Progressive choroidal thinning (leptochoroid) and fundus depigmentation associated with checkpoint inhibitors

Canestraro, Julia; Jaben, Korey; Wolchok, Jedd D.; Abramson, David H.; Francis, Jasmine H.

doi:10.1016/j.ajoc.2020.100799

Cited by 12 publications

(14 citation statements)

References 8 publications

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“…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%

“…The most commonly encountered pattern is posterior choroidal melanosis and surrounding midzonal and peripheral choroidal hypomelanosis [ 16 ]. A second clinical pattern of this disorder is posterior choroidal hypomelanosis with retained peripheral choroidal melanosis [ 17 ]. A third (and least common, in our experience) pattern of acquired bilateral zonal choroidal melanocytic fundopathy is midzonal choroidal hypomelanosis with retained posterior and peripheral choroidal melanosis.…”

Section: Discussionmentioning

confidence: 99%

“…A third (and least common, in our experience) pattern of acquired bilateral zonal choroidal melanocytic fundopathy is midzonal choroidal hypomelanosis with retained posterior and peripheral choroidal melanosis. Cases of the two former types have been evaluated by OCT, and the hypomelanotic areas of the choroid in such cases have been shown to be abnormally thinned relative to comparable fundus areas in unaffected persons [ 16 , 17 ]; in contrast, the melanotic choroidal zones in such cases appear to be relatively normal in thickness. Abramson and coworkers have applied the term “leptochoroid” to the thinned choroidal areas in such eyes [ 16 , 17 ].…”

Section: Discussionmentioning

confidence: 99%

“…Cases of the two former types have been evaluated by OCT, and the hypomelanotic areas of the choroid in such cases have been shown to be abnormally thinned relative to comparable fundus areas in unaffected persons [ 16 , 17 ]; in contrast, the melanotic choroidal zones in such cases appear to be relatively normal in thickness. Abramson and coworkers have applied the term “leptochoroid” to the thinned choroidal areas in such eyes [ 16 , 17 ]. They have associated the first pattern mentioned above with advanced age, and they have associated the second pattern with systemic therapy using checkpoint inhibitors.…”

Section: Discussionmentioning

confidence: 99%

“…The inclusion criterion used to identify cases for this study was a patch of confluent melanotic choroidal hyperpigmentation consistent with congenital choroidal melanocytosis in one or both eyes. Exclusion criteria used to eliminate cases were (1) largest arc length basal diameter of the melanotic choroidal lesion less than 5 mm, (2) measurable thickness of the melanotic choroidal lesion greater than that of the surrounding uninvolved choroid by B-scan ultrasonography, (3) presence of ipsilateral anterior scleral or iridic features of ocular melanocytosis [ 9 , 10 ], (4) acquired bilateral patchy-streaky choroidal melanocytic fundopathy consistent with the fundopathy of cutaneous vitiligo [ 11 , 12 ] and Waardenburg syndrome [ 13 – 15 ], and (5) acquired bilateral zonal choroidal melanocytic fundopathy that is age-related or drug-induced [ 16 , 17 ]. Lesion diameter less than 5 mm was used to exclude typical choroidal nevi, 95% of which have been reported to be smaller than this [ 18 ].…”

Section: Methodsmentioning

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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