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

Abstract:

: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.

journal_name

Indian J Ophthalmol

authors

Kemmanu V,Rathod P,Anaspure H,Yadav NK

doi

10.4103/0301-4738.187681

subject

Has Abstract

pub_date

2016-06-01 00:00:00

pages

469-71

issue

6

eissn

0301-4738

issn

1998-3689

pii

IndianJOphthalmol_2016_64_6_469_187681

journal_volume

64

pub_type

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