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Secondary Congenital Glaucoma Treatment & Management

  • Author: Inci Irak Dersu, MD, MPH; Chief Editor: Hampton Roy, Sr, MD  more...
 
Updated: Mar 14, 2014
 

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

See the list below:

  • 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.
  • Trabeculectomy, shunt devices for patients with useful vision, and cyclocryotherapy and diode laser cyclophotocoagulation for patients with poor vision can be used.
  • Trabeculectomy can be used adjunct with antifibrotic agents (5-fluoruracil and mitomycin-c). 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

See the list below:

  • 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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Contributor Information and Disclosures
Author

Inci Irak Dersu, MD, MPH Associate Professor of Clinical Ophthalmology, State University of New York Downstate College of Medicine; Attending Physician, SUNY Downstate Medical Center, Kings County Hospital, and VA Harbor Health Care System

Inci Irak Dersu, MD, MPH is a member of the following medical societies: American Academy of Ophthalmology, American Glaucoma Society

Disclosure: Nothing to disclose.

Specialty Editor Board

Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Received salary from Medscape for employment. for: Medscape.

Martin B Wax, MD Professor, Department of Ophthalmology, University of Texas Southwestern Medical School; Vice President, Research and Development, Head, Ophthalmology Discovery Research and Preclinical Sciences, Alcon Laboratories, Inc

Martin B Wax, MD is a member of the following medical societies: American Academy of Ophthalmology, American Glaucoma Society, Society for Neuroscience

Disclosure: Nothing to disclose.

Chief Editor

Hampton Roy, Sr, MD Associate Clinical Professor, Department of Ophthalmology, University of Arkansas for Medical Sciences

Hampton Roy, Sr, MD is a member of the following medical societies: American Academy of Ophthalmology, American College of Surgeons, Pan-American Association of Ophthalmology

Disclosure: Nothing to disclose.

Additional Contributors

Andrew I Rabinowitz, MD Director of Glaucoma Service, Barnet Dulaney Perkins Eye Center

Andrew I Rabinowitz, MD is a member of the following medical societies: Aerospace Medical Association, American Academy of Ophthalmology, American Society for Laser Medicine and Surgery, American Academy of Ophthalmology, American Medical Association

Disclosure: Nothing to disclose.

References
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  2. Iwach AG, Hoskins HD Jr, Hetherington J Jr, Shaffer RN. Analysis of surgical and medical management of glaucoma in Sturge-Weber syndrome. Ophthalmology. 1990 Jul. 97(7):904-9. [Medline].

  3. Agarwal HC, Sandramouli S, Sihota R, Sood NN. Sturge-Weber syndrome: management of glaucoma with combined trabeculotomy-trabeculectomy. Ophthalmic Surg. 1993 Jun. 24(6):399-402. [Medline].

  4. Kargi SH, Koc F, Biglan AW, Davis JS. Visual acuity in children with glaucoma. Ophthalmology. 2006 Feb. 113(2):229-38. [Medline].

  5. Yang LL, Lambert SR, Lynn MJ, Stulting RD. Surgical management of glaucoma in infants and children with Peters' anomaly: long-term structural and functional outcome. Ophthalmology. 2004 Jan. 111(1):112-7. [Medline].

  6. Allingham R, Damji K, Freedman S, Moroi S, Shafranov G. Developmental glaucomas with associated anomalies. Shields' Textbook of Glaucoma. 5th ed. Philadelphia, PA 19106: Lippincott Williams & Wilkins; 2005. 252-271.

  7. Cantor LB. Glaucoma associated with congenital disorders. Ritch R, ed. The Glaucomas. St Louis: Mosby; 1989. Vol 2: 931-960.

  8. Eibschitz-Tsimhoni M, Lichter PR, Del Monte MA, Archer SM, Musch DC, Schertzer RM, et al. Assessing the need for posterior sclerotomy at the time of filtering surgery in patients with Sturge-Weber syndrome. Ophthalmology. 2003 Jul. 110(7):1361-3. [Medline].

  9. Facts and Comparisons. Drug Facts and Comparisons. St Louis; 1999.

  10. Freedman S, Walton D. Glaucoma in infants and children. Nelson L, Olitsky S, eds. Harley's Pediatric Ophthalmology. 5th ed. Philadelphia, PA 19106: Lippincott Williams & Wilkins; 2005. Chapter 14, 285-304.

  11. Hittner HM. Aniridia. Ritch R, ed. The Glaucomas. St Louis: Mosby; 1989. Vol 2: 869-884.

  12. Kirwan JF, Shah P, Khaw PT. Diode laser cyclophotocoagulation: role in the management of refractory pediatric glaucomas. Ophthalmology. 2002 Feb. 109(2):316-23. [Medline].

  13. Schottenstein EM. Peter's anomaly. Ritch R, ed. The Glaucomas. St Louis: Mosby; 1989. Vol 2.: 897-903.

  14. Shields MB. Axenfeld-Rieger syndrome. Ritch R, ed. The Glaucomas. St Louis, Mo: Mosby; 1989. Vol 2: 885-95.

  15. Singh OS. Nanophthalmos guidelines for diagnosis and therapy. Albert DM, Jakobiec FA, eds. Principles and Practice of Ophthalmology. 2000. Vol 4: 2846-2859.

  16. Walsh J, Muldoon T. Glaucoma associated with retinal vitreoretinal disorders. Ritch R, Shield MB, Krupin T, eds. The Glaucomas. 1996. Vol 2: 1055-1071.

  17. Weiss JS, Ritch R. Glaucoma in the phakomatoses. Ritch R, ed. The Glaucomas. St Louis: Mosby; 1989. Vol 2: 905-29.

  18. Wilson ME, Buckley EG, Kivlin JD. Pediatric Ophthalmology and Strabismus. AAO, Basic and Clinical Science Course. 1998. 6:330-345.

 
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Axenfeld-Rieger syndrome with iris atrophy, corectopia, and pseudopolycoria.
Female patient with plexiform neurofibroma (NF-1). Upper right eyelid involvement, associated with ipsilateral buphthalmos. In Image A (left), patient is aged 8 months; in Image B (right), patient is aged 8 years.
Female infant with Sturge-Weber syndrome. Facial port-wine nevus involves the left eyelid, associated with ipsilateral buphthalmos.
 
 
 
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