Gastroenterologie
a hepatologie

Congenital extrahepatic portosystemic shunt (Abernethy malformation) complicated by the development of multiple hepatocellular carcinoma –  description of two cases

Irena Míková¹, Jana Jarošová¹, Darina Cupalová², Andrea Vajsová³, Ondřej Fabián^3,4, Eva Sticová ^{Orcid.org  5}, Tomáš Hucl ^{Orcid.org  1}, Libor Janoušek ^{Orcid.org  6}, Jiří Froněk ^{Orcid.org  7}, Pavel Taimr ^{Orcid.org  1}

+ Affiliation
¹ Klinika hepatogastroenterologie, Transplantcentrum, IKEM, Praha
² Pracoviště radiodiagnostiky a intervenční radiologie, IKEM, Praha
³ Pracoviště klinické a transplantační patologie IKEM, Praha
⁴ Ústav patologie a molekulární medicíny 3. LF UK a FTN, Praha
⁵ Pracoviště klinické a experimentální patologie, Transplantcentrum, IKEM, Praha
⁶ Klinika transplantační chirurgie IKEM Praha
⁷ Klinika transplantační chirurgie, Transplantcentrum, IKEM, Praha

Summary

Introduction: Congenital extrahepatic portosystemic shunt (CEPS; Abernethy malformation) can be complicated by hepatic encephalopathy, pulmonary hypertension, or hepatocellular carcinoma (HCC). We present two cases of patients with multiple HCC and simultaneous CEPS. Case descriptions: 17-year old girl with a large portocaval shunt was sent to our clinic for progressive multiple liver nodules with signs of FNH/ adenomas according to MRI. Target bio­psy of one of the nodules proved to be hepatocellular adenoma with risky histological signs, where the finding was not resectable and the patient was indicated for liver transplantation (LT). During uncomplicated LT, the portocaval shunt was closed. The liver explant showed multiple adenomas, some of them have already been transformed to well differentiated HCC. The patient is 2.5 years after LT with good liver function, and without recurrence of HCC. A 62-year old man with a large portosystemic shunt between the superior mesenteric vein and inferior vena cava and with a history of one episode of hepatic encephalopathy was sent to our clinic for a CT finding of two liver nodules with signs of HCC in the field of advanced liver fibrosis (F3 according to elastography). MRI showed 6 liver nodules with a size up to 25 mm, all with signs of HCC, and AFP was 496 µg/ l. The findings exceeded IKEM indication criteria of LT for HCC and the patient was indicated to oncological therapy. Conclusion: Abernethy malformation is associated with increased risk of benign (adenomas) and malignant liver tumours (HCC); thorough surveillance of the patients is necessary.

Keywords

congenital extrahepatic portosystemic shunt, Abernethy malformation, hepatocellular carcinoma, liver transplantation

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