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

Czech version

Authors: I. Miková ¹; J. Jarošová ¹; D. Cupalová ²; A. Vajsová ³; O. Fabián ^3,4; E. Sticová ³; T. Hucl ¹; L. Janoušek ⁵; J. Froněk ⁵; P. Taimr ¹
Authors‘ workplace: Klinika hepatogastroenterologie, Institut klinické a experimentální medicíny, Praha ¹; Pracoviště zobrazovacích metod, Institut klinické a experimentální medicíny, Praha ²; Pracoviště klinické a transplantační patologie, Institut klinické a experimentální medicíny, Praha ³; 2. ústav patologie a molekulární medicíny 3. LF UK a FTN, Praha ⁴; Klinika transplantační chirurgie, Institut klinické a experimentální medicíny, Praha ⁵
Published in: Gastroent Hepatol 2025; 79(4): 247-253
Category: Case Report
doi: https://doi.org/10.48095/ccgh2025247

Overview

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

Sources

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ORCID autorů

I. Míková 0000-0001-7928-7558,

J. Jarošová 0000-0003-0010-0637,

D. Cupalová 0000-0002-6332-0461,

A. Vajsová 0000-0002-8473-8664,

O. Fabián 0000-0002-0393-2415,

E. Sticová 0000-0003-2486-6266,

T. Hucl 0000-0002-5648-4011,

L. Janoušek 0000-0001-7572-8416,

J. Froněk 0000-0003-2379-3886,

P. Taimr 0000-0002-6272-4608.

Doručeno/ Submitted: 16. 7. 2025