Limbal stem cell deficiency secondary to vitrectomy in the context of an autoimmune polyendocrine syndrome type

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.