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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)
Sources
- EyeWiki Cataracts in Children
- EyeWiki Anterior Segment Developmental Anomalies (ASDA)
- AAO Pediatric Cataracts: Overview
- The Wills Eye Manual: Office and Emergency Room Diagnosis and Treatment of Eye Disease; Nika Bagheri MD, Brynn Wajda MD, et al; Lippincott Williams&Wilkins; 7th Edition (2016)
- Kanski’s Clinical Ophthalmology: A Systematic Approach; Jack J. Kanski MD, Brad Bowling MD; Saunders Ltd.; 8th Edition (2015)