British Journal ofOphthalmology 1992; 76: 627-629

CASE REPORTS

Bilateral multiple cavernous haemangiomas of theorbit

Timothy J Sullivan, G William Aylward, John E Wright, Ivan F Moseley, Alec Garner

AbstractOrbital cavernous haemangioma, a commonorbital tumour, is usualiy single and unilateral.We report the first case of histologicallyconfirmed bilateral multiple cavernoushaemangiomas of the orbit.(BrJ7 Ophthalmol 1992; 76: 627-629)

Orbital cavernous haemangioma typically occursas a slow growing well circumscribed lesion inthe lateral part of the middle third of the orbit. 'Patients most commonly present with slowlyprogressive painless proptosis or visualdisturbance." Though multiple lesions havebeen reported in the same orbit' 2 we have tracedonly two reports of cavernous haemangiomas inboth orbits.56 We report the first case of histo-logically confirmed bilateral multiple cavernoushaemangiomas of the orbit.

Orbital Clinic,Moorfields Eye Hospital,City Road, LondonT J SullivanGT AylwardJ E WrightI F MoseleyA GarnerCorrespondence to:Mr J E Wright, ConsultantEye Surgeon, Moorfields EyeHospital, City Road, LondonEC1V 2PD.

Accepted for publication8 April 1992

Case reportA 41-year-old Caucasoid woman noted a mass inthe medial aspect of her right lower lid, in herlate twenties. A plastic surgeon removed thelesion from her anterior orbit. Seven years later a

violaceous lump in her left lower lid was alsoremoved. Pathological examination of theselesions showed both to be typical cavernous

haemangiomas.Over the past 14 months she developed left

proptosis, with intermittent swelling ofthe lowerlid associated with a blue discoloration of theoverlying skin (Fig 1). The swelling did not alterwith changes in posture. She had no pain at anystage, and there were no associated visualsymptoms. She was referred to one of us (JEW)for further evaluation.

Ophthalmic examination revealed a bestcorrected visual acuity of 6/9 OD, attributed toamblyopia, and 6/6 OS. There was 5 mm of leftaxial proptosis, and the left globe was displaceddownwards 3 mm. Two small firm pea-sizedmasses were palpable, one on the anterior edge ofthe inferior orbital margin of the right orbit, andanteroinferior part in the left orbit. The rest ofher ophthalmic examination was normal.Computed tomography (CT) showed the

orbits to be of normal size, although the rightsuperior orbital fissure was bigger than the left.There was about 4 mm proptosis on the left.More than 20 small, rounded masses were seen inrelation to the orbit, varying in diameter from 3

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Figure I Clinical photograph showing left proptosis and theleft lower eyelid swelling.

to 12 mm (Fig 2). Most of the retrobulbar lesionswere intraconal, but similar masses were seeninferior to the globe, in the lids, and in theinferior orbital fissure. One relatively large lesionwas contoured to the posteroinferior surface ofthe globe below the macula, and many lay alongthe optic nerve. At least 10 similar masses werepresent in the right orbit, predominantlyanteriorly, below the globe. The rounded massesshowed variable contrast enhancement. Thesuperior ophthalmic veins were not adequatelydemonstrated, but the cavernous sinuses werenormal. The appearances were consideredtypical of orbital varices.

Intra-arterial digital subtraction angiographywas carried out with injections into the leftcommon, internal, and external carotid arteries(Fig 3). This showed small areas of pooling ofcontrast medium alongside the globe (suppliedby the internal carotid) and posterosuperiorly inthe orbit and overlying the maxilla (supplied bythe external carotid). The left superiorophthalmic vein was not visualised.A diagnosis of orbital varices was made and it

was decided to remove the anterior component ofthe vascular anomaly in the left orbit. At opera-tion three 10 mm diameter, discrete, raspberrycoloured masses were removed from the extra-conal fat of the inferior orbit.

Pathological examination revealed that allspecimens had a similar appearance: wide,endothelial lined, blood-filled channels separ-ated by fibrous trabeculae containing smoothmuscle cells and surrounded by a capsule (Fig 4).The features were typical of cavernoushaemangiomas.

DiscussionCavernous haemangioma has been described as

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Sullivan, Aylward, Wright, Moseley, Gamer

Fig 2A Fig2C

Fig 2B Fig 21)

Figure 2 Axial CT(A,B) before (C,D) after intravenous contrast medium. Innumerable rounded or lobulated masses, 2-8 mmin diameter, lie in the retrobulbar spaces and surround the globes. One ofthe largest (arrowheads) is moulded to the posteroinferiorsurface ofthe left globe, while another (arrow) causes medial kinking of the distal portion ofthe left optic nerve. There is leftproptosis.

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Figure 3 Digital subtraction angiogram, lateral projection (anterior to left). (A,B) Arterial and venous phases ofleft internalcarotid and (C,D) similar phases ofexternal carotid injection (withflashfilling ofinternal carotid) show different small areas of

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Bilateral multiple cavernous haemangiomas ofthe orbit

Figure 4 Histological appearance ofthe haemangiomaremovedfrom the left orbit, showing large bloodfilled channelsseparated byfibrous trabeculae. Frequently as here, thetrabeculae are infiltrated by clusters ofsmall capillaries(haematoxylin and eosin, x 80).

the most common orbital tumour.7-9 The fourprincipal reviews of cavernous haemangiomadescribe multiple lesions in the one orbit in noneof six,3 one of 13,4 two of 66,'° and five of 85patients.' No cases of bilateral cavernous

haemangiomas were seen in any of these largeseries, although two cases of presumed bilateralcavernous haemangiomas have recently beenreported. Fries and Char obtained a tissuediagnosis from one orbit but did not operate onthe contralateral orbit because of the paucity ofsymptoms on that side.5 Johnson et al presumeda diagnosis ol bilateral cavernous haemangiomason clinical, ultrasonographic, and radiologicalgrounds without obtaining tissue from eitherorbit.6Our patient had an unusual history with

multiple lesions, variable and intermittent lowerlid swelling, as well as skin discoloration. Harrisand Jakobiec reported one patient who hadrecurrent spontaneous upper lid ecchymosis. '°CT typically shows cavernous haemangiomas

as well-defined rounded intraconal masses withsmooth contours, of similar density to that ofbrain, which displace normal structures andenhance with intravenous contrast medium." 12The differential diagnosis on CT includesperipheral nerve sheath tumours,'3 1' and rare

lesions such as fibrous histiocytoma'4 '5 orhaemangiopericytoma.4 The clinical, CT, andangiographic features in our patient weresuggestive of primary orbital varices; theophthalmologist should be aware of thisvariant.

It was not until surgery that the possibility ofmultiple cavernous haemangiomas arose. It isnot clear why these characteristically unilateral,solitary lesions should have been multiple inboth orbits in our patient. An association withMaffucci's syndrome (characterised by multipleenchondromas, soft tissue haemangiomas, anda generalised predisposition to neoplasia) andpresumed bilateral orbital cavernous haeman-giomas has been made.6 But neither in ourpatient, nor that reported by Fries and Char,were there other stigmata of this disorder.5

Mr Sullivan's work is supported in part by the RACO/OPSMfellowship.

1 McNab AA, Wright JE. Cavernous haemangiomas of theorbit. Aust NZJ Ophthalmol 1989; 17: 337-45.

2 Harris GJ, Jakobiec FA. Cavernous hemangioma of the orbit.In: Jakobiec FA, ed. Ocular and adnexal tumours.Birmingham: Aesculapius, 1978: 741-81.

3 Kopelow SM, Foos RY, Straatsma BR, Hepler RS, PearlmanJT. Cavernous hemangioma of the orbit. Int Ophthalmol Clin1971; 11: 113-24.

4 Ruchmann MC, Flanagan J. Cavernous hemangiomas of theorbit. Ophthalmology 1983; 90: 1328-36.

5 Fries PD, Char DH. Bilateral cavernous haemangiomas. BrjOphthalmol 1988; 72: 871-3.

6 Johnson TE, Nasr AM, Nalbandian RM, Cappelen-Smith J.Enchondromatosis and hemangioma (Maffucci's syndrome)with orbital involvement. Am J Ophthalmol 1990; 110:153-9.

7 Forrest AW. Intraorbital tumours. Arch Ophthalmol 1949; 41:198-232.

8 Moss HM. Expanding lesions of the orbit. A clinical study of230 consecutive cases. Amj Ophthalmol 1962; 54: 761-70.

9 Reese AB. Tumours ofthe eye, 2nd ed. Hagerstown: Harper andRow, 1976.

10 Harris GJ, Jakobiec FA. Cavernous hemangioma of the orbit.JNeurosurg 1979; 51: 219-28.

11 Moseley IF, Sanders MD. Computerised tomography in neuro-ophthalmology. London: Chapman and Hall, 1982: 53-5.

12 Ruchman MC, Stefanyszyn MA, Flanagan JC, Gonzalez CF,Becker MH. Orbital tumors. In: Gonzalez CF, Becker MH,Flanagan JC, eds. Diagnostic imaging in ophthalmology.Berlin: Springer, 1986: 208-11.

13 Dervin JE, Beaconsfield M, Moseley IF, Wright JE. CTfindings in orbital tumours of nerve sheath origin. ClinRadiol 1989; 40: 475-9.

14 Shields JA, Shields CL, Eagle RC. Cavernous hemangioma ofthe orbit. Arch Ophthalmol 1987; 105: 853.

15 Jacomb-Hood J, Moseley IF. Fibrous histiocytoma of theorbit: computed tomographic findings in ten cases and areview of radiological findings. Clin Radiol 1991; 43:117-20.

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