Keratoconus associated with the Pierre Robin sequence

Abstract

A 30-year-old female with known Pierre Robin sequence presented to the emergency department of the Royal Victorian Eye and Ear Hospital, Melbourne, with acute unilateral hydrops as her first presentation of bilateral keratoconus. Initial management consisted of eye patching, hypertonic saline and cycloplegia. However, the long-term prognosis for visual rehabilitation remained poor in this patient because of the presence of a central corneal opacity and inability to comply with contact lenses. She was not an appropriate candidate for keratoplasty due to severe mental retardation. This report highlights an association of keratoconus in patients with the Pierre Robin sequence.