DOMANDA n. 15

Scott Burk, MD/PhD
Massachusetts Eye and Ear Infirmary, Harvard Medical School, Boston, MA

Fig.1

Fig. 2

 

Figure 1-2: Anterior segment photographs of 2 different individuals with the same corneal condition.
  1. What is your diagnosis?
  2. What is the usual presentation for this condition?
  3. What other conditions may be confused with this disease process?
  4. What is the pathophysiology of this condition?
  5. What is the inheritance pattern of this condition?
  6. What treatment would you recommend?
  7. Does this condition recur in corneal transplants?

 

RISPOSTE

  1. What is your diagnosis?
    Answer: Lattice corneal dystrophy.

     

  2. What is the usual presentation for this condition?
    Answer: Recurrent corneal epithelial erosions usually beginning about the fourth decade of life, leading to progressive scarring and visual loss.

     

  3. What other conditions may be confused with this disease process?
    Answer: Prominent corneal nerves or vessels may simulate lattice lines. Recurrent erosions may resemble HSV keratitis.

     

  4. What is the pathophysiology of this condition?
    Answer: Deposition of amyloid material in the anterior stroma. Lattice corneal dystrophy may be isolated or associated with systemic amyloidosis. When corneal and systemic amyloidosis coexist this is sometimes referred to as the Meretoja variant.

     

  5. What is the inheritance pattern of this condition?
    Answer: Autosomal dominant.

     

  6. What treatment would you recommend?
    Answer: In the early stages of lattice dystrophy treatment focuses on minimizing recurrent erosive episodes with lubrication, as scarring progresses and surface irregularity begins to affect vision rigid gas permiable contact lenses may be helpful. When visual acuity is limited by superficial scarring, superficial keratectomy, excimer phototherapuetic keratectomy, and finally penetrating keratoplasty may be required.

     

  7. Does this condition recur in corneal transplants?
    Answer: Yes, recurrence of lattice dystrophy is common after corneal transplant.