Background:Type 2 Peters ano maly is a rare anted or segment disorder characterized by central corneal leukoma with keratolenticular adhesion and cataract.Performing cataract surgery without corneal tissue transplanta...Background:Type 2 Peters ano maly is a rare anted or segment disorder characterized by central corneal leukoma with keratolenticular adhesion and cataract.Performing cataract surgery without corneal tissue transplantation in patients of type 2 Peters anomaly is extremely rare and challenging.We present a case of type 2 Peters anomaly treated by peeling off the adhesion without penetrating keratoplasty(PKP),in which restoration of corneal transparency is observed.Case presentation:An 11-month-old female infant of type 2 Peters anomaly presented with bilateral corneal opacity with distinct demarcation,keratolenticular adhesion and cataract,which was first noted at the age of 3 months.By peeling off the adhesion from comeal endothelium combined with lensectomy and vitrectomy,there was a gradual reduction in corneal opacity and improvement in visual acuity after surgery over a 2-year period.The visual acuity had improved from light perception preoperatively to 20/50 at the latest follow-up.No sight-threatening postoperative complications were noted.Conclusion:It is safe and effective to peel off the keratolenticular adhesion in patients of type 2 Peters anomaly presented with distinctly demarcated corneal opacity.展开更多
Background:Type 2 Peters anomaly is a rare anterior segment disorder characterized by central corneal leukoma with keratolenticular adhesion and cataract.Performing cataract surgery without corneal tissue transplantat...Background:Type 2 Peters anomaly is a rare anterior segment disorder characterized by central corneal leukoma with keratolenticular adhesion and cataract.Performing cataract surgery without corneal tissue transplantation in patients of type 2 Peters anomaly is extremely rare and challenging.We present a case of type 2 Peters anomaly treated by peeling off the adhesion without penetrating keratoplasty(PKP),in which restoration of corneal transparency is observed.Case presentation:An 11-month-old female infant of type 2 Peters anomaly presented with bilateral corneal opacity with distinct demarcation,keratolenticular adhesion and cataract,which was first noted at the age of 3 months.By peeling off the adhesion from corneal endothelium combined with lensectomy and vitrectomy,there was a gradual reduction in corneal opacity and improvement in visual acuity after surgery over a 2-year period.Her visual acuity had improved from light perception preoperatively to 20/50 at the latest follow-up.No sight-threatening postoperative complications were noted.Conclusion:It is safe and effective to peel off the keratolenticular adhesion in patients of type 2 Peters anomaly presented with distinctly demarcated corneal opacity.展开更多
AIM： To explore the molecular mechanisms in lens development and the pathogenesis of Peters anomaly in Smad4 defective mice. METHODS： Le-Cre transgenic mouse line was employed to inactivate Smad4 in the surface ect...AIM： To explore the molecular mechanisms in lens development and the pathogenesis of Peters anomaly in Smad4 defective mice. METHODS： Le-Cre transgenic mouse line was employed to inactivate Smad4 in the surface ectoderm selectively. Pathological techniques were used to reveal the morphological changes of the anterior segment in Smad4 defective eye. Immunohistochemical staining was employed to observe the expression of E-cadherin, N- cadherin and α-SMA in anterior segment of Smad4 defective mice and control mice at embryonic （E） day 16.5. Real-time quantitative polymerase chain reaction （qPCR） was performed to detect the expression of Snail, Zebl, Zeb2 and Twist2 in lens of Smad4 defective mice and control mice at E16.5. RESULTS： Conditional deletion of Smad4 on eye surface ectoderm resulted in corneal dysplasia, iridocorneal angle closure, corneolenticular adhesions and cataract resembling Peters anomaly. Loss of Smad4 function inhibited E-cadherin expression in the lens epithelium cells and corneal epithelium cells in Smad4 defective eye. Expression of N-cadherin was upregulated in corneal epithelium and corneal stroma. Both E-cadherin and N-cadherin were down-regulated at the future trabecular meshwork region in mutant eye. The qPCR results showed that the expression of Twist2 was increased significantly in the mutant lens （P〈0.01）. CONCLUSION： Smad4 is essential to eye development and likely a candidate pathogenic gene to Peters anomaly by regulating epithelial-mesenchymal transition. Twist2 can be regulated by Smad4 and plays an essential role in lens development.展开更多
文摘Background:Type 2 Peters ano maly is a rare anted or segment disorder characterized by central corneal leukoma with keratolenticular adhesion and cataract.Performing cataract surgery without corneal tissue transplantation in patients of type 2 Peters anomaly is extremely rare and challenging.We present a case of type 2 Peters anomaly treated by peeling off the adhesion without penetrating keratoplasty(PKP),in which restoration of corneal transparency is observed.Case presentation:An 11-month-old female infant of type 2 Peters anomaly presented with bilateral corneal opacity with distinct demarcation,keratolenticular adhesion and cataract,which was first noted at the age of 3 months.By peeling off the adhesion from comeal endothelium combined with lensectomy and vitrectomy,there was a gradual reduction in corneal opacity and improvement in visual acuity after surgery over a 2-year period.The visual acuity had improved from light perception preoperatively to 20/50 at the latest follow-up.No sight-threatening postoperative complications were noted.Conclusion:It is safe and effective to peel off the keratolenticular adhesion in patients of type 2 Peters anomaly presented with distinctly demarcated corneal opacity.
文摘Background:Type 2 Peters anomaly is a rare anterior segment disorder characterized by central corneal leukoma with keratolenticular adhesion and cataract.Performing cataract surgery without corneal tissue transplantation in patients of type 2 Peters anomaly is extremely rare and challenging.We present a case of type 2 Peters anomaly treated by peeling off the adhesion without penetrating keratoplasty(PKP),in which restoration of corneal transparency is observed.Case presentation:An 11-month-old female infant of type 2 Peters anomaly presented with bilateral corneal opacity with distinct demarcation,keratolenticular adhesion and cataract,which was first noted at the age of 3 months.By peeling off the adhesion from corneal endothelium combined with lensectomy and vitrectomy,there was a gradual reduction in corneal opacity and improvement in visual acuity after surgery over a 2-year period.Her visual acuity had improved from light perception preoperatively to 20/50 at the latest follow-up.No sight-threatening postoperative complications were noted.Conclusion:It is safe and effective to peel off the keratolenticular adhesion in patients of type 2 Peters anomaly presented with distinctly demarcated corneal opacity.
基金Supported by the National Natural Science Foundation of China(No.81470617No.81371003)Colleges and Universities Scientific Research Project of Liaoning Province,China(No.L2014305)
文摘AIM： To explore the molecular mechanisms in lens development and the pathogenesis of Peters anomaly in Smad4 defective mice. METHODS： Le-Cre transgenic mouse line was employed to inactivate Smad4 in the surface ectoderm selectively. Pathological techniques were used to reveal the morphological changes of the anterior segment in Smad4 defective eye. Immunohistochemical staining was employed to observe the expression of E-cadherin, N- cadherin and α-SMA in anterior segment of Smad4 defective mice and control mice at embryonic （E） day 16.5. Real-time quantitative polymerase chain reaction （qPCR） was performed to detect the expression of Snail, Zebl, Zeb2 and Twist2 in lens of Smad4 defective mice and control mice at E16.5. RESULTS： Conditional deletion of Smad4 on eye surface ectoderm resulted in corneal dysplasia, iridocorneal angle closure, corneolenticular adhesions and cataract resembling Peters anomaly. Loss of Smad4 function inhibited E-cadherin expression in the lens epithelium cells and corneal epithelium cells in Smad4 defective eye. Expression of N-cadherin was upregulated in corneal epithelium and corneal stroma. Both E-cadherin and N-cadherin were down-regulated at the future trabecular meshwork region in mutant eye. The qPCR results showed that the expression of Twist2 was increased significantly in the mutant lens （P〈0.01）. CONCLUSION： Smad4 is essential to eye development and likely a candidate pathogenic gene to Peters anomaly by regulating epithelial-mesenchymal transition. Twist2 can be regulated by Smad4 and plays an essential role in lens development.
