Retinoschisis

Hirose, Tatsuo; Blodi, Bárbara A.

doi:10.1016/b978-1-4160-0016-7.50184-3

Cited by 2 publications

(5 citation statements)

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“…This is because most of these disorders share common features such as presentation in the early decades of life, night blindness, and mid-peripheral pigmentary changes. [82021222324252627] ESCS being a rare disorder, diagnosis of this entity is a challenge because of relative lack of awareness about this condition and non-availability of electrophysiological testing at all centers. Table 3 provides a useful guide in differentiation and diagnosis of these disorders.…”

Section: Discussionmentioning

confidence: 99%

Enhanced S-cone syndrome: Clinical spectrum in Indian population

Naik

Ratra

Banerjee

et al. 2019

Indian J Ophthalmol

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Purpose: Enhanced S-cone syndrome (ESCS), a rare disorder, is often misdiagnosed as other forms of retinal degenerations, which have a poorer prognosis than ESCS. The aim of this study is to report the varied clinical features of ESCS and distinguish it from other similar disorders. Methods: We retrospectively scrutinized the records of patients with confirmed diagnosis of ESCS and analyzed the findings. Results: We included 14 patients (age range 4–39 years) who were confirmed to have ESCS according to pathognomonic electroretinography (ERG) showing reduced photopic, combined responses, and 30 Hz flicker with reduced L, M cone responses and supernormal S cone responses. The disease presented in the 1 st decade with night blindness and was almost stationary or minimally progressive. Mid-peripheral fundus changes in form of nummular pigmentary alterations, yellow punctate lesions, and macular schisis were noted. The vision ranged from 6/6 to 6/36 with follow-up ranging from 1month to 22 years. Conclusion: ESCS shows varied clinical features ranging from unremarkable fundus to pigment clumping and atrophic lesions. It has good prognosis with patients mostly maintaining their vision. ERG is diagnostic. More awareness and knowledge about this entity can help to differentiate it from other forms of night blindness.

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Section: Discussionmentioning

confidence: 99%

Enhanced S-cone syndrome: Clinical spectrum in Indian population

Naik

Ratra

Banerjee

et al. 2019

Indian J Ophthalmol

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“…Novas rupturas, especialmente as externas, são ligadas à possibildade de descolamento localizado de retina. Este costuma ser não progressivo, esporadicamente infiltrando além de seus limites ou chegando a mácula (1 a cada 178 casos torna-se evolutivo) (25,26,(28)(29)(30)32,37) .…”

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“…Quase 60% dos examinados exibem DVP, presumindo-se que esta separação possa contribuir na interrupção evolutiva. O desaparecimento espontâneo em 8% (quase similar a possibilidade de progressão) (27,28,30,33,38) . As séries de Hirose (26) (245 olhos seguidos por 15 anos) e a de Byer (32) (218 olhos, 9 anos) mostraram que as principais complicações em longos seguimentos são:…”

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“…Na RQ o branqueamento resultante entre as duas é similar; no descolamento são muito diferentes (39) . O movimento de toda a lesão "como uma unidade" na depressão sob a oftalmoscopia binocular indireta (OBI) é típico na RQ (quando os planos se aproximam pela saída do líquido para o vítreo comumente tem-se um DR) (27,28,30) . Esta manobra pode ser aplicada também durante a ecografia, auxiliando a discernir (40,41) .…”

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“…As áreas perirrupturas costumam hiperfluorescer devido ao comprometimento atrófico do epitélio pigmentado retiniano (EPR) (29) . Os defeitos do CV são percebidos apenas nas RQ posteriores ao equador, e não desaparecem mesmo após colapso do cisto (30) (nesta demanda a campimetria manual oferece vantagens). Recentemente a tomografia de coerência óptica (OCT) reforçou as opções para esta distinção.…”

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