Congenital Cataract Clinical Presentation

  • Author: Mounir Bashour, MD, CM, FRCS(C), PhD, FACS; Chief Editor: Hampton Roy Sr, MD   more...
 
Updated: Dec 13, 2011
 

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]
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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.
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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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Contributor Information and Disclosures
Author

Mounir Bashour, MD, CM, FRCS(C), PhD, FACS  Assistant Professor of Ophthalmology, McGill University; Clinical Assistant Professor of Ophthalmology, Sherbrooke University; Medical Director, Cornea Laser and Lasik MD

Mounir Bashour, MD, CM, FRCS(C), PhD, FACS is a member of the following medical societies: American Academy of Ophthalmology, American Association for Pediatric Ophthalmology and Strabismus, American College of International Physicians, American College of Surgeons, American Medical Association, American Society of Cataract and Refractive Surgery, American Society of Mechanical Engineers, American Society of Ophthalmic Plastic and Reconstructive Surgery, Biomedical Engineering Society, Canadian Medical Association, Canadian Ophthalmological Society, Contact Lens Association of Ophthalmologists, International College of Surgeons US Section, Ontario Medical Association, Quebec Medical Association, and Royal College of Physicians and Surgeons of Canada

Disclosure: Nothing to disclose.

Coauthor(s)

Johanne Menassa, MD  Staff Physician, Department of Ophthalmology, University of Laval Hospital, Quebec City

Disclosure: Nothing to disclose.

C Corina Gerontis, MD  Consulting Staff, Departments of Pediatrics and Ophthalmology, Schneider Children's Hospital/Long Island Jewish Medical Center

C Corina Gerontis, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Ophthalmology, American Association for Pediatric Ophthalmology and Strabismus, and American Medical Association

Disclosure: Nothing to disclose.

Specialty Editor Board

Richard W Allinson, MD  Associate Professor, Department of Ophthalmology, Texas A&M University Health Science Center; Senior Staff Ophthalmologist, Scott and White Clinic

Richard W Allinson, MD, is a member of the following medical societies: American Academy of Ophthalmology, American Medical Association, and Texas Medical Association

Disclosure: Nothing to disclose.

Simon K Law, MD, PharmD  Associate Professor of Ophthalmology, Jules Stein Eye Institute, University of California, Los Angeles, David Geffen School of Medicine

Simon K Law, MD, PharmD is a member of the following medical societies: American Academy of Ophthalmology, American Glaucoma Society, and Association for Research in Vision and Ophthalmology

Disclosure: Nothing to disclose.

J James Rowsey, MD  Former Director of Corneal Services, St Luke's Cataract and Laser Institute

J James Rowsey, MD is a member of the following medical societies: American Academy of Ophthalmology, American Association for the Advancement of Science, American Medical Association, Association for Research in Vision and Ophthalmology, Florida Medical Association, Pan-American Association of Ophthalmology, Sigma Xi, and Southern Medical Association

Disclosure: Nothing to disclose.

Lance L Brown, OD, MD  Ophthalmologist, Affiliated With Freeman Hospital and St John's Hospital, Regional Eye Center, Joplin, Missouri

Disclosure: Nothing to disclose.

Chief Editor

Hampton Roy Sr, MD  Associate Clinical Professor, Department of Ophthalmology, University of Arkansas for Medical Sciences

Hampton Roy Sr, MD is a member of the following medical societies: American Academy of Ophthalmology, American College of Surgeons, and Pan-American Association of Ophthalmology

Disclosure: Nothing to disclose.

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. Koc F, Kargi S, Biglan AW, et al. The aetiology in paediatric aphakic glaucoma. Eye. Dec 2006;20(12):1360-5. [Medline].

  4. Birch EE, Cheng C, Stager DR Jr, et al. Visual acuity development after the implantation of unilateral intraocular lenses in infants and young children. J AAPOS. Dec 2005;9(6):527-32. [Medline].

  5. Sidorenko EI, Shirshov MV, Korkh NL. [Preliminary results of primary implantation of flexible intraocular lenses in infants under 1 year of age]. Vestn Oftalmol. Sep-Oct 2005;121(5):37-8. [Medline].

  6. 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].

  7. 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].

  8. 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 2011;118(12):2330-4. [Medline]. [Full Text].

  9. 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 2011;129(8):998-1003. [Medline].

  10. Biglan AW, Cheng KP, Davis JS, et al. Secondary intraocular lens implantation after cataract surgery in children. Am J Ophthalmol. Feb 1997;123(2):224-34. [Medline].

  11. Brady KM, Atkinson CS, Kilty LA, e al. Cataract surgery and intraocular lens implantation in children. Am J Ophthalmol. Jul 1995;120(1):1-9. [Medline].

  12. Buckley E, Lambert SR, Wilson ME. IOLs in the first year of life. J Pediatr Ophthalmol Strabismus. Sep-Oct 1999;36(5):281-6. [Medline].

  13. Cassidy L, Taylor D. Congenital cataract and multisystem disorders. Eye. Jun 1999;13 (Pt 3b):464-73. [Medline].

  14. Cheng KP, Hiles DA, Biglan AW, et al. Management of posterior lenticonus. J Pediatr Ophthalmol Strabismus. May-Jun 1991;28(3):143-9; discussion 150. [Medline].

  15. Mori M, Keech RV, Scott WE. Glaucoma and ocular hypertension in pediatric patients with cataracts. J AAPOS. Jun 1997;1(2):98-101. [Medline].

 
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