Atretic occipital cephalocele

Case contributed by Dr Tamer O. Abdo

Presentation

Occipital swelling.

Patient Data

Age: 7 years
Gender: Male
CT

Brain CT

There is a well-marginated defect with a thin sclerotic border in the midline of the occipital bone measuring 8.5 x 3.3 mm. Superficial to the defect, a well-defined ovoid heterogeneous lesion with areas of fluid attenuation is seen in the scalp measuring 1.8 x 0.8 x 1.9 cm, which appears to communicate with the intracranial space.

Enlarged retrocerebellar CSF space with an intact cerebellar vermis. No communication with the fourth ventricle is seen. Findings are compatible with a mega cisternal magna.

MRI

Brain MRI & MRV

There is a small midline occipital scalp lesion measuring about 2 x 1.6 x 0.8 cm connected to the brain through a small midline occipital bone defect measuring about 8 x 4 mm. It appears heterogeneous and of mixed-signal intensity mainly in T2 images. No vessels, fat, or significant enhancement seen in the mentioned lesion.

No associated intracranial abnormalities.

Case Discussion

Cephaloceles represent herniated intracranial content through a skull defect.

Atretic cephaloceles are involuted cephaloceles containing meninges and neural crests and represent about 50% of all cephaloceles.

The majority of reported cases occurred in the parietal region, although some reports suggest equal incidence on both sides of the lambdoid suture (parietal and occipital).

An increased incidence of intracranial anomalies has been reported with atretic cephalocele, and these include Dandy-Walker syndrome, Walker-Warburg syndrome, and holoprosencephaly, among others.

Our patient went on to have surgical excision of the cephalocele. Histopathology found meningeal tissue within.

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Case information

rID: 83154
Published: 18th Oct 2020
Last edited: 18th Oct 2020
Inclusion in quiz mode: Included
Institution: Ibn Al Haitham hospital