Natália Rybárová, MD, Oční klinika FN Brno, Jihlavská 20, 625 00 Brno, Czech Republic;
Acta Dermatovenerol Croat. 2020 Jul;28(1):29-33.
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.
我们报告了一例成人男性板层状鱼鳞病和瘢痕性睑外翻导致的视力威胁性并发症，该患者通过组织穿透性角膜移植术进行了手术治疗。我们介绍了一例常染色体隐性板层状鱼鳞病病例，患者为 47 岁男性，因右眼原发性角膜炎伴角膜溶解和前房渗出而被转诊至我们的门诊眼科诊所。检查发现存在严重的睑裂闭合不全的瘢痕性睑外翻。患者接受了组织穿透性角膜移植术、白内障摘除术和人工晶状体植入术，无围手术期并发症。随后，由于白色念珠菌的真菌培养阳性，患者接受了 12 天的每日口服氟康唑 200mg 治疗，并接受了 12 天的每日口服阿昔洛韦 250mg 3 次的全身预防唇疱疹感染治疗。术后并发症包括角膜排斥反应和移植物的神经源性上皮缺损不愈合。长期使用环孢素（Ikervis®）和地塞米松滴眼剂治疗导致角膜排斥反应得到解决。使用含有透明质酸、全氟己烷（Evotears®）和维生素 A 软膏的人工泪液进行润滑，缓解了干眼症的症状。患者被转介给皮肤科医生，并开始每天服用 0.5mg/kg 的全身性维甲酸阿维 A 酯治疗。术后 10 个月，患者的视力基于 Snellen 图表为 0.1，角膜移植物稳定。严重瘢痕性睑外翻患者的角膜感染可迅速进展为角膜溶解。良好的患者预后取决于眼科医生、皮肤科医生和整形外科医生对患者管理的跨学科方法。
