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Pediatric cataract surgery
Abhay R. Vasavada and Bharti R. Nihalani
Purpose of review
Pediatric cataract surgery remains a very important and
difficult problem to manage. While dramatic advances
have occurred in this field over the past 10 years, some
technical aspects of surgery, changing refraction and
functional outcome continue to pose significant problems.
The aim of the present review is to update the reader on
advances reported on the topic during the past year.
Recent findings
Manual capsulorhexis still remains a gold standard for the
successful outcome of pediatric cataract surgery. Primary
management of the posterior capsule is mandatory
depending on the age of the child at surgery. Primary
implantation of the intraocular lens after cataract removal
is gaining popularity even in infants and young children.
Short-term results of single-piece Acrysof in pediatric eyes
are encouraging. Predicting axial growth and the refractive
change that accompanies it is one of the major challenges
for long-term care of children after surgery. The evaluation
of rate of axial growth and its correlation with age at
surgery, laterality, aphakia/pseudophakia and visual-axis
obscuration is a positive step in the right direction.
Despite satisfactory technical outcomes, the functional
outcomes remain unpredictable.
Summary
With refinements in surgical techniques, improvisation of
intraocular lenses and better understanding of growth of
the pediatric eye, in the coming years intraocular lens
implantation is likely to become an established mode of
treatment of children even in the youngest age group.
Introduction
Keywords
anterior vitrectomy, cognitive vision, congenital cataract,
pediatric cataract surgery, visual rehabilitation
Indications for surgery
Curr Opin Ophthalmol 17:54–61. # 2006 Lippincott Williams & Wilkins.
Iladevi Cataract and IOL Research Centre, Raghudeep Eye Clinic, Ahmedabad,
India
Correspondence to Dr Abhay R. Vasavada MS, FRCS, Iladevi Cataract IOL
Research Centre, Raghudeep Eye Clinic, Gurukul Road, Memnagar, Ahmedabad,
380 052 India
Tel: 91 79 27492303, 27490909; fax: 91 79 27411200;
e-mail: [email protected]
Current Opinion in Ophthalmology 2006, 17:54–61
Abbreviations
CCC
continuous curvilinear capsulorhexis
IOL
intraocular lens
PCCC posterior continuous curvilinear capsulorhexis
PCO
posterior capsule opacification
VAO
visual axis opacification
# 2006 Lippincott Williams & Wilkins
1040-8738
54
Pediatric cataract is the most common cause of treatable
childhood blindness, accounting for 5–20% of blindness
in children worldwide [1–3]. Managing cataracts in children remains a challenge. Treatment is often difficult
and tedious and requires a dedicated team effort, the
most important members being parents [3]. The timing
of treatment is crucial to the visual development and
successful rehabilitation of children. Managing pediatric
cataract poses important problems related to technical
aspects of surgery, changing refraction and functional
outcome. However, with refinements in surgical techniques, improvisation in quality and designs of intraocular lenses (IOLs) and amblyopia regimes, both technical and functional outcomes of pediatric cataract
surgery are improving.
Etiology of congenital cataract
Zetterstrom and co-authors [4••] in a review and update
of cataracts in children enumerated the etiology of the
cataract in the developed world. The most common
cause of bilateral congenital cataract was idiopathic.
About one third of cases were hereditary, without a systemic disease. Rare causes included metabolic disorders,
systemic abnormalities, intra-uterine infection and a few
ocular conditions. In contrast, unilateral cataract in most
cases was idiopathic. For the first time, Raghu and colleagues [5] reported association of herpes simplex virus
type 1 (HSV-1) with congenital cataract.
Indications for cataract surgery include visually significant central cataracts larger than 3 mm in diameter,
dense nuclear cataracts, cataracts obstructing the examiner’s view of the fundus and cataracts associated with
strabismus [4].
Pre-operative examination
The importance of counseling parents cannot be overstressed. Parents should understand that treatment of
the child starts only after surgery. They need to come
for regular follow-up visits and see that the child wears
glasses or contact lenses despite the IOL implantation;
the child may also need occlusion therapy following surgery. It is important to assess the visual function of the
child, if possible with charts such as preferential looking
charts (Teller acuity card, Keeler, Berkshire, SL4 4AA),
Lea gratings and symbols (Precision vision, Lasalle,
USA), Sheridian Gardiner tests, ‘E’ charts or Snellen’s
Pediatric cataract surgery Vasavada and Nihalani
charts as in older children. In very young children, who
cannot cooperate for vision tests, the ability to fixate or
follow light or objects should be assessed. The presence
of squint or nystagmus should be noted and ocular
movements should be checked.
Preoperative examination with fully dilated pupils if
necessary under anesthesia is mandatory in both eyes.
This should include examination under operating microscope or slit lamp biomicroscope to assess the cataract,
tonometry to rule out any association of glaucoma, measurement of corneal diameter, posterior segment evaluation, keratometry and biometry. The surgeon should
look for a preexisting posterior capsule defect, which
may turn out to be a camouflaged catastrophe [6].
Are pediatric eyes different?
In comparison with adult eyes, pediatric eyes have
greater elasticity of the capsule, lower scleral rigidity
and mitotically active lens epithelial cells, leading to
higher incidence of posterior capsule opacification
(PCO) necessitating primary management of the posterior capsule and thick vitreous gel giving protection
against cystoid macular edema.
Special considerations in pediatric cataract
surgery
The following subsections discuss three areas that
require special consideration.
Technical aspects of surgery
The surgeon should strictly adhere to the principles of
the closed chamber technique, such as valvular incision,
injection of viscoelastic before removing any instrument
from the eye, bimanual irrigation–aspiration and twoport anterior vitrectomy. A 3 mm wide limbal valvular
incision with 1–1.5 mm internal entry or a scleral incision is preferred. Most surgeons prefer to suture these
incisions in view of the low scleral rigidity and a child’s
tendency to rub the eyes. This suturing may induce
astigmatism; hence it is advisable to remove the suture
within 4–6 weeks of surgery. Bradfield and colleagues
[7] demonstrated that small-incision clear corneal cataract extraction with IOL implantation led to minimal
postoperative astigmatism that remained stable over
time.
High-viscosity viscoelastic agents [8] and trypan blue
dye [9,10] are useful adjuncts in pediatric surgery. In a
mature cataract, or where visibility is poor, trypan blue
staining of the anterior capsule is extremely helpful.
Saini and colleagues [11] have stated that Acrysof
IOLs should not be implanted during pediatric cataract
surgery if trypan blue dye is used. Brown and co-authors
[12] have suggested that both trypan blue and acrylic
IOLs are valuable tools in the treatment of pediatric
55
cataracts and should not be declared mutually exclusive
without better evidence.
Anterior lens capsule management
The anterior capsulotomy shape, size and edge integrity
are now recognized as being very important for longterm centration of a capsule bag fixated IOL [13,
14••,15•]. Manual continuous curvilinear capsulorhexis
(CCC) is a popular anterior capsulotomy technique for
adult cataract surgery. The anterior capsule in children,
however, is very elastic. Manual CCC is therefore difficult to perform and control in pediatric eyes. This has
led researchers and surgeons to search for alternative
methods to open the anterior capsule in children [13].
Alternatives currently available include vitrectorhexis,
radio-frequency diathermy and Fugo plasma blade.
Wilson [14••] compared five different anterior capsulotomy techniques using a porcine model. Extensibility
and edge characteristics were reviewed with each technique. Manual CCC was found to produce the most
extensible capsulotomy with most regular edge. These
findings were confirmed by scanning electron microscopy (Trivedi RH, Wilson ME, Bartholomew LR presented at the congress of the American Society of
Cataract and Refractive Surgery; 15–20 April 2005;
Washington DC). Manual CCC remains a gold standard
for resistance to tearing and should be accomplished
whenever possible (Fig. 1). Most surgeons prefer manual CCC for children above 2 years.
Vitrectorhexis has proved to be a good alternative to
manual CCC for young children, especially in the first
Figure 1 Primary manual anterior and posterior continuous
curvilinear capsulorhexis (ACCC, PCCC) showing a smooth
and regular edge
56 Cataract surgery and lens implantation
two years of life when the capsule is very elastic and
difficult to control. Vitrectorhexis is easier to perform
and is a good option for children when anterior vitrectomy is performed as part of primary management. In
contrast, a diathermy-cut capsulotomy, even when performed perfectly, can be seen to have coagulated capsular debris along the edge. In addition, this edge has been
shown experimentally to be less elastic than a manual
CCC [16]. The Fugo blade is a unique cutting instrument that uses plasma for ablating tissue [17,18]. The
Fugo blade helps make a perfectly controlled anterior
capsulotomy of any size, without the risk of a radial
tear. The peculiar structure of the cut edge ensures
that even if a deliberate radial cut is made in the capsulotomy, it will not spontaneously extend towards the
equator. Radio-frequency diathermy and the Fugo
blade are recommended when fibrotic capsules are
encountered or in white cataracts with the absence of
the red reflex.
Titiyal and colleagues [19] recommend postage-stamp
multiple anterior capsulorhexisotomies following IOL
implantation. They have observed that after CCC, anterior capsule opacification and fibrosis were most marked
Figure 4 Anterior vitreous face response (AVR response)
within the posterior capsulorhexis margin at 1.8 year follow-up
in a 5-year-old child with PCCC only
Figure 2 Primary anterior limbal vitrectomy being performed in
a 1-year-old child
Figure 5 A photograph showing proliferative PCO in an 8-yearold child with single-piece Acrysof at 1.5 year follow-up
Figure 3 A well centered single-piece Acrysof in a 2-year-old
boy, showing clear visual axis and early proliferation at the
margin of posterior capsulorhexis at 2.2 year follow-up
Pediatric cataract surgery Vasavada and Nihalani
57
at the margin of capsular rims. They believe capsulorhexisotomies will prevent phimosis of anterior capsule
opening and capsular contraction syndrome.
depends on the age of the child and IOL material [24,
26–28]. The consensus is that primary vitrectomy is not
necessary in children above 5 years (Fig. 2).
Management of the posterior capsule
Bimanual anterior limbal vitrectomy is preferred by
most pediatric cataract surgeons over pars plana vitrectomy. Pars plana vitrectomy is performed for secondary
procedure in eyes with VAO. A transconjunctival sutureless vitrectomy system, being less traumatic, may prove
invaluable in pediatric eyes [29•].
The most frequent and significant problem following
pediatric cataract surgery is PCO [20–23]. The younger
the child, the higher the incidence and the earlier the
onset of PCO. Maintenance of a clear visual axis remains
a high priority when planning management of the posterior capsule in the amblyogenic age range. Guo and
co-authors [15•] have reviewed the literature related to
pediatric cataract surgery and have summarized the
advantages and disadvantages of various approaches
related to surgical management of the anterior capsule,
posterior capsule and anterior vitreous. The consensus is
to perform posterior continuous curvilinear capsulorhexis (PCCC) with anterior vitrectomy in children
under 6–7 years or in children who have poor cooperation for probable Nd:YAG capsulotomy. PCCC without
anterior vitrectomy was found to be a preferred approach
in children above 7 years or for those who cooperate
for Nd:YAG laser capsulotomy at a later time. PCCC
alone may delay the onset of PCO but cannot eliminate
it [15•,24]. The posterior capsule should be left intact in
children above 9–10 years or in those who cooperate for
laser treatment when indicated.
Most surgeons prefer to manage the posterior capsule at
the time of cataract surgery [20,22]. The common practice is to carry out posterior capsulotomy before IOL
implantation if the limbal approach is used (Fig. 1). If
the pars plana approach is used, however, posterior capsulotomy and vitrectomy should be performed after IOL
implantation. Ideally one should aim for a 3.5–4 mm
posterior capsulotomy, which is circular and centric. Posterior capsulotomy can be performed with various
approaches including manual PCCC, vitrectorhexis,
radio-frequency diathermy and the Fugo plasma blade
[15•]. Like ACCC, manual PCCC remains a gold standard for performing posterior capsulotomy. The current
opinion of management of the posterior capsule seems
to be a manual PCCC of 3.5–4 mm size in children
under 7 years of age.
Is vitrectomy necessary?
The anterior vitreous face is closely linked to the posterior capsule. It is more ‘reactive’ in infants and young
children. It acts as a scaffold [25], not only for lens
epithelial cells but also for metaplastic cells. The
inflammatory response in small children is severe and
fibrous membranes may form on the intact anterior
vitreous face, resulting in visual axis opacification
(VAO). Hence anterior vitrectomy along with posterior
capsulotomy is advocated in infants and young children
[15•]. In our experience, the need for vitrectomy
Intraocular lens implantation
Options for optical correction following pediatric cataract surgery are primary IOL implantation, aphakic
glasses and contact lenses. Primary IOL implantation
has become a preferred approach in children above
2 years [30–32]. IOL implantation is still questioned in
children under 2 years as these eyes are most susceptible to intense PCO and excessive uveal inflammation
[4••,26,31,32]. Microcornea, microphthalmos and uveitis
are absolute contraindications whereas aniridia,
glaucoma and persistent hyperplastic primary vitreous
are relative contraindications of IOL implantation.
IOL fixation, material and size are important determinants of immediate and long-term outcome. In-the-bag
fixation is the most preferred site of IOL implantation.
PMMA (polymethyl methacrylate) IOLs have remained
the IOL of choice for many years but the current opinion favors the use of Acrysof [27,28,33–41,42•]. With
Acrysof, the type of PCO is predominantly proliferative.
PCO sets in at a later stage, typically at 14–16 months.
Visual obstruction produced with Acrysof is less severe
than PMMA and is therefore less amblyogenic (Figs 3, 4
and 5).
The preliminary results of single-piece Acrysof have
been encouraging [40,41,42•]. Trivedi and Wilson [40]
believe that single-piece Acrysof SA series IOLs would
be ideal for children. Kugelberg and colleagues [42•]
performed a randomized study on 66 children aged
3 to 15 years to evaluate PCO in children having cataract
surgery with or without anterior vitrectomy. They also
examined properties of single-piece Acrysof SA30AL.
The authors found that children in the younger age
group (≤62 months) had surgery for after-cataract more
often than children in the older age group (P < 0.1) and
patients who did not receive anterior vitrectomy had surgery for after-cataract more often (P < 0.1). They also
found that SA30AL maintained good centration, produced minimal inflammation and was well tolerated in
pediatric eyes. Single-piece Acrysof has extremely flexible haptics, combined with excellent memory, which
makes the lens easy to implant and not prone to deformation. As the haptics unfold slowly, it is easier to
manipulate IOLs in the bag even in the presence of
58 Cataract surgery and lens implantation
PCCC. One-piece construction is believed to be robust
in resisting capsule contracting forces. Also, single-piece
Acrysof adapts to the smallest capsular bag without
becoming decentered. These unique features have led
some surgeons to prefer single-piece over three-piece
Acrysof for pediatric surgery. However, long-term clinical experience is necessary to derive firm conclusions
regarding the biocompatibility of single-piece Acrysof
in pediatric eyes.
Contact lenses seem to be a good option for optical correction [43]. They also provide an answer to the problem
of changing refraction, which often accompanies IOL
implantation. Expenses, compliance and setup, however, are important for contact lens practice. Autrata
and co-authors [44] compared visual acuity, ocular alignment, re-treatment rate and binocular visual outcomes
in children with primary IOL implantation with contact
lens correction for aphakia in one-year old children.The
study included 41 children with unilateral congenital
cataract undergoing cataract surgery with PCCC and
anterior vitrectomy coupled with IOLs (n = 18) or with
contact lenses (n = 23). The mean age at surgery was
3.11 ± 2.65 months. The authors found that the IOL
group had improved visual acuity and binocular visual
outcome with less occurrence of strabismus. The reoperation rate, however, was 78% in the IOL group
compared with 35% in the contact lens group
(P = 0.017). Lambert and colleagues [45] found similar
results while evaluating children undergoing unilateral
cataract surgery during the first six months of life
(n = 25). They observed that visual acuity results were
similar in the two treatment groups (P = 0.99); however,
50% of children in the IOL group compared with 28% in
contact lens group had 20/40 or better visual acuity. A
large randomized clinical trial, the Infant Aphakia Treatment study, is currently under way to compare primary
IOL implantation with contact lens correction in children undergoing unilateral cataract surgery in the first
six months of life [45]. A current trend among highvolume cataract surgeons dealing with pediatric cataracts
and many pediatric ophthalmologists is to consider IOL
implantation as a viable option on a selective basis in
children under 2 years.
Speeg-Schatz and colleagues [46] found that initial rehabilitation of bilateral cataracts with aphakic correction
and secondary IOL implantation in children under
1 year led to predictable postoperative refraction and
fewer complications. Visual rehabilitation in unilateral
aphakia, however, was more difficult because of poor
compliance with contact lenses, which led to a preference for early IOL implantation. For secondary implantation, in-the-bag fixation of foldable IOLs was found to
be associated with a low rate of complications [47]. A
higher rate of decentration, however, was noted in fold-
able lenses compared with PMMA lenses when IOLs
were placed in sulcus, particularly in eyes of myopic
males.
Is optic capture essential?
It was believed that optic capture would allow surgeons
to avoid planned anterior vitrectomy and minimize the
risk of PCO [48–50]. Anterior vitrectomy, however, was
necessary with optic capture even in children above
5 years [49]. Optic capture maintained better IOL centration but was predisposed to an increased inflammatory response [50]. Hence optic capture has become
less popular among pediatric surgeons. Recently, Grieshaber and colleagues [51] performed anterior and posterior vertical capsulotomy with optic entrapment of
IOLs by maintaining anterior hyaloid in children aged
2 months to 8 years. They found that this technique,
although challenging, was safe and ensured clear visual
axis and IOL centration.
Complications of pediatric cataract surgery
The following complications occur frequently during
pediatric cataract surgery.
Visual axis opacification VAO still remains the most
frequent complication of pediatric cataract surgery [20–
23]. The most critical factor influencing the occurrence
of VAO is age. While opacification is nearly universal in
infantile eyes, the incidence decreases with increasing
age. Primary management of the posterior capsule and
anterior vitrectomy are effective in preventing reopacification of visual pathways [52]. The type and
material of the IOL is another factor affecting the
incidence of VAO. A new IOL implanted using an
innovative ‘bag-in-the-lens’ technique seems promising
in greatly reducing or eliminating PCO (Godts DJ, De
Veuster I, Tassignon MJ, et al. presented at the 23rd
Congress of the European Society of Cataract and
Refractive Surgery; 10–14 September 2005; Lisbon). A
Sealed Capsule Irrigation device has a potential clinical
usefulness in reducing the incidence of VAO, which
needs to be confirmed in pediatric eyes [53].
Glaucoma Glaucoma is a recognized complication of
pediatric cataract surgery. Despite improved surgical
techniques, the incidence of glaucoma following
successful cataract removal remains high [54••,55,56]. A
significant number of surgeons regard aphakia as a cause
of glaucoma [56,57]. This glaucoma, however, may be
better described as ‘glaucoma in aphakia and
pseudophakia’ [54••]. The most common type of
glaucoma to develop following congenital cataract
surgery is open-angle glaucoma [54••,55]. The risk
factors include age at surgery, preexisting ocular
abnormalities, type of cataract and the effect of lens
particles, lens proteins, inflammatory cells and retained
Pediatric cataract surgery Vasavada and Nihalani
lens material. In addition, microcornea, secondary
surgery, chronic postoperative inflammation, the type
of lensectomy procedure or instrumentation, pupillary
block and the duration of postoperative observation
have been found to influence the likelihood of
glaucoma after pediatric cataract surgery [54••].
Undergoing lensectomy at a young age, especially in
the first year of life, may be a risk factor for
development of glaucoma [55]. It has been suggested
that the immaturity of the developing infant’s angle
leads to increased susceptibility to secondary surgical
trauma [55]. Hence some surgeons believe it prudent
to consider delaying surgery until the infant is 4 weeks
old in bilateral cases. Glaucoma can occur at any time
after congenital cataract surgery. Therefore, pediatric
aphakic and pseudophakic patients should be routinely
monitored for glaucoma throughout their lives.
Uveal inflammation Intense uveal inflammation or severe
fibrinoid reaction is a concern particularly in infants and
younger children. Addition of heparin to the irrigating
solution has been suggested to reduce postoperative
inflammatory reaction and related complications such
as synechiae, pupil irregularity and IOL decentration
[58]. In our opinion, atraumatic surgical techniques and
in-the-bag fixation of IOLs are other contributing
factors, which may help reduce the inflammatory
response.
Other complications Rabiah and colleagues [59] performed
a retrospective review to determine the frequency and
to identify the predictors of retinal detachment. They
found that retinal detachment is infrequent following
aphakia in pediatric cataract surgery, at least at shortterm follow-up. They believe that a postoperative
aphakic refractive error, more myopic than the ageadjusted aphakic norm, is predictive of retinal
detachment.
Corneal astigmatism [7,60] is recognized as a problem
arising from cataract surgery. Postoperative astigmatism
is of greater importance in children than in adults
because of its adverse effect on vision development
and the risk of amblyopia. Children, however, show
spontaneous regression of astigmatism during the first
5 months of follow-up [60]. An interesting finding is
that relatively less astigmatism was observed in children
having surgery under 3 years [7].
Eye growth and changing refraction
Predicting axial growth, and the refractive change that
accompanies it, is one of the major challenges for longterm care following pediatric cataract surgery [61]. This
is especially true with wide-spread acceptance of fixed
power IOL implantation. Unless the growth of the eye
59
can be accurately predicted, selection of IOL power is a
difficult task [62].
Axial growth after cataract surgery can be attributed to
normal eye growth, other factors – including age at
surgery, visual input, the presence or absence of IOL,
laterality, genetic factors – also influence this process
[61,63••]. The interocular axial length difference was
found to be another important variable influencing
axial growth [64]. Understanding pediatric eye growth
will help in IOL power calculation and the prediction
of refractive changes after IOL implantation.
Functional outcome
Congenital cataract causes visual deprivation that results
in severe amblyopia. Changing refraction with amblyopia poses a grave challenge to visual rehabilitation. Strabismus is another factor influencing visual rehabilitation
following pediatric cataract surgery [65]. Despite a satisfactory technical outcome, functional outcome remains
unpredictable. Children who do not get good visual
acuity can be rehabilitated with cognitive vision [66].
Conclusion
With refinements in surgical techniques, improvisation
of IOLs and better understanding of pediatric eye
growth, IOL implantation will become an established
modality of treatment in the years ahead even for children in the youngest age group.
.
References and recommended reading
Papers of particular interest, published within the annual period of review, have
been highlighted as:
• of special interest
•• of outstanding interest
Additional references related to this topic can also be found in the Current
World Literature section in this issue (pp. 108–109).
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An interesting case report describing a new technique of a sutureless vitrectomy
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Pediatric cataract surgery Vasavada and Nihalani
61 Wilson ME Jr, Trivedi RH. Eye growth after pediatric cataract surgery. Am J
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64 Lal G, Trivedi RH, Wilson ME Jr. et al. Interocular axial length difference in
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