Abernethy Malformation Type 2 and Biliary Atresia Coexistence: A Rare Cause of Infantile Liver Transplant

Abstract

Introduction Abernethy malformation is a very rare condition in which mesenteric venous blood is drained into systemic circulation by an extra hepatic portosystemic shunt. Here we present a case of a female in infantile period who is a liver transplant candidate with biliary atresia and ventricular septal defect (VSD) accompanying Abernethy malformation type 2. Case report The patient who underwent Kasai operation at postnatal day 35 was identified as a liver transplant candidate in the postnatal month 6 due to growth retardation in the infantile period, insufficient weight gain and liver failure (portal hypertension and massive gastrointestinal bleeding). Conclusion Abernethy malformation is divided into two groups as type 1 and type 2; type 2 shunts, which are less common, but more common in males, are not accompanied by other congenital anomalies. Due to the female gender, having biliary atresia and VSD, the development of liver failure at an early stage despite undergoing Kasai operation at the appropriate time, our case differs from the literature. In conclusion, this case is presented in order to indicate the proper and effective use of imaging methods in neonatal cholestasis cases. How to cite this article: Ergon EY, Ermis N, Colak R, Polat B, Ozdemir S, Yildiz M, Kulali F, Ecevit C, Ergun O, Calkavur S. Abernethy Malformation Type 2 and Biliary Atresia Coexistence: A Rare Cause of Infantile Liver Transplant. Euroasian J Hepatogastroenterol, 2018;8(2):163-166.

Case Report: Membranoproliferative Glomerulonephritis, a Rare Clinical Manifestation of Abernethy Malformation Type II

“…Conservative treatment is selected in the absence of overt clinical symptoms. Closing parts of the collateral circulation and ligation of abnormal shunt vessels are considered, with excessive collateral circulation pressure, to be high risk for bleeding due to varicose veins, or intractable hepatic encephalopathy (16,(27)(28)(29). The number of cases similar to our patient reported globally remains limited.…”

Cystic biliary atresia with congenital absence of portal vein

“…Various associated anomalies with CAPV are cardiovascular (atrial septal defect, ventricular septal defect), hepatobiliary (biliary atresia, choledochal cyst), genitourinary (vesicoureteric reflux, pelviureteric junction obstruction), skeletal (vertebral anomalies, radial hypoplasia) and hepatic tumours (hepatocellular carcinoma, hepatoblastoma) 4. To the best of our knowledge, eight cases of biliary atresia associated with CAPV have been reported in the English literature 3–5. Polysplenia was seen in five cases (62.5%) 5.…”

“…To the best of our knowledge, eight cases of biliary atresia associated with CAPV have been reported in the English literature 3–5. Polysplenia was seen in five cases (62.5%) 5. However, the association of CBA with CAPV has never been reported.…”

“…Congenital absence of portal vein (CAPV) is a very uncommon anomaly. Few cases of CAPV have been documented to be associated with BA 3–5. However, this is the first report of CBA to be associated with CAPV.…”

Surgical Ligation for the Treatment of an Unusual Presentation of Type II Abernethy Malformation

“…Type II is rarely encountered but is more common in males and generally unrelated to other congenital anomalies. 4 In our case, we present a patient diagnosed with an unusual pattern of type II Abernethy malformation. To the best of our knowledge, this is the first reported case of type II Abernethy malformation presenting as a portosystemic shunt via the giant portal-inferior vena cava fistula.…”