Liver Transplantation in an Adult With Adenomatosis and Congenital Absence of the Portal Vein: A Case Report

Gordon-Burroughs, S.; Balogh, Julius; Weiner, Morris; Monsour, Howard Paul; Schwartz, Mary R.; Gaber, A. Osama; Ghobrial, Rafik M.

doi:10.1016/j.transproceed.2014.04.012

Cited by 21 publications

(16 citation statements)

References 14 publications

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“…1 HCC Gordon-Burroughs et al (35) (2014) 1 HCC Fernandez-Vega et al (36) (2014) 1 No Patients with GSD IA Matern et al (14) (1999) 14 No Labrune (15) (2002) 1 HCC Lerut et al (13) (2003) 1 HCC Franco et al (37) (2005) 1 HCC Arikan et al (38) (2006) 13 No Davis and Weinstein (39) (2008) 4 No Reddy et al (16) (2009) 1 HCC Manzia et al (40) (2011) 2 HCC…”

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confidence: 99%

Liver transplantation for adenomatosis: European experience

et al. 2016

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The aim of this study was to collect data from patients who underwent liver transplantation (LT) for adenomatosis; to analyze the symptoms, the characteristics of the disease, and the recipient outcomes; and to better define the role of LT in this rare indication. This retrospective multicenter study, based on data from the European Liver Transplant Registry, encompassed patients who underwent LT for adenomatosis between January 1, 1986, and July 15, 2013, in Europe. Patients with glycogen storage disease (GSD) type IA were not excluded. This study included 49 patients. Sixteen patients had GSD, and 7 had liver vascular abnormalities. The main indications for transplantation were either a suspicion of hepatocellular carcinoma (HCC; 15 patients) or a histologically proven HCC (16 patients), but only 17 had actual malignant transformation (MT) of adenomas. GSD status was similar for the 2 groups, except for age and the presence of HCC on explants (P = 0.030). Three patients with HCC on explant developed recurrence after transplantation. We obtained and studied the pathomolecular characteristics for 23 patients. In conclusion, LT should remain an extremely rare treatment for adenomatosis. Indications for transplantation primarily concern the MT of adenomas. The decision should rely on morphological data and histological evidence of MT. Additional indications should be discussed on a case‐by‐case basis. In this report, we propose a simplified approach to this decision‐making process.

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confidence: 99%

Liver transplantation for adenomatosis: European experience

et al. 2016

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“…Interventional radiology procedures include coil embolization for small shunts [8] and use of vascular plugs for large high flow ones [9]. Surgical options [10,11] include shunt ligation for extrahepatic CPSS, liver resection for large multifocal intrahepatic CPSS or focal malignant liver lesions or failed embolization and finally liver transplantation [12] for CPSS with complete agenesis of the portal vein. This child had a large intrahepatic CPSS which was not amenable to embolization or vascular plug insertion due to fear of migration.…”

Section: Discussionmentioning

confidence: 99%

Management of Hepatopulmonary Syndrome in a Child Due to a Large Congenital Intrahepatic Porto-Systemic Shunt

Kedarisetty¹,

Kamble²,

Joseph³

et al. 2019

IJG

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Background: Abernethy malformations are rare vascular anomalies of the portal system which present as extrahepatic congenital portosystemic venous shunts (CPSS). Sometimes they can be intra-hepatic anomalies. There is scarcity of literature on management of these rare anomalies especially intra-hepatic shunts. Case: A five years old child came with the complaints of progressive breathlessness on exertion with effort-intolerance for the past two years. There was no history suggestive of underlying cardiopulmonary illness. On examination, there was cyanosis and clubbing. On evaluation, the imaging showed a large congenital intra-hepatic portosystemic shunt from the left portal vein draining directly into intrahepatic inferior vena cava (IVC) and a hypoplastic right branch of the portal vein leading to a clinical presentation of hepatopulmonary syndrome. Result: The shunt was occluded by placing a covered stent in the IVC across the shunt opening, making sure the openings of hepatic veins and renal vein also were not being covered. There was a significant improvement in oxygenation post procedure with complete disappearance of cyanosis. Conclusion: Covered IVC stent placement is a novel technique for large fusiform dilated intra-hepatic CPSS by closing the shunt flow into the IVC, thereby restoring the physiological flow in the liver.

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“…To our knowledge, this is the first report of kidney transplantation in a CAPV patient. In contrast, a number of CAPV cases that had liver transplantation have been reported . However, they have largely focused on the surgical aspects of this disease including resection of complicated hepatic tumor and reconstruction of the portal vein or bile duct …”

Section: Discussionmentioning

confidence: 99%

“…In contrast, a number of CAPV cases that had liver transplantation have been reported. 7 However, they have largely focused on the surgical aspects of this disease including resection of complicated hepatic tumor 7 and reconstruction of the portal vein or bile duct. 8 We previously noted that the CyA level of the patient had been abnormally elevated when she was given CyA microemulsion for the pediatric disease at age 12 years.…”

Section: Discussionmentioning

confidence: 99%

Pharmacokinetic analysis of cyclosporine in a renal transplant recipient with congenital absence of the portal vein

et al. 2015

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Here we report therapeutic drug monitoring of cyclosporine in a kidney transplant recipient lacking enterohepatic circulation. The patient developed steroidresistant nephrotic syndrome at age 14 years, and was medicated with an oral cyclosporine microemulsion. However, her cyclosporine trough level was unexpectedly elevated, and subsequent investigations showed that she was deficient in drug metabolism as a result of the congenital absence of the portal vein. Her renal function gradually decreased and she became dialysis-dependent at the age of 21 years, and kidney transplantation was planned. Based on pretransplant therapeutic drug monitoring, we started cyclosporine microemulsion at half of the conventional dosage. After transplantation, the dosage was successfully adjusted to achieve a target trough level. The post-transplant course was stable with no symptoms of rejection or cyclosporineassociated nephrotoxicity.

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Liver Transplantation in an Adult With Adenomatosis and Congenital Absence of the Portal Vein: A Case Report

Cited by 21 publications

References 14 publications

Liver transplantation for adenomatosis: European experience

Liver transplantation for adenomatosis: European experience

Management of Hepatopulmonary Syndrome in a Child Due to a Large Congenital Intrahepatic Porto-Systemic Shunt

Pharmacokinetic analysis of cyclosporine in a renal transplant recipient with congenital absence of the portal vein

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