Resolution of hepatopulmonary syndrome after auxiliary partial orthotopic liver transplantation in Abernethy malformation. A case report.
Congenital absence of portal vein and extrahepatic portocaval shunt, also referred to as an Abernethy type 1, is a rare malformation of the mesenteric vasculature. A 9-year-old girl presented with dyspnea on exertion and orthodeoxia. A diagnosis of an Abernethy malformation type 1b and hepatopulmonary syndrome (HPS) was made, and she underwent auxiliary partial orthotopic liver transplantation (APOLT). Symptoms and signs of HPS resolved 2 months after the operation. HPS in Abernethy syndrome is a manifestation of normal portal blood flow bypassing the liver and can be ameliorated by redirecting blood flow to a segment of liver with normal portal anatomy. APOLT is a feasible and successful surgical procedure for patients with Abernethy malformation and HPS.
Department of Pediatrics, Mount Sinai Medical Center, Mount Sinai School of Medicine, New York, NY, USA. sukru.emre@yale.edu
Sukru
S
Emre
Ronen
R
Arnon
Emil
E
Cohen
Raffaella A
RA
Morotti
Dmitriy
D
Vaysman
Benjamin L
BL
Shneider
D002648:Child; D004417:Dyspnea / Q000209:etiology / Q000503:physiopathology / Q000601:surgery; D005260:Female; D020065:Hepatopulmonary Syndrome / Q000150:complications / Q000473:pathology / Q000503:physiopathology / Q000601:surgery*; D006801:Humans; D008102:Liver Circulation; D016031:Liver Transplantation; D018810:Magnetic Resonance Angiography; D010690:Phlebography; D011169:Portal Vein / Q000002:abnormalities* / Q000473:pathology / Q000503:physiopathology; D012039:Regional Blood Flow; D016896:Treatment Outcome