“Fish-eye” Babies: Surgical Management of Ocular Complications in Lamellar Ichthyosis

Lamellar ichthyosis is a rare congenital keratinization disorder, characterized by generalized scaling, inflammation, and significant ocular involvement. Ocular involvement, particularly cicatricial ectropion and lagophthalmos, results from anterior lamellar shortening and exposes the ocular surface to desiccation, recurrent epithelial breakdown, and potential visual loss. Conventional skin-based reconstructive options are frequently limited due to widespread skin involvement. We present two pediatric cases that were surgically managed using vision-preserving techniques. The first underwent bilateral posterior lamellar tarsorrhaphy (PLT) to address lagophthalmos and progressive corneal thinning. The second child was treated with anterior lamellar lengthening using buccal mucous membrane grafts (MMGs) due to the unavailability of healthy skin. Both cases achieved stable eyelid positioning, complete resolution of exposure, and preserved visual potential. These cases underscore the importance of early surgical decision-making and demonstrate how PLT and MMG serve as valuable alternatives in the oculoplastic management of ichthyosis, particularly when conventional grafting is not feasible.