Caroli's disease: Liver resection and liver transplantation. Experience in 33 patients

Kassahun, Woubet Tefera; Kahn, Thomas; Wittekind, Christian; Mössner, Joachim; Caca, Karel; Hauss, Johann; Lamesch, P.

doi:10.1016/j.surg.2005.05.002

Cited by 111 publications

(138 citation statements)

References 36 publications

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“…The occurence of hepatobiliary malignant transformation explained by chronic inflammation of the biliary tree, has been reported in 7%-14% of patients. Death is related to liver failure or complications of portal hypertension [18,19] .…”

Section: Complicationsmentioning

confidence: 99%

Clinical characteristics of Caroli’s syndrome

Yönem

Bayraktar²

2007

WJG

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Caroli's syndrome is characterized by multiple segmental cystic or saccular dilatations of intrahepatic bile ducts associated with congenital hepatic fibrosis. The clinical features of this syndrome reflect both the characteristics of congenital hepatic fibrosis such as portal hypertension and that of Caroli's disease named as recurrent cholangitis and cholelithiasis. The diagnosis depends on both histology and imaging methods which can show the communication between the sacculi and the bile ducts. Treatment consists of symptomatic treatment of cholangitis attacks by antibiotics, some endoscopic, radiological and surgical drainage procedures and surgery. Liver transplantation seems the ultimate treatment for this disease. Prognosis is fairly good unless recurrent cholangitis and renal failure develops.

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Section: Complicationsmentioning

confidence: 99%

Clinical characteristics of Caroli’s syndrome

Yönem

Bayraktar²

2007

WJG

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“…4 The pathology starts in the intrauterine period, 5 and it is characterized by multifocal fibrocystic dilatation of the intrahepatic bile ducts. [4][5][6] Caroli disease can be seen at any age, 4 but it is more common in those in their twenties. 7 Most of the patients present to the hospital with abdominal pain, fever, hyperbilirubinemia, elevated alkaline phosphatase, hepatomegaly, or symptoms of portal hypertension.…”

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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“…Surgical management of CD depends on the extent of the disease process. In localized or monolobar CD, segmental or lobar hepatic resection can be done, especially, when associated with lobar atrophy [9,10]. Resection not only alleviates the septic complications but also prevents development of CCA in the affected lobe.…”

Section: Discussionmentioning

confidence: 99%