Dr.Rama Bharathi Nela

Dr.BOWVIA RAJKUMARI BALU , Dr.Rama Bharathi Nela

Abstract

Purpose:To describe the ophthalmic manifestations in a series of children with congenital lamellar ichthyosis. These cases presented with cicatricial ectropion , hyperkeratotic lesions, madarosis, trichiasis, scales on the lashes, meibomitis, abnormal movements of the upper eyelids, cicatricial lagophthalmos,superficial corneal changes and chronic keratoconjunctivitis.The clinical features and ophthalmic management were studied.
Methods: The case histories of two children presenting were reviewed. All were diagnosed with congenital lamellar ichthyosis.Examination for visual acuity, eyelid and eyelash malposition, lid function and closure done. Corneal examination for exposure was done.
Conclusion : Congenital lamellar ichthyosis is a heterogeneous disorder . The most common eyelid abnormality is cicatricial ectropion of the upper and mainly lower eyelids. Most cases are managed conservatively with surgical correction