Outcomes of surgery for retinal detachment in patients with Stickler syndrome: a comparison of two sequential 20-year cohorts.

Background: Stickler syndrome is a hereditary oculo-systemic disorder where patients are predisposed to retinal detachments which are often complex and challenging to manage. Significant progress has been made regarding the molecular genetics of the condition; however, there is little recent literature on surgery for retinal detachment in Stickler syndrome. Our aim is to describe a population of Stickler patients presenting to Moorfields Eye Hospital with detachment from 1986 to 2003. We looked at patient characteristics, characteristics of detachment, management and anatomical and functional outcomes. We also aim to compare this group from 1986 to 2003 with a past group of Stickler patients treated at Moorfields between 1965 and 1985, reported by (Billington et al. in Trans Ophthalmol Soc UK 104:875-879, 1985). This comparison of 20-year matched cohorts examined patient characteristics, features of detachment, management and anatomical outcome in the two groups using the same definitions as the earlier authors.

Results: In the Stickler group from 1986 to 2003, complete re-attachment rate was 67% for primary scleral-buckle surgery, 84.2% for primary vitrectomy and 78.57% for all surgery in 30 eyes of 23 patients. Overall in this group there was an average increase in Logmar visual acuity of 0.33 and 0.32 in patients undergoing primary cryo-buckle and primary vitrectomy surgery respectively. When comparing the two groups using Fisher's exact test, we found that the group from 1986 to 2003 had significant improvement in re-attachment for detachments with multiple tears and for vitrectomy surgery, compared with the group from 1965 to 1985.

Conclusions: This study shows that despite complicated surgery and often multiple procedures, good anatomical outcomes were achieved as well as useful functional visual results after retinal detachment surgery in Stickler patients. It would also appear that when comparing the group of Stickler patients from 1986 to 2003 with the group from 1965 to 1985 improvements were seen in outcome from vitrectomy surgery and surgery for multiple breaks probably due to advances in technique and technology in vitreoretinal surgery, over the past 4 decades.