Pseudotumor cerebri associated with modified fontan anatomy

Surgical treatment of functional single-ventricle heart disease with a modified Fontan procedure results in elevated central venous pressure. The case report describes a 19-year-old boy with hypoplastic left heart syndrome and Fontan palliation that resulted in frequent debilitating headaches after transcatheter closure of systemic venous-to-pulmonary venous collaterals. Measured increased intracranial pressure and prompt relief of the headaches with lumbar puncture favored a diagnosis of pseudotumor cerebri. After implantation of a lumboperitoneal shunt, the patient's headaches improved dramatically. Headaches are frequent in patients with Fontan circulation. The current literature supports a pivotal role of elevated dural venous sinus pressure in the pathogenesis of pseudotumor cerebri. The high superior and inferior vena caval pressures characteristic of modified Fontan anatomy may account for headaches in some of these patients.