Congenital and Juvenile Cataract

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Bilateral Congenital Cataract

Aetiology

  • Idiopathic
  • Hereditary; typically autosomal dominant
  • Chromosomal aberrations: including Trisomy 21, 18, and 13
  • Associated with syndromes: e.g., Hallermann-Streiff, Crouzon, Apert’s, Pierre Robin Sequence
  • Intrauterine infections: TORCH (toxoplasmosis, others [syphilis, hepatitis B, varicella zoster virus, HIV, parvovirus B19], rubella, cytomegalovirus, herpes simplex virus)
  • Metabolic diseases: including galactosemia, mannosidosis, diabetes mellitus, Fabry, Wilson’s disease
  • Renal: Alport syndrome, Lowe syndrome
  • Ocular anomalies: Aniridia, Anterior Segment Developmental Anomalies (ASDA)
  • Iatrogenic causes: Steroids, radiation

Work-up

  • If a known autosomal dominant inheritance pattern exists, a workup may be omitted
  • Urine analysis: Amino acids (Lowe’s syndrome)
  • Blood tests: Calcium (Hypocalcemia/Hyperparathyroidism), Glucose (Hypoglycemia), TORCH titers; (Galactosemia screening as part of newborn screening in Switzerland)
  • Karyotyping: Trisomy 13, 18, 21, Turner syndrome, Cri-du-Chat syndrome
  • Audiogram: Congenital rubella, Alport syndrome
  • Ultrasound if retina cannot be visualised

Treatment

  • Surgery depending on severity; if clinical significance is suspected, operate after 6-8 weeks

Unilateral Congenital Cataract

Aetiology

  • Idiopathic in 80% of cases
  • Ocular anomalies: Persistent Hyperplastic Primary Vitreous (PHPV), Anterior Segment Developmental Anomalies (ASDA)
  • Traumatic causes
  • Intrauterine infections: TORCH (toxoplasmosis, others [syphilis, hepatitis B, varicella zoster virus, HIV, parvovirus B19], rubella, cytomegalovirus, herpes simplex virus)

Work-up

  • TORCH titers
  • Rule out trauma (child abuse)

Treatment

  • Surgery depending on severity; if clinical significance is suspected, operate at the age of 4-6 weeks

Complications in Aphakia

  • Aphakic Glaucoma in 15% (usually diagnosed 5-6 years postoperatively)

 

Juvenile Cataract

Indication for Cataract Surgery

  • For unilateral cataract: Vision in the affected eye <0.3/0.25 (optotype line acuity)! (As the vision prognosis post unilateral cataract surgery is very poor despite amblyopia therapy, aiming for around 0.3 vision)
  • For bilateral cataract: Better vision prognosis, thus more liberal criteria for surgery

Surgery

  • In addition to phacoemulsification, a posterior capsulectomy and anterior vitrectomy are performed (due to increased inflammatory reaction)
  • Before the age of 2: Aphakia; secondary implantation (intraocular lens or Artisan lens, depending on the integrity of the capsular bag) preferably delayed until the age of 12-14 years
  • Primary IOL implantation from 1-2 years of age (not before 1 year old!)
  • Patients are usually left hyperopic depending on age

Postoperative Care

  • For unilateral aphakia: Aphakic contact lens and from around the age of 2 years, with bifocal or progressive glasses with near addition (+2.5 or +3.0)
  • For bilateral aphakia: Aphakic glasses and from around the age of 2 years, with near addition (+2.5 or +3.0)
  • For pseudophakia: Glasses with near addition on both sides, even in cases of unilateral pseudophakia (according to studies, this leads to better vision outcomes)

 

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