Dandy -Walker Syndrome

1. DANDY-WALKER SYNDROME

2. Normal anatomy

4. Introduction
 Its rare developmental anomaly.

5. Clinical features
 Infancy: Symptoms and signs of hydrocephalus combined with a
prominent occiput.
 Childhood: Signs of cerebellar dysfunction with or without signs of
hydrocephalus.

6. INVESTIGATIONS
 Skull X-ray:
 Usually shows elevation of the transverse sinuses and occipital
bulging, confirming the presence of an enlarged posterior fossa.
 CT scan or MRI:
 dilated 4th ventricle
 Infusion of contrast into the ventricle will determine whether the
4th ventricle communicates with the rest of the ventricular
 Lateral displacement of hypoplastic cerebellar tissue.

7. MANAGEMENT
 When the dilated 4th ventricle communicates with the rest of the
ventricular system, a cysto-peritoneal shunt (CP shunt) suffices and
helps maintain a patent aqueduct.
 When a ‘two-compartment’ hydrocephalus exists, both the encysted 4th
ventricle and the other ventricles require drainage (i.e. with a cysto-
peritoneal and a ventriculo-peritoneal shunt).
 Excision of the cyst membrane (‘marsupialising’ the 4th ventricle) is no
longer thought to normalize CSF flow