Primary Congenital Glaucoma

Author: Gerhard W Cibis, MD; Chief Editor: Hampton Roy Sr, MD more...

Updated: Aug 4, 2011

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Background
Pathophysiology
Epidemiology
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Background

By definition, primary congenital glaucoma is present at birth; however, its manifestations may not be recognized until infancy or early childhood. It is characterized by improper development of the eye's aqueous outflow system, leading to increased intraocular pressure (IOP), with consequent damage to ocular structures, resulting in loss of vision. Although the disease is relatively rare, the impact on visual development can be extreme. Early recognition and appropriate therapy of the glaucoma can significantly improve the child's visual future.

Pathophysiology

Primary congenital glaucoma is restricted to a developmental abnormality that affects the trabecular meshwork. This serves to distinguish it from other childhood glaucomas associated with other ocular and systemic congenital abnormalities, as well as childhood glaucomas that may be secondary to other ocular disorders, such as inflammation, trauma, and tumors.

Frequency

United States

Primary congenital glaucoma is estimated to affect fewer than 0.05% of ophthalmic patients and o.o5% of children. Although patients with the disease account for a significantly higher incidence in institutions for the blind, with various studies suggesting from 2-15%.

International

For genetic reasons, the incidence can be much higher, for example, in Saudi Arabia and among Romanian gypsies.

Mortality/Morbidity

The disease is bilateral in approximately 75% of cases.

Race

Congenital glaucoma affects all races

Sex

Male patients are found to have a higher incidence of the disease, comprising approximately 65% of cases.

Age

Primary congenital glaucoma usually is diagnosed at birth or shortly thereafter, and most cases are diagnosed in the first year of life.

Proceed to Clinical Presentation

Contributor Information and Disclosures

Author

Gerhard W Cibis, MD Clinical Professor, Director of Pediatric Ophthalmology Service, Department of Ophthalmology, University of Kansas School of Medicine

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.

Specialty Editor Board

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.

Simon K Law, MD, PharmD Associate Professor of Ophthalmology, Jules Stein Eye Institute, University of California, Los Angeles, David Geffen School of Medicine

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.

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: Nothing to disclose.

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.

References

Mandal AK, Chakrabarti D. Update on congenital glaucoma. Indian J Ophthalmol. Jan 2011;59 Suppl:S148-57. [Medline]. [Full Text].
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].
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].
Bejjani BA. Primary congenital glaucoma. Gene Tests. Available at http://www.genetests.org/. Accessed September 30, 2004.
DP Edward, Fajarananant TS, et al. A comprehensive update on congenital glaucoma. Current Pediatric Reviews. Feb 2008;4(1):19-30.
Sarfarazi Mansoor , inventors; U of Connecticut. Diagnosis of Primary Congenital Glaucoma. US patent 6207394. March 27 2001.

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