A GIANT OCCIPITAL ENCEPHALOCYSTOCELE: A CASE REPORT

Muhammad Teguh Irianto1 , Muhammad Mundzir Amining1, Handoyo Pramusinto2,

1
Resident of Neurosurgery Division, Department of Surgery, Sardjito General Hospital,
Faculty of Medicine, Public Health and Nursing, Gadjah Mada University, 2 Division of
Neurosurgery, Department of Surgery, Sardjito General Hospital, Faculty of Medicine, Public
Health and Nursing, Gadjah Mada University.

ABSTRACT
Encephalocystocele is a developmental malformation characterized by
brain herniation accompanied by extracranial cystic protrusion of the ventricular
system. Most of the encephaloceles are located on the occipital/posterior part in
Caucasians, whereas encephaloceles in Asians more often appear frontally. The
occipital encephalocystocele that contains the cerebral parenchyma, involving
the cavity linked to the ventricular system, is located above the external
occipital protuberance containing the migrated occipital lobe and the posterior
horn of the lateral ventricle.
A 1-year-old boy weighing 2,730 g was born transvaginally at 33 weeks.
The baby exhibited a large occipital cephalocele (14 x 17 cm in diameter)
covered with skin. No cerebrospinal fluid (CSF) leakage was observed during
the examination. The fontanelle was closed, and no other congenital
abnormalities. Her mother had not been taking the folic supplement.
This case report will discuss a case of a giant occipital
encephalocystocele. This case has no relation with any known reported
syndromes, and it also will be a follow-up on the outcome after 1-year post-
surgery. 1-year after surgery, the child's development is still within normal
limits. At this time, the child can stand unaided and speak 3-5 words clearly.

Keywords: Giant Encephalocystocele Occipital; Neural Tube Defect;

Congenital Malformation