CENTRAL RETINAL ARTERY OCCLUSION SECONDARY TO OPTIC DISK MELANOCYTOMA Pukhraj Rishi MS, DO, Ramesh Venkatesh, MS
Purpose: To report a case of central retinal artery occlusion with cilioretinal artery sparing associated with an optic disk melanocytoma. Methods: Retrospective observational case report. Results: A 29-year-old healthy male adult presented with sudden profound visual loss in the right eye since the past 3 days. His medical history was unremarkable with no history of antecedent trauma. Best-corrected visual acuity was 6/36 in the right eye and 6/6 in the left eye. The left eye was essentially normal. Right afferent pupillary defect was noted. Biomicroscopic examination of right fundus revealed an elevated, deeply pigmented, brown–black mass lesion over the optic disk, suggestive of melanocytoma. Other fundus findings were suggestive of central retinal artery occlusion with cilioretinal artery sparing. Clinical findings were confirmed on fundus fluorescein angiography, optical coherence tomography, ultrasonography, and visual field testing. Severe visual loss can occur because of retinal vascular occlusion in eyes with optic disk melanocytoma. Conclusion: Rapid visual loss and retinal vascular occlusion associated with optic disk melanocytoma may not necessarily be caused by its malignant transformation. It may be caused by tumor necrosis itself. Although rare, it is still important to follow-up such patients closely to look for signs suggestive of malignant transformation. RETINAL CASES & BRIEF REPORTS 6:212–215, 2012
From the Shri Bhagwan Mahavir Vitreoretinal Services, Sankara Nethralaya, Chennai, Tamil Nadu, India.
Case Report A 29-year-old healthy man presented with sudden painless visual loss in the right eye of 3-day duration. His medical history was unremarkable with no history of antecedent trauma. He did not recall having had a prior fundus examination. Best-corrected visual acuity was 6/36 in the right eye and 6/6 in the left eye. The left eye was essentially normal. Right afferent pupillary defect was noted. Intraocular pressure was 14 mmHg in both the eyes. Biomicroscopic examination of the right fundus revealed an elevated, deeply pigmented, brown–black mass lesion over the optic disk, extending to the adjacent retina and choroid. The edges of the lesion were feathery/fibrillated. Almost the entire retina had a pale appearance except for the superior half of the macula, which appeared normally preserved. The retinal arterioles were severely attenuated, and the retinal veins appeared dilated and tortuous. Superficial and deep retinal hemorrhages were noted. Telangiectatic vessels were noted over the superotemporal quadrant of the optic disk (Figure 1). Fundus fluorescein angiography showed a delayed filling of the retinal vessels with sparing of the cilioretinal artery. The tumor remained hypofluorescent throughout the angiogram (Figure 2). Optical coherence tomogram showed thin echogenic line with complete shadowing behind obscuring all details of underlying optic disk and retina. However, subretinal fluid was noted (Figure 3).
M
elanocytoma is a variant of nevus, is deeply pigmented, and usually located on or adjacent to optic disk. Patients with melanocytoma are usually asymptomatic.1 However, severe visual loss can occur under rare circumstances. Melanocytoma can undergo necrosis, causing retinal vascular obstruction2,3 or even rarer still malignant transformation.4 We describe a case that presented with severe visual loss caused by central retinal artery occlusion secondary to optic disk melanocytoma.
The authors report no conflicts of interest. Reprint requests: Pukhraj Rishi, MS, DO, Shri Bhagwan Mahavir Vitreoretinal Services, Sankara Nethralaya, 18 College Road, Chennai 600006, Tamil Nadu, India; e-mail: docrishi@ yahoo.co.in
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CENTRAL RETINAL ARTERY OCCLUSION WITH OPTIC DISK MELANOCYTOMA
Fig. 1. Color fundus photograph of the right eye showing a brown– black mass lesion over the optic disk (white arrow) and extending to adjacent retina (yellow arrow) and choroid (black arrow). The retina has a pale appearance secondary to central retinal artery occlusion except for superior half of the macula because of cilioretinal artery sparing. Telangiectatic vessels are noted over superotemporal quadrant of the optic disk (white arrow with dark outline).
213
Fig. 3. Optical coherence tomogram scan showing an increased retinal thickening and tenting nasal to the fovea with accumulation of subretinal fluid under the nasal fovea.
Ultrasonography showed high surface reflectivity, moderate internal reflectivity, and acoustic solidity, and the lesion measured 1.4 mm in thickness and 6 mm in lateral extent (Figure 4). Visual field testing revealed advanced field loss with central sparing (Figure 5). On the basis of the clinical findings and investigations, optic disk
Fig. 2. Fluorescein angiography showing delayed filling of the retinal arteries and veins with normal filing of the cilioretinal artery (timer in seconds included). Blocked fluorescence is noted because of the presence of retinal hemorrhages and mass lesion over the optic disk.
214
RETINAL CASES & BRIEF REPORTS´ 2012 VOLUME 6 NUMBER 2 melanocytoma with central retinal artery occlusion and sparing of the cilioretinal artery was diagnosed in the right eye.
Discussion
Fig. 4. B-scan ultrasonogram showing an acoustically solid mass at the optic nerve head with high surface reflectivity and moderate internal reflectivity.
Fig. 5. Central (30-2) visual field test showing extensive field loss with preservation of only the central inferior portion of the visual field corresponding to the retinal area supplied by the cilioretinal artery.
The risk of growth of optic disk melanocytoma is reportedly more in cases where the initial tumor thickness is 1.5 mm or more at the time of first diagnosis.5 Notably, the lesion in our case was 1.4 mm thick. However, we did not see any pigmented vitreous seeds. Interestingly, the risk factors for visual loss including retinal extension of tumor and the presence of subretinal fluid were both present.5 Although severe visual loss was noted, extensive involvement of the optic disk by the tumor was not
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CENTRAL RETINAL ARTERY OCCLUSION WITH OPTIC DISK MELANOCYTOMA
apparent. To establish its malignant transformation, a fine needle aspiration biopsy could have been used as a diagnostic aid in our case. However, the tumor size was only 1.4 mm in height, making fine needle aspiration biopsy less safe; hence, it was avoided. Magnetic resonance imaging can detect the extent of optic nerve involvement but cannot rule out microscopic extension of tumor; hence, it was not advised. However, a close follow-up and review after 2 months was advised. Not many cases of melanocytoma of optic disk with central retinal vascular obstruction have been reported.2,3 This case demonstrates that rapid visual loss and presence of signs of retinal vascular occlusion with melanocytoma may not necessarily be caused by its malignant transformation. It may be caused by necrosis within the tumor itself. However, it may still be imperative to follow-up such patients closely to look for signs suggestive of malignant transformation.
Key words: central retinal artery occlusion, cilioretinal artery, melanocytoma, optic disc, tumor. References 1. Shields JA, Demirci H, Mashayekhi A, et al. Melanocytoma of the optic disk: a review. Surv Ophthalmol 2006;51: 93–104. 2. Shields JA, Shields CL, Eagle RC, et al. Central retinal vascular obstruction secondary to melanocytoma of the optic disc. Arch Ophthalmol 2001;119:129–133. 3. Agarwal S, Shanmugam MP, Gopal L, et al. Necrotic melanocytoma of the optic disc with central retinal vascular obstruction. Retina 2005;25:364–367. 4. Shields JA, Shields CL, Eagle RC, et al. Malignant melanoma associated with melanocytoma of optic disc. Ophthalmology 1990;97:225–230. 5. Shields JA, Demirci H, Mashayekhi A, Shields CL. Melanocytoma of optic disc in 115 cases: the 2004 Samuel Johnson Memorial Lecture, part 1. Ophthalmology 2004;111: 1739–1746.
Purpose: To report a case of central retinal artery occlusion with cilioretinal artery sparing associated with an optic disk melanocytoma. Methods: Retrospective observational case report. Results: A 29-year-old healthy male adult presented with sudden profound visual loss in the right eye since the past 3 days. His medical history was unremarkable with no history of antecedent trauma. Best-corrected visual acuity was 6/36 in the right eye and 6/6 in the left eye. The left eye was essentially normal. Right afferent pupillary defect was noted. Biomicroscopic examination of right fundus revealed an elevated, deeply pigmented, brown–black mass lesion over the optic disk, suggestive of melanocytoma. Other fundus findings were suggestive of central retinal artery occlusion with cilioretinal artery sparing. Clinical findings were confirmed on fundus fluorescein angiography, optical coherence tomography, ultrasonography, and visual field testing. Severe visual loss can occur because of retinal vascular occlusion in eyes with optic disk melanocytoma. Conclusion: Rapid visual loss and retinal vascular occlusion associated with optic disk melanocytoma may not necessarily be caused by its malignant transformation. It may be caused by tumor necrosis itself. Although rare, it is still important to follow-up such patients closely to look for signs suggestive of malignant transformation. RETINAL CASES & BRIEF REPORTS 6:212–215, 2012
From the Shri Bhagwan Mahavir Vitreoretinal Services, Sankara Nethralaya, Chennai, Tamil Nadu, India.
Case Report A 29-year-old healthy man presented with sudden painless visual loss in the right eye of 3-day duration. His medical history was unremarkable with no history of antecedent trauma. He did not recall having had a prior fundus examination. Best-corrected visual acuity was 6/36 in the right eye and 6/6 in the left eye. The left eye was essentially normal. Right afferent pupillary defect was noted. Intraocular pressure was 14 mmHg in both the eyes. Biomicroscopic examination of the right fundus revealed an elevated, deeply pigmented, brown–black mass lesion over the optic disk, extending to the adjacent retina and choroid. The edges of the lesion were feathery/fibrillated. Almost the entire retina had a pale appearance except for the superior half of the macula, which appeared normally preserved. The retinal arterioles were severely attenuated, and the retinal veins appeared dilated and tortuous. Superficial and deep retinal hemorrhages were noted. Telangiectatic vessels were noted over the superotemporal quadrant of the optic disk (Figure 1). Fundus fluorescein angiography showed a delayed filling of the retinal vessels with sparing of the cilioretinal artery. The tumor remained hypofluorescent throughout the angiogram (Figure 2). Optical coherence tomogram showed thin echogenic line with complete shadowing behind obscuring all details of underlying optic disk and retina. However, subretinal fluid was noted (Figure 3).
M
elanocytoma is a variant of nevus, is deeply pigmented, and usually located on or adjacent to optic disk. Patients with melanocytoma are usually asymptomatic.1 However, severe visual loss can occur under rare circumstances. Melanocytoma can undergo necrosis, causing retinal vascular obstruction2,3 or even rarer still malignant transformation.4 We describe a case that presented with severe visual loss caused by central retinal artery occlusion secondary to optic disk melanocytoma.
The authors report no conflicts of interest. Reprint requests: Pukhraj Rishi, MS, DO, Shri Bhagwan Mahavir Vitreoretinal Services, Sankara Nethralaya, 18 College Road, Chennai 600006, Tamil Nadu, India; e-mail: docrishi@ yahoo.co.in
212
CENTRAL RETINAL ARTERY OCCLUSION WITH OPTIC DISK MELANOCYTOMA
Fig. 1. Color fundus photograph of the right eye showing a brown– black mass lesion over the optic disk (white arrow) and extending to adjacent retina (yellow arrow) and choroid (black arrow). The retina has a pale appearance secondary to central retinal artery occlusion except for superior half of the macula because of cilioretinal artery sparing. Telangiectatic vessels are noted over superotemporal quadrant of the optic disk (white arrow with dark outline).
213
Fig. 3. Optical coherence tomogram scan showing an increased retinal thickening and tenting nasal to the fovea with accumulation of subretinal fluid under the nasal fovea.
Ultrasonography showed high surface reflectivity, moderate internal reflectivity, and acoustic solidity, and the lesion measured 1.4 mm in thickness and 6 mm in lateral extent (Figure 4). Visual field testing revealed advanced field loss with central sparing (Figure 5). On the basis of the clinical findings and investigations, optic disk
Fig. 2. Fluorescein angiography showing delayed filling of the retinal arteries and veins with normal filing of the cilioretinal artery (timer in seconds included). Blocked fluorescence is noted because of the presence of retinal hemorrhages and mass lesion over the optic disk.
214
RETINAL CASES & BRIEF REPORTS´ 2012 VOLUME 6 NUMBER 2 melanocytoma with central retinal artery occlusion and sparing of the cilioretinal artery was diagnosed in the right eye.
Discussion
Fig. 4. B-scan ultrasonogram showing an acoustically solid mass at the optic nerve head with high surface reflectivity and moderate internal reflectivity.
Fig. 5. Central (30-2) visual field test showing extensive field loss with preservation of only the central inferior portion of the visual field corresponding to the retinal area supplied by the cilioretinal artery.
The risk of growth of optic disk melanocytoma is reportedly more in cases where the initial tumor thickness is 1.5 mm or more at the time of first diagnosis.5 Notably, the lesion in our case was 1.4 mm thick. However, we did not see any pigmented vitreous seeds. Interestingly, the risk factors for visual loss including retinal extension of tumor and the presence of subretinal fluid were both present.5 Although severe visual loss was noted, extensive involvement of the optic disk by the tumor was not
215
CENTRAL RETINAL ARTERY OCCLUSION WITH OPTIC DISK MELANOCYTOMA
apparent. To establish its malignant transformation, a fine needle aspiration biopsy could have been used as a diagnostic aid in our case. However, the tumor size was only 1.4 mm in height, making fine needle aspiration biopsy less safe; hence, it was avoided. Magnetic resonance imaging can detect the extent of optic nerve involvement but cannot rule out microscopic extension of tumor; hence, it was not advised. However, a close follow-up and review after 2 months was advised. Not many cases of melanocytoma of optic disk with central retinal vascular obstruction have been reported.2,3 This case demonstrates that rapid visual loss and presence of signs of retinal vascular occlusion with melanocytoma may not necessarily be caused by its malignant transformation. It may be caused by necrosis within the tumor itself. However, it may still be imperative to follow-up such patients closely to look for signs suggestive of malignant transformation.
Key words: central retinal artery occlusion, cilioretinal artery, melanocytoma, optic disc, tumor. References 1. Shields JA, Demirci H, Mashayekhi A, et al. Melanocytoma of the optic disk: a review. Surv Ophthalmol 2006;51: 93–104. 2. Shields JA, Shields CL, Eagle RC, et al. Central retinal vascular obstruction secondary to melanocytoma of the optic disc. Arch Ophthalmol 2001;119:129–133. 3. Agarwal S, Shanmugam MP, Gopal L, et al. Necrotic melanocytoma of the optic disc with central retinal vascular obstruction. Retina 2005;25:364–367. 4. Shields JA, Shields CL, Eagle RC, et al. Malignant melanoma associated with melanocytoma of optic disc. Ophthalmology 1990;97:225–230. 5. Shields JA, Demirci H, Mashayekhi A, Shields CL. Melanocytoma of optic disc in 115 cases: the 2004 Samuel Johnson Memorial Lecture, part 1. Ophthalmology 2004;111: 1739–1746.
