Bilateral Isolated Choroidal Melanocytosis

Fine, Howard F; Bruè, Claudia; Eandi, Chiara M; Jacobs, Michael I.; Pulitzer, Melissa; Yannuzzi, Lawrence A.

doi:10.1097/icb.0b013e3181737718

Cited by 10 publications

(5 citation statements)

References 6 publications

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“…8 Another report of a similar condition had bilateral multifocal lesions. 9 The findings in both these reports were different than those seen in our patient. 8.…”

Section: Discussioncontrasting

confidence: 76%

Unusual Multifocal Pigmented Lesions of the Uvea in a Patient With Ocular Melanocytosis

Reichstein

Shields

Tran

et al. 2013

RETINAL Cases &Amp; Brief Reports

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“…8 Another report of a similar condition had bilateral multifocal lesions. 9 The findings in both these reports were different than those seen in our patient. 8.…”

Section: Discussioncontrasting

confidence: 76%

Unusual Multifocal Pigmented Lesions of the Uvea in a Patient With Ocular Melanocytosis

Reichstein

Shields

Tran

et al. 2013

RETINAL Cases &Amp; Brief Reports

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“…Because of this finding, we did not consider this patient to have bilateral isolated choroidal melanocytosis. Several cases categorized as bilateral isolated choroidal melanocytosis have been reported [3][4][5][6][7][8]; however, based on our review of the published fundus photographs of those cases, most of them appear to us to have been either acquired patchy-streaky choroidal melanocytic fundopathy or acquired zonal choroidal melanocytic fundopathy, as described above, and not true bilateral isolated choroidal melanocytosis. None of the reported bilateral cases was identified in the neonatal or infantile period of life.…”

Section: Certainty About Clinical Diagnosis (In This Series and In Clinical Practice)mentioning

confidence: 79%

“…The relevant differential diagnosis of isolated choroidal melanocytosis having the clinical characteristics of the cases comprising the current series includes broad-based (diffuse) choroidal nevus [ 19 ], diffuse choroidal melanocytoma [ 28 – 30 ], acquired bilateral patchy-streaky choroidal melanocytic fundopathy associated with systemic disorders such as cutaneous vitiligo [ 11 , 12 ] and Waardenburg syndrome [ 13 – 15 ] but also occurring idiopathically in some individuals [ 5 , 6 ], acquired bilateral zonal choroidal melanocytic fundopathy that is age-related [ 4 , 8 , 16 ] or associated with treatment with certain therapeutic agents [ 17 ], and diffuse uveal melanocytic proliferation associated with an underlying systemic malignancy [ 31 ].…”

Section: Discussionmentioning

confidence: 99%

“…In other cases, there is no concurrent acquired iris depigmentation. Most reported patients with this form of patchy-streaky choroidal melanocytic fundopathy without iris depigmentation have been found to have cutaneous vitiligo [ 11 , 12 ]; however, idiopathic forms of this form of the disorder have also been reported [ 5 , 6 ]. In cases of both Waardenburg syndrome fundopathy and cutaneous vitiligo fundopathy, optical coherence tomography (OCT) studies have generally shown the melanotic patches to be normal in thickness and the hypomelanotic areas to have decreased thickness, presumably due to loss of uveal melanocytes in those areas [ 32 , 33 ].…”

Section: Discussionmentioning

confidence: 99%

“…The overlying retina appears unremarkable ophthalmoscopically and by optical coherence tomography. Just as with complete ocular melanocytosis, most affected persons exhibit unilateral involvement; however, bilateral cases categorized as isolated choroidal melanocytosis have been reported [3][4][5][6][7][8]. Partial (sectoral) forms of complete ocular melanocytosis have been associated with an increased risk of primary uveal melanoma arising from the originally flat melanocytic choroidal lesion [9]; however, the risk in such eyes appears to be much lower than that associated with complete generalized ocular melanocytosis.…”

Section: Complete and Partial Forms Of Ocular Melanocytosismentioning

confidence: 99%

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Isolated choroidal melanocytosis: clinical update on 37 cases

Augsburger

Brooks

Corrêa

2020

Graefes Arch Clin Exp Ophthalmol

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Purpose Isolated choroidal melanocytosis is a congenital melanocytic hyperpigmentation involving the choroid that is not associated with iridic or scleral features of ocular melanocytosis. The purpose of this work was to describe the clinical features and course of a relatively large series of patients with this disorder. Methods A retrospective clinical study of 37 patients with isolated choroidal melanocytosis encountered in a single practice 1986–2018 was done. All lesions were 5 mm or larger in the largest basal diameter, homogeneously melanotic, and completely flat by conventional ocular ultrasonography. Results The 37 patients ranged in age from 2 weeks to 87 years (mean 31.5 years, median 18 years) at initial diagnosis of the melanotic choroidal lesion. Arc length largest basal diameter of the melanotic choroidal lesion ranged from 5.5 to 37 mm (mean 14.6 mm, median 13 mm). The lesion extended beneath the fovea in 18 eyes and to the optic disc margin in 6 eyes. Ten of the lesions straddled the ocular equator, but the center point of all of the lesions was posterior to the equator. The retina was fully attached and appeared normal over the melanotic choroidal lesion in each of these eyes. None of the melanotic choroidal lesions exhibited clumps of orange pigment or drusen on its surface. The lesion was unilateral and unifocal in 36 of the 37 patients. One patient had bilateral choroidal melanocytosis that was isolated in one eye but associated with partial iris melanocytosis in the fellow eye. Three adult patients had a choroidal melanoma localized to the patch of choroidal melanocytosis at baseline. One other adult patient had a choroidal melanoma in the fellow eye at baseline. One pediatric patient had viable unilateral non-familial retinoblastoma in the fellow eye and two adult patients had a classic choroidal nevus in the fellow eye. None of the flat patches of choroidal melanocytosis that were monitored periodically after initial diagnosis expanded appreciably during follow-up ranging from 4.9 months to 15.2 years (mean 5.0 years, median 2.3 years). Conclusions Isolated choroidal melanocytosis is a distinct clinical entity that must be distinguished from broad-based choroidal nevus, choroidal melanocytoma, small choroidal malignant melanoma, acquired bilateral patchy-streaky choroidal melanocytic fundopathy associated with disorders such as cutaneous vitiligo and Waardenburg syndrome, acquired bilateral zonal choroidal melanocytic fundopathy, and diffuse uveal melanocytic proliferation associated with systemic cancer. This disorder appears to predispose affected eyes to development of choroidal melanoma arising from the hypermelanotic patch.

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