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Am J Ophthalmol Case Rep. 2018 Apr 17;11:153-157. doi: 10.1016/j.ajoc.2018.04.007. eCollection 2018 Sep.

Advanced coats-like retinopathy as the initial presentation of Familial Retinal Arterial Macroaneurysms.

American journal of ophthalmology case reports

Sulaiman M Alsulaiman, Leen Abu-Safieh, Abdullah S AlJarallah, Majed AlAbdulhafid, Eman S AlKahtani

Affiliations

  1. Vitreoretinal Division King Khaled Eye Specialist Hospital, Al Oroubah St, Riyadh, Saudi Arabia.
  2. Cardiac Sciences Department, College of Medicine, King Saud University, King Khlaed Road, Riyadh, Saudi Arabia.
  3. Department of Pediatrics, College of Medicine, King Saud University, King Khaled Road, Riyadh, Saudi Arabia.

PMID: 30094394 PMCID: PMC6077123 DOI: 10.1016/j.ajoc.2018.04.007

Abstract

PURPOSE: To describe two young Saudi brothers with bilateral progressive retinal arterial aneurysms and a subtotal exudative retinal detachment with Coats-like presentation in the older sibling as the initial presentation of Familial Retinal Arterial Macroaneurysms (FRAM).

OBSERVATIONS: Two young Saudi brothers with a family history of consanguinity presented with the classic clinical presentation and genetic identification of FRAM. In this report, we describe the presence of prominent peripheral retinal capillary changes mimicking Coats' disease.

CONCLUSIONS AND IMPORTANCE: FRAM can present similar to bilateral Coats' disease and should be considered in the differential diagnosis of Coats-like retinopathy. The diagnosis of FRAM may have a significant implication because of the associated cardiac abnormality, such as supravalvular pulmonary stenosis, which should be evaluated by echocardiography and managed accordingly.

Keywords: Coats’-like retinopathy; Familial retinal arterial macroaneurysms (FRAM); Insulin-like growth factor binding protein 7 (IGFBP7); Retinal arterial aneurysms; Supravalvular pulmonic stenosis

References

  1. Retina. 2002 Oct;22(5):607-15 - PubMed
  2. Am J Hum Genet. 2011 Aug 12;89(2):313-9 - PubMed
  3. Arch Ophthalmol. 2007 Jun;125(6):840-2 - PubMed
  4. Brain. 1987 Jun;110 ( Pt 3):631-48 - PubMed
  5. Ann Neurol. 1982 Oct;12(4):395-8 - PubMed
  6. Ophthalmology. 2012 Mar;119(3):648-9 - PubMed
  7. J Am Coll Cardiol. 1990 Feb;15(2):292-9 - PubMed
  8. Am J Ophthalmol. 2001 May;131(5):572-83 - PubMed
  9. Neuromuscul Disord. 2009 Feb;19(2):140-2 - PubMed

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