Fang Shu,Huang Zhaowei,Zhao Xiaoxia,et al.Association of congenital intestinal atresia and Hirschsprung’s disease: five new cases and its diagnosis and treatment[J].Journal of Clinical Pediatric Surgery,2023,22(09):822-826.[doi:10.3760/cma.j.cn101785-202305044-005]
先天性肠闭锁合并巨结肠的诊断与治疗
- Title:
- Association of congenital intestinal atresia and Hirschsprung’s disease: five new cases and its diagnosis and treatment
- 摘要:
- 目的 总结新生儿先天性肠闭锁合并巨结肠的特点,探讨其诊断与治疗经验。方法 回顾性分析浙江大学医学院附属儿童医院2016年5月至2021年8月收治的5例先天性肠闭锁合并巨结肠患儿的临床资料。5例均行剖腹探查术,均为单发肠闭锁(4例回肠闭锁、1例升结肠闭锁)。其中2例回肠闭锁患儿行闭锁肠段切除及一期吻合术, 2例回肠闭锁及1例结肠闭锁患儿一期行闭锁肠段切除及小肠造瘘术。结果 2例一期行肠吻合术患儿术后因肠梗阻再次行剖腹探查,发现原吻合口通畅,远端结肠仍细小痉挛,行小肠造瘘及肠壁多点活检后确诊合并先天性巨结肠;3例一期行闭锁肠段切除及小肠造瘘术患儿术后恢复良好,其中2例回肠闭锁患儿行下消化道造影及直肠黏膜活检,确诊合并先天性巨结肠,1例升结肠闭锁患儿经一期术中乙状结肠活检确诊先天性巨结肠。5例均行巨结肠根治术,最终确诊先天性巨结肠病理类型为:3例全结肠型巨结肠、1例全肠型巨结肠以及1例长段型巨结肠。5例最终均恢复良好。结论 肠闭锁矫治术中探查远端肠管形态、乙状结肠长度以及完善肠壁活检对肠闭锁治疗方式的选择十分重要。对远端肠管功能存疑的患儿,行肠造瘘术可以减少肠闭锁矫治术后因急腹症再次手术的可能。下消化道钡剂造影及直肠黏膜活检对进一步明确远端肠管形态及功能具有重要价值。
- Abstract:
- Objective To summarize the clinical characteristics of congenital intestinal atresia (CIA) complicating Hirschsprung’s disease (HD) and review its diagnostic and surgical experiences.Methods From May 2016 to August 2021,5 children were diagnosed as CIA combined with HD at Children’s Hospital of Zhejiang University School of Medicine.Retrospective review was conducted for the relevant clinical data.Laparotomy was performed for intestinal obstruction.There were ileal atresia (n=4) and ascending colon atresia (n=1).Two children of ileal atresia underwent atresia intestinal resection with primary anastomosis.Another 2 children of ileal atresia and 1 child of colonic atresia underwent atresia intestinal resection and enterostomy.Results Two children of primary anastomosis underwent a second laparotomy for postoperative intestinal obstruction.Enterostomy was performed for patent anastomosis and microcolon.An association of HD was confirmed by multiple biopsies of distal intestine.Two children of ileal atresia and enterostomy showed abnormal barium enema and both were diagnosed as HD after rectal biopsy.One child of ascending colonic atresia was diagnosed as HD by intraoperative sigmoid biopsy and rectal biopsy.After modified Swenson procedure,all of them survived and recovered well.Total colonic HD (n=3),total intestinal aganglionosis (n=1) and long segment HD (n=1) were confirmed.Conclusion Identification of colonic morphology,length of sigmoid and intestinal biopsy are essential for surgical correction of intestinal atresia.For lowering the possibility of laparotomy,enterostomy is recommended when colonic function is in doubt.Barium enema and rectal biopsy are important tools for ascertaining the morphologies and functions of colon.
参考文献/References:
[1] Adams SD,Stanton MP.Malrotation and intestinal atresias[J].Early Hum Dev,2014,90(12):921-925.DOI:10.1016/j.earlhumdev.2014.09.017.
[2] Sabbatini S,Ganji N,Chusilp S,et al.Intestinal atresia and necrotizing enterocolitis:embryology and anatomy[J].Semin Pediatr Surg,2022,31(6):151234.DOI:10.1016/j.sempedsurg.2022.151234.
[3] 郭卫红,陈永卫,侯大为,等.先天性肠闭锁病死率40年回顾性分析[J].中华小儿外科杂志,2011,32(6):434-437.DOI:10.3760/cma.j.issn.0253-3006.2011.06.010. Guo WH,Chen YW,Hou DW,et al.Morbidity and mortality of congenital intestinal atresia:a review of 40 years[J].Chin J Pediatr Surg,2011,32(6):434-437.DOI:10.3760/cma.j.issn.0253-3006.2011.06.010.
[4] Wang J,Du L,Cai W,et al.Prolonged feeding difficulties after surgical correction of intestinal atresia:a 13-year experience[J].J Pediatr Surg,2014,49(11):1593-1597.DOI:10.1016/j.jpedsurg.2014.06.010.
[5] 王练英,张志波,李正.新生儿肠闭锁的治疗[J].中华小儿外科杂志,2001,22(6):340.DOI:10.3760/cma.j.issn.0253-3006.2001.06.007. Wang LY,Zhang ZB,Li Z.Treatment of neonatal intestinal atresia[J].Chin J Pediatr Surg,2001,22(6):340.DOI:10.3760/cma.j.issn.0253-3006.2001.06.007.
[6] Klein M,Varga I.Hirschsprung’s disease-recent understanding of embryonic aspects,etiopathogenesis and future treatment avenues[J].Medicina (Kaunas),2020,56(11):611.DOI:10.3390/medicina56110611.
[7] Lall A,Gupta DK,Bajpai M.Neonatal Hirschsprung’s disease[J].Indian J Pediatr,2000,67(8):583-588.DOI:10.1007/BF02758486.
[8] Moore SW.Total colonic aganglionosis and Hirschsprung’s disease:a review[J].Pediatr Surg Int,2015,31(1):1-9.DOI:10.1007/s00383-014-3634-3.
[9] Kim PC,Superina RA,Ein S.Colonic atresia combined with Hirschsprung’s disease:a diagnostic and therapeutic challenge[J].J Pediatr Surg,1995,30(8):1216-1217.DOI:10.1016/0022-3468(95)90026-8.
[10] Lauwers P,Moens E,Wustenberghs K,et al.Association of colonic atresia and Hirschsprung’s disease in the newborn:report of a new case and review of the literature[J].Pediatr Surg Int,2006,22(3):277-281.DOI:10.1007/s00383-005-1456-z.
[11] El-Asmar KM,Abdel-Latif M,El-Kassaby AHA,et al.Colonic atresia:association with other anomalies[J].J Neonatal Surg,2016,5(4):47.DOI:10.21699/jns.v5i4.422.
[12] Wang KS,Cahill JL,Skarsgard ED.Omphalocele,colonic atresia,and Hirschsprung’s disease:an unusual cluster of malformations in a single patient[J].Pediatr Surg Int,2001,17(2/3):218-220.DOI:10.1007/s003830000459.
[13] 严佳虞,陈亚军,陈永卫,等.先天性肠闭锁合并巨结肠四例并文献复习[J].中华小儿外科杂志,2020,41(9):806-812.DOI:10.3760/cma.j.cn421158-20190523-00369. Yan JY,Chen YJ,Chen YW,et al.Intestinal atresia complicated with Hirschsprung’s disease:a report of 4 cases with a literature review[J].Chin J Pediatr Surg,2020,41(9):806-812.DOI:10.3760/cma.j.cn421158-20190523-00369.
[14] Gupta M,Beeram MR,Pohl JF,et al.Ileal atresia associated with Hirschsprung disease (total colonic aganglionosis)[J].J Pediatr Surg,2005,40(9):e5-e7.DOI:10.1016/j.jpedsurg.2005.05.064.
[15] Atqiaee K,Hiradfar M,Mashhadi MP,et al.Ileal atresia and total colonic Hirschsprung disease in a 36-week neonate:a case report[J].Clin Case Rep,2023,11(3):e7079.DOI:10.1002/ccr3.7079.
[16] Janik JP,Wayne ER,Janik JS,et al.Ileal atresia with total colonic aganglionosis[J].J Pediatr Surg,1997,32(10):1502-1503.DOI:10.1016/s0022-3468(97)90576-5.
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备注/Memo
收稿日期:2023-5-26。
基金项目:国家自然科学基金面上项目(82171699);浙江省自然科学基金(LY21H150005)
通讯作者:钭金法,Email:toujinfa@zju.edu.cn