title: Incidence of biliary atresia associated congenital malformations: A retrospective multicenter study in China.
abstract: 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).
  
  
  CONCLUSIONS
  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.
authors.affiliation: Department of Surgery, Tianjin Children's Hospital, Tianjin 300134, China. Electronic address: zhanjianghuatj@163.com.
authors.forename: Jianghua
authors.initials: J
authors.lastname: Zhan
authors.affiliation: Department of Pediatric Surgery, Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan 430030, China.
authors.forename: Jiexiong
authors.initials: J
authors.lastname: Feng
authors.affiliation: Department of Surgery, Affiliated Beijing Children's Hospital, Capital Medical University, Beijing 100045, China.
authors.forename: Yajun
authors.initials: Y
authors.lastname: Chen
authors.affiliation: Department of Pediatric Surgery, First Affiliated Hospital, Sun Yat-sen University, Guangzhou 510080, China.
authors.forename: Juncheng
authors.initials: J
authors.lastname: Liu
authors.affiliation: Department of Surgery, Shenzhen Children's Hospital, Shenzhen 518026, China.
authors.forename: Bin
authors.initials: B
authors.lastname: Wang
mesh_terms: D000015:Abnormalities, Multiple / Q000175:diagnosis* / Q000453:epidemiology* / Q000601:surgery; D001656:Biliary Atresia / Q000175:diagnosis* / Q000453:epidemiology* / Q000601:surgery; D002681:China / Q000453:epidemiology; D015331:Cohort Studies; D005260:Female; D006776:Hospitals, Pediatric; D006801:Humans; D007231:Infant, Newborn; D008297:Male; D015995:Prevalence; D011379:Prognosis; D012189:Retrospective Studies; D018570:Risk Assessment; D017678:Sex Distribution
keywords: biliary atresia; extrahepatic congential malformation; multicenter; nonsydromic