bascom palmer eye institute grand rounds kyle alliman, md richard forster, md september 28, 2006

Bascom Palmer Eye InstituteBascom Palmer Eye InstituteGrand RoundsGrand Rounds

U N I V E R S I T Y O F

S C H O O L O F M E D IC IN E

Kyle Alliman, MDKyle Alliman, MD

Richard Forster, MDRichard Forster, MD

September 28, 2006



Case PresentationCase Presentation

• 34 yo female presents to BPEI ER with 34 yo female presents to BPEI ER with redness, discomfort, photophobia, and redness, discomfort, photophobia, and blurry vision OD for 3 days.blurry vision OD for 3 days.

• Seen at BPEI 1 ½ years ago with Seen at BPEI 1 ½ years ago with contact lens intolerance/overwear. contact lens intolerance/overwear. Since then, uses contacts sparingly.Since then, uses contacts sparingly.



• PMHxPMHx• 2 months post-partum2 months post-partum

• PSHxPSHx• s/p C-Section x 2 (1991, 2006)s/p C-Section x 2 (1991, 2006)

• POHxPOHx• h/o myopiah/o myopia• No h/o ocular trauma or surgeryNo h/o ocular trauma or surgery• occasionally uses Elestat for occasionally uses Elestat for

redness/allergiesredness/allergies• noticed “white spot” in right eye since age noticed “white spot” in right eye since age

1212



• MedicationsMedications• multivitaminsmultivitamins

• AllergiesAllergies• NKDANKDA

• SHSH• Lives in MiamiLives in Miami• Works in salesWorks in sales• Denies tob, IVDA. Occasional EtOHDenies tob, IVDA. Occasional EtOH

• FHxFHx• Father with HTN, mother with DM, HTN.Father with HTN, mother with DM, HTN.



• Healthy-appearing female in NADHealthy-appearing female in NAD• BP 128/73, P 77, R 16, T 98.2BP 128/73, P 77, R 16, T 98.2• VAVAcccc

• 20/20 20/20 • 20/20 20/20

• RxRx• OD: -5.25 + 1.25 x 095OD: -5.25 + 1.25 x 095• OS: -5.50 + 0.25 x 065OS: -5.50 + 0.25 x 065

• Ocular MotilityOcular Motility• Orthophoria, full ductions OUOrthophoria, full ductions OU

Initial ExaminationInitial Examination



• Adnexae/FaceAdnexae/Face• WNLWNL

• Visual fieldsVisual fields• Full to confrontation OUFull to confrontation OU

• PupilsPupils• 4 mm 4 mm → 2mm, slightly irregular, no APD OD→ 2mm, slightly irregular, no APD OD• 4 mm 4 mm → 2 mm, no APD OS→ 2 mm, no APD OS

• IOPIOP• 17 mm Hg OD17 mm Hg OD• 14 mm Hg OS14 mm Hg OS

Initial ExaminationInitial Examination



SLE OUSLE OU

• ODOD • Trace injectionTrace injection• Trace cellTrace cell

• See PhotosSee Photos

• OSOS• Conj: W/QConj: W/Q• Cornea: clearCornea: clear• AC: D/QAC: D/Q• Iris: wnlIris: wnl• Lens: clearLens: clear

DFE – wnl OU



Differential Diagnosis?Differential Diagnosis?• Iris cystIris cyst

• Primary Primary • SecondarySecondary

• Amelanotic iris melanomaAmelanotic iris melanoma• Iris nevusIris nevus• Retained FB/FB granulomaRetained FB/FB granuloma• Metastasis to iris/ciliary bodyMetastasis to iris/ciliary body• MedulloepitheliomaMedulloepithelioma• Juvenile xanthogranulomaJuvenile xanthogranuloma• Leukemia/LymphomaLeukemia/Lymphoma• LeiomyomaLeiomyoma• Tapioca melanomaTapioca melanoma• DermoidDermoid• Cogan-Reese/ICECogan-Reese/ICE• Other iris nodules: Lisch, Brushfield spots, Koeppe/BusaccaOther iris nodules: Lisch, Brushfield spots, Koeppe/Busacca



AC UltrasonographyAC Ultrasonography



Anterior Chamber OCTAnterior Chamber OCT



Iris CystsIris Cysts• Rare, outpouchings or deviations of the Rare, outpouchings or deviations of the

iris epithelium or stromairis epithelium or stroma

• 2 main varieties:2 main varieties:• Primary/Spontaneous/CongenitalPrimary/Spontaneous/Congenital• SecondarySecondary

Paintings by Mr. Myers from Berliner



Formal ClassificationsFormal Classifications• Primary cystsPrimary cysts

• Of the iris pigment epitheliumOf the iris pigment epithelium Central (pupillary margin) Central (pupillary margin) MidzonalMidzonal Peripheral (iridociliary)Peripheral (iridociliary)

• Of the iris stromaOf the iris stroma Congenital (children)Congenital (children) Spontaneous (adults)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.



Formal ClassificationsFormal Classifications• Secondary cystsSecondary cysts

• Epithelial downgrowth cysts Epithelial downgrowth cysts Post-surgicalPost-surgical Post-traumaticPost-traumatic

• Pearl cystsPearl cysts• Drug-induced cystsDrug-induced cysts

Cholinergics (pilocarpine, echothiophate, etc.)Cholinergics (pilocarpine, echothiophate, etc.) LatanoprostLatanoprost

• Secondary to intraocular tumorsSecondary to intraocular tumors MedulloepitheliomaMedulloepithelioma Uveal melanomaUveal melanoma

• ParasiticParasiticShields 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.



Secondary CystsSecondary Cysts• Post-SurgicalPost-Surgical

• Post-operative epithelial invasion of the AC results in Post-operative epithelial invasion of the AC results in epithelial downgrowthepithelial downgrowth

• Rarely, downgrowth leads to cyst formationRarely, downgrowth leads to cyst formation Cysts more amenable to treatment than diffuse Cysts more amenable to treatment than diffuse

downgrowthdowngrowth

• Post-traumatic – similar to post-surgicalPost-traumatic – similar to post-surgical• With penetrating injury, epithelium introduced into ACWith penetrating injury, epithelium introduced into AC



Haller JA et al. “Surgical management of anterior chamber epithelial cysts.” Haller JA et al. “Surgical management of anterior chamber epithelial cysts.” American Journal of Ophthalmology.American Journal of Ophthalmology. 135(3), 309 – 313. March, 2003. 135(3), 309 – 313. March, 2003.



Pearl CystsPearl Cysts• Historically, associated with traumaHistorically, associated with trauma• Caused by displaced conjunctival or Caused by displaced conjunctival or

epidermal epitheliumepidermal epithelium• May cause recurrent iritisMay cause recurrent iritis• If cyst ruptures, a secondary mucogenic If cyst ruptures, a secondary mucogenic

glaucoma may resultglaucoma may result

photos from www.eyecancer.com



Pearl CystsPearl Cysts

• Thought to occur with introduction of cilia + Thought to occur with introduction of cilia + epithelium into the ACepithelium into the AC

• 1872, Goldzieher introduced pieces of 1872, Goldzieher introduced pieces of conjunctiva, nasal mucosa, cornea, and conjunctiva, nasal mucosa, cornea, and peripheral nerve tissue into the ACperipheral nerve tissue into the AC• Iris cyst from nasal mucosaIris cyst from nasal mucosa

• 1888, Masse placed various tissues, 1888, Masse placed various tissues, including epithelium into the ACincluding epithelium into the AC• All but epithelium became absorbedAll but epithelium became absorbed

Sitchevska O and Payne BF. “Pearl Cysts of the Iris.” American Journal of Ophthalmology. 34 (6). March, 1951.



Pearl CystsPearl Cysts• 3 stages have been proposed3 stages have been proposed

• Stage 1: quiet stage. Slow progression.Stage 1: quiet stage. Slow progression.• Stage 2: pain, redness, photophobia. Iritis.Stage 2: pain, redness, photophobia. Iritis.• Stage 3: elevated IOP with enlargement of Stage 3: elevated IOP with enlargement of

cyst.cyst.

Courtesy of Richard Forster, MD



Primary Iris CystsPrimary Iris Cysts• Very rare, in absence of h/o trauma or surgeryVery rare, in absence of h/o trauma or surgery• Majority of cases described in children, usually Majority of cases described in children, usually

detected in 1detected in 1stst year of life year of life• Surgery often necessary to prevent amblyopiaSurgery often necessary to prevent amblyopia



Primary iris cystsPrimary iris cysts

• 1998, Lois et al. described a case 1998, Lois et al. described a case series of 17 patients with primary iris series of 17 patients with primary iris cystscysts• All unilateralAll unilateral• 9 pts. under age 10 (52%), 8 (47%) over9 pts. under age 10 (52%), 8 (47%) over

2/9 under 10 with h/o prior amniocenteses2/9 under 10 with h/o prior amniocenteses

• Required treatment: 8/9 < 10 years, 2/8 > Required treatment: 8/9 < 10 years, 2/8 > 10 y.10 y.



Pathogenesis of Congenital Iris CystsPathogenesis of Congenital Iris Cysts• Widely believed to be secondary to Widely believed to be secondary to

entrapment of surface epithelium/ectoderm entrapment of surface epithelium/ectoderm within the eye during lens invaginationwithin the eye during lens invagination• Traction of the zonules on ciliary epithelium Traction of the zonules on ciliary epithelium

faulty apposition of outer and inner layers of optic faulty apposition of outer and inner layers of optic cup?cup?

• Proliferation w/in the neuroepithelial layer?Proliferation w/in the neuroepithelial layer?



HistopathologyHistopathology• Lined by multilayered stratified squamous to cuboidal Lined by multilayered stratified squamous to cuboidal

epithelium with or without goblet cellsepithelium with or without goblet cells• Keratinization reportedKeratinization reported• Corneal endothelial cells have been found covering the cyst Corneal endothelial cells have been found covering the cyst

wallwall

• Similar appearance regardless of originSimilar appearance regardless of origin

Primary Cyst



Implantation cyst



Pearl Cyst



Ultrasound Findings

Marigo FA and Finger PT. “Anterior segment tumors: current concepts and innovations.” Survey of Ophthalmology. 48(6), 569-593. Nov. – Dec, 2003.



Complications Arising from Iris Complications Arising from Iris CystsCysts

• AmblyopiaAmblyopia

• Corneal decompensation/band keratopathyCorneal decompensation/band keratopathy• IritisIritis• Glaucoma Glaucoma • CataractCataract• HyphemaHyphema

• Spontaneous rupture or rupture during surgery Spontaneous rupture or rupture during surgery may result in epithelialization of the ACmay result in epithelialization of the AC



Uveitis and Iris CystsUveitis and Iris Cysts

• Approximately 1% of iris cysts cause an Approximately 1% of iris cysts cause an anterior uveitisanterior uveitis

• Likely secondary to release of mucous Likely secondary to release of mucous or protein debris with subsequent or protein debris with subsequent inflammatory responseinflammatory response• SpontaneousSpontaneous• Traumatic leakTraumatic leak

• When severe, may block trabecular When severe, may block trabecular meshwork meshwork “mucogenic glaucoma” “mucogenic glaucoma”



Treatment OptionsTreatment Options

• Injections into cyst: iodine, radioactive sulfur, Injections into cyst: iodine, radioactive sulfur, trichloroacetic acid, ethanol irrigationtrichloroacetic acid, ethanol irrigation

• Cyst drainageCyst drainage• Laser: argon or xenon, endolaserLaser: argon or xenon, endolaser• DiathermyDiathermy• CryotherapyCryotherapy• MarsupializationMarsupialization• Partial or total resectionPartial or total resection



TreatmentTreatment

• Lois et al, 17 patient case seriesLois et al, 17 patient case series• 5/9pts. < 10 years treated with aspiration + 5/9pts. < 10 years treated with aspiration +

cryotherapycryotherapy 2/5 eventually required excision2/5 eventually required excision

• 3/9 were excised initially3/9 were excised initially

• 2/8 > 10 years treated with argon laser2/8 > 10 years treated with argon laser 1/2 required additional aspiration1/2 required additional aspiration



TreatmentTreatment• 1993, Capo reported on the treatment of 3 1993, Capo reported on the treatment of 3

cases of congenital iris cystscases of congenital iris cysts• Case 1: cyst aspirated, injected with TCACase 1: cyst aspirated, injected with TCA

AC decompensation, glaucoma, RD AC decompensation, glaucoma, RD enucleation enucleation• Case 2: Xenon laser, repeat laser, aspiration, Case 2: Xenon laser, repeat laser, aspiration,

repeat drainage, cryotherapyrepeat drainage, cryotherapy Band keratopathy, glaucoma, corneal ectasia Band keratopathy, glaucoma, corneal ectasia

enucleationenucleation• Case 3: aspiration, viscodissection, iridectomyCase 3: aspiration, viscodissection, iridectomy

No recurrence after 2 years f/uNo recurrence after 2 years f/u



Argon Laser PhotocoagulationArgon Laser Photocoagulation

• 5 patient case series treating implantation iris 5 patient case series treating implantation iris cystscysts

• Laser applied to cyst margins initiallyLaser applied to cyst margins initially• When consolidated, laser applied directlyWhen consolidated, laser applied directly• 100 – 500 um, 0.1 – 0.2 sec, 200 – 750 mW100 – 500 um, 0.1 – 0.2 sec, 200 – 750 mW• 1 to 5 treatment sessions1 to 5 treatment sessions

• 3/5 cysts resolved3/5 cysts resolved• 1/5 cysts shrunk with remnant1/5 cysts shrunk with remnant• 1/5 required surgical excision1/5 required surgical excision

Sugar J, Jampol LM, Goldberg MF. “Argon laser destruction of anterior chamber implantation cysts.” Ophthalmology. 91 (9), 1040 – 1044. Sept, 1984.



Block ExcisionBlock Excision

• 1995, Forster reported on 3 cases of 1995, Forster reported on 3 cases of post-extracapsular iris cysts treated with post-extracapsular iris cysts treated with block excision followed by corneoscleral block excision followed by corneoscleral graft.graft.

• No cases with recurrence at f/u (range 7 No cases with recurrence at f/u (range 7 – 26 months)– 26 months)

• VA ranged from 20/20 to 20/30VA ranged from 20/20 to 20/30



Varying ApproachesVarying Approaches• 2003, Haller et al described 7 patients 2003, Haller et al described 7 patients

with iris cystswith iris cysts• 5 Post-traumatic, 1 post-operative, and 1 5 Post-traumatic, 1 post-operative, and 1

congenitalcongenital• 3/7 treated aggressively3/7 treated aggressively

Excision of cyst + iris, cryoablation of excision Excision of cyst + iris, cryoablation of excision sitesite

• 4/7 treated conservatively4/7 treated conservatively Viscodissection, aspiration, endolaserViscodissection, aspiration, endolaser 1/4 required additional excision1/4 required additional excision

• Post-operative VA slightly better in 2Post-operative VA slightly better in 2ndnd group group



Haller JA et al. “Surgical management of anterior chamber epithelial cysts.” Haller JA et al. “Surgical management of anterior chamber epithelial cysts.” American Journal of Ophthalmology.American Journal of Ophthalmology. 135(3), 309 – 313. March, 2003. 135(3), 309 – 313. March, 2003.



Cyst Prolapse and DiathermyCyst Prolapse and Diathermy

• 2006, Shen et al. reported on 4 patients 2006, Shen et al. reported on 4 patients with congenital iris cysts treatedwith congenital iris cysts treated

• Using blunt dissection and viscoelastic, Using blunt dissection and viscoelastic, the cysts were prolapsed through a limbal the cysts were prolapsed through a limbal incision and excised at their baseincision and excised at their base

• Microdiathermy was then applied to the Microdiathermy was then applied to the basebase

• VA remained stable and no recurrences VA remained stable and no recurrences were noted on f/u (range 1.4 to 6.2 years)were noted on f/u (range 1.4 to 6.2 years)



Back to Our PatientBack to Our Patient

• BPEI ER – mild iritis, Pred Forte QIDBPEI ER – mild iritis, Pred Forte QID

• 5 day f/u – discomfort improved5 day f/u – discomfort improved

• 1 month f/u – redness, discomfort 1 month f/u – redness, discomfort resolved. Cont. to c/o blurry vision.resolved. Cont. to c/o blurry vision.

• Scheduled to f/u in 3 monthsScheduled to f/u in 3 months



Endothelial Cell CountEndothelial Cell Count

OD: Konan Non-Contact Specular Microscopy = 968 cells/mm²

OD: Confoscan 3 Contact Microscopy = 1530 cells/mm²



AbstractAbstract• Title: “Please, I Insyst”Title: “Please, I Insyst”

• Diagnosis: Iris CystDiagnosis: Iris Cyst

• Key Words: iris cyst, pearl cyst, primary cyst, congenital cyst, Key Words: iris cyst, pearl cyst, primary cyst, congenital cyst, secondary cyst implantation cyst, iritissecondary cyst implantation cyst, iritis

• Abstract: A 34 yo female presented to the BPEI ER with complaints of Abstract: A 34 yo female presented to the BPEI ER with complaints of discomfort, redness, photophobia, and a “white spot” OD. Best discomfort, redness, photophobia, and a “white spot” OD. Best corrected VA was 20/20 OD and 20/20 OS. Anterior segment exam corrected VA was 20/20 OD and 20/20 OS. Anterior segment exam revealed mild injection, trace cell, and a whitish iris mass OD. revealed mild injection, trace cell, and a whitish iris mass OD. Examination OS was unremarkable. Dilated exam OU was Examination OS was unremarkable. Dilated exam OU was unremarkable. The patient was given Pred Forte drops 4 times per day 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 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 cystic in nature. Anterior chamber OCT revealed the close proximity to the corneal endothelium. Spectral microscopy showed a slightly low the corneal endothelium. Spectral microscopy showed a slightly low endothelial cell count. However, there is no obvious corneal edema or endothelial cell count. However, there is no obvious corneal edema or evidence of corneal decompensation on exam. Slit-lamp photographs evidence of corneal decompensation on exam. Slit-lamp photographs were taken and the patient will be followed serially for the time being.were taken and the patient will be followed serially for the time being.



ReferencesReferences1. Capo H, Palmer E, and Nicholson DH. “Congenital cysts of the iris stroma.” 1. Capo H, Palmer E, and Nicholson DH. “Congenital cysts of the iris stroma.”

American Journal of Ophthalmology.American Journal of Ophthalmology. 116(2), 228 – 232. Aug, 1993. 116(2), 228 – 232. Aug, 1993. 2. Conway RM et al. “Ultrasound biomicroscopy: role in diagnosis and 2. Conway RM et al. “Ultrasound biomicroscopy: role in diagnosis and

management in 130 consecutive patients evaluated for anterior segment management in 130 consecutive patients evaluated for anterior segment tumours.” tumours.” The British Journal of Ophthalmology.The British Journal of Ophthalmology. 89, 950 – 955. 2005. 89, 950 – 955. 2005.

3. Forster RK. “Corneoscleral block excision of postoperative anterior chamber 3. Forster RK. “Corneoscleral block excision of postoperative anterior chamber cysts.” cysts.” Trans Am Ophthalmol Soc.Trans Am Ophthalmol Soc. 93, 83 – 97. 1995. 93, 83 – 97. 1995.

4. Grutzmacher RD et al. “Congenital iris cysts.” 4. Grutzmacher RD et al. “Congenital iris cysts.” The British Journal of The British Journal of Ophthalmology. Ophthalmology. 71(3), 227 – 234. March, 1987.71(3), 227 – 234. March, 1987.

5. Haller JA et al. “Surgical management of anterior chamber epithelial cysts.” 5. Haller JA et al. “Surgical management of anterior chamber epithelial cysts.” American Journal of Ophthalmology.American Journal of Ophthalmology. 135(3), 309 – 313. March, 2003. 135(3), 309 – 313. March, 2003.

6. Lois N et al. “Primary iris stromal cysts.” 6. Lois N et al. “Primary iris stromal cysts.” Ophthalmology.Ophthalmology. 105(7), 1317 – 105(7), 1317 – 1322. July, 1998.1322. July, 1998.

7. Rosenthal G. “Congenital Cysts of the Iris Stroma.” 7. Rosenthal G. “Congenital Cysts of the Iris Stroma.” Archives of Archives of Ophthalmology.Ophthalmology. 116, 1696. Dec, 1998. 116, 1696. Dec, 1998.

8. Shen CC et al. “Management of Congenital Nonpigmented Iris Cyst.” 8. Shen CC et al. “Management of Congenital Nonpigmented Iris Cyst.” Ophthalmology.Ophthalmology. 113(9), 1639.e1 – 1639.e7. Sept, 2006. 113(9), 1639.e1 – 1639.e7. Sept, 2006.



ReferencesReferences9. Shields JA, Kline MW, Augsberger JJ. “Primary iris cysts: a review of the 9. Shields JA, Kline MW, Augsberger JJ. “Primary iris cysts: a review of the

literature and report of 62 cases.” literature and report of 62 cases.” The British Journal of Ophthalmology.The British Journal of Ophthalmology. 68(3), 152 – 166. March, 1984.68(3), 152 – 166. March, 1984.

10. Shin SY, Stark WJ, Haller J, Green WR. “Surgical management of recurrent 10. Shin SY, Stark WJ, Haller J, Green WR. “Surgical management of recurrent iris stromal cyst.” iris stromal cyst.” American Journal of Ophthalmology.American Journal of Ophthalmology. 130(1), 122-123. 130(1), 122-123. July, 2000.July, 2000.

11. Sitchevska O and Payne BF. “Pearl Cysts of the Iris.” 11. Sitchevska O and Payne BF. “Pearl Cysts of the Iris.” American Journal of American Journal of Ophthalmology.Ophthalmology. 34(6), 833 – 840. July, 1951. 34(6), 833 – 840. July, 1951.

12. Sugar J, Jampol LM, and Goldberg MF. “Argon laser destruction of anterior 12. Sugar J, Jampol LM, and Goldberg MF. “Argon laser destruction of anterior chamber implantation cysts.” chamber implantation cysts.” Ophthalmology.Ophthalmology. 91(9), 1040 – 1044. Sept, 91(9), 1040 – 1044. Sept, 1984. 1984.

13. Tulvatana W et al. “Free Keratin and Dermoid Cyst of the Iris.” 13. Tulvatana W et al. “Free Keratin and Dermoid Cyst of the Iris.” Archives of Archives of OphthalmologyOphthalmology. 123, 402 – 403. March, 2005.. 123, 402 – 403. March, 2005.

14. Yung R and Eiferman RA. “Spontaneous iris stromal cyst: a case report and 14. Yung R and Eiferman RA. “Spontaneous iris stromal cyst: a case report and review of literature.” review of literature.” Annals of Ophthalmology.Annals of Ophthalmology. 24(4), 139 – 142. April, 24(4), 139 – 142. April, 1992.1992.

15. Zhou M et al. “Differential diagnosis of anterior chamber cysts with 15. Zhou M et al. “Differential diagnosis of anterior chamber cysts with ultrasound biomicroscopy: ciliary body medulloepithelioma.” ultrasound biomicroscopy: ciliary body medulloepithelioma.” Acta Acta Ophthalmologica Scandinavica.Ophthalmologica Scandinavica. 84, 137 – 139. 2006. 84, 137 – 139. 2006.



Special ThanksSpecial Thanks

• Sander Dubovy, MDSander Dubovy, MD

• Lejla Mutapcic, MDLejla Mutapcic, MD

• Alex GutierrezAlex Gutierrez

bascom palmer eye institute grand rounds kyle alliman, md richard forster, md september 28, 2006

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