array:24 [ "pii" => "S2173579421000773" "issn" => "21735794" "doi" => "10.1016/j.oftale.2020.12.008" "estado" => "S300" "fechaPublicacion" => "2021-11-01" "aid" => "1912" "copyright" => "Sociedad Española de Oftalmología" "copyrightAnyo" => "2021" "documento" => "article" "crossmark" => 1 "subdocumento" => "rev" "cita" => "Arch Soc Esp Oftalmol. 2021;96 Supl 1:38-51" "abierto" => array:3 [ "ES" => false "ES2" => false "LATM" => false ] "gratuito" => false "lecturas" => array:1 [ "total" => 0 ] "Traduccion" => array:1 [ "es" => array:19 [ "pii" => "S0365669121000289" "issn" => "03656691" "doi" => "10.1016/j.oftal.2020.12.016" "estado" => "S300" "fechaPublicacion" => "2021-11-01" "aid" => "1912" "copyright" => "Sociedad Española de Oftalmología" "documento" => "article" "crossmark" => 1 "subdocumento" => "rev" "cita" => "Arch Soc Esp Oftalmol. 2021;96 Supl 1:38-51" "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">Revisión</span>" "titulo" => "Alteraciones del cristalino en la aniridia congénita" "tienePdf" => "es" "tieneTextoCompleto" => "es" "tieneResumen" => array:2 [ 0 => "es" 1 => "en" ] "paginas" => array:1 [ 0 => array:2 [ "paginaInicial" => "38" "paginaFinal" => "51" ] ] "titulosAlternativos" => array:1 [ "en" => array:1 [ "titulo" => "Crystalline lens alterations in congenital aniridia" ] ] "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" => "fig0065" "etiqueta" => "Figura 13" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr13.jpeg" "Alto" => 1691 "Ancho" => 2167 "Tamanyo" => 264270 ] ] "descripcion" => array:1 [ "es" => "<p id="spar0075" class="elsevierStyleSimplePara elsevierViewall">Cuatro formas de evolución avanzada de la catarata en la aniridia. Arriba izquierda: mujer de 40<span class="elsevierStyleHsp" style=""></span>años con opacidad cortical difusa y núcleo discretamente opalescente. Arriba derecha: mujer de 19<span class="elsevierStyleHsp" style=""></span>años que experimentó rápida pérdida de visión y que presenta una catarata intumescente. Su ojo adelfo, que en ese momento tenía solo pequeñas opacidades subcorticales, desarrolló el mismo aspecto 6<span class="elsevierStyleHsp" style=""></span>meses después. Abajo izquierda: mujer de 45<span class="elsevierStyleHsp" style=""></span>años con importante opacidad irregular y signos de reabsorción parcial. Abajo derecha: hombre de 44<span class="elsevierStyleHsp" style=""></span>años con catarata córtico-nuclear madura, núcleo muy brunescente y subluxación.</p>" ] ] ] "autores" => array:1 [ 0 => array:2 [ "autoresLista" => "F. D’Oria, R. Barraquer, J.L. Alio" "autores" => array:3 [ 0 => array:2 [ "nombre" => "F." "apellidos" => "D’Oria" ] 1 => array:2 [ "nombre" => "R." 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Muñoz-Negrete, M.A. Teus, J. García-Feijoó, M.I. Canut, G. Rebolleda" "autores" => array:5 [ 0 => array:2 [ "nombre" => "F.J." "apellidos" => "Muñoz-Negrete" ] 1 => array:2 [ "nombre" => "M.A." "apellidos" => "Teus" ] 2 => array:2 [ "nombre" => "J." "apellidos" => "García-Feijoó" ] 3 => array:2 [ "nombre" => "M.I." "apellidos" => "Canut" ] 4 => array:2 [ "nombre" => "G." "apellidos" => "Rebolleda" ] ] ] ] ] "idiomaDefecto" => "en" "Traduccion" => array:1 [ "es" => array:9 [ "pii" => "S0365669120304287" "doi" => "10.1016/j.oftal.2020.11.005" "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/S0365669120304287?idApp=UINPBA00004N" ] ] "EPUB" => "https://multimedia.elsevier.es/PublicationsMultimediaV1/item/epub/S2173579421001377?idApp=UINPBA00004N" "url" => "/21735794/00000096000000S1/v1_202111240624/S2173579421001377/v1_202111240624/en/main.assets" ] "itemAnterior" => array:19 [ "pii" => "S2173579421001626" "issn" => "21735794" "doi" => "10.1016/j.oftale.2021.04.003" "estado" => "S300" "fechaPublicacion" => "2021-11-01" "aid" => "1936" "copyright" => "Sociedad Española de Oftalmología" "documento" => "article" "crossmark" => 1 "subdocumento" => "rev" "cita" => "Arch Soc Esp Oftalmol. 2021;96 Supl 1:15-37" "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" => "Aniridia and the ocular surface: Medical and surgical problems and solutions" "tienePdf" => "en" "tieneTextoCompleto" => "en" "tieneResumen" => array:2 [ 0 => "en" 1 => "es" ] "paginas" => array:1 [ 0 => array:2 [ "paginaInicial" => "15" "paginaFinal" => "37" ] ] "titulosAlternativos" => array:1 [ "es" => array:1 [ "titulo" => "Aniridia y superficie ocular: problemas y soluciones médicas y quirúrgicas" ] ] "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" => "fig0025" "etiqueta" => "Fig. 5" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr5.jpeg" "Alto" => 598 "Ancho" => 900 "Tamanyo" => 77317 ] ] "detalles" => array:1 [ 0 => array:3 [ "identificador" => "at0025" "detalle" => "Fig. " "rol" => "short" ] ] "descripcion" => array:1 [ "en" => "<p id="spar0025" class="elsevierStyleSimplePara elsevierViewall">Patient with congenital aniridia and complete limbic insufficiency of long evolution. Extensive conjunctivalization of the cornea is observed.</p>" ] ] ] "autores" => array:1 [ 0 => array:2 [ "autoresLista" => "J. Álvarez de Toledo Elizalde, S. López García, J.M. Benítez del Castillo, J. Durán de la Colina, O. Gris Castejón, J. Celis Sánchez, J.M. Herreras Cantalapiedra" "autores" => array:7 [ 0 => array:2 [ "nombre" => "J." "apellidos" => "Álvarez de Toledo Elizalde" ] 1 => array:2 [ "nombre" => "S." "apellidos" => "López García" ] 2 => array:2 [ "nombre" => "J.M." "apellidos" => "Benítez del Castillo" ] 3 => array:2 [ "nombre" => "J." "apellidos" => "Durán de la Colina" ] 4 => array:2 [ "nombre" => "O." "apellidos" => "Gris Castejón" ] 5 => array:2 [ "nombre" => "J." "apellidos" => "Celis Sánchez" ] 6 => array:2 [ "nombre" => "J.M." "apellidos" => "Herreras Cantalapiedra" ] ] ] ] ] "idiomaDefecto" => "en" "Traduccion" => array:1 [ "es" => array:9 [ "pii" => "S036566912100188X" "doi" => "10.1016/j.oftal.2021.04.003" "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/S036566912100188X?idApp=UINPBA00004N" ] ] "EPUB" => "https://multimedia.elsevier.es/PublicationsMultimediaV1/item/epub/S2173579421001626?idApp=UINPBA00004N" "url" => "/21735794/00000096000000S1/v1_202111240624/S2173579421001626/v1_202111240624/en/main.assets" ] "en" => array:20 [ "idiomaDefecto" => true "cabecera" => "<span class="elsevierStyleTextfn">Review</span>" "titulo" => "Crystalline lens alterations in congenital aniridia" "tieneTextoCompleto" => true "paginas" => array:1 [ 0 => array:2 [ "paginaInicial" => "38" "paginaFinal" => "51" ] ] "autores" => array:1 [ 0 => array:4 [ "autoresLista" => "F. D’Oria, R. Barraquer, J.L. Alio" "autores" => array:3 [ 0 => array:3 [ "nombre" => "F." "apellidos" => "D’Oria" "referencia" => array:2 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">a</span>" "identificador" => "aff0005" ] 1 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">b</span>" "identificador" => "aff0010" ] ] ] 1 => array:3 [ "nombre" => "R." "apellidos" => "Barraquer" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">c</span>" "identificador" => "aff0015" ] ] ] 2 => array:4 [ "nombre" => "J.L." "apellidos" => "Alio" "email" => array:1 [ 0 => "jlalio@vissum.com" ] "referencia" => array:3 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">a</span>" "identificador" => "aff0005" ] 1 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">d</span>" "identificador" => "aff0020" ] 2 => array:2 [ "etiqueta" => "*" "identificador" => "cor0005" ] ] ] ] "afiliaciones" => array:4 [ 0 => array:3 [ "entidad" => "Vissum Innovation, Alicante, Spain" "etiqueta" => "a" "identificador" => "aff0005" ] 1 => array:3 [ "entidad" => "Department of Basic Medical Science, Neuroscience and Sense Organs, University of Bari, Bari, Italy" "etiqueta" => "b" "identificador" => "aff0010" ] 2 => array:3 [ "entidad" => "Instituto Universitario Barraquer, Universitat Autònoma de Barcelona, Barcelona, Spain" "etiqueta" => "c" "identificador" => "aff0015" ] 3 => array:3 [ "entidad" => "División de Oftalmología, Universidad Miguel Hernández, Alicante, 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" => "Alteraciones del cristalino en la aniridia congénita" ] ] "resumenGrafico" => array:2 [ "original" => 0 "multimedia" => array:8 [ "identificador" => "fig0070" "etiqueta" => "Fig. 14" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr14.jpeg" "Alto" => 2086 "Ancho" => 3333 "Tamanyo" => 524176 ] ] "detalles" => array:1 [ 0 => array:3 [ "identificador" => "at0070" "detalle" => "Fig. 1" "rol" => "short" ] ] "descripcion" => array:1 [ "en" => "<p id="spar0070" class="elsevierStyleSimplePara elsevierViewall">Evolution of the patient in <a class="elsevierStyleCrossRef" href="#fig0015">Fig. 3</a> with development of opacities, while the subluxation does not seem to increase significantly. Top: BE appearance at the age of 21 years; cortical opacities have appeared in the inferior sectors. Middle row: the LE at age 27, with progression of cortical opacities and development of opalescent nuclear cataract. Bottom: the RE at age 35, with cortical cataract, more diffuse but nucleus still transparent; the densest opacity coincides with the area of greatest subluxation, as revealed by retroillumination. Bottom right: the LE with diaphragm IOL implantation.</p>" ] ] ] "textoCompleto" => "<span class="elsevierStyleSections"><span id="sec0005" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0025">Introduction</span><p id="par0005" class="elsevierStylePara elsevierViewall">Congenital aniridia is an ocular developmental anomaly associated with mutations in the PAX6 gene, the <span class="elsevierStyleItalic">master gene</span> in ocular development.<a class="elsevierStyleCrossRefs" href="#bib0005"><span class="elsevierStyleSup">1,2</span></a> Said gene regulates other genes, which explains the multiple ocular structures affected, without respecting the classical embryonic layered division. The classic ocular findings in aniridia are a combination of iris underdevelopment, including the presence of only a rudimentary iris stump, nystagmus, foveal hypoplasia, cataracts, glaucoma and keratopathy with features of corneal limbus stem cell deficiency.</p><p id="par0010" class="elsevierStylePara elsevierViewall">The impact of lens changes in aniridia on visual function is variable and often relatively minor among the multiple problems that these patients usually present with. However, they are the aspect of this disease that is most amenable to visual rehabilitation by surgical treatment. This review will focus on the description of the clinically observed changes in the lens of aniridia patients, as well as their etiopathogenesis and surgical treatment.</p></span><span id="sec0010" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0030">Cataract phenotypes</span><p id="par0015" class="elsevierStylePara elsevierViewall">Lens alterations in aniridia can essentially fall into three clinical categories: alterations related to transparency (opacities or cataracts), position (subluxation) which generally reveal zonular weakness, and size and shape (microspherophakia, etc.).</p><span id="sec0015" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0035">Transparency alterations</span><p id="par0020" class="elsevierStylePara elsevierViewall">Lens opacities are very common in eyes with aniridia, and in the literature their prevalence is estimated to be between 50–85%<a class="elsevierStyleCrossRef" href="#bib0015"><span class="elsevierStyleSup">3</span></a>, up to 88% in a Korean cohort of 60 eyes<a class="elsevierStyleCrossRef" href="#bib0020"><span class="elsevierStyleSup">4</span></a> and above 90% in a cohort of 26 Norwegian patients (52 eyes) with congenital aniridia.<a class="elsevierStyleCrossRef" href="#bib0025"><span class="elsevierStyleSup">5</span></a></p><p id="par0025" class="elsevierStylePara elsevierViewall">Cataracts associated with aniridia vary in morphology and are described as polar, cortical, subcapsular, lamellar and more rarely nuclear. Small opacities, either anterior polar, posterior polar or both, are typically present from birth and do not usually compromise vision. This may explain the scant attention given to the presence of cataracts in some studies, even recent ones.<a class="elsevierStyleCrossRef" href="#bib0030"><span class="elsevierStyleSup">6</span></a> In our clinic experience we have found the following clinical types according to lens opacity degree:<ul class="elsevierStyleList" id="lis0005"><li class="elsevierStyleListItem" id="lsti0005"><span class="elsevierStyleLabel">1</span><p id="par0030" class="elsevierStylePara elsevierViewall">Essentially clear lenses or only small anterior, posterior or other off-axis polar opacities. These are usually pediatric patients (<a class="elsevierStyleCrossRef" href="#fig0005">Fig. 1</a>). A clear or nearly clear lens is most often associated with incomplete aniridia (<a class="elsevierStyleCrossRef" href="#fig0010">Fig. 2</a>), but may coexist with glaucoma, microspherophakia, lens subluxation (<a class="elsevierStyleCrossRef" href="#fig0015">Fig. 3</a>), or corneal opacities.</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia><elsevierMultimedia ident="fig0010"></elsevierMultimedia><elsevierMultimedia ident="fig0015"></elsevierMultimedia></li><li class="elsevierStyleListItem" id="lsti0010"><span class="elsevierStyleLabel">2</span><p id="par0035" class="elsevierStylePara elsevierViewall">Small to medium-sized anteriorly predominant opacities of polar, subcapsular or cortical type. There are multiple variants, almost always with bilateral symmetry, from the typical flat polar plaque of about 1 mm, either isolated (<a class="elsevierStyleCrossRef" href="#fig0020">Fig. 4</a>, top left and centre) or associated with peripheral coronary opacity (<a class="elsevierStyleCrossRef" href="#fig0020">Fig. 4</a>, top right), or with annular reinforcement at the edge (<a class="elsevierStyleCrossRef" href="#fig0020">Fig. 4</a>, bottom). Sometimes they are prominent, pyramidal (<a class="elsevierStyleCrossRef" href="#fig0025">Fig. 5</a>), or may have lamellar extensions towards the foveal nucleus or even multiple plaques (<a class="elsevierStyleCrossRef" href="#fig0030">Fig. 6</a>).</p><elsevierMultimedia ident="fig0020"></elsevierMultimedia><elsevierMultimedia ident="fig0025"></elsevierMultimedia><elsevierMultimedia ident="fig0030"></elsevierMultimedia></li><li class="elsevierStyleListItem" id="lsti0015"><span class="elsevierStyleLabel">3</span><p id="par0040" class="elsevierStylePara elsevierViewall">A polar or axially predominant posterior subcapsular cataract, sometimes associated with a polar or anterior subcapsular cataract, which tends to progress peripherally, either in the cortical plane (<a class="elsevierStyleCrossRef" href="#fig0035">Fig. 7</a>) or in a somewhat deeper plane (subcortical or lamellar). It may also occur in cases of partial aniridia (<a class="elsevierStyleCrossRef" href="#fig0040">Fig. 8</a>).</p><elsevierMultimedia ident="fig0035"></elsevierMultimedia><elsevierMultimedia ident="fig0040"></elsevierMultimedia></li><li class="elsevierStyleListItem" id="lsti0020"><span class="elsevierStyleLabel">4</span><p id="par0045" class="elsevierStylePara elsevierViewall">Posterior cataracts more extensive than the preceding group, sometimes described as subcapsular but often concealing a subcortical plane. Typically coralliform or petaloid in appearance, with a series of radial opacities converging to a posterior axial nodule (<a class="elsevierStyleCrossRef" href="#fig0045">Fig. 9</a>).</p><elsevierMultimedia ident="fig0045"></elsevierMultimedia></li><li class="elsevierStyleListItem" id="lsti0025"><span class="elsevierStyleLabel">5</span><p id="par0050" class="elsevierStylePara elsevierViewall">Lamellar (zonular) or cortical ring cataract. Perhaps a variant of the previous one, but predominantly peripheral and with some respect for the visual axis (<a class="elsevierStyleCrossRef" href="#fig0050">Fig. 10</a>), at least until it progresses with age.</p><elsevierMultimedia ident="fig0050"></elsevierMultimedia></li><li class="elsevierStyleListItem" id="lsti0030"><span class="elsevierStyleLabel">6</span><p id="par0055" class="elsevierStylePara elsevierViewall">Nuclear cataract. It may rarely occur as an isolated congenital or infantile form (<a class="elsevierStyleCrossRef" href="#fig0055">Fig. 11</a>). The nuclear component is seen as a developmental form associated with any of the preceding patterns in a somewhat older age group. (<a class="elsevierStyleCrossRef" href="#fig0060">Fig. 12</a>).</p><elsevierMultimedia ident="fig0055"></elsevierMultimedia><elsevierMultimedia ident="fig0060"></elsevierMultimedia></li><li class="elsevierStyleListItem" id="lsti0035"><span class="elsevierStyleLabel">7</span><p id="par0060" class="elsevierStylePara elsevierViewall">Very advanced or mature cataracts. Progressive involvement of the cortex leads to diffuse opacity, whether cortical, cortico-nuclear or irregular. In some cases there is a rapid progression with the formation of an intumescent cataract (<a class="elsevierStyleCrossRef" href="#fig0065">Fig. 13</a>).</p><elsevierMultimedia ident="fig0065"></elsevierMultimedia></li></ul></p><p id="par0065" class="elsevierStylePara elsevierViewall">Finally, there is a group of patients in whom the degree of cataract cannot be accurately established (sometimes not at all) due to the presence of corneal opacities or previous surgery.</p></span><span id="sec0020" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0040">Position alterations</span><p id="par0070" class="elsevierStylePara elsevierViewall">The presence of lens subluxation <span class="elsevierStyleItalic">(ectopia lentis)</span> is also common in aniridia. It has been estimated to occur in at least half of the eyes and almost always consists of an upward displacement. As early as 1947 Beattie<a class="elsevierStyleCrossRef" href="#bib0035"><span class="elsevierStyleSup">7</span></a> described superior subluxation in 12 members of a family of 28 with aniridia, as well as a much higher risk of glaucoma in cases with subluxation. Since glaucoma in aniridia is associated, at least in parts of the eyes, with progressive angle closure by anterior synechiae,<a class="elsevierStyleCrossRef" href="#bib0040"><span class="elsevierStyleSup">8</span></a> it is possible that the presence of subluxation facilitates the formation of such adhesions by lens movements. Zonule degeneration in aniridia is possibly due to alteration (hypoplasia) of the ciliary body, an aspect that has been clinically assessed by ultrasound biomicroscopy.<a class="elsevierStyleCrossRef" href="#bib0045"><span class="elsevierStyleSup">9</span></a> We have found that cortical opacities in subluxated crystalline lenses tend to develop more in the area with increased zonular defect (<a class="elsevierStyleCrossRef" href="#fig0070">Fig. 14</a>).</p><elsevierMultimedia ident="fig0070"></elsevierMultimedia><p id="par0075" class="elsevierStylePara elsevierViewall">Another possible alteration in the position of the lens would be the presence of kerato-lenticular adhesion. This has been described in association with aniridia and causing cataract.<a class="elsevierStyleCrossRef" href="#bib0050"><span class="elsevierStyleSup">10</span></a> We have observed this configuration in an infant with incomplete aniridia, opacity of an entire corneal quadrant and kerato-lenticular adhesion, but still with little lens opacity, all bilateral and symmetrical (<a class="elsevierStyleCrossRef" href="#fig0075">Fig. 15</a>). This is probably a picture of anterior segment dysgenesis analogous to Peters anomaly, which in some cases has also been related to the PAX6 gene.<a class="elsevierStyleCrossRef" href="#bib0055"><span class="elsevierStyleSup">11</span></a></p><elsevierMultimedia ident="fig0075"></elsevierMultimedia><p id="par0080" class="elsevierStylePara elsevierViewall">Even in cases without obvious subluxation, biomicroscopic examination of the zonular region allowing for the absence of the iris often reveals an increased width of the space outside the crystalline equator, typically in the inferior sector (see <a class="elsevierStyleCrossRefs" href="#fig0005">Figs. 1, 3, 9, 12</a>…). This is a sign of incipient zonular distension due to weakness or loss of fibres.</p></span><span id="sec0025" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0045">Size and shape alterations</span><p id="par0085" class="elsevierStylePara elsevierViewall">Changes in the shape of the lens have been described, which could be a consequence of zonular weakness or the manifestation of a malformation of the lens itself. On computed tomography Mehta et al.<a class="elsevierStyleCrossRef" href="#bib0060"><span class="elsevierStyleSup">12</span></a> observed an apparent inversion of the lens curvatures in a case of aniridia, with greater convexity in the anterior than in the posterior, which does not occur in traumatic subluxations. However, B-echography revealed that this change in shape occurred “in the cataract and not in the capsule”. On the other hand, the maintenance of the shape of a membranous cataract after dislocation to the vitreous has been reported in a patient with aniridia who had undergone valve implantation for glaucoma.<a class="elsevierStyleCrossRef" href="#bib0065"><span class="elsevierStyleSup">13</span></a> We have observed in several cases of aniridia an increase in the anterior curvature of the lens, sometimes described as “anterior lenticonus”, which appears more convex than the posterior one in the absence of cataracts (<a class="elsevierStyleCrossRef" href="#fig0080">Fig. 16</a>).</p><elsevierMultimedia ident="fig0080"></elsevierMultimedia><p id="par0090" class="elsevierStylePara elsevierViewall">In cases with microphakia or microspherophakia (<a class="elsevierStyleCrossRef" href="#fig0015">Fig. 3</a>), the small size of the lens may be due to lens hypoplasia, secondary to zonule weakness or even to a process of resorption. When subluxation occurs in infancy, the area with the largest zonular defect is seen to be reduced as well as rounded. This is also seen in other forms of <span class="elsevierStyleItalic">ectopia lentis</span> and seems to be a consequence of the high elasticity of the lens in children. If the zonular weakness is widespread, a soft lens will tend to be spherical in shape and smaller in diameter.</p><p id="par0095" class="elsevierStylePara elsevierViewall">Spontaneous lens resorption has been reported in a Japanese family with aniridia and microcornea, over three generations and perhaps a fourth.<a class="elsevierStyleCrossRef" href="#bib0070"><span class="elsevierStyleSup">14</span></a> We have occasionally observed partial resorption with formation of a membranous cataract, almost always associated with significant subluxation. In at least one case we found – during keratoplasty – the total absence of the lens with no history of surgery or trauma, and no ultrasound evidence of subsequent dislocation, while the fellow eye did have a cataract. This could be spontaneous reabsorption, with or without previous dislocation, or else agenesis of the lens, which we have also observed in eyes with Peters anomaly.</p></span></span><span id="sec0030" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0050">Cataract genomics in aniridia</span><p id="par0100" class="elsevierStylePara elsevierViewall">Aniridia is due to point mutations or chromosomal deletions affecting the activity of the PAX6 gene, located in humans on chromosome 11p13. The previous association with markers on chromosome 2p<a class="elsevierStyleCrossRef" href="#bib0075"><span class="elsevierStyleSup">15</span></a> – also called type I aniridia, while type II was associated with PAX6 – has been disproved by further genetic studies of the same families.<a class="elsevierStyleCrossRef" href="#bib0080"><span class="elsevierStyleSup">16</span></a> Deletions on 11p13 that result in deletion of the PAX6 gene and its neighbouring genes can lead to systemic abnormalities. The classic syndrome occurring in 11p13 deletions is the so-called WAGR syndrome (Wilms’ tumour, aniridia, genitourinary anomalies and mental retardation). Wilms’ tumour and genitourinary anomalies result from disruption of the WT1 gene, a transcription factor gene located in the vicinity of PAX6. Aniridia has also been described in association with systemic findings other than those found in WAGR syndrome: the Gillespie syndrome is a very rare condition presenting with aniridia, cerebellar ataxia and mental retardation.<a class="elsevierStyleCrossRef" href="#bib0085"><span class="elsevierStyleSup">17</span></a> Other mutations in the PAX6 gene have been associated with some cases of Peters anomaly<a class="elsevierStyleCrossRef" href="#bib0055"><span class="elsevierStyleSup">11</span></a> and more recently with bilateral microphthalmia, cataracts, glaucoma and nystagmus in one family, and in another with various ocular (nystagmus, cataracts, foveal hypoplasia, colobomas…) and neurological malformations, but without iridian defect.<a class="elsevierStyleCrossRef" href="#bib0090"><span class="elsevierStyleSup">18</span></a></p><p id="par0105" class="elsevierStylePara elsevierViewall">The mechanisms by which genetic defects cause ocular alterations in aniridia are not precisely understood. Although the cells of the optic vesicle and lens originate from different embryonic layers – respectively the neuroectoderm and the superficial ectoderm – their development involves a series of mutual induction relationships. This leads to the question of whether the alterations in aniridia are due to primary dysfunction of PAX6 activity in one, the other or both cell types. Until recently there was some consensus on some aspects pointing to a predominant role of the lens<a class="elsevierStyleCrossRefs" href="#bib0095"><span class="elsevierStyleSup">19,20</span></a>:<ul class="elsevierStyleList" id="lis0010"><li class="elsevierStyleListItem" id="lsti0040"><span class="elsevierStyleLabel">•</span><p id="par0110" class="elsevierStylePara elsevierViewall">The primary defect that gives rise to the <span class="elsevierStyleItalic">small eye</span> phenotype – due to mutation of PAX6 in the mouse and used as an animal model of aniridia – is the failure of the surface ectoderm to form the crystalline placode.</p></li><li class="elsevierStyleListItem" id="lsti0045"><span class="elsevierStyleLabel">•</span><p id="par0115" class="elsevierStylePara elsevierViewall">The development of the lens vesicle requires PAX6 activity in the prospective lens ectoderm but not in the optic vesicle.</p></li><li class="elsevierStyleListItem" id="lsti0050"><span class="elsevierStyleLabel">•</span><p id="par0120" class="elsevierStylePara elsevierViewall">PAX6 is not essential for the formation of the optic vesicle, although it is involved in later stages of retinogenesis.</p></li></ul></p><p id="par0125" class="elsevierStylePara elsevierViewall">However, it has been shown that PAX6 expression in the mouse optic vesicle is necessary to maintain the contact of the mouse optic vesicle with the lens epithelium, which in turn is necessary for subsequent inductive interactions to take place.<a class="elsevierStyleCrossRef" href="#bib0105"><span class="elsevierStyleSup">21</span></a> More recent studies in chick embryos indicate that PAX6 expression in the optic vesicle does play a role in the regulation of cell survival in the optic vesicle, as well as in lens development (beyond the lens vesicle stage), at least during a narrow time window.<a class="elsevierStyleCrossRefs" href="#bib0110"><span class="elsevierStyleSup">22,23</span></a> Normal ocular development would therefore require the correct expression of PAX6 in <span class="elsevierStyleItalic">both</span> structures.</p><p id="par0130" class="elsevierStylePara elsevierViewall">On the other hand, Collinson et al.<a class="elsevierStyleCrossRef" href="#bib0120"><span class="elsevierStyleSup">24</span></a> observed that, in <span class="elsevierStyleItalic">small eye</span> mouse chimeras with up to 80% PAX6 mutant cells, these were replaced in the lens vesicle by normal cells (which reproduce more actively) from day 16 of development, but not in other ocular tissues. Despite this, ocular size, iris and corneal defects normalised in the fetal and adult periods. Therefore, these aspects of the phenotype could be <span class="elsevierStyleItalic">secondary</span> to the primary alterations of the lens and not to an effect of the PAX6 mutation on these structures. Extrapolating to humans, these authors suggest that partial therapeutic correction of the lens by gene therapy (even if only effective in a percentage of the cells), in embryos with aniridia genotype and identified by prenatal diagnosis<a class="elsevierStyleCrossRef" href="#bib0125"><span class="elsevierStyleSup">25</span></a> could significantly improve several of the clinical defects responsible for subsequent poor vision, ocular degeneration and blindness.<a class="elsevierStyleCrossRef" href="#bib0130"><span class="elsevierStyleSup">26</span></a></p></span><span id="sec0035" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0055">Indications and surgical management</span><p id="par0135" class="elsevierStylePara elsevierViewall">Congenital aniridia complicated by cataract does not require treatment when lens opacity and its effects on visual acuity are mild. In addition, patients with aniridia often have limited visual acuity from birth due to foveal hypoplasia or possible optic nerve hypoplasia. It is important to determine when the decrease in visual acuity is caused by cataract alone or by other ocular pathologies that may decrease vision, especially in patients with foveal hypoplasia. Several methods have been developed to predict retinal visual acuity and how much vision may improve after surgery, from electroretinography or visual evoked potentials to simpler and equally effective methods such as the <span class="elsevierStyleItalic">Vryghem Macular Function Test</span> (MFT), which uses a Parinaud near reading chart with a +8 dioptre addition lens and a Heine ophthalmoscope.<a class="elsevierStyleCrossRef" href="#bib0135"><span class="elsevierStyleSup">27</span></a></p><p id="par0140" class="elsevierStylePara elsevierViewall">However, progressive decline in visual acuity usually occurs in adulthood due to the development of presenile cataracts, which requires surgery with careful preoperative planning. First of all, continuous circular capsulorhexis (CCC) must be treated with great care. The anterior capsules of patients with congenital aniridia are thin and fragile with degenerative changes in the epithelial cells, as has been demonstrated in histological studies,<a class="elsevierStyleCrossRef" href="#bib0140"><span class="elsevierStyleSup">28</span></a> which may predispose to the development of capsular tears.</p><p id="par0145" class="elsevierStylePara elsevierViewall">Secondly, the use of a capsular tension ring (CTR) can significantly reduce the likelihood of postoperative intraocular lens dislocation, postoperative posterior capsule fibrosis (PCO) – patients with congenital aniridia are usually young, so the incidence of PCO is very high – and anterior capsule fibrosis.<a class="elsevierStyleCrossRef" href="#bib0145"><span class="elsevierStyleSup">29</span></a> In addition, many of these patients may have nystagmus, which poses difficulties during Nd:YAG laser treatment. One possible option is to perform posterior CCC with anterior vitrectomy at the time of cataract surgery in these eyes. Wang et al.<a class="elsevierStyleCrossRef" href="#bib0150"><span class="elsevierStyleSup">30</span></a> operated on 10 patients (17 eyes) with cataract and congenital aniridia, with posterior chamber yellow IOL implantation and CTR to stabilise the zonules and prevent future intraocular lens dislocation. In that study, no intraocular lens dislocation occurred and there were no cases of secondary glaucoma or corneal endothelial decompensation. Li et al.<a class="elsevierStyleCrossRef" href="#bib0155"><span class="elsevierStyleSup">31</span></a> performed cataract surgery on 24 eyes (12 patients) with congenital aniridia: most patients had some improvement in visual acuity and quality of vision. <a class="elsevierStyleCrossRef" href="#tbl0005">Table 1</a> summarises the demographic characteristics, visual outcomes and complications of the main studies on patients with cataract and congenital aniridia.</p><elsevierMultimedia ident="tbl0005"></elsevierMultimedia><p id="par0150" class="elsevierStylePara elsevierViewall">The possibility of spontaneous evolution towards subluxation or complete dislocation must be considered in the surgical approach to any case of aniridia with cataract, as these are often young patients with a long life expectancy. For this reason, some authors advise against the use of in-sac fixation for implants<a class="elsevierStyleCrossRef" href="#bib0160"><span class="elsevierStyleSup">32</span></a> or even advocate simple lensectomy without implant when there is clear subluxation.<a class="elsevierStyleCrossRef" href="#bib0165"><span class="elsevierStyleSup">33</span></a> The various techniques of fixation in the absence of capsular support nevertheless allow a lens to be implanted in any case in which it is deemed useful. A study of diaphragm lenses implanted in front of the capsular sac, supported by the ciliary sulcus, indicates good stability after 46 months of mean follow-up, although complications are not avoided in some cases, such as glaucoma deterioration (4 of 19 eyes) or chronic endothelial loss (3 of 11 eyes).<a class="elsevierStyleCrossRef" href="#bib0170"><span class="elsevierStyleSup">34</span></a> Reinhard et al.<a class="elsevierStyleCrossRef" href="#bib0170"><span class="elsevierStyleSup">34</span></a> reported the results of black diaphragm IOL implantation in 19 eyes with congenital aniridia. Glaucoma deterioration occurred postoperatively in 4 of 5 eyes with preoperative glaucoma, while 4 of 14 eyes without preoperative glaucoma developed chronic glaucoma postoperatively. Other authors have also found that prosthetic iris devices, while reducing postoperative photophobic symptoms in patients with congenital aniridia and cataract, may be associated with postoperative secondary glaucoma and long-term corneal endothelial decompensation,<a class="elsevierStyleCrossRefs" href="#bib0175"><span class="elsevierStyleSup">35–39</span></a> highlighting the need for longer-term follow-up studies as well as specific guidelines and protocols on selection criteria. When considering the use of black diaphragm IOLs, we must also emphasise the importance of differentiating these prosthetic devices that received European certification <span class="elsevierStyleItalic">(Conformité Européenne certificate)</span> in 2011 and FDA approval in 2018 from other discredited devices having only a cosmetic function, leading to severe complications with devastating visual consequences,<a class="elsevierStyleCrossRef" href="#bib0200"><span class="elsevierStyleSup">40</span></a> making them unsuitable for implantation in the anterior chamber of the eye for cosmetic purposes.<a class="elsevierStyleCrossRef" href="#bib0205"><span class="elsevierStyleSup">41</span></a></p><p id="par0155" class="elsevierStylePara elsevierViewall">Corneal tattooing (keratopigmentation [KTP]) is a surgical tool that has been used for many decades sporadically for the management of cosmetic problems related to blind eyes, until it was extensively researched and developed by our team. Modern KTP techniques allow for adequate cosmetic and functional results in the treatment of corneal leucomas, mild functional disabilities caused by moderate to severe iris defects, and elective eye colour change.<a class="elsevierStyleCrossRefs" href="#bib0210"><span class="elsevierStyleSup">42–46</span></a> The use of new and extensively studied micronised mineral pigments, with experimental evidence on their tolerability and toxicity,<a class="elsevierStyleCrossRefs" href="#bib0235"><span class="elsevierStyleSup">47,48</span></a> presents an additional advantage over other natural pigments because their particle size is decreased by micronisation procedures and reduces the possibility of developing a foreign body reaction against the pigment being injected into the corneal stroma. KTP has the main advantage of being non-invasive in the sense that it is not intraocular and can be performed without requiring lens removal or without the need for adequate intraocular visibility, as is the case with intraocular techniques. It therefore has a fundamental role in the management of patients with aniridia, but will be of lesser importance in the context of combined cataract surgery with aniridia.</p></span><span id="sec0040" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0060">Conclusions</span><p id="par0160" class="elsevierStylePara elsevierViewall">In most cases of aniridia, lens opacities progress during the first two decades of life to become subcapsular, lamellar or cortical cataracts. Although usually slow, their gradual extension with age or the development of a nuclear component often justifies surgical indication between the third and fifth decades, apart from rapidly progressing and subluxated cases.</p><p id="par0165" class="elsevierStylePara elsevierViewall">When congenital cataracts severely affect the transparency of the eye, extraction before the age of 3 months has been recommended to avoid sensory deprivation nystagmus. However, it is not easy to determine the potential benefit of early removal, as nystagmus is also common in cases of aniridia without cataract, and especially considering the possible complications. In the presence of media opacity, assessment of the status of the macula and optic nerve (and thus of potential visual acuity) is difficult, and even the information that can be obtained by electrophysiological tests is often of limited usefulness. In any case, extreme caution should be exercised when indicating surgery due to the strong inflammatory and cicatricial reaction of ocular tissues in aniridia in general and particularly in infants.</p></span><span id="sec0045" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0065">Funding</span><p id="par0170" class="elsevierStylePara elsevierViewall">This studay was partially funded by the <span class="elsevierStyleGrantSponsor" id="gs0005">Instituto de Salud Carlos III/State Research Agency</span> and by the <span class="elsevierStyleGrantSponsor" id="gs0010">European Regional Development Fund</span>(ERDF) “Una manera de hacer Europa”. Thematic Network of Cooperative Health Research OFTARED, reference RD16/0008/0012.</p></span><span id="sec0050" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0070">Conflict of interest</span><p id="par0175" class="elsevierStylePara elsevierViewall">No conflicts of interest have been declared by the authors.</p></span></span>" "textoCompletoSecciones" => array:1 [ "secciones" => array:12 [ 0 => array:3 [ "identificador" => "xres1614990" "titulo" => "Abstract" "secciones" => array:1 [ 0 => array:1 [ "identificador" => "abst0005" ] ] ] 1 => array:2 [ "identificador" => "xpalclavsec1443666" "titulo" => "Keywords" ] 2 => array:3 [ "identificador" => "xres1614989" "titulo" => "Resumen" "secciones" => array:1 [ 0 => array:1 [ "identificador" => "abst0010" ] ] ] 3 => array:2 [ "identificador" => "xpalclavsec1443665" "titulo" => "Palabras clave" ] 4 => array:2 [ "identificador" => "sec0005" "titulo" => "Introduction" ] 5 => array:3 [ "identificador" => "sec0010" "titulo" => "Cataract phenotypes" "secciones" => array:3 [ 0 => array:2 [ "identificador" => "sec0015" "titulo" => "Transparency alterations" ] 1 => array:2 [ "identificador" => "sec0020" "titulo" => "Position alterations" ] 2 => array:2 [ "identificador" => "sec0025" "titulo" => "Size and shape alterations" ] ] ] 6 => array:2 [ "identificador" => "sec0030" "titulo" => "Cataract genomics in aniridia" ] 7 => array:2 [ "identificador" => "sec0035" "titulo" => "Indications and surgical management" ] 8 => array:2 [ "identificador" => "sec0040" "titulo" => "Conclusions" ] 9 => array:2 [ "identificador" => "sec0045" "titulo" => "Funding" ] 10 => array:2 [ "identificador" => "sec0050" "titulo" => "Conflict of interest" ] 11 => array:1 [ "titulo" => "References" ] ] ] "pdfFichero" => "main.pdf" "tienePdf" => true "fechaRecibido" => "2020-10-27" "fechaAceptado" => "2020-12-15" "PalabrasClave" => array:2 [ "en" => array:1 [ 0 => array:4 [ "clase" => "keyword" "titulo" => "Keywords" "identificador" => "xpalclavsec1443666" "palabras" => array:6 [ 0 => "Aniridia" 1 => "Cataracts" 2 => "Congenital defects" 3 => "Corneal tattoo" 4 => "Keratopigmentation" 5 => "Prosthetic iris devices" ] ] ] "es" => array:1 [ 0 => array:4 [ "clase" => "keyword" "titulo" => "Palabras clave" "identificador" => "xpalclavsec1443665" "palabras" => array:6 [ 0 => "Aniridia" 1 => "Catarata" 2 => "Alteraciones congénitas" 3 => "Tatuaje corneal" 4 => "Queratopigmentación" 5 => "Dispositivos protésicos de iris" ] ] ] ] "tieneResumen" => true "resumen" => array:2 [ "en" => array:2 [ "titulo" => "Abstract" "resumen" => "<span id="abst0005" class="elsevierStyleSection elsevierViewall"><p id="spar0095" class="elsevierStyleSimplePara elsevierViewall">Congenital aniridia is a rare genetic disease associated with mutations in the PAX6 gene. Changes in the lens in aniridia can be alterations of size and shape, of position – which generally reveal zonular weakness and determines subluxation of the lens – and mainly changes in transparency, cataracts, with variable morphology of polar, cortical, subcapsular, lamellar, and more rarely, nuclear cataract. Visual acuity and quality of vision in patients with congenital aniridia complicated by cataracts can be improved by carefully planned surgery, when lack of media transparency justifies surgical indication. Most patients have some improvement in visual acuity and quality of retinal image. Cataract surgery with aniridia is complicated by pathological changes due to the underlying cause of the aniridia. Challenges include corneal opacification, friable capsule and, above all, iris and pupil reconstruction. It can also determine late complications, such as secondary glaucoma or deterioration of pre-existent glaucoma, and corneal endothelial decompensation. After crystalline lens surgery in these patients, either by cataract or dislocation, for visual rehabilitation there are various techniques such as keratopigmentation, prosthetic iris devices or Morcher intraocular lenses with a black diaphragm. An appropriate individualised surgical plan should be selected depending on patient and surgical experience, in order to minimise complications and give the best chance of postoperative success.</p></span>" ] "es" => array:2 [ "titulo" => "Resumen" "resumen" => "<span id="abst0010" class="elsevierStyleSection elsevierViewall"><p id="spar0100" class="elsevierStyleSimplePara elsevierViewall">La aniridia congénita es una enfermedad genética rara asociada a mutaciones del gen PAX6. Las alteraciones del cristalino en aniridia pueden ser de tamaño y forma, de posición – que en general revelan debilidad zonular y posible subluxación del cristalino – y principalmente alteraciones de transparencia. Pueden presentar cataratas con morfología variable desde polares, corticales, subcapsulares y lamelares hasta más infrecuentemente nucleares. La agudeza visual y la calidad de la visión en pacientes con aniridia congénita asociada a cataratas se pueden mejorar mediante una cirugía cuidadosamente planificada cuando la falta de transparencia de los medios justifique la indicación quirúrgica. La cirugía de cataratas con aniridia se complica por alteraciones patológicas asociadas a la aniridia. Los desafíos incluyen opacificación corneal, cápsula friable y sobre todo reconstrucción de iris y pupila; además, pueden aparecer complicaciones tardías como glaucoma secundario o deterioro de glaucoma preexistente y descompensación endotelial corneal. Tras la cirugía cristaliniana en estos pacientes, bien por catarata o luxación, para la rehabilitación visual existen diversas técnicas como la queratopigmentación, los dispositivos protésicos iridianos o las lentes de Morcher con diafragma negro. Debe seleccionarse un método quirúrgico individualizado, según el paciente y la experiencia quirúrgica, que sea apropiado para minimizar las complicaciones y obtener mayores tasas de éxito postoperatorio.</p></span>" ] ] "NotaPie" => array:1 [ 0 => array:2 [ "etiqueta" => "☆" "nota" => "<p class="elsevierStyleNotepara" id="npar0005">Please cite this article as: D’Oria F, Barraquer R, Alio JL. Alteraciones del cristalino en la aniridia congénita. Arch Soc Esp Oftalmol. 2021;96(S1):38–51.</p>" ] ] "multimedia" => array:17 [ 0 => array:8 [ "identificador" => "fig0005" "etiqueta" => "Fig. 1" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr1.jpeg" "Alto" => 1601 "Ancho" => 2167 "Tamanyo" => 246772 ] ] "detalles" => array:1 [ 0 => array:3 [ "identificador" => "at0005" "detalle" => "Fig. " "rol" => "short" ] ] "descripcion" => array:1 [ "en" => "<p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">A 10 year-old girl with complete aniridia and lenses described as “transparent”, although there is a punctate posterior polar opacity in RE and a small peripheral anterior cortical plaque in LE. Note the incipient bilateral inferior zonular distension. The patient has glaucoma that required surgical treatment with a valve implant in the left eye.</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" => 1117 "Ancho" => 1583 "Tamanyo" => 143715 ] ] "detalles" => array:1 [ 0 => array:3 [ "identificador" => "at0010" "detalle" => "Fig. " "rol" => "short" ] ] "descripcion" => array:1 [ "en" => "<p id="spar0010" class="elsevierStyleSimplePara elsevierViewall">IOP of a 4 year-old boy with bilateral incomplete aniridia, clear lens and VA < 0.1, due to possible foveal hypoplasia. IOP was normal (the central white spot is a reflection of the flash).</p>" ] ] 2 => array:8 [ "identificador" => "fig0015" "etiqueta" => "Fig. 3" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr3.jpeg" "Alto" => 1202 "Ancho" => 1583 "Tamanyo" => 151708 ] ] "detalles" => array:1 [ 0 => array:3 [ "identificador" => "at0015" "detalle" => "Fig. " "rol" => "short" ] ] "descripcion" => array:1 [ "en" => "<p id="spar0015" class="elsevierStyleSimplePara elsevierViewall">LE of a 6 year-old boy with bilateral complete aniridia and microspherophakia appearance. The zonule has a weak and distended appearance. The lenses were clear except for a punctate anterior polar opacity, although he already had glaucoma (see evolution in <a class="elsevierStyleCrossRef" href="#fig0070">Fig. 14</a>).</p>" ] ] 3 => array:8 [ "identificador" => "fig0020" "etiqueta" => "Fig. 4" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr4.jpeg" "Alto" => 1889 "Ancho" => 2917 "Tamanyo" => 393137 ] ] "detalles" => array:1 [ 0 => array:3 [ "identificador" => "at0020" "detalle" => "Fig. " "rol" => "short" ] ] "descripcion" => array:1 [ "en" => "<p id="spar0020" class="elsevierStyleSimplePara elsevierViewall">Forms of anterior subcapsular cataract in aniridia. Top left and centre: flat anterior polar plaque of about 1 mm, isolated, in a 22 year-old male. Above right: The same condition, associated with coronary peripheral opacity in a 26-year-old woman. Bottom: central plaque of about 2.5 mm, with peripheral circular enhancement, in a 4 year-old girl who also presented with choroidal coloboma.</p>" ] ] 4 => array:8 [ "identificador" => "fig0025" "etiqueta" => "Fig. 5" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr5.jpeg" "Alto" => 1100 "Ancho" => 2167 "Tamanyo" => 181558 ] ] "detalles" => array:1 [ 0 => array:3 [ "identificador" => "at0025" "detalle" => "Fig. " "rol" => "short" ] ] "descripcion" => array:1 [ "en" => "<p id="spar0025" class="elsevierStyleSimplePara elsevierViewall">Anterior pyramidal cataract in a 5-year-old boy with aniridia, showing remnants of the <span class="elsevierStyleItalic">tunica vascularis</span>.</p>" ] ] 5 => array:8 [ "identificador" => "fig0030" "etiqueta" => "Fig. 6" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr6.jpeg" "Alto" => 1614 "Ancho" => 2167 "Tamanyo" => 221431 ] ] "detalles" => array:1 [ 0 => array:3 [ "identificador" => "at0030" "detalle" => "Fig. " "rol" => "short" ] ] "descripcion" => array:1 [ "en" => "<p id="spar0030" class="elsevierStyleSimplePara elsevierViewall">A 17 year-old male with aniridia and IOP controlled with hypotensive eyedrops. The RE has an “anterior polar” cataract, actually a nodule just in front of the foveal nucleus with lamellar extensions arching backwards and others starting from the posterior pole of the foveal nucleus. The LE has two plaques in the anterior cortex, in a plane just in front of the nuclear interphase.</p>" ] ] 6 => array:8 [ "identificador" => "fig0035" "etiqueta" => "Fig. 7" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr7.jpeg" "Alto" => 1852 "Ancho" => 2167 "Tamanyo" => 291882 ] ] "detalles" => array:1 [ 0 => array:3 [ "identificador" => "at0035" "detalle" => "Fig. " "rol" => "short" ] ] "descripcion" => array:1 [ "en" => "<p id="spar0035" class="elsevierStyleSimplePara elsevierViewall">Female with WAGR syndrome who at least since the age of 2 exhibited posterior and anterior polar subcapsular opacities in BE. She required glaucoma surgery in BE. At the age of 25 the opacities in the LE had progressed a little (top), with discrete posterior peripheral extension, while the RE (bottom) developed a diffuse cortical cataract in addition to the more dense axial opacities.</p>" ] ] 7 => array:8 [ "identificador" => "fig0040" "etiqueta" => "Fig. 8" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr8.jpeg" "Alto" => 1224 "Ancho" => 2167 "Tamanyo" => 154583 ] ] "detalles" => array:1 [ 0 => array:3 [ "identificador" => "at0040" "detalle" => "Fig. " "rol" => "short" ] ] "descripcion" => array:1 [ "en" => "<p id="spar0040" class="elsevierStyleSimplePara elsevierViewall">Partial aniridia associated with central posterior subcapsular opacity with satellites or peripheral branches in a 32 year-old female patient with a family history of aniridia in four generations. There was mild powdery opacity in the embryonal nucleus.</p>" ] ] 8 => array:8 [ "identificador" => "fig0045" "etiqueta" => "Fig. 9" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr9.jpeg" "Alto" => 1737 "Ancho" => 2167 "Tamanyo" => 266215 ] ] "detalles" => array:1 [ 0 => array:3 [ "identificador" => "at0045" "detalle" => "Fig. " "rol" => "short" ] ] "descripcion" => array:1 [ "en" => "<p id="spar0045" class="elsevierStyleSimplePara elsevierViewall">Extensive posterior cortical or subcapsular cataract in two cases of complete aniridia. Above, a 13-year-old patient showing the coralliform or petaloid appearance with branches converging on an axial nodule, both in direct light and under retroillumination. Below, in a 34-year-old patient the plane of the opacities appears subcortical, like a posterior lamellar cataract. In both cases there is some inferior predominance.</p>" ] ] 9 => array:8 [ "identificador" => "fig0050" "etiqueta" => "Fig. 10" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr10.jpeg" "Alto" => 1952 "Ancho" => 2833 "Tamanyo" => 393664 ] ] "detalles" => array:1 [ 0 => array:3 [ "identificador" => "at0050" "detalle" => "Fig. 1" "rol" => "short" ] ] "descripcion" => array:1 [ "en" => "<p id="spar0050" class="elsevierStyleSimplePara elsevierViewall">Four patients with aniridia and approximately annular cataract. The opacities are located in the subcortical or zonular plane (top left, 17 year-old male; centre, 27 year-old female), or in the deep portion of the equatorial cortex (top right, 33 year-old sister of the patient in <a class="elsevierStyleCrossRef" href="#fig0040">Fig. 8</a>). The axial region may be spared or have small opacities, either posterior or anterior (bottom, female aged 34 years).</p>" ] ] 10 => array:8 [ "identificador" => "fig0055" "etiqueta" => "Fig. 11" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr11.jpeg" "Alto" => 1787 "Ancho" => 2167 "Tamanyo" => 253375 ] ] "detalles" => array:1 [ 0 => array:3 [ "identificador" => "at0055" "detalle" => "Fig. 1" "rol" => "short" ] ] "descripcion" => array:1 [ "en" => "<p id="spar0055" class="elsevierStyleSimplePara elsevierViewall">12 year-old boy with aniridia, cataracts seen at birth, glaucoma and psychomotor retardation. Renal alterations were excluded. There is no family history but there is paternal consanguinity. The opacity seems to be limited to the foveal nucleus of the lens.</p>" ] ] 11 => array:8 [ "identificador" => "fig0060" "etiqueta" => "Fig. 12" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr12.jpeg" "Alto" => 1228 "Ancho" => 2167 "Tamanyo" => 160657 ] ] "detalles" => array:1 [ 0 => array:3 [ "identificador" => "at0060" "detalle" => "Fig. 1" "rol" => "short" ] ] "descripcion" => array:1 [ "en" => "<p id="spar0060" class="elsevierStyleSimplePara elsevierViewall">Progression to nuclear cataract in two women with aniridia, aged 41 (left) and 28 years (right). The first, with a moderately brunescent nucleus, previously had only small subcortical or lamellar opacities. The second, with an opalescent nucleus, previously had a dense central posterior subcapsular plaque and is a carrier of a limbal annular homograft. Note the incipient subluxation in both.</p>" ] ] 12 => array:8 [ "identificador" => "fig0065" "etiqueta" => "Fig. 13" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr13.jpeg" "Alto" => 1691 "Ancho" => 2167 "Tamanyo" => 264270 ] ] "detalles" => array:1 [ 0 => array:3 [ "identificador" => "at0065" "detalle" => "Fig. 1" "rol" => "short" ] ] "descripcion" => array:1 [ "en" => "<p id="spar0065" class="elsevierStyleSimplePara elsevierViewall">Four forms of advanced cataract progression in aniridia. Top left: 40-year-old woman with diffuse cortical opacity and discretely opalescent nucleus. Above right: 19-year-old woman who experienced rapid vision loss and intumescent cataract. The fellow eye, which at the time had only small subcortical opacities, developed the same appearance 6 months later. Bottom left: 45 year-old woman with significant irregular opacity and signs of partial resorption. Bottom right: 44 year-old man with mature cortico-nuclear cataract, very brunescent nucleus and subluxation.</p>" ] ] 13 => array:8 [ "identificador" => "fig0070" "etiqueta" => "Fig. 14" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr14.jpeg" "Alto" => 2086 "Ancho" => 3333 "Tamanyo" => 524176 ] ] "detalles" => array:1 [ 0 => array:3 [ "identificador" => "at0070" "detalle" => "Fig. 1" "rol" => "short" ] ] "descripcion" => array:1 [ "en" => "<p id="spar0070" class="elsevierStyleSimplePara elsevierViewall">Evolution of the patient in <a class="elsevierStyleCrossRef" href="#fig0015">Fig. 3</a> with development of opacities, while the subluxation does not seem to increase significantly. Top: BE appearance at the age of 21 years; cortical opacities have appeared in the inferior sectors. Middle row: the LE at age 27, with progression of cortical opacities and development of opalescent nuclear cataract. Bottom: the RE at age 35, with cortical cataract, more diffuse but nucleus still transparent; the densest opacity coincides with the area of greatest subluxation, as revealed by retroillumination. Bottom right: the LE with diaphragm IOL implantation.</p>" ] ] 14 => array:8 [ "identificador" => "fig0075" "etiqueta" => "Fig. 15" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr15.jpeg" "Alto" => 869 "Ancho" => 2167 "Tamanyo" => 148167 ] ] "detalles" => array:1 [ 0 => array:3 [ "identificador" => "at0075" "detalle" => "Fig. 1" "rol" => "short" ] ] "descripcion" => array:1 [ "en" => "<p id="spar0075" class="elsevierStyleSimplePara elsevierViewall">Both eyes of this 2 month-old boy had subtotal aniridia, finely vascularised corneal opacity throughout the lower nasal quadrant and corneal adhesion to the lens in this area, although the lens was still transparent. Note the persistence of remnants of the <span class="elsevierStyleItalic">tunica vasculosa lentis</span>.</p>" ] ] 15 => array:8 [ "identificador" => "fig0080" "etiqueta" => "Fig. 16" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr16.jpeg" "Alto" => 1229 "Ancho" => 2167 "Tamanyo" => 161885 ] ] "detalles" => array:1 [ 0 => array:3 [ "identificador" => "at0080" "detalle" => "Fig. 1" "rol" => "short" ] ] "descripcion" => array:1 [ "en" => "<p id="spar0080" class="elsevierStyleSimplePara elsevierViewall">Two different patients with aniridia and crystalline lenses – with only small opacities – in which the anterior curvature appears increased, greater than the posterior curvature. Hardly any subluxation is visible.</p>" ] ] 16 => array:8 [ "identificador" => "tbl0005" "etiqueta" => "Table 1" "tipo" => "MULTIMEDIATABLA" "mostrarFloat" => true "mostrarDisplay" => false "detalles" => array:1 [ 0 => array:3 [ "identificador" => "at0085" "detalle" => "Table " "rol" => "short" ] ] "tabla" => array:2 [ "leyenda" => "<p id="spar0090" class="elsevierStyleSimplePara elsevierViewall">CTR: capsular tension ring; ECCE: extracapsular cataract surgery; IOL: intraocular lens; NR: not reported.</p>" "tablatextoimagen" => array:1 [ 0 => array:2 [ "tabla" => array:1 [ 0 => """ <table border="0" frame="\n \t\t\t\t\tvoid\n \t\t\t\t" class=""><thead title="thead"><tr title="table-row"><th class="td" title="\n \t\t\t\t\ttable-head\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t" scope="col" style="border-bottom: 2px solid black">Author [year] \t\t\t\t\t\t\n \t\t\t\t\t\t</th><th class="td" title="\n \t\t\t\t\ttable-head\n \t\t\t\t " align="center" valign="\n \t\t\t\t\ttop\n \t\t\t\t" scope="col" style="border-bottom: 2px solid black">Country \t\t\t\t\t\t\n \t\t\t\t\t\t</th><th class="td" title="\n \t\t\t\t\ttable-head\n \t\t\t\t " align="center" valign="\n \t\t\t\t\ttop\n \t\t\t\t" scope="col" style="border-bottom: 2px solid black">Cataract and aniridia patients \t\t\t\t\t\t\n \t\t\t\t\t\t</th><th class="td" title="\n \t\t\t\t\ttable-head\n \t\t\t\t " align="center" valign="\n \t\t\t\t\ttop\n \t\t\t\t" scope="col" style="border-bottom: 2px solid black">Type of operation \t\t\t\t\t\t\n \t\t\t\t\t\t</th><th class="td" title="\n \t\t\t\t\ttable-head\n \t\t\t\t " align="center" valign="\n \t\t\t\t\ttop\n \t\t\t\t" scope="col" style="border-bottom: 2px solid black">Mean age \t\t\t\t\t\t\n \t\t\t\t\t\t</th><th class="td" title="\n \t\t\t\t\ttable-head\n \t\t\t\t " align="center" valign="\n \t\t\t\t\ttop\n \t\t\t\t" scope="col" style="border-bottom: 2px solid black">Visual results \t\t\t\t\t\t\n \t\t\t\t\t\t</th><th class="td" title="\n \t\t\t\t\ttable-head\n \t\t\t\t " align="center" valign="\n \t\t\t\t\ttop\n \t\t\t\t" scope="col" style="border-bottom: 2px solid black">Complications \t\t\t\t\t\t\n \t\t\t\t\t\t</th></tr></thead><tbody title="tbody"><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead rowgroup " rowspan="3" align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Reinard et al. [2000].</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " rowspan="3" align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Germany</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " rowspan="3" align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">17 patients (19 eyes)</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">10 eyes: ECCE; \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " rowspan="3" align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">30 years (range: 10–59)</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " rowspan="3" align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Improvement in 14 eyes, no change in one eye, deterioration in 4 eyes</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">4 eyes with glaucoma deterioration (2 with medical treatment and 2 with surgery); \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">9 eyes: phacoemulsification. \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">4 eyes <span class="elsevierStyleItalic">ex novo</span> glaucoma (2 with medical treatment and 2 with surgery); \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">All: Black diaphragm IOL implant \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">3 eyes endothelial cell loss \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead rowgroup " rowspan="3" align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Park et al. [2010].</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " rowspan="3" align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Korea</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " rowspan="3" align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">33 eyes</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " rowspan="3" align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">8 eyes: phacoemulsification and IOL implantation</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " rowspan="3" align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">29.8 years</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " rowspan="3" align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Improvement in all</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">4 eyes glaucoma deterioration; \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">4 eyes corneal edema; \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">4 eyes corneal transplant \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead rowgroup " rowspan="3" align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Eden et al. [2014].</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " rowspan="3" align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Norway</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " rowspan="3" align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">26 Patients (52 eyes)</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">13 eyes: cataract surgery; \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " rowspan="3" align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">29 years (range: 4–63)</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " rowspan="3" align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">NR</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " rowspan="3" align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">30% associated keratopathy</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">8 eyes: cataract and glaucoma surgery; \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">4 eyes: glaucoma surgery \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead rowgroup " rowspan="2" align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Chang et al. [2014].</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " rowspan="2" align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Korea</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " rowspan="2" align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">63 eyes</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">4 eyes cataract surgery; \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " rowspan="2" align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">31 months (range: 6–66)</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " rowspan="2" align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Improvement in 4 eyes with primary IOL implantation; no improvement in the others.</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " rowspan="2" align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">6 eyes glaucoma (of which 4 treated with surgery)</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">11 eyes cataract surgery and IOL implantation \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Shiple et al. [2015]. \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">United States \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">99 patients \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">32 patients: cataract surgery \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">28.4 years (range: 4–51) \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">NR \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">NR \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Wang et al. [2017]. \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">China \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">10 patients (17 eyes) \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">17 eyes: phacoemulsification with yellow IOL implantation and TCA \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">25.4 years (range: 4–50) \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Improvement in all \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">None \t\t\t\t\t\t\n \t\t\t\t</td></tr></tbody></table> """ ] "imagenFichero" => array:1 [ 0 => "xTab2753642.png" ] ] ] ] "descripcion" => array:1 [ "en" => "<p id="spar0085" class="elsevierStyleSimplePara elsevierViewall">Demographic characteristics, visual outcomes and complications of the main studies on patients with cataract and congenital aniridia.</p>" ] ] ] "bibliografia" => array:2 [ "titulo" => "References" "seccion" => array:1 [ 0 => array:2 [ "identificador" => "bibs0005" "bibliografiaReferencia" => array:48 [ 0 => array:3 [ "identificador" => "bib0005" "etiqueta" => "1" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Genomic structure, evolutionary conservation and aniridia mutations in the human PAX6 gene" "autores" => array:1 [ 0 => array:2 [ "etal" => false "autores" => array:3 [ 0 => "T. 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