Cranial morcellation decompression for refractory idiopathic intracranial hypertension in children

Abstract

Background: Primary idiopathic intracranial hypertension (PIIH) in children is rare and has a poorly understood pathophysiology. It is characterized by raised intracranial pressure (ICP) in the absence of an identified brain lesion. Diagnosis is usually confirmed by the measurement of a high cerebrospinal fluid (CSF) opening pressure and exclusion of secondary causes of intracranial hypertension. Refractory PIIH may lead to severe visual impairment. The purpose of this study was to evaluate a cranial morcellation decompression (CMD) technique as a new surgical alternative to stabilize intracranial pressure in PIIH.

Materials and methods: A literature review was carried out, disclosing only 7 pediatric cases of PIIH treated with surgical skull expansion. In addition, we describe here one case of our own experience treated by CMD.

Conclusions: CMD surgery is a safe and effective option to control refractory PIIH in selected patients.

Keywords: Craniotomy; Idiopathic intracranial hypertension; Papilledema; Pseudotumor cerebri; Technique.