Localized strain characterization of cardiomyopathy in Duchenne muscular dystrophy using novel 4D kinematic analysis of cine cardiovascular magnetic resonance.

BACKGROUND: Cardiomyopathy (CMP) is the most common cause of mortality in Duchenne muscular dystrophy (DMD), though the age of onset and clinical progression vary. We applied a novel 4D (3D + time) strain analysis method using cine cardiovascular magnetic resonance (CMR) imaging data to determine if localized strain metrics derived from 4D image analysis would be sensitive and specific for characterizing DMD CMP. METHODS: We analyzed short-axis cine CMR image stacks from 43 DMD patients (median age: 12.23 yrs [10.6-16.5]; [interquartile range]) and 25 male healthy controls (median age: 16.2 yrs [13.3-20.7]). A subset of 25 male DMD patients age-matched to the controls (median age: 15.7 yrs [14.0-17.8]) was used for comparative metrics. CMR images were compiled into 4D sequences for feature-tracking strain analysis using custom-built software. Unpaired t-test and receiver operator characteristic area under the curve (AUC) analysis were used to determine statistical significance. Spearman's rho was used to determine correlation. RESULTS: DMD patients had a range of CMP severity: 15 (35% of total) had left ventricular ejection fraction (LVEF) > 55% with no findings of myocardial late gadolinium enhancement (LGE), 15 (35%) had findings of LGE with LVEF > 55% and 13 (30%) had LGE with LVEF < 55%. The magnitude of the peak basal circumferential strain, basal radial strain, and basal surface area strain were all significantly decreased in DMD patients relative to healthy controls (p < 0.001) with AUC values of 0.80, 0.89, and 0.84 respectively for peak strain and 0.96, 0.91, and 0.98 respectively for systolic strain rate. Peak basal radial strain, basal radial systolic strain rate, and basal circumferential systolic strain rate magnitude values were also significantly decreased in mild CMP (No LGE, LVEF > 55%) compared to a healthy control group (p < 0.001 for all). Surface area strain significantly correlated with LVEF and extracellular volume (ECV) respectively in the basal (rho = - 0.45, 0.40), mid (rho = - 0.46, 0.46), and apical (rho = - 0.42, 0.47) regions. CONCLUSION: Strain analysis of 3D cine CMR images in DMD CMP patients generates localized kinematic parameters that strongly differentiate disease from control and correlate with LVEF and ECV.

Surgical Management of Tophaceous Gout in the Upper Limb.

PURPOSE: Gouty tophi are a clinical manifestation of hyperuricemia in advanced stages. They can produce pain, functional limitation, and severe deformities. Patients with severe symptoms require short-term symptomatic solutions that standard medical management is not able to provide. The objective of this study was to present the results obtained with the surgical management of tophaceous gout in the upper limb, as well as present a detailed characterization of the disease in the upper limb. METHODS: Databases of the hand surgery service of a quaternary care hospital were reviewed to identify patients aged >18 years old undergoing tophi resection in the upper limbs between 2014 and 2020. Medical history records were reviewed retrospectively, and the relevant data were extracted to establish demographic profile, clinical presentation, anatomic distribution, postoperative outcomes, and additional procedures required. RESULTS: The most frequent symptom was pain (83%), followed by limited range of motion (56%), deformity (50%), and daily living/occupational activity limitation (28%). The main indications for surgical management were the presence of deformity, pain, and/or limited range of motion. The most frequently affected anatomic sites were the metacarpophalangeal joints, followed by the elbows, proximal interphalangeal joints, and proximal phalanges. The postoperative complication rate was 28%. The most common complications were operative site infections and wound dehiscence. Decreased pain was associated with surgical resection. Additional procedures, such as extensor tenorrhaphy and local flaps, were required in 47.2% of patients. CONCLUSION: Surgical resection of tophi can decrease pain. Although surgery is associated with a high rate of complications, most are minor. TYPE OF STUDY/LEVEL OF EVIDENCE: Therapeutic IV.