Assessment, classification and treatment of calcinosis as a complication of juvenile dermatomyositis: a survey of pediatric rheumatologists by the childhood arthritis and rheumatology research alliance (CARRA).

BACKGROUND: There is no standardized approach to the management of JDM-associated calcinosis and its phenotypes. Current knowledge of treatment outcomes is confined to small series and case reports. We describe physician perspectives toward diagnostic approach, classification and treatment directly targeting calcinosis, independent of overall JDM therapy. METHODS: An electronic survey of 22 questions was organized into sections regarding individual practices of assessment, classification and treatment of calcinosis, including perceived successes of therapies. Invitations to complete the survey voluntarily and anonymously were sent to CARRA physician members and the Pediatric Rheumatology Bulletin Board, an electronic list-serv. Results were analyzed by descriptive statistics and chi-square analyses. RESULTS: Of 139 survey responses, 118 were included in analysis. Of these, 70% were based in the USA and 88 (75%) were CARRA members. Only 17% of responders have seen more than 20 cases of calcinosis, and only 28% perform screening imaging studies on new JDM diagnoses. Increasing systemic immunosuppression is first-line therapy for 67% of respondents. Targeted therapy against calcinosis is most often instituted for symptomatic patients. IVIG and bisphosphonates are most frequently used and considered most successful, but many other agents are used. Experienced physicians are more likely to use bisphosphonates, calcium channel blockers and topical sodium thiosulfate (p< 0.002 or lower). CONCLUSIONS: Coexisting JDM disease activity influences whether calcinosis is considered active disease or targeted directly. Experience treating JDM-related calcinosis is low, as are rates of formal screening for calcinosis. Experienced physicians are more likely to use non-immunosuppressive treatments.

Biologic therapies for refractory juvenile dermatomyositis: five years of experience of the Childhood Arthritis and Rheumatology Research Alliance in North America.

BACKGROUND: The prognosis of children with juvenile dermatomyositis (JDM) has improved remarkably since the 1960's with the use of corticosteroid and immunosuppressive therapy. Yet there remain a minority of children who have refractory disease. Since 2003 the sporadic use of biologics (genetically-engineered proteins that usually are derived from human genes) for inflammatory myositis has been reported. In 2011-2016 we investigated our collective experience of biologics in JDM through the Childhood Arthritis and Rheumatology Research Alliance (CARRA). METHODS: The JDM biologic study group developed a survey on the CARRA member experience using biologics for Juvenile DM utilizing Delphi consensus methods in 2011-2012. The survey was completed online by the CARRA members interested in JDM in 2012. A second survey was similarly developed that provided more opportunity to describe their experiences with biologics in JDM in detail and was completed by CARRA members in Feb 2013. During three CARRA meetings in 2013-2015, nominal group techniques were used for achieving consensus on the current choices of biologic drugs. A final survey was performed at the 2016 CARRA meeting. RESULTS: One hundred and five of a potential 231 pediatric rheumatologists (42%) responded to the first survey in 2012. Thirty-five of 90 had never used a biologic for Juvenile DM at that time. Fifty-five of 91 (denominators vary) had used biologics for JDM in their practice with 32%, 5%, and 4% using rituximab, etanercept, and infliximab, respectively, and 17% having used more than one of the three drugs. Ten percent used a biologic as monotherapy, 19% a biologic in combination with methotrexate (mtx), 52% a biologic in combination with mtx and corticosteroids, 42% a combination of a biologic, mtx, corticosteroids (steroids), and an immunosuppressive drug, and 43% a combination of a biologic, IVIG and mtx. The results of the second survey supported these findings in considerably more detail with multiple combinations of drugs used with biologics and supported the use of rituximab, abatacept, anti-TNFα drugs, and tocilizumab in that order. One hundred percent recommended that CARRA continue studying biologics for JDM. The CARRA meeting survey in 2016 again supported the study and use of these four biologic drug groups. CONCLUSIONS: Our CARRA JDM biologic work group developed and performed three surveys demonstrating that pediatric rheumatologists in North America have been using multiple biologics for refractory JDM in numerous scenarios from 2011 to 2016. These survey results and our consensus meetings determined our choice of four biologic therapies (rituximab, abatacept, tocilizumab and anti-TNFα drugs) to consider for refractory JDM treatment when indicated and to evaluate for comparative effectiveness and safety in the future. Significance and Innovations This is the first report that provides a substantial clinical experience of a large group of pediatric rheumatologists with biologics for refractory JDM over five years. This experience with biologic therapies for refractory JDM may aid pediatric rheumatologists in the current treatment of these children and form a basis for further clinical research into the comparative effectiveness and safety of biologics for refractory JDM.

Interaction between osseous and non-osseous vibratory stimulation of the human cadaveric head.

Bone conduction (BC) stimulation can be applied by vibration to the bony or skin covered skull (osseous BC), or on soft tissue such as the neck (non-osseous BC). The interaction between osseous and non-osseous bone conduction pathways is assessed in this study. The relation between bone vibrations measured at the cochlear promontory and the intracranial sound pressure for stimulation directly on the dura and for stimulation at the mastoid between 0.2 and 10 kHz was compared. First, for stimulation on the dura, varying the static coupling force of the BC transducer on the dura had only a small effect on promontory vibration. Second, the presence or absence of intracranial fluid did not affect promontory vibration for stimulation on the dura. Third, stimulation on the mastoid elicited both promontory vibration and intracranial sound pressure. Stimulation on the dura caused intracranial sound pressure to a similar extent above 0.5 kHz compared to stimulation on the mastoid, while promontory vibration was less by 20-40 dB. From these findings, we conclude that intracranial sound pressure (non-osseous BC) only marginally affects bone vibrations measured on the promontory (osseous BC), whereas skull vibrations affect intracranial sound pressure.

Biomechanics of the incudo-malleolar-joint - Experimental investigations for quasi-static loads.

Under large quasi-static loads, the incudo-malleolar joint (IMJ), connecting the malleus and the incus, is highly mobile. It can be classified as a mechanical filter decoupling large quasi-static motions while transferring small dynamic excitations. This is presumed to be due to the complex geometry of the joint inducing a spatial decoupling between the malleus and incus under large quasi-static loads. Spatial Laser Doppler Vibrometer (LDV) displacement measurements on isolated malleus-incus-complexes (MICs) were performed. With the malleus firmly attached to a probe holder, the incus was excited by applying quasi-static forces at different points. For each force application point the resulting displacement was measured subsequently at different points on the incus. The location of the force application point and the LDV measurement points were calculated in a post-processing step combining the position of the LDV points with geometric data of the MIC. The rigid body motion of the incus was then calculated from the multiple displacement measurements for each force application point. The contact regions of the articular surfaces for different load configurations were calculated by applying the reconstructed motion to the geometry model of the MIC and calculate the minimal distance of the articular surfaces. The reconstructed motion has a complex spatial characteristic and varies for different force application points. The motion changed with increasing load caused by the kinematic guidance of the articular surfaces of the joint. The IMJ permits a relative large rotation around the anterior-posterior axis through the joint when a force is applied at the lenticularis in lateral direction before impeding the motion. This is part of the decoupling of the malleus motion from the incus motion in case of large quasi-static loads.

Middle ear adenoma: case report and discussion.

Introduction. Despite modern radiological workup, surgeons can still be surprised by intraoperative findings or by the pathologist's report. Materials & Methods. We describe the case of a 52-year-old male who was referred to our clinic with a single sided conductive hearing loss. He ultimately underwent middle ear exploration and excision of a middle ear tumour followed by second look and ossiculoplasty a year later. Results. Though preoperative CT and MRI scanning were suggestive of a congenital cholesteatoma, the pathologist's report diagnosed a middle ear adenoma. Discussion. Middle ear glandular tumors are extremely rare and, despite numerous histological techniques, continue to defy satisfactory classification. Most surgeons advocate surgical excision though evidence of the tumour's natural course and risk of recurrence is lacking.

[NiTiBOND an optimized self-crimping stapes prosthesis for treatment of otosclerosis]. / NiTiBOND, eine optimierte Steigbügelprothese zur chirurgischen Behandlung der Otosklerose.

BACKGROUND: Compared to traditional stapes prostheses, self-crimping prostheses have been shown to result in similar, if not better, closure of the air bone gap in patients undergoing stapedotomy for otosclerosis. To achieve self-crimping, nitinol, a shape memory alloy, has been used for several years but concerns have been raised regarding possible damage to the incus and its muco-periosteum. We investigate these concerns with regard to the newer NiTiBOND stapes prosthesis in an observational multi-centre study. MATERIAL AND METHODS: In a multicentre, prospective observational study, 76 patients undergoing stapedotomy with the NiTiBond prosthesis across 4 centres were compared to 75 -retrospectively selected control SMart patients. Complications, intra-operative user-friendliness and audiological results at 3 months were documented. RESULTS: Audiological improvement and the rate of complications were similar in both groups. Non inferiority was shown at all frequencies and in the pure-tone average. The NiTiBOND prosthesis was described as very user-friendly. CONCLUSIONS: By eliminating manual crimping, stapedotomy using the NiTiBOND prosthesis can be facilitated and standardized. Furthermore, intraoperative handling characteristics of the prosthesis are very good which may further reduce operative risk. Importantly, we show that these benefits are not to the detriment of audiological outcome. Larger and longer-term studies are required to further evaluate results.

The Bonebridge: preclinical evaluation of a new transcutaneously-activated bone anchored hearing device.

OBJECTIVES: To assess the functional performance of the Bonebridge (BB, MED-EL), a newly-designed transcutaneous bone conduction implant that allows the skin to remain intact and to compare it with the current clinical model of choice, a percutaneous bone conduction implant (BAHA BP100, Cochlear Bone Anchored Solutions AG). MATERIALS AND METHODS: The devices were compared using two methods: (1) Measurements of cochlear promontory acceleration in five cadaver heads: Accelerations of the cochlear promontories on both ipsilateral and contralateral sides were measured using a Laser Doppler system, with free-field sound stimuli of 90 dB SPL in the frequency range of 0.3-10 kHz (2) Measurements of pure-tone sound field thresholds in 5 normally hearing human adult subjects under a condition of simulated hearing loss. For the latter measurements, the devices were applied to the head using a Softband, and measurements were performed in the frequency range of 0.25-8 kHz. Within investigation comparisons (i.e., in cadavers or listeners) and a cross-comparison analysis of the cadaver and human results were done. RESULTS: Results from the cadaver heads showed that the cochlear promontory acceleration with the BB was higher within 10 dB on the ipsilateral side and lower within 5 dB on the contralateral side than the acceleration with the BAHA, in the frequency range of 0.7-10 kHz. The transcranial attenuation of the acceleration for the BB was greater than for the BAHA within 20 dB. For the sound-field threshold assessments with human subjects, the BB and BAHA showed similar threshold improvements of more than 10 dB HL for the ipsilateral side. For the contralateral side, the threshold improvement with the BB was less than with the BAHA, indicating better separation between ipsilateral and contralateral sides. CONCLUSIONS: Preclinical results imply that the BB has functional performance similar to the BAHA and could be beneficial to patients suffering with conductive and mixed hearing losses as well as for those with unilateral impairment. Based on these preliminary results, a carefully designed clinical trial with conservative inclusion criteria can be recommended. This article is part of a special issue entitled "MEMRO 2012".

The development of bone mineral lateralization in the arms.

UNLABELLED: Bone mineral content (BMC) is known to be greater in the dominant arm after the age of 8 years. We studied a group of children and found that BMC sidedness gradually increased up to the age of 6 years and then remained stable into late adolescence. INTRODUCTION: Bone mineral content (BMC) exhibits sidedness in the arms after the age of 8 years, but it is not known whether BMC is greater in the dominant arm from birth or whether lateralization develops in early childhood. To address this, we examined bone mineral status in relation to handedness and age. METHODS: Subjects (N = 158) were children recently initiating glucocorticoids for underlying disease (leukemia 43 %, rheumatic conditions 39 %, nephrotic syndrome 18 %). Handedness was determined by questionnaire and BMC by dual-energy X-ray absorptiometry. RESULTS: Median age was 7.2 years (range, 1.5 to 17.0 years), 49 % was male, and the spine BMD Z-score was -0.9 (SD, 1.3). By linear regression, BMC sidedness in the arms was significantly related to age (r = 0.294, p = 0.0005). Breakpoint analysis revealed two lines with a knot at 6.0 years (95 % CI, 4.5-7.5 years). The formula for the first line was: dominant:nondominant arm BMC ratio = 0.029 × age [in years] + 0.850 (r = 0.323, p = 0.017). The slope of the second line was not different from 0 (p = 0.332), while the slopes for the two lines were significantly different (p = 0.027). CONCLUSIONS: These results show that arm BMC sidedness in this patient group develops up to age 6 years and then remains stable into late adolescence. This temporal profile is consistent with mechanical stimulation of the skeleton in response to asymmetrical muscle use as handedness becomes manifest.

Inversion-recovery single-shot cardiac MRI for the assessment of myocardial infarction at 1.5 T with a dedicated cardiac coil.

OBJECTIVE: The aim of this study was to assess the diagnostic accuracy of imaging myocardial infarction with a two-dimensional (2D) single-shot inversion-recovery (IR)-gradient-echo (GE) sequence compared with a standard 2D segmented IR-GE sequence at 1.5 T using a dedicated cardiac coil. METHODS: 22 patients with myocardial infarction documented in the past 3-12 months were examined at 1.5 T using a 5 channel cardiac coil. Imaging of delayed enhancement was performed 15 min after administration of 0.2 mmol of gadopentetate dimeglumine per kilogram of body weight. Immediately after completion of the single-shot sequence, which allows for coverage of the entire ventricle during a single breath-hold with nine slices, the segmented IR sequence was started. Infarct volumes, infarct transmurality and contrast-to-noise ratios (CNRs) of infarcted and healthy myocardium were compared between both techniques. RESULTS: Despite a moderate, non-significant loss of CNR (CNR(single-shot IR)=31.2±4.1; CNR(segmented IR)=37.9±4.1; p=0.405), the 2D single-shot technique correctly determined infarct size when compared with the standard 2D segmented IR-GE sequence. Assessment of both infarct volume (r=0.95; p<0.0001) and transmurality (r=0.97; p<0.0001) is possible, with excellent correlation of both techniques. CONCLUSION: Single-shot delayed enhancement imaging during a single breath-hold is feasible at 1.5 T with the use of a dedicated cardiac coil. Despite a moderately lower CNR, the single-shot technique allows for fast and accurate determination of infarct size with high spatial resolution and has the potential to reduce electrocardiogram and breathing artefacts.

Impact of iterative reconstruction on CNR and SNR in dynamic myocardial perfusion imaging in an animal model.

OBJECTIVES: To evaluate a new iterative reconstruction (IR) algorithm for radiation dose, image quality (IQ), signal-to-noise-ratio (SNR), and contrast-to-noise-ratio (CNR) in multidetector computed tomography (MDCT) dynamic myocardial perfusion imaging (MPI). METHODS: ECG-gated 256-slice MDCT dynamic MPI was performed in six pigs after subtotal balloon occlusion of one artery. Two 100 kVp protocols were compared: high dose (HD): 150 mAs; low dose (LD): 100 mAs. HD images were reconstructed with filtered back projection (FBP), LD images with FBP and different strengths of IR (L1, L4, and L7). IQ (5-point scale), SNR, and CNR (ischemic vs. normal myocardium) values derived from the HD (FBP) images and the different LD images were compared. RESULTS: Mean SNR values for myocardium were 16.3, 11.3, 13.1, 17.1, and 28.9 for the HD, LD (FBP), LD (L1), LD (L4), and LD (L7) reconstructions, respectively. Mean CNR values were 8.9, 6.3, 7.8, 9.3, and 12.8. IQ was scored as 4.6, 3.3, 4.4, 4.7, and 3.4, respectively. A significant loss of IQ was observed for the LD (L7) images compared to the HD (FBP) images (P < 0.05). CONCLUSION: Appropriate levels of iterative reconstruction can improve SNR and CNR, facilitating radiation dose savings in CT-MPI without influencing diagnostic quality. KEY POINTS: Iterative reconstruction (IR) can reduce radiation dose in myocardial perfusion CT. Our study also demonstrated improvements in image quality (noise, SNR, and CNR). Dynamic CT-MPI could help determine the hemodynamic significance of coronary artery disease. With dynamic CT MPI, myocardial blood flow can be determined quantitatively.

Recurrent phosphaturic mesenchymal tumour of the temporal bone causing deafness and facial nerve palsy.

OBJECTIVE: We describe the first reported case of a phosphaturic mesenchymal tumour, mixed connective tissue variant, invading the temporal bone. CASE REPORT: A female patient presented with increasing deafness. On examination there appeared to be a mass behind an intact tympanic membrane. Further radiological investigation showed a vascular mass occupying the middle ear, mastoid and internal auditory meatus. This was surgically resected and revealed to be a benign phosphaturic mesenchymal tumour, mixed connective tissue variant. The tumour recurred a year later, presenting as facial nerve palsy. A revision procedure was carried out; the tumour was excised with the sacrifice of a segment of the facial nerve, and a facial-hypoglossal nerve anastomosis was performed. CONCLUSION: This case report highlights the occurrence of this benign but sometimes aggressive tumour, of which both clinicians and pathologists should be aware. Early recognition of the condition remains of utmost importance to minimise the debilitating consequences of long-term osteomalacia in affected patients, and to prevent extracranial and intracranial complications caused by the tumour.

MR first pass perfusion of benign and malignant cardiac tumours-significant differences and diagnostic accuracy.

OBJECTIVES: To determine the diagnostic value of magnetic resonance (MR) first pass perfusion in the differentiation of benign and malignant cardiac tumours. METHODS: 24 patients with cardiac tumours (11 malignant, histopathological correlation present in all cases) were examined using MRI. In addition to morphological sequences a saturation-recovery T1w-GRE technique was implemented for tumour perfusion. The maximum relative signal enhancement (RSE[%]) and the slope of the RSE(t)-curve (slopeRSE[%/s]) of tumour tissue were assessed. A t-test was used to identify significant differences between benign and malignant tumours. Sensitivities and specificities were calculated for detection of malignant lesions and were compared with the sensitivity and specificity based on solely morphological image assessment. RESULTS: The RSE and slopeRSE of malignant cardiac tumours were significantly higher compared with benign lesions (p < 0.001 and p < 0.001). The calculated sensitivities and specificities of RSE and slopeRSE for identification of malignant lesions were 100% and 84.6% and 100% and 92.3%, respectively with cut-off values of 80% and 6%/s. The sensitivity and specificity for identification of malignant lesions on the basis of morphological imaging alone were 90.9% and 69.2%. CONCLUSIONS: With first pass perfusion, malignant cardiac masses can be identified with higher sensitivity and specificity compared with morphological image assessment alone.

[Vibration properties of the ossicle and cochlea and their importance for our hearing system]. / Schwingungseigenschaften der Ossikel und der Cochlea und deren Bedeutung für unser Gehör.

The investigations of movements of the eardrum and stapes have shown that at higher frequencies, complex spatial vibration patterns occur in which the individual elements move in very different spatial directions and phase angles. For the stapes, such movements can be divided into piston-like and rotational movements around its short and long axis (tilting or rocking motions). Unlike the piston-like vibrations, rotational rocking motions do not lead to a net volume displacement of cochlear fluid at a certain distance from the footplate. Therefore, according to the current theory of hearing, it is assumed that such tilting movements have no effect on hearing. A number of studies have shown, however, that tilting motions can lead to cochlear activity. Further research is needed to quantify this effect.

Neuroradiology of cholesteatomas.

The relevant aspects of cholesteatomas are reviewed with the emphasis on their diagnosis by using cross-sectional imaging. The indications and limitations of CT and MR imaging and the use of novel MR imaging techniques in the diagnosis of cholesteatomas are described. HRCT of the temporal bone has an excellent spatial resolution, thus even small soft-tissue lesions can be accurately delineated (high sensitivity). However, CT has poor specificity (ie, soft-tissue structures cannot be differentiated). MR imaging with the conventional sequences (T1WI, T2WI, postcontrast T1WI) provides additional information for distinguishing different pathologic entities and for accurately diagnosing primary (nonsurgical) and residual/recurrent (surgical) cholesteatomas. Higher diagnostic specificity is achieved by introducing DW-EPI, delayed postcontrast imaging, DW-non-EPI, and DWI-PROPELLER techniques. Studies using DW-non-EPI and DWI-PROPELLER sequences show promising results related to improved diagnostic sensitivity and specificity for even small (<5 mm) cholesteatomas, thus allowing avoidance of second-look surgery in the future.

Contrast enhanced IR-SSFP examination compared with T1-weighted turbo spin-echo imaging of cardiac tumors and tumorlike lesions.

PURPOSE: To show that the use of an early and delayed contrast enhanced multislice inversion recovery steady state free precession (SS-IR-SSFP) is a valuable substitute for conventional post-contrast fat saturated turbo spin-echo (TSE) T1-weighted images in the assessment of cardiac tumors. MATERIALS AND METHODS: 34 consecutive patients referred for MRI in order to assess cardiac tumors were examined. Shortly after administration of gadopenetate dimeglumine (Gd-DTPA) images were obtained using a SS-IR-SSFP sequence. The inversion time (TI) was set at 350ms to achieve a good demarcation of intracavitary tumor spread. Hereafter 9 slices of a T1w TSE sequence were obtained. Finally a SS-IR-SSFP sequence with an optimized TI to null normal myocardium was employed. Quantitative comparisons were performed by calculating contrast to noise ratios of tumor/myocardium (CNR(tumor/myo)) and CNR of tumor/left ventricular cavity (CNR(tumor/LVC)). Image quality was assessed regarding overall image quality, artifacts and tumor conspicuity. RESULTS: Neither calculation of CNR(tumor/LVC) when comparing the early IR-SSFP and T1w TSE, nor calculation of CNR(tumor/myo) when comparing the late IR-SSFP and T1w TSE sequence resulted in statistically significant differences. However, qualitative assessments revealed significant superior results for the early and the late IR-SSFP images compared to the T1-weighted TSE images (p<0.001). CONCLUSIONS: Image quality and tumor conspicuity were superior and image degradation by artifacts was less on IR-SSFP images compared to TSE images without loss of CNR. Thus the use of IR-SSFP sequences is an attractive alternative imaging method compared to post-contrast T1w TSE imaging in the assessment of cardiac tumors.

Bone conduction thresholds and skull vibration measured on the teeth during stimulation at different sites on the human head.

Vibratory auditory stimulation or bone conduction (BC) reaches the inner ear through both osseous and non-osseous structures of the head, but the contribution of the different pathways of BC is still unclear. In this study, BC thresholds in response to stimulation at several different locations including the eye were assessed, while the magnitudes of skull bone vibrations were measured on the front teeth in human subjects with either normal hearing on both sides or unilateral deafness with normal hearing on the other side. The BC thresholds with stimulation at the ipsilateral mastoid and ipsilateral temporal region were lower than the BC thresholds with stimulation at the other sites, as reported by previous works. The lower thresholds with stimulation at the ipsilateral mastoid and ipsilateral temporal region matched higher amplitudes of skull bone vibrations measured on the teeth, but only at frequencies below 1 kHz. With stimulation at the eye, the thresholds were significantly higher than those with stimulation at the bony sites in the frequency range of 0.25-4 kHz. While skull bone vibrations as measured on the teeth during stimulation at the eye were low for low frequencies, significant bone vibrations were measured at 3 and 4 kHz, indicating different pathways for BC for either the soft tissue or bony site stimulation. This finding contradicts a straightforward relationship between vibrations of the skull bones and BC hearing thresholds.

Prevalent vertebral fractures among children initiating glucocorticoid therapy for the treatment of rheumatic disorders.

OBJECTIVE: Vertebral fractures are an under-recognized problem in children with inflammatory disorders. We studied spine health among 134 children (87 girls) with rheumatic conditions (median age 10 years) within 30 days of initiating glucocorticoid therapy. METHODS: Children were categorized as follows: juvenile dermatomyositis (n = 30), juvenile idiopathic arthritis (n = 28), systemic lupus erythematosus and related conditions (n = 26), systemic arthritis (n = 22), systemic vasculitis (n = 16), and other conditions (n = 12). Thoracolumbar spine radiograph and dual x-ray absorptiometry for lumbar spine (L-spine) areal bone mineral density (BMD) were performed within 30 days of glucocorticoid initiation. Genant semiquantitative grading was used for vertebral morphometry. Second metacarpal morphometry was carried out on a hand radiograph. Clinical factors including disease and physical activity, calcium and vitamin D intake, cumulative glucocorticoid dose, underlying diagnosis, L-spine BMD Z score, and back pain were analyzed for association with vertebral fracture. RESULTS: Thirteen vertebral fractures were noted in 9 children (7%). Of these, 6 patients had a single vertebral fracture and 3 had 2-3 fractures. Fractures were clustered in the mid-thoracic region (69%). Three vertebral fractures (23%) were moderate (grade 2); the others were mild (grade 1). For the entire cohort, mean +/- SD L-spine BMD Z score was significantly different from zero (-0.55 +/- 1.2, P < 0.001) despite a mean height Z score that was similar to the healthy average (0.02 +/- 1.0, P = 0.825). Back pain was highly associated with increased odds for fracture (odds ratio 10.6 [95% confidence interval 2.1-53.8], P = 0.004). CONCLUSION: In pediatric rheumatic conditions, vertebral fractures can be present prior to prolonged glucocorticoid exposure.

[Aberrant internal carotid artery as a cause of pulsatile tinnitus: a difficult diagnosis in MRI?]. / Aberrierende A. carotis interna als Ursache eines pulssynchronen Tinnitus : Schwierige Diagnose in der MRT?

We present the case of a 43-year-old patient with sensorineural hearing loss and the finding of an aberrant internal carotid artery in the left tympanic cavity that was causing pulsatile tinnitus. The aberrant vessel was initially invisible on magnetic resonance imaging (MRI) and was confirmed by high-resolution computed tomography and MR angiography (MRA). Recognition of an aberrant course of an internal carotid artery often requires a combination of MRI and MRA to establish the diagnosis and rule out other differential diagnoses.

The Cutaneous Assessment Tool: development and reliability in juvenile idiopathic inflammatory myopathy.

OBJECTIVES: Clinical care and therapeutic trials in idiopathic inflammatory myopathies (IIM) require accurate and consistent assessment of cutaneous involvement. The Cutaneous Assessment Tool (CAT) was designed to measure skin activity and damage in IIM. We describe the development and inter-rater reliability of the CAT, and the frequency of lesions endorsed in a large population of juvenile IIM patients. METHODS: The CAT includes 10 activity, 4 damage and 7 combined lesions. Thirty-two photographic slides depicting IIM skin lesions were assessed by 11 raters. One hundred and twenty-three children were assessed by 11 paediatric rheumatologists at 10 centres. Inter-rater reliability was assessed using simple agreements and intra-class correlation coefficients (ICC). RESULTS: Simple agreements in recognizing lesions as present or absent were generally high (0.5-1.0). ICCs for CAT lesions were moderate (0.4-0.75) in both slides and real patients. ICCs for the CAT activity and damage scores were 0.71 and 0.81, respectively. CAT activity scores ranged from 0 to 44 (median 7, potential range 0-96) and CAT damage scores ranged from 0 to 13 (median 1, potential range 0-22). The most common cutaneous lesions endorsed were periungual capillary loop changes (63%), Gottron's papules/sign (53%), heliotrope rash (49%) and malar/facial erythema (49%). CONCLUSIONS: Total CAT activity and damage scores have moderate to good reliability. Assessors generally agree on the presence of a variety of cutaneous lesions. The CAT is a promising, semi-quantitative tool to comprehensively assess skin disease activity and damage in IIM.

Malleostapedotomy in revision surgery for otosclerosis.

PURPOSE: The purpose of this study was to analyze the results of malleostapedotomy and to compare them with those of a conventional incus stapedotomy in a series of 82 consecutive surgical revisions in otosclerotic patients. MATERIALS AND METHODS: 82 consecutive revision stapes surgery cases over 5 years were evaluated. The preoperative and postoperative audiometric data of 80 (97.5%) of the patients were obtained. RESULTS: 71 of the patients underwent a functional revision procedure as malleostapedotomy (56, 79%) or as incus stapedotomy (15, 21%). The most common cause of failure of primary surgery was a displaced or malfunctioning prosthesis (86.2%). Pathologic changes of the oval window were found in 80% of the cases. Problems of the incus were identified in 80% and abnormality of the malleus in 48.6% of the cases. The functional success rate of malleostapedotomy (closure within 10 dB) was found to be higher than that of traditional incus stapedotomy (p < 0.05). Overclosure was seen in 12 patients (17%) and a significant sensorineural hearing loss in 2 patients (3%). There were no dead ears in this series. The postoperative hearing results after first revision surgery were better than those after multiple surgical procedures (p < 0.05). CONCLUSIONS: Malleostapedotomy yields better functional hearing results than incus stapedotomy in revision surgery for otosclerosis. The detection of many malleus fixations was the result of the systematic exposure of the anterior malleal process and ligament through an endaural approach with superior canaloplasty.