Management of hydrocephalus associated with occipital encephalocoele using endoscopic third ventriculostomy: report of two cases

Abstract

Background: Occipital encephalocoele is the most common cranial dysraphism in the western hemisphere and is often complicated by hydrocephalus. Management of hydrocephalus and reducing the CSF pressure is crucial in preventing dehiscence at the site of the encephalocoele repair. Methods: Two female patients had presented with occipital encephalocoeles. The first patient (aged 42 days) had undergone repair of the occipital encephalocoele and then developed hydrocephalus with recurrence of the encephalocoele. The second patient (aged 12 months) had hydrocephalus associated with an occipital encephalocoele at initial presentation. Both the patients underwent endoscopic third ventriculostomy (ETV) through a right frontal burr hole. In the first patient, ETV was performed after shunt dysfunction at the age of 9 months. Because she presented with recurrence of the encephalocoele 15 months later, a repeat endoscopic third ventriculostomy was performed. She required a ventriculoperitoneal shunt during the same admission because of the early failure of the ventriculostomy. In the second patient, it was performed before the encephalocoele repair, both ETV and the repair being conducted under the same anesthesia. ETV was performed using a rigid scope and the perforation in the third ventricular floor was enlarged using a No. 4 Fogarty catheter. Results: The first patient had no recurrence of encephalocoele at follow-up of 10 months but she presented with recurrence of the occipital encephalocoele after 15 months. The second patient had no evidence of recurrence at follow-up after 16 months. The lateral and third ventricular volumes had decreased in both the patients at initial follow-up. Conclusion: ETV can be an effective treatment option for encephalocoele-associated hydrocephalus, even in children under the age of 1 year. It may obviate the need for placement of CSF shunts that have a risk of infection and dysfunction. However, delayed failure of ETV may occur as seen in our first patient, indicating the need for careful and long-term follow-up.