Department of Pediatric Surgery, West China Hospital of Sichuan University. #37 Guo-Xue-Xiang, Chengdu, 610041, Sichuan, China.
Pediatric Intensive Care Unit, Department of Critical Care Medicine, West China Hospital of Sichuan University, Chengdu, 610041, China.
Orphanet J Rare Dis. 2021 Apr 30;16(1):193. doi: 10.1186/s13023-021-01835-z.
To determine whether revision laparoscopic Kasai portoenterostomy (RLKPE) is a viable treatment option for patients with biliary atresia (BA) who had undergone initially successful laparoscopic Kasai portoenterostomy (ILKPE).
The medical records of 312 patients with nonsyndromic BA who had undergone ILKPE between May 2009 and May 2017 were retrospectively reviewed. The patients were divided into three groups according to their outcomes after ILKPE: group A: 25 patients who had undergone RLKPE; group B: 203 patients who had undergone ILKPE and required no further surgical intervention; group C: 84 patients with failed ILKPE who had either died or required liver transplantation for survival. The 3-year and 5-year survival with native liver (SNL) rates were compared between groups A and B and between groups A and C. Among the 25 patients in group A, the perioperative data of RLKPE were compared with those of ILKPE.
Of the 312 patients who had undergone ILKPE, 228 reached the normal bilirubin concentration range within 6 months postoperatively. Among them, 25 patients with a sudden cessation of bile flow had undergone RLKPE. Adequate biliary drainage, as evidenced by normalized conjugated bilirubin levels, was achieved in 80% of patients who had undergone RLKPE. The perioperative variables, including the operative time, blood loss, rate of conversion to open surgery and complications of RLKPE, were not significantly different between RLKPE and ILKPE. The 3-year and 5-year SNL rates in patients after RLKPE were 64.0% and 52.0%, respectively, which were not significantly different from the corresponding rates of 86.2% and 73.9%, respectively, in patients after unrevised ILKPE (P > 0.05).
Our data demonstrated that RPLKE is a viable and effective treatment option in patients with sudden cessation of bile drainage after ILKPE. RPLKE can delay the need for liver transplantation, yielding encouraging medium-term patient outcomes.
探讨腹腔镜改良Kasai 肝门空肠吻合术(ILKPE)治疗胆道闭锁(BA)术后胆汁引流突然停止患儿的效果。
回顾性分析 2009 年 5 月至 2017 年 5 月期间我院收治的 312 例非综合征型 BA 患儿的临床资料,根据 ILKPE 术后结局将患儿分为 3 组,A 组:25 例行补救性腹腔镜 Kasai 肝门空肠吻合术(RLKPE);B 组:203 例行 ILKPE 且无需进一步手术治疗;C 组:84 例行 ILKPE 失败且死亡或因生存需要行肝移植。比较 A 组与 B 组、A 组与 C 组患儿 3 年、5 年无病生存(SNL)率。A 组中 25 例行 RLKPE 患儿的围手术期数据与 ILKPE 进行比较。
312 例行 ILKPE 患儿中,228 例术后 6 个月内胆红素浓度恢复正常。其中 25 例患儿出现胆汁引流突然停止,行 RLKPE 后 80%(20/25)患儿胆汁引流通畅,结合胆红素恢复正常。RLKPE 与 ILKPE 患儿的围手术期变量,如手术时间、术中出血量、中转开腹率和并发症发生率等差异均无统计学意义。RLKPE 后患儿的 3 年、5 年 SNL 率分别为 64.0%、52.0%,与未修正 ILKPE 后患儿的 86.2%、73.9%比较差异均无统计学意义(P>0.05)。
ILKPE 术后胆汁引流突然停止患儿行 RLKPE 是一种可行且有效的治疗方法,可延迟肝移植时间,获得良好的中期疗效。