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Seminars in Ophthalmology, 2014; 29(5–6): 414–420

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ISSN: 0882-0538 print / 1744-5205 online
DOI: 10.3109/08820538.2014.959192

REVIEW

Complications of Pediatric Cataract Surgery


Mary C. Whitman and Deborah K. Vanderveen

Department of Ophthalmology, Boston Children’s Hospital, Harvard Medical School, Boston,


Massachusetts, USA
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ABSTRACT
Purpose: Cataract surgery in young children poses different challenges and potential complications compared
to those encountered in adult populations. We performed a literature review of the complications of
pediatric cataract surgery. Methods: Literature review of complications of pediatric cataract surgery. Results:
Complications in children vary based on the age of the patient at surgery and the cause of the cataract. Common
events discussed include increased inflammatory response, opacification of the posterior capsule, lens
reproliferation, pupillary membrane, and amblyopia; less common events include infections, significant
bleeding, and retinal detachment. Conclusion: Complications after cataract surgery in children are often
associated with a robust inflammatory reaction or secondary opacity and, in infants, glaucoma. Late
complications can occur decades later, so that long-term follow-up is required. Though surgery carries
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significant risks, the consequences of no surgery and irreversible deprivation amblyopia in very young children
should be considered.
Keywords: Aphakia, aphakic glaucoma, complications, congenital cataract, pediatric cataract

INTRODUCTION or due to trauma (penetrating or blunt, possibly with


injury to other parts of the eye), or be a result of
There are many potential causes of pediatric cataract, treatments such as radiation or corticosteroid therapy.
and the timing of onset and the cause of the cataract Some children with cataracts have syndromes such as
can affect the risk of surgical complications. Children trisomy 21 or have developmental delays, which
may have congenital cataract, present at birth or make management during the postoperative period
shortly thereafter, which may be unilateral or bilat- and visual rehabilitation phase challenging.
eral. These may be caused by associated diagnoses When consenting families for pediatric cataract
such as genetic disorders, prenatal infections, persist- surgery, we review the risks for cataract surgery that
ent fetal vasculature (PFV), or may be idiopathic. In include immediate events, such as bleeding, inflam-
infants, visually significant congenital cataracts need mation, or risk of infection in the eye, and also discuss
to be removed promptly to prevent irreversible the risks of long-term potential complications, includ-
deprivation amblyopia. Many of the eyes with con- ing opacity of the posterior capsule, secondary mem-
genital cataract, however, also have other abnormal- branes, reproliferation of lens material, glaucoma,
ities that increase the risk of complications. dislocation of the intraocular lens (if placed), retinal
Developmental cataracts, not present at birth but detachment, loss of vision or the eye, and need for
developing during early childhood, may also occur. further treatment or surgery for any of these compli-
Surgery for these cataracts can sometimes be delayed cations. We also emphasize that while some of these
if vision is developing normally, though lamellar or events may occur early, some occur many years in the
posterior cataracts may progress quickly and require future. In addition, children must undergo general
intervention. Some cataracts are acquired in associ- anesthesia for each surgical procedure. Finally, the
ation with other ocular diagnoses such as uveitis, importance of compliance with the visual

Received 18 May 2014; revised 19 June 2014; accepted 29 June 2014; published online 24 September 2014
Correspondence: Mary Whitman, Department of Ophthalmology, Boston Children’s Hospital, Fegan 4, 300 Longwood Ave., Boston, MA 02115,
USA. E-mail: Mary.whitman@childrens.harvard.edu

414
Pediatric Cataract Complications 415

rehabilitation program is discussed, since amblyopia depending on the population studied. Over one-third
in young children can result despite successful of cases are diagnosed within a year of cataract
surgery, and certainly will occur without surgery. removal,6 but glaucoma can also develop years or
In this review, we will discuss management of the decades later.7,8
expected consequences of cataract surgery as well as The cause(s) of aphakic glaucoma are unknown,
the postoperative complications, and the intraopera- but several possible mechanisms have been sug-
tive problems that can make postoperative complica- gested, including chronic trabeculitis from inflamma-
tions more likely. tion, blockade of the angle from retained lens
material,9 changes in the trabecular meshwork due
to exposure to lens epithelial cells,10 chemical factors
INFANTILE CATARACTS from the vitreous, and an abnormal anterior segment,
leading to both the cataract and maldevelopment of
Infant Aphakia Treatment Study the angle.
There are several risk factors for aphakic glaucoma,
The Infant Aphakia Treatment Study (IATS) was a several of which are correlated with each other,
randomized, controlled, multicenter trial of infants including congenital onset of cataract, microcornea,
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with unilateral congenital cataract, and age57 months and younger age (3 months) at cataract sur-
at surgery, comparing primary intraocular lens (IOL) gery.7,11–13 A poorly dilating pupil and congenital
implantation to aphakia and contact lens use.1–3 rubella are also risk factors. Though some have
At both one and five years, there were no signifi- suggested that placement of an IOL at initial surgery
cant differences in visual acuity outcomes between is protective from glaucoma, usually the cohorts are
the groups.4,5 Data on complications were collected biased with IOL placement only performed for older
meticulously, and all participating surgeons were children.14,15 In the IATS trial, there was no significant
experienced pediatric cataract surgeons. Intraopera- difference at one year or at five years in the rate of
tive complications tabulated in IATS included iris glaucoma or diagnosis of glaucoma suspect.16
prolapse, iris damage, hyphema, retained cortex, Aphakic glaucoma is usually first treated with
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corneal clouding, lens fragment in vitreous, and medications: topical beta-blockers and prostaglandin
posterior capsule rupture. These complications were inhibitors, and/or systemic carbonic anhydrase
more common in cases in which an IOL was inhibitors (alphagan should not be used in infants or
implanted and mostly occurred during placement of young children due to the risks of central nervous
the IOL.2 Within the first year of the study, the adverse system depression). If the pressure cannot be normal-
events reported included reproliferation of lens ized with medications, then glaucoma surgery should
material, pupillary membrane, corectopia, retinal be performed. Angle surgery, such as goniotomy or
detachment, wound dehiscence, IOL exchange, vitre- trabeculotomy, as would be used for congenital
ous wick, and glaucoma. By five years, at least one glaucoma, is effective in some cases.17 Some patients
adverse event had occurred in 56% of contact lens will require a trabeculectomy (with mitomycin c) or
patients and 81% of IOL patients, the most common tube shunt, and some may continue to require
being lens reproliferation into the visual axis, pupil- medications after surgery. If it is recognized and
lary membranes, and corectopia in the IOL group, and managed appropriately, children with aphakic glau-
diagnosis of glaucoma or glaucoma suspect in the coma can have good visual acuity.18,19 The major
contact lens group.5 cause of vision loss in young children with unilateral
Though a common cause of unilateral cataract in cataract or glaucoma is amblyopia.
infancy is persistent fetal vasculature (PFV; also Children are also susceptible to steroid-induced
known as persistent hyperplastic primary vitreous, ocular hypertension, which must be monitored in the
PHPV), eyes were excluded from enrollment in IATS perioperative period.20 The rise in intraocular pres-
if the corneal diameter was59 mm or for PFV with in- sure occurs earlier and in more dramatic fashion than
pulling of the ciliary processes. Even so, the rate in the adult population, and should be treated until
of complications for eyes with milder forms of steroids can be discontinued.
PFV enrolled in IATS was higher than for eyes
without PFV.2
Posterior Capsular Opacity,
Lens Re-proliferation, and Pupillary
Aphakic Glaucoma and Pseudophakic Membranes
Glaucoma
Posterior Capsular Opacity (PCO) is caused by pro-
Glaucoma is one of the most common complications liferation of residual lens epithelial cells on the
after cataract surgery in infants. The typical reported posterior capsule. In children under five years of
incidence of aphakic glaucoma ranges from 15–45%, age, posterior capsulotomy and anterior vitrectomy
! 2014 Informa Healthcare USA, Inc.
416 M. C. Whitman and D. K. Vanderveen

are routinely performed as part of the initial cataract Inflammation is especially worrisome in children
extraction to lower the incidence of PCO (which is with uveitic cataracts. The surgery is often difficult
virtually 100% without posterior capsulotomy). due to posterior synechiae and pupillary membranes.
Additionally, reproliferation of lens material occurs The surgeon (and rheumatologist) needs to aggres-
in virtually all pediatric patients to some degree. In sively manage inflammation before surgery, often
young children who are left aphakic, this results in with systemic medications. As with adults, inflam-
development of the Sommering’s ring behind the iris, mation should be controlled for at least three months
or Elschnig pearl formation may occur outside of the before surgery. In young children, however, when
capsular leaflets. Even with IOL implantation, lens amblyopia is a concern, there is pressure to operate as
material will fill in within the capsular bag and may soon as possible. These children should be aggres-
migrate centrally on the posterior capsule or on the sively managed to get the eye quiet as soon as
anterior vitreous face. Pupillary membranes are possible so that surgery can be scheduled. IOLs can be
inflammatory membranes across the pupil. All of used successfully, if inflammation is controlled.27
these cause a secondary opacification of the visual Children are also at risk for TASS (Toxic Anterior
axis that needs to be cleared if decreased visual Segment syndrome), an acute, severe intraocular
responses are noted. inflammation with diffuse corneal edema, presenting
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Older children who have simple PCO may undergo 12–24 hours after cataract surgery.28 It represents a
office laser capsulotomy with Nd:YAG laser, and sterile endophthalmitis, and is thought to be a
those who cannot cooperate for this can have laser reaction to contamination or pH imbalance of solu-
capsulotomy performed under general anesthesia. tions, viscoelastics, intraocular medications, or other
Children with both PCO and reproliferation of cortical items that enter the eye. In pediatric cases, TASS has
lens material or with pupillary membranes will been associated with ethyline-oxide sterilized vitrec-
require a second intraocular surgery (and general tomy packs.29
anesthesthetic).

Infection
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Bleeding
In children, the incidence of infectious endophthalmi-
Bleeding after cataract surgery ranges from simple tis is low (51:1000). Staph and strep are the most
subconjunctival hemorrhage, to significant intraocular common organisms identified. Cases of pediatric
hemorrhages. Vitreous hemorrhage is not uncommon endophthalmitis are associated with nasolacrimal
in eyes with PFV, with up to 10% incidence.12 duct obstruction or upper respiratory infection at
Hyphema can be caused by damage to the iris with the time of cataract extraction. The outcome of
vitrector, instruments, or during corneal suturing. endophthalmitis is often poor, with over half of the
Swan syndrome, resulting from abnormal vessels at patients having LP or NLP vision.30,31
the cataract wound, can later cause spontaneous
hyphema, which can be recurrent.21
Retinal Detachment

Inflammation In children, as in adults, cataract extraction increases


the risk of future retinal detachment. Rabiah et al.32
Inflammation is expected after cataract surgery, and reviewed a series of 1,017 eyes of 579 patients with
children have a more robust inflammatory reaction cataract extraction without IOL implantation at age
than adults. Significant postoperative inflammation 516 yrs. With a mean follow-up of 6.8 years (range
can lead to complications that include secondary 2–18.3 years), they found an incidence of retinal
membranes that block vision and posterior synechiae, detachment of 3.2% (33/1017). The mean time from
and necessitate a second surgery. Intracameral pre- surgery to detachment was 6.8 years (range 0.4 to 14.8
servative-free triamcinolone22,23 or dexamethasone24 years). The risk factors they found associated with
decreases inflammation in children and is especially retinal detachment were more myopic (less hyper-
helpful for those undergoing IOL implantation. opic) aphakic refraction and wound dehiscence.
Dexamethasone has routinely been administered Posterior capsulotomy and anterior vitrectomy were
subconjunctivally at the end of pediatric cases. not found to be risk factors. More recently, in
Adding low-molecular-weight heparin to the irrigat- Denmark, in a series of 1,043 eyes of patients aged
ing solution has been tried as a way to decrease 0–17 at the time of surgery, 25 developed retinal
inflammation, but is not very effective.25 Intraocular detachment a mean of 9.1 years after surgery. They
cefuroxime does not decrease fibrin formation, calculated the 20–year risk as 7% overall, but only 3%
indicating fibrin formation is not due to bacterial among patients with isolated cataract. Retinal detach-
contamination.26 ment was more common in patients with intellectual
Seminars in Ophthalmology
Pediatric Cataract Complications 417

disabilities and those with other ocular or systemic surgery within the first year.2,38 By five years, 37%
anomalies.33 percent of contact lens patients and 43% of IOL patients
had strabismus surgery.5 Other studies, including a
greater variety of cataract patients, also report strabis-
Loss of Vision mus in 24–34% of cataract patients.39,40

Although loss of vision can be caused by posterior


capsular opacity or pupillary membranes (‘‘secondary OTHER RISKS AND COMPLICATIONS
cataract’’) that can be treated, or be the end result of
other complications such as endophthalmitis or ret- Corneal Decompensation
inal detachment that result in poor outcomes despite
treatment, the leading cause of vision loss and poor Children undergoing cataract surgery are also subject
outcomes in young children and infants with cataract to other risks and complications seen in adults, such as
is amblyopia. Amblyopia is particularly challenging damage to the corneal endothelium. Children with a
in unilateral cases, since the phakic eye will naturally history of cataract extraction had decreased number of
be the preferred eye. Amblyopia therapy needs to be endothelial cells with polymegathism and pleomorph-
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started promptly after surgery. The IATS protocol ism, unrelated to the presence of IOL.41 On long-term
called for patching one hour per day per month of life follow-up, a 9.2% loss of endothelial cells was noted
(i.e., five hours per day in a five-month-old) until over 12 years.42 No studies have assessed the corneas
eight months, then half of waking hours, through at of adults who had cataract surgery as children to
least age five.1 Compliance with correction of residual determine if they progress to corneal decompensation.
refractive error and patching, if indicated, is required.
Even children who have an IOL implanted have
significant refractive error in childhood, since the Loss of Accommodation
growth of the pediatric eye will result in a myopic
shift, requiring over-correction regardless of the initial In both adults and children, cataract extraction elim-
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postoperative refraction. Because of the difficulties of inates the ability to accommodate. This is a more
maintaining amblyopia therapy in unilateral cases, profound loss for children, however, since most adults
better VA outcomes are usually found in bilateral undergoing cataract surgery have already been pres-
cataract cases compared to unilateral cases.34,35 byopic for years. Surprisingly, many children have
While amblyopia is not a complication of cataract better pseudoaccommodation than might be expected.
surgery, lack of amblyopia treatment will result in a One series of children with monofocal IOLs implanted
poor visual outcome despite successful surgery, and at age 5 or older, and with low residual refractive
its importance should be recognized. On the other error, showed that 50% of eyes and 75% of patients
hand, avoidance of surgery will also result in (depriv- had 20/40 or better vision uncorrected at both
ation) amblyopia. Amblyopia remains a major barrier distance and near, perhaps due to astigmatism.43
to good outcomes after cataract surgery in young There are a few reports of using multifocal IOLs in
children (58 years of age). children, but there have been no randomized con-
trolled trials.44,45

Need for Further Surgery in Children


after Primary Cataract Surgery Associated Ocular Abnormalities
and Diagnoses
The most common reason for additional surgery is
posterior capsule opacification. In children who did The patient with a congenital cataract has an error in
not have primary posterior capsulotomy, over 60% of eye development, and even in cases of unilateral
one- to six-year-olds require intervention within two cataract, there can be abnormalities of the other eye. In
years from surgery.36,37 Among children 6-13 years of patients treated for unilateral cataract, 41% had at
age at the time of surgery, only 19% require YAG least one abnormality of the phakic eye, including
capsulotomy within two years.37 Other additional decreased vision (20%), nystagmus (19%), cataract
surgeries include glaucoma surgery, secondary IOL (15%), iris heterochromia (9%), myopia (6%), micro-
for eyes left aphakic initially, and strabismus surgery. phthalmos (6%), pupillary miosis (2%), congenital
In the IATS study, 63% of patients in the IOL glaucoma (2%), optic nerve abnormality (2%), aniridia
group required additional intraocular surgery within (1%), and corneal opacity (1%). These abnormalities
the first year vs. 12% in the contact lens group, most were often not apparent at the time of initial treat-
commonly for clearing the visual axis (60% IOL group, ment, underscoring the importance of prompt treat-
11% CL group). Additionally, 18% of the contact lens ment for the cataractous eye, as the phakic eye may
group and 10% of the IOL group had strabismus have other abnormalities.46
! 2014 Informa Healthcare USA, Inc.
418 M. C. Whitman and D. K. Vanderveen

Traumatic Cataracts for necessary surgical procedures. In each case, the


risks of not treating the underlying condition need to
Another cause of cataracts in children is ocular be weighed against the potential risks of anesthesia.57
trauma. These cases present a higher risk of compli-
cations due to the initial trauma and damage to other
ocular structures. The surgeon must worry about
zonular instability, capsular holes, and vitreous loss.
CONCLUSION
Complications (especially corneal scarring, posterior
Cataract surgery in children poses different risks
synechiae, posterior capsular tear, and iris distortion)
and complications than in adults. In adults, cataract
are more common with open globe injury than blunt
surgery can be performed with topical or local
trauma.47,48 Many trauma patients need scleral-fix-
anesthesia, is quick, usually uncomplicated, and
ation of an IOL, if an IOL can be placed.49 About half
allows early restoration of vision without extensive
of the cases have a good visual acuity outcome, but
follow-up. In children, however, general anesthesia is
development of amblyopia is common. Common
required, ocular anatomy and cataract morphology
modes of trauma in the US are paintballs, BBs, and
require alternative techniques and management, need
pens and pencils.50 In contrast, in rural India, bow and
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for secondary surgery is common, and visual rehabili-


arrow injuries are common.47 Perhaps unsurprisingly,
tation after cataract surgery is a long process that
the majority of these injuries occur in boys.
requires parental dedication. Despite these challenges,
good outcomes for all ages are possible, and the
consequence of not operating is usually much greater.
Bilateral vs Unilateral Surgery

In children with bilateral cataracts, there is some


controversy about performing surgery on both eyes DECLARATION OF INTEREST
under the same anesthesia, or scheduling surgery
on two separate days.51 Usually, bilateral cases are The authors report no conflicts of interest. The authors
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done on two separate days to prevent the small alone are responsible for the content and writing of
chance of bilateral endophthalmitis (or bilateral TASS) the paper.
if the solutions or equipment are contaminated.
Others argue, however, that the small risk of bilateral
endophthalmitis needs to be weighed against the
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