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一例罕见的伴有婴儿肥厚性幽门狭窄的威尔基综合征。

A rare case of Wilkie's syndrome with infantile hypertrophic pyloric stenosis.

作者信息

Stamov Petar, Stamova Sylvia

机构信息

Department of General and Operative Surgery, Medicine Faculty, Medical University of Varna, Bulgaria.

Department of Pharmaceutical Chemistry, Pharmacy Faculty, Medical University of Varna, Bulgaria.

出版信息

Med Pharm Rep. 2025 Apr;98(2):257-261. doi: 10.15386/mpr-2724. Epub 2025 Apr 29.

Abstract

Wilkie's syndrome is a rare clinical disorder characterized by partial or complete obstruction of the duodenum. Infantile hypertrophic pyloric stenosis (IHPS) is defined as hyperplasia of the smooth muscle fibers of the pylorus, which is considered responsible for narrowing the pyloric canal and eventually obstructing the gastric outlet. This paper presents a case of a 3-month-old boy who had been vomiting after feeding for one month. The main symptoms, postprandial vomiting, dehydration, alkalosis, and weight reduction, point to the IHPS disease. Imaging studies also confirmed the diagnosis. During the surgical treatment, a concomitant condition known as Wilkie's syndrome was also evidenced. The team performed a complex surgical treatment of a patient diagnosed with both Wilkie's syndrome and IHPS within one single surgical operation. Surgical treatment with pyloromyotomy and duodenal-duodenal anastomosis within one operation is an optimal option for one-stage treatment of patients with IHPS and Wilkie's syndrome.

摘要

威尔基综合征是一种罕见的临床病症,其特征为十二指肠部分或完全梗阻。婴儿肥厚性幽门狭窄(IHPS)的定义是幽门平滑肌纤维增生,这被认为是导致幽门管变窄并最终阻塞胃出口的原因。本文介绍了一名3个月大男婴的病例,该男婴在喂食后呕吐了一个月。主要症状,如餐后呕吐、脱水、碱中毒和体重减轻,指向IHPS疾病。影像学检查也证实了诊断。在手术治疗过程中,还发现了一种名为威尔基综合征的伴随病症。该团队在一次手术中对一名同时被诊断为威尔基综合征和IHPS的患者进行了复杂的手术治疗。一次手术中进行幽门肌切开术和十二指肠-十二指肠吻合术的手术治疗是IHPS和威尔基综合征患者一期治疗的最佳选择。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/7165/12070966/65bbd9a42942/cm-98-257f1.jpg

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