Unusual Discovery after an Examination for Abdominal Pain: Abernethy 1b Malformation and Liver Adenomatosis. A Case Report.
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.
1st Medical Clinic, Department of Gastroenterology;Iuliu Hatieganu University of Medicine and Pharmacy, Cluj-Napoca, Romania. romeochira@yahoo.com.
Romeo Ioan
RI
Chira
Pneumophthisiology Clinical Hospital, Cluj-Napoca, Romania.
Adriana
A
Calauz
Iuliu Hatieganu University of Medicine and Pharmacy;Emergency County Hospital, Department of Radiology, Cluj-Napoca, Romania.
Simona
S
Manole
1st Medical Clinic, Department of Gastroenterology;Iuliu Hatieganu University of Medicine and Pharmacy, Cluj-Napoca, Romania.
Simona
S
Valean
1st Medical Clinic, Department of Gastroenterology;Iuliu Hatieganu University of Medicine and Pharmacy, Cluj-Napoca, Romania.
Petru Adrian
PA
Mircea
D015746:Abdominal Pain / Q000000981:diagnostic imaging / Q000209:etiology*; D000236:Adenoma / Q000000981:diagnostic imaging / Q000209:etiology*; D000293:Adolescent; D005260:Female; D006801:Humans; D008113:Liver Neoplasms / Q000000981:diagnostic imaging / Q000209:etiology*; D011169:Portal Vein / Q000002:abnormalities* / Q000000981:diagnostic imaging; D014057:Tomography, X-Ray Computed; D014463:Ultrasonography; D054079:Vascular Malformations / Q000150:complications* / Q000000981:diagnostic imaging