Author:
Chira Romeo Ioan,Calauz Adriana,Manole Simona,Valean Simona,Mircea Petru Adrian
Abstract
Congenital extrahepatic portosystemic shunt (Abernethy malformation) is a rare condition characterized by developmental abnormalities of the portal venous system resulting in the diversion of the portal blood from the liver to the systemic venous system through a complete or partial shunt of the portomesenteric blood. We report the case of an 18 year-old female examined for abdominal pain, presenting cholestasis syndrome and an elevated serum aspartate aminotransferase level. Liver ultrasound examination revealed the absence of the portal vein with a complete extrahepatic shunt of the portal blood, multiple focal liver lesions, and multiple associated vascular anomalies. A surgical portosystemic shunt and a secondary portosystemic shunt due to portal vein thrombosis were excluded, enabling the diagnosis of a congenital portosystemic shunt. A complex investigation also discovered bone anomalies, and the liver biopsy of the dominant focal lesion revealed adenoma. On a short-term follow-up under hepatoprotective medication, the biochemical parameters improved mildly; however, the size of the main focal lesion increased.Congenital absence of the portal vein often remains an incidental diagnosis. In experienced hands, ultrasonography can diagnose it, but a comprehensive thoraco-abdominal evaluation is compulsory, considering the many potential associated anomalies. In these patients, development of adenomatous liver lesions secondary to Abernethy type Ib malformation represents an indication for liver transplantation.Abbreviations: AMA: anti-mitochondrial antibodies; ASMA: anti-smooth muscle antibody; ANA: anti-nuclear antibody; anti-LKM-1: liver-kidney microsomal type 1 antibodies; CEPS: congenital extrahepatic portosystemic shunt; IVC: inferior vena cava; SMV: superior mesenteric vein; SV: splenic vein.
Publisher
Romanian Society of Gastroenterology and Hepatology
Cited by
6 articles.
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