Ventriculoperitoneal Shunting in Alobar Holoprosencephaly: Does it Work Even When Patient Has No Sign of Raised Intracranial Pressure?

Holoprosencephaly (HPE) is a developmental anomaly of forebrain characterized by a failure of division of the embryonic forebrain into hemispheres. It is associated with a set of facial anomalies at a rate of 80%. Survival rate, particularly in alobar HPE, is quite low. Alobar HPE is usually associated with a large dorsal cyst which might eventually lead to hydrocephalus and raised intracranial pressure. Placement of ventriculoperitoneal (VP) shunt has been reported to be beneficial in symptomatic hydrocephalus accompanying HPE. Here we report a preterm infant born with alobar HPE and undergoing VP shunt placement although there was no sign of raised intracranial pressure. She is 12 months old now having near-normal developmental progress. This case has revealed that the placement of VP shunt, particularly inserting the catheter tip into dorsal cyst of HPE, might be beneficial and contribute to the survival and further brain development even in the absence of the signs of raised intracranial pressure.