acute and chronic fluid misdirection syndrome ... · cataract acute and chronic fluid misdirection...
TRANSCRIPT
CATARACT
Acute and chronic fluid misdirection syndrome:pathophysiology and treatment
Andrzej Grzybowski1,2 & Piotr Kanclerz3
Received: 7 August 2017 /Revised: 10 October 2017 /Accepted: 16 October 2017 /Published online: 6 November 2017# The Author(s) 2017. This article is an open access publication
AbstractPurpose To summarize our current understanding of the spe-cific pathogenic mechanisms of the fluid misdirectionsyndrome and possible treatment methods.Methods We used the PubMed web platform to find relevantstudies using the following keywords: infusion misdirectionsyndrome, aqueous misdirection syndrome, ciliary block,ciliovitreal block, capsular block, intraoperative fluid misdi-rection, subcapsular fluid entrapment, acute intraoperativerock-hard eye syndrome, positive vitreous pressure glaucoma,and malignant glaucoma. Other publications were also con-sidered as a potential source of information when referencedin relevant articles.Results We collected and analyzed 55 articles dated from1951 to 2016. Acute intraoperative rock-hard eye syndromeis characterized by a very shallow anterior chamber with theabsence of suprachoroidal effusion or hemorrhage and no no-ticeable pathology of the iris–lens diaphragm. It usually oc-curs during uneventful phacoemulsification, particularly inhyperopic eyes. The pathophysiology of acute fluid misdirec-tion syndrome is based on inappropriate movement of
balanced salt solution via the zonular fibers. This syndromehas also been described as occurring from hours to months, oryears, after the initial surgery. The pathophysiology of malig-nant glaucoma is based on similar mechanisms of cilio-lenticular block of aqueous flow leading to the misdirectionof aqueous posteriorly into or besides the vitreous gel. Facedwith these situations, vitreous decompression is required, pref-erably with hyaloido-capsulo-iridectomy. In phakic eyes, con-comitant cataract extraction would be desirable.Conclusions We believe both of these clinical conditionsshould be considered as one syndrome. We suggest the termacute fluid misdirection syndrome for the cascade of eventsduring phacoemulsification surgery. Chronic fluid misdirec-tion syndrome better describes the nature of malignantglaucoma.
Keywords Cataract . Glaucoma, angle-closure .
Trabeculectomy . Vitrectomy . Vitreous body
Introduction
The fluid misdirection syndrome is a serious threat in anteriorsegment surgery. It is notoriously difficult to treat, and carriesa generally uncertain prognosis for long-term control of intra-ocular pressure. Our understanding of pathophysiology, riskfactors, accurate diagnosis, and treatment is hampered by in-sufficient uniform clinical case clarification. Previously pro-posed definitions for fluid misdirection syndrome were non-specific and lacking a comprehensive view. It has been alsoreported under different names, including infusion misdirec-tion syndrome, aqueous misdirection syndrome, capsularblock, ciliovitreal block, ciliary block, intraoperative fluidmisdirection, subcapsular fluid entrapment, acute intraoper-ative rock-hard eye syndrome, positive vitreous pressure
* Andrzej [email protected]
1 Department of Ophthalmology, University of Warmia and Mazury,Olsztyn, Poland
2 Institute for Research in Ophthalmology, Foundation forOphthalmology Development, 60-554 Gorczyczewskiego 2/3,Poznan, Poland
3 Department of Ophthalmology, Medical University of Gdańsk,Gdańsk, Poland
Graefes Arch Clin Exp Ophthalmol (2018) 256:135–154https://doi.org/10.1007/s00417-017-3837-0
glaucoma, andmalignant glaucoma. Currently, years after theinitial description by von Graefe in 1869 [1], we now knowmuch more about this uncommon syndrome, but its charac-terization still remains incomplete.
This review summarizes our current understanding ofthe specific pathogenic mechanisms of this rare syn-drome and possible treatment methods. Certainly it willassist in identification of risk factors and make it possi-ble to establish an effective treatment of this dangeroussyndrome.
Methodology
We used the PubMed web platform to find prospective orretrospective studies and case reports.
Our keywords have been strictly defined: infusion misdi-rection syndrome, aqueous misdirection syndrome, ciliaryblock, ciliovitreal block, capsular block, intraoperative fluidmisdirection, subcapsular fluid entrapment, acute intraoper-ative rock-hard eye syndrome, positive vitreous pressureglaucoma. Other publications were also considered as a po-tential source of information when referenced in relevantarticles.
We selected English language articles and divided ourreports into two groups: intraoperative complications(Table 1) and postoperative complications (Table 2).Common risk factors for fluid misdirection syndrome arepresented in Table 3.
Results
We collected and analyzed 55 articles dated from 1951 to2016. Most of the articles related to intraoperative com-plications were published since 2010, though the firstdescription by MacKool is from 1993 [4]. As of now,about 20 cases of this syndrome have been described,commonly during uneventful phacoemulsification. Themost numerous group of cases was collected by Lauet al. [3] Six eyes were described, which was 1.45%cases among all phacoemulsification procedures(Table 1).
Articles referring to malignant glaucoma are definitelymore numerous, and over 200 cases have been describedworldwide. The most abundant was a study described byAl Bin Ali et al. [31] which referred to 69 eyes. Recentstudies focus on the optimal surgical approach, as theoutcome is still not satisfactory (Table 2).
Common risk factors for fluid misdirection syndrome havebeen summarized in Table 3.
Discussion
Mechanisms of acute fluid misdirection syndrome
The fluid misdirection syndrome is a rare clinical conditioncharacterized by an axially very shallow anterior chamberwith the absence of suprachoroidal effusion or hemorrhageand no noticeable pathology of the iris–lens diaphragm. Itusually occurs during uneventful phacoemulsification partic-ularly in hyperopic eyes [3]. It is probably underreported;anecdotally, many surgeons admit having experienced it oc-casionally. The common theme is that it manifests toward theend of irrigation/aspiration (I/A), making the completion of I/A or the insertion of an intraocular lens impossible because offlat anterior chamber. The accumulation of fluid in the poste-rior segment engenders increase in posterior pressure,resulting in anterior displacement of the iris–lens diaphragm,axial and peripheral anterior chamber flattening, and second-ary angle closure. The severity of this condition wasunderlined by using the name acute intraoperative rock-hardeye syndrome.
The integrity of the posterior chamber (PC)–anterior hya-loid membrane (AHM) barrier during phacoemulsificationhas been thoroughly evaluated ex vivo through contrast-enhanced magnetic resonance imaging and in the Miyake–Apple view on porcine eyes [38, 39]. Prolonged irrigationand deflation/inflation of the anterior chamber are risk factorsof AHM detachment, while hydrodissection is associated withan AHM tear [38]. Furthermore, ocular viscosurgical devices(OVD) with higher molecular weight or higher concentrationof sodium hyaluronate predisposed the eye to an increasedrisk of PC-AHM impairment during hydrodissection [39].
In vivo, breaking the PC–AHM barrier is extricated by theresidual cortical fiber irrigation maneuver. It is excessive irri-gation which forces fluids into the PC, thus, the term of infu-sion misdirection syndrome or intraoperative fluidmisdirection has been suggested [4, 6]. The narrow flow isusually generated by a 27G straight or curved hydrodissectioncannula tip. However, it might take place during I/A with atypical irrigation probe, or phacoemulsification at the time ofremoving the last remaining nuclear pieces. It might be no-ticeable that the posterior capsule is flaccid — bulging orbillowing forward [4]. Lau et al. [3] claim that higher levelsof anterior chamber irrigation might be a risk factor.
Miscellaneous routes for balanced salt solution entering theanterior vitreous or Berger’s space have been described.Obviously, a radial extension of capsulorhexis might enabledirect access of fluid into the retrocapsular space [4]. In thesemild cases, administration of OVD into the anterior chambermight be possible, as well as dry aspiration of retrocapsularfluid [5]. Presumably, if irrigation is performed anteriorly tothe anterior capsular flap, fluid could more easilymake its waythrough the zonules.
136 Graefes Arch Clin Exp Ophthalmol (2018) 256:135–154
Tab
le1
Clin
icalmanifestatio
nsof
acuteflu
idmisdirectionsyndrome
Article
Suggestedname
ofsyndrome
No.of
eyes
(incidence)
Perform
edprocedure
Riskfactors
Tim
eof
occurrence
during
surgery
Presum
edpathom
echanism
Suggestedtreatm
ent
Outcome(follow-up
period)
Kam
etal.2016
[2]
Acuteintraoperativ
erock-hardeye
syndrome
Twoeyes
(2/736
phaco
procedures)
Phaco+IO
LN/A
Duringsurgery(not
described)
Freeingtenaciouscortex,direct
irrigatio
nwith
astraight
ora
curved
cannulaoftheinferioror
superior
capsular
bagfornix,
may
contributeto
finding
materialsin
thevitreous.W
hen
repeated
with
asingle,
potentially
powerfuljetof
balanced
saltirrigatedthrougha
syringeandacannula,
may
lead
totransgressionof
thezonularapparatusby
the
balanced
saltsolutionandsm
all
pieces
oflens
material.
Emergentparsplananeedle
aspiratio
nof
retrolenticular
fluidwith
a23-gauge
needle
Good—
ACdeepening
Lau
etal.2014
[3]
Acuteintraoperativ
erock-hardeye
syndrome
Sixeyes
(6/413
phaco
procedures)
Phaco+IO
L-H
igherlevelsof
ACirrigatio
n-Hyperopia
Not
only
from
the
phacoemulsificatio
nbutalsofrom
hydrodissection,
hydrodelineatio
n,andirrigatio
nof
the
posteriorcapsuleand
equatorialcortex
with
ahydrodissection
cannula.
Residualcorticalfiberirrigatio
nmaneuver(w
henresidual
corticalfibersarebeing
removed,the
narrow
stream
ofbalanced
saltsolutio
ngenerated
bythenarrow
hydrodissection
cannulatip
may
distortthe
posteriorcapsulelocally
)resulting
inlocalized
anterior
displacemento
fthecontiguous
posteriorcapsule,creatin
ga
subcapsularor
vitrealspace
throughwhich
thebalanced
salt
solutio
nmay
moveviathe
zonularfibers.
Emergentparsplana(3
mm
from
limbus)needle
aspiratio
nof
retrolenticular
fluidwith
a23-gauge
needle
Goodpost-opvisualacuity
andnorm
alIO
P.Com
plications:
-mild
vitreous
hemorrhage
(one
eye)
-temporalarteritis(one
eye
1month
post-op)
Mackool
etal.
1993
[4]
Infusion
misdirection
syndrome
N/A
Phaco+IO
L-Exfoliatio
n-
Dense/brune-
scentcataract
Duringsurgery,
especially
atthetim
eof
removalof
thelast
nuclearpieces
Aqueous
canbe
directed
posteriorlybehind
anintactlens
andcausecham
bershallowing:
-whenfluidpasses
throughan
openingintheposteriorcapsule
ortheequatorialregion
(especially
likelyafter
can-opener
capsulotom
yor
radialextensionof
capsulorhexis),permitting
directaccess
tothe
retrocapsularspace
-in
thepresence
ofan
intact
capsule(intactrim
bycapsulorhexis)fluidpasses
throughtheregion
ofthe
zonularfibers.Morecommonly
inassociationwith
laxzonular
fibers
-Viscoadaptiv
eagenttothe
AC
-Parsplanadecompression
N/A
Olson
etal.1994
[5]
Subcapsularfluid
entrapment
Five
eyes
Penetrating
keratoplasty
+ECCE+IO
L(twoeyes)
phaco+IO
L(three
eyes)
-OpenskyI/A
-Zonulardialysis
Duringsurgery
-Irrigatio
nanterior
totheACflap
-Zonular
dehiscence
-Dry
aspiratio
n(three
eyes)
-Parsplanaparacentesis
(one
eye)
-Sp
ontaneouscentral
capsulerupture(one
eye)
Good—
ACdeepening
Paracentesisunsuccessful
inoneeye—
IOL
implanted2months
post-op
Graefes Arch Clin Exp Ophthalmol (2018) 256:135–154 137
Tab
le1
(contin
ued)
Article
Suggestedname
ofsyndrome
No.of
eyes
(incidence)
Perform
edprocedure
Riskfactors
Tim
eof
occurrence
during
surgery
Presum
edpathom
echanism
Suggestedtreatm
ent
Outcome(follow-up
period)
Dew
ey2011
[6]
Intraoperativ
efluid
misdirection
Three
eyes
Phaco+IO
L-Coughing
-Sh
ortaxial
length
-Significantly
intumescent
lens
Duringsurgery
N/A
-Viscoadaptiv
eagentsto
theACanddry
aspiratio
n(m
ildcases)
-Sh
arpneedle
decompression,or
automated
vitrectomy
handpieceto
avoid
potentialv
itreous
tractio
n(severecases)
-Benzonatatecapsules
(TessalonPerles)for
patientswith
ahistoryof
coughing,Bsinus
drainage,^
chronic
obstructivepulm
onary
disease,bronchitis,
asthma,sleepapnea,
snoring,useof
supplementalo
xygen,
difficulty
breathingin
thesupine
positio
nfor
anyreason,and
atthe
preoperativ
enurse’s
suspicion.
Good—
ACdeepening
Grzybow
ski
etal.2
014[7]
Acute/chronic
aqueous
misdirection
syndrome
N/A
Phaco+IO
LN/A
Duringsurgery-toward
theendof
irrigatio
n/aspiratio
n
InappropriateBm
ovem
ento
fthe
balanced
saltsolutio
nviathe
zonularfibers^triggeredby
the
unconventio
nalu
seof
the
residualcorticalfiberirrigatio
nmaneuver
Parsplanadecompression
with
vitreous
cutter
Good—
ACdeepening
Wuetal.2016
[8]
Malignant
glaucoma
Thirty-eighteyes
(of1432
eyes
undergoing
anti-glaucoma
surgical
treatm
entfor
prim
aryangle
closure
glaucoma)
-After
trabeculecto-
my(32eyes
—84.2%)
-during
trabeculecto-
my(foureyes
—10.5%)
-aftercombined
glaucoma/-
cataract
surgery(two
eyes
—5.3%
)
Duringor
aftersurgery
1)Ciliolenticular
blockor
anterior
hyaloidobstruction
2)Slacknessof
lens
zonules
3)Severepostoperative
inflam
mation
4)Non
ciliary
block.Itwas
observed
thatthosepatients
who
developedmalignant
glaucomaduring
surgeryall
show
edahigh
stress
leveland
poor
operativecooperation.We
speculated
thatsuch
status
caused
constrictio
nof
extraocularmuscles
pressing
forw
ard,bringing
aboutthe
increase
ofocular
venous
pressure.
Abbreviations
used:AC,anteriorcham
ber;ECCE+IO
L,extracapsular
cataractextractio
nwith
intraocularlens
implantatio
n;I/A,irrigation/aspiratio
n;IO
L,intraocular
lens;IO
P,intraocularpressure;
phaco+IO
L,phacoem
ulsificatio
nwith
intraocularlens
implantatio
n
138 Graefes Arch Clin Exp Ophthalmol (2018) 256:135–154
Tab
le2
Clin
icalmanifestatio
nsof
chronicflu
idmisdirectionsyndrome
Article
Suggested
nameof
syndrome
No.of
eyes
(incidence)
Performed
procedure
Riskfactors
Tim
eof
occurrence
relatedto
time
ofsurgery
Presum
edpathom
echanism
Suggestedtreatm
ent
Outcome(follow-up
period)
Littleetal.1993
[9]
Malignant
glaucoma,
cilio
lenticular
block,
cilio
vitrealb
lock,
iridovitrealb
lock,
aqueousmisdirection
oraqueousdiversion
syndrome
Seveneyes
(7/
12,000
surgeries)
-ECCE+IO
L(foureyes)
-ECCE+IO
L+
trabeculectomy(two
eyes)
-trabeculectomy(one
pseudophakiceye)
N/A
1week–7-
months
Cilio-lenticular
blockof
aqueousflow
leadingto
the
misdirectionof
aqueous
posteriorlyintoor
infrontof
thevitreous
gelleading
tothecharacteristicdiffuse
shallowingof
theAC
accompanied
bya
precipito
usrise
inIO
P.
Nd:YAGlaser
capsulotom
yand/or
vitreolysis
IOPstabilizedin
five
ofseveneyes
Sharmaetal.
2006
[10]
Aqueous
misdirection
syndrome
Five
eyes
-Trabeculectom
y(one
eye)
-Redo-trabeculectomy
(one
eye)
-YAGLPI
(one
eye)
-spontaneous,follo
wing
branch
retin
alvein
occlusion,NdY
AGLPI,
trabeculectomy,
cyclodiode
(one
eye)
-N/A
(though
phaco-em
ulsificatio
nof
thecataractin
thefello
weyehadresultedin
ase-
verepostoperativeaque-
ousmisdirectionsyn-
drom
e)
Hyperopia
1day–3months
Blockageof
anterior
aqueous
flow
atthelevelo
fthe
ciliary
body
combinedwith
aninherent
imperm
eability
defectin
theanterior
hyaloid.Abnormal
anatom
icrelatio
nship
betweentheciliary
body,
anterior
hyaloid,andlens
inhyperopiceyes.A
forw
ard
displacemento
ftheanterior
vitreous
with
appositio
nof
theanterior
hyaloidface
againstthe
lens
andciliary
body
preventsthenorm
alanterior
flow
ofaqueous
humor.S
ubsequent
misdirectionof
aqueous
flow
posteriorlyandits
accumulationin
the
posteriorsegm
ent,with
increasing
posterior
pressure,resultsin
anterior
displacementofthe
iris–lens
diaphragm,axialand
peripheralACflattening,
andsecondaryangleclosure
Corevitrectomy-
phacoemulsificatio
n-vitrectomywith
iridozonulohyaloide-
ctom
y
IOPstabilizatio
nwith
topicalh
ypotensive
agents
Com
plications:
-serosanguinalchoroidal
effusion
inoneeye
(16months/range5–32)
Mengetal.2014
[11]
Ciliaryblockglaucoma
Eleveneyes
(of
nine
patients)
-Trabeculectom
ywith
peripheraliridectomy
Prim
ary
angle
closure
glaucoma
3.9±4.1(range
1–11)days
Lensdisproportionand
lens–ciliarybody
appositio
nin
smalleyesandanterior
hyaloidchanges,with
increasedhydraulic
resistance,are
thoughttobe
themajor
pathophysiologicalfactors.
Inaddition,unfavorable
vitreous
flow
andan
expansionof
thechoroidal
volumein
smalleyesmay
beinvolved
inmalignant
glaucoma.These
anatom
icalcharacteristics
lead
toaqueoushumor
misdirectioninto
the
ACinfusion
followed
bysingleport25-G
anterior
vitrectomy,
phaco+IO
Lin
phakiceyes,posteri-
orcapsulectomywith
anterior
hyaloidface
removaluntil
sudden
pupild
ilatatio
nand
deepeningof
the
ACisachieved
Alth
ough
IOPcontrolw
asachieved
inalleyesafter
surgery,twoeyes
required
long-term
topi-
calantiglaucom
amedi-
catio
n(6–18months)
Graefes Arch Clin Exp Ophthalmol (2018) 256:135–154 139
Tab
le2
(contin
ued)
Article
Suggestednameof
syndrome
No.of
eyes
(incidence)
Perform
edprocedure
Riskfactors
Tim
eof
occurrence
relatedto
time
ofsurgery
Presum
edpathom
echanism
Suggestedtreatm
ent
Outcome(follow-up
period)
vitreous
cavity
andcause
forw
ardmovem
ento
fthe
lens–irisdiaphragm
Rekas
etal.2015
[12]
Malignant
glaucoma
Twenty-twoeyes
(22/1689
glaucoma
surgeries,
2.3%
penetrating
glaucoma
procedures,
0%of
non--
penetrating
procedure)
-Ph
acoiridencleisis
(40.9%
)-Ph
acotrabeculectom
y(22.7%
)-Iridencleisis(18.2%
)-Trabeculectom
y(13.6%
)-Setonvalveim
plantatio
n(4.6%)
Penetrating
glaucoma
surgery
Average
61days
(1–840
days)
Aqueous
humor
accumulates
inthearea
ofthevitreous
cavity
dueto
ciliary
block,
and,as
aresultof
this,there
isan
increase
inthevitreous
pressure
thatistransferred
tothestructures
ofthe
anterior
segm
entcausing
aforw
ardmovem
ento
fthe
lens-irisdiaphragm
25-gauge
vitrectomy
with
iridectomy/iris-lens/-
iridocorneal/iris--
capsuleadhesions
released.
Authorssuggestq
uick
implem
entatio
nof
surgicaltreatm
ent.
IOP≤21
mmHgwith
out
topicalh
ypotensive
agentsin
49.0%
after
12months,
IOP≤21
mmHgwith
amaxim
umof
twotopical
hipotensiveagentsin
85.7%
after12
months
(mean405days
/range
7–1440
days)
Prem
senthil
etal.2012
[13]
Positiv
evitreous
pressure
glaucoma
One
eye
LPI
N/A
1year
Inmalignant
glaucoma,a
viciouscycleof
poorer
vitreous
fluidconductiv
ityandincreasedtransvitreal
pressure
isestablished.This
results
incompression
ofthe
vitreous
gel,progressive
forw
arddisplacemento
fthe
lens-irisdiaphragm
and
eventualdirectclosureof
theACangledespite
the
presence
ofpatent
iridotom
y
PPV, phacoemulsificatio
n,prim
aryposterior
capsulotom
y,and
posteriorcham
ber
IOLim
plantatio
n.
IOPstabilizatio
n(6
months)
Pasaoglu
etal.
2012
[14]
Malignant
glaucoma
Twoeyes
-Trabeculectom
y(one
pseudophakiceye)
-Ph
aco+IO
L,recurrence
ofmalignant
glaucoma
9monthsafterP
PVwith
capsulotom
yand
peripheraliridectomy
(one
eye)
N/A
1week–1month
Relationshipof
thelens,ciliary
body,and
anterior
hyaloid
face
causingan
aqueous
misdirectionandblockade
was
suggestedin
the
pathogenesis.T
heaqueous
hasbeen
thoughttobe
entrappedinside
the
vitreous
cavity
asaqueous
pocketsresulting
inforw
ard
movem
ento
ftheiris-lens
diaphragmwhich
causes
the
secondaryangleclosure
glaucoma.
Patientsweretreated
successfullyby
using
anACapproach
consistin
gof
acapsulo--
hyaloidectom
yand
anterior
vitrectomy
performed
througha
peripheral
iridectomy,creatin
gaperm
anentp
assage
betweentheAC
andvitreous
cavity
byelim
inatingthe
aqueous
misdirection.
IOPstabilizatio
n(5
months)
Zarnowskietal.
2014
[15]
Teneyes
-Ph
acotrabeculectom
y(foureyes)
-Trabeculectom
y(three
eyes)
-phaco+IO
L(three
eyes)
-History
ofprim
ary
angle
closure
-Hyperopia
N/A
Allof
thetissues
(iris,lens
capsule,zonule,and
anterior
vitreous)have
tobe
removed
tocreatea
perm
anentp
assage
between
theACandthevitreous
cavity.Partofthe
problemis
that,duringconventio
nal
Anteriorhyaloidectom
ycombinedwith
peripheral
iridectomy,
zonulectom
y,and
peripheral
capsulectomy
IOPstabilizatio
n(12months/range
6–18.0
months)
140 Graefes Arch Clin Exp Ophthalmol (2018) 256:135–154
Tab
le2
(contin
ued)
Article
Suggestednameof
syndrome
No.of
eyes
(incidence)
Perform
edprocedure
Riskfactors
Tim
eof
occurrence
relatedto
time
ofsurgery
Presum
edpathom
echanism
Suggestedtreatm
ent
Outcome(follow-up
period)
vitrectomyperipheral
vitreous
canhardly
becompletelyremoved,and
thatiswhy
relapseratemay
bevery
high.
Shahid
etal.
2012
[16]
Directlensblock
glaucoma
(0.4–6%
ofincisional
surgeryfor
prim
ary
angle-closure
glaucoma)
-Incisionalsurgeryfor
prim
aryangle-closure
glaucoma
-After
cataractextractio
n-Sp
ontaneously,LPI,
trabeculectomyscleral
flap
suture
lysis,
cyclophotocoagulation,
useof
mioticsand
trabeculectomybleb
needlin
g.Sporadically
inassociationwith
infection,retin
opathy
ofprem
aturity,retinal
detachment,retin
alvein
occlusionandtrauma.
-Tendency
inan individual
(asitoften
occursin
fello
weyes)
Immediate
postoperative
period
tomanyyears
aftersurgery
-Posterior
diversionof
aqueousflow
causes
accumulationof
aqueous
behind
aposteriorvitreous
detachmentw
ithsecondary
forw
ardmovem
ento
fthe
iris-lensdiaphragm
-Laxity
oflens
zonules
coupledwith
pressurefrom
thevitreous
leadstoforw
ard
lens
movem
ent.Avicious
circleissetu
pin
thatthe
higherthepressure
inthe
posteriorsegm
ent,themore
firm
lythelens
isheld
forw
ard.
-Choroidalexpansion
Cycloplegics,aqueous
suppressants,
osmoticagentsand
steroids.
Cataractextractionwith
IOLim
plantatio
n,posterior
capsulotom
yand
vitrectomyin
phakic
eyes.
Inaphakic/-
pseudophakiceyes
thefollowing
procedures
were
performed:
-Nd:YAGlaser
capsulotom
yand
disruptio
nof
anterior
hyaloidface
-Transscleral
cyclodiode
laser
-Vitrectomy.
N/A
Madgulaetal.
2014
[17]
Teneyes
-Ph
acotrabeculectom
y-Trabeculectom
ywith
mito
mycin
-Ph
aco+IO
L-ECCE
-Ph
acovitrectomy
N/A
N/A
Various
anom
aliesoftheciliary
body,choroid,lens,zonule,
andvitreous
which
may
lead
toposteriordiversion
ofaqueoushumor
into
the
vitreous
cavity
have
been
proposed.A
nteriorrotatio
nof
theciliary
body
processes
canlead
tocilio
-lenticular
touchandciliary
block
Zonulo-hyaloido-vitrec-
tomy(anterior
approach)
Malignant
glaucoma
recurred
infour
eyes.
The
reasonsare
blockage
ofthechannel
byvitreous
orfibrin.
Com
plications:
-macular
hole
-cystoidmacular
edem
a-corneald
ecom
pensation
(50.2±27.2
months)
Loisetal.2001
[18]
Malignant
glaucoma
Five ps
eudophakic
eyes
-Ph
acotrabeculectom
y(three
eyes)
-Ph
aco+IO
L(one
eye)
-Trabeculectom
y(one
eye)
N/A
N/A
Existence
ofan
abnorm
alanatom
icrelatio
nship
betweentheciliary
processes,thecrystalline
lens,orIO
Landanterior
vitreous
face,w
hich
leadsto
misdirectionof
aqueous
fluidintothevitreous
cavity
Zonulo-hyaloido-vitrec-
tomy(anterior
approach)
Resolutionof
themalignant
glaucomawas
achieved
inallcases.Inone
patient
with
extensive
anterior
synechiae,bleb
failu
reoccurred
afterthe
resolutio
nof
the
malignant
glaucoma.
Thispatient
was
treated
successfully
with
aguardedfiltrationprocedure
supplementedwith
Graefes Arch Clin Exp Ophthalmol (2018) 256:135–154 141
Tab
le2
(contin
ued)
Article
Suggestednameof
syndrome
No.of
eyes
(incidence)
Perform
edprocedure
Riskfactors
Tim
eof
occurrence
relatedto
time
ofsurgery
Presum
edpathom
echanism
Suggestedtreatm
ent
Outcome(follow-up
period)
5-fluorouracil.
(1–9
months)
Varmaetal.
2014
[19]
Malignant
glaucoma
Twenty
eyes
Phaco+IO
L-Fem
ale
hyperopic
patients
5.8±7.1weeks
Ciliolenticular
block
presum
ably
inducedby
anterior
movem
ento
fthe
lens–irisdiaphragm,p
oor
vitreous
conductiv
ity,and
choroidalexpansion.
-Medicaltherapy:
cycloplegics
and
aqueous
suppressants
-Nd:YAG
Iridozonulohyaloido-
tomy
-ACreform
ation/IO
Lpushback
-Iridozonulohyaloido-
vitrectomy(pars
planaapproach)
Symptom
sresolved
in(IOP
controlw
ithtopical
medications):
Medicaltherapy(twoeyes)
Iridozonulohyaloidotom
y(seven
eyes)
ACreform
ation–IO
Lpushback
(six
eyes)
Iridozonulohyaloidovitrect-
omy(fiveeyes)
Daveetal.2013
[20]
Malignant
glaucoma
Twenty-eight
eyes
Trabeculectom
y(11eyes)
Cataractsurgery
(ten
eyes)
Com
binedcataractand
glaucomasurgery(seven
eyes).
N/A
N/A
Misdirectionof
aqueousintoor
behind
thevitreous
body
thatisresponsibleforan
increase
invitreous
volume
andsubsequent
obliteration
oftheanterior
andposterior
cham
bers.
1.Medicalmanagem
ent
included
topical
cycloplegics
(atropineor
cyclopentolate)and
topical/oralaqueous
suppressants.
Intravenous20%
mannitolinadose
of1mg/kg
was
administeredatthe
discretio
nof
the
treatin
gphysician.
2.Nd:YAGLaser
hyaloidotomyin
pseudophakiceyes
throughan
existin
gperipheraliridotom
yor
beyond
thehaptic
oftheIO
L.
3.Vitrectomy-hyaloid-
otom
y-iridotom
y(parsplana
approach)
4.Transscleral
cyclophotocoagula-
tion
IOP<21
mmHgwith
upto
twotopicalm
edications
in:
1)four
eyes
2)seveneyes
3)four
eyes
4)11
eyes
Intwoeyes
repeated
transscleral
cyclophotocoagulation
was
required
—in
oneeyesymptom
shave
notresolved
(192
days/range
35–425
days)
Stum
pfetal.
2008
[21]
Aqueous
misdirection
syndrome
Five
eyes
-Ph
acoemulsificatio
n(one
eye)
-Ph
acoemulsificatio
nand
trabeculectomy(three
eyes)
-Ph
acotrabeculectom
y(one
eye)
-Hyperopia/
shortaxial
length
(<22.64-
mm)
-Narrow
iridocorn-
ealangle
Directly
after
surgeryto
2years
n/a
Large
posterior
Nd:YAGlaser
capsulotom
y/L
PIwas
ineffectivein
deepeningtheAC.
Transscleral
cyclophtotocoagulat-
ion(9–33pulses)of
Allcasesresponded
rapidly,though
inone
eyerequired
asubsequent
second
applicationayear
later
(15–96
months)
142 Graefes Arch Clin Exp Ophthalmol (2018) 256:135–154
Tab
le2
(contin
ued)
Article
Suggestednameof
syndrome
No.of
eyes
(incidence)
Perform
edprocedure
Riskfactors
Tim
eof
occurrence
relatedto
time
ofsurgery
Presum
edpathom
echanism
Suggestedtreatm
ent
Outcome(follow-up
period)
-Shallo
wAC
2–2.5W
for2sin
1–2quadrants
Heindletal.
2013
[22]
One
eye
Phaco+IO
LN/A
10days
Ultrasound
biom
icroscopy
show
santerior
rotatio
nof
theciliary
body
processes
suggestsablocking
mechanism
betweenciliary
processes,IO
L,lens
capsule,andanterior
vitreous
face.
23-gauge
PPV
combinedwith
iridectomyand
peripheralremoval
oflens
capsule
behind
the
iridectomysiteatthe
1–2-o’clockpositio
n.
Com
pleteresolutio
n(6
months)
Prataetal.2
013
[23]
Malignant
glaucoma
Thirty-oneeyes
-Trabeculectom
ywith
mito
mycin
(14eyes)
-Ph
aco+IO
L(8
eyes)
-Ph
acotrabeculectom
ywith
mito
mycin
(three
eyes)
-Plateau
iris
configura-
tion
Median29
days
(range
2–364days).
Anteriorlyrotatedciliary
inultrasound
biom
icroscopy.
Aplateauirisconfiguration,
definedas
largeand/or
anteriorly
positio
nedciliary
body
abuttin
gtheperipheral
iris,partialv
isibility
orabsenceoftheciliarysulcus,
anirisroot
thatangulates
forw
ardandthen
centrally,
presence
ofacentralflatiris
plane,andirido-angle
contactw
asnoticed
in85%
ofeyes
N/A
N/A
Debrouw
ere
etal.2012
[24]
Malignant
glaucoma
Twenty-four
eyes
-Trabeculectom
y(11eyes)
-Ph
aco+IO
L(eight
eyes)
-Ph
acotrabeculectom
y(one
eye)
-Diode
laser
cyclodestructio
n(one
eye)
-ICCE(one
eye)
-Iridotom
y(one
eye)
-Nd:YAGLPI
(one
eye)
-Hyperopia
-Chronic
angle--
closure
glaucoma
-Sh
allow
-ACafter
surgery
N/A
Anteriorrotatio
nof
theciliary
body,sotheaqueous
produced
bytheciliary
body
cannot
followits
norm
alpathway
and
accumulates
behind
the
iris–lensdiaphragm.
Subsequent
increased
pressure
intheposterior
segm
entresultsin
ananterior
displacemento
fthe
iris– lensdiaphragm,
ACflattening
and
secondaryangle
1)Medicaltreatm
ent
2)Nd:YAGlaser
capsulotom
y+
hyaloidotomy
3)Vitrectomy
4)Anteriorvitrectomy
+iridectomy–-
zonulectom
y5)
Full
vitrectomy–phaco–-
iridectomy–-
zonulectom
y
Relapse
in:
1)100%
eyes
2)75%
eyes
3)75%
eyes
4)66%
eyes
5)0%
eyes
(61days/range
13–228
days)
Aryaetal.2004
[25]
Malignant
glaucoma
One
eye
Nd:YAGlaserposterior
capsulotom
yin
pseudophakiceye
-Alteratio
nin
the
anatom
icrelatio
n-ship
involvingthe
ciliary
body,
anterior
hyaloid
Four
days
B-scanultrasonography
show
edaqueouspocketsinthe
vitreous.
Persistent
liquefactionof
the
vitreous
leadingto
shiftin
gof
vitreous
gelforwardand
misdirectionof
aqueous
posteriorly.
Nd:YAGLPI,m
edical
treatm
entfollowed
byPP
Vwith
air
administrationto
theAC
Recurrence—
8days
after
PPV.R
epeatedPP
VstabilizedIO
P(6
months)
Graefes Arch Clin Exp Ophthalmol (2018) 256:135–154 143
Tab
le2
(contin
ued)
Article
Suggestednameof
syndrome
No.of
eyes
(incidence)
Perform
edprocedure
Riskfactors
Tim
eof
occurrence
relatedto
time
ofsurgery
Presum
edpathom
echanism
Suggestedtreatm
ent
Outcome(follow-up
period)
mem
brane
face,and
vitreous
Brooksetal.
1989
[26]
Malignant
glaucoma
One
eye
NdY
AGLPI
Five
days
Strong
tropine-lik
emydriatics
andstrong
mioticsin
such
patientswith
very
shallow
ACsandan
unstablelens
irisdiaphragm.
Panretinal
photocoagulatio
n.Medicaltreatm
ent
Resolutionaftercancelatio
nof
mydriatics
Wuetal.2016
[8]
Malignant
glaucoma
Thirty-eighteyes
(of1432
eyes
undergoing
antig
laucom
asurgical
treatm
entfor
prim
ary
angle-closure
glaucoma)
-After
trabeculectomy(32
eyes
—84.2%)
-Duringtrabeculectomy
(foureyes
—10.5%)
-After
combined
glaucoma/cataract
surgery(twoeyes
—5.3%
)
Duringor
after
surgery
1)Ciliolenticular
blockor
anterior
hyaloidobstruction
2)Slacknessof
lens
zonules
3)Severepostoperative
inflam
mation
4)Non
ciliary
block.Itwas
observed
thatthosepatients
who
developedmalignant
glaucomaduring
surgeryall
show
edahigh
stress
level
andpoor
operative
cooperation.Wespeculated
thatsuch
status
caused
constrictio
nof
extraocular
muscles
pressing
forw
ard,
bringing
aboutthe
increase
ofocular
venous
pressure.
Allpatientswere
initially
given
medicaltreatm
ent
(foureyes).If
medicaltreatm
ent
failed:
1)laserposterior
capsulotom
ywith
hyaloidotomy(two
pseudophakiceyes)
Phakiceyes
underw
ent
surgicaltreatm
ent
2)anterior
vitrectomy--
reform
ationof
AC
was
chosen
ifIO
Pincreased
non-progressively,
thelens
istransparent
andtheACdepthis
ongradeIIor
shallower
according
toSp
aeth
(13eyes)
3)phaco+IO
Lwith
goniosynechialysis
andreform
ationof
ACwas
indicatedif
progressiveIO
Pincrease
isassociated
with
the
developm
ento
rworsening
ofcataract.A
nterior
vitrectomy
procedurewas
performed
ifAC
failedto
form
during
thesurgery(ten
eyes)
4)vitreous
aspiratio
nor
anterior
vitrectomy
was
performed
ifpatientsdeveloped
malignant
glaucoma
Thirtyof
38eyes
didnot
requiretopicaltreatment
achievingIO
P<21
mmHg.With
topicalh
ipotensive
agents:IOP<21
mmHg
infour
eyes,IOPranged
23–26mmHgin
four
eyes.
Com
plications:
One
eyeexhibitedbleeding
attheentrysiteof
vitrectomyinto
vitreous
cavity.C
orneal
endothelial
decompensation
occurred
tooneeye,and
anothereyeshow
edpositiveresponse
tomedicaltherapybut
allergyto
atropine.
(27.1±9.1months)
144 Graefes Arch Clin Exp Ophthalmol (2018) 256:135–154
Tab
le2
(contin
ued)
Article
Suggestednameof
syndrome
No.of
eyes
(incidence)
Perform
edprocedure
Riskfactors
Tim
eof
occurrence
relatedto
time
ofsurgery
Presum
edpathom
echanism
Suggestedtreatm
ent
Outcome(follow-up
period)
during
surgery(nine
eyes).
Inrefractory
relapsed
eyes
with
lowvisual
potential,transscleral
cyclophotocoagula-
tionwas
performed.
Lazar
etal.1981
[27]
Anteriorpupillary
block
Twoeyes
-Su
rgicalperipheral
iridectomy(one
eye)
-Penetratingkeratoplasty
(one
eye)
8h–3days
The
mechanism
iscaused
byadhesionsbetweentheiris
andhyaloidmem
brane/lens.
Pressure
disparity
between
posteriorandAC.leads
tobackwardmovem
ento
fthe
lens.F
urther
interference
ofaqueousflow
dueto
byiridocornealadhesion.
Aspirationof
fluidfrom
theposterior
cham
berwith
a25-gauge
needlein
theslitlamp—
underslit-lamp
visualization—
throughcornea
and
iris/iridectomy.
Medical:1
%atropine,
10%
phenylephrine,
acetazolam
ide+
hypertonicagents
(one
patient)
Acompletereform
ationof
theACoccurred
immediately
inoneeye
orin
30min
inoneeye
Ozekietal.2010
[28]
Ciliovitrealb
lock
One
eye
Trabeculectom
ywith
unplannedzonulectom
yTw
odays
Ciliovitrealb
lock
caused
bythevitreous
herniatio
nthroughtheperipheral
iridectomyto
thelim
bal
incision
with
flatbleb
andAC
Medical:topical
atropine,
beta-blocker,oral
acetazolam
ide,and
intravenous
mannitol.
Healon5
injected
througha
paracentesis
Thisapproach
was
successful,and
the
malignant
mechanism
didnotrecur
over
aperiod
ofalmost
2weeks,untilamore
rigidanddeep
ACwas
constructedby
cataract
surgery.
Massicotte
etal.
1999
[29]
Pseudomalignant
glaucoma
Twoeyes
Partialv
itrectomywith
C3F 8
tamponade
(leaving
theanterior
hyaloid)
inphakiceyes
1–6days
Obstructio
nof
aqueousflow
,either
byresidualanterior
hyaloidor
byfibrin
and
otherinflammatorydebrisat
thelevelo
ftheciliary
body–zonularapparatus.
Anteriorrotatio
nof
the
ciliary
processes
(arrow
head)andan
axially
shallowand
fibrin-filled
ACwas
found
inultrasound
biom
icroscopy
1)Medical
2)Nd:YAGLPI
and
hyaloidectom
ythroughthezonules
was
performed
and
resultedin
only
transientd
eepening
oftheAC
3)Vitreous
cham
ber
gaswas
aspirated,and
tissueplasminogen
activ
ator
10mgwas
givenintracam
erally
4)Phacoem
ulsificatio
n,air–fluidexchange
with
removalof
C3F 8
gasandperipheral
iridectomyfrom
aposteriorapproach
throughthe
remaining
vitreous,
IOPstabilizatio
nandAC
reform
ation(1
year)
Graefes Arch Clin Exp Ophthalmol (2018) 256:135–154 145
Tab
le2
(contin
ued)
Article
Suggestednameof
syndrome
No.of
eyes
(incidence)
Perform
edprocedure
Riskfactors
Tim
eof
occurrence
relatedto
time
ofsurgery
Presum
edpathom
echanism
Suggestedtreatm
ent
Outcome(follow-up
period)
zonules,andiris(one
eye)
Francisand
Babel2000
[30]
Malignant
glaucoma
One
eye
PPVwith
lensectomy,
scleralb
ucklingand
vitreous
injectionof
18%
SF6
Fourteen
days
Aqueous
misdirection
apparentlyrequires
anintact
hyaloid,perhapswith
decreasedperm
eabilityto
aqueousor
reducedsurface
area
forfluidtransfer.
Hydratio
nof
thevitreous
andincrease
invitreous
volume,exacerbatedby
expansile
gases,may
result
inelevated
IOPandaxial
shallowingof
theAC,even
inthepresence
ofapatent
iridectomy.
Surgicalperipheral
iridectomywith
out
anyeffecton
theAC.
PPVwith
aminor
bucklerelease
ACdeepeningandIO
Pnorm
alization
AlB
inAlietal.
2016
[31]
Aqueous
misdirection
syndrome
Sixty-nine
eyes
-Cataractsurgery
(17eyes
—25%)
-Trabeculectom
y(nine
eyes
—13%)
-Ph
acotrabeculectom
y(43
eyes
—62%)
n/a
The
authorsbelieve
that
removalof
theanterior
hyaloidface
isthekeyto
resolvingthesymptom
sof
fluidmisdirection
syndrome,ratherthan
debulkingthevitreous.
Thoughthey
suggestthata
two-portPP
Vwith
surgical
microscopelig
htingcanbe
aseffectiveas
a3-port
procedure
Two-portPPV
orthree-portPPV
.In
pseudophakiceyes,a
hyaloido-zonulo--
iridectomyand
posteriorcapsultomy
was
performed
Prim
aryfunctio
nalsuccess
rateof
PPVin
reducing
IOPto
<21
mmHgwas
81%,w
ithrecurrence
rate11%.Intraoperative
andpostoperative
complications
included
retin
aldetachmentin
twoeyes
and
endophthalmitisin
one
eye(3–156
months)
Bitrianand
Caprioli2
010
[32]
Aqueous
misdirection
syndrome
Five
eyes
-Ph
acoemulsificatio
n(one
eye)
-Ph
acoemulsificatio
n,trabeculectomy,laser
suture
lysis(one
eye)
-Ahm
edim
plant,
phacoemulsificatio
n,Ahm
edrevision
(one
eye)
-Ph
acoemulsificatio
n,Nd:YAGlaser
capsulotom
y(one
eye)
-Ph
acoemulsificatio
n,Ahm
edim
plant,
Nd:YAGlaser
capsulotom
y(one
eye)
One da
y–8-
months
The
exactp
athophysiology
isnotu
nderstoodfully,but
diverseanom
aliesof
the
ciliary
body,choroid,lens,
zonule,and
vitreous
have
been
suggested,causinga
posteriordiversionof
the
aqueoushumor
into
the
vitreous
cavity.A
nanterior
rotatio
nof
theciliary
body
processes,leadingto
cilio
lenticular
touchand
ciliary
block,hasbeen
suggested.
Anteriorvitrectomy
with
hyaloido--
zonulectom
y,peripheral
iridectomy
(pseudophakiceyes).
The
infusion
linewas
placed
intheACto
deepen
it.
Thissurgicalprocedurewas
successful
inresolving
theaqueousmisdirection
inalleyes.Ananatom
icsuccesshasbeen
achieved,inmostcases
IOPnorm
alized
(1–13months)
Tsaietal.1
997
[33]
Malignant
glaucoma/
ciliary
block
Twenty-fiveeyes
Predom
inantly
trabeculectomy.Also
cataractextractio
n,surgicalperipheral
iridectomy,Molteno
implant
n/a
Anatomicalobstructionof
aqueousflow
attheanterior
hyaloid/zonule-lens/ciliary
processinterface.The
authorsconclude
that
surgicalvitrectomyin
the
presence
ofan
intact
Anteriorvitrectomyor
PPV.A
dditional
concom
itant
cataract
extractio
nshould
beperformed
inphakic
eyes,preferablywith
Various
outcom
esdependingon
the
performed
surgical
procedure.In
eyes
with
pre-existin
gcataract,
combinedlens
extractio
n,prim
ary
146 Graefes Arch Clin Exp Ophthalmol (2018) 256:135–154
Tab
le2
(contin
ued)
Article
Suggestednameof
syndrome
No.of
eyes
(incidence)
Perform
edprocedure
Riskfactors
Tim
eof
occurrence
relatedto
time
ofsurgery
Presum
edpathom
echanism
Suggestedtreatm
ent
Outcome(follow-up
period)
posteriorcapsulemay
preclude
thesurgical
resolutio
nof
aqueous
misdirection.
posterior
capsulotom
y.posteriorcapsulectomy
andsurgicalvitrectomy
should
beperformed
(10day-
s–65
months)
Greenfieldetal.
1999
[34]
Aqueous
misdirection
Teneyes
Baerveldt
glaucoma
drainage
device
implantatio
n.In
oneeye,
concurrent
trabeculectomy.
1–343days
Overfiltratio
nassociated
with
thedrainage
device,w
hich
developedwith
in2months
oftheim
plantatio
nWe
proposethatthissyndrome
results
from
acascadeof
events,w
hich
isinitiated
byexternallig
aturerelease.
Thisproduces
immediate
overfiltrationand
shallowingof
theAC,
anterior
rotatio
nof
the
lens–irisdiaphragm,and
posteriordiversionof
aqueous.
Nd:Yag
hyaloidotomy.
PPValone
(pseudophakiceyes)
orwith
lensectomy
(phakiceye)
Normalizationof
anterior
segm
entanatomywas
achieved
with
aqueous
suppressionand
cycloplegiain
oneeye;
infour
eyes
after
Nd:YAGcapsulotom
y;intwoeyes
afterPP
V;in
oneeyeafterPP
Vwith
lensectomy;
twoeyes
afterPP
Vwith
IOL
explantatio
n(1–23months).
Lynchetal.
1986
[35]
Malignant
glaucoma,
cilio
vitrealb
lock
Four
eyes
-ECCE+IO
Land
iridectomy(one
eyes)
-ECCE+IO
Land
trabeculectomy(one
eye)
-ICCEwith
ACIO
Lim
plantatio
nand
iridectomy(one
eye)
-Smalleyes
with
anatom
i-cally
narrow
A-
C/short
axial
length
-History
ofnarrow
angle
glaucoma
2–7days
Increasedresistance
ofthe
anterior
vitreous
isthought
toinitiateor
perpetuatethe
posteriorflow
ofaqueous
humor.T
hisresistance
might
occuratthelevelo
ftheciliary
body,and
has
been
calledcilio
vitreal
block.
Pseudophakiceyes,especially
afterECCEoftencontain
residualcorticallens
material,which
incitesa
cellu
larandfibrousreactio
nin
theequatorialregion
ofthelens
capsule.Thismay
contributetoblockage
ofthe
aqueousflow
throughthe
lens
zonulesor
betweenthe
ciliary
processesand
capsule.
Nd:YAGhyaloidotomy
(one
eye—
unsuccessful)
PPVwith
excision
ofthelens
capsuleand
zonules(foureyes)
IOPstabilizatio
nwith
orwith
outtopical
medications
and
ACdeepeningin
three
offour
eyes
Tomey
etal.
1987
[36]
Malignant
glaucoma,
ciliary
block,
aqueousmisdirection
four
eyes
-ECCE+IO
L(one
eye)
-ECCE+IO
Lwith
trabeculectomy(three
eyes)
1–10
days
Postoperativewound
leakage
may
beacausativefactor.It
seem
splausiblethatthe
initialforw
ardmovem
entof
theiris-lensdiaphragm
caused
bythewound
leak
(and
perhapsalso
aggravated
bytheabsence
ofiridectomyinsomecases)
startsacycleof
aqueous
misdirectionand
Nd:YAGhyaloidotomy
asfirststep
managem
ent
PPV(twoeyes)
Intwoeyes
IOPwas
stabilizedandAC
deepeningwas
achieved
with
Nd:YAG
hyaloidotomy.In
two
eyes,P
PVwas
required;
inoneeyeitwas
redone
with
IOLremoval
(6–18months)
Graefes Arch Clin Exp Ophthalmol (2018) 256:135–154 147
Tab
le2
(contin
ued)
Article
Suggestednameof
syndrome
No.of
eyes
(incidence)
Perform
edprocedure
Riskfactors
Tim
eof
occurrence
relatedto
time
ofsurgery
Presum
edpathom
echanism
Suggestedtreatm
ent
Outcome(follow-up
period)
subsequent
accumulationin
theposteriorsegm
ent.
Interestingly,
trabeculectomyopenings
may
very
wellb
econsidered
delib
erateleaks.
Harboretal.
1996
[37]
Malignant
glaucoma
Twenty-four
eyes
-Trabeculectom
y(sixeyes)
-Trabeculectom
ywith
mito
mycin
(three
eyes)
-Nd:YAGLPI
(foureyes)
-Centralretin
alvein
occlusion(one
eye)
-ECCE+IO
L(six
eyes)
-ECCE+IO
Lwith
trabeculectomy(four
eye)
1–120days
Posteriormisdirectionof
aqueousflow
intoor
behind
thevitreous
body,w
ithsubsequent
increase
invitreous
volumeand
obliterationof
theanterior
andposteriorcham
bers.
Vitrectomywith
out
lensectomy(nine
eyes)
Vitrectomywith
lensectomy(six
eyes)
Nd:YAGLPI
(twoeyes)
Nd:YAGlaserposterior
capsulotom
y(three
eyes)
ECCE(one
eye)
Surgicalperipheral
iridectomy(one
eye)
ACreform
ation(one
eye)
Argon
lasergonioplasty
(one
eye)
Inphakiceyes,the
initial
PPVwas
successful
insevenof
sveneeyes
that
underw
entlensectom
y,andinfive
ofseveneyes
thatdidnotu
ndergo
lensectomy.In
pseudophakiceyes
the
initialvitrectomywas
successful
in9/10
eyes.
Perioperative
complications
included:
-transientserouschoroidal
detachmentintwoeyes
-transientexudativ
eretin
aldetachmentinoneeye
-suprachoroidal
hemorrhagein
oneeye
(1–89months)
Abbreviations
used:A
C,anteriorchamber;ECCE+IO
L,extracapsularcataractextractio
nwith
intraocularlensim
plantatio
n;ICCE,intracapsularcataractextractio
n;IO
L,intraocularlens;IOP,intraocular
pressure;L
PI,laser
peripheraliridotom
y;Nd:YAG,neodymium:yttrium–aluminum
–garnet;phaco+IO
L,phacoem
ulsificatio
nwith
intraocularlens
implantatio
n;PPV,parsplanavitrectomy
148 Graefes Arch Clin Exp Ophthalmol (2018) 256:135–154
In intact capsules, the zonular dehiscence may enable fluidto flow in an unusual pattern, facilitating its entrapment in thePC. This elucidates why this syndrome is commonly associ-ated with lax zonular fibers, i.e., in exfoliation, dense/brunescent cataracts, or spherophakia. However, it can devel-op in the absence of any clinically detectable zonular fiberweakness or disruption [4], and might be associated withspontaneous PC/vitreous pressure elevation induced by intra-operative coughing [6]. As habitually shallowing of the ante-rior chamber is irreversible, Olson et al. defined the term sub-capsular fluid entrapment.
Optimal treatment for acute fluid misdirection syndrome
Faced with these situations, pars plana decompression is re-quired. Exceptionally spontaneous posterior capsule ruptureand liberation of the entrapped fluid has been described [5].Prior to performing a posterior decompression, the surgeonmust be definitely certain that there is no evidence of choroi-dal effusion or hemorrhage. The decompression might be
done by puncture with a straight needle 3 mm from the rimand then aspiration of retrolenticular fluid [3–5]. Vitreous trac-tion might be a concern when performing this procedure.Furthermore, the treatment has not always been described assuccessful [5].
Hence it would be preferable to use a small-gauge trocar/cannula vitrectomy cutter (23-, 25-, or 27-gauge) [7]. Theincision in the pars plana should be made after displacingthe conjunctiva and then fashioning a beveled incision, as ismodern practice for pars plana entries. The cutter can thenremove retrocapsular fluid using a high cut rate.
Mechanisms of chronic fluid misdirection syndrome
This syndrome has also been described as occurring fromhours to months, or years, after the initial surgery [9].Malignant glaucoma is a recalcitrant and potentially devas-tating secondary angle-closure glaucoma. In 1869, vonGraefe described a rare postoperative complication charac-terized by flattening of the anterior chamber and elevated
Table 3 Common risk factors for fluid misdirection syndrome
Onset of fluid misdirection Risk factor Influence on pathophysiology
During phacoemulsification with IOLimplantation or trabeculectomy
- Higher levels of anteriorchamber irrigation [3]
Residual cortical fiber irrigation maneuver (when residual corticalfibers are being removed, the narrow stream of balanced saltsolution generated by the narrow hydrodissection cannula tip maydistort the posterior capsule locally) resulting in localized anteriordisplacement of the contiguous posterior capsule, creating asubcapsular or vitreal space through which the balanced saltsolution may move via the zonular fibers.
- Coughing [6] or high stress levelwith poor intraoperativecooperation [8]
Increased localized pressure in the eye. Constriction of extraocularmuscles pressing forward, bringing about the increase of ocularvenous pressure.
- Hyperopia [3, 6] Small hyperopic eyes with shallow anterior chamber, leading to adecrease of surgical space.
- Intumescent cataract [4, 6] Posterior capsule flaccidity might result in bulging or billowingforward
- Pseudoexfoliation [4] Lax zonular fibers might facilitate the fluid passage through theregion of the zonular fibers.
During penetrating keratoplasty withextracapsular cataract extraction and IOLimplantation
- Open sky irrigation/aspiration[5])
Irrigation under the iris, anteriorly to the anterior capsule
Following phacoemulsification with IOLimplantation, trabeculectomy orphacotrabeculectomy
- Hyperopia [10, 19, 21] Abnormal anatomic relationship between the ciliary body, anteriorhyaloid, and lens in hyperopic eyes. Anterior movement of thelens–iris diaphragm accompanied with poor vitreous conductivityand choroidal expansion.
- History of angle closure[11, 15, 24, 35]
Increased resistance of the anterior vitreous initiates the posterior flowof aqueous humor.
- Plateau iris configuration [23] Anterior rotation of the ciliary body, so the aqueous produced by theciliary body cannot follow its normal pathway and accumulatesbehind the iris–lens diaphragm.
- Shallow anterior chamberafter surgery [21]
A forward displacement of the anterior vitreous with apposition of theanterior hyaloid face against the lens and ciliary body prevents thenormal anterior flow of aqueous humor.
Abbreviations used: IOL, intraocular lens
Graefes Arch Clin Exp Ophthalmol (2018) 256:135–154 149
intraocular pressure (IOP). As a result of its poor response toconventional treatment, it was called malignant glaucoma[1]. The term was further justified by the devastating effectof using pilocarpine as an attempted treatment for this con-dition. It is recognized to comprise the diagnostic triad of adiffusely flat anterior chamber, high intraocular pressure,and aqueous pooling that is sometimes visible in or in frontof the anterior vitreous. This occurs despite the existence ofa patent iridotomy or iridectomy (Fig. 1). It is best knownfollowing trabeculectomy, but has been reported followinga wide variety of anterior segment procedures, includingcataract extraction or implantation of several glaucomadrainage devices, i.e., Ahmed, Molteno, Baerveldt[32–34]. Furthermore, it can occur subsequent to laser
peripheral iridotomy, surgical peripheral iridectomy,c a p s u l o t o m y , c y c l o p h o t o c o a g u l a t i o n ,phacotrabeculectomy, trabeculectomy scleral flap suture ly-sis, trabeculectomy bleb needling, or initiation of topicalmiotic [5, 10, 12, 24, 40]. Although pars plana vitrectomyis an effective treatment formalignant glaucoma, it does notrule out the development of this syndrome postoperatively[29, 30]. Furthermore cases of malignant glaucoma havebeen described following vitrectomy, particularly in phakiceyes [29, 30, 41]. In a study conducted byMatlach et al., theIOP in malignant glaucoma following trabeculectomy wasslightly lower than after other procedures; furthermore, thisgroup of patients required fewer topical IOP-lowering med-ications [42].
There is an anatomical predisposition for malignantglaucoma. Most patients have chronic angle-closure glauco-ma or an anatomically narrow filtration angle. In other studies,plateau iris configuration and hyperopia have been defined asrisk factors [10, 15, 23, 24]. Additionally, anterior rotation ofthe ciliary body processes in ultrasound biomicroscopy mightbe significant for averting aqueous fluid flow [22]. Malignantglaucoma is indeed more common in women than in men. Inwomen, the location of the lens is more forward than that ofthe lens in men, resulting in not only a 4% shallower anteriorchamber but also a narrower space between the lens equatorand ciliary body. Therefore, women are more prone to devel-oping a misdirection of the aqueous flow [43]. Chandler et al.proposed that laxity of lens zonules coupled with pressurefrom the vitreous leads to forward lens movement [44]. Inspherophakia, zonular dehiscence might be accompanied byincreased lens thickness, hence facilitating anterior chambershallowing [45].
Due to the fact that the aqueous humor secreted by theciliary epithelium is not directed to the anterior chamber, theterm aqueous misdirection syndrome has been proposed [10].It might accumulate in the vitreous or adjacent to it in Berger’sspace. A forward displacement of the anterior vitreous withapposition of the anterior hyaloid face against the lens andciliary body might be significant in averting the flow of aque-ous humor [9]. Furthermore, the aqueous might becomeentrapped inside the vitreous cavity as Baqueous pockets^[14, 40]. Other studies suggest that anterior vitreous and ante-rior hyaloid face condensation result in reduced permeabilityto the aqueous.With that, vitreous detachment would facilitatetrapping fluid in the posterior segment [10]. Little et al. [9]suggest that aqueous might accumulate in front of the anteriorvitreous, and its pooling can be sometimes visible.
The misdirection of aqueous leads to increasing the pres-sure of the posterior chamber vitreous (positive vitreous pres-sure glaucoma) [13] and anterior displacement of the lens–irisdiaphragm. Consequently, a characteristic diffuse shallowingof the anterior chamber can be observed, with subsequentangle closure and rise in intraocular pressure [10]. The
Fig. 1 Mechanism of chronic fluid misdirection syndrome. The aqueoushumor secreted by the ciliary epithelium is not directed to the anteriorchamber (1), which leads to positive vitreous pressure (2). This occursdespite the existence of a patent iridotomy or iridectomy (3).Subsequently a diffusely flat anterior chamber (4) and angle closure (5)can be observed with precipitous rise in intraocular pressure
150 Graefes Arch Clin Exp Ophthalmol (2018) 256:135–154
aqueous misdirection is inevitable — Shaffer and Hoskinspostulated a valve-like mechanism by which aqueous humorwas Bmisdirected^ posteriorly [46]. A vicious circle is set up,in that the higher the pressure in the posterior segment, themore firmly the lens is held forward [13, 16].
Quigley et al. suggest that rather than a one-way ball valvemechanism, choroidal expansion could play a significant rolein malignant glaucoma. In an average human eye, the vitreousvolume is approximately 5000 μl and the choroidal volume isabout 480 μl, while the anterior chamber volume is 150 μl[47]. From solid geometry and ocular dimensions, choroidalexpansion by 20% occupies a volume 100 μl, equal to thevolume of the anterior chamber [48]. A 100-μm uniform cho-roidal expansion could increase IOP to 60 mmHg [47]. Withthe expansion of the choroid, the absolute pressure in eachcompartment of the eye increases. This induces a volume lossfrom the anterior chamber, with increased aqueous outflow.As the pressure in the posterior globe is higher than in theanterior chamber, an anterior movement of the vitreous, car-rying the lens and iris with it, leads to further anterior chambernarrowing. This additionally decreases the outflow, causing avicious circle.
Treatment strategies for chronic fluid misdirectionsyndrome
Malignant glaucoma would be more likely to occur in eyeswith higher than normal resistance to vitreous fluid flow.Normal vitreous does not limit the free passage of water, andits fluid conductivity decreases under an increased pressuredifferential [49]. If the transvitreous flow is limited and insuf-ficient to equalize the difference in pressure between the vit-reous chamber and anterior chamber, the vitreous compressesmore. This further decreases its fluid conductivity. The surfacethrough which aqueous exits the vitreous is limited by theciliary body at its perimeter and apposition of vitreous to thelens centrally, forming a doughnut-shaped zone. The compres-sion of vitreous and its anterior movement gradually limits thediffusional area. Furthermore in hyperopic eyes with smalleraxial length and relatively larger lens the doughnut-shapedzone would be only half as large as normal-sized eyes [48].
Tomey et al. [36] claim, that postoperative wound leakagefollowing cataract surgery may be a causative factor for ma-lignant glaucoma. It seems probable that the initial forwardmovement of the iris–lens diaphragm caused by the woundleak starts a cycle of aqueous misdirection and subsequentaccumulation in the posterior segment. This might be aggra-vated by the absence of iridectomy in some cases.Interestingly, filtration surgery with increased aqueous out-flow might also be considered as a triggering factor.
The treatment strategies for malignant glaucoma aretypically aimed at managing IOP and restoring normalanterior segment anatomy. Medical therapy is reported to
be successful. Cycloplegics draw the lens–iris complexposteriorly, widen the ciliary body diameter, increasingforward diffusional area for fluid to leave the posteriorvitreous cavity. Osmotic agents remove fluid from theeye, and aqueous suppressants decrease its production.Although medical treatment can help partially orcompletely stabilize malignant glaucoma, they do not ad-dress the underlying pressure imbalance; thus the relapserate is described to be as high as 100% [24].
In aphakic and pseudophakic eyes neodymium:yttrium–aluminum–garnet (Nd:YAG) laser iridotomy with anteriorhyaloidotomy and posterior capsulotomy (all through thesame location) might stabilize the intraocular pressure [9].This approach leads to relieving aqueous that might beentrapped within the vitreous. Ultrasound biomicroscopic im-aging revealed that anterior rotation of the ciliary body andanterior chamber shallowing normalize after rupture of theanterior hyaloid face [50]. However, the procedure might havea short-term effect with a high recurrence rate of 75%, pre-sumably because the primary mechanism of misdirection isnot counteracted, allowing new aqueous to accumulate in thevitreous cavity [24]. Furthermore, there might be an inflam-matory reaction in blocking the flow of aqueous across thezonules or between the lens capsule and ciliary processes,especially in eyes with residual cortical lens material [35].Some authors underline the efficacy of transscleral cyclopho-tocoagulation [20, 21]. The coagulative necrosis and shrink-age of the ciliary processes disrupts the ciliary–hyaloid inter-face. In addition to decreasing aqueous production, this dis-ruption may subsequently allow normal aqueous flow andmechanical posterior rotation of the ciliary body. This methodneeds to be considered, although some authors prefer to use anon-destructive intervention, especially in a patients withwell-seeing eyes [20]. Importantly, this approach does notcomplicate a subsequent surgical procedure [21]. It is sug-gested that although cyclophotocoagulation helps to achieveresolution in most eyes, performing vitrectomy withhyaloidotomy and iridectomy with implantation of a glauco-ma drainage device in eyes in which laser hyaloidotomy failedcould be a better option [20].
Thus, in several cases surgical treatment might be neces-sary. Certain authors stress that the greatest chance for perma-nent success is related to quick implementation of surgicaltreatment [51]. Furthermore, eyes with higher IOP and shorteraxial length might be more likely to have a poor prognosis[52]. The fundamentals of the treatment are that evacuation ofvitreous and aqueous humor from the vitreous cavity and es-tablishment of communication with the anterior chamberhelps to stop the vicious mechanism that eventually leads toincreased IOP. Pars plana vitrectomy prevents aqueous accu-mulation inside the vitreous cavity, and it has been reported tobe the treatment of choice for pseudophakic malignant glau-coma. Some authors suggest that a total vitrectomy, rather
Graefes Arch Clin Exp Ophthalmol (2018) 256:135–154 151
than partial removal of the anterior vitreous, is favorable [15].However, this may not be enough to disrupt the cycle of ma-lignant glaucoma, as it is postulated that all of the tissues (iris,lens capsule, zonule, and anterior vitreous) have to be re-moved to create a permanent passage between the anteriorchamber and the vitreous cavity [10, 12, 20, 24]. (Fig. 2)Furthermore, Debrouwere et al. [24] emphasized that totalvitrectomy was not effective in 66% of their patients unlessan zonulectomy was added to the procedure. Part of the prob-lem is that during conventional vitrectomy peripheral vitreouscan hardly be completely removed, and that is why relapserate may be very high [15]. Only total vitrectomy combinedwith zonulectomy, iridectomy, and capsulectomy has beendescribed to be effective in 100% on larger groups of patients[12, 24]. In postoperative follow-up, it is important to main-tain patency of newly created passages by using an Nd:YAGlaser [12]. An alternative surgical treatment for pseudophakicmalignant glaucoma suggested by anterior segment surgeonsis zonulo-hyaloido-vitrectomy [14, 15, 17, 18]. In this proce-dure, the anterior vitrectomy is performed from the anterior
chamber approach, through a tunnel within the iridectomy. Ithas been emphasized that this procedure is safer, as theiridectomy is done within visual sight, in contrast to a blindapproach through the pars plana. The initial results of such aprocedure are good, though recurrence might occur in up to40% of cases. The reason is blockage of the channel by vitre-ous or fibrin [17]. In a recent Saudi Arabian study, the efficacyof vitrectomy combined with hyaloido-capsulo-iridectomyhas been confirmed on a group of 69 eyes. A two-port parsplana vitrectomy with surgical microscope lighting can be aseffective as a 3-port procedure [31]. Some authors underlinethat the removal of the anterior hyaloid face withcapsulectomy is the key to resolving the symptoms of fluidmisdirection syndrome, rather than debulking the vitreous [11,31, 53]. Furthermore, small-gauge techniques might be asefficient as traditional 20-gauge vitrectomy [11].
Most of the cases described in literature do relate topseudophakic eyes. If malignant glaucoma develops ina phakic eye most authors recommend concomitant vitrectomyand cataract extraction [10, 33]. Harbor et al. stress thatlensectomy may be considered in eyes with substantial cornealedema or dense cataract, or when the anterior chamber does notdeepen during vitrectomy [37]. Tsai et al. [33] reported bettersurgical outcome if an additional posterior capsulectomy wasperformed. Sharma et al. [10] suggest performing vitrectomy toreduce posterior pressure, followed by phacoemulsification andsubsidiary vitrectomy followed by zonulo-hyaloidectomy.Chaundry et al. [54] claim that if one eye develops aqueousmisdirection after surgery, prophylactic pars plana vitrectomyduring cataract surgery of the contralateral eye may bebeneficial.
Conclusion
The fluid misdirection syndrome is a rare clinical condition char-acterized by an axially very shallow anterior chamber with theabsence of suprachoroidal effusion or hemorrhage and no notice-able pathology of the iris–lens diaphragm. In all of the describedcases, fluid becomes entrapped in PC. The positive pressure ofthe vitreous chamber does move the lens–iris diaphragm frontal-ly, leading to angle closure and restricting spontaneous resolu-tion. Hyperopia and lax zonular fibers/pseudoexfoliation can in-crease the risk for developing this syndrome. Furthermore, afteran episode of malignant glaucoma in one eye, there is a high riskof a malignant course at the time of surgery or in the postopera-tive period [55]. This proves that all these disorders should betreated as one nosological syndrome.
We suggest the term acute fluid misdirection syndrome forthe cascade of events during phacoemulsification surgery. Thepathophysiology of acute fluid misdirection syndrome isbased on inappropriate movement of balanced salt solutionvia the zonular fibers. This definitely better describes the
Fig. 2 Recommended method of treatment. Vitreous decompressionshould be performed, preferably with hyaloido-capsulo-iridectomy. Thistreatment can be achieved with an anterior (1) or posterior (2) approach.In phakic eyes, concomitant cataract extraction would be desired
152 Graefes Arch Clin Exp Ophthalmol (2018) 256:135–154
nature of the syndrome rather than one of its signs. With that,understanding the pathophysiology will lead to unconditionaland thought-out sorting out of the intervention.
We believe chronic fluid misdirection syndrome better de-scribes the nature ofmalignant glaucoma. It is based on similarmechanisms of cilio-lenticular block of aqueous flow leading tothe misdirection of aqueous posteriorly into or beside the vitre-ous gel, leading to the characteristic diffuse shallowing of theanterior chamber accompanied by a precipitous rise in intraoc-ular pressure. It might seem unreasonable to considermalignantglaucoma a chronic process as, clinically, the marked increaseof intraocular pressure occurs with some rapidity. However, itdoes occur gradually. The first symptom is often an improve-ment in near vision secondary to a myopic shift in refraction asthe lens–iris diaphragm moves forward [16]. Furthermore, theinappropriate movement of aqueous and anterior chambershallowing might be observed several days prior to intraocularpressure elevation. Therefore, malignant glaucoma can be dif-ficult to detect early in its course before elevation in IOP occurs.The prognosis of this condition is good with currently availabletreatment modalities, and "malignant glaucoma" no longer de-serves its historical name. It should be emphasized that usingthe current term leads to misunderstanding. It is necessary toexplain to patients with malignant glaucoma that the term doesnot indicate a neoplastic process, and that glaucomatous dam-age to the optic disc is not always a consequence of thecondition.
It is anticipated that using a clearly specified definition offluid misdirection syndrome will enhance the validity of pub-lished data, assist in the identification of risk factors, andmakeit possible to establish an unified effective treatment for thisdangerous condition.
Acknowledgements Authors thank Dr. Tetsuro Oshika from theDepartment of Ophthalmology, University of Tsukuba, Tsukuba, Japanand Dr. Anders Behndig from the Department of Clinical Sciences/Ophthalmology, Umeå University, Umeå, Sweden for the critical discus-sion of our work.
Funding This study was partially funded by the Institute for Research inOphthalmology, Foundation for Ophthalmology Development, Poznan,Poland. The sponsor had no role in the design or conduct of this research.
Compliance with ethical standards All authors certify that they haveno affiliations with or involvement in any organization or entity with anyfinancial interest (such as honoraria; educational grants; participation inspeakers’ bureaus; membership, employment, consultancies, stock own-ership, or other equity interest; and expert testimony or patent-licensingarrangements), or non-financial interest (such as personal or professionalrelationships, affiliations, knowledge, or beliefs) in the subject matter ormaterials discussed in this manuscript.
For this type of study formal consent was not rerequired.Dr. Grzybowski reports grants, personal fees and non-financial support
from Bayer, non-financial support from Novartis, non-financial supportfrom Alcon, non-financial support from Thea, personal fees and non-financial support from Valeant, non-financial support from Santen, out-side the submitted work.
Open Access This article is distributed under the terms of the CreativeCommons At t r ibut ion 4 .0 In te rna t ional License (h t tp : / /creativecommons.org/licenses/by/4.0/), which permits unrestricted use,distribution, and reproduction in any medium, provided you give appro-priate credit to the original author(s) and the source, provide a link to theCreative Commons license, and indicate if changes were made.
References
1. Graefe A (1869) Beiträge zur Pathologie und Therapie desGlaucoms. Albrecht von Graefes Archiv für Ophthalmologie 15:108–252
2. Kam AW, Chen TS, Wang SB, Jain NS, Goh AY, Douglas CP,McKelvie PA, Agar A, Osher RH, Francis IC (2016) Materials inthe vitreous demonstrated under the operating microscope duringcataract surgery and confirmed histologically. Clin ExpOphthalmol. https://doi.org/10.1111/ceo.12818
3. Lau OCF, Montfort JM, Sim BWC, Lim CHL, Chen TSC, RuanCW, Agar A, Francis IC (2014) Acute intraoperative rock-hard eyesyndrome and its management. J Cataract Refract Surg 40:799–804
4. Mackool RJ, Sirota M (1993) Infusion misdirection syndrome. JCataract Refract Surg 19:671–672
5. Olson RJ, Younger KM, Crandall AS, Mamalis N (1994)Subcapsular fluid entrapment in extracapsular cataract surgery.Ophthalmic Surg 25:688–689
6. Dewey SH (2011) BPlease give me another chance. Intraoperativefluidmisdirection associatedwith unexpected coughing,^ presentedat the annual meeting of the AmericanAcademy of Ophthalmology,Orlando, Florida, USA, October 2011. Syllabus, pp 109–110. http://www.aao.org/pdf/Refractive-Surgery-2011-Syllabus.pdf. Accessed8 Aug 2014
7. Grzybowski A, Prasad S (2014) Acute aqueous misdirection syn-drome: pathophysiology and management. J Cataract Refract Surg40:2167
8. Wu Z-H, Wang Y-H, Liu Y (2016) Management strategies in ma-lignant glaucoma secondary to antiglaucoma surgery. Int JOphthalmol 9:63–68
9. Little BC, Hitchings RA (1993) Pseudophakic malignant glauco-ma: Nd:YAG capsulotomy as a primary treatment. Eye 7(Pt 1):102–104
10. Sharma A, Sii F, Shah P, Kirkby GR (2006) Vitrectomy–phacoemulsification–vitrectomy for the management of aqueousmisdirection syndromes in phakic eyes. Ophthalmology 113:1968–1973
11. Meng L, Wei W, Li Y, Hui X, Han X, Shi X (2015) 25-gauge parsplana vitrectomy for ciliary block (malignant) glaucoma. IntOphthalmol 35:487–493
12. Rękas M, Krix-Jachym K, Żarnowski T (2015) Evaluation of theeffectiveness of surgical treatment of malignant glaucoma inpseudophakic eyes through partial PPV with establishment of com-munication between the anterior chamber and the vitreous cavity. JOphthalmol 2015:873124
13. Premsenthil M, Salowi MA, Siew CM, ak Gudom I, Kah T (2012)Spontaneousmalignant glaucoma in a patient with patent peripheraliridotomy. BMC Ophthalmol 12:64
14. Pasaoglu IB, Altan C, Bayraktar S, Satana B, Basarir B (2012)Surgical management of pseudophakic malignant glaucoma via an-terior segment-peripheral iridectomy capsulo-hyaloidectomy andanterior vitrectomy. Case Rep Ophthalmol Med 2012:794938.https://doi.org/10.1155/2012/794938
15. Zarnowski T, Rękas M (2014) Efficacy and safety of a new surgicalmethod to treat malignant glaucoma in pseudophakia: reply. Eye28:1391–1392
Graefes Arch Clin Exp Ophthalmol (2018) 256:135–154 153
16. Shahid H, Salmon JF (2012) Malignant glaucoma: a review of themodern literature. J Ophthalmol 2012:1–6
17. Madgula IM, Anand N (2014) Long-term follow-up of zonulo-hyaloido-vitrectomy for pseudophakic malignant glaucoma.Indian J Ophthalmol 62:1115–1120
18. Lois N, Wong D, Groenewald C (2001) New surgical approach inthe management of pseudophakic malignant glaucoma.Ophthalmology 108:780–783
19. Varma DK, Belovay GW, Tam DY, Ahmed IIK (2014) Malignantglaucoma after cataract surgery. J Cataract Refract Surg 40:1843–1849
20. Dave P, Senthil S, Rao HL, Garudadri CS (2013) Treatment out-comes in malignant glaucoma. Ophthalmology 120:984–990
21. Stumpf TH, AustinM, Bloom PA,McNaught A,Morgan JE (2008)Transscleral cyclodiode laser photocoagulation in the treatment ofaqueous misdirection syndrome. Ophthalmology 115:2058–2061
22. Heindl LM, Koch KR, Cursiefen C, Konen W (2013) Optical co-herence tomography and ultrasound biomicroscopy in the manage-ment of pseudophakic malignant glaucoma. Graefes Arch Clin ExpOphthalmol 251:2261–2263
23. Prata TS, Dorairaj S, De Moraes CGV, Mehta S, Sbeity Z, Tello C,Liebmann J, Ritch R (2013) Is preoperative ciliary body and irisanatomical configuration a predictor of malignant glaucoma devel-opment? Clin Exp Ophthalmol 41:541–545
24. Debrouwere V, Stalmans P, Van Calster J, Spileers W, Zeyen T,Stalmans I (2012) Outcomes of different management options formalignant glaucoma: a retrospective study. Graefes Arch Clin ExpOphthalmol 250:131–141
25. Arya SK, Sonika KS, Kumar S, KangM, Sood S (2004) Malignantglaucoma as a complication of Nd:YAG laser posteriorcapsulotomy. Ophthalmic Surg Lasers Imaging 35:248–250
26. Brooks AM, Harper CA, Gillies WE (1989) Occurrence of malig-nant glaucoma after laser iridotomy. Br J Ophthalmol 73:617–620
27. Lazar M, Godel V (1981) Anterior pupillary block. ActaOphthalmol 59:221–224
28. Ozeki N, Yuki K, Kimura I (2010) Alternative approach to treatingmalignant glaucoma after trabeculectomy with unplannedzonulectomy. Clin Ophthalmol 4:383–385
29. Massicotte EC, Schuman JS (1999) A malignant glaucoma-likesyndrome following pars plana vitrectomy. Ophthalmology 106:1375–1379
30. Francis BA, Babel D (2000) Malignant glaucoma (aqueous misdi-rection) after pars plana vitrectomy. Ophthalmology 107:1220–1222
31. Al Bin Ali GY, Al-Mahmood AM, Khandekar R, Abboud EB,Edward DP, Kozak I (2017) Outcomes of pars plana vitrectomyin the management of refractory aqueous misdirection syndrome.Retina 37:1916–1922
32. Bitrian E, Caprioli J (2010) Pars plana anterior vitrectomy,hyaloido-zonulectomy, and iridectomy for aqueous humor misdi-rection. Am J Ophthalmol 150:82–87.e1
33. Tsai JC, Barton KA, Miller MH, Khaw PT, Hitchings RA (1997)Surgical results in malignant glaucoma refractory to medical orlaser therapy. Eye 11(Pt 5):677–681
34. Greenfield DS, Tello C, Budenz DL, Liebmann JM, Ritch R (1999)Aqueous misdirection after glaucoma drainage device implantation.Ophthalmology 106:1035–1040
35. Lynch MG, Brown RH, Michels RG, Pollack IP, Stark WJ (1986)Surgical vitrectomy for pseudophakic malignant glaucoma. Am JOphthalmol 102:149–153
36. Tomey KF, Senft SH, Antonios SR, Shammas IV, Shihab ZM,Traverso CE (1987) Aqueous misdirection and flat chamber afterposterior chamber implants with and without trabeculectomy. ArchOphthalmol 105:770–773
37. Harbour JW, Rubsamen PE, Palmberg P (1996) Pars plana vitrec-tomy in the management of phakic and pseudophakic malignantglaucoma. Arch Ophthalmol 114:1073–1078
38. Kawasaki S, Suzuki T, Yamaguchi M, Tasaka Y, Shiraishi A, UnoT, Sadamoto M, Minami N, Naganobu K, Ohashi Y (2009)Disruption of the posterior chamber-anterior hyaloid membranebarrier during phacoemulsification and aspiration as revealed bycontrast-enhanced magnetic resonance imaging. Arch Ophthalmol127:465–470
39. Kawasaki S, Tasaka Y, Suzuki T, Zheng X, Shiraishi A, Uno T,Ohashi Y (2011) Influence of elevated intraocular pressure on theposterior chamber–anterior hyaloid membrane barrier during cata-ract operations. Arch Ophthalmol 129:751–757
40. Mastropasqua L, Ciancaglini M, Carpineto P, Lobefalo L, GallengaPE (1994) Aqueous misdirection syndrome: a complication of neo-dymium: YAG posterior capsulotomy. J Cataract Refract Surg 20:563–565
41. Zacharia PT, Abboud EB (1998) Recalcitrant malignant glaucomafollowing pars plana vitrectomy, scleral buckle, and extracapsularcataract extraction with posterior chamber intraocular lens implan-tation. Ophthalmic Surg Lasers 29:323–327
42. Matlach J, Slobodda J, Grehn F, Klink T (2012) Pars plana vitrec-tomy for malignant glaucoma in nonglaucomatous and in filteredglaucomatous eyes. Clin Ophthalmol 6:1959–1966
43. de Preobrajensky N, Mrejen S, Adam R, Ayello-Scheer S, GendronG, Rodallec T, Sahel J-A, Barale P-O (2010) 23-gaugetransconjunctival sutureless vitrectomy: a retrospective study of164 consecutive cases. J Fr Ophtalmol 33:99–104
44. Chandler PA (1951) Malignant glaucoma. Am J Ophthalmol 34:993–1000
45. Kaushik S, Sachdev N, Pandav SS, Gupta A, Ram J (2006)Bilateral acute angle closure glaucoma as a presentation of isolatedmicrospherophakia in an adult: case report. BMC Ophthalmol 6:29
46. Shaffer RN (1954) The role of vitreous detachment in aphakic andmalignant glaucoma. Trans Am Acad Ophthalmol Otolaryngol 58:217–231
47. Quigley HA, Friedman DS, Congdon NG (2003) Possible mecha-nisms of primary angle-closure and malignant glaucoma. JGlaucoma 12:167–180
48. Quigley HA (2009) Angle-closure glaucoma—simpler answers tocomplex mechanisms: LXVI Edward Jackson Memorial Lecture.Am J Ophthalmol 148:657.e1–669.e1
49. Fatt I (1977) Hydraulic flow conductivity of the vitreous gel. InvestOphthalmol Vis Sci 16:565–568
50. Tello C, Chi T, Shepps G, Liebmann J, Ritch R (1993) Ultrasoundbiomicroscopy in pseudophakic malignant glaucoma.Ophthalmology 100:1330–1334
51. Scott AS, Smith VH (1961) Retrolental decompression for malig-nant glaucoma. Br J Ophthalmol 45:654–661
52. Zhou C, Qian S, Yao J, Tang Y, Qian J, Lu Y, Xu G, Sun X (2012)Clinical analysis of 50 Chinese patients with aqueous misdirectionsyndrome: a retrospective hospital-based study. J Int Med Res 40:1568–1579
53. Byrnes GA, Leen MM, Wong TP, Benson WE (1995) Vitrectomyfor ciliary block (malignant) glaucoma. Ophthalmology 102:1308–1311
54. Chaudhry NA, Flynn HW Jr, Murray TG, Nicholson D, PalmbergPF (2000) Pars plana vitrectomy during cataract surgery for preven-tion of aqueous misdirection in high-risk fellow eyes. Am JOphthalmol 129:387–388
55. Simmons RJ (1972) Malignant glaucoma. Br J Ophthalmol 56:263–272
56. Saunders PP, Douglas GR, Feldman F, Stein RM (1992) Bilateralmalignant glaucoma. Can J Ophthalmol 27:19–21
154 Graefes Arch Clin Exp Ophthalmol (2018) 256:135–154