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The eye is a spherical, hollow structure composed of three tissue layers. The outer layer — the fibrous tunic, comprises the sclera — a white structure — and the cornea, which is transparent. The sclera encompasses some of the ocular surface, most of which is not visible. However, the 'white of the eye' is distinctively visible in humans compared to other species. The cornea, a clear covering at the front of the eye, enables light penetration. The eye's middle...
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CONCENTRIC MACULAR RINGS SIGN IN COMBINED HAMARTOMA OF RETINA AND RETINAL PIGMENT EPITHELIUM.

Lívia da Silva Conci1, Renata Martins Maia1, Rodrigo Pessoa Cavalcanti Lira2

  • 1Department of Ophthalmology, University of Sao Paulo, Sao Paulo, Brazil.

Retinal Cases & Brief Reports
|March 12, 2024
PubMed
Summary
This summary is machine-generated.

Combined hamartoma of the retina and retinal pigment epithelium can present with a concentric macular rings sign. This finding may be associated with specific retinal layer changes, as seen in two case reports.

Keywords:
hamartomaneoplasmsretinaretinal pigment epithelium

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Area of Science:

  • Ophthalmology
  • Retinal Imaging
  • Medical Case Reports

Background:

  • Combined hamartoma of the retina and retinal pigment epithelium (CHRPE) is a rare condition.
  • Diagnostic imaging plays a crucial role in understanding CHRPE.
  • The concentric macular rings sign has not been extensively described in relation to CHRPE.

Purpose of the Study:

  • To describe the concentric macular rings sign in two patients diagnosed with combined hamartoma of the retina and retinal pigment epithelium.
  • To correlate imaging findings with clinical presentation in these cases.

Main Methods:

  • Case report methodology.
  • Utilized spectral domain optical coherence tomography (SD-OCT) for detailed retinal imaging.
  • Employed optical coherence tomography angiography (OCTA) to assess vascularity.
  • Included clinical examination and ultra-widefield imaging.

Main Results:

  • Two patients (26-year-old female with neurofibromatosis type 2, 14-year-old male with diabetes mellitus type 1) presented with CHRPE.
  • Both patients exhibited the concentric macular rings sign on imaging.
  • SD-OCT revealed retinal thickening, disorganization, and a dentate pattern in the outer plexiform and Henle fiber layers.
  • OCTA showed an absent foveal avascular zone in both affected eyes.

Conclusions:

  • Combined hamartoma of the retina and retinal pigment epithelium may be associated with alterations at the outer plexiform and Henle fiber layer interface.
  • The concentric macular rings sign is a potential imaging finding in CHRPE.
  • Further research is warranted to understand the pathogenesis and clinical significance of this sign.