Inherited retinal diseases(IRD)are a leading cause of blindness in the working age population.The advances in ocular genetics,retinal imaging and molecular biology,have conspired to create the ideal environment for es...Inherited retinal diseases(IRD)are a leading cause of blindness in the working age population.The advances in ocular genetics,retinal imaging and molecular biology,have conspired to create the ideal environment for establishing treatments for IRD,with the first approved gene therapy and the commencement of multiple therapy trials.The scope of this review is to familiarize clinicians and scientists with the current landscape of retinal imaging in IRD.Herein we present in a comprehensive and concise manner the imaging findings of:(I)macular dystrophies(MD)[Stargardt disease(ABCA4),X-linked retinoschisis(RS1),Best disease(BEST1),pattern dystrophy(PRPH2),Sorsby fundus dystrophy(TIMP3),and autosomal dominant drusen(EFEMP1)],(II)cone and cone-rod dystrophies(GUCA1A,PRPH2,ABCA4 and RPGR),(III)cone dysfunction syndromes[achromatopsia(CNGA3,CNGB3,PDE6C,PDE6H,GNAT2,ATF6],blue-cone monochromatism(OPN1LW/OPN1MW array),oligocone trichromacy,bradyopsia(RGS9/R9AP)and Bornholm eye disease(OPN1LW/OPN1MW),(IV)Leber congenital amaurosis(GUCY2D,CEP290,CRB1,RDH12,RPE65,TULP1,AIPL1 and NMNAT1),(V)rod-cone dystrophies[retinitis pigmentosa,enhanced S-Cone syndrome(NR2E3),Bietti crystalline corneoretinal dystrophy(CYP4V2)],(VI)rod dysfunction syndromes(congenital stationary night blindness,fundus albipunctatus(RDH5),Oguchi disease(SAG,GRK1),and(VII)chorioretinal dystrophies[choroideremia(CHM),gyrate atrophy(OAT)].展开更多
Background:To compare the arithmetic mean(M-SIA)and the summated vector mean of surgically induced astigmatism(SVM-SIA)according to the incision site after phakic intraocular lens(Visian implantable collamer lens(ICL)...Background:To compare the arithmetic mean(M-SIA)and the summated vector mean of surgically induced astigmatism(SVM-SIA)according to the incision site after phakic intraocular lens(Visian implantable collamer lens(ICL),STAAR Surgical)implantation.Methods:This study comprised 121 eyes of 121 consecutive patients undergoing ICL surgery through a 3.0-mm temporal or superior clear corneal incision.The magnitude and the axis of corneal astigmatism preoperatively and 3 months postoperatively were measured using an automated keratometer.The M-SIA and the SVM-SIA were determined according to the incision site.Results:The magnitude of corneal astigmatism significantly increased from 1.23±0.59 D preoperatively to 1.46±0.72 D postoperatively in the temporal incision group(Wilcoxon signed-rank test,P<0.001),but it significantly decreased from 1.09±0.36 D preoperatively to 0.86±0.41 D postoperatively in the superior incision group(P<0.001).The M-SIA was 0.48±0.30 D,and the SVM-SIA was 0.23±0.52 D at a meridian of 82°in the temporal incision group.The M-SIA was 0.57±0.30 D,and the SVM-SIA was 0.47±0.45 D at a meridian of 1°in the superior incision group.Conclusions:ICL implantation induces the M-SIA by approximately 0.5 D,but the SVM-SIA decreased to 50%and 80%of the M-SIA in magnitude through temporal and superior incisions,respectively.The direction of the SVM-SIA showed a tendency toward corneal flattening to the incisional site.It should be noted that the M-SIA is somewhat different from the SVM-SIA according to the incision site.展开更多
BackgroundTo compare the arithmetic mean (M-SIA) and the summated vector mean of surgically induced astigmatism (SVM-SIA) according to the incision site after phakic intraocular lens (Visian implantable collamer lens ...BackgroundTo compare the arithmetic mean (M-SIA) and the summated vector mean of surgically induced astigmatism (SVM-SIA) according to the incision site after phakic intraocular lens (Visian implantable collamer lens (ICL), STAAR Surgical) implantation.MethodsThis study comprised 121 eyes of 121 consecutive patients undergoing ICL surgery through a 3.0-mm temporal or superior clear corneal incision. The magnitude and the axis of corneal astigmatism preoperatively and 3 months postoperatively were measured using an automated keratometer. The M-SIA and the SVM-SIA were determined according to the incision site.ResultsThe magnitude of corneal astigmatism significantly increased from 1.23 ± 0.59 D preoperatively to 1.46 ± 0.72 D postoperatively in the temporal incision group (Wilcoxon signed-rank test, P < 0.001), but it significantly decreased from 1.09 ± 0.36 D preoperatively to 0.86 ± 0.41 D postoperatively in the superior incision group (P < 0.001). The M-SIA was 0.48 ± 0.30 D, and the SVM-SIA was 0.23 ± 0.52 D at a meridian of 82° in the temporal incision group. The M-SIA was 0.57 ± 0.30 D, and the SVM-SIA was 0.47 ± 0.45 D at a meridian of 1° in the superior incision group.ConclusionsICL implantation induces the M-SIA by approximately 0.5 D, but the SVM-SIA decreased to 50% and 80% of the M-SIA in magnitude through temporal and superior incisions, respectively. The direction of the SVM-SIA showed a tendency toward corneal flattening to the incisional site. It should be noted that the M-SIA is somewhat different from the SVM-SIA according to the incision site.展开更多
基金Supported by grants from the National Institute for Health Research Biomedical Research Centre at Moorfields Eye Hospital NHS Foundation Trust and UCL Institute of Ophthalmology,Macular Society(UK),Fight for Sight(UK),Onassis Foundation,Leventis Foundation,The Wellcome Trust(099173/Z/12/Z)Moorfields Eye Hospital Special Trustees,Moorfields Eye Charity,Retina UK,and the Foundation Fighting Blindness(USA).
文摘Inherited retinal diseases(IRD)are a leading cause of blindness in the working age population.The advances in ocular genetics,retinal imaging and molecular biology,have conspired to create the ideal environment for establishing treatments for IRD,with the first approved gene therapy and the commencement of multiple therapy trials.The scope of this review is to familiarize clinicians and scientists with the current landscape of retinal imaging in IRD.Herein we present in a comprehensive and concise manner the imaging findings of:(I)macular dystrophies(MD)[Stargardt disease(ABCA4),X-linked retinoschisis(RS1),Best disease(BEST1),pattern dystrophy(PRPH2),Sorsby fundus dystrophy(TIMP3),and autosomal dominant drusen(EFEMP1)],(II)cone and cone-rod dystrophies(GUCA1A,PRPH2,ABCA4 and RPGR),(III)cone dysfunction syndromes[achromatopsia(CNGA3,CNGB3,PDE6C,PDE6H,GNAT2,ATF6],blue-cone monochromatism(OPN1LW/OPN1MW array),oligocone trichromacy,bradyopsia(RGS9/R9AP)and Bornholm eye disease(OPN1LW/OPN1MW),(IV)Leber congenital amaurosis(GUCY2D,CEP290,CRB1,RDH12,RPE65,TULP1,AIPL1 and NMNAT1),(V)rod-cone dystrophies[retinitis pigmentosa,enhanced S-Cone syndrome(NR2E3),Bietti crystalline corneoretinal dystrophy(CYP4V2)],(VI)rod dysfunction syndromes(congenital stationary night blindness,fundus albipunctatus(RDH5),Oguchi disease(SAG,GRK1),and(VII)chorioretinal dystrophies[choroideremia(CHM),gyrate atrophy(OAT)].
文摘Background:To compare the arithmetic mean(M-SIA)and the summated vector mean of surgically induced astigmatism(SVM-SIA)according to the incision site after phakic intraocular lens(Visian implantable collamer lens(ICL),STAAR Surgical)implantation.Methods:This study comprised 121 eyes of 121 consecutive patients undergoing ICL surgery through a 3.0-mm temporal or superior clear corneal incision.The magnitude and the axis of corneal astigmatism preoperatively and 3 months postoperatively were measured using an automated keratometer.The M-SIA and the SVM-SIA were determined according to the incision site.Results:The magnitude of corneal astigmatism significantly increased from 1.23±0.59 D preoperatively to 1.46±0.72 D postoperatively in the temporal incision group(Wilcoxon signed-rank test,P<0.001),but it significantly decreased from 1.09±0.36 D preoperatively to 0.86±0.41 D postoperatively in the superior incision group(P<0.001).The M-SIA was 0.48±0.30 D,and the SVM-SIA was 0.23±0.52 D at a meridian of 82°in the temporal incision group.The M-SIA was 0.57±0.30 D,and the SVM-SIA was 0.47±0.45 D at a meridian of 1°in the superior incision group.Conclusions:ICL implantation induces the M-SIA by approximately 0.5 D,but the SVM-SIA decreased to 50%and 80%of the M-SIA in magnitude through temporal and superior incisions,respectively.The direction of the SVM-SIA showed a tendency toward corneal flattening to the incisional site.It should be noted that the M-SIA is somewhat different from the SVM-SIA according to the incision site.
文摘BackgroundTo compare the arithmetic mean (M-SIA) and the summated vector mean of surgically induced astigmatism (SVM-SIA) according to the incision site after phakic intraocular lens (Visian implantable collamer lens (ICL), STAAR Surgical) implantation.MethodsThis study comprised 121 eyes of 121 consecutive patients undergoing ICL surgery through a 3.0-mm temporal or superior clear corneal incision. The magnitude and the axis of corneal astigmatism preoperatively and 3 months postoperatively were measured using an automated keratometer. The M-SIA and the SVM-SIA were determined according to the incision site.ResultsThe magnitude of corneal astigmatism significantly increased from 1.23 ± 0.59 D preoperatively to 1.46 ± 0.72 D postoperatively in the temporal incision group (Wilcoxon signed-rank test, P < 0.001), but it significantly decreased from 1.09 ± 0.36 D preoperatively to 0.86 ± 0.41 D postoperatively in the superior incision group (P < 0.001). The M-SIA was 0.48 ± 0.30 D, and the SVM-SIA was 0.23 ± 0.52 D at a meridian of 82° in the temporal incision group. The M-SIA was 0.57 ± 0.30 D, and the SVM-SIA was 0.47 ± 0.45 D at a meridian of 1° in the superior incision group.ConclusionsICL implantation induces the M-SIA by approximately 0.5 D, but the SVM-SIA decreased to 50% and 80% of the M-SIA in magnitude through temporal and superior incisions, respectively. The direction of the SVM-SIA showed a tendency toward corneal flattening to the incisional site. It should be noted that the M-SIA is somewhat different from the SVM-SIA according to the incision site.
