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Saudi Journal of Ophthalmology (2012) 26, 5560

Cataract Update

Cataract surgery in patients with history of uveitis


Ujwala Baheti, DO, DNB; Sana S. Siddique, MD; C. Stephen Foster, MD, FACS, FACR

Abstract
Cataract surgery in patients with uveitis is not as simple as any senile cataract surgery. Recent evidence suggests that useful visual
outcome can be achieved in most of the cases if they are handled meticulously. Key factors leading to improved visual outcome are
absolute control of preoperative inflammation with diligent use of immunomodulatory drugs, meticulous surgery along with early
detection and care of postoperative complications. Modern technologies in the intraocular lens designs and materials have contributed to the success. In this article, we review the literature on this subject with emphasis on the importance of the use of immunomodulatory drugs to control preoperative and postoperative intraocular inflammation and avoid complications.
Keywords: Uveitis, Cataract, Immunomodulatory drugs, Intraocular lens biocompatibility
2012 Saudi Ophthalmological Society, King Saud University. All rights reserved.
doi:10.1016/j.sjopt.2011.10.003

Introduction

Preoperative evaluation

Cataract is a common complication of uveitis and results


from chronic intraocular inflammation and corticosteroid
use in treating the inflammation. These cataracts are called
complicated cataracts and pose several challenges to the surgeon at every step. Difficulties start from the preoperative
control of inflammation to intraoperative problems like poor
visibility due to band keratopathy, small pupils, posterior synechiae, pupillary membranes, bleeding from abnormal iris
vessels, and unusual anterior capsules. The postoperative
course can be complicated by recurrence of inflammation,
posterior capsular opacification (PCO) and cystoid macular
edema (CME). Inspite of all these difficulties, modern surgical
techniques, refinement in instrumentation, judicious use of
immunomodulatory therapy in control of inflammation and
newer technologies in intraocular lens (IOL) development
have helped in improving the visual outcome in patients with
cataracta complicata. In this review, we discuss the basic principles that we believe should be followed in taking care of patients with uveitic cataracts and also review the current
literature on this subject.

This is the first step in managing patients with uveitic cataract. This involves arriving at an etiological diagnosis of uveitis, selecting appropriate patients who will be benefited
from cataract surgery, giving a judicious prognosis and strict
preoperative control of inflammation.

Systemic examination and laboratory evaluation


A relevant history, detailed ophthalmic and systemic evaluation and review of medical systems along with laboratory
testing often gives a clue to the etiology of uveitis. Eighty
three percent of patients with a confirmed diagnosis of
uveitis have been shown to have an associated systemic disease.17 This is an important step, because the etiology and
type of uveitis is a guide to preoperative control of inflammation, disease course, treatment response and rate of postoperative complications. Cataracts associated with Fuchs
heterochromic iridocyclitis have good results and better
visual prognosis as compared to other types of anterior

Received 20 September 2011; accepted 18 October 2011; available online 29 October 2011.
Massachusetts Eye Research and Surgery Institution (MERSI), United States
Corresponding author. Address: Massachusetts Eye Research and Surgery Institution (MERSI), 5 Cambridge Center, 8th floor, Cambridge, MA 02142,
United States. Tel.: +1 617 621 6377; fax: +1 617 621 2953.
e-mail address: sfoster@mersi.com (C.S. Foster).
Peer review under responsibility
of Saudi Ophthalmological Society,
King Saud University

Production and hosting by Elsevier


Access this article online: www.saudiophthaljournal.com
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56
uveitis as shown by Tejwani et al. who studied the outcomes
of cataract surgery in these patients and found that more
than 80% of patients had 20/40 or better visual acuity and
no significant postoperative inflammation.42 The prognosis
is different in case of Juvenile idiopathic arthritis (JIA)
associated uveitis. BenEzra and Cohen have shown guarded
prognosis of cataract surgery in JIA associated uveitis due
to increased postoperative inflammation and increased incidence of irreversible amblyopia.10 However recent studies
have shown that patients with JIA associated uveitis undergoing cataract surgery had improved visual acuities and
tolerated IOL implantation provided that they had a strict
preoperative and postoperative control of inflammation
which may require immunomodulatory therapy.28,34,43 Cataract surgery in uveitis associated with Behcets disease has
a guarded prognosis as studied by Berker et al. who found
that 20/40 or better visual acuity was achieved in only 45%
of patients undergoing cataract surgery. The most common
cause of decreased vision in these patients was postoperative
macular edema occurring due to severe inflammation and
preexisting pathologies like optic atrophy and epiretinal
membrane.11
Apart from these autoimmune conditions, infectious
causes of uveitis like syphilis, toxoplasmosis, tuberculosis
and viral infections should always be kept in mind and treated
accordingly. Reactivation of toxoplasmosis after cataract surgery has been seen in a significant number of patients in a
study emphasizing the importance of preoperative diagnosis
and treatment with antiparasitic agents.12

Ophthalmic examination
A complete ophthalmic examination is necessary besides
complete systemic examination to rule out associated pathologies in patients with history of uveitis. Uveitis is associated
with various complications like glaucoma, CME, retinal neovascularization, optic atrophy, macular scarring. It is important to diagnose these conditions preoperatively for
appropriate management and for cautious prognostication
of a case. In many of these cases, the view to the posterior
segment is not adequate. Appropriate investigations should
be used wherever necessary for accurate diagnosis and a
careful prognosis in such patients. Techniques like fluorescein
angiography (FA) and optical coherence tomography (OCT)
can detect macular edema while OCT can also detect macular atrophy, epiretinal membranes and vitreomacular traction.
B-scan ultrasonography is a useful tool in assessment of the
posterior segment for vitreous opacities, retinal detachment
and choroidal thickening/detachment. Potential acuity meter
and laser interferometer can be helpful to assess visual potential. A complete ophthalmic examination will help us decide the visual potential and amount of vision loss
attributed by cataract, on which are based the prognosis
and visual outcome of the surgery.

Indications of cataract surgery


Cataract surgery in uveitic patients is not free of risk and
so the decision to operate should be made thoughtfully.
Various indications for cataract surgery in patients with uveitis
include: (1) Phacoantigenic uveitis (active inflammation due
to leakage of lens proteins) where cataract removal is

U. Baheti et al.
obligatory. (2) Visually significant cataract provided that
preoperative inflammation is controlled and there is hope
of improvement in visual acuity after cataract surgery. (3) Cataract that hampers the assessment of fundus in patients with
suspected posterior segment pathologies. (4) Cataract that
obscures adequate visualization of the posterior segment
where posterior segment surgery is indicated.39

Preoperative control of inflammation


This is the most important step in management of patients
with uveitic cataracts. It is generally accepted that the eye
should be quiescent and free of inflammation for at least
3 months before surgery. Many surgeons advocate use of
topical and systemic preoperative corticosteroids to control
inflammation. Corticosteroids have been used with great
benefit for control of uveitis for 60 years. They can be administered locally or systemically and are effective in controlling
inflammation, but long term treatment with systemic corticosteroids has guaranteed side effects and can vary from
weight gain, acne, anxiety, mood changes to more serious
complications like aseptic necrosis of head of the femur, pancreatitis, suppression of adrenals, hypertension, diabetes
mellitus and psychosis. Children below the age of 15 years
are especially prone for growth retardation.32 To combat
the side effects of long term corticosteroids, the role of
immunomodulatory drugs for controlling inflammation has
gained favor. They are especially useful when inflammation
is not controlled even after high dose of corticosteroid for
1 month, control of inflammation requires more than 10 mg
of steroid daily or development of potential side effects
which require stopping or tapering of steroids.23
Recent evidence suggests that the use of immunomodulatory drugs in controlling inflammation has significantly improved the outcome in cataract surgery in uveitis. Our
retrospective case series of cataract surgery in 34 children
with uveitis showed improvement in best corrected visual
acuity (BCVA) post cataract surgery in 85% of our patients.
In this study, 25 of 34 patients had received immunomodulatory therapy for control of inflammation prior to surgery and
we noted better vision in patients who received immunomodulatory therapy, though this difference was not statistically
significant.34 Zaborowski et al. have shared their experience
in treating nine eyes of 6 pediatric patients with uveitic cataract out of which three had JIA associated uveitis. All the patients were treated with cataract extraction and intraocular
lens implantation. Immunomodulatory therapy in the form
of methotrexate was given to 5 of 6 patients preoperatively
as well as postoperatively while 3 patients also received infliximab post operatively and 1 patient received additional
mycophenolate mofetil. They have reported up to 20/30 or
better visual acuity in all nine eyes.44 These above studies
show that adequate preoperative and postoperative inflammation control with use of immunomodulatory drugs wherever necessary leads to better visual outcomes even in
difficult cases of JIA associated uveitis.
There are many different immunomodulatory drugs available, but there are no set guidelines for the use of these
drugs in the control of intraocular inflammation. Immunomodulatory drugs are not free from possible side effects.
Galor et al. compared three immunomodulatory drugs
namely mycophenolate, azathioprine and methotrexate,

57

Cataract surgery in patients with history of uveitis


which were used in treating ocular inflammatory disorders in
a retrospective study of 257 patients. Out of these three,
mycophenolate achieved faster control of inflammation,
while azathioprine had more side effects and discontinuation
rates due to side effects.20 Increased risk of malignancy is a
worrisome complication associated with immunomodulatory
drugs, although a retrospective study done to evaluate the
risk of overall and cancer related mortality in the patients
receiving immunosuppressive therapy for ocular inflammatory diseases showed no statistically significant difference between the patients treated with immunosuppressive drugs
and those not exposed to them. Tumor necrosis factor inhibitors were associated with significantly increased overall and
cancer related mortality, and alkylating agents were also
associated with an increase in cancer mortality, but this was
not statistically significant.24 The choice of immunomodulatory drugs depends on many different factors such as tolerance of the drug by the patient, control of inflammation,
route of administration, side effect profile, general systemic
condition of the patient, and cost of the treatment. The basic
aim should be intolerance to preoperative and postoperative
inflammation with a careful clinical and laboratory assessment
for side effects.

Surgical techniques
The surgeon may face various challenges while operating
on a cataract in a patient with a history of uveitis because
of miotic pupil, posterior synechiae, pupillary membranes,
calcified anterior capsules and poor visualization due to band
keratopathy.

Extracapsular cataract extraction (ECCE) vs


phacoemulsification
Phacoemulsification with intraocular lens is the preferred
technique by most surgeons. Studies have compared phacoemulsification with ECCE and have shown that although postoperative visual acuities did not differ in the two techniques,
there was a decreased incidence of clinically significant
inflammation requiring treatment in phacoemulsification
(41%) as compared to extracapsular extraction (51%) as well
as a decreased rate of postoperative CME, epiretinal membrane formation and posterior synechiae formation with
phacoemulsification technique.16,19,36 This is probably attributed to the decreased surgical time and minimal tissue handling in phacoemulsification technique.

Pars plana vitrectomy


Cataract extraction and IOL placement has shown improved visual acuities in most of the cases of uveitic cataracts;
however those with coexisting posterior segment pathology
and significant vitreous opacities may require additional procedure. Androudi et al. has described combined pars plana
vitrectomy with phacoemulsification in 34 patients with cataract and posterior segment involvement secondary to chronic
uveitis. Out of 34 patients, 22 patients had IOL implantation
and 12 were left aphakic. Post operative visual acuity was improved in 72.2% of patients. The most common cause of decreased postoperative visual acuity was macular edema. They
also reported complications like IOL dislocation requiring

repositioning and intolerance leading to explantation of


IOL.6 Similarly Soheilian et al. in their series of 19 cases treated
with 25 gauge vitrectomy and phacoemulsification with IOL
placement have reported improved visual acuity in 63.2%
patients and found that macular edema and glaucoma were
the common causes of postoperative decreased vision.40
Although combined procedure seems feasible in cases with
associated posterior segment pathology and significant vitreous opacities, it is not free of complications and has to be
tailored according to the patient needs and surgeon comfort.

Surgery in pediatric cases


Most authors have described good outcomes with cataract
extraction and IOL placement in adult population of uveitic
cataracts; however the scenario is more challenging in
children. In young children, the surgery is technically difficult
with increased incidence of postoperative inflammation;
moreover issues of postoperative amblyopia therapy and visual rehabilitation make it more challenging. Earlier reports
suggested lensectomy and pars plana vitrectomy or extracapsular cataract extraction with aphakia to be a safe technique in
managing pediatric uveitic cataracts.30 Recently, most authors
agree with the idea of IOL placement in pediatric complicated
cataracts which was previously thought to be a contraindication.18,27,33,30 BenEzera and Cohen in their retrospective review of 10 eyes of 10 children, out of which five had JIA
associated uveitis have found that the children with JIA associated uveitic cataracts had extended period of postoperative
inflammation with tendency for secondary membrane formation, necessitating second surgical intervention. They also
found that IOL placement was preferable to correction with
contact lenses, as there was poor tolerance to contact lenses
leading to discontinuation and resultant amblyopia especially
with monocular surgery.10 In our series of 34 children of which
13 received an IOL, we noted improvement in 92% of patients
post IOL implantation. IOL was well tolerated in most of the
patients with optimal vision, however JIA associated patients
were the most difficult cases and most of these children did
not achieve improvement in visual acuity. These patients had
a greater tendency to mount postoperative inflammation
and so required to be treated aggressively with immunosuppressive therapy.34 Nemet et al. in a multicentre study of 18 patients revealed no significant difference in the postoperative
course and complication rate in children with and without JIA
associated uveitis. Lam et al. have reported favorable outcomes in 5 patients with JIA associated uveitis undergoing cataract extraction with IOL placement.26
All the above studies have shown that IOL placement is no
longer a contraindication for pediatric cataracts associated
with uveitis and moreover leads to improved visual function
in most of the patients, provided that there is strict control
of preoperative and postoperative inflammation with the
use of immunomodulatory therapy, wherever necessary. Despite this, we should be aware of the fact that the mere presence of an IOL can act as a trigger for persistent inflammation
and can serve as a scaffold for development of inflammatory
membranes and cyclitic membrane leading to hypotony and
may require IOL explantation to halt further damage.22 Adan
et al. have reported two cases of JIA associated uveitic cataracts that required IOL explantation due to severe damage to
macula because of persistent and uncontrolled inflammation

58
post cataract surgery and IOL implantation.3 The issue of use
of IOL in JIA associated uveitis remains controversial, but if
IOL placement is considered, it certainly warrants careful
monitoring for potential side effects and timely intense intervention to treat them.

Intraocular lens biocompatibility and designs


Intra ocular lens (IOL) biocompatibility depends on the
characteristics of the IOL material, design of the lens, surface
properties and its resistance to degradation. Host reactions
to the IOL material, especially in the uveitic eyes, where there
is inherent loss of blood aqueous barrier are equally important. Many authors have studied and compared different
IOL materials in their search for the best material and design
for the uveitic eyes. In a multicentre study, Alio et al. has
compared different IOL materials after phacoemulsification
and lens implantation in 140 uveitic eyes. The different materials that they compared were hydrophobic acrylic, silicone,
poly methyl methacrylate (PMMA), and heparin-surface-modified PMMA (HSM PMMA). They found that the acrylic lens
group had the lowest inflammation and relapse rate at 3 year
follow up among all lenses. They also noticed that posterior
capsule opacification was a significant problem in silicone
lenses as compared to acrylic lenses.4 Similar findings were
noted by Papiliodis et al. who found that acrylic lenses fared
best in terms of post operative inflammation, PCO formation
and need for explantation.31 Roseal et al. compared the two
acrylic materials hydrophobic AcrySof and the hydrophilic
Akreos sharp-edged intraocular lenses in patients with uveitis. They found no significant difference between the two
lens designs in terms of postoperative inflammation and posterior capsular opacification.37 Recently, Abela-Formanek
et al. have compared five different types of IOL materials
consisting of hydrophilic acrylic, hydrophobic acrylic, silicone
and hydrophilic acrylic with heparin coating. After a follow up
for 7 years, they found that the hydrophilic acrylic group had
better uveal biocompatibility in uveitic patients as compared
to other materials but a slight increased propensity to PCO
formation as compared to hydrophobic acrylic lenses.2 This
finding is consistent with their previous report of comparison
of hydrophobic acrylic, hydrophilic acrylic and silicone IOL
with control group on a shorter follow up for 6 months. They
also noticed that sharp edge lenses have less chance of PCO
formation than the round edge lenses.1 All the above studies
indicate that acrylic lenses by the virtue of their uveal biocompatibility should serve best in treating patients with uveitic
cataracts.

Postoperative complications
The story does not end with the cataract extraction and
IOL placement in uveitic patients as the surgeon is most likely
to be confronted with many different complications postoperatively which may include persistent inflammation, posterior capsule opacification, posterior synechiae and macular
edema.

Postoperative inflammation
Postoperative inflammation can be treated with topical or
oral corticosteroids and sometimes may require immuno-

U. Baheti et al.
modulatory therapy. The key features to decrease postoperative inflammation are preoperative control of inflammation
for at least 3 months, minimal manipulation during surgery
and in bag placement of intraocular lens. Intravitreal triamcinolone injection at the time of phacoemulsification has been
described by some authors to decrease the postoperative
inflammation and also as a substitute for systemic steroids,
but increase in intraocular pressure is a concern with this
procedure.5,15

Posterior capsule opacification


PCO formation is one of the common postoperative
complications in uveitic cataracts. Its incidence ranges from
23% to 96% as seen in different studies.16,25,29,35,36 PCO, if
visually disturbing often can be treated with Nd YAG laser
capsulotomy once inflammation subsides. Commonly, there
can be pigment deposition on the IOL, which may be visually
disturbing and needs laser polishing of IOL. In cases, such as
JIA associated uveitis, severe inflammatory membrane can
develop over the IOL as the lens acts as a scaffold for the formation of the inflammatory membrane. These membranes
may need repeated Nd YAG laser disruption or at times
may need surgical membranectomy.

Cystoid macular edema


Cystoid macular edema is another sight threatening complication of uveitic cataract surgery. Its incidence ranges between 21% and 50%.16,21,35,36 Belair et al. used optical
coherence tomography to determine the incidence of CME
after cataract surgery in patients with and without uveitis.
At 3 months follow up, the incidence of CME was more in
uveitic cataracts as compared to non uveitic cataracts; however a statistically significant decrease in occurrence of macular edema was noted in patients whose inflammation was
controlled 3 months prior to surgery and in those who were
preoperatively treated with oral corticosteroids.9 Initial treatment for post cataract surgery CME includes topical nonsteroidal anti inflammatory drops. Different drug delivery
systems have been tried by various authors to treat CME.
Roesel et al. has compared the effect of intravitreal and orbital floor triamcinolone acetonide on macular edema and
found that intravitreal route was more effective than orbital
floor injection in controlling CME and postoperative inflammation in first 3 weeks after surgery. However increase in intra ocular pressure was a concern in both the groups with 2
patients in intravitreal group needing surgical intervention
to treat glaucoma.38 Androudi et al. retrospectively studied
20 eyes for the safety and efficacy of intravitreal triamcinolone acetonide in treatment of uveitic macular edema. They
reported complete resolution of CME in 50% of patients with
persistence or relapse of macular edema in remaining 50% at
mean follow up of 34 weeks. They noted that raised IOP was
transient and responded to medical treatment.7
The effect of triamcinolone acetonide is transient and
macular edema may relapse after the effect of drug wears
off. A sustained drug delivery implant could provide long
term effect. Chieh et al. reviewed 24 eyes of 21 patients,
who underwent a combined phacoemulsification with IOL
implantation and a 3 year sustained drug delivery fluocinolone acetonide implant in vitreous cavity for patients with

59

Cataract surgery in patients with history of uveitis


non infectious posterior uveitis or intermediate uveitis with or
without iridocyclitis. They reported improvement in visual
acuity, decreased rate of recurrence of inflammation and
need for systemic immunosuppression with no intraoperative
complications. The most common complication was raised
IOP and greater than 10 mm Hg increase in IOP was noted
in 21% of patients with 15% requiring glaucoma filtering surgery.14 In the quest of different drugs to treat uveitic macular
edema, Willaims et al. studied the effect of surgical placement of intravitreal dexamethasone implant. Patients were
randomly assigned to receive 350 lg dexamethasone implant, 700 lg dexamethasone implant and observation. Improved visual acuity and decrease in leakage on fluorescein
angiography was observed in both the treatment group,
but raised IOP was a significant problem in most of the
patients.43
As elevated IOP is a potential sight threatening problem
with intraocular steroids, recently intravitreal vascular endothelial growth factor (VEGF) inhibitors have been gaining favor in treatment of uveitic CME. Our retrospective analysis
included 29 eyes of 27 patients with refractory uveitic CME
of various etiologies of which 13 patients received single
intravitreal bevacizumab injection, 6 patients required a repeat intravitreal bevacizumab while 10 patients received a
combination of intravitreal bevacizumab and triamcinolone
acetonide. We found significant improvement in visual acuity
and decreased macular thickness at 1 year follow up.13 Soheilian et al. compared intravitreal bevacizumab and intravitreal
triamcinolone in treatment of refractory uveitic CME in a prospective randomized study and found that both the drugs
were comparable in terms of improved visual acuity; however
visual acuity improvement was more in triamcinolone acetonide group when the factor of corticosteroid induced cataract was removed. Moreover significant macular thickness
reduction was achieved by the triamcinolone acetonide
group.41 Similar findings were noted by Bae et al. who
compared intravitreal bevacizumab (IVB), intravitreal triamcinolone acetonide (IVTA) and posterior subtenons triamcinolone acetonide (PSTA) in refractory uveitic macular edema.
They reported better results with IVTA than those with IVB
or PSTA, although the difference did not reach statistical significance. They noted that the effect of drug lasted for a period of 16 weeks with IVB, 30 weeks with IVTA, and 12 weeks
with PSTA. Increased IOP was a significant problem in steroid
group and (0.5 mmHg greater than baseline) was observed in
5 eyes (45.5%) with IVTA, and 4 eyes (40%) with PSTA as compared to1 eye (10%) with IVB.8 Intravitreal VEGF inhibitor
drugs seem to be a good option in patients at risk of raised
IOP due to corticosteroid response, however repeated injections may be required. Post operative CME is the most
dreaded complication of uveitic cataract surgery and so it
goes without saying that preoperative and post operative
control of inflammation, early detection of CME and aggressive management may be helpful in avoiding permanent
damage.

Conclusion
Cataract surgery in uveitic patients requires extensive
diagnostic evaluation to determine the cause of uveitis, proper patient selection, zero tolerance to preoperative inflammation, vigilant use of immunomodulatory drugs wherever

necessary to control preoperative inflammation, meticulous


surgery, thoughtful decision for implantation of IOL, control
of postoperative inflammation and early detection and
aggressive management of complications. Increasing use of
immunomodulatory drugs has improved the outcomes in
pediatric cases with JIA associated uveitis which however still
remains a challenge to the ophthalmologist and appropriate
decision to the type of surgery and placement of IOL needs
to be done according to the situation.
Further improvement in modes of drug delivery, surgical
techniques and refinement of IOL designs will keep influencing the outcomes in cataracts associated with uveitis.

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