Posterior fossa infected dermoid with congenital heart disease: A novel hypothesis of an unusual association

Intracranial dermoid cysts commonly present as a discharging sinus, local swelling, mass lesion, or abscess formation. These can sometimes be found in association with congenital anomalies. The author presents two original cases of infected posterior fossa dermoid associated with congenital heart diseases (CHDs) that is very rare. The embryologic basis for this unique occurrence is reviewed, and a new hypothesis proposed. Two infants with CHD presented with infected midline posterior fossa dermoid. Excision of the dermoid cyst with the sinus tract was performed. Postoperative period was uneventful. Both the infants had undergone surgery for congenial heart disease a few months prior to the present clinical presentation with uneventful recovery. Infected posterior fossa dermoid cyst without a discharging sinus should prompt a thorough examination to detect CHD. Early diagnosis and timely management results in better outcome.