Abstract
BACKGROUND: Acute compartment syndrome (ACS) is an uncommon but potentially devastating condition. METHODS AND RESULTS: There are scattered case reports and case series in the literature of ACS in persons with haemophilia (PWH), and even fewer in PWH and inhibitors. The management of compartment syndrome in these scenarios is controversial and often anecdotal. In addition haematological outcomes are frequently quoted but functional outcomes are generally overlooked. This article aims to provide an overview of ACS and its contemporary management. We also review the literature and outcomes of patients with haemophilia who develop ACS in an effort to assess the best treatment modality. CONCLUSION: In the majority of cases ACS settles with normalisation of the clotting cascade. Specialist haematological input is mandatory before surgical intervention should be considered, especially in PWH and inhibitors.