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COMBINED HAMARTOMA OF THE RETINA AND RETINAL PIGMENT EPITHELIUM AT PEDIATRIC AGE: Surgical Versus Conservative
Sengul Ozdek1, Ahmet Yucel Ucgul1,2, M Elizabeth Hartnett3
1Department of Ophthalmology, Gazi University, School of Medicine, Ankara, Turkey.
Retina (Philadelphia, Pa.)
|October 13, 2022
Summary
Pars plana vitrectomy improved vision and reduced retinal distortion in pediatric patients with combined hamartoma of the retina and retina pigment epithelium. Conservative management showed poorer outcomes.
Area of Science:
- Ophthalmology
- Retinal Diseases
- Pediatric Ophthalmology
Background:
- Combined hamartoma of the retina and retina pigment epithelium (CHRPE) is a rare condition affecting children.
- Management strategies range from conservative observation to surgical intervention.
Purpose of the Study:
- To compare outcomes of pediatric patients with CHRPE managed conservatively versus those treated with pars plana vitrectomy.
- To evaluate the efficacy of surgical intervention in improving visual acuity and reducing retinal distortion.
Main Methods:
- Retrospective multicenter study of 62 eyes from 59 pediatric patients with CHRPE.
- Patients were divided into surgical (pars plana vitrectomy) and conservative management groups.
- Data collected included visual acuity, optical coherence tomography (OCT) findings, central foveal thickness, and tumor location (Zone 1 vs. Zone 2).
Main Results:
- Pars plana vitrectomy group showed significant improvement in best-corrected visual acuity (68.8% vs. 12.9% in conservative group, P < 0.001).
- Central foveal thickness decreased in the surgical group but increased in the conservative group.
- Posterior tumor location, irregular foveal contour, OCT ellipsoid zone defects, subretinal exudate, and vascular tortuosity were associated with poor visual acuity.
Conclusions:
- Vitreoretinal surgery is safe and effective for improving vision in pediatric CHRPE, particularly in Zone 1 lesions.
- Surgical intervention helps reduce retinal distortion associated with CHRPE.
