2007 年至 2017 年全结肠无神经节细胞症分期和一期剖腹 Soave 手术的临床结果：单中心经验
Clinical Outcomes After Staged and Primary Laparotomy Soave Procedure for Total Colonic Aganglionosis: a Single-Center Experience from 2007 to 2017.
机构信息
Department of General Surgery, Beijing Children Hospital, National Center of Children's Health, Capital Medical University, No. 56 NaLiShi Road, Xicheng District, CN 100045, Beijing, China.
Department of Neonatal Surgery, Beijing Children Hospital, National Center of Children's Health, Capital Medical University, Beijing, China.
出版信息
J Gastrointest Surg. 2020 Jul;24(7):1673-1681. doi: 10.1007/s11605-019-04319-5. Epub 2019 Jul 19.
PURPOSE
To compare the clinical outcomes of the staged laparotomy Soave procedure (SLSP) and primary laparotomy Soave procedure (PLSP) for patients with total colonic aganglionosis (TCA), including some patients who underwent surgical treatment after the age of 1 year.
METHODS
A retrospective, comparative study of TCA patients who underwent SLSP or PLSP at Beijing Children Hospital from 2007 to 2017 was conducted. The primary outcomes were height-for-age (HFA), weight-for-age (WFA), and bowel function score (BFS); the major types of postoperative complications were also analyzed.
RESULTS
Thirty-five patients (SLSP = 14, PLSP = 21) were included. The patients in the SLSP group underwent an enterostomy at a median age of 47 (14, 104) days. The median age at the time of the Soave procedure showed no significant difference between the two groups ([181 (131,346) vs 55 (29, 343)] days, p = 0.072). No significant differences between groups were noted in terms of presenting symptoms, perioperative results, and postoperative outcomes. Twenty-three patients (SLSP = 8, PLSP = 15) were followed for more than 1 year. Both the SLSP and PLSP groups showed similar operative mortality ([1/14, 7.1%] vs [3/21, 14.3%], p = 0.635) and complication rates ([postoperative enterocolitis, (5/8, 62.5%) vs (5/15, 33.3%), p = 0.221] [perianal excoriation, (7/8, 87.5%) vs (10/15, 66.6%), p = 0.369]). Nineteen patients (19/23, 82.6%) had normal growth according to the median percentage of HFA or WFA. Mean BFS was 15.9 ± 3.3 in the 15 patients aged older than 4 years who were followed up. Six patients (SLSP = 1, PLSP = 5) who underwent surgical treatment aged older than 1 year were all alive with good outcomes.
CONCLUSION
PLSP and SLSP had equivalent clinical outcomes, but enterocolitis and perianal excoriation after the Soave procedure need to be managed carefully. PLSP is a feasible option for older TCA patients after conservative treatment.
目的
比较分期剖腹索状手术（SLSP）和一期剖腹索状手术（PLSP）治疗全结肠无神经节细胞症（TCA）患者的临床结果，包括一些在 1 岁后接受手术治疗的患者。
方法
回顾性比较 2007 年至 2017 年在北京儿童医院接受 SLSP 或 PLSP 的 TCA 患者。主要结局指标为身高年龄（HFA）、体重年龄（WFA）和肠道功能评分（BFS）；主要分析术后并发症类型。
结果
纳入 35 例患者（SLSP=14 例，PLSP=21 例）。SLSP 组患者肠造口术的中位年龄为 47（14，104）天。两组患者索状手术的中位年龄无显著差异（[181（131，346）与 55（29，343）]天，p=0.072）。两组在首发症状、围手术期结果和术后结果方面无显著差异。23 例患者（SLSP=8 例，PLSP=15 例）随访超过 1 年。SLSP 组和 PLSP 组的手术死亡率均较低（[1/14，7.1%]与[3/21，14.3%]，p=0.635），并发症发生率也相似（术后肠炎，[5/8，62.5%]与[5/15，33.3%]，p=0.221；肛周糜烂，[7/8，87.5%]与[10/15，66.6%]，p=0.369）。19 例（19/23，82.6%）患者根据 HFA 或 WFA 的中位数百分比正常生长。15 例随访年龄大于 4 岁的患者平均 BFS 为 15.9±3.3。6 例（SLSP=1 例，PLSP=5 例）大于 1 岁接受手术治疗的患者均存活，且结局良好。
结论
PLSP 和 SLSP 的临床结果相当，但索状手术后的肠炎和肛周糜烂需要谨慎处理。PLSP 是 TCA 患者在保守治疗后进行手术治疗的可行选择。
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