case presentation - uveitis · patient presentation cc: fixed, dilated pupil after penetrating...

Case PresentationCase Presentation

Peter Chang, M.D.Peter Chang, M.D.Research FellowResearch Fellow

Massachusetts Eye Research Massachusetts Eye Research and Surgery Institutionand Surgery Institution

9/5/20089/5/2008

Patient PresentationPatient Presentation

CC: Fixed, dilated pupil after penetrating CC: Fixed, dilated pupil after penetrating keratoplasty for scarred cornea, OSkeratoplasty for scarred cornea, OSHPI: 45yo female presents with a fixed, dilated HPI: 45yo female presents with a fixed, dilated left pupil, associated with significant left pupil, associated with significant photosensitivity OS, 5 days after PK for her photosensitivity OS, 5 days after PK for her extensively scarred left cornea. The patient had extensively scarred left cornea. The patient had a history of herpectic keratitis in the left eye a history of herpectic keratitis in the left eye which caused severe scarring, rendering poor which caused severe scarring, rendering poor VA at 20/300. Therefore, PK was done.VA at 20/300. Therefore, PK was done.

Patient Presentation (cont)Patient Presentation (cont)

The transplant was successful without The transplant was successful without complications. Topical steroids and complications. Topical steroids and antibiotics were used both preantibiotics were used both pre--op and op and postpost--op. No mydriatic was used. Both op. No mydriatic was used. Both continuous and interrupted sutures were continuous and interrupted sutures were employed to center the graft.employed to center the graft.


On POD1, Pt presented with conjunctival On POD1, Pt presented with conjunctival injection 2+, corneal graft edema 2+, AC injection 2+, corneal graft edema 2+, AC cell 2+, PERRLA; no wound leakage. VA cell 2+, PERRLA; no wound leakage. VA 20/150, IOP 19. She was maintained on 20/150, IOP 19. She was maintained on Pred Forte q2h and Zymar QID.Pred Forte q2h and Zymar QID.


On POD7, Pt returned with a complaint of On POD7, Pt returned with a complaint of increasing light sensitivity and 8/10 pain, OS. increasing light sensitivity and 8/10 pain, OS. She also noticed in the mirror that her left pupil She also noticed in the mirror that her left pupil had been dilated over the past 2 days.had been dilated over the past 2 days.Exam findings:Exam findings:–– VA 20/125 (20/100ph)VA 20/125 (20/100ph)–– IOP 46 mmHg: drops were inititatedIOP 46 mmHg: drops were inititated–– Conj injection 1+, graft edema 1+, AC cell 1+Conj injection 1+, graft edema 1+, AC cell 1+–– OS pupil diameter was fixed at 8mm, while OD pupil OS pupil diameter was fixed at 8mm, while OD pupil

was PERRLAwas PERRLA–– Wound intact, all sutures buriedWound intact, all sutures buried


On POD8, Pt returned with persistent light On POD8, Pt returned with persistent light sensitivity, though the pain was sensitivity, though the pain was considerably better. Her vision remained considerably better. Her vision remained blurry, and her pupil was still fixed and blurry, and her pupil was still fixed and dilated.dilated.Exam findings:Exam findings:–– Same as POD7, except OS IOP is down to 30 Same as POD7, except OS IOP is down to 30

mmHg: antihypertensive regimen was mmHg: antihypertensive regimen was continued.continued.


POD14: Pt returned with slightly better POD14: Pt returned with slightly better light sensitivity and mild pain. All the postlight sensitivity and mild pain. All the post--operative parameters were improving. The operative parameters were improving. The only exception was the fixed, dilated pupil only exception was the fixed, dilated pupil in the left eye, unresponsive to pilocarpine. in the left eye, unresponsive to pilocarpine. Steroid taper began.Steroid taper began.Exam findings:Exam findings:–– VA 20/100 (20/80ph)VA 20/100 (20/80ph)–– IOP 17mmHgIOP 17mmHg

What is with that Pupil? What is with that Pupil? (Differentials)(Differentials)

DilatationDilatation–– Sympathetic (excitement, fear)Sympathetic (excitement, fear)–– AntiAnti--parasympathetic (ex. drugs)parasympathetic (ex. drugs)–– Midbrain damage (ex. grand mal)Midbrain damage (ex. grand mal)–– Increased IOP (iris ischemia)Increased IOP (iris ischemia)

What is with that Pupil? (cont)What is with that Pupil? (cont)ConstrictionConstriction–– Automonic nervous sytem (sleep)Automonic nervous sytem (sleep)–– NarcoticsNarcotics–– Pilocarpine/phospholine iodidePilocarpine/phospholine iodide–– Pontine hemorrhagePontine hemorrhage–– AgingAging

Irregular shape/sizeIrregular shape/size–– TraumaTrauma −− AniridiaAniridia–– InflammationInflammation −− s/p CE (most common)s/p CE (most common)–– NeovascularizationNeovascularization −− Tadpole pupil (migraine)Tadpole pupil (migraine)–– ColobomaColoboma −− Midbrain damageMidbrain damage

UrretsUrrets--Zavalia Syndrome (UZS)Zavalia Syndrome (UZS)

An uncommon postAn uncommon post--operative complication operative complication in which a pupil remains in which a pupil remains fixed and dilatedfixed and dilated, , accompanied by accompanied by iris atrophyiris atrophy and and occasionally occasionally secondary glaucomasecondary glaucoma. These . These pupils are pupils are unresponsive to miotic agentsunresponsive to miotic agents..The exact features are in fact quite The exact features are in fact quite variable among published reports/series.variable among published reports/series.

HistoryHistory

First described by Dr. UrretsFirst described by Dr. Urrets--Zavalia in Zavalia in 1963, after PK for keratoconus. He 1963, after PK for keratoconus. He associated that with the postassociated that with the post--op mydriatic op mydriatic treatment. He attributed this discovery to treatment. He attributed this discovery to Dr. Castroviejo in the 1940s.Dr. Castroviejo in the 1940s.–– Dr. UDr. U--Z: 6 patients had the syndrome for up to Z: 6 patients had the syndrome for up to

6 weeks, but all resolved spontaneously.6 weeks, but all resolved spontaneously.–– In 1967, Uribe et al described the syndrome in In 1967, Uribe et al described the syndrome in

PK patients without postPK patients without post--op mydratics.op mydratics.

HistoryHistory

Classically linked to PK for keratoconusClassically linked to PK for keratoconusSubsequent reports suggest that UZS can Subsequent reports suggest that UZS can also be seen after: also be seen after: –– Deep lamellar keratoplasty for keratoconusDeep lamellar keratoplasty for keratoconus–– Descemet stripping endothelial keratoplasty for Descemet stripping endothelial keratoplasty for

FuchFuch’’s endothelial dystrophys endothelial dystrophy–– Argon laser peripheral iridoplastyArgon laser peripheral iridoplasty–– Surgical trabeculectomySurgical trabeculectomy–– Phakic ACIOL implantationPhakic ACIOL implantation

EpidemiologyEpidemiology

Incidence: 2.2% Incidence: 2.2% –– 17.7%17.7%This seems to be decreased in the recent This seems to be decreased in the recent years; some even question its continued years; some even question its continued existence.existence.Likely due to improved surgical techniques Likely due to improved surgical techniques and/or different diagnostic standards.and/or different diagnostic standards.

Variable Degrees of DilatationsVariable Degrees of Dilatations

Typically, 3 types of pupil dilatations can Typically, 3 types of pupil dilatations can be seen in UZS:be seen in UZS:–– At least 1.5mm larger than the fellow At least 1.5mm larger than the fellow

unoperated eye, but responds to miotic unoperated eye, but responds to miotic agents (90%)agents (90%)

–– Unreactive and paretic, but slowly returns to Unreactive and paretic, but slowly returns to normal after time (some up to 1 yr)normal after time (some up to 1 yr)

–– Irreversible dilatation with iris atrophyIrreversible dilatation with iris atrophy

Possible MechanismsPossible Mechanisms

Strong intraStrong intra--op mydriasis brings iris into contact op mydriasis brings iris into contact with peripheral cornea, producing peripheral with peripheral cornea, producing peripheral anterior synechiae and glaucoma (Dr. Uanterior synechiae and glaucoma (Dr. U--Z)Z)Relative pupillary blockRelative pupillary blockPrePre--existing pupillary abnormalitiesexisting pupillary abnormalities–– Hyperreaction to mydriatics, as seen in Hyperreaction to mydriatics, as seen in KeratoconusKeratoconus and and

DownDown’’s Syndromes Syndrome–– Iris plataeu syndromeIris plataeu syndrome

Intraoperative trauma causing strangulation of iris Intraoperative trauma causing strangulation of iris vesselsvessels

Possible Mechanisms (cont)Possible Mechanisms (cont)IntraIntra--op IOP elevation causing occlusion of op IOP elevation causing occlusion of scleral vessels which leads to iris ischemiascleral vessels which leads to iris ischemiaDamage to ciliary ganglion during subDamage to ciliary ganglion during sub--tenon tenon injection of anestheticsinjection of anestheticsAbnormalities of the sympathetic nervous Abnormalities of the sympathetic nervous systemsystemOveruse of general anethesiaOveruse of general anethesiaIntraIntra--op exposure to other toxinsop exposure to other toxins

Treatment/PreventionTreatment/PreventionAvoid using mydriatic agents following surgery in Avoid using mydriatic agents following surgery in vulnerable eyes (keratoconic).vulnerable eyes (keratoconic).Laqoutte (1983) proposed a regimen of Laqoutte (1983) proposed a regimen of sympatholytic drop (guanethidine) q4h for 1 day, sympatholytic drop (guanethidine) q4h for 1 day, followed by pilocarpine 2% for several days.followed by pilocarpine 2% for several days.Naumann (1997) recommended performing PI in Naumann (1997) recommended performing PI in all phakic patients undergoing PK.all phakic patients undergoing PK.Special surgical techniques of PK have also Special surgical techniques of PK have also been proposed to prevent iris damage (Loden been proposed to prevent iris damage (Loden and Price, 1998).and Price, 1998).

What Happened to the Patient?What Happened to the Patient?

After 6 months, Pt returned with OS VA of After 6 months, Pt returned with OS VA of 20/40. There was no evidence of 20/40. There was no evidence of inflammation or graft failure. IOP had inflammation or graft failure. IOP had remained in the normal range, so antiremained in the normal range, so anti--glaucoma drops were discontinued.glaucoma drops were discontinued.However, the patient had significant glare However, the patient had significant glare (no photophobia or pain), as the OS pupil (no photophobia or pain), as the OS pupil was still fixed and dilated, with no was still fixed and dilated, with no improvement.improvement.

What Happened to the Patient? What Happened to the Patient? (cont)(cont)

Iris fluorescein angiography disclosed large area Iris fluorescein angiography disclosed large area of nonof non--perfusion in the left iris.perfusion in the left iris.

OD OS

End of the CaseEnd of the Case

Finally, decision was made to reconstruct Finally, decision was made to reconstruct the iris. Afterwards, her left eye had no the iris. Afterwards, her left eye had no more glares.more glares.

ReferencesReferencesUrretsUrrets--Zavalia A. Fixed dilated pupil, iris atrophy and secondary glaucZavalia A. Fixed dilated pupil, iris atrophy and secondary glaucoma: a distinct oma: a distinct clinical entity following penetrating keratoplasty for keratoconclinical entity following penetrating keratoplasty for keratoconus. us. Am J OphthalmolAm J Ophthalmol. . 1963; 56:2571963; 56:257--65.65.Gasset AR. Fixed dilated pupil following penetrating keratoplastGasset AR. Fixed dilated pupil following penetrating keratoplasty in keratoconus y in keratoconus (Castroviejo syndrome). (Castroviejo syndrome). Ann Ophthalmol. Ann Ophthalmol. 1977; 9:6231977; 9:623--8.8.Davies PD, Ruben M. The paretic pupil: its incidence and aetioloDavies PD, Ruben M. The paretic pupil: its incidence and aetiology after keratoplasty gy after keratoplasty for keratoconus. for keratoconus. Br J OphthalmolBr J Ophthalmol. 1975; 59:223. 1975; 59:223--8.8.Tuft SJ, Buckley RJ. Iris ischemia following penetrating keratopTuft SJ, Buckley RJ. Iris ischemia following penetrating keratoplasty for keratoconus lasty for keratoconus (Urrets(Urrets--Zavalia syndrome). Zavalia syndrome). CorneaCornea. 1995; 14:618. 1995; 14:618--622.622.Maurino V., Allan BDS, Stevens JD, et al. Fixed dilated pupil (UMaurino V., Allan BDS, Stevens JD, et al. Fixed dilated pupil (Urrets Zavalia syndrome) rrets Zavalia syndrome) after air/gas injection after deep lamellar keratoplasty for kerafter air/gas injection after deep lamellar keratoplasty for keratoconus. Am J atoconus. Am J Ophthalmol. 2002; 133:266Ophthalmol. 2002; 133:266--8.8.Srinivasan M., Patnaik L. Fixed dilated pupil (UrretsSrinivasan M., Patnaik L. Fixed dilated pupil (Urrets--Zavalia Syndrome) in Corneal Zavalia Syndrome) in Corneal Dystrophies. Dystrophies. Cornea.Cornea. 2004;23:812004;23:81--83.83.Yuzbasioglu E, Helvacioglu F, Sencan S. Fixed, dilated pupil aftYuzbasioglu E, Helvacioglu F, Sencan S. Fixed, dilated pupil after phakic intraocular er phakic intraocular lens implantation. lens implantation. J Cataract Refract SurgJ Cataract Refract Surg. 2006 Jan;32(1):174. 2006 Jan;32(1):174--6.6.Edgar Espana, Alex Ioannidis, Celso Tello, Jeffrey M Liebmann, PEdgar Espana, Alex Ioannidis, Celso Tello, Jeffrey M Liebmann, Paul J Foster, and aul J Foster, and Robert Ritch. UrretsRobert Ritch. Urrets--Zavalia syndrome as a complication of Argon Laser Peripheral Zavalia syndrome as a complication of Argon Laser Peripheral Iridoplasty. Iridoplasty. Br. J. OphthalmolBr. J. Ophthalmol. Sep 2006; doi:10.1136.. Sep 2006; doi:10.1136.Laqoutte F, Thienpont P, Comte P. Proposed treatment of UrretsLaqoutte F, Thienpont P, Comte P. Proposed treatment of Urrets--Zavalia syndrome: Zavalia syndrome: one reversible case (article in French). one reversible case (article in French). J Fr Ophthalmol.J Fr Ophthalmol. 1983;6(3):2911983;6(3):291--4.4.Loden JC, Price FW Jr. Price graftLoden JC, Price FW Jr. Price graft--overover--host technique to manage positive pressure host technique to manage positive pressure during penetrating keratoplasty. during penetrating keratoplasty. J Cataract Refract Surg. J Cataract Refract Surg. 1998;24:7361998;24:736--8.8.

case presentation - uveitis · patient presentation cc: fixed, dilated pupil after penetrating...

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