Lumboatrial shunt in a patient with Crouzon syndrome complicated by pseudotumor cerebri

A 25-year-old man with Crouzon syndrome complicated by pseudotumor cerebri and multiple shunt failures presented with progressive back and neck pain, intermittent headaches, and associated vomiting secondary to shunt infection. Due to his previous history of repeated failure of both ventriculoperitoneal and lumboperitoneal (LP) shunting procedures, the decision was made to place a lumboatrial (LA) shunt via an approach through the internal jugular vein. The procedure was uncomplicated and the man's symptoms were relieved. Despite significant improvement, the LA shunt limited his exercise tolerance, and as an avid runner and weight lifter, he requested reconversion back to an LP shunt. At a follow-up of 20 months, he continued to do well both clinically and radiographically. This case report summarizes the successful placement and use of an LA shunt for the treatment of intracranial hypertension in the setting of Crouzon syndrome.