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Background
The existence of congenital cataracts has been known for more than
200 years. Documented by Duke-Elder, several subtypes are
described; however, a posterior polar cataract is distinct from other
forms of congenital lens opacities.[1]
A posterior polar cataract is a round, discoid, opaque mass that is
composed of malformed and distorted lens fibers located in the
central posterior part of the lens. This location is its most significant
feature, in addition to its proximity to and possible adherence with
the posterior capsule. Moreover, the capsule itself may be weakened.
As such, posterior polar cataract removal is a challenge to the
surgeon because of its adherence to or the associated weakness of the
posterior capsule.
Posterior polar cataract, a distinctive subtype of lens opacity, presents
as an area of degenerative and malformed lens fibers that form an
opacity in the central posterior subcapsular area of the lens. Often,
this opacity is adherent to the lens capsule, thereby making
uncomplicated surgical removal problematic.
Posterior polar cataract surgery is associated with an increased
incidence of rupture of the posterior capsule. Only in recent times
have the advances in control of fluidics and aspiration during
phacoemulsification prompted various approaches to meet this
surgical challenge.
History of the Procedure
Duke-Elder discusses the historical methods of congenital cataract
extraction used prior to the modern introduction of
phacofluidics.[1] He also mentions discission, linear extraction (which
would now be described as irrigation/aspiration), as well as
intracapsular cataract extraction.[1]
In addition, Duke-Elder discusses the association of "irregularities in
the development of lens fibers" and attributes this concept to von
Szily in 1938.[1]
Further, histologic examination has found this to be the case in
posterior polar cataracts.[2, 3, 4] Moreover, genetic analysis using
polymerase chain reactionbased tests have shown that this
autosomal dominant anomaly has been associated with a recurrent 17
bp duplication on the 3(PITX3) gene on chromosome 10.[5]
The histologic changes of the malformed lens fibers are now
recognized to be associated with an adherence of these degenerative
lens fibers to an area of the weakened posterior capsule.
Therefore, the surgical approach must be aimed at reducing the stress
on the weakened posterior capsular. For this reason, various
approaches have been suggested, including limited hydrodissection,
viscodissection, and posterior capsulorrhexis. Still, the reported rate
of capsular rupture in the procedure is too high, sometimes 25% or
more.
Problem
The surgical significance of a posterior subcapsular cataract is an
association with and an adhesion of the lens opacity to the posterior
capsule immediately subjacent to it.
The incidence of capsular tears in this subtype of cataract surgery can
approach an unacceptably high percentage. Some surgeons find that
the tears occur most often during cortical aspiration.
The goal during posterior polar cataract surgery is to remove the
opacity without rupturing the posterior capsule. This can be
accomplished by decreasing stress on the posterior capsule during all
phases of the surgery. Several surgical solutions and approaches have
been proposed, but a technique for uncomplicated and successful
posterior polar cataract extraction continues to remain challenging
and problematic.
Epidemiology
Frequency
The occurrence of posterior polar cataracts is rare; in fact, little is
published on the specific incidence of posterior polar cataracts.
Further, no standardized subtype specific study on this type of
cataract has been conducted. In the Framingham Eye Study, little
prevalence data for posterior polar cataracts are presented. However,
it is well accepted that this is an infrequently encountered form of
cataract and that the prevalence is very low. When seen, it is often as
an inherited trait and is present in several members of the same
family.
The population-based analysis of posterior polar cataract prevalence
has been studied. In summary, 111 cases of posterior polar cataracts
were found against 37,837 population controls without ocular
Aspiration
of
the
subincisional cortex is shown. The dye in the remaining ringlike edge
of the cataract, at the 10-o'clock position, appears to show a small
strand still adhering in the direction of the posterior capsule. A defect
in the capsule has been avoided up to this point.
Finally, once aspiration has been completed, the posterior chamber
intraocular lens can be implanted in the surgeon's usual fashion (see
the image below). When using a viscoelastic, as during the entire
case, care must be taken to avoid overpressurization of the anterior
chamber, creating undue posterior force.
After
careful
cortical
aspiration, a posterior chamber lens is shown. The capsule has
remained intact.
If the capsular opacity resists removal in the above maneuver, the
surgeon may have to resort to a controlled posterior capsulorrhexis
combined with a shallow anterior vitrectomy.
Postoperative Details
The patient should be examined the day after surgery, and the clarity
of the cornea and the depth of the chamber should be assessed. Any
complications should also be addressed.
A topical fourth-generation fluoroquinolone antibiotic as well as a
nonsteroidal anti-inflammatory drug (NSAID) may be instilled.
A refraction is performed 6 weeks postoperatively; at which time, the
eye should be well healed.
Follow-up
Once the eye has stabilized and no problems are present, the patient
can be seen 3 months postoperatively and every 6 months thereafter.
Complications
The complications of wound leak, malpositioned intraocular lenses
(IOLs), andendophthalmitis are well known and do occur in cases of
posterior polar cataract surgery.
Moreover, since this entity occurs in a young age group, refractive
errors tend to increase with age. The choice of IOL power should
take this tendency into account. The necessity of lens exchange in
these patients has not been documented but should be avoided as
much as possible.
Outcome and Prognosis
The patient whose photographs are presented below had a clear
cornea on postoperative day 1. Slit-lamp examination revealed the
eye to be in good pseudophakic order. The patient was given a
topical antibiotic and an NSAID, as described in Postoperative
details.
Aspiration
of
the
subincisional cortex is shown. The dye in the remaining ringlike edge
of the cataract, at the 10-o'clock position, appears to show a small
strand still adhering in the direction of the posterior capsule. A defect
in the capsule has been avoided up to this point.
After
careful
cortical
aspiration, a posterior chamber lens is shown. The capsule has
remained intact.
During the seventh postoperative week, the posterior capsule
opacified rather rapidly, and a YAG laser capsulotomy was
performed. Emmetropia is difficult to achieve in these cases for
several reasons. In this patient, the final refraction was +1.00 -0.50 X
87, and the patient preferred wearing a contact lens. When the patient
reaches the third decade, an excimer laser correction can be
performed if desired.
Without correction, the patient achieves a visual acuity of 20/40 and
often prefers not to wear contacts or spectacles.
Future and Controversies
Posterior polar cataracts have been recognized as a distinctive
subtype for many years. Recent advances in genomics have localized
the chromosomal cause, and further work promises to elucidate the
exact mechanism by which these cataracts form.
Regarding surgery, various approaches have been suggested to
minimize complications, including a bimanual microincisional
approach, the use of viscodissection, a pars plana approach, and
posterior capsulorrhexis. These approaches all address the weakness
of the posterior capsule with its tendency to rupture. Since this
challenge will continue to persist, different surgical approaches will
continue to be offered.
With this in mind, the technique presented herein is offered in the
hope that it leads to fewer cases of capsular rupture.