Increasing Head Circumference from Hydrocephalus, Not Only in Young Children: Case Report of a 7-Year-Old

Abstract

Introduction: Increasing head circumference is a common presentation of hydrocephalus within the first 2 years of life but is extremely rare in older children or adults. Here, we present a rare case of rapidly increasing head circumference, as the sole symptom, as a new diagnosis of hydrocephalus in a 7-year-old boy, who then re-presented with an increasing head circumference at the time of a VPS malfunction a year later. Case: A 7-year-old was referred to neurosurgery for papilledema and increased headaches. The mother reports a rapidly increasing head circumference necessitating changes in hat size at least twice in the last few months. The CT gave the diagnosis of a Dandy-Walker spectrum malformation and hydrocephalus. A VPS was therefore inserted, with a stabilisation of his head circumference for a few months. He presented again with a rapidly increasing head circumference over the course of a few months, from 57 cm up to 59.5 cm, in the setting of progressive headaches consistent with increased ICP. The patient subsequently underwent a shunt valve replacement to improve CSF diversion. Methods: The patient was followed over 18 months from diagnosis to shunt revision, with MRI of the head. Volumetric measurements of the ventricles and head circumference are compared over the same time period. Conclusion: We present a unique case of increasing head circumference in a 7-year-old boy with Dandy Walker spectrum malformation, who then had another rapid increase almost two years later with a shunt dysfunction. To our knowledge, no similar cases of that age were reported in the literature.