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History
Congenital cataracts are present at birth but may not be identified until later in life. Prenatal and family history is
helpful.
Some cataracts are static, but some are progressive. This explains why not all congenital cataracts are
identified at birth.
Anterior polar cataract and nuclear cataract are usually static, although they may rarely progress.
Cataracts that typically progress include posterior lenticonus, persistent hyperplastic primary vitreous, lamellar,
sutural, and anterior or posterior subcapsular. They usually have a better prognosis because they only usually
begin to obstruct the vision after the critical period of visual development has passed.
Not all cataracts are visually significant. If a lenticular opacity is in the visual axis, it usually is considered visually
significant and requires removal.
Cataracts in the center of the visual axis that are greater than 3 mm in diameter are generally considered
visually significant. This principle is furthermore correlated with the clinical ophthalmological examination of the
patient.
A study by the Department of Pediatric Ophthalmology of the Wills Eye Hospital concluded that, in terms of the
risk factor for amblyopia, more important than the cataract size is the anisometropia induced by the congenital
anterior lens opacities (CALOs).[1] Patients with CALOs who have anisometropia of 1 diopter (D) or more are
6.5 times more likely to develop amblyopia.[1]
Physical
A lenticular opacity is called a cataract. Not all cataracts are visually significant.
Description of a congenital cataract must include location, color, density, and shape for purposes of
identification.
An irregular red reflex is the hallmark of visual problems. If an irregular red reflex is detected at the initial
screening, this is usually an indication that a congenital cataract might be present and an ophthalmology
consultation is warranted.
Leukocoria or white reflex can be the presenting sign of a cataract. In fact, in a 2008 study by Haider et al, 60%
of patients who presented with leukocoria had congenital cataracts (18% unilateral and 42% bilateral).[2] Other
causes included retinoblastoma (11% unilateral and 7% bilateral), retinal detachment (2.8% unilateral and 1.4%
bilateral), bilateral persistent hyperplastic primary vitreous (4.2%), and unilateral Coats disease (4.2%).[2]
Slit lamp examination of both eyes (dilated pupil) not only may confirm the presence of a cataract but also may
identify the time when the insult occurred in utero and if there is other systemic or metabolic involvement.
Dilated fundus examination is recommended as part of the ocular examination for both unilateral cataract cases
and bilateral cataract cases.
Causes
The most common etiology includes intrauterine infections, metabolic disorders, and genetically transmitted
syndromes. One third of pediatric cataracts are sporadic; they are not associated with any systemic or ocular
diseases. However, they may be spontaneous mutations and may lead to cataract formation in the patient's
offspring. As many as 23% of congenital cataracts are familial. The most frequent mode of transmission is
autosomal dominant with complete penetrance. This type of cataract may appear as a total cataract, polar
cataract, lamellar cataract, or nuclear opacity. All close family members should be examined.
Infectious causes of cataracts include rubella (the most common), rubeola, chicken pox, cytomegalovirus,
herpes simplex, herpes zoster, poliomyelitis, influenza, Epstein-Barr virus, syphilis, and toxoplasmosis.
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References
1. Ceyhan D, Schnall BM, Breckenridge A, et al. Risk factors for amblyopia in congenital anterior lens opacities.
J AAPOS. Dec 2005;9(6):537-41. [Medline].
2. Haider S, Qureshi W, Ali A. Leukocoria in children. J Pediatr Ophthalmol Strabismus. May-Jun
2008;45(3):179-80. [Medline].
3. Kumar M, Kaur P, Kumar M, Khokhar S, Dada R. Molecular and structural analysis of genetic variations in
congenital cataract. Mol Vis. 2013;19:2436-50. [Medline]. [Full Text].
4. Koc F, Kargi S, Biglan AW, et al. The aetiology in paediatric aphakic glaucoma. Eye. Dec
2006;20(12):1360-5. [Medline].
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intraocular lenses in infants and young children. J AAPOS. Dec 2005;9(6):527-32. [Medline].
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in infants under 1 year of age]. Vestn Oftalmol. Sep-Oct 2005;121(5):37-8. [Medline].
7. Capozzi P, Morini C, Piga S, et al. Corneal Curvature and Axial Length values in children with
Congenital/Infantile Cataract in the first 42 Months of life. Invest Ophthalmol Vis Sci. May 23 2008;[Medline].
8. Hoevenaars NE, Polling JR, Wolfs RC. Prediction error and myopic shift after intraocular lens implantation in
paediatric cataract patients. Br J Ophthalmol. Aug 2011;95(8):1082-5. [Medline].
9. Plager DA, Lynn MJ, Buckley EG, Wilson ME, Lambert SR. Complications, adverse events, and additional
intraocular surgery 1 year after cataract surgery in the infant aphakia treatment study. Ophthalmology. Dec
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10. Vasavada AR, Praveen MR, Vasavada VA, Shah SK, Vasavada V, Trivedi RH. Corneal endothelial
morphologic assessment in pediatric cataract surgery with intraocular lens implantation: a comparison of
preoperative and early postoperative specular microscopy. Am J Ophthalmol. Aug 2012;154(2):259-265.e1.
[Medline].
11. Goggin M, Moore S, Esterman A. Outcome of toric intraocular lens implantation after adjusting for anterior
chamber depth and intraocular lens sphere equivalent power effects. Arch Ophthalmol. Aug
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12. Biglan AW, Cheng KP, Davis JS, et al. Secondary intraocular lens implantation after cataract surgery in
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