Surgical Repair of a Giant Encephalocele with Chiari Malformation Type III- A Therapeutic Challenge

Surgical Repair of a Giant Encephalocele with Chiari Malformation

Abstract

An encephalocele is a congenital neural tube defect characterized by herniation of the intracranial contents through a defect in the cranium. It is caused by failure of closure of the cranial part of the developing neural tube during the first few weeks of foetal life. An encephalocele is called a giant encephalocele when its size is larger than the size of the head. Surgical procedures for giant occipital encephalocele are challenging for both the neurosurgeons and anaesthesiologists due to the large size, associated intracranial anomalies, intraoperative blood loss, intraoperative hypothermia, and prolonged anaesthesia. The index case was antenatally diagnosed to have an encephalocele. Postnatal examination revealed a large meningoencephalocele 10* 10 cm size in the occipital region. MRI brain was suggestive of occipital meningoencephalocele with syrinx involving cervical spinal cord. Baby was operated on day twenty of life. Post operatively, wound site healed with primary intention and was discharged after ten days and child was neurologically normal at the time of discharge.