“Natural history and treatment outcomes in Venolymphatic malformations of the Orbit”
Author: Adriaan Coumou
Base Hospital / Institution: UMC Groningen
Rapid fire oral presentation
Abstract ID: 24-506
Purpose
To retrospectively evaluate radiological and clinical outcomes for patients with venolymphatic or venous malformations (VLM/VM) of the orbit undergoing different treatment modalities, including sclerotherapy, surgery, systemic corticosteroids, or observation. Additionally, we aim to investigate the association between orbital VLM and intracranial vascular anomalies, such as developmental venous anomalies (DVA) and arteriovenous fistulas (AVF).
Methods
We retrospectively reviewed records of 31 patients diagnosed via MRI with VLM or VM in the orbit and/or periorbital area over 25 years. Clinical charts were analyzed to describe the natural history. Pre- and post-procedure volumetric MRI analysis was performed, with radiological responses classified as excellent (over 70% volume reduction) or good (50-70% volume reduction). Clinical responses were categorized as complete (full relief), significant (partial relief), none (no change), or deterioration (worsening symptoms). Additionally, MRI scans were evaluated by a single interventional radiologist for intracranial vascular anomalies, such as DVAs and AVFs.
Results
We analyzed 31 patients (mean age 20.1 years, range 0-54 years; 20 females) with 22 venolymphatic malformations (VLMs) and 9 venous malformations (VMs). Treatment included observation (11 patients, 35.5%), sclerotherapy (14 patients, 70%), surgery (10 patients, 50%), and sirolimus (2 patients, 10%). An excellent radiological response was seen in 2 patients (9.1%), and a significant response in 10 patients (45%). Complete and significant clinical responses were achieved in 13% (4 patients) and 55% (17 patients), respectively. During long-term follow-up, only 3 patients (1%) showed clinical deterioration. Additionally, 6 patients had a DVA and 3 had an AVF, with DVAs associated with extensive involvement of nearby structures.
Conclusion
Sclerotherapy with bleomycin is effective for large VLMs and VMs, with a low complication rate. For malformations with extensive involvement of nearby structures, brain imaging should be included in the diagnosis.
Additional Authors
| First name | Last name | Base Hospital / Institution |
|---|---|---|
| Erik | Buijs | Amsterdam UMC |
| Marta | Mira | Amsterdam UMC |
| Peerooz | Saeed | Amsterdam UMC |
