| 摘要: |
| ［摘要］ 目的 分析产前诊断囊肿型胆道闭锁（CBA）产前及出生后的临床特征和预后情况。方法 回顾性分析广州医科大学附属妇女儿童医疗中心2016年6月1日至2024年6月1日收治的产前超声提示肝门区囊肿并于出生后经手术诊断CBA 20例的临床资料，对其产前和出生后临床特征及预后情况进行分析。所有病例出生后均接受Kasai手术治疗，根据术中造影情况分为Ⅰ型CBA组（9例）和Ⅲ型CBA组（11例），比较两组的临床特征和预后。结果 在产前诊断CBA 20例中，首次超声检查发现肝门区囊肿的孕周中位数为24.00（20.00，24.75）周。出生后手术日龄中位数为43.00（30.75，57.25）d，术后胆管炎发生率为55.00%（11/20），术后3个月黄疸清除率为73.68%（14/19），持续黄疸清除率为75.00%（12/16），自体肝存活率为66.67%（10/15），肝移植率为20.00%（3/15），死亡率为13.33%（2/15）。Ⅰ型CBA组产前首次超声测量囊肿最大径显著大于Ⅲ型CBA组(P<0.05)，两组基本资料、术前末次生化指标、手术相关指标、术后病理结果、术后住院时间和预后情况比较差异无统计学意义（P>0.05）。结论 产前诊断CBA病例可在出生后获得早期治疗，可能有更好的预后。Ⅰ型CBA和Ⅲ型CBA患儿在出生后的临床特征和术后转归方面无明显差异。 |
| 关键词: 囊肿型胆道闭锁 产前诊断 临床特征 预后 |
| DOI：10.3969/j.issn.1674-3806.2025.06.05 |
| 分类号:R 726.5 |
| 基金项目:广州市科技计划项目（编号：2024A03J1008） |
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| Analysis on clinical features of 20 patients with prenatally diagnosed cystic biliary atresia and their prognosis |
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GUAN Xisi1, HUANG Lin2, HOU Longlong1, WANG Junjie1, YU Jiakang1, ZHONG Wei1
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1.Department of Neonatal Surgery, Guangzhou Women and Children′s Medical Center of Guangzhou Medical University, Guangzhou 510623, China; 2.Department of Pediatric Surgery, Shunde Women and Children′s Hospital of Guangdong Medical University, Foshan 528000, China
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| Abstract: |
| ［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 Ⅰ CBA 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. |
| Key words: Cystic biliary atresia(CBA) Prenatal diagnosis Clinical features Prognosis |
