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 IV is presented. He had the typical facies of this disorder, thin skin with atrophic scars and bruised easily. He suffered with congenital clubbed feet, heavy blood loss and poor wound healing following a traumatic compound fracture of the right leg, recurrent spontaneous pneumothoraces for which a pleurectomy was performed, two episodes of vascular rupture following minor trauma, one managed conservatively and the other surgically, and one episode of massive spontaneous vascular rupture which was fatal. In this report we highlight the surgical nature of the presenting complications of EDS type IV and demonstrate the hazards of surgical management. We conclude that, when possible, conservative management is preferable.
Whinney
D
D
Department of Surgery, Weston General Hospital, Weston-super-Mare, Avon, UK.
Nicholson
S
S
Ridley
P
P
D000328:Adult; D016063:Blood Loss, Surgical; D001803:Blood Transfusion; D001808:Blood Vessels / Q000293:injuries; D004535:Ehlers-Danlos Syndrome / Q000150:complications*; D017809:Fatal Outcome; D006406:Hematoma / Q000601:surgery; D006470:Hemorrhage / Q000209:etiology / Q000401:mortality*; D006801:Humans; D007431:Intraoperative Complications / Q000209:etiology; D008297:Male; D010994:Pleura / Q000601:surgery; D011030:Pneumothorax / Q000601:surgery; D011183:Postoperative Complications / Q000209:etiology / Q000401:mortality*; D013908:Thoracotomy