1
Case Report: Phacomorphic Angle Closure
American Academy of Optometry
Submission for Resident's Day 2010
Abstract
A patient presents with intermittent angle closure attacks following hemodialysis treatments,
uncontrolled with patent LPIs. His angle closure is attributed to narrow angles and a phacomorphic
component, with possible contribution from antipsychotic medication and hemodialysis.
I. Case History
A 63 year old white male presents with a chief complaint of a sore, painful, red right eye that began
overnight. He complains of a periorbital and brow headache and decreased vision. He reports that he
has experienced two similar episodes recently, and that each occurred following kidney dialysis.
This patient, Mr. S has an ocular history that is significant for diabetes mellitus type 2 without
retinopathy, mild nuclear cataracts with anterior capsule stellate phenothazide deposits, posterior
vitreous detachments, and dry eyes. He reports that his right eye was hit by a rock many years previous.
He has no previous ocular surgery. His medical history is significant for chronic stage V kidney disease,
hypertension, hyperphosphatemia, anemia, microscopic hematuria , secondary hyperparathyroidism,
pancreatitis, hypercholesterolemia, , seborrheic keratosis, gerd, and schizophrenia. His medications
include simvastatin, risperidone, ziprasidone HCl, calcium acetate, omeprazole, cinacalcet, nephrocaps
and acetaminophen/butalbital /caffeine.
II. Pertinent findings
Visual acuity is 20/400 in the right eye with no
improvement with pinhole and is 20/25+2 in the
left eye. At routine examination five months
previous, visual acuity was measured as 20/20‐ in
each eye. Pupil examination reveals a fixed, mid‐
dilated right pupil, a round, reactive to light left
pupil, and no afferent pupillary defect by reverse
method. Extraocular motilities and confrontation
fields are normal in both eyes. Slit lamp
examination of the right eye shows 4+ conjunctival
injection and a steamy, hazy cornea. The anterior
chamber is shallow in the right eye and moderately
Figure 1. Right eye about two hours after intial presentation. shallow in the left. Von herrick assessment of the
The pupil is mid‐dilated. Corneal haze is difficult to appreciate angle shows iris‐cornea apposition of the right
in this photo. angle and 1/4:1 in the left. Goldmann applanation
tonometry is 52 mmHg in the right eye and 18 mmHg in the left. Examination of the lens shows mild
nuclear sclerosis and phenothiazide anterior capsule deposits in both eyes. Undilated fundus
ophthalmoscopy shows well perfused nerve heads and small cups, 0.25h/v OD and 0.3h/v OS.
Compression gonioscopy in the right eye reveals a closed angle with no opening upon compression.
Gonioscopy of the left eye shows an angle open to the posterior trabecular meshwork 360° with a steep
iris approach and no peripheral anterior synechia.
Kimberly Dong O.D. 2
Figure 2. Anterior chamber of the right eye. The angle and chamber depth is shallow.
Fifty minutes after administration of apraclonidine, pilocarpine 4%, dorzolamide 0.2%/timolol 0.5% and
two 250 mg tablets of acetazolamide, the intraocular pressure in the right eye is still 52 mmHg.
Apraclonidine, pilocarpine 4%, and dorzolamide 0.2%/timolol 0.5% are administered again. Forty
minutes following, the intraocular pressure is 46 mmHg. Two hours later after pilocarpine 1% dosed
every fifteen minutes, intraocular pressure decreases to 21 mmHg and down to 15 after one additional
hour. Two laser peripheral iridotomies are performed. A small amount of bleeding occurs at the site of
the initial iridotomy. Post operative IOP is 8 mmHg and then 11 mmHg forty minutes after the
procedure. The patient is given prednisolone acetate 1% 1gtt QID OD, dorzolamid 0.2%/timolol 0.5%
BID OD and erythromycin ung TID OD.
Five days later, vision is improved to 20/20‐ in both eyes. A prophylactic LPI in the left eye is performed
with no complications.
An A scan ultrasonography is performed s/p LPI OU. The results are as follows:
OD OS
Gonioscopy performed after LPIs done OU shows anterior trabecular meshwork 360° in the right eye
and scleral spur 360° in the left eye.
At one month follow up, the IOP in the right eye is 18 mmHg and examination of the iris shows a barely
open PI at 10 o'clock and a closed PI at 1 o'clock.
A repeat LPI is performed in the right eye. The intraocular pressure at follow up is 35 mmHg despite
three patent LPIs. Gonioscopy in the right eye shows two quadrants of anterior trabecular meshwork
and two quadrants with no angle structures visible. Intraocular pressure is decreased on dorzolamide
0.2%/timolol 0.5% BID, brimonidine 0.2% BID and acetazolamide 500 mg PO and is 9 mmHg at
subsequent follow up.
Kimberly Dong O.D. 3
This patient is seen six weeks later on brimonidine BID OD. The intraocular pressure is elevated at 35
mmHg. The three LPIs are patent.
On both on dorzolamide 0.2%/timolol 0.5% BID and brimonidine 0.2% BID, the IOP is decreased to 13
mmHg at a one week follow up visit.
III. Differential diagnosis
The leading diagnosis in this case is acute angle closure in the right eye with recurrence. Included on the
differential include pupil block, plateau iris syndrome, a phacomorphic component, along with
cumulative effects from this patient's antipsychotic medication, pain medication and hemodialysis
treatment. Other potential, more rare causes include iridociliary cysts, a ciliary body or iris tumors and
malignant glaucoma.
IV. Diagnosis and discussion
This patient was diagnosed with acute angle closure with likely a phacomorphic component. Mr. S
experienced angle closure recurrence despite three patent LPIs. Angle closure can occur through
different mechanisms. An enlarging lens can cause relative pupil block, causing the iris to bow forward,
closing the angle. The growing lens could alternatively push anteriorly on the iris and ciliary body,
causing a more shallow anterior chamber and angle closure. A thickened lens can be due to a mature
cataract or sudden hydration of an immature cataract.11 In the case of mechanical angle closure, laser
peripheral iridotomies may not be effective.1
The lens nuclear sclerosis in this patient was not markedly
more dense than the fellow eye. Both eyes showed mild
nuclear sclerosis and phenothiazide anterior capsule deposits.
The visual acuity was correctable to 20/20‐ in both eyes.
There was no myopic shift noted at routine examination over
a period of years prior to the angle closure attack. Literature
review shows that typical cases of phacomorphic angle
closure involve eyes with dense, mature cataracts, particularly
when compared to the fellow eye.1 The anterior chamber
depth on A scan showed a deeper anterior chamber in the
right eye as compared to the left, as well as a thicker lens.
However it was noted that the scan of the right eye was
difficult to perform, thus may not be the most accurate
results.
Despite these atypical characteristics, a phacomorphic
component likely contributed to this patient's angle closure.
On routine examination prior to the angle closure event, it
was noted that the anterior chamber angles appeared narrow
by Von Herrick technique. A 1/4:1 measurement was found,
Figure 3. Nuclear sclerosis and phenothiazide however on gonioscopy the angle was determined to be open
deposits of the right lens. These photos were to the tip of the ciliary body band with mild iris bowing in both
acquired two years prior to the angle closure eyes. This narrow angle configuration likely makes this
attack. patient more susceptible to phacomorphic changes affecting
the angle. Additionally, it was found later that this patient had zonular dehiscence at 5‐11 o'clock. The
Kimberly Dong O.D. 4
weaken zonules may have allowed the lens to move anteriorly, mechanically causing angle closure.
There is indication that laser peripheral iridotomy may rarely cause zonular damage.3 However, more
likely, this patient's history of trauma to his right eye may have caused zonule damage years previous.
Another cause of secondary angle closure is plateau iris syndrome. A plateau iris configuration occurs
when the iris root is angled forward and with an anteriorly inserted ciliary body. The iris is flat centrally
with a normal appearing anterior chamber depth.2 If angle closure occurs then this is termed plateau
iris syndrome. Because pupil block does not contribute to the process of angle closure in plateau iris,
laser peripheral iridotomy would be ineffective at controlling IOP, making this syndrome part of the
differential diagnosis. A diagnostic feature of plateau iris is the double hump sign seen on indentation
gonioscopy of the iris and angle. The iris has a normal, flat shape centrally, dips peripherally, then is
steeply inserted into the angle. The patient in this case did not have this feature.
Other causes of acute angle closure include iridociliary cysts, ciliary body or iris tumors and malignant
glaucoma. Clinical examination did not indicate signs of these causes. If suspected, additional imaging
including optical coherence tomography and ultrasound biomicroscopy imaging would be helpful in
aiding diagnosis.
Additional factors that may have contributed to this patient's angle closure attack include his long term
use of antipsychotic medication and recent start of a barbiturate pain medication. This patient was
taking ziprasidone HCl 60 mg tab in the morning and 80 mg at night as well as risperidone 1 mg tab one
half tab every morning and one half tab every night for at least a few years. These medications are
atypical antipsychotic drugs for the treatment of schizophrenia. Typical antipsychotics, which include
phenothiazides act by blocking dopamine postsynaptic receptors, histamine receptors, alpha 1
adrenergic receptors and cholinergic M1 receptors.4 Blockage of the latter receptor causes
anticholinergic effects, which includes mydriasis of the pupil, potentially precipitating angle closure.
Atypical antipsychotic drugs act differently by blocking serotonin and dopamine. They have historically
been less likely to cause anticholinergic effects.4 However, ziprasidone has been associated with an
increase in intraocular pressure and is different than other atypical antipsychotics because it has a more
similar mode of action to serotonin reuptake inhibitors (SSRIs). 4 SSRIs have been associated with acute
angle closure because of its serotonin induced mydriatic effect and weak anticholinergic effect.5 Case
reports of patients on an SSRI with subsequent angle closure either very soon after starting or weeks
after starting indicate a link between SSRIs and angle closure. The mechanism is not entirely
understood, but was proposed that the immediate attack was likely due to anticholinergic activity
whereas a delayed reaction was likely due to serotonin because it has a slower and more gradual mode
of action.6
Mr. S presented to urgent care twice over a period of one to two months. He first walked in with a right
sided headache that he noted occurred about two hours after hemodialysis. He was given
acetaminophen 325/butalbital 50/caffeine 40mg to take 1 tab TID as needed for headache pain. The
patient reported that he had experienced another prior episode of right sided headache pain also
following dialysis treatment. He went to urgent care one month later with a similar, but more severe
headache, accompanied by eye pain and severe sensitivity to light. This again occurred less than 24
hours following hemodialysis. This patient likely experienced three episodes of angle closure attacks,
with the third one resulting in complete angle closure. Of note, the patient started acetaminophen
325/butalbital 50/caffeine 40mg after the second attack. The butalbital component of this combination
drug for pain relief is a central nervous system depressant that has anticholinergic properties. It is
possible that this drug may have had a contributory effect on the patient's already presdisposed angle
anatomy.
Kimberly Dong O.D. 5
It is also interesting that all three of these occurrences happened following dialysis treatment.
Hemodialysis is performed on patients with chronic end stage kidney disease to compensate for the
body's poor ability to maintain a normal volume and composition of body fluids.7 It has been shown
that hemodialysis has an effect on intraocular pressure. During hemodialysis water is removed from the
body, causing an increase in plasma colloid osmotic pressure (COP). This increase in COP creates a
gradient between plasma and the aqueous humor pushing water from aqueous to plasma, thus
decreasing IOP. 7 One study showed that patients who have undergone hemodialysis for less than
twelve years experience a signficant decrease in IOP after hemodialysis. Those who had undergone over
twelve years of treatment showed a trend towards increase of IOP after hemodialysis. The proposed
mechanism of action behind this was that the trabecular meshwork outflow becomes impaired over a
long period of hemodialysis treatment due to buildup of abnormal deposits. 8 While consensus over the
relationship between IOP and hemodialysis is still under debate, one case report suggests that during
hemodialysis the aqueous humor production increases due to the shift in osmolarity. In eyes with
normal outflow facility, IOP does not rise, but in eyes with impaired outflow as in narrow or
compromised angles, an increase in IOP results. This case report described a 68 year old woman with
end stage renal disease and neovascular glaucoma. Like Mr. S, this patient experienced a dramatic rise
in intraocular pressure following hemodialysis treatment. 9
V. Treatment and management
When this patient presented with an acute angle closure attack and intraocular pressure of 52 mmHg,
topical and oral medical therapy was initiated including a topical alpha adrenergic agonist, topical beta
blocker, topical and oral carbonic anhydrase inhibitors and pilocarpine. IOP was lowered to 15 mmHg
after a period of four hours. After initiating IOP lowering medication, the patient was sent for emergent
laser peripheral iridotomy, which was performed the same day. Two LPIs were placed in the right eye
and IOP was stable and low after treatment. The patient was sent home with glaucoma medications.
He remained stable for a few weeks, but at follow up one month later, his eye pressure rose again. A
repeat LPI was performed with initial IOP control. However, at follow up IOP continued to rise despite
three patent LPIs. At that point, the patient underwent cataract extraction in the right eye, with
stabilization of IOP following surgery and on brimonidine 0.2% BID OD. During cataract surgery zonular
dehiscence was found at 5‐11 o'clock. Iris hooks were used to stabilize the capsule.
At later follow up, the patient's IOP continued to be controlled at 14 mmHg in the right eye. A dilated
exam and a 30‐2 full threshold Humphrey visual field was performed. The results showed a borderline
glaucoma hemifield test with possible early nasal and inferior defects. Optic nerve evaluation revealed
healthy and symmetrical nerves that appeared intact without focal rim tissue loss. Best corrected visual
acuity returned to 20/20‐. This patient's visual recovery and lack of apparent damage to his optic nerve
heads is consistent with studies showing that the visual return after angle closure attack is directly
related to the duration of the attack. 11 While this patient had a few intermittent episodes of angle
closure, the duration of complete angle closure was less than 24 hours. It has been shown that attacks
of less than two days duration were able to regain vision of 6/12 or better, but attacks lasting longer
than three weeks tended to result in hand motion or light perception. 11
Another possible treatment for phacomorphic glaucoma is argon laser peripheral iridoplasty. A study
was performed that showed this to be a safe and effective initial management.10 For this patient, the
phacomorphic component was not determined until LPIs were found ineffective at maintaining a normal
IOP.
Kimberly Dong O.D. 6
While it is speculated that other factors including antipscyhotic medication and hemodialysis may have
contributed to the development of this patient's angle closure, these interventions would likely not be
discontinued as they are medically necessary. Consultation with the patient's psychiatrist and close
watch of the patient is necessary.
This patient was scheduled to be seen again for an IOP check in six months. He was instructed to return
right away if he experiences any symptoms of an angle closure attack. He continues to use brimonidine
0.2% BID OD. A visual field evaluation will be repeated in one year.
VI. Conclusion
In conclusion, this patient exemplifies a number of features that can contribute to acute angle closure.
While it is difficult to determine which caused his attack, there was likely a cumulative effect that lead to
eventual closure. This patient was anatomically predisposed with narrow angles. He had a history of
eye trauma and was found to have zonular dehiscence, which in hindsight, likely caused the
phacomorphic component. Lens intumescence is another causative possibility. His medications,
especially those with mydriatic and anticholinergic properties could cause angle closure. The effect of
hemodialysis on IOP could have also played a role. In summary, one should carefully monitor and warn
patients who have risk for angle closure. One should watch patients on psychiatric medications and
anticholinergic medications and work with prescribing clinicians if there is suspicion or high risk of angle
closure. While the effect of hemodialysis on IOP maybe be an area that needs further study to
determine trends, it is important to be aware of potential effects, especially in glaucomatous patients.
References
1. Sowka, Joseph. Phacomorphic glaucoma: case and review. Optometry 2006; 77:586‐589.
2. Ritch R, Tham CC, Lam DS. Long‐term success of argon laser peripheral iridoplasty in management of
plateau iris syndrome. Ophthalmology 2004;111:104‐108.
3. Seong M, Kim MJ, Tchah H. Argon laser iridotomy as a possible cause of anterior dislocation of a
crystalline lens. J Cataract Refract Surg 2009;35:190‐192.
4. Souza, Valéria Barreto Novais e et al. Intraocular pressure in schizophrenic patients treated with
psychiatric medications. Arq. Bras. Oftalmol 2008;71:660‐664.
5. Ekea T, Batesa AK, Carrb S. Drug points: Acute angle closure glaucoma associated with paroxetine.
BMJ 1997;314:1387.
6. Ekea T, Carrb S. Acute glaucoma, chronic glaucoma, and serotoninergic drugs. Br J Ophthalmol
1998;82:976.
7. Tokuyama T, Ikeda T, Sato K. Effect of plasma colloid osmotic pressure on intraocular pressure during
haemodialysis. Br J Ophthalmol 1998;82:751‐753.
8. Doshiro A, Ban Y, Kobayashi L, Yoshia Y, Uchiyama H. Intraocular pressure change during
hemodialysis. Am J Ophthalmol 2006;142:337‐339.
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9. Fischer DM, Fleischhauer J, Keusch G. Rise in intraocular pressure during haemodialysis in a patient
with reduced outflow facility. Br J Ophthalmol 2007;91:1091‐1093.
10. Yip PP, Leung WY, Hon CY, Ho CK. Argon laser peripheral iridoplasty in the management of
phacomorphic glaucoma. Ophthalmic Surgery, Lasers & Imaging 2005;36:286‐291.
11. Jain IS, Gupta A, Dogra MR, Gangwar DN, Dhir SP. Phacomorphic glaucoma‐management and visual
prognosis. Indian J Ophthalmol 1983;31:648‐653.