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Nils Mungan, MD, FRCSC; Ken K. Nischal, FRCOphth; Elise Héon, MD, FRCSC; Leslie MacKeen, CRA; J. Williamson Balfe, MD, FRCPC; Alex V. Levin, MD, FRCSC

Arch Ophthalmol. 2000;118:1329-1333.

Objective  To describe the ocular ultrasound biomicroscopy (UBM) findings in patients with cystinosis.

Methods  Six patients with infantile nephropathic cystinosis, aged 16 to 25 years, and 6 controls (matched for age and spherical refractive error) were examined clinically and with UBM. Scleral reflectivity, corneal and iris thickness, central anterior chamber depth, angle width, trabecular meshwork to ciliary process distance, and ciliary sulcus width were measured.

Results  No patient had glaucoma or posterior synechiae, but all had crystals in the trabecular meshwork apparent with gonioscopy. Using UBM, the cornea and iris appeared similar in both groups, but the scleral reflectivity was increased in patients (P = .003). The angle was narrower in patients (mean ± SD, 20° ± 7°) than controls (31° ± 5°, P<.001). The anterior chamber was shallower in patients (2556 ± 197 µm) than controls (2968 ± 284 µm, P<.001). The ciliary sulcus was closed or narrow in all patients (83 ± 112 µm) compared with controls (339 ± 135 µm, P<.001), with a reduction in the trabecular meshwork to ciliary process distance.

Conclusions  This report of ocular UBM findings in cystinosis demonstrated narrowing of the angle and a ciliary body configuration similar to that reported for plateau iris syndrome. Gonioscopy demonstrated crystals in the trabecular meshwork. These findings may explain the predisposition of these patients to glaucoma.

From the Departments of Ophthalmology (Drs Mungan, Nischal, Héon, and Levin and Ms MacKeen) and Nephrology (Dr Balfe), The Hospital for Sick Children, University of Toronto, Toronto, Ontario. Dr Mungan is now with the University of Mississippi Medical Center, Jackson. Dr Nischal is now with the Great Ormond Street Hospital for Children and the Institute of Child Health, London, England.

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