Case report
Sight-threatening Complication of Cicatricial Ectropion in a Patient with Lamellar Ichthyosis – Case Report
Natália Rybárová
orcid.org/0000-0002-7019-6390
; 1Department of Ophthalmology, University Hospital Brno, Brno, Czech Republic
Blanka Pinková
; School of Medicine, Masaryk University, Brno, Czech Republic
Hana Došková
; Department of Ophthalmology and Optometry, St. Anne’s University Hospital, Brno, Czech Republic
Eva Vlková
; 1Department of Ophthalmology, University Hospital Brno, Brno, Czech Republic
Abstract
We report a case of lamellar ichthyosis and sight-threatening
complications of cicatricial ectropion in an adult male patient which was surgically
managed with tectonic penetrating keratoplasty. We present a case
of autosomal-recessive lamellar ichthyosis in a 47-year-old man who was referred
to our outpatient eye clinic for treatment of primary keratouveitis of
the right eye with keratolysis and exudation in the anterior chamber. A diagnosis
of cicatricial ectropion with serious lagophthalmos was established
on examination. The patient underwent tectonic penetrating keratoplasty,
cataract extraction, and intra-ocular lens placement with no perioperative
complications. The patient was subsequently treated with oral fluconazole
200 mg once daily for 12 days due to a positive fungal culture for Candida
albicans and systemic oral acyclovir 250 mg 3 times per day for 12 days as prophylaxis
for a labial herpetic infection. Post-operative complications included
corneal rejection and nonhealing neurotropic epithelial defect of the graft.
Long-term treatment with topical cyclosporine (Ikervis®) and dexamethasone
led to resolution of the corneal rejection. Lubrication with artificial tears
containing hyaluronic acid, perfluorohexyl octane (Evotears®), and vitamin A
ointment led to symptomatic relief of dry eye disease. The patient was referred
to a dermatologist and was started on systemic retinoid acitretin at
a dose of 0.5 mg/kg per day. Ten months after surgery, the patient’s visual
acuity was 0.1 based on the Snellen chart and the corneal graft was stable.
Infection in the cornea can rapidly progress to corneal melting in patients
with severe cicatricial ectropion. A good patient outcome depends on the
interdisciplinary approach to patient management by the ophthalmologist,
dermatologist, and plastic surgeon.
Keywords
ichthyosis, corneal perforation, ectropion, penetrating keratoplasty
Hrčak ID:
246742
URI
Publication date:
18.1.2020.
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