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Description

Abernethy syndrome, known as congenital portosystemic shunt syndrome (CEPS), is characterized by an aberrant connection between the mesenteric and systemic vessels. Normally, the splenic vein (SV) and superior mesenteric vein (SMV) form the portal vein which carries blood to the hepatic sinusoids. Then, blood is returned to the systemic system via the inferior vena cava (IVC). CEPS is divided into type 1 where portal blood bypasses the liver entirely, and type 2 where a hypoplastic portal vein delivers blood to the liver and IVC. Manifestations range from incidental imaging findings to hepatopulmonary syndrome. Early radiological evaluation enables timely medical-surgical intervention. Our report studies a 7 year old female with developmental and endocrine abnormalities found to have CEPS on imaging. We will review the radiological findings, notable manifestations and treatment options.

Publication Date

5-9-2025

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2025 Research Day Corewell Health West, Grand Rapids, MI, May 9, 2025. Abstract 1694

The Many Images of Abernethy Syndrome; Unraveling the Radiologic, Laboratory, and Clinical Findings in an Adolescent Patient Presenting with Congenital Portosystemic Shunt

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