A New Consensus for Paediatric Keratoconus

Constant cooperation to support early diagnosis, good screening programmes, and immediate treatment are among the new European Reference Network (ERN) consensus recommendations for paediatric keratoconus, developed under the auspices of the European Commission.

Established by the European Commission seven years ago, the ERN virtually connects healthcare providers across Europe, particularly for the management of rare diseases. ERN-EYE focuses specifically on rare eye conditions.

“It is composed of 52 full members, covering more than 900 rare eye diseases in six main areas of interest: paediatric ophthalmology, retina, neuro-ophthalmology, anterior segment, genetic testing, and low vision,” Luca Buzzonetti MD explained.

In addition to connecting specialists, ERN-EYE supports clinical research, educational tools, multidisciplinary care, and partnerships with patient associations while helping to improve access to innovation, strengthen national integration, and develop guidelines.

For paediatric keratoconus, a clinical consensus statement was prepared by a team of 15 authors from eight European countries. Professor Buzzonetti served as team coordinator, working alongside three working groups, a medical writer, and a patient representative. The contributors represented the Czech Republic, Estonia, France, Germany, Italy, Lithuania, Slovenia, and Spain.

Prof Buzzonetti described the process as complex from the start, as the project involved several steps before completion. These included defining the target population, establishing a list of keywords, ranking the research questions by importance, and conducting the search, selection, and appraisal of scientific evidence. This was followed by drafting the consensus statements and analysing the Delphi process, which assessed the level of agreement with each recommendation using a five-point scale (0–100%). Statements with more than 75% average agreement were accepted, those with 25–74% agreement were revised, and statements with less than 25% agreement were excluded. The draft was then finalised and approved, leading to the release of the guidelines.

The consensus statement provides an overview of paediatric keratoconus, including its pathophysiology, diagnosis, risk factors, and classification. It emphasises early detection, regular monitoring, patient and family education, and appropriate treatment strategies. The goal is to help healthcare professionals deliver timely care and improve outcomes for affected children. The recommendations are based on a literature review of studies published between 2000 and 2024, using MEDLINE and EMBASE databases with specific keywords for each working group.

The key recommendations include close collaboration between paediatricians and ophthalmologists for early diagnosis; screening at-risk children using tools such as Scheimpflug imaging or retinoscopy; frequent patient monitoring every three to four months initially and then annually until the age of 18; immediate treatment upon early diagnosis; and using epi-off cross-linking as first-line treatment and deep anterior lamellar keratoplasty for advanced cases. The guideline also proposes a management algorithm covering prevention, disease progression, and visual rehabilitation.

International collaboration is still a crucial factor.

“Global initiatives could promote data sharing and the creation of common registries in the attempt to deepen our understanding of paediatric keratoconus and optimise patients’ outcomes,” Prof Buzzonetti said. “In our experience, a significant number of patients still present with a delayed diagnosis, thereby losing the potential benefits of early cross-linking treatment.”

Prof Luca Buzzonetti spoke at the 3rd World Keratoconus Congress 2026 in Florence, Italy.

Luca Buzzonetti MD is Head of the Ophthalmology Department at Bambino Gesù IRCCS Children’s Hospital, Rome, Italy.