Limbal stem cell deficiency (LSCD) is a corneal condition in which the cornea partially or completely loses its regenerative ability. Extensive stem cell loss in the limbal zone leads to permanent corneal epithelial defects and vision loss due to conjunctivalization.

Acquired LSCD is more common compared to primary, genetically driven LSCD. Frequent causes of acquired pediatric LSCD include ocular trauma (e.g., chemical, mechanical, or thermal), autoimmune disease (e.g., Stevens-Johnson syndrome), and chronic allergy (e.g., atopic keratoconjunctivitis). Congenital causes of LSCD include Peters anomaly and aniridia.

Because limbal stem cells do not regenerate, children who have LSCD may experience reduced visual acuity, photophobia, epiphora, blepharospasm, redness associated with chronic inflammation, and recurring attacks of pain due to epitheliopathy. In advanced cases of LSCD, pannus formation, chronic keratitis, scarring, and calcification may occur.

Management

Treatment options include cessation of the offending agent if possible, frequent use of artificial tears, topical corticosteroids, punctal occlusion, autologous serum tears, amniotic membrane grafting, and limbal autografting. In severe bilateral cases, limbal allografting can be considered, but potentially toxic systemic immunosuppressive medications are needed on a lifelong basis (Deng et al, 2020).

Another option for pediatric LSCD is therapeutic scleral lenses, which vault over and protect the cornea and the limbus while providing a healthy aqueous environment for stem cells. Additionally, they improve vision in pediatric LSCD by neutralizing irregular astigmatism from resultant corneal abnormalities (Kojima et al, 2016).

Consider a 15-year-old Caucasian female who had a history of congenital aniridia (Figure 1A), causing LSCD from aniridic keratopathy (Figure 1B). This patient presented with visual acuity of 20/100 and a prescription of +8.00 –3.00 x 180 in both eyes. Due to progressive LSCD corneal changes, this patient was prescribed a large-diameter scleral lens in combination with autologous serum tears for protection of the ocular surface (Figure 1C). This patient’s vision improved to 20/70 with scleral contact lenses.

Summary

LSCD is a rare but serious condition that can quickly turn debilitative. Scleral lenses are a valid treatment option even in pediatric patients and are amenable to combination approaches. CLS

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