Hydrocephalus associated with occipital encephalocele: surgical management and clinical outcome

Abstract

Abstract Background Encephaloceles are cystic congenital malformations in which central nervous system (CNS) structures, in communication with cerebrospinal fluid (CSF) pathways, herniate through a defect in the cranium. Hydrocephalus occurs in 60–90% of patients with occipital encephaloceles. Objective Assessment of the surgical management of hydrocephalus associated with occipital encephalocele and its effect on the clinical outcome. Methods Between October 2015 and October 2019, a retrospective study was conducted on seventeen children with occipital encephaloceles who were operated upon. The presence of progressive hydrocephalus was determined by an abnormal increase in head circumference and an increase in the ventricular size on imaging studies. A ventriculoperitoneal (VP) shunt was applied in patients who had hydrocephalus. The clinical outcome was graded according to the developmental milestones of the children on outpatient follow-up visits. Results The mean age at surgery was 1.6 (range, 0–15) months. There were ten girls (58.8%) and seven boys (41.2%). Ten encephaloceles (58.8%) contained neural tissue. Ten patients (58.8%) had associated cranial anomalies. Eleven children (64.7%) had associated hydrocephalus: four of them (36.4%) diagnosed preoperatively, while seven children (63.6%) developed hydrocephalus postoperatively. Ten of them (90.9%) were managed by VP shunt. All children with hydrocephalus had some degree of developmental delay, including six (54.5%) with mild/moderate delay and five (45.5%) with severe delay. Half of the patients (50%) of the children with occipital encephalocele without hydrocephalus had normal neurological outcome during the follow-up period (p value= 0.034). Conclusions Occipital encephalocele is often complicated by hydrocephalus. The presence of hydrocephalus resulted in a worse clinical outcome in children with occipital encephalocele, so it can help to guide prenatal and neonatal counseling.