Pediatric Chronic Nonbacterial Osteomyelitis of the Jaw: Clinical, Radiographic, and Histopathologic Features.
OBJECTIVE
Chronic nonbacterial osteomyelitis (CNO) is a focal sterile inflammatory osteitis in children that most commonly develops in the long bones, but can occur in any bone. The disease course is variable, ranging from acute and self-resolving isolated lesions to chronic recurrent multifocal osteomyelitis (CRMO), which is frequently associated with extraosseous inflammatory disease. The purpose of this study was to present our clinical experience with CNO of the mandible in children. The specific aims were to 1) document the clinical characteristics, radiographic findings, and histologic features of CNO and 2) determine the percentage of our sample with multifocal disease (CRMO).


METHODS
This is a retrospective case series of patients with mandibular CNO. To be included, patients had to have a mandibular lesion radiographically consistent with osteomyelitis without infection, onset before aged 18 years, and complete records. Medical records were reviewed for history, clinical features, imaging, and pathology. Descriptive data were summarized.


RESULTS
The sample included 22 patients (13 female and 9 male patients) with disease onset at a mean age of 9.05 ± 2.4 years. On presentation, all patients reported mandibular pain and swelling, and 45% had trismus. All had clinical and/or radiographic findings of multifocal intraosseous disease and/or extraosseous inflammatory lesions. Of the patients, 12 (54%) had a documented family history of autoimmune or autoinflammatory disease and 15 (68%) had elevated erythrocyte sedimentation rates during a flare. Computed tomography scans typically showed expansion of the affected mandible with sclerosis of the medullary space, small foci of poorly defined lytic destruction with a lamellated periosteal reaction, and swollen muscles of mastication. Four distinct histologic features were noted including parallel and interconnected osteoid seams, atypical osteoid, areas of woven bone and hypocellular fibroblastic stroma resembling fibrous dysplasia, and patchy nodular fibrosis.


CONCLUSIONS
Pediatric CNO of the mandible has characteristic radiographic and pathologic features and is usually found as one of multiple disease foci in CRMO rather than as an isolated lesion.
Associate Professor, Harvard School of Dental Medicine, Oral Surgeon-in-Chief Department of Plastic and Oral Surgery, Boston Children's Hospital, Boston, MA. Electronic address: bonnie.padwa@childrens.harvard.edu.
Bonnie L
BL
Padwa
Research Assistant, Department of Plastic and Oral Surgery, Boston Children's Hospital, Boston, MA.
Kelley
K
Dentino
Associate Professor, Harvard Medical School, Division Chief, Neuroradiology, Department of Radiology, Boston Children's Hospital, Boston, MA.
Caroline D
CD
Robson
Associate Professor, Harvard School of Dental Medicine, Division of Oral Medicine and Dentistry, Brigham and Women's Hospital, Boston, MA.
Sook Bin
SB
Woo
Assistant Professor, Cummings School or Medicine, University of Calgary, Departments of Pathology and Medical Genetics, Alberta Children's Hospital & Research Institute, Boston, MA.
Kyle
K
Kurek
Instructor, Harvard School of Dental Medicine, Department of Plastic and Oral Surgery, Boston Children's Hospital, Boston, MA.
Cory M
CM
Resnick
D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D002908:Chronic Disease; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D008297:Male; D008336:Mandibular Diseases / Q000175:diagnosis* / Q000000981:diagnostic imaging / Q000473:pathology; D010019:Osteomyelitis / Q000175:diagnosis* / Q000000981:diagnostic imaging / Q000473:pathology; D012189:Retrospective Studies