Osteonecrosis of mandible secondary to sickle cell crisis

Background Sickle cell disease (SCD) is an autosomal recessive hemoglobinopathy causing amino acid substitution in the B-hemoglobin chain of erythrocytes. SCD affects 1 in 500 African American children. SCD is associated with multiorgan morbidity and significant reduction in life expectancy. Vaso-occlusive crisis associated with SCD is characterized by acute pain and local tissue hypoxia and necrosis. The extremities, back, and chest are frequently affected, and jaw involvement is very rare. To the best of our knowledge, only 3 cases of jaw osteonecrosis secondary to SCD have been reported in the English-language literature. Case Summary A 38-year-old African American man with a history of SCD and avascular necrosis of the hips presented to an emergency department with severe pain in his back, legs, and chest due to a vaso-occlusive episode precipitated by physical overexertion. A few days later, the patient complained of left jaw pain and paresthesia. Computed tomography (CT) of the head with intravenous (IV) contrast, maxillofacial CT with IV contrast, and the remainder of the neurologic examinations were unremarkable. The oral medicine service was consulted, and physical examination of the patient demonstrated lingual gingival tissue swelling of the lower left molars. The mass was nonfluctuant with ulceration causing malalignment of tooth number 18. Bone biopsy revealed squamous mucosa with hemorrhagic foci, ulceration, and ectatic blood vessels with fragments of necrotic bone consistent with osteonecrosis. At the patient's 2-week follow-up visit, a small area of exposed necrotic bone was noted in the affected area. Maxillofacial CT without IV contrast showed mixed radiolucency and sclerosis with dehiscence along the lingual cortex of the posterior mandibular body and along the cortex of the retromolar trigone. Chlorhexidine gluconate 0.12% oral solution was prescribed, and debridement of the left posterior mandible and extraction of the lower left molars with local flap reconstruction were recommended by an oral and maxillofacial surgeon. The patient did not return for follow-up management. Conclusions Acute pain and chronic vasculopathy are significant complications of SCD. Jaw involvement in SCD is very rare, as illustrated in this case. It is important for oral health care professionals to understand the pathophysiology and clinical manifestations of SCD.