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成人特发性肥厚性幽门狭窄伴胃十二指肠套叠:一例罕见病例报告。

Adult idiopathic hypertrophic pyloric stenosis presenting with gastroduodenal intussusception: A rare case report.

作者信息

Guo Hou Loo, Nadia Nafasha Baharudin, Sultan Mohamed Arif Hameed, Reynu Rajan, Kosai Nik Ritza, Hamidi Lizawati Rasul

机构信息

Department of General Surgery, National University of Malaysia Faculty of Medicine, Bandar Tun Razak, Malaysia.

Department of Pathology, National University of Malaysia Faculty of Medicine, Bandar Tun Razak, Malaysia.

出版信息

Turk J Surg. 2023 Dec 29;39(4):377-382. doi: 10.47717/turkjsurg.2023.4552. eCollection 2023 Dec.

Abstract

Adult idiopathic hypertrophic pyloric stenosis (AIHPS) is a rare entity first described by Cruveilhier in 1835. There are only approximately 200 cases reported in the English literature to date. Histologically, it may be mistaken for spindle cell neoplasms such as gastrointestinal stromal tumour (GIST). Patients with AIHPS usually present with early satiety, abdominal fullness, postprandial vomiting, epigastric pain, and eructations. Adult intussusception is rare and only accounts for 5% of all intussusceptions. Gastroduodenal intussusception is one of the rare types of adult intussusception. This is more likely to occur when a benign or malignant stomach lesion acts as a lead point. We report a case of AIHPS in a 70-year-old lady presenting with gastroduodenal intussusception. An oesophagogastroduodenoscopy (OGDS) was performed, and it revealed a diffusely thickened and narrowed pyloric antrum. A contrasted computed tomography (CECT) of the thorax and abdomen showed a distended stomach with circumferential thickening of the pylorus. The pre-pyloric antrum was intussuscepting into the pylorus, and the apex is seen within the first part of duodenum. She underwent distal gastrectomy with a Roux-en-y reconstruction via laparoscopic approach and was discharged well. AIHPS is a rare condition and should be a differential in adults presenting with gastric outlet obstruction. We believe in cases of AIHPS presenting with gastroduodenal intussusception, a distal gastrectomy with reconstruction is a reasonable approach. A multidisciplinary approach is essential to obtain the best outcome.

摘要

成人特发性肥厚性幽门狭窄(AIHPS)是一种罕见疾病,最早由克鲁维耶于1835年描述。迄今为止,英文文献中仅报道了约200例病例。在组织学上,它可能被误诊为梭形细胞瘤,如胃肠道间质瘤(GIST)。AIHPS患者通常表现为早饱、腹胀、餐后呕吐、上腹部疼痛和嗳气。成人肠套叠很少见,仅占所有肠套叠的5%。胃十二指肠套叠是成人肠套叠的罕见类型之一。当良性或恶性胃部病变作为引导点时,这种情况更易发生。我们报告一例70岁女性AIHPS患者,表现为胃十二指肠套叠。进行了食管胃十二指肠镜检查(OGDS),结果显示幽门窦弥漫性增厚和狭窄。胸部和腹部的增强计算机断层扫描(CECT)显示胃扩张,幽门周向增厚。幽门窦套入幽门,套叠顶端位于十二指肠第一部内。她通过腹腔镜手术接受了远端胃切除术并进行了Roux-en-y重建,术后恢复良好出院。AIHPS是一种罕见疾病,在出现胃出口梗阻的成人患者中应作为鉴别诊断之一。我们认为,对于出现胃十二指肠套叠的AIHPS病例,行远端胃切除术并重建是一种合理的治疗方法。多学科方法对于获得最佳治疗效果至关重要。

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