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Hepatopulmonary syndrome (HPS) is a rare pulmonary vascular complication of chronic liver disease characterized by dilatation of pulmonary capillaries leading to vascular shunting and systemic hypoxemia. Diagnosis of HPS requires documentation of intrapulmonary vasodilation (IPVD), the two most common imaging studies performed for the detection of IPVD include transthoracic contrast echocardiography (TTCE) and 99m-Tc-macroaggregated albumin scintigraphy (99mTc-MAA scan). TTCE has high sensitivity and thus, is the preferred initial investigation, while 99mTc-MAA scan is highly specific and plays an adjuvant role in diagnosis.

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A 32-year-old man presented with cirrhosis. At 8 years of age, he underwent resection of a craniopharyngioma, which resulted in panhypopituitarism. He underwent self-interrupted hormone replacement therapy at 20 years of age.

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Case Report: Type Ib Abernethy malformation.

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January 2025

Department of Radiology, West China Hospital, Sichuan University, Chengdu, PR China. Electronic address:

Congenital extrahepatic portosystemic shunt, also known as Abernethy malformation, is a rare anatomic vascular malformation. Patients with Abernethy malformation may present with abdominal pain, abnormal liver function tests, hepatopulmonary syndrome, pulmonary hypertension, and/or portosystemic encephalopathy. Accurate identification of the shunt and portal vein and effective management of complications is vital in these patients.

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When encountering severe hypoxemia that does not respond to oxygen supplementation, it is essential to consider underlying right-to-left shunting. Among various diagnostic approaches, the microbubble test via transthoracic echocardiography (TTE) is a simple, noninvasive method for detecting pulmonary arteriovenous shunts, particularly in hepatopulmonary syndrome (HPS). Although microbubbles are usually administered peripherally, using a Swan-Ganz (SG) catheter to inject microbubbles directly into the pulmonary artery may provide even more definitive diagnostic information.

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Childhood-onset craniopharyngiomas, though rare, are intracranial malformations that can cause obesity by disrupting the hypothalamus, a condition that often persists even after tumor resection. This severe obesity increases the risk of diabetes and fatty liver disease in childhood. Concurrently, panhypopituitarism, including growth hormone (GH) deficiency, may develop.

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