关键词: connective tissue disorders nasogastric tube complication small bowel perforation small bowel resection vascular ehlers danlos syndrome

来  源:   DOI:10.7759/cureus.60063   PDF(Pubmed)

Abstract:
Ehlers-Danlos Syndrome (EDS) is a rare connective tissue disorder characterized by mutation in genes that encode or modify collagen. Clinical findings in these patients include skin hyperextensibility, hypermobility of joints, and tissue fragility. Vascular EDS (vEDS) is an autosomal dominant disease typically caused by a mutation in COL3A1, which encodes type III collagen. Presenting signs in the majority of vEDS patients include arterial rupture, uterine rupture, and sigmoid colon perforation. In this case report, the author presents an unusual case of spontaneous small bowel perforation and the creation of a traumatic false passage in the parapharyngeal space during a complicated nasogastric tube insertion in a patient with vEDS.
摘要:
Ehlers-Danlos综合征(EDS)是一种罕见的结缔组织疾病,其特征是编码或修饰胶原蛋白的基因突变。这些患者的临床表现包括皮肤过度扩张,关节过度活动,组织脆弱.血管EDS(vEDS)是一种常染色体显性疾病,通常由编码III型胶原蛋白的COL3A1突变引起。大多数vEDS患者的体征包括动脉破裂,子宫破裂,乙状结肠穿孔。在这个案例报告中,作者介绍了一例罕见的自发性小肠穿孔病例,并在vEDS患者的复杂鼻胃管插入过程中在咽旁间隙产生了创伤性假通道。
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