Review Article

New Management Strategies for Ectopia Lentis

Melissa A. Simon, MD; Catherine A. Origlieri, MD; Anthony M. Dinallo, MD, MPH; Brian J. Forbes, MD, PhD;
Rudolph S. Wagner, MD; Suqin Guo, MD

ABSTRACT
Ectopia lentis refers to displacement of the crystalline
lens in the setting of various systemic and metabolic
disorders. A literature review was conducted to investigate the management of non-traumatic ectopia lentis in the pediatric population, particularly focusing
on surgical intervention. Both limbal and pars plana
approaches for lensectomy are well established in the
literature. Surgical options for intraocular lens implantation in pediatric eyes with ectopia lentis include anterior
chamber intraocular lenses and iris-fixated or scleral-fixated posterior chamber intraocular lenses. Recently, the
use of capsular tension rings has also been described
with promising results. Visual rehabilitation and treatment of amblyopia are essential for patients within the
amblyogenic age group following surgical intervention.
[J Pediatr Ophthalmol Strabismus. 2015;52(5):269-281.]

INTRODUCTION
Ectopia lentis is a rare condition associated with
displacement of the crystalline lens of the eye. The
lens is defined as subluxed when it is displaced but
still contained within the pupillary space, whereas
a luxated lens is completely detached from the ciliary body and can be loose within either the anterior
chamber or the vitreous cavity.1,2 Displacement of
the lens may induce refractive changes such as astigmatism, myopia, or aphakic hyperopia. Patients may

also experience diplopia, particularly if the equator

of the lens crosses the visual axis.1
The underlying pathophysiology of ectopia
lentis involves the disruption or dysfunction of the
zonular fibers.1 Nearly 50% of all cases of lens dislocation are caused by trauma, although ectopia lentis
may occur in predisposed eyes even in the absence of
trauma because this condition frequently occurs in
the setting of systemic disease or metabolic disorder.3
Isolated ectopia lentis, also called simple ectopia
lentis, is an autosomal dominant disorder. This condition may present at birth or later in life, and the
lens generally displaces upward and temporally. Another heritable condition is ectopia lentis et pupillae, in which both the lens and pupil are displaced,
usually in opposite directions. This disorder is autosomal recessive and generally bilateral.1 Microspherophakia, an additional heritable disorder often associated with Weill-Marchesani syndrome, is often
bilateral and characterized by a small and spherical
lens. Patients with microspherophakia typically have
abnormalities in the zonular apparatus, accounting
for the high incidence of lens dislocation in these
eyes.4
Although there are several systemic disorders
associated with ectopia lentis, Marfan syndrome,
WeillMarchesani syndrome, and homocystinuria
account for more than 75% of reported nontraumatic lens displacements. Marfan syndrome is

From The Institute of Ophthalmology and Visual Science, New Jersey Medical School, Rutgers University, Newark, New Jersey (MAS, RSW, SG);
Childrens National Health System, Washington, DC (CAO); Monmouth Medical Center, Long Branch, New Jersey (AMD); and Childrens Hospital of
Philadelphia, Philadelphia, Pennsylvania (BJF).
Submitted: August 29, 2014; Accepted: May 5, 2015
The authors have no financial or proprietary interest in the materials presented herein.
Drs. Simon and Origlieri contributed equally to this work and should be considered as equal first authors.
Dr. Wagner did not participate in the editorial review of this manuscript.
Correspondence: Suqin Guo, MD, The Institute of Ophthalmology and Visual Science, New Jersey Medical School, Rutgers University, 90 Bergen Street,
DOC 6100, Newark, NJ 07101. E-mail: guos1@umdnj.edu
doi: 10.3928/01913913-20150714-02

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an autosomal dominant disorder characterized by an

abnormality in the gene encoding the fibrillin-1 protein, accounting for the typical phenotype of long,
thin limbs, tall stature, joint laxity, and cardiac abnormalities. Up to 80% of patients with Marfan syndrome develop ectopia lentis, and 50% of these cases
occur before the age of 5 years. Lens displacement
is usually bilateral and symmetric, and frequently
occurs in the superotemporal direction. In contrast,
WeillMarchesani syndrome, an autosomal recessive disorder characterized by short stature and short
digits, is associated with inferior dislocation of the
lens. Likewise, up to 90% of patients with homocystinuria experience inferonasal lens displacement.
Lens dislocation in these eyes occurs from progressive zonular degeneration. These patients are usually
tall and thin, and approximately 50% have developmental delay. Thromboembolic events are common
in these patients and may cause death at an early
age; thus, all patients with non-traumatic lens dislocation without an obvious cause should undergo
biochemical screening for homocystinuria.3
The management of ectopia lentis, particularly
in children, is challenging. The goal of this review is
to discuss the management of non-traumatic ectopia lentis in the pediatric population, focusing particularly on the variety of surgical options that exist
for these patients.
VISUAL SEQUELAE AND CONSERVATIVE
MANAGEMENT
The most common cause of uncorrectable poor
vision in non-traumatic ectopia lentis is amblyopia.5-16 Amblyopia, as well as anisometropia, can
lead to poor binocular fixation. Progressive lenticular myopia and astigmatism are often the first signs
of lens dislocation; both may occur when the zonules are disrupted and the lens curvature increases,
or when a displaced lens is tilted.5,6,10,16,17 Myopia in
patients with Marfan syndrome can also be due to
long axial length.5
The lens may assume a range of positions, including minimal dislocation, pupil bisection, dislocation into the anterior chamber, or posterior displacement into the vitreous. The edge of the lens can be
detected in the pupillary space using retinoscopy. The
degree of decreased vision depends on the position
of the lens. If the lens is displaced posteriorly, despite
being central, impairment may be minimal.7 Some
researchers believe that the position of the ectopic
270

lens relative to the pupil correlates more closely with

the degree of amblyopia than do other factors such as
etiology, age, and visual acuity.16,18 Romano et al.18
suggested that it was possible to predict which children with ectopia lentis would develop significant
amblyopia. If the edge of the lens was 1.3 mm from
the center of the pupil (range: 0.3 to 2.3 mm), there
was high probability that the patients vision would
not be correctable with conservative measures.18
Optimal visual correction within the amblyogenic period is critical. Patching may be a part of
this therapy.5,16 However, others hold that occlusion
therapy cannot address the poor visual development
caused by dislocation of the lens.19 Phakic refraction
will typically demonstrate a high degree of myopia
and astigmatism. In cases where lens tilting or movement give a fluctuating or unreliable phakic refraction, aphakic refraction may be advocated.5,7 Some
patients could use an aphakic portion of vision for distance and a phakic portion for near vision, and some
advocate that phakic and aphakic refraction could be
used for monovision, although this would interrupt
binocular vision.5,7 There is some controversy using
phakic and aphakic positions as monovision because
it would eliminate fusion in these children.
Aphakic correction may achieve better vision,
depending on lens position and pupillary space.5,7
Manipulation of the iris has been proposed to expand
the pupillary space by pharmacologic mydriasis,5,7
and historically by laser iridectomy5,20 and iris photocoagulation, although these more aggressive approaches are not current and are controversial.5,21,22
Nd:YAG laser zonulysis has also been performed to
create an aphakic space for refraction.23,24 Photophobia may result from these measures, but visual
acuity may still be optimized and not all patients
require pupillary dilation.7 However, others believe that the pupillary space is still too small after
pharmacological dilation and that such an approach
to aphakic refraction is ineffective.25 Ultimately, the
decision to pursue aphakic or phakic refraction must
be guided by the patients best-corrected visual acuity (BCVA) and as a response to amblyopia therapy.
INDICATIONS FOR SURGERY: HISTORICAL
AND CURRENT REVIEW
Historically, surgery was performed to avoid sequelae of ectopia lentis, including retinal detachment,
cataracts, uveitis, a lens in the anterior chamber or
posterior cavity, imminent complete displacement,
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phacomorphic or phacolytic glaucoma, uncorrectable

vision by refraction, and amblyopia.5,26,27 Historical
approaches, including removal of the lens with capsule forceps, open sky vitrectomy with cryoextraction, discission and aspiration, and intracapsular or
extracapsular extraction, were used in the 1960s,
1970s, and early 1980s, but are no longer reported.5
Before more advanced techniques of microsurgery, visual outcomes from surgical lensectomy
were not reliably improved, and complication rates,
especially retinal detachments, were high.8,9,26,28,29
Furthermore, anesthesia risk for patients with homocystinuria was great.30 Therefore, conservative,
non-surgical management and avoidance of surgery
were advocated.5,31 Instead of surgery, pupillary
dilation and massage were regularly advocated to
move an anteriorly displaced lens back behind the
iris.5,30,32,33 Nevertheless, outcomes with conservative management could also be poor.12
More modern closed microsurgical techniques
with the vitrector revolutionized the surgical approach for ectopia lentis in the 1980s and 1990s.34
Peyman et al.35 first reported success using combined lensectomy and vitrectomy with vitrophage
in ectopia lentis in 1979, with visual improvement
in all 11 eyes regardless of anterior or posterior surgical approach. Girard36 reported similar results in
1981. The closed system technique relieves traction
on the retina and vitreous; it also avoids damage to
the cornea and trabecular meshwork.12
Improvement in anesthesia techniques has made
surgery for patients with homocystinuria safer.11 In
a 1998 study of patients with homocystinuria and
ectopia lentis, only 1 of 82 procedures under general
anesthesia was associated with a complication.37 The
single complication was a transient cerebrovascular
accident that occurred in a patient with no special
precautions taken before or during surgery, and the
patient recovered with no long-term sequelae.37
Even with the improved safety profile of surgical intervention in more recent years, indication for
surgery is not lens subluxation alone. Most commonly, surgery is performed for a lack of improvement in BCVA with conservative management. The
current BCVA threshold for surgery is reported at
20/7012,16,34 or 20/60.6,10,13,38-40
Additional indications for surgery as historically
considered still apply12,17,40 and also include glare, monocular diplopia, and contact lens intolerance.12,16,39,40
Visual rehabilitation may be more effective in a child

with bilateral than unilateral aphakia, similar to children requiring visual rehabilitation after cataract surgery.17 A small retrospective review of children with
ectopia lentis, myopia, and angle-closure glaucoma
showed that lensectomy reverses angle-closure glaucoma when performed early and mitigates the angleclosure glaucoma when performed later.41 In another
study, patients with homocystinuria with a high prevalence of mental delay and inability to test visual acuity
were operated on for lens dislocation, pupillary block,
and optical aberrations. In all 37 eyes with anterior
chamber dislocation, conservative measures were first
tried but failed, and surgery became necessary.37
Much of the available literature stresses that surgical intervention with appropriate visual rehabilitation can prevent a dense amblyopia if performed
early enough, although no controlled trials or retrospective studies have specifically identified an ideal
age for intervention.6,11-13,16,42,43 Romano et al.43
retrospectively reviewed patients with genetic ectopia lentis and found that 50% had significant ametropic functional amblyopia (visual acuity: 20/50 to
20/200), despite appropriate conservative management. The authors recommended early surgery before amblyopia or high axial myopia develops, especially in patients with significant lens displacement
within the center of the pupil that may increase the
risk of developing amblyopia.43 Others believe that
earlier surgery for ectopia lentis before ages 4 or 5
years remains controversial.44
In a study of a single family with familial simple
ectopia lentis, the surgical outcomes in children between the ages of 14 months and 11 years were found
to be superior to the outcomes in their relatives who
underwent surgery at a later adult age, including
outcomes for associated glaucoma; the authors still
concluded that lensectomy should not be performed
for subluxation alone in a patient at any age.25 These
authors believed that adverse sequelae from the subluxation must be present to justify surgery.
In the literature on pediatric cataracts, the importance of early surgical intervention is stressed, as
the visual system becomes sensitive to deprivation at
6 weeks and binocular vision that initially develops
at 3 months of age.45 However, these patients with
cataracts often experience more complete visual deprivation than those with ectopia lentis, and children with ectopia lentis are usually older than those
with congenital cataracts, even if still within the
amblyogenic period.

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SURGICAL APPROACH
Surgical approach remains a matter of much
debate, with no randomized controlled studies in
the literature specifically comparing limbal to pars
plana approaches in patients with ectopia lentis.
Each approach has anatomically strategic advantages and avoids some risks and adverse sequelae of the
other approach. Surgeon comfort level with either
approach may be an important factor.
Limbal

A limbal approach is superior for lenses dislocated into the anterior chamber. This approach also
requires less instrumentation, avoids using a posterior infusion line, and allows for aspiration with
an intact posterior capsule, therefore with reduced
risk for trauma to the vitreous base and retina.10,13,46
Avoidance of a sclerotomy also diminishes the risk
for vitreous hemorrhage or retinal detachment related to this procedure.13 The pars plana does not reach
adult proportions before 7 years of age, and many
authors believe that a posterior approach may be
particularly risky for this immature anatomy.15,46,47
The limbal approach may be easier to perform for
surgeons more experienced with anterior segment
than posterior segment surgery,13 and some advocate that pars plana lensectomies (PPLs) should
be performed only by vitreoretinal surgeons.10,46
Numerous studies support the limbal approach for
patients with non-traumatic ectopia lentis.
In 1990, Bekhi et al.6 published a review of 15
eyes with non-traumatic ectopia lentis operated on
with limbal lensectomy and anterior vitrectomy
between 1977 and 1989. Average patient age at
surgery was 6.15 years. Preoperative visual acuity
after amblyopia treatment ranged from 20/60 to
20/200. Improvement in postoperative visual acuity
was documented in all eyes, ranging from 20/20 to
20/50 during a follow-up period ranging from 8 to
118 months (median: 33 months). The only major
complication was secondary membrane formation
that was treated with Nd:YAG capsulotomy.6
Plager et al.11 performed a similar retrospective
study in 1992 of 29 eyes in children with ectopia lentis operated on with the limbal approach and anterior vitrectomy. All eyes had visual improvement; 27
of 29 eyes had postoperative visual acuity of 20/40
or better, and the poorer visual outcomes were attributed to amblyopia. No significant complications
occurred. Two patients with homocystinuria had
272

bilateral simultaneous surgery under one anesthetic,

with no systemic or ocular complications.11
Subsequent studies confirmed these findings, with
all but a few patients obtaining improved visual acuity,
most to better than 20/40, and those with poorer visual outcomes could generally be attributed to amblyopia. Complications from these studies included:
transient anterior chamber hyphema, vitreous hemorrhage necessitating a trabeculectomy in a patient with
a preoperative pupillary membrane and glaucoma; intractable uveitis requiring a secondary vitrectomy in
an eye with preoperative dislocation of the lens into
the anterior chamber; and eyes with vitreous strands
to the wound, requiring secondary vitrectomy or
Nd:YAG laser treatment in some patients.13,47
A larger retrospective review by Kim et al.16 of
78 eyes of children with ectopia lentis, also treated
with limbal lensectomy and anterior vitrectomy,
reported that surgical trauma to the zonules was
avoided by performing hydrodelineation and maintaining the capsular bag until the end of surgery.
The average patient age at surgery was 5.7 years and
the average length of follow-up was 7.1 years. Visual
acuity improved in all eyes except two, which was
attributed to preoperative amblyopia. One patient
with Marfan syndrome developed a retinal detachment 9 years after surgery.16
PPL

A pars plana approach gains access to any lens

or fragments in the posterior chamber, and it also
minimizes the chance of displacing the lens more
posteriorly during lensectomy.10,34,39 The pars plana approach also avoids possible complications of
the limbal approach, most commonly vitreous loss,
vitreous adhesion to the wound or incarceration,
and need for further surgery due to these complications.10,14,15,39,46-48 A pars plana approach allows for
complete vitreous removal, which can reduce traction on the retina, and any necessary treatment to
the retina can be administered at the time of lensectomy.14,46 Additional sequelae that PPL helps to
avoid include anterior synechiae, anterior wound
dehiscence, pupillary block glaucoma, bullous keratopathy, epithelial or stromal ingrowth, surgically
induced astigmatism, and iris trauma.34
Despite the theoretical risk of retinal detachment
associated with intraocular surgeries in high myopic
eyes and some types of ectopia lentis, many studies
with PPL showed no retinal detachments.10,15,34,39,40
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One group of researchers reviewed 18 eyes after

lensectomy for ectopia lentis and noted no retinal
detachment. The authors suggested that the low
incidence of postoperative detachment may be due
to relative stabilization of axial length after lensectomy.40 The use of closed intraocular microsurgical
techniques and improved surgical equipment have
allowed greater intraoperative control with fewer
complications, including retinal detachment.10
In a study by Hubbard et al.,46 40 eyes with
Marfan syndrome and ectopia lentis that underwent
PPL with complete vitrectomy and intraoperative
panretinal photocoagulation (PRP) reported improved vision in all patients, but concluded prophylactic laser treatment was unnecessary. The postoperative rate of retinal detachment was 6%, which is
higher than in other recent studies of ectopia lentis
in children, but this rate is in part attributed to the
high predisposition of eyes with Marfan syndrome
to develop a retinal detachment.46
In 1987, Reese and Weingeist34 reviewed 12
eyes treated with PPL and anterior vitrectomy. Etiologies of ectopia lentis included idiopathic, Marfan
syndrome, familial, and congenital. The average patient age was 7.8 years, and the average length of follow-up was 5.1 years. All but one patient improved
to a BCVA of 20/40 or better, and the one poor
outcome was attributed to amblyopia. No complications occurred during this study.34
Additional studies of non-traumatic ectopia
lentis in children treated by PPL with anterior vitrectomy had similar results. Complications included: posterior vitreous detachment, transient vitreous
hemorrhage, peripheral anterior synechiae, contact
lens-related complications, and a single retinal detachment 2 years after surgery in a child with Marfan syndrome.12,15,40
Some reviews reported cases with both limbal
and pars plana approach. Hakin et al.10 published
a retrospective review of 44 eyes in children with
ectopia lentis. The surgeons performed both PPL
and limbal lensectomies, according to individual
surgeon preference. Of note, all PPLs in their study
were performed by a vitreoretinal surgeon. It is not
clear whether the location of the subluxed lens influenced the selected approach, but the authors did
mention that in case of a hard nucleus, a PPL approach was advantageous should the lens fall to the
posterior chamber intraoperatively. All patients had
anterior vitrectomies. All eyes had improvement in

vision, and 75% had a postoperative visual acuity of

20/40 or better; poorer outcomes were attributed to
amblyopia. Six of 26 limbal lensectomy eyes required
removal of vitreous strands to the entry sites with a
second surgery, and 1 of 18 PPL eyes had secondary
removal of residual lens. Two of 4 eyes that had 360
of retinal cryotherapy during PPL developed tears,
but none developed detachments. No retinal detachments or glaucoma complications were reported.10
The authors concluded that limbal lensectomy and
PPL were both successful techniques. The limbal
approach requires less instrumentation and reduces
possible trauma to the vitreous base and retina. This
is particularly relevant in children, because the pars
plana does not reach adult proportions. PPLs were all
undertaken by vitreoretinal surgeons in their study.10
Anteby et al.38 reviewed cases where 59 eyes of
children with ectopia lentis were operated on between
1980 and 1994 and an additional 38 eyes were operated
on between 1994 and 2001. The surgeons performed
either limbal lensectomy or PPL with combined anterior vitrectomy in all children. For the later cohort,
PPL was used in 71% of eyes for marked phacodonesis or nearly total subluxation, and the limbal approach was used in 29% of eyes for less marked subluxation. Patient age at the time of surgery was not
statistically different between the two surgical groups,
and postoperative visual acuities were only marginally
better in the later cohort (84.2% BCVA 20/40 or better vs 80%). Complications included one eye in each
study period with a retinal tear or detachment. The authors concluded that surgery with either the limbal or
PPL approach using an ocutome vitrectomy probe was
successful over long-term follow-up. The study also
showed little change in approach or results between the
1980 to 1994 and 1994 to 2001 cohorts.38
Anterior Vitrectomy

Before the advent of automated vitrectomy, the

most common complication of lensectomy for ectopia lentis was vitreous loss.29 Automated vitrectomy
with in-the-bag aspiration allows for total removal
of the anterior vitreous and diminishes vitreous loss,
secondary membrane formation, pupillary block,
and retinal detachment.11,13 Anterior vitrectomy became common practice for lensectomy in children
with ectopia lentis to prevent not only vitreous loss
and displacement, but also posterior opacification
postoperatively.45 Posterior capsular opacity (secondary membrane) is a common complication and

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major risk factor of amblyopia after lensectomy in

children. A primary vitrectomy may be needed during lensectomy for both ectopia lentis and cataract
surgery in young children.
A retrospective review of children with ectopia
lentis who had lensectomies with intraocular lens
(IOL) implantation in posterior capsules left intact
on first surgery for suspicion of zonular weakness
was performed. Thirty-one of 37 (84%) eyes developed visual axis opacification, and 26 had a secondary surgery; 21 of these had discission of the posterior capsule with anterior vitrectomy and 5 had
Nd:YAG capsulotomy. Five eyes had more than one
surgery for the posterior visual axis opacity.42
Similar to children after cataract surgery, pediatric
patients with ectopia lentis who have lensectomies are
prone to develop visually significant posterior capsular
opacity, leading to amblyopia without prompt treatment. Forbes and Guo45 stated that the childs vitreous
is dense and can act as scaffold for the growth of cells
even in the absence of a posterior capsule when reporting on posterior capsular/vitreous face opacification in
pediatric cataract surgery. For these patients with pediatric cataract, they advocate either primary posterior
capsulotomy with anterior vitrectomy or primary posterior capsulotomy with optic capture via the limbal
or pars plana approach.45 Nd:YAG capsulotomy is an
option for posterior capsule opacification, but it may
require multiple procedures and high energy. Although
their discussion did not address patients with ectopia
lentis, it is important to consider better managing anterior vitreous face to achieve best visual outcomes in
patients with ectopia lentis.45,49
Similarly, Kugelberg et al.50 randomized patients with pediatric cataract who all had a posterior capsulorhexis to vitrectomy or no vitrectomy.
Patients younger than 5.2 years were more likely to
require secondary procedures for posterior capsular
opacification without primary vitrectomy. In another study, similar results directed recommendations
for posterior capsulotomy and anterior vitrectomy
in children younger than 5 years.51 However, these
studies evaluated children with cataracts, not ectopia lentis, and the main outcome was posterior capsular/vitreous face opacity, not BCVA.
VISUAL REHABILITATION
In cases of postoperative aphakia, visual rehabilitation must begin as soon as possible, usually within 1 to 2 weeks after surgery; traditionally, this reha274

bilitation has been with refraction, glasses or contact

lens fitting, and any necessary occlusion therapy.10,38
Anteby et al.38 gave children younger than 3 years
a prescription for monovision, whereas those older
than 3 years had multifocal glasses or contacts for
distance and reading glasses. Some believe children
with bilateral aphakia can tolerate aphakic spectacles well.5 Others argue that aphakic glasses are
heavy, awkward, constrict visual fields, and offer
prismatic distortion.52,53 Drawbacks of contact lens
use include cost, possible infection, displacement of
lenses, and noncompliance with use and care.14
Recently, IOL placement has been performed
more frequently as a primary or secondary procedure in pediatric patients with ectopia lentis
requiring surgical intervention. Although IOL
implantation is the subject of much debate, it
is considered appropriate management for children older than 2 years with pediatric cataracts.45
Compared to contact lenses, IOLs have reduced
the chances of inducing corneal epithelial erosion
or infection, and are not dependent on patient
and caretaker cooperation.16 The option of IOL
placement depends on the amount and degree of
capsular support that exists. Options include anterior chamber IOL (AC-IOL), iris-fixated IOL,
and sutured or sutureless transscleral-fixated IOL;
each has its own associated benefits and risks.54
More recently, some surgeons have begun using
capsular tension rings (CTRs) and capsular tension segments (CTSs) in their surgical management of patients with ectopia lentis. Each of these
options is discussed below.
AC-IOL

The original design of the AC-IOL was a closedloop, rigid lens; the newer and more frequently used
design is that of an open-loop, flexible AC-IOL.
These lenses have been associated with iritis, pigment dispersion, glaucoma, hyphema, corectopia,
iris-sphincter erosion, corneal endothelial decompensation, and cystoid macular edema.55-58 Many
of these side effects occurred with either open-loop
or iris-fixated AC-IOLs.58 Although the newer
open-loop design is less associated with the above
risks,57 such complications in the pediatric population are more likely to occur over time given the
obvious longer life expectancy of children. For this
reason, AC-IOLs are not typically the first choice
in pediatric patients with ectopia lentis who require
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lensectomy; however, they may be considered in the

absence of adequate capsular support.
Closed-loop, semiflexible anterior chamber
implants are not commonly used due to high incidence of corneal edema and persistent cystoid
macular edema. Smith et al.59 evaluated 53 eyes that
underwent implantation of closed-loop AC-IOL
(Surgidev Style 10 Leiske IOL; Surgidev Corporation, Goleta, CA). Surgery for IOL removal or exchange was needed in 34 (64%) of 53 eyes and corneal transplantation was needed in 26 (49%) of 53
eyes. Given the intractable inflammatory sequelae
and complications associated with closed-loop ACIOLs, the authors strongly urge discontinuation of
these lenses.59
Some surgeons are cautious about using ACIOLs, specifically in patients with Marfan syndrome
who require lensectomy.60 Because these patients
tend to have large eyes with deep anterior chambers
and recessed angles, the standard AC-IOL may be
too small for implantation, and thus IOL dislocation, corneal decompensation, iritis, or glaucoma
may develop. Another concern in patients with Marfan syndrome is their increased risk of retinal breaks
and detachment, and the presence of an AC-IOL
may make subsequent examination of the retinal
periphery difficult.61 This is especially concerning
in children with Marfan syndrome who will require
routine retinal evaluation for several decades.
In 2005, a retrospective study evaluated AC-IOL
placement in pediatric patients with ectopia lentis
from Marfan syndrome.62 Eight eyes with BCVA
of 20/50 to 20/400 were implanted with a singlepiece, polymethylmethacrylate AC-IOL (model
MTA4UO; Alcon Laboratories, Inc.). Average postoperative BCVA at the most recent follow-up visit
(mean: 12.7 months [range: 9 to 16 months]) was
20/32 (range: 20/50 to 20/20). Complications included pigment deposition on the AC-IOL (3 eyes),
iritis (1 eye), iatrogenic sector iridotomy (1 eye), and
fine keratic precipitates (1 eye).62
Another retrospective study evaluated 4 eyes
in children with idiopathic lens subluxation who
underwent lensectomy via sclerocorneal tunnel
incision and iris fixation of an AC-IOL (Artisan
model 305, Ophthec USA, Inc., Boca Raton, FL)
as a single procedure.63 Preoperative BCVA was
worse than 6/12 in all eyes. Patients were observed
for a minimum of 8 months, and all eyes gained at
least two Snellen lines of BCVA. No postoperative

complications were reported. Interestingly, whereas

no patient underwent a preoperative corneal endothelial cell count, two of the three patients who
had unilateral surgery exhibited no endothelial cell
loss after 8 months compared to their non-operative
eyes. This is in contrast to data showing 40% to
44% endothelial cell loss in children following
AC-IOL placement.64
Much of the data on pediatric AC-IOLs comes
from studies on pediatric cataract extraction without ectopia lentis. In one report, 10 children underwent AC-IOL placement as a secondary procedure
and were observed for 12 to 78 months (mean: 49.2
months).65 Among patients who had reached visual
maturity prior to becoming aphakic, postoperative BCVA was at least 20/40 in all eyes following
AC-IOL placement. However, among amblyopic
eyes BCVA in 40% was 20/400 and no eyes achieved
BCVA better than 20/40. The most common complication related to AC-IOL placement was pigment
deposition on the IOL surface, followed by corectopia, and haptic migration with subsequent glaucoma.
A concern of AC-IOL insertion is the greater postoperative recovery time and astigmatism compared
to the foldable posterior chamber IOL (PC-IOL).66
Iris-fixated PC-IOL

In the absence of adequate capsular support, a

second option for IOL placement in children with
ectopia lentis is an iris-fixated PC-IOL.
Some studies have looked at iris-fixated PC-IOL
following PPL in children with ectopia lentis. A
study from 2008 evaluated 22 pediatric eyes with
ectopia lentis (average age: 5.5 years) in which PPLvitrectomy was performed; 12 eyes then underwent
iris-fixated PC-IOL implantation, whereas the remaining eyes were left aphakic.67 After an average
follow-up of 19.8 months, mean BCVA in cases of
iris-fixated PC-IOL improved from 20/117 to 20/35,
and more than 80% achieved BCVA of 20/40 or better. IOL dislocation occurred in 4 eyes after an average of 5 months due to rotation of the IOL haptic
out of the suture loop. Following IOL resuturing, all
4 eyes achieved a BCVA of at least 20/30.
Other retrospective data have shown similar results for children with ectopia lentis who undergo
PPL-vitrectomy with primary or secondary placement of an iris-fixated PC-IOL.68 In all cases, a
three-piece foldable PC-IOL was sutured to the iris
using a modified Siepser sliding knot.69 IOL disloca-

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275

tion occurred in several eyes after 6 months (range:

1.8 to 9.5 months), and did so independently
of patient age, IOL model, or meridian of suture
placement. None of the IOL dislocations were the
result of suture compromise or erosion. Outcome
following IOL repositioning seemed to vary. One
patient with Marfan syndrome developed iris capture following IOL repositioning, but was successfully managed and remained stable by postoperative
month 17. Another patient with Marfan syndrome
developed retinal detachment 8 months after IOL
repositioning, and the visual acuity declined from
20/50 to hand motions.68
A modified technique of iris suture fixation of a
foldable PC-IOL has been described in children with
ectopia lentis for placement within the sulcus space in
the region of intact zonule, using the remaining part
of the sulcus to guide the proper positioning of the
IOL haptic and iris sutures.56 This study looked at
17 eyes of 9 pediatric patients with ectopia lentis, the
majority of whom had superior lens subluxation associated with Marfan syndrome. Lensectomy was performed in all eyes via an anterior approach through a
small (3 2 mm) capsulorhexis, followed by ablation
of the inferior zonule, capsular bag, and anterior vitreous with a vitrectome. A superior crescent of zonule and capsular bag were left intact to provide sulcus
support to the superior IOL haptic. The monobloc
hydrophobic acrylic IOL (SA60; Alcon Laboratories,
Inc.) was used for this study. Both the superior and
inferior haptics were sutured to the posterior iris.
Mean BCVA improved from 20/153 (range: 20/400
to 20/50) to 20/32 (range: 20/50 to 20/20) over an
average follow-up period of 16.3 months (range: 12
to 25 months). No eyes experienced IOL dislocation throughout this period, but one eye developed
hyphema, synechiae, and corectopia requiring pupilloplasty after 2 months, and another eye developed
aseptic endophthalmitis requiring vitrectomy.56
Literature on adult patients with iris-fixated
PC-IOLs illustrates iris pigment epithelial loss as
a potential intraoperative complication; however,
subsequent development of pigment dispersion
glaucoma has not been reported.70 Postoperative
complications include the formation of peripheral
anterior synechiae overlying the IOL haptics, suture
breakage, and suture erosion into an iris vessel leading to hyphema.70 Some authors support using a 9-0
instead of 10-0 polypropylene suture to decrease the
risk of suture breakage.58,68
276

Scleral-fixated PC-IOL

In addition to the iris-fixated PC-IOL, another

viable option for pediatric eyes in the absence of
adequate capsular support is the transscleral-fixated PC-IOL. This technique, originally described
in adults more than 30 years ago, has been used
(albeit cautiously) in pediatric patients since the
1990s.55,64,65 In 2010, Kim71 evaluated 22 eyes of
11 pediatric patients with ectopia lentis secondary
to Marfan syndrome who underwent lensectomy
and concomitant transscleral-fixated PC-IOL placement. Average age at the time of surgery was 9.8
years (range: 4 to 17 years), and mean follow-up
was 10.4 months (range: 2 to 20 months). Average
BCVA improved from 1.0 logMAR (range: 2.0 to
0.5 logMAR) to 0.18 logMAR (range: 0.5 to -0.1
logMAR). Postoperative complications included
pupillary capture of IOL (2 eyes) and elevated intraocular pressure (IOP) (5 eyes). No eyes developed
retinal detachment in the follow-up period, which
the authors believed was due to visco-dissection of
the posterior capsule from the anterior vitreous face
prior to PC-IOL insertion.71
An earlier review included children with inadequate capsular support secondary to Marfan syndrome (7 eyes), essential lens dislocation (7 eyes),
spherophakia (5 eyes), and perforation with lens
injury (2 eyes).72 Sixteen of these 21 eyes underwent lensectomy via limbal incision and concomitant transscleral-fixated PC-IOL placement (model
CZ70BD; Alcon Laboratories, Inc., or model 722Y;
Pharmacia & Upjohn, Kalamazzo, MI). Mean age
at the time of surgery was 5.8 years (range: 1 to
11 years), and average follow-up was 21.6 months
(range: 9 to 33 months). BCVA improved in 15 eyes
and remained unchanged in 4 eyes; vision in the remaining 2 eyes could not be measured preoperatively
or postoperatively given concurrent developmental
delay. Postoperative complications included mild fibrinoid anterior chamber reaction (2 eyes), posterior
synechiae formation and cells on the IOL surface (4
eyes), and IOL subluxation into the anterior chamber that was managed successfully with pilocarpine
(2 eyes). Because both cases of IOL subluxation occurred with the CZ70BD lens (Alcon Laboratories,
Inc.), it is possible that this was due to the relatively
small optic size (6.5 or 7.0 mm) compared to the pediatric pupil, which is often 7.0 mm in darkness.72,73
No eyes in this review developed cystoid macular
edema, hemorrhage, or retinal detachment.
Copyright SLACK Incorporated

A case report from 2002 described a 5-yearold boy with ectopia lentis and subsequent bilateral superonasal subluxation who underwent bilateral transscleral-fixated IOL placement.74 An IOL
(Acrysof; Alcon Laboratories, Inc.) was inserted via
a polypropylene suture bridging the posterior chamber by way of two sclerotomies placed 180 apart.
After the sutures were adjusted to center the IOL
appropriately, the sutures were then secured beneath each scleral flap. Although complicated by an
IOP spike on the first postoperative day, both eyes
achieved BCVA of 20/40 within 2 weeks (preoperative visual acuity was 20/40 in the right eye and
20/60 in the left eye).74
Some surgeons advocate the importance of anterior vitrectomy when performing lensectomy with
transscleral-fixated PC-IOL placement in children
with ectopia lentis. One retrospective review evaluated 17 pediatric eyes with ectopia lentis, including
Marfan syndrome (9 eyes), ectopia lentis et pupillae (3 eyes), simple ectopia lentis (2 eyes), sporadic
spherophakia (1 eye), and homocystinuria (2 eyes).40
All eyes underwent PPL and anterior vitrectomy; 16
eyes were left aphakic, whereas one eye that had been
aphakic for 6 years underwent secondary placement
of a transscleral-fixated PC-IOL for contact lens
intolerance. BCVA in this patient, who had undergone amblyopia treatment following lensectomy, improved from 20/1600 to 20/100 after placement of a
+26.5-diopter PC-IOL; her refraction changed from
-8.00 +1.25 90 to -1.00 +3.00 80. The only postoperative complication in this patient was transient
IOP elevation to 28 mm Hg in both her operated and
unoperated eyes at postoperative year 5, which resolved spontaneously without optic nerve changes.40
Another retrospective review evaluated 6 pediatric
eyes with ectopia lentis secondary to Marfan syndrome
who underwent lensectomy and anterior vitrectomy
with concomitant transscleral-fixated PC-IOL implantation.75 Average age at the time of surgery was 66.5
months (range: 60 to 73 months), with a mean followup of 71.5 months (range: 12 to 84 months). BCVA
improved from 20/400 to 20/320, 20/63, and 20/32,
respectively, in 3 eyes, and from 20/50 to 20/40 in one
eye. Of the remaining 2 eyes, one remained stable at
central, steady, and maintained in a preverbal child,
and one worsened from 20/200 to no light perception
due to endophthalmitis. Late IOL decentration occurred in one eye (postoperative month not indicated)
due to breakage of the 10-0 polypropylene suture.75

A literature review by Johnston and Charteris57

found that although combined PPL and anterior
vitrectomy was safer in patients with ectopia lentis
who have a greater than baseline risk of retinal detachment, performing a complete pars plana vitrectomy in these eyes may reduce this risk. The authors
pointed out the challenge in placing sutures precisely within the ciliary sulcus, because in eyes without
zonular tension, including those with ectopia lentis,
the ciliary processes tend to contract, thereby narrowing the ciliary sulcus and making PC-IOL placement challenging. Furthermore, to reduce the risk
of hemorrhage induced by damage to the major arterial circle of the iris, needle passes need to be made
through the avascular portion of the ciliary body
when suturing the PC-IOL into place. The authors
also recommended using a single-piece polymethylmethacrylate IOL with a large optic (range: 6.5 to
7.0 mm), haptic-to-haptic diameter of 12.0 to 12.5
mm, posteriorly angled haptics, and preferably haptics with eyelets to allow easier fixation. Although
their review was not specific to either pediatric patients or those with a history of ectopia lentis, the
authors point out that for patients with Marfan syndrome who have undergone vitrectomy, the above
average incidence of hypoplastic iris sphincter muscles may put them at risk for IOL tilt and pupillary
capture of the IOL optic.57
Legitimate concern exists regarding postoperative complications following pediatric transscleralfixated PC-IOL placement, including pupillary
abnormalities, glaucoma (including pigmentary
glaucoma), IOL tilt or decentration, pupillary IOL
capture, uveitis, hemorrhage, cystoid macular edema, retinal detachment, suture erosion or breakage, and endophthalmitis.56,67,76-78 Intraoperatively,
problems such as hypotony, hyphema, or suprachoroidal hemorrhage can occur given the degree of
manipulation required to insert and adequately secure a transscleral-fixated PC-IOL.55 Furthermore,
suture erosion through the conjunctiva may lead
to endophthalmitis; for this reason, it is common
practice to bury suture knots within the sclera or to
conceal them beneath a scleral flap.55,74
A large concern with pediatric eyes is IOL subluxation, because lifelong IOL stabilization is obviously an important surgical goal in children. Interestingly, the sole support for the IOL postoperatively
appears to be the suture itself rather than fibrosis.
Gonioscopy performed at postoperative month

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277

36 in one study55 found that contrary to previous

hypotheses,64 no fibrous proliferation had formed
around the IOL haptics. Some authors recommend
using 9-0 instead of 10-0 polypropylene sutures for
both transscleral- and iris-fixated PC-IOLs, particularly in the pediatric population who require IOL
stabilization for many decades.55,68
Data on children who undergo primary or secondary transscleral-fixated PC-IOL implantation for
congenital or traumatic cataracts have demonstrated
surgical stability over a broad range of follow-up periods (range: 4 to 40 months).55,65,72 Postoperative
BCVA in non-amblyopic eyes improved or remained
stable in most cases. The most common postoperative
complications appear to be persistent inflammation,
increased IOP, and IOL decentration.55,77 Less frequent complications include vitreous hemorrhage,65
cystoid macular edema,77 and suture erosion through
the conjunctiva.77 In some cases, suture erosion has
been managed by shrinking the suture ends with an
argon laser.77 To decrease the risk of suture erosion
and endophthalmitis, one may bury suture knots
within the sclera, cut suture ends flush with the knot,
cauterize suture ends if they begin to erode through
the conjunctiva, or use a scleral flap or donor scleral
patch intraoperatively.77 Some authors advocate haptic fixation via sutureless techniques, including scleral
flaps or tunnels54 or intrascleral gluing.79
In addition to the aforementioned risks, another concern with transscleral-fixated PC-IOLs is
that inserting them, particularly placing the haptics
in the proper location, is essentially a blind procedure.64 When using an ab interno technique, the
needle tip is obscured by the iris (blind approach),
and for this reason many surgeons advocate using
an ab externo technique to minimize the intraocular manipulation.70 The latter approach also offers
the benefit of passing the suture through the sclera
while the eye is closed and firm, which is of particular benefit in vitrectomized eyes.57 Endoscopic
visualization has been employed to overcome the
difficulty of visualization during ab interno sulcus
IOL placement.80
Another obstacle to placement of a sulcus-fixated
IOL is the resultant astigmatism from the relatively
large corneoscleral wound that is required for insertion of a single-piece polymethylmethacrylate IOL.74
Foldable IOLs pose risk given the lack of sufficient
location on the haptic to secure a suture, predisposing to possible lens rotation and movement. Certain
278

IOLs may prove more fitting for this use; not only
can a suture be more firmly secured to a lens with a
terminal knob on its haptic, but newer flexible lenses
permit small eyes to grow without placing tension on
the fixation suture. Furthermore, these lenses may
provide more stability because greater flexibility allows for a longer area of contact between the haptic
and ciliary sulcus. Conversely, greater flexibility, particularly at the hapticoptic junction, compounded
with a smaller optic size (5.5 mm versus 7.0 mm),
may predispose these lenses to subluxation or visual
disturbances in the setting of large pupils.74
CTR

The CTR is a promising option for patients

with ectopia lentis who require lensectomy. Inserted
into the capsular bag, this device provides both intraoperative and postoperative stabilization of the
capsular bagIOL complex. The standard CTR is
an open, compressible ring of polymethylmethacrylate with a single eyelet at each end.81,82 By providing centrifugal force at the capsular equator that
is distributed equally around the zonular apparatus, the CTR stabilizes weak zonules by recruiting
stronger ones.81-83 It also decreases the occurrence
of postoperative capsular traction and posterior capsular opacification formation.82 The ideal CTR is
slightly larger than the capsular bag itself, so that
once in place the ends overlap, thus providing complete circumferential support.82 Another advantage
to the CTR is that it promotes symmetric postoperative capsular contraction, unlike the less controlled
changes observed in the absence of a CTR.82 The
literature shows promising results for both adult and
pediatric patients with ectopia lentis who have undergone lensectomy and in-the-bag IOL implantation with the aid of a CTR.84,85
Currently, there are no standard guidelines for
choosing a CTR size. Lens capsule diameter determines which CTR size is appropriate, and although
related to axial length, it correlates better with the
white-to-white measurement. Thus, when choosing
among the three sizes of currently approved CTRs,
the white-to-white should first be measured with a
caliper or the IOLMaster (Carl Zeiss Meditec, Jena,
Germany). In addition to lens capsule diameter,
the surgeon should also subjectively assess the degree and type of zonular laxity. An eye with a small
white-to-white measurement and not much zonular laxity could do well with a small CTR. On the
Copyright SLACK Incorporated

other hand, an eye with a large white-to-white measurement and marked laxity might do better with a
larger diameter CTR.86
The surgeon may insert the CTR into the capsular bag before or after nuclear extraction, being cautious not to entrap cortical material beneath the CTR
if placed prior to lensectomy.82 The CTR should not
be used in the presence of an anterior or posterior
capsular tear, including noncontinuous capsulorhexis, because ensuing forces may extend small tears that
are already present.82,83 Also in cases involving a previously decentered and fibrosed capsule, suturing a
standard CTR through the capsular bag may induce
astigmatism or capsular tear.71 It is important to note
that the CTR is indicated in cases of mild, generalized
zonular weakness or localized zonular dialysis of less
than 3 or 4 hours.81-83 It is not adequate for recentering a severely subluxed lens or preventing progressive
zonular loss.73,81,82 For either of these conditions, one
may consider a modified CTR or CTS, both of which
may be sutured to the scleral wall. Thus, it is important for the surgeon to consider whether zonular integrity is expected to worsen over time. For instance,
in comparison to ectopia lentis associated with metabolic disorders with generalized zonular dysfunction/
laxity, lens subluxation due to trauma appears to respond well to the use of a CTR, because the remaining zonules are strong and healthy.86
Because capsular tears may result from suturing
the CTR through the capsular bag, the modified
CTR was designed to address this complication.82
The modified CTR has one or two fixation eyelets
that protrude 0.25 mm anteriorly from the ring;
once the modified CTR is in place, these eyelets sit
anterior to the anterior capsule and provide an anchor for sutures while allowing for uncompromised
integrity of the capsule itself.82,83 Typically, the eyelets are sutured in the direction of zonular weakness.42 An adequately sized capsulorhexis (5.5 mm)
decreases the risk of pigment dispersion or inflammation induced by mechanical trauma of the eyelets
against the iris.81,82 Some authors believe that the
modified CTR gives the surgeon greater control of
the capsule during insertion of the IOL.42 Another
benefit to this ring is a square-edge design that decreases the incidence of posterior capsular opacification formation.83
A retrospective study of children and adults
with Marfan syndrome (56 eyes), idiopathic ectopia
lentis (30 eyes), and WeillMarchesani syndrome

(4 eyes) looked at lensectomy with modified CTR

and PC-IOL implantation.66 A modified CTR was
placed either before phacoemulsification and sutured
with 10-0 polypropylene suture or after phacoemulsification and sutured with 9-0 polyproylene suture.
BCVA improved in 79 eyes, remained stable in 9 eyes,
and worsened in one eye. Postoperative complications included symptomatic posterior capsular opacification requiring Nd:YAG capsulotomy (18 eyes),
iritis treated with prolonged topical steroid therapy (3
eyes), increased IOP treated topically (2 eyes), pseudophacodonesis (1 eye), and focal retinal detachment
after 1 month that was successfully managed with
photocoagulation (1 eye). Late IOL decentration occurred in 6 eyes, 5 of which underwent resuturing,
and suture breakage occurred in 9 eyes after an average of 1 year, 5 of which underwent resuturing. All
cases of decentration or suture breakage occurred in
the setting of 10-0 polypropylene suture.66
Another study from 2007 evaluated 37 eyes
in 22 children with ectopia lentis who underwent
lensectomy with insertion of a standard CTR (2
eyes) or modified CTR (33 eyes).42 Each ring was
fixed to the sclera with a 10-0 polypropylene suture
passing either through the eyelet and capsular bag
in cases with CTR or through the anterior eyelet
in cases with modified CTR. All eyes underwent
in-the-bag placement of a single-piece (SN60AT/
SN30AL; Alcon Laboratories, Inc.) or multi-piece
(MA60BM/MA60AC/MA30BA; Alcon Laboratories, Inc.) acrylic IOL. Average BCVA improved
from 0.26 (range: 0.015 to 0.65) to 0.59 (range: 0.2
to 1.0), and among non-amblyopic eyes postoperative BCVA ranged from 0.4 to 1.0. Postoperative
complications included visual axis opacification (31
eyes), IOL dislocation requiring secondary suturing
(2 eyes), ocular surface discomfort related to sutures
requiring trimming under anesthesia (2 eyes), and
anterior synechiae formation (1 eye). No patients in
this study developed glaucoma, retinal detachment,
or endophthalmitis during the follow-up period.42
In addition to the modified CTR, another implant that can be sutured to the scleral wall without
passing the needle through the capsule is the CTS.
This ring segment is designed for placement within
the capsular bag over an area of zonular weakness
and, unlike the standard CTR, can be used in cases
of profound zonular insufficiency.82 Its design is 120
with a radius of 5 mm and anteriorly positioned eyelets.82 Because it can be implanted without a dialing

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279

technique, the CTS can be inserted with less force

compared to the CTR and may even be safe to use in
the setting of discontinuous capsulorhexis, anterior
capsular tear, or posterior capsule rent.81,82 Interestingly, multiple CTSs may be placed within the same
capsular bag, or may be used concomitantly with a
CTR. Furthermore, the CTS offers the option of
removal from the eye following lensectomy if so desired.82 Surgical success has been demonstrated in
adults with Marfan syndrome who undergo phacoemulsification followed by in-the-bag IOL insertion
with the aid of one or two CTSs. After 1 year, all 10
eyes in this study maintained IOL centration, with
8 eyes achieving BCVA of at least 6/12; however,
there remains limited data on CTS use in children
with ectopia lentis.60
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