Pediatric Cataracts - Overview - American Academy of Ophthalmology PDF

11/1/2017 Pediatric Cataracts: Overview - American Academy of Ophthalmology
Clinical Education / Pediatric Ophthalmology Education Center / Browse Topics
NOV 11, 2015
Pediatric Cataracts: Overview

Written By:
M. Edward Wilson, MD
,M. Edward Wilson, MD

Cataract
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Introduction and epidemiology

In children, cataract causes more visual disability than any other form of treatable blindness. Children with
untreated, visually significant cataracts face a lifetime of blindness at tremendous quality of life and
socioeconomic costs to the child, the family, and the society. More than 200,000 children are blind from
unoperated cataract, from complications of cataract surgery, or from ocular anomalies associated with
cataracts.1 Many more children suffer from partial cataracts that may slowly progress over time, increasing
the visual difficulties as the child grows. The cumulative risk of cataract during the growing years is as high
as 1 per 1000.2
The management of cataracts in childhood is tedious and often difficult, requiring many visits over many
years. Success requires a dedicated team effort that often involves parents, primary care pediatricians,
surgeons, anesthesiologists, technicians, orthoptists, low vision rehabilitation specialists, and community
health workers.
Classification (Categorization)
Cataracts in children can be classified using a number of methods including age of onset, etiology, and
morphology.
Age of onset
Congenital/Infantile
While the presence of lens opacities at birth indicates a congenital onset, the diagnosis and recognition of
a lens opacity at a later age does not exclude a congenital onset. It is critical to provide a detailed
description of the type of lens opacities before the cataract is extracted and in the operative note so the
type can be determined and any later study correlating genetic etiology or associated systemic disease
can be done more accurately. Some morphological categories of cataracts such as anterior polar, central
fetal nuclear, and posterior polar clearly indicate a congenital onset, while others such as cortical or
lamellar may be associated either with a later onset or be congenital in nature.
Acquired/Juvenile
https://www.aao.org/pediatric-center-detail/pediatric-cataracts-overview 1/25
This category can be confusing. Strictly speaking, an acquired cataract is one from an external cause, as
opposed to one in which the cause is genetically determined, such as a mutation in one of the crystalline
genes. However, some would use acquired to indicate an onset after infancy, which does not necessarily
indicate a non-genetic cause. Juvenile cataracts are by definition those with an onset in childhood, after
infancy, irrespective of underlying etiology.
Etiology
Genetic
Approximately 50% of childhood cataracts are caused by mutations in genes that code for proteins
involved in lens structure or clarity. Table 1 lists genes in which mutations can cause cataracts.3 While
many of these genes are dominantly inherited, others are autosomal recessive or X-linked. Drawing a
pedigree and recognizing some cataract and ocular phenotypes that are associated with specific mutations
will help determine the probable mode of inheritance and the possible underlying syndrome. Recent
advances in genetic testing, including next generation sequencing, allow the determination of the precise
genetic cause of isolated congenital cataracts in 75% of individual families and 63% of those with
syndromic congenital cataracts.4 Mutations in crystallins account for 50% of isolated (no associated
systemic abnormalities) cataracts, while mutations in the gap junction protein connexins account for 25%
of cases and mutations in genes for heat shock transcription factor-4, aquaporin-0, and beaded filament
structural protein-2 account for the remaining 25%.
Metabolic disorders can cause cataracts, which may have particular morphologies that point to the
underlying cause. Next generation sequencing of genes associated with syndromic or metabolic cataracts
can provide a precise diagnosis if the systemic findings do not allow recognition of the metabolic or
systemic illness. Table 1 summarizes findings in some of the main diseases associated with acquired
syndromic cataracts.
Trauma remains a major cause of acquired cataracts in children. Traumatic cataracts are more common in
boys and can be the result of penetrating or blunt injuries to the eye. One has to be careful in ruling in or
out the presence of an intraocular or intraorbital foreign body, hence the importance of a detailed physical
examination and of imaging studies such as ultrasonography and computed tomography. Magnetic
resonance imaging (MRI) studies are contraindicated if the foreign body is suspected to be metallic.
Table 1. Common causes of congenital or early acquired cataracts3
Disease Location Gene Phenotype OMIM number
AUTOSOMAL DOMINANT
Congenital nuclear cataract and 600886

Hyperferritinemia-cataract syndrome 19q13.33 FTL
elevated serum ferritin
2q33.3 CRYGC Dusty opacity of the fetal nucleus

Coppock-like cataracts with frequent involvement of the 604307
22q11.23 CRYBB2 zonular lens
Volkmann type congenital cataract 1p36 Unknown Central and zonular cataract 115665
Zonular cataracts with sutural

Zonular with sutural opacities 17q11.2 CRYBA1 600881
opacities
Posterior polar 1 (CTPP1) 1p36.13 EPHA2 Opacity located at back of lens 116600
Single well-defined plaque in

Posterior polar 2 (CTPP2) 11q23.1 CRYAB 613763
posterior pole of lens; bilateral
Progressive, disc-shaped, posterior

Posterior polar 3 (CTPP3) 20q11.22 CHMP4B 605387
subcapsular opacity
Single well-defined plaque in

Posterior pole 4 (CTPP4) 10q24.32 PITX3 610623
posterior pole of lens
Mat reflex of posterior capsule that

progresses into well-demarcated
Posterior pole 5 (CTPP5) 14q22-q23 Unknown 610634
disc in posterior pole, forming
opaque plaque
Lenticular opacities located in the

fetal nucleus with scattered, fine,
Zonular pulverulent 1 (CZP1) 1q21.2 GJA8 116200
diffuse cortical opacities and
incomplete cortical 'riders'
Central pulverulent opacity

surrounded by snowflake-like
Zonular pulverulent 3 (CZP3) 13q12.11 GJA3 601885
opacities in anterior and posterior
cortical regions of the lens
Small opacities on anterior surface

Anterior polar cataract 1 14q24-qter Unknown 115650
of lens
Small opacities on anterior surface

Anterior polar cataract 2 17q13 Unknown 601202
of lens
Peripheral blue and white opacities

Cerulean type 1 (CCA1) 17q24 Unknown 115660
in concentric circles
Numerous peripheral blue flakes

Cerulean type 2 (CCA2) 22q11.23 CRYBB2 and occasional spoke-like central 601547
opacities
Cerulean type 3 (CCA3) 2q33.3 CRYGD Progressive blue dot opacities 608983
Needle-like crystals projecting in

Crystalline aculeiform cataract 2q33.3 CRYGD different directions, through or close 115700
to the axial region of the lens
Nonnuclear polymorphic congenital Opacities between the fetal nucleus

2q33.3 CRYGD 601286
cataract and the cortex of the lens
Dense, white opacification around

the anterior and posterior Y sutures,
Sutural cataract with punctate and
22q11.23 CRYBB2 oval punctate and cerulean 607133
cerulean opacities
opacities of various sizes arranged
in lamellar form
Myotonia, muscular dystrophy,

Myotonic dystrophy 1 (DM1) 19q13.32 DMPK cataracts, hypogonadism, frontal 160900
balding, and ECG changes
Lamellar, sutural, polar and cortical 604219

Polymorphic and lamellar cataracts 12q13.3 MIP
opacities
Cataract, autosomal dominant, multiple

3q22.1 BFSP2 Nuclear and sutural opacities. 611597
types 1
AUTOSOMAL RECESSIVE
604168
Congenital cataracts, facial

Congenital cataracts, facial dysmorphism, neuropathy, delayed
dysmorphism, and neuropathy 18q23 CTDP1 psychomotor development, skeletal
(CCFDN) anomalies, microcornea and
hypogonadism
Congenital cataracts, cerebellar

ataxia, muscle weakness, delayed
psychomotor development, short
Marinesco-Sjgren syndrome 5q31.2 SIL1 248800
stature, hypergonadotrophic
hypogonadism, and skeletal
deformities
2q21.3 RAB3GAP1 600118

Warburg micro syndrome 1 Microcephaly, microphthalmia,
microcornea, optic atrophy, cortical
dysplasia, in particular corpus
1q41 RAB3GAP2
Warburg micro syndrome 2 callosum hypoplasia, severe mental 614225
retardation, spastic diplegia, and
hypogonadism
Warburg micro syndrome 3 10p12.1 RAB18 614222
Mental retardation, hypogonadism,

Martsolf syndrome 1q41 RAB3GAP2 212720
microcephaly
Brachycephaly, hypotrichosis,
Hallermann-Streiff syndrome (Francois microphthalmia, beaked nose, skin
6q22.31 GJA1 234100
dyscephalic syndrome) atrophy, dental anomalies, short
stature
Skin atrophy, telangiectasia, hyper-

and hypopigmentation, congenital
Rothmund-Thomson syndrome 8q24.3 RECQL4 skeletal abnormalities, premature 268400
aging, increased risk of malignant
disease
Microcephaly, mental retardation,

Smith-Lemli-Opitz syndrome 11q13.4 DHCR7 270400
hypotonia, , polydactyly, cleft palate
Congenital nuclear cataracts 2 22q11.23 CRYBB3 Nuclear cataract with cortical riders 609741
X-LINKED
Early childhood blindness, mental

Norrie disease Xp11.3 NDP 310600
disorder, sensorineural deafness
Males have dense nuclear

cataracts, microcornea, dental
abnormalities, and developmental
Nance Horan syndrome Xp22.13 NHS 302350
delay. Carrier females have
posterior Y-sutural cataracts with
small corneas
Xu LT, Traboulsi EI. Genetics of congenital cataracts. In: Wilson ME, Trivedi RH, editors. Pediatric Cataract
Surgery: Lippincott, Walters Kluwer 2014. p. 1-8.
Secondary
Uveitis Cataracts develop in patients with uveitis as a result of the chronic ocular inflammation or
secondary to the chronic use of steroids. Surgery for such cataracts can be complicated by severe
postoperative inflammation, hence the need for absence of preoperative inflammation in the anterior
segment of the eye and the pre-, intra-, and post-operative use of various combinations of topical,
subconjunctival, intracameral, and sometimes systemic steroids. Many patients will have a pupillary
membrane that covers the lens and attaches to the iris, making surgery more difficult. Such membranes
can be peeled off of the anterior lens capsule at the time of surgery to facilitate lens removal. The use of an
intraocular lens (IOL) is left to the discretion of the individual surgeon.
Juvenile idiopathic arthritis: One of the more common causes of anterior uveitis in children. The
use of systemic antimetabolites in recent years has led to better control of uveitis in such patients
and to a reduction in the incidence of cataracts.
Other types of uveitis can also cause cataracts either because of the inflammation or as a
complication of steroid use.
Intraocular tumors It is very uncommon for cataracts to develop as a consequence of intraocular

tumors. The lens is characteristically clear in patients with untreated retinoblastoma. Treatments of the
tumor such as radiotherapy may lead to the development of cataracts, in which case timing of cataract
removal has to be very carefully considered and surgery only performed when all tumor in the eye has
been eradicated. Patients with radiation cataracts can have significant ocular surface dryness and will not
tolerate contact lenses, hence the need for intraocular lens (IOL) implantation.
Chronic retinal detachment These cataracts are seen in the setting of injuries or in association with
Stickler syndrome. If the lens is totally opaque, preoperative ultrasonography should be performed to rule
out a chronic retinal detachment. The presence of an afferent pupillary defect is a poor prognostic sign.
Maternal infection (rubella) This type of cataract has not been seen in countries where rubella has
been eradicated, but continues to occur in some parts of the world.
Iatrogenic
Radiation External beam radiation is avoided in patients with retinoblastoma. The eye is typically
shielded if radiation is given to the brain or other parts of the head and neck.
Systemic steroids are very rare causes of cataracts in children. Inhaled steroids for asthma do not
cause cataracts. The typical steroid-induced cataract is posterior subcapsular.
Vitrectomy A large percentage of children who undergo vitrectomy develop cataracts. These are
mostly posterior subcapsular.
Laser for retinopathy of prematurity Cataracts can develop from thermal injury to the lens when
a prominent tunica vasculosa lentis is present.
Morphology
As mentioned above, it is important to utilize the appropriate terminology to describe pediatric cataracts.
The morphology can give a clue to the underlying etiology (isolated or associated with systemic disease),
and possibly to the visual prognosis following surgery.
Diffuse/Total
This is not an uncommon type of congenital cataract. There are no specific causes of diffuse or total
cataracts.
Anterior
Anterior polar The opacity is in the capsule itself and can protrude into the anterior chamber as a small
mammillation. There may be an underlying circular layer of cortical opacity slightly larger than the white
polar opacity. While the majority are stable and do not interfere with vision, some can progress and require
surgical removal. They can be dominantly inherited, especially in bilateral cases. Unilateral cases can be
associated with anisometropia (astigmatism or hyperopia), which if left untreated can cause amblyopia,
even if the cataract itself is not visually significant.
Figure 1. Anterior polar cataract.
Pyramidal These are usually larger than polar cataracts and more likely to progress to visual
significance. They are difficult to remove with a vitrectomy instrument and may require excision and
removal with forceps before the rest of the lens is aspirated.
Figure 2. Pyramidal cataract.
Anterior lenticonus This refers to a thinned-out central anterior capsule with or without anterior cortical
opacities. Anterior lenticonus is said to be characteristic of Alport syndrome. Spontaneous rupture of the
lens can occur, resulting in a hydrated total cataract.
Figure 3. Anterior lenticonus (Courtesy of K. David Epley).
Cortical lamellar
In this type of cataract, the opacification is of a lamella (an ovoid layer of cortex) that can be visualized
between adjacent clear lamellae. These are frequently associated with radial rider opacities. Familial
lamellar cataracts are mostly autosomal dominant and are generally associated with a good visual
prognosis after their removal. They can be stable or may be associated with progressive opacification of
intervening cortex, necessitating removal.
Figure 4. Lamellar cataracts (Top: Courtesy of K. David Epley, MD. Bottom: Courtesy of Faruk H. rge,
MD).
Fetal nuclear
These opacities occupy the central-most part of the lens. They can be dot-like or can be quite dense. They
generally measure 2-3.5 mm and can be associated with microphthalmia. They are said to be associated
with a higher incidence of postoperative glaucoma because of associated microphthalmia and the need for
surgery early in infancy.
Figure 5. Congenital nuclear cataract.
Posterior polar
In this type of cataract, the opacity is in the capsule itself. It is necessary to differentiate posterior polar
from posterior subcapsular cataracts. Posterior polar cataracts are genetically determined and some have
been associated with mutations in PITX3.
Figure 6. Posterior polar cataract.
Posterior lentiglobus (lenticonus)

In this group of conditions, the central and sometimes paracentral posterior capsule is thin and bulges
posteriorly. This usually occurs at the location where the hyaloid system attaches to the eye. The distortion
can cause a localized area of extreme myopic refraction. There may or may not be subcapsular cortical
opacification. Interference with vision can be the result of optical distortion or of capsular opacification.
Most cases are unilateral, although bilateral and familial cases have been reported. Surgery is associated
with good visual outcomes in most cases. Spontaneous rupture of the lens can rarely occur, leading to
abrupt progression to total cataract.
Figure 7. Posterior lentiglobus (lenticonus) cataract. (A) Early clear defect in central posterior capsule
and (B) early opacification of central defect. (C) Ultrasound biomicroscopy of advanced posterior
lenticonus.
Posterior subcapsular
These can be congenital but are more commonly acquired as a result of injury or steroid use. The opacities
are cortical and do not involve the capsule proper.
Figure 8. Posterior subcapsular cataract.
Persistent fetal vasculature (PFV) (severe varieties are still referred to as

persistent hyperplastic primary vitreous)
The lens opacities in patients with PFV are generally capsular and can be associated with shrinkage,
thickening, and vascularization of the capsule. There may be a posterior plaque outside or involving the
lens capsule with a clear lens that nonetheless must be treated as a cataract.
Figure 9. Persistent fetal vasculature.
Traumatic disruption of lens

In children, traumatic anterior lens capsule rupture quickly results in a hydrated white cataract. However, in
children, lens cortex in the anterior chamber may be well tolerated without an intraocular pressure (IOP)
rise. Cataract surgery can often be delayed for a few days or up to 3 or 4 weeks to allow the traumatic iritis
to subside before the cataract and IOL surgery.
Figure 10. Traumatic disruption of lens (Courtesy of K. David Epley).
Evaluation and work-up

Role of vision screening
Vision screening is mandatory to detect cataracts as soon as possible. Late detection may result in poor
visual outcomes. All newborns must have red reflex screening, ideally followed by another red reflex
examination at the 6-8 week neonatal checkup. Red reflex testing is done by using direct ophthalmoscope
from a distance of 1-2 feet in a darkened room. Preschool vision screening (at 3 and 5 years) is often done
in the community. Photo screeners are used in preverbal and verbal children. These may help the
pediatrician save time in screening. They work by a computer analyzing the red reflex for inequality in
color, intensity, or clarity. New screeners utilizing polarized laser light are more accurate at detecting
decreased vision. The presence of any opacities, an absent red reflex, or leukocoria should prompt an
urgent referral to a pediatric ophthalmologist.
Evaluation by the ophthalmologist

A detailed history is taken that includes asking about the childs developmental milestones, and about
health problems in the siblings and parents. Visual assessment is conducted by using age-appropriate
testing. When the child is two months old, vision assessment can be done with forced preferential looking
techniques (eg, Teller acuity cards, Cardiff cards), fixation and following evaluation, and assessing
objection to occlusion of each eye. The presence or absence of nystagmus is noted. Subjective visual
testing (HOTV matching, LEA symbols, or tumbling Es) is done as soon as the child is able to play a
matching game or identify the symbols and letters. These tests can usually be done at age 3 years and
above.
Biomicroscopy (standard or portable slit lamp examination) is completed. Severity and morphology of the
cataract and any associated abnormalities of cornea or anterior segment are documented. Examination of
siblings and parents might indicate inherited cataracts. Intraocular pressure is checked if possible.
If there is a view of the retina, full retinal examination documenting optic nerves, retina, and fovea is
performed. If there is no view, ultrasonography (B-scan) is carried out. If there is trauma, then child abuse
must be ruled out. In unilateral cataracts, laboratory tests are not needed.
For bilateral cataracts, if there is family history of childhood cataracts, the child has no other medical
problems, and the parents have lens opacities, then systemic and laboratory evaluations are not needed. If
there is no family history of cataracts, a pediatric systemic evaluation is required because these cataracts
may be associated with systemic or metabolic disease. Laboratory tests may also be needed. The
ophthalmologist often works in conjunction with a pediatrician and/or a clinical geneticist when directing the
laboratory work-up. A urine test for reducing sugars, TORCH (toxoplasmosis, rubella, cytomegalovirus,
varicella) screening, a Venereal Disease Research Laboratory (VDRL) test for syphilis, and a blood test for
calcium, phosphorus, glucose, and galactokinase levels can be checked.
Most inherited cataracts are autosomal dominant. Recessive and X-linked cataracts are less common.
Genetic testing is a rapidly evolving field. Mutations that cause congenital cataracts have been discovered
in over 100 genes. Using the latest sequencing tests, it will be possible to check all genes involved in
congenital cataracts from one blood sample. This might lead to quicker and cheaper personalized
treatment and counseling by the geneticist.
If cataracts are less than 3 mm in diameter or are of partial density, they may be observed or treated with
dilating drops. Any dense central opacity in the lens of three or more mm in a young child is significant and
requires surgery. In addition to the size of cataract, blackening of the retinoscopic reflex is the most
important factor determining need for a surgery.5 In an older child, any opacity causing a decrease in
quality of life should be considered for surgery. At the same time, the loss of accommodation that occurs
when a childs lens is removed should be taken into account when making a surgical decision. With
increasing age, visual demands of the child increase and the assessment of whether a partial cataract is
visually significant has to be constantly revisited.
Biometry is done to get keratometry measurements, preferably without a speculum. Axial length is often
measured in children by A-scan ultrasound, with the immersion method being more accurate than the
contact method.6,7 Often, these measurements are not possible in clinic and examination under anesthesia
is required. If the child is older and cooperative, and the cataract is not very dense, then optical biometry is
done.
For calculation of the IOL, third-generation theoretical formulae (eg, SRK/T, Holladay I & II, Hoffer Q I & II,
and Haigis) can be used. Target refraction may be aimed for initial hypermetropia (high or low) or
emmetropia. Suggested target refractions for age are given in Table 2.5 Other factors such as amblyopia,
fellow eye condition or refraction, assumed compliance, and parental refractive error should also be taken
into consideration when interpreting the table: one IOL power choice for every age does not work for every
situation.
Table 2. Age at cataract surgery and residual refraction
recommendations for target refraction
Age at cataract surgery Residual refraction (Diopters)
<6 months +6 to +10
6-12 months +4 to +6
1 -3 years +4
3-4 +3
4-6 +2 to +3
6-8 +1 to +2
>8 +1 to 0
Surgery
Who should perform the surgery
Adult cataract surgery is a major emphasis of residency training programs in ophthalmology. The skills
needed to perform adult cataract surgery are also important for performing pediatric cataract surgery, but
additional skills are needed for the pediatric surgery. Pediatric cataract surgery should only be performed
by ophthalmic surgeons who perform them on a weekly or biweekly basis so that they can perform them
with a high level of competency.8 For this reason, most large group practices assign only one surgeon in
their practice to perform these surgeries. When possible, children should be referred to regional centers
where large numbers of pediatric cataract surgeries are performed. After the postoperative period, in most
cases these children can then be followed on a long-term basis by a local doctor and only referred back to
the regional center if problems arise. Pediatric ophthalmologists interested in performing pediatric cataract
surgery should pursue fellowship training at an institution where they will be trained how to perform
pediatric cataract surgery. After completing their fellowship, they should take instructional courses as
needed to incorporate new techniques as they arise. While adult cataract surgeons are usually skillful at
performing intraocular surgery, they often have not been taught the special techniques required to
successfully perform pediatric cataract surgery. If they are interested in performing pediatric cataract
surgery, they should seek out opportunities to learn its best practices either by observation or by taking
instructional courses.
Timing and critical period
In the 1960s, Hubel and Wiesel9 introduced the concept of a latent period and a critical period for visual
development. During the latent period, visual deprivation has no lasting effect on vision in the deprived
eye. After the latent period, there is a critical period during which visual deprivation results in irreversible
vision loss in the deprived eye. The critical period for a child with a cataract extends to age 9-10 years.
Unilateral
The optimal age for performing cataract surgery in a child with a unilateral congenital cataract is generally
agreed to be 6 weeks of age. Birch and Stager10 evaluated the relationship between the age at cataract
surgery and visual outcomes in newborns with a dense unilateral congenital cataract. The model that best
fit their data was bilinear, with no differences in the visual outcomes if the surgery was performed between
birth and age 6 weeks. However, after age 6 weeks, there was a linear decline in visual outcomes related
to the age at cataract surgery. Their model would suggest that there is a 6-week latent period for dense
unilateral cataracts in humans. More recently, Hartmann et al11 found that the age at cataract surgery was
only weakly associated with visual acuity. While the median visual acuity was better among patients who
had cataract surgery between ages 4 and 6 weeks, the association between age at cataract surgery and
the visual outcome was less robust than the data reported by Birch and Stager.
Bilateral
It is generally agreed that bilateral congenital cataracts should be removed by 8 weeks of age to achieve
the best visual outcomes. Lambert and coworkers12 noted that delaying cataract surgery to 10 weeks of
age or later increased the likelihood of a 20/100 or worse visual outcome. Birch and coworkers13 reported
a bilinear relationship between the age of surgery and the visual outcome in infants with dense bilateral
congenital cataracts. Between birth and 14 weeks of age they noted progressively worse visual outcomes
the older a child was at the time of cataract surgery. However, after age 14 weeks until 31 weeks, the
visual outcome was independent of the childs age at the time of cataract surgery. Since it is unclear if
there is a latent period in children with dense bilateral congenital cataracts, the timing of cataract surgery in
these children is often determined by other comorbidities and the increased risk of glaucoma associated
with very early cataract surgery.
Threshold/indication for surgery

Determining the need for surgery in preverbal children
Dense cataracts that block the red reflex before the pupils are dilated and are associated with abnormal
visual behavior should be removed during infancy. Other signs suggestive of visually significant cataracts
are strabismus in a child with a unilateral cataract or nystagmus in a child with bilateral cataracts.
Incomplete cataracts do not always require cataract surgery. If the child has incomplete cataracts and
normal visual behavior and the fundi can be clearly viewed with an ophthalmoscope, cataract surgery
should be deferred. Generally, posterior lenticular opacities are more visually significant than anterior lens
opacities. If the incomplete cataract(s) is unilateral or asymmetrical, part-time patching therapy of the
normal/better eye may be beneficial to improve or maintain vision in the most affected eye.
Visual acuity chart threshold for surgery

Generally, cataract surgery should not be performed on children with bilateral cataracts who have best
corrected visual acuity of 20/40 or better. However, the visual threshold for performing cataract surgery
should be tailored to the needs of the child. For instance, if a child has visual acuity worse than 20/40, but
is doing well in school and does not have any visual behavioral problems, cataract surgery can be deferred
until later. Visual behavior is less helpful in assessing the need for cataract surgery in children with a
unilateral cataract. Generally, if best corrected visual acuity cannot be improved to 20/50 or better with
amblyopia therapy, cataract surgery should be considered.
Visual dysfunction weighed against post-op loss of accommodation

The improvement in visual acuity associated with cataract surgery must be weighed against the loss of
accommodation associated with removing the crystalline lens. While multifocal or accommodative IOLs are
available for adults and may mitigate, somewhat, the loss of accommodation associated with cataract
surgery, they are infrequently implanted in growing children because of the refractive changes that occur
as an immature eye grows. Parents should be told that while their child may see more clearly after
undergoing cataract surgery, the child will have to wear bifocals in order to optimize distance and near
vision.
Informed consent/parenting counseling

The risks and benefits of cataract surgery should be clearly outlined to parents. It is often helpful to show
them models of the eye or illustrations to help them understand what a cataract is and how cataract
surgery will be performed. The importance of amblyopia therapy and optical correction following cataract
surgery should be discussed in detail. The pros and cons of implanting an IOL or creating a posterior
capsulotomy should be discussed with parents. It should also be explained that the US Food and Drug
Administration (FDA) has not approved the implantation of IOLs in children, and their use in children is off-
label.
Immediate sequential bilateral cataract surgery for children

The option of performing immediate sequential bilateral cataract surgery should be discussed with the
parents of infants, particularly if there are comorbidities that increase the risk of general anesthesia. They
should be informed of the risks and benefits associated with immediate sequential bilateral cataract
surgery, including the benefit of administering only one general anesthetic, but the increased risk of
bilateral endophthalmitis.14 It should also be explained that precautions will be taken to reduce the risk of
endophthalmitis, including using different trays of instruments for each eye, disposable cannulas, re-
draping between eyes, and using different lots of irrigating solution and medications for each eye.
Anesthesia management considerations

General anesthesia is required to perform pediatric cataract surgery. The anesthetic agents should be
administered only under the direct supervision of an anesthesiologist with special experience or special
training in pediatric anesthesia. Very young children, especially when born prematurely, will often need to
be hospitalized overnight after cataract surgery because of their increased risk of experiencing apnea after
undergoing general anesthesia. Cataract surgery can be performed as an outpatient procedure in older
children.
Operative techniques
Preoperative preparation is typically done using povidone-iodine. The use of intracameral antibiotics in
either the irrigating solution or injected postoperatively has been extensively tested in adults, and while not
widely practiced among pediatric cataract surgeons, trends forecast more acceptance in the coming years.
Surgical incisions are usually done anteriorly through clear cornea or using a scleral tunnel. If no IOL is to
be placed, a minority of surgeons will opt for a posterior pars plana/plicata approach. Continuous
curvilinear capsulorhexis with or without capsular staining is the gold standard capsulotomy, but
vitrectorhexis also works well and is commonly used in the first few years of age when the capsule is very
elastic. The anterior chamber is maintained with either a separate non-held infusion cannula (an anterior
chamber maintainer) or with matched hand-held bimanual irrigation and aspiration handpieces. Pupil
dilation is enhanced with non-preserved epinephrine or phenylephrine/ketorolac (recently FDA approved
for adults) added to the infusion bottle.
The lens contents are aspirated completely (Figure 11). Phacoemulsification ultrasound energy is never
needed with pediatric cataracts. Hydrodissection is not necessary, but can be used at the surgeons
discretion. However, the large number of pediatric lens opacities associated with posterior capsule
pathology must be noted. Hydrodissection is contraindicated in posterior polar cataracts.
Figure 11. An irrigation/aspiration handpiece removing a lamellar cataract (Courtesy of Faruk H Orge).
A posterior chamber IOL inserted into the capsular bag is always preferred, but ciliary sulcus placement of
a foldable acrylic or single-piece rigid IOL can be done. In cases of no capsular support, posterior chamber
IOLs can be sewn in place; however, placement of iris (claw) fixated lenses is becoming more popular.
In children too young to tolerate a YAG laser posterior capsulotomy in the office, a primary posterior
capsulotomy at the time of initial cataract surgery is recommended. This can be done either before or after
an IOL is placed and can be done anteriorly through the corneal tunnel or posteriorly through the pars
plana. All but the smallest watertight incisions should be closed in children, usually with a synthetic
absorbable 10-0 suture.
Postoperative medications
Antibiotics
After pediatric cataract surgery, either moxifloxacin or tobramycin, the two most widely used antibiotic eye
drops, can be used. The eye drops are instilled four times per day for a week. There is no need to
prescribe systemic antibiotics.
Steroids
Prednisolone eye drops are the mainstay of treatment to control severe inflammation, which is generally
inevitable. In some cases of very severe postoperative inflammation, steroid eye drops must be instilled as
frequently as on an hourly basis. Otherwise, the routine dosage range is 4-8 times per day. Some
surgeons advocate supplementing the topical steroid with oral prednisolone dosed at 1 mg/kg/day for the
first week to help reduce inflammation.
Cycloplegics and mydriatics

Homatropine or atropine eye drops are sometimes used postoperatively as cycloplegics. The possible side
effects of atropine must be discussed with the patients parents.
Follow-up
Pediatric cataract cases are normally examined on the first postoperative day. The next follow-up depends
on the amount of inflammation but is most often at 1 week after surgery. Once both eyes are operated on,
periodic examinations are required to determine refraction, IOP, and retinal evaluation. Glasses or contact
lenses are prescribed as early as possible, preferably within the first week for aphakic correction and within
4 weeks for residual refractive error in pseudophakic children.
Frequency
Typical follow-up frequency is as follows: postoperative day 1, week 1, month 1, month 3, every 3 months
for 2 years, and thereafter every 6 months for 3 years.
Evaluation
It is crucial to check visual acuity, ocular alignment, IOP, refraction, and clarity of the visual axis at every
visit. Should there be any complication detected in any of the follow-up visits, it should be tackled
promptly.
Optical rehabilitation after cataract surgery

Since uncorrected refractive error in the early years can lead to amblyopia, attention to appropriate
refractive correction after cataract surgery is crucial in order to obtain good final visual acuity. For infants
and toddlers, refractive correction should result in good near vision (myopic refraction of approximately -2
diopters). However, correction for distance vision and a bifocal correction for near viewing should be
offered after the age of 2 or 3 years, or by pre-kindergarten. Children who use a contact lens may also
benefit from a spectacle overcorrection after age 2 or 3 years.
Spectacles
For children who have IOL implantation, some residual refractive error is typical and spectacle correction
may be needed for distance and/or near viewing. Additionally, when IOL implantation occurs at an early
age, the growing eye will experience a myopic shift, so that changing refraction is expected with residual
hyperopia in the early years but some degree of myopia expected later. Correction of aphakia with
spectacles may be preferred for infants and young children in whom IOL implantation is not possible or is
purposely delayed. Aphakic spectacles are generally well tolerated, particularly by children who are
bilaterally aphakic. Unilateral aphakia can also be corrected with spectacles, though this is less desirable
because of marked image-size disparity (aniseikonia) and potential disruption of binocular vision, if
present.
Contact lenses
Contact lens correction of aphakia is often planned for very young infants after lensectomy, typically with
either a silicone elastomer lens (extended wear) or rigid gas permeable lens (daily wear). One advantage
of contact lens wear is easy adjustment in power for the rapidly changing refractions encountered in young
children. Contact lens correction of residual refractive error is also possible after IOL implantation, and is
sometimes requested by adolescent patients.
Postoperative Complications and Sequelae

Postoperative complications after pediatric cataract surgery are inversely proportional to the age at the
time of surgery. Associated ocular anomalies, surgical technique, and follow-up duration are some of the
other important variables influencing the prevalence and severity of the postoperative complications after
cataract surgery in children.
Visual axis opacification

If the posterior capsule is left intact at the time of cataract surgery in children, posterior capsule
opacification (PCO) is inevitable. The younger the child, the more acute will be the opacity. After primary
posterior capsulectomy and vitrectomy, visual axis opacification (VAO) is rare in older children; however,
despite posterior capsulectomy and vitrectomy, VAO is commonly observed in infants. VAO in infants
receiving posterior capsulectomy and vitrectomy typically requires surgical removal from 3 months to 1
year after the original surgery, while PCO in older children who had an intact posterior capsule typically
requires Nd:YAG laser or surgical removal of the PCO 2 years or more after cataract surgery.15
Glaucoma
Secondary glaucoma is the most sight-threatening complication of pediatric cataract surgery. Younger age
at the time of surgery is the most commonly reported risk factor. Open-angle glaucoma can develop
months to many years after the surgery, and children must be followed for this regularly for their entire life.
Inflammatory complications
Due to increased tissue reactivity, inflammatory complications (eg, anterior chamber cell and flare, cell
deposits on the IOL optic, posterior synechiae, etc.) are more frequently observed in children. Toxic
anterior segment syndrome (TASS) is a rare inflammatory condition usually observed during the early
postoperative period.
Contact lens related

Bacterial keratitis, corneal opacity due to tight contact lenses, and corneal vascularization are the most
common contact lens-related complications.
IOL malposition
Excessive capsular fibrosis and asymmetric IOL fixation are the most common causes leading to
malposition of an IOL. It can also occur because of traumatic zonular loss and/or inadequate capsular
support. The IOL may have to be repositioned or explanted in some cases when there is significant
decentration/dislocation.
Endophthalmitis
The incidence of postoperative endophthalmitis in children is similar to that reported in adult surgery.
Common organisms are Staphylococcus aureus, Staphylococcus epidermidis, and Streptococcus viridans.
Recent studies in adults have reported a marked decrease in endophthalmitis when intracameral
antibiotics are used. In the US, the absence of an ophthalmic preparation specific for use as an
intracameral injection has slowed adoption of intracameral antibiotics for fear of toxicity from dilution errors
during medication preparation. Studies in adults have used cefuroxime, vancomycin, and undiluted
moxifloxacin.16, 17, 18, 19, 20
Retinal detachment
The incidence of retinal detachment (RD) following pediatric cataract surgery appears to have decreased
markedly as surgical techniques have advanced. However, because RD may develop many years after
surgery, a retinal examination is recommended after cataract surgery at least yearly. This is especially
important for those eyes at higher risk for RD by virtue of a long axial length for age, persistent fetal
vasculature, traumatic cataract, ectopia lentis, Stickler syndrome, repeated surgeries, etc.
Myopic shift
A tendency toward axial elongation and a myopic shift of refraction is well known. This is more concerning
if the child receives an IOL. The younger the child at the time of implantation, the higher the myopic shift.
High myopia in pseudophakic eyes can be treated using spectacles or contact lens. Alternatively, IOL
exchange, piggyback IOL implantation, or corneal refractive surgery may be required.
Other complications
Corneal edema, corneal decompensation, iris prolapse, heterochromia iridis, suture-related complications,
a postoperative IOP spike, astigmatism, ptosis, or phthisis bulbi are other complications reported after
pediatric cataract surgery.
Strabismus
Strabismus can coincide with congenital cataract and is more commonly seen in unilateral cases but not
rare in bilateral cataract cases, especially when nystagmus is present. Esotropia is the most common form
of strabismus in congenital cataract, although cyclovertical strabismus may also contribute to the clinical
picture. In a minority of patients, exotropia of the involved eye is the presenting sign of congenital cataract.
Management of co-existing amblyopia

Deprivation amblyopia is very common in children with unilateral cataract, especially when the opacity is
congenital or infantile. Also, children with bilateral cataracts can develop unilateral or bilateral deprivation
amblyopia when the cataracts are asymmetric, when they are removed too late, or when the aphakia is not
properly corrected. Sensory nystagmus will further limit visual outcome. The management of the amblyopia
should start as soon as possible, since compliance in small infants is better than in 2- to 3-year-old
children. Patching of the sound eye is the mainstay of treatment. However, atropine penalization can be an
alternative if the amblyopic eye can take over fixation. This is quite rare because the aphakic or
pseudophakic eye has lost accommodation and for that reason is always at a disadvantage to the sound
eye, which can accommodate up to 10 diopters depending on the childs age. In bilateral aphakic eyes with
contact lenses, the contact lens of the dominant eye can be removed a few hours or several days per week
as a penalization strategy. The younger the child, the better the effect of amblyopia treatment per hour of
occlusion.
Low vision rehabilitation and quality of life

measures
In cases when the treatment of the congenital cataract is less successful, low vision rehabilitation has an
important role in how the patient can cope with the limited visual capacities in education and daily life. In
most countries, visual rehabilitation and education for visually impaired and blind patients are organized
either by the government, various nongovernmental organizations, or private foundations. The motto
should be: Use the remaining visual function with all other senses to achieve the optimum quality of life.
Future directions
Early detection will allow more timely treatment of pediatric cataract in the future. Vision screening
programs and improved education of primary health care workers and the public will help with this
evolution. Surgical techniques continue to improve and will allow childhood cataract removal with less and
less surgical trauma. Planning for IOL implantation will become easier as our knowledge of myopic shift
and axial globe growth evolve. Ultimately, future IOL technological advances will be aimed at restoration or
preservation of youthful accommodation and the ability to easily compensate for the inevitable myopic shift.
Intracameral medications specifically for ophthalmic use are being developed and these will improve
outcomes for children as they decrease the reliance we now have on the ability of parents to administer
topical medications after surgery.
References
1. Gilbert C. Worldwide causes of blindness in children. In: Wilson ME, Saunders RA, Trivedi RH, eds.
Pediatric Ophthalmology: Current Thought and a Practical Guide. Heidelberg, Germany: Springer;
2009: 47-60.
2. Haargaard B, Wohlfahrt J, Fledelius HC, Rosenberg T, Melbye M. Incidence and cumulative risk of
childhood cataract in a cohort of 2.6 million Danish children. Invest Ophthalmol Vis Sci.
2004;45(5):1316-1320.
3. Xu LT, Traboulsi EI. Genetics of congenital cataracts. In: Wilson ME, Trivedi RH, editors. Pediatric
Cataract Surgery: Techniques, Complications and Management. Philadelphia: Lippincott Williams &
Wilkins; 2014: 1-8.
4. Gillespie RL, O'Sullivan J, Ashworth J, Bhaskar S, Williams S, Biswas S, et al. Personalized
diagnosis and management of congenital cataract by next-generation sequencing. Ophthalmology.
2014;121(11):2124-2137 e1-2.
5. Serafino M, Trivedi RH, Levin AV, Wilson ME, Nucci P, Lambert SR, et al. Use of the Delphi process
in paediatric cataract management. Br J Ophthalmol. 2015. doi: 10.1136/bjophthalmol-2015-307287.
[Epub ahead of print].
6. Trivedi RH, Wilson ME. Prediction error after pediatric cataract surgery with intraocular lens
implantation: Contact versus immersion A-scan biometry. J Cataract Refract Surg. 2011;37(3):501-
505.
7. Trivedi RH, Wilson ME. Axial length measurements by contact and immersion techniques in pediatric
eyes with cataract. Ophthalmology. 2011; 118(3):498-502.
8. Bell CM, Hatch WV, Cernat G, Urbach DR. Surgeon volumes and selected patient outcomes in
cataract surgery: a population-based analysis. Ophthalmology. 2007; 114(3):405-410.
9. Hubel DH, Wiesel TN. The period of susceptibility to the physiological effects of unilateral eye
closure in kittens. J Physiol. 1970; 206(2):419-436.
10. Birch EE, Stager DR. The critical period for surgical treatment of dense congenital unilateral cataract.
Invest Ophthalmol Vis Sci. 1996; 37(8):1532-1538.
11. Hartmann EE, Lynn MJ, Lambert SR, Infant Aphakia Treatment Study Group. Baseline
characteristics of the infant aphakia treatment study population: predicting recognition acuity at 4.5
years of age. Invest Ophthalmol Vis Sci. 2014; 56(1):388-395.
12. Lambert SR, Lynn MJ, Reeves R, Plager DA, Buckley EG, Wilson ME. Is there a latent period for the
surgical treatment of children with dense bilateral congenital cataracts? J AAPOS. 2006;10(1):30-36.
13. Birch EE, Cheng C, Stager DR Jr, Weakley DR Jr, Stager DR Sr. The critical period for surgical
treatment of dense congenital bilateral cataracts. J AAPOS. 2008; 13:67-71.
14. Dave H, Phoenix V, Becker ER, Lambert SR. Simultaneous vs sequential bilateral cataract surgery
for infants with congenital cataracts: Visual outcomes, adverse events, and economic costs. Arch
Ophthalmol. 2010; 128(8):1050-1054.
15. Wilson ME, Jr., Trivedi RH, Buckley EG, Granet DB, Lambert SR, Plager DA, et al. ASCRS white
paper. Hydrophobic acrylic intraocular lenses in children. J Cataract Refract Surg. 2007;
33(11):1966-1973.
16. Braga-Mele R, Chang DF, Henderson BA, Mamalis N, Talley-Rostov A, Vasavada A. ASCRS Clinical
Cataract Committee. Intracameral antibiotics: Safety, efficacy, and preparation. J Cataract Refract
Surg. 2014; 40(12):2134-2142.
17. Tan CS, Goh AG, Ngo WK, Lim LW, Fam HB. Safety of intracameral antibiotic use after cataract
surgery. J Cataract Refract Surg. 2014; 40(11):1940-1941.
18. Shorstein NH, Winthrop KL, Herrinton LJ. Decreased postoperative endophthalmitis rate after
institution of intracameral antibiotics in a Northern California eye department. J Cataract Refract
Surg. 2013; 39(1):8-14.
19. Espiritu CR, Caparas VL, Bolinao JG. Safety of prophylactic intracameral moxifloxacin 0.5%
ophthalmic solution in cataract surgery patients. J Cataract Refract Surg. 2007; 33(1):63-68.
20. Beselga D, Campos A, Castro M, Fernandes C, Carvalheira F, Campos S, Mendes S, Neves A,
Campos J, Violante L, Sousa JC. Postcataract surgery endophthalmitis after introduction of the
ESCRS protocol: a 5-year study. Eur J Ophthalmol. 2014; 24(4):516-519.
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Clinical Education / Pediatric Ophthalmology Education Center / Browse Topics

NOV 11, 2015

Pediatric Cataracts: Overview

,M. Edward Wilson, MD

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Introduction and epidemiology

Disease Location Gene Phenotype OMIM number

Congenital nuclear cataract and 600886

2q33.3 CRYGC Dusty opacity of the fetal nucleus

Zonular cataracts with sutural

Single well-defined plaque in

Progressive, disc-shaped, posterior

Single well-defined plaque in

Mat reflex of posterior capsule that

Lenticular opacities located in the

Central pulverulent opacity

Small opacities on anterior surface

Small opacities on anterior surface

Peripheral blue and white opacities

Numerous peripheral blue flakes

Needle-like crystals projecting in

Nonnuclear polymorphic congenital Opacities between the fetal nucleus

Dense, white opacification around

Myotonia, muscular dystrophy,

Lamellar, sutural, polar and cortical 604219

Cataract, autosomal dominant, multiple

Congenital cataracts, facial

Congenital cataracts, cerebellar

2q21.3 RAB3GAP1 600118

Mental retardation, hypogonadism,

Skin atrophy, telangiectasia, hyper-

Microcephaly, mental retardation,

Early childhood blindness, mental

Males have dense nuclear

Intraocular tumors It is very uncommon for cataracts to develop as a consequence of intraocular

Figure 1. Anterior polar cataract.

Figure 2. Pyramidal cataract.

Figure 3. Anterior lenticonus (Courtesy of K. David Epley).

Figure 5. Congenital nuclear cataract.

Figure 6. Posterior polar cataract.

Posterior lentiglobus (lenticonus)

Figure 8. Posterior subcapsular cataract.

Persistent fetal vasculature (PFV) (severe varieties are still referred to as

Figure 9. Persistent fetal vasculature.

Traumatic disruption of lens

Figure 10. Traumatic disruption of lens (Courtesy of K. David Epley).

Evaluation and work-up

Evaluation by the ophthalmologist

Age at cataract surgery Residual refraction (Diopters)

<6 months +6 to +10

Timing and critical period

Threshold/indication for surgery

Visual acuity chart threshold for surgery

Visual dysfunction weighed against post-op loss of accommodation

Informed consent/parenting counseling

Immediate sequential bilateral cataract surgery for children

Anesthesia management considerations

Cycloplegics and mydriatics

Optical rehabilitation after cataract surgery

Postoperative Complications and Sequelae

Visual axis opacification

Contact lens related