Abstract
Background: While trauma to the upper extremity is known to cause acute compartment syndrome (ACS), nontraumatic causes of ACS of the upper extremity are rare. Nontraumatic ACS of the upper extremity can lead to adverse outcomes if not recognized early. There are limited reports of spontaneous ACS published in the literature. The aim of this comprehensive systematic review is to increase awareness among plastic surgeons and hand surgeons of this acute event and provide an algorithmic approach to management in the acute setting through an illustrative case example. Methods: A comprehensive systematic review of published literature was conducted in the Medline/PubMed database with the search terms, "compartment syndrome," "extremity," "spontaneous," "nontraumatic," and "atraumatic" without timeframe limitations. Articles were identified and included in this review based on ACS localization in the upper extremity and etiology of nontraumatic, spontaneous origin. Results: Sixteen publications and 19 total cases of nontraumatic ACS of the upper extremity from 1993 to 2016 met our search criteria. A bleeding disorder was the etiology in three cases, systemic anticoagulation in three cases, infection in six cases, and unknown in three cases. The remaining four cases included systemic sclerosis, Ehlers-Danlos syndrome, rhabdomyolysis, and McArdle disease. Conclusions: Nontraumatic causes of ACS of the upper extremity include infection, anticoagulation therapy, and bleeding disorders. Even though trauma is the most common cause of ACS, clinicians should be aware of these other potential causes of ACS in the nontraumatic setting. Appropriate medical and surgical intervention should be done to avoid potential adverse outcomes.