Atlas of Ophthalmology

Acute Zonal Occult Outer Retinopathy (AZOOR) (Colour Image, FFA, OCT, ERG, VF)

Retina -> Distrofias y Degeneraciones Hereditarias -> Epitelio Pigmentario de la Retina (EPR)
28 years of age, female; since 6 weeks photopsia with unaffected central vision; visual field defect at left eye in the superior temporal quadrant; intermitted headache. VA OD cc 0,9 , OS cc 0,9; IOP RA 14 mmHg, LA 14 mmHg. Acute zonal occult outer retinopathy (AZOOR) is a rare unilateral or bilateral disease of unknown etiology. AZOOR is characterized by focal degeneration of photoreceptors. Acute zonal occult outer retinopathy is characterized by the rapid loss of outer retinal function, usually associated with photopsia. Central vision is often unaffected. Changes on the ERG and visual field defects are present in virtually all patients In this group of predominantly white individuals, average age at presentation is about 35 years, and the male:female ratio is 1:3. The majority of patients complained of the acute onset of a scotoma, which is often associated with photopsia. Visual acuity is 20/40 or better in the majority of eyes. Fundus examination is often unremarkable. Often blind spot enlargement, with or without other field defects, were observed. Mainly in all patients electroretinographic abnormalities were recorded. AZOOR belongs to a spectrum that included multiple evanescent white dot syndrome (MEWDS), acute idiopathic blind spot enlargement (AIBSE) syndrome, acute macular neuroretinopathy (AMN), presumed ocular histoplasmosis (POHS), punctate inner choroidopathy (PIC), and multifocal choroiditis and panuveitis (MFC). Various treatments have been attempted in patients with AZOOR--including systemic corticosteroids, other systemic immunosuppressive agents, and different antimicrobials--but none have been proven effective. Literature: Dinelli M. Monson, Justine R. Smith. Acute Zonal Occult Outer Retinopathy. Survey of Ophthalmology.Volume 56, Issue 1, January–February 2011, Pages 23–35.

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