J. Neurol. Neurosurg. Psychiat., 1968, 31, 81-84

A case of intermittent exophthalmosL. J. DE LIMA' AND H. PENZHOLZ

From the Department of Neurosurgery, Stadt. Krankenhaus Neukolln, West Berlin, Germany

Intermittent exophthalmos was first described bySchmidt (1805) as a clinical entity in an infant andhas since been recognized as a rare but strikingcondition, its rarity being evidenced from thepaucity of reported cases. Two important papersrelating to this topic have been the subject of acomprehensive review of the world literature in thefirst half of this century-the first by Birch-Hirsch-feld in 1930 and the second by Walsh and Dandy in1944, and since then scarcely anything more hasbeen added to, our knowledge of this condition thatis not contained in these reports.

Brauston and Norton in 1963, adding two casesof their own to the number previously reported,brought the total up to 137. In recent years, a surveyof the IndexMedicus reveals a fewmorecontributionsto the list to which the authors wish to add thispersonal case.

CASE REPORT

The patient, G. B., was a 41-year-old woman whosechief complaint was a distressing feeling of fullness inthe right eye in certain bodily positions which wasimmediately relieved on sitting or standing upright. Shestated that at the age of 6 years, there was a suddenslight protrusion of the right eyeball which within twohours was normal again. At age 20, she was operatedupon for a hemangioma over the right upper eyelid whichfirst made its appearance at the age of 14 and hadslowly increased in size since then. At 24, she noticedthat the right eye would 'pop' out of its socket with anyform of excitement, forward bending of the head, orwhen she walked into a warm room from the outsidecold in the winter months, but, as she attained the erectposture, the eye swelling would disappear almost asrapidly as it proptosed and she would be comfortableagain. This situation became slowly and progressivelyworse over the years, until at the age of 39 she noticedthat lying in bed, and especially on her right side, pro-duced a proptosis which became almost unbearable.Other than the feeling of fullness, there was no pain inthe eye, no disturbance of vision, and no pulsations orunpleasant noises heard. The left eye was normal at alltimes. There was no history of trauma.

PHYSICAL EXAMINATION The patient was of normalbuild and average intelligence. Blood pressure was 120/70.'Present address: 30 Admiral Street, Charlottetown, P.E.T., Canada.

General physical examination was within normal limitsand, except for the following findings in the right eye, theneurological examination was unremarkable. In theerect posture of the head, inspection showed a slightenlargement of the right palpebral fissure (R = 12 mm;L = 11mm). Enophthalmos was clearly evident (R =15-5 mm; L = 17 mm). There was a small varicosity nearthe inner canthus of the eye. The pupils reacted normallyto light and accommodation. Eye movements werenormal. There was no diplopia, nystagmus, or ptosis.When light was thrown on the right eye, a faint pulsationwas visible in the eye and upper lid, but it was notpalpable and no bruit could be heard. The optic discswere physiological, while acuity, colour, and fields ofvision were within normal limits.The eye inexophthalmos (Fig. Ib) was best studied with

the patient lying on her right side and the head tiltedslightly forward. It could also be evoked by throwing thehead backward, or when artificial means were employedto increase intracranial pressure-for example, by pres-sing the ipsilateral jugular vein or on forced expirationwith compression of the nostrils. Within a few secondsthe eye would proptose to its maximum with congestion

i.~~~~~ ~ ~~~~~~~~~~~~~~~~...ott se

FIG. l a. The eye in the normalposition.

FIG. lb. The eye in exophthalmos.81

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of the sclera and rapid filling of a cluster of veins over theorbital ridge. The right palpebral fissure was narrowed(R = 6 mm; L = 9 mm) and the eyeball seemed to bedisplaced forward, downward, and outward, but eye

movements were possible in all directions. The exoph-thalmos measured R = 20 mm; L = 17 mm. The pupilwas of normal size and reacted promptly to light. Therewas no diplopia and the pulsation seen in the erect

posture was visibly increased but again was not palpableand no bruit was heard. On assuming the erect posture.the exophthalmos would disappear as rapidly as itpresented itself. The patient did not complain of any

pain but within 5 min of proptosis was clearly uncom-

fortable.

FIG. 2. The radiograph of the skull shows erosion of theorbital roof.

FIG. 3. Right orbitalphleblography.

Routine blood and urine examinations were normal.Blood serology was negative. The E.E.G. was unre-markable. The radiograph of skull and orbits (Fig. 2)revealed small areas of erosion of the right orbital roof.

Bilateral carotid angiograms (external and internalfillings) were inconclusive for any vascular malformationsor tumours within the intracranial chamber or orbitalcavities. A right orbital phlebography (Fig. 3) using theangular vein on the right side was carried out and showed(a) a cluster of vessels over the right supraorbital ridge,(b) a large vein crossing the left orbital cavity, presum-ably the left ophthalmic, and (c) numerous extracranialveins of normal size.Under a presumptive diagnosis of intermittent exoph-

thalmos, probably caused by orbital varices, the patientwas subjected to a right fronto-temporal craniotomy. Onelevating the right frontal lobe with the dura intact, theerosions in the anterior half of the orbital roof wereseen and some of them extended through the depth ofthe bone, visualizing large, tortuous, and dilated veinswithin the orbit. The dura was next opened and sur-prisingly on the inner surface of its orbital portion was anarea approximately 2 cm x 1 cm containing coils ofvessels, knit together and closely adherent to the dura.These vessels were bright red in colour, pulsation inthem was not clearly visible, but they were probablylargely arterial coils conveying blood to the dilatedveins within the orbit. No large artery was recognized asbeing the main feeder of this dural arteriovenous mal-formation. The vessels were ligated and excised and afew were cauterized. The right orbit was unroofed andtwo or three large dilated veins forming part of the uppergroup were picked up, ligated, and excised. The clusterof subcutaneous vessels overlying the supraorbitalridge was similarly treated (Fig. 4).The patient has been examined periodically since her

operation, and a year later she reports that she has hadno further attacks of exophthalmos nor can it be evokedby such means as were used before surgery.

DISCUSSION

The complete syndrome ofintermittent exophthalmosis characterized by an alternating exophthalmosand enophthalmos and usually develops graduallyand progressively. It has been seen at all ages, frombabes in arms to those in the evening of life, butthe great majority ofcases present with symptoms andsigns in the second and third decades. A case ofbilateral involvement has yet to be seen and asurvey of the literature shows a slight predilectionfor the left eye.

Vascular malformations within the orbit orwithin it and the intracranial cavity have been foundresponsible for the disorder. According to Duke-Elder (1952) varicosities form 90% of the orbitalanomalies, but arteriovenous aneurysms, hemangio-mas, and lymphangiomas have also been encount-ered.As regards symptomatology, the most common

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A case of intermittent exophthalmos

Arter ovenousmalformation

Optic nerve.

FiG. 4. The artist's ir

complaint appears to be a bulging forward of theeyeball with a distressing feeling of fullness withinit under conditions that would induce venousstasis within the affected orbit-for example,bending of the head forward or backward, excite-ment or straining, or even sudden changes oftemperature as experienced by the patient in thecase report. Eye pain is a complaint in only aminority of cases. The exophthalmos usuallyreaches its maximum within a few seconds andthe patient is forced to seek relief by adoptingsuch bodily positions to which he or she nas becomeaccustomed. In the exophthalmic state the patient'sdistress is obvious. The eyeball is pushed forwardor downward and outward to as much as 30 mm orso. It may be congested and vision itself not infre-quently reduced. Changes have also sometimes beenobserved in the pupil, which is dilated or narrowed.Eye movements may be restricted and diplopia mayoccur. Examination of the fundus may revealslight congestion.Walsh and Dandy (1944) observed pulsation of

the eyeball in their patient and, in concludingdogmatically that an arterial component was pres-ent in the lesion concerned, proved it at operation.In our patient, faint pulsations were observed inthe enophthalmic eye and these became morepronounced in the exophthalmic state. The weak-ness of the pulsations in our case was probably due

Frontal bone

I S ubcutaneous' _venouslangioma

.,I':. D il]ated

o rb i talIwa;,veins

mpression of the lesion.

to the fact that they were transmitted throughsieve-like openings in the orbital roof rather thanthrough a widely dilated superior orbital fissure aswas present in the case of Walsh and Dandy (1944).

In most of the reported cases, radiographs ofskull and orbits were found to be negative, thoughthe presence of an enlarged orbit or the superiororbital fissure has been noted in some cases. In aninteresting review of the clinical material at theNational Hospital and the Atkinson Morley inLondon, Rischbieth and Bull (1958) discussed thesignificance of enlargement of the superior orbitalfissure and concluded with these remarks: 'Theclue provided by widening of the superior orbitalfissure in the diagnosis of cases of "orbital tumour"is valuable, since those lesions confined to the orbitalcavity produce no bony erosion, whereas those withan intracranial extension show erosion in a highproportion of cases.' In our case there was erosionof the orbital roof.

Since attaining neurosurgical importance, manycases in recent years have had carotid angiography.This study has invariably been reported as normal,but even under these circumstances it would befallacious to rule out the presence of an intracraniallesion, and especially if it be borne in mind thatdural vascular malformations have been known tooccur without the faintest trace of their presence onroutine angiography (Obrador and Urquiza, 1952;

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Verbiest, 1962) and which was so well exemplifiedin our own case. In fact the only clue to the presenceof the intracranial lesion in this patient was theorbital erosion.Krayenbuhl discussed orbital phlebography in

his valuable paper in 1962 and used the procedurehimself in a case of intermittent exophthalmos.In a more recent publication, Arseni, Mihailesu,Simionescu, and Le-Xuan-Trung (1965) reportedtheir experiences in a series of 40 cases with orbitallesions of various types, and deduced some inter-esting conclusions from interpretation of the venouspatterns seen on their radiographs. In our opinion,orbital phlebography in intermittent exophthalmoshas one important value-that is, it helps to definethe various communications that exist between thevarices in the orbit and the intracranial chamber. It isknown that each orbit has two sets of veins (upperand lower) which unite to form the main ophthalmicat the orbital apex and which eventually drains intothe cavernous sinus. It is also known that the veinsdraining the lids, temporal region, and the face arein direct communication with these veins in theorbit of one or both sides, and, as in our case, aresometimes engorged. If therefore the venous stasis inthe orbit in intermittent exophthalmos is influencedlargely by changes in intracranial pressure, it wouldbe reasonable to conclude that if only some of thesedilated orbital veins were ligated at operation and/oreven the main ophthalmic on the correspondingside were tied off, the remainder of the dilated veinsin the orbit could still be influenced by changes inintracranial pressure through the ophthalmic veinof the opposite side, should a communication exist.In the case reported, orbital phlebography showedfilling of a large vein, probably the ophthalmic inthe contralateral orbit, a finding that could be ofprognostic significance.The syndrome, though self-limiting, is a progressive

one and a retrobulbar haemorrhage is an occasionalcomplication. Less frequently abducens paralysis,optic atrophy, and even sudden blindness havebeen reported.The treatment of intermittent exophthalmos has

been a lamentable tale of failures and complications.On the basis ofpathogenesis it would seem clear thatthe aim of any operative procedure should be either(1) excision of all the offending veins in the orbit, or(2) interruption of these veins from their counter-parts in the intracranial chamber. Most neuro-surgeons and acknowledged ophthalmologists todaywould agree that the transcranial approach to the

orbit is the more acceptable, for it allows a widerexposure and a better definition of the lesionstherein. Admittedly, at the time of this publication,our patient has been followed up for too short a per-iod to permit any operative conclusions. Never-theless, it would seem to us on the basis of patho-genesis that, when an arteriovenous aneurysm ispresent in intermittent exophthalmos, it is impera-tive that the entire malformation including theorbital varices if present be excised as far as thelimits of surgery will allow.

SUMMARY

A case of intermittent exophthalmos of the righteye occurring in a 41-year-old woman is reported.The cause was proven to be an intracranial arterio-venous aneurysm and orbital varices. Through afronto-temporal approach, the anomalies concernedwere extirpated with complete cure of the exoph-thalmos.The literature on the subject has been reviewed

and the syndrome discussed briefly with reference toits clinical features, radiological observations, andtreatment.

The authors wish to thank Miss E. W. Sweezy, head ofthe Medical Illustrations Department, Queen MaryVeterans Hospital, Montreal, for the preparation ofFigure 4.

REFERENCES

Arseni, C., Mihailescu, N., Simionescu, M., and L-Xuan-Trung.(1965). Orbital Phlebography-Its value for the aetiologicaldiagnosis of unilateral exophthalmos. Acta Neurochir. (Wein),12, 521-542.

Birch-Hirschfeld, A. (1930). Der Intermittirende Exophthalmus.In Handbuch der Gesamten Augenheilkunde, edited byGraefe, A., and Saemisch, T. Springer, Berlin. 2nd ed., band 9,abt 1, pp. 105-149.

Brauston, B. B., and Norton, E. W. D. (1963). Intermittent Exoph-thalmos. Amer. J. Ophthal., 55, 701-708.

Dandy, W. E. (1941). Results following the transcranial operativeattack on orbital tumors. Arch. Ophthal. (Chic.) 25, 191-213.

Duke-Elder, W. S. (1952). Textbook of Ophthalmology, Vol. 5, pp.5397-5403, 5627. Kimpton, London.

Krayenbuhl, H. (1962) The value of orbital angiography fordiagnosis of unilateral exophthalmos. J. Neurosurg., 19,289-301.

Obrador, S., and Urquiza, P. Cited by Verbiest, H. (1962).(1952). Angicme art6rioveineux de la tente du cerivelet.

Folia psychiat. neurol. 55, 385.Rischbieth, R. H. C., and Bull, J. W. D. (1958). The significance of

enlargement of the superior orbital fissure. Brit. J. Radiol.,31, 125-135.

Schmidt (1805). Cited by Duke-Elder, W. S. (1952).Verbiest, H. (1962). Arterial and arteriovenous aneurysms of the

posterior fossa. Psychiat. Neurol. Neurochir. (Amst.), 65,329-369.

Walsh, F. B., and Dandy, W. E. (1944). On the pathogenesis ofintermittent exophthalmos. Arch Ophthal. (Chic.) 32, 1-10.

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