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Congenital Cataract

  • Author: Mounir Bashour, MD, PhD, CM, FRCSC, FACS; Chief Editor: Hampton Roy, Sr, MD  more...
 
Updated: Mar 22, 2016
 

Background

A cataract is an opacification of the lens. Congenital cataracts usually are diagnosed at birth. If a cataract goes undetected in an infant, permanent visual loss may ensue. Not all cataracts are visually significant. If a lenticular opacity is in the visual axis, it is considered visually significant and may lead to blindness. If the cataract is small, in the anterior portion of the lens, or in the periphery, no visual loss may be present.

Unilateral cataracts are usually isolated sporadic incidents. They can be associated with ocular abnormalities (eg, posterior lenticonus, persistent hyperplastic primary vitreous, anterior segment dysgenesis, posterior pole tumors), trauma, or intrauterine infection, particularly rubella.

Bilateral cataracts are often inherited and associated with other diseases. They require a full metabolic, infectious, systemic, and genetic workup. The common causes are hypoglycemia, trisomy (eg, Down, Edward, and Patau syndromes), myotonic dystrophy, infectious diseases (eg, toxoplasmosis, rubella, cytomegalovirus, and herpes simplex [TORCH]), and prematurity.

See What the Eyes Tell You: 16 Abnormalities of the Lens, a Critical Images slideshow, to help recognize lens abnormalities that are clues to various conditions and diseases.

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Pathophysiology

The lens forms during the invagination of surface ectoderm overlying the optic vesicle. The embryonic nucleus develops by the sixth week of gestation. Surrounding the embryonic nucleus is the fetal nucleus. At birth, the embryonic and fetal nuclei make up most of the lens. Postnatally, cortical lens fibers are laid down from the conversion of anterior lens epithelium into cortical lens fibers.

The Y sutures are an important landmark because they identify the extent of the fetal nucleus. Lens material peripheral to the Y sutures is lens cortex, whereas lens material within and including the Y sutures is nuclear. At the slit lamp, the anterior Y suture is oriented upright, and the posterior Y suture is inverted.

Any insult (eg, infectious, traumatic, metabolic) to the nuclear or lenticular fibers may result in an opacity (cataract) of the clear lenticular media. The location and pattern of this opacification may be used to determine the timing of the insult as well as the etiology.

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Epidemiology

Frequency

United States

Incidence is 1.2-6 cases per 10,000.

International

Incidence is unknown. Although the World Health Organization and other health organizations have made outstanding strides in vaccinations and disease prevention, the rate of congenital cataracts is probably much higher in underdeveloped countries.

Mortality/Morbidity

Visual morbidity may result from deprivation amblyopia, refractive amblyopia, glaucoma (as many as 10% post surgical removal), and retinal detachment.

Metabolic and systemic diseases are found in as many as 60% of bilateral cataracts.

Mental retardation, deafness, kidney disease, heart disease, and other systemic involvement may be part of the presentation.

Age

Congenital cataracts usually are diagnosed in newborns.

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Contributor Information and Disclosures
Author

Mounir Bashour, MD, PhD, CM, FRCSC, FACS Assistant Professor of Ophthalmology, McGill University Faculty of Medicine; Clinical Assistant Professor of Ophthalmology, Sherbrooke University; Medical Director, Cornea Laser and Lasik MD

Mounir Bashour, MD, PhD, CM, FRCSC, FACS is a member of the following medical societies: American Academy of Ophthalmology, American Association for Pediatric Ophthalmology and Strabismus, American College of International Physicians, American College of Surgeons, American Medical Association, American Society of Cataract and Refractive Surgery, American Society of Mechanical Engineers, American Society of Ophthalmic Plastic and Reconstructive Surgery, Biomedical Engineering Society, Canadian Medical Association, Canadian Ophthalmological Society, Contact Lens Association of Ophthalmologists, International College of Surgeons US Section, Ontario Medical Association, Quebec Medical Association, Royal College of Physicians and Surgeons of Canada

Disclosure: Nothing to disclose.

Coauthor(s)

C Corina Gerontis, MD Consulting Staff, Departments of Pediatrics and Ophthalmology, Schneider Children's Hospital/Long Island Jewish Medical Center

C Corina Gerontis, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Ophthalmology, American Association for Pediatric Ophthalmology and Strabismus, American Medical Association

Disclosure: Nothing to disclose.

Johanne Menassa, MD Staff Physician, Department of Ophthalmology, University of Laval Hospital, Quebec City

Disclosure: Nothing to disclose.

Specialty Editor Board

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.

J James Rowsey, MD Former Director of Corneal Services, St Luke's Cataract and Laser Institute

J James Rowsey, MD is a member of the following medical societies: American Academy of Ophthalmology, American Association for the Advancement of Science, American Medical Association, Association for Research in Vision and Ophthalmology, Florida Medical Association, Sigma Xi, Southern Medical Association, Pan-American Association of Ophthalmology

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

Richard W Allinson, MD Associate Professor, Department of Ophthalmology, Texas A&M University Health Science Center; Senior Staff Ophthalmologist, Scott and White Clinic

Richard W Allinson, MD is a member of the following medical societies: American Academy of Ophthalmology, American Medical Association, Texas Medical Association

Disclosure: Nothing to disclose.

References
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