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Posterior amorphous corneal dystrophy (PACD) (OMIM 612868) is a rare autosomal dominant disorder characterized by partial or complete posterior lamellar corneal opacification, decreased corneal thickness and flattening of the corneal curvature. PACD is associated with heterozygous deletions in chromosome band 12q21.33 harboring DCN, KERA, LUM, and EPYC which encode small leucine-rich proteoglycans. We report on a 7-year-old male patient with PACD who had an interstitial deletion of 1.3 Mb in 12q21.33. His mother carried a balanced insertional translocation involving this 12q21.33 segment which was inserted into the proximal part of the long arm of one chromosome 13. The patient corroborates previous observations that PACD is a contiguous gene syndrome caused by combined haploinsufficiency of DCN, KERA, LUM, and EPYC and provides the first example of a balanced chromosome rearrangement involving 12q21.33 in an unaffected parent.

We assess the accuracy of the Visante anterior segment optical coherence tomographer (AS-OCT) and present improved formulas for measurement of surface curvature and axial separation. Measurements are made in physical model eyes. Accuracy is compared for measurements of corneal thickness (d(1)) and anterior chamber depth (d(2)) using-built-in AS-OCT software versus the improved scheme. The improved scheme enables measurements of lens thickness (d(3)) and surface curvature, in the form of conic sections specified by vertex radii and conic constants. These parameters are converted to surface coordinates for error analysis. The built-in AS-OCT software typically overestimates [mean +/- standard deviation (SD)]d(1) by + 62 +/- 4 mu m and d(2) by + 4 +/- 88 mu m. The improved scheme reduces d(1) (-0.4 +/- 4 mu m) and d(2) (0 +/- 49 mu m) errors while also reducing d(3) errors from + 218 +/- 90 (uncorrected) to + 14 +/- 123 mu m (corrected). Surface x coordinate errors gradually increase toward the periphery. Considering the central 6-mm zone of each surface, the x coordinate errors for anterior and posterior corneal surfaces reached + 3 +/- 10 and 0 +/- 23 mu m, respectively, with the improved scheme. Those of the anterior and posterior lens surfaces reached + 2 +/- 22 and + 11 +/- 71 mu m, respectively. Our improved scheme reduced AS-OCT errors and could, therefore, enhance pre- and postoperative assessments of kera-torefractive or cataract surgery, including measurement of accommodating intraocular lenses. (C) 2007 Society of Photo-Optical Instrumentation Engineers.