Pediatr Radiol (1994) 24:437-471

Pediatric Radiology 9 Springer-Verlag 1994

European Society of Pediatric Radiology, 31st Congress Palais des Congr~s, Brussels/Belgium, June 1-3, 1994

Abstracts of Oral Presentations and Poster Presentations Edited by P. G. Small, Nottingham

438

Oral Presentations Familial Visceral Myopathy - the Role for Radiology

Mohammed-S-H, Freeman-N, Nayar-R Green-R, Devi-L Department of Radiology, Royal Hospital, Muscat Sultanate of Oman Familial visceral myopathy (FVM) is one of the primary causes of chronic intestinal pseudo-obstruction (CIPO). It is a rare genetic disease characterised by degeneration and fibrous replacement of the smooth muscle of the GI tract and sometimes of the urinary tract. It causes defective intestinal motility. The usual clinical diagnosis is intestinal obstruction, but laparotomies fail to demonstrate mechanical obstruction. The definitive diagnosis is histopathological, which requires special techniques, based on full thickness biopsy. It should be the last investigation to resort to, as it is notoriously associated with peritoneal soiling in this disease. Radiologists should be aware of FVM and direct early attention to it, when confronted with young patients with long standing, recurrent complaints of abdominal distension and pain, nausea, vomiting, diarrhoea and/ or constipation. Correct radiological and manometric studies can help to reach a correct differential diagnosis of the CIPO syndromes to permit early, relevant therapy; especially with the newer gastro-intestinal motility drugs, like cisapride. This communication is based on our experience with 6 patients, age range 8-15 y, 4 of whom are siblings and 3 have histologically proven diagnosis of FVM. Its main purpose is to emphasize the radiological manifestations of FVM and the importance of radiology in diagnosis and management. The main radiological features of FVM as observed in our material and from a review of the literature are (i) non-specific abdominal distension, coprostasis, sometimes air-fluid levels in the bowel (ii) normal to mildly dilated oesophagus with delayed clearance, occasionally with gastro-esophageal reflux and oesophagitis, (iii) normal stomach, rarely with delayed emptying (iv) megaduodenum, with poor peristalsis and delayed emptying (v) dilated, hypoperistaltic small bowel, with up to several days delayed emptying (vi) dilated, redundant, occasionally dehaustrated colon, with faecal impaction (vii) US demonstrates a thin intestinal wall and poor peristalsis. Sometimes a dilated gallbladder and urinary tract is observed (the latter can also be seen on IVU). Complications, following surgery, will be illustrated with 2 cases. The role of radiology is to suggest the possibility of FVM, in order to initiate manometric and special histological examinations, restrain unnecessary surgery and to start medical therapy for the individual patient and genetic counselling for the involved families.

Autosomai Recessive Osteopetrosis With Renal Tubular Acidosis and Intracranial Calcification

Rismar-J, Cumming-W-A, Larsson-S-G, Mc-Donald-R King Faisal Specialist Hospital and Research Centre Riyadh, Kingdom of Saudi Arabia The purpose of this presentation is to illustrate the imaging findings in the syndrome of autosomal recessive osteopetrosis associated with renal tubular acidosis, intracranial calcification and carbonic anhydrase 11 deficiency. To do this a retrospective review was made of the medical records and imaging studies made in 24 patients with this disorder. These children, who have a characteristic facial appearance showed delayed growth retarded bone age and variable mental retardation. Anemia was rare but visual impairment was not uncommon. The osteopetrosis which is generally milder than in other autosomal recessive forms predisposes to fracture. There is a characteristic pattern of intracranial calcification evolving during

early childhood involving the basal ganglia and the greywhite matter junction in the hemispheres. We conclude that the presence of this syndrome should be considered particularly in those children with less severe forms of autosomal recessive osteopetrosis who show delayed growth and mental retardation, and beyond early childhood the pattern of intracranial calcification is characteristic.

Hyperplastic Callus Formation in Osteogenesis Imperfecta, Plain Film, CT and MRI Correlation in Two Patients

Kramer-P-P-G, Keessen-W, Beek-F-J-A, Pruys-J-E-H, University Hospital For Children and Youth, Utrecht, Netherland Hyperplastic callus formation in Osteogenesis Imperfecta (OI) is an unusual but well-recognized entity. In a population of 70 patients we only encountered it in 2 cases. Probably the incidence of osteosarcoma in OI is no different from the normal population, but the similarity between the clinical and radiological appearances of these two diseases requires extreme caution. Hyperplastic callus formation is not expected to occur in all fractures or interventions in subjects with OI. Since it runs in the family of our second patient, the liability to develop hyperplastic callus is probably caused by a specific genetic defect or mutation. There are very few articles about it in the radiological literature, but it is well-documented in the orthopaedic literature. We present the plain films, CT and MR images of two patients who both have had many fractures. The first one developed hyperplastic callus formation of the pelvis and the other one in the femora on both sides. The family history of the second patient revealed that his father has had several courses of irradiation for hyperplastic callus formation and in his aunt similar symptoms had led to biopsy and amputation. The clinical symptoms in hyperplastic callus formation are indistinguishable from those occurring in osteosarcoma. Biopsy can differentiate between malignancy and hyperplastic callus. The clinical symptoms will disappear following the administration of indomethacin although the local swelling will remain. The use of Gadolinium in MRI does not give more information or differentiation.

Cherubism - Report of A Familial Case

Diard-F, Chateil-J-F, Pinsolle-J, Rivel-J, Vuillecard-E, Service de Radiologie, H6pital Pellegrin, Bordeaux, France A black twelve year-old boy, from Gabon came to medical attention with a two year history of a slowly increasing painless, symmetrical swelling of the mandible unattended by any other symptoms. The initial onset was followed by a period of minimal growth. However, a rapid expansion had occurred during the last six months, with asymmetrical development, malposition and loss of the teeth with sub-mandibular and cervical lymphadenopathy, causing progressive and very severe deformation of the lower face. X-rays showed multiple destructive lesions with well demarcated outlines, involving the whole mandible, sparing the condylar processes and the maxilla, associated with agenesis, malposition or "floating" teeth. A diagnosis of Cherubism was suggested. CT with axial and coronal views confirmed the multiple expansive, lytic lesions of the mandible, asymmetrical development of permeative nodules into the gingivo-buccal sulci and the external soft tissues of the jaw. The child underwent surgery with a conservative remodelling curettage via an endobuccal approach. The aesthetic and functional results were excellent. Pathologic examina-

439 tion of the specimen confirmed the results of the biopsy. His mother had also been operated on for similar lesions at the age of fourteen, with a good longlasting result. One threeyear-old sister out of two brothers and three sisters presented with a slight swelling of the mandible. This involvement of three members of the same family clearly illustrates the autosomal dominant inheritance of the disease. Clinical radiological, and pathological documentation is presented allowing the description and the discussion of the principal differential diagnosis of this well known but very rare disease.

Double Heterozygous Form of Achondroplasia and Spondylo-Epiphyseal Dysplasia Congenita (SEDC)

lage plate of the physis. Streptococcus Beta hemolytic (group B) infection produces larger quantities of fluid in the joint with a subtle synovial hypertrophy. Osseous lesions are localized in the trabecular bone of the metaphysis (secondary spongiosa). The initial US allows the choice of a specific treatment. US is used for follow-up, reducing the number of X-rays, controls the therapeutic effects, rapidly detects complications, and looks at the integrity of the cartilaginous epiphyseal rim. X-ray is still useful and necessary for the acute period and during the late follow-up. Conclusion. US helps the physician to make a rapid diagnosis and allows a close survey without radiation. The prognostic value of color Doppler findings in the early phase has yet to be evaluated for synovial and osseous epiphyso-metaphyseal vascularisation.

Fliegel-C-P, Gunthard-J, Buhler-E, Ohnacker-H, RutishauserM, Children's Hospital Basel, Romergasse 8, Ch-4005 Basel, Switzerland

Role of MRI in the Acute Phase of Salter Harris IV Fracture of the Lower Extremity of the Tibia

A rare case of double heterozygosity for achondroplasia and SEDC is presented. The expected early death in the neonatal period did not occur and the patient survived for more than twelve months suffering from pulmonary hypertension and respiratory failure. The diagnosis was suspected because her mother suffered from SEDC and her father from achondroplasia. The clinical and radiographic features were compatible with both dysplasias although some of the characteristic radiographic findings were remarkably different from the heterozygous forms of achondroplasia and SEDC. Compatible with SEDC were retarded bone age, flattening and dorsal wedging of vertebral bodies, short tubular bones and a hypoplastic odontoid process. Compatible with achondroplasia were large skull, prominent forehead, short vertebral pedicles, the pelvic configuration, short tubular bones with flaring ends, trident hands with short phalanges and metacarpals. Two important skeletal findings were missing: narrowing of interpedicular distances of lumbar spine and the characteristic configuration of the proximal femora. Additional findings were palatoschisis and an abnormal type II collagen on histological examination. The case is presented as one of the rare occurrences of a combination of two skeletal dysplasias (achondroplasia and SEDC). Clinical radiographic and pathological features will be demonstrated.

Significance of Ultrasonic Findings in Septic Osteoarthritis of the Hip in Neonate

Baud-C, Ferran-J-L, Couture-A, Veyrac-C, Department of Pediatric Radiology, Arnaud de Villeneuve Hospital, 34295 Montpellier Cedex 5, France Purpose: To determine the usefulness of high frequency sonography (US) in the early phase of the septic hip in newborn, for diagnosis and follow-up. Material and Methods: In 8 years, 16 cases of neonatal septic hip have been examined by US. Using a high frequency probe US is performed during the period of invasion and the close following time after the first symptoms. The results were compared and correlated with the clinical, radiological and biological findings. Results. In all of the cases presented, US easily showed the lesion of the joint. The first examination was diagnostic in 14/16. The two other patients had a simple osteitis. US is specially accurate when the pain is slight or masked by other localisations or by a more diffuse septic disease. The sensitivity of US is higher than X-ray for the detection of small amounts of fluid within the joint, particularly in cases of bilateral lesions or if the quality of radiography is poor. There is a relationship between the type of the causal infectious agent and ultrasonic findings. Iatrogenic factors are a more common etiology. In case of infection by Staphylococcus, synovial fluid is sparse and associated with a variable thickening of the synovial layer. The osseous locations are predominantly found in the primary spongiosa near the carti-

Petit-P, Panuel-M, Jouve-J-L, Kervella-H, Faure-F BourliereNajean-B, Devred-R Service de Radiologie Pediatrique, C H U Timone Enfant, 13385 Cedex S Marseille, France MRI has been recently used to evaluate physeal injury after fracture. From January 93 to January 94, of the 21 patients referred to our institution for fracture of the lower extremity of the tibia, 8 patients were radiographically considered to present a Salter Harris (SH) IV fracture These patients were examined by MRI within the first week after trauma. All exams were performed using a 1 5 T magnet with gradient - echo sequences in sagittal and coronal planes. MRI was performed to assess its ability (1) to define the precise direction of the fracture, (2) to determine the size and number of fragments, and (3) to assess the benefit of MRI versus plain film in term of diagnosis and immediate therapeutic management. Our results showed that the fracture and the fractured fragments were always better seen with MRI. However, in no case did MRI modify the surgical treatment planned. In our work in progress, MRI has not proved to be useful in term of orthopaedic management in the acute phase of SH IV fracture of the lower extremity of the tibia.

Juvenile Rheumatoid Arthritis MRI and Ultrasound in the Initial Evaluation and Follow-UP After Intraarticular Steroid Injection

Eich-G, Halle-F, Seger-R, Willi-U-V, Division of Radiology, The University Childrens Hospital, Zurich, Switzerland Juvenile rheumatoid arthritis (JRA) is a heterogenous group of diseases characterized by chronic relapsing inflammation of synovia in one or more joints. Disease onset is before the age of 16 years. The disease process often leads to deformity of the joints and invalidity. It was shown to be the most frequent chronic joint disorder of children in developed countries. Therapy at present consists mostly of antiinflammatory drugs, which may be given systemically or intraarticularly. Intraarticular steroid therapy in large joints is preferred because of fewer and less severe systemic side effects and potentially higher local efficacy. Imaging may be particularly helpful for optimal titration of the injected drug. The aim of our study was to compare US with MRI in assessing the affected joints before therapy and following intraarticular steroid injection. MRI was considered to be the "gold-standard". Material and Methods: A prospective study was performed on 12 patients with J R A in large joints (hip, knee, tibio-talar joint). Pre-therapeutic assessment included a complete clinical, rheumatological work-up with radiographic documentation, US and MRI of the affected joint(s). Joint fluid was aspirated and analyzed. Following intraarticular steroid injection clinical and US follow-up examinations at I w and i m were performed. Later, if clinically indi-

440

cated, MRI was performed after i m to monitor treatment response. Results. MRI and US could demonstrate pannus formation and effusion, but differentiation was less distinct by US. Baker cysts and popliteal lymph nodes were visible both on MRI and US. MRI in addition could demonstrate articular cartilage, allowing the assessment of cartilage erosion, which cannot reliably be assessed by US. Bone erosions and marrow edema were not present in our patients. Epiphyseal overgrowth was best assessed by plain radiography. Conclusion. MRI at present is the best toot to assess the morphologic abnormalities of the joints in JRA. Initial staging of the joints may be done with plain films and MRI. US is a useful tool to assess effusion and pannus formation and therefore may be used to monitor treatment response following intraarticular steroid injection.

Imaging of Sacroiliitis in Children

Tschappeler-H, Department of Diagnostic Radiology, Pediatric Radiology, University Hospital, Inselspital, Ch-3010 Berne, Switzerland Inflammatory disease of the pediatric sacroiliac joint poses an important indication for imaging and diagnosis, as rapid treatment is essential. Twelve children aged from 1 to 15 years, 8 girls and 4 boys form the basis of this retrospective study, which was undertaken in order to evaluate the optimal imaging method. Nine patients had infectious, 3 non-infectious sacroiliitis; one boy had bilateral disease, otherwise the side-distribution was almost homogeneous (6 right, 5 left side). For primary diagnosis the following methods were used: conventional X-ray examination (8), bone scintigraphy (10); CT (3); MRI (6). One single modality was applied in 2 children, 2 different techniques were used in 6, 4 patients had an initial work-up by 3 or 4 methods. In bacterial sacroiliitis all 5 initial conventional studies were normal or equivocal; 7 of 9 scintigraphies were abnormal but unspecific, whereas all 3 CT and all 4 MRI-examinations were pathological. In non-infectious arthritis all 3 conventional examinations were abnormal, the only scintigraphy performed was normal, both MRI studies were abnormal, Conclusion: Early and prompt diagnosis of infectious sacroiliitis is best established by CTor MRI. Both methods allow delineation of jointspace pathology, involvement of neighbouring osseous structures and extension into the soft tissue is readily demonstrated. As MRI is less invasive it should be the method of choice.

Extension of Early Hematogenous Osteomyelitis in Children, Evaluation With MRI

Ka&er-S, Jorulf-H, Department of Pediatric Radiology, St Goran Childrens Hospital, Stockholm, Sweden The treatment of acute hematogenous osteomyelitis includes both long-term antibiotics and removal of pus and debris by surgery. Before curettage, an exact delineation of the infected space is required. Several studies in adults have shown that MRI is superior to other modalities in evaluation of musculoskeletal infection. We studied twelve pediatric patients, age 511 years, with clinical suspicion of osteomyelitis. They were investigated with plain X-ray, US, bone scintigraphy and MRI. The limited value of conventional radiography in the early stage of osteomyelitis is well known. US proved to be a useful diagnostic instrument in detecting subperiosteal abscesses only. Bone scintigraphy seemed to underestimate the extension of an osteomyelitic lesion in the metaphysis. In four cases, MRI also demonstrated epiphyseal involvement not suggested by bone scintigraphy. In two patients, a later follow-up, demonstrated remaining signal abnormalities in spite of absence of clinical symptoms. Conclusion: MRI seems to give valuable information about the extent of an infectious process and is useful for mapping before biopsy procedure and surgical drainage.

Stroke in HIV Infected Children: Radiologic Findings and Clinical Correlations

Sebag-G, Philippet-R Blanche-S, Rodesch-G Griscelli-S, Tardieu-M, H6pital Des Enfants-Malades, Paris and H6pital Bicetre Le Kremlin Bicetre, France Purpose: To describe the radiologic features in 4 HIV-infected children with acute hemiplegia and underline differences in etiology and prognosis despite a similar initial clinical presentation. Materials and Methods: In a 10 years period, four of 380 HIV-infected children followed in our institution had a stroke. All these children were severely immunodepressed. Neuroradiologic studies included CT, MR imaging, MR angiography and digitalized subtraction angiography. Results: In two cases, aneurysmal dilation of major cerebral arteries, thrombosis of small cortical vessels and infarcts with a watershed distribution were found. Both children had a history of frequent infections and had suffered repeated neurologic events ending in severe clinical deterioration and death. An infectious causative agent of cerebral arteritis was strongly suspected but not detected. The other two patients had a more favourable outcome. An isolated sylvian artery thrombosis was found in one, while in the other, HIV-related focal necrosis was shown on brain biopsy. Conclusion: Since the survival of HIV-infected children has improved through better management, these neurologic accidents could become more frequent. Signs of cerebral arteritis should be ruled out using imaging. The poor prognosis of this complication warrants thorough tests to distinguish it from the other causes of strokes which seem to have a better outcome.

Steroid Responsive Encephalitis: The "Pseudo-Leigh" Pattern

Dacher-J-N, Cohen-P-A, Adamsbaum-C, Le-Dosseur-R Kalifa-G, Department of Pediatric Radiology, H6pital Charles Nicolle, Rouen and Department of Radiology, H6pital St Vincent de Paul, Paris, France The aim of the study was to report the cases of 6 patients with steroid responsive encephalitis presenting multifocal and grossly symmetric high-signal abnormalities on T2-weighted images mainly involving basal ganglia and the brainstem. Materials and Methods: Medical records and imaging studies of the 6 patients aged 6 months to 8 years were retrospectively reviewed. Results: Alteration of consciousness and dystonia were found in all patients. They otherwise showed multiple and variable neurological symptoms. A recent history of infection was found in 5 cases. In all of them, MRI studies showed high-signal intensities on T2-weighted images predominating in the caudate lobes, lentiform nuclei and brainstem (the "pseudo-Leigh" pattern). Metabolic, toxic or acute infectious diseases were ruled out by extensive investigations. Steroid therapy was initially efficient in all patients and induced a dramatic improvement of the clinical status of 4 severely affected patients. Neurological outcome was excellent in 5 patients with a minimal follow-up time of 9 months, even though the regression of MR anomalies was variable and unpredictable. The child with severe sequelae was the only one to show multiple high-signal loci within basal ganglia on both T1 and T2-weighted images strongly evocative of haemorrhage. Conclusions: Such MR findings in children have been essentially reported in mitochondrial diseases and after toxic exposures. However, the differential diagnosis of acute demyelinating encephalomyelitis should be considered as stereoid therapy can be spectacularly efficient. Physiopathological hypotheses will be considered. Prognosis seems to be good except when evidence of haemorrhage is found.

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Brain Functional MR Imaging in Children. A Study of Language Localization in Patients With Complex Partial Seizures

Hertz-Pannier-L, Gaillard-W-D, Mott-S, Weinstein-S, Conry-J, Bookheimer-S, Cuenod-C-A, Kolodgie-M, Theodore-W-H, Le-Bihan-D, Radiology Dept, Clinical Center, National Institutes of Health, Bethesda, Md, U S A Introduction: Language localization is crucial in the surgical evaluation of patients with intractable partial epilepsy if language is to be spared during lobectomy. Current means of establishing hemispheric language dominance (WADA) and cortical localization (corticography) are invasive, and risky, especially in pediatric patients. The ability to lateralize and localize language function noninvasively is of great interest. Functional MRI (FMRI) in adults identifies cortex activated during sensorimotor and cognitive tasks, by detecting local variations of blood flow and oxygenation, but has not been studied in children. Objectives: To establish the feasibility and safety of FMRI in children by studying language activation in a pediatric cohort with complex partial seizures (CPS). Methods: 5 right handed children (2 females and 3 males), mean age 10.7 years (range 9. 1-13.2), with onset of CPS at mean age 4.9 years (range 1.0-8.5), were studied with EEG, MRI, and FMRI. Three had a temporal lobe focus (2 right, i left); 2 had an indeterminate focus. Structural 1.5 T MRI was normal in 2, showed mesial temporal sclerosis in 2, (i right, 1 left), and a right temporal cystic mass in 1. Two right handed adult normal volunteers were also studied. FMRI was performed by acquiring images continuously during a paradigm alternating 2 activation periods (44 seconds each) with rest periods. During activation, the subjects were asked to silently generate words beginning by a given letter, as fast as possible. Two surface coils were placed symmetrically on the anterior part of the head. Coronal images were acquired on the frontal lobe, using echo-planar imaging (EPI) with TR=4s, TE=40 ms, flip angle:90 degrees, FOV=16 cm, Matrix= 64 x 64, slice thickness=5 ram. Data analysis screened for regions exhibiting a significant difference in signal between rest and activation (correlation > 0.5 and Z score >1.5). Results: In the 2 adults coronal acquisition reproduced results of previous studies performed with sagittal slices and demonstrated activation of inferior and anterior frontal regions predominantly on the left side (Broca's area), associated with bilateral activation of the premotor cortex. One child study showed predominantly left language activation, two demonstrated bilateral inferior frontal activation. All three had diffuse activation of cingulate and premotor areas. Two studies were obscured by motion artifact. All children complied with the task and remained still for the study (75 minutes) without ill effect. Discussion: Coronal acquisition may be superior to sagittal slices for side to side comparison and for the assessment of language lateralization. The prominence of diffuse activation may reflect a general alerting response to the task among children, or reorganization of language cortex as a consequence of intractable seizures Movement is a particularly significant issue in pediatric FMRI studies, and might be resolved by reregistration algorithms. FMRI is a safe and well tolerated technique in children and is a promising tool for studying brain organization and development in children.

Evolving Pituitary Hormone Deficiency is Associated With Pituitary Vasculopathy. Snap-Shot MR Dynamic Study in Children With Hypopituitarism, Diabetes Insipidus and Langerhans Cell Histiocytosis

Beluffi-G, Maghnie-M, Genovese-E, Arico-M, Villa-A, Calcante-S, Campani-R, Severi-F, Department of Pediatric Radiology, University of Pavia, Irccs Policlinico S Matteo, Italy

Snap-shot dynamic MRI allows measurement of time to enhancement of the posterior and anterior pituitary lobes. By this technique, the vascular supply of the pituitary gland can be explored. Twenty-one patients (11 M and 10 F, age 417 years) were evaluated 8 with biopsy-proven Langerhans cell histiocytosis of whom 3 had diabetes insipidus (DI), 3 had "idiopathic" DI, and 10 had isolated GH deficiency. Posterior pituitary hyperintensity was absent in 5/6 patients with DI. Controls were 10 age-matched subjects. Enhancement of the cerebral vessels of the posterior pituitary, and of the entire anterior pituitary occurred within 7.4-18.5 sec, 7.4-18.5 sec, and 18.4-25.9 sec respectively in all but 4 patients with central DI (4/4) and thick stalk (3/4). These patients differed from all other subjects, initial posterior pituitary enhancement occurred, not simultaneously with that of the cerebral vessels, but only after 4.5-9.2 sec delay (p < 0 005). Enhancement of the entire anterior pituitary occurred within 18.4-25.9 sec in all subjects while it was significantly prolonged (32.2-64.4 sec; p < 0 005) in the 4 patients with delayed posterior pituitary enhancement. These patients showed progressive pituitary volume and function deterioration in the follow-up. The times to enhancement of the posterior and anterior pituitary were significantly correlated (p < 0 0001). Snap-shot technique provides new information on the blood supply of the hypothalamic pituitary area. Enhancement time exceeding 20 sec for the posterior pituitary and 30 sec for the anterior pituitary suggests abnormal vascularization in the hypophyseal arteries and portal system. Pituitary vasculopathy characterizes a subset of patients with central DI, even without stalk granulomatosis, who are at high risk of developing multiple hormone deficiency "Idiopathic" isolated. GH-deficient patients with small anterior pituitary have normal pituitary blood supply. In cases of central DI and/ or isolated GH deficiency, this is an advance towards discriminating between patients with different prognoses.

The Etiological Diagnosis of True Cushing's Disease in Children. A Challenge for the Radiologist

Kalifa-G, Adamsbaum-C, Carel-J-C, AndrE-C, Oppenheim-C Bougneres-P-F, Chaussain-J-L, Department of Radiology, Saint Vincent de Paul Hospital, Paris, France True Cushing's disease in children represents 2/3 of the incidence of Cushing syndromes. It may be life threatening. In children, in addition it bears a major risk for normal growth and puberty. The diagnosis must be made as early as possible. True Cushing's disease is defined by hypercortisolism secondary to excessive pituitary A C T H secretion. The diagnosis is based upon the Dexamethasone suppression test or the Metapyrone test. According to the above criteria, seven patients have been selected (age 9 to 15 y) (sex-ratio: 4 boys, 3 girls). We wish to present the results of pituitary MRI investigations, performed in order to disclose a secreting lesion (adenoma). T1 and T2 sequences were obtained, completed by dynamic studies after contrast injection. The MRI diagnosis was correlated with surgical findings and the pathological data. Three adenomas were found on MRI and confirmed at surgery and pathology. Two other adenomas were suggested, one turned out to be a small incidental non-secreting, cystic lesion, the other one was not found at surgery, or by pathological examination. In both cases, symptoms persisted after surgery and the children had to be treated with OP'DDD. One patient had a normal MRI, surgery was normal but his status improved after partial resection of the pituitary gland. No hyperplasia or adenoma could be identified. In the last case, MRI was normal, no surgery has been performed and the child is under chemotherapy ( O P ' D D D ) If we compare the results of this small series to the literature, the success rate may be considered as slightly disappointing. A technical problem is not likely as the method is similar to the one used in adults. Two other explanations

442 can be proposed. The time interval between clinical onset and MRI examination is probably shorter in children than in adults, as attention is paid earlier to obesity for instance. The other possibility is that excessive A C T H pituitary secretion is related to diffuse hyperplasia and not to a localized secreting tumor. Larger series are required to confirm these hypotheses. In conclusion, radiologists should be aware of the difficulties in identifying A C T H secreting lesions of the pituitary gland in children presenting with true Cushing's disease.

Vermian Agenesis Without A Posterior Fossa Cyst

Adamsbaum-C, Moreau-V, Lair-Milan-F, Bulteau-C, LalandeG, Kalifa-G, Department of Radiology, Saint Vincent de Paul Hospital, Paris, France MRI now allows us to distinguish between several types of cerebellar malformations (CM) besides true atrophy: CM associated with posterior fossa cyst (Dandy Walker syndrome and variant) and CM without a cyst. In this group, the vermis and/ or the hemispheres can be involved. We wish in this paper to report 8 cases of isolated vermian agenesis without any posterior fossa cyst or hemispheric abnormalities. In all these patients, MRI disclosed absence of the inferior vermis, hypoplasia of the superior vermis, a narrow sagittal cleft separating the hemispheres ("buttock" sign) and 4th ventricle deformity. The clinical presentation (5 days to 8-year-old patients) appeared as follows: ocular motor abnormalities, (8 cases), developmental delay, (5 cases), dysmorphic abnormalities, (5 cases), congenital retinal dystrophy, (3 cases), respiratory difficulties, (1 case) Only one patient had notable supra-tentorial lesions (gyration disorder). Consanguinity was often found (4 cases). A relationship between Joubert syndrome and this entity has to be considered, as well as neurophysiological correlation with vermian lobules defects. Embryology of the cerebellum is considered for a better understanding of these congenital abnormalities. In summary, vermian malformations probably represent a specific developmental abnormality. They must be recognized in different clinical circumstances particularly in cases with ocular motor disturbance.

From 1988 to 1992, 46 patients aged 2 w to 18 y, (mean age 4 7 y, 25 F : 21 M), were examined by US through the closed eyelid for eye abnormalities with an Acuson 128 using a 7MHz linear transducer. Forty-five eyes were abnormal and 47 eyes were normal confirmed clinically, with CT or at operation. A wide variety of abnormalities were visualized including cataracts (20), retinal detachment (11), vitreous hemorrhage (15), small anterior chamber (5), microphthalmia (4), retrolental fibrodysplasia (4), posterior lenticonus (2), tumors (5), and persistent hyperplastic primary vitreous (1). Vitreous echoes or strands were present in 15. Multiple abnormalities were present in 16 eyes (14 7 %). Each abnormality had a specific spectrum of appearance with cataracts showing either rim hyperechogenicity or complete hyperechogenicity of the lens. The appearance of retinal detachment varied according to the severity. Chronic complete retinal detachment showed funnelshaped, broad, linear, posterior chamber strands anchored at the optic nerve root. The size of the globes, chambers and lens could be readily measured and compared with the contralateral side aiding diagnosis of abnormal size of all or part of the globe. A tiny anterior chamber was present in one child with glaucoma. Vitreous hemorrhage showed as variable intensity low level echoes. Retrolental fibroplasia showed dense hyperechoic material filling the posterior chamber. Primary persistent hyperplastic vitreous (failure of regression of the primary vitreous, tunica vasculosa lentis) showed as a thick strand extending between the posterior surface of the lens and the area of the optic nerve head. Retinoblastomas, hemangiomas, and lymphangiomas appeared of uniform soft tissue echogenicity. Localization was helpful in aiding diagnosis. Doppler flow patterns were non specific except in one hemangioma clinically suspected of being an encephalocele. One other hemangioma did not show any increase in flow on Doppler study. In all cases US was quick and easy to perform without sedation and gave more information than physical examination alone, especially as the appearance was often specific. The spectrum of normal and abnormal appearance will be demonstrated.

Radiological Evaluation of Biliary Complications Following Liver Transplantation in Children

Prognostic Value of MRI in Unilateral Moderate Ventricular Dilatation of the Fetal Brain

Griffith-J, John-P-R, Department of Radiology, Childrens Hospital, Birmingham, UK

Elmaleh-A, Sonigo-P, Kahn-A-P, Baraton-J, H6pital des Enfants-Malades, Paris, France

Purpose: Biliary complications occur more frequently in children than in adults following liver transplantation. Clinical symptomatology is often non-specific and diagnostic imaging plays an important role. The aim of this study was to review our experience of imaging in children with biliary complications following liver transplantation in a major British transplant centre. Methods: 138 consecutive liver transplants in 112 children were retrospectively reviewed. The relevant imaging studies, clinical notes and operation details were analysed. Sixty (43 %) of the grafts were whole livers, 77 (55.6 %) were reduced livers and one (0 7 %) was a split liver. Four types of biliary anastomoses were utilised, namely choledochojejunostomy (59%) choledochodochostomy (32 6%) cholecystodochostomy (4 3 %) and cholecystoieiunostomy (3 6 %). Results: Biliary complications occurred in 36 grafts (26 %) in 34 patients. (30 4 %). Bile leakage occurred in 17 grafts (12 3 %) and typically presented in the early post-operative period (median 10 days). Over half of these leaks occurred at the site of anastomosis. Ultrasound was confirmatory in the vast majority of cases and cholangiography, when performed (5) accurately localised the site of leakage. Concomitant biliary stenoses were present in two cases. Isolated biliary obstruction or duct dilatation was present in 16 grafts.(11 6 %) and may present either early < 2 weeks (9) or late > two weeks (7). Ultrasound and/or cholangiographic abnormalities were present in all cases. Obstruction was due to anastomotic strictures (6), non anastomot-

Brunelle-E

The neurologic prognosis of unilateral moderate dilatation of the cerebral ventricular system in the fetus is not known. However, it is common practice to follow-up the pregnancy if the dilatation is below 11 mm and in the absence of chromosomal abnormality and with a negative infection work-up. We reviewed 11 cases of mild unilateral ventricular dilatation and correlated the MRI findings with the neurologic out-come. Material and Methods: Eleven fetuses aged from 21 to 32 weeks of amenorrhea were studied by antenatal MRI. Unilateral moderate ventricular dilatation was diagnosed in all (greater than 7 ram). Postnatal neurologic assessment was obtained in all but 4 patients. Results: Five patients are well and neurologically normal. Four patient are lost to follow-up. Two patients have hemiplegia and the MRI showed associated findings suggesting hemorrhage. Conclusion: Fetal cerebral MRI should be performed in patients with mild unilateral ventricular dilatation. Indeed associated hemorrhage is of poor prognosis.

The Sonographic Spectrum of Pediatric Ocular Disease

Stringer-D-A, Lewis-R Phillips-R-R, Poskitt-K-J, NewmanD-E, BC Childrens Hospital, Vancouver, British Columbia, Canada

443 ic strictures (5) and intraluminal material (2). Three patients had incidental biliary dilatation (i. e. in the absence of clinical symptoms or biochemical cholestasis). Conclusion: Diagnostic imaging modalities have a very important role to play in the post-operative surveillance of paediatric liver transplants, not least in the diagnosis of biliary tract complications.

found between the US appearances and other indices of metabolic control. Seven patients were found to have hepatic tumours, 6 with GSD-I and one with GSD-III. Those with GSD-I and tumours tended to have the more severe hepatic parenchyreal changes. We conclude that US may be useful in identifying patients with GSD-I at risk of hepatic tumour formation.

Mucocele of the Cystic Duct Remnant After Liver Transplantation. A Series of 4 Children

Architectural Changes of the Liver in Tyrosinemia. Radiologic-Pathologic Correlations

Pariente-D, Mignon-F, Urvoas-E,

Dubois-J, Garel-L, Patriquin-H, Patenaude-Y, Paradis-K For-

Riou-J-Y, H6pital de Bicetre, Le Kremlin Bicetre, France

Husson-B,

Biliary complications are frequent after liver transplantation (LT) in children, accounting for 20 % in our series of more than 300 LT performed over the past 8 years. They are mainly secondary to hepatic artery thrombosis or anastomotic stricture. Mucocele of the cystic duct remnant is a rare cause of extrinsic compression of the common bile duct (CBD) by the blind mucosa-lined allograft cystic duct. The clinical and radiological findings of 4 cases are described. Our patients, 2 girls and 2 boys, aged from 3 to 15 ys were transplanted with a reduced-sized liver in 2 and a choledochocholedochostomy in 2. They were explored by means of Doppler ultrasound (US) in all cases, percutaneous transhepatic cholangiography in 2 and preoperative cholangiography in 1. Biological and US findings of biliary obstruction were often delayed occurring from 2 to 9 months after LT. Two of our cases required a surgical resection of a biliary anastomotic stricture. In 3 cases the diagnosis was only made at surgery, but in one it was suggested on US and confirmed by percutaneous puncture of the tension mucocele. In 2 cases percutaneous balloon dilatation was attempted with immediate and complete inflation of the balloon, but with persistance of the obstruction on a later control examination. In conclusion we would like to stress the following points. Findings of a round fluid collection impinging the CBD on US and/or extrinsic compression on the antero-posterior or lateral films of cholangiography should suggest this diagnosis. No attempt at interventional radiology should be made, as the treatment is surgical.

Hepatic Ultrasound Findings in the Glycogen Storage Diseases

Lee-P, Mather-S, Owens-C, Leonard-J, Dicks-Mireaux-C, The Hospital For Sick Children, Great Ormond Street, London, UK The glycogen storage diseases (GSDs) are a group of inherited disorders of a number of enzymes controlling glucose production from glycogen in the liver. The clinical picture varies considerably, the most common being hepatomegaly and growth delay. Secondary biochemical consequences include fasting hypoglycaemia, hyperlipidaemia and hyperuricaemia accompanied histologically by excess glycogen and fat deposition within the liver. In recent years the introduction of effective dietary therapy has greatly improved prognosis, although without entirely correcting the metabolic derangements. Ultrasound has been demonstrated to be of value in a number of conditions affecting the liver, in particular steatoses in alcoholic liver disease and malnutrition. There are few reports of it's use in the hepatic GSDs and those that exist have described the appearances of liver tumours. We performed hepatic US to assess parenchymal echogenicity and to evaluate it's role in monitoring the metabolic derangements. We postulated that hepatic US might indicate the effectiveness of therapy and correlate with eventual outcome. Seventy patients with the GSDs were examined, 27 had GSD-I, 24 had GSD-III and 19 had GSDs VI/IX. Thirty-one (45 %) had normal or mild parenchymal changes, 41% of those with GSD-I, 25 % with GSD-III and 11% with GSDs VI/IX had marked changes. No relationships were

get-S, St-Vii-D, Russo-R H6pital Sainte-Justine, Montreal, Quebec, Canada Purpose: To compare the radiological and pathological appearance of the liver of patients transplanted for tyrosinemia. Methods: All patients undergoing liver transplantation between July '85 and August '93 were examined with contrast enhanced CT, US (grey scale and Doppler) and measurements of serum alpha-foetoprotein levels(AFP). At the time of transplantation, the status of the native liver was assessed and correlated with gross and macroscopic sections. A comparison between in vivo and in vitro anatomy was made, with specific reference to the incidence and appearance of space occupying lesions (regeneration nodules and hepatocarcinomas), as characterized by architecture distinct from the surrounding liver, displacement of vessels, and/or presence of neovascularisation visible at Doppler US. Results: There were 22 patients (10/1=, 12/M) aged 5 m to 16 years 8 m, (mean age: 4 y 3m), transplanted for the following reasons: 9 patients for neurological crises with various degrees of liver dysfunction and 13 patients for progressive liver failure with or without presence of nodules. Seven patients had multiple nodules 0 1 to 1 5 cm in diameter, 6 of these were detected prior to transplantation, as hypo or hyperechogenic lesions or high or low attenuation on CT. With the exception of 2 carcinomas and 6 premalignant lesions (cellular dysplasia) all nodules identified in vivo were regeneration nodules. We failed to distinguish carcinomas from benign nodules. No tumor vessels were identified with contrast CT or Doppler US. Conclusion: The cirrhosis which accompanies tyrosinemia is characterized by multiple regeneration nodules. Distinction between such nodules and carcinoma is impossible using CT, grey scale and Doppler US and levels of serum AFE

The Detection of Early Liver Disease in Children With Cystic Fibrosis Using Grey Scale and Colour Doppler Ultrasound

Hollman-A-S, Davies-M-L, Paton-J-Y, Evans-T-J Coutts-J-AR Coutts-N-A, McColl-J, Departments of Radiology and Paediatric Medicine, Royal Hospital For Sick Children, Glasgow, Scotland Introduction: With improvement in survival of patients with cystic fibrosis liver disease with portal hypertension and variceal haemorrhage is now a significant cause of morbidity and death. Clinical and biochemical signs of early hepatic disease are unreliable. If potential treatments are to be evaluated, then non-invasive techniques, such as ultrasonography(US), are required. A prospective study is reported using grey scale and colour Doppler US to detect liver disease in children with cystic fibrosis. Methods: Two groups of children underwent detailed abdominal US assessment using a strict protocol. 104 children with cystic fibrosis were matched with age and sex with 104 normal children (attending for routine renal US with no cardiac, respiratory or liver disease). An independent evaluation was made to assess whether the children with cystic fibrosis had clinical evidence of liver disease or not. Clinical and US data was compared between the two groups. Results: Of

444 104 cystic fibrosis patients (age range 2 m- 15 8 y, mean 7 16 y; M/59:F/45). 14 children had clinical evidence of liver disease, whilst 32 had US evidence of an abnormal liver. The diameter of the portal vein was larger in cystic fibrosis patients than in controls, and larger still with CF with clinical liver disease. The portal vein velocity in well established liver disease was lower with damping of the hepatic venous waveform, but both parameters were often normal in early clinical liver disease. Eleven children had gallstones, but only i had gallstones and liver disease. The gall-bladder wall is thickened with liver disease but in one child, the coexistence of gallstones made this finding difficult to interpret. All children with CF had larger spleens than the control population, and this difference was more marked in those with liver disease. Conclusion: 1) US demonstrates early liver abnormality in CF patients before clinical detection is possible. 2) Grey scale US alone is adequate to detect early liver disease, but colour Doppler US is useful in the evaluation of severe liver disease with portal hypertension.

Role of Color Vascular Ultrasound in Congenital Hepatic Vein Anomalies in Children

Harkanyl-Z, Tasnadi-G, Pasz!i-I, Bitvai-K, Department of Radiology, Semmelweis Medical University Budapest, Department of Radiology and Surgery, Heim Pal Childrens Hospital, Budapest, Hungary Purpose: Color Doppler imaging (CDI) and recent introduction of color velocity imaging (CVI) can aid the assessment of hepatic vein anomalies in children. Five pediatric cases with different congenital anomalies are analyzed. Method and patients: 2D real-time imaging, color Doppler and duplex Doppler studies were performed on Hitachi EUB 40, ATL UM-9 H D I systems; CVI studies were performed on a Philips P-700 unit. In two cases MRI was done by Siemens Magnetom system. Three boys and two girls were examined several times. The longest follow-up time is 3 y. Age range of our patients is 1-12 y. Results: Final diagnosis was based on clinical presentation and on grey-scale and CDI in all cases. Angiography was performed in one, MRI in two cases and surgery was performed in two cases. Diagnosis: Cases of congenital arterioportal shunt, hepatic web, Budd-Chiari syndrome with portal hypertension, portal cavemoma and persistent ductus venosus were made. Conclusion: CDI and CVI are cost-effective and reliable techniques in the morphological and functional evaluation of different hepatic vein anomalies. Invasive radiological procedures are necessary if therapeutic intervention is planned.

The Ductus Venosus in Premature Infants. A Color and Duplex Doppler Study

infants, because of its narrower width, often less than one mm. Ductus length varied from 0.8 to 1.8 cm. Initial flow velocity ranged from 0.07 to 0.6 m/sec. In all the infants the ductus was patent at the age of 2-4 days. In 69 % of cases it was open at one week, in 44 % at 9-10 days, in 11% at two weeks, and in 3 % of infants the ductus was still open at 18 d and beyond. We present graphic demonstration of ductus closure with age in premature infants, comparing infants of gestational age less than 32 wwith infants of gestational age between 32-36 w. We also compare these results to those in our study of full-term infants. Conclusion: The ductus venosus is consistently detectable and patent in premature infants. It tends to remain open for a longer period of time than in full-term infants.

Diagnostic Difficulties in A Toddler With A Pancreatic Abscess

Lombay-B, Kiss-A, Nyari-E, Monus-A, Ladanyi-E, County Teaching Hospital, Miskolc and County Hospital Eger, Hungary A one and a half year-old-boy with poor growth and development was admitted. Celiac disease or other malabsorbtion syndromes were suspected, but a jejunal biopsy and several biochemical investigations had given normal findings. An abdominal US was performed which revealed a huge cystic lesion (6.5 x 4.5 cm in size).in the middle abdomen which seemed to be mainly in the lesser sac. There was a large amount of echogenic debris within the cyst which was covered by a thin wall. Serum and urine amylase and lipase level was normal by the repeated analyses and there was no sign of acute or chronic pancreatitis, multisystemic and metabolic diseases. There was no trauma in the history. The only positive finding was Streptococcus alfa-hemolyticus cultured from the bile (duodenum fluid sample). CT was performed showing pancreatic swelling and nearly the same findings as the US. There was a moderate contrast enhancement in the rim of the cyst. The lesion was thought to be an infected gastric (enteric) cyst or some other retroperitoneal cyst as the pancreatic origin was not supported clinically. Because of the unclarified origin a percutaneous drainage was opposed and surgery was suggested. At surgery a huge pancreatic abscess was revealed and operated on by the Jurasz's technique making an anastomosis between the cyst and the stomach. The boy recovered completely. Conclusion: In infancy and in toddlers pancreatitis is very rare and in 25 % of the cases neither the history nor the clinical and laboratory data led to the correct diagnosis. In our case the US and CT revealed the lesion which was caused by a streptococcal infection in the pancreas. This fact suggested any anomaly of the pancreas which has not yet been clarified in our young patient.

Herskovitz-M, Loberant-N, Barak-M, Ben-Elisha-M, Hersch-

Radiologic Findings in 4 Children With Pancreatic Transection Successfully Treated Non-Operatively

kowitz-S, Roguin-N, Western Galilee Regional Hospital, Nahariya, Israel

Lucaya-J, Vazquez-E, Chait-R Wesson-D, Daneman-A, De-

Our objective was to study the anatomy, flow pattern and time of closure of the ductus venosus in premature infants. This is a follow-up to our study in full-term neonates (Amer J Roentgen 1992; 159: 1083-1085). We examined 41 premature infants of gestational age 28-36 weeks, who met the following criteria: normal Apgar score, normal physical examination, no need for supplemental oxygen or blood transfusion at the time of the initial examination. The infants were examined initially at age of 2-4 d, and subsequently every 3 or 4 d until ductus closure was confirmed. Grey scale, color and duplex Doppler US were performed each time. The length and width of the ductus, and the flow pattern were recorded for each exam. Results: The ductus was in general less apparent in premature infants than it had been in the population of full-term

Distal pancreatic resection has been the treatment of choice for pancreatic transection due to blunt abdominal trauma. This paper presents the clinical and radiographic findings in 4 children with pancreatic transection who were successfully treated non-operatively. Cross-sectional imaging (US and CT) showed the presence of pseudocyst formation in all 4. In 3 of these, a pancreatic fracture was also clearly visualized and in the 4th, an ERCP showed complete disruption of the pancreatic duct. Three patients required pseudocyst drainage and the 4th was managed with nasojejunal feeding. Follow-up sinogram of the pseudocyst drain in one showed complete pancreatic duct disruption as only the duct in the body and tail of

partmento Radiologia Pediatrica, Hospital Universitari Materno-Ifantil Vall D'Hebron, Barcelona, Spain

445 the pancreas was demonstrated by this injection. Follow-up US and CT showed the pancreatic tail and adjacent body to be normal in i and to be decreased in size in 2. No recurrent pseudocysts occurred. Bowel rest is essential (total parental nutrition or elemental diet via a nasojejunal tube) until pancreatic inflammation has subsided. All 4 patients have done well and are now asymptomatic. The imaging procedures enabled accurate visualization of the site of pancreatic transection, duct disruption and presence of pseudocysts at the time of diagnosis or during follow-up and this facilitated appropriate clinical decision making.

Chest CT Findings of Pulmonary CMV Infection in Lung Transplantation Patients

Kervella-H, Faure-F, Bourliere-B, Petit-R Gaubert-J-Y, ViardL, Garbe-L, Badier-M, Camilleri-S, Metras-D, Noirclerc-M, Devred-R CHU Timone Enfants, Marseille, France Purpose: To evaluate chest CT findings of CMV pneumonia (PCMV) in lung transplantation patients. Materials and Methods: From May '88 to March '93, 28 lung transplantations were performed in children (5 to 16 years). The post- operative management included chest CT, scintigraphy, lung function studies, broncho-alveolar lavage and transbronchial biopsies (BTB). Moreover, a CT was performed in case of progressive complications. Twelve children presented 16 cases of PCMV diagnosed by transbronchial biopsies. Chest CT, including HRCT study, were performed in 13 cases at the time of pneumonia and just before BTB. Results were compared with clinical severity. Results: In all patients CT demonstrated diffuse infiltration lung disease which included septal (13), and non septal (9), linear opacities + nodular opacities (10), peribronchial thickening (12), ground glass opacities (8), air space consolidations (12). A correlation was observed between the profusion of lung changes and pneumonia severity. Conclusion: Diffuse infiltrative lung disease is a good indicator of PCMV in this population. CT can locate pathological areas to target for transbronchial biopsies which remains the "gold standard" for PCMV and can reflect the disease severity.

Diaphragmatic Paralysis in Children. Diagnosis by TMMode Ultrasound

Urvoas-E, Fausser-C, Lipsich-J, Taleb-R, Pariente-D, H6pital de Bicetre, Le Kremlin-Bicetre, France Diagnosis of diaphragmatic paralysis is classically made by fluoroscopy or B-mode US. The use of time-motion (TM)mode has been only described in two cases in the literature, and we would like to report our experience of this method of exploration in this difficult diagnosis at the pediatric age. Material and Methods: From July 92 to January 93, 30 patients aged from 1 day to 14 years (average +/- SD=4.2 + 4.5 years) were explored by B-mode and TM-mode ultrasound for suspicion of diaphragmatic paralysis and after plication in one ease. All patients were explored spontaneously breathing and lying supine. Bilateral exploration with a probe of 3.5 or 5 MHz was performed with coronal oblique scans in all cases sagittal and recurrent oblique scans when possible. Direction and amplitude of the diaphragmatic excursion pattern of the transition between inspiration and expiration (sharp and brief or smooth and prolonged) were noted. All patients underwent inspiratory and expiratory chest radiography or fluoroscopy. Results: Examination was considered normal in 7 patients and TM-mode demonstrated movement of normal direction and amplitude, and sharp aspect of the transition between inspiration and expiration. Diaphragmatic paralysis was present in 9 patients and was secondary to phrenic neuropathy (3), thoracic and abdominal surgery (3), catheter puncture (2)

and birth injury (1). It was unilateral in 7 cases (left-sided 4), (right-sided 3) and bilateral in 2 cases. TM-mode demonstrated a paradoxical movement, a reduced amplitude and a prolonged transitional zone with smooth aspect of the TM-tracing. Diaphragm dysfunction was present in the 14 other patients. TM-mode demonstrated a movement of normal direction but with a reduced amplitude of less than 50 % of the controlateral side. TM tracing was smooth in 13 cases (abdominal surgery= 10, neuropathy = 1, myopathy = 1 congenital eventrat i o n = l ) and sharp in 1 case (plication). Conclusion: When compared to B-mode, TM-mode allows to record movements of the diaphragm with a more objective way. It can identify direction of the movement even if movements are fast and of weak amplitude and thus can differentiate paralysis from dysfunction. Reproducible quantification is useful in follow-up to monitor stability or recovery of diaphragmatic function. Moreover, this low-cost non irradiating imaging can be performed at bedside and is available on all basic devices.

Portable Chest Imaging. A Comparison of Storage Phosphor and Screen Film Systems. Part 2: Results

Ducou-Le-Pointe-H, Montagne-J-P, Maccia-C. Fery-Lemonnier-E, Service de Radiologie, H6pital D'Enfants ArmandTrousseau, 75012 Paris, Caats, 94230 Cachan Cedit Assistance Publique-Hopitaux de Paris, 75100 Paris, France Purpose of the study: To assess the possibility of reducing patient exposure with an X-ray quality equal to conventional radiography. Methods: 71 patients had 4 consecutive chest Xrays. The first one with a screen film combination speed of 400. The following three were done in a random order with a Fuji storage phosphor system, one with same kV and mAs, an other with same kV and mAs -30 %, the last one with same kV and mAs -60 %. The X-rays were randomly read on the same viewing box by 3 radiologists. A score was given according to quality criteria defined by the Lake Starnberg Group. Results: Dosimetry, mean values: Conventional X-ray, 146 micro Gy +/- 127 Storage phosphor.(SP) same parameters 135 ,micro Gy +/- 92 SP same kV, mAs.-30 % 99 micro Gy +/- 111 SP same kV, mAs -60 % 73 micro Gy +/-67 Quality criteria. Conventional X-ray is never statistically significantly better than storage phosphor images. Conventional X-ray is considered worse than SP by the junior radiologist, SP -60 % is considered worse than conventional X-ray, SP 100% and SP -30 % by the two senior radiologists. No differences are detected between SP -30 % and SP 100 %. Conclusion: A potential reduction of exposure is offered by the storage phosphor system. The inter reader variability is confusing. It may be due to a better acceptance of digital images by a young radiologist than by older ones trained on conventional radiology. Further studies based on diagnostic efficacy need to be conducted.

Digital Chest Radiography of Newborns. Comparison on Diagnostic Accuracy of Hard Copy Film Versus Soft Copy Images

Durand-C, Fran~ois-R Nugues-F, Baudain-R Department of Pediatric Radiology of Grenoble, Ph Baudain, France Purpose: The aim of the study was to compare observer performance of digital hard copy film and interactive soft-copy display in the routine practice of pediatric radiology. Materials and Methods. Thirty-two chest radiographs of newborns obtained on storage phosphor system (FCR ACI+) were displayed on a work station and on laser camera films. Thirteen experienced pediatric radiologists evaluated the two display modalities at different moments: hard copy with two postprocessed images (unenhanced and edge-enhanced images) and

446 soft copy on a workstation. Five abnormalities were selected for analysis: air effusions, air bronchograms, hazy vessels, pulmonary interstitial emphysema, and position of small newborns' catheters. Sensibility and specificity were calculated and Receiver Operating Characteristic (ROC analysis) curves were generated for each abnormality and display format. Results: For the detection of air effusions, air bronchograms interstitial emphysema, no statistically significant differences were found. For hazy vessels, the sensitivity was identical, while the specificity was significantly better (p < 0,001 ) on the hard copy. Finally, for the detection of the position of catheters, observer performance was better on the interactive soft copy display. Conclusion: The efficacy of display of soft copy is not significantly different from that of hard copy. These results lead us to continue to study the feasibility of PACS for the pediatric intensive care unit.

Spiral CT and 3D Reconstruction of Vascular Rings and Associated Tracheobronchial Anomalies

Katz-M, Konen-E, Rozenman-J, Szeinberg-A, Itzchak-Y, She-

tional 0.6 sec scans were reconstructed with a standard algorithm and helical studies with standard and bone algorithms. Three blinded observers evaluated identically presented images for sharpness, motion and visibility of segmental and subsegmental bronchi. Results: There was no statistically significant difference in image sharpness, motion and visibility of bronchi for standard algorithm helical and conventional techniques. However, image sharpness (p < 0.001) and visibility of bronchi (p < 0.001) were greater for bone algorithm helical than for standard algorithm conventional images. Conclusion: Helical CT is preferable to 0.6 sec scanning in the evaluation of children incapable of breath-holding, because bone algorithm can be used to improve image sharpness and anatomic definition.

Spiral CT in Children. Intra-Individual Comparison With Conventional CT

Benz-Bohm-G, Landwehr-P, Doelken-W, Lackner-K, Department of Radiology/Pediatric Radiology, University of Cologne, Germany

ba-C, Medical Center, Tel-Hashomer 52621, Israel Purpose: To present the application of spiral-CT scanning and computerized three-dimensional (3D) color-coded reconstruction of vascular and tracheobronchial congenital anomalies in the pediatric age group. Patients and Methods: Two symptomatic children were referred for spiral CT examination following positive barium esophagram for vascular ring. In one child a complete double arch was demonstrated and the other child had a left aberrant pulmonary artery. Additionally one adult, referred for spiral CT examination for an unrelated condition, was diagnosed, incidentally, with a right aberrant subclavian artery. The examinations were performed by Elscint Twin equipment, capable of performing 1 rotation/sec at 120 KV and 200 mA, with a table speed of 2.0 mm/sec at an interval of 32.1 sec/examination. The slice thickness was 1.1 mm slice increment 0.6 with a real overlapping of 1.5 pitch Contrast material was administered, half by rapid injection.(1.5 ml/sec) and half by continuous injection (0.5 ml/sec) by a power injector. Chloral hydrate was used (60-80 mm/kg) as sedation. Colorcoded 3D reconstructions from spiral-CT data of the area of interest were obtained using an independent Elscint Omni View unit. Results: Color-coded 3D reconstruction of two-dimensional images permit simultaneous visualization of great vessels and associated tracheobronchial anomalies. The relationship between these structures is demonstrated from different orientations providing additional information to standard two-dimensional projections. Conclusion: Spiral-CT and colorcoded 3D reconstructions represent a non-invasive method of examination of vascular rings and associated tracheobronchial anomalies. This examination is an additional or an alternative tool to other invasive or non-invasive techniques in evaluation of vascular anomalies in children.

Comparison of Helical and Coventioaual Chest CT In The Uncooperative Pediatric Patient

Effrnan-E-L, Cox-T-D, White-K-S, Weinberger-E, Childrens Hospital and Medical Center, Seattle, Washington, U S A Purpose: Chest CT in uncooperative children is often limited by respiratory motion and poor anatomic definition. We compared the quality of conventional and helical CT images obtained in children unable to breath-hold. Materials and Methods: Two serial conventional 0.6 sec and helical scan studies ( G E HiSpeed Advantage CT) with comparable mAs and equal collimation were obtained in 16 patients at a mean study interval of 3.8 months. Mean patient age was 3.7 years (range 1-7). Scans were obtained during quiet respiration. Conven-

In the last two years we performed 160 spiral CT examinations on 135 pediatric patients. Conventional or spiral CT of identical regions was performed in the case of follow-up on 22 children. In this group of patients we were able to compare both CT modalities using a S O M A T O M Plus S scanner (Siemens company, Erlangen, F R G ) . The examinations focused on pathologies of the skull, brain, spine, thorax and abdomen. As far as possible an identical collimation was used in both CT modalities on the same patient. Usual spiral CI pitch factors were set to I to 1.5. Due to the limited heat capacity of the Xray tube, radiation dose had to be reduced in spiral compared to conventional CT by a factor of 0.3 to 0.5. The results of our study are as follows: Spiral C1 allows for a dramatic reduction of total examination time, so only brief or no sedation is necessary. Motion artifacts are significantly reduced, so the quality of 2D and 3D reconstructions is improved. Despite the radiation dose reduction in spiral CT, resulting in a moderate reduction of signal-to-noise ratio, the image quality was comparable to conventional CT from the diagnostic point of view. This can be explained by the reduction of motion artifacts, the better utilization of intravenous contrast agents and the possibility of overlapping slice reconstructions. According to our results we conclude that spiral CT is the best method for the majority of pediatric CT indications. This is especially true for seriously ill or injured children.

Spiral CT in Children. How to Use Contrast Media?

Neuenschwander-S, Briand-Y, Servois-V, Ollivier-L~ BonnerotV, Institute Curie, Paris, France Spiral CT requires new practices in the administration of contrast media. Our purpose is to evaluate 6 months experience of abdominal and thoracic spiral CT in children in terms of vascular enhancement and its relationship to the type of contrast medium and the injection. Methods: We reviewed the data of 57 spiral CT scans (Elscint CT Twin) with intravenous administration of contrast medium. Children under 5 were sedated with chloral hydrate. A power injector (MCT - Medrad) was used in 55 examinations. Thoracic examination was performed in 26 children, aged 10 months to 17 y (reed 12 y). The length of scanning was between 9.5 and 25 cm, with a scan time of 14 to 30 sec (med:16 sec). The contrast medium was injected through a peripheral vein with a 22 G (n = 18) or a 24 G (n=4) angiocatheter, or a central catheter (n= 4), at injection rates of 0.8 to 1.5 ml/sec; in 17 cases, we used a biphasic rate. All patients received an ionic contrast medium ( H O C M Ioxithalamate - Guerbet) 1.2 to 2 ml / kg with a 30 % iodine con-

447 centration. In 22 cases 1.2 to 1.5 ml / kg at 38 % in 4 cases. The delay between initiation of contrast material injection and scanning was 17 to 60 sec (reed 35 sec). Abdominal examination was performed in 26 children, aged 15 days to 16 y (mod 3 y). The length of scanning was between 9 and 37 cm, with a scan time of 15 to 31 sec (med 25 sec). The contrast medium was injected through a central catheter (n = 12) or a peripheral vein with a 24 G (n = 10) or a 22 G (n = 4) angiocatheter, at injection rates of 0.7 to 1.5 ml/sec. 24 patients received ioxithalamate 1.5 to 2 ml / kg at 38 %, 2 patients received a non-ionic contrast medium (LOCM iopamidol). The delay between initiation of contrast material injection and scanning was 20 to 55 sec (med 30 sec) 2 patients, aged 4 mths and 11 y, had a liver examination only and received 2.5 ml/kg at a rate of 0 5 ml/sec, and 1.5 ml/kg at a rate of 1 m l / s e c of 38 % ioxithalamate respectively, with a scan delay of 20 sec. 3 patients, aged 5, 6 and 11 y, had both thoracic and abdominal examinations. They received 2 to 2.2 ml/kg of 38 % ioxithalamate at a rate of 0.7 and 0.8 ml/sec. The length of scanning was between 28.5 and 37.5 cm, with a scan time of 20 to 30 sec and a scan delay of 30 or 48 sec. The scan was performed in a single examination in 2 patients and in two successive steps in 1 patient. In each patient incidents or adverse reactions were noted, and vascular enhancement was graded into 4 groups of increasing quality from 1 to 4. Failure of the spiral technique occurred in 6 cases, 2 were non evaluable; in the 4 other cases the scans were simply delayed from 1 to 5 min., but images were obtained. Minor adverse reactions to contrast media were observed with 38 % ioxithalamate in 2 cases vomiting (1) urticaria (1). In 3 cases, also with 38 % ioxithalamate children moved during scanning but in all cases mild motion artifacts did not alter information. Among the 25 evaluable thoracic examinations i was in group 1 (bad opacification because of spiral technique failure), 5 in group 2, 15 in group 3 and 4 in group 4. Among the 25 evaluable abdominal examinations, 3 were in group 1 (all because of incidents) 2 in group 2, 3 in group 3 and 17 in group 4. The 2 liver examinations and the 2 thoracoabdominal examinations performed in a single run were in group 4. The last thoracic and abdominal examination performed in 2 successive steps was classified 4 for the thorax and 2 for the abdomen. Conclusion: 1.5 ml / kg of a 30 % H O C M appears sufficient to obtain good mediastinal vessel enhancement and a biphasic rate seems to improve hilar enhancement. The most frequent shortcoming of this study was a venous flash, which could be avoided by optimizing the scan delay or scanning from bottom to top. Liver and abdominal examinations with 1.5 m l / k g of 38 % H O C M at a rate of 0.7 to 1 m l / s e c and a delay of about 30 sec yielded excellent results.

Full Film Less Ultrasound Imaging Using A Picture Archiving and Communication System

Piqueras-J, Carreno-J, Enriquez-G, Aso-C, Lucaya-J, Hospital Universitari Materno-Infantil, Vall D'Hebron, Barcelona, Spain We present two years of experience of a film-less pediatric ultrasound unit using a commercial PACS. Every day 20-24 examinations are performed, 4-10 of them mobile in the neonatal or pediatric CCU's. Two ultrasonography (US) units (Acuson 128 and A l o k a 650) are connected to a CommView PACS (Philips Medical Systems), by video frame grabbers, hnage resolution is 660 • 576 pixels (256 grey levels). The aquisition is activated by pressing a footswitch, and is completed in 0.5-1.5 seconds. Mobile examinations are acquired by small framegrabber devices (RDS Adquisition AgfaMatrix Instruments, CA) with removable magnetic discs; once back in the department, images are downloaded to the PACS. A diagnostic workstation (DWS, CommView EDWS) equipped with four high resolution monitors, and one gigabyte magnetic disc, is de-

voted to the ultrasound section. A film laser printer (Kodak KELP) is shared with other DWS. Hardcopies are only printed for slide generation and scientific sessions. The PACS has all modalities connected (computed radiography, fluoroscopy, CT and MRI). We have eliminated the handling of cassettes and films saving one hour of nursing time per day. The mean total number of images per examination has risen from 12 to 20 and the number of examinations per day has increased about 10 %. Image quality is as good as conventional hardcopy being independent of processor problems. Quality control can be checked on screen immediately after the examination. Image zooming and window level and width adjustment helps in diagnosis in many cases. Reporting is done always by dictation. A list of undictated examinations is presented and moving to the following exam is done by a single keystroke. The display of an exam is done in 7-16 sec. Previous examinations of any modality can be retrieved and displayed simultaneously on any DWS. Reports are printed on the original examination request. US exams are archived on an optical disc library. We have suffered several problems, e.g. one day of reported examinations was lost before optical archiving, after a magnetic disc failure, and three periods of 3-4 days where the system was out of order for repair of PACS subsystems. We conclude that PACS can be successfully applied to US without major technical problems. Similar results can be expected in other radiology environment where workload and image size are well suited for current PACS technology.

Digital Subtraction Radiography in Diagnostics of Bronchial Anatomy for Detection of Visceral Situs

Ivanitsky-A, Bakulev Center For Cardiovascular Surgery, Moscow, Russia Purpose: To present the new radiologic technic for detection of bronchial anatomy. Digital subtraction radiography without contrast injection was used. Investigations were performed on X-ray system Siregraph-D with Digitron-3 (Siemens, Germany) during 4-6 sec with 8 frames per sec. Then selection of frames with optimal image of bronchial anatomy was made. In the period 1992-1993 100 patients with congenital heart disease were investigated. 9 of them (7 male, 2 female, 9 months 16 years old) had anomalies of bronchial situs: 3 - situs inversus, 2 - right and 4 - left isomerism. Subsequent angiography and operating data confirmed left position of the venous atrium m cases with bronchial situs inversus, syndrome asplenia in cases with right bronchial isomerism, and syndrome polysplenia in cases with left bronchial isomerism. Conclusion: Digital subtraction is the simple and effective method of visualisation of bronchial anatomy for detection of visceral situs.

Patient Dose in Paediatric Computed Tomographic Examinations. Results From A Pilot Field Study in Bavaria

Horwitz-A-E, Schneider-K, Kohn-M-R Freidhof-C Endemann-B, Ernst-G, Scheurer-C, Panzer-W, Menzel-M-L, Paediatric Radiology, Childrens Hospital, University of Munich, Mtinchen, Germany Computed tomographic examination (CT) frequency in children is much higher than that of magnetic resonance imaging because of the extensive availability of CT and relative ease of handling of these young patients. CT has a high diagnostic potential when compared to conventional radiography; however, patient dose is also considerably higher. The goal of this study is to gather information on the state of the art of paediatric CT examinations in this region of Germany together with the corresponding patient dose. We surveyed 43 clinics and private radiologist's offices in Bavaria and its neighbouring states on CT equipment and radiographic technique for the three

448 most frequent paediatric CT examinations (skull, chest and abdomen). A t the same time, we measured dose using thermoluminescent dosemeters. These were positioned fi-ee-in-air in the centre of the axis of rotation for the respective standard radiographic settings. Where appropriate, the survey information was correlated with the measured dose. Analysis of the results show that CT technique for children is not standardized and that there is large variation in patient dose for comparable CT machines. Dose is not dependent on the age of the equipment, but is mostly determined by a radiographic CT technique which is geared for high image quality, i.e. high mAs-settings, thin slices and absence of additional filtration. For example for the standard of a CT skull examination for the age group ranging from 5 to 12 years, our survey showed a dose variation from 23 to 150 m G y per slice. For chest examinations of the same group, the range was 15 to 61 m G y per slice and 14.2 to 101.5 mGy for the abdomen. Similar wide variations were found for the respective examinations in the younger age groups (0 to 1 and 1 to 5 years). In consequence, we recommend lower mAs-settings and increased filtration to obtain dose reduction accepting a lowered but still tolerable image quality. Each department studied will receive feedback in an anonymous comparison with the others. Further studies on dose and image quality, using a child equivalent phantom are in preparation.

ing increase in echogenicity in their contralateral kidneys. Doppler examination was performed in one patient. Slowed systole was detected in segmental or interlobar intrarenal arteries, as has been reported in patients with severe renal stenosis. Owing to anatomical difficulties, percutaneous angioplasty was ruled out and both affected kidneys were removed. Surprisingly, routine renal US performed 20 days later showed both right kidneys to be normal. To our knowledge, this is the first report of increased echogenicity in the contralateral kidney of patients with unilateral RAS. These alterations very likely represent hyperfiltration and increased excretion of Na and water known to occur in the normal kidney secondary to overproduction of renin and angiotensin II by the ischemic kidney. Conclusion: Hypertensive patients with severe unilateral renal stenosis may show increased echogenicity of the contralateral kidney. In our experience, this sonographic finding is reversible and does not necessarily imply a permanent anatomic lesion.

Computerized Radiation Dose Monitoring in Children

It is well known that urate infarction is often observed in the kidneys of severe and prolonged hypoxic human neonates. Materials and Methods: The examinations were performed in 12 adult rabbits. I group - 7 rabbits - received 0.8 g/kg urate solution i v only once, II group - 5 rabbits - received 0.1 g/kg urate solution i v 4 times, every second hour. Four hours tourniquet test was used in both groups for modelling shock and sensitizing the kidneys. Each animal served as its own control. Before drug administration, and 4, 8 and 24 hours later the kidneys were examined by ultrasound (US), (7.5 MHz transducer) and the plasma level of uric acid (UA) was measured. Result: Group I exhibited rather severe hypervolaemic circulation insufficiency, U A precipitation could not been proved by US. Group II U A level at 8 hours was significantly increased, 4 hours US reveled enlarged kidneys, 8 hours increased echogenicity of the pyramids and cortex. The kidneys were not further enlarged, 24 hours increased echogenicity of the pyramids was unchanged. Conclusion: These experimental results suggest the possibility of examining the occurrence of urate nephropathy in the kidney of severe hypoxic human neonates.

Wesenberg-R-L, University of South Alabama, Department of Radiology, 2451 Pillingim Street, Mobile, A l a b a m a 36617, USA We have developed and installed at the University of South A l a b a m a an accurate, user-friendly, cost-effective computerized radiation dose monitoring system. The system provides immediate feedback of radiation dose administered to the child during fluoroscopy by the pediatric radiologist. Any of the fluoroscopic parameters which are changed during the procedure (e g.-kVp, mAs, field size, magnification etc.) are immediately evident on an L E D read-out attached to the fluoroscopic TV monitor. A t the end of the procedure there is a printout of exactly how much radiation dose was administered at fluoroscopy, with spot films and radiographic overhead films plus total radiation dose for the entire procedure. Other systems have been developed to determine exposure area product. However, this is the first system in the world to compute actual radiation absorbed dose in gram-rads within an error range of plus or minus five percent. Awareness of radiation dose administered by the pediatric radiologist as well as adjusting of fluoroscopic techniques has resulted in a reduction of 80 % to 85 % of radiation administered to pediatric patients. Generalized usage of this sophisticated computer system could result in at least a 50 % reduction in GSD (genetic significant dose) to all children.

Reversible Sonographic Alterations in the Contralateral Kidney in Two Children With Unilateral Renal Stenosis

Enriquez-G, Castello-F, Lucaya-L Aso-C, Hospital Universitari Materno-Infantil, Vall D'Hebron, Barcelona, Spain Renal arterial stenosis (RAS) is a cause of hypertension in pediatric patients. Angiography is the standard radiological procedure for establishing diagnosis. On conventional renal sonography, patients with RAS normally present a small-sized affected kidney. However, no altered echogenicity of the affected or contralateral kidneys is usually observed. Two hypertensive children 2 months old and 26 months old, respectively, with severe left renal artery stenosis secondary to fibromuscular dysplasia are described. Initial renal US showed a mislead-

Experimental Sonographic Morphology of Uric Acid Nephropathy

Horvath-I, Zewdu-M, Streitman-K, Pinter-S, Department of Radiology, Albert Szent-Gyorgyi Medical University, Szeged, Hungary

Radiological Manifestations of Sarcoidosis With Renal Involvement in A Child

Sayegh-N, Bensar-F, Mamou-Mani-T, Lallemand-D, H6pital Des Enfants-Malades, Paris, France Sarcoidosis is rare in children. It affects primarily lungs, skin and eyes. Liver, spleen, peripheral lymph nodes, salivary glands and kidneys are seldom affected. Radiologic signs of renal involvement have not been reported in children. An 8year-old girl was referred for evaluation of a severe pulmonary insufficiency with infiltrative lesions and hilar lymph nodes on chest X-Ray and CT. Sarcoidosis and tuberculosis were suspected. Abdominal US revealed retroperitoneal adenopathy with abnormal echogenicity patterns of both kidneys, with similar anomalies on abdominal CT. The diagnosis was confirmed by renal biopsy.

Case Report: Carcinoid Tumor Of The Prostate

Udjus-Teitelbaurn-K, Childrens Hospital of Eastern Ontario, 401 Smyth Road Ottawa, Ontario, Canada

449 Carcinoid tumors occur rarely in children. A case of a sevenyear-old boy with this condition is presented including imaging appearances on ultrasound, CT and MR. The location of the tumor in the prostate gland has never before been described in a child.

Endovaginal Ultrasound Using Miniature High Resolution Transducers for Evaluation of Vaginal Tumor in Children: Preliminary Report

Rosenberg-H-K, Liu-J-B, Merton-D-A, Goldberg-B-B, Meadows-A-T, Albert Einstein Medical Center, Thomas Jefferson University Hospital and The Childrens Hospital, Philadelphia, Pennsylvania, U S A Ultrasound (US) of the pelvis is useful for the initial diagnosis of vaginal tumors in young children. Following response to chemotherapy and/or radiation treatment, transabdominal or periheal sonography cannot provide complete evaluation of the vaginal wall for residual or recurrent tumor. We therefore assessed the efficacy of endovaginal US using miniature catheterbased transducers for the evaluation of vaginal tumors in children. Methods: We utilized a Diasonics IVUS real time US unit equipped with a 6.2 French, either 12.5 or 20 MHz catheter-based transducer was inserted directly into the vagina in two young girls who were previously treated for vaginal tumors. CASE 1; This 20-month-old female presented with a history of vaginal bleeding during the preceding 11 days. CT of the chest and abdomen were negative and CT of the pelvis was reported to have shown a solid vaginal mass with no evidence of pelvic extension. The bone scan was negative. Two biopsies of the vaginal wall revealed botryoid embryonal rhabdomyosarcoma. Her treatment included radiation therapy and chemotherapy. C A S E 2; This 8-month-old female presented with a 6 week history of blood on her diaper and an introital mass. Chest radiographs were normal and the bone scan was negative. Abdominal pelvic US revealed a 2.8 x 1.6 x 2.0 cm solid pelvic mass in the region of the vagina appearing not to involve the uterine cervix. Pelvic sonography utilizing a water enema technique helped to define the rectum as the organ of origin. Vaginoscopy and multiple vaginal biopsies were done, with removal of 50 percent of the tumor from the right posterior later al wall. Pathologic diagnosis was endodermal sinus tumor and the child was treated with chemotherapy. Results: Cross-sectional real time US images of the walls of the vagina, obtained with the miniature transducers, showed no evidence of vaginal mass in these two cases, In one patient a 6 French plastic catheter was inserted alongside the US probe and sterile water was hand-injected, but there appeared to be no additional advantage. Conclusion: It is concluded, based on this limited experi ence, that routine pelvic US (to rule out mass, lymphadenopathy, and ascites), combined with endovaginal catheter-based miniature US transducers is reliable and cost-effective for the follow-up of young girls treated for vaginal cancer and may obviate the need for CT, M R I and hydrosonovaginography.

Can Transcranial Doppler Sonography (TCD) Detect Cerebrovascular Disease in Children With Sickle Cell Disease?

Verlhac-S, Bernaudin-F, Brugieres-P, Tortrad-D, Mage-K, Gaston-A, Reinert-P H, Departments of Radiology, Pediatrics and Neuroradiology, Intercommunal and Henri Mondor Hospitals, Creteil, France Stroke, especially thrombosis is a common and severe complication in SCD, with an estimated prevalence of clinical stroke syndromes from 13 to 17 %. The mean age of these patients is 7.7 years and the recurrence rate for stroke up to 67 %. Recent MRI studies have reported a high percentage of silent infarcts

(11% to 16 %). Initially cerebrovascular disease was believed to be caused by small vessel occlusion with sludging and congestion by the sickled red blood cells. With the advent of angiography most cerebral infarctions in SCD are known to be associated with an occlusive vasculopathy involving the large cranial arteries. TCD has been proved to be an effective modality for stroke prediction in SCD. We systematically performed TCD on SCD pediatric patients cared for in our center. Moreover M R I and M R A were done on patients over the age of 7 years and on those with abnormal TCD. When a stenosis was suspected by TCD an arteriography was performed after decreasing HbS level to under 30 % and hydration. 92 TCD were performed on 72 children (56 SS, 13 SC and 3 SB thai) during an 18-month follow-up. Mean M C A velocity was found higher (113 + 46 cm/sec) in SS group than in SC group (81+25 cm/ sec). Abnormal flow was not found in SC group but in 9/56 SS patients undetectable M C A flow (2) and mean flow velocity > 170 cm/sec (8), one patient having undetectable flow on one side and accelerated flow on the other side. The 2 patients with unilaterally undetectable M C A flow had experienced a stroke and MCA-thrombosis was confirmed at arteriography. Among the 8 cases with high velocity flow the 4 cases with a mean M C A flow > 190 cm/sec had stenosis found at arteriography even though only 2/4 patients had experienced a stroke. In the 4 cases with 170> flow velocity < 190 cm/sec arteriography has been performed in 2 cases (1/2 abnormal). Mean M C A velocity was found within normal limits 6 months later in the two other cases. We conclude that TCD is very useful in SCD patients for intracranial arterial stenosis detection. Such selected patients should be evaluated by arteriography and considered for intensive therapies such as BMT or transfusion program.

Periventricular Echodensities and Hemorrhagia Incidence and Correlation to Perinatal Risk Faclors Diagnosis by Cranial Ultrasound in Preterm Infants With Birth Weight Of 1500 Grams Or Less

Hesser-U, Katz-Salamon-M, Mortensson-W, Flodmark-O Forssberg-H, Department of Pediatric Radiology, St Gorans Hospital Karolinska Hospital, Stockholm, Sweden Classification of pathomorphological findings at cranial US has usually been based on the criteria elaborated by Papile et al (1978). They focused the hemorrhage in the germinal matrix, its possible rupture into and distention of the ventricles as well as extension into the adjacent parenchymal tissue. The knowledge that periventricular echodensities may represent ischemic-hemorrhagic lesions, shown by Hill in 1982 necessitated a modification included in recent reports (Volpe et at al 1987, de Vries et al 1992). The aim of this report was to describe the findings at brain US using a partly modified interpretation of the US findings to elucidate the relationship between risk factors in the neonatal period versus hemorrhagia and intraparenchymal echodensities. From 1988 to 1993, 260 consecutively born infants weighting 1500 g or less were studied with US at the neonatal intensive care unit at Karolinska Hospital, Stockholm. The aim was to examine the infants at least 3 times, at day 1-3, at day 5-7 and at 3 weeks or at discharge. Pathology due to bleeding was classified in 4 grades (B~4) similar to Volpe's grading. Abnormality in the paraventricular white matter was classified, regardless of size and duration, in 4 groups.(Wl_4): 1) Subtle echodensity of diffuse type, 2) Definite, distinctive echodensity, 3) Cystic formation, 4) Echodensity of hemorrhagic type or brain edema (coincides with B4). Neonatal risk factors included patient data (sex, gestational age and weight), antenatal factors (maternal toxicosis, maternal septicemia, placental bleeding or abnormal CTG), asphyxia at birth (low Apgar score), and postnatal riskfactors, circulatory (PDA) and respiratory (RDS, pneumothorax, assisted ventilation and surfactant treatment). Results: 48 patients had

450 abnormalities in the paraventricular white matter (15 W1, 12 W2, 10 W3 and 11 W4), and 60 patients had subependymal (28), ventricular (21), and/or intraparenchymal bleeding (11). Statistical analysis using stepwise multiple regression showed the following strongest and statistically significant risk factors: B14 assisted ventilation; W14 asphyxia at birth and surfactant treatment; B i and B2 low gestational age, B3 pneumothorax, B4(W4)surfactant treatment and asphyxia; W 1 and W 2 no significant risk factor found, W 3 asphyxia. The explanatory variables (R square) were, however, low, indicating a multifactorial cause. Conclusion: Subependymal/intraventricular hemorrhage and white matter echodensities have partly different risk factors; the abnormalities irrespective of type had multifactorial genesis. Surfactant treatment may increase the risk of ischemic related lesions in the paraventricular white matter.

Hyperechoic Thickened Ependyma. Sonographic Demonstration and Significance in Neonates

Rypens-F, Avni-E-E Vermeylen-D, David-R Matos-C. Hospital Erasme, Bruxelles, Belgium Hyperechoic thickening of the ependyma is supposedly related to ventriculitis yet, in our experience, this sign is more often observed without any infection but in association with subacute intraventricular hemorrhage (IVH). The aim of this study is to determine the circumstances of detection, the time of apparition, the sonographic evolution and the significance of these ventricular appearances. Sixty premature neonates have been prospectively studied from December 1992 to January 1994. Cranial sonograms have been performed at least twice a week during the acute phase of the neonatal hospitalization and weekly thereafter until final discharge. (Follow-up sonographic examinations have been obtained in all abnormal cases). The sonographic aspects were correlated with complementary investigations (MRI, infection work-up) when available. Intracranial hemorrhage was detected in 29 patients and hyperchoic thickening of the ependyma was observed in 23 of them. All the 23 neonates had IVH (14 grade II intracranial hemorrhages, 6 grade III, and 3 grade IV). In 14/23 hyperchoic ependyma was detected about 3 days (0-6 d) after the detection of intraventricular hemorrhage. In the other patients (7 with initially grade I hemorrhage and 2 without known hemorrhage), thickened ependyma was the first sign detected, allowing a retro-diagnosis of grade II hemorrhage. In all patients but one, the hyperechoic ependyma persisted at least for 2 months after disappearance of other sonographic signs of IVH. Six patients with intracranial hemorrhage did not present any particular aspect of the ependyma; 5 of them had hemorrhage localised in the germinal matrix and I had a limited intraparenchymal hematoma. None had IVH. MR examinations were performed in 32 neonates (18 patients of them had hyperchoic ependyma). It demonstrated the presence of hemosiderin and ferritin ependymal deposits only in patients with hyperechoic ependyma. Intravenous contrast injection performed in one of these patients showed enhancement of the ependyma. No neonate in our series had sign of infection. Furthermore lumbar punctures were performed in the first 4 cases of our series, showing only crenelated red cells and no signs of infection. Among the 23 patients, 6 developed transient ventricular dilatation, and 1 necessitated surgical intervention. One neonate developed marked dilatation one month after onset of the hemorrhage. In this patient a diagnosis of hemorrhage was made thanks to the hyperechoic ependyma. It is still closely followed clinically and sonographically. Hyperchoic thickened ependyma in premature neonates results most often from subacute intraventricular hemorrhage easily detected, it allows an "a retro" diagnosis of grade II hemorrhage and ascertains a clinical and sonographic follow-up in newborns at risk for secondary hydrocephaly.

Early MR Detection of Cortical and Subcortical HypoxicIschemic Encephalopathy in Full-Term Infants

Christophe-C, Clercx-A, Blum-D, Hasaerts-D, Segebarth-C, Perlmutter-N, H6pital Universitaire des Enfants Reine Fabiola, Ulb Bruxelles, Belgium In the neonatal period, US and CT often provide disappointing results in the assessment of brain damage in full-term infants. In contrast, spin-echo (SE) and inversion-recovery (IR) MR pulse sequences, optimized for the examination of neonates and infants, yield exquisite contrasts between cerebral lesions and normal parenchyma. Four full-term infants with severe hypoxic-ischemic encephalopathy were examined with MRI, first, during their first 2 weeks of life and later, after several months of life. MRI was performed at .5 Tesla (Phillips T5). A Tl-weighted SE sequence in the sagittal plane (TR =.500 ms, TE=35 ms) was followed by a phase-sensitive IR sequence (TR=1600 ms, TI= 600 ms, TE=35 ms) and a combined proton-density weighted SE sequence (TR=3700ms TE=35/ 150 ms) in the axial plane. The combination of these MR sequences has permitted the detection of various coexisting lesions in these infants. In particular, diffuse postnatal involvement of cerebral cortex and subcorfical white matter (WM) is demonstrated. Cortical hyperintensity on both proton-density and Tl-weighted images is probably related to cellular necrosis which is distributed diffusely or parasagittally. Hyperintense, frontal, subcortical WM edging on proton-density images results from the increase of water concentration, induced either by infarct or by edema. Diffuse WM areas of low intensity on T1 images and of high intensity on T2 images are presumably related to cytotoxic and/or vasogenic edema, proportional to the underlying damaged tissues. On follow-up MRI, several months later, the importance of cortical atrophy and of the myelination delay appears related to the importance of the lesions detected during the postnatal period. Staging of the lesions with MRI in the early postnatal period has a good predictive value with regard to neurodevelopmental outcome.

Biomechanics of the Lumbar Spinal Cord in Children Studied by MRI

Brunelle-F, Sebag-G, Baraton-J, Pierre-Kahn-A, H6pital Des Enfants-Malades, Paris, France Purpose: To analyse the movement of the lumbar spinal cord by MRI in normal children and in tethered cord syndrome. Material and Methods: 10 normal children and 20 children with tethered cord were studied by MRI (1.5 T). A sagittal T1 sequence was used for morphological analysis. An axial sequence was used for quantitative study of the spinal cord movement at the level of T l l for normal children and tethered cord syndrome. A CINE PCF sequence was used (TR 50 TE rain, 0=30 degrees; GRASS). Phase encoded velocity of 10 cm/s was used, 16 acquisitions were obtained per cardiac cycle. An ROI was placed on the spinal cord and CSF spaces to measure the velocity of the spinal cord and CSE Results: Lumbar CSF exhibits a systolodiastolic flow pattern similar to the intracranial CSF in all patients. Normal patients: Spinal cord exhibits a normal craniocaudal displacement with an offset of 3/16 cardiac cycle as compared to the CSF pattern. The maximal velocity is 1 to 2 cm/s. Tethered patients: Compared to normal patients 18/20 showed no movement of the spinal cord at all. Two patients with lipoma of the filum terminale had normal biomechanics of the spinal cord, and were not operated upon. Conclusion: MRI is a valuable tool to display normal and abnormal biomechanics of the spinal cord. It plays a role in selecting patients eligible for surgery.

451

Stenosis of the Nasal Cavity and Pituitary Agenesis. A Rare But Not Fortuitous Association. Report of 2 Cases

Hassan-M, Garel-C, Leger-J, Francois-M, Czernichow-R Department of Radiology, Robert Debre Hospital, 48 Bd Serurier, 75019 Paris, France We report 2 cases of neonates presenting the association of a stenosis of the nasal cavity and a pituitary agenesis. Both were full-term neonates. Their parents were first-degree cousins. In their first hours of life, they were both admitted to an intensive care unit because of respiratory distress. A nasal obstruction was suspected and CT demonstrated stenosis of the nasal cavity. During clinical follow-up, both neonates presented deep hypoglycemic attacks. Moreover, the physical examination showed a narrow palate in one baby. Both neonates had a complete anterior pituitary insufficiency without diabetes insipidus. On the skull radiographs, their sella turcica was shallow; one neonate had a single median incisor. An MRI study of the brain revealed a complete absence of the anterior and posterior pituitary gland and a Chiari I malformation. Hormone therapy was instituted at 1 month 1/2. The neurologic status of these two children is presently very poor. Stenosis of the nasal cavity is very rare and pituitary agenesis is an exceptional condition. They both belong to midline anomalies. To the best of our knowledge, such an association has never been reported. The clinical relevance of this association will be discussed.

Cranial Sonography in the Detection of Acute Intracranial Complications in Infants on E C M O: An Analysis of the Efficacy of Current Imaging Protocols

Tucker-J-l, Bramson-R-T, Blickman-J-G, Kruatrachue-A, Massachusetts General Hospital, Boston, Massachusetts, USA Current cranial US protocols for infants on ECMO have evolved empirically to entail a rigid schedule of frequent imaging regardless of the clinical status of the patient. Growing cost containment imperatives necessitate a scientific analysis of the efficacy of current protocols with the ultimate goal of optimizing the yield of cranial sonography in this setting. Materials and Methods: From February, '88 to May, '93, fiftyeight infants at a median and modal age of 1 day (range hours-94 days) required ECMO for up to 3 weeks secondary to cardiopulmonary insufficiency related to a variety of principle diagnoses, most commonly hyaline membrane disease or meconium aspiration. These infants underwent cranial US prior to, at 1-4 day intervals during, and at least once following ECMO. Results: No infant had US evidence of intracranial hemorrhage or ischemic changes at initiation of ECMO. US identified possible or definite acute intracranial hemorrhage in seventeen infants (29 %) during ECMO, of which nine (53%) represented intraventricular hemorrhage of Grade II or less. Analysis of the data suggests that clinical indicators such as an arrest or significant blood pressure change identify ECMO patients at increased risk of acute intracranial hemorrhage and, thus, can be used to streamline US protocols. Conclusion: Current cranial US protocols in ECMO patients are not maximally efficacious. Incorporation of an analysis of clinical parameters routinely followed in these NICU patients should identify those at increased risk of acute intracranial hemorrhage increasing the yield of this sensitive but time and cost intensive modality.

Ultrasonographic Evaluation of the Tongue-Mouth Complex. Normal and Pathological Results

Garel-C, Elmaleh-M, Francois-M, Hassan-M, Department of Radiology, Robert Debre Hospital, 48 Bd Serurier, 75019 Paris, France

An ultrasonographic study of the tongue and the floor of the mouth was performed in 30 healthy children (age 1 day15 years) in order to assess the normal US anatomy of this region. The scans were performed in sagittal and transversal planes with a 7.5 mHz transducer and a fluid-path. Moreover 17 children (aged from i d to 13 y) presenting with various local clinical symptoms underwent an US examination. This series included 3 abscesses of the tongue, 1 sublinguitis, 1 ranula, 1 lingual thyroid gland, 2 thyroglossal cysts, 3 dermoid cysts and 6 vascular malformations. The US findings were correlated with surgery and pathology in 15 cases, with the clinical follow-up in 1 case (sublinguitis) and with the nuclear study in 1 case (lingual thyroid gland). In each case, US could anatomically locate the lesion with a very good accuracy. The evaluation of the type of the lesion by US proved to be correct in 16 cases. We conclude that US of the tongue and the floor of the mouth in children yields an overall very good accuracy as a way of investigating masses and pseudo-masses of this region.

MRI and MRA as Guides for Treatment of Vascular Malformations

Fitz-C-R, Towbin-R, Kaye-R, Childrens Hospital of Pittsburgh, Pa, USA The interventional section of our department as part of a "vascular anomaly" team sees a large number of children referred for treatment. Patients selected by the team (surgeons, dermatologists, radiologists, and pathologists) for possible interventional treatment need an efficient diagnostic and treatment protocol to avoid unneeded procedures. The study is a retrospective and prospective determination of a protocol based on magnetic resonance imaging (MRI) and magnetic resonance angiography (MRA). Methods: All patients had MRI on a CE Signa 1.5 Tesla system. T1, proton density, and T2 images were obtained in appropriate planes. Based on these, M R A using 2D phase contrast with various velocities or 3D time of flight MRI and/or contrast MRI, or no further exam was done. Confirming catheter angiography with embolization, venography with sclerotherapy or sclerotherapy alone were treatments used. Direct injection of 100 % alcohol was used for sclerotherapy. 24 patients aged 16 w to 17 y were examined. 18 neck and face lesions, mostly of the nose and mouth, 7 body, and 2 limb lesions were examined and 23 were treated. Arteriovenous (AVM) and venous malformations (VM) lymphangiomas, and cystic hygromas (CH) were diagnosed. Results: 11 patients had MRI and M R A suspicious for AVM (abnormal vessel and blush on MRA) confirmed by catheter angiography and treatment by embolization. 10 patients had MRIs suspicious for slow flow VA, CH or lymphangioma, 3 of these had confirmatory negative MRA, and 2 of 3 early in the study had negative catheter angiograms. One of the 7 without M R A had catheter angiography showing capillary hemangiomas not treatable by interventional techniques. 3 of the 24 patients had M R A and catheter studies that were inconclusive or not in agreement due to technical factors or lesion characteristics small size of approximately 1.5 cm or infiltrating adjacent tissues. Conclusion: MRI combined with M R A when needed are highly reliable techniques to decide whether catheter angiography with embolisation or sclerotherapy should be done. Small lesions with limited flow and infiltrative lesions may be harder to assess.

Fast Sequential Postcontrast MR Imaging Differentiates Tumor From Perineoplastic Edema in Children With Musculoskeletal Neoplasm

Lang-P, Honda-G, Johnston-J, Roberts-T, Grampp-S, Geschwind-J-F, Genant-H-K, Gooding-C-A, Department of Radiology, University of California, San Francisco, Ca 94143, USA

452 Current MR imaging techniques including postcontrast scans are limited in evaluating the extraosseous extent of a bone tumor, although this information is crucial in decisions regarding limb-sparing procedures. The interface between the tumor and the surrounding soft-tissue is often ill-defined and extraosseous tumor may be mistaken for non-tumorous perineoplastic edema. We evaluated the use of fast sequential MRI following bolus administration of gadopentetate-dimeglumine in differentiating between extraosseous tumor and perineoplastic edema and between other neoplastic and non-neoplastic tissues present in these lesions. Methods: Sixteen patients with primary bone tumors were studied with a fast gradient-echo sequence (TR=39 msec TE=5 msec, Theta=60 degrees, 1 NEX, fat saturated acquisition time 3.5 sec scan) immediately after bolus administration of 0.1 mmol/kg body weight Gd-DTPA. Images were acquired sequentially for a total of 161 sec. Additionally, conventional T1 and T2-weighted MR sequences were obtained (precontrast double-echo G R E 600/14, 30/30 degrees, STIR 1500/20/170; pre- and postcontrast SE 800/30 SE fat saturated 500/30). Correlation with large stab histologic sections of the excised tumors or large core biopsy specimen was performed in all patients. Slope values of contrast enhancement were determined using an exponential fitting algorithm. A one-way ANOVA was employed to compare slope values of the different tissues. When the overall F-test showed statistical significance at 95 %, a Fisher PLSD test was used for multiple comparisons. Results: Mean slope values were as follows: viable extraosseous tumor 8.73, (range 39.98-1.02), edematous muscle without tumor infiltration 0.505 (0.67-0.25), normal muscle 0.421 (0.98-0.11), subcutaneous fat 0.242 (0.76-0.04), necrotic tumor.-0.13 (0.05- -0.23) (no significant enhancement), fibrous tissue 0.60 (0.71-0.43), normal marrow 0.20 (0.9-0.06). It is striking that no overlap was seen in slope values of viable tumor and edematous muscle as well as normal adjacent softtissues. The differences in slope values between viable tumor and edematous muscle without tumor infiltration viable tumor and normal muscle, viable tumor and subcutaneous fat as well as between viable tumor and necrotic tumor, fibrous tissue and normal marrow were statistically significant. These tissues can be differentiated using this technique. Conclusion: Unlike conventional M R images, slope values derived from fast sequential MR images following bolus administration of Gd-DTPA afford differentiation between extraosseous tumor and non-neoplastic normal and edematous softtissues. These images provide precise localization of the tumor interface and can thereby guide the surgeon in planning limb-sparing procedures. Slope values also help to differentiate between viable and necrotic tumor which can be used to define the biopsy site.

MR Signal Characteristics of Adult Cadaveric Bone Allografts in Three Children With Primary Bone Tumors Treated With Limb Salvage Therapy

Levin-T-L, Miller-T-T, Berdon-W-E, Ruzal-Shapiro-C, Rosenfield-N, Panicek-D-M, Dick-H-S, Babies Hospital, Department of Radiology, Columbia-Presbyterian Medical Center, 3975 Broadway, New York, USA Objective: To report the magnetic resonance {MR) appearance of adult cadaveric bone allografts in children following wide en bloc resection and limb salvage treatment of malignant bone tumors. Materials and Methods: MR imaging of the adult cadaveric bone allograft in three patients (two with limb salvage resection of osteogenic sarcoma and one with limb salvage resection of Ewing's sarcoma) was performed with T1 and T2 weighted spin echo sequences. The patients were between 5 m and 2 1/2 y following transplantation. Results: In all patients, MR of the allografts demonstrated decreased signal on TI weighted images and increased signal on T1 weighted images without evidence of recurrent disease. Conclusion: These pre-

viously unreported signal characteristics appear to be a normal finding in cadaveric bone allografts and should not be mistaken for recurrence. Revascularization of the allograft is limited to the function between the allograft and host bone and cannot account for these findings. The finding may reflect persistent marrow necrosis within the allograft marrow cavity.

Congenital Fibrosarcoma. Radiological Aspects

Sucher-E, Porat-S, Abu-Dalu-K, Amir-G, Ariel-I, Tadmor-O, Rabinowitz-R-G, Bar-Ziv-J, Department of Orthopedic Surgery, Hadassah Univ Hospital, Jerusalem, Israel Three patients with congenital fibrosarcoma are described. The age at the time of diagnosis ranged between 6 w and 3 y. The lower limb was involved in 2 patients, the upper limb in one only. All cases presented with large tumor masses which were considered initially as vascular tumors (lymphangiomas or hemangiomas). The US examinations and the CT scans performed revealed multiseptated areas of fluid density, a multitude of large supplying vessels together with more uniform tumor masses with no calcification and little, if any bone involvement. The treatment was by amputation in two cases and debulking in one, with good results during the follow-up period of 1.3 years. A fourth case was diagnosed antenatally and the pregnancy was terminated. In this case similar radiological and pathological findings were noted.

MRI of Normal Age-Related Bone Marrow. Cranial and Vertebral Bone

Taccone-A, Occhi-M, Dell-Acqua-A, Oddone-M, Giannina Gaslini Childrens Hospital, Genova, Italy To evaluate the MR features of normal cranial and vertebral bone marrow at different pediatric ages, the authors retrospectively analyzed 733 Tl-weighted MR examinations of the cranial bone and 250 of the spine. Bone marrow signal intensity on short TR/TE images was compared with that of fat and normal muscles. Concerning cranial bone, the bone marrow change of skull base, calvaria zygomatic bone, mandibular symphysis and apical petrous bone was evaluated. The patients were divided into six age groups: 0-1 year, 1-2, 3-6, 710, 11-15 and 16-24 years. Tl-weighted images were graded according to the patterns of physiologic conversion from red to yellow marrow and to the pneumatization of the sphenoid simls. The signal intensity of all anatomic segments was as low as that of muscle or inferior in all patients younger than 3 months. After this age, there is continued conversion to high signal intensity yellow marrow, with different features in the various anatomic segments. The first anatomic segments to become hyperintense were zygomatic bone and mandibular symphysis followed by presphenoid and basisphenoid regions basiocciput, calvaria and petrous apex. Regarding the spine vertebral body bone marrow was evaluated. Three age groups were studied: 0-6 and 7-24 months, 320 years. The most interesting changes in the developing spine occur at very young ages (first two years). From birth to about 3 months of age, the vertebral bone marrow appears markedly hypointense, because of hematopoietic tissue and probably greater trabecular bone amounts. From 3 to 6 months, the ossification centers gradually increase in signal intensity starting from the end- plates. From 7-8 months of age on the vertebral bodies assume the mild hyperintensity characterizing the whole childhood period, because of the relatively large red marrow fraction. The morphology of the vertebral bodies was evaluated The variability of the signal and the morphology of the disk were also evaluated. It is necessary to know the normal MR age related appearance of red and yellow marrow, in order to identify abnormal pat-

453 terns as well as marrow infiltration by tumours or other pathologic processes.

Prediction of Stability of Non-Displaced or Minimally Displaced Fractures of the Lateral Humeral Condyle

Finnbogason-T, Mortensson-W, Lindberg-L, Dept Paediatric Radiology, St Gorans Childrens Hospital, Stockholm, Sweden Subsequent displacement of non-displaced and minimally displaced fractures of the lateral humeral condyle while immobilised in plaster, may contribute to severe complications. To prevent this, prophylactic nailing or frequent X-rays and immediate nailing, if increased displacement occurs, has been practised in our hospital. The present prospective investigation aims at describing radiological criteria to assess the stability of these fractures, while immobilised in plaster. The clinical material comprised 112 children aged 1 to 11 years, with a mean age of 5.4 years. All fractures were diagnosed at the day of injury. Initially the displacement was none or at most 2 ram. According to the radiographic findings, the fractures were allocated to one of three groups stable fractures (A), fractures with ambiguous risk (B) and high risk (C) respectively, of subsequent displacement. All children were treated with splinting only. Results: The table below shows the number of

Group

No

Risk ratio

95 % confidence interval

A (n = 65) B (n = 35) C (n = 12)

0 6 5

0.00 0.17 0.42

0.00-5.52 6.56-33.65 15.17-72.33

fractures with subsequent displacement in each group, risk ratio, and the 95 % confidence interval. The subsequent displacement was between 1 and 2 mm, in one case 3 mm. In essence, the defined criteria were efficient in identifying the stable and the high risk fractures with high reliability. The group of fractures with indeterminate risk of subsequent displacement was fairly large - one third of the total material. The implication of these findings upon the treat-ment strategy of the fractures will be analysed in a separate report.

Femoral Head Shape in Legg-Calve-Perthes Disease. Correlation Between Conventional Radiography, Arthrography and Magnetic Resonance Imaging

Hochbergs-P-L, Eckerwall-G, Egund-N, Jonsson-K, Wingstrand-H, Department of Radiology, University Hospital, Lund, Sweden. Purpose: The shape of the femoral head is essential for the prognosis in Legg-Calve-Perthes disease. Degenerative disease will be delayed in onset and be less severe if the joint is congruous and the femoral head spherical at skeletal maturity. The purpose of this study was to correlate the bony head shape on conventional radiography with the cartilaginous head shape on arthrography and magnetic resonance imaging (MRI), and to compare the cartilaginous outlines obtained on arthrography with MRI. Materials and Methods: Twentythree children with Legg-Calve-Perthes disease were evaluated with conventional radiography, arthrography and MRI. Anteroposterior conventional radiographs and arthrograms with the hip in neutral position were used for measurements. MRI was performed using a 0.3-T permanent-magnet unit. Tl-weighted images in the coronal plane through the center of the femoral head were used to measure the cartilaginous outlines. The center of the femoral head was defined as the

midpoint of the maximum transverse diameter. Measurements were obtained from the center of the femoral head to the bony or cartilaginous outline at 30o intervals. The measurements were plotted in a scattergram and were analysed with polynomial regression of the fifth order. Results: On conventional radiography the bony outline of the affected hip was flattened laterally with maximum point at 75 o from the baseline. There was no variation in cartilaginous outline comparing arthrography and MRI. As in conventional radiography the cartilaginous outline was flattened laterally but with maximum at 60o. The unaffected hips did not reveal differences between the bony outline and the cartiloginous outline Conclusion: The bony shape obtained by conventional radiography does not reflect the cartilage femoral shape obtained by MRI or arthrography.

CT Survey of Laminotomies for Spinal Surgery

Schmit-P, Husson-B, Pariente-D, Zerah-M, Service de Radiopediatrie, Hopital de Bicetre, 78 Avenue Du General Leclerc, F-94270 Le Kremlin Bicetre, France Spinal deformity frequently occurs after spinal cord surgery. It is related to spinal instability due to the posterior approach of the cord through laminectomies. In order to reduce the incidence of such a deformity laminotomies instead of laminectomies can be performed. To evaluate the normal post-operative CT appearance and the efficacy of the procedure, we reviewed the files of 21 patients (14 girls and 7 boys) who underwent spinal cord surgery with an "en bloc" laminotomy approach between April '88 and December '93. Patient ages ranged from 2 m to 16 y (mean 5 y 2 m). Pathological processes include 16 intramedullary and 5 extramedullary masses. In 9 patients, spinal deformity was present prior to surgery. The level of the "en bloc" laminotomy procedure was cervical in 11 patients, thoracic in 19 and lumbar in 7. The extent of the laminotomy involved 3 to 10 neural arches, with a mean of 6.5 levels. Antikyphosis brace was used in every patient. According to the referring pathology, spinal radiotherapy was performed in 5 patients. Follow-up ranged from 1 to 40 m (mean 13 m). In 4 patients spinal deformity was present after spinal surgery, but in only one patient there was no pre-laminotomy deformity. Repeated CT monitored periosteal reaction, callus formation and final consolidation of laminotomy sites. Consolidation was definitively obtained in 8 patients. Its time of occurrence was highly variable with a range of 8 to 36 m (mean 20 m). Re-interventions were mandatory 4 times (3 patients) and laminotomies were again performed. One of these patients underwent laminotomy approach 3 times, total consolidation occurred after the first 2 procedures; after the third one, 50 % of the laminotomy sites were consolidated. 12 months after surgery the patient is still under survey and no spinal deformity did occur. Laminotomy approach allows good exposure of the spinal cord without the poor orthopedic prognosas of laminectomy, if mandatory re-intervention can easily be performed using the same approach and with the same good orthopaedic result. CT must be performed in the first post-operative week to be sure of the good reposition of neural arches. CT survey must be long enough to allow laminae consolidation monitoring and is helpful in determining the duration of antikyphosis brace use. Absence of spinal deformity allows easy MR survey of the cord status.

Serial Skeletal Radiographs in Infant Abuse

Kleinman-P-K, University of Massachusetts Medical Center, Worcester, Ma, USA

454 The purpose of this study was to demonstrate the value of serial extremity radiographs in cases of suspected infant abuse The radiographic findings in six instances of suspected infant abuse with serial radiographs were reviewed. Serial examinations were of value in 1) documenting injuries not visible on initial studies; 2) dating metaphyseal fractures and 3) providing insights into the process of repair of metaphyseal lesions. Serial radiographs may be of value in the routine evaluation of suspected infant abuse and further systematic study with larger numbers of patients is encouraged.

Nail Dysplasia Syndrome With Anonychia and Absence and/or Hypoplasia of Distal Phalanges Thomas-P-S, Nevin-N-C Royal Belfast Hospital For Sick Children and Dept of Medical Genetics, Queens University, Belfast, N Ireland Absence or hypoplasia of the nails and terminal phalanges of the fingers and toes is a feature of several congenital malformation syndromes in which there is associated mental and/or growth retardation. There is only a solitary report (from Israel) of familial absences and/or hypoplasia of the terminal phalanges occurring as an isolated phenomenon without other abnormalities. We describe the radiological and clinical findings of this condition in 4 members from 2 generations of a Northern Irish family.

Postnatal Investigation and Early Management of Congenital Hydronephrosis Lamont-A-C, Owen-R-J-T, Brookes-J-P, The Leicester Royal Infirmary, Leicester, UK Introduction: Medical and paramedical staff involved in the postnatal assessment of infants born in Leicester have a wide range of skill and experience and those infants with antenatally diagnosed abnormalities may require several hospital attendances before the investigation work-up is complete. The purpose of this study was to assess the value of an investigation protocol in reducing hospital attendances for infants with congenital hydronephrosis, by management from within the radiology department, both for diagnosis and early follow-up. Material and Methods: During 1993, consecutive pregnancies were screened for fetal abnormalities by ultrasound at the 1618 week, and if fetal hydronephrosis was identified, again at 34 weeks. Those with renal dilatation were referred to a paediatric radiologist and entered the protocol. A post-natal scan was performed within one week of birth, if abnormality persisted, a course of prophylactic antibiotics was initiated and a cysto-urethrogram performed to identify those with vesicoureteric reflux. Those without reflux proceeded to DTPArenography to detect pelvi-ureteric junction (PUJ) obstruction. Fully investigated patients were then referred to a paediatric renal clinic for appropriate surgical or long-term medical management. Results: 4268 screening examinations were performed. Seventy fetal renal tract dilatations were identified at the 34 week stage. 30% had postnatal hydronephrosis and 25 % had either reflux or PUJ obstruction and were referred to the paediatric clinics. Conclusion: Our results show that the system can run smoothly and allow accurate and reliable diagnosis with subsequent follow-up. Excessive patient clinic attendances for investigation planning can be avoided.

Antenatal Versus Postnatal Ultrasound Screening for Congenital Uropathy Barakat-M, Sweeney-L, Savage-J-M, Royal Belfast Hospital For Sick Children, Belfast, Northern Ireland

Published data suggest that 1:500 babies have a detectable renal tract abnormality on antenatal scan. Early detection allows for intervention to mediate renal damage by prevention of infection or surgical correction. We wish to determine the value of screening all newborn infants before hospital discharge for such problems. During a one year period, twentynine newborn infants, twenty-one boys and eight girls, had renal abnormalities detected; fourteen were detected antenatally from 5,000 live births (group i), and fifteen were detected on the second day of life from screening 654 randomly selected neonates as part of a pilot study (group ii). When apparent abnormality was detected antenatally, a post-natal ultrasound scan before hospital discharge was performed confirming abnormalities in all fourteen. All infants were started on prophylactic antibiotic Trimethoprim in the first week of life. Subsequently all twenty-nine infants were investigated as follows: 1) repeat US at 1 month of age; 2) if abnormality was still detected, a micturating cysto-urethrogram (MCUG) was performed; 3) radioisotope T99 DMSA scan if reflux was detected + / - M A G 3 renogram for differential renal function and evaluation of obstruction. Results: In group I of fourteen babies with antenatal diagnosis of hydronephrosis, seven were confirmed to have significant hydronephrosis, five had dysplatic multicystic kidneys and two had duplex kidneys. Three had vesico-ureteric reflux (VUR), two had reduced function associated with obstruction and three had reduced function associated with scarring. Four had dilated pelvi-calyceal systems without obstruction or reflux. In group II twelve had hydronephrosis, one had significant inter-renal size discrepancy and two had duplex kidneys. None had VUR, significant obstruction was present in six with reduced function in one and one had a horse shoe kidney. In summary out of fourteen infants detected antenatally, ten had significant abnormality on full investigation and of fifteen infants detected postnatally, seven had a significant abnormality. Antenatal detection rate was 1:500 and postnatal detection rate 1:100. Conclusion: Routine antenatal scanning fails to detect up to two thirds of significant urological abnormalities which can be detected on postnatal scanning.

Ureteropelvic Junction Obstruction of the Lower Pole in Complete Duplication of the Ureter Fernbach-S-K, Zawin-J, Lebowitz-R-L, Childrens Memorial Hospital, Chicago, I1, U S A Retrospective review at two hospitals revealed an anomaly associated with complete ureteral duplication: ureteropelvic junction obstruction (UPJO) of the lower moiety. Most prior reports have described this problem in patients with incomplete ureteral duplication. The uroradiologic studies of 15 children (10 boys, 5 girls) with lower pole UPJO were reviewed. In 7, prenatal sonography had detected an abnormality; in the other 8, the anomaly was found when studies were performed for urinary tract infection. At VCUG, 6 had reflux into the affected lower pole, allowing differentiation between complete duplication and bifid ureter. Pelvicaliceal dilatation disproportionate to the size of the refluxing ureter, dilution of the refluxed contrast material in the dilated pelvis, and a delayed film of the abdomen showing retention of contrast in the pelvicaliceal system confirmed the diagnosis of UPJO. In 2 children there was contralateral lower pole UPJO (1 complete, 1 bifid ureter). Contralateral UPJO of a nonduplicated kidney was present in 2. Corroborative functional studies were performed in all children and retrograde ureterograms in many. Lower pole UPJO must therefore be considered when there is hydronephrosis of this segment.

455

Yet Another Study of the Use of DMSA Scintigraphy in Urinary Tract Infection

Kenney-I-J, Barnes-J,

Burwood-R-J, Lutkin-J-E, Royal Alexandra Hospital For Sick Children, Brighton, England D M S A scintigraphy is recognised as the best method for detecting renal scarring in children. Its routine use has been advocated in initial investigation of proven urinary tract infection. We wish to present a prospective study of all children referred to all Brighton paediatricians over a 2 year period for investigation of UTI. After initial investigations with ultrasound(US) + / - M C U , the children were allocated as follows: 1) D M S A indicated - by the results of US + / - M C U . When initial investigation was considered normal, the children were age matched and allocated randomly to have either 2) D M S A now or 3) D M S A delayed (in 12 months' time). Results: (Table). Finally hospital and general practitioner records were

DMSA Group 1 2 3

all

abnormal

45 88 78

23 3 2

painstakingly searched for the results of the urine specimen which prompted referral, to determine which children had a genuine UTI. This was defined as follows. Proven U T I - > 105 pure growth and > 100 white blood cells/ml Equivocal U T I > 105 pure growth or > 100 white blood cells/ml, or result not available. No UTI - < 105 pure growth and < 100 white blood cells/ml (Table). Conclusion: We believe that initial investiga-

UTI

all

DMSA+

Provenuti Equivocal None

112 52 47

17 5 6

tion with US + / - M C U can screen out the large majority of children who do not need immediate further investigation. It is clear that US misses focal renal scarring but we are not convinced that the small number of +ve D M S A scans in whom initial investigation was negative, (5 cases out of 166, 3 %) justifies the radiation and financial burden. It may be preferable to employ our scarce resources to investigate all children with urinary tract symptoms, given that the number of positive D M S A studies in unproven UTI's (11) was higher than those in whom initial investigation was negative (5).

Inter- and Intraobserver Variance of the Renal Resistive Index in Healthy Children - Clinical Implications

Sorantin-E, Fotter-R, Uggowitzer-M, Qehenberger-F, Paediattic Radiology Division, Department of Radiology University Hospital Graz, Auenbruggerplatz 34, A - 8036 Graz, Austria Ultrasound (US) is one of the first choice imaging methods for the evaluation of the urinary tract especially in children. Grey scale US allows the determination of anatomy with high precision. Duplex and colour coded duplex sonography allow the visualisation of blood flow within the vessels of the organ and measurements can be done. One of these, the renal resistive index (RI), is believed to reflect peripheral resistance. This index is widely used in the initial assessment and in fol-

low-up of many renal diseases although little work was done concerning observer dependent variability. Purpose: The aim of this prospective study was to evaluate the inter- and intraobserver variance concerning the renal resistive index in healthy children. Material and Methods: 7 healthy children (range 6 to 14a mean 9.4a) comprised the study group. There was no history of any previous urinary tract pathology. Informed consent was obtained in all parents. All children were investigated by two of the authors (SE and UM) on two identical ultrasound machines (Acuson XPI128). Each patient was investigated always at the same unit in order to avoid bias in inconsistency of the machines. Each patient was examined twice in alternating order by each of the authors on the same day. During the period of investigation each of the authors kept his results strictly to himself. All measurements were done after voiding. The selection of Doppler tracings was based on a previous publication and the built-in software of the ultrasound machine was used to calculate the resistive index sup 1. Results: Overall 347 RI values were obtained in the 7 children. The mean RI was 59.5 +/- 6.6 % with a range from 50.1 to 64.9 % (p=0.0001). Further 5.8 % of all RI values were higher than 70 %. 5 measurements have to be performed in order to obtain a mean RI value with a precision of 2 %, 10 measurements provide a precision of 1.3 %. The RI values between left and right side did not differ significantly (p=0.56). There was no overall statistical significant difference between both examiners (p=0.87 - interobserver error). There was an overall decrease of the resistive index between the first and second examination from 60.58 to 58.56 (p=0.0001, intraobserver error) Conclusion: - If obeying a strict protocol there is a low, insignificant interobserver error. Therefore follow-up examinations can be done confidently by different investigators. The intraobserver error reveals a decrease of the RI about 2 % which may be stress related to a primarily unfamiliar situation. This is essential to keep in mind in interpreting follow-up exams with only minor changes in RI. A minimum of 5, better 10, measurements have to be done for a mean RI value with a precision of 1.3-2 %. In this small study the mean RI values of healthy children, older than 6 years, differ with statistically significance. In about 6 % of the measurements the RI values are greater than 70%. Hence sporadically RI values higher than 70 % should be treated with care and may not indicate pathology. Mean values out of about S to 10 RI values should be calculated and comparison with the contra-lateral kidney should be done.

Differentiation of Cystic Renal Neoplasm From Cystic Renal Dysplasia by Ultrasound

Duncan-A-W, Charles-A-K, Berry-P-J, Departments of Paediatric Radiology and Paediatric Pathology, Bristol Royal Hospital For Sick Children, St Michaels Hill, Bristol, U K It may be difficult to distinguish a cystic renal neoplasm from multicystic dysplasia or polycystic disease by ultrasound examination, particularly if only part of the kidney is affected. We report two cystic renal tumours (a cystic Wilms' tumour and a cystic partially differentiated nephroblastoma) in which ultrasound examination showed small cysts within the septa separating the larger cysts as a distinctive feature. This observation was confirmed by C T o f one case and direct pathological examination of both cases. Other scans of non-neoplastic cystic renal lesions were examined retrospectively and the feature of the cysts within septa was not observed. A literature review showed this feature illustrated but uncommented upon in scans of cystic renal neoplasms. We suggest that if ultrasound imaging shows 'cysts within septa' in a cystic renal lesion then a neoplastic process should be considered prompting further urgent evaluation.

456

Pheochromocytomas. Modern Trends in Evaluation

Hoeffel-J-C, Galloy-M-A, Paolucci-R, Marcon-E H6pital D'Enfants, Rue Du Morvan, 54511 Vandoeuvre Cedex, France Retrospective study of 11 cases. 20 % of all pheos are found in the pediatric population and more than one third of these children have multiple tumors, most of which are recurrent. Most of our cases were hypertensive. One presented with an unexplained fever. Three cases had bilateral tumors. Computed tomography was positive each time it was done. Diagnosis was based on finding increased urinary vanyline mandelic acid excretion. One case presented with ectopic thoracic tumor. Two familial cases occurred without associated disease. All patients were treated by tumor excision. All cases were benign. Three had recurrences. Two cases were associated with bone lesions of the digits and of the knees.

Diagnostic Imaging and Evolution of Cystic Congenital Adrenal Neuroblastoma (16 Cases)

Deffrenne-Pracros-P, Pracros-J-R Couanet-D, Panuel-M, Chateil-J-F, Didier-F, Pariente-D, Neuenschwander-S, Multicentric French Study, Department of Radiology, Debrousse, France

val rate was 91%. Over two thirds of the infants made an uneventful recovery and required minimal radiological input. However, a minority underwent a more protracted course. Common findings on plain radiography included bowel wall thickening and luminal dilatation, often accompanied by abdominal distension. More serious and uncommon complications included necrotizing enterocolitis (9%), short bowel (12 %), atretic bowel (8 %) and motility disorders (9 %). Contrast examinations were sometimes necessary to differentiate mechanical and functional obstruction. A 'small bowel enema' technique was found to be superior to the conventional follow-through examination in these cases. Significant cholestatic jaundice was seen in 11%, usually resulting from prolonged parenteral nutrition in patients with short bowel. This complication was monitored with serial US. We conclude that the overall prognosis in gastroschisis is now very favourable, and this should be taken into account when parents are counselled following antenatal diagnosis. Despite this, a few infants undergo a more protracted course and have a poorer prognosis particularly those with small bowel atresia.

AIDS-Related Lymphoma. Unique Features in Pediatric Patients

Siskin-G-P, Hailer-J-O, Miller-S-L, Sundaram-R, SUNY, Purpose: The purposes of this study were to illustrate the difficulties in establishing the diagnosis of cystic congenital neuroblastoma, to determine the diagnostic criteria and the evolution of this pathology. Material and Methods: From 1982 to 1993, sixteen patients (eight girls and eight boys eight on both sides) were diagnosed as cystic congenital adrenal neuroblastomas in the prenatal period of these, eight cases were found during the antenatal period. The following diagnostic methods were used: US (16), CT (5), MRI (1), MIBG (6), elevation of catecholamines (11), and adrenal or metastasis puncture (2). Results: Diagnostic criteria: size of the cystic adrenal mass 2 to 7 cm, hepatic metastasis 7/16 (4 pts presented cystic metastasis), elevation of catecholamines 6/11. MIBG + 5/6, adrenal or metastasis puncture + 1/2, US CT, MRI no specific aspect cystic or haemorrhagic mass with/without septations. Treatment: surgery 15/16 (7 pts were treated only by surgery), adjuvant chemotherapy: 7/16 adjuvant radiotherapy 1/16, 1 patient did not have any treatment at presentation. Evolution: recovery after initial treatment 12 pts (4/7 pts with a Pepper syndrome), recurrence 2 pts (1/7 pt with a Pepper syndrome), deaths 2 pts (2/7 pts with a Pepper syndrome). Conclusion: there is no specific aspect at US, CT, or MRI to differentiate cystic congenital adrenal neuroblastoma from adrenal haematoma or cystic lymphangioma except in cases of hepatic metastatic involvement. MIBG is specific and sensitive, catecholamines and cytology are not always conclusive; management is controversial and the evolution is often good even in stage IV-S.

Gastroschisis: A Radiological and Clinical Review

Ramsden-W-H, Arthur-R-J, Cusick-E, Martinez-D, The General Infirmary, Belmont Grove, Leeds, UK Gastroschisis is said to have an increasing incidence and is commonly diagnosed antenatally. Prenatal diagnosis can lead to difficult management decisions both before and after birth. Although antenatal sonographic appearances are well described, the postnatal radiographic findings are less well documented. A retrospective clinicoradiogical review of the condition was undertaken, combining its morbidity and mortality with its radiological features and their impact upon management. The study included 66 consecutive cases managed at our institution over a period of 11 years 6 months. All patients underwent surgery in the first 24 hours of life, and the overall survi-

Health Science Center, Brooklyn, USA Objective: The purpose of this study was to present the largest series of children with AIDS-related lymphoma and to compare the radiologic, clinical, and pathologic features with those of adult patients with AIDS-related lymphoma. Materials and Methods: We retrospectively reviewed the medical records and radiologic studies of eight children with AIDSrelated lymphoma. These children (ages 1-15) were treated at three medical centers in Brooklyn, New York. The imaging examinations included US, CT, MRI, and plain films. Results: The sites of lymphomatous involvement in our patients were widely distributed, including rare sites such as bone, testicles, and kidneys. Two patients presented with primary CNS lymphoma. Two other patients presented with primary, intraabdominal lymphoma with spread to the liver, spleen, kidneys, adrenal glands, and bone. The remaining patient presented with testicular lymphoma with spread to bone. All of our patients presented with site-specific symptomatology including seizures, respiratory distress, testicular swelling, and hepatosplenomegaly. Pathologically, the examined lesions were either intermediate (diffuse large cell) or high-grade (immunoblastic, Burkitt's type). In addition, four patients were tested and found to have evidence of prior exposure to EBV. Conclusions: Our results show that children with AIDS-related lymphoma have unique features when compared with adults. The clinical presentations differ in that adults typically present with "B-type" symptomatology. In addition, our results contradict previous assertions that extra-CNS lymphoma is rare in children. The fact that three of our children presented with lymphoma in rare sites may prove to be an important point of differentiation between AIDS-related lymphoma in children and adults.

Sonography and Color Doppler of Colonic Inflammatory Diseases in Children

Ferran-J-L, Couture-A, Veyrac-C, Baud-C, Department of Pediatric Radiology, Arnaud de Villeneuve Hospital, Montpellier Cedex, France Purpose: To determine the usefulness and accuracy of sonography and color Doppler US in the diagnosis of large bowel inflammatory conditions. Material and Methods: Prospectively US with color Doppler has been used in 37 children who suf-

457 fered from acute or chronic inflammatory diseases of the colon. Routine grey scale examination with graded compression allowed visualisation of the entire colon most of the time. Specific criteria evaluated the size of the colon's wall, the extension of the disease, the site of maximal enlargement, the pattern of the different layers of the wall and the color Doppler findings. Results: Infectious diseases (Salmonella, Shigella) of the colon always showed a concentric wall thickening with enlargement of the submucosal hyperchoic layer. Yersinia showed increasing size of the mucosa in the terminal bowel with a huge hypervascularisation. Crohn's disease showed marked wall thickening with disappearing concentric aspect of the different layers (mucosa, submucosa, muscularis) during the acute phases of recurrence with enlargement of the serosa. Typhlitis showed a thickening of the mucosa with confusion of the different layers. Hemolytic-uremic syndrome is characterized by a mural thickening without increased color doppler signal. Conclusions: Grey scale US and color Doppler seem to be useful and accurate tools in the assessment of inflammatory diseases of the colon. Crohn's disease may be followed up by US. Infectious diseases are rapidly diagnosed and US allows the appropriate treatment. High frequency probes with graded compression US of the colonic inflammatory diseases is now a routine and the number of barium enemas decrease day after day in our department for colon imaging.

Strictures of the Colon in Cystic Fibrosis

King-S-J, Van-Velzen-D, Carty-H, Heaf-D, Royal Liverpool Childrens NHS Trust, Eaton Road, Liverpool, UK Cystic fibrosis patients may develop recurrent colicky abdominal pain, right lower quadrant mass and signs of intestinal obstruction known as distal intestinal obstruction syndrome. Patients who do not respond to medical management require radiological investigation. We describe the radiological and histopathological findings in six children age 2-13.4 years (mean 6 7 years) with cystic fibrosis who presented in this way over a seven month period. Five children had ultrasound and contrast enema examinations. There was wall thickening of the ascending colon on ultrasound and contrast enema revealed a stricture of the ascending colon in all five. Five children had a right hemicolectomy. One child had resection of the terminal ileum and caecum. A patient who had undergone right hemicolectomy developed symptoms of intestinal obstruction five months later. Radiological investigations revealed a stricture in the descending colon which was resected. All children had histopathological changes of post ischaemic ulceration repair with mucosal and submucosal fibrosis in resected specimens. The only consistent change in management of these children was a switch from conventional enteric coated pancreatic enzymes to high strength products 12-15 months before presentation. Colonic strictures should be considered in cystic fibrosis patients who do not respond to medical management of distal intestinal obstruction syndrome.

Oro-Pharyngeal Swallowing in Infancy

Newman-L-A, Cleveland-R-H, Hillman-R-E, Gilbert-R-J, Blickman-J-G, Jaramillo-D, Childrens Hospital, Boston, Mass, USA Purpose: Diagnosis of infant dysphagia requires understanding of the normal infant swallow. Anatomic differences and lack of neurologic maturation are responsible for differences between infant and adult swallowing. However, there is a paucity of date delineating the parameters of the infant swallow. The purpose of the study was to obtain normal infant swallowing data.

Methods: Five swallows of twenty full term infants from birth to six months with normal GI evaluations and no history of neurologic or swallowing abnormalities were examined using video fluoroscopy. A liquid barium suspension (50 % wt/vol) was administered via bottle and nipple. Variables were determined by viewing the videotape by three pediatric radiologists and frame by frame computer analysis. Results: Computer analysis revealed a bolus head acceleration as it approached the cervical esophagus without a concomitant bolus tail acceleration. Variables which described oral transit showed greater variability across infants than pharyngeal transit variables. For example, maximum value for suck and oral transit time (x= 1.9 sec, s. d. - 1.8, range =0.26-5.76 sec) and sucks per swallow (x=3.44, s.d. = 2.95 range -- 1.0-11.0) was 22.15 and 11 times greater respectively than minimum value. In contrast, maximum pharyngeal transit time (x=0.61, s.d. =0.1, range = 0.32--0.78) was only 2.44 times greater than minimum. Two additional pharyngeal transit variables, velocity of bolus head and velocity of bolus tail, also had smaller ranges similar to pharyngeal transit time. Infants collected material in various sites in the oral cavity during oral transit including mid tongue, posterior tongue, and valleculae. Eighty percent of the infants exhibited residue of material in the valleculae upon completion of the swallow. All infants exhibited stripping tongue motion, absent nasopharyngeal reflux, and absent penetration of material into the supraglottic space. Conclusions: (1) Acceleration of the bolus head hypothetically represents the entrance of the bolus into the cervical esophagus. Explanations for acceleration include a) pressure differential between pharynx and cervical esophagus, b) Bernouilli effect of the bolus entering a narrow tube producing acceleration or c) a combination of a and b. (2) Across infants, factors describing oral transit were more variable than pharyngeal transit (3). Pooling and residue in the valleculae, abnormal in the adult swallow, were common in infants.

Pitfalls of Ultrasound in Appendiceal Peritonitis

Geoffray-A, Chami-M, Filippigh-P, Valla-J-S, Fondation Lenval, 57 Av. de la Californie, Nice, France Ultrasound is now a well known technique in the diagnosis of appendicitis. With the new technology (high frequency transducer) and if the radiologist is well trained the positive diagnosis is made in most of the cases. However, diagnosis may be difficult or even mistaken in cases of peritonitis. The purpose of this study is to review the different patterns that may be encountered in appendiceal peritonitis even normal examination in a few cases. We present a series of 24 cases with surgical treatment in all cases. Results of US will be described and discussed. In 9 cases, US was not contributive and even falsely reassuring in 4. This is important to know because a normal USd should not delay surgical treatment if the clinical examination suggests peritonitis.

Partially Reduced Intussusception: When are Repeated Delayed Attempts Appropriate?

Connolly-B, Daneman-A, Alton-D-F, Shuckett-B, Ein-S-H, Department of Diagnostic Imaging, The Hospital For Sick Children, 'Toronto, Canada The imaging techniques for diagnosis and monitoring of reduction of intussusception by fluoroscopy and ultrasound continue to evolve. The common goal of all of these new protocols is to reduce the intussusception by enema in as many patients as possible and avoid laparotomy with its potential morbidity. We have previously reported a series of patients who became asymptomatic following reduction of intussusceptions from the colon without contrast filling of the terminal ileum. Sur-

458 gery was withheld and the patients remained asymptomatic. We now report two infants in whom the initial attempt at reduction by enema achieved partial reduction from the descending colon to the transverse colon in one, and from the splenic flexure to the caecum in the other. Both patients became asymptomatic and clinically stable. In light of the clinical stability and in consultation with the surgeons laparotomy was deferred. Following several repeated enemas the intussusceptions were successfully reduced after 20 hours and 24 hours

respectively. Ultrasound was used to confirm the persistence of the intussusception before repeat air enema. Both patients remained asymptomatic and did not require surgery. In infants with partially reduced intussusception we suggest that if the patient becomes asymptomatic and stable, surgery can be safely delayed to permit further attempts at enema reduction. Close cooperation with the surgeon and careful clinical monitoring of the patient are essential requirements for this proposal.

Poster Presentations Intracranial Complications Associated With Extracorporeal Membrane Oxygenation in Neonates Patterns and Frequency

Rapoport-R-J, Gross-G-W, Saini-C-R Shaber-G-S, Thomas Jefferson University Hospital, Philadelphia, USA Neonates on ECMO life-support are at increased risk for a variety of intracranial complications, including hemorrhage, ischemic and hemorrhagic infarction, cerebral edema, hydrocephalus, white matter disease, and atrophy. The daily neurosonograms during ECMO and post-ECMO neuro CT and neuro MRI exams of 260 patients were reviewed and all demonstrated abnormalities. Major intracranial complications (e.g. grade 3 hemorrhage, infarction, PVL) occur infrequently. Almost half of all ECMO patients have no imaging abnormality. This exhibit demonstrated the range and the variety of intracranial complications of ECMO, with comparison between neurosonography, neuro CT, and neuro MRI.

Abdominal Complications Associated With Extracorporeal Membrane Oxygenation (ECMO) in Neonates

Gross-G-W, Saini-C-R Shaber-G-S, Thomas Jefferson University Hospital, Philadelphia, USA ECMO in neonates can be associated with abdominal changes that vary from Clinically innocuous to life-threatening. The clinical records, radiographs, and abdominal sonograms of 260 neonates treated with ECMO were reviewed. The range of intra-abdominal abnormalities and complications occurring during ECMO were determined. Frequently encountered, but clinically insignificant findings include a gasless abdomen, limited ascites, and mild renal collecting system dilatation. Calculi and/or sludge within the gallbladder are common and often associated with hyperbilirubinemia. Massive ascites, intra-abdominal or retroperitoneal hemorrhage, and intestinal perforation are clinically significant but occur less frequently. This exhibit illustrated the range of radiographic and sonographic complications and imaging findings involving abdominal structures occurring in ECMO patients.

Radiographic and Sonographic Appearances of the Thoracic 4) Complications of Extracorporeal Membrane Oxygenation (ECMO)

Saini-C-P, Gross-G-W, Shaber-G-S, Thomas Jefferson Ulaiversity Hospital, Philadelphia, USA. Thoracic complications of ECMO in neonates, identified by chest radiography and thoracic sonography, vary widely in their appearances. The medical records, chest radiographs, and thoracic sonograms performed on 260 consecutive ECMO neonates were reviewed and all complications invol-

ving thoracic structures were tabulated. Complications varied considerably in frequency and included malposition and/or migration of various essential tubes and catheters, a variety of abnormal patterns of air collections, pleural effusions that varied considerably in clinical significance, and a variety of acute intrathoracic hemorrhages. Thoracic complications of ECMO are common and varied in nature, but have recognizable patterns of radiographic and sonographic presentation. The range of radiographic and sonographic appearances of thoracic complications of ECMO, along with their clinical significance, were be presented.

Chest Radiographic Appearances in Neonates on Extra Corporeal Membrane Oxygenation (ECMO) - Common Findings and Variations

Shaber-G-S, Gross-G-W, Saini-C-R Thomas Jefferson University Hospital, Philadelphia, U S A Chest radiographs of neonates on ECMO present a wide range of potentially confusing but not necessarily clinically significant findings. Daily portable A P chest radiographs on 260 neonates treated with ECMO were reviewed and the range and frequency of possible findings were tabulated. A wide rate of radiographic appearances in an uncomplicated course of ECMO therapy were identified, including variations in cannula position, variable degrees and patterns of pulmonary opacification, pleural effusions, miscellaneous additional support lines, and alterations in appearance to the abdomen. This exhibit demonstrates the range of chest radiographic findings in uncomplicated courses of ECMO in neonates.

Clinical Significance of the Conus Medullaris Ending at the Level of L2

Dominguez-R, Martinez-D, Rollins-N, Department of Radiology, Childrens Medical Center & University of Texas Southwestern Medical Center, 5323 Harry Hines Blvd., Dallas, Tx 75235, U S A The normal location of the conus medullaris (CM) varies from T. n to L v A conus ending at L. 3 or lower levels is considered indicative of a tethered cord; but the clinical significance of a conus ending at L. 2 level is uncertain. The purpose of this retrospective study is to clarify the clinical status of patients whose CM ends at L~. One hundred and seven children (age 2 weeks to 20 years) underwent MRI of the spine to evaluate a possible tethered cord. In 68 of these patients the CM was at T n to L z and they had normal imaging findings of the cord and its coverings. Nineteen other children had the CM ending at L 3 or lower and were compatible with a tethered cord. This was later confirmed by surgery. The remaining 20 patients had the CM at L 2 and all but 2 had abnormal neurological findings: anal atresia, (with hydronephrosis and/or neurogenic bladder), distal neuron pathology (club feet, hyperreflexia of

459 the lower extremity) and/or moderate to severe scoliosis or were being followed for previous tethered cord after surgical release. We conclude that a cord ending at L 2 level in any child should be closely correlated with the clinical status as in most cases it is also associated with spinal dysraphism or other abnormalities of the neural canal.

Sonographic Determination of the Position of the Conus Medullaris in Preterm and Term Newborns and Infants

Beek-F-J-A, De-Vries-L-S, Gerards-L-J, Kramer-P-P-G, University Childrens Hospital ~ erlands

Utrecht, The Neth-

A study mode to determine the position of the conus medullaris relative to the vertebral column in preterm and term babies and infants and to define the rate of the ascensus meduL laris. Materials and methods: 94 children without congenital abnormalities were examined with ultrasound (US) of the spinal canal. The level of the conus medullaris was related to the middle of a vertebral body or a vertebral interspace. This level was determined by using sonographic landmarks or by comparing the number of ossified vertebral bodies on US with the number on abdominal radiographs. The level of the conus medullaris was related to the 50th percentile of the postconceptual age with the Kruskal-Wallis test. Results: For a level of the conus at Thn-L1, the 50th percentile of the postconceptual age was 58 weeks, for L 1 39 weeks, for L1-L2 38 weeks, for L 2 34 weeks, for L2-L3 34 weeks, for L 3 30 weeks. Statistical analysis showed a significant ascensus of the conus medullaris (p=0.0027). Conclusion: A statistically significant trend towards an ascensus medullaris is present in our study group. The conus medullaris in newborns is on average at a higher level than the L2-L3 level which is mentioned in most textbooks.

Color Doppler Ultrasound in the Evaluation of Pediatric Ocular and Orbital Disorders

Kovacs-R, HarkanyL-Z, Senyl-K, Nemeth-J, Department of Radiology Heim Pal Children's Hospital, Department of Radiology & 1st Department of Opthalmology, Semmelweis Medical University, Budapest, Hungary Color Doppler was used in 11 infants and children with different ophthalmological problems. The aim of this retrospective study to determine indications for the use of color Doppler in pediatric ocular and orbital diseases. Methods and patients: Color Doppler imaging and duplex Doppler studies were p e r formed by A T L UM-9 H D I system with 7-10 MHz linear and 7.5 MHz phased array probes. Direct scanning was applied through the palpebra without sedation. 6 boys and 5 girls were examined with an age range of 6 months and 16 years. Color Doppler and duplex study of orbital and ocular masses was performed with the lowest velocity range and wall filter settings. Central retinal artery and vein, ophthalmic artery and superior ophthalmic vein were interrogated for spectrum analysis and measurement. Orbital M R I was performed in one case. Results: Diagnosis of our cases as follows, retinoblastoma (n=2), intraorbital hematoma (n=l) chronic uveitis (u=l) vitreous membrane (n=3) retinal ablation (n=l) retrobulbar mass with not identified origin (n=2). Doppler signals were present in all tumours and inflammatory masses. Color Doppler was able to demonstrate circulation in cases with vascular anomalies. Conclusion: Based on previous studies in adults and on our present study in pediatric population color Doppler is useful in the differential diagnosis of ocular and orbital masses and vascular anomalies.

Orbital and Ocular Growth in the Fetus

Faure-F, Denis-D, Volot-F, Scheiner-C, Bourliere-Najean-B, Petit-R Devred-R CHU Timone Enfants, 13385 Marseille Cedex 5, France The aim of this study was to determine the links between the horizontal orbital diameter (HOD), the corneal diameter (CD), the axial length of the globe (AL) and the fetal growth. Sixty fetuses (17-40 FW) were examined. H O D was determined with radiological procedm'e, CD was determined with a calliper and A L was evaluated with ophthalmic ultrasonographic procedures. The data were correlated to gestational age (GA), fetal weight (FW), fetal height (FH), head circumference (HC) and biparietal diameter (BPD). The ocular growth is closely correlated to H C growth. The H O D related to the BPD is among the parameters which is both the most accurate for orbital growth and the best to predict the growth of the eye. This study leads to stress the interest of the fetal ultrasonographic measurement of the H O D in prenatal screening

Sonographic Evaluation of Superficial Dermoids of the Head Preliminary Experience

Peled-N, Decarie-J-C, Zuker-R, Bar-Ziv-J, Hadassah Hospital, Jerusalem, Israel To determine the value of high resolution sonography in the preoperative evaluation of superficial dermoids of the head. Material and methods: This was a prospective study of patients referred for preoperative evaluation of suspected superficial dermoids of the head. A total of 16 patients were examined. dermoids were: 11 periorbital, 2 lateral parietal, 1 retroauricular, 1 lateral frontal, 1 lateral occipital. Midline nasal dermoids were excluded because of the known high frequency of intra-cranial extension, and the complex anatomy of the sinus tract of the dermoid in this location. Results: The size of the dermoids ranged between 6 mm-20 mm (mean 12.9 mm). 15/16 were hypoechoic, 12/15 had a thin rim, and 3/15 had a thick rim, and 1/16 was isoechoic. Bone scalloping was detected by ultrasound in 7 patients. Confirmation by surgery and/ or CT was obtained in all patients,-by surgery in 11 patients and by CT in 10 patients. No intracranial or intraorbital extension was found in any of the 16 cases. The ability of U.S to identify extension of a sinus tract intracranially, was demonstrated in two patients with midline occipital cephalocele. Conclusion: High resolution sonography is accurate in evaluating superficial dermoids of the head. It does not require sedation and there is no radiation as in CT scan. It is our opinion that sonography should be the preoperative modality of choice in these cases.

Juvenile Capillary Hemangioma Simulating an Extracranial Meningioma

Ladanyi-E, Juhasz-J, Dobronyi-I, Paraicz-E, Hantos-G, BalintK, County Hospital, Eger and National Institute of Neurosurgery, Budapest, Hungary A one-day-old term male baby was admitted because of a mass in the right retroauricular region, Ultrasonography revealed a mass above the posterolateral fontanelle. A small part of the tumor was located intracranialy and seemed to be a solid mass with mild hyperechogenicity. It could not be differentiated from the dura. The mass came up like a buttonhole tumor through the fontanelle beneath the galea, and some larger vessels were visible within the mass. CT scans showed a homogenous, mildly hyperdense mass with contrast enhancement suggesting the diagnosis of meningioma. A t surgery the tumor appeared to be a fibrous meningioma macroscopically consistent

460 with the findings on frozen section. Histology was a typical juvenile capillary angioma. Conclusion: craniofacial capillary hemangiomas are benign tumors and generally appear shortly after birth. Most often they have no clinical significance and spontaneous resolution begins in the 1st - 2nd years of life. They may grow rapidly and may mimic other tumors. In our case the localization and structure of the lesion led to prompt surgical treatment.

Spinal Cord Alterations Associated With Ano-Rectal Malformation: MRI Evaluation

Schingo-P, Malena-S, *Bizzarri-G, Fariello-G, Rivosecchi-H, Ospedale Pediatrico Bambino Gesu,* Ospedale Regina Apostolurum, Albano Roma, Italy

A 9-year-old girl presented with a left-sided torticollis, which had been present during at least two years. The torticollis was painless. However, rotation was severely restricted. At the age of seven the patient had tonsillitis and developed trismus, but recovered promptly after antibiotic treatment. Only after this episode was the torticollis recognized. Plain radiographs did not reveal the cause of the torticollis. On tomography misalignement was observed between C 1 and C 2. On MRI a small soft tissue mass between the anterior arch of C~ and the dens was interpreted as possible granulation tissue, concomitant with Grisel's syndrome. 3D-CT depicted the true cause of the torticollis as an atlantoaxial rotary dislocation with interlocking on the left side. Signs of secondary osteoarthritis indicated a duration of this abnormality for longer than two years. Further enquiry revealed a history of injury of falling downstairs head first when aged two years. Broken incisor teeth were the only damage noted. Discussion: In a child a torticollis can be compensated for years, by extra motion in the unaffected segments. If this ability is lost the deformity will become apparent. Causes of torticollis in childhood are:-fibromatosis colli in the neonatal period, congenital abnormalities at the craniocervical junction, brain stem tumors and laxity of the transverse atlantal ligament following nasopharyngeal infection, known as Grisel's syndrome. Torticollis as a remnant of cervical spine injury is a rare cause, but this presentation is typical in this age group. Nowadays, more cases should be detected particularly with 3D-CT reconstruction. In this case it is unknown whether the nasopharyngeal infection may have led to laxity of the transverse atlantal ligament and thus worsened the torticollis. Operative correction can be hazardous and may lead to cord damage so conservative treatment is advisible.

Ano-rectal malformations (ARM) are due to an arrest or to a disorder of bowel development and of caudal migration. They can be associated to a wide number of malformations involving the spine,the genito-urinary tract, the cardio-vascular system, the trachea and the oesophagus. The value and the increased availability of MRI has allowed accurate diagnosis in spinal dysraphisms. The object of our work was: 1) to evaluate the incidence of these lesions and to identify a group of patients who were at risk for neurological deficits. 2) to refer these patients for spinal cord surgery in order to improve the sphincteral innervation and function. From January 1990 to December 1993 we used MRI in 62 patients affected by A R M aged from 2 months to 14 years. We found spinal cord alterations in 55 % of our patients: 22 presented with lipoma of the filum terminale; 4 with hydromyelia; 2 with tethered cord; 2 with lipomyelomeningocele and 2 with of caudal regression syndrome. The high incidence of spinal complications in this series suggests that MRI should be included in the protocol of investigation of A R M patients for correct surgical planning and adequate evaluation of ano-rectal function.

Paesano-P-L, Minio-Paluello-G, Weber-G, Prina-Cerai-L,

MRI Study in Children With Currarino Triad

Chiumello-G, Del-Maschio-A, Dept. of Radiology, Scientific Institute, H S. Raffale University Hospital, Milan, Italy

Ngo-L, Bugman-P, Le-Coultre-C, Argyropoulou-M, Depts of Pediatric Radiology and Pediatric Surgery, Hopital Cantonal Universitaire de Geneve, Switzerland The Currarino triad includes a sacral bony defect, anorectal malformation and a presacral mass. The aim of this study was to present the signs of this entity by MRI. Four children (one boy and three girls) presenting with the signs of the Currarino triad were examined by MRI using a 1.5 Tesla MR unit. The spinal canal and the pelvis were studied using spin-echo, T1 (TR= 500, TE=25 ) both with and without intravenous administration of Gadolinium, and with T2 (TR=2000, TE=25) sequences. Results: MRI showed partial sacral agenesis in three children and coccygeal agenesis in one. All of them had anorectal malformations which were well-seen on MRI. Two children had presacral teratoma. In one case, the lesion was homogenous and solid; in the second one, there was a cystic component. The others two patients had anterior lipomeningocele with tethered cord. No Arnold-Chiari malformation was detected. In one case with a history of meningitis, barium enema showed a neuroenteric fistula. Conclusion: MRI is a very good modality in the study of the Currarino triad. The nature of the presacral mass is better characterized; the bony lesions and the anorectal malformation are also well demonstrated.

An Unusual Cause of Torticollis

Zonderland-H-M, Bos C-F-A, Vielvoye-G-J, Bode-P-J, Bakker-D, University Hospital Leiden and Juliana Children's Hospital, The Hague, The Netherlands

Ultrasound of Thyroid in Young Diabetic Patients: Correlation With Laboratory Data

Autoimmune thyroiditis is more frequent in diabetic children and adolescents than in the normal population. Therefore we have compared thyroid ultrasound (US) patterns and laboratory data in these patients. Materials and methods: 24 non consecutive diabetic patients positive to thyroid autoantibodies (group 1) and 25 non consecutive young diabetic patients with a duration of the disease longer then five years, negative to anti-microsomes (anti-M) and anti-thyroglobulin (anti-TG) (group 2) were studied. All 49 patients underwent FT3, FT4 and TSH measurements, T R H and US examination of the thyroid with a linear probe at 7-13 MHz. Results: All patients of group ~' (24) showed presence of anti-M autoantibodies whereas only 15/24 (62 %) of them were positive to anti-TG autoantibodies. 18/24 (75 %) were euthyroid, 4/24 (17 %) had subclinical hypothyroidism and the remaining 2/24 (8 %) had subclinical hyperthyroidism (TSH alterations). US patterns suggestive of thyroiditis (polycyclic profiles and/or parenchyreal inhomogeneity and/or hypoechoic texture) were present in 13/24 (54 %); who were randomly distributed in the different subcategories. The remaining patients did not show any US alteration. All patients with subclinical hyper-or hypothyroidism 6 (25 %), had a pathological US. T R H - test was abnormal in 9/24 (37,5 %); 8 of whom (33 %) had either TSH or US alterations. None of the patients of group 2 showed US abnormalities suggestive of thyroiditis and all laboratory measurements were within normal limits. Conclusion: Our data suggest that thyroid US examination, TSH and determination of anti-M and anti-TG autoantibodies allow discrimination of thyroiditis.

461

Imaging of Pulmonary Sequestration: Report of Six Cases

Valdes-P, Ceres-L, Alonso-I, Lopez-R Gutierrez-J.A, Hospital Materno Infantil, Malaga, Spain We present six cases of pulmonary sequestration in three males and three females, with ages ranging from three months to 13 years. Imaging consisted on conventional X.R. (n=6), ultrasound (n=3), doppler and colour doppler ultrasound (n=2), C.T. (n=2), M. R.I. (n=2) and arteriography (n-l). We present the different findings in each imaging modality, and discuss their utility. We briefly show the current theories about the embryogenesis of the pulmonary sequestration and another related congenital anomalies (examples of these -adenomatoid cystic malformation, scimitar syndrome are presented as differential diagnosis.). When colour doppler ultrasound was available, we found it a very useful tool in the diagnosis of this pathology, and we include it in the protocol of study when pulmonary sequestration is suspected.

calculi seldom diagnosed in childhood. The high rate of diagnosed sibs suggest, that this is a familiar disease, and for unknown reasons it is most common in Turkey. We present 2 asymptomatic turkish sibs, a 4 year-old boy and his 7 year-old sister, with PAM confirmed by transbronchial lung biopsy. Conventional chest roentgenogram and high resolution CT demonstrated widespread intra-alveolar calcifications of both lungs. The lesions were sharply defined and under 1 mm in diameter. High resolution CT showed high concentration of microliths along the bronchiovascular bundles and in the (sub)pleural parenchyma. The more severe changes in the elder sib suggest, that the lung disease may be progressive in our patients. The described family with the consanguineous, non-affected parents, two affected children and one unaffected child confirms the autosomal recessive inheritance of PAM (Mc Kusick 265100). In addition, the affected girl had clinical symptoms consistent with autosomal recessive Waardenburg II-syndrome (Mc Kusick 206920), raising the question, whether this is an occurrence by chance or a contiguous gene syndrome.

The Antenatal Detection of Infradiaphragmatic Pulmonary Sequestration. Report of 3 Cases and Review of 10 Published Cases

Bronchiolitis Obliterans Organizing Pneumonia (BOOP) in a Child

Baunin-C-H, Puget-C, Railhac-J-J, Chateil-J-F, Diard-F,

Mage-K, Verlhac-S, Dieu-S, Lemerle-S, La-Peyraque-A-L,

Imagerie Pediatrique, Hopital Purpan, Toulouse, France

Reinert-P-H, Service de Radiologie et Pediatrie, Hopital Intercommunal De Creteil, 40 Avenue de Verdun, 94000 Creteil, France

Pulmonary sequestration is part of the spectrum of bronchopulmonary foregut malformation. Usually located in the thorax, it may be found in the retroperitoneum. We report 3 cases of prenatal detection of Infradiaphragmatic Pulmonary Sequestration (IPS) and review 10 published cases. The purpose of this study is to describe the antenatal and postnatal findings in IPS, and to discuss the pathogenic and therapeutic problems. In the antenatal period, fetal ultrasonography detected a suprarenal mass in all 13 cases. In the postnatal period, various imaging modalities were performed, including Ultrasonography, CT scan, MRI and color-flow Doppler. In most cases (10/ 13), the definitive diagnosis was not appreciated, and the primary diagnoses were other more common retroperitoneal masses, such as Neuroblastoma. The definitive diagnosis was made by histology which showed the characteristic changes of Extralobar Sequestration, associated with some histologic features of Cystic Adenomatoid Malformation type 2 in 3 cases. The presence of Extralobar Sequestration requires a search for other congenital anomalies which may be present in up to 60 % of cases. The frequency of associated abnormalities in our study was much less. The natural progression of IPS is unknown, because to date such lesions have been successfully resected. However in one of our cases, surgery has not yet been performed. In this case, the diagnosis was suggested by the demonstration of the feeding vessel using color-flow Doppler. There is no evidence of a clinical or other associated congenital anomaly. The child is now 15 months old. During this follow up period, no changes in the mass has been noticed and no complication has occurred. In summary, IPS, although rare, should be considered in the differential diagnosis of a fetal or newborn suprarenal mass. The demonstration of the feeding vessel can confirm the diagnosis. The major problems result from the uncertainty about the spontaneous evolution of this condition.

Sibs With Pulmonary Alevolar Microlithiasis (PAM)

Schrnidt-H, Lorcher-U, Kitz-R, Zielen-S, Ahrens-R Konig-R, Center of Diagnostic Radiology, J.W. Goethe-University, 60596 Frankfurt, Theodor Stern Kai 7, Germany PAM is a rare lung disease of unknown etiology characterized by progressive formation of innumerable tiny intra-alveolar

A 14-year-old boy was admitted with deteriorating health, the diagnosis was acute lymphoblastic leukemia. Before any treatment, the chest radiograph and CT scan demonstrated bilateral peripheral pulmonary nodules and mediastinal nodes. All the infections samplings were negative, the broncho-alveolar lavage showed 58 % polynuclear. The thoracoscopy-guided biopsy showed a BOOR A steroid treatment was began and the pulmonary opacities quickly disappeared. In 1985 (Epler), idiopathic BOOP was described as a distinct entity: BOOP is defined as granulation tissue plugs within lumens of small airways and granulation tissue extending into alveolar ducts and alveoli. CT scan gives a better assessment of disease pattern and distribution (asymmetric patchy air space consolidation, nodules) than does the chest radiograph and is a guide to optimal biopsy site. BOOP may be due to a variety of causes including inhalation of toxic gases, connective tissue disorders, viral infection, and bone marrow and heart-lung transplantation; however, in most cases of BOOP in adults, no cause is found. In children with malignant disease, BOOP has been reported in a few cases. Most of them were treated for acute leukemia. Conclusion: Lung nodules do not always signal metastatic disease in pediatric patients with known malignancies but radiologic imaging of B O O P is not specific. Histologic examination is necessary for the accurate diagnosis of B O O P

Reliability of Frontal Chest X-Ray in the Diagnosis of Pulmonary Opacities

Patenaude-Y, Blais-C, Leduc-C-R Department of Radiology, Boston Childrens Hospital, Mass, U S A The world health organization (WHO) recommends that the routine investigation of children with suspected pneumonia should not include lateral chest X-rays. However the reliability of the frontal view alone in the diagnosis of pulmonary opacities is not known. We report on the evaluation of the reliability of frontal chest X-rays used alone. Material and methods: We studied prospectively 373 consecutive chest X-rays of children aged from 0 to 17 years, examined for suspected pneumonia. One radiologist interpreted the frontal view during regular clinical activity, and the diagnoses were compared with those

462 of three radiologists who interpreted both frontal and lateral X-rays. The diagnosis to be made was the presence of alveolar opacities. Because there are no gold standard available for the detection of pulmonary opacities, the probability that the radiologist using the frontal X-ray alone would agree as often with the 3 radiologists using both views as they agree between themselves was computed (William's index). Results: William's index for rater reliability was 0,98 for all ages (95 % confidence interval: 0,94-1,00). For children aged < 2 years the index was 0,99 (95 % CI: 0,95-1,00) and for those aged 2 and older 0,96 (95 % CI: 0,94-1,00). Thus a radiologist from an independent group using both frontal and lateral views would agree 98 % of the time with a radiologist using only a frontal view. of the 83 opacities confirmed by group review on both views, 59 were also seen on the frontal view alone. The causes of disagreement were independent of age. In 8 of 24 instances, densities were seen on the frontal view alone but were deemed localized peribronchial confluences, not opacities. The converse was also present where 10 to 27 densities seen on frontal view were not judged opacities by the group using frontal-lateral views. There was a total of 5 opacities seen on lateral view alone and 4 opacities missed on the frontal view alone. Conclusions: The last two decades have seen a change in the pattern of consultation for respiratory disease in children. Parents bring their children earlier in the course of the disease and for many reasons the chest radiographs are sought earlier. Segmental and lobar pneumonias are thus rarer and discriminating the signs of early alveolar filling in early pneumonia is more frequent. The interpretation of bronchial thickening and peribronchial alveolar confluences as opacities is a problem remaining on either frontal or frontal-lateral views. We report no systematic gain in using frontal and lateral views. The lateral yielded only 5 more opacities out of 357 examinations and 19 undetected opacities were not dependant on the use of a lateral view. Detecting a definite pulmonary opacity on the frontal view alone predicts its presence on frontal-lateral views. This study clearly supports the W H O recommendation of using frontal views alone in the evaluation of febrile children with important respiratory symptoms.

Radiographic Chest Evaluation in Broncho-pulmonary Dysplasia in One Year Old Children

Breysem-L, De-Boeck-K, Smet-M-H, Van-Lierde-S, Devlieger-H, Marchal-G-E Department of Radiology, University Hospitals K. U. L., Leuven, Belgium Purpose: To investigate the relationship between the radiographic chest findings, the clinical variables and the pulmonary function tests in one year old patients with a history of neonatal lung disease; Methods: The pulmonary status of 51 infants with neonatal lung disease requiring artificial ventilation is reevaluated clinically and radiographically at the corrected age of one year. Twenty-two patients had developed bronchopulmonary dysplasia. The radiographic score was defined as the presence or absence of cardiomegaly, hyperinflation, emphysema and interstitial lung disease (modified from Toce). Morphometric information such as thoracic width, anteroposterior dimensions of the thoracic cage and insertion angles of the ribs were measured. These findings were correlated with the clinical measurements. Minute ventilation, dynamic lung compliance, in- and expiratory pulmonary resistance were obtained for each patient except one. Statistical analysis was done using the chi square test or the Mann-Whitney-U test as appropriate. Results: The chest X-rays of patients with bronchopulmonary dysplasia showed more frequently hyperinflation, focal zones of emphysema and interstitial lung disease (p < 0,05 for all). Chest depth measured at the level of rib 2, 5 and 8 was significantly smaller in patients with bronchopul-

monary dysplasia (p < 0,05). Chest width was significantly smaller at the level of rib 8 only (p < 0,05). There was a good correlation between clinical and radiological measurements of the thoracic depth and width at the level of rib 5 (resp r=0,44, p < 0,001 and r=0,38, p < 0,006). Inspiratory and expiratory pulmonary resistance were significantly higher in BPD patients (p < 0,05). Conclusions: There is a good correlation between the clinical and radiological measurements of the thoracic depth and width at the level of rib 5. Patients with BPD have significantly more pulmonary abnormalities and a flatter chest on chest X-rays in comparison with non BPD. We suggest that the abnormal pulmonary function can be responsible for these findings.

Absence of Left Pericardium. Report of 18 Pediatric Cases

Hoeffel-J-C, Mainard-L, Worms-A-M, Marcon-E Galloy-MA, Hopital D'Enfants Rue du Morvan 54511 Vandoeuvre Cedex, France Retrospective study of 18 cases of absence of the left pericardium (mostly total). Plain postero-anterior and lateral views of chest radiograph and fluoroscopic evaluation of the heart remain the most essential tools for the diagnosis. In cases of total absence of left pericardium, the heart is shifted to the left, close to the left lateral thoracic wall but there is no intracardiac abnormality. The left cardiac border is markedly hyperkinetic at fluoroscopy. In cases of partial absence of left pericardium, there is a bulging of left middle segment of the heart. MR1 shows the absence of pericardium and confirms the initial diagnosis. Lack of visualization of the left pericardium is visible on MRI associated with interposition of lung between the aorta and the main pulmonary artery. Absence of left pericardium in most instances is an asymptomatic condition and requires no further investigations. Diagnostic pneumothorax is obsolete. The radiologic pattern of absence of pericardium must be well known to the radiologists and would assist in avoiding unnecessary invasive procedures. In case of partial left pericardial defect there may be, as in one of our children who suffered chest pain, herniation of the left atrial appendage. The surgical procedure included excision of the appendage and closure of the defect. Lateral radiograph of the chest would be helpful in differentiating from pectus excavatum.

Congenital Thymic Cysts Two Different Presentations

Unsinn-K-M, Gassner-I, Fink-C, Herzog-B, Fliegel-C, The University Childrens Hospital Innsbruck, Austria and University Childrens Hospital, Basel, Switzerland A rare cause of mediastinal or cervical enlargement in children are congenital thymic cysts. Diagnosis can be established by mediastinal ultrasound, CT or MRI. Complications are infection or hemorrhage. We present two cases of congenital thymic cysts. Case 1 presented in-utero with a cystic mediastinal mass. Postpartum US revealed a cyst within the thymus that increased during the first two years of life to extend from the level of the aortic arch to the diaphragm. Thereafter spontaneous regression occured. Diagnosis were confirmed by CT. Case 2 presented with an indolent supraclavicular mass at the age of 11 years. US and CT revealed a cystic mass of 8 cm in diameter. Diagnosis of an infected thymic cyst was established histologically after surgical removal. We conclude that one should include thymic cyst in differential diagnostic considerations of mediastinal or cervical masses in children. Because of spontaneous regression of the cyst in our first patient, we discuss the possibility to assume a wait and see attitude in asymptomatic cases. Complications (infection or hemorrhage) make surgical intervention necessary.

463 Rare Aortic Arch Complex Anomalies: the 4th, 5th and 6th Aortic Arches - Their Protean Faces

Formanek-A, Csader-M, Haviar-D, Hudecek-M Childrens Cardiocenter, Children's University Hospital, Bratislava, Slovakia Among the many aberrations of the aortic arch complex, the majority are relatively easy to understand and to diagnose. However a few, usually associated with a complex congenital heart defect, harbor unexpected features. As long as the responsibility of the radiologist is to perform and to report angiographic studies, he should be familiar with the anatomy of these unusual anomalies. The purpose of this pictorial essay is to present four very rare anomalies of the aortic arch complex due to faulty development of the 4th, 5th and 6th aortic (branchial) embryonic arches, in each case the embryologic events (only the essential ones) - in words and in pictures -, the relevant imaging technique and radiologic manifestation and the clinical importance of the anomaly are given. The following entities are discussed: 1. Origin of the ductus arteriosus (BotaUi) from the left sub-clavian artery (aberration of the 6th arch). 2. Double lumen (barrel) aortic arch persistence of the 5th aortic arch). 3. Left subclavian artery as a first branch of an right aortic arch (regression of the 4th and persistence of the 5th aortic arch). 4. Interruption of the aortic arch (Type b) and origin of the right subclavian artery from the descending aorta distal to the origin of the left subclavian artery (regression of the 4th arch and persistence of both 5th aortic arches). Lung Perfusion Scintigraphy in Patients With Congenital Heart Disease

Boothroyd-A-L, McDonald-E, Carty-H, Royal Liverpool Childrens NHS Trust, Eaton Road, Liverpool, L12 2AR England Lung perfusion scans requested over a five year period for children with known congenital heart disease were reviewed retrospectively. A total of 102 lung perfusion scans were performed and comparable pulmonary arteriograms were available in 63 children. The scans were requested by cardiologists to assess branch pulmonary artery stenosis (30 cases), suspected pulmonary embolus (31 cases), pulmonary hypoplasia (1 case), pulmonary hypertension (1 case) and an embolised P D A occlusion device (1 case). There was agreement between scintigraphy and angiography in 50 cases and discrepancy in 13 cases. Scintigraphy was more sensitive in detecting small perfusion defects, particularly those seen only on oblique views. Pulmonary arteriography was more specific in identifying the cause of the perfusion abnormalities. The two techniques yield complementary information about lung perfusion. Perfusion scintigraphy is safe in children with congenital heart disease even with known pulmonary to systemic shunting. Many pitfalls could be avoided by careful choice of injection site, by knowledge of previous surgery and comparison with relevant angiograms. Cardiovascular MRI in Children

Castellote-A, Lucaya-J, Vazquez-E, Vera-J, Garcia-Pena-R Casaldaliga-J, Hospital Universitari Materno-Infantil, Vall D'Hebron, Barcelona, Spain In recent years, magnetic resonance imaging (MRI) has emerged as a powerful method for studying the heart and great vessels and has been successfully applied in children. This technique has the ability to obtain images in any plane, to display large field of view and to have natural contrast provided by flowing blood. On spin-echo (SE) sequences the

blood shows little or no signal intensity, while on the gradientrecalled echo (GRE) sequences blood displays high signal intensity. The most common indications of MR imaging are based on morphologic obsenations, although functional analysis of the heart can be achieved with the use of G R E techniques. The authors review their experience in the last two years of 64 cases with cardiovascular anomalies. The age range was from 13 days to 22 years. These cardiovascular anomalies include: aortic coartations (alone or in combination with other congenital heart disease), aneurysmal dilatations of ascending aorta in Marfan's syndrome, vascular rings, agenesis or hypoplasia of central pulmonary arteries, complex congenital heart diseases, pericardial disease, cardiac masses and heteroataxic syndromes. Some postoperative follow-up procedures are included. We present a pictorial review of the most representative cases. Pulmonary Metastases in Wilms Tumor - CXR Versus CT

Wootton-S-L, Rumack-C-M, Albano-E-A, Riggs-J-M, StrainJ-D, The Childrens Hospital and University of Colorado Health Sciences Center, Denver, Co, U S A Purpose: To compare the efficacy of chest radiography (CXR) and chest CT in the evaluation of pulmonary metastases from Wilms tumor. Materials and methods: A retrospective study of 83 patients with Wihns tumor treated from 1980 through 1993 identified 13 cases with pulmonary nodules. CXR's and chest CI's obtained within one month of each other were reviewed in a blinded fashion by three pediatric radiologists. The size, number and location of pulmonary metastases were recorded. A confidence level as to the presence or absence of pulmonary metastases was determined. Pathology, staging, treatment and survival were reviewed. Results: In 81 of 83 patients, CXR and chest CT concurred in the diagnosis or exclusion of pulmonary metastases. In two cases the CXR and chest CTwere discrepant: (1) A nodule identified on CT alone was resected and proved to be a granuloma. (2) A cardiophrenic angle mass was seen on CT only in a patient with diffuse metastatic disease to the liver. Conclusion: Chest radiography is a cost effective and accurate method for the diagnosis or exclusion of pulmonary metastases in patients with Wilms tumor. Chest CT should be reserved for cases in which CXR's are inconclusive or in those patients being considered for resection of metastases. Imaging of Breast Lesions in Infants and Adolescent Girls

Arboucalot-F, Chateil-J-F, Boisserie-Lacroix-M, Diard-F, Service de Radiologie A, Hopital Pellegrin-Enfants, P1. A. RabaLeon, 33076 Bordeaux, France The purpose of this exhibit is to illustrate the spectrum of breast disease in the pediatric age. The first part shows the normal development of the breast in childhood. A large variety of diseases are illustrated and discussed: - Hypoplasia or agenesis of the breast (e.g., Poland disease), - True or false premature thelarche, with or without precocious puberty: pelvic and adrenal sonography is always helpful, - Juvenile gigantomastia: mammary proeminent enlargement due to hormonal changes in puberty, - Adenofibroma is the most frequent breast tumor in adolescent girl; this lesion is usually solitary. Sonography shows an homogenous and hypoechoic mass. Adenofibromas are rarely multiple: adenofibromatosis, Juvenile adenofibroma: This'lesion may be solitary or multiple. It manifests rapid growth, with a large size, with a stromal hypercellularity, differential diagnosis includes cystosarcoma phyllodes - Hyperplastic cystic disease, with multiple regular echo-free nodules, - Retro-areolar cyst is rare, with a cyst behind the nipple. It gives sometimes an abcess; - Breast abcess may occur during

464 the neonatal period, or later in the adolescent age, - Juvenile papillomatosis, which constitutes a risk for breast cancer, - Malignant mammary masses (e.g. rhabdomyosarcoma metastasis, Hodgkin disease) have to be suspected when the tumor is heterogenous, multiple. Childhood breast carcinoma is exceptional. Aspiration cytology is helpful for the diagnosis when a malignancy is suspected. Malignant breast lesions are rare in this age group. Clinical history and sonographic findings are helpful for the diagnosis. Mammography has not to be performed in most of cases. Conservative treatment by clinical observation or local excision is advocated for most patients. Biopsy during early life has to be avoided, if possible, because of risk of secondary breast hypoplasia.

Radiological Evaluation of Patellar Malalignment Using a New Frame For Axial Imaging of the Patella

Vahasarja-V, Lanning-R Kinnunen-R Serlo-W, Departments of Diagnostic Radiology and Paediatrics, University of Oulu, Oulu, Finland Patellar malalignment is a common cause of knee problems: in children and adolescents. The problem in diagnosing and treating the patellar-malaligmnent is that there is not any standard radiological method generally accepted. The purpose of this study was to create a standard method for visualisation and measuring of radiological values of the patellofemoral articulation in patellar malalignment. The study group compromised 53 girls and 6 boys aged 8 to 17 years (mean age 13.2 years) with patellar malalignment that had not been operated on. An axial roentgenogram was exposed using a special frame keeping the knees in 20 degrees flexion during imaging. The x-ray tube was directed in a cephalad direction and the frame stabilised the cassette. The images were produced with the patient supine and the quadriceps muscles relaxed. The following indices were measured from the x-ray films: The sulcus angle (SA), the lateral patellofemoral angle (LPA), the lateral patellar displacement, lateral patellar tilt (LPT) and the Insall index (Ii). 79 of the knees were symptomatic and 39 asymptomatic. 19 had bilateral symptoms. The mean LPA was significantly smaller, and the mean LPD and LPT were significantly greater in the symptomatic group. There was no significant difference of the mean SA between symptomatic and asymptomatic knees. The mean Ii for symptomatic knees was 0.78 (SD 0.13), normal values being 0.99 (SD 0.084). Our conclusion is that the Laurin's technique used with a special frame keeping the knees in 20 degrees flexion can give standard conditions for the measurements of radiological indices in the diagnostics and in the planning of realignment operation for patellar malalignment.

Persisting Pain in the Hip in Children: the Value of MRI (Fat Suppressed Sequence)

Minio-Paluello-G-B, Paesano-P-L, Del-Maschio-M, De-Gaspari-A, De-Pellegrin-M, Del-Maschio-A, Department of Radiology, Scientific Institute, University of Milan Raffaele, Milan, Italy Purpose: To assess the MRI features in persisting pain in the hip in children. Materials and methods: 14 consecutive patients aged from 1.6 to 15.2y (mean 6.5) were selected for the study. At clinical examination all patients had a limp who cannot bear weight on one leg, or had other symptoms suggesting pain in the hip persisting after 5 days of anti-inflammatory therapy. All patients were studied with x-rays, sonography, and MRI (0.ST). Coronal plane on SE T l W sequences, SE T l W fat suppressed sequences (SEFS), SE T2 W sequences and gradient-echo (RGB) T I W like (90 degrees) were obtained. Slice thickness were 5-3 mm respectively for SE and

G R E sequences. Prospective evaluation of MRI findings were made on morphology and on signal intensities of epiphyses and metaphyses. Conclusive diagnosis was assessed by means of clinical and imaging follow-up (6 months). Results: 2 Pts, had transient synovitis, 5 Pts. had Perthes disease, 2 Pts. had Meyer dysplasia, 1 Pt. had initial slipped capital femoral epiphysis, 1 Pt. had fracture, 1 Pt. had extraarticular cause of pain and 2 Pts. had not any detectable cause. In all cases (14/ 14) MRI helped to confirm or establish clinical, x-ray and / or US features. MR1 was particularly helpful in confirming the diagnosis in two cases: 1) A case of suspected Meyer dysplasia. MRI revealed early Perthes disease 2) A case of suspected slipped capital femoral epiphysis. MR1 revealed an incomplete fracture. SEFS sequences improving contrast, especially in bone marrow, were better than SE T1 W and G R E T1 W like sequences. Conclusions: MRI, especially using fat suppressed imaging, in persisting symptoms localized in the hip, permits to detect the presence or absence of pathology and confirm the clinical, X-ray and ultrasonographic features changing diagnosis and therapy.

Results of Systematic Post Mortem Radiographic Study in Perinatal Death

Ben-Romdhane-H, Sellier-N, Bucourt-M, Combier-E, CodercE, Callard-P, Hopital Jean Verdier, 93 143, Bondy, France Based on a public health program, organized under the care of the General Council of our department (Seine St Denis France), we have taken in our institution, in the analysis of 550 perinatal death occurring between 20 and 41 weeks of amenorrhea. The authors analyze the value of systematic skeletal radiography for the final diagnosis (overall value of 50 %). The results were correlated with clinical, antenatal US, chromosomal and foeto-placental data. In some cases, especially for the central nervous system or spinal diseases, postnatal US or CT bring further informations. Special attention was given to the bone maturation, growth and structure and mineralisation. Such anomalies were found in 40 %. In this epidemiologic study, intra uterine growth delay is the main data, related to insufficient obstetrical follow-up. Bone maturation curves are now helpful for antenatal ultrasound and maturation advances are mainly found in chromosome 21 Trisomy syndrome. In infectious diseases, skeletal X ray is normal in 55 %. The incidence of bone dysplasia is 1.1%; those of chromosomal abnormalities is 9.1% and those of malformations is 11.5 %. Such program assists genetic councelling, sonographic screening, obstetrical follow-up and furthermore leads to decrease of perinatal mortality.

Metacarpophalangeal Pattern Profile Analysis as an Aid in Recognizing Dyschondrosteosis

Rosenborg-M, Department of Pediatric Radiology, St Ooran Childrens Hospital and Karolinska Hospital, Stockholm, Sweden Dyschondrosteosis (Leri-Weill syndrome) is inherited as an autosomally dominant bone dysplasia. It is characterized by a mesomelic short stature and a Madelung deformity of the wrist. The expressiveness is very variable, and mild cases often remain undiagnosed or become apparent by chance when the wrist for other reasons is radiographed. The Madelung deformity can also be seen as an isolated finding. The isolated wrist deformity and dyschondrosteosis are regarded as different phenotypes of a common genetical defect. Pattern profile analysis of the hand according to Poznanski has proved useful in the diagnosis of certain bone dysplasias. We use the method in routine work-up in dealing with skeletal dysplasias and congenital malformation syndromes. For practical reasons we have

465 computerized the method which makes it more useful and very much less time consuming. Having analysed thirty odd cases, we propose two metacarpophalangeal profile patterns, which might reflect different genetic expressions of this syndrome. We consider the method as a valuable tool in mildly expressed cases and for screening of relatives.

Chronic Familial Hyperhosphatasemia: Report of a Case

Kararnan-C, Oezdemir-S, Bilaloglu-P, Kizilcan-M, Tamac-N-I, Department of Radiology and Pediatric Clinics of Ankara, Hospital of The Turkish Ministry of Health, Ankara, Turkey Chronic familial hyperphosphatasemia is a rare autosomal recessive disorder characterized with an increased turnover of the membranous bones. A 12 year-old girl was admitted to pediatric clinics with a history of delayed development, bowing of her legs and hearing loss. Her physical examination revealed dwarfism, macrocephaly and gross skeletal deformities. Abnormally increased serum alkaline and acid phosphates values were found. The radiographic findings were as follows: In the skull, widened diploe was observed, there was enlargement of the medulla, and thinning of the cortices in the tubular bones of the extremities. The diaphyses were thickened and bowed especially in the femora and tibiae. Audiometry showed bilateral conductive hear loss. The patient's clinical, biochemical and radiological findings were compatible with the diagnosis of chronic familial hyperphosphatasemia. External fixation for the bowing of tiNae, and calcitonin, 100 U/day was administered. By reviewing the recent literature, the diagnostic features and the treatment of chronic familial hyperphosphatasemia will be discussed.

Hip Sonography in Infants at Risk - Correlation With Radiography

Donoghue-V, Murphy-J-F-A, Mc-Mahon-A, Mc-Manus-F, Departments of Radiology, Neonatology and Orthopaedics, National Maternity Hospital, Holles St. and The Childrens Hospital, Temple St. Dublin, Ireland We have performed hip ultrasonography at day 3 of life, and at 6 weeks in 63 infants (126 hips) 'at risk' for congenital dislocation of the hip (C.D.H.). These included infants with a first degree relative with C. D. H., Breech presentation and "click" at birth. All infants had a hip radiograph at 4 months of age. No infant had treatment between birth and 4 months. The sonographic technique reported by Graf was used. There was correlation between the sonographic exams at 3 days, 6 weeks and the hip radiograph in 102 hips. Type II a- hips reverted to normal in 7. 3 Type II c hips at Day 3 reverted to normal at 6 weeks. In 3 hips the pattern changed from Type I a at 3 days to II a- at 6 weeks and a normal radiograph at 4 months. 4 normal hips at Day 3, reverted to Type II c at 6 weeks and had acetabular angles of 35 degrees or more at 4 months. All continue to improve without treatment at 9 months. 7 hips had Type II c or I I I a at Day 3. This pattern continued to improve at 6 weeks, 4 months and 6 months without treatment. In conclusion, we feel that the state of the hip on sonography is better assessed at 6 weeks than Day 3 of life. Though our numbers are as yet small our study also suggests that treatment recommended by Graf based on his classification leads to over treatment. It also confirms that there are as yet a number of factors we still do not understand about the development of the acetabulum and hip instability.

Neonatal Hip Sonography From Anatomy to Sonography

Gomes-H, Ouedraogo-T-H, Bakhache-P, Adnet-J-J, Pediatric Imaging Department, Chr Reims 51100, France

Ultrasonography has gained wide acceptance for the diagnosis of hip dysplasia, however the neonatal screening remains controversial. Objective: 1) understanding specific anatomic patterns of the perinatal hip; 2) checking if these are taken in account by the current US techniques; 3) defining a reliable US approach. Subjects and Methods: Two studies were simultaneously run : * anatomic study of 20 post mortem hips, 8 fetal (> 32 weeks G.A.), 6 newborn, 6 infants (age: 2-3 months), neonatal morphologic and dynamic US screening of 7750 hips. US predictive value was assessed on 1370 instable and 1000 stable hips left untreated and revisited at 1 month by US and 4 months by X-rays. Results of the anatomic study: Major specific patterns characterized the neonatal hip : the quite exclusive cartilaginous nature of the acetabulum and absence of a bony cavity which appeared well after birth, the importance of the osseous components which even if very small gave the cavity stiffness and solid cover, but mainly the progressive bulging of the posterosuperior osseous roof which revealed fundamental for the femoral stability. In the fetuses, large posterosuperior uncovered areas could be easily put out of shape leading to femoral head dislocation. Whereas in the neonate solid osseous covering prevented any deformity. Thus, neonatal pathologic hip appears to be a persistent fetal cartilaginous type configuration, with a lack of adequate bony coverage. This, featuring the deformability of the structure leading ultimately to hip dislocation. Investigating the neonatal hip should therefore emphasize the development of the postero superior bony area along with the deformability of the uncovered cartilaginous mass. Results: Bony coverage was best appreciated by coronal morphologic examinations (i. e. Graf's technique). However the deformation range in this coronal plan was quite small due to the fact that major alterations took place more posteriorly. Three dimensional cartilaginous deformation can hardly be visualized but it can be deduced from the femoral head position after stress manoeuvre. Many dynamic techniques have been described. Their limitations being, inaccurate stress, small interfaces and above all 3D components of the head shift. Precise head localisation requiring biplanar investigations, no single technique was able to represent simultaneously the three vectors of the movement. Most of the dynamic studies showing only abduction were of limited value. The accuracy of US approach was determined on the follow-up of more than 2000 hips. Despite the previously mentioned technical limitations the simultaneous use of morphological and dynamic examinations had a good sensitivity (99.5 %) and a good positive predictive value (95 %), the pathological hips representing 1% and the doubtful 5 % of all cases. Conclusion: Neonatal hip US presents technical difficulties due to specific anatomy, but the simultaneous use of morphologic and dynamic evaluation turns out quite accurate for neonatal screening.

Analysis of Field Size in Chest Radiographs of Premature Babies and Older Infants

Schneider-K, Fichtner-C, Horwitz-A-E, Kohn-M-M, Endemann-B, Klinikum Innenstadt der LMU, Dr. Von Haunersches Kinderspital, Lindwurmstr. 4, D-80337, Munich, Germany A Working Document on "Quality Criteria for Diagnostic Radiographic Images in Paediatrics" was published in 1992 by the Commission of the European Community. Definitions for field size of x-ray films were given, but were imprecise. The purpose of this study was to evaluate the practicability of these definitions for field sizes for chest x-rays of infants. The field size of the original radiographs from 89 children's clinics participating in a Europe-wide survey were measured for this analysis. Evaluation of the chest films showed extensive variations in field size, ranging from too narrowly collimated fields to complete babygrams. Correctly sized films were rare. The

466 ideal field size could often not be measured because the upper field border was cut off. Positioning of the lower border was often too low. Based on the CEC-Document definitions, roughly 10-25 % were within the recommendations, 70-85 % of the films were poorly collimated and close to 20 % of chest x-ray films of prematures exceeded an ideal field definition by more than 200 %. With respect to the radiation protection of the paediatric patient such variations are alarming because chest x-rays are the most frequent X-ray examination in this age group with a high repeat rate. More precise definitions for stricter collimation of the X-ray field are absolutely mandatory. Further quality control and assurance measures must be taken and present measures should be improved to increase image quality and radiation protection of paediatric patients.

Placentomegaly, Massive Adrenal Hyperplasia, Cystic Hamartoma of the Lung, and Multiple Mesenchymal Hamartomas of the Liver: A Variant of the BeckwithWiedemann Syndrome?

Eich-G-F, Stallmach-T, Willi-U-V, Division of Radiology, University Childrens Hospital, and University Hospital, Zurich, Switzerland The case of a premature born female baby is described. At the 22nd week gestational age placentomegaly was noted. Cesarian section was performed in the 31st week due to preeclampsia. Histologic evaluation of the placenta revealed massive hydrops of the stem villi without associated trophoblast hyperplasia, a finding which may be associated with the BeckwithWiedemann syndrome. On examination of the newborn baby a small omphalocele and a mass in the left upper quadrant of the abdomen were found, macroglossia or gigantism, however, were not present. Investigation by US and CTrevealed a left suprarenal mass with a "cerebral" texture. Neuroblastoma was considered and a 6 cm mass was removed. On histological examination a massive adrenal hyperplasia of the fetal cortex was found without evidence for malignancy. Such adrenal hyperplasia had been described previously in cases with Beckwith-Wiedemann syndrome. Within the lower lobe of the right lung an air-filled cystic lesion was noted at birth on radiography and CT. This lesion slowly enlarged over time to 5 cm diameter, compressing the rest of the right lung. Therefore resection had to be performed at 2 months of age. Histology showed a partially cystic and solid Hamartoma. The liver was enlarged at birth but homogenous in texture on ultrasound. Follow-up ultrasound and CT studies, however, demonstrated the appearance of multiple enhancing and partly cystic lesions within the liver. One of these lesions enlarged to 9 cm in diameter. Because malignancy could not be ruled out a partial hepatectomy at the age of 8 months was performed, Histology showed a mesenchymal hamartoma with hemangiomatous elements. The combination of the multiple tumor-like conditions in this infant is unique. Some parallels to the Beckwith-Wiedemann syndrome exist. However, the clinical appearance does not fulfill all the requirements for this syndrome. Therefore we would like to present the findings in this infant as an "unknown," the etiology of which still has to be elucidated.

Failure of Postprandial Gallbladder Contraction in Active Coeliac Disease

Kovi-R, Arato-A, Micskey-E, Szonyi-L, Kis-E, Department of

lenge, as well as in 15 controls. Less than 50 % decrease in the volume of the gallbladder postprandially was regarded as impaired contraction. In children with active coeliac disease the postprandial volume was significantly higher compared to that of controls (77 + 4.2 % of the volume on an empty stomach, mean _+SEM vs 35 + 3.1%, p 0.01). In contrary, on a gluten free diet the gallbladder contraction (32 + 2.6 %) was similar to that of the controls, and in all but one children the gallbladder function was normal. In children after gluten challenge, in whom no symptoms of malabsorption was observed, the gallbladder contraction was only slightly decreased (42 + 5.6 %). We infer that in active coeliac disease mucosal damage reducing the secretion of cholecystokinin-pancreozyrain may lead to the impaired contraction of the gall-bladder. Decreased output of bile may contribute to the steatorrhea in coeliac disease.

Congenital Tuberculosis Complicated by Portal Hypertension

Hara-A, Kondoh-S, Itoh-M, Ishida-H, Hayashi-A, KamagataS, P Kiyose Childrens Hospital, Kiyose-City, Tokyo, Japan Congenital tuberculosis is very rare, but remains critical in infants. Congenital tuberculosis has been thought to be acquired in two ways; transplacentally via the umbilical vein, inducing a primary liver complex, and aspiration or swallowing of infected material, resulting in primary complex either in the lungs or in the gastrointestinal tract. Congenital tuberculosis is known to be a cause of portal hypertension. Detecting a primary complex in the liver is important for early diagnosis. We present a rare case of congenital tuberculosis complicated by portal hypertension well demonstrated on CT. A one month old female infant was referred to our institution with abdominal distention, fever, and tachypnea. Her initial radiograph of the chest and abdomen on admission revealed diffuse miliary shadows in both lungs, and marked hepatosplenomegaly. Unenhanced CT scan of the abdomen at the time of the admission showed multiple calcifications in the liver, spleen, and at the area of the porta hepatis, suggesting congenital tuberculosis. A mantoux reaction was positive. Her mother had been well during this pregnancy, and had been febrile immediately after the delivery. Tuberculous lesions were proved from mother's uterine mucosa by biopsy. Positive tubercle bacilli culture result was obtained from gastric fluid four weeks after infant's admission. Then the diagnosis of congenital tuberculosis infected via the umbilical vein was made. She was treated with antituberculous therapy, and was getting better until nine months of age. The second CT at five months of age demonstrated increased calcifications in both number and size in the same area as before. At one year of age, hematemesis began due to ruptured esophageal varices. A superior mesenteric arteriography revealed an obstruction of the portal trunk with cavernous transformation, and multiple varices in the coronary vein, and the esophagus. She died at one year and nine months of age. Numerous tuberculous lesions were obtained in the lungs, liver, spleen, porta hepatis, and lymph nodes at autopsy. The portal trunk was almost completely obstructed by tuberculous granulomas in the porta hepatis. CT clearly demonstrated tiny calcifications in the primary complex of congenital tuberculosis, and was very useful for both early' diagnosis and follow up examination.

Radiology, Semmelweis Medical University, Budapest, Hungary

Cystic Fibrosis: Qualitative and Quantitative MR Evaluation of the Pancreatic Lesion

We studied the postprandial gallbladder contraction in response to a test meal with ultrasound examination in 8 children with active coeliac disease before dietary treatment, in 22 children on a gluten free diet and in 5 after a gluten chal-

Ferrozzi-F, Castriota-Scanderbeg-A, Campodonico-F, DeChiara-F, Bacchini-E, Battistini-A, Instituto Di Scienze Radiologiche, Ospedale Sollievo Della Sofferenza, S. Giovanni Rotondo, Italy

467 Purpose: To evaluate the role of Magnetic Resonance (MR) in the detection and quantification of pancreatic damage in cystic fibrosis (CF); to correlate the MR findings with clinical data of esoendocrine insufficiency related to the disease; to verify the relationships between the genotype and pancreatic lesion degree. Materials and methods: 29 patients affected by CF, aged from 5 and 34 years (mean 15.1 years) and 15 normal subjects were evaluated by M R (0.5 T unit). Abdominal transaxial SE T1 -weighted (TR 500 TE 30) and mixed (TR 500 TIE 30 T R I R 1150 T1 150) sequences were performed. Water was orally administered as the contrast medium (350-500 ml.) before each MR examination. Functional tests (fat excretion and insulin -response evaluation) were performed to determinate exocrine and endocrine status. Genetic study of the most frequent mutations (F 508, G542 X, R553 X, 1717-1-G-A) was also included. Results: M R images show 4 different patterns of pancreatic involvement. A) diffuse hyperintensity of variable degree with residual lobular pattern (14/29); B): homogeneously hyperintensity without lobular pattern (2/29); C) hyperintensity with focal areas of hypointensity (3/29); D) no structural or signal intensity changes, generally associated with glandular hypotrophy (9/29). Quantitative calculation revealed a reduction of Tl-numbers between 2 and 63 % versus normal subjects. Conclusions: statistical analysis demonstrated high correlation between the pancreatic endocrino-esocrine insufficiency and MR findings. The mutation F 508 homozygote shows a significant correlation with exocrine lesion.

Percutaneous Transhepatic Balloon Dilatation (PTBD) of Post Transplant Biliary Strictures in Reduced Liver Grafts

John-P-R, Kelly-D-A, Mayer-A-D, Buckels-J-A-C, Radiology

turbance and immunosuppression. Orthotopic liver transplantation is increasingly common in children for both fulminant hepatic failure and end stage chronic liver disease and imaging is often requested for varied neurological indications e. g. depressed consciousness, seizures, focal signs and sepsis. We analysed our experience of Cranial CT in children from the Kings College Hospital transplant programme, 1988 to 1993, where 81 children (36 boys:45 girls; age 4 weeks to 16 yrs. mean age of 7.0 years ) received 104 transplants with a patient survival of 80 % (65 children). 14 patients had 25 cranial CT scans post transplant, from 4 days to i year post graft (8 within 1 month, 3 further within 2 months, 3 at 6 months to I year.) All scans were reviewed by a neuroradiologist. 5 patients had "Normal" scans 4 patients had Generalised Atrophy (rated moderate to severe) 2 had multifocal abnormalities: - 1 Lymphoma, - 1 Leucoencephalopathy. 3 had focal abnormalities: - 1 Infarct, - 1 AV malformation with haemorrhage, - 1 Intracerebral haemorrhage(? AVM). We reviewed these results with respect to reason for graft, presenting neurology and time after transplant. Comparison was made with any pre transplant CT's. A review of the literature suggests we are seeing many fewer infective lesions .e.g. Aspergillus, than in the adult and previously described child populations and also less ischaemia and myelinolysis. This is almost certainly related to increasing clinical expertise and prompt recognition and correction of hypotensive episodes, electrolyte imbalance and better management of immunosuppression and infection prophylaxis. MRI will play a larger role in the future, specially in the assessment of white matter disease

Rhabdomyosarcoma of the Biliary Tract in Children: Report of Three Cases

Department and Liver Unit, Children'S Hospital, Birmingham, UK

Lopez-R-P, Valdes-P, Ceres-L, Alonso-I, Hospital Materno In-

Interventional radiology plays an important role in the management of children undergoing liver transplantation. To date there are few reports on the percutaneous management of post transplant biliary strictures in young children with reduced grafts. The aim of this study is to describe our early experience of PTBD in such grafts. Methods: Case notes, imaging and interventional procedures, pathology and laboratory data were retrospectively studied on our first 3 children undergoing PTBD in the past year. Results: PTBD was performed on 3 reduced (segments 2 and 3) grafts in 3 children (aged 2wks-3yrs at transplant) 4 months-l.5 yrs post transplantation. Graft ischaemia was present following transplantation in 1 case. Prior to PTBD all had progressive intragraft biliary dilatation on ultrasound with elevated serum alkaline phosphatase levels (> 1700 iu/1). Cholangitis was present in 2. High common duct strictures (present in all at PTC) were successfully dilated with "coronary" and "conventional" balloon catheters. At follow-up (1-7 mths) all are generally well and 2 have complete resolution of biliary tree dilatation on ultrasound with reduced alkaline phosphatase levels by 1500 iu/1. Conclusion: Our early experience in young children shows PTBD can improve biliary drainage across reduced liver graft biliary strictures. This may be a useful alternative to conventional surgery.

Biliary tract rhabdomosarcoma is an unusual tumor. We report three cases seen at our institution between 1.987 and 1.993. All cases were studied with U.S., C.T. and M. R. I. The image findings wilt be discussed, and the differential diagnosis with other hepatic tumors will be established.

Cranial CT in the Imaging of Children With Neurological Symptoms Post Liver Transplantation

Witham-F, Haigh-S-E King-S-J, Razak-M, Dawson-J, Kings College Hospital, London, UK Neurological complications are a significant cause of morbidity and mortality after all forms of organ transplantation because of vascular insults, haematological and electrolytic dis-

fantil, Malaga, Spain

Imaging in Splenic Abnormalities

Noel-B, Taghreed-T, Children's Research Centre, Our Lady's Hospital For Sick Children, Dublin, Ireland In this presentation, we briefly review intrinsic abnormalities of the spleen detected in a ten year period at Our Lady's Hospital for Sick Children up to 1993. Cases of generalised splenomegaly due to leukaemia, portal hypertension and systemic inflammatory lesions are excluded due to lack of significant radiological features. The remaining thirteen cases are as listed below: Accessory spleen - 2 cases; cysts - 1 small, 2 giant; trauma - 5; malignant lesions - 1 histiocytosis, 1 Hodgkins nodule. Such findings are well documented and illustrated in the radiology literature. However, the role of imaging including sonography, radionuclide scan and computed tomography followed by interventional drainage is briefly described in i giant splenic cyst. One other case, with a false sonographic sign of splenic laceration with ascites following trauma is also described and illustrated. Peritoneal fluid in a deep normal splenic notch simulated laceration and the actual source of haemeperitoneum was a vertically disposed laceration high in right lobe of the liver around the midline. Awareness of this pitfall is stressed along with reinforcing the need for computed tomography in cases of severe abdominal trauma.

468

Recurrent Abdominal Pain. Recognition of Chronic Mesenteric Lymphadenitis by Ultrasound

Malignant Blue Naevus in a Child of 7 Years - MRI Appearances

Metreweli-C, Bergman-J, Sullivan-P Department of Diagnostic

Maskell-T, Lamont-A-C, Shannon-R-S, Leicester Royal Infirmary, Leicester, UK

Radiology and Imaging, Chinese University of Hong Kong Prince of Wales Hospital Shatin, N.T., Hong Kong Recurrent abdominal pain (RAP) (as defined by Apley) is a common problem yet has been a long running mystery in paediatrics that has not been significantly altered by diagnostic imaging until now. Only a small number of children will have some structural abnormality such as duplication, mesenteric cyst, malrotation and urinary tract abnormalities but the large majority of children have so far been considered to be "normal". We have noted in 30 mainly Chinese children with R A P (Apley criteria) that 65 % of them have enlarged mesenteric lymph nodes i. e. chronic mesenteric lymphadenitis. Mesenteric nodes are visible in most children but are usually below 3 mm short axis diameter. Nodes over 5 mm in diameter are found in children with chronic viral, bacterial and protozoal infections. Therefore some children with R A P may be suffering from chronic infection or allergy. Although our figures are relatively small there appears to be three groups of children that can be identified. Those who complain of upper abdominal or epigastric pain in whom nodes are normal in size. Those with periumbilical pain who tend to have left sided mesenteric lymphadenitis and those with hypogastric pain who tend to have right sided mesenteric lymphadenitis. Ultrasound identifies these children in whom a more diligent search for the cause may be indicated and prevent the wrongful attribution of psychogenic causes to be blamed on the child (or its parents).

The Radiologic-Pathologic Correlation of Aids in Children

Wolfson-B-J, Faerber-E-N, Feingold-A, Raphael-S-A, Kochhar-O-S, Crisci-K, Geller-E, Smergel-E, Greenberg-B, St. Christophers Hospital For Children and Temple University School of Medicine, Philadelphia, Pa, USA AIDS is an increasing cause of morbidity and mortality in infants and children. Several infections, such as pneumocystis carinii and atypical mycobacterium, are common causes of illness or death in these children. Secondary fungal or bacterial infections also occur but are less common. HIV infection itself is known to cause damage to the brain and may damage other organs as well. Examples of these infections will be presented correlating multimodality imaging with the pathologic specimens. Entities shown will include H I V infections of the brain, nephropathy associated with HIV, lymphoproliferative disorder of the distal ileum and atypical mycobacterium of the abdomen.

Malignant Lymphoma in Children - Ultrasound Diagnosis

Brzewski-M, Gadomski-A, Biejat-A, Marcinski-A, Departament of Pediatric Radiology and Department of Pediatric Oncology, Warsaw Medical Faculty, Marszal Kowska 24, 00-576 Warszawa, Poland Between 1986 and 1990, 65 children with proved malignant lymphoma were examined by sonography as a part of the initial staging of the disease. The authors describe a rational investigative protocol and place of ultrasonography in this protocol. Our clinical material, based on the staging classification is as follow: I stage - 5 children, II stage - 10 children, III stage 25 children, IV stage - 25 children. Mediastinum, solid abdominal viscera, abdominal and pelvic lymph nodes, thyroid, testes and female genital organs were investigated by ultrasound. The analysis of ultrasound semiology of malignant lymphoma is present.

Malignant blue naevus is a rare tumour of the skin which may arise de novo or from a pre-existing cellular blue naevus, it has usually been regarded as an unusual, and less aggressive form of malignant melanoma. However there are a number of case reports of both local and metastatic spread, supporting the contention that this is an extremely aggressive neoplasm. It is an unusual tumour in children. We present the MR findings in the case of a 7 year old boy with malignant blue naevus of the scalp and metastatic spread to regional lymph nodes.

Oncologic Emergencies: Cardiac Compression in Mediastinal Masses

Occhi-M, Oddone-M, De-Caro-E, Calza-G, De-Bernardi-B, Toma-R G. Gaslini Institute, Genova, Italy Because of the potential life-threatening effects of malignant pericardial effusion and cardiac compression effective therapy can prolong survival and permit an appropriate treatment of the underlying disease. In the presence of cardiac mechanical complications of mediastinal masses the goal of the first therapy is the palliation of symptoms. Nevertheless a general consensus regarding the therapeutical approaches has still to be agreed upon by surgeons and clinicians. So far a variety of treatment approaches have been reported in the literature: catheter drainage of pericardial effusions pericardial surgery aggressive chemotherapy or radiotherapy without pericardiectomy. The authors report their experience in the management of complicated mediastinal masses imaged by US CT and MRI. The underlying diseases were: non Hodgkin lymphoma (2 cases), rhabdomyosarcoma (2), metastatic Wilms' tumor (1). In two cases a thoracotomy with pericardial window was performed: this approach allowed a histologic diagnosis of the masses and no postoperative complications occurred. In the other cases aggressive chemotherapy was given with good results in terms of a prompt reduction of mass size and improvement of clinical condition. In our experience rapidly growing masses of unknown histology require surgical approach both for diagnostic biopsy and if necessary for safe drainage of pericardic effusion. Sometimes however, bone marrow aspirates and/or bone biopsies are available to confirm clinical, radiological data suggesting lymphoma; in this case prompt aggressive chemotherapy is the treatment of choice.

Spontaneous Reduction of Intussusception Documented by Sonography

Papadopoulou-F, Efremidis-S, Georgopoulou-V, Kouri-A, kalovoulos-M, Hippokration General Hospital, Thessaloniki, Greece Three cases with spontaneous reduction of intussusception are presented All patients had clinical and sonographic findings highly suggestive of intussusception. The first patient went for surgery and no intussusception was found. In the second patient therapeutic barium enema documented the spontaneous reduction since no intussusception was demonstrated. Follow up sonography confirmed the absence of intussusception. In the third child reduction occurred during diagnostic sonography. The size of intussusception was seen to be gradually diminishing and a few minutes later only the thickened iliocolic valve was seen. Rapid and obvious clinical improvement accompanied the spontaneous reduction in the last two cases. We conclude that spontaneous reduction of intussusception

469 should be always considered in cases of rapid clinical improvement of a child with recently diagnosed intussusception. Spontaneous reduction can be documented by sonography.

Prostaglandin Induced Gastric Mucosal Hyperplasia and Outlet Obstruction in Infants

Kjellin-I-B, Young-L-W, Loma Linda University Childrens Hospital, Division of Pediatric Radiology, 11234 Anderson Street, Loma Linda, California 92354, USA E-type prostaglandins are useful in managing infants with ductus dependent critical congenital cardiac defects. A n association between prostaglandin E 1 therapy and gastric outlet obstruction from antral hyperplasia was recently reported by other investigators. We provide information of our experience at L L U C H with this complication in particular its radiographic and sonographic appearances. Materials and methods: 133 neonates and infants at L L U C H who received prostaglandin E~ therapy between November 1985 and April 1993 were evaluated retrospectively for radiographic and/or sonographic evidence of gastric outlet obstruction. Results: 17 infants underwent upper gastrointestinal radiopaque exams. In eight infants there were definite gastric antral abnormalities including narrowing and thickened mucosal folds. Two infants showed elongation and narrowing extending to the pylorus. Seven infants had suggestive gastric antropyloric findings. Two infants had suboptimal visualization of the antropyloric region. The radiographic and sonographic findings were confirmed intraoperatively in two infants. Conclusion: The large experience at L L U C H with prostaglandin E 1 therapy in infants with ductal dependent congenital heart disease confirms the significant complication of prostaglandin E a induced gastric antral hyperplasia. This paper contributes additional evidence for raising the index of suspicion to recognize the clinical signs and to perform confirmatory imaging examinations for prostaglandin E 1 gastric outlet obstruction.

Post-Traumatic Duodenal/Jejunal Haematomas in Children

Miron-M-C, Pracros-J-R Filiatrault-D, Foray-R Grimard-G, H6pital Debrousse, Lyon, France and Hopital Ste-Justine, Montreal, Canada Duodenal/jejunal haematoma is an uncommon disorder which usually follows blunt abdominal trauma which can be mild or even initially forgotten. The clinical presentation is non specific. Accurate radiological diagnosis is important as conservative management is the rule if there is no intestinal perforation or severe associated lesion. Aims: The aims of this study were to describe the clinical presentation, causes, localization, radiological investigation, and treatment of duodenal and small bowel haematomas. Material and methods: From 1980 to 1992, ten cases of duodenal/jejunal haematomas were seen at H6pital Debrousse, Lyon and H6pital Ste-Justine, Montreal. Six girls and four boys aged from 2 to 9 years old were reviewed. Results: Vomiting is the most common symptom and abdominal guarding is the most frequent clinical sign. Fever does not exclude the diagnosis as three patients presented with a febrile episode. In only four cases the traumatic etiology was evident. Upper GI studies were performed at presentation in seven patients; all patients presented complete obstruction at the site of haematoma. Six haematomas were located at the duodenum, one at the proximal jejunum and three at both duodenum and jejunum. All patients were investigated by US. They all presented with a heterogeneous mass. No associated lesion at US was noted although two patients presented with pancreatic contusion at CT scan, one of these had also a pancreatic pseudo cyst. Five patients were treated conservatively

with an average hospital stay of nineteen days compare to the average hospital stay of thirteen days for the four patients treated surgically. One patient was managed both conservatively and surgically. Conclusions: Clinical presentation in haematomas of duodenum and jejunum is non specific. Vomiting is the most common symptom. Fever does not exclude the diagnosis. In face of acute abdomen consider intestinal trauma. The majority of haematomas are located at the duodenum. US is the imaging modality of choice. CT scan can be helpful to describe the associated lesions and to support the management. Upper GI studies are useful in patient followup. Conservative treatment is the rule for non complicated intestinal haematoma although this treatment can be reassessed after a few days.

"Blue Nevus" Transdiaphragmatic Duplication of the Alimentary Tract

Zupancic-Z, Brencic-E, Department of Radiology University Medical Center, Ljubljana, Slovenia Transdiaphragmatic duplication (TD) of the alimentary tract is a rare congenital anomaly with the duplication elements above and below the diaphragm. It is an early embryological error occuring at three to six weeks of development. The cause is uncertain but some explanations of the pathogenesis are offered: TD can be explained by the endodermal-ectodermal adhesion or by the split notochord theory. As we diagnosed TD in a six month old girl we present the imaging studies with surgical and pathologic findings of the anomaly. Patient: A 6 month old girl came to the hospital because of intermittent vomiting for three months. Physical examination and laboratory values were normal. The vomiting stopped with antireflux upright positioning. Ultrasound (US) of the abdomen as the first diagnostic study revealed near to the porta hepatis, a spherical cystic mass with an echogenic rim suggestive of a dilated segment of the alimentary tract. Barium upper GI study was negative except for gastroesophageal reflux. But a repeated US demonstrated in addition to the cystic mass, a connection of the mass with a fluid filled tubular structure behind the liver. It was extending through the diaphragm into the right posterior thorax to the level of the bifurcation of the trachea as demonstrated subsequently by CT. On chest X-ray the intrathoracic portion was presented as an area of increased round density below the lower right cardiac border. There was no evidence of skeletal or other anomalies. The preoperative diagnosis was transdiaphragmatic duplication. At surgery a right posterior mediastinal cystic tumor extending from the trachea along the esophagus through the esophageal hiatus to the duodenum was found and removed. The tumor did not communicate with the alimentary tract. The histopathologic examination confirmed the diagnosis of a duplication with the wall containing smooth muscles and esophageal and gastric mucosal lining. Interesting enough the outer surface of the intrathoracic portion of the duplication was covered with numerous black areas their histology consistent with blue nevus. The postoperative course was uneventful and the girl was discharged ten days after the operation.

Crohn's Disease in Very Unusual Sites in Children: Radiographic Findings

Koumanidou-C, Sivit-C, Zarifi-M, Vritsiou-M, Kakavakis-K, Radiology Department of "Agia Sophia" Children's Hospital, 11527 Goudi Athens, Greece and Radiology Department of Childrens National Medical Center, 111 Michigan Avenue, Washington DC., U S A Crohn's disease (regional enteritis) is a transmural granulomatous disease that may affect the small bowel or colon and is

470 quite rare in children. The aim of this study is to present the radiographic patterns of 5 children with Crohn's disease in very unusual sites: In the first case the disease involved the second portion of the duodenum; in the second case it was localized throughout the colon and the terminal ileum; in the third case it involved only the jejunum and in the two last cases the appendix. In the first case the upper GI examination revealed persistent narrowing of the second portion of the duodenum with thickening of mucosal folds and persistent dilation of the 3rd portion, with pooling of barium in this region. In the second case the barium enema demonstrated diffuse mucosal irregularity and spiculation in the terminal ileum and throughout the colon including the appendix. In the third case the barium enema showed a cobblestone appearance of the mucosa and rigidity of the jejunum only. In the last two cases the Crohn's appendix was diagnosed by US as acute appendicitis. The surgery and the biopsy results revealed Crohn's disease. The following barium enema revealed that Crohn's disease involved the appendix in a cobblestone pattern.

Doppler Sonography of Infants and Children With Acute Scrotal Pain

Patriquin-H-B, Yazbeck-S, Trinh-B, Jequier-S, Burns-P-N, Grignon-A, Filiatrault-D, Garel-L, Dubois-J, H6pital SainteJustine Montreal Quebec Canada. Introduction: In the past any boy with acute scrotal pain underwent surgical intervention to exclude the diagnosis of testicular torsion. Scintigraphy and Doppler sonography have become the investigations of choice to evaluate testicular perfusion especially in adults. In babies Doppler US is limited by the sensitivity of available equipment to the slow flow in tiny intratesticular arteries. Methods: We evaluated the accuracy of Doppler US in detecting testicular blood flow in 65 boys (aged 0 18 yrs) with acute scrotal symptoms using high frequency color and pulsed Doppler modes with low wall filter and pulse repetition frequency. The examination was started on the normal side. Central intratesticular flow signals were taken as evidence of testicular perfusion. The children were then evaluated with scintigraphy soJ'gery or 3-6 m. clinical follow-up. Results: Doppler US was successful in 59/65 boys. 17/19 torsions were accurately diagnosed. Two false positive examinations were due to intermittent torsion. There were no false positive results. The correct sonographic diagnosis was made in 23/24 boys with epididymitis 6/13 torsions of the appendix testis 6/6 hematomas and 3/3 incarcerated hernias. Conclusion: In our series of babies and children Doppler US had a sensitivity of 89 % and specificity of 100 % in the diagnosis of testicular torsion.

False Positive Sonograhic Diagnosis in Intussusception of the Small Bowel

Pracros-J-P, Miron-M-C, Foray-R Grimard-G, Chapelon-C, Abighosn-J, Gbazi-C, Hopital Debrousse, Lyon, France The purpose of this study was to assess patterns and reliability of US in the diagnosis of intussusception of the small bowel. Material and methods: From 1983 to 1993, all paediatric patients in our center presenting as acute abdomen were investigated by US using 5 and 7.5 MHz probes. Forty-one cases of intussusception of the small boweI were found. Twenty-six boys and fifteen girls aged from six weeks to thirteen years old were reviewed. Results: All cases of intussusception of the small bowel were made with US except for one complex form. In this case of chronic ileo-ileal intussusception with volvulus and perforation the precise diagnosis was made at surgery. Based on the US findings we subdivided the intussusception of the small bowel in three different types: 1) the acute

form was found in fourteen patients, all were ileo-ileal, and in most of cases we identified a new distinctive sign, "the half moon crescent sign". This image represents the fluid trapped between the external and internal layers of the intussuscepturn. 2) The chronic form was found in only one patient, the jejunum was involved, and a normal peristaltism of the internal central loop was noticed which seems to be characteristic of this type of intussusception. 3) The transitory form was present in twenty-five patients. Twenty of these, presented as ileoileal intussusception, the other five were jejunojejunal. A t ultrasound, it was possible to define the site of intussusception according to the aspect of the bowel involved. All intussuscepturns had a diameter less than two centimetres and all resolved during the ultrasonographic examination. It is important to note that of the forty-one cases of intussusception of the small bowel, eleven cases were proved by pathology to be secondary to an intestinal disorder such as Meckel's diverticulum, polyp, intestinal lymphoma, Henoch-Sch6nlein purpura, or celiac disease. Conclusion: US is the imaging modality of choice in detection of intussusception of the small bowel. It is a reliable technique where we can determine the type and the site of intussusception in the presence of the new signs that we described: the "half moon crescent sign" for the acute form and the persistence of normal peristaltis of the internal loop of the intussusceptum in the chronic form.

Simple lntratesticular Cysts in Children. Preoperative Sonographic Diagnosis

Garcia-C-J,, Zuniga-S, Rosenberg-H-J, Arce-J-D, Iglesias-M, Hospital Clinico Universidad Catolica and Clinica Santa Maria, Santiago, Chile Simple intratesticular cysts are rare in children and have been considered the most uncommon cause of scrotal cystic masses in this age group. The exact etiology of these lesions is unclear. High-resolution ultrasonography has been proved to be a reliable and accurate imaging modality in the evaluation of scrotal abnormalities in children. We report the sonographic findings in 4 boys aged from 4 to 10 months, who presented with a scrotal mass and had a simple intratesticutar cyst. Sonography showed a characteristic appearance in all of them and provided the necessary information to do conservative surgery with testicular preservation in three infants. In one infant the cyst involved most of the testicle and orchidectomy was performed. Pathological examination revealed a single smooth-walled cystic structure, with clear and serous fluid, lined with flattened epithelial cells in all of them. Although epidermoid cysts of the testis are mostly cystic, they contain some echoes within them that make it possible to differentiate them from simple cysts. The same happens with some kind of mature testicular teratomas, that rarely might present a predominantly cystic appearance on sonography.

The Adreno-Genital Syndrome Intraperitoneal Complications

Bie]at-A, Golatowski-S, Marcinski-A, Department of Pediatric Radiology and Department of Pediatrics and Endocrinology, Warsaw Medical Faculty, Marszal Kowska 24, 00-576 Warszawa, Poland Deficiency of an C - 21 hydroxylase an enzyme necessary for the adrenal production of cortisol is a cause of adrenogenital syndrome - congenital adrenal hyperplasia (CAH). It results in excessive accumulation of adrenogenic precursors and is the most common cause of female pseudohermaphroditism. The authors present results of a study carried in a group of 59 girls with C A H and pseudohermaphroditism hospitalized in our institution in a period from 1963 to 1991. In 8 girls from

471 this group we have observed an intraperitoneal complication due to the urine reflux from the urogenital sinus to the vagina, uterus and peritoneal cavity. Such a complication has produced peritoneal infection and peritonitis. An analysis of clinical data and results of imaging examinations (vaginography, micturition cystouretrography and ultrasound studies) are presented.

Doppler Sonography in the Follow-Up of Children With the Antenatal Diagnosis of Unilateral Hydronephrosis

difficult to characterize. The following exams - US, M C U G and IVU together with endoscopy confirmed a polymalformative pathology characterized by a left renal agenesis, with a lower blind ureter and a cyst of the seminal vesicle. The intraoperative control confirmed the imaging diagnosis, showing abnormalities of the bladder wall and the confluence of the blind dysplastic ureter into the seminal vesicle. We briefly discuss some embryological aspects of the genito-urinary malformation and the role of imaging that, in such clinical situations, can often give images that are difficult to interpret.

Santos-J-F, Lobo-L, Afonso-J-G, Serviso De Radiologia, Hospital De Santa Maria Av. Prof. Egas Moniz 1500 Lisboa, Portugal

Renal Stones: Transitory Phenomenon in Sick Neonates?

Purpose: To evaluate and to compare the resistive index (R.I.) of the dilated and undilated kidneys in children with the antenatal diagnosis of unilateral hydronephrosis. Methods: The R.I. was determined in the two kidneys of 10 boys aged from 9 to 30 months. The cystography was performed in all cases and V. U. R. was excluded. Every six months a renogram was performed in all the patients, with R. G.F. > 40 % and without an obstructive pattern (after I.V. furosemide). Results: in all cases the R. I. of the dilated kidney was superior to the R. I. of the undilated kidney and consequently the R. I. R. > 1. Conclusions: The small number of cases only can enhance several points: 1 - The conservative attitude followed in these situations, without reflux or deterioration of renal function. 2 The impossibility of R.I. to affirm an obstructive pattern in these circumstances. 3 - The interest in comparing the R.I. of patients in this situation with a group with defined obstruction.

Kis-E, Verebely-T, Kovi-R, Mattyus-I, INST.: First Depart-

Primary Obstructive Megaureter - Long-Term Results

Gruchalska-E, Biejat-A, Gruchalski-J, Department of Pediatric Radiology and Pediatric Surgery, Warsaw Medical Faculty, Marszal Kowska 24, 00-576 Warszawa, Poland Twenty-three children with primary obstructive megaureter were diagnosed and operated upon between 1982-1992. The youngest child was 2 weeks of age and the oldest was 13 years of age. The main clinical symptom was urinary tract infection. The diagnosis was established based on ultrasonography, urography and voiding cystourethrography. In 22 children obstructed segment of the ureter was excised and the ureter was reimplanted (in 8 children ureteral tailoring was also performed). In i child primary nephrectomy was performed. The results of treatment were as follows: In 19 children long term results were good, and only in 3 children long term results were unsatisfactory and secondary nephrectomies were needed.

Complex Genito-Urinary Malformation, With Left Kidney Agenesis, Ipsilateral Displastic Urether and Cyst of the Seminal Vesicle

Da-Pozzo-G-L, Cerri-M, Selvaggio-G, Maestri-L, SchiavettiE, Service of Radiology and Ecography, Osp. Dei Bambini V. Buzzi, Milano, Italy This report deals with a six year old boy who came to hospital with recurrent U.T.I. The preliminary sonographic study revealed left renal agenesis with a suspected ipsilateral ureterocele and a dilated and tortuous left pelvic ureter, apparently related to a cystic structure near the bladder, which was at first

ment of Paediatrics and Department of Radiology of Semmelweiss Medical University, Budapest, Hungary Nephrolithiasis is rare in the newborn infant. Two-thirds of children have a predisposing factor such as metabolic disease, infection, congenital anomalies, intravenous hyperalimentation, urinary diversion procedures, hyperparathyroidism and furosemide therapy. We report five patients seen in a twoyear period with nephrolithiasis detected by routine US. Two neonates have been treated with obstructive uropathy, one with intestinal obstruction and two with respiratory distress because of meconium aspiration. A few months after the successful therapy of the main pathology, the renal stones disappeared without any special therapy, in all five patients. The genesis of neonatal nephrolithiasis is discussed.

Massive Tumoral Calcinosis in a Mantainance Haemodialysis Child. The Contribution of Different Imaging Techniques to a Correct Diagnosis.

Bongini-U, Lavoratti-G, Seracini-D, Vichi-G-F, Department of Pediatric Radiology, A. Meyer Hospital, Florence, Italy Although tumoral calcinosis can develop in adult uremic patients it is however an infrequent finding, characterized by massive periarticular calcium deposition within multiple cavities containing giant cells. Despite a number of studies devoted to soft tissue calcifications in uremia, the etiology of this complication remains poorly understood; although secondary hyperparathyroidism has been recognized to play an important role in the pathogenesis, recent studies underline the possible role of other factors like magnesium, D-vitamin and K-vitamin overload, metabolic alkalosis during and after the dialysis session, and focal tissue injury. We describe a case of child, on haemodialysis since first months of life, affected by a severe secondary hyperparathyroidism. At the age of 15 years the patient suddenly and quickly developed a massive periarticular, parascapular, pseudotumoral calcification followed, one year later, by an analogue, controlateral manifestation. We emphasized, in our case, the importance of imaging integration, supporting the radiological features with CT and especially MR and arteriographic findings, to get to the right nature diagnosis without recurring to biopsy. After all that, combining history, physical examination and such different imaging techniques can solve the difficult differential diagnosis problems with massive tumoral calcinosis, an infrequent finding in adults and extremely rare in a child on haemodialysis.