Indication of liver transplantation for jaundice-free biliary atresia with portal hypertension

Introduction Portal hypertension often occurs in biliary atresia (BA). The subsequent development of esophageal varices and bleeding from these varices are a well-known complication. We aim to describe the incidence and severity of variceal bleeding in patients with BA. In addition, we describe the characteristics of patients who experienced variceal bleeds. Materials and Methods We included all infants treated for BA at our center between March 1987 and August 2015. Variceal bleeding was defined as hematemesis and/or melena with presence of varices at endoscopy. Findings at endoscopy and ultrasound, laboratory tests, clearance of jaundice, fibrosis-grade at Kasai portoenterostomy, and several varices prediction scores were documented. Routine endoscopies were not performed. Results In this study, 74 patients were included. During follow-up, 18 out of 74 patients (24%) developed variceal bleeding at an age of 9 months (range, 4–111). Twelve patients were listed for liver transplantation at the time of bleeding. Patients who did not clear their jaundice developed variceal bleeds more often and earlier in life. Bleeds were treated with sclerotherapy, banding, or octreotide. Four patients did not receive treatment. No bleeding-related mortality occurred. Conclusion One-fourth of the children diagnosed with BA experience variceal bleeds during follow-up. Most of these children are younger than 1 year and often already listed for transplantation. Major complications did not occur after variceal bleeding.

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A young adult with biliary atresia and liver decompensation

Thai Journal of Hepatology ◽

10.30856/th.jhep2019vol2iss1_10 ◽

2019 ◽

Vol 2 (1) ◽

pp. 38-40

Author(s):

Panyavee Pitisuttithum ◽

Piyawat Komolmit

Keyword(s):

Liver Transplantation ◽

Liver Cirrhosis ◽

Lymph Node ◽

Portal Hypertension ◽

Biliary Atresia ◽

Liver Decompensation ◽

Native Liver ◽

Peribiliary Cysts ◽

Collateral Vessels ◽

Paraesophageal Lymph Node

Biliary atresia is a progressive fibro-obliterative disease of the bile duct, commonly diagnosed within a few months of life. After hepatoportoenterostomy was successfully performed, about one fourth of biliary atresia patients have grown up to the age of twenty with their native liver. However, progression to liver cirrhosis, development of portal hypertension, or cholangitis are the major problems of adult with biliary atresia. Monitoring of these complications and listing for liver transplantation whenever indicated are the key when caring adult with biliary atresia. Figure 1 แสดง CT abdomen พบว่ามีตับโต และมีลักษณะของตับแข็งและ ภาวะความดันของระบบหลอดเลือด portal สูงขึ้น (collateral vessels, splenomegaly, ascites) พบการขยายตัวของท่อน้ำดีในตับ (multifocal dilated ducts and peribiliary cysts along bilateral intrahepatic ducts) และต่อมน้ำเหลืองโตในช่องท้อง (mesenteric, gastrohepatic, hepatoduodenal, aortocaval, para aortic, right anterior diaphragmatic, lower paraesophageal lymph node ขนาดสูงสุด 1.8 ซม.)

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Extrahepatic Biliary Atresia: The Importance of Early Referral

JMS SKIMS ◽

10.33883/jms.v17i1.234 ◽

2014 ◽

Vol 17 (1) ◽

pp. 39-40

Author(s):

Wani Sajad ◽

Bhat Nisar ◽

Aejaz Baba ◽

Gowhar Mufti ◽

Khursheed Ahmad Sheikh

Keyword(s):

Liver Transplantation ◽

Liver Cirrhosis ◽

Portal Hypertension ◽

Biliary Atresia ◽

Bile Ducts ◽

Early Referral ◽

Diffusion Of Information ◽

Extrahepatic Biliary Atresia ◽

Extrahepatic Bile Ducts ◽

And Diffusion

Extrahepatic biliary atresia (EHBA), characterized by obliteration or discontinuity of extrahepatic bile ducts, is still the major cause for liver transplantation among children nowadays [1]. All untreated children eventually die due to complications resulting from portal hypertension and liver cirrhosis. The exchange and diffusion of information that can make the diagnosis of EHBA easier is of utmost importance, since prognosis is improved when patients are surgically treated by portoenterostomy in the first 2 months of life. JMS 2014;17(1):39-40

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Congenital Cholestatic Syndromes: What Happens When Children Grow Up?

Canadian Journal of Gastroenterology ◽

10.1155/2007/581247 ◽

2007 ◽

Vol 21 (11) ◽

pp. 743-751 ◽

Cited By ~ 12

Author(s):

Simon C Ling

Keyword(s):

Liver Transplantation ◽

Portal Hypertension ◽

Biliary Atresia ◽

Alagille Syndrome ◽

Nutritional Deficiencies ◽

Published Data ◽

Caroli Disease ◽

Progressive Encephalopathy ◽

Biliary Sepsis ◽

Affected Parent

Although advances in the management of children with congenital cholestasis have enabled many to survive into adulthood with their native livers, even the most common of these conditions remains rare in adult hepatology practice. Among four congenital cholestatic syndromes (biliary atresia, Alagille syndrome, Caroli disease and congenital hepatic fibrosis, and progressive familial intrahepatic cholestasis), the published data on outcomes of the syndromes into adulthood suggest that a spectrum of severity of liver disease can be expected, from cirrhosis (almost universal in adults with biliary atresia who have not required liver transplantation) to mild and subclinical (eg, in the previously undiagnosed affected parent of an infant with Alagille syndrome). Complications associated with portal hypertension and nutritional deficiencies are common, and other associated features of the cholestatic syndrome may require appropriate attention, such as congenital heart disease in Alagille syndrome. Indications for liver transplantation include synthetic failure, progressive encephalopathy, intractable pruritus, recurrent biliary sepsis and recurrent complications of portal hypertension. Improved understanding of biliary physiology will hopefully translate into improved therapy for children and adults with cholestasis.

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De novo gastric cancer developing after liver transplantation from deceased donor for biliary atresia: a case report

Surgical Case Reports ◽

10.1186/s40792-021-01210-x ◽

2021 ◽

Vol 7 (1) ◽

Author(s):

Naruki Higashidate ◽

Suguru Fukahori ◽

Shinji Ishii ◽

Nobuyuki Saikusa ◽

Naoki Hashizume ◽

...

Keyword(s):

Gastric Cancer ◽

Liver Transplantation ◽

Biliary Atresia ◽

De Novo ◽

Epigastric Pain ◽

Gastroesophageal Junction ◽

Deceased Donor ◽

Lymphoproliferative Disease ◽

Endoscopic Examination ◽

De Novo Malignancy

Abstract Background Apart from Kasai’s procedure, liver transplantation (LTx) has dramatically improved the outcome of children with biliary atresia (BA). However, de novo malignancy has been reported to be one of the major causes of late mortality after LTx among adults. We report a rare case of de novo gastric cancer developing after LTx for BA received during childhood. Case presentation A 21-year-old male patient who had undergone LTx for BA at age 2 years occasionally visited our outpatient clinic due to symptoms of epigastric pain and dysphagia. Endoscopic examination and computed tomography revealed advanced gastric cancer at the gastroesophageal junction with multiple liver metastases. Despite systemic chemotherapy, the disease progressed, resulting in patient’s death 2 years after the diagnosis. Conclusions De novo malignancy in the absence of post-transplant lymphoproliferative disease is rare in pediatric patients who received LTx. To the best of our knowledge, no report has been available on the development of gastric cancer after LTx for BA during childhood. Primary physicians should therefore establish a follow-up plan for patients receiving LTx for BA considering the potential for the development of de novo malignancy, including gastric cancer, despite its rarity.

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