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Cataract, Congenital: Treatment & Medication

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
Coauthor(s): Johanne Menassa, MD, Staff Physician, Department of Ophthalmology, University of Laval Hospital, Quebec City; C Corina Gerontis, MD, Consulting Staff, Departments of Pediatrics and Ophthalmology, Schneider Children's Hospital/Long Island Jewish Medical Center
Contributor Information and Disclosures

Updated: Jun 8, 2009

Treatment

Medical Care

Medical therapy is directed at the prevention of amblyopia.

Surgical Care

  • Cataract surgery is the treatment of choice and should be performed when patients are younger than 17 weeks to ensure minimal or no visual deprivation. Most ophthalmologists opt for surgery much earlier, ideally when patients are younger than 2 months, to prevent irreversible amblyopia and sensory nystagmus in the case of bilateral congenital cataracts. The delay in surgery is because of glaucoma. Since glaucoma occurs in 10% of congenital cataract surgery, many surgeons delay the cataract surgery.
    • Unfortunately, the improved surgical techniques of the 1990s have not lowered the incidence of glaucoma from the series published in the 1980s. The development of glaucoma (which occurs in later years) only occurs in cataract eyes that undergo surgery. This may be in part due to the immaturity of the angle at the time of surgery. A delay of a few weeks allows the angle of the immature eye to develop.
    • Koc and colleagues concluded that early age at cataract extraction and microcornea are risk factors for delayed-onset glaucoma.3
  • Extracapsular cataract extraction with primary posterior capsulectomy and anterior vitrectomy is the procedure of choice (via limbal or pars plana approach). Intracapsular cataract extraction in children is contraindicated because of vitreous traction and loss at the Wieger capsulohyaloid ligament. Vitrectomy instrumentation is the preferred method since the lens material is very soft. The whole procedure can be performed using one intraocular instrument. Young eyes develop capsular opacification very quickly, necessitating primary capsulectomy at the time of cataract extraction.
  • A study is underway in the United States to determine if intraocular lens placement in children younger than 6 months is a viable option. (Several articles have already been published in British journals.)
  • A study by the Retina Foundation of the Southwest in Texas compared intraocular lens (IOL) implantation with aphakic contact lenses (CLs) after the extraction of a unilateral cataract.4 Patients were as young as 6 months. They concluded that IOLs and aphakic CLs support similar visual acuity development after surgery for a unilateral cataract. IOLs may support better visual acuity development when compliance with CL wear is moderate to poor or when a cataract is extracted in a patient older than 1 year.
  • A study with promising preliminary results concerns the primary implantation of flexible IOLs in infants younger than 1 year.5 The population studied includes infants aged 3-11 months who have different forms of unilateral congenital cataracts.
  • A 2008 study by Capozzi and colleagues showed that, in the first 42 months of age, corneal power (Km) and axial length (AL) values are significantly different according to age.6 These findings have implications for the calculation of IOL power. Km values were significantly greater, and AL readings were shorter, in younger children (p<0.001). No differences according to gender were found. As a group, eyes from unilateral cataract cases had significantly longer AL readings than those from bilateral cataract cases (p=0.029). In a small subgroup of unilateral cataract cases, for which readings from the clear lens eye were available (n. 39), Km values of the affected eye were significantly greater than that of the fellow healthy eye (p=0.007).

Consultations

  • An ophthalmology consultation is essential to prevent visual loss as well as to make the appropriate diagnosis of the type of cataract.
  • A genetics evaluation is warranted if bilateral cataracts or any other anomalies are present.

Diet

Restriction of galactose, if galactosemia is present, may reverse the progression of the classic "oil droplet" cataract.

More on Cataract, Congenital

Overview: Cataract, Congenital
Differential Diagnoses & Workup: Cataract, Congenital
Treatment & Medication: Cataract, Congenital
Follow-up: Cataract, Congenital
References
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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. 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. 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].

  8. 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].

  9. 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].

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

  11. 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].

  12. 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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Further Reading

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Keywords

congenital cataract, congenital cataracts, cataract extraction, cataract surgery, deprivation amblyopia, refractive amblyopia, glaucoma, vision loss, visual deficit, blindness, rubella

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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.

Medical Editor

Richard W Allinson, MD, Associate Professor, Department of Surgery, 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.

Pharmacy Editor

Simon K Law, MD, PharmD, Assistant Professor of Ophthalmology, Jules Stein Eye Institute; Chief of Section of Ophthalmology Surgical Services, Department of Veterans Affairs Healthcare Center, West Los Angeles
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.

Managing Editor

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

CME Editor

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.

 
 
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