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The congenital porto‐caval fistula: A unique presentation and novel intervention
Author(s) -
Hoover Wynton,
Ackerman Veda,
Schamberger Marcus,
Kumar Manjusha,
Marshalleck Francis,
Hoyer Mark
Publication year - 2008
Publication title -
pediatric pulmonology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.866
H-Index - 106
eISSN - 1099-0496
pISSN - 8755-6863
DOI - 10.1002/ppul.20727
Subject(s) - medicine , hyperammonemia , coagulopathy , presentation (obstetrics) , fistula , surgery , pediatrics
Congenital porto‐caval fistulas are uncommon vascular malformations with a varied clinical presentation beginning in infancy and spanning late adulthood. We report a 14‐year‐old male who presented with dyspnea and cough. His past medical history was significant for a chronic non‐immune hemolytic anemia, thrombocytopenia, coagulopathy and a learning disability. He was found to have severe pulmonary hypertension and hyperammonemia associated with a large congenital porto‐caval fistula. The abnormal vessel was occluded via endovascular covered stent placement in the vena cava. His pulmonary hypertension has improved remarkably while his chronic anemia, thrombocytopenia and ammonia have normalized to allow improved cognitive performance. Pediatr Pulmonol. 2008; 43:196–199. © 2007 Wiley‐Liss, Inc.