From A… to… Z(OOR): The Clinical Spectrum of Acute Zonal Occult Outer Retinopathy

Margaret Wang; Ama Sadaka; Thomas Prager; Andrew G Lee; Francesco Pellegrini; Daniele Cirone; Luca De Simone; Luca Cimino

doi:10.1080/01658107.2017.1400076

From A… to… Z(OOR): The Clinical Spectrum of Acute Zonal Occult Outer Retinopathy

Neuroophthalmology. 2017 Dec 21;42(4):215-221. doi: 10.1080/01658107.2017.1400076. eCollection 2018 Aug.

Authors

Margaret Wang¹, Ama Sadaka², Thomas Prager¹, Andrew G Lee^{1

2

3

4

5}, Francesco Pellegrini⁶, Daniele Cirone⁷, Luca De Simone^{8

9}, Luca Cimino⁹

Affiliations

¹ Blanton Eye Institute, Department of Ophthalmology, The Methodist Hospital, Houston, Texas, USA.
² Department of Ophthalmology, Department of Ophthalmology, Baylor College of Medicine, Houston, Texas, USA.
³ Departments of Ophthalmology, Neurology, and Neurosurgery, Weill Cornell Medical College, Houston, Texas, USA.
⁴ Department of Ophthalmology, The University of Texas Medical Branch, Galveston, Texas, USA.
⁵ Department of Ophthalmology, The University of Iowa Hospitals and Clinics, Iowa City, Iowa, USA.
⁶ Neuro-Ophthalmology Service, Department of Ophthalmology, De Gironcoli Hospital, Conegliano, TV, Italy.
⁷ Uveitis Service, Department of Ophthalmology, Murri Hospital, Fermo, FM, Italy.
⁸ Department of Ophthalmology, University Campus Bio-Medico of Rome, Rome, RM, Italy.
⁹ Immunology Eye Unit, Azienda USL IRCCS, Reggio Emilia, RE, Italy.

Abstract

Acute idiopathic blind spot enlargement (AIBSE) is often caused by Acute Zonal Occult Outer Retinopathy (AZOOR), an outer retinal disease. We report two illustrative cases of AZOOR. The first one was a 21-year-old white female who presented with a scotoma and "shimmering lights" in her left eye. In the second case, a 73-year-old white female was referred for evaluation of a "bitemporal hemianopsia" that started years prior, with no clinical significant photopsias. To our knowledge, case two is the longest documented duration of bilateral, progressive, and chronic, idiopathic, enlargement of the blind spot (CIBSE) documented in the English language ophthalmic literature.

Keywords: Acute zonal occult outer retinopathy; autofluorescence; ellipsoid zone; enlarged blind spot.