Secondary Congenital Glaucoma Treatment & Management

Updated: Jul 27, 2020
  • Author: Inci Irak Dersu, MD, MPH; Chief Editor: Hampton Roy, Sr, MD  more...
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Treatment

Medical Care

In managing secondary congenital glaucoma, medical therapy is the first-line treatment.

Topical beta-blockers, carbonic anhydrase inhibitors (CAI), and prostaglandin analogs can be used. Alpha-2-adrenergic agonists should be avoided in children younger than 3 years due to possible apnea and other central nervous system adverse effects. Topical beta-blockers should be used very carefully in neonates and should be avoided in premature babies. Timolol should be started low dose as 0.25% initially. Bradycardia and bronchospasm can be a problem in some children.

When beta-blockers are contraindicated, topical CAIs (dorzolamide or brinzolamide bid or tid) can be used. They can still be helpful to add even if the child is taking oral CAIs (oral acetazolamide or methazolamide). Acetazolamide is used with food in divided doses at the dose of 10-20 mg/kg/d. Adverse effects of topical CAIs on graft survival have been questioned.

Miotics are not used as often as they used to be. Pilocarpine is only used before and after goniotomy or trabeculectomy. Echothiophate iodide can still be used successfully in aphakic glaucoma in some cases.

Prostaglandin analogs have not been extensively studied in children. However, they appear to be safe. Lengthening of eyelashes with drops is more excessive in children.

Depending on whether the glaucoma is early or late onset or depending on its severity, patients may respond to treatment, but surgery is necessary in most cases.

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Surgical Care

Patients may need multiple procedures for adequate IOP control.

If the cornea is clear, goniotomy or trabeculotomy may be performed first.

Goniotomy works best in primary congenital glaucoma. Children with enlarged cornea and more severe angle deformation and with associated systemic abnormalities do not do as well. Trabeculotomy is advantageous for eyes when the cornea is cloudy and goniotomy cannot be performed.

Trabeculotomy ab interno with the Trabectome (Neomedix) and goniotomy-assisted transluminal trabeculotomy (GATT) are newer options for pediatric glaucomas. In these techniques, a goniolens over the cornea is used to identify the angle structures, requiring the cornea to be clear.

Trabeculectomy, shunt devices for patients with useful vision, and cyclocryotherapy and diode laser cyclophotocoagulation for patients with poor vision can be used. [8]

Trabeculectomy can be used adjunct with antifibrotic agents (5-fluoruracil and mitomycin-c). [9, 10] Antifibrotic agents increase the success rate; however, bleb-related infections are also a risk. Therefore, children with avascular bleb must be closely observed.

Shunt devices are usually used when trabeculectomy fails in adults. In secondary congenital glaucoma, however, particularly in those younger than age 2 years, shunt devices may work better as first-line surgical treatment in comparison to trabeculectomy.

Cycloablation, which includes cyclocryotherapy, transscleral diode laser, or endoscopic diode laser, can be used when medical and other surgical treatments are exhausted.

Repeated cyclodiode treatments may be necessary to control IOP. Compared with adults, the success rate of cyclodiode treatments is lower in children because of their faster recovery of ciliary body function; however, it is still a viable option. In patients with Peters anomaly, performing penetrating keratoplasty early to prevent amblyopia if the corneal opacities are dense is suggested. The result of unilateral keratoplasty generally is poor.

Patients with nanophthalmos may need laser peripheral iridectomy and laser peripheral iridoplasty.

The need for posterior sclerectomies during filtering surgery in patients with Sturge-Weber syndrome has been questioned. Instead, the application of viscoelastic material seemed to reduce the complication rate after decompression of the eye.

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Consultations

Genetic consultation helps to subclassify the diseases and sometimes to predict the prognosis.

Consult with other specialties regarding the existing systemic abnormalities.

Axenfeld-Rieger syndrome: Patients may need workup for associated systemic abnormalities, so referring to a pediatrician or an internist is important.

Phakomatoses have typical ocular findings. Because of their systemic nature, evaluation by the proper specialty is necessary.

For the sporadic type of aniridia, consultation with nephrology is necessary to evaluate the possibility of Wilms tumor.

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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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Long-Term Monitoring

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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