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Optometry (2006) 77, 586-589

Phacomorphic glaucoma: Case and review


Joseph Sowka, O.D.
Nova Southeastern University, College of Optometry, Ft. Lauderdale, Florida.
KEYWORDS
Phacomorphic
glaucoma;
Phacolysis;
Lens-induced
glaucoma;
Argon laser peripheral
iridoplasty

Abstract Phacomorphic glaucoma is a lens-induced secondary angle closure glaucoma that may occur
as a result of mature cataract formation. A patient with markedly asymmetric cataract and anterior
chamber depth was referred after the development of significant eye pain and elevated intraocular
pressure that did not respond to topical antiglaucoma medications. The presence of an asymmetric
mature lens, angle closure, and intraocular pressure elevation in the affected eye led to the diagnosis of
phacomorphic glaucoma. Because the patient was systemically ill, physically frail, and had poor vision
in the affected eye since birth, secondary to strabismic amblyopia, initial therapy was medically directed
toward pain management. Cycloplegia, corticosteroids, and aqueous suppressants successfully ameliorated the patients intraocular pressure and adequately controlled pain. However, progression to
phacolysis and subsequent failure of pain management necessitated referral for lens extraction. The
diagnosis and mechanism of phacomorphic glaucoma is discussed along with a review of current
treatment modalities.
Optometry 2006;77:586-589

Intraocular pressure elevation can be induced by the


crystalline lens through several mechanisms and represents
a significant cause of secondary glaucoma.1-3 Although
lens-induced glaucomas tend to be more common in developing countries where citizens have less access to surgical
care,1-3 they can and do occur in developed nations when
individuals, for a variety of reasons, allow cataracts to fully
mature and progress to hypermaturity. Phacomorphic glaucoma is a known cause of secondary angle closure glaucoma, the clinical appearance of which must be recognized
to institute appropriate management.

Case report
A 48-year-old black woman with cerebral palsy, sickle cell
disease, and arthritis was referred urgently for a glaucoma
Corresponding author: Joseph Sowka, O.D., Nova Southeastern University, College of Optometry, 3200 South University Drive, Ft. Lauderdale, Florida 33328.
E-mail: jsowka@nova.edu

consultation owing to elevated intraocular pressure (IOP)


and ocular pain in the left eye (O.S.) that had not responded
to topical glaucoma therapy. She presented to the referring
optometrists office with a painful left eye. Her left eye had
been moderately painful for approximately 3 months prior,
but the intensity of the pain had increased over the last
several days. Because the patient was unable to maintain
fixation in the biomicroscope, intraocular pressure evaluation at that time was performed with a Tono-Pen (Mentor,
Santa Barbara, California), with resultant readings of 19
mmHg in the right eye (O.D.) and 39 mmHg O.S. Gonioscopy reportedly had been attempted but was unsuccessful
because of the patients lack of cooperation. A dilated
fundus examination found angioid streaks in the right eye
without any evidence of sickle cell retinopathy. Funduscopic views of the left eye were unattainable because of a
dense cataract.
The referring optometrist initially used apraclonidine
0.5% in the office to reduce the intraocular pressure, without
success. The patient was prescribed travoprost 0.004% every evening O.S. When seen for follow-up 7 days later, her
intraocular pressure measured 45 mmHg O.S. (by Tono-

1529-1839/06/$ -see front matter 2006 American Optometric Association. All rights reserved.
doi:10.1016/j.optm.2006.08.013

Joseph Sowka

Issue Highlight

Pen), and travoprost was deemed ineffective. The patient


was then prescribed pilocarpine 2% 3 times a day and
brimonidine 0.2% twice a day O.S. and referred for evaluation and management.
On presentation for consultation 7 days later, the patient
reported that her left eye was still painful with no reduction
in pain with the use of the medications. Her medical history
was significant for sickle cell disease, mild cerebral palsy,
and osteoarthritis. She was using Bextra (valdecoxib; G.D.
Searle & Co., Skokie, Illinois) for pain associated with
sickle cell disease and osteoarthritis. The patient was frail
with limited mobility, for which she used a wheelchair. She
reported that she had a constant left eye turn since birth with
poor visual acuity in that eye, had no changes in vision in
either eye, and was most concerned about the pain in her left
eye.
Unaided visual acuity was 20/100 O.D., with refractive
improvement to 20/40 with a 3.00 spherical correction
and bare light perception O.S. The right pupil reacted to
light, whereas the left pupil was medically miotic. There
was a relative afferent pupil defect O.S. by reverse testing.
Confrontation visual field screening was full O.D. The
patient could not be screened in the left eye because of poor
vision. Ocular muscle testing found no restrictions in either
eye; however, there was a 45-prism diopter constant left
exotropia present at distance and near.
Biomicroscopic evaluation found a normal anterior segment O.D. with the exception of a mild nuclear sclerotic
cataract. The left eye showed normal adnexa and a fully
mature nuclear sclerotic cataract that was much denser than
that in the fellow eye. Funduscopic views through the left
cataract were not possible. The anterior chamber appeared
to be deep in the right eye and much shallower in the left
eye. Goldmann applanation tonometry was performed successfully. Intraocular pressures were 18 mmHg O.D. and 35
mmHg O.S. Gonioscopy found no angle structures in any
view in the left eye; however, there were areas of peripheral
anterior synechiae. There was no neovascularization of the
iris or angle. Gonioscopy was unsuccessful in the right eye
because of increasing lack of patient cooperation.
The patient had phacomorphic glaucoma diagnosed O.S.
based on the asymmetric maturity of the left cataract with
anterior chamber shallowing and angle closure. A deep
anterior chamber in the right eye as well as the presence of
myopia further supported a lens-induced mechanism of
angle closure. Previous use of apraclonidine, pilocarpine,
and travoprost had apparently not yielded any significant
success in either reducing the IOP, changing angle configuration, or ameliorating the patients discomfort. Because of
little visual potential in the left eye and pain being the
patients most significant concern, medical therapy was
instituted to address the discomfort. Pilocarpine and brimonidine were discontinued, and prednisolone acetate 1% 4
times a day, scopolamine 0.25% twice a day, and timolol
maleate 0.5% gel-forming solution every day in the morning O.S. were prescribed. The patient was reappointed for 1

587
week but told to call immediately if her pain did not
improve or if it worsened within the next several days.
The patient returned as scheduled and reported good
medical compliance. Her visual acuity was unchanged in
each eye. She now reported that her ocular pain had greatly
diminished and was now only occasional. She had no ocular
pain at the time of the visit. Intraocular pressures were 19
mmHg O.D. and 40 mmHg O.S. Her left pupil was pharmacologically dilated. The patient was instructed to continue the medications prescribed at the last visit as well as to
resume brimonidine bid O.S. The patient was reappointed
for 3 months with the instructions to return immediately
should there be any increase in pain or other changes.
At the 3 month follow-up, the patient reported good
medical compliance and no further instances of ocular pain.
Intraocular pressures by Goldman applanation were 16
mmHg O.D. and 25 mmHg O.S. There were no changes in
her biomicroscopic evaluation. Because there was no potential for improved functional vision O.S., and her pain was
well managed medically, she was deemed not to be a good
candidate for lens extraction. She continued all medications.
The patient was successfully followed up medically for
the next 7 months. However, at her most recent progress
evaluation, she reported that she had developed significant
pain again in her left eye as well as general photophobia.
The visual acuity was unchanged in the right eye but had
decreased to no light perception in the left eye. Additionally, the left cataract was visibly whiter on gross inspection.
Her family members had also noticed that her pupil seemed
whiter than in the past month.
Biomicroscopic evaluation found a hypermature, lytic
cataract O.S. with a moderate anterior chamber reaction.
Intraocular pressures were 18 mmHg O.D. and 38 mmHg
O.S. It was determined that the phacomorphic lens had now
become phacolytic. As a result, it was felt that medical
control would no longer be appropriate to manage the
patients pain and that the patient would now benefit from
medically necessary cataract extraction. Ultrasonography,
planned to ascertain if an intraocular process may have
contributed to the asymmetric cataract formation, was abandoned in favor of a surgical consultation for cataract extraction. The consulting surgeon agreed that lens removal was
medically necessary despite poor visual potential. At the
time of this writing, the patient is scheduled for cataract
extraction O.S (see Figure 1).

Discussion
As the name implies, phacomorphic (phaco lens; morph
shape) glaucoma develops secondary to the shape of the
lens. Angle closureacute, subacute, or chronic can be
triggered by a mature or intumescent cataract and occurs in
eyes with previously open angles as well as in those with
narrow, occludable angles. This is encountered more often
in developing countries where health care is not easily

588

Figure 1

Optometry, Vol 77, No 12, December 2006

Phacolytic lens in another patient.

accessible, and patients present late, often waiting until the


cataract becomes mature. It is a common belief among these
patients that cataracts should not be removed until vision
drops to the level of hand movements or light perception.4
Typically, an advanced, intumescent cataract will be present
in the affected eye. Visual acuity is reduced, often to a level
of hand motion or worse.1,3 In addition, there will be a
shallow anterior chamber. In eyes with markedly asymmetric cataract formation, the depth of the anterior chambers
may be accordingly disparate, which can be very diagnostic.
On occasion, phacomorphic glaucoma will occur not because of mature cataract formation but because of
spherophakia in Weill-Marchesani syndrome.5-8 Pain is a
very common presenting accompanying symptom, being
present in 71% of cases in one report.1 Intraocular pressure is significantly elevated, often exceeding 30 to 40
mmHg.1,9
Thickening of the lens during cataractogenesis can result
in a relative pupil block, with resultant iris bomb and
subsequent angle closure glaucoma. This may occur commonly in patients with already shallow anterior chamber
angles, and cataractogenesis enhances the physiology of
angle closure. However, when angle closure occurs in
myopic eyes or in those patients manifesting a deep fellow
eye anterior chamber, then phacomorphic glaucoma is the
primary mechanism.1,4 Further, the swelling of the lens may
cause it to press on the iris and ciliary body forcing them
anteriorly and shallowing the anterior chamber. Thus, there
can be an acute angle closure attack without relative pupil
block as a mechanism, which may not respond to laser
peripheral iridotomy (LPI).
Medical therapy is often used initially in an attempt to
reverse the process and acutely lower the intraocular pressure. Beta-blockers, -2 adrenergic agonists, topical corticosteroids, topical or oral carbonic anhydrase inhibitors, and
oral hyperosmotic agents all may be systematically used.
However, the use of pilocarpine is somewhat controversial
because it has the effect of increasing axial lens thickness
and causing anterior lens movement, resulting in the anterior chamber becoming more shallow, worsening the condition.10,11 Superior IOP control and shortening the dura-

tion of the attack preoperatively is essential in improving


the final visual outcome.12
It has long been believed that surgical management of
patients with phacomorphic glaucoma tends to be associated
with complications and poor visual outcomes.1,13,14 Although complications can and do occur surgically, successful outcomes are not infrequent.3,12,15 However, surgical
therapy may not be indicated when visual potential is
known to be poor because of glaucomatous damage or other
pre-existing conditions.
In any case of phacomorphic glaucoma, especially those
with the presence of iris bomb indicating pupil block, LPI
remains a strong component of early management. In one
review of 10 patients undergoing LPI for phacomorphic
glaucoma, it was seen that the acute angle closure attack
was prevented successfully or reversed by the procedure in
all cases.4
Argon laser peripheral iridoplasty (ALPI) is rapidly becoming an accepted management of phacomorphic glaucoma.9,16-18 ALPI is a thermally induced iridoretraction
procedure designed to pull the iris from its apposition
against the cornea and trabecular meshwork.18
In one report involving 21 eyes of 21 patients having
ALPI as an initial step in the management of phacomorphic
glaucoma, it was seen that IOP was controlled successfully
in 81% of eyes, with a statistically significant decrease at 2
and 24 hours postoperatively. Elective cataract surgery was
performed in 16 of the 17 successful cases. In 12 patients
completing at least 12 months of follow-up, IOP was normal
without medications in 11 eyes, and only 1 patient required
antiglaucoma eye drops to control IOP. All patients maintained an open angle without further surgical intervention.
No complications directly attributable to ALPI were noted.
It was seen that ALPI was a safe and efficacious measure for
the initial management of phacomorphic glaucoma, obviating the need to operate in highly inflamed eyes in an
emergency setting.17
Tham et al.9 reported on 10 consecutive patients with
acute phacomorphic glaucoma receiving an immediate standard treatment consisting of atropine, timolol, and ALPI and
found that only 1 patient additionally required systemic
acetazolamide to control IOP. Ultimately, all eyes underwent cataract extraction without complications within 4
days after ALPI. There were no complications associated
with ALPI. The investigators concluded that ALPI is a safe
and effective first-line treatment for acute phacomorphic
angle closure, obviating the need for systemic IOP medications.
It is important to distinguish phacomorphic from phacolytic glaucoma; the patient presented herein developed both
conditions. As noted, phacomorphic glaucoma is a closed
angle situation, whereas phacolytic glaucoma is predominately an open-angle condition. Phacolysis refers to the
dissolution of the hypermature crystalline lens. As a cataract
progresses to hypermaturity, the lens shrinks with loss of
high molecular weight (HMW) soluble proteins through an
intact lens capsule.19 This incites an immune-mediated

Joseph Sowka

Issue Highlight

cellular response, with the proteins being engulfed by macrophages, which congregate on the surface of the lens and
become trapped within the trabecular meshwork.19 When
the accumulation of macrophages and HMW-soluble proteins physically block and impede aqueous outflow through
the trabecular meshwork, a secondary pressure rise known
as phacolytic glaucoma is said to ensue.20

Conclusions
Although lens-induced glaucomas are not encountered commonly in populations that have ready access to cataract
surgery, they can easily occur in patients who allow cataracts to mature. Lens-induced glaucomas represent a significant percentage of secondary glaucomas.3 A mature cataract can induce angle closure and phacomorphic glaucoma,
whereas a hypermature cataract can induce phacolytic glaucoma. In some cases, such as the one presented here, more
than 1 lens-induced mechanism may occur in a patient.
Although cataract extraction typically is the definitive treatment for lens-induced glaucomas, there are steps that must
be taken in advance to address the acute nature of the
condition. Also, for a variety of reasons, some patients may
not be good surgical candidates, which may be the underlying reason that a cataract may not have been addressed
earlier and allowed to mature. Alternate chronic medical
therapies may then be more appropriate for that particular
patient situation.
The patient presented here was a poor surgical candidate
because of no potential visual improvement and frail health.
Because the pain was the most significant concern, medical
rather than surgical therapy was initially successfully instituted. However, the progression from phacomorphic to
phacolytic glaucoma with an inability to medically control
pain necessitated a change in therapeutic approach.

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