Reduction cranioplasty for macrocephaly with long-standing hydrocephalus and non-fused fontanelle in Chiari malformation type I

Introduction: Because hydrocephalus is diagnosed and treated at an early stage in pediatric patients, pediatric neurosurgeons rarely encounter patients with hydrocephalic macrocephaly. There are even fewer cases of infants with long-standing hydrocephalus in whom macrocephaly progresses and is accompanied by skull defect due to malunion of suture lines despite long-term CSF diversion treatment.

Case report: We report the case of a male infant with Chiari malformation type I who presented with congenital hydrocephalus and occipital encephalocele that progressed to hydrocephalic macrocephaly with frontal skull defect, despite numerous cerebrospinal fluid diversion operations. The patient eventually recovered successfully after reduction cranioplasty.