Management Of Unilateral Keratoconus Presenting As Acute Hydrops

To report a rare instance of unilateral keratoconus presenting as acute hydrops and the management strategies adopted for it

Department of Ophthalmology, Jawaharlal Institute of Postgraduate Medical Education and Research (JIPMER), Puducherry

A sixteen-year-old girl presented with sudden onset vision loss in the right eye associated with watering for 3 days. She reported progressive diminution of vision in her right eye for the past 6 months. On examination her BCVA was OD:1/60, OS:6/6; the right eye showed extreme paracentral corneal thinning (334µm) central corneal edema with bullae, normal left eye. The left eye topography showed a near normal cornea (K1: 44.3D, K2: 46.2D; astigmatism: 1.8D at 157◦, thinnest local: 503µm). The right eye AS-OCT revealed Descemet’s membrane breach with intrastromal fluid clefts in the central cornea, confirming corneal hydrops due to keratoconus. Right eye intracameral isoexpansile C3F8 was injected with minimal resolution at 1 week, so, compression sutures were additionally placed, and the hydrops resolved with central corneal scarring. However, she developed cataract in her right eye and her BCVA was 2/60.  She underwent right eye phacoemulsification with posterior chamber IOL implantation in the bag, but BCVA improved to only 3/60 due to the extreme corneal thinning and central corneal scar. Right eye optical penetrating keratoplasty was done (graft 7.5mm, host 7mm). Visual rehabilitation was done by selective suture removal and custom contact lenses. Her BCVA is 6/9 in the right eye (at 2 years follow-up)

Our case highlights the challenging clinical course of aggressive unilateral keratoconus presenting as hydrops while the contralateral eye remained in a quiescent phase. Descemetopexy with C3F8 failed and also led to iatrogenic cataract, while compression sutures helped in the early resolution of the hydrops