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International Ophthalmology

, Volume 38, Issue 5, pp 2179–2182 | Cite as

Full-thickness macular hole associated with congenital simple hamartoma of retinal pigment epithelium (CSHRPE)

  • Panagiotis Stavrakas
  • Athanasios Vachtsevanos
  • Efthymia Karakosta
  • Nikos Kozeis
  • Magdalini Triantafylla
  • Paraschos Tranos
Case Report

Abstract

Purpose

To report a unique case of congenital simple hamartoma of retinal pigment epithelium (CSHRPE) associated with full-thickness macular hole (FTMH).

Methods

A 14-year-old female with a previous diagnosis of simple hamartoma of retinal pigment epithelium in the left eye presented with a 3-week onset of reduced visual acuity in the same eye. Complete ophthalmological examination with spectral domain ocular coherence tomography (SD-OCT) and autofluorescence imaging was carried out. Results were compared with the last 4-year follow-up data.

Results

On examination, visual acuity in the left eye was counting fingers compared to 20/100 documented in the last follow-up. Fundoscopy revealed a nodular, heavily pigmented, circumscribed foveal mass, with 0.5 mm basal diameter and 1.1 mm thickness corresponding to the simple RPE hamartoma. Although the latter remained unchanged throughout the follow-up period, a new lesion at the inferior edge of the hamartoma was noted. SD-OCT confirmed the presence of a FTMH with no evidence of associated epiretinal membrane, vitreomacular traction or posterior vitreous detachment. After explaining the risks and benefits of vitrectomy for FTMH, the patient declined surgery.

Conclusions

CSHRPE may have associated features such as feeding vessels, retinal surface wrinkling, pigmented vitreous cells, retinal exudation and vitreoretinal adhesion. The case presented herein broadens the clinical spectrum of CSHRPE and emphasizes that despite the benign nature of the lesion, which remains stable without growth, visual loss may occur, attributable to foveal traction and development of FTMH. We postulate that protrusion of the tumor into the vitreous cavity results in tissue stretching and development of anteroposterior and tangential forces which may lead to development of macular hole.

Keywords

Congenital simple hamartoma of retinal pigment epithelium Full-thickness macular hole Benign tumor of the retina Vitreoretinal traction 

Notes

Compliance with ethical standards

Conflict of interest

The authors declare that they have no conflict of interest.

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Copyright information

© Springer Science+Business Media B.V. 2017

Authors and Affiliations

  1. 1.2nd Department of Ophthalmology, National and Kapodistrian University Athens Medical School“Attikon” University General HospitalsAthensGreece
  2. 2.“Ophthalmica” Institute of OphthalmologyThessalonikiGreece

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