Serious corneal complications and undiagnosed floppy eyelid syndrome; A case series and a 10-year retrospective review

PURPOSE: To describe three individuals with severe keratitis and a substantial delay before floppy associated eyelid syndrome (FES) was identified, and to estimate the prevalence of severe corneal disease in individuals with FES. METHODS: We defined severe keratitis as corneal ulceration, vascularization or scar that affected vision. We recorded the clinical characteristics, the duration of symptoms before the diagnosis of FES, subsequent management and outcome. Then, to determine the proportion of individuals with FES who had severe corneal disease, we interrogated the Moorfields Eye Hospital electronic patient record (EPR) for the diagnosis of FES made in the ten-year interval from 2008. RESULTS: Three individuals presented with severe progressive keratitis (median duration of symptoms 19 months, range 2–48 months). All were male and with a high body mass index (BMI, range 38.9–41.2). In each the etiology of the keratitis was unclear before FES was identified. All had very lax lids and were aware they had periods of lid malposition during sleep. None mentioned symptoms of obstructive sleep apnoea (OSA) until they or their partner were directly questioned. The management of keratitis included both medical and surgical corneal treatments, with tarsorrhaphy and lid shortening surgery. We identified an additional 104 cases of FES from the EPR, of which 4 (3.8%) had severe keratitis. CONCLUSIONS: FES can be missed unless signs of lid laxity are directly elicited. A delay in diagnosis can result in clinical deterioration, with unnecessary investigations and treatments. An assessment for FES should be included as part of the evaluation of individuals with severe or chronic keratitis.