Endoscopic cyst fenestration in the treatment of uniloculated hydrocephalus in children

Clinical article

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Object

The treatment of uniloculated hydrocephalus is a difficult problem in pediatric neurosurgery. Definitive treatment is surgical, yet the approach remains controversial. This study evaluates the role of endoscopic cyst fenestration (ECF) in the management of this disease.

Methods

Thirty-one pediatric patients with uniloculated hydrocephalus who underwent endoscopic surgery, performed by the author, between May 1999 and December 2010 constitute the patient group for this study. The patients included 17 boys and 14 girls, with ages ranging from 5 months to 5 years (mean 22.9 months). Patients with multiloculated hydrocephalus were not included. The patients' charts were reviewed for demographic data, radiological findings, information regarding morbidity, improvement of hydrocephalus, incidence of recurrence, shunt dependency, and the need for shunt revision.

Results

Neuroepithelial cysts were the most common cause (17 cases), followed by postoperative gliosis due to previous shunt infection (9 cases), intraventricular hemorrhage (3 cases), and meningitis (2 cases). Multiplanar MRI was reliable in making the diagnosis and is indicated if CT shows disproportionate hydrocephalus. Surgical treatment included ECF (31 cases), endoscopic revision of malfunctioning preexisting shunts (9 cases), endoscopic third ventriculostomy (4 cases), and placement of a new shunt (3 cases). Endoscopic cyst fenestration was easily performed in all the cases, with devascularization of the cyst wall by coagulation to prevent recurrence. Improvement of hydrocephalus was observed in 26 cases (83.9%). Among the group of patients without prior shunts (22 cases), 3 patients (13.6%) required repeat ECF and 3 patients (13.6%) required placement of a shunt (new shunt placement). In the 9 patients with preexisting shunts, endoscopy reduced the mean rate of shunt revision from 2.7 revisions per year before fenestration to 0.25 per year after fenestration. Four of these 9 patients had multiple shunts, which could be converted to a single shunt; however, repeat ECF was necessary in all 9 patients. With a mean follow-up duration of 4.3 years, none of the patients with a prior shunt was able to become shunt-independent, whereas 86.4% of patients without a prior shunt were able to avoid shunt placement. Endoscopic complications were reversible (unilateral subdural effusion in 5 cases, minor arterial bleeding in 2 cases, CSF leakage in 1 case), and there was no death (0%).

Conclusions

Endoscopic cyst fenestration is recommended in the treatment of uniloculated hydrocephalus because it is effective, simple, minimally invasive, and associated with low morbidity and mortality rates. The fact that all previously shunt-treated patients needed repeat ECF and that none of these patients was able to become shunt-independent makes it clear that uniloculated hydrocephalus due to postoperative gliosis induced by previous shunt infection carries the worst prognosis.

Abbreviations used in this paper:AED = antiepileptic drug; ECF = endoscopic cyst fenestration; ETV = endoscopic third ventriculostomy.

Article Information

Address correspondence to: Nasser M. F. El-Ghandour, M.D., 81 Nasr Road, Nasr City, Cairo, Egypt. email: elghandour@yahoo.com.

Please include this information when citing this paper: published online February 1, 2013; DOI: 10.3171/2012.12.PEDS12379.

© AANS, except where prohibited by US copyright law.

Headings

Figures

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    Intraoperative photographs. A: The cyst wall before fenestration, showing the typical bluish appearance. B: Example of a cyst that is not adherent to the ependyma. The arrow indicates the Monro foramen, obstructed by the cyst. C: A diathermy probe is used to coagulate the cyst wall and make an initial perforation. D: The resulting endoscopic ventriculocystostomy. E: Collapse of the cyst at the end of the procedure, with opening of the Monro foramen and restoration of the CSF pathways. F: Catheter placement. After the ECF is performed and the old malfunctioning ventricular catheter is removed, a new one is placed in the optimal position under endoscopic visualization in order to drain both the cyst and the ventricular system.

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    Axial T1-weighted MR imaging. Left: Preoperative image showing a huge intraventricular neuroepithelial cyst obstructing both Monro foramina, resulting in hydrocephalus. Right: Postoperative image obtained 3 months after ECF showing significant reduction in both cyst and ventricle size, with restoration of ventricular architecture.

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