• Of the congenital variety, about one-third are inherited, one-third are associated with systemic genetic, metabolic, or maternal infectious disorders, and about one-third are idiopathic.
• A small number occur associated with other primary ocular abnormalities.
• Developmental cataracts can result from some metabolic disorders, toxic agents (steroids, radiation), and localized trauma.

• Represent a derangement of the normal developmental growth of the crystalline fibers of the central lens nucleus or peripheral cortex
• Frequently classified according to their morphology or etiology.
• Age at which they began or occurred is often determined by the location of the opacity within the lens.
• Any central opacity of 3 mm or more produces substantial visual disability.

• Lack of removal of a visually significant cataract at the appropriate early time can lead to irreversible deprivation amblyopia so that no amount of surgery, optical correction, and amblyopia therapy is of any help.
• Surgical removal of a cataract, while technically not usually difficult with today’s microsurgical instruments, leaves the eye without a lens (aphakic) and unable to focus without some type of optical correction (spectacles, contact or intraocular lenses). Unless rapid restoration of optical correction occurs, irreversible deprivation amblyopia may still occur after the cataract is removed. This is especially true when cataracts are unilateral.
• The aphakic pediatric eye is more prone to elevated intraocular pressures (glaucoma) and early retinal detachments, either of which can cause permanent visual loss, than a normal eye with its own lens.

• Prior to 1980, the majority of children treated for monocular cataracts had their best corrected vision only in the 20/200 to 20/800 range (legal blindness—best corrected visual acuity of 20/200 or worse); and bilateral cataracts usually obtained only best corrected visual acuities in the 20/80 to 20/200 range.
• Early surgery, better surgical techniques, and rapid optical correction now affords frequent corrected visual acuities in the 20/40 or better range for bilateral cataracts, and in the 20/40 to 20/200 range for monocular cataracts.
• In all cases, the onset or presence of nystagmus before the cataract is removed is an ominous sign for poor outcome.
• In general, successful treatment of the cataract in a child can be extremely difficult and must be accomplished very early in life if the cataract is congenital.
• Useful vision in children with unilateral cataracts in the newborn period can be restored/obtained only if the surgery is completed within the first 8 to 12 weeks of life.
• After 8 to 12 weeks, because of irreversible deprivation amblyopia, visual restoration becomes progressively more difficult to impossible.
• With congenital bilateral cataracts, the prognosis for visual rehabilitation is slightly better providing surgical removal and optical correction is also accomplished early, preferably by 3 months of age.
• Later onset has better prognosis because the visual system is more mature.

• Fetal alcohol syndrome
• Chromosomal disorders such as Down syndrome, trisomy 13-15, Turner syndrome
• Many craniofacial and mandibulofacial syndromes
• Dermatological syndromes such as congenital ichthyosis, hereditary ectodermal dysplasia, infantile poikiloderma
• Skeletal syndromes such as Marfan and Conradi syndromes
• Renal disorders such as Lowe or Alport syndrome
• Neurofibromatosis
• Myotonic dystrophy
• Metabolic and endocrine disorders such as galactosemia, hypoglycemia, diabetes mellitus
• Fabry disease and hypoparathyroidism
• Local ocular disorders with cataracts include aniridia, many of the anterior chamber dysgenesis syndromes, trauma, and those associated with ocular inflammatory processes (chronic iritis, uveitis) such as juvenile rheumatoid arthritis.

Question: Decreased visual responses
Significance: Cataracts may decrease vision

Question: Sun sensitivity (squinting in bright light)
Significance: Cataracts may increase light sensitivity

Question: Strabismus
Significance: Strabismus may indicate loss of vision in one eye

Question: White pupil
Significance: White pupil cataracts appear as white object in pupil

Question: Unequal or abnormal pupillary reflections in photographs
Significance: Cataract will block red reflex

Question: Nystagmus
Significance: Nystagmus is an ominous sign for degree of vision loss

Question: Careful family and prenatal history
Significance: 8% to 23% of cataracts are genetic

Question: Positive familial history or known history of disorder associated with cataracts
Significance: See associated disease section

Finding: Decreased vision
Significance: Cataracts may decrease visual acuity

Finding: Strabismus
Significance: May indicate loss of vision in one eye

Finding: White pupil (leukokoria) on flashlight examination
Significance: Cataracts appear as white pupil

Finding: Unequal or poor red fundus reflections by direct ophthalmoscopy
Significance: Cataract will interfere with seeing red reflex

Finding: Visual acuity assessment
Significance: Determine if cataracts caused visual loss

Finding: Presence/absence of nystagmus
Significance: Nystagmus is poor prognostic sign

Finding: Bilaterality of disease
Significance: Most bilateral cataracts are idiopathic, hereditary, or secondary to systemic disease

Finding: Size of globe
Significance: Micropthalmia suggests congenital cataracts

Finding: Thorough physical examination for systemic syndrome
Significance: Many diseases are associated with cataracts. See associated disease sections

• Bicromicroscopy
• Ultrasonography to visualize media behind opacity
• Electrophysiological analysis of visual system
• Selective work-up
• TORCH titers, including syphilis
• Urine for reducing substance (galactosemia), protein, and pH (Lowe syndrome)
• Blood glucose, calcium, phosphate
• Quantitative amino acids, RBC enzyme levels (galactokinase, gal-1-uridyltransferase)
• Genetic consultation; chromosome analysis

• With monocular, small cataracts, sometimes simple dilatation of the pupil of the cataractous eye and occlusion of the normal eye help overcome mild amblyopia and preserve acceptable vision without surgical removal of the cataract. Glasses may or may not be of additional help.
• With visually significant cataracts, surgical removal is the only way to begin therapy. However, without optical correction of the operated, aphakic eye, visual rehabilitation is impossible.
• Prior to age 2, optical correction of aphakia is most frequently accomplished with contact lenses or spectacles when the cataracts are bilateral. In unilateral cases, successful visual rehabilitation always requires extensive occlusion therapy to the normal eye for years. In this instance, optical correction of the unilateral aphakia is best obtained with a contact lens. Even when visual rehabilitation of the monocular aphakia is successful, normal binocular vision with three-dimensional and perfect depth perception is rarely possible.
• Refractive corneal surgery for optical correction of the aphakic is only rarely indicated in children and is not very successful.
• In children older than 2 years, especially those with unilateral cataracts, intraocular lenses (IOLs) have proved successful in appropriate cases, and are now used more frequently. They do afford faster visual rehabilitation and excellent chances of normal binocular vision.

• Without treatment, progressive visual loss is the natural course of a visually significant cataract. When the opacity is present at birth or very early in life, the visual loss quickly becomes irreversible.
• Once surgical removal and optical correction is started, the child, parents, and physician enter into an intensive and long rehabilitation period, lasting until visual maturity and stability is reached (usually, around 9 years old). Following this, yearly eye examinations are the minimum requirement.
• Parental and educational support services may be needed for those with residual visual handicap.
• Special local, state, and federal services for the visually handicapped and/or blind may be required, as not all children who have successful surgical results will have good vision.

• Lack of early diagnosis and treatment
• Lack of understanding of irreversible deprivation amblyopia

Q: Is surgical removal the same as visual cure?
A: No. This is the beginning of treatment that includes optical correction and amblyopia therapy.

Q: Once the cataract is removed, is intensive, extensive follow-up needed?
A: Yes. The visual prognosis is directly related to post-surgical treatment compliance.

Q: Is the cataract easier to treat when the child is older?
A: No. Irreversible deprivation amblyopia develops when the child is older, which precludes the chance for normal vision.

Childhood cataracts and other lens disorders. In: Del Monte MA, eds. Pediatric ophthalmology and strabismus: basic and clinical science course, section 6. San Francisco: American Academy of Ophthalmology, 1994–95:93–95.

Gimbel HV, Basti S, Ferensowicz M, DeBroff BM. Results of bilateral cataract extraction with posterior chamber intraocular lens implantation in children. Ophthalmology 1997;104:1737–1743.

Hertle RW, Quinn GE, Schaffer DB, Markowitz GD, Granet DB, Napolitano JA. Visual rehabilitation after cataract extraction in children using intraocular lenses. From the Proceedings of the Joint ISA & AAPO&S Meeting, Vancouver, Canada, June 19–23, 1994. In: Lennerstrand G, ed. Update on strabismus and pediatric ophthalmology. Boca Raton: CRC Press, Inc., 1995:511–515.

Hiles DA, Hered RW. Disorders of the lens. In: Isenberg SJ, ed. The eye in infancy. Chicago: Year Book, 1989:284–319.

Nelson LB, Ullman S. Congenital and developmental cataracts. In: Tasman W, Jaeger EA, eds. Duane’s clinical ophthalmology. Philadelphia: JB Lippincott, 1994:1–10.

von Noorden GK, Crawford MLJ. The effects of total unilateral occlusion versus lid suture on the visual system of infant monkeys. Invest Ophthalmol Vis Sci 1981;21:142–146.

Wiesel TN, Hubel DH. The period of susceptibility to the physiological effects of unilateral eye closure in kittens. J Physiol 1970;206:419–436.