CORNEAL TOPOGRAPHY IN EHLERS-DANLOS-SYNDROME

Purpose: To assess the use of corneal topography in conjunction with s litlamp biomicroscopy and retinoscopy to diagnose keratoconus in a lar ge group of patients with Ehlers-Danlos syndrome (EDS). Setting: Kresg e Eye Institute, Wayne State University, Detroit, Michigan. USA.Method s: Thirty-six patients (72 eyes) with genetically typed EDS had slitla mp biomicroscopy, retinoscopy, and videokeratography with the EyeSys i nstrument. The presence or absence of slitlamp keratoconus findings wa s correlated to a presumptive diagnosis based on corneal topography us ing derived topographic indexes associated with keratoconus. These top ographic indexes included central corneal power, (CCP), difference in CCP, inferosuperior asymmetry (I-S) value, and asphericity (Q). Axial and profile difference maps were generated and analyzed for findings s uggestive of keratoconus. Results: In 72 eyes, no keratoconus was foun d using slitlamp biomicroscopy. No eye had an I-S value greater than 1 .60 diopters (D), 2 eyes had a CCP greater than 46.50 D, and 2 eyes ha d a Q value less than -1.00. Eight of 36 pairs of eyes had fan interey e CCP greater than 0.92 D. In both eyes of the patient with Q values l ess than -1.00, the profile difference maps were mildly abnormal. Conc lusions: Slitlamp biomicroscopy of the cornea was unremarkable in all patients. Only 1 patient had Q values and profile difference maps that were mildly suggestive of keratoconus. Even after adding topography t o the examination, it appears that keratoconus in a known population o f patients with EDS remains rare.