Percutaneous Drainage and Ablation as First Line Therapy for Macrocystic and Microcystic Orbital Lymphatic Malformations

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Abstract

Purpose:

To review the management of orbital lymphangi-omas and to propose a new treatment for both macrocystic and microcystic lymphatic malformations of the orbit.

Methods:

A retrospective case series of all patients from the authors’ practice from 2001 to the present who met the histopathologic and/or diagnostic imaging criteria for orbital lymphatic malformation was reviewed. Lymphatic malformation was diagnosed if there was a multilobulated pattern on CT or a cystic internal structure on ultrasonography. In patients that were treated, macrocysts (≥1 cm) were treated with dual-drug chemoablation (sequential intracystic sodium tetradecyl sulfate and ethanol); doxycycline injections were used for microcysts. The goal of treatment was complete cyst ablation documented by ultrasonography or MRI.

Results:

Twenty patients met the inclusion criteria. They were separated in 3 groups based on the anatomical location of the lymphatic malformation: deep, superficial, or combined. Deep orbital lymphatic malformation presented in 14 patients (70%), superficial presented in 4 patients (20%), and both deep and superficial presented in 2 patients (10%). Thirteen of the 20 patients underwent percutaneous sclerotherapy. Of those treated, 7 patients (53.8%) had lymphatic malformations (LM), while 6 patients (46.2%) had venous-lymphatic malformations (VLMs). The average number of treatments required to achieve complete cyst ablation in patients with LM was 1.7. The average number of treatments required for patients with VLM was 3.0; however, some of these patients continue to have the venous component of their lesions treated. Clinically, all treated patients maintained or improved an average of one Snellen line (–0.16 decimal Snellen equivalent) from their preoperative visual acuity to their last recorded follow-up visit. There was a mean reduction in proptosis of 2.4 mm (p = 0.003, confidence interval [CI] 0.838 to 3.962), which was statistically significant. There were no recurrences (0%) in patients who completed treatment with cyst ablation (n = 8) at an average follow-up period of 43 months (range 6–96, standard deviation 30). There were no data available as to the recurrence status of one patient. Four patients were still undergoing treatment for a venous component at the time of this review.

Conclusions:

Percutaneous sclerotherapy provides a safe and effective treatment for both macrocystic and microcystic orbital lymphatic malformations as a primary treatment or for recurrence after surgical intervention.

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