Mesh : Male Humans Adolescent Child Adult Ehlers-Danlos Syndrome, Type IV Ehlers-Danlos Syndrome / complications diagnosis Hematoma / diagnostic imaging etiology Pyloric Stenosis / complications

来  源:   DOI:10.11405/nisshoshi.120.662

Abstract:
Ehlers-Danlos syndrome (EDS) is a relatively rare syndrome characterized by excessive stretching of the skin and joints and hemorrhage and increased tissue fragility due to abnormal collagen. Particularly, vascular type EDS has been associated with type III collagen-rich aorta and intestinal tract fragility, owing to which young people are at a risk of spontaneous arterial rupture, aneurysm, aortic dissection, and gastrointestinal perforation. However, there have been few reports regarding gastric submucosal hematoma caused by EDS. Herein, we presented the case of a 29-year-old man who was diagnosed with EDS during his childhood and used to attend another hospital. He visited our hospital after developing epigastralgia and vomiting. The patient presented with pyloric stenosis caused by gastric antrum submucosal hematoma as revealed via abdominal contrast-enhanced computed tomography (CT), esophagogastroduodenoscopy (EGD), and endoscopic ultrasonography. He was admitted and underwent conservative therapy in addition to fasting, drip infusion, and tube feeding. The follow-up CT and EGD revealed hematoma shrinkage, after which he was discharged as no further deterioration was observed. Thus, based on the observed EDS characteristics, an accurate diagnosis is warranted to avoid unnecessary invasive therapy.
摘要:
Ehlers-Danlos综合征(EDS)是一种相对罕见的综合征,其特征是皮肤和关节过度拉伸,出血以及由于胶原蛋白异常而导致的组织脆性增加。特别是,血管型EDS与富含III型胶原的主动脉和肠道脆性有关,因此,年轻人有自发性动脉破裂的风险,动脉瘤,主动脉夹层,和胃肠道穿孔。然而,关于EDS引起的胃粘膜下血肿的报道很少。在这里,我们介绍了一例29岁男性,他在童年时期被诊断患有EDS,过去曾在另一家医院就诊.他在出现胃痛和呕吐后去了我们医院。通过腹部对比增强计算机断层扫描(CT)显示,患者表现为由胃窦粘膜下血肿引起的幽门狭窄,食管胃十二指肠镜检查(EGD),和超声内镜.他入院并接受了保守治疗,除了禁食,滴注,管喂食。随访CT及EGD显示血肿缩小,此后,由于没有观察到进一步的恶化,他出院了。因此,根据观察到的EDS特征,准确的诊断是必要的,以避免不必要的侵入性治疗.
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