z-logo
open-access-imgOpen Access
Brittle cornea syndrome: A tale of three brothers
Author(s) -
Kunal Mandlik,
Rakesh A Betdur,
R Rashmita,
Shivananda Narayana
Publication year - 2022
Publication title -
indian journal of ophthalmology/indian journal of ophthalmology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.542
H-Index - 51
eISSN - 1998-3689
pISSN - 0301-4738
DOI - 10.4103/ijo.ijo_2894_21
Subject(s) - sclera , medicine , cornea , connective tissue disorder , ophthalmology , connective tissue , ehlers–danlos syndrome , dermatology , surgery , pathology
Brittle cornea syndrome (BCS) is a genetic connective tissue disorder with discernible ocular features such as blue scleral and thin cornea that predominantly presents in younger children. We herein describe cases of three siblings with BCS, two of whom presented to us with open globe injuries following trivial trauma. Clinical examination of the other eye in both showed diffusely thin corneas and blue sclera. A systemic evaluation revealed sensorineural hearing loss and hyperextensible joints. The third sibling was screened and found to have features concurrent with BCS. This report highlights the challenges faced in the management of ocular injuries and consecutive complications in these patients.

The content you want is available to Zendy users.

Already have an account? Click here to sign in.
Having issues? You can contact us here