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Institution: CHRISTIAN MEDICAL COLLEGE VELLORE - TAMIL NADU, India
Purpose: Fibroadipose vascular anomaly (FAVA), a recent entry in the nomenclature of vascular malformations was first introduced by Alomari et al. in 2014 followed by multiple case series around the world.This study,the largest in the world, aims to shine light on this entity which is commonly misdiagnosed and managed suboptimally.
Methodology: A retrospective study of 50 patients diagnosed with FAVA at the vascular surgery department at Christian Medical College, Vellore from August 2016 to February 2022 were included in our study.The clinical, radiological, pathological data, procedures performed, and the outcomes were analysed.Patients were followed up for minimum of 6 months clinically and with ultrasound if recurrence was suspected.
Results: Patients were initially diagnosed most commonly misdiagnosed as venous malformation in 22 patients. The gender distribution was equal in number with an age ranging from 6 years to 63 years. The calf was involved in majority of the cases.The overlying skin was spared in all cases. Surgical resection provided the maximum relief of symptoms as most of the resections were done after multiple attempts with other modalities especially sclerotherapy. Postoperatively 5 patients had a seroma and 2 surgical site infections which were managed conservatively. No recurrence was noted in the cohort.
Conclusion: Fibroadipose vascular anomaly (FAVA), though a recently named pathology, it has evolved rapidly and is no longer a rare entity. To our knowledge this is the largest case series reported in English literature. It is best managed in a multi- disciplinary approach involving vascular surgery, interventional radiology, pathology and occupational therapy. Surgical excision appears to be the best form of treatment. In summary, a proper diagnosis based on clinical findings and imaging is crucial which when followed by appropriate management would improve the outcome.Recent advances like targeting mTOR pathways seem worth exploring.