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成人先天性巨结肠症的分类与外科治疗:一项回顾性研究

The Classification and Surgical Treatments in Adult Hirschsprung's Disease: A Retrospective Study.

作者信息

Ma Shengzhe, Yu Yue, Pan Anfu, Gong Haifeng, Lou Zheng, Liu Lianjie, Hao Liqiang, Meng Ronggui, Sui Jinke, Zhang Wei

机构信息

Department of Colorectal Surgery, Changhai Hospital, Shanghai, China.

出版信息

Front Med (Lausanne). 2022 Apr 8;9:870342. doi: 10.3389/fmed.2022.870342. eCollection 2022.

DOI:10.3389/fmed.2022.870342
PMID:35463022
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC9024099/
Abstract

PURPOSE

To explore the treatments and short-term effects of different types of adult Hirschsprung's disease.

METHODS

89 patients treated in Shanghai Changhai Hospital were retrospectively analyzed. According to the patient's medical history, clinical manifestations, auxiliary examination and postoperative pathological results, the patients were divided into adult congenital megacolon, adult idiopathic megacolon, ganglion cell deficiency (types I and II), toxic megacolon and iatrogenic megacolon, The Treatment methods and short-term prognosis of patients in each group were summarized.

RESULTS

41 cases of Hirschsprung's disease in adults and low anterior resection or pull-out low anterior resection was performed, and 35 patients with idiopathic Megacolon were treated with one-stage subtotal colon resection under the condition of adequate preoperative preparation. Some patients admitted for emergency intestinal obstruction received conservative treatment first or underwent elective surgery after colonoscopic decompression was improved; two patients with ganglion cell deficiency subtotal colectomy were performed to remove the dilated proximal bowel segment and the narrow distal bowel segment; three patients with toxic Hirschsprung's disease underwent colostomy in mild cases, while subtotal colorectal resection was required in severe cases; Iatrogenic megacolon was diagnosed in eight cases and the optimum operation should be selected according to the specific conditions of patients.

CONCLUSION

Adult Hirschsprung's diseases were divided into adult congenital hirschsprung's disease, idiopathic Hirschsprung's disease, ganglion cell deficiency, toxic hirschsprung's disease, and iatrogenic Hirschsprung's disease. Different types of surgical treatments for Hirschsprung's disease in adults should be selected according to the specific diagnosis. All patients with adult Hirschsprung's diseases have good short-term outcomes after surgical treatment.

摘要

目的

探讨不同类型成人先天性巨结肠症的治疗方法及短期疗效。

方法

回顾性分析上海长海医院收治的89例患者。根据患者病史、临床表现、辅助检查及术后病理结果,将患者分为成人先天性巨结肠、成人特发性巨结肠、神经节细胞缺乏症(Ⅰ型和Ⅱ型)、中毒性巨结肠和医源性巨结肠,总结每组患者的治疗方法及短期预后。

结果

41例成人先天性巨结肠症患者行低位前切除术或拖出式低位前切除术,35例特发性巨结肠患者在充分的术前准备下接受一期全结肠切除术。部分因急性肠梗阻入院的患者先接受保守治疗或在结肠镜减压改善后接受择期手术;2例神经节细胞缺乏症患者行全结肠切除术,切除扩张的近端肠段和狭窄的远端肠段;3例中毒性先天性巨结肠症患者轻症行结肠造口术,重症则需行全结直肠切除术;8例诊断为医源性巨结肠,应根据患者具体情况选择最佳手术方式。

结论

成人先天性巨结肠症分为成人先天性巨结肠症、特发性先天性巨结肠症、神经节细胞缺乏症、中毒性先天性巨结肠症和医源性先天性巨结肠症。成人先天性巨结肠症的不同类型应根据具体诊断选择不同的手术治疗方法。所有成人先天性巨结肠症患者手术治疗后短期疗效良好。

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Ir J Med Sci. 2022 Dec;191(6):2669-2675. doi: 10.1007/s11845-021-02906-7. Epub 2022 Jan 6.
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Stercoral ulcer after Hirschsprung's disease surgery.先天性巨结肠症手术后的粪性溃疡
Clin Case Rep. 2021 Dec 26;9(12):e05239. doi: 10.1002/ccr3.5239. eCollection 2021 Dec.
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Idiopathic Megacolon-Short Review.
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Diagnostics (Basel). 2021 Nov 15;11(11):2112. doi: 10.3390/diagnostics11112112.
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Long-term surgical and patient-reported outcomes of Hirschsprung Disease.先天性巨结肠症的长期手术和患者报告结果。
J Pediatr Surg. 2021 Sep;56(9):1502-1511. doi: 10.1016/j.jpedsurg.2021.01.043. Epub 2021 Feb 13.
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Pediatr Int. 2021 Sep;63(9):1095-1102. doi: 10.1111/ped.14596. Epub 2021 Jul 29.
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