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Investigation into multi-centre diagnosis and treatment strategies of biliary atresia in mainland China

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Abstract

Background

Biliary atresia (BA) is an obstructive hepatobiliary disease which manifests during infancy. Kasai portoenterostomy (KPE) is the preferred operation for BA, supplemented with glucocorticoids, antibiotics, and choleretic agents. A great deal of research has been carried out regarding diagnosis, operation, and adjuvant therapies of BA, but no consensus had been reached. To understand the variation in diagnosis and treatment strategies of BA across mainland China and to help achieve a unified treatment strategy in the future, this investigation was carried out.

Methods

This investigation was conducted via electronic questionnaire. The centres were divided into three groups based on their annual caseload: low (0–20)-, mid (21–40)-, and high (≥ 41)-volume group. Differences in the clinical practice among three groups were analyzed by Chi-square test and considered statistically significant at P < 0.05.

Results

41 Centres from 26 different administrative regions were involved. The average age at KPE was mainly 51–60 days (39%, 16/41) and 61–70 days (32%, 13/41). The annual caseload was 0–20 patients in 17 centres, 21–40 patients in 11 centres, and > 40 patients in 13 centres. Preoperative ultrasound and intraoperative cholangiography were performed in all centres. Low-volume centres had a high proportion of MRI (P = 0.005), while the high-volume group had a high proportion of LSM (P = 0.015). Open KPE without liver mobilisation is the most common surgical procedure (71%, 29/41). Open KPE without liver mobilisation was more commonly used in low-volume group (P = 0.044), and laparoscopic KPE was mainly used in high-volume group (P = 0.011). The spur anti-reflux intestinal valve was performed in more than half of the centres (51%, 21/41). The length of the Roux-en-Y loop was ≥ 30 cm in the majority of centres (78%, 32/41). Glucocorticoids and antibiotics were used in most centres (90%, 37/41; 100%, 41/41) with marked variations in type, administration, dose, and duration. Oral ursodeoxycholic acid (UDCA) was used in 38 centres, in varying doses of 10–20 mg/kg/day. The duration of oral UDCA was over a year in 19 centres.

Conclusion

Mainland China has a large number of patients with biliary atresia. Diagnostic and surgical methods vary from centre to centre and are related to its caseload. In most centres, KPE is supplemented with glucocorticoids, antibiotics, and choleretic agents without a standard regimen.

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Acknowledgements

We are grateful to the following institutions for providing the data for this study (in no particular order): The Children's Hospital, Zhejiang University School of Medicine, Children's Hospital of Nanjing Medical University, Children's Hospital of Shanxi, Fujian Provincial Maternity and Children’s Hospital, Binzhou Medical University Hospital, Hunan Children’s Hospital, The Second Hospital of Hebei Medical University, The First Affiliated Hospital of Zhengzhou University, Shengjing Hospital of China Medical University, The First Affiliated Hospital of Xinjiang Medical University, Children's Hospital of Shanghai/Children's Hospital of Shanghai Jiaotong University, Tongji Hospital Affiliated to Tongji Medical College of Huazhong University of Science and technology, Urumqi Children's Hospital, Wuhan Children's Hospital Affiliated to Tongji Medical College of Huazhong University of Science and Technology, Xuzhou Children's Hospital, Shenzhen Children's Hospital, Hainan Women and Children's Medical Center, Beijing Children's Hospital Affiliated to Capital Medical University, The Seventh Medical Center of PLA General Hospital, Hainan Women and Children's Medical Center, Anhui Women and Child Health Care Hospital, Fujian Medical University Union Hospital, Guangzhou Women and Children’s Medical Center, Henan Children's Hospital, The First Affiliated Hospital of Guangxi Medical University, Xi'an Children's Hospital, The First Affiliated Hospital of Dali University, General Hospital of Ningxia Medical University, Affiliated Hospital of Zunyi Medical University, Changchun Children's Hospital, Shandong Provincial Hospital, The Second Affiliated Hospital of Xi'an Jiaotong University, The Second Affiliated Hospital of Harbin Medical University, Children's Hospital of Chongqing Medical University, Guiyang Children's Hospital, West China Hospital of Sichuan University, The Affiliated Children's Hospital of Capital Institute of Pediatric, Children's Hospital of Hebei Province.

Funding

This study was funded by the National Natural Science Foundation of China (Grant number 81570471) and Tianjin Health Bureau special grant (Grant number 14KG129).

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Authors and Affiliations

  1. Graduate College, Tianjin Medical University, Tianjin, 300070, People’s Republic of China

    Qipeng Zheng, Liang Ge, Jinfu Jia, Qingyun Gou & Jinfeng Zhao

  2. Department of General Surgery, Tianjin Children’s Hospital, LongYan Road 238, Beichen District, Tianjin, 300134, People’s Republic of China

    Shujian Zhang & Jianghua Zhan

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  1. Qipeng Zheng
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Correspondence to Jianghua Zhan.

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Zheng, Q., Zhang, S., Ge, L. et al. Investigation into multi-centre diagnosis and treatment strategies of biliary atresia in mainland China. Pediatr Surg Int 36, 827–833 (2020). https://doi.org/10.1007/s00383-020-04679-z

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