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The management of Keratoconus in an Irish Intellectual Disability Population

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First Author: K.Stephenson IRELAND

Co Author(s):    B. Power   D. Malata   B. Quill   C. Murphy   W. Power        

Abstract Details

Purpose:

To assess an intellectual disability (ID) cohort in Ireland including systemic and ocular (visual acuity and tomography) clinical characteristics, prevalence of keratoconus (KC) and to describe an appropriate clinical algorithm for assessment and treatment of KC in this cohort.

Setting:

The study was carried out at a single tertiary referral ophthalmic hospital (the Royal Victoria Eye & Ear Hospital, Dublin, Ireland) under the care of three consultant ophthalmic / corneal surgeons.

Methods:

A search of operating theatre and clinic electronic records identified eligible patients (search terms: corneal collagen crosslinking (CXL), general anaesthetic (GA) and KC). Retrospective chart review was conducted assessing corneal diagnosis, visual acuity (VA) and tomography data between treated and untreated groups. ID subtype and severity along with systemic syndromic effects were documented. Statistical analysis was performed via SPSS (IBM Corporation) comprising 1-way ANOVA tests. This study abided by local guidelines regarding general data protection regulations.

Results:

Mean age of the 24 patients was 31.9 years with 66.2% male. ID type was Down Syndrome (T21, 66.6%), autism (25%) and other (8.3%). History of atopy was confirmed in 33.3%. KC was diagnosed in 98% of eyes with 45.8% having untreatable advanced disease (33.3% bilateral), 39.6% amenable to CXL (20.8% bilateral) and 6.3% having corneal transplantation. There were no severe systemic adverse events despite 37.5% congenital heart defects in the T21 cohort. Two eyes had delayed epithelial healing (10.5%). VA was significantly better for CXL group (LogMAR 0.58) than advanced group (1.21), p=0.02.

Conclusions:

KC is strikingly prevalent in the ID population. Ireland has the highest rate of Down Syndrome in Europe (26.3:10,000 live births) thus there is a large population at risk of corneal blindness. Visual loss contributes to intellectual disability and dementia progression. This group is rarely suitable for corneal transplantation and CXL is a safe and effective intervention to prevent corneal blindness in this already socially restricted group. We propose an algorithm for investigation and treatment of KC in ID and also advocate early screening via a paediatric Down Syndrome clinic to catch KC while non-invasive treatment is an option.

Financial Disclosure:

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