Ma Jeffrey, Baqai Jeanine
Department of Ophthalmology, Feinberg School of Medicine, Northwestern University, 645 North Michigan Avenue, Suite 440, Chicago, IL, USA.
Am J Ophthalmol Case Rep. 2019 Jul 10;15:100513. doi: 10.1016/j.ajoc.2019.100513. eCollection 2019 Sep.
To report a case of severe bilateral descemetoceles in a patient with alpha-1 antitrypsin (A1AT) deficiency during intensive care unit hospitalization.
A 42-year-old male presented with sub-acute bilateral vision loss during an intensive care unit hospitalization following liver and kidney transplantations. On exam, this patient's best-corrected visual acuity was 20/80 in both eyes. There were bilateral descemetoceles inferotemporally in both eyes with overlying epithelial defects and dense surrounding punctate epithelial staining. The patient was initially treated with gatifloxacin drops and frequent lubricating ointment. Given the concern for impending perforation, cyanoacrylate glue with bandage contact lens was applied to both eyes. His best corrected visual acuity remained unchanged in the right eye and improved to 20/30 in the left eye. Upon medical stabilization, anterior lamellar graft was performed in the right eye, with plans for the same treatment in the left eye in the future.
As A1AT is found in the tear film and is believed to play a role in regulating protease activity in the cornea, we hypothesize that this patient's A1AT deficiency exacerbated the progression of corneal ulceration leading to severe descemetocele formation.
报告1例α-1抗胰蛋白酶（A1AT）缺乏患者在重症监护病房住院期间发生严重双侧角膜后弹力层膨出的病例。
一名42岁男性在肝肾移植后的重症监护病房住院期间出现亚急性双侧视力丧失。检查时，该患者双眼最佳矫正视力均为20/80。双眼颞下象限均有双侧角膜后弹力层膨出，伴有上皮缺损及周围密集的点状上皮染色。患者最初接受加替沙星滴眼液和频繁使用润滑眼膏治疗。鉴于担心即将发生穿孔，对双眼应用了氰基丙烯酸酯胶水和绷带式隐形眼镜。其右眼最佳矫正视力保持不变，左眼提高到20/30。病情稳定后，右眼进行了前板层移植，计划未来对左眼进行同样的治疗。
由于在泪膜中发现了A1AT，且认为其在调节角膜中的蛋白酶活性方面发挥作用，我们推测该患者的A1AT缺乏加剧了角膜溃疡的进展，导致严重的角膜后弹力层膨出形成。
