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Vol. 97. Issue 1.
Pages 48-51 (January 2022)
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Vol. 97. Issue 1.
Pages 48-51 (January 2022)
Short communication
Congenital hypertrophy of the retinal pigment epithelium associated with acquired retinoschisis and microcystic degeneration: The importance of multimodal imaging
Hipertrofia congénita del epitelio pigmentario de la retina asociada a retinosquisis adquirida y degeneración microquística: La importancia del estudio con imagen multimodal
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Jorge Fernández-Engrobaa,b, Rafael Olleroa,b, Anna Soldevilaa,b,
Corresponding author
anna.soldevila@barraquer.com

Corresponding author.
a Centro de Oftalmología Barraquer, Barcelona, Spain
b Institut Universitari Barraquer, Universitat Autònoma de Barcelona, Barcelona, Spain
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Abstract

We present an illustrated clinical case with multimodal representation of a congenital hypertrophy of the depigmented retinal pigment epithelium (CHRPE) associated with acquired retinoschisis and microcystic degeneration (MD) in a 64-year-old hypermetropic, asymptomatic woman. Retinography showed a whitish lesion with pigmented edges, slightly raised, accompanied by a lower area with a microcystic pattern. The complementary tests carried out allowed to determine a well-defined diagnosis of exclusion. B-mode ultrasound showed a smooth, cupuliform and cystic elevation characteristic of acquired retinoschisis, and fluorescein angiography could rule out the presence of a vascular tumor. The hypoautofluorescence of the lesion determined the depigmentation of the CHRPE, while the slight perilesional hyperautofluorescence corresponded to the associated retinoschisis and DM. This is the first case described in the literature of an acquired retinoschisis associated with CHRPE.

Keywords:
Congenital hypertrophy of retinal pigment epithelium
Acquired retinoschisis
Microcystic degeneration
Resumen

Presentamos un caso clínico ilustrado con representación multimodal de una hipertrofia congénita del epitelio pigmentario de la retina (HCEPR) despigmentada asociada con una retinosquisis adquirida y a una degeneración microquística (DM) en una mujer hipermétrope de 64 años, asintomática. En la retinografía se evidenciaba una lesión blanquecina con bordes pigmentados, levemente sobreelevada, acompañada de un área inferior con patrón microquístico. Las pruebas complementarias realizadas permitieron determinar un diagnóstico de exclusión bien definido. La ecografía en modo B mostraba una elevación lisa, cupuliforme y quística característica de la retinosquisis adquirida, pudiendo descartar mediante la angiografía fluoresceínica la presencia de un tumor vascular. La hipoautofluorescencia de la lesión determinaba la despigmentación de la HCEPR, mientras que la tenue hiperautofluorescencia perilesional se correspondía con la retinosquisis y la DM asociadas. Se trata del primer caso descrito en la literatura de una retinosquisis adquirida asociada con una HCEPR.

Palabras clave:
Hipertrofia congénita del epitelio pigmentario de la retina
Retinosquisis adquirida
Degeneración microquística

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