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肛门直肠畸形合并先天性巨结肠:一例报告。

Anorectal malformation combined with Hirschsprung's disease: a case report.

作者信息

Fan Jiansen, Liu Mingkun, Lin Yu, He Yuanbin, Fang Yifan

机构信息

Fujian Children's Hospital (Fujian Branch of Shanghai Children's Medical Center), College of Clinical Medicine for Obstetrics & Gynecology and Pediatrics, Fujian Medical University, Fuzhou, China.

出版信息

Front Pediatr. 2023 May 24;11:1182342. doi: 10.3389/fped.2023.1182342. eCollection 2023.

Abstract

Anorectal malformation (ARM) and Hirschsprungs disease (HSCR) are frequently associated with other congenital malformations, but rarely with one another. We describe the case of a child with intermediate anorectal malformation who underwent ARM correction. This child experienced recurrent postoperative symptoms, including intestinal obstruction, nutrition intolerance, and weight loss. The child was diagnosed with Hirschsprung's disease by colon barium contrast and pathological findings from a rectal biopsy, and subsequently underwent pull -through procedure after conservative treatment failed. After six months of postoperative follow-up, the patient still experiences occasional episodes of enteritis, but the symptoms are substantially less severe than they were before surgery, and the patient's weight is slowly increasing. We described a case of a child who had ARM combined with HSCR. Although the association between ARM and HSCR is uncommon, severe constipation or enteritis following complete correction of ARM in the absence of anal stricture should prompt consideration for HSCR. Before the second stage of ARM surgery, pay close attention to the barium enema examination, as an abnormal shape may indicate the presence of HSCR.

摘要

肛门直肠畸形(ARM)和先天性巨结肠病(HSCR)常与其他先天性畸形相关,但彼此之间很少关联。我们描述了一名患有中度肛门直肠畸形的儿童接受ARM矫正手术的病例。该患儿术后反复出现肠梗阻、营养不耐受和体重减轻等症状。通过结肠钡剂造影和直肠活检的病理结果,该患儿被诊断为先天性巨结肠病,在保守治疗失败后随后接受了拖出术。术后随访六个月,患者仍偶尔出现肠炎发作,但症状比手术前明显减轻,且体重在缓慢增加。我们描述了一例患有ARM合并HSCR的儿童病例。尽管ARM与HSCR之间的关联并不常见,但在没有肛门狭窄的情况下,ARM完全矫正后出现严重便秘或肠炎应考虑HSCR。在ARM手术的第二阶段之前,要密切关注钡剂灌肠检查,因为异常形态可能提示存在HSCR。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/e69c/10244652/504a863e5997/fped-11-1182342-g001.jpg

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