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长发公主综合征:一例伴有小肠并发症的毛粪石病例。

Rapunzel Syndrome: A Case of Trichobezoar with Small Bowel Complications.

作者信息

Narra Ramakrishna, Guntamukkala Anusha, Rao Chanda Bhaskara, Begum Tanveer

机构信息

Department of Radio-diagnosis, Katuri Medical College, Guntur, Andhra Pradesh, India.

Department of Pediatric Surgery, Guntur Medical College, Guntur, Andhra Pradesh, India.

出版信息

Surg J (N Y). 2022 Nov 6;8(4):e293-e296. doi: 10.1055/s-0042-1757777. eCollection 2022 Oct.

Abstract

Rapunzel syndrome is characterized by a large trichobezoar in the stomach with a tail extending beyond the pylorus into the small bowel, causing mechanical obstruction of the small bowel. A 7-year-old girl presented to the emergency room with severe epigastric pain. Computed tomography suggested trichobezoar causing jejuno-jejunal intussusceptions, bowel wall thickening, and dilated small bowel loops proximal to the obstruction. On laparotomy, two concealed perforations were noted at the duodenojejunal (DJ) junction and 40 cm distal to the DJ junction. An enterotomy incision was given at the antimesenteric border of the distal jejunal perforation site, and the mass was successfully extracted. Primary repair was done at the DJ perforation site, and resection was followed by an end-to-end anastomosis at the distal jejunal perforation site. Surgery confirmed a complex mass of tangled hair within the gastric cavity with a tail extending into the pylorus of the stomach and small intestine, consistent with trichobezoar.  Computed tomography is superior to other radiological imaging modalities for diagnosing trichobezoars as it helps diagnose and demonstrate mechanical bowel complications.

摘要

长发公主综合征的特征是胃内有一个大的毛粪石,其尾部延伸至幽门以外进入小肠,导致小肠机械性梗阻。一名7岁女孩因严重上腹部疼痛被送往急诊室。计算机断层扫描提示毛粪石导致空肠-空肠套叠、肠壁增厚以及梗阻近端小肠肠袢扩张。剖腹手术时,在十二指肠空肠(DJ)交界处及DJ交界处远端40厘米处发现两个隐匿性穿孔。在远端空肠穿孔部位的系膜对侧缘做肠切开切口,成功取出肿块。DJ穿孔部位进行了一期修复,远端空肠穿孔部位切除后进行了端端吻合。手术证实胃腔内有一团纠结的毛发形成的复杂肿块,其尾部延伸至胃幽门和小肠,符合毛粪石表现。计算机断层扫描在诊断毛粪石方面优于其他放射成像方式,因为它有助于诊断并显示肠道机械性并发症。

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