Management and Outcomes of Glaucoma in Children with Stickler Syndrome.

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.