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Idiopathic maculopathy in eyes with regressed retinopathy of prematurity
Kyoung Min Lee1, Jeong Hun Kim, Young Suk Yu
1Department of Ophthalmology, College of Medicine, Seoul National University, Seoul, 110-744, Republic of Korea.
Insights
Idiopathic maculopathy in former preterm infants with regressed retinopathy of prematurity presents as geographic atrophy. This rare condition, distinct from cicatricial changes, shows no progression over time.
Area of Science:
- Ophthalmology
- Retinal Diseases
- Pediatric Ophthalmology
Background:
- Idiopathic maculopathy is a rare posterior pole condition observed in eyes with regressed retinopathy of prematurity.
- It is crucial to differentiate this entity from cicatricial changes associated with retinopathy of prematurity.
Purpose of the Study:
- To describe the clinical characteristics of idiopathic maculopathy in eyes with regressed retinopathy of prematurity.
- To distinguish idiopathic maculopathy from cicatricial changes in retinopathy of prematurity.
Main Methods:
- Retrospective review of medical records and fundus photographs of eight eyes (four patients) with idiopathic maculopathy.
- Exclusion of posterior polar cicatricial changes.
- Ophthalmologic examinations included visual acuity, refractive error, funduscopy, fluorescein angiography (FAG), and optical coherence tomography (OCT).
Main Results:
- Maculopathy presented as depigmented geographic atrophy with FAG showing RPE window defects.
- OCT revealed foveal depression with preserved internal retinal architecture.
- All patients had retinopathy of prematurity, with maculopathy detected after laser photocoagulation in most cases; it was bilateral and associated with myopia.
- Visual acuity varied, and no progression was observed over an 8-year follow-up.
Conclusions:
- Idiopathic maculopathy is a distinct posterior polar change in regressed retinopathy of prematurity, separate from cicatricial changes.
- The condition appears independent of prior laser photocoagulation treatment.
- No progression was noted, suggesting a fair visual prognosis.
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.
