Chiari III malformation with occipital encephalocele

Case contributed by Mostafa Elfeky
Diagnosis almost certain

Presentation

Large occipital cystic lesion since birth.

Patient Data

Age: 3 months
Gender: Male

An occipital meningoencephalocele is noted where part of posterior fossa contents, mainly the left cerebellar hemisphere and overlying meninges are herniating through a 1.8 cm defect in the right occipital region forming a cystic structure measuring 9 x 7.7 cm.

Crowded posterior fossa with downward displacement of the right cerebellar tonsil through the foramen magnum.

Distorted 4th ventricle and narrowed cerebral aqueduct with consequent mild supratentorial ventricular dilatation with no subependymal CSF permeation.

Mild cervical syrinx.

Case Discussion

Findings are consistent with large occipital meningoencephalocele, with herniated part of posterior fossa contents and secondary hydrocephalus and cervical syrinx, collectively representing Chiari III malformation.

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