RevBrasAnestesiol.2017;67(5):541---543
REVISTA
BRASILEIRA
DE
ANESTESIOLOGIA
PublicaçãoOficialdaSociedadeBrasileiradeAnestesiologiawww.sba.com.br
CLINICAL
INFORMATION
Anesthetic
considerations
for
a
patient
with
hereditary
angioedema
---
A
clinical
case
Maria
J.L.
Vilac
¸a
∗,
Filipa
M.
Coelho,
Ana
Faísco,
Cristina
Carmona
HospitalProfessorDoutorFernandoFonseca,Servic¸odeAnestesiologia,Reanimac¸ãoeTerapêuticadaDor,Amadora,Portugal
Received14February2015;accepted23March2015 Availableonline18September2016
KEYWORDS
Hereditary angioedema; Immune-hemotherapy; Prophylaxis
Abstract Hereditaryangioedema(HAE),withanestimatedprevalenceof1:50,000,isarare butpotentiallyfataldisease.Itmaypresentwithrecurrentsystemicedemaofthesubcutaneous tissueandmucousmembranes.PatientswithHAEareatincreasedriskforclinicalworsening withsurgicalstress,andmaydeveloprespiratorydistresssyndromeduetoimpairedairwayand hemodynamic instability.The perioperativemanagementofthese patients requiresspecific interventions.Wepresentaclinicalcaseofawoman,50yearsold,withHAEtypeIIscheduled
forureteralstentplacementviaendoscopicapproach.
©2015SociedadeBrasileiradeAnestesiologia.Publishedby ElsevierEditoraLtda.Thisisan openaccessarticleundertheCCBY-NC-NDlicense( http://creativecommons.org/licenses/by-nc-nd/4.0/).
PALAVRAS-CHAVE
Angioedema hereditário;
Imuno-hemoterapia; Profilaxia
Considerac¸õesanestésicasperanteumdoentecomangioedemahereditário---Caso
clínico
Resumo Oangioedemahereditário (AEH),comuma prevalênciaestimadade 1:50000 pes-soas,éumadoenc¸araramaspotencialmentefatal.Podeseapresentarcomedemasistêmico recorrentedotecidosubcutâneoedasmucosas.OsdoentescomAEHtêmumriscoacrescido deagudizac¸ãoclínicacomoestressecirúrgico,podemdesenvolversíndromesdedificuldade respiratóriaporcompromissodaviaaéreaedeinstabilidadehemodinâmica.Aabordagem peri-operatóriadessesdoentesrequerintervenc¸õesespecíficas.Apresentamosum casoclínicode umamulherde50anoscomAEHtipoIIindicadapararealizarureteroscopiacomcolocac¸ãode
stent.
©2015SociedadeBrasileiradeAnestesiologia.PublicadoporElsevierEditoraLtda.Este ´eum artigo OpenAccess sobumalicenc¸aCCBY-NC-ND( http://creativecommons.org/licenses/by-nc-nd/4.0/).
∗Correspondingauthor.
E-mail:[email protected](M.J.Vilac¸a).
http://dx.doi.org/10.1016/j.bjane.2015.03.007
542 M.J.Vilac¸aetal.
Introduction
Hereditaryangioedema(HAE)isanautosomaldominant
dis-ease characterizedby quantitative or qualitative changes
in the level of the first complement cascade component
inhibitor(orC1esteraseinhibitor---C1-INH),allowing
uncon-trolledactivation of the classicalcomplement cascade.1,2
HAE classification had two variants phenotypes: type I
is characterized by low plasma levels of normally
func-tional C1-INH; type II is characterized by the presence
of normal or high plasma levels of C1-INH nonfunctional
or abnormally functional.1 More recently, the existence
of a third type of an estrogen-dependant HAE with
nor-malfunctionaland quantitativelevelsofC1-INH hasbeen
identified.3
Despite its name, C1-INH not only acts on the
complementcascade,italsoinhibitsproteasesfrom
coagu-lation/fibrinolysiscascadeandkininpathways.InHAE,the
associatedsymptomsaremainlyduetointerferenceonkinin
pathwayswithincreasedbradykininproduction. Clinically,
HAEmaypresentwithsystemicandrecurrentedemaof
sub-cutaneoustissueormucousmembrane,withinvolvementof
thegastrointestinalsystem.Itcanbemisinterpretedasan
acuteabdomencondition;airwayconditionwithrespiratory
distress,laryngospasm,bronchospasm,andchoking;
hemo-dynamicinstabilityduetoanaphylacticshockanddeath.1,4
Traumaistheprecipitatingfactoroftheclinicalpicturein
about one third of patients. Other triggering factors are
infection,anxiety, and estrogens. In a significant number
ofcases,theprecipitatingfactorisnotidentified.5,6
Case
report
We describe the perioperative anesthesia care to a 50
yearsold woman scheduled for urologic elective surgery:
ureteroscopy with stenting for non-radiopaque kidney
stones. The patient had a diagnosis of HAE type II and a
positivefamily history (father andbrother withthe same
condition).Shewasbeingfollowed-upatthehospitalwith
immunoallergologyconsultationandhadnoregulartherapy.
After careful preanesthetic evaluation, and in
accor-dance with the preoperative protocol of the
immune-hematology service of our hospital and her
immunoaller-gologyconsultation, prophylaxis was performed withfour
oraldosesofstanozolol4mg(Winstrol®)twodayspriorto
surgeryandadministrationof1000UofC1-INHconcentrate
(Berinert®)45minbeforesurgery,atslowintravenousbolus.
Premedicationwithdiazepam1mgwasgiven atthe night
beforesurgeryandonthemorningofsurgery.
Onthedayofsurgeryanduponarrivalintheoperating
room,shewasmonitoredwithstandardmonitoringand
pre-treatedwithintravenousmidazolam(2.5mg).Subarachnoid
blockadewastheanesthetictechniqueofchoice,performed
with a 25G beveled needle at L3---L4 level with
bupiva-caine0.5%(15mg),withmedianapproachandablockade
uptoT10.Parecoxib (40mg)wasadministeredand
antibi-oticprophylaxisperformedwithintravenouscefoxitin(2g).
According to the protocol, 500U of C1-INH concentrate
(Berinert®) were maintained on standby in the operating
room,intheeventofanacuteclinicalpicture.
Thesurgerylasted20min;thepatientremained
hemo-dynamically stable during surgery and in the immediate
postoperative period. Postoperative care did not require
specificityassociatedwithHAE,andthepatientwastreated
withintravenousparacetamolandIVparecoxib,antibiotic
andfluidtherapy.Thepatientwasdischargedafter24h.
Discussion
There arenumerous factorsprecipitatingacute
exacerba-tionsofHAE,particularlyperioperativeanxietyandsurgical
trauma, hence the importance of an adequate
premedi-cation.
Theacuteepisodeapproach,withsymptomsandvarying
severity,iscontroversial.Evidence-basedmedicinesuggests
that acute exacerbations may not respond to treatment
withepinephrine,antihistamines,orglucocorticoids,amore
specific therapy is required.5,7 The initial approach of a
severeacuteepisodeshouldincludetheuseofrecombinant
C1-inhibitor drugsorbradykininreceptorantagonists.2,3,6,7
When these drugs are not available, other approaches
includetheuseofhigherdosesofandrogens,suchas
dana-zolor derivatives, tranexamicacid (TA), epinephrine(not
always effectively), analgesic control, fluid therapy, and
intensive supportive therapy.5,6 The use of fresh frozen
plasmaremainscontroversialduetoitstheoreticalpotential
toexacerbateandperpetuatetheshock.6
The most appropriate approach for this pathology is
theprophylaxis.5,6Thus, inelective surgery,theapproach
shouldincludeanxiolyticpremedicationand:
(a) Use of recombinant factor C1-INH in the following
dosages:500U(if<50kg);1000U(if>50kg,but<100kg)
or4000U(if>100kg)30---60minpriortothesurgical
pro-cedure,withdailyrepetitioniftheriskofprecipitating
anacuteepisoderemainshigh.
(b) Androgenderivatives, uptofive tosevendaysbefore
surgery.
(c) Eventually TA, although not as effective as androgen
derivatives.
(d) Doubleprophylactictherapy,aswasouroption.
Theregional anesthesiatechniqueoption alsopresents
advantagesovergeneralanesthesia,asitdoesnotimplyan
airwayactivemanipulationthat,bytriggeringanepisodeof
exacerbationwithamorelocalizedimpact,couldleadtoa
laryngealedema.
In conclusion, HAE is a rare disease, with a few
contact by most anesthesiologists. Due to the potential
for originating severe complications, it requires a careful
perioperativepreparation,withtheinvolvementof
multidis-ciplinaryteams(immuno-hemotherapists,surgeons,critical
care physicians, immunoallergologists, and
anesthesiolo-gists),adoptionofanappropriateprophylaxis,andcorrect
monitoringofthepatient.
Conflicts
of
interest
Anestheticconsiderationsforapatientwithhereditaryangioedema---Aclinicalcase 543
References
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