Yan Jiayu,Wang Kexin,Chen Yajun.Long-term outcomes of Soave procedure for Hischspung’s disease: analysis of 55 cases with over 10 years of follow-up[J].Journal of Clinical Pediatric Surgery,2026,(05):430-435.[doi:10.3760/cma.j.cn101785-20260211-00066]
Soave手术治疗先天性巨结肠的远期疗效(附随访10年以上的55例分析)
- Title:
- Long-term outcomes of Soave procedure for Hischspung’s disease: analysis of 55 cases with over 10 years of follow-up
- 关键词:
- Hirschsprung病; 预后; 影响因素分析; 外科手术; 儿童
- Keywords:
- Hirschsprung Disease; Prognosis; Root Cause Analysis; Surgical Procedures; Operative; Child
- 摘要:
- 目的 总结Soave手术治疗先天性巨结肠(Hirschsprung’s disease,HD)的远期疗效,分析远期预后的可能影响因素。方法 回顾性收集2011年1月至2015年12月由首都医科大学附属北京儿童医院普外科同一手术团队采用Soave手术治疗的91例HD患儿资料,并于2026年1月进行电话随访,所有患儿Soave术后超过10年,随访内容包括生长发育情况(身高、体重)和排便功能(Rintala评分系统,满分为20分)。结果 男73例、女18例,其中短段型HD 25例(25/91,27.5%),常见型55例(55/91,60.4%),长段型8例(8/91,8.8%),全结肠型3例(3/91,3.3%),行Soave手术年龄为1.3(0.7,2.9)岁。共55例(55/91,60.4%)获得随访,平均随访12.7(11.0,13.8)年,随访年龄为14.7(13.4,16.0)岁,分别有52例(52/55,94.5%)、53例(53/55,96.4%)患儿的年龄别身高和BMI处于正常范围,Rintala评分为20.0(19.0,20.0)。不同病理类型(短段型、常见型和长段型)HD患儿的切除肠管长度差异有统计学意义(P<0.001),但远期生长发育和排便功能,组间差异无统计学意义(P>0.05)。通过限制性立方样条函数对切除肠管长度和Rintala评分进行建模,发现切除肠管长度与Rintala评分具有相关性(P=0.0038),切除肠管长度>40 cm后Rintala评分下降趋势明显。结论 Soave手术治疗HD患儿的远期疗效可,切除肠管长度较长可能是导致患儿远期排便功能不良的主要因素。
- Abstract:
- Objective Tosummarize the long-term outcomes of the Soave procedure for Hirschsprung’s disease (HD) and to analyze potential factors affecting long-term prognosis.Methods A retrospective study was conducted on 91 children with HD who underwent the Soave procedure performed by the same surgical team at Beijing Children’s Hospital,Capital Medical University,between January 2011 and December 2015.Telephone follow-up was performed in January 2026,and all patients had postoperative follow-up of more than 10 years.Follow-up data included growth and development (height and weight) and bowel function assessed using the Rintala scoring system (maximum score:20).Results Among the 91 patients,73 were male and 18 were female.The distribution of pathological types was as follows:short-segment HD in 25 cases (25/91,27.5%),classic-segment HD in 55 cases (55/91,60.4%),long-segment HD in 8 cases (8/91,8.8%),and total colonic HD in 3 cases (3/91,3.3%).The median age at surgery was 1.3 (0.7,2.9) years.A total of 55 (55/91,60.4%) patients were successfully followed up,with a median follow-up of 12.7 (11.0,13.8) years and a follow-up age of 14.7 (13.4,16.0) years.Among these patients,52 (94.5%) and 53 (96.4%) had age-appropriate height and BMI within the normal range,respectively.The Rintala score was 20.0(19.0,20.0).There was a significant difference in the length of resected bowel among different pathological types (short-segment,classic-segment,and long-segment HD) (P<0.001).However,no significant differences were observed in long-term growth or bowel function among the groups (P>0.05).Restricted cubic spline analysis demonstrated a significant overall association between the length of resected bowel and Rintala score (P=0.0038),with a noticeable decline in Rintala score when the resected length exceeded 40 cm.Conclusion The Soave procedure provides satisfactory long-term outcomes in children with HD.A longer length of resected bowel may be a major factor associated with poor long-term bowel function.
参考文献/References:
[1] Morera C, Nurko S.Hirschsprung’s disease.Management[J].Aliment Pharmacol Ther, 2024, 60(S1):S66-S76.DOI:10.1111/apt.18068.
[2] Ma ZH, Tang J, Xie N, et al.Comparative efficacy of Soave versus Swenson procedures for Hirschsprung disease:a systematic review and meta-analysis[J].Pediatr Surg Int, 2025, 42(1):53.DOI:10.1007/s00383-025-06260-y.
[3] 谢川平, 严佳虞, 陈亚军, 等.先天性巨结肠Soave术后远期排便功能不良的危险因素分析[J].临床小儿外科杂志, 2024, 23(5):426-429.DOI:10.3760/cma.j.cn101785-202304013-005. Xie CP, Yan JY, Chen YJ, et al.Risk factors for long-term bowel function outcomes after Soave procedure for Hirschsprung’s disease[J].DOI:10.3760/cma.j.cn101785-202304013-005.
[4] Gosain A, Frykman PK, Cowles RA, et al.Guidelines for the diagnosis and management of Hirschsprung-associated enterocolitis[J].Pediatr Surg Int, 2017, 33(5):517-521.DOI:10.1007/s00383-017-4065-8.
[5] Yan JY, Chen YW, Ding CL, et al.Clinical outcomes after staged and primary laparotomy soave procedure for total colonic aganglionosis:a single-center experience from 2007 to 2017[J].J Gastrointest Surg, 2020, 24(7):1673-1681.DOI:10.1007/s11605-019-04319-5.
[6] 中华医学会小儿外科学分会肛肠学组、新生儿学组.先天性巨结肠的诊断及治疗专家共识[J].中华小儿外科杂志, 2017, 38(11):805-815.DOI:10.3760/cma.j.issn.0253-3006.2017.11.002. The Group of Anorectum, the Group of Neonatology, the Society of Pediatric Surgery, Chinese Medical Association.The guidelines of diagnosis and treatment of Hirschspung’s disease[J].Chin J Pediatr Surg, 2017, 38(11):805-815.DOI:10.3760/cma.j.issn.0253-3006.2017.11.002.
[7] World Health Organization.Child growth standards.[2009-11-12].https://www.who.int/tools/child-growth-standards/standards.
[8] Neuvonen MI, Kyrklund K, Rintala RJ, et al.Bowel function and quality of life after transanal endorectal pull-through for Hirschsprung disease:controlled outcomes up to adulthood[J].Ann Surg, 2017, 265(3):622-629.DOI:10.1097/SLA.0000000000001695.
[9] Xu TO, Levitt MA, Feng C.Controversies in Hirschsprung surgery[J].World J Pediatr Surg, 2024, 7(3):e000887.DOI:10.1136/wjps-2024-000887.
[10] Merga TG, Assefa HG, Erge MG, et al.Post pull-through bowel function outcome and contributing factors in children; a cross-sectional study from low resource setting[J].BMC Surg, 2025, 25(1):260.DOI:10.1186/s12893-025-02999-2.
[11] Davidson JR, Kyrklund K, Eaton S, et al.Long-term surgical and patient-reported outcomes of Hirschsprung Disease[J].J Pediatr Surg, 2021, 56(9):1502-1511.DOI:10.1016/j.jpedsurg.2021.01.043.
[12] Short SS, Durham MM, Rollins MD.Hirschsprung disease outco-mes[J].Semin Pediatr Surg, 2022, 31(2):151160.DOI:10.1016/j.sempedsurg.2022.151160.
[13] Pini Prato A, Erculiani M, Novi ML, et al.Delayed diagnosis in Hirschsprung disease[J].Pediatr Surg Int, 2024, 40(1):65.DOI:10.1007/s00383-024-05657-5.
[14] Xie CP, Yan JY, Wang KX, et al.Surgical outcomes for patients with rectosigmoid Hirschsprung disease who underwent transanal endorectal pull-through after 1 year of age[J].BMC Surg, 2024, 24(1):273.DOI:10.1186/s12893-024-02560-7.
[15] Onishi S, Kaji T, Nakame K, et al.Optimal timing of definitive surgery for Hirschsprung’s disease to achieve better long-term bowel function[J].Surg Today, 2022, 52(1):92-97.DOI:10.1007/s00595-021-02356-9.
[16] Verkuijl SJ, Meinds RJ, van der Steeg AFW, et al.Functional outcomes after surgery for total colonic, long-segment, versus rectosigmoid segment Hirschsprung disease[J].J Pediatr Gastroenterol Nutr, 2022, 74(3):348-354.DOI:10.1097/MPG.0000000000003355.
[17] Wang KX, Xie CP, Yan JY, et al.Total transanal endorectal pull-through versus laparoscopic-assisted approach in children with rectosigmoid Hirschsprung’s disease:a systematic review and meta-analysis[J].J Laparoendosc Adv Surg Tech A, 2025, 35(1):80-88.DOI:10.1089/lap.2023.0448.
[18] Ban HT, Chieu VM, Huynh QTV, et al.Long-term outcomes of laparoscopic-assisted versus complete transanal endorectal pull-through technique for classic segment Hirschsprung’s disease[J].Pediatr Surg Int, 2024, 41(1):26.DOI:10.1007/s00383-024-05924-5.
[19] Elrouby A, Shehata S, Waheeb S, et al.One stage transanal versus one stage laparoscopic-assisted transanal endorectal pull-through in managing Hirschsprung’s disease in pediatric age group; a retrospective study[J].BMC Surg, 2025, 25(1):59.DOI:10.1186/s12893-025-02768-1.
[20] Durazo CIM, Frei P, Go SGG, et al.Postoperative outcomes of robotic-assisted vs.laparoscopic pull-through surgery in pediatric Hirschsprung’s disease:a systematic review and meta-analysis[J].Pediatr Surg Int, 2025, 41(1):228.DOI:10.1007/s00383-025-06137-0.
[21] Frischer JS, Rymeski B.Complications in colorectal surgery[J].Semin Pediatr Surg, 2016, 25(6):380-387.DOI:10.1053/j.sempedsurg.2016.10.008.
[22] Peng CH, Chen YJ, Pang WB, et al.Redo transanal soave pull through with or without assistance in Hirschsprung disease:an experience in 46 patients[J].Eur J Pediatr Surg, 2021, 31(2):182-186.DOI:10.1055/s-0040-1710028.
[23] Chi SQ, Guo JL, Zhang X, et al.Resuturing without enterostomy for the treatment of Early-Stage anastomotic leaks after laparoscopic soave procedure in Hirschsprung’s disease[J].J Laparoendosc Adv Surg Tech A, 2020, 30(12):1295-1300.DOI:10.1089/lap.2020.0640.
[24] de Andres Crespo M, Bystr?m C, Tyraskis A, et al.Long-term effects of Hirschsprung disease in adults:meta-analysis and patient-level regression study[J].BJS Open, 2025, 9(6):zraf107.DOI:10.1093/bjsopen/zraf107.
[25] Tran VQ, Mahler T, Dassonville M, et al.Long-term outcomes and quality of life in patients after soave pull-through operation for Hirschsprung’s disease:an observational retrospective study[J].Eur J Pediatr Surg, 2018, 28(5):445-454.DOI:10.1055/s-0037-1604115.
[26] 谢川平, 严佳虞, 陈亚军.先天性巨结肠术后大便失禁的诊疗进展[J].中华小儿外科杂志, 2024, 45(10):953-958.DOI:10.3760/cma.j.cn421158-20231116-00499. Xie CP, Yan JY, Chen YJ.Advances in the diagnosis and treatment of postoperative fecal incontinence in Hirschsprung disease[J].Chin J Pediatr Surg, 2024, 45(10):953-958.DOI:10.3760/cma.j.cn421158-20231116-00499.
[27] Saadai P, Trappey AF, Goldstein AM, et al.Guidelines for the management of postoperative soiling in children with Hirschsprung disease[J].Pediatr Surg Int, 2019, 35(8):829-834.DOI:10.1007/s00383-019-04497-y.
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备注/Memo
收稿日期:2026-2-11。
通讯作者:陈亚军,Email:chenyajunmd@aliyun.com