Analysis on clinical features of 20 patients with prenatally diagnosed cystic biliary atresia and their prognosis

GUAN Xisi; HUANG Lin; HOU Longlong; WANG Junjie; YU Jiakang; ZHONG Wei

doi:10.3969/j.issn.1674-3806.2025.06.05

Abstract：Objective To analyze the prenatal and postnatal clinical features and the prognosis of the patients with prenatally diagnosed cystic biliary atresia(CBA).Methods The clinical data of 20 patients who were admitted to Guangzhou Women and Children's Medical Center of Guangzhou Medical University from June 1,2016 to June 1,2024 and were prenatally indicated to have hepatic portal cysts by ultrasonography and were diagnosed with CBA by surgery after birth were retrospectively analyzed.The prenatal and postnatal clinical features and the prognosis of these patients were analyzed.All the patients received Kasai's procedure after birth.The patients were divided into type Ⅰ CBA group(9 patients)and type Ⅲ CBA group(11 patients)according to their intraoperative angiography results.The clinical features and prognosis were compared between the two groups.Results In the 20 patients with prenatally diagnosed CBA,the median value of gestational age at which hepatic portal cysts were detected by the first prenatal ultrasonography in them was 24.00(20.00,24.75)weeks.The median age of the patients receiving operations after birth was 43.00(30.75,57.25)days.The incidence of postoperative cholangitis was 55.00％(11/20).The jaundice clearance rate at 3 months after operation was 73.68％(14/19).The continuous jaundice clearance rate was 75.00％(12/16).The survival rate of autologous liver was 66.67％(10/15).The liver transplantation rate was 20.00％(3/15).The mortality rate was 13.33％(2/15).The maximum diameter of the cysts measured by the first prenatal ultrasonography in the type ⅠCBA group was significantly larger than that in the type Ⅲ CBA group(P＜0.05).There were no statistically significant differences between the two groups in terms of the patients' basic information,the last biochemical indicators before operation,the operation-related indicators,the postoperative pathological results,the postoperative hospital stay and the prognosis(P＞0.05).Conclusion The patients with prenatally diagnosed CBA can obtain early treatment after birth and may have a better prognosis.There are no significant differences in postnatal clinical features and postoperative outcomes between the pediatric patients with type Ⅰ CBA and those with type Ⅲ CBA.