The acronym FAVA (Fibro-Adipose Vascular Anomaly) has been recently given to a distinct vascular entity that is characterized by fibrofatty infiltration of muscle, unusual phlebectasia with pain, and contracture of the affected extremity. We report a new case of FAVA in a 10-year-old girl with pain in her right lower leg and equinus contracture. As in our case, FAVA typically presents in young females with calf involvement and limited ankle dorsiflexion with local pain. FAVA should be considered as a differential diagnosis when evaluating vascular anomalies in the lower extremities.
Since the introduction of propranolol in the treatment of complicated infantile hemangiomas (IH) in 2008, other different beta-blockers, including timolol, acetabutolol, nadolol and atenolol, have been successfully used for the same purpose. Various hypotheses including vasoconstriction, inhibition of angiogenesis and the induction of apoptosis in proliferating endothelial cells have been advanced as the potential beta-blocker-induced effect on the accelerated IH involution, although the exact mechanism of action of beta-blockers remains unknown. This has generated an extraordinary interest in IH research and has led to the discovery of the role of the renin-angiotensin system (RAS) in the biology of IH, providing a plausible explanation for the beta-blocker induced effect on IH involution and the development of new potential indications for RAS drugs such as angiotensin-converting enzyme inhibitors and angiotensin-receptor blockers in the treatment of IH. This review is focused on the current use of cardiovascular drugs in the treatment of IH.
Purpose
Pathologic fractures occur in 5-10% of pediatric osteosarcoma cases and have historically been considered a contraindication to limb salvage. We purposed to describe the radiographic features of pathologic fracture and examine its impact on local recurrence rates, functional outcomes and overall survival.
Methods
We retrospectively analyzed patients at our institution from 1990-2015 with pathologic fracture at diagnosis or during neoadjuvant chemotherapy. We selected a control group of 50 osteosarcoma patients of similar age and gender without pathologic fracture from 1990-2015. Functional outcomes were scored using Musculoskeletal Tumor Society (MSTS) criteria. Chi square test was used for comparative analysis of groups.
Results
Thirty-six patients with 37 pathologic fractures form the study cohort. Of patients who received surgery, 18/34 patients with fracture underwent amputation, compared to 8/48 in the non-fracture group (p=0.007). Indications for amputation in fracture patients were tumor size (n=7), neurovascular involvement (n=6), and tumor progression during neoadjuvant chemotherapy (n=5). Only one patient (2.9%) in the fracture group who underwent limb salvage suffered local recurrence. Of patients who received neoadjuvant chemotherapy, 25/34 fracture patients showed poor histological response, compared to 24/47 non-fracture patients. (p=0.044) There was no statistically significant difference in overall survival between the two groups (p=0.96). Functional outcomes were significantly lower in fracture patients (median=17.5) than non-fracture patients (median=24) (p=0.023).
Conclusions
Radiographic features of pathologic fractures were highly variable in this population. Limb salvage surgery can be performed without increased risk of local recurrence. Patients with pathologic fracture suffer worse functional outcomes, but show no decrease in overall survival.
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