DOMANDA n. 14

 

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


Fig. 1

Fig. 2

Fig. 3

 

Figure 1-3: Anterior segment photographs of 3 different individuals with the same corneal condition.
  1. What is your diagnosis?
  2. Will this process extend to involve the entire cornea?
  3. What is the inheritance pattern of this condition?
  4. When does this condition become evident?
  5. What are the symptoms of this condition?
  6. What would light microscopy show in this condition?
  7. What treatment would you recommend?

RISPOSTE

  1. What is your diagnosis?
    Answer: Discrete milky white, granular deposits in the anterior stroma are characteristic of granular dystrophy.

     

  2. Will this process extend to involve the entire cornea?
    Answer: No, granular dystrophy does not extend to the limbus.

     

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

     

  4. When does this condition become evident?
    Answer: The disease is evident early in life, but patients remain asymptomatic for years.

     

  5. What are the symptoms?
    Answer: Slowly progressive visual loss, rarely below 20/200. Recurrent erosions are rare.

     

  6. What would light microscopy show in this condition?
    Answer: Clumps of hyaline material concentrated in the anterior stroma that stains well with Masson's trichrome stain.

     

  7. What treatment would you recommend?
    Answer: In the early stages granular dystrophy no treatment is needed. When visual acuity is limited, penetrating keratoplasty offers a relatively good prognosis though the disease may recur after many years.