Rollon-Mayordomo Angel, Mataix-Albert Beatriz, Espejo-Arjona Francisco, Herce-Lopez Javier, Lledo-Villar Leticia, Caparros-Escudero Carlos, Infante-Cossio Pedro
Department of Oral and Maxillofacial Surgery, Virgen Macarena University Hospital, Seville, Spain.
Department of Ophthalmology, Virgen Macarena University Hospital, Seville, Spain.
Ophthalmic Plast Reconstr Surg. 2022;38(2):e49-e51. doi: 10.1097/IOP.0000000000002086.
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.
在此，作者报告了一例罕见病例，一名6岁男孩患有右侧Goldenhar综合征和三叉神经发育不全，并发神经营养性角膜病变（NK）。尽管使用了治疗性隐形眼镜并多次进行临时性睑裂缝合术，但NK仍恶化，出现中央角膜瘢痕、新生血管形成和明显的基质变薄，存在角膜穿孔风险。Cochet-Bonnet感觉测量显示角膜完全麻醉。为尽量减少额外的角膜并发症，患者接受了腓肠神经自体移植与对侧滑车上神经吻合的间接角膜神经化手术。在24个月的随访中，未观察到上皮缺损、并发症或复发情况。角膜感觉显著改善，感觉测量评分为20毫米，出现反射性眨眼。该病例强调，在Goldenhar综合征的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，因此可将其视为避免难治性NK的早期治疗选择。
