From the Guidelines
Glaucoma in neonates is a rare condition that typically presents with distinctive findings such as buphthalmos, epiphora, photosensitivity, and corneal clouding, and intraocular pressure measurement should be performed whenever a child has or is at risk for glaucoma, as stated in the 2023 pediatric eye evaluations preferred practice pattern 1. When evaluating neonates for glaucoma, it is essential to consider the potential signs and symptoms, including enlarged corneal diameter, corneal clouding or edema, tearing, photophobia, and elevated intraocular pressure (IOP).
- Key findings in glaucoma in neonates include:
- Enlarged corneal diameter (>10.5mm)
- Corneal clouding or edema
- Tearing
- Photophobia
- Buphthalmos (enlarged eyeball)
- Elevated intraocular pressure (IOP) Examination may reveal Haab's striae (breaks in Descemet's membrane), increased cup-to-disc ratio, and myopia. Primary congenital glaucoma is the most common form, typically presenting within the first year of life. Initial management usually involves surgical intervention rather than medications, with goniotomy or trabeculotomy being the preferred first-line procedures, as these methods have shown to be effective in reducing IOP and preventing vision loss 1. Medications may be used temporarily before surgery or as adjunctive therapy, including beta-blockers like timolol 0.25% (one drop twice daily), carbonic anhydrase inhibitors like dorzolamide 2% (one drop three times daily), and alpha-2 agonists like brimonidine (avoided in infants under 2 years due to CNS depression risk). Prostaglandin analogs are less effective in congenital cases. Early diagnosis and treatment are crucial to prevent irreversible vision loss, as the developing eye is particularly vulnerable to pressure-related damage. Regular follow-up examinations are essential to monitor IOP control and assess for disease progression or treatment complications. It is also important to note that an orbital/periorbital plexiform neurofibroma (OPPN) should be considered in infants and young children who present with buphthalmos and/or glaucoma, as reported in the 2017 study on orbital/periorbital plexiform neurofibromas in children with neurofibromatosis type 1 1. However, the 2023 study on pediatric eye evaluations preferred practice pattern provides more recent and relevant guidance on the evaluation and management of glaucoma in neonates 1.
From the Research
Glaucoma Findings in Neonates
- Glaucoma in neonates is a severe form of childhood glaucoma and is not always due to primary congenital glaucoma (PCG) 2
- The incidence of neonatal-onset glaucoma (NOG) is rising due to advances in neonatal care, but it remains an under-reported entity 2
- A study of 94 eyes of 53 babies with NOG found that 35 (66%) had PCG, while others had conditions such as neonatal congenital ectropion uveae, congenital rubella syndrome, Peter's anomaly, and Sturge-Weber syndrome 2
Clinical Profile and Outcome
- The mean age at presentation and surgery for NOG was 24.8±21.9 and 36.7±29.9 days, respectively 2
- Additional glaucoma surgery was required in 43 of the 94 eyes (45.7%) 2
- PCG had significantly better outcomes than other glaucomas at all time points, with 28.3% of eyes having good vision, 34.7% having moderate visual impairment, and 16% being blind 2
Treatment Options
- Various pharmacotherapeutic options are available for glaucoma in children, including topical adrenoceptor blockers, topical and systemic carbonic anhydrase inhibitors, prostaglandin (PG) analogs, adrenoceptor agonists, parasympathomimetics, and combined preparations 3
- However, none of these drugs have been licensed by regulatory agencies for use in children, and evidence from randomized controlled trials in the pediatric population is sparse 3
- A systematic review and meta-analysis found that fixed-combination drugs containing 0.5% timolol can effectively lower intraocular pressure in patients with primary open-angle glaucoma (POAG) and ocular hypertension (OHT) 4