Limbal Stem Cell Deficiency: Demographics and Clinical Characteristics of a Large Retrospective Series at a Single Tertiary Referral Center

Abstract

The aim of this study was to characterize a large cohort of patients presenting to a single referral center for limbal stem cell deficiency (LSCD).Methods: A retrospective chart review of all patients with a clinical diagnosis of LSCD from 2002 to 2015 was performed. Demographics, etiology, previous ocular surgeries, visual acuity, and treatment were assessed.Results: Seven hundred thirty-eight eyes of 434 patients (51.4% male subjects) were diagnosed with LSCD. The mean presenting age was 42.9 years, 70% presented with bilateral disease, and overall vision was poor. The most common etiologies were congenital aniridia (30.9%), chemical or thermal injuries (20.6%), contact lens (16.8%), Stevens-Johnson syndrome (SJS, 10.4%), and iatrogenic (7.3%). Congenital aniridia had a significantly increased association with glaucoma or ocular hypertension (P , 0.0001). Chemical or thermal injuries (P = 0.0007), SJS (P , 0.0001), and mucous membrane pemphigoid (P , 0.0001) had a significantly increased association with eyelid pathology. The mean logMAR best corrected visual acuity (analysis excluded eyes with light perception and no light perception) at presentation was 1.145 (;20/280). Keratoplasty performed (before presentation at our center) without first addressing the LSCD was seen in 80 eyes; all keratoplasties failed due to recurrence of the LSCD. Conclusions:Patients presenting with LSCD were on average middle aged without sex predominance. LSCD was most commonly bilateral, comprised a wide range of etiologies, and decreased vision substantially. Ocular comorbidities may need to be treated before treating the LSCD surgically. Finally, not addressing the LSCD (primary issue) first can result in keratoplasty failure.

Why Are Corneal Specialists Resistant to Treating Patients Who Have Severe Ocular Surface Disease With Limbal Stem Cell Deficiency?

“…Superficial keratectomy (with or without amniotic membrane) and phototherapeutic keratectomy can buy time with only short-term visual improvement for mild disease with anterior stromal scarring. 18 Ultimately, this strategy will be unsuccessful because it fails to address the underlying problem of limbal stem cell failure.…”

“…Penetrating keratoplasty (PK) is the most common erroneously applied corneal procedure we encounter for patients with total LSCD. 18 Corneal specialists should be well aware that there is no chance of success with a primary PK for these patients. 3,[18][19][20][21][22][23] The epithelium of the donor cornea only survives from days in many cases to a few months in the best scenario.…”

“…18 Corneal specialists should be well aware that there is no chance of success with a primary PK for these patients. 3,[18][19][20][21][22][23] The epithelium of the donor cornea only survives from days in many cases to a few months in the best scenario. The patient with total LSCD has no ability to produce a heathy corneal epithelium.…”

“…The total LSCD leads to failed PK due to ocular surface failure, inflammation, and neovascularization of the cornea with subsequent rejection and worse vision. 18,[24][25][26][27] With rejection comes a sensitized immune system to donor corneal antigens potentially sensitizing the host to future donors, thus making subsequent OSSTx more difficult. 28,29 This scenario is likely compounded in the setting of multiple PKs (ie, increased sensitization from additional antigen exposure) that are often performed for these patients.…”

Cataract surgery in setting of ocular surface disease

“…Early subtle sings of corneal conjunctivalization include late staining fluorescein pattern; the dye tends to pool on the affected area because the abnormal conjunctival/metaplastic epithelium layer tends to be thinner and lacks cell-cell tight junctions (Fig. 2) [25,26].…”