Dandy walker syndrome with giant occipital meningocele

Case contributed by Ali Alsmair
Diagnosis certain

Presentation

A newborn patient brought to the radiology department with a large head and a mass protruding from the head-neck junction posteriorly.

Patient Data

Age: 1 day
Gender: Female

There are foci of diffusion restriction in the right periventricular white matter, bilateral parietal lobes and in the left temporal lobe, consistent with acute infarctions.

Evidence of a large posterior fossa cyst causing enlargement of the posterior fossa resulting in an indentation of the torcula herophili, and shows communication with the fourth ventricle resulting in moderate dilatation of the lateral and third ventricles. This large cyst shows communication with an occipital meningocele. They measure collectively about 7.5 x 9 x 15.5 cm (AP/TR/CC).
Hypoplasia of the vermis and cephalad rotation of the vermian remnant are also noted. 
This large cystic lesion causes compression of the brainstem and upper cervical cord, with resultant thinning. 
Features are consistent with Dandy Walker malformation with associated giant occipital meningocele.

Syringomyelia of most of the spinal cord is noted.

Segmentation anomalies of the cervical vertebrae.
Conus medullaris is located at the level of the mid-L2 vertebral body.
Normal corpus callosum. 
No intracranial hemorrhage or shift of midline structures. 
No tonsillar herniation. 
Normal sella.

Case Discussion

Giant occipital meningocele with Dandy walker syndrome is an extremely rare syndrome, only 31 cases reported till the year of 2017 1.

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