Asian Journal of Surgery

Asian Journal of Surgery

Volume 40, Issue 6, November 2017, Pages 429-433
Asian Journal of Surgery

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

https://doi.org/10.1016/j.asjsur.2016.04.003Get rights and content
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open access

Summary

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.

Keywords

biliary atresia
extrahepatic congential malformation
multicenter
nonsydromic

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Conflict of interests: The authors declare that they have no conflicts of interests.