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What
are the causes of congenital cataract?
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My baby’s pediatrician
referred us to a pediatric ophthalmologist for confirmation of congenital
cataract. What should we expect from the consultation? Will blood exams be
requested?
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How is
congenital cataract treated?
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Will my
child see after surgery for congenital cataract?
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Is there a
link between infantile cataract and glaucoma?
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Does congenital cataract present with glaucoma?
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What are the causes of
congenital cataract?
Bilateral congenital cataracts are often
inherited as an autosomal dominant trait, however, recessive and x-linked
patterns have also been described. Approximately 5-10% of bilateral cataracts
are associated with a systemic disorder. Unilateral infantile cataracts are
rarely caused by a systemic disease, except in some cases of intrauterine
infections such as rubella. About 20-30% of rubella cataracts are unilateral.
Thus, the presence of a unilateral cataract does not completely rule out the
possibility of an associated systemic disorder, but it is highly
suggestive that the cataract is caused by local dysgenesis. Asymmetric
bilateral lens opacities may be misinterpreted as a unilateral cataract.
Below is a list of common causes
of congenital cataracts:
Unilateral Cataracts
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Idiopathic
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Posterior lenticonus
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Persistent Hyperplastic Primary
Vitreous
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Traumatic
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Intrauterine infection (such as
rubella)
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Anterior Segment Dysgenesis
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Posterior Pole Tumor
Bilateral Cataracts
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Idiopathic
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Hereditary (without systemic
disease)
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Autosomal dominant
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Autosomal recessive
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X-linked
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Systemic diseases
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Intrauterine infection
(TORCHES)
-
Ocular abnormalities (such as
aniridia, anterior segment dysgenesis)
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My baby’s pediatrician
referred us to a pediatric ophthalmologist for confirmation of congenital
cataract. What should we expect from the consultation? Will blood exams be
requested?
The first part of the
consultation is the history. The parents will be asked questions regarding the
onset of the cataract and developmental milestones of your child. The family
history is also critical. Then, a complete ophthalmic evaluation shall be
done. If a hereditary type of cataract is suspected, both the parents should be
examined. Laboratory workup is not necessary if the cataracts can be positively
defined as hereditary without other systemic abnormalities. The required
laboratory tests for congenital cataract include serum TORCH titer and VDRL. A
urine test for reducing substance (sugar) after a milk feeding is necessary for
bilateral cases. The blood exam is costly but important, particularly if the
examination does not clearly reveal a specific diagnosis. The serum TORCH titer
will help rule out an intrauterine infection.
Children with congenital
cataracts, particularly those with questionable inheritance pattern and possible
systemic conditions, are referred to clinical geneticists for co-management.
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How is congenital cataract treated?
The main goal for treating
congenital cataract is to provide a clear retinal image as soon as possible to
avoid irreversible amblyopia, the most common cause of poor vision after
cataract surgery in children. Amblyopia may occur whether only one eye or both
eyes are involved, so visually significant congenital cataract must be visually
rehabilitated as soon as possible. Treatment is urgent because bilateral
congenital cataracts that obscure the visual axis will often result in sensory
nystagmus and a bilateral poor visual outcome if not treated by 2 months of age.
The use of intraocular
lenses is the accepted method for treating aphakia (absence of a natural lens)
in children that underwent cataract surgery. Because of the significant change
in eye size during the first year of life, implantation of ocular lenses in
infants is controversial. By 2 years of age, however, the eye is almost adult
size. For the most part, intraocular lenses are becoming the standard in
children older than 2 years old. Since the posterior capsule invariably
opacifies after a lens implantation, a secondary YAG capsulotomy or secondary
procedure is usually necessary. Complications of cataract surgery are unusual,
but late complications include retinal detachment, immediate or late glaucoma,
retinal hemorrhages, and endophthalmitis.
The treatment does not stop
after the surgery. Visual rehabilitation continues by the use of spectacles and
contact lenses and amblyopia therapy.
Spectacles are an option to
correct bilateral aphakia in children. However, they do not provide constant
correction. They are not used for monocular aphakia because of the unilateral
magnification of the aphakic lens. Contact lenses are the standard treatment
for either unilateral or bilateral aphakia in newborns. Aphakic spectacles can
be used as a backup for contact lenses in bilaterally aphakic children.
Occlusion therapy is a must
after surgery in monocular congenital cataract in order to treat amblyopia. The
amount of patching should be based on the severity of the amblyopia. The role
of the parents in the treatment of amblyopia is critical. They should be
educated about the importance of a clear retinal image, and the need for
occlusion therapy.
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Unlike adult cataract,
the management of cataracts in children is a big challenge because the role of
early visual rehabilitation is critical in the prevention of irreversible
amblyopia. Until two decades ago, many ophthalmologists would not even attempt
to do surgery on unilateral congenital cataracts because of the poor prognosis
associated with this condition. Ophthalmologists today operate as early as the
first few weeks of life, and the prognosis is much improved.
In general, patients
with monocular cataracts have a very poor prognosis for obtaining fusion, and
almost all cases eventually develop strabismus. However, if surgery and optical
correction are provided by 2 months of age, good visual acuity and good
binocularity with stereopsis are possible. The important point in the
management of these cases is that very early surgery (less than 2 months of age)
is performed with immediate contact lens fitting, and part-time monocular
occlusion for amblyopia is initiated during the first few months of life.
Binocular cataracts can
also cause amblyopia. It is important that binocular cataracts are treated with
urgency and are operated during the first few weeks of life. If visually
significant bilateral congenital cataracts are not cleared by 2 months of age,
patients will develop sensory nystagmus and very poor visual acuity in most
cases. Surgery by 2 months of age is definitely the treatment of choice.
However, older children who present late should still be considered for cataract
surgery, even though they present with bilateral cataracts and nystagmus. The
exceptions to this are those patients with an abnormality of the retina or optic
nerve, such as aniridia. Cataract surgery usually does not improve vision in
these cases because the anatomic abnormality of the optic nerve or macula limits
the visual acuity potential of the child.
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Is there a
link between infantile cataract and glaucoma?
Yes, there are several links between these
two conditions. All children who undergo cataract surgery, especially in
infancy, are at life long risk for developing glaucoma after surgery. Although
this risk is not very high, the serious visual effects of glaucoma can be
detected even at the earliest stage. This is why regular follow-up with the
pediatric ophthalmologist is important, even after the surgery. Some eyes with
congenital cataract are also at risk for glaucoma even if no surgery is done
yet. This only means that there are other problems in the eye other than just
the cataract. Some eye conditions that are associated with glaucoma would
include eye and body syndromes such as aniridia (absent iris), anterior segment
dysgenesis, congenital rubella syndrome, and persistent hyperplastic primary
vitreous (PHPV). These eye conditions sometimes present initially with cataract
and glaucoma.
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Does congenital cataract present with glaucoma?
The
following conditions present with infantile cataract and glaucoma:
Aniridia literally means absence of an
iris. However, this is somewhat a misnomer because there is always some
amount of iris tissue present. This condition commonly presents with other
ocular problems that involve the other parts of the eye such as cataract, lens
subluxation, glaucoma, optic nerve hypoplasia, and foveal hypoplasia. The
latter two results in poor vision.
Anterior segment dysgenesis is a spectrum
of eye disorders that involves the anterior part of the eye. These diseases
present abnormal development of the cornea, iris and lens during
embryogenesis. The structural abnormalities that result from the dysgenesis
produce corneal opacity, glaucoma, and cataract.
PHPV represents an abnormal regression of
the primitive hyaloid vascular system in the eye. This produces a
fibrovascular stalk that connects the optic disc and the posterior part of the
lens (posterior cataract). A retrolental membrane forms at the posterior part
of the lens and it may extend to the ciliary processes. Over time, the
membrane can contract, pulling the ciliary processes centrally. If left
untreated, severe forms of PHPV can lead to shallowing of the anterior chamber
and angle closure glaucoma.
Systemic findings of congenital rubella
syndrome include congenital heart defects, hearing loss, and mental
retardation. Ocular findings include pigmentary retinopathy (25%), cataract
(15%), strabismus (20%), microphthalmos (15%), optic atrophy (10%), corneal
haze (10%), glaucoma (10%), and phthisis bulbi (2%). The retinopathy is
stable and usually does not affect vision. Rubella cataracts are caused by
invasion of the lens by the rubella virus, and are bilateral in 80% of all
cases. These cataracts may present with a hazy cornea caused by either
congenital glaucoma or keratitis. Treatment of the cataract involves removing
the entire lens cortex, since the patient’s tendency to postoperative
inflammation is increased if residual cortex is left after the surgery.
This is an x-linked disorder that presents
with bilateral congenital cataracts and often with bilateral congenital
glaucoma. Infants show severe developmental delay, hypotonia, and renal
failure with aminoaciduria. The visual prognosis is poor since there is
progression of neurological and renal deterioration. Death occurs in late
childhood. A dilated slit lamp examination of the patient’s mother shows
multiple punctate white snowflake opacities of the lens periphery.
There are several ways of reaching the ophthalmologists of EYE REPUBLIC Ophthalmology Clinic:
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ONLINE ACCESS
WEBSITES.
http://www.EyeRepublic.com.ph - EYE REPUBLIC
Ophthalmology Clinic
http://www.OCP.com.ph - Ophthalmic
Consultants Philippines Co. -
http://www.LASIK.com.ph - Refractive
Surgery Resource
http://www.Cataract.com.ph - Cataract Surgery Resource
http://www.Eye.com.ph - Eye
Information Online
http://www.EyeDoc4Kids.com.ph - Eye Information for Kids
http://www.Retina.com.ph - Retina
Surgery Resource
http://www.Glaucoma.com.ph - Glaucoma
Online
http://www.Uveitis.com.ph - Uveitis Online
EMAIL. After writing down your comments,
suggestions, problems and/or questions, kindly tell us how
to get in touch with you by providing your name, email,
home/office numbers, and mobile phone.
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Glaucoma Service - glaucoma@EyeRepublic.com.ph
Cataract Service - cataract@EyeRepublic.com.ph
Doctors - eyemd@EyeRepublic.com.ph
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EYE REPUBLIC
Ophthalmology Atlas
CLINIC INFORMATION
Mobile E-Yellow Pages. Via
SMS, text LUK4 EYEREPUBLIC
(send to 2851 for Globe and Sun Cellular, and 2951 for Smart). |
EYE REPUBLIC Ophthalmology Clinic
Manila
3/F Don Santiago Building Units 309-310
1344 Taft Avenue, Ermita
Manila, 1000 Philippines
Direct and Fax: (632) 536-2398
Trunk Line: (632) 523-8271 to 79 local 30
Mobile: (63917) 899-2020
Map and directions
|
EYE REPUBLIC
Ophthalmology Clinic
Asian Hospital
and Medical Center
5/F Medical Office
Building (MOB) Suite 509
2205 Civic Drive,
Filinvest, Alabang
Muntinlupa City,
1781 Philippines
Direct:
(632) 771-9253
Direct and Fax:
(632) 771-9254
Mobile: (63917) 795-2020
Map and Directions
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EYE REPUBLIC Ophthalmology Clinic
Medical City
6/F Medical Arts Tower Inc (MATI) Suite 602
MERALCO Compound, Ortigas Avenue
Pasig City, 1604, Philippines
Direct and Fax: (632) 632-7846
Mobile: (63917) 537-2020
Map and directions
|
EYE REPUBLIC
Ophthalmology Clinic
St. Luke's
Medical Center
6/F
Cathedral Heights Building Complex (CHBC)
North Tower Suite 614
279 E. Rodriguez
Sr. Boulevard
Quezon City, 1102
Philippines
Direct and Fax:
(632) 407-3883
Mobile: (63917) 855-2020
Map and directions
|
CLINIC HOURS
First-Come, First-Served
Monday to Saturday 9:00 AM to 6:00 PM
All clinics are closed on Sundays and Holidays
|
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CONGENITAL CATARACT information compiled by
Dr. Barbara L. Roque
and initially uploaded on May 1, 2005.
Last updated on
September 19, 2007. |
