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Vol 7 (2022): Continuous Publishing
Original paper
Published online: 2022-12-09
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Results of surgical management of the retinal detachment in pediatric patients with Marfan and Stickler syndrome

Aleksandra Czarnek-Chudzik1, Mario Damiano Toro12, Patrycja Lesiuk1, Vincenza Bonfiglio3, Robert Rejdak1, Katarzyna Nowomiejska1
DOI: 10.5603/OJ.2022.0026
·
Ophthalmol J 2022;7:163-171.
Affiliations
  1. Chair and Department of General and Pediatric Ophthalmology, Medical University of Lublin, Poland
  2. Faculty of Medicine, Collegium Medicum Cardinal Stefan Wyszynski University, Warsaw, Warsaw, Poland
  3. Department of Experimental Biomedicine and Clinical Neuroscience, Ophthalmology Section, University of Palermo, Palermo, Palermo, Italy

open access

Vol 7 (2022): Continuous Publishing
ORIGINAL PAPERS
Published online: 2022-12-09

Abstract

Background: The purpose of this study was to report the anatomical and functional outcomes in the surgical treatment of retinal detachment (RD) in pediatric patients suffering from Marfan (MFS) and Stickler syndrome (SS).

Material and methods: Retrospective consecutive case series of eight eyes of four patients with SS and MFS. Retinal reattachment and visual acuity, as well as complications of surgical procedures have been evaluated during
the follow-up period.

Results: Total RD was reported in five of the eight eyes of these patients. In one patient, it was bilateral. As a surgical treatment, pars plana vitrectomy (PPV) with silicone oil was performed in four eyes. Proliferative vitreoretinopathy was the reason for the secondary PPV in three eyes. Scleral buckling was conducted in two eyes of bilateral RD. Laser treatment was performed as prophylaxis in all fellow eyes. The visual acuity in eyes with RD ranged from light perception to 0.1 in two cases. The retina was finally attached in all cases. Imaging with OCT-A in a patient with SS revealed a reduced vascular density in the eye after PPV compared to the eye treated with laser.

Conclusions: Multiple surgical procedures are needed to attach the retina in patients with SS and MFS. However, despite the improvement of anatomical conditions — reattachment of the retina, the visual acuity after RD surgery is not satisfactory in patients suffering from SS and MFS.

Abstract

Background: The purpose of this study was to report the anatomical and functional outcomes in the surgical treatment of retinal detachment (RD) in pediatric patients suffering from Marfan (MFS) and Stickler syndrome (SS).

Material and methods: Retrospective consecutive case series of eight eyes of four patients with SS and MFS. Retinal reattachment and visual acuity, as well as complications of surgical procedures have been evaluated during
the follow-up period.

Results: Total RD was reported in five of the eight eyes of these patients. In one patient, it was bilateral. As a surgical treatment, pars plana vitrectomy (PPV) with silicone oil was performed in four eyes. Proliferative vitreoretinopathy was the reason for the secondary PPV in three eyes. Scleral buckling was conducted in two eyes of bilateral RD. Laser treatment was performed as prophylaxis in all fellow eyes. The visual acuity in eyes with RD ranged from light perception to 0.1 in two cases. The retina was finally attached in all cases. Imaging with OCT-A in a patient with SS revealed a reduced vascular density in the eye after PPV compared to the eye treated with laser.

Conclusions: Multiple surgical procedures are needed to attach the retina in patients with SS and MFS. However, despite the improvement of anatomical conditions — reattachment of the retina, the visual acuity after RD surgery is not satisfactory in patients suffering from SS and MFS.

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Keywords

retinal detachment; pediatric patients; Marfan syndrome; Stickler syndrome

About this article
Title

Results of surgical management of the retinal detachment in pediatric patients with Marfan and Stickler syndrome

Journal

Ophthalmology Journal

Issue

Vol 7 (2022): Continuous Publishing

Article type

Original paper

Pages

163-171

Published online

2022-12-09

Page views

3450

Article views/downloads

373

DOI

10.5603/OJ.2022.0026

Bibliographic record

Ophthalmol J 2022;7:163-171.

Keywords

retinal detachment
pediatric patients
Marfan syndrome
Stickler syndrome

Authors

Aleksandra Czarnek-Chudzik
Mario Damiano Toro
Patrycja Lesiuk
Vincenza Bonfiglio
Robert Rejdak
Katarzyna Nowomiejska

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