The favorable prognosis of cystic biliary atresia may be related to early surgery and mild liver pathological changes

Zheng, Qipeng; Yang, Fang; Yan, Zhao; Liu, Gengxin; Mengdi, Li; Hu, Xiaoli; Zhan, Jianghua

doi:10.1007/s00383-021-05030-w

Cited by 5 publications

(2 citation statements)

References 28 publications

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“…Early diagnosis and surgery were not contributing factors to the favorable outcomes of patients with patent intact hepatic ducts. This finding contrasts with the previous findings reported by others, where they attributed the favorable outcomes to the younger age at surgery for patients with cystic biliary atresia (2,11). A further pathophysiological investigation is required to identify the contributing factors to favorable outcomes in cystic biliary atresia with patent intact hepatic ducts.…”

Section: Discussioncontrasting

confidence: 99%

“…Although ALT and GGT at the time of surgery were comparable, this rapid improvement suggests better bile drainage (although bilirubin levels were not significantly better) and possibly less fibrotic damage to the liver parenchyma before the surgery. This finding is in line with a recent report where cystic biliary atresia exhibited fewer fibrotic features in biopsy histology obtained at the time of diagnosis (11). Although it is rare, investigating distinct subgroups will help understand the etiology of biliary atresia by segregating a unique cohort from the heterogenic population.…”

Section: Discussionsupporting

confidence: 93%

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The Outcome of Patients With Cystic Biliary Atresia With Intact Proximal Hepatic Ducts Following Hepatic-Cyst-Jejunostomy

Asai

Wang

et al. 2022

Journal of Pediatric Gastroenterology &Amp; Nutrition

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Objectives: To determine the outcomes of patients with cystic biliary atresia by correlating the anatomy of the hepatic ducts with the choice of biliary reconstruction surgery. Background: The Kasai hepatoportoenterostomy (Kasai) is the initial surgical procedure offered to most patients with biliary atresia. In contrast, a hepatic-cyst-jejunostomy has been reported to be effective in patients with the cystic form of biliary atresia. Methods and Results:We performed an international multicenter retrospective review. Two hundred eighty-seven patients were included, and 33 cases of cystic biliary atresia were identified. Outcomes were the serum total bilirubin level 3 months post-surgery and native liver survival at 2 years of age and were compared between cases who received the Kasai versus hepatic-cyst-jejunostomy in correlation to the anatomy of proximal hepatic ducts. The patients were categorized into 3 anatomical groups: patent intact hepatic ducts (n = 10), patent hypoplastic hepatic ducts (n = 13), and obliterated hepatic ducts (n = 10). All 10 patients with patent intact hepatic duct group underwent hepatic-cyst-jejunostomy, and 9 experienced bile drainage and native liver survival. Among the 13 patients with hypoplastic hepatic ducts, 11 underwent the Kasai procedure, and 9 had bile drainage, whereas 2 underwent hepatic-cyst-jejunostomy, and one survived with the native liver. All of the patients with obliterated hepatic ducts underwent the Kasai procedure; 5 established biliary drainage and survived with the native liver. Of 5 who did not drain, 3 underwent liver transplantation.Conclusions: In patients with cystic biliary atresia, the subset with a connection between cyst and intrahepatic bile ducts via intact proximal hepatic ducts had favorable clinical outcomes following hepatic-cyst-jejunostomy.

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Section: Discussioncontrasting

confidence: 99%

Section: Discussionsupporting

confidence: 93%