Acute Zonal Occult Outer Retinopathy (AZOOR) and Related Diseases

  • Stephen H. Tsang
  • Tarun SharmaEmail author
Part of the Advances in Experimental Medicine and Biology book series (AEMB, volume 1085)


  • Acute zonal occult outer retinopathy (AZOOR) is a presumed inflammatory disorder with outer retinal dysfunction.

  • Typically, the onset is acute and it is unilateral, with symptoms of photopsias and nasal field loss; scotoma is usually contiguous with the optic nerve. Later, the other eye is involved in nearly three fourths of patients. The central vision remains good in most cases.

  • Patients are usually young women with myopia.

  • Fundus: May be normal in the beginning, but may show a grayish-white line at the border of normal and involved retina, usually in peripapillary area. This line disappears within weeks and is replaced with an orange zone. With time, retinal vessels attenuate and a large zone of retinal pigment epithelium (RPE) depigmentation appears, sort of a sector retinitis pigmentosa (RP) or unilateral or asymmetric RP.

  • Rarely, mild vitritis may occur, and relative afferent pupillary defect (RAPD) is present in about 75% of cases.


Phenocopies Acute zonal occult outer retinopathy AZOOR 

Suggested Reading

  1. Jung JJ, Khan S, Mrejen S, Gallego-Pinazo R, Cunningham ET Jr, Freund KB, et al. Idiopathic multifocal choroiditis with outer retinal or chorioretinal atrophy. Retina. 2014;34:1439–50.CrossRefGoogle Scholar
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  3. Sarraf D, Rahimy E, Fawzi AA, Sohn E, Barbazetto I, Zacks DN, et al. Paracentral acute middle maculopathy: a new variant of acute macular neuroretinopathy associated with retinal capillary ischemia. JAMA Ophthalmol. 2013;131:1275–87.CrossRefGoogle Scholar
  4. Yannuzzi LA, Jampol LM, Rabb MF, Sorenson JA, Beyrer C, Wilcox LM Jr. Unilateral acute idiopathic maculopathy. Arch Ophthalmol. 1991;109:1411–6. Erratum in: Arch Ophthalmol. 1992;110:449CrossRefGoogle Scholar

Copyright information

© Springer International Publishing AG, part of Springer Nature 2018

Authors and Affiliations

  1. 1.Jonas Children’s Vision Care, Bernard & Shirlee Brown Glaucoma Laboratory, Columbia Stem Cell Initiative-Departments of Ophthalmology, Biomedical Engineering, Pathology & Cell Biology, Institute of Human Nutrition, Vagelos College of Physicians and Surgeons, Columbia UniversityNew YorkUSA
  2. 2.Department of Ophthalmology, Columbia UniversityEdward S. Harkness Eye Institute, NewYork-Presbyterian HospitalNew YorkUSA

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