Acute compartment syndrome in haemophilia

Acute compartment syndrome (ACS) is characterized by an increase in pressure (intramuscular pressure) within a muscle compartment, which reduces capillary perfusion threatening tissue survival. Persistence of this increased pressure for a few hours will result in necrosis of muscle and nerve tissue, with contracture in the affected limb and permanent loss of function. For that reason, early treatment and diagnosis of ACS is fundamental. Diagnosis should be based on physical examination (pain on stretching the involved muscles) and on an objective measurement of the limb perfusion pressure (DBP minus intramuscular pressure) within the affected compartment. To obtain a reliable clinical diagnosis, the patient must be evaluated every 1-2  h. In children and in unconscious patients, where the level of pain cannot be appropriately determined, an accurate clinical diagnosis is unfeasible, hence the importance of measuring compartment pressure. A fasciotomy should be performed when the limb perfusion pressure is less than 30  mmHg when averaged over a 12-h period (monitored every 1-2  h). Only 16 studies have been published on haemophilic patients with ACS, which report on a total of 34 cases. If symptoms or pressure measurements are suggestive of ACS, an extensive fasciotomy will be required. Unfortunately, fasciotomy is not exempt from complications such as the need of subsequent surgery because of a delay in wound healing, the need of a skin graft, pain, cosmetic problems, nerve injury, permanent muscle weakness and chronic venous insufficiency. Overlooked compartment syndrome remains one of most common causes of malpractice lawsuits. In haemophilia, adequate substitution of coagulation factor must be the first step. The main principle of surgical treatment is an extensive fasciotomy.