The Prognosis of Type III Biliary Atresia with Hilar Cyst
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ORIGINAL ARTICLE
The Prognosis of Type III Biliary Atresia with Hilar Cyst Quan-yuan Shan 1 & Bao-xian Liu 1 & Zhi-hai Zhong 2 & Hua-dong Chen 2 & Yu Guo 3 & Xiao-yan Xie 1 & Wen-ying Zhou 1 & Hong Jiang 2 & Lu-yao Zhou 1 Received: 14 November 2019 / Accepted: 26 October 2020 # Dr. K C Chaudhuri Foundation 2020
Abstract Objective To compare the outcome of biliary atresia (BA) patients with and without hilar cyst on preoperative ultrasound. Methods A single center retrospective review of patients of BA with (n = 27) and without hilar cyst (n = 27) over a 5 y period was done. The patients were analyzed using propensity score matching to reduce selection bias. All patients were diagnosed as type III BA by histologic examination and cholangiograms. Clinicopathological characteristics and survival outcomes were compared between the two groups. Results There were no significant intergroup differences between baseline characteristics and outcomes after Kasai portoenterostomy surgery in two groups. BA with hilar cyst group showed comparable survival outcomes to the BA without cyst group (cumulative 1-y, 2-y and 5-y overall survival rates with native liver 61.4% vs. 65.8%, P = 0.041; 45.0% vs. 49.0%, P = 0.57; 45.0% vs. 49.0%, P = 0.57). And the Kaplan–Meier survival curves showed no significant difference in cumulative survival with native liver between the two groups (P = 0.58). Conclusions Type III BA with hilar cyst had no better prognosis compared with Type III BA without cyst. Keywords Biliary atresia . Cyst . Ultrasonography . Prognosis
Introduction Biliary atresia (BA) is a destructive, inflammatory, obliterative cholangiopathy that develops in 1/5000 to 1/19,000 newborns [1]. BA affects varying lengths of both the extra and intrahepatic biliary tract and is usually classified, according to the most proximal level of obstruction, into 3 types. Type I BA is atresia of the common bile duct (CBD), which is often
* Hong Jiang [email protected] * Lu-yao Zhou [email protected]
cystic. Type II BA is atresia of the hepatic duct and can contain visible patent ducts at porta. Type III BA (>90%) is atresia at the porta hepatis, and only microscopic ductules can be detected [1, 2]. In 6–20% of BA cases [3–5], a single or multiple cysts, which usually could be clearly visualized on Ultrasound scan [6, 7], are present at diagnosis in any part of the extrahepatic biliary tract. Some of these cases belong to Type I BA, and others to Type III BA. Nevertheless, some researchers suggest that BA with hilar cyst is a clinically distinct subtype associated with projected improved outcomes [8, 9], while other researchers have not found this [10]. To clarify this issue, authors retrospectively reviewed the characteristics of preoperative ultrasonography (USG) findings and the prognosis of 27 BA patients who had hilar cyst and had undergone Kasai portoenterostomy (KPE) surgery, and compared them with a propensity score matched pair control group of 27 BA patients without hilar cyst.
1
Department of Medical Ultrasonics, Insti
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