Hosono Isao, Ogiso Takuya, Matsuba Tomohiko, Takayanagi Masahiro
Department of Gastroenterology, Chutoen General Medical Center.
Nihon Shokakibyo Gakkai Zasshi. 2023;120(8):662-670. doi: 10.11405/nisshoshi.120.662.
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.
埃勒斯-当洛综合征(EDS)是一种相对罕见的综合征,其特征为皮肤和关节过度伸展、出血以及由于胶原蛋白异常导致组织脆性增加。特别是,血管型EDS与富含III型胶原蛋白的主动脉和肠道脆性有关,因此年轻人有自发动脉破裂、动脉瘤、主动脉夹层和胃肠道穿孔的风险。然而,关于EDS引起胃黏膜下血肿的报道很少。在此,我们报告了一例29岁男性病例,他童年时被诊断为EDS,之前一直在另一家医院就诊。他因上腹部疼痛和呕吐前来我院就诊。腹部增强计算机断层扫描(CT)、食管胃十二指肠镜检查(EGD)和内镜超声检查显示,该患者因胃窦黏膜下血肿导致幽门狭窄。他入院后接受了保守治疗,包括禁食、静脉输液和鼻饲。随访CT和EGD显示血肿缩小,此后由于未观察到进一步恶化,他出院了。因此,基于观察到的EDS特征,有必要进行准确诊断以避免不必要的侵入性治疗。