In India, hundreds of children are born with a genetic condition called ‘Peters anomaly’ (PA), a rare, congenital disorder that leads to corneal opacities at birth. If untreated, it can leave a child without vision for the rest of their life. PA typically affects the central, front layers of the cornea, leading to various ‘zones’ of corneal opacities. In infants with PA, the front portion of the eye is defectively developed due to genetic abnormalities. Sometimes, the iris and cornea adhere at various points, or the lens can fail to fully separate from the cornea during development. In people with PA, parts of the corneal endothelium—a single layer cell lining that is a barrier that maintains the cornea’s hydration and transparency—is abnormal leading to fluid retention and corneal opacities.
The standard way to treat an eye affected by PA is a ‘full-thickness’ corneal transplant, a Penetrating Keratoplasty (PK). However, PK is a challenging and complex procedure which requires high-quality donor corneas. It has many post-operative challenges, and so it has only modest success.
A previous review from LVPEI, among others, report 50-100% graft failures over time and other post-surgical complications. There is an urgent need for an alternative that can save the sight of these infant children.
A new paper by Dr Muralidhar Ramappa and others in the Ophthalmic & Physiological Optics presents evidence for a new surgical technique, “Selective Endothelialectomy in Peters Anomaly” (SEPA), as a viable alternative. In SEPA, only the abnormal region of the endothelium is selectively removed instead of a full transplant. The central idea of this approach is that once the abnormal layers are selectively removed, neighboring cells migrate or enlarge into the gap and restore visual function.
The paper reviews the surgical outcomes of 34 eyes of 28 children who underwent SEPA over eight years (2012-2019). 17 children (60.7%) were male, while a third had parental consanguinity. 29 eyes (85%) showed mild to moderate corneal clearing, indicating endothelial healing. Visual outcomes continue to improve in 32 eyes (94%) over time. This is strong evidence for the viability of this approach.
‘Three separate studies have completed SEPA or its iterations in 120 children with Peters anomaly,’ says Dr Muralidhar Ramappa, Faculty Ophthalmologist, Child Sight Institute at LVPEI and SEPA’s inventor. ‘Our long-term experience has shown that SEPA is a safe, effective, and superior surgical alternative to full-thickness corneal transplantation in children with a broad spectrum of corneal opacities due to Peters anomaly. At LVPEI, we believe SEPA would benefit hundreds of children with PA-related corneal blindness worldwide.’