1. Congenital Extrahepatic Portosystemic Shunts (Abernethy Malformation): An International Observational Study
- Author
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Baiges, Anna, Turon, Fanny, Simon-Talero, Macarena, Tasayco, Stephanie, Bueno, Javier, Zekrini, Kamal, Plessier, Aurelie, Franchi-Abella, Stephanie, Guerin, Florent, Mukund, Amar, Eapen, CE, Goel, Ashish, Shyamkumar, Nidugala Keshava, Coenen, Sandra, De Gottardi, Andrea, Majumdar, Avik, Onali, Simona, Shukla, Akash, Jose Carrilho, Flair, Nacif, Lucas, Primignani, Massimo, Tosetti, Giulia, La Mura, Vicenzo, Nevens, Frederik, Witters, Peter, Tripathi, Dhiraj, Tellez, Luis, Martinez, Javier, Alvarez-Navascues, Carmen, Fraile Lopez, Miguel Lopez, Procopet, Bogdan, Piscaglia, Fabio, de Koning, Barbara, Llop, Elba, Romero-Cristobal, Mario, Tjwa, Eric, Monescillo-Francia, Alberto, Senzolo, Marco, Perez-LaFuente, Mercedes, Segarra, Antonio, Sarin, Shiv Kumar, Hernandez-Gea, Virginia, Patch, David, Laleman, Wim, Hartog, Hermien, Valla, Dominique, Genesca, Joan, Carlos Garcia-Pagan, Juan, Garcia-Criado, Angeles, Darnell, Anna, Belmonte, Ernest, Ferrusquia-Acosta, Jose, Magaz, Marta, Vidal-Gonzalez, Judit, Horia, Stefanescu, Nicoara-Farcau, Oana, Joseph, Philip, Zachariah, Uday, Moses, Vinu, Mammen, Suraj, Ahmed, Munawwar, Koshy, George, Eapen, Anu, Sajith, KG, Vyas, FL, Raju, RS, Rymbai, Manbha L, Arulkumar, S, Ramachandran, Jeyamani, Elias, Elwyn, Baiges A., Turon F., Simon-Talero M., Tasayco S., Bueno J., Zekrini K., Plessier A., Franchi-Abella S., Guerin F., Mukund A., Eapen C.E., Goel A., Shyamkumar N.K., Coenen S., De Gottardi A., Majumdar A., Onali S., Shukla A., Carrilho F.J., Nacif L., Primignani M., Tosetti G., La Mura V., Nevens F., Witters P., Tripathi D., Tellez L., Martinez J., Alvarez-Navascues C., Fraile Lopez M.L., Procopet B., Piscaglia F., de Koning B., Llop E., Romero-Cristobal M., Tjwa E., Monescillo-Francia A., Senzolo M., Perez-LaFuente M., Segarra A., Sarin S.K., Hernandez-Gea V., Patch D., Laleman W., Hartog H., Valla D., Genesca J., Garcia-Pagan J.C., Gastroenterology & Hepatology, Pediatrics, and Surgery
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0301 basic medicine ,Male ,Vascular Malformations ,medicine.medical_treatment ,International Cooperation ,Liver transplantation ,0302 clinical medicine ,Malalties hereditàries ,Hepatopulmonary syndrome ,610 Medicine & health ,Child ,Hepatic encephalopathy ,Portal Vein ,Malalties del fetge ,Liver Neoplasms ,vascular diseases of the liver ,Middle Aged ,Abernethy ,Child, Preschool ,030211 gastroenterology & hepatology ,Female ,medicine.symptom ,Genetic diseases ,Adult ,medicine.medical_specialty ,Adenoma ,Adolescent ,Hypertension, Pulmonary ,Asymptomatic ,03 medical and health sciences ,Young Adult ,extrahepatic portosytemic shunt ,medicine ,Humans ,Liver diseases ,Aged ,Retrospective Studies ,Abernethy Malformation ,Hepatology ,business.industry ,Infant ,Retrospective cohort study ,medicine.disease ,Pulmonary hypertension ,Surgery ,Renal disorders Radboud Institute for Molecular Life Sciences [Radboudumc 11] ,030104 developmental biology ,Hepatic Encephalopathy ,Congenital malformation ,Portosystemic shunt ,business ,Hepatopulmonary Syndrome - Abstract
Contains fulltext : 220051.pdf (Publisher’s version ) (Closed access) 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 observational, 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.
- Published
- 2018