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Neurotrophic Keratitis in a Pediatric Patient With Goldenhar Syndrome and Trigeminal Aplasia Successfully Treated by Corneal Neurotization.
Ophthalmic Plastic and Reconstructive Surgery ( IF 1.2 ) Pub Date : 2021-11-16 , DOI: 10.1097/iop.0000000000002086
Angel Rollon-Mayordomo 1 , Beatriz Mataix-Albert 2 , Francisco Espejo-Arjona 2 , Javier Herce-Lopez 1 , Leticia Lledo-Villar 2 , Carlos Caparros-Escudero 3 , Pedro Infante-Cossio 4
Affiliation  

Herein, the authors report an unusual case of a 6-year-old boy with right-sided Goldenhar syndrome and trigeminal nerve aplasia who developed neurotrophic keratopathy (NK). Despite the use of therapeutic contact lenses and multiple temporary tarsorrhaphy, NK worsened showing a central corneal scar, neovascularization, and significant stromal thinning, with risk of corneal perforation. Cochet-Bonnet esthesiometry revealed complete corneal anesthesia. To minimize additional corneal complications, the patient underwent indirect corneal neurotization by a sural nerve autograft anastomosed to the contralateral supratrochlear nerve. At 24-month follow up, no epithelial defects, complications, or recurrence were observed. Significant improvements in corneal sensitivity with esthesiometry score of 20 mm and reflex blinking were achieved. This case highlights corneal anesthesia should be suspected among Goldenhar syndrome ophthalmologic abnormalities and monitored before corneal changes become irreversible. Since corneal neurotization can successfully improve corneal sensation, it could be considered as an early therapeutic option to avoid refractory NK.

中文翻译:

角膜神经化成功治疗了一名患有 Goldenhar 综合征和三叉神经发育不全的小儿患者的神经营养性角膜炎。

在此,作者报告了一个不寻常的病例,一名 6 岁男孩患有右侧 Goldenhar 综合征和三叉神经发育不全,并发展为神经营养性角膜病 (NK)。尽管使用治疗性隐形眼镜和多次临时性睑板缝合术,NK 仍恶化,出现中央角膜疤痕、新生血管和显着的基质变薄,并有角膜穿孔的风险。Cochet-Bonnet 审美测量显示角膜完全麻醉。为了尽量减少额外的角膜并发症,患者通过与对侧滑车上神经吻合的自体腓肠神经进行间接角膜神经化。24 个月随访时,未观察到上皮缺陷、并发症或复发。角膜敏感性显着改善,美学评分达到 20 毫米,反射性眨眼也得到改善。该病例强调,在 Goldenhar 综合征眼科异常中应怀疑角膜麻醉,并在角膜变化变得不可逆转之前进行监测。由于角膜神经化可以成功改善角膜感觉,因此可以将其视为避免难治性 NK 的早期治疗选择。
更新日期:2021-11-16
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