Evidence-Based Clinical Practice Guideline for Diagnosis, Treatment, and Follow-Up of Keratoconus Patients.

Abstract

Keratoconus is a progressive corneal ectatic disorder characterized by focal stromal thinning and conical protrusion, typically involving the central or paracentral cornea. Disease progression may lead to significant visual impairment and reduced quality of life. Reported global prevalence ranges from 0.17 to 40 cases per 1000 persons, reflecting differences in diagnostic criteria, imaging technologies, and study populations. Early detection, timely intervention, and risk-based follow-up are essential to limit progression and reduce visual morbidity. A systematic literature search did not identify existing clinical practice guidelines for keratoconus diagnosis and management with sufficient methodological quality to support adoption or adaptation. Therefore, a de novo evidence-based clinical practice guideline was developed. The objective was to provide recommendations for timely diagnosis, risk stratification, interventional treatment, and follow-up of pediatric and adult patients with keratoconus, aiming to standardize clinical criteria and support decision-making among healthcare professionals. The guideline was developed through systematic evidence synthesis and the Grading of Recommendations Assessment, Development and Evaluation (GRADE) approach to assess certainty of evidence and strength of recommendations. The evidence base included systematic reviews, diagnostic accuracy studies, randomized clinical trials, and observational studies, according to each clinical question. Recommendations addressed risk factors, diagnostic and staging criteria, progression assessment, corneal cross-linking protocols, intrastromal corneal ring segments, keratoplasty techniques, and individualized follow-up. Most recommendations were conditional, reflecting low or very low certainty of evidence in several domains. This guideline provides a practical framework for standardized keratoconus care while explicitly identifying areas where evidence remains limited and further research is needed.