Behrens-Baumann-Dust Syndrome

Microphthalmos, Dandy-Walker anomaly, and ocular anomalies (optic disc atrophy, lid adhesion, and/or ankyloblepharon).

Oculocerebral Dysplasia.

Two sibs have been described. Autosomal recessive inheritance was suggested.

A 16-year-old girl with bilateral microphthalmos, aplasia of the right optic nerve, and a large cerebellar Dandy-Walker cyst was described. She was mentally normal, and no clinical consequences from the Dandy-Walker cyst were mentioned. Her 13-year-old brother had unilateral optic nerve aplasia with ipsilateral cryptophthalmus and contralateral microphthalmos. He suffered from significant mental retardation.

If a Dandy-Walker malformation is present with signs of compression and increased intracranial pressure, a lumbar puncture for spinal anesthesia is contraindicated and anesthetic management must address the problem of raised intracranial pressure. Adequate anxiolysis is often helpful in the management of mentally delayed patients, but in the presence of increased intracranial pressure may have more adverse than beneficial effects. No other specific anesthetic implications are expected from the described ophthalmologic features.