Retinal detachment and prophylaxis in type 1 Stickler syndrome.

Abstract:

PURPOSE:To report the prevalence of retinal detachment (RD) and results of prophylaxis against detachment from a giant retinal tear in a large cohort of patients with type 1 Stickler syndrome. DESIGN:Retrospective study. PARTICIPANTS:Two hundred four type 1 Stickler syndrome patients. METHOD:Pedigrees and individuals with type 1 Stickler syndrome were identified from the vitreous research clinic and divided into 3 groups. Group 1 consisted of patients who received no prophylaxis (control group). Group 2 consisted of patients who had bilateral 360 degrees prophylactic cryotherapy (study group). Group 3 consisted of patients referred with unilateral RD for surgical repair and who underwent prophylaxis in the fellow eye (mixed group). MAIN OUTCOME MEASURES:Retinal status after prophylaxis, with failure of prophylaxis being defined as the development of RD or retinal tears needing further retinopexy. RESULTS:Of 111 patients who had no prophylactic retinopexy (group 1; mean age, 49 years), 73% (81/111) suffered RD and 48% (53/111) were bilateral. Of 62 patients who had bilateral prophylactic cryotherapy (group 2; mean age, 21 years), 8% (5/62) suffered failure of prophylaxis. There were no cases of bilateral detachments. The mean follow-up period was 11.5 years. In 31 patients who had unilateral prophylactic cryotherapy to the fellow eye (group 3; mean age, 36 years), failure occurred in 10% (3/31) of cases with a mean follow-up of 15.5 years. The prevalence of failure of prophylaxis in treated patients was significantly less than prevalence of RD in untreated patients (chi2(1) = 119.2, P<0.001). CONCLUSION:Prophylactic cryotherapy substantially reduces the risk of RD in type 1 Stickler syndrome and, in this series, eliminated the risk of bilateral detachments.

journal_name

Ophthalmology

journal_title

Ophthalmology

authors

Ang A,Poulson AV,Goodburn SF,Richards AJ,Scott JD,Snead MP

doi

10.1016/j.ophtha.2007.03.059

subject

Has Abstract

pub_date

2008-01-01 00:00:00

pages

164-8

issue

1

eissn

0161-6420

issn

1549-4713

pii

S0161-6420(07)00340-5

journal_volume

115

pub_type

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