Deep Compartment Syndrome Without Myonecrosis: A Case Report on a Rare Complication of Sickle Cell Disease

Abstract

Compartment syndrome is a rare manifestation of vaso-occlusive crisis, a serious complication of sickle cell disease (SCD), which is an inherited hemoglobinopathy. During a visit to Norway, an otherwise healthy, 20-year-old male from Ghana was admitted to Oslo University Hospital (Day 1) because of increasing pain in the hip and thighs that did not respond adequately to non-opioid painkillers. Despite initial treatment with intravenous fluids and opioids, his pain intensified. Careful clinical inspection supported by an MRI examination revealed focal, high-signal-intensity muscle edema of the anterior compartment of the thigh, almost exclusively limited to the vastus intermedius muscles. There were no MRI findings or blood biochemistry evidence for myonecrosis or rhabdomyolysis, and a diagnosis of deep compartment syndrome appeared to be the most likely explanation for his pain. We decided to continue with a conservative treatment approach, and the patient did not undergo a fasciotomy or blood transfusion therapy. On Day 7 after admission, his condition improved markedly, and he was discharged on Day 11 whereupon he returned to Ghana. This case is a reminder that, although rare, deep compartment syndrome can be a severe manifestation of vaso-occlusive crisis in SCD and should be considered in patients with severe, deep muscular pain in the absence of other explanatory factors.