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Comment & Response |

Macular Caldera in North Carolina Macular Dystrophy Only an Illusion of Posterior Pole Staphyloma

Connie Chen, MD1; Rahul N. Khurana, MD2; Hendrik Scholl, MD1; Morton Goldberg, MD1
[+] Author Affiliations
1Department of Ophthalmology, Wilmer Eye Institute, Johns Hopkins University, Baltimore, Maryland
2Northern California Retina Vitreous Associates, Mountain View
JAMA Ophthalmol. 2014;132(6):786-787. doi:10.1001/jamaophthalmol.2014.1705.
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To the Editor We read with interest the article by Schoenberger and Agarwal titled “Intrachoroidal Cavitation in North Carolina Macular Dystrophy.”1 They describe 2 patients who had bilateral macular “staphylomas.” We disagree with the nomenclature (staphyloma) used to describe these advanced macular lesions and favor the term macular caldera, as previously explained by Khurana et al.2Staphyloma refers to a definite outpouching of the sclera that is lined with uveal tissue. Although Schoenberger and Agarwal demonstrate with optical coherence tomography (OCT) the posterior bowing of the central macula, the entire sclerochoroidal interface is not well visualized on their conventional spectral-domain OCT.

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Figure 2.
B-Scan Ultrasonographic Image From Patient With North Carolina Macular Dystrophy

B-scan ultrasonographic image captured through the macular lesion shows no outpouching of the sclera.

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Figure 1.
Enhanced-Depth Imaging Optical Coherence Tomographic Images of the Advanced Macular Lesion in North Carolina Macular Dystrophy

Only minimal (microscopic) convexity of the sclera underlying the macular lesion is seen. Note absence of choroidal attenuation or stretching. Red arrowheads indicate lesion borders; white arrowheads, sclerochoroidal interface.

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June 1, 2014
Anita Agarwal, MD; Scott D. Schoenberger, MD
1Vanderbilt Eye Institute, Vanderbilt University Medical Center, Nashville, Tennessee
JAMA Ophthalmol. 2014;132(6):787. doi:10.1001/jamaophthalmol.2014.1781.
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