Unilateral anterior persistent fetal vasculature in a child with blepharophimosis-ptosis-epicanthus inversus syndrome: A surgical challenge
Blepharophimosis-ptosis-epicanthus inversus syndrome (BPES) is a rare autosomal dominant genetic disease. It is clinically characterized by four major features; blepharophimosis, ptosis, epicanthus inversus, and telecanthus. We report a case of a 1-year-old female with BPES with unilateral anterior...
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| Format: | Article |
| Language: | English |
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Wolters Kluwer Medknow Publications
2016-01-01
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| Series: | Indian Journal of Ophthalmology |
| Subjects: | |
| Online Access: | http://www.ijo.in/article.asp?issn=0301-4738;year=2016;volume=64;issue=6;spage=469;epage=471;aulast=Kemmanu |
| _version_ | 1818316484691099648 |
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| author | Vasudha Kemmanu Pragnya Rathod Hemant Anaspure Naresh K Yadav |
| author_facet | Vasudha Kemmanu Pragnya Rathod Hemant Anaspure Naresh K Yadav |
| author_sort | Vasudha Kemmanu |
| collection | DOAJ |
| description | Blepharophimosis-ptosis-epicanthus inversus syndrome (BPES) is a rare autosomal dominant genetic disease. It is clinically characterized by four major features; blepharophimosis, ptosis, epicanthus inversus, and telecanthus. We report a case of a 1-year-old female with BPES with unilateral anterior persistent fetal vasculature (PFV). On examination, she was found to have all the clinical features of BPES, along with calcified and partially absorbed cataract with elongated ciliary processes in her left eye. B-scan of left eye showed attached retina with no evidence of posterior PFV. Systemic examination was normal. She underwent cataract surgery with primary posterior capsulotomy with intraocular lens implantation under general anesthesia. Literature search did not reveal any previous reports of unilateral anterior PFV and BPES. The clinical features, other associations, and the difficulties in the surgical management of this condition are discussed. |
| first_indexed | 2024-12-13T09:22:10Z |
| format | Article |
| id | doaj.art-2ea9f59a56a4421fb8bdb2ff165b68af |
| institution | Directory Open Access Journal |
| issn | 0301-4738 1998-3689 |
| language | English |
| last_indexed | 2024-12-13T09:22:10Z |
| publishDate | 2016-01-01 |
| publisher | Wolters Kluwer Medknow Publications |
| record_format | Article |
| series | Indian Journal of Ophthalmology |
| spelling | doaj.art-2ea9f59a56a4421fb8bdb2ff165b68af2022-12-21T23:52:42ZengWolters Kluwer Medknow PublicationsIndian Journal of Ophthalmology0301-47381998-36892016-01-0164646947110.4103/0301-4738.187681Unilateral anterior persistent fetal vasculature in a child with blepharophimosis-ptosis-epicanthus inversus syndrome: A surgical challengeVasudha KemmanuPragnya RathodHemant AnaspureNaresh K YadavBlepharophimosis-ptosis-epicanthus inversus syndrome (BPES) is a rare autosomal dominant genetic disease. It is clinically characterized by four major features; blepharophimosis, ptosis, epicanthus inversus, and telecanthus. We report a case of a 1-year-old female with BPES with unilateral anterior persistent fetal vasculature (PFV). On examination, she was found to have all the clinical features of BPES, along with calcified and partially absorbed cataract with elongated ciliary processes in her left eye. B-scan of left eye showed attached retina with no evidence of posterior PFV. Systemic examination was normal. She underwent cataract surgery with primary posterior capsulotomy with intraocular lens implantation under general anesthesia. Literature search did not reveal any previous reports of unilateral anterior PFV and BPES. The clinical features, other associations, and the difficulties in the surgical management of this condition are discussed.http://www.ijo.in/article.asp?issn=0301-4738;year=2016;volume=64;issue=6;spage=469;epage=471;aulast=KemmanuAnterior persistent fetal vasculatureblepharophimosis-ptosis-epicanthus inversus syndromecongenital cataract |
| spellingShingle | Vasudha Kemmanu Pragnya Rathod Hemant Anaspure Naresh K Yadav Unilateral anterior persistent fetal vasculature in a child with blepharophimosis-ptosis-epicanthus inversus syndrome: A surgical challenge Indian Journal of Ophthalmology Anterior persistent fetal vasculature blepharophimosis-ptosis-epicanthus inversus syndrome congenital cataract |
| title | Unilateral anterior persistent fetal vasculature in a child with blepharophimosis-ptosis-epicanthus inversus syndrome: A surgical challenge |
| title_full | Unilateral anterior persistent fetal vasculature in a child with blepharophimosis-ptosis-epicanthus inversus syndrome: A surgical challenge |
| title_fullStr | Unilateral anterior persistent fetal vasculature in a child with blepharophimosis-ptosis-epicanthus inversus syndrome: A surgical challenge |
| title_full_unstemmed | Unilateral anterior persistent fetal vasculature in a child with blepharophimosis-ptosis-epicanthus inversus syndrome: A surgical challenge |
| title_short | Unilateral anterior persistent fetal vasculature in a child with blepharophimosis-ptosis-epicanthus inversus syndrome: A surgical challenge |
| title_sort | unilateral anterior persistent fetal vasculature in a child with blepharophimosis ptosis epicanthus inversus syndrome a surgical challenge |
| topic | Anterior persistent fetal vasculature blepharophimosis-ptosis-epicanthus inversus syndrome congenital cataract |
| url | http://www.ijo.in/article.asp?issn=0301-4738;year=2016;volume=64;issue=6;spage=469;epage=471;aulast=Kemmanu |
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