Acute Compartment Syndrome in Congenital and Acquired Haemophilia

Acute compartment syndrome (ACS) is a surgical emergency defined as an increase in intramuscular pressure within a compartment that compromises capillary perfusion and threatens the survival of compartment tissues. A sustained increase in compartment pressure causes irreversible necrosis of nerves and muscles, leading to permanent damage.

To conduct a narrative review of the literature on the diagnosis and treatment of ACS in persons with congenital and acquired haemophilia (PwH) METHODS: An English-language literature search for articles published before 28 February 2024 was performed in PubMed (MEDLINE) and the Cochrane Library using "haemophilia and compartment syndrome" as keywords.

We found 77 articles in PubMed (of which 19 were eliminated as not strictly related to the topic) and 1 in the Cochrane Library (which was repeated in PubMed). In total, there are 83 published cases (61 of congenital haemophilia and 22 of acquired haemophilia).

Misdiagnosis or failure to perform early haematological treatment in PwH suffering from ACS, followed by extensive fasciotomy if haematological treatment does not stop the problem, may result in permanent disability of the affected limb. The prognosis will depend on the intensity and duration of the compartment pressure elevation. For PwH, adequate clotting factor replacement should be the first step of treatment, as it may be effective in some cases of ACS. In general, factor levels of 50%-100% should be maintained in the perioperative period; thereafter, the dose can be gradually reduced to maintain levels at 50% for approximately 2 weeks or longer if necessary.