Endoscopic transethmodial decompression of a thrombosed orbital venous malformation. (Original Article).

Abstract

We describe the case of a 37-year-old woman who experienceda painful thrombosis of an orbital venous malformation. We successfully treated her with surgical decompression via a computer-assisted, endoscopic transethmoidal orbitotomy. Removal of the lamina papyracea and evacuation of the thrombus provided immediate symptomatic relief Decompression via an endoscopic trans-ethmoidal route provides a more direct and less invasive approach than other techniques.

Introduction

Orbital venous malformations usually manifest as intermittent unilateral proptosis during the second or third decade of life. In most cases, management of these malformations is conservative and supportive. More aggressive interventions--in the form of vascular clipping, cauterization, evacuation, or excision--is reserved for those lesions that threaten vision or cause severe pain. (1-6) In this article, we report the case of a patient who required acute intervention for a painful thrombosed orbital venous malformation.

Case report

A 37-year-old woman came to us with a 2-day history of positional right orbital pain and lower-eyelid ecchymosis. Her orbital discomfort increased with movement of the eye and during physical activity. She denied vision loss or diplopia. Her medical history included a nasal fracture at 4 years of age and rhinoplasty at 19 years.

Examination revealed lower-eyelid ecchymosis and 1 mm of proptosis on the right and blepharoptosis bilaterally (figure 1). The patient's visual acuity was 20/20 in each eye. Her ocular motility was globally limited as a result of the orbital discomfort. The results of pupillary, visual-field, slit-lamp, and ophthalmoscopic examinations were normal. Physical findings were unchanged with dependent head positioning and with Valsalva's s maneuver. Intranasal findings were normal.

The patient underwent computed tomography (CT) and magnetic resonance imaging (MRI) (figure 2). CT showed a well-demarcated 2.0 x 1.0-cm soft-tissue mass In the medial right orbit. On MRI, the mass had a heterogenous signal intensity whose characteristics were consistent with those of subacute hemorrhage and thrombosis. The combined clinical and imaging findings strongly supported a diagnosis of thrombosed orbital venous malformation. Outpatient management with oral acetaminophen/oxycodone and restricted physical activity was prescribed.

During re-evaluation 48 hours later, the patient reported that conservative management had failed to control her pain. In view of this treatment failure, we offered surgical decompression of the malformation. We performed a computer-assisted orbitotomy via an endoscopic transethmoidal approach (figure). Removal of the lamina papyracea and evacuation of the orbital thrombus provided immediate symptomatic relief Complete hemostasis was achieved following the thrombus evacuation. The patient's proptosis and dysmotility resolved immediately. At 18 months of follow-up, her physical findings remain normal.

Discussion

Prior to the publication of the Orbital Society's 1999 recommendations, (7) orbital venous malformations characterized by a low degree of vascular flow were broadly termed orbital varices. The nomenclature change was precipitated by a debate over the differentiation of orbital varices from lymphangiomas. This was important given that histopathologic confirmation of orbital varices is uncommon.

Two vascular features of orbital venous malformations play a role in the lesion's characteristic appearance: (1) an absence of valves in the orbital veins and (2) a low degree of intrinsic blood flow. The absence of valves allows for induced variability in venous pressure, which promotes the physical expansion and deflation of the malformation. Thus, lowering the head position or performing Valsalva's maneuver clinically precipitates intermittent proptosis. (1-3) A low degree of intrinsic blood flow through the malformation predisposes it to intravascular thrombosis. When orbital venous malformations hemorrhage and/or thrombose, they produce sustained proptosis and pain. (1-3)

The diagnosis of orbital venous malformation is usually not made histopathologically but rather by the clinical picture and supportive imaging studies. The utility of multiple imaging modalities--including CT, MRI, sonography, venography, and color Doppler imaging--in the diagnosis of orbital venous malformations has been established. (1-6) The advantages of MRI in this condition include not only its noninvasive nature but its ability to demonstrate intralesional hemorrhage and thrombosis. MRI signal intensities from a thrombus depend on the oxygenation and degradation of its contained hemoglobin. (3) The ring-like, layered MRI signal configuration and the juxtapositioning of a vein, as was seen in our patient, are characteristic of intravascular thrombosis. (1, 4-6, 8)

Although most orbital venous malformations are managed conservatively, intervention is indicated for those that cause intractable pain and for those that threaten vision. Modem surgical instrumentation and technique have expanded the role of endoscopic surgery. Selected lesions of the medial orbit have been shown to be amenable to endoscopic transethmoidal diagnostic and therapeutic procedures. (9,10) The proximity of our patient's malformation to the medial orbital wall and the presence of a large intralesional thrombus suggested that the malformation could be safely decompressed into the ethmoid sinuses. We found the endoscopic transethmoidal approach to be more direct and less invasive than previously described techniques.

References

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(3.) Rubin PA, Remulla ND. Orbital venous anomalies demonstrated by spiral computed tomography. Ophthalmology 1997;104: 1463-70.

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(5.) Cohen JA, Char DH, Norman D. Bilateral orbital varices associated with habitual bending. Arch Ophthalmol 1995:113:1360-2.

(6.) Foroozan R, Shields CL, Shields JA, et al. Congenital orbital varices causing extreme neonatal proptosis. Am J Ophthalmol 2000; 129:693-4.

(7.) Harris GJ. Orbital vascular malformations: A consensus statement on terminology and its clinical implications. Orbital Society. Am J Ophthalmol 1999;127:453-5.

(8.) Ida M, Kurabayashi T, Tanaka A, et at. Thrombosed varix in the submandibular space. Dentomazillofac Radiol 1998;27:367-70.

(9.) Neumann AM, Jr., Pasquale-Niebles K, Bhuta T, Sillers MJ. Image-guided transnasal endoscopic surgery of the paranasal sinuses and anterior skull base. Am J Rhinol 1999;13:449-54.

(10.) Froehlich P. Pransky SM, Fontaine P. et al. Minimal endoscopic approach to subperiosteal orbital abscess. Arch Otolaryngol Head Neck Surg 1997:123:280-2.

From the Department of Otolaryngology-Head and Neck Surgery (Dr Gigantelli and DR. Leopold), the Department of Ophthalmology (Dr. Giantelli), and the Department of Radiology (Dr. Arthur), University of Nebraska Medical Center, Omaha, and the Department of Ophthalmology, Creighton University School of Medicine, Omaha (Dr. Gagnon).

Reprint request: James W. Gigantelli, MD, Department of Ophthalmology, 985540 Nebraska Medical Center, Omaha, NE 68198-5540. Phone: [phone]; fax: [phone]; e-mail: [e-mail]

This study was supported in part by an unrestricted grant from Research to Prevent Blindness of New York City. The authors have n proprietary interest in the development or marketing of any drug, compound, or technique noted in this manuscript.