Orthognathic surgery in a patient with sickle cell disease: A case report and review of literature

Sickle cell disease is a genetic hemoglobinopathy that has the potential to affect any organ of the body. Patients with sickle disease have high morbidity and mortality creating safety concerns among patients and physicians about surgery in this subset of population. Factors that predispose SCD patient to complications are divided into patient-related and surgery-related factors. Patient factors associated with increased complication rate include the type and severity of SCD. Orthognathic surgery involves osteotomies, major movement of upper or lower jaw that, although safe, have the potential of list of complications in healthy individuals. Therefore, complications are expected to be higher in SCD population. This complicates the decision of surgery and mandates careful perioperative management. Key points • Sickle cell disease is a genetic hemoglobinopathy that has the potential to affect any organ of the body. Patients with sickle disease have high morbidity and mortality creating safety concerns among patients and physicians about surgery in this subset of population. • SCD is characterized by anemia and four types of crises, namely: hemolysis, sequestration, aplastic and vaso-occlusive crises. The latter is a painful condition that could not be attributed to any diagnosis. VOCs resemble and predispose infarcted tissues to infections (e.g. osteomyelitis). • Factors that predispose SCD patient to complications are divided into patient-related and surgery-related factors. Patient factors associated with increased complication rate include the type and severity of SCD. • As Part of head and neck surgeries, orthognathic surgeries may be considered moderate-risk procedures. Orthognathic surgery is an invasive procedure that involves osteotomies, major movement of upper or lower jaws and the possibility of blood loss have the potential of list of complications in healthy individuals. • The selection of surgical procedures in SCD population may be based on individual variations in disease baseline, oral hygiene and the demand of the surgical procedure. Careful perioperative management with experienced medical team and nursing staff is necessary.