ESCRS - PO0141 - Unusual Association Of Axenfeld- Rieger Syndrome With Keratoconus

Unusual Association Of Axenfeld- Rieger Syndrome With Keratoconus

Published 2023 - 41st Congress of the ESCRS

Reference: PO0141 | Type: Case report | DOI: 10.82333/apdm-7h90

Authors: Georgiana Camburu* 1 , Rossella Spena 2 , Davide Demaria 2 , Marco Pellegrini 2 , Angeli Christy Yu 2 , Massimo Busin 2

1Department of Ophthalmology,Ospedali Privati Forlì, “Villa Igea”,Forlì,Italy, 2Department of Ophthalmology,Ospedali Privati Forlì, “Villa Igea”,Forlì,Italy;Department of Translational Medicine,University of Ferrara,Ferrara,Italy

 

To present an unusual case of Axenfeld-Rieger syndrome associated with keratoconus which underwent mushroom penetrating keratoplasty (PK).

Ospedali Privati Forlì, "Villa Igea", Forlì, Italy

A 25-year-old Caucasian male presented with progressive blurring of vision in the left eye. Snellen visual acuity was 20/80. Slit-lamp examination revealed posterior embryotoxon, iris stromal hypoplasia, iridocorneal adhesions, and corectopia in both eyes. IOP was 16 mmHg in both eyes. Gonioscopy revealed prominent Schwalbe's line. Fundus examination was unremarkable. Anterior segment OCT demonstrated inferonasal steepening corresponding to localized stromal thinning and posterior corneal elevation. Kmax was 48.1 D and topographic astigmatism was 2.7 D. Mushroom PK was performed.

This case demonstrates an unusual association of an anterior segment dysgenesis with keratoconus. Anterior segment OCT serves as an effective tool for the evaluation of Axenfeld-Rieger syndrome.