Progressive keratoconus

Specialty: Ophthalmology.

• progressive corneal ectasia
• progressive cone cornea
• progressive corneal thinning

Why it occurs

• Primary progressive bilateral keratoconus (ectatic disease of multifactorial etiology with genetic component)
• Chronic and vigorous eye rubbing (traumatic mechanism of cellular microtrauma, frequently associated with atopic keratoconjunctivitis, chronic pruritus or blepharitis)
• Down syndrome (presents a high incidence of keratoconus due to collagen laxity and constant rubbing)
• Floppy Eyelid Syndrome (lax eyelid that causes frictional keratopathy and ectasia)
• Marfan syndrome or Ehlers-Danlos syndrome (associated with laxity and fragility in collagen fibers)

Initial workup

Computerized corneal topography (anterior and posterior elevation maps, and quantitative pachymetric map to evaluate curvature and rule out localized thinning of the corneal stroma); Ultrasonic corneal pachymetry; Slit lamp biomicroscopy looking for typical signs (Vogt's striae in the stroma, epithelial Fleischer ring due to iron deposits at the base of the cone, or corneal vertex scar).

Standard management

• 5% hypertonic sodium chloride in ophthalmic ointment — apply a thin layer to the conjunctival sac every 12 hours in the event of acute corneal dropsy, in order to osmotically dehydrate the cornea and accelerate the resolution of the edema
• Olopatadine — 0.2% eye drops, 1 drop per day in the conjunctival sac to effectively control ocular itching in patients with atopy or allergic conjunctivitis, completely eradicating the habit of eye rubbing
• Artificial tears without preservatives — apply every 4 hours to reduce corneal friction
• Riboflavin (vitamin B2 eye drops used in the operating room to soak the cornea during the surgical procedure of corneal cross-linking by UVA light, indicated to structurally stabilize progressive keratoconus).