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成人肠神经节发育不良罕见病例报告及文献复习。

Rare case of adult intestinal hypoganglionosis and review of the literature.

机构信息

Colorectal Surgery Unit, Dandenong Hospital, Monash Health, 135 David Street, Dandenong, VIC, Australia.

Department of Surgery, School of Clinical Sciences at Monash Health, Monash University, Clayton, VIC, Australia.

出版信息

Clin J Gastroenterol. 2021 Apr;14(2):599-607. doi: 10.1007/s12328-021-01342-5. Epub 2021 Jan 27.

DOI:10.1007/s12328-021-01342-5
PMID:33502729
Abstract

Intestinal hypoganglionosis is a rare condition in adults. We report a case of intestinal hypoganglionosis in the mid-distal transverse colon to splenic flexure in a 65-year-old female patient presenting with altered bowel habit and abdominal distension, and reviewed the current literature on this topic. Our patient had a medical history of neurofibromatosis type 1. A preoperative computed tomography (CT) scan demonstrated a grossly dilated transverse colon without obstruction. A laparotomy for subtotal colectomy was performed, with histopathology demonstrating intestinal hypoganglionosis.

摘要

肠无神经节细胞增生症在成人中较为罕见。我们报告了一例发生于横结肠中下段至脾曲的肠无神经节细胞增生症病例,患者为 65 岁女性,表现为排便习惯改变和腹胀,同时对该主题的现有文献进行了复习。我们的患者有神经纤维瘤病 1 型病史。术前计算机断层扫描(CT)显示横结肠明显扩张但无梗阻。行次全结肠切除术,组织病理学显示肠无神经节细胞增生症。

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Arch Pathol Lab Med. 2019 Feb;143(2):235-243. doi: 10.5858/arpa.2017-0524-RA. Epub 2018 Aug 8.
3
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4
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Virchows Arch. 2017 Jun;470(6):679-685. doi: 10.1007/s00428-017-2128-9. Epub 2017 Apr 19.
5
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Dig Liver Dis. 2017 Jan;49(1):104. doi: 10.1016/j.dld.2016.10.002. Epub 2016 Oct 18.
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