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Posterior Polar Cataract

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

defects. Even among congenital cataracts, posterior polar cataracts


are rare. The results of the study concluded that isolated congenital
cataracts were preventable by maternal rubella and influenza
vaccination.[6]
Other population studies have not focused on the incidence of
posterior polar cataracts. Most studies based data on the incidence of
all types of cataracts, which were part of the focus of the
Framingham Eye Study, but found mainly age-related cataracts,
although posterior subcapsular opacities were reported to occur in the
younger subjects of the group studies. The Framingham Eye Study
did not specifically address the incidence of posterior polar cataracts
as a separate group.[7] Other studies focused on incidence and
etiology and nutrition.[8, 9]
Etiology
A posterior subcapsular cataract is a distinctive, often very sharply
demarcated opacity, discoid in shape and adjacent and merging with
the posterior capsule. This cataract represents degenerative lens
fibers that have developed abnormally. The lens fibers are
surrounded by globular structures that contain a highly refractile
material. A weakness of and adhesion to the posterior capsule
immediately subjacent to the area of cataract is also associated with
this type of cataract.
The etiology of this congenital condition has been noted for many
years. For some time, it has been recognized that posterior polar
cataracts seemed to follow an autosomal dominant inheritance pattern
with an occasional sporadic mutation. The direct cause of the
lenticular fiber malformation during lens development has not been
well understood.[10, 11, 12]
Genetic analysis by Addison and colleagues, as well as by Burdon
and colleagues, however, has focused attention on a mutation on
the PITX3 gene.[13, 14] This gene was found to control the
development of the anterior segment of the eye. The duplication of
the 17 base pairs in this gene apparently alters the normal
development of the anterior segment, leading to a posterior polar
cataract. The exact mechanism through which this pathology is
produced remains unclear.
Recent advances in genomics techniques, such as single-nucleotide
polymorphism (SNP) and microsatellite markers, have led to an
increased understanding of the mechanism. A specific example,
relating to the formation of posterior poplar cataract, is detailed in a
report from Berry et al.[15] This report describes a pedigree,
comprising 4 generations of a family, with more than 6 members
diagnosed with posterior polar cataract after full eye examinations at
the Moorefields Eye Hospital cataract clinic. Further, a genomewide
linkage was then performed by single-nucleotide polymorphism and
microsatellite markers. An autosomal dominant base pair deletion
(542delC) mutation was noted in the homeobox of gene PITX3 in
10q. This is one of the transcription factors critical for normal lens
induction and development. The authors found this base pair to cosegregate with all the affected family members with posterior polar
cataract.
Because the understanding of posterior polar cataract, an autosomal
dominant condition, is continually expanding, genetic counseling for
the parents remains very important.
Pathophysiology
A posterior polar cataract has an autosomal dominant inheritance
pattern. However, it has also been noted as a result of gene
mutation.[13] The proximal cause of the abnormality is expressed as
an abnormality in lens development, specifically in the lens fibers
that fail to develop normally and form an opacity close to and
sometimes adherent to the posterior capsule.
Maldevelopment of the lens fibers produces an opacity composed of
disorganized, misshapen, and malformed lens fibers and vacuoles in
the region of the posterior polar capsule. At times, this discoid
opacity can actually adhere to the posterior capsule. The remainder of
the lens can appear normal, and the pathology is limited to the
specific area mentioned above. The genomic etiology has been
localized to the chromosomes mentioned above. Its challenge as a
surgical problem remains significant.
Presentation
Posterior polar cataract presents as a distinctive discoid lens opacity
situated posteriorly, adjacent to the posterior capsule. Duke-Elder
mentions stationary as well as progressive cataracts.[1]
Posterior polar cataract is easily seen and often clearly delineated. In
its early stages, it can be seen interfering with the normal light reflex.

Fully formed, it presents as a dense, circular plaque in the central


posterior part of the lens. It can be surrounded by vacuoles and
smaller areas of degenerated lens material. Often, the only benefit to
the surgeon is that the defect is so clearly visible.
This entity is rare and can be seen to progress from its initial
discovery. Surgical therapy is indicated once visual acuity is affected.
Further, because of its mode of inheritance, ophthalmic examinations
should be scheduled for family members.
Indications
Once visual acuity is diminished, amblyopia can develop and cataract
removal is indicated. Since this type of lens opacity usually occurs
directly in the visual axis, it does diminish vision more rapidly as
compared to lens opacities that are located more peripherally.
Moreover, since posterior polar cataracts can occur in a younger age
group, the possibility of amblyopia must be considered. The case for
early intervention is made on the basis that visual acuity impairment
can lead to amblyopia.
The Lens Opacities Classification System (LOCS) that indicates the
significance of the lens opacity is not clearly applicable here. A
separate posterior polar cataract classification system has yet to be
formally introduced.
The indication for posterior capsular cataract removal is often only a
matter of time. Certainly, a visual acuity that is worse than 20/40 is a
valid indication. While the patient can make the choice for surgery
when the vision impairs daily activity, it is the surgeon's judgment
that should determine the time of intervention.
Since posterior polar cataracts can present in the first several months
of life, surgical intervention is indicated in the presence of a visually
significant opacity. The suspicion of amblyopia predisposes to very
early removal of the lens opacity. This is especially true if an
unexplained decrease in visual acuity occurs without a concurrent
change in the posterior polar cataract.
Relevant Anatomy
A posterior polar cataract consists of dysplastic lens fibers, which, in
their migration posteriorly from the lens equator, exhibit progressive
lens opacity with the formation of a characteristic discoid posterior
polar plaquelike cataract.
Histology has shown that posterior polar cataracts consist of lens
fibers that have developed abnormally. Further, the lens fibrils have
degenerated and, in some cases, are directly attached to the posterior
capsule. Often, the posterior polar opacified plaque is surrounded by
a less distinct area of fibrillar degeneration as well as vacuoles filled
with liquefied material.
The current literature points to a genomic cause of this entity. Several
chromosomal abnormalities, the majority of which appear to exhibit
an autosomal dominant inheritance pattern, are present in a familial
distribution in posterior polar cataract cases. Because these cataracts
appear to occur in familial groups, the focus has narrowed in on
several specific chromosomes. These genomic aspects are also
discussed in Etiology. Future work may shed light on the more
detailed genomic defect of this subtype of lens opacity.
Contraindications
Contraindications include any concurrent medical condition that
precludes cataract surgery. A complete ocular history and a family
history must be obtained. After a full ophthalmic examination, any
concurrent ocular pathology must be stabilized prior to surgical
intervention.
Ocular
conditions,
such
as retinal
detachment or glaucoma, as well as infections of any type must be
treated. Once the patient is stabilized, surgery can then proceed.
The surgeon should use the same criteria to determine if surgery is
appropriate in a posterior polar cataract case as that for a routine
case.
Laboratory Studies
Apart from a complete blood count (including hemoglobin and
hematocrit), no laboratory examinations have been shown to be
necessary prior to posterior polar cataract surgery.
Imaging Studies
A chest radiograph may be obtained prior to surgery.
Other Tests
A-scan ultrasonography should be performed.
Although routinely done prior to almost all preoperative workups,
keratometry readings should be performed to aid in intraocular lens
(IOL) calculations.

Electrocardiography should be performed to rule out any associated


disorders, which have been mentioned but often do not contraindicate
posterior polar cataract surgery.
Diagnostic Procedures
The diagnosis of a posterior polar cataract is self-evident on slit-lamp
examination and does not require special diagnostic procedures
beyond a full ophthalmic examination. Slit-lamp examination and
pupillary retroillumination allow a good evaluation of the visual
significance of the opacity. The anterior vitreous should be examined
carefully to ensure that it is free of opacity or capsular adherence.
Histologic Findings
A posterior polar cataract has been shown to present as an opacity in
the subcapsular portion of the lens that consists of malformed,
disorganized, and degenerated lens fibers. As these fibers migrate
posteriorly from their origin at the equator, increased degenerative
changes have been shown as they reach the posterior pole. This
results in a dense discoid opacity that is often surrounded by an area
of vacuoles and abnormal lens fibers. Often, these abnormal lens
fibrils are connected to the underlying posterior capsule.
Staging
Posterior polar cataracts have not been staged, probably because of
their rare occurrence.
At present, the Lens Opacities Classification System (LOCS) uses
slit-lamp images to grade color and opalescence, as well as
retroillumination images to grade cataracts and posterior subcapsular
opacities. Cataract severity is graded on a decimal scale that
reportedly shows good correlation between different observers. This
system, however, does not specifically deal with posterior polar
cataracts.
The literature is sparse on this aspect of posterior polar cataracts. The
average practice encounters relatively few cases. Grading of posterior
polar cataracts was reported by Forster and colleagues in
2006.[16] Out of 33 patients aged 1 week to 8 years with lens
opacities, 3 patients were noted to have posterior polar cataracts. The
grading was done subjectively and also objectively with the use of
photographs. Grading used a scale of 0-10. Posterior polar cataracts
graded a 6 indicated surgical removal.
A suggested concept would be to classify these opacities according to
size and density. The size range would be 0.5-3 mm in increments of
0.5 mm. Density would be staged from 1+ to 4+ with increasing
density as judged by the ability to visualize the posterior capsule.
Moreover, posterior polar cataracts can sometimes be associated with
other lens opacities, which can be classified according to the LOCS.
Finally, the main hindrance in fully adopting a functional grading
system for posterior polar cataracts appears to be the small sample
sizes used. This is because the entity is rare. The author would be
glad to receive case reports, which could be correlated into a
meaningful classification algorithm.
Medical Therapy
Therapy for posterior polar cataracts is primarily surgical, but safety
is a consideration since this entity poses a special challenge. The
timing of the intervention should be early enough to prevent the
development of amblyopia.
A thorough pediatric and/or intermedical evaluation for associated
systemic findings in all forms of congenital cataract, including
posterior polar cataracts, is indicated. Kronenberg and colleagues
reported on a rare syndrome with a rare neurocutaneous syndrome in
which a chart review revealed significant eye abnormalities.[17] DukeElder cited a series of associated conditions, including ectodermal
dysplasia, Rothmund disease, scleroderma, incontinentia pigmenti,
congenital dyskeratosis, congenital ichthyosis, and congenital
atrophy of the skin.[1]
Surgical Therapy
Posterior polar cataract surgery is challenging, even with the most
advanced techniques available.[18, 19, 20, 21] The surgical pitfalls have
been well documented in the ophthalmic literature. The importance
of being aware of these surgical pitfalls and avoiding them cannot be
overemphasized. It has been suggested that where hydrodelineation is
difficult, prechopping of the anterior nucleus should be initially
performed and any cracking of the nucleus should be limited so as to
not involve the area near the poster pole or the capsule.[22] As
mentioned below, hydrodissection should be avoided because it can,
in itself, cause the abnormally weakened posterior capsule to tear.
Preoperative Details

The preoperative clearance for posterior polar cataract surgery does


not need to include more than that obtained prior to a routine cataract
surgery.
A full and careful pediatric workup should be obtained with an
emphasis on uncovering any associated systemic abnormalities.
Appropriate subspecialty referrals should be obtained in the event of
any significant findings.
Assuming the absence of any associated systemic or ocular disease,
the preoperative preparation for posterior polar cataract surgery can
be limited to a routine preoperative workup and clearance.
Intraoperative Details
Posterior polar cataract surgery is associated with an increased
incidence of posterior capsular rupture. Undue stress on the capsule
must be minimized. The main difficulty is the adherence of the
abnormally formed lens fibers to the posterior capsule and its
associated weakness. Morever, the risk of poster capsule rupture
(PCR) increases significantly when the capsular opacification is
larger than 3 mm.[23] Once the surgeon is aware of these dangers, the
technique can be altered to meet these challenges.
For this reason, special lower flow and aspiration settings should be
used. This situation is described in the following case, which is used
for illustrative purposes. Topical 4% Xylocaine anesthesia is instilled
prior to inserting the lid speculum and positioning the eye to the
operative microscope. The cataract is inspected and typically presents
as a central posterior discoid mass. This can be surrounded by an area
of degenerated fibrillar elements (see the image below).

The posterior polar cataract


is seen as a dense discoid opacity. The central fibrous mass consists
of degenerated lens fibers surrounded by several small globular
vacuoles containing what appears as a refractile substance as well as
some lens degenerative fiber material.
A limbal side port incision can be used to instill 1% preservative-free
Xylocaine solution into the anterior chamber.
Surgery begins in the usual manner with a clear corneal incision and
capsulorrhexis. Care should be taken to minimize posterior pressure
during all maneuvers.
Hydrodissection should be avoided because the fluid wave created
may extend to the weak area of the posterior capsule and in itself
produce a tear. Rotation of the cataract should also be avoided to
prevent the application of any inadvertent torquing forces being
transmitted to the posterior capsule.
Instead, the nucleus and the cortex should be outlined using
hydrodilineation. The goal is to leave the posterior pole as
undisturbed as possible by creating a hydrodissected plane separating
the nucleus and the epinucleus. This should create a cushion of the
cortex and the epinucleus, which should then cushion and protect the
region of the central posterior capsule.
The anterior cortex and the nucleus are then removed using low
aspiration and as little phacoemulsification as possible. Moreover,
once the nucleus is removed, irrigation and aspiration, and especially
phaco, should be directed obliquely as much as possible. Direct
application of these forces to the posterior polar cataract should be
avoided. These forces should be aimed obliquely so as not to cause
inadvertent stress on the delicate posterior capsule. Indeed, it is
sometimes possible to hydrodissect part of the discoid opacity free to
the point where it can be safely removed (see the image below).

Removal of the nucleus and


much of the cortex has been completed with the aid of
hydrodissection. Almost all of the posterior cortical opacity has also
been aspirated. Only indirect irrigation and aspiration was used.
Hydrodelineation has been avoided. There appears to be a ringlike
remnant of the opacity that may have some adherence to the capsule.
Once this is achieved, the surgeon is still faced with the epinucleus
and, more significantly, any residual posterior capsular adhesion.
Dye has been introduced as an aid to ocular surgery. [24] As seen in the
image below, the peripheral remnant of the opacity still remains. This
presents the possibility of an area of capsular adherence, and care
must still be exercised in aspirating this area.

Removal of the nucleus and


much of the cortex has been completed. Highly dilute fluorescein dye
has been instilled to stain the remaining epinucleus but especially the
remaining ringlike opacity. The capsule is still intact since the stain is
limited to the anterior chamber. This aids in careful cortical
aspiration without damage to the intact posterior capsule.
For this reason, highly diluted fluorescein has been instilled to
delineate this remnant of the posterior polar cataract. Indeed, the
appearance of this remnant is enhanced, and the 2- and 9-o'clock
positions of the ring look especially suspicious (see the image
above). Further, the integrity of the posterior capsule is verified
because the dye has not spread into the region of the anterior
vitreous.
Additional indirect irrigation can be used to further loosen and
hydrate the remaining opacity using the silicone tip, which is an
additional recommended safety feature. The remainder of the
epinuclear cortex can then be aspirated, using vacuum levels as low
as practical to avoid any additional stress (see the image below).

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.

The posterior polar cataract


is seen as a dense discoid opacity. The central fibrous mass consists
of degenerated lens fibers surrounded by several small globular
vacuoles containing what appears as a refractile substance as well as
some
lens
degenerative
fiber
material.

Removal of the nucleus and


much of the cortex has been completed with the aid of
hydrodissection. Almost all of the posterior cortical opacity has also
been aspirated. Only indirect irrigation and aspiration was used.
Hydrodelineation has been avoided. There appears to be a ringlike
remnant of the opacity that may have some adherence to the capsule.

Removal of the nucleus and


much of the cortex has been completed. Highly dilute fluorescein dye
has been instilled to stain the remaining epinucleus but especially the
remaining ringlike opacity. The capsule is still intact since the stain is
limited to the anterior chamber. This aids in careful cortical
aspiration without damage to the intact posterior capsule.

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.