Dandywalker syndrome

This rare developmental anomaly comprises:

1. Dilatation of the lateral and third ventricles (but to a lesser extent than the fourth ventricle) .

2. Widely separated, hypoplastic cerebellar hemispheres, with a small hypoplastic vermis, displaced rostrally.

Other developmental anomalies occur in 65% of patients.

2. Widely separated, hypoplastic cerebellar hemispheres, with a small hypoplastic vermis, displaced rostrally.

Enlarged posterior fossa with high tentorium cerebelli and transverse sinuses.

Cystic dilatation of the 4th ventricle - usually related to congenital absence of the foramina of Luschka and Magendic.

5. Thin, transparent membrane containing ependymal colls and occasionally cerebellar tissue.

Enlarged posterior fossa with high tentorium cerebelli and transverse sinuses.

Cystic dilatation of the 4th ventricle - usually related to congenital absence of the foramina of Luschka and Magendic.

5. Thin, transparent membrane containing ependymal colls and occasionally cerebellar tissue.

CLINICAL PRESENTATION

Infancy: Symptoms and signs of hydrocephalus (page 361) combined with a prominent occiput. Childhood: Signs of cerebellar dysfunction with or without signs of hydrocephalus.

INVESTIGATIONS

Skull X-ray: Usually shows elevation of the transverse sinuses and occipital bulging, confirming the presence of an enlarged posterior fossa.

CT scan:

Confirms dilatation of the 4th ventricle with lateral displacement of hypoplastic cerebellar tissue.

Differentiate from:

— Midline arachnoid cyst

— Englarged cisterna magna distinguish from Dandy-Walker by identifying cerebellar tissue or septum between the cyst and the 4th ventricle

MANAGEMENT

Excision of the cyst membrane, 'marsupialising' the 4th ventricle. Alternatively, and more simply, cystoperitoneal shunt.

PROGNOSIS

Marked neurological impairment prior to treatment carries a poor outlook. In less impaired patients, the prognosis relates more to the presence of other developmental anomalies.

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