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Congenital Extrahepatic Portosystemic Shunts (Abernethy Malformation): An International Observational Study
Author(s) -
Baiges Anna,
Turon Fanny,
SimónTalero Macarena,
Tasayco Stephanie,
Bueno Javier,
Zekrini Kamal,
Plessier Aurélie,
FranchiAbella Stéphanie,
Guerin Florent,
Mukund Amar,
Eapen C.E.,
Goel Ashish,
Shyamkumar Nidugala Keshava,
Coenen Sandra,
Gottardi Andrea,
Majumdar Avik,
Onali Simona,
Shukla Akash,
Carrilho Flair José,
Nacif Lucas,
Primignani Massimo,
Tosetti Giulia,
Mura Vicenzo,
Nevens Frederik,
Witters Peter,
Tripathi Dhiraj,
Tellez Luis,
Martínez Javier,
ÁlvarezNavascués Carmen,
Fraile López Miguel López,
Procopet Bogdan,
Piscaglia Fabio,
Koning Barbara,
Llop Elba,
RomeroCristobal Mario,
Tjwa Eric,
MonescilloFrancia Alberto,
Senzolo Marco,
PerezLaFuente Mercedes,
Segarra Antonio,
Sarin Shiv Kumar,
HernándezGea Virginia,
Patch David,
Laleman Wim,
Hartog Hermien,
Valla Dominique,
Genescà Joan,
GarcíaPagán Juan Carlos
Publication year - 2020
Publication title -
hepatology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 5.488
H-Index - 361
eISSN - 1527-3350
pISSN - 0270-9139
DOI - 10.1002/hep.30817
Subject(s) - medicine , asymptomatic , portosystemic shunt , hepatocellular carcinoma , liver transplantation , portal hypertension , hepatopulmonary syndrome , surgery , portopulmonary hypertension , hepatic encephalopathy , incidence (geometry) , cirrhosis , radiology , transplantation , physics , optics
Congenital extrahepatic portosystemic shunt (CEPS) or Abernethy malformation is a rare condition in which splanchnic venous blood bypasses the liver draining directly into systemic circulation through a congenital shunt. Patients may develop hepatic encephalopathy (HE), pulmonary hypertension (PaHT), or liver tumors, among other complications. However, the actual incidence of such complications is unknown, mainly because of the lack of a protocolized approach to these patients. This study characterizes the clinical manifestations and outcome of a large cohort of CEPS patients with the aim of proposing a guide for their management. This is an o bservational, multicenter, international study. Sixty‐six patients were included; median age at the end of follow‐up was 30 years. Nineteen patients (28%) presented HE. Ten‐, 20‐, and 30‐year HE incidence rates were 13%, 24%, and 28%, respectively. No clinical factors predicted HE. Twenty‐five patients had benign nodular lesions. Ten patients developed adenomas (median age, 18 years), and another 8 developed HCC (median age, 39 years). Of 10 patients with dyspnea, PaHT was diagnosed in 8 and hepatopulmonary syndrome in 2. Pulmonary complications were only screened for in 19 asymptomatic patients, and PaHT was identified in 2. Six patients underwent liver transplantation for hepatocellular carcinoma or adenoma. Shunt closure was performed in 15 patients with improvement/stability/cure of CEPS manifestations. Conclusion: CEPS patients may develop severe complications. Screening for asymptomatic complications and close surveillance is needed. Shunt closure should be considered both as a therapeutic and prophylactic approach.