IMPORTANT: We currently have a number of bugs related to image cropping and are actively trying to resolve them. In the meantime, we have disabled cropping. Apologies for any inconvenience. Stay informed: radiopaedia.org/chat

Atretic occipital cephalocele

Case contributed by Tamer O. Abdo
Diagnosis certain

Presentation

Occipital swelling.

Patient Data

Age: 7 years
Gender: Male

Brain CT

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.

Brain MRI & MRV

mri

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.

How to use cases

You can use Radiopaedia cases in a variety of ways to help you learn and teach.

Creating your own cases is easy.

Updating… Please wait.

 Unable to process the form. Check for errors and try again.

 Thank you for updating your details.