Glaucoma, Secondary Congenital Follow-up

• Patients rarely need hospitalization for IOP control.

• Closely monitor patients with Axenfeld-Rieger anomaly to detect early signs of developing glaucoma.
• Closely monitor patients with neurofibromatosis in childhood. Occurrence of new ocular complications (eg, glaucoma) decreases in adulthood.
• Periodically examine patients with neurofibromatosis using gonioscopy to assess for the risk of developing angle-closure component over presence of open angles.
• Children with Sturge-Weber syndrome should periodically have complete ocular examinations to detect early signs of ocular complications.
• Starting from an early age, periodically monitor children with von Hippel-Lindau disease.

• Beta-blockers, parasympathomimetics, alpha-adrenergic agonists, prostaglandin analogs, and carbonic anhydrase inhibitors can be used.

Obtaining IOP under 19 mm Hg in the pediatric population with glaucoma may prevent optic nerve damage. Treating amblyopia early can achieve good visual outcome in the long term.

• Complications include intractable glaucoma despite numerous interventions. Open-angle glaucoma can develop into angle-closure glaucoma or neovascular glaucoma. Eyes eventually can be phthisical.
• Sturge-Weber syndrome (encephalofacial angiomatosis)

  • These eyes carry high risk for expulsive choroidal hemorrhage upon entering the eye causing sudden pressure change.
  • They are more likely to develop choroidal effusion from choroidal cavernous hemangioma.
  • Reducing the IOP as low as possible and performing posterior sclerotomies prior to entering the eye may reduce complications.
  • In a study by Iwach et al, intraoperative choroidal expansion was detected in 24% of patients who underwent trabeculectomy.^[2]
  • In Agarwal's series of 18 patients with Sturge-Weber syndrome who underwent the trabeculotomy-trabeculectomy procedure, the following complications were noted: intraoperative hyphema (22.2%), vitreous loss (16.7%), and vitreous hemorrhage (5.6%).^[3]
• von Hippel-Lindau (retinal angiomatosis)

  • This condition consists of capillary proliferation that leaks on fluorescent angiography.
  • Iridocyclitis and neovascularization of the iris cause neovascular glaucoma.

• Prognosis in secondary congenital glaucoma is guarded.

  • Earlier age at onset of glaucoma usually is more difficult to manage. Patients need multiple procedures, each of which has its own risks.
  • Associated ocular problems (eg, strabismus, cataract, microphthalmia, amblyopia) also worsen the prognosis.
  • In the study by Kargi et al, visual function was evaluated retrospectively with an average follow-up of 11.6 years in 204 eyes of 126 patients who had childhood glaucoma including congenital glaucoma and secondary glaucoma with or without syndrome association.^[4] They found that decreased final visual acuity (less than 20/40 is considered as decreased vision) is strongly correlated with amblyopia and optic nerve damage. Anisometric or strabismic amblyopia was seen, but deprivation amblyopia was the most common type in syndrome-associated glaucoma. Cornea- and lens-associated problems were more common on syndrome-associated glaucoma; therefore, their final visual acuity was worse than other groups at the end of the follow-up period.
  • In a series by Yang et al of 34 eyes of 19 children with Peters anomaly, IOP control with or without antiglaucoma medicine was achieved in 11 eyes (32%) after 1 or more surgical procedures.^[5] The visual outcome was poor due to glaucomatous optic neuropathy, amblyopia, and other associated anomalies.
  • Agarwal et al studied 18 eyes of patients with Sturge-Weber syndrome who underwent the combined trabeculotomy-trabeculectomy procedure. The follow-up (mean, 42 mo) results are as follows: IOP was controlled in 11 eyes (61.1%), and visual acuity was better than 6/60 (20/200) in 8 patients.^[3]
  • Patients with Lowe syndrome have a poor life expectancy.

• For excellent patient education resources, visit eMedicine's Glaucoma Center. Also, see eMedicine's patient education articles Glaucoma Overview, Glaucoma FAQs, and Understanding Glaucoma Medications.