Normatov Inessa, Kesavan Anil, Srikumar Pillai B, McConnie Randolph M
Department of Pediatrics, Rush University Medical Center, Chicago, IL.
Department of Pediatric Gastroenterology, Hepatology and Nutrition, Rush University Medical Center, Chicago, IL.
ACG Case Rep J. 2016 Jan 20;3(2):104-6. doi: 10.14309/crj.2016.14. eCollection 2016 Jan.
The Ehlers-Danlos syndromes (EDS) are a group of connective tissue disorders characterized by triad of joint hypermobility, skin extensibility, and tissue fragility. Ehlers-Danlos syndrome type IV places patients at risk for life-threatening, spontaneous, vascular or visceral rupture due to reduced or abnormal secretion of type III collagen. We present an adolescent male who was found to have a perisigmoid abscess with a fistula connecting to adjacent sigmoid colon secondary to undiagnosed EDS type IV. Conservative management with antibiotics and bowel rest was pursued to allow for elective resection for his acute complicated diverticulitis at a safer time.
埃勒斯-当洛综合征（EDS）是一组结缔组织疾病，其特征为关节活动过度、皮肤延展性和组织脆弱三联征。IV型埃勒斯-当洛综合征患者由于III型胶原蛋白分泌减少或异常，面临危及生命的自发性血管或内脏破裂风险。我们报告一名青少年男性，他因未被诊断出的IV型EDS继发乙状结肠周围脓肿并伴有与相邻乙状结肠相连的瘘管。采取了抗生素和肠道休息的保守治疗方法，以便在更安全的时间对其急性复杂性憩室炎进行择期切除。
