rev bras hematol hemoter. 2015;37(4):272–274
w w w . r b h h . o r g
Revista
Brasileira
de
Hematologia
e
Hemoterapia
Brazilian
Journal
of
Hematology
and
Hemotherapy
Case
Report
An
unexpected
full
neurological
recovery
after
cardiac
arrest
in
a
sickle
cell
anemia
patient
with
bilateral
cervical
carotid
artery
disease
Aline
Cristina
Peluccio
Martins,
Gisele
Sampaio
Silva,
Samuel
Ademola
Adegoke,
Daniela
Laranja
Gomes
Rodrigues,
Josefina
Aparecida
Pellegrini
Braga,
Maria
Stella
Figueiredo
∗UniversidadeFederaldeSãoPaulo(UNIFESP),SãoPaulo,SP,Brazil
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r
t
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e
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o
Articlehistory: Received2April2015 Accepted16April2015 Availableonline18May2015
Introduction
Sickle cell anemia (SCA) is ahereditary hemoglobinopathy causedbyhomozygosityforsicklehemoglobin(HbS).HbSis causedbyasinglemutationinthe-globingene(HBB,glu6val) resultinginthepresenceofvalineinsteadofglutamicacidin thesixthpositionofthe-globinchain.1,2HbScanform
poly-merswhendeoxygenatedleadingtovaso-occlusionandtissue ischemiawhichmayresultinseverepainofthechest,back, abdomen,orextremities.2,3
Acutechestsyndrome(ACS)isanacuteillness character-izedbyfeverand/orrespiratorysymptoms,accompaniedby newpulmonaryinfiltratesonachestX-ray.4–10Itistheleading
causeofhospitalizationandintensivecareunitadmission,4,6
aswellasthemostcommoncauseofdeathinadultpatients withSCAevenamongthoseonhydroxyurea(HU)therapy.8,9
Factorssuchasinfection,fatembolism,iatrogenicfluid over-load,hypoventilationfrom painor narcoticanalgesics,and
∗ Correspondingauthor at:UniversidadeFederaldeSãoPaulo,UNIFESP,EscolaPaulista deMedicina,RDr Diogode Faria,825,Vila
Clementino,CEP04037-002SãoPaulo,SP,Brazil.
E-mailaddress:stella.figueiredo@unifesp.br(M.S.Figueiredo).
vaso-occlusionofthepulmonaryvasculaturecontributetothe pathogenesisofACS.1,2,7Neurologiccomplicationsfollowing
ACS occurin about22% ofadultsand 8%ofchildrenwith SCA.10Thepathophysiologyofneurologicaldeficitsinpatients
withACSseemstoberelatedtoanabruptdecreasein oxy-genationinthevascularbedofthecentralnervoussystem.
Acuteischemicstroke(AIS) isadevastatingneurological complicationofSCA,occurringinabout8%ofHbSSchildren bytheirseconddecadeoflife.ChildrenwithSCAandstrokes frequently present withvasculopathyin thedistalinternal carotid and middle cerebral arteries, although extracranial vasculopathycanalsobepresent.2Otherlesscommon
neu-rologic complications of SCA include hemorrhagic stroke, transientischemicattack,silentcerebralinfarct,duralvenous sinus thrombosis, posteriorreversible encephalopathy syn-drome(PRES),andmigraine.5,11,12
Althoughocclusionofcervicalcarotidarteriesisaknown cause of AIS, these vessels are not routinely screened in sickle celldisease.Thesepatientsare commonlyexamined
http://dx.doi.org/10.1016/j.bjhh.2015.04.003
revbrashematolhemoter.2015;37(4):272–274
273
Figure1–Brainmagneticresonanceimageshowing sequelaeintherighttemporalregion.
withneurologicalimagingsuchasmagneticresonance imag-ing(MRI);magneticresonanceangiographyandtranscranial Dopplersonography(TCD).However,theseexamsaremainly carried out to identify lesions in the major intracerebral vessels.12
WereportacaseofanadultSCApatientwhopresented withACSfollowedbycardiacarrestandcomaduetocerebral hypoperfusion.
Case
report
A23-year-old female withSCA and apast medicalhistory oftwoepisodesofischemic stroke wasadmittedto hospi-talduetofever,tachypnea,thoracicpain,hypoxemia,cough andwheezing.Shehad beenonchronicbloodtransfusions for14years sinceherfirststrokeandhad alsobeentaking hydroxyureafortwoyears.Thisstrategywaschosendueto thedifficultyinmaintainingtheidealintervalbetween trans-fusionsasaresultofalloimmunization.
Auscultationofthechestrevealedreducedbreathsounds at the left pulmonary base. A chest X-ray showed a left lowerlobeinfiltrateandtheoxygensaturationwas95%with a continuous positive airway pressure using 50% oxygen. Despite treatment for ACS(supportive care, fluid manage-ment,oxygenation,chestphysiotherapyandantibiotics),two days after hospital admission the patient suffered cardio-respiratoryarrest.Afterasuccessfulresuscitation,although sherespondedtopain,shehadnospontaneouseyeopening andnoverbalresponse.Shealsohadbilateraldeviationofeye movementtotheright,butnootherclinicalsignsofmeningeal irritation.Thediagnosisofcoma(GlasgowComaScore5)was subsequentlymade.
Brainmagneticresonanceimagingafterthecardiacarrest showedonlyoldischemicareascompatiblewithherprevious strokes(Figure1).Acomputerizedtomographyangiography revealedseverestenosis ofthe leftcervical internalcarotid artery and occlusion of the right cervical internal carotid artery(Figure2).TCDshowedsymmetricbutdecreasedblood
Figure2–Computedtomographyangiographywithpartial andtotalobstructionoftheleftandrightcervicalcarotid arteries.
flowvelocitiesinbothmiddlecerebralarteries(time-averaged maximummeanvelocity:40cm/s),aswellascollateralflow fromtheposteriorcirculation.
Sheremainedincomafor19daysandduringthisperiod shewastreatedwithmultipleexchangeblood transfusions inordertomaintaintheHbSconcentrationat<30%.Shehad fullneurologicalrecoverywithoutnewdeficits(GlasgowComa Score15).Shehasbeenfollowedupandtreatedwithchronic blood transfusionsandacetylsalicylate(aspirin) forthe last threeyears.
Discussion
WedescribeapatientwithSCAwhopresentedwithACS com-plicatedbycardiacarrestandcoma.Despitehavingbilateral cervicalcarotidarterydisease,thepatienthadfull neurologi-calrecovery.Inpatientsresuscitatedfromcardiacarrest,the neurologicaloutcomedependsonimmediaterestorationof systemic circulation and oxygenation tomeet the cerebral oxygen demand.13 Inour patient,cerebral flowwas
proba-blyevenmorecompromisedduringcardiacarrestbecauseof bilateralcervicalcarotidarterydisease.
274
revbrashematolhemoter.2015;37(4):272–274howeverwedid nothavean evaluationofherintracranial vasculaturebeforethecardiacarrest.
Collateralcirculationcandevelopinpatientswith intracra-nial and cervical artery occlusions. For instance, major collateral anastomoses can occur between the ophthalmic branchoftheinternalcarotidarteryandtheterminaltwigsof theinternalandexternalmaxillarytributariesoftheexternal carotidarteries.Adequatecollateralvesselsconferprotection intheeventofischemiclesions.16Ourpatienthadlow
cere-bralbloodflowvelocitiesintheanteriorcirculationwithrobust collateralflowthroughtheposteriorcirculation.Herexcellent neurologicaloutcomewasprobablyrelatedtothepresenceof posteriorcirculationcollateralvessels.
Themanagementofsymptomaticcervicalcarotidartery disease in patients without SCA is well established, with revascularizationbeingthegoldstandardtreatment.18–20 In
patients with SCA and cervical carotid artery disease, the impactoftherapiessuchasendarterectomyorangioplasty withstenting isuncertain. Rather, patientscontinue to be managed with chronic blood transfusions similar to those withintracranialcarotidarterydisease.
In conclusion, a good neurological outcome inpatients withbilateralcervicalcarotidarterydiseaseispossibleevenin thesettingofextremelylowcerebralbloodflowasseenduring cardiacarrest.Theroleofcollateralcirculationbetween extra-andintra-cranialvesselsinpatientswithSCAneedsfurther evaluationsincethiscouldinfluencethedecisiontoconsider revascularization therapies inpatients with cervical artery disease.Also,thevalueofcervicalcarotidarteryDoppler ultra-sound scanningshould bestudiedprospectively, sincethis quickand non-invasive proceduremay identifypatients at increasedriskofstrokewhowouldotherwisebemisdiagnosed asPRESorseveralotherdifferentialdiagnosisofSCApatients withacuteneurologicmanifestations.
Conflicts
of
interest
Theauthorsdeclarenoconflictsofinterest.
Acknowledgment
A.C.P.M.wassupportedbyFAPESP(12/19346-1)
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