Juvenile open-angle glaucoma: a clinicopathological update and review

Area of Science:

• Ophthalmology
• Glaucoma Research
• Genetics of Eye Diseases

Background:

• Juvenile open-angle glaucoma (JOAG) is a rare primary open-angle glaucoma variant affecting individuals aged 3-40 years.
• Characterized by early onset, elevated intraocular pressure, normal gonioscopic angles, optic disc cupping, and visual field loss.
• Includes variants like juvenile ocular hypertension and juvenile normal tension glaucoma.

Purpose of the Study:

• To review the clinical features, pathophysiology, risk factors, genetics, diagnosis, and management of JOAG.
• To highlight recent advances in the clinical setting and therapeutic strategies for JOAG.
• To emphasize the importance of early detection and comprehensive care for JOAG.

Main Methods:

• Retrospective literature review of PubMed and Google (2000-2025).
• Diagnostic criteria include clinical examination, tonometry, gonioscopy, CCT, slit lamp biomicroscopy, OCT, OCT angiography, and automated perimetry.
• Evaluation of optic disc changes and retinal nerve fiber layer loss.

Main Results:

• Trabeculodysgenesis is the primary pathology, hindering aqueous humor outflow, classified into four gonioscopic subtypes.
• Key risk factors include male gender and myopia; MYOC gene mutations are commonly implicated.
• Medical therapy is first-line; selective laser trabeculoplasty shows promise. Surgical intervention is reserved for cases with inadequate IOP control.

Conclusions:

• JOAG is a heterogeneous and complex disease requiring a multidisciplinary approach.
• Screening at-risk individuals can lead to earlier diagnosis and prevent visual disability.
• Prognosis hinges on diagnosis stage, patient adherence, and timely management, necessitating lifelong follow-up.