Abernethy malformation type 1b - acute spontaneous hepatic hemorrhage

Case contributed by Dr Liz Silverstone

Presentation

Abrupt onset shock and neck pain, systolic blood pressure 75 mmHg

Patient Data

Age: 40
Gender: Female

Large acute subcapsular hepatic hematoma compressing the liver parenchyma. Moderate hemoperitoneum.

Anomalous portal vein draining into the confluence of the IVC and hepatic veins, bypassing the liver.

Large hepatic artery and veins. Enlarged peripheral hepatic veins. Heterogeneous liver enhancement. Elongated right lobe.

Right pleural effusion and pulmonary atelectasis. Scoliosis.

Case Discussion

This rare malformation is known as Abernethy type 1b, occurring in females who may present with congenital heart disease.

This patient was diagnosed with ASD, VSD and cor triatriatum in childhood and these required surgical correction. Her congenital extrahepatic portosystemic shunt was not recognized until this acute presentation as she has no features of hepatic encephalopathy. Her portal vein connects to the IVC bypassing the liver; the liver is exclusively supplied by the hepatic artery. 

Hepatic angiogram demonstrated large hepatic arteries, but no active hemorrhage or vascular malformations. There were no hepatic tumors. Hemorrhage was presumably related to the enlarged blood vessels. The subcapsular hematoma resolved following percutaneous drainage and laparoscopic washout by the liver surgeon.

NB. Practical point: include vasculature in your systematic review. Vascular anomalies and diseases are frequently overlooked!

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