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Published on: February 15, 2022
Management and Outcomes of Glaucoma in Children with Stickler Syndrome
Adam Jacobson1, Safa Rahmani2, Brenda L Bohnsack2
1Department of Ophthalmology and Visual Sciences, University of Michigan, Ann Arbor, Michigan.
Insights
Glaucoma affects 14% of children with Stickler syndrome. Infantile glaucoma requires drainage devices, while post-surgery glaucoma may need medication or surgery, impacting vision.
Area of Science:
- Ophthalmology
- Genetics
- Pediatrics
Background:
- Stickler syndrome is a genetic disorder affecting connective tissues.
- Ocular manifestations are common in Stickler syndrome, including glaucoma.
- Early diagnosis and management are crucial for preserving vision.
Purpose of the Study:
- To assess the prevalence, management, and outcomes of glaucoma in pediatric patients with Stickler syndrome.
- To identify risk factors and specific glaucoma subtypes within this population.
- To evaluate the effectiveness of different treatment strategies.
Main Methods:
- Retrospective observational case series of 97 children diagnosed with Stickler syndrome.
- Data collected included ocular examinations, genetic information, glaucoma diagnoses, and surgical history.
- Analysis focused on best-corrected visual acuity (BCVA), glaucoma type, and surgical interventions.
Main Results:
- 14% of patients with Stickler syndrome developed glaucoma.
- Glaucomatous eyes were more myopic and had worse final BCVA.
- Infantile-onset glaucoma (n=5) required glaucoma drainage devices (GDDs); post-intraocular surgery glaucoma (n=9) varied in management, with some needing GDDs.
Conclusions:
- Glaucoma is a frequent complication in children with Stickler syndrome.
- Infantile glaucoma management often necessitates angle bypass surgery for IOP control.
- Post-surgical glaucoma may respond to medical therapy, but GDD placement can be complex due to prior ocular surgeries.
Purpose:
Assess management and outcomes of glaucoma in children with Stickler syndrome.
Design:
A retrospective observational case series.
Subjects:
Children (less than 18 years of age) with Stickler syndrome.
Methods:
Review of 97 patients who presented between 2010 and 2025 with a clinical diagnosis of Stickler syndrome and had at least 2 documented eye exams. Examination findings, genetic information, ocular diagnoses, and surgeries were collected.
Main Outcome Measures:
Best-corrected visual acuity, type of glaucoma, type of glaucoma surgeries.
Results:
Fourteen of 97 patients (14%) with Stickler syndrome were diagnosed with glaucoma at a median age of 8.5 interquartile range [0.6, 12.3] years. There was no difference in age at presentation (P = 0.84) or length of follow-up (P = 0.29) between patients with and without glaucoma, but glaucomatous eyes were significantly more myopic at presentation (P = 0.02) and had worse final best-corrected visual acuity (P < 0.0001). Nine eyes of 5 patients (4 type I and 1 type II) had infantile-onset glaucoma, and all eyes required glaucoma drainage device implantation. Eleven eyes of 9 patients (2 type I, 4 type II, and 3 unknown) developed glaucoma following intraocular surgery (10 after retinal detachment and 1 after lensectomy), and 3 eyes required glaucoma drainage device placement. The other 8 eyes with glaucoma were medically managed.
Conclusions:
Glaucoma is frequently diagnosed in children with Stickler syndrome. In the infantile-onset form, angle surgery does not seem to yield long-term intraocular pressure control, and these children typically require angle bypass surgery. Glaucoma following intraocular surgery may respond to medications, but some do require glaucoma drainage device placement, which can be complicated by prior scleral buckle or silicone oil.
Financial Disclosure(S):
Proprietary or commercial disclosure may be found in the Footnotes and Disclosures at the end of this article.
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