A Case Of Corneal Perforation In Acute Hydrops Secondary To Keratoconus

We present a rare case of corneal perforation in acute hydrops. This patient was seen and managed in the outpatient ophthalmology department. Keratoconus is a progressive corneal condition which features central corneal thinning and protrusion. Corneal hydrops is an acute complication in which Descemet’s membrane breaks under tension then retracts or coils due to its elastic structure. Aqueous can then enter the weakened stroma developing cystic spaces which cause marked corneal oedema.¹ It is a typically self-limiting condition with oedema resolving over 2-4 months as Descemet’s reattaches to overlying stroma and the endothelium migrates to cover defects.

Corneal perforation in acute corneal hydrops is uncommon.² Rarely, the process of intrastromal clefts can result in a track through from anterior chamber to epithelium in which aqueous humour can leak. Previous cases have notably been treated with corneal glue or grafting.³ Pressure patching, bandage contact lens and hypotensive agents can also be used as more conservative treatment.² 

A 38-year-old female presented acutely with sudden onset of left eye pain, light sensitivity and reduced vision. A longstanding history of unilateral keratoconus with poor vision in the left eye and atopic conjunctivitis was noted. The patient was referred  to the on-call ophthalmology registrar who reviewed her in the outpatient setting. On examination with slit lamp the conjunctiva was injected and patient was highly photosensitive. Cornea was noted to have central corneal oedema. When fluorescein was instilled a brisk seidel-positive aqueous leak was noted at an area central to the corneal oedema. The anterior chamber was noted to be deep with a regular shape to pupil and no hypopyon. Red reflex was visible but no further fundal examination possible. In the acute setting a silicon hydrogel bandage contact lens was applied and patient was commenced on topical antibiotics and cycloplegics. Anterior segment OCT showed the break in Descemet’s and helped to visualise the intrastromal clefts from corneal oedema and the tract they formed to allow aqueous fluid to pass through. At follow up 1 week later the corneal oedema persisted, but the aqueous leak had spontaneously resolved. The patient is still undergoing follow up. If corneal stromal oedema persists then a further management option would be to consider an intracameral injection of SF6 gas to seal the Descemet’s break.

Corneal perforation in acute hydrops and keratoconus is a rare occurrence. The structural changes that occur during corneal hydrops can be examined using imaging techniques such as anterior segment OCT. This can also help monitor progress and response to treatment.¹ In this case these images helped to visualise the source of aqueous leak and allowed us to plan management accordingly. We were able to manage conservatively with bandage contact lens and the patient’s condition settled without the need for urgent surgical intervention.