Introduction Age at Kasai portoenterostomy (KPE) has been identified as a predictive factor for native-liver survival in patients with biliary atresia (BA). Outcomes of pediatric liver transplantation (LT) have improved over recent years. It has been proposed to consider primary LT as a treatment option for late-presenting BA infants instead of attempting KPE. We present our experience with patients older than 90 days undergoing KPE.

Materials and Methods A retrospective chart review of patients with BA undergoing KPE at our institution between January 2010 and December 2020 was performed. Patients 90 days and older at the time of surgery were included. Patients' characteristics, perioperative data, and follow-up results were collected. Eleven patients matched the inclusion criteria. Mean age at KPE was 108 days (range: 90–133 days).

Results Postoperative jaundice clearance (bilirubin < 2 mg/dL) at 2-year follow-up was achieved in three patients (27%). Eight patients (73%) received a liver transplant at a mean of 626 days (range: 57–2,109 days) after KPE. Four patients (36%) were transplanted within 12 months post-KPE. Two patients died 237 and 139 days after KPE due to disease-related complications. One patient is still alive with his native liver, currently 10 years old.

Conclusion Even when performed at an advanced age, KPE can help prolong native-liver survival in BA patients and offers an important bridge to transplant. In our opinion, it continues to represent a viable primary treatment option for late-presenting infants with BA.

简介 Kasai 门肠造口术 (KPE) 的年龄已被确定为胆道闭锁 (BA) 患者自身肝脏存活率的预测因素。近年来，儿童肝移植 (LT) 的结果有所改善。有人建议考虑将原发性 LT 作为晚期 BA 婴儿的治疗选择，而不是尝试 KPE。我们介绍了接受 KPE 的 90 天以上患者的经验。

材料与方法 对 2010 年 1 月至 2020 年 12 月在我院接受 KPE 的 BA 患者进行回顾性图表审查。包括在手术时 90 天及以上的患者。收集患者的特征、围手术期数据和随访结果。11 名患者符合纳入标准。KPE 的平均年龄为 108 天（范围：90-133 天）。

结果 3 名患者（27%）在 2 年的随访中实现了术后黄疸清除（胆红素 < 2 mg/dL）。8 名患者（73%）在 KPE 后平均 626 天（范围：57-2,109 天）接受了肝移植。4 名患者 (36%) 在 KPE 后 12 个月内进行了移植。由于疾病相关并发症，两名患者在 KPE 后 237 天和 139 天死亡。一名患者仍然活着，他的原生肝脏目前已 10 岁。

结论 即使在高龄时进行，KPE 也可以帮助延长 BA 患者的天然肝脏存活率，并为移植提供重要的桥梁。我们认为，对于晚期出现的 BA 婴儿，它仍然是一种可行的主要治疗选择。