A Rare Case of Partially Ossified Sacrospinous Ligament Causing Sciatic Nerve Compression.

Ossification of sacrospinous ligament induces a great risk for maintaining the stability of the pelvis. The sacrospinous ligament, along with the sacrotuberous ligament, plays a distinct role in the sacroiliac joint and pubic symphysis stability. The ossification may cause compression of neurovascular structure traversing through the greater and lesser sciatic foramen. Here we report a case of unilateral heterogenous ossification of the left sacrospinous ligament causing sciatic nerve compression and sciatic pain. A 22-year-old Bangladeshi woman, mother of one child, presented with complaints of pain in the lower back, left buttock and back of the upper thigh. Clinical examination and investigations revealed a diagnosis of the partially ossified sacrospinous ligament with sciatic nerve compression. Total excision of heterotrophic calcification and partial excision of left sacrospinous ligament through posterior approach by a left paramedian incision over the lower back was performed under general anaesthesia. On outpatient follow-up visits at 2 weeks and 6 weeks post-surgery, complete disappearance of pain was observed, and the patient was able to return to regular productive life activity. In this report, we presented a rare case of ossified sacrospinous ligament causing sciatic nerve compression with unknown etiology. The surgical approach performed, total excision of heterotrophic calcification and partial excision of left sacrospinous ligament through the posterior approach helped to preserve the pelvic stability with a good clinical outcome.

An Uncommon Presentation of Eagle Syndrome in a Primary Care Patient with Chronic Neck Pain: A Case Report and Literature Review.

BACKGROUND Eagle syndrome is an uncommon medical illness that can manifest as neck pain in primary care. It results from an abnormally unilateral or bilateral long styloid process that may compress and affect adjacent structures, which leads to the symptoms. Classical Eagle syndrome has been commonly reported, but this case highlights the uncommon involvement of autonomic nerve dysfunction. CASE REPORT This case report details a 43-year-old woman with chronic neck pain for 5 years who saw numerous medical professionals and underwent 8 physiotherapy sessions. Marginal improvement of her neck pain and recent development of imbalance and a floating sensation prompted escalation of radiological imaging that eventually led to the diagnosis of Eagle syndrome. She was subsequently subjected to tonsillectomy and styloidectomy to address the sources of her neck pain. CONCLUSIONS Neck pain is a common complaint in primary care, but Eagle syndrome is often overlooked due to its complex symptoms, which mimic other conditions resulting in missed diagnoses and prolonged diagnostic evaluations. To improve patient care and outcomes, primary care physicians should consider Eagle syndrome when evaluating neck pain. This involves taking a detailed clinical history, conducting a thorough physical examination, using appropriate imaging techniques, and knowing the treatment options. By considering this potential diagnosis, primary care physicians, other healthcare professionals, and physical therapists play an important role in referring these patients to an otorhinolaryngologist or a maxillofacial surgeon for a comprehensive evaluation and management.

Percutaneous endoscopic transforaminal decompression surgery for symptomatic double-level lumbar spinal stenosis with ossification.

This study aimed to explore the short-term effects of percutaneous endoscopic transforaminal decompression (PETD) for the treatment of symptomatic double-level lumbar spinal stenosis (LSS) with ossification. Twenty-eight patients diagnosed with double-level lumbar spinal stenosis who underwent double-level PETD surgery between January 2021 and January 2023 at our institution. General information, such as age, sex, disease duration, hospitalization time, and operation time, was recorded. Magnetic resonance imaging (MRI) dural sac cross-sectional area (DSCA) was recorded to assess the degree of spinal canal decompression. The White-Panjabi scoring system (WP) was used to assess preoperative and postoperative lumbar spine stability. Pre- and postoperative visual analog scale (VAS) and Oswestry Disability Index (ODI) scores were recorded to assess symptom improvement, and surgical efficacy was evaluated using the modified Macnab evaluation criteria at the 1-year postoperative follow-up. The types and risks of complications were also recorded. The patient's 1-year postoperative follow-up MRI showed that both L3/4 and L4/5 DSCA were significantly enlarged compared with preoperative values (P < .001). There was no significant difference in the WP scores at 3 months postoperatively compared with those preoperatively (P > .05). The VAS scores for hip and lower extremity pain at 3 days, 3 months, and 1 year postoperatively were significantly lower than those preoperatively (P < .001), and the ODI scores at 3 months and 1 year postoperatively were significantly lower than those preoperatively (P < .001). There were no significant differences in hip pain, lower extremity pain VAS scores, or ODI scores between the postoperative follow-up time points (P > .05). There was 1 case of lower limb numbness and 1 case of neuroedematous pain in the postoperative period, and all patients had no complications, such as dural sac tear, infection, or recurrence. The 1-year postoperative follow-up was assessed as excellent in 17 cases, good in 9 cases, and possible in 2 cases using the modified Macnab criteria, with an excellent rate of 92.9%. The efficacy of double-level PETD for symptomatic double-level LSS is clear, the local stability of the lumbar spine is less affected, and the risk is low, which can reduce the chances of reoperation in patients. Thus, it is a recommended surgical procedure.

Clinical and Genetic Insights into Desbuquois Dysplasia: Review of 111 Case Reports.

Skeletal disorders encompass a wide array of conditions, many of which are associated with short stature. Among these, Desbuquois dysplasia is a rare but severe condition characterized by profound dwarfism, distinct facial features, joint hypermobility with multiple dislocations, and unique vertebral and metaphyseal anomalies. Desbuquois dysplasia is inherited in an autosomal recessive manner, with both the DBQD1 (MIM 251450) and DBQD2 (MIM 615777) forms resulting from biallelic mutations. Specifically, DBQD1 is associated with homozygous or compound heterozygous mutations in the CANT1 gene, while DBQD2 can result from mutations in either the CANT1 or XYLT1 genes. This review synthesizes the findings of 111 published case reports, including 54 cases of DBQD1, 39 cases of DBQD2, and 14 cases of the Kim variant (DDKV). Patients in this cohort had a median birth weight of 2505 g, a median length of 40 cm, and a median occipitofrontal circumference of 33 cm. The review highlights the phenotypic variations across Desbuquois dysplasia subtypes, particularly in facial characteristics, joint dislocations, and bone deformities. Genetic analyses revealed a considerable diversity in mutations, with over 35% of cases involving missense mutations, primarily affecting the CANT1 gene. Additionally, approximately 60% of patients had a history of parental consanguinity, indicating a potential genetic predisposition in certain populations. The identified mutations included deletions, insertions, and nucleotide substitutions, many of which resulted in premature stop codons and the production of truncated, likely nonfunctional proteins. These findings underscore the genetic and clinical complexity of Desbuquois dysplasia, highlighting the importance of early diagnosis and the potential for personalized therapeutic approaches. Continued research is essential to uncover the underlying mechanisms of this disorder and improve outcomes for affected individuals through targeted treatments.

Effectiveness of indomethacin in preventing Heterotopic Ossification: a systematic review and meta-analysis of randomized controlled trials.

BACKGROUND: Heterotopic ossification (HO) is a frequent complication of joint trauma or surgery, commonly occurring after hip replacements, acetabular or other joint injuries, or surgeries. Indomethacin has long been used to prevent HO and is considered the first-line therapy. However, its effectiveness and necessity for HO prevention are still debated due to mixed evidence about its efficacy and potential side effects. METHODS: Following PRISMA guidelines, this systematic review and meta-analysis evaluated randomized controlled trials using the PICO framework. Searches were conducted across PubMed, Embase, Cochrane Library, and Web of Science. Data were extracted and assessed based on the evidence levels of the selected articles. This study was registered with the International Platform of Registered Systematic Review and Meta-analysis Protocols (INPLASY). RESULTS: This analysis included 665 patients, with 347 in the Indomethacin group and 318 in the No Indomethacin group. The outcomes analyzed-HO, Gastrointestinal Side Effects, and Bone Ununion-indicated that indomethacin effectively prevents HO. The meta-analysis revealed that the Indomethacin group experienced a significant reduction in the occurrence of grade I-II HO compared to the No Indomethacin group, but not for grade III-IV HO. Gastrointestinal side effects were notably higher in the Indomethacin group. The incidence of bone nonunion was higher in the Indomethacin group, although not statistically significant. CONCLUSIONS: The meta-analysis suggests that indomethacin is effective in preventing HO, particularly for Brooker grade I-II, rather than Brooker grade III-IV. Special attention should be given to gastrointestinal complications when using indomethacin. Further prospective randomized controlled studies are required to confirm these findings.

Neurogenic Heterotopic Ossification of the Hip: a Case Report.

Heterotopic ossification (HO) denotes aberrant osteogenesis in extra-skeletal tissues, often associated with neurological disorders, total hip arthroplasty, and specific traumatic scenarios. Neurogenic heterotopic ossification manifests prominently subsequent to traumatic brain injury or spinal cord injury, with Guillain-Barre Syndrome presenting an infrequent etiological link. This article details the case of a 56-year-old female diagnosed with Guillain-Barre Syndrome, who developed neurogenic heterotopic ossification around both hips within two years of disease onset. The patient's medical history included mechanical ventilation, incomplete tetraplegia, and prolonged immobilization. A conclusive diagnosis of HO was established through radiological and clinical assessments. After neurogenic heterotopic ossification was confirmed, the patient had surgery to remove the lesions, radiation therapy, and medication treatments as planned. Physical therapy was introduced one week post-surgery, with subsequent follow-ups tracking improvements in pain levels, range of motion (ROM), and Activities of Daily Living scores. Key words: neurogenic heterotopic ossification, Guillain-Barre syndrome, hip, excision.

Cant1 Affects Cartilage Proteoglycan Properties: Aggrecan and Decorin Characterization in a Mouse Model of Desbuquois Dysplasia Type 1.

Desbuquois dysplasia type 1 (DBQD1) is a recessive chondrodysplasia caused by mutations in the CANT1 gene, encoding for the Golgi Calcium-Activated Nucleotidase 1 (CANT1). The enzyme hydrolyzes UDP, the by-product of glycosyltransferase reactions, but it might play other roles in different cell types. Using a Cant1 knock-out mouse, we demonstrated that CANT1 is crucial for glycosaminoglycan (GAG) synthesis; however, its impact on the biochemical properties of cartilage proteoglycans remains unknown. Thus, in this work, we characterized decorin and aggrecan from primary chondrocyte cultures and cartilage biopsies of mutant mice at post-natal day 4 by Western blots and further investigated their distribution in the cartilage extracellular matrix (ECM) by immunohistochemistry. We demonstrated that the GAG synthesis defect caused by CANT1 impairment led to the synthesis and secretion of proteoglycans with shorter GAG chains compared with wild-type animals. However, this alteration did not result in the synthesis and secretion of decorin and aggrecan in the unglycanated form. Interestingly, the defect was not cartilage-specific since also skin decorin showed a reduced hydrodynamic size. Finally, immunohistochemical studies in epiphyseal sections of mutant mice demonstrated that the proteoglycan structural defect moderately affected decorin distribution in the ECM.

Bronchiectatic Actinomycosis with Osseous Metaplasia Masquerading as Lung Cancer.

After the publication of the original article, the authors noticed an error in the departmental affiliation of one of the contributors, Don MASCARENHAS. The corrected version of the department is provided below, and the original article has been updated accordingly. Archana BHAT < sup > 1 < /sup > , Manjunath J < sup > 1 < /sup > , Don MASCARENHAS < sup > 2 < /sup > < br / > Department of < sup > 1 < /sup > Pathology and < sup > 2 < /sup > Pulmonology, Father Muller Medical College, MANGALORE, INDIA.

Pharmacologic or genetic targeting of peripheral nerves prevents peri-articular traumatic heterotopic ossification.

Heterotopic ossification (HO) is a pathological process that commonly arises following severe polytrauma, characterized by the anomalous differentiation of mesenchymal progenitor cells and resulting in the formation of ectopic bone in non-skeletal tissues. This abnormal bone growth contributes to pain and reduced mobility, especially when adjacent to a joint. Our prior observations suggested an essential role of NGF (Nerve Growth Factor)-responsive TrkA (Tropomyosin Receptor Kinase A)-expressing peripheral nerves in regulating abnormal osteochondral differentiation following tendon injury. Here, we utilized a recently developed mouse model of hip arthroplasty-induced HO to further validate the role of peripheral nerve regulation of traumatic HO. Nerve ingrowth was either modulated using a knockin transgenic animals with point mutation in TrkA, or local treatment with an FDA-approved formulation of long acting Bupivacaine which prevents peripheral nerve growth. Results demonstrate exuberant sensory and sympathetic nerve growth within the peri-articular HO site, and that both methods to reduce local innervation significantly reduced heterotopic bone formation. TrkA inhibition led to a 34% reduction in bone volume, while bupivacaine treatment resulted in a 50% decrease. Mechanistically, alterations in TGFß and FGF signaling activation accompanied both methods of local denervation, and a shift in macrophages from M1 to M2 phenotypes was observed. In sum, these studies reinforce the observations that peripheral nerves play a role in the etiopathogenesis of HO, and that targeting local nerves represents a potential therapeutic approach for disease prevention.

Involvement of the Wnt/ß-catenin signalling pathway in heterotopic ossification and ossification-related diseases.

Heterotopic ossification (HO) is a pathological condition characterized by the formation of bone within soft tissues. The development of HO is a result of abnormal activation of the bone formation programs, where multiple signalling pathways, including Wnt/ß-catenin, BMP and hedgehog signalling, are involved. The Wnt/ß-catenin signalling pathway, a conserved pathway essential for various fundamental activities, has been found to play a significant role in pathological bone formation processes. It regulates angiogenesis, chondrocyte hypertrophy and osteoblast differentiation during the development of HO. More importantly, the crosstalk between Wnt signalling and other factors including BMP, Hedgehog signalling, YAP may contribute in a HO-favourable manner. Moreover, several miRNAs may also be involved in HO formation via the regulation of Wnt signalling. This review aims to summarize the role of Wnt/ß-catenin signalling in the pathogenesis of HO, its interactions with related molecules, and potential preventive and therapeutic measures targeting Wnt/ß-catenin signalling.

Immobile to Ambulation: Complex Multijoint Pathologies in a Patient with Advanced HIV Disease: A Case Report.

CASE: A 30-year-old man with a history of advanced HIV disease (AHD) presented with bilateral equinocavus, leg, and foot muscle paresis, Brooker grade 4 heterotopic ossification of hips and knee stiffness, and was unable to sit upright, stand independently, or walk. Electromyography showed demyelinating sensorimotor and axonal polyneuropathy of lower extremities. Multiple surgeries of bilateral hips, ankles, and feet enabled joint mobility, plantigrade feet, and independent ambulation. CONCLUSION: Patients with AHD may develop multijoint pathologies, secondary to HIV, antiretroviral therapy, or prolonged immobility, resulting in loss of ambulation and independence. Restoring ambulation may necessitate multiple surgeries, with potential for success.

The Impact of Endplate Coverage on Heterotopic Ossification Following Cervical Disc Replacement: A Systematic Review and Meta Analysis.

STUDY DESIGN: Systematic review and meta-analysis. OBJECTIVE: Describe the impact of endplate coverage on HO in cervical disc replacement (CDR). SUMMARY OF BACKGROUND DATA: CDR is a motion-sparing alternative to anterior cervical discectomy and fusion. However, the high prevalence of heterotopic ossification threatens to diminish range of motion and limit this benefit associated with CDR. MATERIALS AND METHODS: A systematic review was performed in accordance with the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines. EMBASE and PubMed databases were queried. Results were deduplicated and screened. Relevant studies were included. All metrics that were reported in ≥3 studies were aggregated for analysis. SPSS was used to perform the meta-analysis. RESULTS: A total of 10 studies were included in the systematic review. Endplate coverage was assessed using a wide variety of measurements, including anteroposterior implant depth (ID), endplate depth (ED), exposed endplate depth (EED), implant depth to endplate depth ratio (ID:ED), EED to ED ratio (EED:ED), implant width (IW) to endplate width (EW) ratio (IW:EW), and the implant area (IA) to endplate area (EA) ratio (IA:EA). No evidence has linked ID (three studies) to HO. Mixed evidence has linked ID:ED (3/5) and IW:ED (1/2) to HO. All available evidence has linked ED (2), EED (4), EED:ED (2), and IA:EA (1) to HO. In our meta-analysis, ID was not found to be a significant risk factor for HO. However, EED and ID:ED were found to be significant risk factors for HO formation. CONCLUSIONS: Exposed endplate, especially as assessed by EED and ID:ED, is a significant risk factor for HO. Surgeons should focus on preoperative planning and intraoperative implant selection to maximize endplate coverage. While optimizing technique and implant selection is crucial, improved implant design may also be necessary to ensure that appropriate implant-endplate footprint matching is possible across the anatomic spectrum.

Anatomical and Radiological Findings of Abnormal Calcified Structures within and around the Foramen Transversarium and Its Clinical Implications.

BACKGROUND: The foramen transversarium is a vital anatomical structure found in the cervical vertebrae of the spine. Typically, it serves as a passageway for important neurovascular structures, including the vertebral artery and vein, as well as the vertebral nerve. However, abnormal calcification or ossification of soft tissues in and around this area can lead to various clinical implications. Understanding the presence and implications of abnormal ossified structures in and around the foramen transversarium is crucial for clinicians involved in the diagnosis and management of cervical spine disorders. AIMS: Accordingly, this present study was designed to evaluate the abnormal ossified structures anatomically and radiologically within and around the foramen transversarium. MATERIALS AND METHODS: This study was conducted on 182 (26 sets of cervical vertebrae) dried human cervical vertebrae obtained from the respective departments of anatomy and on 190 (95 males and 95 females) adult patients who visited the radiology department for neck-related problems such as stiff neck, neck/shoulder pain, dizziness, vertigo, imbalance, visual disturbances, and cognitive impairment. RESULTS: Among 182 examined cervical vertebrae, unilateral complete accessory foramen transversarium was found in 23 vertebrae (12.63%), bilateral complete in 19 (10.44%), bilateral incomplete in 6 (3.29%), unilateral complete double in 4 (2.19%), and unilateral complete absence of foramen transversarium in 3 (1.64%). Stenosis due to aberrant osteophytes was noted in 9 vertebrae (4.9%). Out of 190 patients, three males presented with cervical kyphosis, severe spinal canal stenosis, and spinal cord compression due to ossification of the posterior longitudinal ligament and osteophyte complexes at C3-C6, with the most significant compression at C5-C6. CONCLUSION: A thorough understanding of abnormal ossifications in and around the foramen transversarium is crucial for the management of cervical spine disorders; imaging modalities such as X-ray, computed tomography, and magnetic resonance imaging are crucial for recognizing and intervening in these cases, which is essential to prevent adverse neurological outcomes associated with vertebral artery involvement.

Résumé Contexte:Le foramen transversarium est une structure anatomique vitale trouvée dans les vertèbres cervicales de la colonne vertébrale. Généralement, il sert de passage pour d'importantes structures neurovasculaires, notamment l'artère et la veine vertébrale, ainsi que le nerf vertébral. Cependant, anormal la calcification ou l'ossification des tissus mous dans et autour de cette zone peut entraîner diverses implications cliniques. Comprendre la présence et Les implications des structures ossifiées anormales dans et autour du foramen transversarium sont cruciales pour les cliniciens impliqués dans le diagnostic et prise en charge des troubles de la colonne cervicale.Objectifs:En conséquence, cette présente étude a été conçue pour évaluer les structures ossifiées anormales anatomiquement et radiologiquement à l'intérieur et autour du foramen transversarium.Matériels et méthodes:Cette étude a été menée sur 182 (26 ensembles de vertèbres cervicales) vertèbres cervicales humaines séchées obtenues auprès des départements d'anatomie respectifs et sur 190 (95 hommes et 95 femmes) patients adultes qui ont consulté le service de radiologie pour des problèmes liés au cou tels qu'une raideur de la nuque, des douleurs au cou/à l'épaule, des étourdissements, vertiges, déséquilibre, troubles visuels et troubles cognitifs.Résultats:Parmi 182 vertèbres cervicales examinées, unilatérales completes un foramen transversarium accessoire a été trouvé dans 23 vertèbres (12,63%), bilatéral complet dans 19 (10,44%), bilatéral incomplet dans 6 (3,29%), double complet unilatéral chez 4 (2,19 %) et absence complète unilatérale de foramen transversarium chez 3 (1,64 %). Sténose due à une aberration des ostéophytes ont été notés dans 9 vertèbres (4,9 %). Sur 190 patients, trois hommes présentaient une cyphose cervicale, une sténose sévère du canal rachidien, et compression de la moelle épinière due à l'ossification du ligament longitudinal postérieur et des complexes ostéophytes en C3­C6, le plus compression importante en C5­C6.Conclusion:Une compréhension approfondie des ossifications anormales dans et autour du foramen transversarium est crucial pour la gestion des troubles de la colonne cervicale; modalités d'imagerie telles que les rayons X, la tomodensitométrie et la résonance magnétique l'imagerie est cruciale pour reconnaître et intervenir dans ces cas, ce qui est essentiel pour prévenir les conséquences neurologiques indésirables associées avec atteinte de l'artère vertébrale.

A rare mass with atypical localization: Heterotopic ossification associated with flexor hallucis longus.

Heterotopic ossification (HO), characterized by the formation of ectopic bone, is a benign mass observed in soft tissues. Depending on its location, it can cause symptoms beyond compression, such as mechanical blockage when associated with joints, leading to limitations in joint movements. In the majority of cases, involvement of the hip and elbow joints is common, while HO can sometimes be observed in atypical locations. Trauma, head injury, and spinal cord injuries are well-recognized risk factors for HO development. However, on rare occasions, in non-traumatic cases are identified without any known risk factors. Herein, we present a rare non-traumatic HO case associated with the flexor hallucis longus (FHL) tendon in a 58-year-old female patient. She complained of pain under the first toe of her right foot while wearing shoes for a year, and a mass was detected on the plantar surface of the foot along with limitation of movement in the first metatarsophalangeal joint. Further examinations revealed that the identified mass was a mature HO lesion. Surgical treatment was performed, and during one-year follow-up, the pain subsided, and joint movements returned to normal, resulting in a satisfactory outcome. In conclusion, although many cases of HO are associated with traumatic injuries, it can sometimes be idiopathic, as in our case, and rarely it is accompanied tendon such as FHL in the foot.

Characterization of flare-ups and impact of garetosmab in adults with fibrodysplasia ossificans progressiva: a post hoc analysis of the randomized, double-blind, placebo-controlled LUMINA-1 trial.

Fibrodysplasia ossificans progressiva (FOP) is an ultra-rare disorder, characterized by progressive heterotopic ossification (HO) and painful soft-tissue inflammatory flare-ups. This was a post hoc analysis from a phase 2 (NCT03188666) trial in which adults with FOP received intravenous anti-activin A antibody garetosmab 10 mg/kg or placebo every 4 wk over 28 wk (Period 1), followed by a 28-wk open-label treatment and extension (Periods 2 and 3). Here we describe flare-ups, their relationship to new HO lesions, and the impact of garetosmab on flare-ups. Volume of new HO lesions was measured by CT. Patient-reported flare-ups were defined by any 2 of the following: new onset of pain, swelling, joint stiffness, decrease in movement, or perceived presence of HO. Flare-ups were experienced by 71% (17/24) of placebo-treated patients, 59% (10/17) of whom developed a new HO lesion irrespective of flare-up location; 24% of flare-ups location-matched new HO lesions. Twenty-nine new HO lesions occurred in the placebo cohort by week 28, of which 12 (41%) occurred in the same location as new or ongoing flare-ups. A higher volume of newly formed heterotopic bone (week 28) occurred in placebo-treated patients who had experienced a prior flare-up vs those without (median [Q1:Q3] of 16.6 [12.0:31.1] vs 3.2 cm3). Garetosmab was previously shown to decrease patient-reported flare-up frequency in Period 1; here, garetosmab reduced the median (Q1:Q3) duration of patient-reported flares (15.0 [6.0:82.0] vs 48.0 [15.0:1.00] d) and the severity of flare-ups vs placebo. Frequency of corticosteroid use was numerically reduced in those treated with garetosmab (40.0%) vs placebo (58.3%). In this analysis, 71% of placebo-treated adults with FOP experienced flare-ups over 28 wk, which were associated with an increased volume of newly formed heterotopic bone. Garetosmab reduced the severity and duration of flare-ups, with effects sustained during the entire trial.

Fibrodysplasia ossificans progressiva (FOP) is a very rare genetic disorder caused by mutations in the ACVR1 gene. People with FOP experience growth of new bone in places where bone does not usually develop. Soft tissues (like skeletal muscles) and connective tissues (like tendons and ligaments) are gradually replaced by bone beyond the normal skeleton­a process called heterotopic ossification (HO). People with FOP experience flare-ups, which are painful swellings of the soft tissues. In this clinical study in people with FOP, we looked at the number of flareups, whether flareups were linked to new HO lesions, and the impact of garetosmab (a monoclonal antibody) on flareups. At random, about half the patients received placebo, or inactive drug, with the other half receiving garetosmab, the study drug. Of the patients who received placebo, 71% had flare-ups and 59% percent of those who had flare-ups also had a new HO lesion, which was not always related to the location of the flare-up. We have previously shown that garetosmab reduces the number of flareups patients report. In this study, we show that garetosmab reduces the length and pain severity of flare-ups too. The treatment effects were maintained for the whole study.

Identification of Risk Factors for Primary Osteoporosis: The Role of Cervical Ligament Ossification.

BACKGROUND Long-term clinical practice has suggested a possible association between ossification of cervical ligament (OCL) and primary osteoporosis (POP). However, there is a lack of relevant research data. This study aimed to clarify the potential relationship between OCL and POP, and propose new strategies for preventing the onset of POP. MATERIAL AND METHODS The study involved 107 patients. The patients' diagnosis included OCL (ossification of the posterior longitudinal ligament, ossification of the ligamentum flavum, and ossification of the nuchal ligament) and POP. Bone mineral density (BMD), types of OCL, types of ossification of posterior longitudinal ligament, age, sex, serum calcium, serum phosphorus, alkaline phosphatase, type I collagen amino-terminal extension peptide, type I collagen degradation products, osteocalcin N-terminal molecular fragments, 25-hydroxyvitamin D, and history of taking steroid drugs were collected. SPSS24.0 and GraphPad Prism 8 were used to obtain the risk factors for POP. RESULTS One-way analysis of variance found that OCL, ossification of posterior longitudinal ligament, alkaline phosphatase, and osteocalcin N-terminal molecular fragments had statistical significance on BMD of the femoral neck (P<0.05). The independent sample t test showed that patient sex had statistical significant effect on BMD (femoral neck) (P=0.036). Incorporating the above factors into multiple linear regression analysis, it was found that OCL, alkaline phosphatase, and osteocalcin N-terminal molecular fragments were risk factors affecting BMD of femoral neck (P<0.05). CONCLUSIONS OCL, osteocalcin N-terminal molecular fragments, and alkaline phosphatase are risk factors for POP.

Molecular Developmental Biology of Fibrodysplasia Ossificans Progressiva: Measuring the Giant by Its Toe.

When a genetic disease is characterized by the abnormal activation of normal molecular pathways and cellular events, it is illuminating to critically examine the places and times of these activities both in health and disease. Therefore, because heterotopic ossification (HO) in fibrodysplasia ossificans progressiva (FOP) is by far the disease's most prominent symptom, attention is also directed toward the pathways and processes of bone formation during skeletal development. FOP is recognizable by effects of the causative mutation on skeletal development even before HO manifests, specifically in the malformation of the great toes. This signature skeletal phenotype is the most highly penetrant, but is only one among several skeletal abnormalities associated with FOP. Patients may present clinically with joint malformation and ankylosis, particularly in the cervical spine and costovertebral joints, as well as characteristic facial features and a litany of less common, non-skeletal symptoms, all stemming from missense mutations in the ACVR1 gene. In the same way that studying the genetic cause of HO advanced our understanding of HO initiation and progression, insight into the roles of ACVR1 signaling during tissue development, particularly in the musculoskeletal system, can be gained from examining altered skeletal development in individuals with FOP. This review will detail what is known about the molecular mechanisms of developmental phenotypes in FOP and the early role of ACVR1 in skeletal patterning and growth, as well as highlight how better understanding these processes may serve to advance patient care, assessments of patient outcomes, and the fields of bone and joint biology.

Surgical Treatment of Heterotopic Ossification of the Bilateral Shoulder and Elbow Joints Caused by Burn Injury: A Case Report.

CASE: A 27-year-old man sustained chemical burns affecting 54% of his body caused by steam and acetic acid at a dyeing factory. He developed restricted bilateral elbow and shoulder motion because of heterotopic ossification (HO) beginning 3 months after the incident. The skin healed within 1 year, but ankylosis developed because of progressing ossification. We performed HO surgical excision in 4 stages. Two years after the final surgery, the function of both upper extremities had recovered. CONCLUSION: For HO caused by severe burns, improvement in upper extremity function can be achieved even if surgery is performed after skin healing.

Three-Dimensional Morphological Analysis of the Suprascapular Notch in Patients with Arthroscopic Rotator Cuff Repair.

Background: The morphology of the suprascapular notch (SSN) and the ossification of the superior transverse suprascapular ligament (STSL) are risk factors for injury of the suprascapular nerve (SN) during arthroscopic shoulder procedures. The purpose of the current study was to compare preoperative clinical and radiologic characteristics between patients with and without STSL ossification and to evaluate SSN morphology in patients who underwent arthroscopic rotator cuff repair using a 3-dimensional (3D) reconstructed model. Methods: Patients who underwent arthroscopic rotator cuff repair and were given a computed tomography (CT) scan from March 2018 to August 2019 were included in this study. Patients were divided into 2 groups: those without STSL ossification (group I) and those with STSL ossification (group II). Tear size of the rotator cuff and fatty infiltration of rotator cuff muscles were assessed in preoperative magnetic resonance imaging. The morphology of the SSN was classified following Rengachary's classification. The transverse and vertical diameters of the SSN and the distances from anatomical landmarks to the STSL were measured. All measurements were completed using a 3D CT reconstructed scapula model. Results: A total of 200 patients were included in this study. One hundred seventy-eight patients (89.0%) without STSL ossification were included in group I, and 22 patients (11.0%) with STSL ossification were included in group II. Group II showed a significantly advanced age (61.0 ± 7.4 vs. 71.0 ± 7.3 years, p < 0.001) and a shorter transverse diameter of SSN (10.7 ± 3.1 mm vs. 6.1 ± 3.7 mm, p < 0.001) than group I. In the logistic regression analysis, age was an independent prognostic factor for STSL ossification (odds ratio, 1.201; 95% confidence interval, 1.112-1.296; p < 0.001). Patients in type VI showed significantly shorter transverse diameters than other types (p < 0.001). The patient with type I showed a significantly shorter distance from the articular surface of the glenoid to the SSN than those with other types (p < 0.001). Conclusions: In the 3D morphological analysis, age was the independent factor associated with STSL ossification in patients who underwent arthroscopic rotator cuff repair. Type VI showed significantly shorter transverse diameters than other types. Type I showed a significantly shorter distance from the articular surface of the glenoid to the SSN than other types.