Congenital intrahepatic arterioportal fistula presenting as severe undernutrition and chronic watery diarrhea in a 2-year-old girl.
Intrahepatic arterioportal fistula (IAPF) is a rare cause of portal hypertension in young children. We report the case of a 2-year-old girl with severe undernutrition, chronic watery diarrhea, and gastrointestinal bleeding because of a congenital intrahepatic arterioportal fistula. Radiographic embolization and surgical ligation of the left hepatic artery were attempted, with no resolution of the symptoms. So, a left lobectomy was performed, with excellent results and prompt disappearance of the diarrhea. Hepatectomy should be considered as a definitive and reliable therapy for congenital IAPF.
Pediatric Surgery Division, Pediatric Liver Transplantation Unit and Laboratory of Research in Pediatric Surgery (LIM 30), University of Sao Paulo Medical School, Sao Paulo, Brazil. cristannuri@hotmail.com
Ana Cristina Aoun
AC
Tannuri
Uenis
U
Tannuri
Fabiana Roberto
FR
Lima
Luiz Roberto Schlaich
LR
Ricardi
Antonio José Gonçalves
AJ
Leal
Marcos Marques
MM
da Silva
D001164:Arteriovenous Fistula / Q000175:diagnosis / Q000601:surgery*; D001165:Arteriovenous Malformations / Q000175:diagnosis / Q000601:surgery; D002648:Child; D015362:Child Nutrition Disorders / Q000175:diagnosis / Q000628:therapy; D004621:Embolization, Therapeutic / Q000379:methods; D004932:Esophageal and Gastric Varices / Q000601:surgery; D005260:Female; D006471:Gastrointestinal Hemorrhage / Q000601:surgery; D006498:Hepatectomy / Q000379:methods*; D006499:Hepatic Artery / Q000002:abnormalities* / Q000601:surgery*; D006801:Humans; D006975:Hypertension, Portal / Q000601:surgery; D008026:Ligation / Q000379:methods; D011169:Portal Vein / Q000002:abnormalities* / Q000601:surgery*; D016896:Treatment Outcome; D003969:Vipoma / Q000175:diagnosis / Q000628:therapy