DOMANDA n. 5

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

Figure 1: The fundus photo demonstrates massive subretinal exudate in the macula of a child.

  1. What is your diagnosis of this lesion
  2. What is the natural history of this disease?
  3. Who are afflicted with this disease?
  4. How common is it for this condition to be bilateral?
  5. What is the pathogenesis of this condition?
  6. What might a fluorescein angiogram show?
  7. What is the treatment for this disease?
 

RISPOSTE

Figure 1: The fundus photo demonstrates massive subretinal exudate in the macula of a child.
  1. What is your diagnosis of this lesion
    Answer: The large amount of exudate in the macula is classic in appearance for Coats' disease

     

  2. What is the natural history of this disease?
    Answer: The disease tends to wax and wane with acute exacerbations and periods of quiescence, however, the course is typically progressive. There is increasing subretinal exudation with resultant exudative retinal detachment which may be quite extensive.

     

  3. Who are afflicted with this disease?
    Answer: Typically, the disease affects young children and adolescents. There is an adult form which is often associated with hypercholesterolemia. The male to female ratio is 3:1. There is no racial or ethnic predilection. There is no evidence of genetic transmission.

     

  4. How common is it for this condition to be bilateral?
    Answer: The disease is unilateral in 80% of patients.

     

  5. What is the pathogenesis of this condition?
    Answer: Coats' disease is felt to be due to an idiopathic loss of the blood retinal barrier. Electron microscopy reveals a loss of retnal vascular endothelium and pericytes.

     

  6. What might a fluorescein angiogram show?
    Answer: The fluorescein angiogram may highlight the retinal vascular abnormality. Vascular telangiectasia, and aneurysms may be the predominant feature or may be very subtle. The abnormal vessels tend to show early and persistent leakage. Areas of capillary drop-out may also be visualized.

     

  7. What is the treatment for this disease?
    Answer: Macula threatening exudate and early retinal detachment may be treated by photocoagulation. Large exudative detachments, however, may require drainage and treatment with laser or cryotherapy.