Open AccessCongenital extrahepatic portosystemic shunt: description of four cases and review of the literature
Author(s) -
Francesca Romana Ponziani,
Mariella Faccia,
Maria Assunta Zocco,
V. Giannelli,
A. Pellicelli,
Giuseppe Maria Ettorre,
Nicoletta De Matthaeis,
Fabrizio Pizzolante,
Anna Maria De Gaetano,
Laura Riccardi,
Maurizio Pompili,
Gian Ludovico Rapaccini
Publication year - 2018
Publication title -
journal of ultrasound
Language(s) - English
Resource type - Journals
eISSN - 1971-3495
pISSN - 1876-7931
DOI - 10.1007/s40477-018-0329-y
Subject(s) - medicine , portosystemic shunt , portopulmonary hypertension , neonatal cholestasis , radiology , portal hypertension , liver biopsy , shunt (medical) , venography , abdominal pain , hepatic encephalopathy , cirrhosis , biopsy , liver transplantation , biliary atresia , thrombosis , transplantation
Congenital extrahepatic portosystemic shunt (CEPS), also known as Abernethy malformation, is a rare condition in which the splenomesenteric blood drains directly into a systemic vein, bypassing the liver through a complete or partial shunt. The diagnosis is frequently made during childhood in the setting of neonatal cholestasis, hypergalactosemia, failure to thrive, mental retardation or other congenital defects. In adulthood, CEPS is usually found incidentally during diagnostic work-up for abdominal pain, liver test abnormalities, liver nodules, portopulmonary hypertension, portopulmonary syndrome or portosystemic encephalopathy. The diagnosis depends on imaging and portal venography, but sometimes only liver biopsy can be resolutive, demonstrating the absence of venules within the portal areas. Here we report four recent cases of Abernethy malformation diagnosed in young adults, in which ultrasound (US) was the initial imaging technique and allowed to suspect the diagnosis. Furthermore, we reviewed clinical presentations, associated anomalies and treatment of the 310 cases of CEPS previously reported in the literature.