I N T R A C R A N I A L O P H T H A L M I C A R T E R Y ANEURYSM WALTER H . STERN, M.D., AND J. TERRY ERNEST, M.D.

Chicago, Illinois The ophthalmic artery arises from the in­ ternal carotid artery just as the internal ca­ rotid artery pierces the dura mater after tra­ versing the cavernous sinus. The intracranial course of the ophthalmic artery begins below and lateral to the optic nerve and passes to the medial side of the optic nerve before entering the orbit through the optic foramen. Aneurysms at the junction of the ophthalmic artery with the internal carotid artery are rare 1 and Krayenbiihl and Yasargil 2 found only seven such aneurysms among 290 intra­ cranial aneurysms. Because of their intimate association with the optic nerve, these aneurysms cause initial visual symptoms without other ocular signs and may be mis­ taken for retrobulbar neuritis. Recent studies indicate that despite their large size, aneurysms in this region rarely rupture. We describe a patient with an intra­ cranial ophthalmic artery aneurysm that caused sudden loss of vision followed by death 36 hours after rupture. CASE REPORT

A 45-year-old black man was seen in the emer­ gency room complaining of a sudden loss of vision in the left eye on arising in the morning. There was no headache, nausea, or vomiting. Several hours later, he complained of a throbbing pain be­ hind the left eye that increased with medial and lateral movement of the eye. He also had inter­ mittent, severe, left-sided throbbing frontal head­ ache. Physical examination revealed an alert and co­ operative black man with blood pressure of 140/90 and a pulse of 84 beats per minute. There were no bruits and no neck stiffness. Visual acuity was R.E.: 20/20 and 4 point, and L.E.: hand motion at two feet. The extraocular movements were full. From the Eye Research Laboratories, Depart­ ment of Ophthalmology, University of Chicago, Chicago, Illinois. This study was supported in part by Public Health Service grant EY-0523 from the National Institutes of Health. Reprint requests to Walter H. Stern, M.D., Pritzker School of Medicine, Department of Oph­ thalmology, 950 E. 59th St., Chicago, IL 60637.

The left pupil was mid-dilated and the swinging flashlight test disclosed consensual contraction of the left pupil followed by marked dilation when the left eye was directly stimulated. The right pupillary response was normal. Corneal sensation was in­ tact in both eyes. The applanation tensions were normal. Visual fields with a Goldmann perimeter using a 64-mm white test object with a relative in­ tensity of 1.00 log unit disclosed a 20-degree island of vision temporally and superiorly in the left eye. The right visual field was normal. Ophthalmoscopic examination revealed normal optic disks, vessels, and maculae in both eyes. Ophthalmodynamometry was performed with a Baillert ophthalmodynamometer and revealed equal diasto!ic blood pressures in both ophthalmic arteries. The remainder of the physical examination, including neurologic evalua­ tion, was normal. The patient was admitted to the hospital with a tentative diagnosis of retrobulbar neuritis. Twelve hours after admission, a lumbar puncture was per­ formed and after several attempts pink cerebrospinal fluid was obtained that did not clear. Open­ ing cerebrospinal fluid pressure was 275 mm H 2 0 and closing pressure was 150 mm H»0. A leaking intracranial aneurysm was suspected and the pa­ tient was scheduled for a carotid arteriogram. Sev­ eral hours after the lumbar puncture, however, the patient became incoherent and a respiratory ar­ rest ensued. With the patient on a respirator, left and right carotid arteriograms were performed, but the intracranial vessels did not fill with con­ trast material. The patient died shortly thereafter. Pathology—At autopsy, a ruptured saccular aneurysm, 14 mm in diameter, was dis­ covered at the junction of the left carotid and left ophthalmic arteries compressing the left optic nerve medially into a thin ribbon (Fig. 1). We noted another small saccular aneurysm of the right anterior communicat­ ing artery. A subarachnoid hemorrhage was present. Histologic examination of the aneurysm showed a partially calcified wall. Examina­ tion of the optic nerve in the area of com­ pression revealed almost total loss of nerve fibers (Fig. 2 ) . Distal to this area, the optic nerve was normal.

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DISCUSSION

Intracranial ophthalmic artery aneurysms

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Fig. 1 (Stern and Ernest). The saccular aneurysm, 14 mm in diameter, at the left carotidophthalmic artery junction.

have not been well studied as they are usually not distinguished from the more common aneurysms of the internal carotid artery branches. However, a cooperative study of 2,695 intracranial ruptured and nonruptured aneurysms indicated that 5.4% occur at the carotid-ophthalmic artery junction.3 If only nonruptured aneurysms in this series are considered, the incidence of carotid-ophthal­ mic artery aneurysms rises to 13%. This last figure indicates that carotid-ophthalmic artery aneurysms commonly do not rupture despite their usually large size. Fifty percent of aneurysms located at the carotid-ophthal­ mic artery junction were greater than 25 mm in diameter and 75% were greater than 10 mm. In follow-up studies of nine unruptured, untreated ophthalmic artery aneu­ rysms for an average of 45 months, only two had fatal subarachnoid hemorrhages, despite

AUGUST, 1975

the fact that three aneurysms were larger than 11 mm and four were larger than 25 mm.4 It is unclear why ophthalmic artery aneurysms are usually larger than other aneurysms of the internal carotid artery. Most case reports describing carotidophthalmic artery aneurysms emphasized monocular central visual field loss.5"11 Some­ times there was progression of the visual field defect to the superior nasal field in the involved eye, then loss of superior temporal field in the contralateral eye, and finally blindness on the side of the lesion. None of the case reports prior to the cooperative study documented spontaneous rupture of a carotid-ophthalmic artery aneurysm. The re­ sults from the cooperative study reinforce the feeling gleaned from the few case reports that aneurysms at the carotid-ophthalmic ar­ tery junction tend to be larger than most others located on the internal carotid artery and tend not to rupture spontaneously as fre­ quently as other internal carotid artery aneurysms. Their propensity to produce vi­ sual symptoms is related not only to their lo­ cation beneath the optic nerve, but to their tendency to grow to such a large size without rupturing. The presenting complaints of patients with nonruptured carotid-ophthalmic artery an­ eurysms do not differ greatly from those with fusiform dilations of the internal ca­ rotid artery described by Dandy.12 The coop­ erative study results indicate that while head­ ache and orbital pain are the most common complaints, 29% of patients with nonrup­ tured carotid-ophthalmic artery aneurysms have visual loss. In another perspective, of the 15 patients in the cooperative study with visual loss due to an unruptured aneurysm, nine had an aneurysm near the carotidophthalmic artery junction. The sudden loss of vision in our patient followed by rupture of the aneurysm is dif­ ferent than most clinical presentations of this class of aneurysm. The patient may not have previously noted progressive loss of vision in

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Fig. 2 (Stern and Ernest). The optic nerve showing the compression necrosis caused by the saccular carotid-ophthalmic artery aneurysm (hematoxylin-eosin, x50).

the left eye. Evidence that would favor this interpretation is the large size of the an­ eurysm and the calcification of the aneurysm wall. However, the lack of optic pallor on physical examination and the normal histologic appearance of the optic nerve distal to the site of compression suggest an acute ex­ pansion of an existing aneurysm. The clinical presentation was complicated by the presence of intermittent, severe retrobulbar pain that was exacerbated by ocular movements. While pain on movement of the eye has often been associated with retrobulbar neuritis,13 in this case it may have been due to blood in the optic nerve sheath. Ophthalmic artery aneurysms are the most likely aneurysms of the internal carotid ar­ tery to produce visual symptoms that may be mistaken for retrobulbar neuritis in the early stages of the disease. Repeated careful ex­ amination of the visual fields may suggest a

progressive lesion and influence the clinician to consider further diagnostic studies such as carotid angiography. This is a lesion that may be amenable to surgery. SUMMARY

A 45-year-old black man with a carotidophthalmic artery aneurysm developed sud­ den loss of vision in his left eye, a middilated pupil, and pain on movement of the left eye. A temporal island of vision was present in the left eye and the right visual field was normal. There were no other neuro­ logic abnormalities. The aneurysm spon­ taneously ruptured, resulting in the death of the patient. REFERENCES

1. Bull, J. W. D.: Contribution of radiology to the study of intracranial aneurysms. Br. Med. J. 2:1701, 1962. 2. Krayenbiihl, H., and Yajargil, M. G.: Das

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Hirnaneurysma. Basel, J. R. Geigy, 1958, p. 15. 3. Locksley, H. B.: Report on the cooperative study of intracranial aneurysms and subarachnoid hemorrhage, section 5, part 1. Natural history of subarachnoid hemorrhage, intracranial aneurysms and arteriovenous malformations. J. Neurosurg. 25: 219, 1966. 4. : Report on the cooperative study of intracranial aneurysms and subarachnoid hemor­ rhage, section 5, part 2. Natural history of sub­ arachnoid hemorrhage, intracranial aneurysms and arteriovenous malformations. J. Neurosurg. 25:321, 1966. 5. Walsh, F. B., and Hoyt, W. F.: Clinical Neuroophthalmology. Baltimore, Williams and Wilkins Co., 1969, vol. 2, p. 1748. 6. Hauser, M. J., and Gass, H.: Optic nerve pres­ sure by aneurysm relieved by decompression of

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optic nerve. Arch. Ophthalmol. 48:627, 1952. 7. Offret, G, and Godde-Jolly, D.: Les anevrismes de I'artere ophtalmique. Arch. Ophtalmol. 16:388, 1956. 8. Goldin, R. R., and Silver, M. L.: Ophthalmic artery aneurysm. Radiology 68:727, 1957. 9. Arseni, M. C, and Lasco, F.: L'anevrysme de I'artere ophtalmique. Rev. Otoneuroophtalmol. 39; 83, 1967. 10. Alexander, R. L.: Aneurysm of the ophthal­ mic artery. Can. J. Ophthalmol. 5 :248, 1970. 11. Cunningham, R. D., and Sewell, J. J.: An­ eurysm of the ophthalmic artery with drusen of the optic nerve head. Am. J. Ophthalmol. 72:743, 1971. 12. Dandy, W. E.: Intracranial Arterial An­ eurysms. New York, Hafner Press, 1969, p. 34. 13. Adie, W. J.: Aetiology and symptomatology of disseminated sclerosis. Br. Med. J. 2:997, 1932.

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Intracranial ophthalmic artery aneurysm.

A 45-year old black man with a carotid-ophthalmic artery aneurysm developed sudden loss of vision in his left eye, a mid-dilated pupil, and pain on mo...
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