Hydrocephalus associated with congenital intracranial anomalous venous return: a case report and review of the literature

Objective To study the clinical features and management of the hydrocephalus associated with congenital intracranial anomalous venous return. Methods Combined with review of the literatures, the history and clinical data of a patient suffered from hydrocephalus associated with congenital intracranial anomalous venous return, whose diagnosis was confirmed by CT, MRI, MRV scan and DSA and programmable ventriculoperitoneal shunting was given, were analysed. Results The hydrocephalus associated with congenital intracranial anomalous venous return was rare. Commonly, the patient had a portwine-colored nevus of the head and neck at birth, a better tolerance to intracranial hypertention. Damage to the venous return may induce the decompensation of intracranial pressure regulation. DSA can make sure of the anomalous venous return. Programmable ventriculoperitoneal shunting can control the shunting pressure according to the patient's reaction at any time. A little higher shunting pressure was often necessary in order to keep the venous return. Conclusions Congenital intracranial anomalous venous return should be considered to those who had a port-wine-colored nevus of the head and neck at birth, DSA is helpful to confirm the diagnosis, programmable ventriculoperitoneal shunting can control the rapid increase in head circumference due to the hydrocephalus associated with congenital intracranial anomalous venous return safely and effectively. Key words: Anomalous venous return;  Hydrocephalus;  Sturge-Weber syndrome