Posters

Non-surgical management of exposure keratopathy in harlequin ichthyosis cicatricial ectropion

Poster Details

First Author: G.Kalantzis UK

Co Author(s):    E. Drimtzias   B. Chang           

Abstract Details

Purpose:

To describe a case of bilateral cicatricial ectropion repair in a harlequin baby with sight-threatening bilateral upper and lower eyelid retraction following the non-surgical use of hyaluronic acid.

Setting:

St James University Hospital, Leeds, UK.

Methods:

Interventional case report of a 4-month old baby with harlequin ichthyosis who developed severe corneal scarring due to corneal exposure and was treated with subcutaneous eyelid injections of hyaluronic acid.

Results:

A 4-month old boy with harlequin ichthyosis had severe bilateral upper- and lower-eyelid ectropion, despite treatment with oral retinoids and intense skin emollients. When corneal scarring started due to corneal exposure, he was treated with 1 mL of Restylane SubQ into each eyelid. Recurrence of ectropion necessitated repeat treatment at age 9 months and 16 months. At age 18 months, ectropion surgery (full-thickness skin grafts) was deemed necessary. The patient displaced one of the grafts postoperatively, resulting in recurrence of left corneal ulceration. A repeat dose of SubQ was again injected into the upper eyelid, achieving better corneal coverage.

Conclusions:

We present a case of bilateral corneal exposure due to cicatricial ectropia in a child with harlequin ichthyosis, which was managed with subcutaneous hyaluronic acid fillers before skin grafting. Our case underlines that the use of Restylane in patients with ichthyosis can be an effective, repeatable method of eyelid expansion that can act as a good temporizing method, often delaying the need for more invasive surgical procedures. FINANCIAL DISCLOUSRE: NONE