Successful Treatment of Orbital Lymphatic Malformation Using Foam Sclerotherapy with 3% Sodium Tetradecyl Sulfate: A Case Report

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Dear Editor,

Orbital lymphatic malformations (LMs), also known as lymphangiomas, are benign, hamartomatous vascular lesions primarily found in children. Historically, complex surgeries had been tried as a treatment, leading to high recurrence and poor visual outcomes. Recently, percutaneous intralesional sclerotherapy, especially with bleomycin, OK-432 (Picibanil), and sodium tetradecyl sulfate (STS), has become a preferred initial treatment due to fewer complications and better outcomes [1,2]. Written informed consent for publication of the research details and clinical images in this case report was obtained from the patient.

A 75-year-old woman, with a medical history of papillary thyroid carcinoma, presented with 6-day history of binocular diplopia and left-eye proptosis. Orbital magnetic resonance imaging identified a lymphatic or venolymphatic malformation within the left orbit (Fig. 1A–1D). Despite her visual acuity being preserved at 20 / 20 and normal light reflexes in both eyes, she exhibited restricted extraocular motility in all directions, with marked impairment of supraduction rated at −2, and experienced worsened vertical binocular diplopia upon upward gaze. Upon examination, the patient exhibited a proptosis in the left eye, which measured 18 mm by exophthalmometry, a notable increase from the 15 mm observed in the right eye. Treatment was initiated with 8 mg/day of oral methylprednisolone, along with 2 mg/day of sirolimus. One week after the treatment, the patient complained of continuing vertical diplopia and agreed to more potent treatment. Subsequent magnetic resonance imaging scans of the orbit showed no major changes since the last imaging. The decision was made to have a percutaneous foam sclerotherapy under imaging guidance. During the sclerotherapy procedure, an angiographic study confirmed the lesion as an orbital LM. The procedure was performed under local anesthesia, percutaneously with imaging guidance in the hybrid operating room. Once the sclerotherapy catheter was positioned within the LM, lymphatic fluid was drained, and the lesion was thoroughly flushed with saline. A mixture of 0.5 mL STS and 2 mL of room air was used to create the foam for the sclerotherapy, which was then administered (Fig. 1E, 1F, and Supplementary Video 1). Intraoperative computed tomography imaging confirmed the successful injection of the STS foam into the LM, with minimal leakage occurring (Fig. 1G, 1H). The patient’s gaze limitation, proptosis, and binocular diplopia showed improvement the following day. Her vision and intraocular pressure remained normal, despite some bruising around the left eye. Four weeks after the treatment, an orbital computed tomography scan verified the complete regression of the orbital LM, no proptosis and gaze limitations were observed (Fig. 1I, 1J).

Fig. 1

Diagnostic and therapeutic imaging in the management of orbital lymphatic malformation patient. T2- and T1-weighted magnetic resonance imaging images show a 3.6 mL volume (23 × 20 × 15-mm size) of lymphatic malformation or venolymphatic malformation in the orbit, particularly involving the inferior area. (A–D) The lymphatic malformation consists of fluid-filled spaces of varying sizes and has caused proptosis in the left eye. (E,F) The image taken during sclerotherapy, with angiography, demonstrates that the sclerosant was well applied within the malformation. (G,H) Intraoperative computed tomography images reveal the sclerosant clearly instilled into the lesion, highlighting the targeted treatment area as well. (I,J) Postoperative computed tomography images taken 1 month after surgery show significant regression of the malformation, indicative of successful therapeutic intervention.

LMs are histologically characterized by dilated sacs or locules lined with endothelium and supported by connective tissue, contain protein-rich fluid. These lesions, which can be macrocystic, microcystic, or a mixture of both. They present considerable treatment challenges due to the risk of complications like internal bleeding, infection, amblyopia, and reduced visual acuity.

Research involving small patient cohorts, up to 13 individuals, has shown the effectiveness of percutaneous sclerotherapy for treating orbital LMs using various sclerosants, such as STS and bleomycin, with different techniques. Furthermore, a more comprehensive study with 29 patients has validated the success of image-guided percutaneous sclerotherapy in managing orbital LMs, specifically utilizing STS [3,4].

STS, a detergent-like anionic surfactant buffered with 2% benzyl alcohol, induces death in endothelial cells due to its cytotoxic effects. Its high effectiveness allows for using reduced volumes for sclerotherapy, a significant advantage in the orbital area where space is limited. However, the merits of using smaller volumes are tempered by potential side effects. These can include significant swelling within the first 5 days after the procedure and a risk of neurological damage, both of which are dose-dependent [4].

The efficacy of foam sclerotherapy is primarily attributable to its mechanism that obstructs blood flow and replaces blood within the vessels, thus amplifying the interaction between the sclerosant and the vessel wall. This approach necessitates a lower dosage of STS, thereby mitigating the risk of adverse effects [5]. In this case report, administering a minimal volume of 0.5 mL STS combined with 2 mL of air effectively treated the orbital LM, which had a volume of 3.6 mL, without eliciting significant neurological side effects or inflammatory reactions.

In conclusion, foam sclerotherapy utilizing 3% STS presents as an efficacious and safe treatment option for orbital LMs. Additional research is warranted to reinforce the empirical support for this approach in the management of orbital LMs.

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