ORIGINAL PAPER Progression of gyrate atrophy measured with ultra-wide- field imaging Guillermo Salcedo-Villanueva . Miguel Paciuc-Beja . Cristina Villanueva-Mendoza . Mariana Harasawa . Jesse M. Smith . Raul Velez-Montoya . Jeffrey L. Olson . Scott C. Oliver . Naresh Mandava . Hugo Quiroz-Mercado Received: 27 August 2014 / Accepted: 13 May 2015 / Published online: 26 May 2015 Ó Springer Science+Business Media Dordrecht 2015 Abstract The study aims to determine the progres- sion of gyrate atrophy by measuring the area growth of chorioretinal atrophic lesions using ultra-wide-field images (UWFI). A retrospective, observational, and comparative study was conducted and UWFI (200°) were obtained from two patients with gyrate atrophy at baseline and follow-up. Measurements of atrophy were obtained for three types of lesions: Solitary atrophic lesions (SAL), De novo solitary lesions (DNSL), and peripapillary atrophy (PPA). Compar- ison of baseline and follow-up was done using t tests. Two patients with gyrate atrophy were included. Patient 1 presented 16 SAL, 5 DNSL, and PPA measured for both eyes (BE). Overall area growth (OAG) for SAL (expressed in decimals) presented a mean of 3.41, r 3.07. DNSL area for BE presented a mean of 1586.08 P 2 , r 1069.55. OAG for PPA presented a mean of 1.21, r 0.17. Patient 2 presented 5 SAL, no DNSL, and PPA was measured for BE. OAG for SAL presented a mean of 1.58, r 1.05 (range 1.02–3.47). OAG for PPA presented a mean of 1.05, r 0.001. Gyrate atrophy progression can be determined by measuring the changes in area using UWFI. Keywords Choroid Á Gyrate atrophy Á Retina Á Retina pigment epithelium Á Retinal distrophies Introduction Gyrate atrophy of the choroid and retina (OMIM # 258870) is a rare dystrophy of the posterior segment clinically characterized by the triad of progressive chorioretinal degeneration, early cataract formation, and type II muscle fiber atrophy. Its incidence varies, being highest in Finland, with an estimated frequency of 1 in 50,000 individuals. It is a progressive condition associated with significantly increased plasma or- nithine levels. The condition is inherited as autosomal recessive [1, 2]. Patients develop circular areas of chorioretinal atrophy which are distributed around the peripheral fundus. As the disease progresses, lesions coalesce and spread posteriorly. The macula and consequently the central vision are often preserved into the 4th or 5th decade of life. Patients typically report night blindness, loss of peripheral vision, or both, from the second decade of life; myopia and early G. Salcedo-Villanueva (&) Á M. Paciuc-Beja Á M. Harasawa Á H. Quiroz-Mercado Department of Ophthalmology, Denver Health Medical Center, 777 Bannock St., Denver, CO 80204, USA e-mail: salcedovilla@gmail.com G. Salcedo-Villanueva Á M. Paciuc-Beja Á J. M. Smith Á R. Velez-Montoya Á J. L. Olson Á S. C. Oliver Á N. Mandava Á H. Quiroz-Mercado Rocky Mountains Lions Eye Institute, University of Colorado School of Medicine, 1675 Aurora Ct., Aurora, CO 80045, USA C. Villanueva-Mendoza Asociacio ´n Para Evitar la Ceguera en Me ´xico, Vicente Garcı ´a Torres # 46, 04030 Mexico, DF, Mexico 123 Int Ophthalmol (2016) 36:111–120 DOI 10.1007/s10792-015-0085-3