RevBrasAnestesiol.2017;67(6):663---669
REVISTA
BRASILEIRA
DE
ANESTESIOLOGIA
PublicaçãoOficialdaSociedadeBrasileiradeAnestesiologia www.sba.com.brLETTERS
TO
THE
EDITOR
Anesthesia
for
intestinal
obstruction
in
a
six
years
old
child
with
cerebro-oculo-facio-skeletal
syndrome
Anestesia
para
obstruc
¸ão
intestinal
em
uma
crianc
¸a
de
seis
anos
de
idade
com
síndrome
COFS
DearEditor,
Wereportouranesthesiamanagementofa6yearsoldgirl
with Cerebro-Oculo-Facio-Skeletal (COFS) Syndrome who
wasconsultedasintestinalobstructioninemergency
depart-mentofourhospital.Shewasalreadyafollow-uppatientof
pediatricdepartment anddiagnosedasCOFSsyndromeby
thempreviously.
She had characteristic signs of thesyndrome including
growth failure, weighning 6kg, cachexia, micrognathism,
microcephaly. She had small mouth opening and dental
abnormalities,flexurecontracturesatherelbowandknees.
Her preoperative blood tests were within normal limits
except C-reactive protein (40mg.dL−1) and White Blood
Cell(18×103).Plevraleffusionandabdominalvolvuluswas
foundinherradiologicalimages.Herinitialvitalsignswere
innormalrange.Intravenousaccesswasestablishedby 24
gaugecatheters. Anesthesiawasinduced withsevoflurane
8%inair---oxygenmixtureand12mcgfentanylintravenously.
Intubation was done with a cuffed 4.0mm endotracheal
tubesuccesfully.Anesthesiamaintenancewasprovidedwith
sevoflurane 2% in air---oxygen mixture, and fentanyl. She
wasventilated in pressure control ventilation. There was
noanestheticproblemsduringtheoperationthatcontinued
for 135min. After adequate breathing spontaneously, she
wasextubatedsmoothly.
COFSsyndromeisinitiallydescribedbyPenaandShokeir
in 1974 and has been recognized as a rare, autosomal
recessive disorder characterized by DNA repair defect.1
Cockayne syndrome Type II is known as COFS syndrome.
Degenerative problem of the brain and spinal cord is
usually seen beforebirth. Clinical findings are
psychomo-tor development delay, neurological dysfunction,
periph-eralneuropathy,microcephaly,micrognathism, hypotonia,
hyporeflexia,convulsions, and congenitalcataract. And is
associatedwith feeding difficulties, sensorineural hearing
loss,coxavalga,kneeflexioncontracture.1Progressive
neu-rologicaldegenerationcan lead toa risk of hyperkalemia
byusingsuccinylcholinein thesepatients.Reports canbe
seen describing the anesthesia management of Cockayne
syndrome in the literature but there was no about the
anesthesia management of COFS syndrome. Difficult
air-waymanagementwasoneof themostimportantproblem
inanesthesiaprocedure.Also,duetothecontractionsand
osteoporosis,adequatecareshouldbeconsideredwhile
giv-ingpositionduringthesurgery.
Thatis difficulttopredict the effectsof muscle
relax-ants’inthesepatientsfortheyarecachectic,almostlackof
musclewithinsufficientdevelopmentoftheneuromuscular
junctionforthis,theyareneedtobeavoided.Wesawthat
onlyonecasethatmusclerelexantwasnotusedduring anes-thesiaintheliterature.2Endotrachealintubationwasdone
viasevofluranecombinedwithnitrousoxideinthispatient
whowasscheduledforliverbiopsy,andtheprocedurelasted
for13min.Wedidnotusemusclerelexantsininductionand
maintenanceofanesthesia, too.Our procedurecontinued
135min.Weusedweight-appropiateendotrachealtubethan
age-appropiateonebutweprefferredcuffed---tubetoavoid theriskofaspiration.
Inconclusion;alloftheseassociatedanomaliesand
dys-functionsrequiringattentionandexperience.Usingsmaller
endotracheal tubes and avoiding muscle relexants during
intubationcanbekeptinmindduringanesthesia
manage-mentofCOFSsyndrome.
Conflicts
of
interest
Theauthorsdeclarenoconflictsofinterest.
References
1.LyonsK.unusualbrainand/orneuromuscularfindingswith asso-ciateddefectsCerebro-Oculo-Facio-Skeletal(COFS)Syndrome. Smith’srecognizablepatternsofhumanmalformation.7thed; 2013.p.234.
664 LETTERSTOTHEEDITOR
AhmetYükseka,YükselElab,ElifDo˘ganBakib,∗,
SerdarKokulub
aAfyonKocatepeUniversity,FacultyofMedicine,
AnesthesiologyandReanimationDepartment, Afyonkarahisar,Turkey
bKocatepeUniversity,FacultyofMedicine,Anesthesiology
andReanimationDepartment,Afyonkarahisar,Turkey
∗Correspondingauthor.
E-mail:elifbaki1973@mynet.com(E.D.Baki).
http://dx.doi.org/10.1016/j.bjane.2016.02.004
0104-0014/
©2016SociedadeBrasileiradeAnestesiologia.Publishedby ElsevierEditoraLtda.ThisisanopenaccessarticleundertheCC BY-NC-NDlicense( http://creativecommons.org/licenses/by-nc-nd/4.0/).
Severe
hypoxemia
follows
hypoxic
pulmonary
vasoconstriction
and/or
hypoxic
pulmonary
vasoconstriction
inhibition
by
inhaled
anesthetics:
prognostic
potential
of
100%
shunt
fractions
Hipoxemia
grave
após
vasoconstric
¸ão
hipóxica
pulmonar
e/ou
inibic
¸ão
da
vasoconstric
¸ão
hipóxica
pulmonar
por
anestésicos
inalatórios:
potencial
prognóstico
de
frac
¸ões
de
shunt
de
100%
DearEditor,
HypoxicPulmonaryVasoconstriction(HPV)wasfirstreported
byBindsleyetal.inadultpatientsandwasmanagedusing
double lumen catheters to ventilate one lung with 100%
oxygen and the other with 95% N2 and 5% oxygen during
intravenousbarbiturateandfentanylanesthesia.1However,
‘‘HPV inhibition’’ has predominantly been attributed to
theuse of inhalation agents andis considered as acause
of hypoxia duringanesthesia. Moreover,both in vitro and
in vivo studies have demonstrated that inhalation agents
inhibit HPV under a range of conditions,2---5 and
sevoflu-ranehasbeenshowntodecreaseHPVinadose-dependent
manner.4,6,7
Case
description
Herewereportacaseofseverehypoxemiaduring
sevoflu-raneinduction ina 6year-oldboy whowasscheduled for
adenotonsillectomy.The patientwastreated according to
AmericanSocietyofAnesthesiology(ASA)classIandweighed
22kg. Pre-operative assessments, physical examinations,
andlaboratoryinvestigationswere unremarkable,andthe
patienthadahemoglobinlevelof12mg.dL−1anda
hemat-ocritof36%.Followingtransfertotheoperatingroomwith
nopre-medication,routineElectrocardiography(ECG),
non-invasiveblood pressure, andSpO2 levels weremonitored.
Anesthesiawasintroduced viaa facemaskand apediatric
circlesystemproviding8%sevofluranein100%oxygenata
flow rate of 6L.min−1. Subsequently, sevoflurane
concen-trationswerereducedto5%withinthefirstminuteandto
2% onlossof eyelash reflexes.Following intravenous
can-nulation, rocuronium(0.6mg.kg−1) wasadministered,and
SpO2 levelsrapidly andprogressively decreased from 98%
to 38% at 10 and 15min of induction, respectively, with
no clinical explanation. As a consequence, the patients’
HeartRate(HR)suddenlydecreasedfrom109to90bpmin
responsetohypoxia, andsevofluraneconcentrations were
reducedto2%andsubsequently discontinuedprior to
tra-chealintubationandventilationwith100%oxygen.Efficient lungventilationwaspossiblethroughouttheperiod.A
clini-calimprovementwasobservedwithinsecondsofintubation
andventilation, andSpO2 levelsand HRreturnedto100%
and 118bpm, respectively. No blood pressure
abnormali-ties were observed during the procedure, and the rapid
restorationofSpO2andHRwith100%oxygenwasconsidered
symptomatic of an adverse drug reaction. Thus,
anesthe-siawasmaintainedwith1%---1.5%sevofluraneandoxygenin
50%N2O.
Discussion
HPV is considered as a protective mechanism that
opti-mizessystemicoxygendelivery.Thus,theinhibitionofHPV
by inhalationalagents isbelieved tocausehypoxia during
anesthesia.However,theprotectiveeffectsofHPVagainst
hypoxia maydepend onthesize ofaffected lung regions.
Lungtissuesaregloballyaffectedbyallpulmonary
anesthet-ics, andsevofluranehas been shown toinducepulmonary
vessel dilatation in normoxia.7 Therefore, HPV should be
considered as a multifactorial response tolocal or global
pulmonaryhypoxiaduringacutehypoxia.Thus,thepresent
observationssuggestthatthedegreeofacutelocalshunting
canbeusedtodeterminewhetherHPVmechanism is
use-ful ornot.The typeofanesthetics canbesignificantwith
regardtothisreaction.
Itiswidelyacceptedthatinhalationagentshaveglobal
effects on pulmonary vessels during either induction or
maintenance of anesthesia. Thus, hypoxemia because of
globalHPVislikelyinthepresenceof100%intrapulmonary
shuntfractions,andtheprotectiveeffectsofHPV-influenced
perfusiontothebetterventilatedlungregionstoimprove
oxygenation may be abolished under these conditions. In
contrast,inhibitionofHPVbyinhalationagentsincommonly
affected lungsleads torapidonsetof hypoxemiabecause
of normal effects on perfusion and alveolar hypoxia and
directeffectsof anesthesia. Thus,theeffectofHPV
inhi-bitionbyinhalationagentsorhypoxiamaydependonglobal
effectsonlungsandtheensuingshuntfractions.Therefore,
