vascular rings slings

8/12/2019 Vascular Rings Slings

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CXRCXR


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DiagnosisDiagnosis::

Vascular RingVascular Ring-- probable double aortic archprobable double aortic arch


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Vascular Rings & SlingsVascular Rings & Slings

Morning ReportMorning Report

December 13, 2005December 13, 2005


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IntroductionIntroduction

Vascular ringVascular ringrefers to a variety ofrefers to a variety of

congenital vascular anomalies that encirclecongenital vascular anomalies that encircleand compress the esophagus and tracheaand compress the esophagus and trachea

Complete orComplete ortrue ringtrue ringvs. Incompletevs. Incomplete

Abnormalities of the aortic arch and itsAbnormalities of the aortic arch and itsbranching arteries make up most of thebranching arteries make up most of the

vascular ringsvascular rings(exception(exception-- pulmonary artery sling)pulmonary artery sling)


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EmbryologyEmbryology

In the embryonic aortic arch,In the embryonic aortic arch,the ventral and dorsal aortathe ventral and dorsal aorta

are connected by six primitiveare connected by six primitiveaortic archesaortic arches

Regressions or persistence ofRegressions or persistence of

the primitive arches cause thethe primitive arches cause thedifferent vascular ringdifferent vascular ringanomaliesanomalies

AAleftleftororrightrightaortic arch isaortic arch isdefined by thedefined by the mainstemmainstembronchusbronchus that is crossed by thethat is crossed by theaorta archaorta arch (not to the side of the(not to the side of themidline the aorta descends)midline the aorta descends)

www.childsdoc.org/spring98/vascular


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Clinical PresentationsClinical Presentations

Respiratory SymptomsRespiratory Symptoms(Most Common)(Most Common)

InspiratoryInspiratory stridorstridor

noisy breathingnoisy breathingoror

wheezingwheezing Chronic coughChronic cough

Recurrent respiratoryRecurrent respiratoryinfectionsinfections

Hoarse cryHoarse cry ALTE/apneaALTE/apnea (infants)(infants)

Feeding SymptomsFeeding Symptoms(More common in older pts)(More common in older pts)

Gagging or choking w/Gagging or choking w/

foodfood

Recurrent emesisRecurrent emesis

DysphagiaDysphagia (typically in older(typically in olderchildren/ especially with solidchildren/ especially with solid

foods)foods)

Failure to thriveFailure to thrive (food(foodavoidance)avoidance)

Symptoms are often made worse w/ feeding and during intercurrent illness


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Physical ExamPhysical Exam

Often NormalOften Normal

Poor weight gain (if severe compression)Poor weight gain (if severe compression)

Pulmonary exam may reveal wheezing,Pulmonary exam may reveal wheezing, stridorstridor,,dyspneadyspnea, retractions, retractions

Abnormal positioning may be observedAbnormal positioning may be observed Lie w/ neck extended, back arched when supine toLie w/ neck extended, back arched when supine to

minimize tracheal narrowingminimize tracheal narrowing


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Vascular RingsVascular Rings

CompleteComplete

(encircle and compress the(encircle and compress theesophagus and trachea)esophagus and trachea)

Double aortic archDouble aortic arch

Right aortic arch w/Right aortic arch w/aberrant leftaberrant left

subclaviansubclavian (and left(and leftligamentumligamentum arteriosumarteriosum))

IncompleteIncomplete

(compress but do not encircle(compress but do not encirclethe esophagus and trachea)the esophagus and trachea)

Aberrant rightAberrant rightsubclaviansubclavian arteryartery

AnomalousAnomalous innominateinnominatearteryartery

Pulmonary vascularPulmonary vascular

slingsling Right aortic arch w/Right aortic arch w/

mirror imagemirror image

branchingbranching

These two anomaliesmake up >95% of allcomplete vascular rings


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Park: Pediatric Cardiology for Practitioners, 4th ed., Copyright 2002 Mosby, Inc


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Normal Aortic Arch AnatomyNormal Aortic Arch Anatomy

(aka innominate

artery)

www.pediatriconcall.com/fordoctor/Diseasesandcondition/Difficultydia.asp


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Double Aortic ArchDouble Aortic Arch

Most common vascularMost common vascularring (40%)ring (40%)

Persistence of both rightPersistence of both rightand leftand left branchialbranchial archesarches

Infants present early inInfants present early in

life w/ respiratory distresslife w/ respiratory distress((stridorstridor) and feeding) and feedingproblemsproblems

RightRight

--arch dominant isarch dominant is

most common (75%)most common (75%)

Usually an isolatedUsually an isolatedanomalyanomaly



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Right Aortic Arch w/ Aberrant LeftRight Aortic Arch w/ Aberrant Left

SubclavianSubclavianArteryArtery Second most common vascularSecond most common vascular

ring (30%)ring (30%)

The ring is formed by the rightThe ring is formed by the rightaortic arch w/ left sidedaortic arch w/ left sidedligamentumligamentum arteriosumarteriosum

attached to an aberrant LSAattached to an aberrant LSA

Present later in life (3Present later in life (3--9mo)9mo)b/cb/c the ring isthe ring islooserloosercompared to double aortic archcompared to double aortic arch

Not often associated w/Not often associated w/congenital heart defectscongenital heart defects(except w/(except w/mirror imagemirror image

branchingbranchingtype)type)



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Right Aortic Arch AnomaliesRight Aortic Arch Anomalies


Complete Ring Incomplete Ring


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Incomplete Vascular RingsIncomplete Vascular Rings

AnomalousAnomalous InnominateInnominateArteryArtery TheThe innominateinnominate arises from the left side of the aortic arch andarises from the left side of the aortic arch and

compresses the tracheacompresses the trachea Asymptomatic or causes mild respiratory symptomsAsymptomatic or causes mild respiratory symptoms

BariumBarium EsophagramEsophagram is normal;is normal; BronchoscopyBronchoscopy can be diagnosticcan be diagnostic

Commonly associated with other cardiac defectsCommonly associated with other cardiac defects

Aberrant RightAberrant Right SubclavianSubclavianArteryArtery RSA arises from the descending aorta and courses behind theRSA arises from the descending aorta and courses behind the

esophagusesophagus Usually asymptomaticUsually asymptomatic

Mild feeding problems may occur/swallowing dysfunctionMild feeding problems may occur/swallowing dysfunction

Usually an isolated anomalyUsually an isolated anomaly


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Anomalous Left Pulmonary ArteryAnomalous Left Pulmonary Artery

Pulmonary Vascular SlingPulmonary Vascular Sling Left PA arises from the RPALeft PA arises from the RPA

and courses behind theand courses behind thetrachea and in front of thetrachea and in front of the

esophagus to enter theesophagus to enter the hilumhilumof the left lungof the left lung

Respiratory AND feedingRespiratory AND feeding

problems may occurproblems may occur--symptoms may be severesymptoms may be severe

EsophagramEsophagram showsshows anterioranterior

compression onlycompression only

BronchoscopyBronchoscopy is required tois required tor/or/o associated trachealassociated trachealstenosisstenosis w/ complete trachealw/ complete tracheal

ringsrings ringring--sling complexsling complex(50%)(50%) www.childsdoc.org/spring98/vascular


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DiagnosisDiagnosis

CXRCXR(AP and lateral)(AP and lateral) Tracheal narrowing and/or displacement (lateral)Tracheal narrowing and/or displacement (lateral)

Aortic arch locationAortic arch location Tracheal Position: abnormal deviation to the leftTracheal Position: abnormal deviation to the left

(due to the aorta coursing over the right(due to the aorta coursing over the right mainstemmainstem bronchus)bronchus) ORORMidline position with double aortic archMidline position with double aortic arch

AtelectasisAtelectasis, hyperinflation, or pneumonia may be present, hyperinflation, or pneumonia may be present(especially in pulmonary artery slings)(especially in pulmonary artery slings)

BariumBarium EsophagramEsophagram

Most important and reliable diagnostic toolMost important and reliable diagnostic tool Except in anomalousExcept in anomalous innominateinnominate arteryartery

PosteriorPosterior (and anterior) compression of esophagus on lateral(and anterior) compression of esophagus on lateral Except in pulmonary artery slingExcept in pulmonary artery slinganterioranterior compression onlycompression only


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CXRCXR

www.uhrad.com/ pedsarc/peds056a.jpg


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Tracheal DeviationTracheal Deviation

www.pediatriconcall.com/fordoctor/Diseasesandcondition/Difficultydia.asp


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BariumBarium EsophagramEsophagram


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Other ImagingOther Imaging ECHOECHO

Recommended to exclude other cardiac defects and visualizes theRecommended to exclude other cardiac defects and visualizes thevascular ringvascular ring

Very useful in diagnosing pulmonary artery slingVery useful in diagnosing pulmonary artery sling

CT/MRICT/MRI Identify vascular structures and anatomy ofIdentify vascular structures and anatomy of tracheobronchialtracheobronchial treetree

AngiographyAngiography ConsideredConsideredgold standardgold standard, but rarely needed for diagnosis, but rarely needed for diagnosis

BronchoscopyBronchoscopy

Use as a diagnostic tool is controversialUse as a diagnostic tool is controversial Recommended for diagnosis of a vascular sling toRecommended for diagnosis of a vascular sling to r/or/o concomitantconcomitant

tracheal rings (and useful for aberranttracheal rings (and useful for aberrant innominateinnominate artery)artery)


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ManagementManagement

Asymptomatic patients need no surgical treatment, evenAsymptomatic patients need no surgical treatment, evenwhen anomalies are found incidentallywhen anomalies are found incidentally

Medical management is recommended for infants withMedical management is recommended for infants withmild symptomsmild symptoms

Careful feeding w/ soft foodsCareful feeding w/ soft foods Aggressive treatment of pulmonary infectionsAggressive treatment of pulmonary infections

Respiratory distress,Respiratory distress, h/oh/o recurrent pulmonary infections,recurrent pulmonary infections,apneicapneic spells, FTT are indications for surgicalspells, FTT are indications for surgicalinterventionintervention


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Surgical ManagementSurgical Management

Double Aortic ArchDouble Aortic Arch Division of the smaller of the two arches (usually the left archDivision of the smaller of the two arches (usually the left arch))

Right Aortic Arch w/ Aberrant LSARight Aortic Arch w/ Aberrant LSA Division of theDivision of the ligamentumligamentum arteriosumarteriosum

AnomalousAnomalous InnominateInnominateArteryArtery

Surgical suturing of the artery to the sternum (if >75% trachealSurgical suturing of the artery to the sternum (if >75% trachealnarrowing)narrowing)-- rarely indicatedrarely indicated

Aberrant rightAberrant right SubclavianSubclavianArteryArtery Surgical interruption of the artery (rarely indicated)Surgical interruption of the artery (rarely indicated)

Anomalous Left Pulmonary ArteryAnomalous Left Pulmonary Artery (Pulmonary Vascular(Pulmonary VascularSling)Sling) Surgical division andSurgical division and reimplantationreimplantation of the LPAof the LPA


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ComplicationsComplications

Surgical Mortality rate is generally low (

vascular rings slings

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