Journal of Neuro-Ophthalmology, June 1983, Volume 3, Issue 2

B-scan ultrasonic evaluation of a dilated superior ophthalmic vein in orbital and retro-orbital arteriovenous anomalies.

All patients between 1977 and 1982 who presented with unilateral exophthalmos were evaluated with contact B-scan ultrasonography. Of these, eight patients were diagnosed as having retro-orbital or orbital arteriovenous anomalies (two carotidcavernous sinus fistulas, four dural arteriovenous malformations, and two orbital arteriovenous malformations). On B-scan ultrasound, all of these patients demonstrated a dilated superior ophthalmic vein. None of the other patients with unilateral proptosis demonstrated this finding. With recent advances in treatment of these conditions, early diagnosis becomes increasingly important. Contact B-scan ultrasonography (which is widely available, convenient, and expedient) can be used for early diagnosis of arteriovenous anomalies in the orbit and cavernous sinus areas. Other ultrasonic techniques such as A-scan, standardized A-scan, or immersion B-scan, are equally reliable in the recognition of a dilated superior orbital vein. In the authors' opinion, however, these are less convenient, more time consuming, and require more expertise for similar results.

f. Clin. Neuro-ophthalmoJ. 3: 105-108, 1983. B-Scan Ultrasonic Evaluation of a Dilated Superior Ophthalmic Vein in Orbital and Retro-orbital Arteriovenous Anomalies MARLENE R. MOSTER, M.D. JOHN S. KENNERDELL, M.D. Abstract All patients between 1977 and 1982 who presented with unilateral exophthalmos were evaluated with contact B-scan ultrasonography. Of these, eight pa­tients were diagnosed as having retro-orbital or orbital arteriovenous anomalies (two carotidcavernous sinus fistulas, four dural arteriovenous malformations, and two orbital arteriovenous malformations). On B-scan ultrasound, all of these patients demonstrated a di­lated superior ophthalmic vein. None of the other pa­tients with unilateral proptosis demonstrated this finding. With recent advances in treatment of these conditions, early diagnosis becomes increasingly im­portant. Contact B-scan ultrasonography (which is widely available, convenient, and expedient) can be used for early diagnosis of arteriovenous anomalies in the orbit and cavernous sinus areas. Other ultrasonic techniques such as A-scan, standardized A-scan, or immersion B-scan, are equally reliable in the recogni­tion of a dilated superior orbital vein. In the authors' opinion, however, these are less convenient, more time consuming, and require more expertise for simi­lar results. Introduction The differential diagnosis of unilateral exoph­thalmos includes dysthyroid orbitopathy, orbital and optic nerve tumors, orbital pseudotumor, ocu­lar infections, metastatic tumors of the orbit, lac­rimal gland tumors of the orbit, angiomas, lacrimal gland tumors, and orbital and retro-orbital anom­alies which include carotidcavernous sinus fistula, dural arteriovenous malformations, and orbital ar­teriovenous malformation. I. ~ The arteriovenous anomalies share common signs of exophthalmos arterialization of the epibulbar veins, headache, decreased vision, impaired extraocular movements, venous stasis on the fundus exam, and secondary From the Departments of Ophthalmology and Neurology, Eye and Ear Hospital, University of Pittsburgh, Pittsburgh, Penn­sylvania. June 1983 glaucoma 4 Bruits and pulsating exophthalmos which are common in carotid cavernous sinus fis­tulas, are only present occasionally in dural arte­riovenous malformations and orbital AVMs. The recognition of a dilated superior ophthalmic vein has been described in the CT scan literature in patients found to have dural arteriovenous mal­formations and carotidcavernous sinus fistulas. 6 . 7 However, this finding has only been rarely empha­sized in the literature for the ultrasonic evaluation of these conditions.;l. 16. 17 Method The B-scan ultrasonogram can identify a dilated superior ophthalmic vein most conveniently by a contact method. In our patients, the contact method using aquasonic jelly placed copiously over the closed lid was used. This can be done with any of the currently available B-scan contact ultrasonic units. With the B-scan transducer placed appropriately on the closed lid, vertically oriented, on the globe between the transducer and the superior orbital area, the dilated superior ophthalmic vein can be seen as a round sonolucent structure of low internal reflectivity, located medial and superior to the optic nerve in the superior orbital fat pad. When the transducer is placed horizontally with the trans­ducer top aimed medially across the globe, one sees a curvilinear sonolucent tube in the superior orbital fat pad, extending from lateral to medial behind the globe. The dilated vein is compressible with careful pressure on the globe directed at the sonolucent structure. Care must be taken not to misinterpret dislocation of the vein from compres­sion. Case Reports Case 1 A 73-year-old, diabetic female noticed promi­nence and redness of her left eye for 7 months. She was treated with antibiotics for 2 months before 105 Contact B-Scan Ultrasonography Figure 1. P<lticnt with drteriobriz<ltion of the epibulbdr veins <lnd conjunctiva extending to the limbus. referral. Visual acuity was 20/70 right eye, 20/60 left eye. Pupillary reactions and visual fields were normal. Hertel exophthalmometry showed an 8­mm left proptosis. Ocular motility revealed severe restriction of abduction of the left eye with less severe limitation in other fields of gaze. The right eye motility was normal. Tensions by applanation tonometry were 10 mm right eye and 28 mm left eye. Slit lamp biomicroscopy showed arterializa­tion of the episcleral veins extending to the limbus in the left eye (Fig. 1). The fundus exam showed early venous stasis of the left eye only. A bruit over the left orbit was heard. B-scan ultrasonography demonstrated a dilated superior ophthalmic vein in both the horizontal and vertical projections. The patient was then re­ferred for arteriography, which showed a dural arteriovenous malformation on the left supplied by branches of both the external and internal carotid arterial systems. In a 2-week interval, the patient's proptosis increased to 9 mm on the left and intra­ocular pressure increased to 36. The patient was referred to neuroradiology for treatment. Case 2 A 49-year-old man noted bulging of the eye for 2 months, first attributing this to seasonal allergies. There was no history of trauma. Visual acuity was 20/20 in the left eye, 20/15 in the right eye. Pu­pillary responses, ocular motility, visual fields, and color vision were normal. Hertel exophthalmome­try showed a 3-mm left proptosis with positive resistance to retropulsion of the globe. Applanation 106 tonometry was 17 mm in both eyes. Slit lamp biomicroscopy of the conjunctiva and sclera re­vealed tortuous and dilated conjunctival and epis­cleral vessels only in the left eye. Fundus exam was normal in both eyes. TRH stimulation test was normal. B-scan ultrasound showed dilated superior ophthalmic vein in both horizontal and vertical projections in the left eye. CT scan revealed an orbital apex mass with bony destruction and a prominent superior ophthalmic vein. In a 3-month interval, the left proptosis increased to 5 mm. Tensions remained normal. Orbital venography showed conspicuous nonfilling of the left superior ophthalmic vein and arteriography showed a high arteriovenous flow "mass" which was dorsal to the optic nerve, suggestive of an orbital AYM. An attempt at the intraarterial catheterization with a balloon in 1979 was unsuccessful. By 1 year, left proptosis became stable at 7 mm and patient was again referred to neuroradiology for treatment with embolization technique.'~ Case 3 A 71-year-old, diabetic female noted a promi­nence and redness of both eyes with horizontal diplopia for 1 month duration. There was no his­tory of trauma. Visual acuity was 20/25 and 20/ 30. There was a bilateral sixth nerve paresis. Her tensions by applanation tonometry were 34 and 36 mm Hg. She had 3 mm of bilateral proptosis. Slit lamp biomicroscopy showed arterialization of the epibulbar veins in both eyes. There was no bruit. Journal of Clinical Neuro-ophthalmology Moster, Kennerdell Figure 2. B-scan ultrasound displaying dilated superior ophthdlmic vein in the superior orbItal fdt pad. Transducer is in the verticdl position. Y = vItreous, SOY = superior ophthalmic vein. SLR = superior rectus levator complex, ON = optic nerve. Figure 3. B-scan ultrasound of a dildted superior orhthdlmil vein with th" tr.lnsduc"r In .1 horizontal position. One secs d curvIlll1edr s"mllucent tub" III til(' ,u['erlllr lHb,tdl f.lt ['.ld V = vitreous, SOY = superior ophthalmic vein. The fundus exam was also normal in both eyes. Ultrasonic evaluation revealed similar findings as in the preceding patients, with a dilated superior ophthalmic vein in both the horizontal and vertical projections (Figs. 2 and 3). CT scan of the orbits revealed bilateral exophthalmos without evidence of extraocular muscle enlargement. Arteriogram June 1983 confirmed the suspicion of a bilater.ll dural arterio­venous malformation, primarily on the left side. The patient was followed for I month, at which time she noted increased proptosis and persistent diplopia. Her tensions increased to 40 mm in both eyes on pilocarpine and timoptic. The patient underwent a therapeutic emboliza- 107 Contact B-Scan Ultrasonography tion procedure of the external carotid arterial sys­tem, particularly the internal maxillary arteries on both sides. There was significant therapeutic im­provement of the proptosis and vascular conges­tion, although the bilateral sixth nerve paresis per­sisted. Medial rectus recessions of both eyes were performed to align the eyes in the primary position. Comment The diagnosis of arteriovenous anomalies of the orbit in cavernous sinus regions should be consid­ered when patients present with proptosis, epibul­bar vessel congestion, lid edema, and ophthalmo­plegia. An orbital bruit may be in the carotid cavernous sinus fistulas, but is often absent in arteriovenous malformations.5 The clinical signs may be subtle and correct diagnosis is often de­layed. It is important to be aware of the significance of arterialization of the conjunctival venules mim­icking conjunctival inflammation in these cases. Three out of seven of our patients were initially treated for nonspecific ocular inflammation with antibiotics before the eventual referral to the med­ical center. The recognition of a dilated superior ophthalmic vein in orbital and retro-orbital arteriovenous anomalies has been well-described by angiography and more recently by CT scan. However, the B­scan ultrasonic pattern of the superior ophthalmic vein dilatation has not been emphasized in the literature as being recognized by a current, inex­pensive, expedient, office diagnostic test..3· 16. 1, This finding is highly significant in that it points to a lesion causing venous engorgement in the orbit such as deep orbital and arteriovenous malforma­tion, a carotid cavernous fistula, or a dural sinus arteriovenous malformation. We demonstrated the presence of a dilated su­perior ophthalmic vein by B-scan ultrasound in eight out of eight patients with arteriovenous anomalies of the orbit and retro-orbital regions. This finding is specific in that no patients with other causes of exophthalmos demonstrated a di­lated superior ophthalmic vein on ultrasound, and were then referred for either angiography or digital subtraction angiography to confirm the diagnosis. There have been recent advances in the treat­ment of arteriovenous malformations with embo­lization of the inert particles or tissue adhesives to occlude the vessels feeding the malformation.ll . I:3 The availability of these techniques as well as the need for early treatment of elevated intraocular pressure increase the importance of early diagno­sis. B-scan ultrasound units which are often avail­able in the community can be used for early diag­nosis and direct referral arterial or venous angiog­raphy. Therefore, definitive treatment can be con­sidered both by the minimization or elimination of the abnormality itself or to better control the ve- 108 nous stasis glaucoma suffered by the eye in patients who are not surgical candidates. 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Neurasurg 8: 52-55, 1981. 14. Klepach, GL., Wray, S.H., Roberson, GH., and Dallow, R.L.: Bilateral dural arteriovenous malfor­mations simulating dysthyroid ophthalmopathy. Ann.Ophthalmol. 10: 1519-1523, 1978. IS. Flanagan, J.C: Tumors of the lids and orbits. Oph­thalmol 86: 896-913, 1979. 16. Phelps, CD., Thompson, H.S., and Ossoinig, K.C: The diagnosis and prognosis of atypical carotid-cav­ernous fistula (red eye shunt syndrome). Am. ]. Ophthalmol. 93: 423-436, 1982. 17. Ossoinig, K.C: A-scan echography and orbital dis­ease. Mod. Probl. Ophthalmol. 14: 203, 1975. Write for reprints to: John S. Kennerdell, M.D., Eye and Ear Hospital, 230 Lothrop Street, Pittsburgh, Penn­sylvania 15213. Journal of Clinical Neuro-ophthalmology