J Korean Ophthalmol Soc > Volume 60(12); 2019 > Article
Journal of the Korean Ophthalmological Society 2019;60(12):1323-1328.
DOI: https://doi.org/10.3341/jkos.2019.60.12.1323    Published online December 15, 2019.
Ocular Manifestations, Treatment Outcome and Clinical Course of Infective Keratitis of Keratitis-Ichthyosis-Deafness Syndrome.
Ki Young Son, Dong Won Paik, Eun Hye Cho, Daehwan Shin, Hye In Woo, Tae Young Chung, Dong Hui Lim
1Department of Ophthalmology, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea. ldhlse@gmail.com
2Department of Laboratory Medicine and Genetics, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea.
3Department of Ophthalmology, Kangbuk Samsung Hospital, Sungkyunkwan University School of Medicine, Seoul, Korea.
4Department of Preventive Medicine, Graduate School of Medicine, The Catholic University of Korea, Seoul, Korea.
키드증후군의 안과 증상 및 감염각막염 치료와 경과에 대한 증례
손기영1 · 백동원1 · 조은혜2 · 신대환3 · 우혜인2 · 정태영1 · 임동희1,4
성균관대학교 의과대학 삼성서울병원 안과학교실1, 성균관대학교 의과대학 삼성서울병원 진단검사의학교실2, 성균관대학교 의과대학 강북삼성병원 안과학교실3, 가톨릭대학교 의학전문대학원 예방의학교실4
Correspondence:  Dong Hui Lim,
Email: ldhlse@gmail.com
Received: 20 June 2019   • Revised: 23 July 2019   • Accepted: 6 December 2019
Abstract
PURPOSE
To report the ocular manifestations and treatment outcomes of infective keratitis in a patient with keratitis-ichthyosis-deafness (KID) syndrome and to report a newly discovered mutation in the GJB2 gene in Korea. CASE SUMMARY: A 32-month-old boy was referred to the Ophthalmology Clinic for bilateral corneal opacity and glare. The patient showed alopecia, hyperkeratotic skin in both limbs, and hearing loss in both ears. Ocular examination showed loss of eyebrows and eyelashes, hyperkeratotic lesions of the eyelids, mucopurulent discharge in the eyelids, and opacity and scarring with superficial vascularization and conjunctivalization in both corneas. Molecular analysis showed a pathogenic variant in the GJB2 gene and confirmed the diagnosis of KID syndrome. Superficial keratectomy and amniotic membrane transplantation were performed in both eyes. Corneal opacity recurred in the left eye; treatment with bevacizumab eyedrops was instituted and penetrating keratoplasty was performed in the left eye. Corneal stromal opacity relapsed in the left eye; 5% vancomycin and 5% ceftazidime eyedrops were started and methicillin-resistant Staphylococcus aureus was cultured from a corneal scraping specimen. CONCLUSIONS: A careful observation of ocular manifestations in KID syndrome is needed to prevent infective keratitis and limbal cell deficiency; intensive antibiotic eyedrop treatment is recommended to prevent permanent visual impairment.
Key Words: Eye manifestations;Keratitis;Keratitis-Ichthyosis-Deafness syndrome


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