Central Areolar Choroidal Dystrophy (CACD) and Age-Related Macular Degeneration (AMD): Differentiating Characteristics in Multimodal Imaging

Smailhodzic, Dženita; Fleckenstein, Monika; Theelen, Thomas; Boon, Camiel J F; Huet, Ramon A. C. van; Ven, Johannes P. H. van de; Hollander, Anneke I. den; Schmitz-Valckenberg, Steffen; Hoyng, Carel B.; Weber, Bernhard H. F.; Holz, Frank G.; Klevering, B. Jeroen

doi:10.1167/iovs.11-7926

Cited by 63 publications

(59 citation statements)

References 60 publications

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“…It is appropriate for the phenotype of central areolar choroidal dystrophy, a dominantly inherited macular dystrophy, which is different from the AMD phenotype and is caused by mutations in the PRPH2 gene ( Fig. 4 ) [26] . This disease commonly manifests as a unifocal atrophic area with a ring of pigmentary changes or -better visible -speckled increased fundus autofluorescence (FAF) intensities around a large central patch of decreased FAF intensities.…”

Section: The Semantics and Origin Of The Term "Geographic"mentioning

confidence: 99%

The Journey of “Geographic Atrophy” through Past, Present, and Future

Schmitz-Valckenberg¹

2017

Ophthalmologica

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“Geographic atrophy” is a concise term that has been firmly established for the description of the end-stage manifestation of nonexudative age-related macular degeneration (AMD). “Geographic lesions” resembling sharply demarcated continents on a map have been originally described in the German literature in 1854 (landkartenartiger/inselförmiger Zungenfratt) for a manifestation later called “geographic tongue” in English. In 1970, Gass was the first to describe “geographic areas of atrophy” in “senile macular choroidal degeneration.” Within a decade, the disease itself was named “geographic atrophy.” Today, various meanings of the term are used in parallel both in research and in routine clinical care. Currently, we are on the verge of better understanding the different forms of atrophy development, manifestation, and progression in AMD, which will pave the way for a more rational approach to their nomenclature and classification.

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Section: The Semantics and Origin Of The Term "Geographic"mentioning

confidence: 99%

The Journey of “Geographic Atrophy” through Past, Present, and Future

Schmitz-Valckenberg¹

2017

Ophthalmologica

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“…With regard to the word “areolar” which had been commonly used in the context of atrophic late AMD (“areolar atrophy” or similar), the primary definition of “areola” is a small ring of color around a center portion, as about the nipple of the breast or the part of the iris surrounding the pupil of the eye This term may be appropriate to describe the phenotype of monogenetically inherited “central areolar choroidal dystrophy” (CACD) which is typically characterized by a ring of pigmentary changes or speckled fundus autofluorescence changes around a large central patch of atrophy 16 . By contrast, the term would neither refer to the shape of atrophy itself nor appreciate the multifocality of atrophy commonly presented in late-stage atrophic AMD.…”

Section: Semantic Considerationsmentioning

confidence: 99%

Geographic Atrophy

Schmitz-Valckenberg

Sadda

Staurenghi

et al. 2016

Retina

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Purpose There is a lack of agreement regarding the types of lesions and clinical conditions that should be included in the term “geographic atrophy”. Varied and conflicting views prevail throughout the literature and are currently used by retinal experts and other health care professionals. Methods We reviewed the nominal definition of the term “geographic atrophy” and conducted a search of the ophthalmological literature focusing on preceding terminologies as well as the first citations of the term “geographic atrophy” secondary to age-related macular degeneration (AMD). Results According to the nominal definition, the term “geography” stands for a detailed description of the surface features of a specific region, indicating its relative position. However, it does not necessarily imply that the borders of the region must be sharply demarcated or related to any anatomical structures. The term “geographical areas of atrophy” was initially cited in the 1960s in the ophthalmological literature in the context of uveitic eye disease and shortly thereafter also for the description of variants of “senile macular degeneration”. However, no direct explanation could be found in the literature as to why the terms “geographical” and “geographic” were chosen. Presumably the terms were used as the atrophic regions resembled the map of a continent or well-defined country borders on thematic geographical maps. With the evolution of the terminology, the commonly used adjunct “of the retinal pigment epithelium” was frequently omitted and solely the term “geographic atrophy” prevailed for the non-exudative late-stage of AMD itself. Along with the quantification of atrophic areas, based on different imaging modalities and the use of both manual and semi-automated approaches, various and inconsistent definitions for the minimal lesion diameter or size of atrophic lesions have also emerged. Conclusions Reconsideration of the application of the term “geographic atrophy” in the context of AMD appears to be prudent given ongoing advances in multi-modal retinal imaging technology with identification of various phenotypic characteristics, and the observation of atrophy development in eyes under anti-angiogenic therapy.

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“…The differential diagnosis of this condition included pericentral retinitis pigmentosa [2], pericentralpigmentary retinopathy [3], pericentral retinal dystrophy [4], circinatechoroidal sclerosis [5], annular choroidal sclerosis [6], choroidal sclerosis [7], and central areolar choroidal dystrophy [8].…”

Section: Case Reportmentioning

confidence: 99%

“…Central areolar choroidal dystrophy is a hereditary retinal disorder that affects the macula, resulting in progressive and usually profound visual loss [8]. The hallmark feature of the disorder is a well-defined atrophy of the retinal pigment epithelium and the choriocapillaris involved the fovea.…”

confidence: 99%

Posterior Polar Hemispheric Choroidal Dystrophy

Makino¹

2015

J Clin Res Ophthalmol

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Central Areolar Choroidal Dystrophy (CACD) and Age-Related Macular Degeneration (AMD): Differentiating Characteristics in Multimodal Imaging

Cited by 63 publications

References 60 publications

The Journey of “Geographic Atrophy” through Past, Present, and Future

The Journey of “Geographic Atrophy” through Past, Present, and Future

Geographic Atrophy

Posterior Polar Hemispheric Choroidal Dystrophy

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