Incidence of Secondary Hydrocephalus after Excision of Huge Encephaloceles in Neonates: Case Study

Background  Encephaloceles presents as a protrusion of the cranial contents through a defect in the cranium. The most common sites of occurrence are the occipital and frontonasal regions. The surgical outcome is reported to be satisfactory; however, the incidence of hydrocephalus in patients with encephaloceles is variable in the literature. This study investigated the relationship between the size of the encephaloceles and the occurrence of hydrocephalus. Patient and Methods  Data of all neonates with encephaloceles who presented to our institution from September 2012 to September 2014 were collected. Surgery was performed during the first 2 weeks of age. Encephaloceles with a maximal diameter > 10 cm were included in the study, and the clinical picture, surgical technique, pre- and postoperative imaging, and follow-up were analyzed. Results  Nineteen cases were included in this study. The mean follow-up period was 7 months. Sixteen cases were occipital; three were frontal. In all patients the maximum diameter was > 10 cm. However, in four patients it was > 18 cm. Postoperative ventriculomegaly occurred in seven cases. Of these, four patients needed a permanent ventriculoperitoneal shunt implantation (21%). Wound dehiscence occurred in two patients who required secondary sutures with a favorable outcome. One patient died 2 weeks after the surgery due to a poor general condition and wound infection. Conclusion  Early surgical excision provides effective treatment of huge encephaloceles. Overall, 21% of cases require cerebrospinal fluid (CSF) diversion afterward depending on associated anomalies. Despite their size, giant encephaloceles can have an excellent prognosis with no need for further treatment or CSF diversion.