Spontaneous shunt independence in paediatric tumour-related hydrocephalus: case series and review of the literature

Abstract

Purpose: Hydrocephalus is commonly associated with paediatric posterior fossa tumours and their resection. This is commonly managed by ventriculoperitoneal shunt insertion, which is associated with a lifelong risk of malfunction, necessitating revisional surgery. Few opportunities ever arise for the patient to be free of the shunt and this risk. We describe three patients shunted for tumour-related hydrocephalus who subsequently developed spontaneous shunt independence. We discuss this in the context of the literature.

Methods: A single-centre retrospective case series analysis was performed using a departmental database. Case notes were retrieved from a local electronic records database, and images were reviewed using national Picture Archiving and Communication Systems.

Results: Over a 10-year period, 28 patients underwent ventriculoperitoneal shunt insertion for tumour-related hydrocephalus. Of these, 3 patients (10.7%) went on to have their shunts successfully removed. Age at presentation varied from 1 to 16 years. In all cases, the patient required shunt externalization due to shunt or intra-abdominal infection. This was used as an opportunity to challenge the need for ongoing cerebrospinal fluid (CSF) diversion. In one case, this occurred only several months after a shunt blockage with intracranial pressure monitoring that proved her shunt dependence. All three patients tolerated this challenge, their shunt systems were removed without complication, and they remain free of hydrocephalus at last follow-up.

Conclusion: These cases reflect our poor understanding of the heterogenous physiology of patients with shunted hydrocephalus and underline the importance of challenging the need for CSF diversion at any appropriate opportunity.

Keywords: Hydrocephalus; Posterior fossa tumour; Shunt independence; Ventriculoperitoneal shunt.