de Ribot Francesc March, de Ribot Anna March, Núñez Pérez Javier, Huang Joshua M, Huang Jordan J, Martinez Jaime D
Department of Ophthalmology, Otago University, Dunedin, New Zealand.
Department of Ophthalmology, Girona University, Girona, Spain.
Am J Ophthalmol Case Rep. 2025 Sep 2;40:102421. doi: 10.1016/j.ajoc.2025.102421. eCollection 2025 Dec.
Autoimmune polyendocrine syndromes (APS) are rare diseases characterized by immunologic activity against multiple endocrine organs. Bilateral keratitis and limbal stem cell deficiency are manifestations described.
A 28-year-old man presented to our service with discomfort in his left eye. Past medical history included autoimmune polyendocrine syndrome, under hormonal treatment. Ocular history included an idiopathic bilateral peripheral occlusive vascular retinal disease, with areas of non-perfusion, neovascularization, and bleeding, treated with laser photocoagulation. He presented with a vitreous hemorrhage which was treated with a 20-gauge pars plana vitrectomy and endolaser. On review of family history, the patient reported having an older brother also affected by APS, developing the same bilateral retinal affection.
Examination of the anterior segment of the left eye revealed a superior corneal epithelial irregularity. Fundus examination was significant for retinal arteries with segmental blood flow, box-carring, along with peripheral laser scars over previously ischemic areas. Corneal scraping and impression cytology demonstrated goblet cells on the corneal surface epithelium. A diagnosis of limbal stem cell insufficiency was made, and the patient was started on ocular lubricating drops and topical corticosteroids. At follow-up, the patient has been stable.
We describe a case of iatrogenic limbal stem cell insufficiency related to vitrectomy in a patient with autoimmune polyendocrine syndrome, a risk factor for this presentation. We propose that surgical trauma near the limbus and damage to the corneal sub-basal nerve plexus during vitrectomy may result in the development of limbal stem cell deficiency in a predisposed eye, as postulated in this patient with an APS.
自身免疫性多内分泌腺综合征（APS）是罕见疾病，其特征为针对多个内分泌器官的免疫活性。文献中描述了双侧角膜炎和角膜缘干细胞缺乏是其表现。
一名28岁男性因左眼不适前来就诊。既往病史包括自身免疫性多内分泌腺综合征，正在接受激素治疗。眼部病史包括特发性双侧周边闭塞性视网膜血管疾病，有非灌注区、新生血管形成和出血，曾接受激光光凝治疗。他出现玻璃体积血，接受了20G玻璃体切割术和眼内激光治疗。在询问家族病史时，患者报告其哥哥也患有APS，并出现了相同的双侧视网膜病变。
左眼眼前节检查发现角膜上皮上缘不规则。眼底检查显示视网膜动脉有节段性血流、箱样改变，以及先前缺血区域的周边激光瘢痕。角膜刮片和印迹细胞学检查显示角膜表面上皮有杯状细胞。诊断为角膜缘干细胞功能不全，患者开始使用眼部润滑滴眼液和局部糖皮质激素治疗。随访时，患者病情稳定。
我们描述了一例自身免疫性多内分泌腺综合征患者因玻璃体切割术导致医源性角膜缘干细胞功能不全的病例，这是出现这种情况的一个危险因素。我们提出，玻璃体切割术期间角膜缘附近的手术创伤和角膜基底膜下神经丛损伤可能导致易感眼发生角膜缘干细胞缺乏，正如本例患有APS的患者所推测的那样。
