Pediatric cataract: challenges and future directions
Pediatric cataract: challenges and future directions
Anagha Medsinge 0 1
Ken K Nischal 0 1
0 University of Pittsburgh School of Medicine , Pittsburgh, PA , USA
1 Pediatric Ophthalmology, Strabismus, and Adult Motility, Children's Hospital of Pittsburgh of University of Pittsburgh Medical Center , , UPMC
Cataract is a significant cause of visual disability in the pediatric population worldwide and can significantly impact the neurobiological development of a child. Early diagnosis and prompt surgical intervention is critical to prevent irreversible amblyopia. Thorough ocular evaluation, including the onset, duration, and morphology of a cataract, is essential to determine the timing for surgical intervention. Detailed assessment of the general health of the child, preferably in conjunction with a pediatrician, is helpful to rule out any associated systemic condition. Although pediatric cataracts have a diverse etiology, with the majority being idiopathic, genetic counseling and molecular testing should be undertaken with the help of a genetic counselor and/or geneticist in cases of hereditary cataracts. Advancement in surgical techniques and methods of optical rehabilitation has substantially improved the functional and anatomic outcomes of pediatric cataract surgeries in recent years. However, the phenomenon of refractive growth and the process of emmetropization have continued to puzzle pediatric ophthalmologists and highlight the need for future prospective studies. Posterior capsule opacification and secondary glaucoma are still the major postoperative complications necessitating long-term surveillance in children undergoing cataract surgery early in life. Successful management of pediatric cataracts depends on individualized care and experienced teamwork. We reviewed the etiology, preoperative evaluation including biometry, choice of intraocular lens, surgical techniques, and recent developments in the field of childhood cataract.
children; pediatric cataract; infantile cataract; aphakia; pseudophakia
open access to scientific and medical research
Pediatric cataract is one of the major causes of preventable childhood blindness,
affecting approximately 200,000 children worldwide, with an estimated prevalence
ranging from three to six per 10,000 live births.1–3 Pediatric cataracts may be
congenital if present within the first year of life, developmental if present after infancy,
or traumatic. Early diagnosis and treatment are of crucial importance to prevent the
development of irreversible stimulus-deprivation amblyopia. The management of
pediatric cataract should be customized depending upon the age of onset, laterality,
morphology of the cataract, and other associated ocular and systemic comorbidities.
Recent advances in surgical techniques, intraocular lens (IOL) composition and
designs, increased understanding about the neurobiology of visual development, and
early postoperative use of contact lenses for optical rehabilitation have contributed to
improved outcomes after pediatric cataract surgery. Furthermore, early diagnosis can
be achieved by genetic counseling and testing in cases of hereditary cataracts.4
However, certain issues specific to pediatric eyes, such as increased postoperative
inflammation, axial growth after cataract extraction, implant-power calculation,
secondary glaucoma, posterior-capsule opacification (PCO), and amblyopia management,
are still major obstacles to achieving good visual outcomes
in childhood cataract surgery.5–9
Examination of the child
The evaluation of a child with a cataract begins with a detailed
history including family history; a prenatal history including
maternal drug use and febrile illnesses with rash; and birth
history, especially birth weight, since low birth weight may
81 be associated with idiopathic bilateral congenital cataracts.10
l-u02 A developmental history should be carefully assessed, and
-J21 if required, review should be sought to exclude metabolic
no or systemic related etiologies. A history of the onset of the
.773 lenticular opacities, laterality, and progression is also
impor.711 tant. Unilateral cataracts are usually isolated, but they are
.y35b4 vmaossctuclaotmurme o(nPlFyVf)o;u11nadlstoo,boetahsesroocciautleadr wabinthorpmerasliisttieens,t sfeutcahl
/om as lenticonus/lentiglobus may be associated.
.rscse l.y A detailed ocular examination is carried out either in
ep on the office or in the operating room. This should include
.vdoww lsunae slit-lamp biomicroscopy to assess the size, location, density
of lenticular opacity, capsular changes, such as preexistent
/:/spw rspeo posterior capsular defects, and other associated
anteriortth ro segment developmental anomalies. In addition, measurement
from F of intraocular pressures and corneal diameters are performed.
ded Fundus examination in partial cataracts and ultrasound
lnao examination in total cataracts may reveal posterior-segment
odw abnormalities that may affect the visual outcome. Ultrasound
lygo biomicroscopy can be informative in children with
anteriorlom segment developmental anomalies and PFV, and also in the
thha assessment of posterior capsular support while considering
lpO secondary IOL implantation (Figure 1).12–14 In children under
iilcnaC a1f2temr othnethyshoafvaegbee,eintifsesdommieltki.mes possible to examine them
In preverbal children who are uncooperative for standard
visual acuity testing, fixation behavior, fixation preference,
and objection to occlusion should be checked. In younger
infants with poorly developed fixation, a red reflex test can be
performed in a darkened room with a direct ophthalmoscope
along with undilated retinoscopy to assess the visual
significance of the lens opacity. A central cataract larger than 3 mm
in diameter, unilateral cataract associated with strabismus,
and bilateral cataract with nystagmus are considered visually
significant.15 Asking about the visual interaction of the child
at home with the family members, also helps in determining
the severity of visual dysfunction.
It is important to check the visual acuity in older
cooperative children, if possible with preferential looking cards (Teller
acuity card, Keeler, etc), or other vision tests such as “E” charts
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or Snellen chart appropriate to the age of the child. In cases of
lamellar or posterior subcapsular cataracts, the visual acuity
in an examination lane with dim light may appear normal or
reasonable, but glare testing where visual acuity drops by two
or more lines, will reveal potential difficulty in daylight. The
glare test is performed by shining a bright light to the side of
each eye as the child attempts to read the vision chart.
visual evoked potential
For preverbal, less cooperative, or developmentally delayed
children, pattern visual evoked potential (VEP) can be used to
assess the effect of the opacity on the visual pathway, thereby
indirectly reflecting visual significance of the opacity.16–18
Flash VEPs may be useful in complete cataracts to establish
the gross integrity of the visual pathways.19
Family-album tomography scan
The parents can be asked to bring the family photograph
album if they are unsure about the onset of cataract.
Especially when an older child presents with unilateral cataract,
inspection of the red reflex in old photographs may give a
clue as to whether the cataract was present during the critical
period of visual development, or not.19
examine the family members
A dilated slit-lamp examination of the parents and any sibling
should be performed, which may reveal previously
undiagnosed lenticular changes indicative of an inherited cause for
the child’s cataract (Figure 2). Female carriers of X-linked
conditions associated with males presenting with
congenital cataracts may show lenticular changes. Some important
examples are punctate lens opacities in a mother of a male
child with Lowe syndrome, and Y-sutural opacities in a
mother of a male child with Nance–Horan syndrome.16,20,21
Morphology of pediatric cataract: specific diagnostic
A detailed description of the morphology of a pediatric
cataract may not only help in the diagnosis of a specific condition
but also in planning the management with regard to surgical
as well as nonsurgical treatment. Morphologically, pediatric
cataracts can be broadly classified into the cataracts involving
the entire lens, central cataracts, anterior cataracts, posterior
cataracts, punctate lens opacities, coralline cataracts, sutural
cataracts, wedge-shaped cataract, and cataracts associated
with PFV.11 In this review, we want to highlight cataracts with
typical morphology specific to certain systemic conditions
or syndromes that may be helpful to diagnosis.
Total cataracts can be sporadic or hereditary in nature
(Figure 3); they can also be seen in Down’s syndrome and
congenital rubella syndrome.11 Early surgical intervention is
mandatory to prevent the development of amblyopia.
Anterior polar cataracts are not pathognomonic of any
particular condition, but are commonly seen in patients with
aniridia9 (Figure 4A). They can appear as dot-like,
plaquelike, or in the form of a pyramid. Pyramidal cataracts are
the severe form of anterior capsulolenticular opacities in the
form of a pyramid protruding into the anterior chamber, and
have also been described in children with retinoblastoma and
Ehlers–Danlos syndrome (Figure 4B).22,23
Anterior subcapsular cataracts are associated with uveitis,
trauma, irradiation, and atopic dermatitis. Anterior lenticonus
is a bilateral condition seen in Alport syndrome, and rarely
in Waardenburg syndrome.24,25
Oil-droplet cataracts are the nuclear opacities typically
seen in infants with galactosemia (Figure 5). However,
other forms of cataract, such as posterior subcapsular or
small nuclear and cortical opacification, are also described
in galactosemia.26 The changes are reversible with early
Sunflower cataract is a type of anterior subcapsular
cataract almost only seen in Wilson’s disease, an
autosomalrecessive condition with a defect in the metabolism of
copper leading to accumulation of copper in the liver and basal
ganglia. These lenticular opacities are also reversible with
treatment with penicillamine.27
Posterior subcapsular cataract can be drug-induced
(steroids most commonly) or a complication of radiation
therapy for ocular and periocular tumors.28,29 It has also been
reported in systemic conditions, such as Turner’s syndrome,
Fabry’s disease, Bardet–Biedl syndrome, and
neurofibromatosis type 2.30–34
Membranous cataracts are the disk-like opacities formed
after spontaneous resorption of lens material. These are
typically seen in Hallermann–Streiff syndrome.35 This condition
has also been described in children with congenital rubella
syndrome, Lowe syndrome, and PFV.36–38
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in female carriers of Nance–Horan syndrome, with affected
males presenting with dense visually significant cataracts.20
Although 60% of pediatric cataracts are idiopathic,40 based
on the antenatal history, family history, and the type of
cataract, a baseline laboratory workup may be required
in some cases. Extensive laboratory investigations are
not usually indicated for unilateral cataracts, as most of
them are isolated, nonhereditary, and without any systemic
associations.40,41 However, in any child with a positive
maternal antenatal history suggestive of infection or the
presence of microcephaly, deafness, cardiac
abnormalities, and/or developmental delay should be investigated
for varicella, herpes simplex, toxoplasmosis, rubella, and
syphilis (TORCHS).42–45 Traumatic etiology, particularly of
nonaccidental cause, must always be ruled out in any case
of unilateral cataract.
Children with bilateral cataracts may be considered in
the following way: 1) well infant – rule out galactosemia
and TORCHS; 2) well toddler – rule out galactokinase
deficiency; 3) jaundiced infant with failure to thrive – rule
out galactosemia, urine screening for reducing substances
and erythrocyte assays are effective ways to diagnose
galactosemia;26,46 4) unwell infant or child, eg, in a child
with coexistent congenital glaucoma, hypotonia, and
developmental delay, oculocerebrorenal syndrome (Lowe
syndrome) should be ruled out by checking the urine for
amino acids.21 Specific conditions may need specific
testing, eg, serum calcium, phosphorus, and glucose testing
should be tailored based on the child’s systemic exam.47,48
.vdowww lssunaeo typically associated with Stickler syndrome in addition to
Wedge-shape cataracts are partial lenticular opacities,
/:sp rpe Conradi–Hünermann syndrome, neurofibromatosis type 2,
tthm roF and Fabry’s disease.11,39
frod Punctate cortical opacities sparing the nucleus are
charead acteristically seen in carriers of X-linked recessive Lowe
lonw syndrome and in children with Down’s syndrome.11,21,40
ydo Radiating spoke-like cortical punctate opacities are also
logo consistent with Fabry’s disease.31
lam Sutural cataracts are visually insignificant opacities along
thph the Y-sutures of the lens. They are often found as an incidental
laO finding on routine examination. They have been described
All of these investigations are usually best performed in
conjunction with general health assessment of the child by
Evaluation by a geneticist is helpful for determining the
inheritance pattern and to identify associated syndromes.
Genetically based cataracts account for 8%–29% of all
congenital cataracts, with the majority being
autosomal-dominant in inheritance.49 Autosomal-recessive and X-linked
patterns have also been isolated.50,51 Clinical and genetic
heterogeneity is well documented in autosomal-dominant
cataracts.52 More than 40 different genes and various loci
have been identified with congenital cataracts.53
Mutations in the genes responsible for the maintenance of lens
clarity, such as the crystallin and connexin genes, are the
most commonly described in the etiology of nonsyndromic
inherited cataracts.49,54 Mutations in the genes coding for
transcription factors, aquaporin (Maf), beaded filament
structural protein, vimentin, and lens intrinsic membrane
proteins have also been reported.49,54
Mutations in the α-crystallin gene tend to cause nuclear,
lamellar, zonular, and posterior polar cataracts. In addition
to primary cataract, mutation in CRYAA has been
associated with microcornea. Phenotypic variability is commonly
observed with mutations in the β-crystallin genes.55
Mutations in the developmental genes, such as PAX6, FOXE3,
PITX3, and MAF have also been implicated with cataract
as a part of anterior-segment developmental anomalies.53
Anterior polar cataracts are commonly seen with PAX6
mutations with or without aniridia, whereas PITX3 mutations
predominantly cause posterior polar cataracts.56,57
Furthermore, genes responsible for major syndromic
cataracts include OCRL (Lowe syndrome),21,58 GALK117q
(galactosemia),58 GLA (Fabry’s disease), and NHS (Nance–
Horan cataract–dental syndrome).59
Indication for cataract surgery depends upon how much
visual function is affected. The mere presence of a lenticular
opacity does not indicate surgical removal. Peripheral lens
opacities, punctate opacities with intervening clear zones,
and opacities less than 3 mm in diameter can be observed
closely and successfully managed by treating the associated
amblyopia by patching and glasses.60 For example, anterior
polar and pyramidal cataracts are not visually significant per
se, but can be associated with significant progressive corneal
astigmatism, which can lead to decreased visual acuity and
amblyopia.61 In addition, in small central opacities, a larger
area of clear visual axis can be achieved by pharmacological
dilatation.62 In a recent report, Birch et al found that abnormal
visual acuity and contrast sensitivity in young children with
partial cataracts was associated with poor long-term visual
acuity outcomes.63 This suggests that while conservative
treatment can be used, it must be done so with caution, and
ancillary tests such as glare testing must be utilized to ensure
the lens opacity is not visually significant.
A critical period for visual development has been described
in the first 6 weeks of life, during which the vision is
subcortically mediated and the infant is relatively resistant to
amblyopia.64 Extraction of unilateral congenital cataracts by
4–6 weeks and bilateral congenital cataracts within the first
6–8 weeks of life can prevent the development of
stimulusdeprivation amblyopia, strabismus, and nystagmus.65,66
Prompt optical rehabilitation and occlusion therapy can result
in good visual acuity with fusion and stereopsis.67
A child’s eye is unique
Pediatric eyes are different from adult eyes. They are smaller
in size at birth, with changing axial length and corneal
curvature over a period of time. The mean axial length of a
newborn eye is 16.5 mm. There is rapid growth of eyes in
the first 18 months, which increases to 23 mm by 13 years of
age.68 Similarly, there is a change in the corneal curvature:
from 51.2 D in newborns to 43.5 D in adults.69,70 They have
a thin and less rigid sclera, a more elastic capsule, and a risk
of severe inflammatory response after surgery. Furthermore,
a child has a longer life span after cataract removal, with
a potential for irreversible visual loss due to amblyopia.
Therefore, presurgical evaluation of a growing child’s eye is
a little complex. Moreover, the process of emmetropization
is potentially disrupted.
Preoperative evaluation: biometry
Optical correction after pediatric cataract surgery may be
18 achieved by aphakic glasses, contact lenses, or primary IOL
l-02u implantation. Despite the increasing popularity of primary
-J12 IOL implantation, particularly in young infants, prediction of
on eye growth over a period of time and choosing an
appropri.737 ate IOL power to prevent the unexpected refractive change
.171 is still a challenge. In a recent survey, approximately 70%
.rssce l.y period of time, it is essential to predict eye growth and have
ep on an accurate biometry in order to choose the appropriate IOL
.vdoww lsunae ipnotwoethresufuitteudref.oArecaccuhractehimldeaats uthreemtiemnetsooffsauxrgiaelrylenangdthwaneldl
/:s re keratometry in the office setting may be difficult because of
tth ro poor patient cooperation and poor fixation, and most of the
from F time biometry has to be performed under sedation or general
edd anesthesia in the operating room. Immersion biometry has
lnoa been shown to be more predictable than the contact method
odw for IOL-power calculation children.72
While the general consensus among pediatric cataract
surgeons worldwide is partial undercorrection at the time
of surgery to balance the postoperative myopic shift, some
studies have reported a reduction in axial elongation, and
some have reported an increase in axial elongation in young
children.73–75 Axial elongation has been reported to be more
after unilateral cataract extraction in the operated eye than
bilateral cataract surgery.5 Recent work has documented
axial growths in children operated on at 10 years of age,
and advised a refractive target of residual mild hyperopia
at the time of surgery in older children.76 Considering these
issues, the choice of IOL power should be individualized
based on the child’s need and refractive status of the other
eye in unilateral cases.
An accurate IOL-calculation formula is required for proper
selection of IOL power. SRK II, Holladay 1, Holladay 2,
Hoffer Q, SRK/T, and Pediatric IOL Calculator (a
computer program) have been used to calculate the desired IOL
power in children, with variable results.6,7,77 Various factors,
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including inaccuracies of axial length measurements in the
supine position, biometry technique, keratometry values,
phenomenon of pseudoaccommodation, variability in IOL
position due to capsule fibrosis, and increased corneoscleral
elasticity in children, have been suggested to contribute to
prediction errors.77–81 Additionally, in children younger than
2 years, smaller axial lengths, shallower anterior chambers,
and changes in corneal curvature and corneal thickness may
lead to higher prediction errors.7,82 In the Infant Aphakia
Treatment Study (IATS), less than half of the children were
within 1.00 D of target refraction, with the greatest
prediction errors in eyes with axial lengths of 18 mm or less.83 The
recent report of IATS recommended Holladay 1 and SRK/T
formulae for infant eyes.84 However, at 5 years, they found
refractive errors ranging from +5.00 to -19.00 D, and the
authors believe that the inability to predict axial elongation in
infantile eyes was the primary reason for such a wide range
of refractive errors.75,85
Absolute prediction errors are common even after
secondary IOL implantation, and are reported to range from 0.9±0.9 D
to 2.15±1.68 D.86–89 Refraction-based formulae by Hug, Khan
and Algeed for estimation of secondary posterior-chamber IOL
power provide comparable results to those obtained by standard
biometry-based formulas, and can be useful in difficult
situations when standard biometry cannot be performed or when it
is not available in the operating room.86 It is important to note
that the Hoffer Q equation relies on an anterior-chamber depth
(ACD) value and not on the A-constant. The ACD value is the
predicted postoperative ACD. The value given by
pharmaceutical companies for each IOL is that for adults. It is larger
than that expected for children and especially infants. To use
a more accurate ACD value, the author measures the ACD of
both eyes and then predicts the ACD, using this value for the
Hoffer Q. As a rule of thumb, 1 mm is added to the preoperative
ACD, but this may be modified in unilateral cases, depending
on what the ACD is of the unaffected eye. The author uses an
empiric rule whereby children under the age of 3 months are
left +8.00 D, 3 months to 1 year +6.00 D, 1–2 years +4.00 D,
2–3 years +3.00 D, 3–5 years +2.00 D, 5–7 years +1.00 D, and
7 years and after +0.5 D until the age of 11 years. These
numbers may be modified in unilateral cases, depending upon the
refraction of the other eye, so as to cause minimal anisometropia
2 years after surgery.
Type of IOL
In recent years, acrylic IOLs have gained popularity over
polymethyl methacrylate (PMMA) IOLs, which had
remained the IOL of choice for many years.71,90,91 In children,
AcrySof IOLs are considered better than PMMA IOLs in
terms of greater biocompatibility and smaller incision size
with use of foldable design, with late onset and lower rate of
PCO formation. Hydrophobic acrylic IOLs are used by 93%
of pediatric cataract surgeons. The one-piece lenses (SA or
SN series) for in-the-bag fixation and three-piece lenses (MA
series) for sulcus fixation are preferred.71 In children with
uveitic cataracts, decreased postoperative inflammation has
been reported with the use of heparin-surface-coated PMMA
IOLs.92 Silicone IOLs are used less frequently, because of
an increased rate of capsule contraction.93
In special situations, such as in children with dislocated
lenses either due to trauma or systemic conditions, such
as Marfan’s syndrome (Figure 6), transscleral sutured
monofocal or multifocal IOLs may be considered for visual
rehabilitation. However, limited studies in children have
been reported, with a 12%–24% rate of spontaneous
dislocation of IOL from suture breakage over a follow-up period
of 10 years. This is concerning in the pediatric population
because of their longevity.94–96 Secondly, transscleral sutured
IOL is considered a blind procedure with low accuracy of
placement of haptics in the sulcus.97
Alternatively, iris-fixated IOLs, such as iris-sutured IOLs
and iris-claw IOLs, have been reported with short-term
success with complications, including retinal detachment after
dislocation, hyphema, synechiae, ectopic pupil, fibrinous
uveitis, and vitreous strands in the wound.98,99 Few studies
exist that report the use of angle-supported anterior-chamber
IOLs in older children.100,101 A modified capsular tension
ring with posterior-chamber IOL implantation is another
option in children with partial loss of zonular support.102
The indications for multifocal IOLs in children are
debatable. Simultaneous distance and near vision without the aid
of glasses or contact lenses can be achieved with the use of
multifocal IOLs. This is especially important in children,
as they lose accommodation once the cataract is removed.
Refractive shift during eye growth as well as amblyopia
due to loss of contrast sensitivity associated with
multifocal IOLs are the main concerns of most pediatric cataract
surgeons.71 Although limited studies in children have shown
improved stereopsis and spectacle independence with the
use of multifocal IOLs, studies with long-term follow-up
Cataract surgery in children is challenging, because of
increased scleral elasticity, thicker corneas, increased risk
of trauma from eye rubbing, less compliance with activity
restriction, and most importantly the effect of postoperative
astigmatism on amblyopia. While scleral tunnel incision was
preferred by most pediatric cataract surgeons because it was
thought to induce less postoperative astigmatism, recent
studies have shown clinically insignificant difference between
the two types of incisions, with spontaneous regression of
astigmatism over a period of time.106 Superior incisions are
commonly performed compared to the temporal approach,
probably in view of less risk of injury and postoperative
endophthalmitis. As the flattening of the cornea occurs along
the incisional meridian, there is a tendency toward
withthe-rule astigmatism with the temporal approach in young
children.107 The temporal approach is desirable in deep-seated
eyes and in children with previous or planned filtration
surgery superiorly. However, there is no study comparing
the astigmatic outcome after pediatric cataract surgery with
the superior and temporal approaches. Lensectomy through
pars plana approach can also be done if IOL insertion is not
To prevent wound leakage due to the reasons mentioned,
suturing of all the wounds, including paracentesis with either
a 10-0 or 9-0 Vicryl or nylon suture, is recommended.
Absorbable sutures are preferred, in order to avoid a second visit to
the clinic or operating room for suture removal. Research has
shown that the use of 10-0 Vicryl caused astigmatism, but
that this astigmatism dissipated after 6 weeks.108
Continuous, smooth, and well-centered anterior
capsulotomy is a prerequisite for safe lens implantation. Currently
used techniques for pediatric anterior capsulotomies include
vitrectorhexis, manual continuous curvilinear capsulorhexis
(CCC), can-opener, and radio-frequency diathermy.109–112
Other devices, such as the plasma blade, diacapsutom, and
pulsed-electron avalanche knife, have been suggested to
minimize zonular tension and prevent peripheral CCC extension,
but have not become popular.113 Elasticity and thickness of
the anterior capsule in young children makes manual CCC the
most difficult technique, with a steep learning curve. It can be
performed either with a cystotome or forceps. Manual CCC
81 has been shown to produce the most extensible capsulotomy
l-20u and the smoothest edge with scanning electron microscopy
-J12 evaluation in a porcine model.114 Although manual CCC
no has been the gold standard, some surgeons prefer to use the
.737 vitrector for very young patients and manual CCC for older
.117 children.109 The problem with the vitrectorhexis technique is
/.yb534om ttmhwaaotn-uiintaclisiCsinCooCnt prhouabsshua–svtpeucrolylmtsetpecaehrpnedilqeutaoernmdineagvneculuoarlpveCedCinbCyc.hNTilirdsarcdehinat.iloThnhaaesl
.rscse l.y been shown to produce consistent-size capsulorhexis
openep on ings with minimal risk of extension or tears (Figure 7).115,116
.vdoww lsunae femtosecond laser-assisted anterior and posterior
Recently, Dick and Schultz described promising results with
mmiuelstiipnlefoaunrtecrhioilrdrceanp.s11u7lTorihtieyxailseottoamlaidevsotcoapterepvoesntatgaen-tsetraimorpfrom F capsule phimosis.118 For proper centration of the IOL, the
edd anterior capsulotomy should be smaller than the IOL optic. An
laon ideal capsulotomy for an IOL with optic diameter 5.5–6 mm
odw should be 4–5 mm. Smaller capsulotomies may result in
llyoogm sbeavgedreiacmaeptseurlaorccpuhrismoovseisr,6asm1o5n%thss.h11r9inkage of the capsular
Techniques for cataract extraction
Surgical techniques for pediatric cataract are constantly
evolving to minimize the complication rate. Soft
consistency of pediatric cataracts makes phacoemulsification
unnecessary.120 Lens removal can be conducted through an
anterior approach by manual irrigation and aspiration or
through the pars plana using a vitrector. In rare cases with
membranous cataracts or retrolenticular membranes in PFV,
intraocular scissors are required.120 An anterior-chamber
maintainer used for continuous irrigation prevents the
collapse of the anterior chamber during withdrawal of the
instruments during the surgery.121 It also aids in pupillary dilatation
with the use of 1:1,000 epinephrine added to the balanced
salt solution.122 The use of heparin in the balanced salt
solution and intracameral recombinant tissue-plasminogen
activator has been shown to reduce the fibrinous reaction and
pigment deposits on the IOL.123,124 However, the beneficial
effect of heparin on early postoperative inflammation has
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been questioned in a recent randomized controlled trial.125
Recently, the use of a transconjunctival 25-gauge vitrectomy
system has been gaining popularity for lens removal in
Maintenance of a clear visual axis is critical for a good
postoperative visual outcome after pediatric cataract
extraction. If the posterior capsule is left intact, 100% of eyes
less than 4 years of age develop significant PCO.127,130 The
anterior vitreous face acts a scaffold for the proliferation
of lens epithelial cells (LECs); therefore, it is essential to
combine primary posterior capsulotomy (PPC) with anterior
vitrectomy in infants and young children.128,129 It can also
be considered in older children who are poor candidates
for possible neodymium-doped yttrium aluminum garnet
(Nd:YAG) laser capsulotomy. PPC can be performed either
through a limbal or pars plana approach. The opening for
PPC should be smaller than the anterior capsulotomy.
Staining of the posterior capsules with trypan blue 0.1%
may facilitate the procedure of PPC.130 PPC using the
twoincision push–pull technique has been shown to give
consistent results without vitreous loss during the procedure.115,116
Praveen et al reported triamcinolone-assisted vitrectomy
for visualization of the anterior vitreous during pediatric
Optic capture of the IOL was considered another
surgical technique that would obviate the need for anterior
vitrectomy and prevent the development of PCO.132
However, Vasavada et al believed that anterior vitrectomy was
necessary with optic capture even in older children.133
Furthermore, it is a difficult technique to perform with
singlepiece IOLs without haptic angulation. Posterior vertical
capsulectomy with optic entrapment by Grieshaber et al
showed promising results in 68 children aged 2 months to 8
years, with clear visual axes for 5–12 years postoperatively.
Although this procedure is technically challenging, anterior
vitrectomy is seldom required.134
Postoperatively, subconjunctival or intracameral steroids
are recommended to suppress inflammation in the immediate
postoperative period.135,136 Intracameral recombinant
tissueplasminogen activator has also been shown to reduce the
fibrinous reaction and pigment deposits on the IOL after
pediatric cataract surgery, though there is a risk of hyphema.124
Decrease in the rates of postoperative endophthalmitis in
adults has been reported with the intracameral use of
antibiotics. However, there is no study showing its effectiveness
in the pediatric population.
Postoperative increased inflammatory response in children
can lead to fibrinous reactions, pigment deposits on the IOL,
decentration of the IOL, and posterior synechiae. Toxic
anterior-segment syndrome, which is a well-recognized
comreported in children.137 Even capsular blockage syndrome
has been reported following pediatric cataract surgery.138
Secondary glaucoma is the most feared complication of
pediatric cataract surgery, and is commonly seen in infants.
Studies, including a recent report of the IATS, showed
plication after uneventful adult cataract surgery, has also been
that IOL implantation does not seem to protect from the
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development of secondary glaucoma.85,139 However, in a
recent meta-analysis, Mataftsi et al found that glaucoma
risk after infantile cataract surgery appears to be associated
with surgery within the first month of life and additional
intraocular surgical procedures, but not with primary IOL
implantation. Therefore, the factors influencing the risk for
postoperative glaucoma remain an unresolved issue.8
Measurement of central corneal thickness in aphakic
81 as well as pseudophakic eyes is essential to diagnose
l-20u true glaucoma.140 Therefore, monitoring for glaucoma is
-J21 mandatory for any child undergoing cataract surgery early
on in life. Retinal detachment is a rare late complication of
.737 pediatric cataract surgery.141 It is not known whether the
.711 pars plana approach is associated with increased risk for
.534 retinal detachment.
/ybom comVpilsiucaatlioanxisafotepracpiefidciaattiroicn craetmaraaincts stuhregmeroys.tAclothmomugohn
.rcsse l.y PPC, anterior vitrectomy, use of hydrophobic acrylic IOL,
ep on or in-the-bag IOL implantation have been effective in
.vdoww lsunae preventing or delaying the occurrence of PCO, it is still a
major concern in infantile eyes. Severe capsular phimosis
/:sw rseo can also occlude the visual axis. Nd:YAG laser capsulotomy
tth ro or surgical membranectomy can be performed to clear the
from F visual axis. Sealed-capsule irrigation using either distilled
edd water or 5-fluorouracil has been shown to be successful in
lona reducing the incidence of visual axis opacification in adult
dow eyes by killing the LECs.142,143 This device may help pediatric
lyog cataract surgeons to overcome the problem of PCO, but the
lom risk of endothelial cell loss cannot be underestimated. The
thah development of the bag-in-the-lens technique by Tassignon
lpO et al shows promise, because by using this lens the LECs
ilicanC raeredutrcaipnpgeddrabmetwateiceanlltyhethaentreartieoorfanPdCpOo.s14t4erior capsules, thus
The modalities for visual rehabilitation after pediatric cataract
extraction include IOL implantation, aphakic glasses, and
contact lenses. Aphakic glasses are an efficient method for
visual rehabilitation in children up to 4 years of age.
Addition of a bifocal segment is needed in children 4 years of
age or older. Contact lenses are usually well tolerated, and
the power can be changed until the child is ready for IOL
implantation with predictable postoperative refraction. Rigid
gas-permeable contact lenses are preferred by the majority of
clinicians.145 Good visual acuity can be achieved with contact
lens fitting within 3 weeks of surgery in unilateral aphakes.146
A recent report of the IATS comparing outcomes of contact
lens and IOL correction showed no significant difference in
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median visual acuity between two groups at 5 years,85 but
showed more complications in the IOL group. No matter what
rehabilitation method is used, surveillance is needed.
Unlike adults, visual outcome after a successful cataract
surgery in children finally depends on the timely management
of amblyopia, especially after unilateral cataract
extraction. Generally, patching after pediatric cataract surgery is
continued until it is believed that the child is no longer at risk
of developing amblyopia. However, there is no consensus
about the amount and duration of patching therapy
necessary to achieve a good visual result. Extensive occlusion
therapy during early infancy may disrupt the development
of binocularity.147 Compliance with patching has been shown
to be associated with good postoperative visual outcomes
in unilateral and bilateral cataracts, more so in monocular
cataract.148 Therefore, educating the parents about the
importance of amblyopia therapy postsurgery is crucial.
Despite the better understanding of pathophysiology of
visual maturation in children and advancements in surgical
technology and instrumentation, childhood cataract remains
a challenge to pediatric ophthalmologists.
Prevention of PCO is a major problem faced by the
entire pediatric ophthalmology community worldwide.
Capsule-irrigating devices customized for pediatric eyes,
which will reduce the speed and severity of proliferation of
LECs, or future research discovering the drugs antagonizing
the effect of factors responsible for PCO are warranted.142,143
Recently, the results in an animal model indicated that there
was a beneficial effect on postoperative inflammation with
dexamethasone-coated IOLs.149 It is reasonable to assume
that IOLs will be designed that can deliver slow-release
molecules to reduce inflammation and obviate the need for
topical anti-inflammatory treatment.
Post-operative prediction error is another issue, and there
is a need to develop an IOL-calculation formula customized
for pediatric eyes to minimize it. Furthermore, future studies
are needed to look for the factors, other than secondary
glaucoma, affecting postoperative axial growth, so that surgeons
will have the ability to predict postoperative refraction and
implant an accurately powered IOL in pediatric eyes. There
may also be a place for modified implantation techniques and
modified IOL designs, such as multicomponent adjustable
IOLs (Infinite Vision Optics, Strasbourg, France) to combat
Appropriate measures, such as immunization programs
for rubella and measles and neonatal screening, can reduce
the incidence in developing countries. Finally, continued
genetic studies that will allow clinicians to better understand
the natural history and likely outcome of the cataract are
The authors report no conflicts of interest in this work.
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