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Congenital cataract

Definition

A congenital cataract is a clouding of the lens of the eye that is present at birth. The lens of the eye is normally clear. It focuses light that comes into the eye onto the retina.

Alternative Names

Cataract - congenital

Causes

Unlike most cataracts, which occur with aging, congenital cataracts are present at birth.

Congenital cataracts are rare. In most people, no cause can be found.

Congenital cataracts often occur as part of the following birth defects:

  • Chondrodysplasia syndrome
  • Congenital rubella
  • Conradi-Hünermann syndrome
  • Down syndrome (trisomy 21)
  • Ectodermal dysplasia syndrome
  • Familial congenital cataracts
  • Galactosemia
  • Hallermann-Streiff syndrome
  • Lowe syndrome
  • Marinesco-Sjögren syndrome
  • Pierre-Robin syndrome
  • Trisomy 13

Symptoms

Congenital cataracts most often look different than other forms of cataract.

Symptoms may include:

  • An infant does not seem to be visually aware of the world around them (if cataracts are in both eyes)
  • Gray or white cloudiness of the pupil (which is normally black)
  • The "red eye" glow of the pupil is missing in photos, or is different between the 2 eyes
  • Unusual rapid eye movements (nystagmus)

Exams and Tests

To diagnose congenital cataract, the infant should have a complete eye exam by an ophthalmologist. The infant may also need to be examined by a pediatrician who is experienced in treating inherited disorders. Blood tests or x-rays may also be needed.

Treatment

If congenital cataracts are mild and do not affect vision, they may not need to be treated, especially if they are in both eyes.

Moderate to severe cataracts that affect vision, or a cataract that is in only 1 eye, will need to be treated with cataract removal surgery. In most (noncongenital) cataract surgeries, an artificial intraocular lens (IOL) is inserted into the eye. The use of IOLs in infants is controversial. Without an IOL, the infant will need to wear a contact lens.

Patching to force the child to use the weaker eye is often needed to prevent amblyopia.

The infant may also need to be treated for the inherited disorder that is causing the cataracts.

Outlook (Prognosis)

Removing a congenital cataract is usually a safe, effective procedure. The child will need follow-up for vision rehabilitation. Most infants have some level of "lazy eye" (amblyopia) before the surgery and will need to use patching.

Possible Complications

With cataract surgery there is a very slight risk of:

  • Bleeding
  • Infection
  • Inflammation

Infants who have surgery for congenital cataracts are likely to develop another type of cataract, which may need further surgery or laser treatment.

Many of the diseases that are associated with congenital cataract can also affect other organs.

When to Contact a Medical Professional

Call for an urgent appointment with your baby's health care provider if:

  • You notice that the pupil of one or both eyes appears white or cloudy.
  • The child seems to ignore part of their visual world.

Prevention

If you have a family history of inheritable disorders that could cause congenital cataracts, consider seeking genetic counseling.

Gallery

Eye
The eye is the organ of sight, a nearly spherical hollow globe filled with fluids (humors). The outer layer or tunic (sclera, or white, and cornea) is fibrous and protective. The middle layer (choroid, ciliary body and the iris) is vascular. The innermost layer (the retina) is nervous or sensory. The fluids in the eye are divided by the lens into the vitreous humor (behind the lens) and the aqueous humor (in front of the lens). The lens itself is flexible and suspended by ligaments which allow it to change shape to focus light on the retina, which is composed of sensory neurons.
Crossed eyes
People are very sensitive to other individuals' eye positions. By looking at another person's eye position, one can very effectively gauge where they are looking. People are also sensitive to eyes that are not looking in the same direction, which is referred to as crossed eyes (strabismus). Other more specific medical terms refer to eyes turned either outward or inward, or that are abnormally rotated. Any appearance of crossed eyes in young children should be immediately evaluated, as should recent onset of crossed eyes in an adult.
Rubella on an infant's back
Rubella is often called three-day measles. Because of the very effective vaccine, rubella is seldom seen today. The rubella vaccine is given in combination with measles (rubeola) and mumps. (Image courtesy of the Centers for Disease Control and Prevention.)
Eye
The eye is the organ of sight, a nearly spherical hollow globe filled with fluids (humors). The outer layer or tunic (sclera, or white, and cornea) is fibrous and protective. The middle layer (choroid, ciliary body and the iris) is vascular. The innermost layer (the retina) is nervous or sensory. The fluids in the eye are divided by the lens into the vitreous humor (behind the lens) and the aqueous humor (in front of the lens). The lens itself is flexible and suspended by ligaments which allow it to change shape to focus light on the retina, which is composed of sensory neurons.

References

Cioffi GA, Liebmann JM. Diseases of the visual system. In: Goldman L, Schafer AI, eds. Goldman-Cecil Medicine. 26th ed. Philadelphia, PA: Elsevier; 2020:chap 395.

Lambert SR, Cotsonis G, DuBois L, et al; Infant Aphakia Treatment Study Group. Long-term effect of intraocular lens vs contact lens correction on visual acuity after cataract surgery during infancy: a randomized clinical trial. JAMA Ophthalmol. 2020;138(4):365-372. PMID: 32077909 pubmed.ncbi.nlm.nih.gov/32077909/.

Lambert SR, Wilson ME Jr, Plager DA, Lloyd IC. Update on pediatric cataracts. AAPOS Webinar. May 12, 2017. www.aao.org/annual-meeting-video/update-on-pediatric-cataracts. Accessed November 16, 2021.

Örge FH. Examination and common problems in the neonatal eye. In: Martin RJ, Fanaroff AA, Walsh MC, eds. Fanaroff and Martin's Neonatal-Perinatal Medicine. 11th ed. Philadelphia, PA: Elsevier; 2020:chap 95.

Wevill M. Epidemiology, pathophysiology, causes, morphology, and visual effects of cataract. In: Yanoff M, Duker JS, eds. Ophthalmology. 5th ed. Philadelphia, PA: Elsevier; 2019:chap 5.3.

Last reviewed September 3, 2021 by Franklin W. Lusby, MD, Ophthalmologist, Lusby Vision Institute, La Jolla, CA. Also reviewed by David Zieve, MD, MHA, Medical Director, Brenda Conaway, Editorial Director, and the A.D.A.M. Editorial team..

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