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BRIEF COMMUNICATION
Year : 2016  |  Volume : 64  |  Issue : 6  |  Page : 469-471

Unilateral anterior persistent fetal vasculature in a child with blepharophimosis-ptosis-epicanthus inversus syndrome: A surgical challenge


1 Department of Pediatric Ophthalmology and Strabismus, Narayana Nethralaya, Super Specialty Eye Hospital and Post Graduate Institute of Ophthalmology, Bengaluru, Karnataka, India
2 Department of Pediatric Ophthalmology and Strabismus, Narayana Nethralaya, Bengaluru, Karnataka, India
3 Department of Vitreo Retina Services, Narayana Nethralaya, Bengaluru, Karnataka, India

Correspondence Address:
Vasudha Kemmanu
Department of Pediatric Ophthalmology and Strabismus, Narayana Nethralaya, Super Specialty Eye Hospital and Post Graduate Institute of Ophthalmology, 121/C, Chord Road, Rajajinagar, 1st "R" Block, Bengaluru - 560 010, Karnataka
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0301-4738.187681

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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.


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