Endoscopic third ventriculostomy in the fourth ventricle outlet obstruction associated with chiari malformation type I and syringomyelia: Case report

Object The aim of this study was to evaluate the efficacy of endoscopic third ventriculostomy (ETV) in patients with Chiari malformation Type I (CM-I) and hydrocephalus with or without syringomyelia. Methods The authors identified, in a prospective endoscopy database, 16 adults and children (age range 2–68 years) with CM-I and hydrocephalus that had been managed with ETV. They reviewed the clinical features and radiographic findings for all patients. Fifteen patients underwent ETV as a primary treatment, whereas 1 patient underwent the procedure at the time of shunt failure. All patients had symptomatic hydrocephalus with either aqueductal or fourth ventricular outflow obstruction. The mean duration of follow-up was 42 months. Results Fifteen patients (94%) remain shunt free following ETV for CM-I. Five (83%) of the 6 patients with a syrinx had improvement or resolution of the syrinx following ETV. Six patients (37.5%) underwent foramen magnum decompression for persistent CM-I– or syrinx-related symptoms. There was no cerebrospinal fluid leakage or intracranial pressure–related problem following foramen magnum decompression. Conclusions Endoscopic third ventriculostomy provides a durable method of treatment for hydrocephalus associated with CM-I. It is effective as a primary treatment, and the authors advocate its use as a replacement for routine ventriculoperitoneal shunt insertion in these patients. Management of the hydrocephalus alone is often sufficient and may obviate decompression, although a significant proportion of patients will still need both procedures.

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Fourth Ventricle Outlet Obstruction and Diverticular Enlargement of Luschka Foramina in a Child with Down Syndrome

Pediatric Neurosurgery ◽

10.1159/000511088 ◽

2020 ◽

pp. 1-4

Author(s):

Valentina Orlando ◽

Pietro Spennato ◽

Maria De Liso ◽

Vincenzo Trischitta ◽

Alessia Imperato ◽

...

Keyword(s):

Down Syndrome ◽

Fourth Ventricle ◽

Endoscopic Third Ventriculostomy ◽

Obstructive Hydrocephalus ◽

Radiological Finding ◽

Outlet Obstruction ◽

Third Ventriculostomy ◽

Viable Option ◽

Case Presentation

Introduction: Hydrocephalus is not usually part of Down syndrome (DS). Fourth ventricle outlet obstruction is a rare cause of obstructive hydrocephalus, difficult to diagnose, because tetraventricular dilatation may suggest a communicant/nonobstructive hydrocephalus. Case Presentation: We describe the case of a 6-year-old boy with obstructive tetraventricular hydrocephalus, caused by Luschka and Magendie foramina obstruction and diverticular enlargement of Luschka foramina (the so-called fourth ventricle outlet obstruction) associated with DS. He was treated with endoscopic third ventriculostomy (ETV) without complications, and a follow-up MRI revealed reduction of the ventricles, disappearance of the diverticula, and patency of the ventriculostomy. Conclusion: Diverticular enlargement of Luschka foramina is an important radiological finding for obstructive tetraventricular hydrocephalus. ETV is a viable option in tetraventricular obstructive hydrocephalus in DS.

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