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

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Related Studies

Ontology: Congenital cataract (C0009691)

Definition (NCI) Cataract that is present at birth.
Concepts Congenital Abnormality (T019)
ICD9 743.30
ICD10 Q12.0
SnomedCT 156899008, 231966004, 609587005, 204123005, 79410001
English CATARACT CONGENITAL, Congenital cataract unspecif., Congenital cataract, unspecified, Cataract, congenital, congenital cataract (diagnosis), congenital cataract, Cataract congenital, Congenital cataract NOS, cataracts congenital, congenital cataracts, cataract congenital, Congenital Cataract, Congenital cataracts, Cataracts, congenital, Congenital cataract, unspecified (disorder), Congenital cataract (morphologic abnormality), Congenital cataract (disorder), cataract; congenital, congenita; congenita cataract, congenital; cataract, Congenital cataract, NOS, Congenital cataract [Ambiguous], Congenital cataract
Italian Cataratta congenita, Cataratta congenita, non specificata
Dutch congenitaal cataract, congenitaal cataract niet-gespecificeerd, cataract; congenitaal, congenita; cataracta membranacea congenita, congenitaal; cataract, Congenitaal cataract, cataract, congenitaal
French Cataracte congénitale non précisée, CATARACTE CONGENITALE, Cataracte congénitale
German kongenitaler Katarakt, kongenitaler Katarakt, unspezifisch, Cataracta congenita, KATARAKT KONGENITAL, Katarakt kongenital
Portuguese Catarata congénita NE, CATARATA CONGENITA, Catarata congénita
Spanish Catarata congénita no especificada, CATARATA CONGENITA, catarata congénita, no especificada (trastorno), catarata congénita, no especificada, catarata congénita (anomalía morfológica), catarata congénita (trastorno), catarata congénita, Catarata congénita
Japanese 詳細不明の先天性白内障, 先天性白内障, ショウサイフメイノセンテンセイハクナイショウ, センテンセイハクナイショウ
Czech Vrozená katarakta, blíže neurčená, Vrozená katarakta
Korean 선천 백내장
Hungarian Veleszületett szürkehályog, nem meghatározott, Veleszületett szürkehályog, Veleszületett cataracta