title: Congenital extrahepatic portocaval shunt (Abernethy type 2), huge liver mass, and patent ductus arteriosus--a case report of its rare clinical presentation in a young girl.
abstract: The authors report a case of congenital extrahepatic portocaval shunt with a hypoplastic portal vein. This type of shunt is called an Abernethy type 2 shunt, where the portal vein and inferior vena cava are connected side to side, and a hypoplastic portal flow exists in the liver parenchyma. This case of a young girl is complicated with a cardiac anomaly and a large hyperplastic nodule in the liver. This phenotype usually is seen in the Abernethy type 1 shunt, which is a side-to-end shunt with the complete absence of a portal vein. In this context, the current case is very unique in its clinical manifestation, and this phenotype has never been reported. Therefore, it is important to speculate the etiology and treatment of this kind of abnormality and its associated hepatic mass.
authors.affiliation: Department of Pediatric Surgery and Department of Pathology, Tokyo University Hospital, Tokyo, Japan.
authors.forename: Yutaka
authors.initials: Y
authors.lastname: Kanamori
authors.forename: Kohei
authors.initials: K
authors.lastname: Hashizume
authors.forename: Yoshihiro
authors.initials: Y
authors.lastname: Kitano
authors.forename: Masahiko
authors.initials: M
authors.lastname: Sugiyama
authors.forename: Toru
authors.initials: T
authors.lastname: Motoi
authors.forename: Tsuyoshi
authors.initials: T
authors.lastname: Tange
mesh_terms: D000015:Abnormalities, Multiple / Q000601:surgery; D001284:Atrophy; D001921:Brain / Q000473:pathology; D002675:Child, Preschool; D003951:Diagnostic Errors; D004374:Ductus Arteriosus, Patent / Q000150:complications*; D005260:Female; D006390:Hemangioendothelioma / Q000175:diagnosis; D006801:Humans; D006965:Hyperplasia; D006975:Hypertension, Portal / Q000209:etiology; D008099:Liver / Q000098:blood supply / Q000473:pathology; D008113:Liver Neoplasms / Q000175:diagnosis; D008642:Mesenteric Veins / Q000002:abnormalities; D010641:Phenotype; D011169:Portal Vein / Q000002:abnormalities*; D013162:Splenic Vein / Q000473:pathology; D014682:Vena Cava, Inferior / Q000002:abnormalities*