Primary Congenital Glaucoma
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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.
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.
United States
Primary congenital glaucoma is estimated to affect less 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.
The disease is bilateral in approximately 75% of cases.
Congenital glaucoma affects all races
Male patients are found to have a higher incidence of the disease, comprising approximately 65% of cases.
Primary congenital glaucoma usually is diagnosed at birth or shortly thereafter, and most cases are diagnosed in the first year of life.
López-Garrido MP, Medina-Trillo C, Morales-Fernandez L, Garcia-Feijoo J, Martínez-de-la-Casa JM, García-Antón M, et al. Null CYP1B1 genotypes in primary congenital and nondominant juvenile glaucoma. Ophthalmology. 2013 Apr. 120(4):716-23. [Medline].
Gupta V, Jha R, Srinivasan G, Dada T, Sihota R. Ultrasound biomicroscopic characteristics of the anterior segment in primary congenital glaucoma. J AAPOS. 2007 Dec. 11(6):546-50. [Medline].
Tamcelik N, Ozkiris A. Long-term results of viscotrabeculotomy in congenital glaucoma: comparison to classical trabeculotomy. Br J Ophthalmol. 2008 Jan. 92(1):36-9. [Medline].
Abu-Amero KK, Deepak PE. Primary Congenital Glaucoma. GeneReviews. Available at http://www.ncbi.nlm.nih.gov/books/NBK1135/. Accessed: August 8, 2013.
Edward DP, Vajaranant TS, Al-Shahwan S, Bejjani BA. A comprehensive update on congenital glaucoma. Curr Ped Rev. Feb 2008. 4(1):19-30.
Mansoor S. Diagnosis of Primary Congenital Glaucoma. March 27, 2001;
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, American Ophthalmological Society
Disclosure: Nothing to disclose.
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, American Academy of Ophthalmology
Disclosure: Nothing to disclose.
Andrew A Dahl, MD, FACS Assistant Professor of Surgery (Ophthalmology), New York College of Medicine (NYCOM); Director of Residency Ophthalmology Training, The Institute for Family Health and Mid-Hudson Family Practice Residency Program; Staff Ophthalmologist, Telluride Medical Center
Andrew A Dahl, MD, FACS is a member of the following medical societies: American Academy of Ophthalmology, American College of Surgeons, American Intraocular Lens Society, American Medical Association, American Society of Cataract and Refractive Surgery, Contact Lens Association of Ophthalmologists, Medical Society of the State of New York, New York State Ophthalmological Society, Outpatient Ophthalmic Surgery Society
Disclosure: Nothing to disclose.
Simon K Law, MD, PharmD Clinical Professor of Health Sciences, Department 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, Association for Research in Vision and Ophthalmology, American Glaucoma Society
Disclosure: Nothing to disclose.
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.
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.
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, California Association of Ophthalmology, San Diego County Ophthalmological Society, Society of Military Ophthalmologists
Disclosure: Nothing to disclose.
Primary Congenital Glaucoma
Research & References of Primary Congenital Glaucoma|A&C Accounting And Tax Services
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