Disease specific determinants of cardiopulmonary fitness in systemic sclerosis.

OBJECTIVES: We aimed to quantify the burden of exercise intolerance in systemic sclerosis (SSc) and explore the disease features that contribute to impaired exercise capacity (measured as peak oxygen uptake, peak VO2) to provide novel mechanistic insights into the causes of physical disability in SSc. METHODS: Thirty-three SSc patients with no history of cardiac disease and no active myositis underwent cardiac and skeletal muscle MRI, transthoracic echocardiography, pulmonary function tests and cardiopulmonary exercise testing (CPET). CPET results were compared to an age-, sex-, and weight-matched controls with no overt cardiopulmonary disease. Native T1 and T2-mapping sequences were used to quantify diffuse fibroinflammatory myocardial disease and qualitative assessment of skeletal muscle oedema was performed. The associations between parameters of cardiorespiratory function and skeletal muscle abnormalities and peak VO2 were evaluated with linear regression analysis. RESULTS: Exercise capacity was markedly impaired in SSc and significantly reduced when compared to control subjects (percent predicted peak VO2: 70% vs 98%, p < 0⋅01). Diffuse myocardial fibroinflammatory disease (p < 0⋅01) and skeletal muscle oedema (p = 0⋅01) were significantly associated with reduced exercise capacity. There was no association between impaired exercise capacity and left ventricular ejection fraction. CONCLUSION: SSc is associated with marked functional impairment that is not explained by commonly used parameters of cardiac function such as left ventricular ejection fraction. Rather, only more sensitive measures of organ involvement are associated with impaired exercise tolerance. Our results show diffuse interstitial changes of the myocardium and skeletal muscle affect oxygen uptake and are important contributors to functional limitation in SSc.

Using magnetic resonance imaging to map the hidden burden of muscle involvement in systemic sclerosis.

BACKGROUND: Skeletal muscle can be directly affected by systemic sclerosis (SSc); however, a significant burden of SSc-associated myopathy is undetected because clinical parameters such as weakness and creatine kinase (CK) are unreliable biomarkers of muscle involvement. This study presents qualitative and quantitative magnetic resonance imaging (MRI) findings that quantify the prevalence of myopathy and evaluate any association between skeletal and cardiac muscle involvement in SSc. METHODS: Thirty-two patients with SSc who fulfilled the 2013 American College of Rheumatology/European League Against Rheumatism classification criteria underwent skeletal muscle MRI in addition to cardiac MRI. Skeletal muscles were independently assessed by two musculoskeletal radiologists for evidence of oedema, fatty infiltration and atrophy. Skeletal muscle T2 mapping times and percentage fat fraction were calculated. Linear regression analysis was used to evaluate the clinical and myocardial associations with skeletal muscle oedema and fatty infiltration. Cardiac MRI was performed using post gadolinium contrast imaging and parametric mapping techniques to assess focal and diffuse myocardial fibrosis. RESULTS: Thirteen participants (40.6%) had MRI evidence of skeletal muscle oedema. Five (15.6%) participants had fatty infiltration. There was no association between skeletal muscle oedema and muscle strength, creatine kinase, inflammatory markers or fibroinflammatory myocardial disease. Patients with skeletal muscle oedema had higher T2-mapping times; there was a significant association between subjective assessments of muscle oedema and T2-mapping time (coef 2.46, p = 0.02) and percentage fat fraction (coef 3.41, p = 0.02). Diffuse myocardial fibrosis was a near-universal finding, and one third of patients had focal myocardial fibrosis. There was no association between skeletal myopathy detected by MRI and burden of myocardial disease. CONCLUSIONS: MRI is a sensitive measure of muscle oedema and systematic assessment of SSc patients using MRI shows that myopathy is highly prevalent, even in patients without symptoms or other signs of muscle involvement. Similarly, cardiac fibrosis is highly prevalent but occurs independently of skeletal muscle changes. These results indicate that novel quantitative MRI techniques may be useful for assessing sub-clinical skeletal muscle disease in SSc.

Dual-energy contrast-enhanced CT to evaluate scaphoid osteonecrosis with surgical correlation.

To evaluate the validity of contrast enhanced dual energy CT using a lung perfusion algorithm in assessing for post-traumatic scaphoid proximal pole avascular necrosis. From Aug 2013 to Aug 2016, 18 patients (19 wrists, 16 males, 2 females, mean age 28 years) were assessed as high-risk for proximal pole scaphoid avascular necrosis by a single surgeon following a scaphoid fracture and were referred for contrast-enhanced dual energy CT. 8 wrists had specimens sent for correlative histological analysis and 11 were correlated with operative notes. Eight surgical specimens were sent to histology and showed a 100% correlation (8/8) with the DECT findings. The remaining 11 wrists that did not have a specimen sent had in-surgery findings that also correlated with DECT. A single case was discrepant (1/11) due to presence of an intra-osseous ganglion, which was reported as osteonecrosis on CT, but considered viable at surgery. No case was called viable on CT that proved to be necrotic at either surgery or histologically. Contrast-enhanced dual energy CT using a perfusion algorithm is an innovative and promising method in evaluating viability of the post-trauma proximal pole of scaphoid.

Prevalence of Extracranial Venous Narrowing on Magnetic Resonance Venography Is Similar in People With Multiple Sclerosis, Their Siblings, and Unrelated Healthy Controls: A Blinded, Case-Control Study.

PURPOSE: The study sought to assess and compare the prevalence of narrowing of the major extracranial veins in subjects with multiple sclerosis and controls, and to assess the sensitivity and specificity of magnetic resonance venography (MRV) for describing extracranial venous narrowing as it applies to the chronic cerebrospinal venous insufficiency theory, using catheter venography (CV) as the gold standard. METHODS: The jugular and azygos veins were assessed with time-of-flight MRV in this assessor-blinded, case-control study of subjects with multiple sclerosis, their unaffected siblings, and unrelated controls. The veins were evaluated by diameter and area, and compared with CV. Collateral vessels were also analyzed for maximal diameter, as a potential indicator of compensatory flow. RESULTS: A high prevalence of extracranial venous narrowing was demonstrated in all study groups, collectively up to 84% by diameter criteria and 90% by area, with no significant difference between the groups when assessed independently (P = .34 and .63, respectively). There was high interobserver variability in the reporting of vessel narrowing (kappa = 0.32), and poor vessel per vessel correlation between narrowing on MRV and CV (kappa = 0.064). Collateral neck veins demonstrated no convincing difference in maximum size or correlation with jugular narrowing. CONCLUSION: There is a high prevalence of narrowing of the major extracranial veins on MRV in all 3 study groups, with no significant difference between them. These findings do not support the chronic cerebrospinal venous insufficiency theory. Although MRV has shown a high sensitivity for identifying venous narrowing, time-of-flight imaging demonstrates poor interobserver agreement and poor specificity when compared with the gold standard CV.

A Prospective Pilot Investigation of Brain Volume, White Matter Hyperintensities, and Hemorrhagic Lesions after Mild Traumatic Brain Injury.

Traumatic brain injury (TBI) is among the most common neurological disorders. Hemorrhagic lesions and white matter hyperintensities (WMH) are radiological features associated with moderate and severe TBI. Brain volume reductions have also been observed during the months following injury. In concussion, no signs of injury are observed on conventional magnetic resonance imaging (MRI), which may be a true feature of concussion or merely due to the limited sensitivity of imaging techniques used so far. Moreover, it is not known whether volume reductions are due to the resolution of trauma-related edema or a true volume loss. Forty-five collegiate-level ice hockey players (20 females) and 15 controls (9 females), 40 players underwent 3-T MRI for hemorrhages [multi-echo susceptibility-weighted imaging (SWI)], WMH (three-dimensional fluid-attenuated inversion recovery), and brain volume at the beginning and the end of the hockey season. Concussed athletes underwent additional imaging and neuropsychological testing at 3 days, 2 weeks, and 2 months after injury. At the end of the hockey season, brain volume was reduced compared to controls by 0.32% (p < 0.034) in the whole cohort and by 0.26% (p < 0.09) in the concussed athletes. Two weeks and 2 months after concussion, brain volume was reduced by -0.08% (p = 0.027) and -0.23% (p = 0.035), respectively. In athletes, the WMH were significantly closer to the interface between gray matter and white matter compared to controls. No significant changes in the number of WMH over the duration of the study were found in athletes. No microhemorrhages were detected as a result of concussion or playing a season of ice hockey. We conclude that mild TBI does not lead to transient increases in brain volume and no new microbleeds or WMH are detectable after concussion. Brain volume reductions appear by 2 weeks after concussion and persist until at least 2 months after concussion. Brain volume is reduced between the beginning and the end of the ice hockey season.

Gouty arthropathy: Review of clinico-pathologic and imaging features.

Gout is a common inflammatory arthropathy in adults, with the prevalence increasing in males of older age. It occurs when monosodium urate (MSU) crystals are deposited in joints and connective tissue causing inflammation. The gold standard for the diagnosis of gout is the demonstration of negatively birefringent, needle-shaped MSU crystals through synovial fluid aspiration. However, this is an invasive technique and may not always be conclusive or feasible. Imaging techniques have been developed to aid in diagnosis of gout non-invasively. Radiography has a low utility in the early diagnosis of gout and demonstrates erosions in late stages. Ultrasound (US) has a high overall sensitivity in diagnosing gout with the 'double contour' sign having a high specificity. Magnetic resonance imaging is good at detecting tophi, bone marrow oedema and erosions, but has a limited role in diagnosis because of its high cost and limited availability. Conventional computed tomography (CT) has no role in the routine diagnosis of gout before development of erosions and tophi. A newer technology, dual-energy CT (DECT) has been shown to be able to detect MSU crystals burden with high accuracy. It has a higher specificity and lower sensitivity that US in gout diagnosis. However, because of radiation exposure and cost, it has a better utility in diagnosing clinically suspected gout complicated by other concurrent rheumatologic conditions or if radiography, US and synovial aspiration are inconclusive or not feasible. This paper will review the clinico-pathologic and imaging features of gouty arthropathy.

Biopsy and the diagnostic evaluation of musculoskeletal tumours: critical but often missed in the 21st century.

Bone and soft-tissue sarcomas are rare and heterogeneous malignancies arising from tissues of mesenchymal origin. Treatment planning is informed by accurate diagnosis for which biopsy is the diagnostic standard. Biopsy in the setting of suspected malignancy is a technically challenging procedure that should only be performed at specialist institutions. Without the requisite expertise, they can compromise the viability of reconstructive procedures and may make necessary amputation to achieve adequate surgical margins. The risk of complications arising from the procedure must be minimized and therefore biopsy should always be preceded by imaging. There must be no attempt at biopsy or excision prior to referral if there is any suspicion of malignancy. Patients with suspected bone and soft-tissue tumours are best evaluated and treated at specialist sarcoma centres under the care of expert multidisciplinary teams. Prompt referral to a specialist sarcoma centre should always be made prior to biopsy for any suspicious mass that is painful, progressively increasing in size, greater than 5 cm in diameter, deep to deep fascia or recurs following inadvertent excision.

Musculoskeletal desmoid tumours: Pre- and post-treatment radiological appearances.

This study was aimed to illustrate the pre- and post-treatment imaging findings of musculoskeletal desmoid tumours and describe current treatment methods. Imaging of histologically proven cases of desmoid tumours at St. Vincent's Hospital, Melbourne, were obtained via picture archiving communication system (PACS) and then assessed by two musculoskeletal radiologists. Suitable imaging both pre- and post-treatment were then obtained from PACS. All imaging chosen were de-identified. Ninety-two patients were found to have histologically proven cases of desmoid tumours between January 2000 and December 2013. Six patients with extra-abdominal tumours were selected, where pre- and post-treatment imaging was available. Desmoid tumours can occur in many areas of the body. Treatment of desmoids are varied. Although wide-margin surgery has been the traditional form of treatment, it still cannot guarantee absence of tumour recurrence despite microscopically tumour-free margins. Other forms of treatment such as non-steroidal anti-inflammatory drugs, radiotherapy, chemotherapy, tyrosine kinase inhibitors and also the conservative 'watch and wait' approach have been suggested, which show varying results.

Musculoskeletal desmoid tumours: Diagnostic imaging appearances.

This study aimed to discuss the role medical imaging has on diagnosis of musculoskeletal desmoid tumours and to describe their radiological appearances on various imaging modalities. Imaging of histologically proven cases of desmoid tumours at St. Vincent's Hospital Melbourne were obtained via picture archiving communication system (PACS) and then assessed by two musculoskeletal radiologists. Suitable imagings were obtained from PACS. All imaging chosen was de-identified. Desmoid tumours can occur in many areas of the body. Imaging plays an important role in the diagnosis of these tumours and magnetic resonance imaging has been the gold standard for imaging and is the most accurate in terms of assessing tumour margins and involvement of surrounding structures.

Imaging assessment of congenital and acquired abnormalities of the portal venous system.

Abnormalities of the portal venous system are a heterogeneous group of conditions that can cause substantial morbidity and mortality and may lead to complications during surgery or percutaneous interventions involving the portal venous system. High-resolution computed tomography, ultrasonography, and magnetic resonance imaging permit a comprehensive, noninvasive evaluation of the portal venous system, enabling the detection of both structural and functional abnormalities. However, an understanding of the embryologic development of the normal portal venous anatomy and anatomic variants is essential to accurately interpret the imaging findings. Knowledge of the characteristic appearances of abnormalities of the portal venous system allows a more confident diagnosis, permitting timely treatment and more informed guidance of surgical procedures and percutaneous interventions, which may lead to an improved outcome.

Clinical Images. An unusual case of appendicitis.

Situs inversus may be identified as an incidental finding when investigating abdominal pain in the acute emergency setting. We report a case of a 46-year-old man who presented with left-sided acute appendicitis on a background of situs inversus. The clinical presentation was left lower-quadrant pain. Clinically, the diagnosis of diverticulitis was made until review of plain-film imaging raised the possibility of situs inversus, and thus appendicitis. Cross-sectional imaging studies and laparoscopy confirmed the diagnosis of acute left-sided appendicitis. He underwent laparoscopic appendectomy with an uncomplicated postoperative course.