Incidence of biliary atresia associated congenital malformations: A retrospective multicenter study in China

被引:36
作者
Zhan, Jianghua [1 ]
Feng, Jiexiong [2 ]
Chen, Yajun [3 ]
Liu, Juncheng [4 ]
Wang, Bin [5 ]
机构
[1] Tianjin Childrens Hosp, Dept Surg, Tianjin 300134, Peoples R China
[2] Huazhong Univ Sci & Technol, Tongji Hosp, Dept Pediat Surg, Wuhan 430030, Hubei, Peoples R China
[3] Capital Med Univ, Affiliated Beijing Childrens Hosp, Dept Surg, Beijing 100045, Peoples R China
[4] Sun Yat Sen Univ, Affiliated Hosp 1, Dept Pediat Surg, Guangzhou 510080, Guangdong, Peoples R China
[5] Shenzhen Childrens Hosp, Dept Surg, Shenzhen 518026, Peoples R China
关键词
biliary atresia; extrahepatic congential malformation; multicenter; nonsydromic; STOOL COLOR CARD; SPLENIC MALFORMATION; ANOMALIES; DISEASE; TAIWAN;
D O I
10.1016/j.asjsur.2016.04.003
中图分类号
R61 [外科手术学];
学科分类号
摘要
Background: Some patients with biliary atresia (BA) have associated anomalies. Our study aimed to investigate the incidence of BA-associated malformations in mainland China, and compare the results with those reported in the Western literature. Methods: Clinical data were collected retrospectively from five medical centers in mainland China. BA patients were diagnosed and confirmed by laparotomy with intraoperative cholangiography and liver biopsy. Cases were divided into isolated type BA and BA with associated anomalies, including polysplenia, situs inversus, intestinal malrotation, and cardiovascular anomalies. Results: A total of 851 BA patients were recruited from Tianjin, Beijing, Wuhan, Guangzhou, and Shenzhen. Patients were grouped as follows: Type I, 13 cases (1.5%); Type II, five cases (0.6%); Type III, 833 cases (97.9%). Forty-two (4.94%) patients had 54 associated congenital abnormalities. The intra-abdominal anomalies included polysplenia (n = 4, 1 fusion between liver and spleen), situs inversus (n = 2), and intestinal malrotation (n = 3). The cardiovascular anomalies included atrial septal defect and ventricular septal defect (n = 29), patent foramen ovale (n = 1), patent ductus arteriosus (n = 4), and other cardiac malformations (n = 3, including coronary sinus dilation, left superior vena cava, Tetralogy of Fallot). Conclusion: Our data showed that spleen anomaly is not as common as reported in the Western literature. The difference may suggests different genetic and environmental risk factors for BA. (C) 2016 Asian Surgical Association and Taiwan Robotic Surgical Association. Publishing services by Elsevier B.V.
引用
收藏
页码:429 / 433
页数:5
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