CASE REPORT

JANUARI 2016

Urrets-Zavalia Syndrome (Fixed and Dilated Pupil)

Following Uneventful Cataract Surgery
Fadly, Yunita, Hamzah, Abrar

Oleh :

Muhammad Fadly Hidayat

Pembimbing :

dr. Yunita, Sp.M(K), M.Kes

dr. Hamzah, Sp.M(K)
dr. Muh. Abrar Ismail, Sp.M, M.Kes

KONSENTRASI PENDIDIKAN DOKTER SPESIALIS TERPADU

( COMBINED DEGREE )
BAGIAN ILMU KESEHATAN MATA PROGRAM STUDI BIOMEDIK
PROGRAM PASCA SARJANA
FAKULTAS KEDOKTERAN UNHAS
MAKASSAR
2016
 Urrets-Zavalia Syndrome (Fixed and Dilated Pupil)
Following Uneventful Cataract Surgery
Fadly Hidayat, Yunita, Hamzah, Abrar
Department of Ophtalmology, Faculty Of Medicine, Hasanuddin University
Hasanuddin Teaching Hospital, Makassar

ABSTRACT

Purpose : To report a case of urrets-zavalia syndrome following uneventful cataract

surgery
Methods : A case report
Case report : A 57 years old female presented with glare on right eye 3 days following
cataract surgery. Examination of the right eye showed iris atrophy and fixed dilated
pupil, the absence of light reflex, pigment of the iris appears on the surface of
intraocular lens. There are no history of complication recorded during surgery. Fellow
eye was operated by same surgeon with BCVA 20/20 and no found abnormality.
Result : The patient were given a miotic agent drops every 2 hours, and steroid every 6
hours daily and tappered off every week. In the next visit showed no respond on therapy,
the pupil still fixed and dilated.
Conclusion : The exact etiology of urrets-zavalia syndrome is unknown and different
mechanisms have been proposed.This condition may need preventive strategies to avoid
complication. This case shows an intrinsic susceptibility for urrets-zavalia syndrome
which alerts surgeons in the case of second eye surgery
Keywords : Urrets-Zavalia syndrome, Dilated Pupil, Fixed Pupil, penetrating
keratoplasty, atropine, mydriatics
INTRODUCTION
Urrets-Zavalia Syndrome (UZS) was
first described as a syndrome consisting
of a fixed, dilated pupil with iris atrophy
following penetrating keratoplasty
1
(PKP) in 1963. Other manifestations
that are not essential for the diagnosis
are posterior synechiae, ectropion uvea,
pigment dispersion, anterior subcapsular
lens opacities and secondary glaucoma
syndrome.
At first this syndrome had been
attributed to the keratoconus. However
this syndrome was described after PKP
for corneal dystrophy, deep anterior
lamellar keratoplasty (DALK), descemet
stripping endothelial keratoplasty
(DSEK), trabeculectomy, laser
iridoplasty, iatrogenic mydriasis and
after implantation of phakic intraocular
lenses.2-10
Although Zavalia associated this
syndrome with postoperative treatment
with mydriatics, then Uribe supposed a
process of postoperative spontaneous
mydriasis, poorly responsive to miotics,
in patients with keratoconus undergoing
PKP.11 Thus it could happen where
mydriatics had not been prescribed. The
precise contributing mechanism has not
been known so far but fibrinous uveitis,
iris vessel strangulation and pupillary
block have been proposed.8
Hereby we reported case of urrets
zavalia syndrome post cataract surgery
CASE REPORT
A 57 years old female presented to us
clinic with complaint of glare on the
right eye since 3 days after cataract
surgery. The patient acknowledge that
her vision was improved after surgery,
however she felt glare on a bright light
environment. Examination of the right
eye showed atrophy of the iris, dilated
pupil, absence of light reflex, there was
no relative afferent pupillary defect,
pigments of the iris were present on
intraocular lenses surface, intraocular
lense is located in the bag. Eye
movements are normal on both eyes.
Both eyes are pseudophacic with
BSCVA on right and left eye are 20/25
and 20/20 respectively. Funduscopy
examination showed normal findings.
Medications given after catarct surgery
are antibiotics and steroid eye drops.
The patient was also given beta blocker
eye drop. On the day of surgery, there
were 3 cataract surgeries. Patient were
operated with surgeon using similar
consumables with same technique. None
of them reported to have similar sign
and symptomps. The patient’s fellow
eye were operated 1 month earlier with
same surgeon and no adverse outcomes
found. Since the first day after surgery
the right eye had an increase of the intra
ocular pressure, and another finding is
that the conjunctiva is hiperemic and the
cornea appears edema. On the next visit,
these signs were absent. At first control,
we got signs fixed and dilated pupil, and
then the patient was given chlolinergic
parasympathomimetic (pilocarpine)
every 2 hours for 1 day usage and other
drugs such as antibiotic and steroid drop
were proceed. In the next control, we
still got fixed and dilated pupil, and no
light reflex. It showed no response to
pilocarpine administration so that the
administration was stopped.
Figure 1. Shown dilated pupil and atrophy of the
iris
DISCUSSION
A fixed, dilated pupil will not react to
light or accommodation16, around 7 mm
in diameter, is often irreversible, not
respond to pharmacological treatment
and will usually be noticed during the
immediate postoperative period.17,18 As
with the theory above, in this case, the
patient had fixed and dilated pupil that
do not respond to the administration of
chlolinergic parasympathomimetic for
24 hours.
Jastaneiah et al reported that a
dilated pupil will be detected by the first
two postoperative days in 80.9% of
patients.19 Similarly, Davies and Ruben
found most cases of UZS to occur at the
first postoperative day. A few present
later, but not after the 12th postoperative
day.20 Others, however, stated that
although UZS will typically occur 1 or 2
weeks after PKP, it does not occur
within the first postoperative days.21,22
UZS is also reported to occur up to 5
months postoperatively.23 In this patient
was found glare since 3 days post
surgery, from this sign we can assume
that fixed and dilated pupil occurred in
the same time.
The pathophysiology of this
syndrome is still unclear. The leading
explanation for the development of UZS
is ischemic atrophy of the sphincter
muscle secondary to iris strangulation
with resultant pupil dilatation. The cause
for iris ischemia could be an acute post-
operative increase in IOP,15,24
compression of iris vessels against the
Figure 2. Iris pigments on IOL surface
incision edge of the host cornea as the
lens-iris diaphragm moves forwards
during surgery.20 Some argue that UZS
is a pupillary block phenomenon.25,26
The rise of IOP also found on the first
day post operation.
In several reports where IOP
measurements were not available or IOP
was normal, patients reported
experiencing pain, nausea, and vomiting
during the night, often symptoms of a
transient increase in IOP, before a fixed
and dilated pupil was observed.15,27,28 As
we observed in this patient.
Urrets and Zavalia also suggested
that the strong mydriasis produced by
atropine at the time of surgery brought
iris in contact with peripheral cornea to
produce peripheral anterior synaechiae
and secondary glaucoma.1 Though,
glaucoma is not the integral part of the
syndrome.13 UZS may develop even in
cases where no mydriatic drops were
applied.4
Davies and Ruben also suggested
that direct iris trauma during surgery
could result in strangulation of iris
vessels in the mid-periphery and
ischemic paralysis of the sphincter
pupillae.15 In this case we found iris
atrophy cause by injury during operation
Cataract surgery may result in fixed
and dilated pupils.29,30 Viscoelastic
material left in the anterior chamber
angle,15 and the hypromellose used as
viscoelastic material was suspected to
be toxic to the sphincter or vasculature
of the iris, resulting in a rate of 16.7% of
sphincter atrophy and fixed dilated ocular pressure a day after cataract
pupil.30 Jastaneiah also suggested surgery.
Residues of either viscoelastics or CONCLUSION
detergents can lead to inflammation,
which is usually considered toxic The exact etiology of urrets-zavalia
anterior segment syndrome. This can syndrome is unknown and different
elevate the IOP, impair iris sphincter mechanisms have been proposed. This
functions, and possibly result in a may complicate preventive strategies for
permanently dilated fixed pupil as well this syndrome. UZS can occur even
as atrophy of the iris.19 In this patient, thought under optimal operation
we did not find the residual of condition. Experienced surgeon, the
viscoelastic left on anterior chamber but technique and use of the same material
it can not be guarantee because of the still has the same chance to occur this
viscoelastic substance is transparence syndrome. This case shows an intrinsic
made difficult to evaluated. susceptibility for urrets-zavalia
A fixed, dilated pupil does not cause syndrome which alerts surgeons in the
a decrease in visual acuity23 but can case of second eye surgery
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