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MANAGEMENT OF

SUBLUXATED CATARACTS
The surgeon must remain flexible and be prepared to handle a greater degree
of difficulty than was anticipated during preoperative assessment.
BY ARUP BHAUMIK, MD; AND SANTANU MITRA, MBBS, DOMS
Surgical management of ectopia lentis is one of the major
challenges faced by cataract
surgeons today. Ectopia lentis
signifies a displacement or
malposition of the crystalline
lens, irrespective of cause.
It may occur congenitally
or as part of developmental anomalies, as found in Marfan
syndrome, homocystinuria, Ehlers-Danlos syndrome, hyperlysinemia, sulfite oxidase deficiency, simple primary ectopia
lentis, and congenital aniridia syndrome.1,2 Pseudoexfoliation
syndrome (PXF) is probably the most common cause of adultonset zonular dehiscence. Subluxation may also result from
blunt external trauma or iatrogenic zonular dehiscence during
complicated cataract surgery.3,4 This article offers five pearls
for managing these challenging cases.
CLINICAL EVALUATION
Proper evaluation of patient historyincluding family
history, relevant trauma history, and history of onset with
vision-related symptomsis the first step in the clinical evaluation of patients with ectopia lentis. Phacodonesis, an important
sign of subluxation, is best evaluated in undilated or fully dilated pupils. The ophthalmic examination should be comprehensive and assess both the anterior and posterior segments.
Hoffman et al5 classified the degree of subluxation into
three broad groups: (1) minimal to mild subluxation, in
which the lens edge uncovers 0% to 25% of the dilated pupil;
(2) moderate lens subluxation, in which the lens edge uncovers
25% to 50% of the dilated pupil; and (3) severe subluxation, in
which the lens edge uncovers more than 50% of the pupil.
It is best to examine a patient with a severely subluxated
lens in both upright and supine positions. The change in the
position of the lens with different head positions helps to
indicate the severity of subluxation.
The degree of zonular loss may be localized, as in focal trauma
or congenital defects, or there may be an extensive generalized
74 CATARACT & REFRACTIVE SURGERY TODAY EUROPE | APRIL 2015

weakness of zonular fibers, as in Marfan syndrome or PXF. A


round lens edge indicates total loss of zonules or lax zonules.
The already difficult situation of lens subluxation may
become more challenging in the presence of a hard cataract, a
rigid pupil, an iris coloboma, vitreous in the anterior chamber,
anterior subluxation of the lens with a very shallow anterior
chamber, or a very deep anterior chamber as in myopia.
SURGICAL PLANNING
In these types of cases, local anesthesia is preferable over
topical anesthesia. Preoperative intravenous mannitol
(1,000 to 2,000 mg/kg of body weight) can reduce vitreousassociated complications during surgery.
In eyes with ectopia lentis, the phacoemulsification technique used depends on the degree of zonulopathy and
its underlying pathophysiologic origin. When the zonular
abnormality is less than 3 contiguous clock hours, slow and
careful surgery may be sufficient for a successful surgical
outcome, with adjunctive support from capsule retractors.
When the area of zonular dialysis is small, with otherwise
strong zonular attachment elsewhere, as in trauma, a capsular tension ring (CTR) may not be routinely required.
On the other hand, the use of a CTR is mandatory even in
mild cases of zonular laxity that have a progressive pathologic
nature, as in PXF, Weill-Marchesani syndrome, Marfan syndrome,
or retinitis pigmentosa. These are cases in which the zonular
pathology worsens with time, and the CTR can be helpful for
secondary fixation of the bag if necessary. It is wise to implant a
CTR in every case in which zonular disturbance is suspected.
In younger patients with such risk factors, bag fixation to
the sclera with a capsular tension segment (CTS) is advisable
from the beginning. The operating surgeon is the ultimate
judge, but he or she should remain flexible in these cases
and be prepared to handle a greater degree of difficulty than
anticipated during the preoperative assessment.
The remainder of this article focuses on five pearls for this
challenging surgery.
(Continued on page 70)

CATARACT FUNDAMENTALS

(Continued from page 74)


INCISION
The modern cataract surgeon is likely accustomed
to using a clear corneal temporal incision. The
dictum in eyes with lens subluxation is that the main incision
should not lie directly over the area of zonulopathy when possible. The paracentesis wound for a second instrument should
be more corneal to prevent injury to the rhexis margin, which
tends to be slightly anteriorly tented after fixation of iris hooks.
Management of preexisting vitreous prolapse in the anterior chamber must be done at the beginning of surgery. This
can be accomplished with triamcinolone-assisted two-port
anterior vitrectomy. In this situation, the capsular bag must be
supported with hooks, even if the area of subluxation is small.
CAPSULORRHEXIS
The capsulorrhexis is the most challenging step
of cataract surgery in eyes with ectopia lentis
because capsulorrhexis completion and bag preservation are
mandatory for successful phacoemulsification. Both initiation
and completion of the capsulorrhexis are more demanding in
this setting than in standard cases. Making the first puncture
is difficult due to a lack of zonular countertraction and to the
highly elastic capsules seen in comparatively young eyes.
Staining of the capsule with trypan blue dye can aid in this
situation, as the dye reduces the elasticity of the capsule and
makes penetration of the capsule easier.6 Caution is needed
in the event of a loss of the anterior-posterior barrier due
to zonular deficiency, as trypan blue can stain the vitreous,
leading to a loss of the red reflex after simple irrigation of
the dye into the anterior chamber. It is safer to paint a few
drops of dye directly across the anterior capsule under an
ophthalmic viscosurgical device (OVD).
The first capsular puncture can be made with a standard cystotome or a straight 25-gauge needle (Figure 1). If this cannot be
done easily, the crossed-swords capsule pinch approach using
two 180 opposing 30-gauge needle tips can be used to pierce
the capsule and create a starting point for the capsulorrhexis.
In most unstable lenses, microforceps or a hook may be
used to provide countertraction during the continuous tear
of the capsule. This second instrument should be placed at
least 2 to 3 clock hours from the leading edge of the capsulorrhexis to avoid runaway (Figure 2). An eccentric rhexis can be
made according to the shape of an eccentric lens to ultimately
achieve a round, central rhexis. In extreme situations, microincision forceps can be useful to access the capsule from multiple microincision paracenteses around the circumference.
The ideal capsulorrhexis should have at least a 2-mm
margin between the capsulorrhexis edge and the lens
equator to adequately support the CTR, Cionni ring, or
Ahmed CTS (all available from multiple manufacturers) in
the capsular bag.
70 CATARACT & REFRACTIVE SURGERY TODAY EUROPE | APRIL 2015

FUNDAMENTAL NO. 2: FIGURES

Figure 1. Puncture of an elastic anterior capsule with a longtipped cystotome.

Figure 2. Countertraction with a Kuglen hook during


capsulorrhexis creation (A) and countertraction with an iris
hook and centration of a subluxated lens (B).

STABILIZATION OF THE
CAPSULAR BAG
If zonular dysfunction involves a large area, for
instance in the range of 3 to 6 clock hours, then the capsule
must be supported during surgery. This can be done using
flexible iris retractors strategically placed through limbal stab
incisions to hook the capsulorrhexis edge and support the

However, putting the CTR in with the lens still in the bag
is challenging. Sometimes the obstructed progress of the
CTR through the bag may lead to more damage to zonular
fibers,8,9 and a larger area of zonular weakness may result.
Additionally, cleaning of the cortex from the equator can be
hampered by the CTR. Most surgeons would ideally prefer
to follow this rule: Place the CTR as late as you can, but as
soon as you must.5
Segmental designs with eyelets, such as the Cionni or
Ahmed CTS, can be sutured to the sclera to stabilize focal
defects. Alternatively, the CTS can be fixed by passing an iris
hook through an eyelet.
PHACOEMULSIFICATION
The next important phase of surgery is
phacoemulsification of the cataractous lens.
Zonulopathy makes the situation more difficult, as mobilization of the nucleus in the bag is nearly impossible due to
a lack of countertraction. The risk of damaging the bag can

FUNDAMENTAL NO. 3: FIGURES


A

Figure 3. Capsular bag stabilization using multiple capsular


hooks.

Figure 4. Capsular bag stabilization with Mackool capsular


hooks (A) and MicroSurgical Technology capsular hooks (B).

Figure 5. Stabilization of a subluxated lens is accomplished


using an Ahmed CTS by hooking the fixation eyelet with an iris
hook.

Figure 6. Capsular bag stabilization using a capsular hook


placed through a prelimbal incision in a deep anterior chamber.

APRIL 2015 | CATARACT & REFRACTIVE SURGERY TODAY EUROPE 71

CATARACT FUNDAMENTALS

bag (Figure 3).7 The disadvantage of this approach is that the


hook ends can inadvertently tear the rhexis edge. They also
tend to bring the capsular bag into a more anterior plane,
and sometimes in extreme subluxation the lens itself can
be in the anterior chamber. Capsular hooks, therefore, are a
better alternative, as they support the bag by its equator and
keep it distended, reducing the risk of aspiration of the bag
equator (Figure 4).
The capsular bag can be stabilized with an Ahmed CTS by
hooking the fixation eyelet with an iris retractor (Figure 5). In
patients with deep anterior chambers, a capsular hook may
be placed through a prelimbal stab incision to reduce the
chance of the hook slipping (Figure 6).
The best way to stabilize a compromised capsular bag is
to place a CTR; this approach maintains an expanded capsular bag, supports the zonular apparatus, and can facilitate
safe phacoemulsification. The primary question, however,
is when to place the CTR. Some surgeons prefer to put it in
early so that the weak bag is supported from the beginning.

CATARACT FUNDAMENTALS

be reduced with meticulous and repeated hydrodissection


and viscodissection, crucial steps in this procedure. Slowmotion phacoemulsifcation with low parameters is mandatory. In this mostly younger population, cataracts tend to
be soft and easily aspirated with irrigation and aspiration
only. Harder cataracts are more difficult to crack. If chopping can be successfully initiated, removal of the first pie
segment creates more space in the bag, thereby reducing
zonular stress during subsequent chopping.
In our clinic, repeated injection of hydroxypropyl methylcellulose in the bag and anterior chamber is facilitated with
the use of the Visco pump (available in India). This simple,
motorized, footpedal-controlled device can be used to
continuously inject OVD through a second sideport at the
desired site with an irrigating cannula (Figure 7).

FUNDAMENTAL NO. 5: FIGURES

Figure 8. CTR insertion with an injector, maintaining the arc of


the capsular bag.

LONG-TERM BAG FIXATION


Available tools for bag fixation include standard CTRs, the Cionni ring, and the Ahmed
CTS. The process of selecting which tool to use is ruled
by the degree of zonular damage and its chance of progression.

FUNDAMENTAL NO. 4: FIGURE

Figure 9. Implantation of an Ahmed CTS at the capsular bag


equator with a hook.

Figure 7. Injection of an OVD with a cannula placed through a


second sideport incision.

In the case of a small area of dialysis, a standard CTR


will suffice, inserted manually or with an injector system
with the vector forces directed toward the area of zonular
weakness (Figure 8). If the damage is larger than 4 clock
hours, with a grossly decentered bag, a standard CTR may
be augmented with a CTS. This combination of a standard CTR with a CTS is likely a better option than using a
Cionni ring because of their ease of insertion and fixation
with less zonular stress involved. A Cionni ring should be
72 CATARACT & REFRACTIVE SURGERY TODAY EUROPE | APRIL 2015

Figure 10. A Cionni ring or Ahmed CTS can be fixed to the


sclera by taking multiple suture bites without a flap or knot.

the primary choice only if the capsulorrhexis is larger than


planned.5
For long-term fixation of the bag, additional CTS anchorage
over a standard CTR is necessary.
Placement of a Cionni ring, which is done in a clockwise
direction, is more technically challenging. Preloading the
leading eyelet with a 10-0 nylon suture makes placement
around the first few clock hours much easier, adding ten-

AT A GLANCE
Ectopia lentis signifies a displacement or malposition
of the crystalline lens.
Pseudoexfoliation syndrome is probably the most
common cause of adult-onset zonular dehiscence.
In patients with subluxated lenses, a comprehensive
ophthalmic examination should assess both the
anterior and posterior segments.
In ectopia lentis, the phacoemulsifcation technique
used depends on the degree of zonulopathy and its
underlying pathophysiologic origin.

sion to the leading suture to reduce the arc of curvature


and prevent the device from hanging up in the capsular
fornix.
An Ahmed CTS can be placed directly into the bag fornix in the desired clock hour (Figure 9). The fixation device
should overhang the capsular margin anteriorly and be
placed directly over the area of greatest weakness. The
sutures may be placed ab interno through the scleral wall or
exteriorized by making scleral pockets. Some surgeons prefer
to use an ab externo technique. A temporary knot is made,
and, after making the eyeball tight with an OVD, suture tension is further titrated to achieve maximum IOL centration.
The final knot can be buried under a corneoscleral pocket or
scleral flap. The suture can be fixed to the sclera by multiple
horizontal bites without a knot (Figure 10).
Suture material with a more permanent nature is preferable for fixation. Whereas 10-0 polypropylene has a

WATCH IT NOW
Watch a series of pearls for managing ectopia lentis, one
of the major challenges faced by cataract surgeons.

tendency to hydrolyze in 5 to 10 years and, thus, should be


avoided,10 9-0 polypropylene has a longer survival record.
However, the exact time interval of degradation is yet to
be documented. Polytetrafluoroethylene (Gore-Tex CV-8;
W.L. Gore and Associates) sutures have excellent longevity.
Some surgeons prefer to suture a Cionni ring or Ahmed
CTS to the sclera after IOL implantation to achieve better
centration.
FINAL THOUGHTS
IOL selection will depend on how successfully the surgery is
completed. If everything goes as planned, no rhexis damage or
posterior capsular rupture occurs, and the bag is fixated centrally
with a CTR or CTS, then in-the-bag implantation of a one- or
three-piece foldable IOL can be performed. In the case of an
absolutely centrally fixated bag, a multifocal or toric IOL may be
considered. In eyes with PXF, the IOL may be implanted in the
sulcus with the optic captured in the anterior capsulorrhexis.
If damage to the rhexis or a posterior capsular rent occurs,
the use of a CTR or CTS should be avoided. These are
extreme situations, and IOL selection must be modified, as
anterior chamber, scleral- or iris-fixated, or glued IOLs are
then the only appropriate options.
For a video demonstration of management of subluxated
cataracts, visit http://eyetube.net/?v=apodi. n
1. Merriam JC, Zheng L. Iris hooks for phacoemulsification of the subluxated lens. J Cataract Refract Surg. 1997;23:1295-1297.
2. Santoro S, Sannace C, Cascella MC, Lavermicocca N. Subluxated lens: phacoemulsification with iris hooks. J Cataract
Refract Surg. 2003;29:2269-2273.
3. Ton Y, Michaeli A, Assia EI. Repositioning and scleral fixation of the subluxated lens capsule using an intraocular anchoring device in experimental models. J Cataract Refract Surg. 2007;33:692-696.
4. Cionni RJ, Osher RH. Endocapsular ring approach to the subluxed cataractous lens. J Cataract Refract Surg. 1995;21:245-249.
5. Hoffman RS, Snyder ME, Devgan U, Allen QB, Yeoh R, Braga-Mele R. Management of the subluxated crystalline lens. J
Cataract Refract Surg. 2013;39:1904-1915.
6. Cionni RJ, Osher RH, Marques DMV, Marques FF, Snyder ME, Shapiro S. Modified capsular tension ring for patients with
congenital loss of zonular support. J Cataract Refract Surg. 2003;29:1668-1673.
7. Vasavada V, Vasavada VA, Hoffman RO, Spencer TS, Kumar RV, Crandall AS. Intraoperative performance and postoperative outcomes of endocapsular ring implantation in pediatric eyes. J Cataract Refract Surg. 2008;34:1499-1508.
8. Ahmed II, Cionni RJ, Kranemann C, Crandall AS. Optimal timing of capsular tension ring implantation: Miyake-Apple
video analysis. J Cataract Refract Surg. 2005;31:1809-1813.
9. Dietlein TS, Jacobi PC, Konen W, Krieglstein GK. Complications of endocapsular tension ring implantation in a child with
Marfans syndrome. J Cataract Refract Surg. 2000;26:937-940.
10. Price MO, Price FW Jr, Werner L, Berlie C, Mamalis N. Late dislocation of scleral-sutured posterior chamber intraocular
lenses. J Cataract Refract Surg. 2005;31:1320-1326.

Arup Bhaumik, MD

Senior Consultant, Department of Cataract, Cornea and


Refractive Services, Disha Eye Hospitals and Research
Centre, West Bengal, India
na
 rupbhaumik_cal@yahoo.co.in
nF
 inancial disclosure: Inventor (Visco pump)
n

Santanu Mitra, MBBS, DOMS

Senior Consultant, Department of Cataract, Orbit &


Oculoplasty, Disha Eye Hospitals, Barrackpore,
West Bengal, India
ns
 antanu_mitra60@yahoo.co.in
nF
 inancial disclosure: None
n

APRIL 2015 | CATARACT & REFRACTIVE SURGERY TODAY EUROPE 73

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