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Case of the Month

by Dr Noreen Norfaraheen Lee Abdullah MD (UKM), MMed Radiology (UKM), AM(Mal)

Answer to Case Of the Month 
Occipital Encephalocoele

Discussion of Findings
The head is distorted in shape. There is a defect at the occipital bone with herniation of brain tissue (the occipital lobe) and its ventricles together with the meningeal covering through the defect area. This is an occipital encephalocele.

Within the encephalocele, the cerebrospinal fluid (CSF) is intermixed with blood. CSF is also seen within the subcutaneous tissue. There is intraventricular hemorrhage with dilated ventricles in the cranium. Layering of the blood is demonstrated. There is also intraparenchymal hemorrhage in the vertex. Blood tracked along the falx cerebri, which will be consistent with subarachnoid hemorrahge.

Occipital Encephalocoele

This phenomenon occurs because there is a mesodermal defect in the skull and dura resulting in extracranial extension of the intracranial structures. Encephaloceles are located in the midline and the most common region to arise from is the occipital region. The next common is at the nasofrontal area. Less commonly encountered are the lateral encephalocele, anterior fontanelle encephalocele, orbital encephalocele and base of skull encephalocele.

Occipital encephaloceles are the most serious as they contain portions of the cerebellum or occipital lobes. Removal will result in much neurological deficit. Nasofrontal encephaloceles are more of a cosmetic problem. Orbital encephalocele occurs when there is a defect in the sphenoid bone. Brain tissue protrudes into the orbit and the patient presents with exopthalmos, which is pulsating in nature.

Encephaloceles are readily diagnosed clinically. The child presents with a bulging external soft tissue mass. The underlying calvarial defect is easily seen radiographically. Imaging with CT scan demonstrates more information and tells us the contents within the encephalocele. Infratentorial and supratentorial structures are herniated into the defect with equal frequency in occipital encephaloceles.

Ventriculomegaly is a common finding. Brain hemorrhage occurs quite frequently as the occipital encephalocele is easily traumatized during delivery. The prognosis is poor with 50% survival in newborns and 74% will be mentally retarded.
About the Author 
Dr Noreen, a Consultant Radiologist is currently with the Universiti Sains Malaysia, Kubang Kerian, Kelantan, Malaysia.
 
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Tuesday, 04 January 2005