Disproportionately Large Communicating Fourth Ventricle: Pearls for Diagnosis and Management

Introduction Disproportionately large communicating fourth ventricle (DLCFV) is an unusual presentation of communicating hydrocephalus, in which patients with hydrocephalus have a disproportionately enlarged fourth ventricle in the absence of obstructive pathology. We present six cases of DLCFV which, to date, is the largest series of this relatively rare condition. We highlight the significance of diagnosis and its differentiation from trapped fourth ventricle (TFV) and discuss the nuances for optimal management of DLCFV. Methods Retrospective case series of consecutive patients with DLCFV, managed by the senior author (LT) over a 10-year period. Results Six cases were identified, five of whom had previous posterior fossa surgery and one with previous encephalitis. All patients presented with cerebellar signs, the initial group had unsuccessful initial management with typical cerebrospinal fluid (CSF) diversion. Consistent symptom resolution was achieved by the application of negative CSF pressures via external ventricular drainage (EVD), maintained with subsequent ventriculopleural shunt (VPL), valveless lumbopleural shunt (LPS) or valveless ventriculoperitoneal shunt (VPS), or proceeding directly to a low-pressure system. Conclusions DLCFV is a diagnosis characterised by cerebellar dysfunction, with or without cranial nerve palsies, often in the setting of previous posterior fossa pathology. Optimal management relies on knowledge of this unique diagnostic entity, and use of an EVD at negative pressures to confirm symptomatic and radiological improvement prior to definitive treatment.