Caroli's disease and orthotopic liver transplantation

Habib, Shahid; Shakil, Obaid; Couto, O. F. M.; Demetris, Anthony J.; Fung, John J.; Marcos, Amadeo; Chopra, Kapil

doi:10.1002/lt.20719

Cited by 82 publications

(93 citation statements)

References 17 publications

(15 reference statements)

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“…4,7 In the literature, most patients who are treated with liver transplant for Caroli disease are adult patients, 8 and graft and patient survival rates are no different from those shown in patients who undergo orthotopic liver transplant for other causes of chronic liver disease. 7,8 Our patient was an 8-year-old female patient. She had good graft survival with no complications and no rejection episodes.…”

Section: Discussionmentioning

confidence: 99%

Untitled

Akdur

Kırnap²,

Soy³

et al. 2017

Exp Clin Transplant

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Objectives: This study sought to evaluate the efficacy of liver transplant for unusual liver diseases. Materials and Methods:The results of 476 patients who underwent liver transplant from 1988 to January 2015 were retrospectively analyzed. Two hundred forty-five of them were adult patients and 231 of them were pediatric. Thirty-one patients had unusual liver disease. Results: Of the 31 patients with unusual liver disease, 9 (29%) were adult and 22 (71%) were pediatric patients. In the pediatric group, indications for liver transplant were Alagille syndrome (n = 5), Crigler-Najjar syndrome type 1 (n = 5), glycogen storage disease (n = 3), oxalosis (n = 3), familial hypercholesterolemia (n = 2), alpha-1-antitrypsin deficiency (n = 2), Caroli disease (n = 1), and cystic neuroblastoma metastasis (n = 1). Six patients (27.2%) had acute rejection episodes and were successfully treated with pulse steroids. In 2 patients, retransplant was performed for chronic rejection. Three patients died during follow-up (13.6%): 2 because of sepsis and 1 because of cranial hemorrhage. In the adult group, indications for liver transplant were neuroendocrine tumor metastasis (n = 1), liver angiosarcoma (n = 1), familial hypercholesterolemia (n = 2), alveolar hydatid disease (n = 2), cystic fibrosis (n = 1), congenital hepatic fibrosis (n = 1), and oxalosis (n = 1). Four patients (44.4%) had acute rejection episodes and were successfully treated with pulse steroid therapy. One patient died due to the recurrence of primary disease (liver angiosarcoma) during follow-up (11.1%). Conclusions: Advances in liver transplant and our understanding about unusual liver disease have led to significant improvements in managing these diseases. Liver transplant effectively treats the underlying defect and the complications of portal hypertension, or risk of malignancy for those disorders, in which the liver is affected.

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Section: Discussionmentioning

confidence: 99%

Untitled

Akdur

Kırnap²,

Soy³

et al. 2017

Exp Clin Transplant

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“…7,[11][12][13][14] The most common indication for transplantation in these series was recurrent cholangitis. Most patients had associated polycystic kidney disease or hepatic fibrosis.…”

Section: Discussionmentioning

confidence: 99%

“…In a report from the University of Pittsburgh, 10 of 30 patients required retransplantation, and in this report, the overall mortality was 39%. 7 The examination of the European Liver Transplant Registry, the largest to date, however, showed an overall survival of 80.9% in 110 patients with Caroli's disease 11 ; of these, 16 patients had simultaneous liver and kidney transplants, and 15 patients were retransplanted, likely because of vascular thrombosis. A detailed analysis of the European registry, however, was not provided in this study.…”

Section: Discussionmentioning

confidence: 99%

“…Similarly, we also did not find a survival advantage in younger patients as reported previously. 7 The optimal management of Caroli's disease is not well defined because of the rarity of the disease and paucity of published data. The disease may present as monolobar or multilobar disease.…”

Section: Discussionmentioning

confidence: 99%

“…Although extended hepatectomies could be performed for multilobar disease, it is not recommended, as liver transplantation is associated with a better outcome on the basis of small studies. 1,[6][7][8] The objective of the current study was to examine the posttransplant outcomes of an unselected cohort of patients with Caroli's disease with the United Network for Organ Sharing (UNOS) database.…”

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confidence: 99%

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Caroli's disease and outcomes after liver transplantation

2007

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Caroli's disease is a rare autosomal recessive disorder characterized by intrahepatic cystic dilatation of the bile ducts that, when progressive, leads to intrahepatic stones, recurrent cholangitis, portal hypertension, cholangiocarcinoma, and liver failure. Liver transplantation is a promising curative option for advanced Caroli's disease. The aim of this study was to determine the outcomes of liver transplantation in unselected patients with Caroli's disease and recommend an evidencebased therapeutic algorithm for the management of Caroli's disease. Of the 78,124 patients transplanted in the United States between 1987 and 2006, 104 had Caroli's disease; 96 of these underwent liver alone, and 8 underwent combined liver/kidney transplantation. The patient survival and graft survival were analyzed by Kaplan-Meier survival analysis, and risk of death and risk of graft loss were analyzed by Cox proportional hazards regression. The overall 1-, 3-, and 5-year graft (79.9%, 72.4%, and 72.4%) and patient (86.3%, 78.4%, and 77%) survival rates were excellent for patients after liver transplantation. For combined liver/kidney transplantation (n ϭ 8), the 1-year patient survival and graft survival were 100%. Proportional hazards analysis identified Asian ethnicity, elevated bilirubin, requirement of life support or hospitalization prior to transplantation, and a cold ischemia time greater than 12 hours as associated with increased risk of both graft loss and death. A history of prior transplant or prior abdominal surgery was also associated with increased risk of graft loss. In conclusion, liver transplantation is an excellent treatment option for patients with advanced Caroli's disease and should be considered in a timely fashion to prevent worsening complications including refractory cholangitis and cholangiocarcinoma.

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Percutaneous closure of nonrestrictive aortopulmonary window in three infants

Trehan¹,

Nigam²,

Tyagi³

2008

Cathet Cardio Intervent

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Aortopulmonary (AP) window, a communication between ascending aorta and pulmonary artery, is usually nonrestrictive and causes severe pulmonary vascular obstructive disease early in life. Only in 10% of the cases it is restrictive. There are sporadic case reports [1-8] of device closure of AP window which are mostly confined to these restrictive AP windows, that too in adults or relatively older children. Till date there is a single case report of device closure of nonrestirctive AP window in an infant [8]. We report our single experience of device closure of large, nonrestrictive AP windows in 3 infants. Percutaneous closure of AP window in each of the three patients was done by a different type of device i.e. duct occluder, muscular VSD occluder and perimembranous VSD occluder.

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Caroli's disease and orthotopic liver transplantation

Cited by 82 publications

References 17 publications

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Caroli's disease and outcomes after liver transplantation

Percutaneous closure of nonrestrictive aortopulmonary window in three infants

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