Seminars in Ophthalmology

ISSN: 0882-0538 (Print) 1744-5205 (Online) Journal homepage: http://www.tandfonline.com/loi/isio20

Melanocytoma of the Optic Nerve Head, ThirtyMonth Follow-Up Hasan Eldaly & Zeiad Eldaly To cite this article: Hasan Eldaly & Zeiad Eldaly (2014): Melanocytoma of the Optic Nerve Head, Thirty-Month Follow-Up, Seminars in Ophthalmology, DOI: 10.3109/08820538.2013.874485 To link to this article: http://dx.doi.org/10.3109/08820538.2013.874485

Published online: 07 Feb 2014.

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Date: 05 November 2015, At: 15:16

Seminars in Ophthalmology, Early Online, 1–6, 2014 ! Informa Healthcare USA, Inc. ISSN: 0882-0538 print / 1744-5205 online DOI: 10.3109/08820538.2013.874485

C ASE REPORT

Melanocytoma of the Optic Nerve Head, Thirty-Month Follow-Up Hasan Eldaly1 and Zeiad Eldaly2

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Aswan Eye and Laser Center, Aswan, Egypt and 2Ophthalmology Department, Assiut University Hospital, Assiut, Egypt

ABSTRACT Introduction: Optic nerve head (ONH) melanocytoma is a benign pigmented tumor that is detected as a dark brown mass on or adjacent to the optic disc. Ultrasonography, optical coherence tomography (OCT), and fundus fluorescein angiography (FFA) can be used to differentiate it from other pigmented tumor as well as to monitor tumor growth. Visual field changes are variable in ONH melanocytoma. Usually, ONH melanocytoma has a stationary course with few cases of progressive growth or malignant transformation. Case Presentation: A 54-year-old female complained of blurring of vision. Best-corrected visual acuity (BCVA) was 0.9 in both eyes. On routine fundus examination, an elevated pigmented lesion was found, involving the optic nerve head of the left eye. B-scan ultrasound demonstrated a mass arising from ONH. FFA revealed blocked fluorescence corresponding to pigmented mass. HR-OCT scans showed a dome-shaped, elevated ONH lesion with highly reflective layer and optical shadowing behind it, obscuring most of the optic nerve head details. On regular follow-up for 30 months, the lesion had neither changed in its size nor affected the patient’s visual acuity. Conclusion: ONH melanocytoma is a benign tumor with rare malignant transformation and visual impairment. Keywords: Blocked fluorescence, blurring of vision, malignant transformation, optical coherence tomography, pigmented optic nerve head lesion

detected by use of OCT.5,6 Visual field defects can be detected in up to 90% of patients with optic disc melanocytoma. The most commonly reported visual field abnormality was enlargement of the blind spot.7,8

INTRODUCTION Melanocytoma of the optic disc is a benign tumor that is usually detected as a dark brown mass on or adjacent to the optic nerve head. It is thought to derive from dendritic uveal melanocytes of the lamina cribrosa.1,2 Histologically, a melanocytoma is a magnocellular nevus with round to oval cells which occurs more commonly in dark-skinned individuals. Only 1 to 2% of the cases show a malignant transformation.3 Diagnostic tools, such as ultrasonography, visual field analysis, FFA, and OCT can be used with different diagnostic values.4 Visual loss may occur due to several reasons, such as spontaneous necrosis or compressive optic neuropathy, which be seen during follow-up. It can also be associated with subretinal fluid, cystoid retinal edema, retinal traction, and epiretinal membrane, which can be

CASE PRESENTATION In January 2011, a 54-year-old female complained of blurring of vision in both eyes. Glasses were prescribed to her with BCVA was 0.9 in both eyes. Intra-ocular pressure was 14 in both eyes. On routine fundus examination, an elevated pigmented lesion was found, involving upper, lower, and nasal quadrants of the optic nerve head of the left eye extending to nasal juxta-papillary chorio-retina. Slit-lamp biomicroscopy of the right eye did not show any

Received 23 July 2013; accepted 8 December 2013; published online 2 2 2 Correspondence: Zeiad Eldaly, Opthalmology Department, Assiut University Hospital, Assiut, Egypt. E-mail: [email protected]

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H. Eldaly and Z. Eldaly unchanged since the beginning of our observation (Figure 7).

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DISCUSSION

FIGURE 1. B-scan ultrasound (left eye). A small, dome-shaped mass arising from the optic nerve head could be seen. The mass had homogenous, moderate to high internal reflectivity without any choroidal excavation or orbital shadowing. 31  22 mm (300  300 DPI).

pigmented lesion. Anterior segment assessment of both eyes showed no associated conjunctival melanocytosis. B-scan ultrasound demonstrated a mass arising from the optic nerve head (Figure 1). The mass had homogenous, moderate to high internal reflectivity without any choroidal excavation or orbital shadowing. FFA showed hypofluorescence corresponding to the blocked choroidal and retinal fluorescence by the pigmented lesion in both early and late phases with absence of leakage around the mass and dual circulation (Figure 2). Humphrey Visual Field analysis of the left eye revealed lower arcuate and upper nasal scotomas, while analysis of the right eye revealed no significant abnormality (Figure 3). HR-OCT scans showed a dome-shaped, elevated ONH lesion with highly reflective layer with optical shadowing behind it, obscuring most of the optic nerve head details. The highly reflective layer produced by the mass was continuous with the high reflective signal produced by the adjacent retinal nerve fiber layer. OCT scans revealed no subretinal fluid, cystoid retinal edema, retinal traction, or epiretinal membrane (Figure 4). Peri-papillary retinal nerve fiber layer (RNFL) thickness map showed important fiber loss at the site of the lesion, in comparison to the right eye (Figure 5). Based on clinical appearance and data collected from various ophthalmic investigations, the lesion was diagnosed as melanocytoma of the optic nerve head and the patient was advised to have periodic examinations. On regular visits of our patient from January 2011 until July 2013, visual acuity remained unchanged during our follow-up. A B-scan ultrasound was done during follow-up (Figure 6). There were no changes observed in mass size, echogenicity, or choroidal excavation. Fundus examination and FFA remained

Melanocytoma is a deeply pigmented variant of melanocytic nevus that affects the optic disk with limited peripapillary involvement. It may increase in size in 10–15% of cases.3 The size of ONH melanocytoma does not usually exceed 1–2 disc diameter, except in one case where Joffe et al. reported a six disc diameter ONH melanocytoma.9 Follow-up of our patient for 30 months revealed neither enlargement of the lesion nor significant visual impairment. This is confirmed by serial fundus examinations, ultrasonography and FFA. On visual field analysis, an enlarged blind spot and nasal scotoma could be recognized. However, visual field changes were stationary and did not affect the visual acuity of the patient. In addition to enlargement of the blind spot, other visual field abnormalities have been described, such as arcuate defect, nasal step, or paracentral scotoma caused by mechanical compression on the nerve fiber bundles or its microcirculation.8 By ocular ultrasound, ONH melanocytoma acquires a small, dome-shaped elevation with high echogenicity and absent internal acoustic hollowness and choroidal excavation characteristic for choroidal melanomas. Blocked retinal and choroidal fluorescence corresponding to the lesion with no leakage in the late phases are FFA characteristics of ONH melanocytoma. Gupta et al. reported similar findings by both ophthalmic ultrasound and FFA.10 HR-OCT of the optic nerve head and peripapillary zone may be useful in the diagnosis and differentiation of ONH melanocytoma. HR-OCT revealed a dome-shaped elevated lesion from the surface of the optic nerve head and infero-nasal peripapillary zone with high reflective surface and back-shadowing over the underlying structures. The high reflectivity surface of the lesion is continuous with high reflectivity of RNFL with no associated involvement of peripapillary structures. This is one of the advantages of OCT in differentiating ONH melanocytoma from peripapillary choroidal melanoma, in which high reflective surface is continuous with high reflective band of retinal pigment epithelium-Bruch membrane complex. Back-shadowing of the ONH melanocytoma and incapability of assessing its internal structure are drawbacks of OCT in such cases as reported by Chaudhary et al.11 This could be attributed to high pigmentary content, which blocks the transmission of light through the ONH melanocytoma. Antcliff et al.1 Seminars in Ophthalmology

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Optic Nerve Head Melanocytoma 3

FIGURE 2. Fundus photography (top) and FFA (bottom left, early phase and bottom right, late phase) of the left eye. There is a pigmented lesion involving most of the optic nerve head. Hypofluorescence is corresponding to blockage by pigmented lesion with no leakage. 77  64 mm (300  300 DPI).

FIGURE 3. Visual field analysis of both eyes. It reveals lower arcuate and upper nasal scotoma with enlarged blind spot. No abnormality is detected in visual field analysis of the right eye. 210  112 mm (96  96 DPI).

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H. Eldaly and Z. Eldaly

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Chaudhary et al.11 and Go¨nu¨l et al.6 reported similar OCT features of optic nerve head melanocytoma in a different series of cases. In spite of the stationary course of the lesion previously mentioned, with stability of good visual acuity through our follow-up period, cases with severe visual impairment have been reported. Al-Rashaed et al. have reported visual impairment

FIGURE 4. HR-OCT (ONH radial scans) of left eye. Elevated ONH lesion with highly reflective layer and back-shadowing over the underlying structures. 264  516 mm (72  72 DPI).

FIGURE 6. Serial B-scan ultrasound of left eye (February 2011 (top), May 2012 (middle), and June 2013 (bottom)). There is no change in mass size, echogenicity, or choroidal excavation. 148  310 mm (96  96 DPI).

FIGURE 5. RNFL thickness map (A: right eye; B: left eye). RNFL loss could be seen corresponding to the location of the lesion in the left eye. Normal bi-hump configuration of RNFL thickness curve is seen in the right eye. 20  4 mm (300  300 DPI). Seminars in Ophthalmology

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Optic Nerve Head Melanocytoma 5

FIGURE 7. Serial fundus photography and FFA of the left eye (left column: February 2011; right column: June 2013). There is no change in lesion morphology, dimensions, or fluorescein patterns. 76  61 mm (300  300 DPI).

secondary to ONH melanocytomas associated with optic atrophy, foveal choroidal neovascularization, optic disc odema, macular exudation, and peripapillary subretinal fluid.4 Rai et al. and Zimmerman et al. have reported cases with glaucomatous field defect which necessitated closer follow-up.12,13 Intracranial meningioma can also be associated with optic disc melanocytoma and should be suspected if visual field defects respecting the vertical midline are found.14,15 In a single case, Shields et al. have reported a case of central retinal vein occlusion secondary to ONH melanocytoma.16

CONCLUSION Melanocytoma of the optic disc is a benign tumor that rarely causes visual impairment. However, visual acuity may be impaired by several mechanisms such as tumor necrosis, progressive enlargement, and associated rare tumor-related complications. It is important to follow up every case with melanocytoma of the optic disc as long as possible. !

2014 Informa Healthcare USA, Inc.

DECLARATION OF INTEREST The authors report no conflicts of interest. The authors alone are responsible for the content and writing of the paper.

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H. Eldaly and Z. Eldaly 12. Rai S, Medeiros FA, Levi L, Weinreb RN. Optic disc melanocytoma and glaucoma. Semin Ophthalmol 2007;22: 147–150. 13. Zimmerman LE, Garron LK. Melanocytoma of the optic disk. Int Ophthalmol Clin 1962;2:431–440. 14. Shinoda K, Hayasaka S, Nagaki Y, et al. Melanocytoma of the left optic nerve head and right retrobulbar optic neuropathy compressed by a tuberculum sellae meningioma. Ophthalmologica 2002;14:161–163. 15. Walsh TJ, Packer S. Bilateral melanocytoma of the optic nerve associated with intracranial meningioma. Ann Ophthalmol 1971;3:885–888. 16. Shields JA, Shields CL, Eagle Jr RC, et al. Central retinal vascular obstruction secondary to melanocytoma of the optic disc. Arch Ophthalmol 2001;119:129–133.

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7. Usui T, Shirakashi M, Kurosawa A, et al. Visual disturbance in patients with melanocytoma of the optic disk. Ophthalmologica 1990;201:92–98. 8. Osher RH, Shields JA, Layman PR. Pupillary and visual field evaluation in patients with melanocytoma of the optic disc. Arch Ophthalmol 1979;97:1096–1099. 9. Joffe L, Shields JA, Osher RH, Gass JD. Clinical and follow-up studies of melanocytomas of the optic disc. Ophthalmology 1979;86:1067–1078. 10. Gupta V, Gupta A, Dogra MR, Pandav SS. Progressive growth in melanocytoma of the optic nerve head. Indian J Ophthalmol 1995;43:198–200. 11. Chaudhary R, Arora R, Mehta DK, Singh M. Optical coherence tomography study of optic disk melanocytoma. Ophthalmic Surg Lasers Imaging 2006;37:58–61.

Seminars in Ophthalmology

Melanocytoma of the Optic Nerve Head, Thirty-Month Follow-Up.

Optic nerve head (ONH) melanocytoma is a benign pigmented tumor that is detected as a dark brown mass on or adjacent to the optic disc. Ultrasonograph...
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