Azoor: Retinopatia externa oculta zonal aguda associada com doença autoimune / Azoor: Acute Zonal Occute Outer retinopathy associated with autoimmune disease

Resumo Retinopatia externa oculta zonal aguda (AZOOR) foi descrita pela primeira vez por Gass em 1993 como uma síndrome com perda rápida de uma ou mais zonas extensas dos segmentos externos da retina. Paciente masculino, 35 anos, portador de doença de Crohn, queixando-se de dor ocular eventual e nictalopia em olho direito desde infância. Em uso regular de azatioprina e mesalazina. melhor acuidade visual 20/20 AO. À fundoscopia, lesões hiperpigmentadas em arcada temporal inferior de olho direito, em treliça, acompanhando vasculatura local. Após exclusão de diagnósticos diferenciais chegou-se ao diagnóstico de Azoor. Azoor é uma síndrome idiopática caracterizada por um quadro agudo início de fotopsia, escotoma ou ambos e é tipicamente associado a uma perda persistente de função visual que envolve uma ou mais zonas da retina externa. Apesar dos sintomas clássicos de fotopsia, o paciente em questão teve uma apresentação clínica atípica. Descrevemos um caso ocorrido em indivíduo do sexo masculino em região periférica apresentando afinamento coroidiano e com doença autoimune associada. Dessa forma, acreditamos que é necessária maior investigação para verificar a etiologia da alteração coroideana e da associação com a doença específica.

Abstract Acute zonal occult external retinopathy (AZOOR) was first described by Gass in 1993 as a syndrome with rapid loss of one or more large areas of the external retinal segments. Male, 35 years, with Crohn's disease complaining of occasional eye pain and right eye nictalopia since childhood. In regular use of azathioprine and mesalazine. better visual acuity 20/20 OU. At fundoscopy, hyperpigmented lesions in the right temporal arcade of the right eye, in trellis, accompanying local vasculature. After exclusion of differential diagnoses, Azoor's diagnosis was reached. Azoor is an idiopathic syndrome characterized by an acute onset of photopsy, scotoma or both and is typically associated with a persistent loss of visual function involving one or more areas of the external retina. Despite classic photopsy symptoms, the patient in question had an atypical clinical presentation. We describe a case of a peripheral male with choroidal thinning and associated autoimmune disease. Thus, we believe that further investigation is necessary to verify the etiology of choroidal alteration and its association with the specific disease.

A Case Report of Acute Zonal Occult Outer Retinopathy

PURPOSE: We present a case of acute zonal occult outer retinopathy (AZOOR) that has not been reported in Korea. METHODS: A 26-year-old female with photopsia and visual field defect in the left eye was referred to our hospital. There were no fundus abnormalities to explain the visual field defect. Electroretinography, indocyanine green angiography, neurologic examination, cerebrospinal fluid tapping and brain magnetic resonance imaging were performed for further evaluation. RESULTS: Visual acuity was 20/20 in both eyes. Visual field examination revealed a peripheral scotoma in the right eye and marked constriction of visual field in the left eye. Electroretinography showed normal response in the right eye and no response in the left eye. There was an afferent pupillary defect in the left eye and vitreous cells in both eyes. Indocyanine green angiography, neurologic examination, cerebrospinal fluid tapping and brain magnetic resonance imaging showed no abnormal findings. CONCLUSIONS: The possibility of AZOOR should be considered in a patient with photopsia, visual field defect, acute loss of outer retinal function confirmed with electroretinography, and minimal or no fundus and angiographic changes, especially in a young woman.