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Purpose of Review <p>Venous-lymphatic malformations of the orbit are congenital, low-flow vascular anomalies composed of mixed venous and lymphatic channels that can cause intermittent proptosis, hemorrhage, and compressive optic neuropathy. We present a summary of clinical evaluation, imaging, surgical management, sclerotherapy, and medical treatment.</p> Recent Findings <p>Treatment is indicated for vision-threatening, disfiguring, or symptomatic lesions, with the goal of relieving orbital pressure, preserving vision, and improving cosmesis. Recent studies of medical therapeutics, including sirolimus and <i>PIK3CA-</i>targeted molecular therapy, have demonstrated promising results, though larger trials have yet to be performed. Newer sclerosing agents, especially doxycycline and bleomycin, may be more effective and well-tolerated than traditional agents. Enhanced surgical strategies such as the use of fibrin glue or adjunctive sclerotherapy are likely to increase efficacy while decreasing intra-operative complications.</p> Summary <p>Multiple treatment options exist for orbital venous-lymphatic malformations; therapy must be selected based on urgency, lesion characteristics, and patient goals. Certain sclerosing agents and oral sirolimus are likely to achieve satisfactory clinical responses, though surgical resection is the mainstay for urgent debulking.</p>

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Update on Treatment of Orbital Venous-Lymphatic Malformations

  • 期刊论文

  • 发表日期：

    2025年12月4日

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  • Tahvi Frank,
  • Nickisa Hodgson,
  • Rabih Hage

摘要

Purpose of Review

Venous-lymphatic malformations of the orbit are congenital, low-flow vascular anomalies composed of mixed venous and lymphatic channels that can cause intermittent proptosis, hemorrhage, and compressive optic neuropathy. We present a summary of clinical evaluation, imaging, surgical management, sclerotherapy, and medical treatment.

Recent Findings

Treatment is indicated for vision-threatening, disfiguring, or symptomatic lesions, with the goal of relieving orbital pressure, preserving vision, and improving cosmesis. Recent studies of medical therapeutics, including sirolimus and PIK3CA-targeted molecular therapy, have demonstrated promising results, though larger trials have yet to be performed. Newer sclerosing agents, especially doxycycline and bleomycin, may be more effective and well-tolerated than traditional agents. Enhanced surgical strategies such as the use of fibrin glue or adjunctive sclerotherapy are likely to increase efficacy while decreasing intra-operative complications.

Summary

Multiple treatment options exist for orbital venous-lymphatic malformations; therapy must be selected based on urgency, lesion characteristics, and patient goals. Certain sclerosing agents and oral sirolimus are likely to achieve satisfactory clinical responses, though surgical resection is the mainstay for urgent debulking.