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64 MDCT 3D Assessment of Complex Congenital Heart and Bilateral Tracheal Bronchi: A Case Report

ศุภÔกÒ กฤษณÕไพºูÅย์1*สุภÒพÃ โÃยมณÕ2

64 MDCT 3D Assessment of Complex Congenital Heart and Bilateral Tracheal Bronchi: A Case Report.Supika Kritsaneepaiboon1, Supaporn Roymanee2

1Department of Radiology, 2Department of Pediatrics, Faculty of Medicine, Prince of Songkla University, Hat Yai, 90110, Thailand.*E-mail: supikak@yahoo.comSongkla Med J 2015;32(2):103-107

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¤ำÒสำÒ¤Ñญ:กลุม่อาการทีไ่มส่ามารถแยกซา้ย-ขวาได,้กลุม่อาการไมม่มีา้ม,ภาวะแขนงหลอดลมงอกจากทอ่ลมใหญ่

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ศุภÔกÒ กฤษณÕไพºูÅย์ แÅะ สุภÒพÃ โÃยมณÕ

Abstract: Thetrachealbronchusmanifestsavarietyoftracheobronchialanomalies.Bilateraltrachealbronchiarerare.Anomaloustrachealbronchusisusuallyasymptomatic. Case report: Wereportan8-yearoldgirlwhohadunderlyingcomplexcongenitalheartdiseases:uni-ventricularheart,tricuspidatresia,andseverepulmonarystenosisandwhowasincidentallyfoundtohaveaspleniasyndromeandbilateraltrachealbronchifromthecontrastenhancedmultidetectorcomputedtomography(MDCT). Conclusion:Thebilateraltrachealbronchiaremoreoftenfoundinpatientswithaspleniasyndromeandconsideredbilateralrightbronchi.

Keywords:asplenia,heterotaxysyndrome,trachealbronchus

Introduction Trachealbronchusisrelativelyrareandoftenincidentallydiscoveredduringbronchoscopeoratchestcomputedtomography(CT).1-3Thepresen-tationcanvaryfromasymptomaticclinicalwithconservativetreatmentorrespiratoryproblemsinintubatedpatients.2-4Trachealbronchusismorecommoninpatientwithassociatedconditionsuchascongenitalheartdiseasethaninpatientswithoutcongenitalheartdisease.2Thiscasepresentedbilateraltrachealbronchiwithheterotaxysyndrome.

Case report An8-yearoldgirlpresentedwithunderlyingcomplexcongenitalheartdiseases:uni-ventricularheart,tricuspidatresia,andseverepulmonarystenosis.ThepatientunderwentbilateralBlalockTaussigshuntandthenbilateralGlennoperationat1yearofageandlateralFontanoperationat7yearsofage.Shehadnohistoryofrecurrentpneumonia.

ThechestCTwasperformedtoevaluatecausesofdesaturation.ArterialandvenousenhancedCTscanofthechest(PhillipsBrilliance64slice,protocol:110mAs,120kVp,2mm,40mLofUltravist370)showedtricuspidatresia,rightsidedaorticarch,severesupra-valvularpulmonarystenosis,multiplesmallaorto-pulmonarycollateralsandhepaticcongestion(Figures1Aand1B).ThelateralFontanSVCandIVCpathwaysshowednothrombusorstenosis(Figures2Aand2B)indelayedscanimages.Aspleniasyndromeandbilateraltrachealbronchi(displacedapicalsubtype)wereincidentallyfoundwithnoassociatedatelectasis(Figures3A,3Band3C).

Discussion Atruetrachealbronchusistheupperlobebronchusarisingfromthelateralwallofthetracheausuallywithin2cmandupto6cmfromthecarinaanditcouldbetiny,stenoticorblind-ended.The

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Complex Congenital Heart Disease and Bilateral Tracheal Bronchi

Kritsaneepaiboon S and Roymanee S.

incidenceofrightandlefttrachealbronchusis0.1%-2%and0.3%-1%respectively.1,2Thetracheal

bronchusisclassifiedintodisplacedorsuper-

numerarysubtype.Itisdefinedasdisplacedtype

ifonebranchoftheupperlobebronchusismissing

anddescribedassupernumerarysubtypewhenthe

upperlobebronchushasanormalandcomplete

trifurcation.Thedisplacedsubtypeismorecommon

thansupernumerarysubtype.1,5Anomaloustracheal

bronchusisusuallyasymptomatic;however,recurrent

pneumonia,bronchiectasis,focalemphysema,

persistentrightupperlobeatelectasisinintubated

patientsorrespiratorydistresscanoccur,which

resultingfromimpaireddrainageattheoriginof

trachealbronchus.1,2,5Theanglebetweenthetracheal

bronchusandtracheacanvaryfrom22°to108°.

Childrenwithgreater-angletrachealbronchusor

small-sizedtrachealbronchushavelesschanceof

accidentalendotrachealintubutionthanthesmall-

angletrachealbronchustooccludethelumenof

trachealbronchusleadingtoobstructedsegmental

airwayanddeficientventilationoftheremaining

lung.2AssociatedanomaliesincludeDown’ssyndrome,

ribabnormalities,VATERsyndrome,congenital

heartdiseases,abnormalpulmonarylobulation,and

anomalouspulmonaryarterialsupplyandvenous

drainage.4-6Althoughtruebilateraltrachealbronchi

areextremelyrare,ithasbeenreportedupto24%

ofpatientswithaspleniasyndrome.7Patientswith

heterotaxysyndrome,asplenicsubtype,havetwo

“rightsides”andabsentspleen.Thebronchialpattern

inaspleniasyndromeisbilateralrightsidedbronchi

orbilateraleparterialbronchi,thusthebilateral

trachealbronchiarebelievedtobebilateralright

bronchi.2,7MDCTisthenon-invasiveandreliable

imagingtechniquetodiagnosetrachealbronchus

andalsoassociatedotheranomalies,particularly

forthreedimensional(3D)assessment.7,8The

sensitivityofMDCTtodiagnosetrachealbronchus

is100%andcanbeassistivetodetectorexclude

thisairwayabnormality.7Furthermore,theMDCT

can provide the distance between the tracheal

bronchusandcarina,thesizeoftrachealbronchus,

theanglebetweenthetrachealbronchusandthe

trachea,andparticularity3D-MDCTinparticularly

candynamicallydemonstratethespecialrelation-

shipbetweenarterialandtrachealanomalies.7,8

Figure 1A AxialenhancedMDCTshowstricuspid atresia(whitearrow)seenashypodense fattytissuepreventingconnection betweenrightatriumandhypoplastic rightventricle(*).Rightsidedaortic arch(grayarrow)isalsodemonstrated.

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โäหÑÇใจพÔกÒÃแต่กำÒà¹ÔดซѺซ้อ¹Ã่ÇมกѺภÒÇะแ¢¹§หÅอดÅม§อกจÒก·่อÅมใหญ่

ศุภÔกÒ กฤษณÕไพºูÅย์ แÅะ สุภÒพÃ โÃยมณÕ

Figure 2A Coronalmulti-planarreformation (MPR)enhancedMDCTshowinglateral Fontantunnel(*)withpatentsuperior venacava(SVC)(arrow)pathway.

Figure 1B MDCT-3Dvolumerenderingview ofcardiacandgreatvesselsshowing multiplesmallmajoraortopulmonary collateralarteries(MAPCAs)(arrows) arisingfromtheproximaldescending thoracicaorta.

Figure 3A Coronalminimal-intensityprojection (minIP)imagerevealsbilateraltracheal bronchi(displacedapicalsubtype) (arrows).

Figure 2B CoronalMPRenhancedMDCTshowing lateralFontantunnel(*)withpatent inferiorvenacava(IVC)(arrow) pathway.Thespleenisabsentatleft upperabdomen(openarrow)indicating aspleniaorrightisomerism.

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Complex Congenital Heart Disease and Bilateral Tracheal Bronchi

Kritsaneepaiboon S and Roymanee S.

Figure 3CCoronalMPRMDCT(lungwindow) displayingbilateralminorfissures indicatingbilateraltrilobedlungs(arrows) inrightisomerism.

Figure 3B 3D-shadedsurfacedisplay(SSD)opaque lungimagedisplayingbilateraltracheal bronchi(arrows).

Conclusion The bilateral tracheal bronchi are more

often found in patients with congenital heart

disease and asplenia syndrome considered to be

bilateral right bronchi. 3D-MDCT is the reliable,

noninvasive and powerful technique to demon-

strate associated arterial and tracheal anomalies.

References 1.GhayeB,SzapiroD,FanchampsJM,etal.Congenital

bronchialabnormalitiesrevisited.Radiographics2001;

21:105-19.

2.MingZ,LinZ.Evaluationoftrachealbronchusin

ChinesechildrenusingmultidetectorCT.Pediatr

Radiol2007;37:1230-4.

3.CopeR,CampbellJR,WallM.Bilateraltracheal

bronchi.JPediatrSurg1986;21:443-4.

4.O’SullivanBP,FrassicaJJ,RayderSM.Tracheal

bronchus:acauseofprolongedatelectasisin

intubatedchildren.Chest1998;113:537-40.

5.KumagaeY,JingujiM,TanakaD,etal.Anadult

caseofbilateraltruetrachealbronchiassociated

withhemoptysis.JThoracImaging2006;21:293-5.

6.ChauKW,NgDK,ChongAS,etal.Tracheal

bronchus.HongKongMedJ2003;9:71-2.

7.MingZ,HongS,BiaoJ.Imagesincardiovascular

medicine.Aspleniasyndromewithbilateraltracheal

bronchi.Circulation2008;118:196-7.

8.WatabnabeK,UeseK,HiguchiO,etal.Three-

dimensionalcomputedtomographicfindingsof

bilateraltrachalbronchus.PediatrCardiol2009;30:

87-8.