eMedicine Specialties > Ophthalmology > Intraocular Pressure

Glaucoma, Secondary Congenital: Follow-up

Author: Inci Irak-Dersu, MD, Assistant Professor, Director of Glaucoma Service, Department of Ophthalmology, University of Arkansas College of Medicine, Jones Eye Institute
Contributor Information and Disclosures

Updated: Feb 15, 2008

Follow-up

Further Inpatient Care

  • Patients rarely need hospitalization for IOP control.

Further Outpatient Care

  • 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.

Inpatient & Outpatient Medications

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

Complications

  • 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.3
    • 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%).4
  • 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

  • Prognosis in secondary congenital glaucoma is guarded.
    • Earlier age onset glaucoma usually is more difficult to manage. Patients need multiple procedures, each of which has its own risk.
    • Associated ocular problems (eg, strabismus, cataract, microphthalmia, amblyopia) also worsen the prognosis.
    • 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 that had 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.4
    • Patients with Lowe syndrome have a poor life expectancy.

Patient Education

Miscellaneous

Medicolegal Pitfalls

  • A chromosomal test is available for diagnosis of von Hippel-Lindau disease. Informing health insurance companies of positive test results remains controversial.
 


More on Glaucoma, Secondary Congenital

Overview: Glaucoma, Secondary Congenital
Differential Diagnoses & Workup: Glaucoma, Secondary Congenital
Treatment & Medication: Glaucoma, Secondary Congenital
Follow-up: Glaucoma, Secondary Congenital
Multimedia: Glaucoma, Secondary Congenital
References
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References

  1. Lopes JE, Wilson RR, Alvim HS, Shields CL, Shields JA, Calhoun J, et al. Central corneal thickness in pediatric glaucoma. J Pediatr Ophthalmol Strabismus. Mar-Apr 2007;44(2):112-7. [Medline].

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

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

  4. Agarwal HC, Sandramouli S, Sihota R, Sood NN. Sturge-Weber syndrome: management of glaucoma with combined trabeculotomy-trabeculectomy. Ophthalmic Surg. Jun 1993;24(6):399-402. [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. Jan 2004;111(1):112-7. [Medline].

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

  7. 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. Jul 2003;110(7):1361-3. [Medline].

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

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

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

  11. Shields MB. Textbook of Glaucoma. 3rd ed. Baltimore: Williams & Wilkins; 1992:235-257, 348-351.

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

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

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

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

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

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Further Reading

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Keywords

secondary congenital glaucoma, developmental glaucoma, open angle, closed angle, vision loss, visual deficit, congenital ocular anomalies, congenital ocular abnormalities, systemic anomalies

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Contributor Information and Disclosures

Author

Inci Irak-Dersu, MD, Assistant Professor, Director of Glaucoma Service, Department of Ophthalmology, University of Arkansas College of Medicine, Jones Eye Institute
Inci Irak-Dersu, MD is a member of the following medical societies: American Academy of Ophthalmology, American Glaucoma Society, and Arkansas Medical Society
Disclosure: Nothing to disclose.

Medical Editor

Andrew I Rabinowitz, MD, Consulting Staff, Department of Ophthalmology, Barnet Dulaney Perkins Eye Center
Andrew I Rabinowitz, MD is a member of the following medical societies: Aerospace Medical Association, American Academy of Ophthalmology, and American Medical Association
Disclosure: Nothing to disclose.

Pharmacy Editor

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
Disclosure: Nothing to disclose.

Managing Editor

Martin B Wax, MD, Clinical Professor, Department of Ophthalmology, University of Texas Southwestern Medical School; Vice President, Ophthalmology Research and Development, Head, Ophthalmology Discovery Research, Alcon Labs, Inc
Martin B Wax, MD is a member of the following medical societies: American Academy of Ophthalmology, American Glaucoma Society, and Society for Neuroscience
Disclosure: Alcon Labs Salary Employment

CME Editor

Lance L Brown, OD, MD, Ophthalmologist, Affiliated With Freeman Hospital and St John's Hospital, Regional Eye Center, Joplin, Missouri
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, and Pan-American Association of Ophthalmology
Disclosure: Nothing to disclose.

 
 
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