In Reply We thank Chen and colleagues for their keen interest in our article. The terms staphyloma, coloboma, and caldera have all been used previously to describe the grade 3 phenotype of NCMD. We agree that the lesion is not a coloboma given the lack of absence of tissue. The term caldera applies to those lesions where the sclerochoroidal junction beneath the lesion is continuous or nearly continuous with the adjacent normal posterior wall of the eye as in the example by Chen and colleagues. We have to point out that the sclerochoroidal junction of our patients shown in Figure 1C and D and Figure 2C, E, and F demonstrates a degree of outpouching that is absent in their example. In addition, the choroid within this posterior bowing is thinned to a significant extent. Even though the OCT images in our article were not from EDI-OCT, the sclerochoroidal junction can be visualized clearly throughout most of the scan length. Given the clearly demonstrable bowing out of the sclera and choroid, we used the term staphyloma rather than caldera. It is most likely that the degree of thinning of the posterior wall of the eye in this phenotype of NCMD varies in different patients, with some demonstrating minimal thinning resembling a caldera and others demonstrating more thinning resembling a staphyloma. Newer imaging studies with swept-source and 3-dimensional imaging will help us define the boundaries better in the future. For now, we agree that both terms caldera and staphyloma seem to apply to the condition.
Agarwal A, Schoenberger SD. Macular Caldera in North Carolina Macular Dystrophy—Reply. JAMA Ophthalmol. 2014;132(6):787. doi:10.1001/jamaophthalmol.2014.1781