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Posterior Segment Characterization in Children With Pierson Syndrome
Ophthalmic Surgery, Lasers & Imaging Retina
|November 24, 2020
Summary
Pierson syndrome, a rare genetic disorder, presents with significant posterior segment abnormalities, including high myopia and incomplete retinal vascularization. Early detection of complications like retinal detachment is crucial for management.
Area of Science:
- Ophthalmology
- Genetics
- Retinal Diseases
Background:
- Pierson syndrome is a rare genetic disorder characterized by congenital nephrotic syndrome and microcoria.
- Ocular manifestations, particularly posterior segment and retinal features, are not well-defined.
Purpose of the Study:
- To describe the posterior segment and retinal characteristics in patients with Pierson syndrome.
- To highlight the importance of thorough ophthalmic examination in managing this condition.
Main Methods:
- Retrospective chart review of nine patients diagnosed with Pierson syndrome.
- Ophthalmic history, ocular examination, retinal imaging, and surgical interventions were analyzed.
- Median follow-up duration was 17 months.
Main Results:
- All nine patients (16 eyes) exhibited high myopia with tessellated fundus, optic disc pallor, and abnormal retinal vascularization.
- Parapapillary chorioretinal atrophy was observed in 75% of eyes.
- Rhegmatogenous retinal detachment (RRD) occurred in 7 eyes, with recurrence after surgical repair in all cases.
Conclusions:
- Pierson syndrome is associated with high axial myopia and incomplete peripheral retinal vascular maturation.
- Regular posterior segment examination is vital for identifying RRD and neovascularization.
- Management of RRD in Pierson syndrome is challenging due to high recurrence rates.
