Zeng Jixiao,Xu Xiaogang.Some focal issues of standardizing the diagnosis and treatment of Hirschsprung’s disease[J].Journal of Clinical Pediatric Surgery,2021,20(03):201-207.[doi:10.12260/lcxewkzz.2021.03.001]
先天性巨结肠诊疗规范化的再思考
- Title:
- Some focal issues of standardizing the diagnosis and treatment of Hirschsprung’s disease
- 关键词:
- 先天性巨结肠/诊断; 先天性巨结肠/外科学; 手术后并发症
- 分类号:
- R726.1;R574
- 摘要:
- 先天性巨结肠(Hirschsprung’s disease,HSCR)是小儿肛肠外科常见疾病,规范化的诊疗可以减少HSCR的误诊与误治,降低手术并发症的发生率和再次手术的概率。然而,我国大陆地区HSCR的诊疗策略经过数十年发展与演变仍未统一,一些诊疗的关键标准仍欠规范。近年来国内关于HSCR的临床研究和指南针对以上问题进行了深入探讨,但仍有部分患者未能从中获益。HSCR的规范化治疗对于患者实现个体化诊疗意义重大,标准化、规范化的流程有助于HSCR的精准诊断与手术治疗。本文就HSCR的精准诊断、手术时机、手术方式选择以及新技术应用等方面进行阐述。
- Abstract:
- Hirschsprung’s disease (HSCR) is a common disease in pediatric anorectal surgery.Standardized diagnosis and therapy may reduce its misdiagnostic and therapeutic errors,lower its incidence of complications and minimize redo pull-through procedure.However,its strategies of diagnosis and treatment in mainland China has not been unified after decades of development and evolution and some focal issues requires standardization.Recently domestic clinical studies and guidelines on HSCR have extensively discussed these issues.However,some patients have not benefited.Standardizing its diagnosis and treatment is essential for achieving individualized protocols for HSCR patients so as to ensure an accurate diagnosis and an optimal timing of operation.
参考文献/References:
1 Veras LV,Arnold M,Avansino JR,et al.Guidelines for synoptic reporting of surgery and pathology in Hirschsprung disease[J].J Pediatr Surg,2019,54(10):2017-2023.DOI:10.1016/j.jpedsurg.2019.03.010.
2 汤绍涛,常晓盼.对微创外科在先天性巨结肠应用现状及未来趋势的思考[J].临床小儿外科杂志,2020,19(1):1-6.DOI:10.3969/j.issn.1671-6353.2020.01.001. Tang ST,Chang XP.Retrospective review and future prospects of technological evolutions of mini-invasive surgery for Hirschsprung’s disease[J].J Clin Ped Sur,2020,19(1):1-6.DOI:10.3969/j.issn.1671-6353.2020.01.001.
3 中华医学会小儿外科学分会内镜外科学组.腹腔镜先天性巨结肠手术操作指南(2017版)[J].中华小儿外科杂志,2017,38(4):247-254.DOI:10.3760/cma.j.issn.0253-3006.2017.04.002. Section of Endoscopy,Branch of Pediatric Surgery,Chinese Medical Association:Guideline for Laparoscopic Pull-through for Hirschsprung’s Disease (2017 Edition)[J].Chin J Pediatr Surg,2017,38(4):247-254.DOI:10.3760/cma.j.issn.0253-3006.2017.04.002.
4 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. Groups of Anorectum and Neonatology,Society of Pediatric Surgery,Chinese Medical Association: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.
5 高亚.先天性巨结肠的诊断与治疗挑战与机遇[J].中华小儿外科杂志,2018,39(6):401-403.DOI:10.3760/cma.j.issn.0253-3006.2018.06.001. Gao Y.Challenges and opportunities of diagnosing and treating Hirschsprung’s disease[J].Chin J Pediatr Surg,2018,39(6):401-403.DOI:10.3760/cma.j.issn.0253-3006.2018.06.001.
6 谢崇,潘伟康,高亚,等.我国大陆地区先天性巨结肠诊疗情况调查[J].中华小儿外科杂志,2018,39(6):411-418.DOI:10.3760/cma.j.issn.0253-3006.2018.06.003. Xie C,Pan WK,Gao Y,et al.Surveying the diagnosis and management of Hirschsprung’s disease in mainland China[J].Chin J Pediatr Surg,2018,39(6):411-418.DOI:10.3760/cma.j.issn.0253-3006.2018.06.003.
7 曾纪晓.先天性巨结肠非计划性再手术的相关问题[J].临床小儿外科杂志,2018,17(2):94-98.DOI:10.3969/j.issn.1671-6353.2018.02.004. Zeng JX.Related issues of unscheduled re-operation for the treatment of Hirschsprung’s disease[J].J Clin Ped Sur,2018,17(2):94-98.DOI:10.3969/j.issn.1671-6353.2018.02.004.
8 Friedmacher F,Puri P.Rectal suction biopsy for the diagnosis of Hirschsprung’s disease:a systematic review of diagnostic accuracy and complications[J].Pediatr Surg Int,2015,31(9):821-830.DOI:10.1007/s00383-015-3742-8.
9 de Lorijn F,Boeckxstaens GE,Benninga MA.Symptomatology,pathophysiology,diagnostic work-up,and treatment of Hirschsprung disease in infancy and childhood[J].Curr Gastroenterol Rep,2007,9(3):245-253.DOI:10.1007/s11894-007-0026-z.
10 Muise ED,Cowles RA.Rectal biopsy for Hirschsprung’s disease:a review of techniques,pathology,and complications[J].World J Pediatr,2016,12(2):135-141.DOI:10.1007/s12519-015-0068-5.
11 De La Torre L,Wehrli LA.Error traps and culture of safety in Hirschsprung disease[J].Semin Pediatr Surg,2019,28(3):151-159.DOI:10.1053/j.sempedsurg.2019.04.013.
12 Friedmacher F,Puri P.Current practice patterns of rectal suction biopsy in the diagnostic work-up of Hirschsprung’s disease:results from an international survey[J].Pediatr Surg Int,2016,32(8):717-722.DOI:10.1007/s00383-016-3907-0.
13 Zani A,Eaton S,Morini F,et al.European Paediatric Surgeons’Association Survey on the Management of Hirschsprung Disease[J].Eur J Pediatr Surg,2017,27(1):96-101.DOI:10.1055/s-0036-1593991.
14 Ad?güzel ǔ,A?engin K,K?r???o?lu I,et al.Transanal endorectal pull-through for Hirschsprung’s disease:experience with 50 patients[J].Ir J Med Sci,2017,186(2):433-437.DOI:10.1007/s11845-016-1446-2.
15 Neuvonen MI,Kyrklund K,Lindahl HG,et al.A population-based,complete follow-up of 146 consecutive patients after transanal mucosectomy for Hirschsprung disease[J].J Pediatr Surg,2015,50(10):1653-1658.DOI:10.1016/j.jpedsurg.2015.02.006.
16 Zhu T,Sun X,Wei M,et al.Optimal time for single-stage pull-through colectomy in infants with short-segment Hirschsprung disease[J].Int J Colorectal Dis,2019,34(2):255-259.DOI:10.1007/s00384-018-3179-3.
17 Langer JC.Laparoscopic and transanal pull-through for Hirschsprung disease[J].Semin Pediatr Surg,2012,21(4):283-290.DOI:10.1053/j.sempedsurg.07.002.
18 Levitt MA,Dickie B,Pe?a A.The Hirschsprungs patient who is soiling after what was considered a "successful" pull-through[J].Semin Pediatr Surg,2012,21(4):344-353.DOI:10.1053/j.sempedsurg.2012.07.009.
19 V? PA,Thien HH,Hiep PN.Transanal one-stage endorectal pull-through for Hirschsprung disease:experiences with 51 newborn patients[J].Pediatr Surg Int,2010,26(6):589-592.DOI:10.1007/s00383-010-2599-0.
20 Lu C,Hou G,Liu C,et al.Single-stage transanal endorectal pull-through procedure for correction of Hirschsprung disease in neonates and nonneonates:A multicenter study[J].J Pediatr Surg,2017,52(7):1102-1107.DOI:10.1016/j.jpedsurg.2017.01.061.
21 De La Torre L,Langer JC.Transanal endorectal pull-through for Hirschsprung disease:technique,controversies,pearls,pitfalls,and an organized approach to the management of postoperative obstructive symptoms[J].Semin Pediatr Surg,2010,19(2):96-106.DOI:10.1053/j.sempedsurg.2009.11.016.
22 De la Torre-Mondragón L,Ortega-Salgado JA.Transanal endorectal pull-through for Hirschsprung’s disease[J].J Pediatr Surg,1998,33(8):1283-1286.DOI:10.1016/s0022-3468(98)90169-5.
23 Kim AC,Langer JC,Pastor AC,et al.Endorectal pull-through for Hirschsprung’s disease-a multicenter,long-term comparison of results:transanal vs transabdominal approach[J].J Pediatr Surg,2010,45(6):1213-1220.DOI:10.1016/j.jpedsurg.2010.02.087.
24 Ishikawa N,Kubota A,Kawahara H,et al.Transanal mucosectomy for endorectal pull-through in Hirschsprung’s disease:comparison of abdominal,extraanal and transanal approaches[J].Pediatr Surg Int,2008,24(10):1127-1129.DOI:10.1007/s00383-008-2231-8.
25 El-Sawaf MI,Drongowski RA,Chamberlain JN,et al.Are the long-term results of the transanal pull-through equal to those of the transabdominal pull-through? A comparison of the 2 approaches for Hirschsprung disease[J].J Pediatr Surg,2007,42(1):41-47.DOI:10.1016/j.jpedsurg.2006.09.007.
26 Obermayr F,Szavay P,Beschorner R,et al.Outcome of transanal endorectal pull-through in patients with Hirschsprung’s disease[J].Eur J Pediatr Surg,2009,19(4):220-223.DOI:10.1055/s-0029-1220682.
27 Miyano G,Takeda M,Koga H,et al.Hirschsprung’s disease in the laparoscopic transanal pull-through era:implications of age at surgery and technical aspects[J].Pediatr Surg Int,2018,34(2):183-188.DOI:10.1007/s00383-017-4187-z.
28 Stensrud KJ,Emblem R,Bj?rnland K.Anal endosonography and bowel function in patients undergoing different types of endorectal pull-through procedures for Hirschsprung disease[J].J Pediatr Surg,2015,50(8):1341-1346.DOI:10.1016/j.jpedsurg.2014.12.024.
29 Thomson D,Allin B,Long AM,et al.Laparoscopic assistance for primary transanal pull-through in Hirschsprung’s disease:a systematic review and meta-analysis[J].BMJ Open,2015,5(3):e006063.DOI:10.1136/bmjopen-2014-006063.
30 Arts E,Botden SM,Lacher M,et al.Duhamel versus transanal endorectal pull through (TERPT) for the surgical treatment of Hirschsprung’s disease[J].Tech Coloproctol,2016,20(10):677-682.DOI:10.1007/s10151-016-1524-5.
31 Levitt MA,Hamrick MC,Eradi B,et al.Transanal,full-thickness,Swenson-like approach for Hirschsprung disease[J].J Pediatr Surg,2013,48(11):2289-2295.DOI:10.1016/j.jpedsurg.2013.03.002.
32 Deng X,Wu Y,Zeng L,et al.Comparative analysis of modified laparoscopic swenson and laparoscopic Soave procedure for short-segment hirschsprung disease in children[J].Eur J Pediatr Surg,2015,25(5):430-434.DOI:10.1055/s-0034-1384647.
33 Yokota K,Uchida H,Tainaka T,et al.Single-stage laparoscopic transanal pull-through modified Swenson procedure without leaving a muscular cuff for short- and long-type Hirschsprung disease:a comparative study[J].Pediatr Surg Int,2018,34(10):1105-1110.DOI:10.1007/s00383-018-4318-1.
34 张茜,汤绍涛,曹国庆,等.da Vinci机器人辅助腹腔镜Soave拖出术治疗先天性巨结肠[J].中国微创外科杂志,2016,16(2):165-167,184.DOI:10.3969/j.issn.1009-6604.2016.02.019. Zhang Q,Tang ST,Cao GQ,et al.Robotic-assisted Soave pull-through for Hirschsprung’s disease in infants[J].Chin J Min Inv Surg,2016,16(2):165-167,184.DOI:10.3969/j.issn.1009-6604.2016.02.019.
35 Bischoff A,Frischer J,Knod JL,et al.Damaged anal canal as a cause of fecal incontinence after surgical repair for Hirschsprung disease-a preventable and under-reported complication[J].J Pediatr Surg,2017,52(4):549-553.DOI:10.1016/j.jpedsurg.2016.08.027.
36 Yamataka A,Miyano G,Takeda M.Minimally invasive neonatal surgery:Hirschsprung disease[J].Clin Perinatol,2017,44(4):851-864.DOI:10.1016/j.clp.2017.08.006.
37 Yamataka A,Yazaki Y,Koga H,et al.How best to expose the entire surgical anal canal in the operative field during transanal pull-through for Hirschsprung’s disease:a crucial step that determines success[J].Pediatr Surg Int,2019,35(2):199-202.DOI:10.1007/s00383-018-4395-1.
38 Miyano G,Koga H,Okawada M,et al.Rectal mucosal dissection commencing directly on the anorectal line versus commencing above the dentate line in laparoscopy-assisted transanal pull-through for Hirschsprung’s disease:Prospective medium-term follow-up[J].J Pediatr Surg,2015,50(12):2041-2043.DOI:10.1016/j.jpedsurg.2015.08.022.
39 Wada T,Kawada K,Takahashi R,et al.ICG fluorescence imaging for quantitative evaluation of colonic perfusion in laparoscopic colorectal surgery[J].Surg Endosc,2017,31(10):4184-4193.DOI:10.1007/s00464-017-5475-3.
40 van den Bos J,Al Taher M,Schols RM,et al.Near infrared fluorescence imaging for real-time intraoperative guidance in anastomotic colorectal surgery:a systematic review of literature[J].J Laparoendosc Adv Surg Tech A,2018,28(2):157-167.DOI:10.1089/lap.2017.0231.
41 Rentea RM,Halleran DR,Ahmad H,et al.Preliminary use of indocyanine green fluorescence angiography and value in predicting the vascular supply of tissues needed to perform cloacal,anorectal malformation and Hirschsprung reconstructions[J].Eur J Pediatr Surg,2020,30(6):505-511.DOI:10.1055/s-0039-1700548.
42 Pini Prato A,Arnoldi R,Dusio MP,et al.Totally robotic soave pull-through procedure for Hirschsprung’s disease:lessons learned from 11 consecutive pediatric patients[J].Pediatr Surg Int,2020,36(2):209-218.DOI:10.1007/s00383-019-04593-z.
43 徐晓钢,曾纪晓,刘斐,等.吲哚菁绿荧光血管显像技术在单孔腹腔镜巨结肠根治术中的应用[J].中国微创外科杂志,2021,21(2):165-168. Xu XG,Zeng JX,Liu F,et al.Application of indocyanine green fluorescence angiography in single-port laparoscopic radical operation for Hirschsprung’s disease[J].Chin J Min Inv Surg,2021,21(2):165-168.
44 Sch?ffel N,Gfroerer S,Rolle U,et al.Hirschsprung disease:critical evaluation of the global research architecture employing scientometrics and density-equalizing mapping[J].Eur J Pediatr Surg,2017,27(2):185-191.DOI:10.1055/s-0036-1583536.
45 Tomuschat C,Zimmer J,Puri P.Laparoscopic-assisted pull-through operation for Hirschsprung’s disease:a systematic review and meta-analysis[J].Pediatr Surg Int,2016,32(8):751-757.DOI:10.1007/s00383-016-3910-5.
46 Scholfield DW,Ram AD.Laparoscopic Duhamel procedure for Hirschsprung’s disease:systematic review and meta-analysis[J].J Laparoendosc Adv Surg Tech A,2016,26(1):53-61.DOI:10.1089/lap.2015.0121.
47 Gosemann JH,Friedmacher F,Ure B,et al.Open versus transanal pull-through for Hirschsprung disease:a systematic review of long-term outcome[J].Eur J Pediatr Surg,2013,23(2):94-102.DOI:10.1055/s-0033-1343085.
48 Taguchi T,Obata S,Ieiri S.Current status of Hirschsprung’s disease:based on a nationwide survey of Japan[J].Pediatr Surg Int,2017,33(4):497-504.DOI:10.1007/s00383-016-4054-3.
49 向磊,李娜萍,武海燕,等.先天性巨结肠及其同源病诊断热点讨论[J].中华小儿外科杂志,2016,37(4):248-252.DOI:10.3760/cma.j.issn.0253-3006.2016.04.003. Xiang L,Li NP,Wu HY,et al.Controversies concerning diagnostic guidelines for Hirschsprung’s disease and allied disorders[J].Chin J Pediatr Surg,2016,37(4):248-252.DOI:10.3760/cma.j.issn.0253-3006.2016.04.003.
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备注/Memo
收稿日期:2020-12-24。
基金项目:国家自然科学基金(编号:82070528);广东省自然科学基金(编号:2018A030313570);先天性巨结肠发病机制及转归预后前瞻性队列研究(编号:ChiCTR-eoc-17013386)
通讯作者:曾纪晓,Email:zengjixiao@163.com