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M.L. Livani, C. Teodori, F. Tortelli, R. Malagola, E.M. Vingolo; Coat's Like Disease Associated to Retinitis Pigmentosa: Casual Association or Inherited Defect? . Invest. Ophthalmol. Vis. Sci. 2003;44(13):4853.
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Purpose: Coat's like disease is rarely associated with retinitis pigmentosa, even if actually is still difficult to understand if this association may be occasional or may be due to a genetically determined mechanism. In this view our two patients from an ADRP family may contribute to understand this pathology. Material: an ADRP pedigree with 10 affected patients was examined firs time in 1992 two individual of generation IV were also affected by Coat’s Like disease. Both patients were followed for a period of 10 years with a complete ophthalmological examination including FA, VF and ERG testing. Results: we observed the main pedigree of three generation and both affected patients presented a retinal capillary anomalies with massive exudation and retinal non-rhegmatogenous detachment in OU in infero temporal quadrant. Mother of these patients affected by RP did not presented any exudative response. Discussion Coat’s like disease was observed in literature in different incidence up 3,6% of patients. Our experience shows that even if microteleangectasies are frequently observed in tapetoretinal degenerations exudation and retinal detachment are less frequent, only to these two main symtoms are specifically present in the Coat’s like disease. Moreover these findings are not inherited in fact in our pedigree only two patients on ten had this charachteristic, so we think that, as observed in other cases of chorio-retinal scars, a vascular shunt between choriocapillary and retinal system may cause a surcharge of blood flow in the capillary network starting the exudative response. In this view we consider that Coat’s like disease and Retinitis Pigmentosa are two different entities casually associated.
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