Transcript Corneal Interface Morphology After Descemet Stripping

Urrets-Zavalia Syndrome Following
Descemet Stripping Endothelial Keratoplasty
Claire Y. Chu, MD
Pawan Prasher, MD
Eric Dai, MD
R. Wayne Bowman, MD
V. Vinod Mootha, MD
Department of Ophthalmology
University of Texas Southwestern Medical Center at Dallas, Texas
Introduction
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Descemet stripping endothelial keratoplasty (DSEK) has emerged as an
elegant alternative to penetrating keratoplasty (PK) for the treatment of
endothelial disorders such as Fuchs endothelial dystrophy.
DSEK minimizes the risk of PK associated postoperative complications
such as wound dehiscence, refractive astigmatism, prolonged recovery
time.
However, pupillary block glaucoma continues to be reported in DSEK
outcomes studies as a rare complication (0.5 - 3.8%) associated with the
introduction of the air bubble for graft-host apposition.
Pupillary block had previously been described in association with iris
atrophy and a fixed nonreactive pupil, collectively designated UrretsZavalia syndrome.
UZ occurs as a rare complication of penetrating keratoplasty, laser
peripheral iridotomy, and deep lamellar keratoplasty.
We describe Urrets-Zavalia syndrome in a series of patients undergoing
DSEK for Fuchs endothelial dystrophy.
Purpose
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To examine risk factors associated with
Urrets-Zavalia syndrome in patients
undergoing DSEK
Materials and Methods
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Four patients undergoing DSEK for Fuchs
endothelial dystrophy with postoperative changes
consistent with Urrets-Zavalia syndrome were
analyzed retrospectively.
 All eyes underwent clinical examination, with
followup between 4 and 8 months
Results
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Mean age was 77.5 +/- 8.1 years
Three patients had a history of prior ocular surgery, including
CEIOL, scleral buckle for retinal detachment repair, and strabismus
surgery
Preoperative BCVA ranged from 20/40 to 20/70
All patients required rebubbling during the postoperative course for
management of graft dislocation
Of the three patients who developed pupillary block glaucoma, one
required rebubbling whereas two others required rebubbling in
addition to repeat DSEK for graft failure and dislocation
One patient had no history of prior ocular surgery and did not
develop pupillary block glaucoma, but still developed UZ syndrome.
This patient was a glaucoma suspect based on family history and
optic atrophy in the affected eye
Postoperative BCVA improved, ranging from 20/25 to 20/50
Postoperative Pupillary Defects
A
B
A. Pupillary changes after DSEK with postoperative pupillary block
glaucoma, graft dislocation, and reapposition of the graft in the
operating room. B. Fellow eye.
Patient Clinical Data
Age
Gender
Preoperative
BCVA
Postoperative
BCVA
Prior Ocular
Surgery
Operative
Course
Pupillary
block
glaucoma
Nonreactive
pupil
1
82 yo M
20/70
20/30
Cataract
extraction
DSEK
Graft dislocation
+
+
2
68 yo M
20/70
20/50
None
DSEK
Graft dislocation
Repeat DSEK
+
+
3
86 yo F
20/70
20/50
Scleral buckle
DSEK
Graft dislocation
Repeat DSEK
+
+
4*
74 yo F
20/60
20/25
Strabismus
DSEK
Graft dislocation
*Glaucoma suspect based on positive family history and increased optic atrophy in the affected eye.
+
Discussion
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The most common postoperative complication of DSEK is graft
dislocation (1.5 - 14%)
Pupillary block glaucoma has been reported as a rare postoperative
complication of DSEK at a rate between 0 -3%.
The pathogenesis of Urrets-Zavalia syndrome is thought to be
pupillary block glaucoma resulting in iris ischemia, which in turn leads
to a fixed dilated pupil.2
Risk factors predisposing patients to iris ischemia may thus increase
the likelihood of UZ -- increased age, multiple intraocular
manipulations, postoperative pupillary block glaucoma, glaucoma
suspect.
Techniques to minimize graft dislocation in DSEK may minimize the
need for additional manipulation and also the risk of pupillary block
glaucoma -- peripheral recipient bed scraping, removal of interface
fluid, thorough irrigation of viscoelastic from the interface.3,4,5
Conclusions
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A dilated pupil is a rare complication following penetrating
keratoplasty and deep anterior lamellar keratoplasty, but can also
be associated with DSEK.
Patients with Fuchs endothelial dystrophy with a complicated
postoperative course may be at greater risk of iris ischemia and
Urrets-Zavalia syndrome.
Continued evolution of surgical techniques will enhance
outcomes and minimize complications of DSEK.
References
1. Price MO, Price FW. Descemet's stripping endothelial keratoplasty. Curr Opin Ophthalmol. 2007 18:290-4.
2. Koenig SB, Covert DJ. Early Results of Small-Incision Descemet’s Stripping and Automated Endothelial Keratoplasty.
Ophthalmology. 2007 114:221-226.
3. Maurino V, Allan BDS, Stevens JD, Tuft SJ. Fixed Dilated Pupil (Urrets-Zavalia Syndrome) After Air/Gas Injection After
Deep Lamellar Keratoplasty for Keratoconus. Am J Ophthalmol 2002 133:266-268.
4. Terry MA, Shamie N, Chen ES, Hoar KL, Friend DJ. Endothelial Keratoplasty: A Simplified Technique to Minimize Graft
Dislocation, Iatrogenic Graft Failure, and Pupillary Block. Ophthalmology 2007 122(5):686-92
5. Silvera DA, Fabrizio MJ, Goins KM. The Characterization of Interface Haze Following DLEK. IOVS 2007;48:ARVO EAbstract 4715.