eMedicine Specialties > Ophthalmology > Intraocular Pressure

Glaucoma, Primary Congenital: Treatment & Medication

Author: Gerhard W Cibis, MD, Clinical Professor, Director of Pediatric Ophthalmology Service, Department of Ophthalmology, University of Kansas, Kansas City
Coauthor(s): Robert C Urban, Jr, MD, Medical Director, Glaucoma Associates, Oaklake Medical Center; Andrew A Dahl, MD, Director of Ophthalmology Teaching, Mid-Hudson Family Practice Institute, The Institute for Family Health; Assistant Professor of Surgery (Ophthalmology), New York College of Medicine
Contributor Information and Disclosures

Updated: Feb 13, 2009

Treatment

Medical Care

Primary congenital glaucoma almost always is managed surgically. Medical therapy is used only as a temporizing measure prior to surgery and to maximize pressure control after surgery.

Surgical Care

The primary surgical techniques are designed to eliminate the resistance to aqueous outflow created by the structural abnormalities in the anterior chamber angle. This may be accomplished through an internal approach with goniotomy or through an external approach with trabeculotomy.

  • Goniotomy is a technique in which abnormal tissue is incised under direct visualization with the aid of a goniolens. This presumably relieves the compressive traction on the anterior uvea on the trabecular meshwork, which eliminates any resistance imposed by incompletely developed inner trabecular meshwork.
  • Viscotrabeculotomy (canalotomy) uses a high-viscosity viscoelastic to open the canal.2
  • In trabeculotomy, the Schlemm canal is identified by external dissection, and the trabecular meshwork is incised by passing a probe into the canal and, then, rotating it into the anterior chamber. One advantage of this procedure is that it can be performed in eyes with cloudy corneas, which is not the case with goniotomy.
  • Both goniotomy and trabeculotomy have their advocates; however, reported success rates for both procedures are approximately 80%. The worst prognosis occurs in infants with elevated pressures and cloudy corneas present at birth. The most favorable outcome is seen in infants operated between the second and eighth month of life. Surgery has been found to be less effective in preserving vision, with increasing age.
  • When multiple goniotomies and/or trabeculotomies have failed, the surgeon usually resorts to a filtering procedure, such as trabeculectomy. This may be accomplished either with or without antimetabolites. Should these procedures fail, shunts may be used. In those situations, in which all else has failed, ciliary body destructive procedures may be useful.

More on Glaucoma, Primary Congenital

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

  1. Gupta V, Jha R, Srinivasan G, Dada T, Sihota R. Ultrasound biomicroscopic characteristics of the anterior segment in primary congenital glaucoma. J AAPOS. Dec 2007;11(6):546-50. [Medline].

  2. Tamcelik N, Ozkiris A. Long-term results of viscotrabeculotomy in congenital glaucoma: comparison to classical trabeculotomy. Br J Ophthalmol. Jan 2008;92(1):36-9. [Medline].

  3. Bejjani BA. Primary congenital glaucoma. Gene Tests. Available at http://www.genetests.org/. Accessed September 30, 2004.

  4. DP Edward, Fajarananant TS, et al. A comprehensive update on congenital glaucoma. Current Pediatric Reviews. Feb 2008;4(1):19-30.

  5. Sarfarazi Mansoor , inventors; U of Connecticut. Diagnosis of Primary Congenital Glaucoma. US patent 6207394. March 27 2001.

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Further Reading

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Keywords

primary congenital glaucoma, childhood glaucomas, glaucoma in children, increased intraocular pressure, increased IOP, optic nerve damage, vision loss, blindness, aqueous outflow

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Contributor Information and Disclosures

Author

Gerhard W Cibis, MD, Clinical Professor, Director of Pediatric Ophthalmology Service, Department of Ophthalmology, University of Kansas, Kansas City
Gerhard W Cibis, MD is a member of the following medical societies: American Academy of Ophthalmology, American Association for Pediatric Ophthalmology and Strabismus, and American Ophthalmological Society
Disclosure: Nothing to disclose.

Coauthor(s)

Robert C Urban, Jr, MD, Medical Director, Glaucoma Associates, Oaklake Medical Center
Robert C Urban, Jr, MD is a member of the following medical societies: Alpha Omega Alpha and American Academy of Ophthalmology
Disclosure: Nothing to disclose.

Andrew A Dahl, MD, Director of Ophthalmology Teaching, Mid-Hudson Family Practice Institute, The Institute for Family Health; Assistant Professor of Surgery (Ophthalmology), New York College of Medicine
Andrew A Dahl, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Ophthalmology, American College of Surgeons, American Medical Association, American Society of Cataract and Refractive Surgery, and Wilderness Medical Society
Disclosure: Nothing to disclose.

Medical Editor

Neil T Choplin, MD, Adjunct Clinical Professor, Department of Surgery, Section of Ophthalmology, Uniformed Services University of Health Sciences
Neil T Choplin, MD is a member of the following medical societies: American Academy of Ophthalmology, American Glaucoma Society, Association for Research in Vision and Ophthalmology, and California Medical Association
Disclosure: Nothing to disclose.

Pharmacy Editor

Simon K Law, MD, PharmD, Assistant Professor of Ophthalmology, Jules Stein Eye Institute; Chief of Section of Ophthalmology Surgical Services, Department of Veterans Affairs Healthcare Center, West Los Angeles
Simon K Law, MD, PharmD is a member of the following medical societies: American Academy of Ophthalmology, American Glaucoma Society, and Association for Research in Vision and Ophthalmology
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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