Rhegmatogenous Retinal Detachment in Stickler Syndrome
Bibliographic record
Abstract
Stickler syndrome is an inherited connective tissue disorder with severe ocular manifestations. It is the leading syndromic cause of rhegmatogenous retinal detachment (RRD) particularly in the pediatric population. To review the prevalence, prophylactic efficacy and surgical outcomes of RRD in Stickler syndrome. Our protocol was registered on PROSPERO (CRD420251070425). We searched six databases and ClinicalTrials.gov from inception to June 2025. Studies reporting RRD-specific data in genetically or clinically confirmed Stickler syndrome were included. The primary outcome was RRD prevalence. Secondary outcomes included prophylactic efficacy and final surgical repair success. Meta-analysis was performed using the random-effects model, with assessment of heterogeneity and publication bias. Risk of bias was assessed using the Joanna Briggs Institute (JBI) the Newcastle-Ottawa Scale (NOS) tools. Evidence certainty was assessed using the (GRADE) framework. A total of 49 studies involving 3,492 individuals were included. Thirty-one studies reported the prevalence of RRD in individuals, with a pooled estimate of 41.9% (95% CI 33.78%–50.20%; I 2 = 86.3%), while 23 studies reported prevalence in eyes, with a pooled estimate of 26.1% (95% CI 19.64%–33.13%; I 2 = 82.1%), with higher rates in type 1 Stickler syndrome. Twelve studies of prophylactically treated individuals showed an RRD incidence of 8.7% (95% CI 7.09%–10.48%; I 2 = 0.0%), and 15 studies of prophylactically treated eyes showed an incidence of 5.1% (95% CI 4.02%–6.27%; I 2 = 23.5%). Prophylaxis reduced the odds of RRD by 92% (OR 0.08, 95% CI 0.04–0.17), with moderate-certainty evidence in patients (I 2 = 76.9%) and low-certainty evidence in eyes (I 2 = 83.5%). Fifteen studies reported surgical outcomes, with a pooled final anatomical success rate of 85.5% (95% CI 73.13%–94.74%; I 2 = 83.9%), supported by moderate-certainty evidence, and the highest success of 93.6% (95% CI 87.26%–97.98%; I 2 = 0.0%) was observed in eyes treated with combined scleral buckling + vitrectomy. Overall, most included studies were of acceptable methodological quality. RRD is highly prevalent in Stickler syndrome. Prophylaxis significantly reduces this risk, while surgery remains effective. These findings support early surveillance and prophylaxis, and larger prospective trials comparing prophylactic and surgical techniques.
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How this classification was reachedexpand
Full frame machine prediction
Teacher imitationNot calibrated prevalence, not ground truth. Human validation pending. The Gemma side is a direct model label for every work in the frame, read from the title-only record. The Codex side is a classifier learned from the 10,348 direct Codex labels and calibrated to design-weighted sample rates; fields without enough sample support carry no Codex call. Candidate is the union of the two sides; consensus is their intersection. These outputs are machine_predicted_unvalidated and are not human labels.
Distilled classifier scores by category (both heads)
| Category | Codex | Gemma |
|---|---|---|
| Metaresearch | 0.009 | 0.024 |
| Meta-epidemiology (narrow) | 0.001 | 0.001 |
| Meta-epidemiology (broad) | 0.007 | 0.010 |
| Bibliometrics | 0.004 | 0.003 |
| Science and technology studies | 0.000 | 0.001 |
| Scholarly communication | 0.002 | 0.001 |
| Open science | 0.001 | 0.001 |
| Research integrity | 0.002 | 0.001 |
| Insufficient payload (model declined to judge) | 0.003 | 0.000 |
Machine scores (provisional)
The two teacher heads of the student model, read on this work. A score orders the frame for review; it never asserts a category, and the validation status ships verbatim with every row.
Baseline scores from an immature model (maturity gate not passed, 7 training rounds). Scores rank; they never assert a category.
score_only:v0-immature-baseline · verbatim from the scoring run: score_only means the number may rank works, and no category label ships from itClassification
machine, unvalidatedMachine predicted; a candidate call from one source (direct Gemma or distilled Codex), not a consensus.
How this classification was reached, model by model and score by score, is at the end of the page under "How this classification was reached".