Abernethy malformation in an adult with intermittent hematochezia

Cruz, K. C., Marcos, D., Cua, I. Y.

Significance: Abernethy malformation is rare anomaly of the splanchnic venous system wherein the portomesen­teric blood drains into a systemic vein. It is also an extremely rare cause of chronic lower gastrointestinal bleeding.

Case Presentation: We present a 33-year old female presenting with a 20-year history of intermittent hematochezia with multiple blood transfusion. She denied abdominal pain and changes in bowel habits. She is post-hemorrhoidectomy. Physical examination was unremarkable.

Management: Colonoscopy showed descending and sigmoid colon varices. Laboratory examinations were normal except for anemia(10g/dl) and hypoalbuminemia(2.9g/dL). Computed tomography of the whole abdomen and abdominal aorta showed an absent intrahepatic portal vein. Findings were suggestive of Type II Abernethy Malformation. Liver ultrasound and doppler showed intermittent portal venous flow in the expected location of the right and left portal vein, unobstructed hepatic venous system and increased resistance in the hepatic bed arterial flow. Patient underwent mesenteric venoangiogram for possible embolization which was aborted due to the variant vascular anatomy. She underwent mesocaval shunt creation between the Inferior Vena Cava and Superior Mesenteric vein and was maintained on Coumadin.

Recommendation: Abernethy malformation requires urgent diagnosis to prevent complications.  Patients may benefit from early surgery especially if embolization is not feasible.