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J Pediatr Surg. 2019 Aug 30. pii: S0022-3468(19)30567-6. doi: 10.1016/j.jpedsurg.2019.08.027. [Epub ahead of print]

Intrahepatic Cystic Lesions in Children with Biliary Atresia After Kasai Procedure.

Author information

1
Department of Pediatric Surgery, Children's Hospital of Fudan University, Shanghai Key Laboratory of Birth Defect, Shanghai, China.
2
Department of Pediatric Surgery, Children's Hospital of Fudan University, Shanghai Key Laboratory of Birth Defect, Shanghai, China. Electronic address: rdong@fudan.edu.cn.
3
Department of Pediatric Surgery, Children's Hospital of Fudan University, Shanghai Key Laboratory of Birth Defect, Shanghai, China. Electronic address: szheng@shmu.edu.cn.

Abstract

BACKGROUND:

Intrahepatic cystic lesion (ICL) is a common complication for biliary atresia post-Kasai portoenterostomy. The purpose of this study was to review the cases in our hospital and assess the correlation between characteristics of ICL and clinical outcomes.

METHODS:

We retrospectively analyzed 787 cases of biliary atresia from 2012 to 2016. Demographics, clinical details, and postoperative outcomes were reviewed.

RESULTS:

A total of 76 patients were diagnosed with ICLs using ultrasound post-Kasai procedure, and the incidence was 9.7%. Preoperative characteristics showed no significant differences between ICL (+) and ICL (-) groups. Nearly 70% (53/76) of the patients with ICLs had a history of cholangitis. The 2-year native liver survival rate was 60.4% for those with a history of cholangitis and 87% for those without (P = 0.017). Further analysis showed that the 2-year native liver survival rate was 42.9% for those diagnosed within 3 months post-Kasai procedure, 54.2% for those diagnosed between 3 and 6 months, and 80.0% for those diagnosed beyond 6 months (P = 0.002), while no significance was observed for type (P = 0.094) or site (P = 0.406) of ICL.

CONCLUSION:

Patients with ICLs had a high incidence of cholangitis. The prognosis was closely related with the history of cholangitis and the onset time of ICLs.

LEVEL OF EVIDENCE:

Level II.

KEYWORDS:

Biliary atresia; Intrahepatic cystic lesions; Kasai procedure

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