Initial management of hydrocephalus associated with Chiari malformation Type I–syringomyelia complex via endoscopic third ventriculostomy: an outcome analysis

Caroline Hayhurst M.R.C.S.1, Jibril Osman-Farah M.D.1, Kumar Das M.R.C.P., F.R.C.R.2, and Conor Mallucci M.B.B.S., F.R.C.S.1
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  • 1 Departments of Neurosurgery and
  • | 2 Neuroradiology, The Walton Centre for Neurology and Neurosurgery, Liverpool, United Kingdom
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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.

Abbreviations used in this paper:

CM-I = Chiari malformation Type I; CSF = cerebrospinal fluid; ETV = endoscopic third ventriculostomy; ICP = intracranial pressure; MR = magnetic resonance; VP = ventriculoperitoneal.

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