Long-term anatomical and functional outcomes of surgical treatment of retinal complications in children and adolescents with Stickler syndrome between 2004 and 2021.

BACKGROUND: Stickler syndrome (STL) is an inherited progressive connective tissue collagen disorder. STL is the most common hereditary cause of retinal complications, retinal tears, and the development of retinal detachment (RD) in childhood. The aim of the study was to evaluate the long-term anatomical and functional results of surgical treatment of retinal complications in children and adolescents affected by STL. METHODS: A retrospective, single-center study was performed a cohort of children with STL who underwent retinal surgery between 2004 and 2021. RESULTS: The study group consisted of nine children; the mean age at the time of the retinal tear with/without retinal detachment was 7.2 (2-10) years, and the mean follow-up period was 9.6 (5-16) years. Pathogenic variants COL2A1 (5 children) and COL11A1 (3 children) were confirmed in our cohort. In total, we operated on 13 eyes, 11 eyes with complicated RD and two eyes with multiple retinal defects, but without RD. At the end of the follow-up period, an attached retina was achieved 77% (10 eyes) with or without silicone oil tamponade: cryopexy alone was successful in one eye (10%), scleral buckling (EB) in five eyes (50%), and vitrectomy with silicone oil tamponade combined with EB in four eyes (40%). The mean number of surgeries was 2.3 per eye. The resulting best corrected visual acuity ranged from 0.03 to 0.1 in one eye, from 0.16 to 0.4 in two eyes, and from 0.5 to 1.0 in 7 eyes. CONCLUSION: Repair of retinal tears with/without retinal detachment in patients with Stickler syndrome often requires multiple surgeries with combinations of cryopexy, scleral buckling, and/or vitrectomy with silicone oil tamponade. Treatment of the ocular complications arising from STL requires long-term comprehensive care.

[Retinal detachment surgery: trends over 15 years]. / Ablatiochirurgie: Trends über 15 Jahre.

INTRODUCTION: The aim of the study was to investigate the changes in the treatment of patients with retinal detachment over the past 15 years. METHOD: Files of all 5101 patients hospitalized for retinal detachment surgery between January 2005 and March 2020 were retrospectively analyzed. RESULTS: The number of retinal detachment surgeries over the past 15 years increased continually from 150 to 600 per annum. During the summer months an increased incidence of retinal detachment of approximately 20% could be observed compared to the winter months. Anatomical success rates of emergency surgery carried out at weekends were not lower than those of scheduled routine procedures performed during the week. The relative proportion of buckle surgery decreased significantly from more than 60% down to 5% between 2005 and 2019. While initially an additional encircling buckle was employed in most cases of primary vitrectomy, this technique has almost completely disappeared in recent years without a deterioration of success rates. Liquid silicone tamponade was applied less frequently over the years. The overall anatomical success rates showed a slight trend towards improved results over the past 15 years. CONCLUSION: The strategies for surgical repair of retinal detachment have changed considerably in recent years: less buckle surgery, more vitrectomy, less add-on encircling buckles and less silicone tamponade. A slight trend towards better anatomical success rates could be observed.

[Removal of higher viscosity oil through a hybrid 2-port 23/25 gauge pars plana vitrectomy]. / Hybrid-2-Port-23/25-G-Pars-plana-Vitrektomie zur Entfernung von Öl höherer Viskosität.

This article presents a new hybrid method of a combined 2­port 23/25G pars plana vitrectomy for removal of silicone oil (5700 centistokes) compared to a 25G vitrectomy. In this hybrid technique the infusion is performed through a 25G cannula and the oil removal through a 23G cannula. The duration of the surgery and the intraocular pressure (IOP) in both groups were compared. The oil removal using the hybrid technique was performed significantly faster with a reduction of the time by 32.2% but there was no difference in the IOP. In conclusion, silicone oil removal through a 25G cannula is generally possible but oil removal with the new hybrid technique is much faster.

Effect of Intravenous Mannitol on Intraocular Pressure in Vitrectomized Silicone-Oil-Filled Eyes.

PURPOSE: To evaluate the effect of intravenous mannitol (IVM) on intraocular pressure (IOP) in vitrectomized eyes. METHODS: Thirty-one patients with raised IOP after retinal surgery with silicone oil injection were included. Patients were administered IVM (20% solution, 1g/Kg, over 30 minutes) and IOP was noted at regular intervals. IOP reduction in vitrectomized eyes (Group 1) was compared with the normal eyes (Group 2). RESULT: Percentage IOP reduction was higher in Group 1 than in Group 2 at all time intervals, 24.5% vs. 19.2% at 15 minutes (p=0.34), 22.6% vs. 9.8% at 45 minutes (p=0.003), 19.1% vs. 9.9% at two hours (p=0.023), and 16.1% vs. 7.8% at four hours (p=0.04), respectively. In Group 1, 40% eyes had an IOP reduction of 2-6 mmHg while 30% eyes had IOP reduction >6 mmHg at four hours post-IVM. CONCLUSION: IVM is useful for short-term IOP reduction in vitrectomized eyes. IOP reduction is independent of baseline IOP, and sustained and higher as compared to normal eyes.