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array:24 [ "pii" => "S217357942300018X" "issn" => "21735794" "doi" => "10.1016/j.oftale.2022.12.003" "estado" => "S300" "fechaPublicacion" => "2023-04-01" "aid" => "2073" "copyright" => "Sociedad Española de Oftalmología" "copyrightAnyo" => "2023" "documento" => "simple-article" "crossmark" => 1 "subdocumento" => "crp" "cita" => "Arch Soc Esp Oftalmol. 2023;98:233-7" "abierto" => array:3 [ "ES" => false "ES2" => false "LATM" => false ] "gratuito" => false "lecturas" => array:1 [ "total" => 0 ] "Traduccion" => array:1 [ "es" => array:19 [ "pii" => "S0365669123000011" "issn" => "03656691" "doi" => "10.1016/j.oftal.2022.12.006" "estado" => "S300" "fechaPublicacion" => "2023-04-01" "aid" => "2073" "copyright" => "Sociedad Española de Oftalmología" "documento" => "simple-article" "crossmark" => 1 "subdocumento" => "crp" "cita" => "Arch Soc Esp Oftalmol. 2023;98:233-7" "abierto" => array:3 [ "ES" => false "ES2" => false "LATM" => false ] "gratuito" => false "lecturas" => array:1 [ "total" => 0 ] "es" => array:13 [ "idiomaDefecto" => true "cabecera" => "<span class="elsevierStyleTextfn">Comunicación corta</span>" "titulo" => "Atrofia coriorretiniana pigmentada paravenosa de presentación unifocal y unilateral" "tienePdf" => "es" "tieneTextoCompleto" => "es" "tieneResumen" => array:2 [ 0 => "es" 1 => "en" ] "paginas" => array:1 [ 0 => array:2 [ "paginaInicial" => "233" "paginaFinal" => "237" ] ] "titulosAlternativos" => array:1 [ "en" => array:1 [ "titulo" => "Unifocal and unilateral pigmented paravenous retinochoroidal atrophy" ] ] "contieneResumen" => array:2 [ "es" => true "en" => true ] "contieneTextoCompleto" => array:1 [ "es" => true ] "contienePdf" => array:1 [ "es" => true ] "resumenGrafico" => array:2 [ "original" => 0 "multimedia" => array:7 [ "identificador" => "fig0010" "etiqueta" => "Figura 2" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr2.jpeg" "Alto" => 4082 "Ancho" => 2925 "Tamanyo" => 821386 ] ] "descripcion" => array:1 [ "es" => "<p id="spar0040" class="elsevierStyleSimplePara elsevierViewall">Campimetría computerizada 24-2 (A y B). En ojo izquierdo (a pesar de la baja fiabilidad) se aprecia una disminución generalizada de la sensibilidad con un defecto casi completo en hemicampo superior congruente con la lesión retiniana (A). En ojo derecho aparecen defectos parcheados en región superior e inferior nasal debido a artefactos por mala colaboración (B). En la angiografía de ojo izquierdo (C), se observa una hiperfluorescencia compatible con degeneración del epitelio pigmentario de la retina y bloqueo de la fluorescencia en las áreas de acumulación de pigmento a lo largo de los vasos retinianos inferiores. En la OCT macular (D y E) se observa un agujero lamelar (D) y una disminución del grosor de la capa de células ganglionares, sobre todo en sector inferior (E).</p>" ] ] ] "autores" => array:1 [ 0 => array:2 [ "autoresLista" => "J.C. Castaño Silos, I. Sanchez-Guillen, I. Almorín-Fernández-Vigo, M. Jerez Fidalgo, J.I. Fernández-Vigo" "autores" => array:5 [ 0 => array:2 [ "nombre" => "J.C." "apellidos" => "Castaño Silos" ] 1 => array:2 [ "nombre" => "I." "apellidos" => "Sanchez-Guillen" ] 2 => array:2 [ "nombre" => "I." "apellidos" => "Almorín-Fernández-Vigo" ] 3 => array:2 [ "nombre" => "M." "apellidos" => "Jerez Fidalgo" ] 4 => array:2 [ "nombre" => "J.I." "apellidos" => "Fernández-Vigo" ] ] ] ] ] "idiomaDefecto" => "es" "Traduccion" => array:1 [ "en" => array:9 [ "pii" => "S217357942300018X" "doi" => "10.1016/j.oftale.2022.12.003" "estado" => "S300" "subdocumento" => "" "abierto" => array:3 [ "ES" => false "ES2" => false "LATM" => false ] "gratuito" => false "lecturas" => array:1 [ "total" => 0 ] "idiomaDefecto" => "en" "EPUB" => "https://multimedia.elsevier.es/PublicationsMultimediaV1/item/epub/S217357942300018X?idApp=UINPBA00004N" ] ] "EPUB" => "https://multimedia.elsevier.es/PublicationsMultimediaV1/item/epub/S0365669123000011?idApp=UINPBA00004N" "url" => "/03656691/0000009800000004/v2_202304071613/S0365669123000011/v2_202304071613/es/main.assets" ] ] "itemSiguiente" => array:19 [ "pii" => "S2173579423000324" "issn" => "21735794" "doi" => "10.1016/j.oftale.2023.01.003" "estado" => "S300" "fechaPublicacion" => "2023-04-01" "aid" => "2081" "copyright" => "Sociedad Española de Oftalmología" "documento" => "simple-article" "crossmark" => 1 "subdocumento" => "crp" "cita" => "Arch Soc Esp Oftalmol. 2023;98:238-42" "abierto" => array:3 [ "ES" => false "ES2" => false "LATM" => false ] "gratuito" => false "lecturas" => array:1 [ "total" => 0 ] "en" => array:13 [ "idiomaDefecto" => true "cabecera" => "<span class="elsevierStyleTextfn">Short communication</span>" "titulo" => "The effect of prolonged and nocturnal use of contact lenses on the cornea: about a case of corneal decompensation and decreased endothelial count" "tienePdf" => "en" "tieneTextoCompleto" => "en" "tieneResumen" => array:2 [ 0 => "en" 1 => "es" ] "paginas" => array:1 [ 0 => array:2 [ "paginaInicial" => "238" "paginaFinal" => "242" ] ] "titulosAlternativos" => array:1 [ "es" => array:1 [ "titulo" => "El efecto del uso prolongado y nocturno de las lentes de contacto en la córnea. A propósito de un caso de descompensación corneal y descenso del recuento endotelial" ] ] "contieneResumen" => array:2 [ "en" => true "es" => true ] "contieneTextoCompleto" => array:1 [ "en" => true ] "contienePdf" => array:1 [ "en" => true ] "resumenGrafico" => array:2 [ "original" => 0 "multimedia" => array:8 [ "identificador" => "fig0015" "etiqueta" => "Fig. 3" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr3.jpeg" "Alto" => 3374 "Ancho" => 3341 "Tamanyo" => 1150223 ] ] "detalles" => array:1 [ 0 => array:3 [ "identificador" => "at0015" "detalle" => "Fig. " "rol" => "short" ] ] "descripcion" => array:1 [ "en" => "<p id="spar0015" class="elsevierStyleSimplePara elsevierViewall">Endothelial count, corneal pachymetry and central corneal thickness (CCT or central) post-treatment. RE left image and LE right image. The CCT has decreased compared to the first visit: RE 567 μm and LE 560 μm. The endothelial count is 1,278 cells/mm<span class="elsevierStyleSup">2</span> in the RE and 1,073 cells/mm<span class="elsevierStyleSup">2</span> in the LE.</p>" ] ] ] "autores" => array:1 [ 0 => array:2 [ "autoresLista" => "G. Castilla Martinez, C.P. Tarazona Jaimes, C.E. Monera Lucas" "autores" => array:3 [ 0 => array:2 [ "nombre" => "G." "apellidos" => "Castilla Martinez" ] 1 => array:2 [ "nombre" => "C.P." "apellidos" => "Tarazona Jaimes" ] 2 => array:2 [ "nombre" => "C.E." "apellidos" => "Monera Lucas" ] ] ] ] ] "idiomaDefecto" => "en" "Traduccion" => array:1 [ "es" => array:9 [ "pii" => "S0365669123000230" "doi" => "10.1016/j.oftal.2023.01.006" "estado" => "S300" "subdocumento" => "" "abierto" => array:3 [ "ES" => false "ES2" => false "LATM" => false ] "gratuito" => false "lecturas" => array:1 [ "total" => 0 ] "idiomaDefecto" => "es" "EPUB" => "https://multimedia.elsevier.es/PublicationsMultimediaV1/item/epub/S0365669123000230?idApp=UINPBA00004N" ] ] "EPUB" => "https://multimedia.elsevier.es/PublicationsMultimediaV1/item/epub/S2173579423000324?idApp=UINPBA00004N" "url" => "/21735794/0000009800000004/v2_202304071550/S2173579423000324/v2_202304071550/en/main.assets" ] "itemAnterior" => array:19 [ "pii" => "S2173579423000336" "issn" => "21735794" "doi" => "10.1016/j.oftale.2023.03.007" "estado" => "S300" "fechaPublicacion" => "2023-04-01" "aid" => "2083" "copyright" => "Sociedad Española de Oftalmología" "documento" => "article" "crossmark" => 1 "subdocumento" => "rev" "cita" => "Arch Soc Esp Oftalmol. 2023;98:220-32" "abierto" => array:3 [ "ES" => false "ES2" => false "LATM" => false ] "gratuito" => false "lecturas" => array:1 [ "total" => 0 ] "en" => array:13 [ "idiomaDefecto" => true "cabecera" => "<span class="elsevierStyleTextfn">Review</span>" "titulo" => "Effectiveness of topical insulin for the treatment of surface corneal pathologies" "tienePdf" => "en" "tieneTextoCompleto" => "en" "tieneResumen" => array:2 [ 0 => "en" 1 => "es" ] "paginas" => array:1 [ 0 => array:2 [ "paginaInicial" => "220" "paginaFinal" => "232" ] ] "titulosAlternativos" => array:1 [ "es" => array:1 [ "titulo" => "Efectividad de la insulina tópica en el tratamiento de patologías de la superficie corneal" ] ] "contieneResumen" => array:2 [ "en" => true "es" => true ] "contieneTextoCompleto" => array:1 [ "en" => true ] "contienePdf" => array:1 [ "en" => true ] "resumenGrafico" => array:2 [ "original" => 0 "multimedia" => array:8 [ "identificador" => "fig0005" "etiqueta" => "Fig. 1" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr1.jpeg" "Alto" => 2370 "Ancho" => 2508 "Tamanyo" => 228514 ] ] "detalles" => array:1 [ 0 => array:3 [ "identificador" => "at0005" "detalle" => "Fig. " "rol" => "short" ] ] "descripcion" => array:1 [ "en" => "<p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">Flowchart for articles selection.</p>" ] ] ] "autores" => array:1 [ 0 => array:2 [ "autoresLista" => "M.P. Castro Mora, J. Palacio Varona, B. Perez Riaño, C. Laverde Cubides, D.V. Rey-Rodriguez" "autores" => array:5 [ 0 => array:2 [ "nombre" => "M.P." "apellidos" => "Castro Mora" ] 1 => array:2 [ "nombre" => "J." "apellidos" => "Palacio Varona" ] 2 => array:2 [ "nombre" => "B." "apellidos" => "Perez Riaño" ] 3 => array:2 [ "nombre" => "C." "apellidos" => "Laverde Cubides" ] 4 => array:2 [ "nombre" => "D.V." "apellidos" => "Rey-Rodriguez" ] ] ] ] ] "idiomaDefecto" => "en" "Traduccion" => array:1 [ "es" => array:9 [ "pii" => "S0365669123000254" "doi" => "10.1016/j.oftal.2023.01.008" "estado" => "S300" "subdocumento" => "" "abierto" => array:3 [ "ES" => false "ES2" => false "LATM" => false ] "gratuito" => false "lecturas" => array:1 [ "total" => 0 ] "idiomaDefecto" => "es" "EPUB" => "https://multimedia.elsevier.es/PublicationsMultimediaV1/item/epub/S0365669123000254?idApp=UINPBA00004N" ] ] "EPUB" => "https://multimedia.elsevier.es/PublicationsMultimediaV1/item/epub/S2173579423000336?idApp=UINPBA00004N" "url" => "/21735794/0000009800000004/v2_202304071550/S2173579423000336/v2_202304071550/en/main.assets" ] "en" => array:19 [ "idiomaDefecto" => true "cabecera" => "<span class="elsevierStyleTextfn">Short communication</span>" "titulo" => "Unifocal and unilateral pigmented paravenous retinochoroidal atrophy" "tieneTextoCompleto" => true "paginas" => array:1 [ 0 => array:2 [ "paginaInicial" => "233" "paginaFinal" => "237" ] ] "autores" => array:1 [ 0 => array:4 [ "autoresLista" => "J.C. Castaño Silos, I. Sanchez-Guillen, I. Almorín-Fernández-Vigo, M. Jerez Fidalgo, J.I. Fernández-Vigo" "autores" => array:5 [ 0 => array:3 [ "nombre" => "J.C." "apellidos" => "Castaño Silos" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">a</span>" "identificador" => "aff0005" ] ] ] 1 => array:4 [ "nombre" => "I." "apellidos" => "Sanchez-Guillen" "email" => array:1 [ 0 => "inessanchezguillen@gmail.com" ] "referencia" => array:3 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">a</span>" "identificador" => "aff0005" ] 1 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">b</span>" "identificador" => "aff0010" ] 2 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">*</span>" "identificador" => "cor0005" ] ] ] 2 => array:3 [ "nombre" => "I." "apellidos" => "Almorín-Fernández-Vigo" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">b</span>" "identificador" => "aff0010" ] ] ] 3 => array:3 [ "nombre" => "M." "apellidos" => "Jerez Fidalgo" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">a</span>" "identificador" => "aff0005" ] ] ] 4 => array:3 [ "nombre" => "J.I." "apellidos" => "Fernández-Vigo" "referencia" => array:2 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">c</span>" "identificador" => "aff0015" ] 1 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">d</span>" "identificador" => "aff0020" ] ] ] ] "afiliaciones" => array:4 [ 0 => array:3 [ "entidad" => "Departamento de Oftalmología, Hospital Perpetuo Socorro, Badajoz, Spain" "etiqueta" => "a" "identificador" => "aff0005" ] 1 => array:3 [ "entidad" => "Centro Internacional de Oftalmología Avanzada, Badajoz, Spain" "etiqueta" => "b" "identificador" => "aff0010" ] 2 => array:3 [ "entidad" => "Centro Internacional de Oftalmología Avanzada, Madrid, Spain" "etiqueta" => "c" "identificador" => "aff0015" ] 3 => array:3 [ "entidad" => "Departamento de Oftalmología, Hospital Clínico San Carlos, Instituto de Investigación Sanitaria San Carlos (IdISSC), Madrid, Spain" "etiqueta" => "d" "identificador" => "aff0020" ] ] "correspondencia" => array:1 [ 0 => array:3 [ "identificador" => "cor0005" "etiqueta" => "⁎" "correspondencia" => "<span class="elsevierStyleItalic">Corresponding author</span>." ] ] ] ] "titulosAlternativos" => array:1 [ "es" => array:1 [ "titulo" => "Atrofia coriorretiniana pigmentada paravenosa de presentación unifocal y unilateral" ] ] "resumenGrafico" => array:2 [ "original" => 0 "multimedia" => array:8 [ "identificador" => "fig0005" "etiqueta" => "Fig. 1" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr1.jpeg" "Alto" => 1950 "Ancho" => 2007 "Tamanyo" => 461798 ] ] "detalles" => array:1 [ 0 => array:3 [ "identificador" => "at0005" "detalle" => "Fig. " "rol" => "short" ] ] "descripcion" => array:1 [ "en" => "<p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">Colour fundus photograph and autofluorescence of both eyes. The retinogram is unremarkable in the right eye (A). The left eye shows chorioretinal atrophy with pigment distribution following the inferior temporal arcade and a lamellar hole (B). The autofluorescence image of the right eye is unaltered (C) and the left eye shows a hypoautofluorescent area with defined borders in the area of the lesion (D).</p>" ] ] ] "textoCompleto" => "<span class="elsevierStyleSections"><span id="sec0005" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0025">Introduction</span><p id="par0005" class="elsevierStylePara elsevierViewall"><span class="elsevierStyleItalic">Paravenous pigmented chorioretinal atrophy</span> (PPCRA) was described in 1937 by Brown<a class="elsevierStyleCrossRef" href="#bib0005"><span class="elsevierStyleSup">1</span></a> as an entity characterised by areas of retinal atrophy and pigment accumulation in areas close to the venous arcades. PPCRA has been given multiple names such as retinochoroiditis radiate,<a class="elsevierStyleCrossRef" href="#bib0005"><span class="elsevierStyleSup">1</span></a> striated, congenital retinal pigmentation, pigmented paravenous chorioretinal atrophy and, more recently, PPCRA.<a class="elsevierStyleCrossRef" href="#bib0010"><span class="elsevierStyleSup">2</span></a></p><p id="par0010" class="elsevierStylePara elsevierViewall">This entity usually occurs sporadically, although familial cases have been described and the etiology is unknown.<a class="elsevierStyleCrossRef" href="#bib0015"><span class="elsevierStyleSup">3</span></a> Several etiopathogenic mechanisms have been proposed, such as a primary dysfunction of the retinal pigment epithelium or the development of retinal vessel abnormalities. Associations have been described with systemic inflammatory pathology, such as Behçet's disease; infectious, such as rubella, tuberculosis or syphilis; and genetic alterations, such as mutation in the CRX gene.<a class="elsevierStyleCrossRef" href="#bib0010"><span class="elsevierStyleSup">2</span></a></p><p id="par0015" class="elsevierStylePara elsevierViewall">The diagnosis of PPCRA is primarily clinical, based on the characteristic fundus appearance. Regarding complementary tests, autofluorescence shows large areas of hypoautofluorescence with distinct borders in the atrophic zone. Fluorescein angiography reveals transmitted hyperfluorescence or chorioretinal atrophy, depending on the severity of the lesion, without fluorescein leakage.<a class="elsevierStyleCrossRef" href="#bib0010"><span class="elsevierStyleSup">2</span></a> In electroretinograms (ERG), reduced B-wave amplitude is the most common alteration, followed by reduced A-wave amplitude and delayed latency. Visual field changes depend on pigment distribution and atrophy,<a class="elsevierStyleCrossRef" href="#bib0010"><span class="elsevierStyleSup">2</span></a> while on optical coherence tomography (OCT) the inner retinal layers may be spared or show significant thinning.<a class="elsevierStyleCrossRef" href="#bib0020"><span class="elsevierStyleSup">4</span></a> Recent work with angio-OCT (OCTA) has contradictory results regarding decreased choroidal perfusion.<a class="elsevierStyleCrossRefs" href="#bib0025"><span class="elsevierStyleSup">5,6</span></a></p><p id="par0020" class="elsevierStylePara elsevierViewall">PPCRA is usually bilateral and symmetrical with variable expressivity. Few cases are asymmetric or unilateral<a class="elsevierStyleCrossRefs" href="#bib0015"><span class="elsevierStyleSup">3,7</span></a> like the one presented below.</p></span><span id="sec0010" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0030">Clinical case</span><p id="par0025" class="elsevierStylePara elsevierViewall">A 50-year-old woman of African origin attended the emergency department with pain and progressive decrease in left eye vision (LE). Her personal history included rheumatoid arthritis, sickle cell disease and intestinal malabsorption syndrome.</p><p id="par0030" class="elsevierStylePara elsevierViewall">Examination revealed visual acuity of 20/20 in the right eye and 1/20 in the right eye. Slit-lamp examination of the anterior segment showed marked hyperemia in the inferior bulbar conjunctiva of the right eye, with congestion of the scleral vessels, compatible with diffuse scleritis. the LE fundus showed a lamellar macular hole (LMH) and atrophy-hypertrophy lesions of the retinal pigment epithelium around the inferior temporal venous arcade, with significant pigment mobilisation in the form of bony spicules, extending to the posteroquatorial retina (<a class="elsevierStyleCrossRef" href="#fig0005">Fig. 1</a>B).</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia><p id="par0035" class="elsevierStylePara elsevierViewall">Additional tests include fundus autofluorescence, which showed a hypoautofluorescent image with well-defined borders in the area of the lesion (<a class="elsevierStyleCrossRef" href="#fig0005">Fig. 1</a>D). Fluorescein angiography showed hyperfluorescence compatible with degeneration of the retinal pigment epithelium, blockage of fluorescence in the areas of pigment accumulation along the retinal vessels and repletion defects in some vessels compatible with partial occlusions. No fluorescein leakage could be observed (<a class="elsevierStyleCrossRef" href="#fig0010">Fig. 2</a>C). An OCT was performed and confirmed the funduscopic image of LMH (<a class="elsevierStyleCrossRef" href="#fig0010">Fig. 2</a>D). The visual field revealed a generalised decrease in sensitivity with an altitudinal defect in the superior hemifield compatible with the LE retinal lesion (<a class="elsevierStyleCrossRef" href="#fig0010">Fig. 2</a>A). Finally, electrophysiological tests showed latencies, amplitudes and morphology within normal limits in the structured stimulus (pattern) visual evoked potentials. The pattern ERG (ERG-P) shows decreased amplitudes and a normal N95/P50 ratio, and the ERG-Ganzfeld (ERG-G) also showed a significant reduction in the amplitude of the responses of the different scotopic and photopic phases, congruent with a macular lesion due to LMH. The right eye showed no alterations in visual evoked potentials -P or ERG (P or G).</p><elsevierMultimedia ident="fig0010"></elsevierMultimedia><p id="par0040" class="elsevierStylePara elsevierViewall">The patient was diagnosed with unilateral PPCRA and diffuse scleritis in the left eye. Treatment was prescribed with oral corticosteroids and infliximab for the scleritis and follow-up by Ophthalmology and Rheumatology.</p></span><span id="sec0015" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0035">Discussion</span><p id="par0045" class="elsevierStylePara elsevierViewall">PPCRA is an entity characterised primarily by its typical funduscopic appearance of symmetrical, bilateral pigment accumulation and chorioretinal atrophy distributed along the retinal veins, starting at some distance from the optic nerve head. Pigmentation is typically in bony spicule. Due to morphological similarities with retinitis pigmentosa (RP) (especially the pericentral, sectorial and atypical types), PPCRA has sometimes been described as a variant of this disease. However, unlike PPCRA, in RP nyctalopia and campimetric involvement are present and tend to progressively worsen.<a class="elsevierStyleCrossRef" href="#bib0010"><span class="elsevierStyleSup">2</span></a> Vascular sclerosis and alterations in the papilla and macula<a class="elsevierStyleCrossRef" href="#bib0025"><span class="elsevierStyleSup">5</span></a> typical of RP are also absent in PPCRA.</p><p id="par0050" class="elsevierStylePara elsevierViewall">Other differential diagnoses include helical peripapillary chorioretinal atrophy, serpiginous choroidopathy, angioid streaks, cone dystrophy, Stickler syndrome, choroideremia due to gyrate atrophy, Wagner's dominant vitreoretinal degeneration, sarcoidosis, syphilis, acute retinal necrosis, cytomegalovirus retinitis, tuberculous disseminated choroiditis, onchocerciasis, toxoplasmosis, frosted branch angeitis and various disorders called pseudoretinitis pigmentosa.</p><p id="par0055" class="elsevierStylePara elsevierViewall">PPCRA usually presents asymptomatically and is generally detected incidentally.<a class="elsevierStyleCrossRef" href="#bib0015"><span class="elsevierStyleSup">3</span></a> It does not affect vision, except in specific cases where central vision may be slightly impaired.<a class="elsevierStyleCrossRef" href="#bib0010"><span class="elsevierStyleSup">2</span></a> However, in the present case, the patient was not asymptomatic as she reported decreased visual acuity caused by LMH and pain due to the accompanying scleritis. The work of Xiang and Wei<a class="elsevierStyleCrossRef" href="#bib0040"><span class="elsevierStyleSup">8</span></a> presents a case of PPCRA with LMH with very low vision attributed to the significant atrophy of the nerve fibre layer on OCT. Similar to the work of Xiang and Wei,<a class="elsevierStyleCrossRef" href="#bib0040"><span class="elsevierStyleSup">8</span></a> the patient in our case had LMH with very low vision, which can be attributed to atrophy of the ganglion cell layer seen on OCT (<a class="elsevierStyleCrossRef" href="#fig0010">Fig. 2</a>E). In addition to LMH, other macular changes have been described in the literature including: cystoid macular edema, exudates, epiretinal membrane, central areolar macular atrophy and macular coloboma. It is not known whether these macular changes are associated with the disease phenotype or are independent.<a class="elsevierStyleCrossRef" href="#bib0010"><span class="elsevierStyleSup">2</span></a></p><p id="par0060" class="elsevierStylePara elsevierViewall">The appearance of these lesions unilaterally and unifocally, as in the case of our patient, is extremely unusual and there are only 3 cases published in the literature.<a class="elsevierStyleCrossRefs" href="#bib0015"><span class="elsevierStyleSup">3,7,9</span></a> The 3 published cases are females (as in the present case) of varying ages (from 23 to 79 years). In all cases the presentation was sporadic and one case included a family history of the disease.<a class="elsevierStyleCrossRef" href="#bib0045"><span class="elsevierStyleSup">9</span></a> Visual acuities varied from 20/20 to 20/40 and in one case it was associated with bilateral epiretinal membrane.<a class="elsevierStyleCrossRef" href="#bib0015"><span class="elsevierStyleSup">3</span></a></p><p id="par0065" class="elsevierStylePara elsevierViewall">There is no treatment for PPCRA, except for associated complications.<a class="elsevierStyleCrossRef" href="#bib0010"><span class="elsevierStyleSup">2</span></a> Prognostically, PPCRA lesions do not usually progress unlike RP.<a class="elsevierStyleCrossRef" href="#bib0015"><span class="elsevierStyleSup">3</span></a> Some cases of very slow ERG and visual field progression have been reported.<a class="elsevierStyleCrossRef" href="#bib0050"><span class="elsevierStyleSup">10</span></a> However, more studies and longer follow-up of these unilateral and unifocal cases are needed to rule out the possibility of other lesions appearing in the fellow eye.</p></span><span id="sec0020" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0040">Conclusion</span><p id="par0070" class="elsevierStylePara elsevierViewall">PPCRA is a rare bilateral retinal degeneration, typically asymptomatic and non-progressive, characterised by atrophy of the retinal pigment epithelium, choroid and outer retinal layers. It can be associated with other ocular disorders and systemic inflammatory diseases, and its unilateral and unifocal presentation as in the present case is extremely unusual.</p></span><span id="sec0025" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0045">Conflict of interest</span><p id="par0075" class="elsevierStylePara elsevierViewall">No conflicts of interest were declared by the authors.</p></span></span>" "textoCompletoSecciones" => array:1 [ "secciones" => array:10 [ 0 => array:3 [ "identificador" => "xres1876907" "titulo" => "Abstract" "secciones" => array:1 [ 0 => array:1 [ "identificador" => "abst0005" ] ] ] 1 => array:2 [ "identificador" => "xpalclavsec1628166" "titulo" => "Keywords" ] 2 => array:3 [ "identificador" => "xres1876908" "titulo" => "Resumen" "secciones" => array:1 [ 0 => array:1 [ "identificador" => "abst0010" ] ] ] 3 => array:2 [ "identificador" => "xpalclavsec1628165" "titulo" => "Palabras clave" ] 4 => array:2 [ "identificador" => "sec0005" "titulo" => "Introduction" ] 5 => array:2 [ "identificador" => "sec0010" "titulo" => "Clinical case" ] 6 => array:2 [ "identificador" => "sec0015" "titulo" => "Discussion" ] 7 => array:2 [ "identificador" => "sec0020" "titulo" => "Conclusion" ] 8 => array:2 [ "identificador" => "sec0025" "titulo" => "Conflict of interest" ] 9 => array:1 [ "titulo" => "References" ] ] ] "pdfFichero" => "main.pdf" "tienePdf" => true "fechaRecibido" => "2022-11-01" "fechaAceptado" => "2022-12-27" "PalabrasClave" => array:2 [ "en" => array:1 [ 0 => array:4 [ "clase" => "keyword" "titulo" => "Keywords" "identificador" => "xpalclavsec1628166" "palabras" => array:4 [ 0 => "Pigmented paravenous chorioretinal atrophy" 1 => "Pigmented paravenous retinochoroid atrophy" 2 => "Paravenous atrophy" 3 => "Lamellar macular hole" ] ] ] "es" => array:1 [ 0 => array:4 [ "clase" => "keyword" "titulo" => "Palabras clave" "identificador" => "xpalclavsec1628165" "palabras" => array:4 [ 0 => "Atrofia coriorretiniana pigmentada paravenosa" 1 => "Atrofia retinocoroidea pigmentada paravenosa" 2 => "Atrofia paravenosa" 3 => "Agujero macular lamelar" ] ] ] ] "tieneResumen" => true "resumen" => array:2 [ "en" => array:2 [ "titulo" => "Abstract" "resumen" => "<span id="abst0005" class="elsevierStyleSection elsevierViewall"><p id="spar0015" class="elsevierStyleSimplePara elsevierViewall">Paravenous pigmented chorioretinal atrophy (PPRCA) is a generally multifocal, bilateral and symmetric rare entity associated with autoimmune diseases and other ocular complications.</p><p id="spar0020" class="elsevierStyleSimplePara elsevierViewall">We present the clinical case of a patient with rheumatoid arthritis who attended for pain of several days. He presented decreased visual acuity of the left eye (LE), nodular scleritis and chorioretinal atrophy with pigment accumulation in bone spicules in the inferior temporal vascular arcade and lamellar macular hole (AML). The right eye shows no alterations. LE autofluorescence (AF) shows a hypoautofluorescence lesion with defined edges. Fluorescein angiography (FAG) shows hyperfluorescence consistent with retinal pigmentary epithelial degeneration and blockage in pigment areas. The visual field (VC) reveals a defect in the superior hemifield.</p><p id="spar0025" class="elsevierStyleSimplePara elsevierViewall">This case describes an atypical unifocal and unilateral PPRCA. This variant must be known to make a correct differential diagnosis, as well as to provide adequate prognostic information.</p></span>" ] "es" => array:2 [ "titulo" => "Resumen" "resumen" => "<span id="abst0010" class="elsevierStyleSection elsevierViewall"><p id="spar0030" class="elsevierStyleSimplePara elsevierViewall">La atrofia coriorretiniana pigmentada paravenosa (PPRCA) es una entidad infrecuente, asociada a enfermedades autoinmunes y otras complicaciones oculares, generalmente multifocal, bilateral y simétrica.</p><p id="spar0035" class="elsevierStyleSimplePara elsevierViewall">Se presenta el caso clínico de una paciente con artritis reumatoide que acude por dolor de varios días. Presenta disminución de agudeza visual de ojo izquierdo (OI), escleritis nodular y atrofia coriorretiniana con acumulación de pigmento en espículas óseas en arcada vascular temporal inferior y agujero macular lamelar (AML). El ojo derecho no presenta alteraciones. La autofluorescencia (AF) del OI muestra hipoautofluorescencia de bordes definidos en la lesión. La angiografía con fluoresceína (AFG) evidencia hiperfluorescencia compatible con degeneración del epitelio pigmentario retiniano y bloqueo en las áreas de pigmento. El campo visual (CV) revela un defecto altitudinal en hemicampo superior.</p><p id="spar0040" class="elsevierStyleSimplePara elsevierViewall">Este caso describe una PPRCA atípica unifocal y unilateral. Se debe conocer esta variante para realizar un correcto diagnóstico diferencial, así como proporcionar una información pronóstica adecuada.</p></span>" ] ] "multimedia" => array:2 [ 0 => array:8 [ "identificador" => "fig0005" "etiqueta" => "Fig. 1" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr1.jpeg" "Alto" => 1950 "Ancho" => 2007 "Tamanyo" => 461798 ] ] "detalles" => array:1 [ 0 => array:3 [ "identificador" => "at0005" "detalle" => "Fig. " "rol" => "short" ] ] "descripcion" => array:1 [ "en" => "<p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">Colour fundus photograph and autofluorescence of both eyes. The retinogram is unremarkable in the right eye (A). The left eye shows chorioretinal atrophy with pigment distribution following the inferior temporal arcade and a lamellar hole (B). The autofluorescence image of the right eye is unaltered (C) and the left eye shows a hypoautofluorescent area with defined borders in the area of the lesion (D).</p>" ] ] 1 => array:8 [ "identificador" => "fig0010" "etiqueta" => "Fig. 2" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr2.jpeg" "Alto" => 4082 "Ancho" => 2925 "Tamanyo" => 822900 ] ] "detalles" => array:1 [ 0 => array:3 [ "identificador" => "at0010" "detalle" => "Fig. " "rol" => "short" ] ] "descripcion" => array:1 [ "en" => "<p id="spar0010" class="elsevierStyleSimplePara elsevierViewall">24–2 computerised campimetry (A and B). The left eye (despite poor reliability) shows a generalised decrease in sensitivity with an almost complete defect in the superior hemifield congruent with the retinal lesion (A). The right eye shows patchy defects in the upper and lower nasal region due to artefacts caused by poor collaboration (B). Angiography of the left eye (C) shows hyperfluorescence compatible with degeneration of the retinal pigment epithelium and blockage of fluorescence in the areas of pigment accumulation along the inferior retinal vessels. Macular OCT (D and E) shows a lamellar hole (D) and a decrease in the thickness of the ganglion cell layer, especially in the inferior sector (E).</p>" ] ] ] "bibliografia" => array:2 [ "titulo" => "References" "seccion" => array:1 [ 0 => array:2 [ "identificador" => "bibs0005" "bibliografiaReferencia" => array:10 [ 0 => array:3 [ "identificador" => "bib0005" "etiqueta" => "1" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Retino-choroiditis radiata" "autores" => array:1 [ 0 => array:2 [ "etal" => false "autores" => array:1 [ 0 => "T.H. Brown" ] ] ] ] ] "host" => array:1 [ 0 => array:2 [ "doi" => "10.1136/bjo.21.12.645" "Revista" => array:7 [ "tituloSerie" => "Br J Ophthalmol." 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