Rare mandibular condition presenting as trismus: an exploration of IgG4-related disease.

A woman in her 30s presented with a 12-month history of reduced mouth opening and swelling on the right side of her mandible. The swelling was non-tender and firm on palpation. The swelling began to increase in size after the extraction of her carious wisdom tooth. Histopathological and serological examinations confirmed the diagnosis of IgG4-related disease, manifested as a mass in the mandible. The patient was prescribed oral corticosteroids at a tapering dosage over 8 weeks. After 3 months, there was an improvement in the patient's mouth opening and a reduction in the size of the swelling. The patient remains in follow-up care. Including IgG4-related disease in the list of potential diagnoses for oral soft tissue masses is crucial, given their positive response to medical treatment, highlighting the significance of an accurate diagnosis to prevent unnecessary surgery, with oral lesions potentially serving as early indicators before multiorgan complications arise.

Chronic Diffuse Sclerosing Osteomyelitis of the Mandible: The Use of Bisphosphonates as a Treatment for a Rare and Challenging Condition.

Chronic diffuse sclerosing osteomyelitis is a very rare condition, described as a non-suppurative, inflammatory disease of the bone and characterized by a proliferative endosteal reaction, which clinically reveals itself with cyclic pain of the jaw and swelling. We reported two clinical cases, where patients suffered recurrent swelling and pain at the mandible irradiating to the preauricular area, denying any previous trauma or significant medical history. Odontogenic causes were excluded. An initial treatment with antibiotics and NSAIDs temporarily relieved the symptoms without complete resolution, prompting further investigations. After a comprehensive array of diagnostic tools (X-rays, CT scans, scintigraphy, bone biopsy, serum markers), both patients were diagnosed with chronic diffuse sclerosing osteomyelitis of the mandible. Bisphosphonates (clodronate and zolendronate) with different treatment schemes were used to treat the condition, until a full recovery from symptoms was reported. Bisphosphonates could therefore represent an effective option in managing this rare but impactful condition. Further research is warranted to better understand the underlying mechanisms of the disease and to optimize treatment strategies.

Collaborative approach to paediatric chronic non-bacterial osteomyelitis of the mandible: Great Ormond Street Hospital case series.

This paper outlines a 10-patient case series of chronic non-bacterial osteomyelitis (CNO) of the mandible at a tertiary paediatric hospital in the UK. Our findings highlight the homogeneous presenting signs and symptoms of an intermittently painful, swollen angle and ramus of the mandible. We present the typical laboratory investigative findings (normal inflammatory markers) and imaging appearances (sclerosis and periosteal oedema). Our paper outlines an investigation protocol, including recommendations for extraoral bone biopsies and systemic magnetic resonance imaging (MRI). We explain the importance of multidisciplinary care, with combined care by rheumatologists and infectious disease specialists. Finally we demonstrate the efficacy of our treatment algorithm for oral non-steroidal anti-inflammatory drugs (NSAIDs), and in those cases refractory to NSAIDS, intravenous pamidronate. This paper provides a useful addition to the literature by informing OMF surgeons of this rare condition and given the clinical equipoise in treatments, it can hopefully guide clinicians in an investigation pathway and management protocol.

Osteoradionecrosis in a Torus Mandibularis Treated by Antimicrobial Photodynamic Therapy: A Case Report.

Background: Osteoradionecrosis (ORN) of the jaws is a late complication after radiotherapy to head and neck cancer. Objective: To describe a rare case of ORN of the torus mandibularis that was successfully managed exclusively with antimicrobial photodynamic therapy (aPDT). Case report: A 72-year-old man presented an exposed necrotic bone observed in the torus mandibularis, extending to the lingual alveolar ridge with no edema nor suppuration. The treatment provided a noninvasive treatment leading to spontaneous sequestrectomy of the torus in 2 weeks with complete mucosal repair in 5 weeks and absence of lesion signs and/or symptoms even after 6 months of follow-up. Conclusions: The aPDT indicated to be a satisfactory treatment for ORN affecting torus mandibularis, a region with surgical limitations, avoiding surgery.

Actinomyces-induced Osteomyelitis of the Mandible - A Rare Disease?

BACKGROUND: Actinomyces species are commensal oral cavity flora that can cause jaw osteomyelitis. Osteomyelitis of the jaw by Actinomyces is rare, and its presentation can be confused with many different pathologies. CASE PRESENTATION: This is the case of a 61-year-old female with breast cancer and on chemotherapy as well as non-invasive carcinoma of the tongue who initially presented to the dentist with white spots in the right mandible near the incisors associated with right mandible pain and swelling. Actinomyces-induced osteomyelitis of the mandible was diagnosed. The patient was treated with penicillin V for 6 weeks along with a course of hyperbaric oxygen therapy, which resulted in the complete resolution of the infection. CONCLUSION: In summary, jaw osteomyelitis caused by Actinomyces should always be part of the differential diagnosis; as these organisms are commensal flora. The symptoms manifested are non-specific, and such a diagnosis could be easily missed, resulting in delay of care and disease progression.

Mandibular Osteoradionecrosis: Defining the Microbial Milieu and Antimicrobial Resistance at the Time of Rescue Flap Surgery.

OBJECTIVE: To report the microorganisms and their antibiotic sensitivity profile from tissue cultures and stains at the time of anterolateral thigh fascia lata (ALTFL) rescue flap for management of mandibular osteoradionecrosis (ORN). METHODS: Retrospective chart review of patients who underwent ALTFL rescue flap for native mandibular ORN between 2011 and 2022. RESULTS: Twenty-six cases comprising 24 patients (mean age 65.4 years, 65.4% male) with mandibular ORN from whom tissue cultures and gram stain were obtained at the time of ALTFL rescue flap. 57.7% grew bacterial species, while 34.6% grew fungal species. Multibacterial speciation was noted in 26.9% of cultures. A combination of bacterial and fungal growth was also seen in 15.4% of cases. All gram-positive cocci (GPC) were pansensitive to antibiotics except for one case of Staphylococcus aureus, which was resistant to levofloxacin. Gram-negative bacilli (GNB) species were isolated in 50.0% of cases. All fungal growth was due to Candida species. No growth was noted in 23.1% of cases. Multidrug resistance was noted in 53.8% of cases when GNB was isolated. CONCLUSION: We report 76.9% of our cases of mandibular ORN had microbial growth from tissue cultures obtained at the time of the ALTFL rescue flap. Fungal growth was noted in a substantial number of cases and should be obtained as a specimen when pursuing culture-driven antibiotic therapy. Most GPCs were pansensitive to antibiotics, while GNBs were often the harbinger of multidrug resistant mandibular ORN. LEVEL OF EVIDENCE: 4 Laryngoscope, 134:166-169, 2024.

Bony Canal Method of Dexamethasone Injections in Aggressive Form of Central Giant Cell Granuloma-Case Series.

Central Giant Cell Granuloma constitutes approximately 7% of benign tumors of the jaws. The aggressive form of CGCG clinically behaves like a classic semi-malignant neoplasm. In the literature, the suggested method of treatment of aggressive forms of CGCG is curettage or resection with the margin of 0.5 cm. Surgical treatment, especially in the developmental age, entails disturbances in the growth and differentiation of tissues and deforms and disturbs the functioning of the stomatognathic system. Alternative treatment methods of the CGCG presented in this article lead to the patient avoiding a mutilating procedure and improve their quality of life. The aim was to present alternative method of treatment of aggressive forms of Central Giant Cell Lesion of the jaws-injections of dexamethasone into the tumor mass through drilled bony canals. Here, we present the three cases of aggressive forms of CGCG of jaws treated with dexamethasone injections into the tumor mass. Two cases resulted in regression of the tumor, which was confirmed in histologic evaluation after remodeling surgery. Those two patients were uneventful and showed no signs of tumor recurrence at 8 and 9 years of thorough follow-up, respectively. The third patient was qualified for the mandible resection due to the enlargement of the lesion and destruction of the cortical bone. According to our observations, if the proper patient discipline, and thorough, careful clinical and radiological examinations are provided, the dexamethasone injections could be a recommended method of treatment of intraosseous giant cell granuloma. The indication is restricted to the cases with preserved bony borders despite deformation. Additionally, leaving vital teeth in the lesion is also possible.

The W9 peptide inhibits osteoclastogenesis and osteoclast activity by downregulating osteoclast autophagy and promoting osteoclast apoptosis.

The W9 peptide has been shown to act as a receptor activator for nuclear factor-κB ligand (RANKL) antagonist and tumor necrosis factor (TNF)-α antagonist, which can promote bone formation and inhibit bone resorption. Studies on the W9 peptide at the cellular level have mainly focused on osteoblasts, and little research on the mechanism by which the W9 peptide regulates osteoclasts has been reported, which was the aim of this work. In this study, a rat mandibular defect model was established in vivo and implanted with hydrogel containing the W9 peptide for 2 weeks and 4 weeks, and histochemical staining was used to evaluate the formation of new bone and the changes in osteoclasts. RAW264.7 cells were cultured in vitro for osteoclast induction, and different concentrations of W9 peptide were added. Tartrate resistant acid phosphatase staining, monodansylcadaverine staining, TdT-mediated dUTP Nick-End Labeling assay, real-time PCR and Western blot were used to detect osteoclast differentiation, autophagy and apoptosis. Our results showed that the W9 peptide could reduce osteoclastogenesis and osteoclast activity induced by RANKL, and these effects were partly due to the inhibition of osteoclast autophagy. On the other hand, the W9 peptide could promote mature osteoclast apoptosis, in which autophagy might play an antagonistic role. Taken together, these results suggest that the W9 peptide inhibits osteoclastogenesis and osteoclast activity by downregulating osteoclast autophagy and promoting osteoclast apoptosis. Our results will benefit the development and application of new small molecule peptides for the treatment of bone resorption diseases.

Mechanisms of acute hypercalcemia in pediatric patients following the interruption of Denosumab.

PURPOSE: Denosumab is a fully human monoclonal anti-RANK-L antibody that is clinically used to counteract the bone loss induced by exacerbated osteoclast activity. Indeed, its binding to RANK-L prevents the interaction RANK-L/receptor RANK that is essential for osteoclastogenesis and bone resorbing activity. Although there are many medications available to treat bone loss diseases, including bisphosphonates, Denosumab is highly effective since it reduces the bone erosion. The use in pediatric patients is safe. However, some concerns are related to the interruption of the treatment. Indeed, in this study, we reported hypercalcemia in two pediatric patients and alterations of circulating osteoclast precursors. METHODS: Peripheral Blood Mononuclear Cells (PBMC) were isolated from two pediatric patients with hypercalcemia after Denosumab interruption and from 10 controls. Cytofluorimetric analysis and in vitro osteoclastogenesis experiments were performed. RESULTS: Increase of CD16-CD14+CD11b+ cells was revealed in PBMC from patients reflecting the enhanced in vitro osteoclastogenesis. CONCLUSION: Our data suggest that precautions must be taken when Denosumab therapy is interrupted and gradual decrease of dose and/or timing of treatment should be performed. To prevent the onset of hypercalcemia that could be in the discontinuation phase, cytofluorimetric analysis of PBMC should be performed to evaluate osteoclast precursors.

Dilemma in the Treatment of a Central Giant Cell Granuloma.

Management of central giant cell granuloma (CGCG) presents a clinical challenge. While eradicating a lesion known for its high recurrence rate calls for radical surgical approaches, these cause significant esthetic and functional impairment. We present an eight-year-old boy suffering from an extraordinarily large CGCG expanding into the mandible and base of the mouth in the whole anterior region. Combined treatment with surgical intervention and corticosteroid application was successfully applied, and all six attached dental germs could be preserved. Different approaches for clinical management in pediatric cases are discussed.

Etanercept prevents TNF-α mediated mandibular bone loss in FcγRIIb-/- lupus model.

Patients with systemic lupus erythematosus are at increased risk for alveolar bone loss due to periodontitis possibly as a result of a pathogenic immune response to oral bacteria and inflammation. The aim of the present study was to investigate whether an anti-TNF-α antagonist could prevent mandibular bone loss in the FcγRIIb-/- mouse model of lupus. Mice lacking FcγRIIb had decreased cancellous and cortical bone volume at 6 months of age. Etanercept increased cancellous but not cortical bone volume in WT and increased both cancellous bone volume and cortical thickness in FcγRIIb-deficient mice. FcγRIIb deficiency decreased mRNA levels for osteoblast marker genes, Osx, Col1a1 and Alp without any change in osteoclast marker genes. Etanercept increased Osx, Alp, and Ocn in both WT and FcγRIIb-/- mice. Osteoclast marker genes including TNF-α, Trap and RANKL/OPG ratio was decreased in WT. Serum markers of proinflammatory cytokines, TNF-α, IFNγ, IL-6, and IL-17A, were increased in FcγRIIb-/- mice and etanercept antagonized these effects in FcγRIIb-/- mice. Etanercept increased serum PTH levels in the FcγRIIb-/- mouse model of lupus. Our results suggest that deletion of FcγRIIb induces osteopenia by increasing the level of proinflammatory cytokines. Etanercept is effective in preventing mandibular bone loss in FcγRIIb-/- mice, suggesting that anti-TNF-α therapy may be able to ameliorate mandibular bone loss in SLE patients with periodontitis.

Local treatment of experimental mandibular osteomyelitis with an injectable biomimetic gentamicin hydrogel using a new rabbit model.

Mandibular osteomyelitis (OM) is a challenging disease. Our objective was to assess a new OM model in rabbits induced by arsenic trioxide and to assess the efficacy of local treatment of OM using injectable gentamicin-collagen hydrogels (GNT-COLL). OM was induced unilaterally by controlled confinement of arsenic trioxide paste to the root canal of lower incisors of rabbits, while OM progression was characterized for 16 weeks. On the other hand, two injectable COLL hydrogels functionalized with GNT were prepared and characterized for physicochemical properties; a simple GNT-COLL and a nanohydroxyapatite (nHA)- loaded hydrogel (GNT-COLL/nHA). The two hydrogels were evaluated to treat OM model, while a multidose intramuscular GNT solution served as positive control. Outcomes were assessed by standard methods at 4 and 12 weeks post-surgery. The clinical, radiographical, and histopathological findings provided evidence for the validity of the arsenic-induced OM. The results demonstrated that a single intra-lesional injection of the two hydrogels was more suppressive to OM compared to multidose systemic GNT. The composite GNT-COLL/nHA hydrogel proved to induce early preservation of alveolar bone (ridge) length and higher amount of bone area\total area at 4 weeks (40.53% ± 2.34) followed by GNT-COLL (32.21% ± 0.72). On the other hand, the positive control group revealed the least ridge length and bone area\total area (26.22% ± 1.32) at 4 weeks. Both hydrogels successfully arrested OM with no signs of recurrence for up to 12 weeks. Therefore, results support the greater advantages of the composite hydrogel as an osteogenic/antibiotic delivery system in OM treatment.

Treatment of Blepharospasm and Oromandibular Dystonia with Botulinum Toxins.

Blepharospasm and oromandibular dystonia are focal dystonias characterized by involuntary and often patterned, repetitive muscle contractions. There is a long history of medical and surgical therapies, with the current first-line therapy, botulinum neurotoxin (BoNT), becoming standard of care in 1989. This comprehensive review utilized MEDLINE and PubMed and provides an overview of the history of these focal dystonias, BoNT, and the use of toxin to treat them. We present the levels of clinical evidence for each toxin for both, focal dystonias and offer guidance for muscle and site selection as well as dosing.

[Practical experience in treating mandibular osteomyelitis ("lumpy jaw") with gamithromycin in a muntjac]. / Praktische Erfahrungen zur Behandlung einer mandibulären Osteomyelitis ("lumpy jaw") mit Gamithromycin bei einem Muntjak.

Mandibular osteomyelitis is common in domestic and wild ruminants. Inflammation of the mandible is not only caused by Actinomyces ssp., but numerous additional bacteria may also lead to this disease. A privately owned muntjac was presented to the clinic due to reluctant food intake and a solid mass on the corpus of the right mandible. Cranial radiography led to the diagnosis mandibular osteomyelitis. The muntjac was treated with gamithromycin (Zactran®) for 50 days, which led to an improvement of food intake as well as a size reduction of the mandibular mass. Radiographic imaging 18 and 28 months after treatment revealed a satisfactory mandibular remodeling, however complete loss of the molar teeth occured. Solitary long-term treatment with gamithromycin may represent a successful method for treating mandibular osteomyelitis in ruminants.

Outcome of different treatments for chronic diffuse sclerosing osteomyelitis of the mandible: a systematic review of published papers.

Treating chronic diffuse sclerosing osteomyelitis (DSO) is challenging and many treatments have been reported. However, we know of no standard protocol or guidelines. In this systematic review of relevant publications we provide an overview of the different treatments used. We made an electronic search of PubMed, Medline, Embase, Web of Science, and the Cochrane Library databases, for papers that described the treatment of DSO of the mandible. The search yielded 48 papers that applied to all inclusion criteria, resulting in 16 case reports, 13 case series, 18 retrospective clinical cohort studies, and one randomised controlled trial. Reported treatment options included different operations; the use of antibiotics, anti-inflammatories, and antiresorptive medication; conservative treatment; and hyperbaric oxygen. Surgical treatment resulted in a low success rate and was associated with higher morbidity than other treatments. Conservative treatment, and that of bisphosphonates, yielded more promising results, so conservative treatment and bisphosphonates seem to be the most promising therapeutic options. However, because of the high risk of bias, no firm conclusions can be drawn, and larger studies with clear inclusion criteria and specified endpoints are needed.

SAPHO Syndrome Involving the Mandible Treated With 99Tc-MDP.

SAPHO (synovitis-acne-pustulosis-hyperostosis-osteitis) syndrome is a chronic inflammatory disease involving multiple organs such as skin and bones. At present, its etiology and pathogenesis are still unclear. Due to the variety of clinical manifestations and the small number of SAPHO syndrome involving the mandible, accurate diagnosis is difficult for oral and maxillofacial surgeons. Here, the authors report that a male patient with SAPHO syndrome involving the maxillofacial skin and mandible, followed for 3 years. We used Tc-MDP (technetium-99 conjugated with methylene disphosphonate) (commercially known as Yunke) to treat this disease and achieved significant clinical treatment. This suggests that Tc-MDP can be used as a bisphosphonate to treat SAPHO syndrome.

Pediatric chronic nonbacterial osteomyelitis of the mandible: Seattle Children's hospital 22-patient experience.

BACKGROUND: Studies evaluating treatment responses for chronic nonbacterial osteomyelitis (CNO) are lacking. We aimed to measure and compare response rates of medical treatments, time to response of medical treatments among patients with CNO of the mandible, and describe bacterial contamination rates from biopsy. METHODS: We conducted a retrospective chart review of all patients diagnosed with CNO of mandible between 2003 and 2017 and extracted demographic, clinical, laboratory, imaging and surgical data. Detailed medication use and response to medications were recorded. The primary outcome was response to medical treatments defined as improvement of presenting symptoms, inflammatory markers, and imaging if available. Medical treatments included nonsteroidal anti-inflammatory drugs (NSAIDs), glucocorticoids, disease modifying anti rheumatic drugs (DMARDs), anti-tumor necrosis factor (TNF) therapy, and pamidronate. Descriptive analysis was performed when appropriate. Multivariable logistic regression and Kaplan-Meier curves were applied to compare the responses to medical treatments and time to full response. RESULTS: We identified 22 patients with a median age of 11 and 36% were female. Four patients (18%) had multifocal bone lesions. CT findings (n = 21) showed lytic lesions (62%) and sclerosis (90%). MRI (n = 14) revealed hyperintensity within bone marrow (100%), soft tissue (71%) and bony expansion (71%). Non-antibiotic treatments including NSAIDs (n = 18), glucocorticoids (n = 10), DMARDs (n = 9), anti-TNF therapy (n = 5) and pamidronate (n = 6) were applied. Rates of full responses to anti-TNF therapy (60%) and pamidronate (67%) were higher than that to NSAIDs (11%) (p < 0.05). Patients receiving pamidronate responded more rapidly than those receiving anti-TNF therapy (median two vs 17 months, p = 0.01) when there was a full response. All had bone biopsies. Intraoral biopsy was performed in 12 of 13 operated in our center and the most common contaminants were Neisseria spp and Streptococcus viridians. CONCLUSION: Both anti-TNF and pamidronate appeared superior to NSAIDs alone in treating mandibular CNO. Patients receiving pamidronate responded faster than those receiving anti-TNF therapy.