Ankylosing spondylitis (AS) is a chronic immune‐mediated inflammatory arthropathy primarily affecting the axial skeleton but may involve peripheral joints, particularly the hips. Avascular necrosis (AVN) of the femoral head represents a severe, underrecognized complication whose pathogenesis in AS is multifactorial—encompassing disease‐intrinsic inflammatory vasculopathy, iatrogenic glucocorticoid exposure, and, potentially, a prothrombotic state. We report the case of a 28‐year‐old HLA‐B27–positive male with a decade‐long history of AS who presented with bilateral AVN of the femoral heads superimposed on secondary hip osteoarthritis, compounded by severe microcytic anemia (hemoglobin 6.5 g/dL) and a recent COVID‐19 infection. The patient was initiated on infliximab (TNF inhibitor) for disease control and underwent staged bilateral total hip arthroplasty (left hip: March 2023; right hip: October 2024), achieving marked functional recovery. This case underscores the importance of prospective hip surveillance in AS, delineates the mechanistic nexus between systemic inflammation, corticosteroid use, and AVN, and highlights the perioperative considerations unique to biologic‐treated AS patients. A multidisciplinary rheumatology‐orthopedic approach is essential to optimize outcomes in this high‐complexity population.
Klippel‐Trenaunay syndrome (KTS) is a rare congenital vascular overgrowth disorder characterized by capillary and venous or lymphatic malformations with limb hypertrophy. Deep intramuscular involvement in children is uncommon and may resemble other vascular‐overgrowth syndromes. We report a 12‐year‐old boy with right lower‐limb swelling since birth, recurrent cellulitis, chronic ulceration and purulent discharge, progressive limb enlargement, and four previous soft‐tissue excisions. Examination showed bilateral nodular ulcerated lesions and vascular skin changes. Laboratory testing demonstrated persistent microcytic anemia and raised inflammatory markers. Doppler ultrasonography identified soft‐tissue hypertrophy, macrodactyly, calcified cavernous vascular lesions, superficial venous dilatation, incompetent perforators, and venous reflux, while deep veins remained patent. Contrast‐enhanced magnetic resonance imaging showed extensive intramuscular malformations involving the gastrocnemius and tibialis muscles without marrow or joint involvement. The calcified lesions initially raised concern for Maffucci syndrome; however, the absence of enchondromas and histopathologic confirmation of benign cavernous vascular malformations supported KTS. Progressive symptomatic masses were treated with surgical debulking, drainage, wound care, and culture‐directed antibiotics. Recovery was uneventful, with continued conservative surveillance. This case shows how combined clinical assessment, Doppler ultrasonography, magnetic resonance imaging, and histopathology can distinguish atypical pediatric KTS from skeletal dysplasias and guide treatment when genetic testing and multidisciplinary vascular services are limited.
Background: Pulmonary hypertension (PH) is associated with increased pulmonary arterial pressure and impairment of right ventricular (RV) function. Reliable characterization of RV performance is important for prognosis, risk assessment, and therapeutic planning. Cardiac magnetic resonance (CMR) is considered the reference technique for quantifying RV volumes and function, but its expense and limited availability can restrict routine implementation. Echocardiography is more widely accessible; however, the extent to which individual echocardiographic indices correspond with CMR-derived RV ejection fraction (RVEF) remains incompletely defined. Objective: Our objective was to determine the correlation of RV fractional area change (RV-FAC), RV free-wall longitudinal strain (RV-FWLS), and RV global longitudinal strain (RV-GLS) with CMR-derived RVEF in individuals with PH. Methods: PubMed, Embase, ClinicalTrials.gov, Scopus, and the Cochrane Library were searched, yielding 404 records; six studies fulfilled the eligibility criteria. Studies were eligible when they enrolled patients with PH and provided extractable correlations between at least one prespecified echocardiographic measure and CMR-derived RVEF. Correlation coefficients were converted with Fisher’s z transformation, combined using common-effect and random-effects approaches, and then transformed back to correlation coefficients. Pooled mean RV-FAC was evaluated as a separate outcome. Between-study heterogeneity was quantified with I2 and τ2, with random-effects estimates emphasized for interpretation. Results: Each evaluated echocardiographic measure was positively correlated with CMR-derived RVEF. RV-FWLS had the largest pooled correlation and the greatest consistency (r = 0.79, 95% CI: 0.73–0.83; I2 = 0.0%). The pooled correlation for RV-GLS was 0.75 (95% CI: 0.60–0.85; I2 = 80.3%), while that for RV-FAC was 0.72 (95% CI: 0.61–0.80; I2 = 61.8%). The random-effects pooled mean RV-FAC was 31.10% (95% CI: 26.77–35.43), with substantial heterogeneity (I2 = 90.9%). Conclusions: RV-FWLS showed the highest numerical and most consistent association with CMR-derived RVEF, suggesting that it may serve as a useful non-invasive adjunct for RV assessment in PH, especially where CMR is not readily available. Larger studies using harmonized acquisition and analysis methods are required for confirmation.