Diagnosis and management of limbal stem cell deficiency, challenges, and future prospects

ABSTRACT

Introduction: Corneal limbal stem cell deficiency is a multifaceted, sight-threatening disease process that can be challenging to diagnose and treat.

Areas covered: This review discusses normal corneal limbal stem cell health, causes and diagnosis of limbal stem cell deficiency, and the medical and surgical management of limbal stem cell deficiency.

Expert opinion: Recently established consensus guidelines for the diagnosis and management of limbal stem cell deficiency will help improve the diagnosis, management, and collaborative study design regarding this disease. When medical management fails, a variety of autologous and allogenic limbal stem cell transplant techniques may be tried. However, these treatments potentially have high morbidity and may be short-lived. Newer strategies of ex vivo cell culture and keratoprosthesis implants may help improve visual outcomes in patients faced with bilateral limbal stem cell deficiency.

KEYWORDS:

• Limbal stem cell deficiency
• limbal stem cell transplantation
• amniotic membrane
• cultured limbal epithelial transplantation

Declaration of interest

The authors have no relevant affiliations or financial involvement with any organization or entity with a financial interest in or financial conflict with the subject matter or materials discussed in the manuscript. This includes employment, consultations, honoraria, stock ownership or options, expert testimony, grants or patents received or pending, or royalties.

Reviewer disclosures

Peer reviewers on this manuscript have no relevant financial or other relationships to disclose.

Article highlights

• The Cornea Society’s Limbal Stem Cell Working Group has established consensus guidelines for the diagnosis and management of limbal stem cell deficiency to provide consistency in the diagnosis, staging, and management of limbal stem cell deficiency and to facilitate the ability to compare research in this field

• While impression cytology is still the gold standard for diagnosis of limbal stem cell deficiency, imaging modalities such as anterior segment optical coherence tomography and in vivo confocal microscopy are being increasingly utilized to improve diagnostic accuracy

• Early medical management may prevent progression of limbal stem cell deficiency and its sequelae

• Autologous limbal stem cell transplantation techniques can restore limbal stem cell health in patients with unilateral limbal stem cell deficiency and obviate the need for systemic immunosuppression, but carry the risk of iatrogenic limbal stem cell deficiency

• Patients with bilateral limbal stem cell deficiency require allogenic limbal stem cell transplantation or Keratoprosthesis, which have high rates of complication, require systemic immunosuppression, and may not be successful long term

• New techniques for ex-vivo cell culture and Keratoprosthesis implants are being developed to address the needs of patients with bilateral limbal stem cell deficiency