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Ultrasound Biomicroscopy in Asymmetric Pigment Dispersion Syndrome and Pigmentary Glaucoma

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Ultrasound Biomicroscopy in Asymmetric Pigment Dispersion Syndrome and Pigmentary Glaucoma CLINICAL SCIENCES Ultrasound Biomicroscopy in Asymmetric Pigment Dispersion Syndrome and Pigmentary Glaucoma Fabio N. Kanadani, MD; Syril Dorairaj, MD; Alan M. Langlieb, MD, MPH, MBA; Wisam A. Shihadeh, MD; Celso Tello, MD; Jeffrey M. Liebmann, MD; Robert Ritch, MD Objective: To identify differences in anterior chamber Results: There were no differences in lens thickness anatomy among patients with asymmetric pigment dis- (P=.33), refractive error (P=.84), or axial length (P=.99) persion syndrome and no other discernible cause for the between more and less affected eyes. However, the asymmetry. mean ± SD iris concavity (PϽ.001), iris-lens contact dis- tance (P=.02), and distance from the scleral spur to the Methods: Ultrasound biomicroscopy and A-scan iris insertion (0.42±0.11 vs 0.29±0.06 mm) (P=.002) biometry were performed on both eyes of 13 patients were greater in the more affected eye of each patient. with asymmetric pigment dispersion syndrome with- out a known cause for asymmetric involvement. A Conclusion: A more posterior iris insertion predis- radial perpendicular image in the horizontal temporal poses to the phenotypic expression of pigment disper- meridian detailing the scleral spur, angle anatomy, and sion syndrome. iris configuration was obtained for each eye by 2 examiners. Arch Ophthalmol. 2006;124:1573-1576 N PIGMENT DISPERSION SYNDROME Phenotypic expression is typically bi- (PDS), friction between the poste- lateral and symmetric. A marked asym- rior iris surface and the anterior zo- metric involvement is unusual, and a cause nular bundles causes the disinte- should be sought when present. Asym- gration of iris pigment epithelial metry may occur because a second con- cells and the release of pigment granules, dition, such as cataract formation or ex- Author Affiliations: I 10 11 which are then dispersed by aqueous cur- traction, Horner syndrome, or Adie Department of Ophthalmology, 1,2 12 The New York Eye and Ear rents. The liberated pigment is depos- pupil, limits the involvement in 1 eye or Infirmary (Drs Kanadani, ited throughout the anterior segment. The because the occurrence or development of Dorairaj, Shihadeh, Tello, classic diagnostic triad consists of a Kruken- exfoliation syndrome13 or angle reces- Liebmann, and Ritch), and berg spindle, slitlike radial midperipheral iris sion led to higher intraocular pressure and Glaucoma Service, Manhattan transillumination defects, and increased pig- more frequent glaucoma in the doubly in- Eye, Ear & Throat Hospital, mentation of the trabecular meshwork. The volved eye.14 In some patients, no reason Department of Ophthalmology, angle is typically wide open, the iris is in- for asymmetry can be discovered even af- New York University School of serted posteriorly, and the configuration of ter a thorough clinical examination. In this Medicine (Dr Liebmann), New the peripheral iris is posteriorly concave. El- article, we describe 13 such patients in York, and Department of Ophthalmology, New York evated intraocular pressure develops in whom a more posterior iris insertion in the Medical College, Valhalla many patients and may lead to glaucoma- more involved eye was detected by ultra- (Dr Ritch); and Department of tous damage (pigmentary glaucoma). sound biomicroscopy (UBM). Ophthalmology, Wilmer Eye Pigment dispersion syndrome is an au- Institute, The Johns Hopkins tosomal dominant disorder.3 Myopia pre- METHODS University School of Medicine, disposes to its phenotypic expression and Baltimore, Md (Dr Langlieb). is present in approximately 80% of af- Dr Kanadani is now with the fected patients.4-6 The most significant risk High-frequency high-resolution anterior Department of Ophthalmology, factors for the development of the pheno- segment UBM (UBM P-40; Paradigm Medical Santa Casa Hospital, University typic expression of PDS are young age, male Industries, Salt Lake City, Utah) and A-scan of Minas Gerais, Belo biometry (A/B scan; Sonomed Inc, Lake Suc- sex, myopia, white race/ethnicity, and a posi- Horizonte, Brazil. Dr Shihadeh 1 cess, NY) were performed before pharmaco- is now with the Faculty of tive family history. Although men and logic pupillary manipulation in both eyes of all Medicine, Jordan University women are equally affected, men are more patients with asymmetric PDS without dis- of Science & Technology, likely to develop glaucoma, in approxi- cernible cause of the asymmetry. A sagittal im- Irbid, Jordan. mately a 3:1 ratio.7-9 age in the horizontal temporal meridian in both (REPRINTED) ARCH OPHTHALMOL / VOL 124, NOV 2006 WWW.ARCHOPHTHALMOL.COM 1573 ©2006 American Medical Association. All rights reserved. Downloaded From: https://jamanetwork.com/ on 09/30/2021 C AC I CP LC Iris Configuration “0” Figure 1. Ultrasound biomicroscopy appearance of the anterior chamber of the more affected eye of patient 4 in Table 1. AC indicates anterior chamber; C, cornea; CP, ciliary processes; I, iris; LC, lens capsule; white arrow, iris root; black arrow, scleral spur. Figure 3. Less affected eye of patient 5 in Table 1. The dashed black line corresponds to the straight line connecting the posterior surface of the iris root to the pupillary margin. It is positive if convex, and it is negative if concave. sured included the linear extent of iris-lens contact distance (ILCD), iris configuration (Figure 2 and Figure 3), and dis- tance from the scleral spur to the iris insertion. The iris configu- ration was measured by drawing a line connecting the most pe- ripheral and most central points of the iris pigment epithelium (reference line) and by measuring the largest perpendicular dis- tance from the line to the iris pigment epithelium. A concave or convex surface was determined to exist when there was a mea- surable difference between the plane of the iris pigment epithe- lium and the reference line. Negative values were assigned to con- cave irides, and positive values were assigned to convex irides. A planar iris received a 0 value. Measurements of lens thickness and axial length were obtained using A-scan biometry. Statistical evaluation was performed using t test when evalu- ating differences between more and less affected eyes. The soft- ware program JMP 4.0 was used (SAS Institute Inc, Cary, NC). RESULTS Figure 2. More affected eye of patient 5 in Table 1. The dashed black line Eleven men and 2 women were included in the study. corresponds to the straight line connecting the posterior surface of the iris root to the pupillary margin. The iris configuration is estimated by drawing a The mean ± SD patient age was 44.9±7.3 years. Only 1 perpendicular line (bar) at the peak of the iris concavity to the dashed line. patient did not have higher intraocular pressure in the more affected eye, and 9 of 13 patients had glaucoma- eyes detailing the Schwalbe line, scleral spur, and iris root in- tous optic neuropathy in at least 1 eye. sertion was obtained for each eye by 2 masked examiners (S.D. Patients’ intraocular pressure and cup-disc ratio are given and C.T.) under standardized room lighting conditions. Mea- in Table 1. All patients had 20/20 visual acuity. The surements were made using the software UBM Pro2000 Para- mean ± SD horizontal corneal diameters were 12.7±0.3 and digm Medical Industries). Corneal diameter was measured us- 12.6±0.5 mm, lens thicknesses were 4.24±0.45 and ing a handheld caliper. 4.01±0.39 mm, and axial lengths were 24.7±0.7 and The following 3 landmarks were used as reference points 24.7±0.8 mm in more and less affected eyes, respectively for UBM measurements (Figure 1 and Figure 2): (1) the (PϾ.05 for all). Schwalbe line (the termination of Descemet membrane, which The UBM measurements are given in Table 2. There was appears as a hyperreflective line in the posterior aspect of the a more concave iris configuration (−0.28 vs 0.08 mm), in- cornea), (2) the scleral spur (a hyperlucent wedge at the an- terior edge of a line separating corneal tissue and ciliary muscle creasediridolenticularcontact(1.44vs0.91mm),andgreater fibers [Figure 1]), and (3) the iris root insertion (the anterior- distance from the scleral spur to the iris insertion (0.42 vs most insertion of the iris into the ciliary body). 0.29 mm) in the eyes with more severe PDS. There was no The UBM image measurements were made by a masked ob- difference in lens thickness or axial length to account for server (S.D. or C.T.) using a random image order. Variables mea- the asymmetry of the anterior segment appearance. (REPRINTED) ARCH OPHTHALMOL / VOL 124, NOV 2006 WWW.ARCHOPHTHALMOL.COM 1574 ©2006 American Medical Association. All rights reserved. Downloaded From: https://jamanetwork.com/ on 09/30/2021 Table 1. Patients’ General Features Intraocular Pressure, mm Hg Cup-Disc Ratio Patient No. Age, y More Affected Eye Less Affected Eye More Affected Eye Less Affected Eye 1 27 27 18 0.6 0.4 2 46 15 14 0.1 0.25 3 45 33 19 0.9 0.3 4 42 37 14 0.5 0.4 5 46 18 20 0.5 0.6 6 46 17 17 0.5 0.5 7 47 26 20 0.3 0.3 8 47 16 16 0.85 0.4 9 53 14 14 0.6 0.5 10 40 20 20 0.75 0.75 11 36 28 20 0.9 0.5 12 55 13 13 0.8 0.6 13 61 14 14 0.7 0.3 Table 2. Ultrasound Biomicroscopy Measurements Scleral Spur–Iris Root Distance* Iris Configuration† Iris-Lens Contact Distance‡ Variable More Affected Eye Less Affected Eye More Affected Eye Less Affected Eye More Affected Eye Less Affected Eye Mean, mm 0.42 0.29 −0.28 0.08 1.44 0.91 SD 0.11 0.06 0.15 0.25 0.43 0.57 *P = .002.
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