The congenital porto‐caval fistula: A unique presentation and novel intervention

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 ane...

Full description

Saved in:
Bibliographic Details
Published in:Pediatric pulmonology 2008-02, Vol.43 (2), p.196-199
Main Authors: Hoover, Wynton, Ackerman, Veda, Schamberger, Marcus, Kumar, Manjusha, Marshalleck, Francis, Hoyer, Mark
Format: Article
Language:English
Subjects:
abernethy malformation
Adolescent
Aminoacid disorders
Anemia - complications
Anemias. Hemoglobinopathies
Biological and medical sciences
Chronic Disease
congenital porto‐caval fistula
Cough - etiology
Diseases of red blood cells
Dyspnea - etiology
Errors of metabolism
General aspects
Hematologic and hematopoietic diseases
hemolytic anemia
Humans
hyperammonemia
Hyperammonemia - complications
Hyperammonemia - etiology
Hypertension, Pulmonary - etiology
Learning Disorders - etiology
Male
Medical sciences
Metabolic diseases
Pneumology
Portal Vein - diagnostic imaging
portosystemic shunt
Pulmonary Artery - pathology
pulmonary hypertension
Pulmonary hypertension. Acute cor pulmonale. Pulmonary embolism. Pulmonary vascular diseases
Stents
Thrombocytopenia - complications
Tomography, X-Ray Computed
Treatment Outcome
Vascular Fistula - complications
Vascular Fistula - congenital
Vascular Fistula - diagnosis
Vascular Fistula - diagnostic imaging
Vascular Fistula - therapy
Vena Cava, Inferior - diagnostic imaging
Citations: Items that this one cites
Items that cite this one
Online Access:Get full text
Tags: Add Tag
No Tags, Be the first to tag this record!
Description
Summary: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.
ISSN:8755-6863
1099-0496
DOI:10.1002/ppul.20727