Case Reports
Anterior Sacral Meningocele With Presacral Cysts: Report of a Case Zoran Krivokapic´, M.D., Ph.D., F.R.C.S.,1 Nikica Grubor, M.D.,1 ˇ olovic´, M.D., Ph.D.1 Marjan Micev, M.D., Ph.D.,1 Radoje C 1
Digestive System Diseases Institute, First Surgical Clinic, Clinical Center of Serbia, Belgrade, Serbia and Montenegro Anterior sacral meningocele is a rare anomaly most frequently presenting as a presacral mass. Since the first description in 1837, approximately 150 cases have been reported. The case presented is a 37-year-old female in whom an asymptomatic presacral mass was discovered during her first delivery. Because normal delivery was impossible, a cesarean section was performed. A year later, in a regional hospital a “cystic presacral tumor” was treated with biopsy and drainage. Four years later, she developed constipation caused by perineal compression for which she was admitted to our department in which two anterior presacral cysts were excised. The recovery was complicated with meningitis, which was successfully treated with antibiotics. Whenever a presacral mass is found, anterior sacral meningocele has to be a diagnostic consideration. The symptoms are usually related to the compression on rectum, bladder, and sacral nervous plexus. Rectal examination and radiography of the pelvis with the sacral bone showing the “scimitar sign” are the main diagnostic methods. Myelography, computed tomography, and magnetic resonance imaging are the best methods for identifying the precise anatomy of sacral meningocele and for proper planning of the operation. Transvaginal or transrectal aspiration and drainage are not advised, because they may result in a lethal outcome caused by sepsis. [Key words: Sacrum; Anterior meningocele; Presacral cysts; Operation]
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nterior sacral meningocele (ASM) is a congenital cystic structure containing the cerebrospinal fluid, which results from a hernia through a defect on the anterior surface of the sacral bone. The outer wall of the hernial protrusion is a dural membrane and the Correspondence to: Nikica Grubor, M.D., Digestive System Diseases Institute, First Surgical Clinic, Clinical Center of Serbia, Koste Todorovic´ a 6, Belgrade, Serbia and Montenegro, e-mail:
[email protected] Dis Colon Rectum 2004; 47: 1965–1969 DOI: 10.1007/s10350-004-0664-2 © The American Society of Colon and Rectal Surgeons Published online: 11 October 2004
internal one is an arachnoid membrane.1 The sac is linked to the dural pouch with a thin neck. Because of its rarity, ASM can be difficult to diagnose preoperatively. The surgeon treating ASM must be acquainted with this congenital deformity because errors in the therapy may result in fatal outcome.
REPORT OF A CASE A 37-year-old female had been admitted for surgical treatment of a previously diagnosed presacral cyst. The cyst was first identified in 1993 during pregnancy. Because a normal delivery was impossible, a cesarean section was performed. The cyst was again identified after gynecologic examination in 1994. The patient was operated on in a regional hospital. The procedure included biopsy of the cyst. Analysis of the biopsy sample revealed a cyst covered with a thin, singlelayer epithelium. The cyst wall also included smooth muscle tissue with little connective tissue and a small number of epithelial secretion-capable cells. The possibility of retained embryonal development was considered. Even after the intervention, previous symptoms continued to persist in the form of constipation, sensation of pressure on the perineum, and somewhat more frequent urination. Digital rectal examination revealed a soft-fluctuant mass compressing the back wall of the rectum. The laboratory data were within normal limits with a erythrocyte sedimentation rate of 20 mm per 1 hour. The ultrasound examination indicated a clearly defined cystic formation in the lesser pelvis. The abdomen and pelvis CT revealed a
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presacral, retroperitoneal, oval-defined mass, measuring 85 mm × 89 mm × 140 mm in diameters. The cyst exerted pressure on the rectum and uterus to the left and frontward (Fig. 1). A standard x-ray of the pelvis in the anteroposterior and lateral positions (Fig. 2) revealed a defect of the sacral bone, starting from S1 with a “scimitar sign,” and the complete absence of the coccygeal bone. At laparotomy, no abnormalities were observed in the abdominal organs. In the lesser pelvis, retroperitoneally, a large cystic mass obstructing the access to the lesser pelvis was found. With careful dissection, its ribbon-shaped communication with the anal canal was identified (Fig. 3). The cyst was completely excised, and once the “ribbon” was cut, a small opening on the posterior commissure of the anus was observed. The defect in the posterior anal canal was suture closed through a transanal approach. Once this cyst was removed, another smaller one was observed, originating from the anterior aspect of the sacral bone. Palpation revealed the side of the cyst like a “scimitar.” A careful excision was performed and the dural pouch closed with individual sutures. Postoperative recovery was uneventful. On the tenth postoperative day, the patient complained of headache, photophobia, and difficulty in walking, and meningitis was diagnosed. [Lumbar punction: liquor clear, 246 cell elements/mm 3 (differentiation: 48 percent polymorphonuclear, 52 percent lymphocytes, 0.77 saccharum, 1.45 proteins). The liquor culture after lumbar puncture was sterile; slidex 0; hemoculture sterile.] Postoperative magnetic resonance imaging (MRI) of the lumbosacral part of the spine and lesser pelvis was performed (first without and then with the intravenous contrast as well) as the MRI myelography. The defect of the sacral bone has been observed from level S1. The projection of this congenital defect also revealed still fresh postoperative scar tissue, and immediately above it the continuation of the spinal canal and a slightly expanded dural pouch was noticed suturing (Fig. 4). The patient was treated with high doses of antibiotics (Cephalosporin, Gentamicin, and Metronidazole), as well as with antiedematous and symptomatic therapy. After ten days of therapy with antibiotics, a lumbar punction was again performed (liquor clear, 0 cell elements/mm3, saccharum 2.0 proteins 0.25, culture 0, slidex 0). The neurologic state was returning to normal, meningeal symptoms and headache disappeared. The patient was discharged 37 days after the operation. She remains symptom-free to date.
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Figure 1. CT scan of the pelvis demonstrating a large presacral mass, 140 mm × 85 mm × 89 mm in diameter, and right-sided sacral agenesis. Figure 2. Radiography of the pelvis showing the absence of the right-side of the sacral bone from level S1.
Gross review of the presacral cyst showed a tense polycystic mass with maximal diameter 140 mm × 85 mm × 65 mm, filled with clear watery liquid. Outer and inner surfaces of cystic loculi were smooth and gray, focally erythematous, and with rare, slightly increased cuboidal to cylindrical epithelium, focally associated with rare mucin-productive glands (Fig. 5). In addition, cystic epithelium appeared multilayered
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Figure 5. Enteric type of epithelial lining covered most of presacral cyst.
Figure 3. Anatomic relation of the anterior meningocele with the spinal canal (A) and of the enteric cyst with the anal canal (B).
mal canalicular outpouching, with maximal diameters measuring 60 mm × 50 mm × 40 mm. On opening, the cystic attachment to sacrum revealed smooth membranous inner surface, with a similarly clear watery content. Microscopic examination revealed irregular shaped cystic formation with multiple clefts, lined by meningothelial cells. In the cystic wall, there were smooth muscle bundles, blood vessels, and disorganized nerve structures, including nerve fascicles and Pacinian corpuscles (Fig. 6). There were no associated enteric epithelial elements in this cyst. The findings were diagnostic of an anterior sacral meningocele combined with an enteric presacral cyst with a major defect of the sacral bone and complete absence of the coccygeal bone.
DISCUSSION
Figure 4. Postoperative myelography showing the spinal canal and lightly expended dural pouch.
in some areas with scattered but evident squamous differentiation, including rare foci of parakeratosis and hyperkeratosis, or interspersed with enteric elements. Outer fibromyxomatous layer of cystic wall contained small microcystic satellites. Another cyst, located in sacral region showed a unilocular cystic mass on gross appearance with proxi-
ASM is a rare congenital cystic tumor. In 1837, Back published a report on the first ASM case, which led to obstruction during childbirth and the subsequent death of the parturient female.2 Coller and Jackson3 in 1943 found 23 patients described in literature. Eder4 in 1949 found 45 described cases. In 1958, Silvis et al.5 found 58 and Haddad6 found 53, including 2 of his own patients, operated cases. By that time, the total number of registered cases was still < 80. By 1977, the number of identified cases was approximately 100 and by 1988 approximately 150.7 Some 85 percent of the patients were female and the condition was linked to gender.8 Yates et al.9 also raised the possibility of male-to-male transmission and believed that the disease is transmitted autosomally and dominantly. Most of the symptoms appear because of pressure on surrounding structures: rectum, bladder, female genitals,
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Figure 6. Nerve fascicles and bundles with Pacinian structures surrounding cleft of meningocele.
and sacral nerve roots. They include: constipation, irregular menstruation, frequent urination, and infections, nonspecific pains in the lower parts of the back sometimes radiating into the legs, hypalgesia in the perineum zone, paresthesia, reduced tonus of the anal sphincter, and tonus of the detrusor.10 Headaches appear because of the intermittent increase of the intracranial pressure caused by the direct compression on the broad neck of the cyst, preventing the transfer of the cerebrospinal liquor into the meningocele reservoir. It is believed that this mechanism causes headaches in approximately one-third of patients. The transfer of cerebrospinal liquor causes the extension of the pain-sensitive structures inside the posterior fossa.11 This symptom typically appears in childhood with sudden headaches during strain, defecation, crouching, and often during coitus in adults. Meningitis appears in case of a microperforation or a major canal of communication (iatrogenic) between the rectum and the meningocele, which permits the passage of bacteria to the meningocele. Like any other large pelvic mass, ASM can obstruct childbirth (dystocia).12–14 With small and soft cysts that are asymptomatic, a vaginal delivery may be possible. More frequently, delivery is difficult or nearly impossible15 requiring a cesarean section, as in the case of our patient. The differential diagnosis between ASM and other presacral cysts and tumors is extremely important. In the pre-1960 period, because of mistakes in the preoperative diagnosis, a very high surgical mortality was recorded, reaching 40 percent. One must primarily consider the possibility of ASM. A digital-rectal examination shows a soft, fluctuant, presacral mass. Radiography indicating the so-called “scimitar-shaped sacrum” is pathognomic because the size of the ASM
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affects the development of the coccygeal and sacral bone giving it a characteristic shape. Other associated anomalies are uterus duplication, anal stenosis, duplication of the rectum, teratomas, epidermoid cysts, and, rarely, teratocarcinoma.16 CT scan with contrast or MRI clearly defines the cyst and connection with the dura. If these methods fail to reveal the neck of the cyst, a myelogram might be indicated, especially in the lateral position, even at a later stage to help identify the level of communication. The treatment of choice is resection and ligation. The duraside and the sacral side are sutured in a watertight manner to prevent infection of the cerebrospinal fluid. In females of childbearing age and when the cyst is asymptomatic, removal is recommended to allow vaginal delivery. A posterior approach can be used for the resection of an ASM when it is positioned low. In case of a cyst positioned higher, it should be dealt with by transabdominal approach. The abdominal approach was first successfully used by Roux17 and later by Weber. 18 The posterior, presacral, Kraske’s approach had been recommended by Pupovac.19 In the case of our patient, surgery was performed with a combined transabdominal and transanal approach. In 1938, Adson20 introduced ligation of the ASM stem pedicle after sacral laminectomy. In their case, the communication channel was closed from the posterior aspect of the cyst, and they had no mortality in 19 patients. If the ASM stem is inadequately ligated, or in case of an incorrect preoperative or intraoperative diagnosis, the results were poor. Most of the patients died because of complications, such as arachnoiditis with paraplegia,21 liquor fistula, and prolonged meningitis.22
CONCLUSIONS We believe that every “elastic” mass, located retrorectally, requires detailed and complete diagnostic workup. An anterior presacral meningocele is seldom associated with other presacral cysts. Biopsy may result in a lethal outcome caused by sepsis. Treatment is surgical removal.
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13. Shidler FP, Richards V. Anterior sacral meningocele: report of three cases. Ann Surg 1943;118:913–8. 14. Sherman RM, Caylor HD, Long L. Anterior sacral meningocele: report of three cases. Am J Surg 1950;79:743– 7. 15. Sobrado CW, Mester M, Simonsen OS, Justo CR, de Abreu JM, Habr-Gama A. Retrorectal tumors complicating pregnancy: report of two cases. Dis Colon Rectum 1996;39:1176–9. 16. Christensen, MA, Blatchford, GJ. “Presacral tumors in adults” In: Beck, DE, Wexner, SD, eds. Fundamentals of anorectal surgery. New York: McGraw-Hill, 1992: 386–401. 17. Roux S. Contribution a l etude de la meningocele sacree anterieure. Rev Med Suisse Romande 1918;38:47–71. 18. Weber EA. Propos d’un cas de meningocele anterior sacree. Rev Fr Gynec Obstet 1921;16:377–92. 19. Pupovac D. Zur Kenntnis der pathologischen Anatomic und genese der hydromeningocele sacralis anterior. Arb Geb Klin Chir 1903;1:533–49. 20. Adson AW. Spina bifida cystica of the pelvis: diagnosis and surgical treatment. Minn Med 1938;21:468–75. 21. Sutton D. Sacral cysts. Acta Radiol 1963;1:787–95. 22. Cramer H. Meningocele sacralis ventralis. Acta Neurochir (Wien) 1960;9:139–51.