Idiopathic maculopathy in eyes with regressed retinopathy of prematurity

Graefes Arch Clin Exp Ophthalmol. 2010 Aug;248(8):1097-103. doi: 10.1007/s00417-010-1355-4. Epub 2010 Mar 23.


Background: To describe idiopathic maculopathy in eyes with regressed retinopathy of prematurity, which differs from cicatricial changes in retinopathy of prematurity.

Setting: institutional.

Patients: patients were former preterm infants who had undergone fundus examinations for retinopathy of prematurity between December 1993 and May 2002. Posterior polar cicatricial change was excluded. The medical records of eight eyes (four patients) with photo-documented idiopathic maculopathy were reviewed retrospectively.

Main outcome measures: complete ophthalmologic examinations including best corrected visual acuity (BCVA), refractive error, funduscopic examination, fluorescein angiography (FAG), and optical coherence tomography (OCT).

Results: Maculopathy was characterized by depigmented geographic atrophy. FAG showed window defects due to atrophy of retinal pigment epithelium. OCT showed depression in the fovea with retained internal architecture. In all cases patients had retinopathy of prematurity, and all except one had been treated with laser photocoagulation. In the case without laser photocoagulation, maculopathy was observed on the first exam. In the other cases, no maculopathy was observed on the first exam, but was detected after laser photocoagulation. No patient had a family history of hereditary retinal dystrophy. In all cases, maculopathy was noted in both eyes with myopia. BCVA was variable (0.0 approximately 0.82, 0.32 +/- 0.32 logMAR). No progression was observed over the course of 8 years.

Conclusion: Idiopathic maculopathy is a rare posterior polar change that differs from the cicatricial changes seen in retinopathy of prematurity, and is independent of prior laser photocoagulation. No progression was observed, and visual prognosis was fair.

MeSH terms

  • Adolescent
  • Atrophy
  • Child
  • Electrooculography
  • Electroretinography
  • Female
  • Fluorescein Angiography
  • Gestational Age
  • Humans
  • Infant, Newborn
  • Infant, Premature
  • Laser Coagulation
  • Macula Lutea / pathology*
  • Male
  • Refractive Errors / diagnosis
  • Retinal Diseases / diagnosis
  • Retinal Diseases / etiology*
  • Retinal Pigment Epithelium / pathology
  • Retinopathy of Prematurity / complications*
  • Retinopathy of Prematurity / diagnosis
  • Retrospective Studies
  • Tomography, Optical Coherence
  • Visual Acuity / physiology