Stickler syndrome. Epidemiology of retinal detachment

Arch Soc Esp Oftalmol. 2015 Jun;90(6):264-8. doi: 10.1016/j.oftal.2014.11.001. Epub 2015 Mar 25.
[Article in English, Spanish]


Objective: A review was performed on all patients with Stickler syndrome that had been treated in our Centre since it was diagnosed, in order to evaluate the risk of suffering a retinal detachment (RD).

Methods: A total of 14 patients, diagnosed by clinical criteria, were included. The following variables were evaluated: age, gender, ocular background, follow-up, initial and final visual acuity (VA), optical prescription, prophylactic treatment, surgery and techniques performed. The risk age to suffer a RD, as well as cataracts, was determined by using the Kaplan-Meier survival curve analysis.

Results: From a total of 5 men and 9 women, the median initial VA was 0.35, which was the same as the final VA. The median optical prescription was -9.5 D myopia. The median of follow-up was 7 years. Ocular background was 4 RD cases and 2 Lasik surgeries. The operations performed were 8 RD, 12 cataract, 2 glaucoma, 2 macular hole, and one endotropia. The median age of RD was 20 years and cataract 34 years. As regards surgical tecnique, 4 scleral buckle cases, and 4 scleral buckle+pars plana vitrectomy cases were formed. The prophylactic treatments performed were: one scleral buckle case, 4 endolaser photocoagulation, and one cryotherapy. Two of which presented with RD.

Conclusion: In the series presented, retinal detachment in Stickler syndrome mainly occurs in the second decade of life, with cataracts mainly developing in the fourth decade.

Keywords: Agujero macular; Cataract; Catarata; Desprendimiento de retina; Glaucoma; Macular hole; Retinal detachment; Sickler syndrome; Síndrome de Stickler; Vitrectomy; Vitrectomía.

MeSH terms

  • Adolescent
  • Adult
  • Arthritis / epidemiology*
  • Arthritis / surgery
  • Child
  • Child, Preschool
  • Connective Tissue Diseases / epidemiology*
  • Connective Tissue Diseases / surgery
  • Female
  • Follow-Up Studies
  • Hearing Loss, Sensorineural / epidemiology*
  • Hearing Loss, Sensorineural / surgery
  • Humans
  • Male
  • Ophthalmologic Surgical Procedures / statistics & numerical data
  • Retinal Detachment / epidemiology*
  • Retinal Detachment / surgery
  • Retrospective Studies
  • Treatment Outcome
  • Young Adult

Supplementary concepts

  • Stickler syndrome, type 1