Congenital Varicella Syndrome with Aplasia Cutis Congenita and Aqueduct Stenosis: A Rare Combination

Pritish Kumar Sahoo, Anwesha Panigrahi

Abstract: Congenital varicella syndrome is a rare disorder characterised by skin scars, limb deficits, ocular and CNS manifestation. However congenital varicella presenting as Aplasia cutis with obstructive hydrocephalus is extremely rare in literature. Aplasia cutis congenita is characterised by localised or generalised condition characterised by absence of skin. Hydrocephalus is a pressure dependent enlargement of the cerebral ventricles. Hydrocephalus caused by aqueductal anomaly is extremely rare. I report a case of Congenital varicella syndrome presenting with generalised tonic clonic seizure, with MRI Brain showing aqueductal stenosis with Aplasia cutis congenita on the scalp.

Keywords: Congenital varicella syndrome, Aplasia cutis congenita, hydrocephalus, aqueduct stenosis, vp shunt