Surgical management of ichtyosis-related ectropion. Description of four cases and a literature review

Abstract

Ichtyosis is an heterogeneous group of diseases characterized by formation of hyperkeratotic, squamous, tile-like scales virtually on the whole body. Depending on the severity and localization of the symptoms, the disease may lead to serious complications including ectropion of the eyelids, which may further lead to ocular disorders, including blindness. The aim of the work was to outline major characteristics of the entity and to focus on surgical methods of ichtyosis-related eyelid ectropion management. Four cases of ichtyosis-related eyelid ectropion, which occurred in patients presenting severe symptoms of the disease treated surgically in the clinic between 2003-2011, were described. In all patients the eyelid contractures were successfully released and relief of conjunctival inflammation was obtained. No postoperative complications occurred. In two patients, a secondary operation was necessary due to skin graft contracture and ectropion recurrence. In some cases, ichtyosis may lead to serious ocular complications. Surgical management of eyelid ectropion is challenging due to the shortage of available lesion-free skin for grafting and due to possible skin graft contracture leading to recurrence.

Management of congenital ichthyoses: European guidelines of care, part two

“…It should ideally be undertaken before keratinization of the palpebral conjunctiva occurs. The main issue is relapse, which may occur rapidly, and subsequent topical therapy remains necessary. Autologous skin grafts are the most commonly reported surgical interventions.…”

Ocular manifestations, complications and management of congenital ichthyoses: a new look

“…Regardless of harvest site, repeat surgery due to skin graft contracture is often necessary. Zachara et al 86 reported four patients who required 19 surgical graft procedures, 10 of which were to upper eyelids involving autografts harvested from inner arm, postauricular, supraclavicular and groin sites. Split-thickness skin grafts in a 25-year-old patient have also been reported 87.…”

Successful correction of ichthyosis‐related ectropion by autografts

“…2 The most widespread ocular manifestation is cicatricial ectropion and mostly seen as lamellar type. 3,4 Cicatricial ectropion can be congenital or can be seen in the further phases of life. 5 As characteristics of ichthyosis disease, trans epidermal dehydration as well as contractions and loss of elasticity in eyelid skin arise as a result of compensatory hyperproliferation.…”

“…[5][6][7] Other ocular manifestations such as lagophthalmos, conjunctival hyperemia, exposure keratopathy, recurrent corneal ulcer mostly develop due to cicatricial ectropion. [3][4][5][6]8 Furthermore, the disease itself may also cause ocular manifestations with systemic involvement. 3 Patients complain about photophobia, pain, tearing, foreign body sensation and loss of vision.…”