Secondary Congenital Glaucoma Follow-up

Updated: Mar 13, 2014
  • Author: Inci Irak Dersu, MD, MPH; Chief Editor: Hampton Roy, Sr, MD  more...
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Follow-up

Further Outpatient Care

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  • 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.
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Further Inpatient Care

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  • Patients rarely need hospitalization for IOP control.
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Inpatient & Outpatient Medications

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

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.

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Complications

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  • 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.
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Prognosis

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  • 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.
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Patient Education

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