SURGICAL PRESENTATIONS OF EHLERS-DANLOS SYNDROME TYPE-IV - A CASE-REPORT

The case of a 28 year old man with Ehlers-Danlos syndrome (EDS) type I V is presented, He had the typical facies of this disorder, thin skin with atrophic scars and bruised easily. He suffered with congenital cl ubbed feet, heavy blood loss and poor wound healing following a trauma tic compound fracture of the right leg, recurrent spontaneous pneumoth oraces for which a pleurectomy was performed, two episodes of vascular rutpure following minor trauma, one managed conservatively and the ot her surgically, and one episode of massive spontaneous vascular ruptur e which was fatal. In this report we highlight the surgical nature of the presenting complications of EDS type IV and demonstrate the hazard s of surgical management. We conclude that, when possible, conservativ e management is preferable.