Treating and managing glaucoma in children with Stickler Syndrome

Adam Jacobson, MD, and colleagues retrospectively investigated how glaucoma affects the eyes of pediatric patients with Stickler syndrome and what may determine the best management course. Jacobson, the first study author from the University of Michigan, Ann Arbor, reported their findings at the 51^st annual meeting of the American Association for Pediatric Ophthalmology and Strabismus in Boston, March 18-22.

Stickler syndrome, a rare genetic disease resulting from mutations in types I and XI collagen, affects multiple structures in the eye; the typical ocular manifestations of the disease include high myopia and retinal detachments. Numerous systemic effects include premature arthritis, cleft palate, micrognathia, and hearing loss in addition to the ocular manifestations.

In addition to high myopia and retinal detachments, glaucoma also develops in many patients, but there is not a great deal of information about its incidence, management, and outcomes. This recognition was the basis for a study focusing on glaucoma in this patient population.

Jacobson and colleagues conducted a retrospective review in which they identified 97 pediatric patients, ie, younger than 18 years, who presented between 2010 and 2025 with a clinical diagnosis of Stickler syndrome and who had undergone a minimum of two examinations. The study defined glaucoma as two measurements of intraocular pressure that exceeded 21 mmHg with corneal signs, cupping of the optic nerve, and/or buphthalmos.

They published their findings in Ophthalmology Glaucoma.¹

What did the data analysis of the patients with Stickler Syndrome show?

Jacobson reported that 20 eyes of 14 patients were diagnosed with glaucoma at a median age of 8.5 years, which translated to a 14% rate of glaucoma in this patient cohort.

This investigation identified 2 distinct glaucoma populations, ie, glaucoma that developed after intraocular surgery, such as scleral buckle or vitrectomy, or infantile-onset glaucoma, which has some similarities to primary congenital glaucoma, that developed before age 1 year.

Nine eyes of five patients had infantile-onset glaucoma, and in 11 eyes of 9 patients the glaucoma developed after intraocular surgery.

“Retinal detachments were more common in patients with glaucoma than those without (P < 0.0001). The glaucomatous eyes were characterized by more myopia and worse best-corrected visual acuity than the fellow eyes without glaucoma in unilateral cases (P = 0.02, P < 0.0001, respectively),” he described.

The glaucoma that developed after an intraocular surgery responded well to topical anti-hypertensive drops; 75% of these patients only needed drops during follow-up and no surgery.

The patients with infantile-onset glaucoma underwent an angle surgery, ie, goniotomy or trabeculotomy. However, a difference in this patient population compared to primary congenital glaucoma, according to Jacobson, was that all of these patients required a trabecular bypass surgery. “They did far better with tube shunts than with angle surgery,” he stated.

These findings, he explained, help with prognostication and parental counseling. “We know about myopia and retinal detachment in these patients, but not a lot has been published on the risk and types of glaucoma and outcomes,” he explained.

At the last follow-up visit, 4 eyes had retinal detachments, 3 eyes required another glaucoma surgery, and 1 eye each had endophthalmitis, corneal decompensation, and chronic hypotony.

“Historically, in pediatric glaucoma, many treatments and the thought process have been anecdotal. Recently, the childhood glaucoma community has pushed for a more evidence-based practice to determine the best treatments not only for childhood glaucoma as a whole but also for tailoring treatments for different etiologies. Pediatric eyes are not just smaller versions of adult eyes; everything is different. They respond to drops and surgeries differently. The push for evidence-based treatment has provided a better understanding of childhood glaucoma,” Jacobson stated.

Jacobson was joined in this study by Safa Rahmani and Brenda Bohnsack, MD, both from Lurie Children's Hospital, Chicago, and Northwestern University, Evanston, Ill.

Reference

1. Jacobson A, Rahmani S, Bohnsack BL. Management and outcomes of glaucoma in children with Stickler syndrome. Ophthalmol Glaucoma. 2025; published online December 27; https://doi.org/10.1016/j.ogla.2025.12.009