Multifocal recurrent atraumatic acute compartment syndrome in an adolescent

Recurrent, atraumatic acute compartment syndrome (ACS) is an uncommon and poorly understood phenomenon. We report a female in her teens who experienced two distinct episodes of atraumatic ACS. The first episode involved the right lower extremity, and the second, the right upper extremity. Each case required emergent fasciotomy and involved postoperative complications, requiring repeat surgical intervention and vacuum-assisted closure. Extensive multidisciplinary evaluation, including rheumatologic, hematologic, and genetic testing, failed to identify an etiology. Given the recurrence and localization to separate limbs, a focal vascular abnormality is suspected, supported by comparison with a similar reported case successfully treated with angiographic embolization. This case underscores the importance of prompt surgical intervention and multidisciplinary evaluation and highlights the potential role of vascular malformations in cases of unexplained compartment syndrome.