Congenital Cataract

Aka: Congenital Cataract, Cataract in Newborns

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II. Definitions

  1. Congenital Cataract
    1. Congenital lens opacity

III. Epidemiology

  1. Rare, uncommon cause of Amblyopia
  2. Incidence: 0.4% of newborns
  3. Responsible for 15-20% of blindness in children

IV. Causes: Primary (Congenital Cataract)

  1. Idiopathic in most cases
  2. Hereditary in one third of cases

V. Causes: Secondary (infantile Cataract)

  1. Congenital Infections (TORCH Infection)
    1. Toxoplasmosis
    2. Syphilis
    3. Rubella
    4. Cytomegalovirus
    5. Herpes Simplex Virus
  2. Galactokinase deficiency (Metabolic Disorder)
    1. Cataracts may be sole clinical finding
  3. Developmental
    1. Down Syndrome
    2. Turner's Syndrome
  4. Systemic Disease
    1. Wilson's Disease
    2. Alport's Syndrome

VI. Types

  1. Congenital Cataract
    1. Nuclear Cataract, nonprogressive
  2. Infantile Cataract
    1. Lamellar Cataract, progressive

VII. Exam: Screening

  1. Red Reflex
    1. Dull irregular or absent Red Reflex (or White Pupil)
  2. Visual Acuity
  3. Strabismus (Strabismus often seen 2nd to Cataract)

VIII. Management

  1. Lens Cataract Extraction
    1. Unilateral Cataract
      1. Best outcome for extraction age under 2 months
    2. Bilateral Cataract
      1. Less urgent then unilateral Cataract
      2. Extract Cataract at age 4 to 6 months of age
    3. Lens implants not used in children
      1. Safety of lens implants unclear in children
  2. Contact Lens placement
  3. Treat Amblyopia

IX. References

  1. Berson (1987) Ophthalmology Study Guide, AAO, p. 95-110
  2. Essman (1992) Am Fam Physician 46(4):1243-52 [PubMed]
  3. Mills (1999) Am Fam Physician 60(3):907-16 [PubMed]
  4. Russell-Eggitt (1997) J Cataract Refract Surg 23:664-8 [PubMed]
  5. Simon (2001) Am Fam Physician 64(4):623-8 [PubMed]

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