Idiopathic cardiac ossification with Chiari mesh in the right atrium: a case report and literature review.

Background: Idiopathic cardiac osseous metaplasia in the right atrium of a 9-year-old boy, accompanied by right atrial Chiari network and right pulmonary artery embolism. This case is rare and can easily be misdiagnosed. Case Description: We encountered a case of a 9-year-old boy with a 3.5 cm diameter neoplasm in the right atrium. Preoperative imaging diagnosis could not determine the nature of the tumor, and the initial clinical suspicion of cardiac myxoma. After admission, a cardiotomy to remove foreign bodies and a pulmonary artery thrombectomy were performed. Conclusions: Idiopathic cardiac osseous metaplasia is relatively rare, and it is even rarer to be accompanied by a Chiari network in the right atrium. Due to the location and characteristics of the lesion in this case, it is easy to be misdiagnosed as atrial myxoma in clinical practice. Whether it is idiopathic osseous metaplasia or myxoma, it needs to be performed surgical treatment and pathological examination can easily rule out the diagnosis of myxoma. However, as idiopathic cardiac metaplasia is difficult to encounter in clinical work and there are few reports in the literature, clinicians and pathologists need to consult more relevant literature. Learn to understand and master the disease through multi-party consultation.

Differential impact of chronic intermittent hypoxia and stress changes on condylar development.

OBJECTIVES: This study aimed to determine the effects of chronic intermittent hypoxia (CIH) and stress change (SC) on the development of the condyle in mouth breathing rats. DESIGN: A total of 120 4-week-old rats were randomly assigned to one of five groups. The control (Ctrl) group was the blank control and the intermittent nasal obstruction (INO) group was the positive control. Mild CIH (mCIH) and severe CIH (sCIH) groups were developed by adjusting environmental oxygen concentration and monitoring real-time blood oxygen saturation (SpO2). The SC group was developed using INO, increased environmental oxygen concentration, and real-time SpO2 monitoring. Six rats from each group were sacrificed for analysis at 0, 1, 2, or 4 weeks. RESULTS: Similar to the INO group, condyle and mandibular body development in the sCIH group, but not in the mCIH group, was significantly inhibited compared with the Ctrl group. The SC group had inhibited development of the condyle, especially of the posterior zone, but had minimal impact on the growth of the mandible. CONCLUSION: The inhibitory effects of CIH on the development of the condyle and mandibular body were SpO2-dose-dependent. When SC occurred, inhibited development was observed in the posterior zone of condyle but not the whole mandible. These findings provide important insights for targeted interventions that address the consequences of mouth breathing in children.

Dysplasia Epiphysealis Hemimelica (Trevor Disease) of the Talus in a Toddler: A Case Report With a Long Follow-up.

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Animal models of temporomandibular joint heterotopic ossification: a scoping review.

There are few animal models for heterotopic ossification of the temporomandibular joint (TMJ-HO). This scoping review provides an overview of current knowledge on the induction methods and specific conditions required to produce TMJ-HO in various animal models. Two independent reviewers selected papers from the PubMed, Web of Science, and Cochrane Library databases. The inclusion criteria were articles in English, in vivo studies, and a TMJ-HO induction method. Observational, in vitro, human studies, reviews, and book chapters were excluded. Twenty-four publications were included. HO was surgically, genetically, or chemically induced through single or combined defects in the condyle, articular disc, and temporal bone in animal models (sheep=9 studies, mouse=5, rat=4, rabbit=2, pig=2, goat=1, dog=1, monkey=1) specific for traumatic TMJ-HO (n=4), ankylosis (n=9), osteoarthritis (n=8), experimental disc perforation (n=1), status post-TMJ replacement (n=1), and status post bilateral sagittal split osteotomy (n=1). TMJ-HO remains challenging to study due to its multifactorial etiology and largely unknown pathogenesis, which varies between species. There is a need for more accurate, reproducible animal models that can be extrapolated to human TMJ-HO and a consolidated clinical classification system to allow for meaningful future research.

Clinical and radiological response of Maffucci related enchondromas to mutant IDH1 inhibitor Ivosidenib.

Ollier Disease (OD) and Maffucci syndrome (MS) is a rare bone disorder that affects the growth and development of the bones, with an estimated prevalence of 1 in 100,000 people. It is associated with somatic mosaicism of isocitrate dehydrogenase-1 (IDH1) or 2 (IDH2) pathogenic variants. Ivosidenib is indicated for the treatment of acute myeloid leukemia and locally advanced or metastatic cholangiocarcinoma and is currently investigated in low-grade glioma with a susceptible isocitrate dehydrogenase-1 (IDH1) pathogenic variant, but its effects in patients with OD or MS are unknown. We here report the first case of a patient with MS who was treated with Ivosidenib for recurrent IDH-1 mutated glioma. Besides the stabilization of the tumor size, the patient observed significant improvement in his enchondromas that became stiffer, with reduced pain, and significant modification of the mineralization of the enchondromas observed on X-rays. This first case report provides hope for the medical management of patients suffering because of OD or MS. Future clinical research is urgently needed to evaluate long-term benefit risk profile of IDH inhibitors in these rare diseases.

Ossification of neurofibroma in neurofibromatosis type 1, a case report of a rare presentation.

INTRODUCTION: Although musculoskeletal involvement of Neurofibromatosis type 1 (NF1) has been well documented, bone formation, or ossification, within neurofibroma, has been scarcely documented in literature. Here, we report a rare case of ossified neurofibroma in a patient with long history of NF1. PRESENTATION OF CASE: 73-Year-old female with childhood-onset NF1 and surgical history of resection for multiple neurofibromas, presented with right ptosis and eyebrow ptosis. A growing tumor on the right eyebrow was surgically resected. Microscopically, the dermal tumor consists of bland spindle cells with thin, wavy nuclei, without atypia, showing S100 immunoreactivity, consistent with neurofibroma. Multiple metaplastic bone formation composed of mature bone trabeculae surrounding adipose tissue were apparent. DISCUSSION: Up to date, ossification of neurofibroma has been scarcely reported in literature. The etiology is unclear but might involve the response to chronic stress and tissue damage over the years, and/or might indicate the potential differentiation plasticity of mesenchymal stem cell-like population. CONCLUSION: The unusual presentation of ossification provides insights on the pathogenesis and differentiation plasticity of neurofibroma.

Patient-Reported Outcomes and Patient Satisfaction Following Surgery for Thoracic Myelopathy.

Introduction: The association between postoperative patient-reported outcomes (PROs) and patient satisfaction remains poorly defined in patients undergoing surgery for thoracic myelopathy. This study aimed to investigate PROs and patient satisfaction following surgical intervention for thoracic myelopathy. Methods: A prospective cohort of 133 patients who underwent surgery for thoracic myelopathy at 13 hospitals between April 2017 and August 2021 was enrolled. Patient demographics and perioperative complications were recorded. PROs were assessed using questionnaires administered preoperatively and 1 year postoperatively, including the EuroQol-5 dimension, physical and mental component summaries of the 12-item Short-Form Health Survey, Oswestry Disability Index, and numerical rating scales for low back, lower extremity, and plantar pain. Patients were categorized into two groups: satisfied (very satisfied, satisfied, and slightly satisfied) and dissatisfied (neither satisfied nor dissatisfied, slightly dissatisfied, dissatisfied, and very dissatisfied). Results: The mean age of the patients was 66.5 years, comprising 87 men and 46 women. The most common diagnoses were ossification of the ligamentum flavum (48.8%) and thoracic spondylotic myelopathy (26.3%). Seventy-four (55.6%) and 59 (44.3%) patients underwent decompression surgery and underwent decompression with fusion, respectively. Eight patients required reoperation due to postoperative surgical site infection, hematoma, and insufficient decompression in four, three, and one patient. Ninety (67.7%) patients completed both the preoperative and postoperative PRO questionnaires, all of which demonstrated significant improvement. Among them, 58 (64.4%) and 32 (35.6%) reported satisfaction and dissatisfaction with their treatment, respectively. The satisfied group showed superior improvement in PROs than the dissatisfied group, although there were no significant differences in complication rates between the two groups. Conclusions: The 64.4% satisfaction rate observed in patients undergoing surgery for thoracic myelopathy was lower than that reported in previous studies on cervical or lumbar spine surgery. The dissatisfied group exhibited significantly poorer quality of life (QOL) and higher pain scores than the satisfied group.

Soft tissue aneurysmal bone cyst presenting as an enlarging neck mass: Case report and review of the head and neck literature.

Soft tissue aneurysmal bone cysts (STABCs) are rare neoplasms histopathologically identical to aneurysmal bone cysts. These benign lesions are characterized by thin, peripheral ossification and no skeletal continuity. STABC may be difficult to distinguish from myositis ossificans (MO) and malignant entities from imaging and fine needle aspiration, due to rarity and overlapping features. We present a case of a STABC occurring in the paraspinal cervical muscles. The imaging, histopathology, molecular analysis, and treatment are discussed. Four other published cases of STABC of the head and neck are reviewed.

Arthroscopic Resection of Symptomatic Ossicles of the Medial Malleolus Combined With Deltoid Ligament Repairs in Athletes: A Report of Three Cases.

We report three cases of young athletes with symptomatic ossicles of the medial malleolus who underwent arthroscopic resections of the ossicle combined with deltoid ligament repair. A differential diagnosis of the ossicles beneath the medial malleolus, accessory ossification center, avulsion fracture resulting in pseudoarthrosis, and accessory bone such as os subtibiale has been proposed. However, it is difficult to differentiate them clearly. Most of these ossicles are asymptomatic, although they can cause chronic medial ankle pain, especially in young athletes who require surgical treatment. All three patients had pain in the distal part of the medial malleolus, which restricted their sports activities. Plain radiographs of all three cases revealed a well-defined, round-shaped bony lesion beneath the medial malleolus. Ultrasonographic imaging, magnetic resonance imaging, and arthroscopic findings revealed that ligament attachment to the ossicle varies in volume and type according to the cases. In other words, the mechanisms through which the existence of the ossicles affects the stability of the ankle joint and foot alignment are different in each case, indicating that deltoid ligament repair is necessary according to the cases. In all three cases, we performed arthroscopic resections of ossicles combined with deltoid ligament repairs, achieving favorable short-term clinical outcomes.

The involvement of epidural fat in ossification of the ligamentum flavum: From the perspective of exosomal proteome.

Ossification of the ligamentum flavum (OLF) is the primary etiology of thoracic spinal stenosis. The functional properties of epidural fat (EF), an adipose tissue located in close proximity to ligamentum flavum (LF), have been scarcely investigated. The metabolic state of adipocytes significantly influences their functionality, and exosomes play a pivotal role in intercellular communication. This study aimed to investigate the role of EF-derived exosomes in OLF and characterize their protein profile by proteomics analysis. Our findings demonstrate that exosomes obtained from EF adjacent to OLF possess the ability to enhance osteogenesis of fibroblasts in vitro. Furthermore, proteomics analysis revealed metabolic dysfunction in EF adipocytes and identified lactate dehydrogenase A (LDHA) as a potential mediator involved in the development of OLF. This study provides new insights into the pathogenic mechanism underlying OLF and offers a theoretical basis for preventing and treating ligament ossification.

Perioperative complications and cost of posterior decompression with fusion in thoracic spine for ossification of the posterior longitudinal ligament and ossification of the ligamentum flavum -a comparative study using a national inpatient database.

BACKGROUND: Although posterior decompression with fusion (PDF) are effective for treating thoracic myelopathy, surgical treatment has a high risk of various complications. There is currently no information available on the perioperative complications in thoracic ossification of the longitudinal ligament (T-OPLL) and thoracic ossification of the ligamentum flavum (T-OLF). We evaluate the perioperative complication rate and cost between T-OPLL and T-OLF for patients underwent PDF. METHODS: Patients undergoing PDF for T-OPLL and T-OLF from 2012 to 2018 were detected in Japanese nationwide inpatient database. One-to-one propensity score matching between T-OPLL and T-OLF was performed based on patient characteristics and preoperative comorbidities. We examined systemic and local complication rate, reoperation rate, length of hospital stays, costs, discharge destination, and mortality after matching. RESULTS: In a total of 2,660 patients, 828 pairs of T-OPLL and T-OLF patients were included after matching. The incidence of systemic complications did not differ significantly between the T-OPLL and OLF groups. However, local complications were more frequently occurred in T-OPLL than in T-OLF groups (11.4% vs. 7.7% P = 0.012). Transfusion rates was also significantly higher in the T-OPLL group (14.1% vs. 9.4%, P = 0.003). T-OPLL group had longer hospital stay (42.2 days vs. 36.2 days, P = 0.004) and higher medical costs (USD 32,805 vs. USD 25,134, P < 0.001). In both T-OPLL and T-OLF, the occurrence of perioperative complications led to longer hospital stay and higher medical costs. While fewer patients in T-OPLL were discharged home (51.6% vs. 65.1%, P < 0.001), patients were transferred to other hospitals more frequently (47.5% vs. 33.5%, P = 0.001). CONCLUSION: This research identified the perioperative complications of T-OPLL and T-OLF in PDF using a large national database, which revealed that the incidence of local complications was higher in the T-OPLL patients. Perioperative complications resulted in longer hospital stays and higher medical costs.

Intraoperative and Postoperative Ultrasonographic Spinal Cord Evaluation of Cervical Double-Door Laminoplasty.

BACKGROUND: Ultrasonography is a useful tool for the localization, morphology, and characterization of lesions and is increasingly being applied to spinal cord evaluation in cervical spine diseases. However, in conventional cervical laminoplasty, detailed evaluation is difficult because of ultrasound attenuation. Therefore, the purpose of this study was to perform a cervical laminoplasty using a modified surgical technique and evaluate the spinal cord. METHODS: The spinal cord was evaluated intraoperatively and one week postoperatively in 11 patients with cervical compressive myelopathy. Double-door laminoplasty was selected as the surgical method, and the shape and placement of the bone graft between the expanded laminas were devised to reduce ultrasonic attenuation, such that there was a large space in which the dura mater was visible. RESULTS: Intraoperative and postoperative spinal cord decompression, claudication, and pulsation were confirmed in all cases. A more precise diagnosis was possible using ultrasound attenuation using the grafted bone between the laminas as an indicator. CONCLUSION: Intraoperative and postoperative spinal cord decompression status and wave patterns of modified cervical double-door laminoplasty can be evaluated using ultrasonography. Ultrasound-based evaluations of the spinal cord may provide new insights.

Embryonic development and cranial ossification of the Japanese Aodaisho, Elaphe climacophora (Serpentes: Colubridae): with special reference to the prootic bone and auditory evolution in snakes.

Snakes show remarkably deviated "body plan" from other squamate reptiles. In addition to limb loss, they have accomplished enormous anatomical specialization of the skull associated with the pit organs and the reduction of the tympanic membranes and auditory canals in the outer ears. Despite being the most diverse group of snakes, our knowledge of the embryonic staging for organogenesis and cranial ossification has been minimal for Colubridae. Therefore, in the present observation, we provide the first embryonic description of the Japanese rat snake Elaphe climacophora. We based our study on the Standard Event System (SES) for external anatomical characters and on a description of the cranial ossification during post-ovipositional development. We further estimated the relative ossification timing of each cranial bony element and compared it with that of selected other snakes, lizards, turtles, and crocodilians. The present study shows that the relative ossification timing of the palatine and pterygoid bones is relatively early in squamates when compared to other reptiles, implying the developmental integration as the palate-pterygoid complex in this clade and functional demands for the unique feeding adaptation to swallow large prey with the help of their large palatine and pterygoid teeth. Furthermore, unlike in species with pit organs, the prootic bone of Ela. climacophora is expanded to provide articulation with the supratemporal, thereby contributing to the hearing system by detecting substrate vibration. We also demonstrate that the relative timing of the prootic ossification is significantly accelerated in colubrids compared to snakes with pit organs. Our finding suggests that the temporal changes of the prootic ossification underpin the evolution of the perception of the ground-bourne sound signals among snakes.

Regulation mechanism of endochondral ossification in Rana zhenhaiensis during metamorphosis based on histomorphology and transcriptome analyses.

Endochondral ossification plays a crucial role in the limb development of amphibians. This study explored the ossification sequence in the hindlimb of Rana zhenhaiensis tadpoles and the correlation between thyroid hormones (THs) and endochondral ossification via histomorphology and transcriptional analyses. Our results suggest that ossification of the femur and tibiofibula was initiated during the period of high THs activity (metamorphosis climax). In addition, the results of differentially expressed gene analyses in the hindlimb and tail showed that systemic factors, transcription factors, and locally secreted factors interacted with each other during the metamorphosis climax to regulate the occurrence of endochondral ossification. These results will enrich the morphological data of anurans and provide scientific reference for the evolutionary history of vertebrates.

Radiographic evaluation of the forelimb bone development in maned wolves (Chrysocyon brachyurus).

This study aimed to assess the fusion of growth plates and the development of secondary ossification centres in the forelimb bones of maned wolves (Chrysocyon brachyurus), contrasting the findings with established data from domestic dogs. Three maned wolves, comprising one male and two females, initially aged between 3 and 4 months, were subjected to monthly radiographic evaluations until 10-11 months of age, followed by bimonthly assessments until 18-19 months of age, encompassing both forelimbs. The closure times of growth plates were observed as follows: supraglenoid tubercle (7-8 months), proximal humerus (17-19 months), distal humerus (8-9 months), medial epicondyle of the humerus (8-9 months), proximal ulna (9-10 months), proximal radius (13-15 months), distal ulna (13-15 months) and distal radius (17-19 months). Statistical analysis revealed significant differences in the areas of secondary ossification centres in the proximal epiphyses of the humerus and radius, respectively, observed from the initial evaluation at 8-9 months and 6-7 months. Conversely, the epiphyses of the supraglenoid tubercle, distal humerus, proximal ulna, distal ulna, medial epicondyle of the humerus and distal radius did not exhibit significant area differences between 3-4 months and 4-5 months, yet notable distinctions emerged at 5-6 months. In summary, while the radiographic appearance of epiphyseal growth plates and secondary ossification centres in maned wolves resembles that of domestic dogs, closure times vary. These findings contribute to understanding the dynamics of epiphyseal growth plates in this species.

Heterotopic ossification of the elbow joint in a child: Successful surgical resection - A case report.

Background: This case report describes the occurrence of a rare heterotopic ossification of the elbow joint in a child, caused by inappropriate movement after trauma. A successful operation to remove heterotopic ossification was described in the report with satisfactory results. Case presentation: A 7-year-old boy suffered a supracondylar fracture of the humerus after an accidental fall, and after immobilization with a cast, improper movement resulted in heterotopic ossification of the elbow joint, which severely affected joint function. The heterotopic ossification was surgically removed and a complete recovery was demonstrated at 18 months follow-up. The heterotopic ossification was successfully removed with good elbow function and no recurrence at 18 months follow-up. Conclusions: The purpose of this report is to show the good results with surgical treatment of heterotopic ossification of the elbow joint in children,when conservative treatment does not work.

Malignant character of an ossified posterior longitudinal ligament in a hyperflexion injury: A case report.

The present study reports the case of a 50-year-old obese male with ankylosing spondylitis, Scheuermann's kyphosis. The patient was asymptomatic concerning the ectopic ossification of the posterior longitudinal ligament (OPLL) at the cervical spine; he developed quadriparesis and respiratory insufficiency following minor head trauma. Even though trauma to the cervical spine in patients with OPLL is common, to the best of our knowledge, this is the first reported case of an extensive osteophyte with a lethal outcome after syncope. In rare occasions, it may be present with syncope and potentially lethal outcomes, particularly when precipitated by trauma. Therefore, the management of OPLL with significant canal stenosis should not be unnecessarily delayed.

Increased combined anteversion is associated with larger posterior acetabular rim ossification in patients with femoroacetabular impingement syndrome.

PURPOSE: (1) To determine the prevalence, magnitude and distribution pattern of acetabular rim ossification in patients with femoroacetabular impingement syndrome (FAIS) and (2) to determine the association between acetabular rim ossification and rotational abnormalities of the hip. METHODS: Patients underwent hip arthroscopic surgery for FAIS at our institute between January 2021 and May 2022 were retrospectively reviewed. Patients were included if preoperative computed tomography (CT) images of the operated hip and ipsilateral distal femur were available for the measurement of femoral and acetabular anteversion. The presence and size of acetabular rim ossification were evaluated on coronal CT sections for the superior half of the acetabulum on each clockface location. The associations between acetabular rim ossification and radiographic parameters of hip rotational morphology were examined. RESULTS: A total of 214 hips were included. Acetabular rim ossification was found in 167 hips (78%) and the most common locations were 10 and 11 o'clock. Patients presenting with acetabular rim ossification had a mean size of 4.6 ± 1.6 mm. It was the largest at 9 o'clock position (4.9 ± 2.2 mm), with a decreasing trend in size from posterior to anterior. Logistics regression analysis found age was associated with the occurrence of posterior ossification (p = 0.002). Linear regression analysis found age (p = 0.049) and male sex (p < 0.001) were significantly correlated with the size of ossification. Patients with increased cranial combined anteversion had larger posterior ossification than patients with normal and decreased cranial combined anteversion (4.2 ± 2.9 vs. 3.1 ± 2.5 mm, p = 0.016; 4.2 ± 2.9 vs. 2.5 ± 2.4 mm, p = 0.005). CONCLUSION: Increased combined anteversion is associated with greater posterior acetabular rim ossification. The presence and size of acetabular rim ossification are positively associated with older age and male sex. LEVEL OF EVIDENCE: Level III.

Foxc1 and Foxc2 regulate growth plate chondrocyte maturation towards hypertrophy in the embryonic mouse limb skeleton.

The Forkhead box transcription factors Foxc1 and Foxc2 are expressed in condensing mesenchyme cells at the onset of endochondral ossification. We used the Prx1-cre mouse to ablate Foxc1 and Foxc2 in limb skeletal progenitor cells. Prx1-cre;Foxc1Δ/ Δ;Foxc2Δ/Δ limbs were shorter than controls, with worsening phenotypes in distal structures. Cartilage formation and mineralization was severely disrupted in the paws. The radius and tibia were malformed, while the fibula and ulna remained unmineralized. Chondrocyte maturation was delayed with fewer Indian Hedgehog-expressing, prehypertrophic chondrocytes forming and a smaller hypertrophic chondrocyte zone. Later, progression out of chondrocyte hypertrophy was slowed, leading to an accumulation of COLX-expressing hypertrophic chondrocyte zone and formation of a smaller primary ossification center with fewer osteoblast progenitor cells populating this region. Targeting Foxc1 and Foxc2 in hypertrophic chondrocytes with Col10a1-cre also resulted in an expanded hypertrophic chondrocyte zone and smaller primary ossification center. Our findings suggest that Foxc1 and Foxc2 direct chondrocyte maturation towards hypertrophic chondrocyte formation. At later stages, Foxc1 and Foxc2 regulate function in hypertrophic chondrocyte remodelling to allow primary ossification center formation and osteoblast recruitment.

Comparative analysis of 'functional excision' of heterotopic ossification around the hip - computed tomography (CT) navigation guided versus conventional excision.

PURPOSE: We aim to analyse and compare the efficacy of the excision of HO around the hip with and without CT-guided navigation. We also aim to compare the functional outcome between CT navigation guided versus conventional excision of HO. PATIENTS AND METHODS: This study is a retrospective analysis of prospectively collected data from 2015 to 2022. There were 23 patients (24 hips) in the final cohort. Intraoperative CT navigation guided excision was performed in 7 hips and conventional excision of HO was done in 17 hips. The HO was classified by Brooker's grading in radiographs. CT scan was taken preoperatively in all patients to exactly identify the volume, location and preoperative planning. The functional outcome was analysed according to Harris Hip Score (HHS) and International Hip Outcome Tool (iHOT) for self-ambulatory patients and improvement in the sitting or nursing care was assessed in patients mobilising with wheelchair or walker support. Any complications or recurrence noted postoperatively and in follow-up were recorded. RESULTS: The mean follow-up was 41.2 months in the CT navigation-guided excision group and 55 months in the conventional excision group. According to Brooker's grading, grade IV was present in 20 hips and grade III in four hips. Twelve patients were self-ambulatory and the other 12 patients were requiring support for mobilisation. There was a significant improvement in the HHS from 21.3 ± 3.7, 18.3 ± 2.5 preoperatively to 75.2 ± 8.3, 72.2 ± 4.3 postoperatively in the CT navigation guided and conventional group respectively (p < 0.001) in the self-ambulatory group. There was one anterior wall and one partial posterior wall fracture in the conventional group. One patient in the conventional group had a deep infection and recurrence. One patient had a superficial infection and another had superficial vein thrombosis in the CT guided excision group. CONCLUSION: Intraoperative CT navigation helps to exactly localize the HO and facilitates safe excision. Functional excision of the HO leads to better nursing care and functional outcomes between both groups.