Global alignment taking into account the cervical spine with odontoid hip axis angle (OD-HA).

INTRODUCTION: Global alignment analysis is of upmost importance in adult spinal deformity patients (ASD). Numerous parameters exist in the literature to measure global alignment based upon C7 or T1. One common limitation of these parameters is that they neglect the cervical segment which is essential in spinal compensatory mechanisms and in horizontal gaze preservation. A recent stereoradiography analysis of asymptomatic subjects introduced a new 3D parameter (ODHA), defined as the angle between the vertical reference line and the line joining the odontoid tip (OD) to hip axis center (HA). Thus, the goal of this study was to analyze 3D global alignment of ASD patients using the new parameter odontoid hip axis angle and its relationship to other spinal parameters. METHODS: In this prospective study, 90 adult patients with lumbar scoliosis (Cobb > 20°) were included. All subjects underwent low dose biplanar X-rays with 3D spinal reconstructions. Based on published normative values of ODHA, we defined abnormally high value as mean ODHA of asymptomatic subject + 2SD (i.e., ODHA > 6.1°). Values of 3D radiographic parameters and ODI were compared between patients with ODHA > 6.1° and < 6.1°. RESULTS: Mean ODHA was 5+/- 3.6° (0.4° to 18.6°). 22 patients had abnormally high ODHA. They were older than the 68 other patients (68+/- 9y vs 53+/- 14y, p = 0.001), without any significant difference in terms of sex, BMI and rate of rotatory subluxation (54% vs 62%, p = 0.06). However, coronal and sagittal deformity was more important in patients with abnormal ODHA (larger Cobb angle, coronal malalignment, pelvic tilt and lower lumbar lordosis). Patients with abnormal ODHA had significantly worst ODI (50+/- 23 vs 30+/- 18, p = 0.0005). CONCLUSION: Extreme values of ODHA are observed in significantly older patients with significant functional impairment. In addition, in these patients with sagittal malalignment with loss of lumbar lordosis, who recruit compensatory mechanisms such as pelvic retroversion, the cervical area is also involved with a posture in cervical hyperlordosis to maintain the head over the pelvis. Thus, ODHA is an interesting parameter allowing a more comprehensive alignment measurement taking into account the mechanisms of compensation of the cervical spine to the pelvis.

Assessment of an AI Aid in Detection of Adult Appendicular Skeletal Fractures by Emergency Physicians and Radiologists: A Multicenter Cross-sectional Diagnostic Study.

Background The interpretation of radiographs suffers from an ever-increasing workload in emergency and radiology departments, while missed fractures represent up to 80% of diagnostic errors in the emergency department. Purpose To assess the performance of an artificial intelligence (AI) system designed to aid radiologists and emergency physicians in the detection and localization of appendicular skeletal fractures. Materials and Methods The AI system was previously trained on 60 170 radiographs obtained in patients with trauma. The radiographs were randomly split into 70% training, 10% validation, and 20% test sets. Between 2016 and 2018, 600 adult patients in whom multiview radiographs had been obtained after a recent trauma, with or without one or more fractures of shoulder, arm, hand, pelvis, leg, and foot, were retrospectively included from 17 French medical centers. Radiographs with quality precluding human interpretation or containing only obvious fractures were excluded. Six radiologists and six emergency physicians were asked to detect and localize fractures with (n = 300) and fractures without (n = 300) the aid of software highlighting boxes around AI-detected fractures. Aided and unaided sensitivity, specificity, and reading times were compared by means of paired Student t tests after averaging of performances of each reader. Results A total of 600 patients (mean age ± standard deviation, 57 years ± 22; 358 women) were included. The AI aid improved the sensitivity of physicians by 8.7% (95% CI: 3.1, 14.2; P = .003 for superiority) and the specificity by 4.1% (95% CI: 0.5, 7.7; P < .001 for noninferiority) and reduced the average number of false-positive fractures per patient by 41.9% (95% CI: 12.8, 61.3; P = .02) in patients without fractures and the mean reading time by 15.0% (95% CI: -30.4, 3.8; P = .12). Finally, stand-alone performance of a newer release of the AI system was greater than that of all unaided readers, including skeletal expert radiologists, with an area under the receiver operating characteristic curve of 0.94 (95% CI: 0.92, 0.96). Conclusion The artificial intelligence aid provided a gain of sensitivity (8.7% increase) and specificity (4.1% increase) without loss of reading speed. © RSNA, 2021 Online supplemental material is available for this article.

Volume of spinopelvic muscles: comparison between adult spinal deformity patients and asymptomatic subjects.

PURPOSE: Spinal muscles are a major component of posture in spinal pathologies and changes to the spine with aging. Specifically, spinopelvic muscles may compensate for underlying anomalies such as pelvic retroversion, knee flexion, and cervical or thoracic spinal balance abnormalities. To increase understanding between muscular characteristics and compensatory mechanisms, this study aimed to compare the volume of spinopelvic muscles in adults with a spinal deformity (ASD) to a control group of well-aligned adult subjects. METHODS: Twenty-eight lumbar ASD patients [Cobb angle > 20°, > 40 years old (yo)] were prospectively included and compared to 35 normal subjects divided into 2 different groups: one group of young (Y) subjects (n = 23, < 20 yo) and one group of old (O) subjects (n = 12, > 40 yo). All subjects had a fat/water separation MRI (from C7 to the knees). Volumetric 3D reconstructions of 30 spinopelvic muscles were performed and muscles volumes were compared. RESULTS: Mean age was 60 ± 16 yo, without significant differences between the ASD and O groups (57 ± 11 yo). Age and BMI were smaller in the young group. Mean Cobb angle of the ASD group was 45 ± 11°. Comparing the ASD and O groups, total muscular volume was similar; however, erector spinae (0.24 ± 0.06 vs 0.68 ± 0.08 dm3, p = 0.001), iliopsoas (0.49 ± 0.09 vs 0.60 ± 0.09 dm3, p = 0.001) and obliquus (0.42 ± 0.08 vs 0.50 ± 0.08 dm3, p = 0.02) were significantly smaller in the ASD group. Comparing the Y and the ASD groups, total muscular volume was higher in the Y group than the ASD group (+ 3.3 dm3, p = 0.003) and erector spinae (0.24 ± 0.06 vs 0.74 ± 0.08, p = 0.0001), gluteus medius (0.51 ± 0.07 vs 0.62 ± 0.13, p = 0.01) and vastus lateralis (1.33 ± 0.21 vs 2.08 ± 0.29, p = 0.001) were significantly bigger in the Y group. CONCLUSION: This is the first study to compare volume of spinopelvic muscles between ASD patients and a control group without spinal deformity. Our results demonstrate that muscular degeneration has a double origin: aging and deformity. Erector spinae, iliopsoas, and obliquus are the muscles most affected by degeneration.

Relationships between radiographic parameters and spinopelvic muscles in adult spinal deformity patients.

INTRODUCTION: While the clinical impact of coronal and sagittal alignment in adult spinal deformity (ASD) patients (pts) is established, there is a paucity of data in terms of axial plane deformity and potential association between muscle degeneration and 3D deformity. The purpose of this study was to analyze spinopelvic muscles characteristics in association with the 3D deformity of ASD patients. METHODS: This is a prospective cohort study; primary lumbar scoliosis patients (Cobb > 20°) were enrolled and sustained a low-dose X-rays with 3D spinal reconstructions and a fat/water separation MRI (from C7 to the knee). Volumetric 3D reconstructions and fat infiltration (FI) of 6 muscles groups were performed. Relationships between muscular data, radiographic parameters and health-related quality of life were investigated. Patients were stratified and compared based on the SRS classification, the odontoid-hip axis (ODHA) angle (> or < 6.1°) and occurrence of rotatory subluxation. RESULTS AND DISCUSSION: Twenty-eight patients were enrolled with a mean age of 60 ± 16yo and mean body mass index of 26 ± 4 kg/m2 without differences between groups. There were a moderate sagittal malalignment and a Cobb angle of 45 ± 11° (table). Muscular volume was smaller in patients with more severe deformity (p > 0.05). Pts with ODHA > 6.1° or pelvic incidence minus lumbar lordosis > 10° had significantly higher FI for the 6 muscular groups, patients with pelvic tilt > 20° had significantly higher FI for erector spinae, hip flexors and extensors (p < 0.05). SF36-PCS significantly correlated with the muscular volume; SRS and Oswestry disability index correlated with the erectors spinae volume (p < 0.05). CONCLUSION: This study analyzed for the first time the relationship between 3D radiographic parameters and muscular characteristics in ASD. Sagittal malalignment is associated with increased FI and decreased muscle volumes with poor outcomes. These slides can be retrieved under Electronic Supplementary Material.

CHRNG-related nonlethal multiple pterygium syndrome: Muscle imaging pattern and clinical, histopathological, and molecular genetic findings.

Mutations in the CHRNG gene cause autosomal recessive multiple pterygium syndrome (MPS). Herein we present a long-term follow-up of seven patients with CHRNG-related nonlethal MPS and we compare them with the 57 previously published patients. The objective is defining not only the clinical, histopathological, and molecular genetic characteristics, but also the type and degree of muscle involvement on whole-body magnetic resonance imaging (WBMRI). CHRNG mutations lead to a distinctive phenotype characterized by multiple congenital contractures, pterygium, and facial dysmorphism, with a stable clinical course over the years. Postnatal abnormalities at the neuromuscular junction were observed in the muscle biopsy of these patients. WBMRI showed distinctive features different from other arthrogryposis multiple congenita. A marked muscle bulk reduction is the predominant finding, mostly affecting the spinal erector muscles and gluteus maximus. Fatty infiltration was only observed in deep paravertebral muscles and distal lower limbs. Mutations in CHRNG are mainly located at the extracellular domain of the protein. Our study contributes to further define the phenotypic spectrum of CHRNG-related nonlethal MPS, including muscle imaging features, which may be useful in distinguishing it from other diffuse arthrogryposis entities.

Muscular MRI-based algorithm to differentiate inherited myopathies presenting with spinal rigidity.

OBJECTIVES: Inherited myopathies are major causes of muscle atrophy and are often characterized by rigid spine syndrome, a clinical feature designating patients with early spinal contractures. We aim to present a decision algorithm based on muscular whole body magnetic resonance imaging (mWB-MRI) as a unique tool to orientate the diagnosis of each inherited myopathy long before the genetically confirmed diagnosis. METHODS: This multicentre retrospective study enrolled 79 patients from referral centres in France, Brazil and Chile. The patients underwent 1.5-T or 3-T mWB-MRI. The protocol comprised STIR and T1 sequences in axial and coronal planes, from head to toe. All images were analyzed manually by multiple raters. Fatty muscle replacement was evaluated on mWB-MRI using both the Mercuri scale and statistical comparison based on the percentage of affected muscle. RESULTS: Between February 2005 and December 2015, 76 patients with genetically confirmed inherited myopathy were included. They were affected by Pompe disease or harbored mutations in RYR1, Collagen VI, LMNA, SEPN1, LAMA2 and MYH7 genes. Each myopathy had a specific pattern of affected muscles recognizable on mWB-MRI. This allowed us to create a novel decision algorithm for patients with rigid spine syndrome by segregating these signs. This algorithm was validated by five external evaluators on a cohort of seven patients with a diagnostic accuracy of 94.3% compared with the genetic diagnosis. CONCLUSION: We provide a novel decision algorithm based on muscle fat replacement graded on mWB-MRI that allows diagnosis and differentiation of inherited myopathies presenting with spinal rigidity. KEY POINTS: • Inherited myopathies are rare, diagnosis is challenging and genetic tests require specialized centres and often take years. • Inherited myopathies are often characterized by spinal rigidity. • Whole body magnetic resonance imaging is a unique tool to orientate the diagnosis of each inherited myopathy presenting with spinal rigidity. • Each inherited myopathy in this study has a specific pattern of affected muscles that orientate diagnosis. • A novel MRI-based algorithm, usable by every radiologist, can help the early diagnosis of these myopathies.

Clinical and imaging hallmarks of the MYH7-related myopathy with severe axial involvement.

INTRODUCTION: MYH7 gene mutations are related to a heterogeneous group of skeletal and cardiac myopathies. METHODS: We evaluated clinical and muscle MRI changes in patients with mutations in the rod domain of MYH7, including 1 with mosaicism and 3 with novel missense mutations. RESULTS: Patients presented in childhood with a distal and axial phenotype. Biopsy findings were variable. Half of the cases displaying some type of core pathology, including minicores and eccentric cores. Most patients demonstrated internal bands of infiltration ("inverted-collagen-VI sign") in multiple muscles, particularly the soleus, and prominent atrophy and fatty infiltration of the tongue and the paraspinal, gluteus minimus, sartorius, gracilis, tibialis anterior, and extensor digitorum longus muscles. DISCUSSION: Muscle imaging findings in patients with axial involvement provide significant clues permitting the distinction between MYH7-related myopathies and other axial myopathies such as those related to SEPN1 and LMNA genes. Muscle Nerve 58: 224-234, 2018.

Pre-surgical CT-assessment of neurogenic myositis ossificans of the hip and risk factors of recurrence: a series of 101 consecutive patients.

BACKGROUND: Neurogenic Myositis Ossificans (NMO) is a rare disabling pathology characterized by peri-articular heterotopic ossifications following severe peripheral or central nervous system injuries. It results in ankylosis and vessels or nerves compressions. Our study aimed to describe the pre-operative findings of patients with NMO of the hip using biphasic computerized tomography (CT). METHODS: Between 2006 and 2012, we retrospectively analyzed 101 consecutive patients with hip NMO. We analyzed all CTs and surgical reports following a standardized grid depicting the osteoma and its relations with joint capsule, vessels and nerves and bone mineralization. We studied surgical complications and recurrence during follow-up. Chi2-test and Fischer's test were performed to compare qualitative values with respectively normal and non-normal distribution. Quantitative values were analyzed with a one factor analysis of variance (ANOVA) test. Agreement between pre-surgical CT and surgical observations was evaluated with Cohen's kappa test. RESULTS: Correlation between pre-operative CT and surgical findings was excellent regarding relationships with vessels (0,82) and was good concerning relationships with sciatic nerves (0.62) and with joint capsule (0.68). Close contact or disruption of joint capsule (p = 0.005), joint space narrowing (p = 0.007) and bone demineralization (p < 0.001) were correlated with NMO recurrence. CONCLUSIONS: Biphasic enhanced-CT allows pre-operative assessment of NMO with good correlation to surgical observations and helps prevent surgical complications.

Subcutaneous Phaeohyphomycosis Due to Pyrenochaeta romeroi Mimicking a Synovial Cyst.

Opportunistic subcutaneous fungal infections are increasing nowadays due to the growing number of medical conditions causing immunosuppression, especially organ transplant. The incidence rate of subcutaneous phaeohyphomycosis is very low. Most studies found are case reports. They showed a wide variation of clinical presentations. Pyrenochaeta romeroi, a fungus from the Dematiaceae group is a saprophyte found in soil and plants and a possible causative agent of phaeohyphomycosis. We present a rare case of subcutaneous phaeohyphomycosis caused by P. romeroi mimicking a synovial cyst in a diabetic patient.