Transcript 2006.09.26- Iris Cyst

Bascom Palmer Eye Institute
Grand Rounds
Kyle Alliman, MD
Richard Forster, MD
September 28, 2006
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Case Presentation
• 34 yo female presents to BPEI ER with
redness, discomfort, photophobia, and
blurry vision OD for 3 days.
• Seen at BPEI 1 ½ years ago with
contact lens intolerance/overwear.
Since then, uses contacts sparingly.
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• PMHx
2 months post-partum
• PSHx
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s/p C-Section x 2 (1991, 2006)
• POHx
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h/o myopia
No h/o ocular trauma or surgery
occasionally uses Elestat for
redness/allergies
noticed “white spot” in right eye since age
12
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• Medications
•
multivitamins
• Allergies
•
NKDA
• SH
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Lives in Miami
Works in sales
Denies tob, IVDA. Occasional EtOH
• FHx
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Father with HTN, mother with DM, HTN.
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Initial Examination
• Healthy-appearing female in NAD
• BP 128/73, P 77, R 16, T 98.2
• VAcc
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20/20
20/20
• Rx
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OD: -5.25 + 1.25 x 095
OS: -5.50 + 0.25 x 065
• Ocular Motility
•
Orthophoria, full ductions OU
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Initial Examination
• Adnexae/Face
•
WNL
• Visual fields
•
Full to confrontation OU
• Pupils
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4 mm → 2mm, slightly irregular, no APD OD
4 mm → 2 mm, no APD OS
• IOP
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17 mm Hg OD
14 mm Hg OS
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SLE OU
• OD
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• OS
Trace injection
Trace cell
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See Photos
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Conj: W/Q
Cornea: clear
AC: D/Q
Iris: wnl
Lens: clear
DFE – wnl OU
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Differential Diagnosis?
• Iris cyst
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Primary
Secondary
Amelanotic iris melanoma
Iris nevus
Retained FB/FB granuloma
Metastasis to iris/ciliary body
• Medulloepithelioma
• Juvenile xanthogranuloma
• Leukemia/Lymphoma
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Leiomyoma
Tapioca melanoma
Dermoid
Cogan-Reese/ICE
• Other iris nodules: Lisch, Brushfield spots, Koeppe/Busacca
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AC Ultrasonography
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Anterior Chamber OCT
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Iris Cysts
• Rare, outpouchings or deviations of the
iris epithelium or stroma
• 2 main varieties:
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Primary/Spontaneous/Congenital
Secondary
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Paintings by Mr. Myers from Berliner
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Formal Classifications
• Primary cysts
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Of the iris pigment epithelium
Central (pupillary margin)
 Midzonal
 Peripheral (iridociliary)
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Of the iris stroma
Congenital (children)
 Spontaneous (adults)

Shields JA, Kline MW, Augsberger JJ. “Primary iris cysts: a review of the literature
and report of 62 cases. The British Journal of Ophthalmology. 86:3, 152-66. 1984.
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Formal Classifications
• Secondary cysts
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Epithelial downgrowth cysts
Post-surgical
 Post-traumatic
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Pearl cysts
Drug-induced cysts
Cholinergics (pilocarpine, echothiophate, etc.)
 Latanoprost
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Secondary to intraocular tumors
Medulloepithelioma
 Uveal melanoma

•
Parasitic
Shields JA, Kline MW, Augsberger JJ. “Primary iris cysts: a review of the literature
and report of 62 cases.” The British Journal of Ophthalmology. 86:3, 152-66. 1984.
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Secondary Cysts
• Post-Surgical
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Post-operative epithelial invasion of the AC results in
epithelial downgrowth
Rarely, downgrowth leads to cyst formation
 Cysts more amenable to treatment than diffuse
downgrowth
• Post-traumatic – similar to post-surgical
•
With penetrating injury, epithelium introduced into AC
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Haller JA et al. “Surgical management of anterior chamber epithelial cysts.”
American Journal of Ophthalmology. 135(3), 309 – 313. March, 2003.
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Pearl Cysts
• Historically, associated with trauma
• Caused by displaced conjunctival or
epidermal epithelium
• May cause recurrent iritis
• If cyst ruptures, a secondary mucogenic
glaucoma may result
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photos from www.eyecancer.com
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Pearl Cysts
• Thought to occur with introduction of cilia +
epithelium into the AC
• 1872, Goldzieher introduced pieces of
conjunctiva, nasal mucosa, cornea, and
peripheral nerve tissue into the AC
•
Iris cyst from nasal mucosa
• 1888, Masse placed various tissues,
including epithelium into the AC
•
All but epithelium became absorbed
Sitchevska O and Payne BF. “Pearl Cysts of the Iris.”
American Journal of Ophthalmology. 34 (6). March, 1951.
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Pearl Cysts
• 3 stages have been proposed
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Stage 1: quiet stage. Slow progression.
Stage 2: pain, redness, photophobia. Iritis.
Stage 3: elevated IOP with enlargement of
cyst.
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Courtesy of Richard Forster, MD
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Primary Iris Cysts
• Very rare, in absence of h/o trauma or surgery
• Majority of cases described in children, usually
detected in 1st year of life
• Surgery often necessary to prevent amblyopia
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Primary iris cysts
• 1998, Lois et al. described a case series
of 17 patients with primary iris cysts
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All unilateral
9 pts. under age 10 (52%), 8 (47%) over
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2/9 under 10 with h/o prior amniocenteses
Required treatment: 8/9 < 10 years, 2/8 >
10 y.
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Pathogenesis of Congenital Iris Cysts
• Widely believed to be secondary to
entrapment of surface epithelium/ectoderm
within the eye during lens invagination
•
Traction of the zonules on ciliary epithelium 
faulty apposition of outer and inner layers of optic
cup?
• Proliferation w/in the neuroepithelial layer?
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Histopathology
• Lined by multilayered stratified squamous to cuboidal
epithelium with or without goblet cells
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Keratinization reported
Corneal endothelial cells have been found covering the cyst
wall
• Similar appearance regardless of origin
Primary Cyst
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Implantation cyst
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Pearl Cyst
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Ultrasound Findings
Marigo FA and Finger PT. “Anterior segment tumors: current concepts and innovations.” Surv
of Ophthalmology. 48(6), 569-593. Nov. – Dec, 2003.
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Complications Arising from Iris
Cysts
• Amblyopia
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Corneal decompensation/band keratopathy
Iritis
Glaucoma
Cataract
Hyphema
• Spontaneous rupture or rupture during
surgery may result in epithelialization of the
AC
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Uveitis and Iris Cysts
• Approximately 1% of iris cysts cause an
anterior uveitis
• Likely secondary to release of mucous
or protein debris with subsequent
inflammatory response
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Spontaneous
Traumatic leak
• When severe, may block trabecular
meshwork  “mucogenic glaucoma”
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Treatment Options
• Injections into cyst: iodine, radioactive sulfur,
trichloroacetic acid, ethanol irrigation
• Cyst drainage
• Laser: argon or xenon, endolaser
• Diathermy
• Cryotherapy
• Marsupialization
• Partial or total resection
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Treatment
• Lois et al, 17 patient case series
•
5/9pts. < 10 years treated with aspiration +
cryotherapy

2/5 eventually required excision
•
3/9 were excised initially
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2/8 > 10 years treated with argon laser

1/2 required additional aspiration
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Treatment
• 1993, Capo reported on the treatment of
3 cases of congenital iris cysts
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Case 1: cyst aspirated, injected with TCA

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Case 2: Xenon laser, repeat laser,
aspiration, repeat drainage, cryotherapy
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AC decompensation, glaucoma, RD 
enucleation
Band keratopathy, glaucoma, corneal ectasia
 enucleation
Case 3: aspiration, viscodissection,
iridectomy

No recurrence after 2 years f/u
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Argon Laser Photocoagulation
• 5 patient case series treating implantation iris
cysts
• Laser applied to cyst margins initially
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•
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When consolidated, laser applied directly
100 – 500 um, 0.1 – 0.2 sec, 200 – 750 mW
1 to 5 treatment sessions
• 3/5 cysts resolved
• 1/5 cysts shrunk with remnant
• 1/5 required surgical excision
Sugar J, Jampol LM, Goldberg MF. “Argon laser destruction of anterior chamber
implantation cysts.” Ophthalmology. 91 (9), 1040 – 1044. Sept, 1984.
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Block Excision
• 1995, Forster reported on 3 cases of
post-extracapsular iris cysts treated with
block excision followed by corneoscleral
graft.
• No cases with recurrence at f/u (range 7
– 26 months)
• VA ranged from 20/20 to 20/30
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Varying Approaches
• 2003, Haller et al described 7 patients
with iris cysts
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5 Post-traumatic, 1 post-operative, and 1
congenital
3/7 treated aggressively

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Excision of cyst + iris, cryoablation of excision
site
4/7 treated conservatively
Viscodissection, aspiration, endolaser
 1/4 required additional excision

•
Post-operative VA slightly better in 2nd
group
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Haller JA et al. “Surgical management of anterior chamber epithelial cysts.”
American Journal of Ophthalmology. 135(3), 309 – 313. March, 2003.
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Cyst Prolapse and Diathermy
• 2006, Shen et al. reported on 4 patients
with congenital iris cysts treated
• Using blunt dissection and viscoelastic,
the cysts were prolapsed through a
limbal incision and excised at their base
• Microdiathermy was then applied to the
base
• VA remained stable and no recurrences
were noted on f/u (range 1.4 to 6.2
years)
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Back to Our Patient
• BPEI ER – mild iritis, Pred Forte QID
• 5 day f/u – discomfort improved
• 1 month f/u – redness, discomfort
resolved. Cont. to c/o blurry vision.
• Scheduled to f/u in 3 months
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Endothelial Cell Count
OD: Konan Non-Contact Specular Microscopy = 968 cells/mm²
OD: Confoscan 3 Contact Microscopy = 1530 cells/mm²
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Abstract
•
Title: “Please, I Insyst”
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Diagnosis: Iris Cyst
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Key Words: iris cyst, pearl cyst, primary cyst, congenital cyst,
secondary cyst implantation cyst, iritis
•
Abstract: A 34 yo female presented to the BPEI ER with complaints of
discomfort, redness, photophobia, and a “white spot” OD. Best
corrected VA was 20/20 OD and 20/20 OS. Anterior segment exam
revealed mild injection, trace cell, and a whitish iris mass OD.
Examination OS was unremarkable. Dilated exam OU was
unremarkable. The patient was given Pred Forte drops 4 times per day
OD. On follow-up, an anterior chamber B-scan proved the mass to be
cystic in nature. Anterior chamber OCT revealed the close proximity to
the corneal endothelium. Spectral microscopy showed a slightly low
endothelial cell count. However, there is no obvious corneal edema or
evidence of corneal decompensation on exam. Slit-lamp photographs
were taken and the patient will be followed serially for the time being.
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References
1. Capo H, Palmer E, and Nicholson DH. “Congenital cysts of the iris
stroma.” American Journal of Ophthalmology. 116(2), 228 – 232.
Aug, 1993.
2. Conway RM et al. “Ultrasound biomicroscopy: role in diagnosis and
management in 130 consecutive patients evaluated for anterior
segment tumours.” The British Journal of Ophthalmology. 89, 950 –
955. 2005.
3. Forster RK. “Corneoscleral block excision of postoperative anterior
chamber cysts.” Trans Am Ophthalmol Soc. 93, 83 – 97. 1995.
4. Grutzmacher RD et al. “Congenital iris cysts.” The British Journal of
Ophthalmology. 71(3), 227 – 234. March, 1987.
5. Haller JA et al. “Surgical management of anterior chamber epithelial
cysts.” American Journal of Ophthalmology. 135(3), 309 – 313.
March, 2003.
6. Lois N et al. “Primary iris stromal cysts.” Ophthalmology. 105(7), 1317
– 1322. July, 1998.
7. Rosenthal G. “Congenital Cysts of the Iris Stroma.” Archives of
Ophthalmology. 116, 1696. Dec, 1998.
8. Shen CC et al. “Management of Congenital Nonpigmented Iris Cyst.”
Ophthalmology. 113(9), 1639.e1 – 1639.e7. Sept, 2006.
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References
9. Shields JA, Kline MW, Augsberger JJ. “Primary iris cysts: a review of
the literature and report of 62 cases.” The British Journal of
Ophthalmology. 68(3), 152 – 166. March, 1984.
10. Shin SY, Stark WJ, Haller J, Green WR. “Surgical management of
recurrent iris stromal cyst.” American Journal of Ophthalmology.
130(1), 122-123. July, 2000.
11. Sitchevska O and Payne BF. “Pearl Cysts of the Iris.” American
Journal of Ophthalmology. 34(6), 833 – 840. July, 1951.
12. Sugar J, Jampol LM, and Goldberg MF. “Argon laser destruction of
anterior chamber implantation cysts.” Ophthalmology. 91(9), 1040 –
1044. Sept, 1984.
13. Tulvatana W et al. “Free Keratin and Dermoid Cyst of the Iris.”
Archives of Ophthalmology. 123, 402 – 403. March, 2005.
14. Yung R and Eiferman RA. “Spontaneous iris stromal cyst: a case
report and review of literature.” Annals of Ophthalmology. 24(4),
139 – 142. April, 1992.
15. Zhou M et al. “Differential diagnosis of anterior chamber cysts with
ultrasound biomicroscopy: ciliary body medulloepithelioma.” Acta
Ophthalmologica Scandinavica. 84, 137 – 139. 2006.
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Special Thanks
• Sander Dubovy, MD
• Lejla Mutapcic, MD
• Alex Gutierrez
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