Vaso-occlusive crisis in the cervicofacial region in sickle cell anemia

Sickle cell anemia (SCA) is an autosomal recessive disorder characterized by a point mutation in the β-globin chain of the hemoglobin protein, causing abnormal folding. This can lead to sickling of red blood cells, agglutination, and ultimately vascular obstruction, which rarely occurs in the cervicofacial region.

This report reviews a rare case of vaso-occlusive crisis (VOC) in the cervical vertebrae and mandible, manifesting as severe mandibular pain of unknown origin in a patient with SCA. The patient experienced diffuse body pain, 3 days in duration, and most prominent in the right hemimandible. Symptoms were refractory to hydration and analgesics, requiring intensive care unit admission. Jaw pain persisted, and magnetic resonance imaging of the face was significant for enhancement in the right posterior hemimandible with surrounding soft-tissue inflammatory changes. Differential diagnoses included osteonecrosis, osteomyelitis, odontogenic infection, extramedullary hematopoiesis, and VOC. The patient lacked signs and sequelae of infection or hematopoiesis; therefore, the pain was attributed to bone infarcts secondary to VOC. The patient was placed on a multimodal analgesic regimen with resolution of symptoms and was discharged in stable condition.

This case illustrates an unusual manifestation of severe jaw pain of unknown origin that was determined to be caused by VOC of the mandible. This highlights the need for dentists to understand SCA pathophysiology and their integral role in the diagnosis of atypical facial pain in patients with SCA. Dentists may be the first to recognize manifestations of SCA, emphasizing their role in patient education and interdisciplinary care.