Rapid Orbital Swelling Following Sclerotherapy of Orbital Venous Malformation

Low-flow vascular malformations of the orbit present many challenges for the treating physician. The highest degree of success has been described for a multidisciplinary treatment approach. A key element of this approach has been image guided sclerotherapy by percutaneous injection of sclerosing agents. Image guided sclerotherapy has proven highly effective with good to excellent results possible in 75%-90% of patients.¹ Other less invasive methods such as carbon dioxide have also been described.² In this case report, we have described a complication following percutaneous sclerotherapy and its effective management.

Case Report

A 3.5-year-old female patient presented with history of a slowly growing swelling on the right upper eyelid for 2 years. There was a history of increase in size of the swelling on crying. There was no history of pain or sudden increase in size. On examination, there was a smooth, oval, diffuse subcutaneous swelling with bluish discoloration involving the right medial upper lid. The swelling was compressible. There was no increased warmth or tenderness and there were no pulsations or bruit over the swelling. The child had proptosis of the right eye. Hertel Exophthalmometry readings measured 21 mm right eye and 18 mm left eye. The vision was 6/12 in both eyes. There were small hemangiomas on the hard palate. Otherwise systemic examination was normal.

MRI showed a well defined predominantly extraconal lesion in the superomedial aspect of the right orbit, which was of high signal intensity on T2W and low signal intensity on T1W images with internal septations. Examination showed the lesion was anechoic with thin septations. There was no arterialized flow on Doppler examination. On compression release, there was expansion of the lesion with color flow. Based on the clinical and imaging findings, diagnosis of venous malformation of the right orbit was made. We decided to treat the patient with image guided percutaneous sclerotherapy.

Technique

The procedure was performed in a digital subtraction angiography suite (Siemens Multistar T.O.P.), under general anesthesia with aseptic precautions. Dexamethasone 0.1 mg/kg was given intravenously. Ultrasound guidance was used for the initial access using two 23 g scalp vein needles (Figure 1).

Free reflux of blood was noted. Venography was performed with 50% contrast, which was injected under blank roadmap to assess the volume (Figure 2). The estimated volume was approximately 4 mL. There was no immediate opacification of the veins, suggesting that the venous malformation was a sequestered type. Sclerotherapy was performed using 2 mL of sodium tetradecyl sulfate (Setrol 3% W/V) and 2 mL of contrast. There was no extravasation noted during the procedure. At the end of sclerotherapy, there was increase in the orbital swelling.

Post-procedural monitoring showed a marked increase in proptosis, lid edema (Figure 3A), tight lids, and tense periorbital and orbital tissues onto retropulsion. All ocular movements were absent. There was a grade 3 relative afferent pupillary defect (RAPD) and the intraocular pressure (IOP) measured with a tonopen was 60 mm Hg (normal <20 mm Hg). We diagnosed the patient with orbital compartment syndrome (OCS). The patient underwent an emergency lateral canthotomy and inferior cantholysis (LCC) under general anesthesia. A hemostat was applied horizontally across the lateral canthus, with the tip directed towards the lateral orbital margin and clamped for one minute. A blunt tipped tenotomy scissor was used to make a full thickness incision until the orbital rim. Inferior cantholysis was performed with the same scissors. The cut end of the lower lid was lifted up, and with the tip of the scissors pointing towards the nose,  the inferior limb of the lateral canthal tendon was cut from its attachment to the orbital margin. The patient was also started on tablet acetazolamide 10 mg/kg in 4 divided doses. One and a half hours after the procedure the intraocular pressure came down to 31 mm Hg.

On the second day after sclerotherapy, there was a reduction in the proptosis; IOP was 27 mm Hg with only a grade I RAPD. The patient was discharged on the 4th day post-procedure. On discharge her vision was 6/12, the ocular movements were full, and there was no RAPD.
At 6-month follow-up, there was approximately 50% reduction in the orbital swelling. The vision and the ocular movements were normal.

Discussion

Venous malformations are categorized as low-flow vascular malformations within the domain of vascular anomalies.³ They are present at birth, undergo pari passu growth, and present clinically because of symptoms related to mass effect or stasis.³ Differentiation from other vascular and nonvascular entities often requires an imaging work-up that includes ultrasound with Doppler and MRI.

Percutaneous image-guided sclerotherapy has become the mainstay of treatment for venous malformations.³ Following introduction of a sclerosant into the vascular spaces, the endothelial-cidal action of the sclerosant starts with thrombus formation and thrombophlebitis. This usually results in temporary swelling of the lesion, which subsides within few days. Such an expansion in a closed space like orbit can lead to orbital compartment syndrome.

LCC has most frequently been used for the treatment of OCS secondary to retrobulbar hemorrhage,⁴ and still remains the most commonly used procedure to alleviate this condition.⁵ The orbit is a closed space bounded on 4 sides by the orbital walls and anteriorly by the eyelids, which are held in place by the medial and lateral canthal tendons attached to the orbital margin.⁵ Moreover, the orbital contents are contained within facial compartments with not much scope for expansion. Any sudden increase in the orbital volume, as in this case by injection of sclerosant and the thrombophlebitis, raises the intraorbital pressure. A rise in the intraorbital pressure reduces the perfusion to the hypoxia sensitive retina and optic nerve and can cause permanent visual damage if not dealt with at the appropriate time.⁶

OCS is characterized by painful proptosis associated with a decrease in vision, a tense orbit, restriction of ocular movements, RAPD, and increased intraocular tension.5 Fundus examination may reveal venous congestion, cherry red spot secondary to non perfusion of the central retinal artery, or optic nerve head edema.⁷ By disinserting the lid from the orbital margin, LCC works by decompressing the orbit, thus reducing the built-up intraorbital pressure. It has been shown as an effective procedure in preventing visual loss due to OCS.4 The advantages of LCC lie in its simplicity and speed of results. Most canthotomies heal well with scarring and do not require a second procedure for their repair.⁵

We recommend ophthalmologic assessment not only prior to sclerotherapy of an orbital venous malformation, but also soon after the procedure and in the immediate post-procedural period. Lateral canthotomy may be required anytime in the first few hours after the procedure. It is a simple sight-saving procedure that can be reversed a few days later, if needed, once the swelling subsides.

Conclusions

Percutaneous sclerotherapy is a useful technique in treating venous malformation of the orbit. Rapid reversible orbital swelling may result following sclerotherapy in permanent optic nerve damage, which may be prevented by timely intervention reducing the intra-orbital pressure by lateral canthotomy and inferior cantholysis.

References

1. Lewin JS. Low-flow vascular malformations of the orbit: a new approach to a therapeutic dilemma. AJNR Am J Neuroradiol. 2004;25(10):1633-1634.
2. Arat YO, Mawad ME, Boniuk M. Orbital venous malformations: current multidisciplinary treatment approach. Arch Ophthalmol. 2004;122(8):1151-1158.
3. Legiehn GM, Heran MK. Venous malformations: classification, development, diagnosis, and interventional radiologic management. Radiol Clin North Am. 2008;46(3):545-597.
4. Goodall KL, Brahma A, Bates A, Leatherbarrow B. Lateral canthotomy and inferior cantholysis: an effective method of urgent orbital decompression for sight threatening acute retrobulbar haemorrhage. Injury. 1999;30(7):485-490.
5. Lima V, Burt B, Leibovitch I, PrabhakaranV, Goldberg RA, Selva D. Orbital compartment syndrome: the ophthalmic surgical emergency. Surv Ophthalmol. 2009;54(4):441-449.
6. Knoop KJ, Dennis WR, Hedges JR. Ophthalmologic Procedures. In: Roberts JR, Hedges JR, eds. Clinical Procedures in Emergency Medicine. 5th ed. Philadelphia: W.B.Saunders. 2010:1141-1177.
7. Castro E, Seeley M, Kosmorsky G, Foster JA. Orbital compartment syndrome caused by intraorbital bacitracin ointment after endoscopic sinus surgery. Am J Ophthalmol. 2000;130(3):376-378.

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From the ¹Department of Radiology and the ²Department of Ophthalmology, Christian Medical College, Vellore, Tamil Nadu, India.

Disclosure: The authors have completed and returned the ICMJE Form for Disclosure of Potential Conflicts of Interest. The authors report no conflicts of interest regarding the content herein.

Manuscript received February 8, 2012 and final version accepted March 13, 2013.

Address for correspondence: Dr Shyamkumar Nidugala Keshava, Professor, Department of Radiology, Christian Medical College, Vellore, Tamil Nadu, India. Email: aparna_shyam@cmcvellore.ac.in