Brady, Kathryn

Butler, Kelci

TeKippe, Michael

SHARE @ Advocate Health - Midwest

Mandibular Mayhem: An Uncommon Cause of Facial Swelling Kelci Butler, DO; Kathryn Brady, DO; Michael TeKippe, MD, PhDAdvocate Children’s Hospital, Advocate Health Care, Oak Lawn, ILBackgroundFacial swelling is a common pediatric complaint and is often due to infectious causes, such as lymphadenitis, parotitis, sinusitis, or dental infection. PurposeWhen antibiotics fail and symptoms become subacute/chronic, further work up is indicated to evaluate for other disease processes such as oncologic or autoimmune. The purpose of this case is to highlight a unique presentation of facial swelling non-responsive to antibiotic therapy.Case DescriptionHistory of Presenting Illness A previously healthy 8-year-old female presented with a three-week history of progressive right-sided facial swelling and tenderness, which failed to respond to oral antibiotic therapy.Physical ExamOn exam, she had right cheek swelling with focal tenderness over the ramus of the mandible (Figure 1). There was no overlying induration, warmth, or erythema. She had normal dentition without caries.Figure 1. 8-year-old female who presented with progressive, right-sided facial swelling and focal tenderness over the ramus of the mandible. Diagnostic EvaluationLab EvaluationHer laboratory evaluation was notable for an elevated ESR with normal CBC, and negative CRP, procalcitonin, and blood culture. Imaging Studies CT neck demonstrated no evidence of abscess but did show periosteal reaction at the right mandibular angle with a 9mm osseous erosion, suggestive of osteomyelitis (Figure 2). MRI of the face confirmed mandibular osteomyelitis. Given her reassuring laboratory workup and lack of infectious source, concern for nonbacterial osteomyelitis was raised. A full body MRI was obtained to evaluate for other potential sites of involvement, but none were identified. Procedural EvaluationA bone biopsy was obtained, and pathology revealed inflammatory changes without histologic evidence of an infectious or neoplastic process. References1. Hofmann SR, Kapplusch F, Girschick HJ, Morbach H, Pablik J, Ferguson PJ, Hedrich CM. Chronic Recurrent Multifocal Osteomyelitis (CRMO): Presentation, Pathogenesis, and Treatment. Curr Osteoporos Rep. 2017 Dec;15(6):542-554. doi: 10.1007/s11914-017-0405-9. PMID: 29080202; PMCID: PMC5705736.2. Zhao DY, McCann L, Hahn G, Hedrich CM. Chronic nonbacterial osteomyelitis (CNO) and chronic recurrent multifocal osteomyelitis (CRMO). J Transl Autoimmun. 2021 Mar 20;4:100095. doi: 10.1016/j.jtauto.2021.100095. PMID: 33870159; PMCID: PMC8040271.OutcomesDiagnostic CriteriaThis case was most suspicious for Chronic Nonbacterial Osteomyelitis (CNO) versus Chronic Recurrent Multifocal Osteomyelitis (CRMO). Our patient meets both published diagnostic criteria (Jansson and Bristol criteria) for diagnosis of CNO/CRMO. Follow UpOur patient has been followed by both Pediatric Infectious Diseases and Pediatric Rheumatology. She continued to fail to respond to two different antibiotics. Stopping antibiotics while continuing scheduled NSAIDs has led to improvement in swelling and tenderness. Discussion/ConclusionChronic Nonbacterial Osteomyelitis (CNO) and Chronic Recurrent Multifocal Osteomyelitis (CRMO) are autoinflammatory bone disorders that present with insidious, recurrent bone pain in one or multiple sites. Distinguishing between bacterial infection and CNO/CRMO: involvement of bones not typically associated with bacterial osteomyelitis such as the clavicle, mandible, and vertebrae can be suggestive of CNO/CRMO. In this patient, normal dentition strongly suggested a nonbacterial process, as bacterial infection of the mandible usually spreads from caries/bacterial infection of the teeth.DiagnosisWhole body MRI is the gold standard for diagnosis and follow-up for CNO/CRMO due to the frequency of multi-focal involvement. Bone biopsy is often required to exclude other disease processes such as infection or malignancy. Histopathology typically shows nonspecific inflammation similar to infectious osteomyelitis; however, cultures are negative and bacteria are not found.ManagementWhile CNO/CRMO can mimic bacterial osteomyelitis, the first line of treatment is NSAIDs rather than antibiotics. OutcomesThe diagnosis of CNO/CRMO is often delayed, however early recognition and diagnosis is crucial for prevention of complications such as fractures and management of chronic, recurrent pain, as up to 50% or more of patients develop clinically significant flares.Figure 2. CT neck with evidence of osteomyelitis of the right mandibular ramus and angle with periosteal reaction and associated myositis of the adjacent muscles of mastication. 

Mandibular Mayhem: An Uncommon Cause of Facial Swelling

https://institutionalrepository.aah.org/cgi/viewcontent.cgi?article=2097&context=advocategme

Mandibular Mayhem: An Uncommon Cause of Facial Swelling

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