|Year : 2004 | Volume
| Issue : 3 | Page : 238-41
Choroidal melanoma with macular hole.
S Narang, S Kochar, Kamal Singh Pannu, N Kalra, R Gupta, S Sood
Department of Ophthalmology, Govt. Medical College Hospital, Chandigarh, India
|Date of Submission||07-Oct-2002|
|Date of Acceptance||17-Jun-2003|
Department of Ophthalmology, Govt. Medical College Hospital, Chandigarh
Source of Support: None, Conflict of Interest: None
A rare case of choroidal melanoma with exudative retinal detachment and a full-thickness macular hole is reported in an Indian patient.
Keywords: Choroidal melanoma, macular hole, retinal detachment
|How to cite this article:|
Narang S, Kochar S, Pannu KS, Kalra N, Gupta R, Sood S. Choroidal melanoma with macular hole. Indian J Ophthalmol 2004;52:238
Choroidal melanoma, which usually presents in the fifth decade of life is not very common in India. The tumour arises from the melanocytes in the uveal stroma derived from the neural crest. Although a few cases of choroidal melanoma have been documented to have rhegmatogenous retinal detachment with detectable retinal breaks, the co-existence of a macular hole with choroidal melanoma is extremely rare. We report a case of choroidal melanoma with a full-thickness macular hole at initial presentation.
| Case report|| |
A 45-year-old female presented with the chief complaint of gradual painless progressive diminution of vision in the left eye of four months' duration. There were no other ocular complaints. Her systemic history was unremarkable.
On ocular examination, her visual acuity in the right eye was 6/6 and in the left eye was 1/60. Intraocular pressure was 12mmHg in both eyes. There was a relative afferent pupillary defect in the left eye. Anterior and posterior segment examination of the right eye including visual fields were within normal limits. Slitlamp biomicroscopy of the left eye showed a normal anterior chamber and +2 inflammatory cellular reaction in the vitreous. Pigmented cells were not seen in the vitreous cavity. Fundoscopy of the left eye through a dilated pupil showed grade I media clarity and an elevated, well defined, orangeish-yellow, round to oval, subretinal mass about four disc diameters in size in the macular area. The overlying retina showed some pigmentary changes and a full-thickness macular hole about 500 µ in diameter [Figure - 1]. A smooth grey dome-shaped elevation of the inferior and inferotemporal retina with shifting subretinal fluid was seen, suggestive of exudative retinal detachment. The exudative retinal detachment commu-nicated with the macular hole in the supine position. There were no other detectable peripheral retinal breaks.
Fundus fluorescein angiography showed early hypofluorescence corresponding to the mass lesion with double circulation pattern, characterised by simultaneous visibility of both choroidal and retinal vessels in the arterial phase [Figure - 2], top). Late dye transit phase showed diffuse hyperfluorescence with dye leakage [Figure - 2], bottom). Ultrasonography with 5-10 MHz high frequency linear transducer demonstrated a well circumscribed subretinal mass of low to moderate echogenecity measuring 1.02 x 0.91x 0.47cm temporal to the optic disc with acoustically quiet areas along with a retinal detachment [Figure - 3], top). Colour Doppler imaging showed increased vascularity of the lesion with a double circulation pattern due to visibility of both choroidal and retinal vessels [Figure - 4], [Figure - 5]. Magnetic resonance imaging revealed a well defined curvilinear lesion involving the posterior part of the left globe temporal to the optic nerve. The lesion was mild to moderately hyperintense on T1 weighted image [Figure - 3], bottom left) and hypointense on T2 weighted image [Figure - 3], bottom right). Thorough systemic examination and investigations including complete haemogram, liver and renal function tests, X-ray chest, ultrasound abdomen and proctoscopy to rule out secondaries, were within normal limits.
A diagnosis of small posterior pole choroidal melanoma of the left eye, with a macular hole and an exudative retinal detachment, without any detectable metastasis was made. Transpupillary thermotherapy was carried out with a 3.0 mm spot size, a 700 mW power setting and one minute exposure time. Three months after treatment, fundoscopy revealed a decrease in tumour size with increased pigmentation [Figure - 1], Inset). The exudative retinal detachment was markedly reduced in extent and the minimal residual shifting fluid did not communicate with the macular hole in the supine position. On ultrasono-graphic evaluation the tumour had reduced in size to 0.88 x 0.38 x 0.70 cm and 0.72 x 0.61 x 0.31cm at 3 and 6 months' follow up respectively. Colour Doppler imaging demonstrated reduced vascularity of the mass lesion. The visual acuity however did not show any improvement after treatment.
| Discussion|| |
Choroidal melanoma associated with a macular hole is rare. It has been reported only on two earlier occasions., Uffer and Zografos were the first to report the association of a macular hole and choroidal melanoma with an accompanying rhegmatogenous retinal detachment. In the case described by Balestrazzi et al occurrence of macular hole leading to an inferior rhegmatogenous retinal detachment occured three months after transpupillary thermotherapy for choroidal melanoma. Since the tumour site was remote from the macula, transpupillary thermotherapy was unlikely to have caused the macular hole. However, Balestrazzi et al advocate future cautious evaluation of transpupillary thermotherapy. The macular hole was diagnosed histopathologically only after enucleation in a case reported by Dunn et al.
In our case the macular hole was seen on stereoscopic posterior segment evaluation at the time of initial presentation before any treatment intervention. Retinal degenerative changes like thinning and cystoid oedema over the tumour apex are common in choroidal melanoma. Cystoid macular oedema has also been reported, even when the tumour is remotely located from the posterior pole. It could be hypothesised that the macular hole in the present case followed cystoid macular oedema. It is well known that inflammatory cellular reaction in choroidal melanoma in the vitreous may be part of a generalised ocular inflammatory response secondary to choroidal melanoma, occurring as a part of host response to tumour antigens. Inflammation-induced increase in capillary permeability could have resulted in cystoid macular oedema leading to a macular hole. Additionally, exudative retinal detachment could have contributed to the development of cystoid changes in the macula. Since fundus fluorescein angiography does not provide any information on the macula, we believe optical coherence tomography (OCT) could highlight any changes in the overlying vitreous. But OCT was not done in the present case since the facility was not available.
The presence of a macular hole with choroidal melanoma should lead to careful evaluation as regards to local spread of the tumour, particularly when the hole is located over the mass. The absence of a "collar-button" appearance of the tumour on B-scan ultrasonography was suggestive that it was confined to the choroid with an intact Bruch's membrane. Moreover, the absence of pigmented cells in the vitreous cavity was further indication against vitreous seeding or local spread of the tumour. Hence, the patient was considered for transpupillary thermotherapy.
Our case of malignant melanoma with co-existent macular hole was unique. Although transpupillary thermotherapy seems to be effective in treating choroidal melanoma even in the presence of a macular hole, follow up for future development of rhegmato-genous retinal detachment is mandatory in such cases.
| References|| |
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[Figure - 1], [Figure - 2], [Figure - 3], [Figure - 4], [Figure - 5]