Childs Nerv Syst (2010) 26:1699–1704 DOI 10.1007/s00381-010-1180-4
ORIGINAL PAPER
Endoscopic third ventriculostomy before tumor surgery in children with posterior fossa tumors, CCHE experience Mohamed Ahmed El Beltagy & Hazem Mostafa Kamal & Hala Taha & Madeha Awad & Nada El Khateeb
Received: 17 February 2010 / Accepted: 10 May 2010 / Published online: 26 May 2010 # Springer-Verlag 2010
Abstract Objective This is a retrospective study to evaluate the effectiveness of endoscopic third ventriculostomy (ETV) performed in children with posterior fossa tumors and hydrocephalus in an attempt to classify the selected cases who could benefit from ETV as a permanent CSF diversion procedure. Methods During the period between January 2008 and December 2008, 40 patients with posterior fossa tumors and associated hydrocephalus were treated inside the Children’s Cancer Hospital, Egypt (CCHE)—with ETV in order to relieve the increased intracranial pressure while awaiting their definite tumor surgery. Results ETV was successful in relieving hydrocephalus clinically and radiologically in 26 patients with different posterior fossa pathologies, with the highest success rate in glioma (100%), followed by 50% in ependymoma and 47.6% in medulloblatoma. In the other 14 cases, preoperative ETV failed in permanently resolving hydrocephalus M. A. El Beltagy (*) : H. M. Kamal Department of Neurosurgery, Cairo University Hospital, Children’s Cancer Hospital (57357), Cairo, Egypt e-mail:
[email protected] H. Taha Department of Pathology, Children’s Cancer Hospital, Cairo, Egypt M. Awad Department of Pediatric Oncology, Children’s Cancer Hospital, Cairo, Egypt N. El Khateeb Department of Research, Children’s Cancer Hospital, Cairo, Egypt
and patients required the insertion of a ventriculoperitoneal (VP) shunt after their posterior fossa surgery and during their follow-up period. Conclusion ETV should be considered as an alternative procedure to VP shunt in controlling severe hydrocephalus related to posterior fossa tumors, to relieve symptoms quickly during the preoperative period while patients await their definite tumor excision. Patients with ependymomas and gliomas, with totally excised tumors, are better candidates for ETV than those with medulloblastomas. However, ETV cannot always prevent postoperative hydrocephalus in all cases of posterior fossa tumor, the thing that makes using postoperative VP shunt an alternative. Keywords Hydrocephalus . Posterior fossa tumor . VP shunt . Endoscopic third ventriculostomy
Introduction Endoscopic third ventriculoscopy (ETV) is one of the options considered by some authors as an initial surgical procedure for the management of obstructive hydrocephalus related to posterior fossa tumors [1]. Endoscopic third ventriculostomy and other related procedures are now commonly used to treat a wide array of neurosurgically managed conditions. A seemingly limitless number of neurosurgical applications await the endoscope. The endoscopic procedure in general was expected—by some authors years ago—to become a routine in modern neurosurgical practice and training [2]. Despite that, some authors still prefer other ways for treating hydrocephalus secondary to posterior fossa tumors such as the preoperative ventriculoperitoneal (VP) shunt [3] or external ventricular drainage (EVD) when necessary [4].
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Others recommended the pretreatment with steroids and subcutaneous ventricular catheter reservoir when possible [5]. Age and etiology are the most important factors influencing the success rate of ETV. Failure rates are reported by some authors to be particularly high during the first year of age [6]. We are mainly trying to find a relation between the pathology of the posterior fossa tumor and its impact on ETV success, and to identify the risk factors which would affect the success of ETV when performed before tumor surgery.
Materials and methods During the period between January 2008 and December 2008, 40 patients with posterior fossa tumors and associated hydrocephalus were treated in the Children’s Cancer Hospital-Egypt (CCHE) by ETV after their admission and while awaiting their scheduled time for surgical excision. Cerebrospinal fluid (CSF) samples were taken from all patients after ETV through lumbar puncture for cytology to look for tumor cells seedling, and were correlated with their spinal magnetic resonance imaging (MRI) findings. CSF sampling was performed by lumbar puncture to exclude any possible false negative results that might be obtained from sampling during the ETV. Tumor excision for all patients was performed in a prone position within the first week after the ETV. All Patients were followed-up clinically, by MRI brain and MRI spine within the first 48 h after surgery and every 3 months during the first 2 years. Patients were divided according to their pathology into: 13 cases with astrocytomas; with age ranging between 2 and 14 years (mean age=8 years), six cases with ependymomas; with age ranging between 1 and 5 years (mean age=3 years), and 21 cases with medulloblastomas; with age ranging between 1 and 16 years (mean age=8.5 years). In patients who did not require VP shunt insertion during the first year after surgery, ETV was considered to be successful. While in those patients who required VP shunt insertion at any time during their follow-up period, ETV was considered to be a failure. ETV failure was diagnosed both clinically by the development of pseudomeningiocele, which required repeated aspiration, and/or clinical manifestations of increased ICT, and radiologically by showing findings of hydrocephalus.
Results ETV was successful in relieving hydrocephalus clinically and radiologically in 26 patients with different posterior fossa pathologies out of 40 patients, with an overall success rate of 65% (Table 1).
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In all 13 astrocytoma cases (GI and GII), ETV was successful with a success rate of 100%, while in ependymoma, three out of six cases required VP shunt insertion, with time difference—between ETV and VP shunt insertion— ranging from 2 to 3 weeks. The remaining three patients had a functioning ETV during the follow-up period (50% success rate). In cases of medulloblastomas, they showed the lowest success rate as 11 cases out of 21 required VP shunt insertion with a success rate of 47.6% (ten cases).The time difference between the ETV and VP shunt insertion varied from 2 to 16 weeks (mean time difference=9 weeks). ETV was successful in a total of 26 patients who did not need a VP shunt within their follow-up period which ranged from 6 to 20 months with a mean of 13 months (Fig. 1). Correlating the age with the success rate, we identified that, all patients with successful ETV were above 1.5 years. There were six cases out of 14 with an age equal to or below this age with failed ETV (42.9%; Table 2).
Discussion Many discussions have been raised against routine VP shunt insertion before definite posterior fossa surgery. Epstein et al. [7] reported a 10% rate of upward herniation in cases of posterior fossa tumors subjected to preliminary VP shunting. Hoffman et al. [8] and Fiorillo et al. [9] described the spreading of medulloblastomas through ventriculo-peritoneal shunts. These arguments led neurosurgeons to propose other methods to avoid the preshunting. Several authors [4, 10] suggested corticosteroid therapy, early surgery, and external ventricular drainage when needed. Steroids may help to reduce the posterior fossa swelling, but still preoperative drainage is needed as despite steroids, there are still serious hazards from the increased ICP such as deterioration in the level of consciousness or visual affection due to papilledema. Although the preoperative VP shunt is still considered the procedure of choice for managing acute hydrocephalus in children with posterior fossa tumors, arriving in bad neurological conditions at a peripheral hospital, it has many complications including spreading of medulloblastomas [9], shunt obstruction, shunt malfunction (even without ventricular dilatation in some cases) [11], shunt infection [12], and other less common complications such as abdominal complications [13], inguinal hernia, perforation of the colon, shunt disconnection, bladder perforation, CSF cysts, and intestinal obstruction. Due to these complications, some authors recommended ETV before tumor removal [1]. Bhatia et al. [14] reported 87.1% success rate for ETV performed before tumor resection with 7.5 years followup. Sainte-Rose et al. [15] showed 98.5% immediate
Childs Nerv Syst (2010) 26:1699–1704 Table 1 Cases with successful ETV
GTR gross total resection, STR subtotal resection
Cases
1701 Age (years)
Pathology
Extend of surgery
CSF cytology
Tumor location
1 2 3 4 5 6 7 8 9 10 11 12 13 14 15
6 14 12 13 10 7 6 7 5 3 5 2 13 1.7 4
Astrocytoma GI Astrocytoma GI Astrocytoma GI Astrocytoma GI Astrocyctoma GI Astrocytoma GI Astrocytoma GII Astrocytoma GI Astrocytoma GII Astrocytoma GI Astrocytoma GII Astrocytoma GI Astrocytoma GII Ependymoma Ependymoma
GTR GTR STR GTR STR GTR GTR STR STR GTR GTR STR STR GTR GTR
Negative Negative Negative Negative Negative Negative Negative Negative Negative Negative Negative Negative Negative Negative Negative
Cerebellar Cerebellar Brain Stem Cerebellar Brain stem Cerebellar Cerebellar Brain stem Brain stem Cerebellar Cerebellar Brain stem Brain stem 4th ventricular 4th ventricular
16 17 18 19 20 21 22 23 24 25 26
5 3 16 10 6 12 6 1.5 5 7 12
Ependymoma Medulloblastoma Medulloblastoma Medulloblastoma Medulloblastoma Medulloblastoma Medulloblastoma Medulloblastoma Medulloblastoma Medulloblastoma Medulloblastoma
STR GTR GTR GTR GTR GTR GTR STR GTR GTR GTR
Negative Negative Negative Negative Negative Negative Negative Negative Positive Negative Negative
4th 4th 4th 4th 4th 4th 4th 4th 4th 4th 4th
symptomatic improvement of their patients when performing ETV prior to tumor surgery with mean follow-up of 786 days, and a 94% rate of shunt-free patients after tumor removal. Nevertheless, they acknowledge this resulting in a proportion of patients undergoing an “unnecessary” procedure. Hopf et al. [16] have reported 17 patients harboring posterior fossa tumors in whom CSF diversion was performed using ETV. In these cases, the hydrocephalus was successfully managed in 13 cases (76%). When performed prior to tumor resection, an ETV represents an efficient way to obtain emergency control of obstructive hydrocephalus, having both a curative effect on intracranial hypertension prior to tumor removal and a prophylactic effect by preventing the development of hydrocephalus after tumor removal [15]. The posterior fossa surgery might therefore be delayed for several days to complete the imaging workup and to perform microsurgery in optimal conditions [1]. The low incidence of persistent hydrocephalus led some authors to believe that the routine use of preoperative ETVs was not entirely justified [17]. Practically, one must recognize that pediatric posterior fossa surgery is not always easy to integrate into a busy
ventricular Ventricular ventricular ventricular ventricular ventricular ventricular ventricular ventricular ventricular ventricular
elective operative schedule within 48-72 h following admission and in a busy center as CCHE; it’s sometimes difficult to plan for the excision of posterior fossa tumor within 3 or 4 days following admission. Therefore, we routinely performed ETV in obstructive hydrocephalus due to posterior fossa tumors before the definite tumor excision which could be then postponed 3– 5 days due to the waiting list of patients. This would be in an attempt to limit the insertion of VP shunt in all hydrocephalic patients, as not all would need a permanent VP shunt after the excision of their tumors. We are trying to retrospectively assess which group of posterior fossa tumors had benefited from the performance of preoperative ETV and which had needed the permanent insertion of VP shunt. This might help to guide us and others to the selection of ETV candidates, when ETV or VP shunt is to be performed before the definite surgery of the tumor. Thus, trying to avoid the so called “unnecessary” preoperative ETV. In our study, we noticed that ETV had a low success rate in children below 1.5 years of age with open anterior fontanel. From our results, it is clear that low-grade gliomas have the highest success rate (100%), followed by 50% in
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Fig. 1 Sagittal MRI with contrast of an 8-year-old male patient showing fourth ventricular Medulloblastoma. a Preoperative MRI, b immediate postoperative MRI, c 6 months follow-up, d 1 year follow-up, all showing functioning ETV with no hydrocephalic changes
a
b
c
d
Table 2 Cases with VP shunt due to ETV failure Cases
Age (years)
1 2 3
1 1 5
4 5 6 7 8 9 10 11 12 13 14
9 12 1.5 1.5 5 11.4 1.5 5 1 9 8
Pathology
Extend of surgery
The time between ETV and VP (days)
CSF cytology
Tumor location
Ependymoma Ependymoma Ependymoma
STR STR GTR
23 14 12
Negative Negative Positive
4th ventricular 4th ventricular 4th ventricular
Medulloblastoma Medulloblastoma Medulloblastoma Medulloblastoma Medulloblastoma Medulloblastoma Medulloblastoma Medulloblastoma Medulloblastoma Medulloblastoma Medulloblastoma
GTR GTR GTR GTR GTR GTR GTR GTR GTR STR GTR
50 14 62 45 26 34 120 31 13 20 28
Negative Negative Negative Positive Positive Negative Positive Negative Positive Positive Negative
4th 4th 4th 4th 4th 4th 4th 4th 4th 4th 4th
ventricular ventricular ventricular ventricular ventricular ventricular ventricular ventricular ventricular ventricular ventricular
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ependymomas and 47.6% in medulloblatomas. The six cases of ependymoma were too small a number to evaluate the success of ETV, but at least it seems that the cases of low-grade pathologies with GTR and negative CSF cytology seem to be good candidates to avoid the insertion of VP shunt. Of course, we cannot predict what the pathology of the case before excision is, but at least from the scans of the patients, low-grade gliomas and ependymomas could sometimes be distinguished from medulloblastomas through certain radiological findings. We can assume that the lower success rate in case of medulloblastomas even when achieving total resection of the tumor may be attributed to the higher grading and CSF Seeding in certain medulloblastomas. Some authors [18] suggested that medulloblastomas might be more infiltrative than other tumor types in the lateral recesses of the fourth ventricle, thus impeding CSF outflow. The prone position used in all of our patients was emphasized by others to be accompanied by a more significant intraoperative bleeding, stagnation of blood and debris in the surgical field, increased risk of contamination of the subarachnoid space, and possibly postoperative adhesions [1], but the immediate postoperative MRI scans performed to all our patients showed none of these complications, at least there were no MRI differences between the failed and the successful cases regarding any of the abovementioned changes. In our cases, ETV could still be revised after failure but this would add to the hospitalization days for the patients and would postpone their adjuvant therapy, which is not always feasible in overloaded centers. Some had suggested that hydrocephalus occurring postoperatively has multiple etiologies: swelling of the cerebellum, alteration in CSF re-absorption due to surgery-induced subarachnoid hemorrhage, and development of adhesions at the level of the fourth ventricle outlets and the adjacent cisterns [1]. Many authors recommended VP shunt after the tumor removal but did not deny that ETV was a safe way even after the tumor removal. Morelli et al. [18] confirmed that a routine postoperative ETV is indicated for treating persistent hydrocephalus. Also, Tamburrini et al. [19] suggested that ETV should be regarded as the best surgical option for the management of persistent hydrocephalus following posterior fossa tumor excision. Tamburrini et al. [20] reported that the postoperative ETV had a high success rate while poor results were obtained in children with tumor seeding and/or evidence of positive CSF cultures. Still, a preoperative ETV could render patients’ postoperative course less complicated because it eliminates the risk of CSF infection related to EVD or VP shunt and minimizes the risk of over drainage because it provides more physiological CSF drainage than do other procedures [1].
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From our study, we can identify that the patients who could benefit from ETV before definite tumor surgery and who could continue to have a functioning ETV after the excision could be (1) patients above 1.5 years old, (2) those with a postoperative MRI showing complete tumor excision, (3) cases without metastatic disease in the CSF, and (4) whom their pathologies prove to be low grade gliomas followed by ependymomas and least in medulloblastomas, especially if small without extension in the lateral recesses of the fourth ventricle. Applying these criteria, when considering ETV as a method for CSF diversion before posterior fossa tumor surgery, would avoid or at least decrease the incidence of the unnecessary ETV.
Conclusion ETV should be considered as an alternative procedure to VP shunt in controlling severe hydrocephalus related to posterior fossa tumors to relieve symptoms quickly during the preoperative period when patients should wait for their definite tumor excision. Patients with ependymomas and gliomas, with totally excised tumors, are better candidates for ETV than those with medulloblastomas. However, ETV cannot always prevent postoperative hydrocephalus in all cases of posterior fossa tumor, the thing that makes using postoperative VP shunt an alternative.
References 1. Ruggiero C, Cinalli G, Spennato P, Aliberti F, Cianciulli E, Trischitta V, Maggi G (2004) Endoscopic third ventriculostomy in the treatment of hydrocephalus in posterior fossa tumors in children. Childs Nerv Syst 20:828–833 2. Li KW, Nelson C, Suk I, Jallo GI (2005) Neuroendoscopy: past, present, and future. Neurosurg Focus 19(1):E1 3. Albright L, Reigel DH (1977) Management of hydrocephalus secondary to posterior fossa tumors. J Neurosurg 46:52–55 4. Muszynski CA, Laurent JP, Cheek WR (1994) Effect of ventricular drainage and dural closure on cerebro-spinal fluid leaks after posterior fossa tumor surgery. Pediatr Neurosurg 21:227–231 5. Schmid UD, Seiler RW (1986) Management of obstructive hydrocephalus secondary to posterior fossa tumors by steroids and subcutaneous ventricular catheter reservoir. J Neurosurg 65:649–653 6. Jones RF, Stening WA, Brydon M (1990) Endoscopic third ventriculostomy. Neurosurgery 26:86–91 7. Epstein F, Murali R (1978) Pediatric posterior fossa tumors: hazards of the “preoperative” shunt. Neurosurgery 3:348–350 8. Hoffman HJ, Hendrick EB, Humphreys RP (1976) Metastasis via ventriculoperitoneal shunt in patients with medulloblastoma. J Neurosurg 44:462–466 9. Fiorillo A, Maggi G, Martone A, Migliorati R, D’Amore R, Alfieri E, Greco N, Cirillo S, Marano I (2001) Shunt-related abdominal metastases in an infant with medulloblastoma:longterm remission by systemic chemotherapy and surgery. J Neurooncol 52:273–276
1704 10. Rappaport ZH, Shalit MN (1989) Perioperative hydrocephalus secondary to infratentorial brain tumors. Acta Neurochir(Wien) 96:118–121 11. McNatt SA, Kim A, Hohuan D, Krieger M, McComb JG (2008) Pediatric shunt malfunction without ventricular dilatation. Pediatr Neurosurg 44:128–132 12. Lim ME, Hoffman JA, Kim KS (1999) Recurrent ventriculoperitoneal shunt infection due to nontypeable Haemophilus influenzae. Clin Infect Dis 28:147–148 13. Bryant MS, Bremer AM, Tepas JJ, Mollitt DL, Nquyen TQ, Talbert JL (1988) Abdominal complications of ventriculoperitoneal shunts. Case reports and review of the literature. Am Surg 54:50–55 14. Bhatia R, Tahir M, Chandler CL (2009) The management of hydrocephalus in children with posterior fossa tumours: the role of pre-resectional endoscopic third ventriculostomy. Pediatr Neurosurg 45:186–191 15. Sainte-Rose C, Cinalli G, Roux FE, Maixner R, Chumas PD, Mansour M, Carpentier A, Bourgeois M, Zerah M, Pierre-Kahn A, Renier D (2001) Management of hydrocephalus in pediatric patients with posterior fossa tumors: the role of endoscopic third ventriculostomy. J Neurosurg 95:791–797
Childs Nerv Syst (2010) 26:1699–1704 16. Hopf NJ, Grunert P, Fries G, Resch KD, Perneczky A (1999) Endoscopic third ventriculostomy: outcome analysis of 100 consecutive procedures. Neurosurgery 44:795–806 17. Bognar L, Borgulya G, Benke P, Madarassy G (2003) Analysis of CSF shunting procedure requirement in children with posterior fossa tumors. Childs Nerv Syst 19:332–336 18. Morelli D, Pirotte B, Lubansu A, Detemmerman D, Aeby A, Fricx C, Berré J, David P, Brotchi J (2005) Persistent hydrocephalus after early surgical management of posterior fossa tumors in children: is routine preoperative endoscopic third ventriculostomy justified? J Neurosurg 103:247–252 19. Tamburrini G, Pettorini BL, Massimi L, Caldarelli M, Di Rocco C (2008) Endoscopic third ventriculostomy: the best option in the treatment of persistent hydrocephalus after posterior cranial fossa tumour removal? Chils Nerv Syst 24:1405–1412 20. Tamburrini G, Massimi L, Caldarelli M, Di Rocco C (2008) Antibiotic impregnated external ventricular drainage and third ventriculostomy in the management of hydrocephalus associated with posterior cranial fossa tumours. Acta Neurochir (Wien) 150:1049–1056