Orthopaedic Manifestations of Sickle Cell Disease

Abstract

Sickle cell disease (SCD) is an autosomal recessive disorder that results in hemolytic anemia related to abnormal hemoglobin and erythrocyte levels. SCD is characterized by vascular occlusive episodes, visceral sequestration, and aplastic or hemolytic crises. These crises most commonly occur in bone. The orthopaedic manifestations of SCD comprise much of the morbidity associated with this disorder. Osteonecrosis and osteomyelitis are among the most disabling and serious musculoskeletal complications in patients with SCD. Effective management of the bone and joint sequelae requires an accurate diagnosis, an understanding of the pathophysiology of the disease, and knowledge of available medical and surgical treatment alternatives. The major orthopaedic manifestations of SCD are osteonecrosis, osteomyelitis, septic arthritis, and bone infarction. Patients with SCD require close monitoring in the perioperative period because of the risk for vasoocclusive crisis.

Perioperative care of children with sickle cell disease: A systematic review and clinical recommendations

“…The use of preoperative transfusion is a source of significant controversy. Case‐by‐case decision‐making is the standard of care in the majority of reports . Prospective clinical studies on this question are presented in Table (all primary research in Table S2).…”

“…Intraoperative monitoring varies. Per standard anesthetic care, blood pressure, cardiac rhythm and rate and oxygenation are monitored strictly to avoid hypoxia . Some groups perform more invasive systematic monitoring such as the continuous measurement of central venous pressure, arterial blood pressure, regular arterial blood gas exchange and/or urine output determination (by Foley catheterization) .…”

“…The central goal during surgery is to avoid any triggering factor for erythrocyte sickling leading to vaso‐occlusion and hemolysis. Strategies include avoiding vascular stasis with proper positioning, and swiftly reversing hypotension . Vasoconstricting amines or other similar drugs, which are theoretically associated with a risk of peripheral vascular stasis, should if possible not be among the first line approaches to hypotension according to many .…”

“…Vasoconstricting amines or other similar drugs, which are theoretically associated with a risk of peripheral vascular stasis, should if possible not be among the first line approaches to hypotension according to many . Hydration to avoid hyperviscosity and dehydration is considered essential . Hypothermia is not tolerated .…”

“…One review suggests that hypothermia remains possibly safe for cardiac and neurosurgical procedures . Proactive body warming strategies, including higher operating room temperature, coverage with warm blankets, exposure to warming lights and use of warm fluids have been reported . Oxygenation has to be strictly controlled and hypoxia needs to be avoided.…”