Spontaneous Regression Of Corneal Hydrops In A Down’S Syndrome Patient

Keratoconus is an ectatic corneal disorder, classically described as progressive, non inflammatory and characterised by central corneal thinning, protrusion and irregular myopic astigmatism. Acute corneal hydrops is an incompletely-understood complication of keratoconus, characterised by marked corneal oedema caused by a break in Descemet membrane, allowing aqueous to enter the corneal stroma and epithelium. We present the case of spontaneous regression of a coreal hydrops in a patient with Down’s sydrom

 This is an 18-year-old patient with Down's syndrome and a poorly monitored heart defect who presented to the emergency department with a unilateral drop in visual acuity of the left eye with tearing and photophobia.

Ophthalmological examination revealed a total corneal opacity of the OG, with some secretions; the rest of the examination was unremarkable; examination of the right eye appeared normal.

An OCT of the cornea showed a rupture of the descmet with odéma in the stroma and epithelium confirming the diagnostic of corneal hydrops: stage 5a keratoconus.A therapeutic decision was made to perform a predescmesic suture with gas injection into the anterior chamber; while awaiting the patient's conditioning, he was placed on topical corticoids and hypotonic medications . The patient's family refused surgery.

The patient returned 7 days after the 1st consultation, with an almost total disappearance of the corneal opacity.

The prevalence of keratoconus has been reported to vary in different studies internationally, from 8.8 to 54.4 per 100 000. Corneal hydrops is relatively uncommon and is estimated to occur in 2.6 – 2.8% of patients with keratoconus.

Although acute hydrops is usually self-limiting and clinical signs of oedema typically resolve after two to four months, it often leaves a vision-impairing scar necessitating and expediting the need for corneal transplantation. In some cases, corneal neovascularisation may occur and this has significant implications on the patient’s future management and prognosis. In our case ; the oedema regressed in 7 days with a small central corneal scar . 

Corneal hydros is a complication of keratoconus ; Down’s syndrome is a factor risk of complication . Effective management of acute corneal hydrops in keratoconus is based on recognising and addressing the risk factors, treating the acute event effectively and promptly to reduce the duration of oedema and its complications, and ultimately successful corneal transplantation with acceptable long-term graft survival rates.