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伴有胆管炎和胰腺炎的长发公主综合征——一例罕见病例报告

Rapunzel syndrome with cholangitis and pancreatitis - A rare case report.

作者信息

Vellaisamy Rajendran, Iyer Shruti, Chandramohan Servarayan Murugesan, Harikrishnan Sakthivel

机构信息

Department of General Surgery, Madras Medical College, EsoIndia Research Volunteer, Chennai 600003, India.

Sri Ramachandra Institute of Higher Education and Research, EsoIndia Research Volunteer, No. 1, Ramachandra Nagar, Porur, Chennai 600116, India.

出版信息

Open Med (Wars). 2020 Nov 14;15(1):1137-1142. doi: 10.1515/med-2020-0243. eCollection 2020.

Abstract

Rapunzel syndrome, defined by the presence of a trichobezoar extending from the stomach to the small intestine, is a rare cause of intestinal obstruction. It usually presents with vague symptoms; however, it can also present with complications such as perforation, peritonitis and obstructive jaundice. We report a rare case of a 37-year-old woman with Rapunzel syndrome complicated by acute cholangitis and pancreatitis and analyse the diagnosis and management of this complicated pathology. Although she reported a history of trichotillomania and trichophagia, she had been asymptomatic for ten years. We review the steps of diagnosis, highlighting the importance of a thorough clinical history and detailed examination, with supporting evidence from the contrast-enhanced computed tomography (CECT) scan. She was successfully managed with gastrotomy and trichobezoar removal. She had an uneventful postoperative recovery and was discharged after psychiatric counselling. To our knowledge, this is the first case of Rapunzel syndrome in a young female presenting with both cholangitis and pancreatitis.

摘要

长发公主综合征是指胃内毛发团块延伸至小肠,是肠梗阻的罕见病因。其症状通常不明确;然而,也可能出现穿孔、腹膜炎和梗阻性黄疸等并发症。我们报告一例罕见的37岁女性长发公主综合征病例,该病例并发急性胆管炎和胰腺炎,并分析这种复杂病理情况的诊断和治疗。尽管她有拔毛癖和食毛症病史,但已十年无症状。我们回顾诊断步骤,强调全面临床病史和详细检查的重要性,并辅以增强计算机断层扫描(CECT)的证据支持。她通过胃切开术成功移除毛发团块。术后恢复顺利,经心理咨询后出院。据我们所知,这是首例年轻女性长发公主综合征并发胆管炎和胰腺炎的病例。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/f239/7718640/bc29c76a63ec/j_med-2020-0243-fig001.jpg

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