TNF-alpha blockade in Primary Chronic Non-Bacterial Osteomyelitis of the Mandible.

OBJECTIVES: Primary chronic Non-Bacterial Osteomyelitis of the jaw is a rare auto-inflammatory disease of unknown aetiology that bears pathophysiological resemblance to both the synovitis, acne, pustulosis, hyperostosis, and osteitis (SAPHO) syndrome in adults and chronic recurrent multifocal osteomyelitis (CRMO) in children. Both SAPHO and CRMO respond to TNF-alpha blockade. Previously reported treatment regimens in CNOM including non-steroidal anti-inflammatory drugs, corticosteroids, antibiotics, anti-resorptive therapy, and surgery all bear disappointing results. TNF- α blockade is suggested as a treatment option by some experts but this is not backed by any clinical data.We sought to retrospectively and exhaustively report our experience of anti-TNF alpha therapy in refractory CNOM. METHODS: Fifteen patients with refractory CNOM and high disease burden were referred to our centre. TNF- α blockade was attempted in 10 cases, given its efficacy in neighbouring diseases, its good tolerance profile and failure of previous treatment strategiesWe herein retrospectively report detailed outcomes for all patients having received anti-TNF alpha therapy for this indication in our centre. RESULTS: TNF-α-targeting therapy resulted in a rapid and sustained remission in a majority of patients with CNOM, without serious adverse events. Treatment was tapered and stopped without relapse in some patients despite a refractory course of several years. Male sex seems to be associated with a poorer outcome. CONCLUSION: Our results suggest that blocking TNF-α is efficient and safe in CNOM.

Prevalence of diffuse idiopathic skeletal hyperostosis (DISH) and early-phase DISH across the lifespan of an American population.

OBJECTIVES: DISH is a common musculoskeletal disorder; however, the imaging features and disease continuum from early to advanced stages is poorly understood. The purpose of this study was to evaluate the prevalence of DISH and early-phase DISH in an American population and to assess the extent and pattern of ectopic mineralization across the thoracic spine. METHODS: Data were retrieved in collaboration with the Rochester Epidemiology Project. We conducted a retrospective image evaluation of a sample of individuals over 19 years of age with CT of the thoracic spine from a Northern US catchment area. Stratified random sampling by age and sex was used to populate the study. We examined the prevalence and extent of ectopic mineralization along the thoracic spine using previously established criteria. RESULTS: A total of 1536 unique images (766 female and 770 male individuals) including 16 710 motion segments were evaluated for imaging features of the continuum of DISH. Collectively, 40.5% of all motion segments evaluated displayed evidence of ectopic mineralization in the thoracic spine. The prevalence of early-phase DISH was 13.2% (10.4% of female and 15.8% of male individuals). The prevalence of established DISH was 14.2% (7.4% of female and 20.9% of male individuals). Remarkable heterogeneity was detected in individuals within each disease classification, based on the extent of the thoracic spine affected and degree of mineralization. CONCLUSIONS: The continuum of imaging features associated with DISH is detected in more than one in four adults and both sexes in an American population.

Analysis of palatal marginal alveolar exostosis and palatal torus using cone-beam computed tomography.

The aim of the present study was to analyze palatal marginal alveolar exostosis (PMAE) and palatal torus (PT). Cone-beam computed tomography (CBCT) of the maxilla in multiplanar sections and volumetric renderings were used to assess this. PT and PMAE were classified according to location and morphology. Height, width, length, and thickness of the overlying mucosa were determined. The prevalence of PT and PMAE was assessed according to sex and age group. The correlation between the occurrence of PMAE and PT was also evaluated. A total of 385 CBCT scans were examined. PT was found in 38.70% of the sample and located more frequently in the middle third of the maxilla (52.35%) with a flat shape (42.95%). PMAE was found in 54.80% of the sample, bilaterally in 56.40% of the cases, and located more frequently in the molar region (62.42%) in the form of small nodules (36.97%). The mucosa covering PMAE was generally thicker than that over PT. The use of CBCT for the identification of PT and PMAE in vivo showed high frequencies of both conditions. The occurrence of PMAE was independent of the presence of PT.

Utility of MRI-based vertebral bone quality scores and CT-based Hounsfield unit values in vertebral bone mineral density assessment for patients with diffuse idiopathic skeletal hyperostosis.

This study investigated bone mineral density assessment for patients with DISH. DXA-based T-scores overestimated bone quality, while MRI-based VBQ scores and CT-based HU values provided accurate assessments, particularly for advanced degenerative cases. This enhances accurate evaluation of BMD, crucial for clinical decision-making. PURPOSE: To investigate the diagnostic effectiveness of DXA, MRI, and CT in assessing bone mineral density (BMD) for diffuse idiopathic skeletal hyperostosis (DISH) patients. METHODS: Retrospective analysis of 105 DISH patients and 116 age-matched controls with lumbar spinal stenosis was conducted. BMD was evaluated using DXA-based T-scores, MRI-based vertebral bone quality (VBQ) scores, and CT-based Hounsfield unit (HU) values. Patients were categorized into three BMD subgroups. Lumbar osteophyte categories were determined by Mata score. Demographics, clinical data, T-scores, VBQ scores, and HU values were collected. Receiver operating characteristic (ROC) analysis identified VBQ and HU thresholds for diagnosing normal BMD using DXA in controls. Correlations between VBQ, HU, and lumbar T-score were analyzed. RESULTS: Age, gender, and BMI showed no significant differences between DISH and control groups. DISH patients had higher T-score (L1-4), the lowest T-score, and Mata scores. VBQ and HU did not significantly differ between groups. In controls, VBQ and HU effectively diagnosed normal BMD (AUC = 0.857 and 0.910, respectively) with cutoffs of 3.0 for VBQ and 104.3 for HU. DISH had higher normal BMD prevalence using T-scores (69.5% vs. 58.6%, P < 0.05), but no significant differences using VBQ (57.1% vs. 56.2%, P > 0.05) and HU (58.1% vs. 57.8%, P > 0.05). Correlations revealed moderate correlations between HU and T-scores (L1-4) in DISH (r = 0.642, P < 0.001) and strong in controls (r = 0.846, P < 0.001). Moderate negative correlations were observed between VBQ and T-scores (L1-4) in DISH (r = - 0.450, P < 0.001) and strong in controls (r = - 0.813, P < 0.001). CONCLUSION: DXA-based T-scores may overestimate BMD in DISH. VBQ scores and HU values could effectively complement BMD assessment, particularly in DISH patients or those with advanced lumbar degeneration.

Synovitis, Acne, Pustulosis, Hyperostosis, Osteitis, Palmoplantar Pustulosis, and Neutrophilic Dermatoses: A GRAPPA 2023 Annual Meeting Update.

Synovitis, acne, pustulosis, hyperostosis, and osteitis (SAPHO) syndrome and chronic nonbacterial osteomyelitis (CNO) are rare autoinflammatory/autoimmune conditions seen in adults and children. Although osteoarticular manifestations are the primary distinguishing features of SAPHO, over half of patients also have palmoplantar pustulosis (PPP). These and other associated disorders such as acne, inflammatory bowel disease, and hidradenitis suppurativa are characterized, at least in the early stages, by neutrophilic infiltration. The bone and skin manifestations exhibit both innate and adaptive immune responses and therefore share similar pathogenic molecules and overlapping treatment targets. At the Group for Research and Assessment for Psoriasis and Psoriatic Arthritis (GRAPPA) 2023 annual meeting, a 3-part presentation provided an overview of current efforts at establishing consensus on diagnosis/classification, treatment, and core outcome sets for SAPHO/CNO; an overview of PPP in SAPHO and as a standalone condition; and finally, an overview of the role of the neutrophil in these disorders.

The IFITM5 Ser40Leu variant can manifest as prenatal Caffey disease.

We report on a female neonate with a clinico-radiological presentation in keeping with a lethal form of prenatal Caffey disease (PCH). She had antenatal and postnatal features of severely bowed long bones, small chest, diaphyseal hyperostosis and polyhydramnios and died shortly after birth. Initial testing excluded COL1A1-related PCH, as an OI gene panel, consisting of COL1A1, COL1A2, CRTAP, and P3H1 genes, was negative. Targeted sequencing using a gene panel was performed and a de novo heterozygous, likely pathogenic variant in IFITM5: c.119C > T(p.Ser40Leu) was identified, which was previously described to cause a severe form of progressively deforming osteogenesis imperfect (OI). To our knowledge, variants in IFITM5 have not been reported in infantile Caffey disease (ICH) or PCH. Given that the pathogenesis of PCH is largely unknown, we postulate that a subset of PCH may be associated with variants in IFITM5.

How Does Social Inequality Alter Relationships Between Porous Cranial Lesions and Mortality? Examining the Relationship Between Skeletal Indicators of Stress, Socioeconomic Status, and Survivorship in a Pediatric Autopsy Sample.

BACKGROUND: In prior exploration of modern and archeological populations, lower SES has been associated with an increased risk of mortality. However, SES is often difficult to ascertain in archeological populations. Thus, explorations of skeletal lesions and their association with mortality may be subject to confounding factors that alter the strength and/or direction of this association. METHODS: The present study uses data from a modern, documented coronial pediatric dataset to examine the association between porous cranial lesions (PCLs) (cribra orbitalia [CO] and porotic hyperostosis [PH]) and age at death while controlling for SES, as inferred through housing type, with manufactured or apartment housing identified as reflecting individuals from lower SES backgrounds in this context. We include 887 (535 males, 352 females) individuals aged 0.5-20.9 years from New Mexico who died between 2011 and 2022. Kaplan-Meier survival analysis was used to assess survivorship as related to PCLs and SES. RESULTS: Low SES is associated with lower survivorship. CO does not have a significant association with age at death when not controlling for SES; PH alone is associated with older age at death. Disadvantaged individuals with PCLs have significantly reduced survivorship than those with higher SES. DISCUSSION AND CONCLUSIONS: The findings of this study demonstrate that low SES results in reduced survivorship, and those with low SES and PCLs have worse survivorship than less disadvantaged individuals with PCLs. Thus, the strong contribution of SES to mortality necessitates the consideration of the sociocultural context as a confounding factor when examining associations between variables of interest (such as lesions) and mortality in both past and present populations.

Surgical outcomes of anterior column reconstruction for spinal fractures caused by minor trauma-preoperative examination of the number of intervertebral bone bridges is key to obtaining good bone fusion.

BACKGROUND: To achieve good bone fusion in anterior column reconstruction for vertebral fractures, not only bone mineral density (BMD) and bone metabolism markers but also lever arms due to bone bridging between vertebral bodies should be evaluated. However, until now, no lever arm index has been devised. Therefore, we believe that the maximum number of vertebral bodies that are bony and cross-linked with the contiguous adjacent vertebrae (maxVB) can be used as a measure for lever arms. The purpose of this study is to investigate the surgical outcomes of anterior column reconstruction for spinal fractures and to determine the effect of bone bridging between vertebral bodies on the rate of bone fusion using the maxVB as an indicator of the length of the lever arm. METHODS: The clinical data of 81 patients who underwent anterior column reconstruction for spinal fracture between 2014 and 2022 were evaluated. The bone fusion rate, back pain score, between the maxVB = 0 and the maxVB ≥ 2 patients were adjusted for confounding factors (age, smoking history, diabetes mellitus history, BMD, osteoporosis drugs, surgical technique, number of fixed vertebrae, materials used for the anterior props, etc.) and analysed with multivariate or multiple regression analyses. The bone healing rate and incidence of postoperative back pain were compared among the three groups (maxVB = 0, 2≦maxVB≦8, maxVB ≧ 9) and divided by the maxVB after adjusting for confounding factors. RESULTS: Patients with a maxVB ≥ 2 had a significantly higher bone fusion rate (p < 0.01) and postoperative back pain score (p < 0.01) than those with a maxVB = 0. Among the three groups, the bone fusion rate and back pain score were significantly higher in the 2≦maxVB≦8 group (p = 0.01, p < 0.01). CONCLUSIONS: Examination of the maxVB as an indicator of the use of a lever arm is beneficial for anterior column reconstruction for vertebral fractures. Patients with no intervertebral bone bridging or a high number of bone bridges are in more need of measures to promote bone fusion than patients with a moderate number of bone bridges are.

Inferior Glenoid HyperOSTosis (GHOST) Lesion Development Following Reverse Total Shoulder Arthroplasty.

BACKGROUND: Patients undergoing reverse shoulder arthroplasty (RTSA) have been noted on postoperative radiographs to have a curved bony overgrowth on the inferior glenoid neck. This study aims to investigate potential risk factors for and postoperative effects of these ossifications, here called glenoid hyperostosis (GHOST) lesions due to their location. METHODS: A retrospective review of patients undergoing reverse total shoulder arthroplasty between 2007 and 2020 at a single institution was performed. Predictors including gender, age, implant sizing, and preoperative diagnosis were examined using logistic regression analysis. Outcomes including readmission rate, revision rate, preoperative and postoperative range of motion, visual analog scale (VAS) pain scores, and American Shoulder and Elbow Surgeons (ASES) functional outcome scores were also analyzed using two-sample t-tests. RESULTS: Preoperative and postoperative radiographs of 170 primary reverse shoulder arthroplasty patients were assessed. 25.9% of RTSAs were identified to have a GHOST lesion. When adjusting for race, age, BMI, preoperative diagnosis, male patients were still associated with 2.28 odds of developing a GHOST lesion compared to female patients (95% CI: 1.08 - 4.86). Other elements such as age, race, BMI, laterality, preoperative diagnosis, implant manufacturer, and implant sizing demonstrated no statistically significant association to GHOST lesion presence. Postoperatively, GHOST lesion development was not associated with range of motion or ASES score. However, presence of GHOST lesions on radiographs was associated with increased pain scores for patients at 2 months (p = 0.034) and 12 months (p = 0.029) postoperatively. DISCUSSION: Inferior glenoid hyperostosis (GHOST) lesions is a common and potentially benign finding following reverse shoulder arthroplasty, with unclear etiology. Risk factors for GHOST lesions included male gender, while patient demographics, implant type or size, shoulder lateralization and distalization were not associated with lesion formation. Clinically, greater short-term VAS scores were seen in patients with GHOST lesions. However, there were no differences observed between the two groups in ASES scores or postoperative range of motion at later time points. Further research is needed to identify risk factors and assess the clinical implications of GHOST lesions.

Hyperostosis in meningioma: a retrospective exploration of histological correlates.

PURPOSE: Meningiomas are the most common type of primary brain tumour. Hyperostosis is commonly associated but remains incompletely understood. This study aimed to evaluate the relationship between meningioma-associated hyperostosis and other tumour variables. MATERIALS AND METHODS: We retrospectively analysed 245 patients with 263 cranial meningiomas (202 CNS WHO grade 1, 53 grade 2, and 8 grade 3) who underwent surgery over a three-year period. Meningiomas adjacent to the skull were included. Demographic, radiological, and tumour characteristics were analysed using standard statistical methods. RESULTS: Hyperostosis was evident in 99 (38%) of meningiomas. The most common subtypes were meningothelial, transitional, fibrous, atypical, and anaplastic. There were no statistically significant relationships between hyperostosis and bone invasion, and CNS WHO grade and histological subtype. Hyperostosis was more common in skull base meningiomas than in convexity meningiomas (p = 0.001). Ki-67 index was significantly related to CNS WHO grade but not histological subtype when grade was considered. Mean Ki-67 index was higher in meningiomas without hyperostosis (p = 0.03). There was no such relationship with bone invasion (p = 0.29). Univariate and multivariate analysis revealed that Ki-67 index was negatively correlated with hyperostosis (p = 0.03), while bone invasion (p < 0.001) and skull base location (p = 0.03) were positively correlated with hyperostosis. CONCLUSIONS: Hyperostosis did not appear to be related to CNS WHO grade or histological subtype. Proliferative activity appeared to be higher in meningiomas without hyperostosis and hyperostosis was associated with evidence of bone invasion and skull base location.

Long-term effects of diffuse idiopathic skeletal hyperostosis on physical function: A longitudinal analysis.

BACKGROUND: Cross-sectional studies on diffuse idiopathic skeletal hyperostosis have focused on its incidence and related factors. However, the long-term changes caused by the disease remain unclear. This longitudinal cohort study aimed to elucidate the progression of diffuse idiopathic skeletal hyperostosis and its effects on physical function, health-related quality of life, and spinal alignment. METHODS: We recruited 255 older adults (87 men and 168 women; average age, 71.3 years in 2014) who attended local health checkups in 2014 and 2020. Height, body weight, body mass index, blood pressure, grip strength, functional reach, and bone mineral density were measured. The prevalence, location, number of ossified contiguous vertebrae, and spinopelvic parameters were estimated using whole-spine standing radiographs. For health-related quality of life assessment, the Oswestry disability index and EuroQuol-5D were obtained. We performed a 1:1 case-control study with age and sex-matched patients with and without diffuse idiopathic skeletal hyperostosis and analyzed progression over a 6-year period. RESULTS: In 2014, 39 (15.3%) of 255 patients were diagnosed with diffuse idiopathic skeletal hyperostosis (24 males and 15 females), which occurred more frequently in the elderly and males. In 2020, 12 (4.3%) patients were newly diagnosed with diffuse idiopathic skeletal hyperostosis, and 28 (71.7%) of 39 patients diagnosed in 2014 showed varying degrees of progression. Compared with age- and sex-matched patients without diffuse idiopathic skeletal hyperostosis, patients with the condition had higher body mass index and lumbar bone mineral density, larger sagittal vertical axis, and greater T1-pelvic angle. Changes in physical function and spinal-pelvic parameters during the 6-year period did not differ between the groups. CONCLUSIONS: Over a 6-year period, the prevalence of diffuse idiopathic skeletal hyperostosis increased by 4.3%, and it progressed in 71.7% of patients. However, it had little effect on longitudinal physical function, health-related quality of life, and spinopelvic parameters in older adults.

Prevalence and characteristics of osseous bridging between vertebral bodies in the cervical spine: A skeletal study.

Osseous bridging (OB) in three or more segments of motions (SOMs) of the mobile spine was initially defined as diffuse idiopathic skeletal hyperostosis (DISH), located particularly in the thoracic spine (T-spine). This pathological phenomenon is often characterized by calcification and ossification, which take place simultaneously or separately. The soft tissues, mainly ligaments and entheses, are calcified, with bone formation not originating from the anterior longitudinal ligament (ALL). DISH formation can involve osteophytes, which are created by the ossification process and can involve soft tissue such as the ALL. The ALL can also be calcified. Until recently, the prevalence of DISH in the general population was considered low (0%-5%) and rare in the cervical spine (C-spine). In a cross-sectional observational skeletal study, we investigated the prevalence and location of C-spine OB between vertebral bodies with fewer than three SOMs. We tested a large sample (n = 2779) of C-spines housed in the Cleveland Museum of Natural History (Ohio, USA). The human sources of the samples had died between the years 1912 and 1938 and represented both sexes and two different ethnic groups: Black Americans and White Americans. The process development can be seen on the ALLs as calcification, osteophytosis, and candle-shaped. Among all of the specimens, 139 (5%) were affected by OB, mostly in one SOM. Prevalence tended to be higher in women, White Americans, and the older age group. The levels most affected were C3-C4, followed by C2-C3 and subsequently, C5-C6. OB involving two consecutive SOMs was found only at C5-C7. We believe it is important to respond to the presence of a single SOM with a presumptive diagnosis of OB and to follow up, identify whether the diagnosis is correct, and take preventive action if possible. There is a need for updated diagnostic criteria and research approaches that reflect contemporary lifestyle factors and their impact on spine health.

The clinical manifestations of hyperostosis frontalis interna: A qualitative systematic review of cases.

Hyperostosis frontalis interna (HFI) is a condition defined as abnormal bone growth on the posterior aspect of the frontal bone. Despite uncertainties regarding its etiology and prognosis, clinicians typically consider HFI a benign pathology. There are no studies organizing all the possible manifestations of the disease. The present study aims to organize all the clinical manifestations of HFI within the current case report/series literature. A blinded PRISMA-guided search of HFI case reports and case series yielded 43 relevant articles and provided 110 patients for analysis. The symptoms presenting alongside HFI were extracted and tabulated. We found high-frequency clinical manifestations of HFI (>20% of patients) to include headaches, obesity, vertigo/dizziness symptoms, cognitive decline, and depression. An additional 15 symptoms were tabulated at frequencies found to be less than 20%. Based on our analysis, we suggest the constellation of high-frequency symptoms can offer a more comprehensive clinical picture of symptomatic HFI which may be valuable to consider for clinicians and future researchers in the field of HFI.

Hyperostosis Frontalis Simulating Metastatic Deposits: Unmasked on SPECT-CT.

Skeletal scintigraphy has a pivotal role in detecting a number of bone pathologies, but it has its own limitations because of 2D image acquisition. Hybrid imaging acts as a savior in these cases where it is difficult to distinguish between benign and malignant lesions just on the basis of planar images. We present one such case of known breast carcinoma with abnormal increased radiotracer uptake in the skull which was difficult to characterize as benign lesion such as hyperostosis frontalis or metastatic osseous lesion. The importance of describing this case is to have a thorough understanding of hyperostosis patterns and to not confuse it with metastatic deposits in patients with known malignancies.

[Convexity hyperostotic meningioma en plaque: a systematic review]. / Ploskostnye giperostoticheskie meningiomy svoda cherepa. Sistematicheskii obzor literatury.

BACKGROUND: Planar hyperostotic meningiomas account for 2-9% of intracranial meningiomas. They are characterized by planar node following the contours of the inner surface of the skull. Hyperostosis is present in most cases. Timely diagnosis of skull base tumors is usually simple due to early involvement of the cranial nerves. However, convexity meningiomas en plaque usually reach large dimensions that complicates surgery and radiotherapy. OBJECTIVE: To analyze the current state of diagnosis, molecular biology and surgical treatment of hyperostotic meningiomas en plaque. MATERIAL AND METHODS: A systematic review was performed in accordance with the PRISMA guidelines. Searching for literature data included the following keywords: «planar meningioma¼, «hyperostotic meningioma¼, «meningioma en plaque¼, «infiltrative meningioma¼. We reviewed the PubMed and Google Scholar databases until May 2023 and enrolled only full-text Russian-, English- or French-language reports. RESULTS AND DISCUSSION: Among primary 332 reports, 35 references met the inclusion criteria. We found less severity or absence of focal neurological symptoms, comparable incidence of intracranial hypertension and no histological differences between planar and nodular meningiomas. Analysis of molecular biological features of planar meningiomas, including cell cultures, is feasible. There is no consensus regarding surgical treatment and radiotherapy. Most publications are case reports. CONCLUSION: The results of treatment of planar hyperostotic meningiomas, especially large and giant ones, are unsatisfactory. There is no a generally accepted algorithm for treating patients in the literature. This problem requires further research.

Association between serum interleukin-17 levels and ectopic bone formation in OPLL patients with DISH.

OBJECTIVE: To investigate the relationship between the severity and morphology of heterotopic ossification in the spinal ligaments including sacroiliac (SI) joints, and serum interleukin-17 (IL-17) levels in patients with ossification of the posterior longitudinal ligament (OPLL) with or without diffuse idiopathic skeletal hyperostosis (DISH), as well as a non-OPLL group. METHODS: A total of 103 patients with OPLL (DISH (-), n = 50; DISH (+), n = 53) and 53 age- and gender-matched controls were included. The serum levels of IL-17 were analyzed, and the severity of ectopic ossification and the morphology of ectopic bone formation were evaluated. The SI joint morphological variations were categorized into four types. RESULTS: No significant differences were found in serum IL-17 levels between the OPLL and control groups. However, the DISH (+) group showed higher IL-17 levels than the DISH (-) group, especially in female patients (p = 0.003). Additionally, IL-17 levels were positively correlated with the number of Flat vertebral units, meaning one of the characteristics of DISH ossification type (R2 = 0.199, p = 0.012). IL-17 levels in type 4 were significantly higher in the DISH (+) group than in the DISH (-) group. CONCLUSIONS: The morphological characteristics of paravertebral bone formation in the entire spine, including the SI joint, are likely associated with serum IL-17 levels in OPLL. These findings provide pathological and serological evidence of local inflammation contributing to paravertebral ossification of OPLL patients.

Clinical and therapeutic diversity in adult chronic nonbacterial osteomyelitis (CNO) of the sternocostoclavicular region: a meta-analysis.

OBJECTIVES: Chronic nonbacterial osteomyelitis (CNO) is a rare inflammatory bone disease. The distinct CNO subtype that affects the anterior chest wall is descriptively named sternocostoclavicular hyperostosis (SCCH) and mainly occurs in adults. Literature on CNO/SCCH is scattered and lacks diagnostic and therapeutic consensus. METHODS: Systematic review and meta-analysis aiming to characterize clinical presentation and therapeutic modalities applied in adult CNO/SCCH patients. Untransformed numerical data and double-arcsine transformed proportional data were pooled in a random effects model in R-4.0.5; proportions were reported with 95% CI. RESULTS: Forty studies were included, containing data on 2030 and 642 patients for aim 1 and 2, respectively. A female predisposition (67%, 95% CI 60, 73) and major diagnostic delay (5 years 95% CI 3, 7) were noted. Clinical presentation included chest pain (89%, 95% CI 79, 96) and swelling (79%, 95% CI 62, 91). Patients suffered from pustulosis palmoplantaris (53%, 95% CI 37, 68), arthritis (24%, 95% CI 11, 39) and acne (8%, 95% CI 4, 13). Inflammatory markers were inconsistently elevated. Autoantibody and HLA-B27 prevalence was normal, and histopathology unspecific. Increased isotope uptake (99%, 95% CI 96, 100) was a consistent imaging finding. Among manifold treatments, pamidronate and biologicals yielded good response in 83%, 95% CI 60, 98 and 56%, 95% CI 26, 85, respectively. CONCLUSION: CNO/SCCH literature proves heterogeneous regarding diagnostics and treatment. Timely diagnosis is challenging and mainly follows from increased isotope uptake on nuclear examination. Biopsies, autoantibodies and HLA status are non-contributory, and biochemical inflammation only variably detected. Based on reported data, bisphosphonates and biologicals seem reasonably effective, but due to limitations in design and heterogeneity between studies the precise magnitude of their effect is uncertain. Fundamentally, international consensus seems imperative to advance clinical care for CNO/SCCH.

High Circulating FGF-23 and Its Relationship with Severity of Spinal Involvement in Diffuse Idiopathic Skeletal Hyperostosis.

The primary objective of this study was to determine the role of fibroblast growth factor 23 (FGF-23) in the pathogenesis of diffuse idiopathic skeletal hyperostosis (DISH). A total of 61 patients with DISH and 61 age- and sex-matched control patients without DISH were included in this study. The serum FGF-23, creatinine, inorganic phosphate, calcium, albumin, albumin-adjusted calcium and alkaline phosphatase, and C-reactive protein were assessed in both groups. Based on the extent of ossification, DISH group was further divided into T-DISH and L-DISH subgroups. Data were comparatively analyzed between DISH and Non-DISH groups and among T-DISH, L-DISH, and Non-DISH groups, respectively. Besides, the number of ossification segments of all DISH patients was quantified and the correlation between the number of ossification segments and the serum concentration of FGF-23 was analyzed. The results revealed that serum FGF-23 was significantly higher in DISH group than in Non-DISH group, regardless of gender. Interestingly, serum Pi was significantly lower in DISH group than in Non-DISH group. Moreover, a significant difference in serum FGF-23 among T-DISH, L-DISH, and Non-DISH groups was also observed. In contrast to Non-DISH group, both T-DISH and L-DISH subgroups displayed significantly higher serum FGF-23 level. Although the mean value was relatively higher in L-DISH subgroup, no statistically significant difference was found between T-DISH and L-DISH subgroups. In addition, a moderately positive correlation was identified between the number of ossification segments and the serum level of FGF-23. It can be concluded that serum FGF-23 could serve as a positive biomarker for DISH and may play a significant role in ectopic ossification in DISH.

Post-traumatic spinal hematoma in diffuse idiopathic skeletal hyperostosis (DISH).

OBJECTIVES: To determine the incidence of spinal hematoma and its relation to neurological deficit after trauma in patients with spinal ankylosis from diffuse idiopathic skeletal hyperostosis (DISH). MATERIALS AND METHODS: A retrospective review of 2256 urgent or emergency MRI referrals over a period of 8 years and nine months revealed 70 DISH patients who underwent CT and MRI scans of the spine. Spinal hematoma was the primary outcome. Additional variables were spinal cord impingement, spinal cord injury (SCI), trauma mechanism, fracture type, spinal canal narrowing, treatment type, and Frankel grades during injury, before and after treatment. Two trauma radiologists reviewed MRI scans blinded to initial reports. RESULTS: Of 70 post-traumatic patients (54 men, median age 73, IQR 66-81) with ankylosis of the spine from DISH, 34 (49%) had spinal epidural hematoma (SEH) and 3 (4%) had spinal subdural hematoma, 47 (67%) had spinal cord impingement, and 43 (61%) had SCI. Ground-level fall (69%) was the most common trauma mechanism. A transverse, AO classification type B spine fracture (39%) through the vertebral body was the most common injury type. Spinal canal narrowing (p < .001) correlated and spinal cord impingement (p = .004) associated with Frankel grade before treatment. Of 34 patients with SEH, one, treated conservatively, developed SCI. CONCLUSIONS: SEH is a common complication after low-energy trauma in patients with spinal ankylosis from DISH. SEH causing spinal cord impingement may progress to SCI if not treated by decompression. CLINICAL RELEVANCE STATEMENT: Low-energy trauma may cause unstable spinal fractures in patients with spinal ankylosis caused by DISH. The diagnosis of spinal cord impingement or injury requires MRI, especially for the exclusion of spinal hematoma requiring surgical evacuation. KEY POINTS: • Spinal epidural hematoma is a common complication in post-traumatic patients with spinal ankylosis from DISH. • Most fractures and associated spinal hematomas in patients with spinal ankylosis from DISH result from low-energy trauma. • Spinal hematoma can cause spinal cord impingement, which may lead to SCI if not treated by decompression.

Bilateral compressive optic neuropathy and outer retinopathy due to optic canal hyperostosis in a child with isolated vitamin a deficiency.

PURPOSE: Vitamin A plays a crucial role in rod phototransduction, with deficient levels manifesting as night blindness. Animal models have demonstrated bone dysplasia in the setting of hypovitaminosis A. We present a rare case of bony overgrowth leading to bilateral compressive optic neuropathy, combined with outer retinopathy, in a paediatric patient secondary to isolated vitamin A deficiency. METHODS: A single case report was conducted from Toronto, Canada. RESULTS: A 12-year-old boy with known autism spectrum disorder presented with a 9-month history of progressive painless vision loss. Vision was 20/300 and hand motion in the right and left eye, respectively. Fundus photography demonstrated bilateral optic atrophy and yellow lesions notably in the right eye far periphery. Optical coherence tomography (OCT) imaging demonstrated thinning of the retinal nerve fibre layer, alterations in the ellipsoid zone, as well as retinal pigment epithelium deposits. Computed tomography imaging demonstrated sphenoid bone thickening with narrow optic canals and moderate optic atrophy bilaterally. Full-field electroretinogram (ERG) demonstrated mildly reduced dark adapted (DA) 0.01 b-wave amplitudes and electronegative configuration of DA 3.0 and DA 10.0 ERG; the light adapted ERGs were normal. The patient was treated with pulse vitamin A therapy. Subsequently, the DA ERG normalized, outer retinal changes reversed and vision stabilised; no surgical intervention was conducted. CONCLUSION: This case represents a rare presentation of compressive optic neuropathy with concomitant outer retinopathy secondary to isolated vitamin A deficiency. Despite improvement in outer retinal integrity on OCT imaging and ERG testing results following vitamin A supplementation, no functional improvement was obtained due to severe optic atrophy.