Management Strategies for Orbital Lymphatic-Venous Malformations: Insights from a Case Series

Abstract

INTRODUCTION: Orbital venous-lymphatic malformations (VLMs) are complex vascular anomalies with diverse clinical manifestations that pose significant therapeutic challenges due to their proximity to critical orbital structures and risk of visual loss. Surgery carries high risk and laser therapy is limited to superficial lesions, whereas imaging-guided sclerotherapy with bleomycin offers a minimally invasive, effective, and repeatable treatment, particularly for deep or retrobulbar VLMs. However, microcystic subtypes often respond poorly, with recent studies suggesting oral sirolimus as a promising alternative in refractory cases.

We aimed to describe the clinical presentation, management and outcomes of orbital VLM cases across different age groups.

METHODS: A retrospective study was conducted on selected patients with orbital VLMs treated at Unidade Local de Saude de Coimbra between July 2010 and September 2025 to represent the spectrum of management approaches available. All patients underwent ophthalmic examination and orbital imaging with computed tomography and/or magnetic resonance. Clinical photographs, treatment response, number of sessions, and procedure-related complications were systematically documented and analyzed.

RESULTS: Five patients (ages 9–52 years) with orbital VLMs were analyzed. Treatment modalities included intralesional bleomycin, oral sirolimus and surgical excision, with most patients undergoing multimodal therapy. Intralesional bleomycin achieved significant lesion regression and symptomatic improvement in all treated cases, although one patient required ongoing therapy for residual maxillary involvement. Sirolimus was used in two refractory cases, showing lesion stabilization, while surgical excision achieved complete resolution in two patients with well-circumscribed lesions. Visual acuity was preserved at 20/20 in four patients. No major ophthalmic or systemic complications were observed across treatment modalities.

CONCLUSION: Management of orbital VLMs remains challenging due to their proximity to critical structures, risk of recurrence and hemorrhagic complications. In this series, multimodal therapy—including intralesional bleomycin, sirolimus and surgery—was well tolerated, with no complications. Surgical excision and embolization achieved favorable outcomes in selected cases with well-defined lesions. Intralesional bleomycin provided effective lesion control and symptom relief, while sirolimus demonstrated promising results in refractory microcystic lesions. Overall, individualized, multimodal treatment strategies of orbital VLMs appear safe and effective, supporting their use across a wide range of patient ages, lesion types, and disease severities.