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Pseudotumor cerebri associated with modified Fontan anatomy
Hans Fuchs1, Dhiraj Singh, Clarence Greene
1Department of Pediatrics, Louisiana State University Health Sciences Center and Children's Hospital of New Orleans, 200 Henry Clay Avenue, New Orleans, LA, 70118, USA.
Pediatric Cardiology
|August 18, 2012
Summary
Patients with Fontan circulation may experience headaches due to elevated intracranial pressure. This case report highlights pseudotumor cerebri as a potential cause, treatable with shunting.
Area of Science:
- Cardiology
- Neurology
- Pediatric Surgery
Background:
- Modified Fontan procedure for single-ventricle heart disease can lead to elevated central venous pressure.
- Headaches are a common complaint in patients with Fontan circulation.
Observation:
- A 19-year-old male with hypoplastic left heart syndrome and Fontan palliation developed severe headaches post-collateral closure.
- Increased intracranial pressure was measured, and headaches resolved temporarily with lumbar puncture, suggesting pseudotumor cerebri.
Findings:
- The patient's pseudotumor cerebri and headaches significantly improved after lumboperitoneal shunt implantation.
- Elevated dural venous sinus pressure is implicated in pseudotumor cerebri pathogenesis.
- High vena cava pressures in Fontan anatomy may contribute to headaches.
Implications:
- Pseudotumor cerebri should be considered in Fontan patients with new-onset severe headaches.
- Management may involve cerebrospinal fluid diversion, such as shunting.
- Understanding the link between Fontan physiology and intracranial pressure is crucial for patient care.
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