May 2008
Volume 49, Issue 13
Free
ARVO Annual Meeting Abstract  |   May 2008
Coats Like Disease and Retinitis Pigmentosa: Casual Association or Inherited Defect?
Author Affiliations & Notes
  • S. Putano
    Ophthalmology, Univ La Sapienza of Rome, Rome, Italy
  • E. Perrotta
    Ophthalmology, Univ La Sapienza of Rome, Rome, Italy
  • D. Domanico
    Ophthalmology, Univ La Sapienza of Rome, Rome, Italy
  • E. Vingolo
    Ophthalmology, Univ La Sapienza of Rome, Rome, Italy
  • Footnotes
    Commercial Relationships  S. Putano, None; E. Perrotta, None; D. Domanico, None; E. Vingolo, None.
  • Footnotes
    Support  None.
Investigative Ophthalmology & Visual Science May 2008, Vol.49, 2183. doi:https://doi.org/
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      S. Putano, E. Perrotta, D. Domanico, E. Vingolo; Coats Like Disease and Retinitis Pigmentosa: Casual Association or Inherited Defect?. Invest. Ophthalmol. Vis. Sci. 2008;49(13):2183. doi: https://doi.org/.

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      © ARVO (1962-2015); The Authors (2016-present)

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Abstract

Purpose: : Coats Like disease is rarely associated to retinitis pigmentosa, even if is still difficult to understand whether or not this association is accidental or due to a genetically determined mechanism. In this view our two patients from an ADRP family may contribute to understand this pathology.

Methods: : An ADRP pedigree with 10 patients mean age 26 y.o. (range 4-63 y.o.)affected was first examined in 1992, Two siblings of IV generation were also affected by Coats Like disease. Both patients underwent a complete ophthalmologic examination including BCVA, anterior and posterior segment examination FA, VF and ERG. This patients were followed up for a period of 15 years. Each parameters was recorded and statistically analized

Results: : We observed the ADRP pedigree of three generations and two brothers of generation IV presented a retinal capillary anomalies with massive essudation and retinal non rhegmatogenous detachment in both eyes in infero-temporal quadrant suggestive Coats Like disease. The mother of this patients affected by RP did not presented any exudative response.

Conclusions: : In our ADRP pedigree only two patients on ten had a Coats like disease, so we think that as observed in other cases of chorio-retinal scars, a vascular shunt between choriocapillary and retinal system may cause an increasing of blood flow in the capillary network determining the exudative response. In this view we consider that Coats Like disease and Retinitis Pigmentosa are two different disease whose association is not statistically significative.

Keywords: retinal degenerations: hereditary • retinal pigment epithelium • degenerations/dystrophies 
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