Urrets-Zavalia Syndrome Following Descemet Stripping Endothelial Keratoplasty

1
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 OphthalmologyUniversity of Texas
Southwestern Medical Center at Dallas, Texas
2
Introduction

• 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
  Urrets-Zavalia 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.

3
Purpose

• To examine risk factors associated with
  Urrets-Zavalia syndrome in patients undergoing
  DSEK

Materials and Methods

• 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

4
Results

• 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

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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.
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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.
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Discussion

• 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

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Conclusions

• 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 18290-4. 2. Koenig SB, Covert
DJ. Early Results of Small-Incision Descemets
Stripping and Automated Endothelial Keratoplasty.
Ophthalmology. 2007 114221-226. 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 133266-268.
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
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