ABSTRACT

An elderly woman presented with fever, dehydration, orbitalinflammation, total external and internal ophthalmoplegia andblindness, resembling the clinical appearance at presentationof severe orbital inflammatory disease or mucormycosis.Orbital computed tomography scanning demonstrated aretrobulbar orbital mass. Subsequent B-scan ultrasoundexamination confirmed the orbital mass but also demon-strated a mass within the eye. At lateral orbitotomy, extra-scleral spread of an entirely necrotic intraocular melanomawas demonstrated. As computed tomography scanning maynot be able to delineate an entirely necrotic intraocularmalignant melanoma, B-scan ultrasonography should be con-sidered in patients with orbital inflammation, especially in thepresence of a retrobulbar mass.

Key words: choroidal malignant melanoma, orbital inflam-mation.

CASE REPORT

An 85-year-old female nursing home resident presented tocasualty with a 3-day history of a red right eye together withswollen lids, proptosis and serous discharge. She had beeninitially treated with topical chloramphenicol by her generalpractitioner. Apart from multi-infarct dementia and hyper-tension, her past medical history was unremarkable.

She was febrile (37.5°C) and mildly dehydrated. Ophthal-mologic examination showed that she had no perception oflight in the right eye. The pupils were equal in size but theright pupil was fixed, with an indirect relative afferent pupildefect. She had marked chemosis, orbital oedema, proptosis,complete ptosis, total external and internal ophthalmoplegiaand corneal oedema (Fig. 1). No fundal view was obtainedbecause of corneal oedema and a very dense cataract. The

intraocular pressure was 50 mmHg and there was pain onretropulsion of the globe.

Intravenous cephalothin was commenced in order tocover the possibility of orbital cellulitis. Otolaryngologicalreview revealed normal nasal mucosa and no evidence ofmucormycosis was demonstrated in the nasopharynx.Orbital computed tomography (CT) scan on admissiondemonstrated periorbital soft tissue swelling and proptosiswith a retrobulbar mass, but the sinuses were clear (Fig. 2).The eye was reported by the consultant radiologist to benormal, although there was questionable thickening of theposterior sclera.

Three days later, she was afebrile, and the chemosis andlid swelling had improved. One week after admission,orbital B-scan ultrasound examination showed that the rightglobe was filled with highly echogenic material with a solidretrobulbar mass of homogeneous echogenicity (Fig. 3).Colour Doppler showed no evidence of abnormal bloodflow related to the mass. Two weeks later, the proptosis andchemosis had largely resolved, and ocular rotations hadimproved. The intraocular pressure was 12 mmHg. Rubeosisiridis was now visible as the cornea had cleared.

Frozen section of the biopsy of the retrobulbar mass at the time of lateral orbitotomy demonstrated malignantmelanoma. The eye and its attached mass were thereforeremoved en bloc, followed by closure of the lateral orbito-tomy. Macroscopic examination of the specimen showed anirregular retrobulbar mass 25 mm in diameter (Fig. 4). Adarkly pigmented choroidal malignant melanoma withretinal detachment was found intraocularly. The choroidalmelanoma was of a mixed epithelioid and spindle cell type(Fig. 5). The intraocular melanoma was entirely necrotic(Fig. 6) and the extrascleral extension was partly necrotic,extending to its posterior edge. Features of necrotizing episcleritis, panophthalmitis, rubeosis, cataract, intraocularhaemorrhage and central retinal artery occlusion werepresent. The patient achieved reasonable cosmesis followingsurgery and fitting of an ocular prosthesis.

Clinical and Experimental Ophthalmology (2001) 29, 97–99

Case Report

Orbital inflammation in a patient with extrascleral spread ofchoroidal malignant melanomaAlvin SF Goh BSc(Med)(Hons) FRANZCO, Ian C Francis FRANZCO FASOPRS, Medduma B Kappagoda FRANZCO FRCOphth and Marijan Filipic FRCPA FRANZCO(Hon) Ocular Plastics Unit, University of New South Wales, Prince of Wales Hospital, Randwick, Sydney, Australia

� Correspondence: Dr Ian C Francis, Suite 12, Chatswood Grove, 12–14 Malvern Avenue, Chatswood, NSW 2067, Australia. Email: if@student.unsw.edu.au

DISCUSSION

This case presented a number of management problems.The patient was a nursing home resident with multi-infarctdementia, making the time course of the history of her visualloss somewhat unreliable. The differential diagnosisincluded orbital cellulitis, mucormycosis, orbital inflamma-tory disease, inflammation in a retrobulbar orbital tumourand severe thyroid orbitopathy. The patient’s clinical pre-sentation of ptosis, external and internal ophthalmoplegia,blindness, low-grade fever and mild dehydration was similarto the clinical picture of rhino-orbitocerebral mucormy-cosis.1 Mucormycosis was excluded by otolaryngologicalreview and the CT scan. The patient appeared to show apartial response to intravenous antibiotics, as has beenreported in three previous cases.2

Ocular and orbital inflammation may be the first clinicalsign in patients with malignant melanoma of the choroid, asdemonstrated in the present case. In a series of 450 enucle-ated eyes with either malignant melanoma of the choroid orciliary body, 22 (4.9%) presented initially with ocularinflammation (including episcleritis, uveitis, endophthalmi-tis and panophthalmitis).3 Six out of the 22 patients pre-sented with panophthalmitis and all of them had large,

necrotic tumours. Even in the absence of extrascleral exten-sion, intraocular malignant melanomas can also present with ocular inflammation.3,4 Choroidal melanoma withextrascleral extension can produce proptosis and inflam-mation simulating panophthalmitis.5 However, necroticmalignant melanoma without extrascleral extension produc-ing proptosis has also been described.6 Mauriello et al. pos-tulated that the principal cause of orbital inflammation dueto intraocular tumour was probably necrosis, where large

98 Goh et al.

Figure 1. Photograph of the patient demonstrating completeright ptosis and chemosis.

Figure 2. Computed tomography scan showing soft tissueswelling, proptosis and a retrobulbar tumour on the right side.

Figure 3. B-scan ultrasound showing intraocular echogenic material and a solid homogeneous retrobulbar mass.

Figure 4. Enucleation specimen showing an irregular darkly pigmented mass behind the globe.

amounts of inflammatory mediators are released upon celldeath, but inflammation was also felt to result from secretionof inflammatory mediators from the tumour.2

A reported case of disappearance of ocular malignantmelanoma on CT scan after total spontaneous necrosis20 months following the initial diagnosis supported ourfindings.6 This was due to loss of tumour density followingnecrosis. While CT scanning provides accurate estimates ofthickness and basal diameters of posterior uveal melanomas,Augsburger et al. recognized that the use of both CT scan-ning and ultrasonography is more valuable than using eitherof them alone.7

The present case illustrated that CT scanning was unableto detect the necrotic intraocular tumour although it delin-eated the retrobulbar mass. Further, B-scan ultrasonographydid not display the characteristic appearance of a choroidalmelanoma, because of total necrosis of the tumour. How-ever, the appearance of non-homogeneous, highly echo-genic intraocular material is consistent with necroticmalignant melanoma.

The present case supports the notion that both CT scan-ning and ultrasonography should be performed in caseswhere intraocular malignant melanomas are suspected in thepresence of orbital inflammation. However, even then thediagnosis may not be clear until exploratory surgery in somecases. Bond et al. concluded that gadolinium-enhanced T1-weighted magnetic resonance imaging (MRI) appears to bethe most sensitive imaging method for detecting choroidalmelanoma.8 Thus MRI may need to be included in the management of some patients, although the appearance of

necrotic malignant melanoma of the choroid has not yetbeen characterized by MRI study.

REFERENCES

1. Downie JA, Francis IC, Arnold JJ, Bott LM, Kos S. Suddenblindness and total ophthalmoplegia in mucormycosis. Aclinicopathological correlation. J. Clin. Neuro-ophthalmol. 1993;13: 27–34.

2. Mauriello Jr JA, Tello C, Kishore K, Hidayat AA. Carcinomaof the ciliary epithelium presenting as orbital cellulitis. Orbit1996; 15: 47–51.

3. Fraser DJ, Font RL. Ocular inflammation and hemorrhage asinitial manifestation of uveal malignant melanoma. Arch.Ophthalmol. 1979; 97: 1311–14.

4. Rose GE, Hoh HB, Harrad RA, Hungerford JL. Intraocularmalignant melanomas presenting with orbital inflammation.Eye 1993; 7: 539–41.

5. Reese AB, Archila EA, Jones IS, Cooper WC. Necrosis ofmalignant melanoma of the choroid. Am. J. Ophthalmol. 1970;69: 91–104.

6. Hardman Lea SJ, Livesey SJ, Lowe J, Rothwell I, Haworth SM.Disappearance of ocular malignant melanoma on comput-erised scan after spontaneous necrosis: clinical, radiologicaland pathological features. Eye 1991; 5: 748–50.

7. Augsburger JJ, Peyster RG, Markoe AM, Guillet EG, ShieldsJA, Haskin ME. Computed tomography of posterior uvealmelanomas. Arch. Ophthalmol. 1987; 105: 1512–16.

8. Bond JB, Haik BG, Mihara F, Gupta KL. Magnetic resonanceimaging of choroidal melanoma with and without gadoliniumcontrast enhancement. Ophthalmology 1991; 98: 459–66.

Orbital inflammation 99

Figure 5. Malignant melanoma of the choroid of a mixed epithe-lioid (left) and spindle (right) cell type.

Figure 6. The entirely necrotic intraocular malignant melanoma.