Recurrent massive hematoma after trivial trauma in Ehlers-Danlos syndrome: a case report highlighting diagnostic urgency and surgical challenges
Abstract
Musculocontractural Ehlers-Danlos syndrome (mcEDS) is a rare connective tissue disorder characterized by abnormal collagen structure, tissue fragility, and predisposition to disproportionate hematoma formation following trivial trauma. Large expanding hematomas may lead to tissue necrosis, thereby requiring urgent decision-making and rapid intervention. We report a case of a 10-year-old boy with mcEDS and Dandy-Walker syndrome who presented after a minor fall with rapidly progressive swelling of the right lower limb and a history of recurrent post-traumatic hematomas. Clinical deterioration prompted urgent exploration, revealing a large, organized hematoma, and friable devitalized tissues. Extensive debridement, staged wound care, and split-thickness skin grafting were performed. Microbiology was negative, confirming a traumatic hematoma with secondary soft-tissue compromise related to the connective tissue disorder. Minor trauma in patients with mcEDS may cause severe limb-threatening hematomas with tissue necrosis. Early recognition and prompt surgical intervention are essential to prevent ischemic injury and improve reconstructive outcomes.
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