Hamaoui K, Riaz A, Hay A, Botha A
Department of Renal and Transplant Medicine, Hammersmith Campus, Imperial College London, London, UK.
Ann R Coll Surg Engl. 2012 Jan;94(1):e5-7. doi: 10.1308/003588412X13171221499306.
Ehlers-Danlos syndrome (EDS) is a rare disorder caused by abnormalities in the synthesis and structure of collagen. The resultant tissue fragility and weakness can lead to multiple surgical conditions. In this report we present the very rare and life threatening case of a massive spontaneous diaphragmatic rupture in a 35-year-old man with EDS and reflect on the literature, highlighting points to consider when managing such complex patients.
埃勒斯-当洛综合征(EDS)是一种由胶原蛋白合成和结构异常引起的罕见疾病。由此导致的组织脆弱和虚弱会引发多种外科疾病。在本报告中,我们介绍了一名35岁患有EDS的男性发生巨大自发性膈肌破裂这一极其罕见且危及生命的病例,并对相关文献进行思考,强调在处理此类复杂患者时需要考虑的要点。