Congenital Uniocular Cataract Management Strategy

Denis Stark 

first posted in 2001   Updated September 24 2014

Traditional Management Recommendations:

Extraction of Cataract and correction of vision with a Contact Lens + a regime of Occlusion of the normal eye.
Needs to be performed before 2 months of age if visual improvement is to be expected. (Appendix 1 Amblyopia )
Surgery after that date of reduced benefit.

Effectivity of this Therapy:

Even with this ideal management a very limited number of patients gain useful vision because

  • The difficulty involved in maintaining a contact lens and occlusion.
  • Aphakia (absence of ocular lens) is almost as powerful an amblyogenic factor as the cataract itself.
  • These children invariably will progress to an insertion of an intraocular lens a a later date. This latter procedure with a sutured IOL is less desirable than the primary "in the bag" procedure with the IOL placement within the patient's lens capsule.

If no surgery is performed - there is no chance of visual recovery.

Modern  Management


Removal of Cataract and Insertion of an Intraocular Lens.
This still ideally should be performed in first months of life.
The strength of the lens should initially undercorrect the refractive error.
Occlusion + a contact lens may still be necessary for a few months- not years.

Spectacles will be necessary.

Advantages of this therapy


A closer simulation of ‘normal refraction’
Even early this century some series - very small with limited followup were supporting the benefit of this therapy. (Sinskey, O’Keefe, Lambert, Cheng,Vasavada)

As time has progressed the advocates of this therapy have multiplied. Our team has advocated and pursued this therapy for 15 years. We have avoided, to date, any of the serious complications mentioned below. Visual results have been rewarding in the majority of cases but disappointing in some. Although the surgery is complex our surgeon's skill and experience has made this operation appear routine. The management of a congenital cataract remains a long term commitment for the patient, the parents and the ophthalmic team.

Disadvantages of this therapy


More complicated surgery - more potential complications
Still uncertain response.
Advantages ar becoming more obvious.

Theoretical advantages


Improved chance of recovery of some vision because of better stimulation by better focus.
Still reduced chance of recovery - dependent on age at time of surgery and age when catarat developed.
Visual improvement is likely but end result sometimes may be only poor to moderate.
.

Theoretical disadvantages


Still a limited chance of visual recovery.
Will require patching to ovecome amblyopia spectacle correction will be required and possibly a contact lensin the first 6 montns

Complications of the Surgery


The Worst: Possible loss of the eye 1/10000

-Short term possibilty of  infection 1/1000


-Long term followup is required because other problems may require management including Corneal complications, glaucoma, retinal detachment,

The need for more operations unplanned at this stage may eventuate


Glaucoma
retinal
corneal

The need for more planned operations


Exchange of IOL
Addition of IOL
Possible Clearing of visual pathway

With a very experienced Cataract Surgeon , in spite of the risks enumerated cataract extraction and simultaneous lens insertion probably gives an infant the best chance of obtaining the best visual result.

        Appendix

Amblyopia  (Click here for more details regarding AMBLYOPIA)

The vision of an infant is still developing. Anything, which interferes with this development, can result in permanent failure of vision.

The factors which can be involved:

1 Poor focus - the need for corrective lens.

2.An opacity in the line of vision

3. Poor aim- a turned eye.

Congenital cataract results in an opacity in the line of vision.

If this cataract is removed the focus of the eye is then extremely poor.

- Unless a lens is placed in the system

Thus we have 2 factors adding to the effect of visual failure.

Time: The first months of life are critical. The failure to stimulate the visual brain in this period has a very severe effect on visual development making it, in the past, ALMOST IMPOSSIBLE TO DEVELOP VISION LATER.

Today with modern surgery this window appears to have been lengthened but still early intervention is still of extreme importance