Magnetic resonance imaging overestimates patellar height compared with radiographs.

PURPOSE: To evaluate the inter-observer and inter-method reliability for patellar height measurements between conventional radiographs (CR) and magnetic resonance imaging (MRI) using one or two slices. METHODS: This was a reliability study, with 60 patients divided in two groups: 30 patients with patellar instability (patella group) and 30 patients with anterior cruciate ligament or meniscus injury (control group). CR and MRI were evaluated by two independent observers. Insall-Salvati index (IS) and Caton-Deschamps index (CD) were measured using three different methods: CR, one-slice MRI or two-slice MRI. Intra-class correlation coefficients (ICC) were calculated for inter-observer reliability and inter-method reliability. Bland-Altman agreement was also calculated. RESULTS: The inter-observer reliability was very good for the IS with ICCs of 0.93, 0.84 and 0.82, for the CR, one-slice MRI and two-slice MRI, respectively. Similarly, for the CD the ICCs were good, 0.76, 0.80 and 0.75 for the CR, one-slice MRI and two-slice MRI, respectively. No differences were found between the patella and the control group. The inter-method analysis results were: ICCs for IS (0.83, 0.86, 0.93) and CD (0.72, 0.82, 0.83), for the comparisons of CR/one-slice MR, CR/two-slice MRI and one-slice MRI/two-slice MRI, respectively. The Bland-Altman mean differences showed an 8% and a 7% increase on IS values with one-slice MRI and two-slice MRI compared to CR results, while the increase was of 9% and 1% in CD for the respective comparisons with CR. CONCLUSION: MRI can overestimate patellar height compared to CR, as much as an 8% increase in Insall-Salvati values when using one- or two-slice MRI measurements, and up to a 9% increase in Caton-Deschamps value when using the one-slice MRI method. It is recommended to use the CR as the preferred method when measuring patellar height. LEVEL OF EVIDENCE: III.

Acetabular Global Insufficiency in Patients with Down Syndrome and Hip-Related Symptoms: A Matched-Cohort Study.

BACKGROUND: The etiology of hip instability in Down syndrome is not completely understood. We investigated the morphology of the acetabulum and femur in patients with Down syndrome and compared measurements of the hips with those of matched controls. METHODS: Computed tomography (CT) images of the pelvis of 42 patients with Down syndrome and hip symptoms were compared with those of 42 age and sex-matched subjects without Down syndrome or history of hip disease who had undergone CT for abdominal pain. Each of the cohorts had 23 male and 19 female subjects. The mean age (and standard deviation) in each cohort was 11.3 ± 5.3 years. The lateral center-edge angle (LCEA), acetabular inclination angle (IA), acetabular depth-width ratio (ADR), acetabular version, and anterior and posterior acetabular sector angles (AASA and PASA) were compared. The neck-shaft angle and femoral version were measured in the patients with Down syndrome only. The hips of the patients with Down syndrome were further categorized as stable (n = 21) or unstable (n = 63) for secondary analysis. RESULTS: The hips in the Down syndrome group had a smaller LCEA (mean, 10.8° ± 12.6° compared with 25.6° ± 4.6°; p < 0.0001), a larger IA (mean, 17.4° ± 10.3° compared with 10.9° ± 4.8°; p < 0.0001), a lower ADR (mean, 231.9 ± 56.2 compared with 306.8 ± 31.0; p < 0.0001), a more retroverted acetabulum (mean acetabular version as measured at the level of the centers of the femoral heads [AVC], 7.8° ± 5.1° compared with 14.0° ± 4.5°; p < 0.0001), a smaller AASA (mean, 55.0° ± 9.9° compared with 59.7° ± 7.8°; p = 0.005), and a smaller PASA (mean, 67.1° ± 10.4° compared with 85.2° ± 6.8°; p < 0.0001). Within the Down syndrome cohort, the unstable hips showed greater femoral anteversion (mean, 32.7° ± 14.6° compared with 23.6° ± 10.6°; p = 0.002) and worse global acetabular insufficiency compared with the stable hips. No differences between the unstable and stable hips were found with respect to acetabular version (mean AVC, 7.8° ± 5.5° compared with 7.6° ± 3.8°; p = 0.93) and the neck-shaft angle (mean, 133.7° ± 6.7° compared with 133.2° ± 6.4°; p = 0.81). CONCLUSIONS: Patients with Down syndrome and hip-related symptoms had more retroverted and shallower acetabula with globally reduced coverage of the femoral head compared with age and sex-matched subjects. Hip instability among those with Down syndrome was associated with worse global acetabular insufficiency and increased femoral anteversion, but not with more severe acetabular retroversion. No difference in the mean femoral neck-shaft angle was observed between the stable and unstable hips in the Down syndrome cohort. LEVEL OF EVIDENCE: Prognostic Level III. See Instructions for Authors for a complete description of levels of evidence.

Why are bone and soft tissue measurements of the TT-TG distance on MRI different in patients with patellar instability?

PURPOSE: To determine whether the tibial tuberosity-to-trochlear groove distance (TT-TG) and patellar tendon-to-trochlear groove distance (PT-TG) are equal, whether the bony and cartilaginous points coincide in the trochlea, and whether the insertion of the PT coincides with the most anterior point of the TT in patients with patellar instability. METHODS: Fifty-three MRI scans of patients with patellar instability were examined. TT-TG and PT-TG were measured by three examiners in 31 knees. Additionally, the bone-cartilage distance in the trochlea [trochlear cartilage to trochlear bone (TC-TB)] and the distance between the mid-point of the PT insertion and the most anterior point of the TT (PT-TT) were measured by one examiner. The intraclass correlation coefficient was used to evaluate the reliability of the measurements between the three examiners. The relationships between the measurements were determined, the means of the measurements were calculated, and the correlations between PT-TG and TT-TG, PT-TT, and TC-TB were assessed. RESULTS: The ICC was above 0.8. PT-TG was 3.7 mm greater than TT-TG. The TC and TB coincided in 73 % of cases, and the mean TC-TB was 0.3 mm. The PT was lateral to the TT in 94 % of the cases, and the mean PT-TT was 3.4 mm. The Pearson's correlation coefficients between PT-TG and TT-TG, PT-TT, and TC-TB were 0.946, 0.679, and 0.199, respectively. CONCLUSION: TT-TG underestimated PT-TG, primarily due to the lateralization of the PT insertion relative to the most anterior point of the TT. CLINICAL RELEVANCE: our study shows that in patients with patellar instability, there are differences in the absolute values of TT-TG and PT-TG, as previously reported for patients without patellar instability. Hence, normal cut-off values based on case-control studies of TT-TG cannot be equivalently used when measuring PT-TG to indicate TT medialization in patients with patellar instability. It is also important to note that the clinical outcomes cannot be directly compared between patients evaluated using TT-TG versus PT-TG measurements. LEVEL OF EVIDENCE: III.

Is there a relationship between spinal instability in neoplastic disease and Tokuhashi scoring system?

Spinal instability neoplastic score (SINS) classification evaluates spinal stability by adding together six radiographic and clinical components. The objective of this study was to verify the association between SINS and Tokuhashi scoring system (TSS) score. Fifty-eight patients with vertebral metastases were admitted from 2010 to 2014 at Hospital do Servidor Público Estadual de São Paulo. They were evaluated according to their SINS and Tokuhashi SS score. Fourteen patients (24.13 %) scored from 0 to 6 points (stable spine), 37 (63.79 %) scored from 7 to 12 (potentially unstable), and 7 (12.06 %) scored from 13 to 18 (unstable). In stable spine patients according to SINS, the mean TSS score was 9.2. In potentially unstable spine patients, the mean TSS score was 8.24. In unstable spine patients, mean TSS score was 6.28. There was a statistically significant difference of the TSS score between stable and unstable patients. After evaluating TSS score in each patient, the worse the SINS, the worse was also the TSS score.

Tratamiento de la inestabilidad acromioclavicular crónica / Treatment of chronic acromioclavicular joint instability

Valorar los resultados de la técnica quirúrgica asistida por artroscopía indicada para el tratamiento de la inestabilidad acromioclavicular crónica (IAC), basada en la fijación coracoclavicular (CC) no-rígida más reconstrucción CC anatómica con aloinjerto tendinoso. Se incluyó a los pacientes con IAC intervenidos entre 2008 y 2012. Las valoraciones clínicas se realizaron mediante el SF36, la EVA y el DASH, aplicados en la visita previa (VPI) a la intervención y en la última visita de seguimiento (UVS). El Constant score y la Escala de Satisfacción General (0-10) se aplicaron en la última visita de seguimiento. Se valoró el desarrollo de subluxaciones secundarias. Se incluyeron 10 pacientes. Edad media 41 años [rango 33-55]. Seguimiento medio 25.50 meses [rango 24-30].En todos los pacientes el tratamiento quirúrgico se indicó cuando el tratamiento conservador fracasó. Cuestionarios aplicados en la VPI y en la UVS: 1. SF36 físico: VPI 29.60 ± 3.41 y UVS 59.58 ± 1.98 (p = 0.000); 2. SF36 mental: VPI 46.57 ± 3.80 y UVS 56.62 ± 1.89 (p = 0.000); 3. EVA: VPI 5.17 ± 2.40 y UVS 1.67 ± 2.07 (p = 0.022); y 4. DASH: VPI 63.33 ± 23.56 y UVS 2.61 ± 1.79 (p = 0.000). El Constant score y la satisfacción general de la UVS fueron 95.56 ± 3.28 y 9.22 ± 0.67 respectivamente. No hubo subluxaciones secundarias. El tratamiento de la IAC mediante un dispositivo de suspensión CC y una reconstrucción anatómica de los ligamentos CC asistida por artroscopía, puede ofrecer una mejoría significativa de la calidad de vida de los pacientes y representa una estrategia que al contemplar una fijación CC mecánica primaria, puede minimizar las posibilidades de fracaso y desarrollo de subluxaciones secundarias.

The purpose of this paper is to assess the results obtained with the arthroscopy-assisted surgical technique for the treatment of chronic acromioclavicular joint instability (CACJI), based on non-rigid coracoclavicular (CC) fixation and anatomical CC reconstruction with a tendinous allograft. Patients with CACJI who underwent surgery between 2008 and 2012 were included in the study. Clinical assessments included SF36, VAS and DASH, applied at the preoperative visit (POV) and at the last follow-up visit (LFUV). The Constant score and the General Satisfaction Score (0-10) were applied at the last follow-up visit. Occurrence of secondary subluxations was assessed. Ten patients were included; mean age was 41 years (range 33-55). Mean follow-up was 25.50 months (range 24-30). Surgical treatment was indicated in all patients after failure of conservative treatment. Questionnaires applied at the POV and the LFUV showed the following results: 1. SF36: physical, POV = 29.60 ± 3.41 and LFUV = 59.58 ± 1.98 (p = 0.000); 2. SF36 mental, POV = 46.57 ± 3.80 and LFUV = 56.62 ± 1.89 (p = 0.000); 3. VAS: POV = 5.17 ± 2.40 and LFUV: 1.67 ± 2.07 (p = 0.022); and 4. DASH: POV = 63.33 ± 23.56 and LFUV = 2.61 ± 1.79 (p = 0.000). The Constant score and the general satisfaction at the LFUV were 95.56 ± 3.28 and 9.22 ± 0.67, respectively. There were no secondary subluxations. Treatment of CACJI with a CC suspension device and arthroscopically-assisted anatomical reconstruction of CC ligaments may provide a significant quality of life improvement to patients. It is a strategy that, upon considering primary mechanical CC fixation, may minimize the chance of failure and occurrence of secondary subluxations.

Traumatic Shoulder Dislocation Among Adolescents: Hill-Sachs Lesion Volume and Recurrent Instability.

BACKGROUND: Accurate assessment of Hill-Sachs lesions (HSLs) and their relationship to the glenoid track is essential to optimizing management following traumatic shoulder dislocation. The purpose of this study was to measure the size of HSLs by a novel method with magnetic resonance (MR) arthrography and to investigate the relationship between lesion size and instability history in adolescents (below 19 y of age) with a history of traumatic dislocation. METHODS: All shoulder MR arthrograms queried to identify HSLs over a 4-year period were retrospectively reviewed and independently evaluated by 2 blinded musculoskeletal radiologists. HSLs were evaluated on axial T1-weighted fat-saturated images. For each consecutive image slice in which the HSL was identified, a freehand region of interest was constructed along the compression fracture cavity. Region of interest sum was multiplied by image slice thickness (and gap between slices if present) to generate a total HSL volume (mm). Subject chart review was then performed to acquire demographic data, including shoulder dislocation history. RESULTS: A total of 30 patients met inclusion criteria and included 22 boys (mean age, 16.3 y) and 8 girls (mean age, 16.4 y). Four patients had a history of 1 traumatic dislocation, 12 had a history of 2, and 14 had a history of ≥3. The average size of the HSL for all patients was 3.8 mm (range, 0.52 to 11 mm). There was no significant difference in HSL volume between the 21 skeletally immature (3.7 mm) and 9 skeletally mature (4.2 mm) patients (P=0.67). Patients with a history of 1, 2, or 3+ dislocations had an average HSL volume of 1.3, 3.7, and 4.7 mm, respectively. The measurement method revealed excellent interreader reliability (P=0.00). There was a statistically significant difference between dislocation history group mean HSL volumes (P=0.019), as well as a statistically significant difference between the number of dislocations and lesion volume (P=0.011). CONCLUSIONS: HSLs can be effectively measured in adolescent patients using MR arthrography and patients with larger HSLs have more recurrent instability episodes, potentially meriting greater and earlier attention to the defect. LEVEL OF EVIDENCE: Prognostic/diagnostic study level IV.

Can ultrasound of plantar plate have normal appearance with a positive drawer test?

OBJECTIVES: The aims of this study were (1) to evaluate the reliability of ultrasound (US) examination in the identification and measurement of the metatarsophalangeal plantar plate (MTP-PP) in asymptomatic subjects and (2) to establish the correlation of US findings with those of physical examination and magnetic resonance imaging (MRI), once it is an important tool in the evaluation of the instability syndrome of the second and third rays. MATERIALS AND METHODS: US examinations of the second and third MTP-PPs were performed in eight asymptomatic volunteers, totaling 32 MTP joints, by three examiners with different levels of experience in musculoskeletal US. Plantar plate dimensions, integrity and echogenicity, the presence of ruptures, and confidence level in terms of structure identification were determined using conventional US. Vascular flow was assessed using power Doppler. US data were correlated with data from physical examination and MRI. RESULTS: MTP-PPs were ultrasonographically identified in 100% of cases, always showing homogeneous hyperechoic features and no detectable vascular flow on power Doppler, with 100% certainty in identification for all examiners. There was excellent US inter-observer agreement for longitudinal measures of second and third toe MTP-PPs and for transverse measures of the second toe MTP-PP. The MTP drawer test was positive for grade 1 MTP instability in 34.4% of joints with normal US results. Transverse MTP-PP measures were significantly higher in individuals with positive MTP drawer test. US measures and characteristics of MPT-PPs were positively correlated with those of MRI. CONCLUSIONS: US is efficient in identifying and measuring MPT-PPs and may complement physical examination. A grade 1 positive MTP drawer test may be found in asymptomatic individuals with normal MPT-PPs, as assessed by imaging.

[Treatment of chronic acromioclavicular joint instability]. / Tratamiento de la inestabilidad acromioclavicular crónica.

The purpose of this paper is to assess the results obtained with the arthroscopy-assisted surgical technique for the treatment of chronic acromioclavicular joint instability (CACJI), based on non-rigid coracoclavicular (CC) fixation and anatomical CC reconstruction with a tendinous allograft. Patients with CACJI who underwent surgery between 2008 and 2012 were included in the study. Clinical assessments included SF36, VAS and DASH, applied at the preoperative visit (POV) and at the last follow-up visit (LFUV). The Constant score and the General Satisfaction Score (0-10) were applied at the last follow-up visit. Occurrence of secondary subluxations was assessed. Ten patients were included; mean age was 41 years (range 33-55). Mean follow-up was 25.50 months (range 24-30). Surgical treatment was indicated in all patients after failure of conservative treatment. Questionnaires applied at the POV and the LFUV showed the following results: 1. SF36: physical, POV = 29.60 ± 3.41 and LFUV = 59.58 ± 1.98 (p = 0.000); 2. SF36 mental, POV = 46.57 ± 3.80 and LFUV = 56.62 ± 1.89 (p = 0.000); 3. VAS: POV = 5.17 ± 2.40 and LFUV: 1.67 ± 2.07 (p = 0.022); and 4. DASH: POV = 63.33 ± 23.56 and LFUV = 2.61 ± 1.79 (p = 0.000). The Constant score and the general satisfaction at the LFUV were 95.56 ± 3.28 and 9.22 ± 0.67, respectively. There were no secondary subluxations. Treatment of CACJI with a CC suspension device and arthroscopically-assisted anatomical reconstruction of CC ligaments may provide a significant quality of life improvement to patients. It is a strategy that, upon considering primary mechanical CC fixation, may minimize the chance of failure and occurrence of secondary subluxations.

MRI evaluation of the MTP plantar plates compared with arthroscopic findings: a prospective study.

BACKGROUND: Instability of the lesser metatarsophalangeal (MTP) joints has been widely reported and plantar plate insufficiency is a key part of this pathologic process. The diagnosis is made clinically but can be aided by imaging studies, particularly magnetic resonance imaging (MRI); however, the sensitivity and accuracy of this method compared to direct visualization of these lesions has not yet been established, nor has interobserver accuracy of MRI been assessed for evaluation of plantar plate pathology. In this study, our goals were to identify the accuracy of the MRI in describing plantar plate tears when compared to direct arthroscopic visualization using an anatomic grading system and to test the influence of an anatomic grading system in the accuracy of the MRI readings. METHODS: We evaluated the clinical exam, MRI scans, and arthroscopic findings of 35 patients with lesser MTP instability. RESULTS: Using an anatomic grading system, a distinct improvement in the radiological evaluation and interpretation occurred. Knowledge of the pattern of plantar plate tears by a radiologist enabled them to locate and describe the type of tears of the plantar plate on the MRI. The amount of training and the experience of the radiologist were also important factors in our study. The senior radiologists had much better levels of accuracy (Group A, 77.0%; Group B, 88.5%) than less experienced radiologists. CONCLUSION: Prior knowledge of the pathophysiology and morphological types of lesions of the plantar plates was helpful for accurate identification and description of the tears by the radiologist. LEVEL OF EVIDENCE: Level II, prospective comparative study.

Evolução etária do ângulo de carregamento do cotovelo: estudo clínico-radiográfico / Evolution of the carrying angle of the elbow: a clinical and radiographic study

OBJETIVO: Avaliar clínica e radiograficamente o ângulo de carregamento do cotovelo (ACC) determinando uma curva de normalidade de acordo com faixas etárias (da infância à maturidade esquelética) e comparar as medidas clínicas e radiográficas. MÉTODOS: Avaliamos 510 indivíduos (1020 cotovelos), com idades entre 1 e 18 anos, distribuídos em grupos de 30 conforme faixas etárias, com intervalo de 1 ano. Excluímos os portadores de: fraturas do cotovelo, sequelas, malformações, doenças genéticas, afecções inflamatórias e frouxidão ligamentar. Mensuramos clinicamente o ACC bilateralmente com goniômetro, onde obtivemos duas medidas por dois examinadores onde média destas foi considerada. Realizamos radiografias ântero-posteriores dos cotovelos e aferimos os ângulos formados pelos eixos do úmero e da ulna. Todos os dados foram analisados estatisticamente pelo teste t student. RESULTADOS: Determinamos uma curva de normalidade onde observamos aumento deste parâmetro conforme a progressão da idade. Não observamos diferença significante entre as medidas clínicas e radiográficas. CONCLUSÃO: A média do ângulo de carregamento para o sexo feminino foi 12,78º ± 5,35 e para o masculino 11,20º ± 4,45. Este valor aumenta progressivamente da infância até os 16 anos quando observamos estabilização. Não houve diferença estatística significante das medidas clínicas e radiográficas.

OBJECTIVE: This paper has the purpose of evaluate the elbow carrying angle by clinic and radiographic examination in normal children and determine the range of normality according to age from childhood to skeletal maturity and also check if there is a statistically significant difference between the clinical and radiographic measurements. METHODS: We evaluated 510 persons with ages varying from 1 to 18 years distributed in groups with 30 subjects according to the age group with 1-year interval. We performed radiographic examination of the elbow and measured the angle formed by the long axis of the humerus and ulna. The data were statistically analyzed using the student t-test. RESULTS: We determined a normal curve of the study population where there was an increase of this parameter with the progression of age. No statistically significant difference between the clinical and radiographic measures. CONCLUSION: The average of the elbow carrying angle was 12,78 ± 5,35 degrees for females and 11,20 ± 4,45 degrees for males. This values increase progressively from childhood until 16 years when we notice stabilization. There was no statistical difference between the clinical and radiographic measurements.

Evaluation of the lateral pterygoid muscle using magnetic resonance imaging.

OBJECTIVES: The aims of this study were to evaluate the visibility of the lateral pterygoid muscle (LPM) in temporomandibular joint (TMJ) images obtained by MRI, using different projections and to compare image findings with clinical symptoms of patients with and without temporomandibular disorders (TMD). METHODS: In this study, LPM images of 50 participants with and without TMDs were investigated by MRI. The images of the LPM in different projections of 100 TMJs from 35 participants (70 TMJs) with and 15 participants (30 TMJs) without clinical signs and symptoms of TMD were visible and analysed. RESULTS: The oblique sagittal and axial images of the TMJ clearly showed the LPM. Hypertrophy (1.45%), atrophy (2.85%) and contracture (2.85%) were the abnormalities found in the LPM. TMD signs, such as hypermobility (11.4%), hypomobility (12.9%) and disc displacement (20.0%), could be seen in TMJ images. Related clinical symptoms, such as pain (71.4%), articular sounds (30.4%), bruxism (25.7%) and headache (22.9%), were observed. CONCLUSIONS: Patients with TMD can present with alterations in the LPM thickness. Patients without TMD also showed alterations, such as atrophy and contracture, in TMJ images. Recognition of alterations in the LPM will improve our understanding of clinical symptoms and pathophysiology of TMD, and may lead to a more specific diagnosis of these disorders.

Lateral mass screw fixation of the atlas: surgical technique and anatomy.

The use of lateral mass atlas screws is an important technique to achieve fusion and stability at the craniocervical region affected by different pathologies (degenerative, traumatic, inflammatory, neoplastic, or congenital). This paper describes the anatomy and techniques necessary for proper insertion of posterior C1 lateral mass screws, using anatomic dissection and intraoperative pictures. Knowledge of the anatomy and the surgical technique of insertion of C1 lateral mass screws are of paramount importance to have good surgical results.

Ullrich congenital muscular dystrophy and Bethlem myopathy: clinical and genetic heterogeneity.

UNLABELLED: Ullrich congenital muscular dystrophy (UCMD), due to mutations in the collagen VI genes, is an autosomal recessive form of CMD, commonly associated with distal joints hyperlaxity and severe course. A mild or moderate involvement can be occasionally observed. OBJECTIVE: To evaluate the clinical picture of CMD patients with Ullrich phenotype who presented decreased or absent collagen VI immunoreactivity on muscular biopsy. RESULTS: Among 60 patients with CMD, two had no expression of collagen V and their clinical involvement was essentially different: the first (3 years of follow-up) has mild motor difficulty; the second (8 years of follow-up) never acquired walking and depends on ventilatory support. A molecular study, performed by Pan et al. at the Thomas Jefferson University, demonstrated in the first a known mutation of Bethlem myopathy in COL6A1 and in the second the first dominantly acting mutation in UCMD and the first in COL6A1, previously associated only to Bethlem myopathy, with benign course and dominant inheritance. CONCLUSION: Bethlem myopathy should be considered in the differential diagnosis of UCMD, even in patients without fingers contractures; overlap between Ullrich and Bethlem phenotypes can be supposed.

Ullrich congenital muscular dystrophy and Bethlem myopathy: clinical and genetic heterogeneity

Ullrich congenital muscular dystrophy (UCMD), due to mutations in the collagen VI genes, is an autosomal recessive form of CMD, commonly associated with distal joints hyperlaxity and severe course. A mild or moderate involvement can be occasionally observed. OBJECTIVE: To evaluate the clinical picture of CMD patients with Ullrich phenotype who presented decreased or absent collagen VI immunoreactivity on muscular biopsy. RESULTS: Among 60 patients with CMD, two had no expression of collagen V and their clinical involvement was essentially different: the first (3 years of follow-up) has mild motor difficulty; the second (8 years of follow-up) never acquired walking and depends on ventilatory support. A molecular study, performed by Pan et al. at the Thomas Jefferson University, demonstrated in the first a known mutation of Bethlem myopathy in COL6A1 and in the second the first dominantly acting mutation in UCMD and the first in COL6A1, previously associated only to Bethlem myopathy, with benign course and dominant inheritance. CONCLUSION: Bethlem myopathy should be considered in the differential diagnosis of UCMD, even in patients without fingers contractures; overlap between Ullrich and Bethlem phenotypes can be supposed.

A distrofia muscular congênita (DMC) com hiperextensibilidade articular distal (fenótipo Ullrich) associa-se a mutações nos genes do colágeno VI e corresponde a um grave quadro congênito de herança autossômica recessiva e curso progressivo, ocasionalmente mostrando menor gravidade. OBJETIVO: Avaliar o quadro clínico dos pacientes com DMC tipo Ullrich que apresentam imunoexpressão baixa ou ausente do colágeno VI na biópsia muscular. RESULTADOS: Entre 60 pacientes com DMC, dois mostravam imunomarcação negativa do colágeno VI. Mostravam-se clinicamente essencialmente diferentes: o primeiro, com 8 anos de idade e três de seguimento mostra leve dificuldade motora; o segundo, com 14 anos de idade e 8 de seguimento, não deambula e apresenta insuficiência respiratória. O estudo molecular, realizado na Thomas Jefferson University por Pan et al., revelou no primeiro, no gene COL6A1, mutação típica da miopatia de Bethlem, que tem curso benigno e herança autossômica dominante; e no segundo a primeira mutação de efeito dominante e do gene COL6A1, previamente associado apenas à miopatia de Bethlem. CONCLUSÃO: A miopatia de Bethlem deve constar no diagnóstico diferencial da DMC tipo Ullrich, mesmo na ausência das típicas contraturas dos dedos; pode existir sobreposição dos fenótipos Ullrich e Bethlem.

Aging of the elastic and collagen fibers in the human cervical interspinous ligaments.

BACKGROUND CONTEXT: The ligaments consist of collagen bands intermingled with elastic fibers that support hundreds of pounds of stress per square inch. In the spine the basic functional unit comprises vertebrae, intervertebral disc and ligament tissues. The interspinous ligaments with the function of limiting the spine flexion are exposed to a traumatic and degenerative process that promotes pain or instability. It has been shown that aging induces structural changes to capsular, fascial and ligamentous structures, mainly to the elastic and collagen fibers. However, the relative changes with age in elastic and collagen fibers have not been quantified. PURPOSE: Examine the changes in the arrangement and amount of the elastic and collagen fibers of the human cervical interspinous ligament and attempt to correlate them with age. STUDY DESIGN/SETTING: Histomorphometric analysis of ligament samples harvested during surgery. PATIENT SAMPLE: We studied the dorsal portion of this ligament from 17 patients aged 16 to 69 years. OUTCOME MEASURES: Fraction of collagen and elastic fibers with linear regression analysis correlating fraction versus age. METHODS: The elastic and collagen fibers were identified by selective staining methods, and a blinded investigator using an image analysis system performed the histomorphometry. RESULTS: There is an age-related progressive increase in collagen and mature and elaunin elastic fibers responsible to elasticity. However, these elastic fibers showed structural degenerative changes with aging. Furthermore, there is an age-related decrease of oxytalan elastic fibers responsible to resistance. CONCLUSIONS: The aged interspinous ligament showed loss of elasticity that could alter the flexion limiting of the vertebral column.

Localized medial triquetral-hamate instability: anatomy and operative reconstruction-augmentation.

Localized medial triquetral-hamate instability (LTHMI) is a relatively uncommon form of wrist instability. After an initial traumatic lesion elongating the triquetral-hamate (TH) complex (TH medial ligament and floor of the extensor carpi ulnaris [ECR] sheath), pain occurs during professional activities demanding ulno-radial movements (polo, tennis, music playing). Imbrication of the TH complex and augmentation with the ECU tendon is suggested for LTHMI.