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Faculty of Medical Sciences

“Natural History and Treatment Outcomes of Venolymphatic Malformations of the Orbit”

da Veiga Fernandes de Mira, Marta (2024) “Natural History and Treatment Outcomes of Venolymphatic Malformations of the Orbit”. thesis, Medicine.

Full text available on request.

Abstract

Purpose: This study retrospectively evaluated the progression of orbital venolymphatic and venous malformations (OVLM/VM) and assessed imaging and clinical outcomes for patients treated with sclerotherapy, surgery, sirolimus, or 'watchful waiting.' We also explored the association between orbital VLMs and intracranial vascular anomalies (IAs). Methods: Patients diagnosed with a VLM or VM in the orbit and/or periorbital area via MRI or CT-scan over a 34-year period were retrospectively reviewed and placed into either the intervention or 'watchful waiting' group. All MRI-scans were reviewed by an interventional radiologist. Lesion size was assessed qualitatively on T1/T2-weighted MRI at baseline and post-procedure. Clinical and imaging responses were categorized by outcome. Results: Thirty-two patients (24 VLMs; 8 VMs) were included. Of these, 21 (65.6%) underwent intervention (sclerotherapy n=14 [66.7%]; surgery n=5 [23.8%]; sirolimus n=2 [9.5%]), while 11 (34.4%) were managed with "watchful waiting." Clinical responses were complete in 4 (19%), significant in 13 (61.9%), unchanged in 3 (14.3%), and deteriorated in 1 (4.8%). Imaging outcomes showed significant reduction in 13 (61.9%), mild reduction in 4 (19%), no change in 2 (9.5%), and deterioration in 2 (9.5%). Sclerotherapy (ST) led to 73% of treatment successes. Symptomatic recurrences occurred in 9 patients (64.3%) of the ST group. The correlation between clinical and imaging outcomes was significant (Spearman’s ρ=0.703, p=0.0024). IAs were present in 7 patients (29.9%) and were linked to extensive involvement of nearby structures (p=0.005), particularly the neck (p=0.0173). Conclusion: The variable and unpredictable natural progression of OVLMs suggests treatment should be guided by clinical presentation rather than imaging. Young patients with extensive lesions often need immediate intervention, while those initially under 'watchful waiting' often require treatment later. Sclerotherapy with Bleomycin is safe and highly effective, but frequent recurrences require ongoing treatment. An association with IAs was found, warranting brain MRI for cases with extensive involvement. This abstract will be presented at the 42nd Annual Meeting of the European Society of Ophthalmic Plastic & Reconstructive Surgery (ESOPRS).

Item Type: Thesis (UNSPECIFIED)
Supervisor name: Prof. dr. Saeed, Peerooz and Drs. Coumou, Adriaan
Faculty: Medical Sciences
Date Deposited: 06 Sep 2024 12:58
Last Modified: 06 Sep 2024 12:58
URI: https://umcg.studenttheses.ub.rug.nl/id/eprint/3753

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