Background/Purpose:  Chronic nonbacterial osteomyelitis (CNO) of the mandible is a rare osseous autoinflammatory disease. Diagnosis is based on characteristic clinical, laboratory and imaging features. Our aim was to describe the phenotype and response to treatment in children diagnosed at our center.

Methods:  We conducted a retrospective chart review of the patients diagnosed and followed for at least 6 months from 1988-2012. Parameters recorded at the last 2 visits (within 6 months interval): pain, abnormal ESR/CRP, imaging features, including bone and/or soft tissue edema, bone enhancement, ongoing medication and/or surgery. For each of these parameters, an average score was calculated based on the presence (1) or absence (0) of that feature; 0.5=presence at 1 visit, absent/unknown at the 2^nd visit. Mean and SEM were reported. Statistical comparison between 2 groups was performed with t-test. P<0.05 was considered statistically significant

Results: 11 patients (8F:3M) with CNO of the mandible were reviewed (Table). Mean age at diagnosis was 8.4 years (3-11 yrs.); follow-up, 67±13 months (9-155 mos). 6/11 (55%) children were followed primarily/exclusively by rheumatologists and 5, by dental surgeons. 9/11 (82%) had isolated CNO of the mandible; the last 2, each had 1 additional femoral lesion. 3/11 children (27%) had other immune manifestations: insulin resistance and antibodies against insulin receptors, autoimmune neutropenia and bilateral granulomatous uveitis. Mandibular biopsies were performed in 10/11 patients (91%); all were consistent with CNO. Blood and bone cultures were negative in all patients tested. All patients were prescribed antibiotics. 5/11 (45%) children underwent surgical interventions. 6/6 patients followed by rheumatologists received NSAIDs; 1/6 (17%), also received biphosphonates. No patient was treated with steroids or biological therapy. The patients followed by surgeons had pain at the last 2 visits (0.62±0.13), vs those followed by rheumatologists (0) (P=0.02).

Conclusion: Pain at the last 2 follow-ups is common in patients that received only operative treatment. A major shift in the therapeutic approach of CNO of the mandible occurred in the last two decades, from exclusive surgical interventions to anti-inflammatory therapy and rheumatologic follow-up. CNO of the mandible can be associated with other immune manifestations.

	Demographics			Treatment		Last 2 visits
Pt #	Gender	Age at dx (years)	FU (ms)	Medication	# of surgeries	Pain	Abnormal ESR/CRP	Radioogical signs of inflam-mation	Treatment	Type of treatment	Primary specialist
1	F	11	28	0	0	1	uk	uk	0	None	Dentist
2	F	10	144	Naproxen prn	5	1	uk	uk	0.5	Surgery; None	Dentist
3	F	6	86	0	2	0.5	uk	0.5	0.5	Surgery; None	Dentist
4	F	10	123	0	4	0.5	uk	uk	0	Surgery; None	Dentist
5	M	5	9	0	1	uk	uk	uk	0.5	Surgery; None	Dentist
6	M	13	73	Naproxen	4	0	0.5	0.5	1	NSAID	Rheum
7	F	10	24	Indocid	0	0	uk	uk	0	None	Rheum
8	F	10	58	Naproxen	0	0	1	0	1	NSAID	Rheum
9	M	3	39	Naproxen	0	0	1	uk	0	None	Rheum
10	F	5	95	Indocid;Pamidronate	0	0	0	0.5	1	NSAID	Rheum
11	F	10	55	Naproxen, then Indocid	0	0	1	uk	0	None	Rheum

 Table. Demographic, clinical, laboratory, imaging features and treatment approaches in pediatric CNO of the mandible. FU=follow-up; dx=diagnosis; uk=unknown.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/chronic-nonbacterial-osteomyelitis-of-the-mandible-in-children-a-tertiary-center-experience/