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Congenitallobaremphysema(CLE)isapotentiallyreversiblethoughpossiblylifethreateningcauseof
respiratorydistressintheneonate.
Seetheimagesbelowofcongenitallobaremphysema.

Afrontalradiographofthechestinaneonateshows
markedoverdistentionoftheleftupperlobewithmediastinalshifttotheright.

Histopathologyofcongenitallobaremphysemawith

markedoverdistentionofallalveoli.
overexpandedandshowsnootherintrinsicabnormality.

Resectedlobeis

Congenitallobaremphysemaismostoftendetectedinneonatesoridentifiedduringinutero
ultrasound.Anomaliesareinfrequentandusuallypresentatbirth.Lobardistentioncanbevisible
duringinuteroultrasoundasanoverinflated,fluidfilledlobe;inlessseverecases,thediagnosisis
madeininfancyorchildhood.[1,2,3,4,5,6]
CLEalmostalwaysinvolvesonelobe,withratesofoccurrenceasfollows:

Leftupperlobe41%

Rightmiddlelobe34%

Rightupperlobe21%

Congenitallobaremphysemahas2forms:

Hypoalveolar(fewerthanexpectednumberofalveoli)

Polyalveolar(greaterthanexpectednumberofalveoli)

Thethoraxontheinvolvedsideishyperresonantwithdecreasedorabsentbreathsoundsand
transillumination.Progressiverespiratorydistressfrombirthreflectsthedegreeofemphysema;
symptomsareattheirworstinthefirstmonth.Occasionally,patientspresentinlaterchildhoodor
adulthood.
Congenitallobaremphysema(ie,congenitallesion)shouldbedifferentiatedfromSwyerJames
syndrome(ie,acquiredpulmonaryabnormalitysecondarytoinfection).
InSwyerJamessyndrome,infectionresultsinthefollowing:

Vascularcompromise

"Pruning"ofperipheralpulmonaryvasculature

Smallbuthyperlucentlung(astheopposite,normallunggrows,theinvolvedlungdoesnot
growandappearsmoreradiolucent)

Congenital lobar emphysema

Author
Christopher M Oermann, MD
Section Editors
Joseph A Garcia-Prats, MD
Gregory Redding, MD
Deputy Editor
Alison G Hoppin, MD
INTRODUCTION
Congenitallobaremphysema(CLE)isadevelopmentalanomalyofthelowerrespiratorytractthatis
characterizedbyhyperinflationofoneormoreofthepulmonarylobes[1,2].OthertermsforCLE
includecongenitallobaroverinflationandinfantilelobaremphysema[35].
EPIDEMIOLOGY
Congenitallobaremphysema(CLE)isararecongenitalmalformationwithaprevalenceof1in20,000
to1in30,000[6].CLEwasdiagnosedin10of70patientswithcongenitalmalformationsofthelung
seenfrom1970to1995atChildren'sNationalMedicalCenterinWashington,DC[7].Dependingupon
thepatternofreferrals,othertertiarymedicalcentersmaytreatoneortwocasesperyear[3,8].
Malesappeartobeaffectedmorethanfemales,inaratioof3:1[1].Thereasonforthemale
predominanceisunknown[1,3,5].
PATHOGENESIS
Progressivelobarhyperinflationislikelythefinalcommonpathwaythatresultsfromavarietyof
disruptionsinbronchopulmonarydevelopment.Theseresultfromabnormalinteractionsbetween
embryonicendodermalandmesodermalcomponentsofthelung.Disturbancesmayleadtochangesin
thenumberofairwaysoralveolioralveolarsize[9].However,adefinitivecausativeagentcannotbe
identifiedinapproximately50percentofcases[10].
Themostfrequentlyidentifiedcauseofcongenitallobaremphysema(CLE)isobstructionofthe
developingairway,whichoccursin25percentofcases[1].Airwayobstructioncanbeintrinsicor
extrinsic,withtheformermorecommon.Thisleadstothecreationofa"ballvalve"mechanismin
whichagreatervolumeofairenterstheaffectedlobeduringinspirationthanleavesduringexpiration,
producingairtrapping.

Abstract
Summary
Congenitallobaremphysema(CLE)characterizedbyoverdistensionandairtrappingintheaffected
lobeisoneofthecausesofinfantilerespiratorydistressrequiringsurgicalresectionofaffectedlobe.
Atinduction,positivepressureventilationcanexpandtheemphysematouslobecompressingthe
normallungresultinginseverecardiovascularcompromise.Wereportacaseof28dayoldbabywith
CLEpostedforemergencylobectomy.Strategiestopreventhyperinflationandanaesthetic
considerationsofvarioustechniquesadoptedforlungseparationininfantshavebeenreviewed.

Keywords: Congenitallobar emphysema, Neonatal anaesthesia,

Hyperinflation of emphysematous lung, Positive pressure ventilation
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Introduction
Congenitallobaremphysema(CLE)characterizedbyunilobaralveolardistensionsecondaryto
bronchomalaciaorabsentcartilageisararecondition.Thisdiseasepresentsasrespiratorydistressdue
totheventilationperfusionmismatchasaresultofthehyperinflatedlungcausingcompression
atelectasisontheipsilateralorthecontralateralsidewithmediastinalshift.In1954GrossandLewis,
publishedthefirstcasereportofCLE.1Malebabiesareaffectedmoreoftenthanfemaleintheratioof
3:2.2.Theincidenceofleftupperlobeinvolvementis43%,rightmiddlelobe32%,rightupperlobe
20%,andbilateralinvolvement20%.3
Theexactetiologyofthediseaseisnotknown,butseveralintrinsicandextrinsiccauseshavebeen
postulated4.Presentingfeaturesintheseinfantscanbedyspnea,tachypnea,retraction,wheezing,
coughing,cyanosis,andasymmetricbreathsounds.Intheseinfants,thereisincreasedintrathoracic
pressurebecauseofhyperinflationofoneormorepulmonarylobes,leadingtomediastinalshiftand
atelectasisoftheipsilateralorcontralaterallobesofthelung.Thiscausesdisplacementofheartsounds,
decreasedvenousreturn,andvaryingdegreesofventilationperfusionmismatch,whichleadsto
hypoxia.Chestradiographshelptodiagnosebutisnotdefinitive4.ACTscanconfirmsthediagnosis
andmayruleoutassociatedanomalousvascularslings.Associatedcongenitalheartdiseaseorvascular
anomaliesmayoccurin12%14%ofthesepatients5.Thus,allpatientsshouldhaveadequate
preoperativecardiacevaluationbyechocardiographyandCTscan.Cardiaccatheterizationand
angiographyarenecessaryinchildrenwithknownorsuspectedcongenitalcardiovascularlesions.

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Case report
A28dayoldmalebabyweighing3.4Kgwasreferredasacaseofperinatalasphyxiawithrespiratory
distressnotrespondingtomedicalmanagement.
Onexamination,babywastachypnoeicwithflaringofalaenasiandsubcostalretraction.Thepulse
ratewasaround150/min.Onexaminationoftherespiratorysystem,decreasedbreathsoundsontheleft
hemithoraxwasnoted.Onexaminationofthecardiovascularsystem,theheartsoundswereshiftedto
theoppositeside.Nogrosscardiacanomalywasfound.
Followinginvestigationswerecarriedout:completebloodcount,bloodglucose,bloodurea,serum
creatinineandelectrolytesandchestXray(PAandlateralview.ChestXrayshowedincreased

translucencyontheleftsidewithtrachealandmediastinalshifttotherightside.CTscanconfumedthe
diagnosisofCLEoftheleftupperlobe(Fig1,,2
2).

Fig 1 & 2
CT thorax showing hyperlucent, hyperexpanded left upper lobe
causing compression of the remaining lung and mediastinal shift to
right.
Theneonatewaspostedforleftupperlobectomy.

Anaesthetic management:
Preoperativeexaminationrevealedtachycardiaandtachypnoeawithsignsofrespiratorydistress.On
auscultation,thereweredecreasedbreathsoundsonthelefthemithorax.Thecardiovascularsystem
wasnormal.Oxygensaturation(SpO2)was84%inair,buttherewasnovisiblecyanosis.Routine
hematologicalandbiochemicalinvestigationswerewithinnormallimits.Echocardiographyruledout
anyassociatedcongenitalcardiacanomalies.
ThebabywaslabeledasASAIIIE.Thebabywaswrappedinwarmcottonwoolgamgeesandplaced
ontheheatingmattress.Cardioscopeandpulseoximeterwasattachedtothebaby.Ryle'stubewas
aspiratedwithasyringe.Beforestartinganaesthesia,asurgeonwasscrubbedtoperformemergency
thoracostomyifrequired.
Antisialogogueatropine0.0lmg.kg1andfentanyl3mcgwasgivenintravenouslyandrectal
paracetamolsuppository80mgwasplaced.Thebabywaspreoxygenatedfor5minutesandthen
graduallysevofluranewasstarted.Gentlemanualventilationwasperformedviathefacemask.
Afterintroducinglaryngoscope,a3.5sizeendotrachealtubewasinserted.Thebabywasconnectedto
anaesthesiamachinethroughJacksonReesmodificationofAyre'sTpiece.
Spontaneousventilationwasmaintainedusing100%oxygen,12%sevofluranewithgentlemanual
ventilation.Saturationonpulseoximeterwas98%followingintubation
Theneonatewasplacedintruerightlateralposition
Monitoringincludedelectrocardiogram,invasivebloodpressure,SpO2,ETCO2andrectaltemperature.
IVfluidsweretitratedaccordingtoHolidaySegarformulatoreplacefastingandmaintenance
requirements.Bloodlosswasreplaced.Vitalsignsweremaintainedinnormalrangethroughout
surgery.

Onceresectionoftheaffectedlobewascompleted,controlledlungventilationwithatracuriumasthe
neuromuscularblockingagentwasstarted.Nitrousoxidewasaddedthence.
Bloodgasesintraoperativelyandpostoperativelywerewithinnormallimits.Attheendofoperation,
intercostalblockwasgivenwith3mlof0.125%bupivacaine,andresidualneuromuscularblockwas
reversedwithneostigmine0.15mgalongwithatropine0.03mgIV.Theinfantwasextubatedwhen
spontaneousrespirationwassufficienttomaintainSpO2>90%inair.Later,thechildwaskeptinan
oxygenenrichedenvironmentinthepediatricintensivecareunitundercontinuousSpO2andEKG
monitoring.At72hoursthechestdrainwasremovedafterfullexpansionoftheresiduallung.Restof
thepostoperativeperiodwasuneventful,andthechildwasdischargedafter7days.
Lungbiopsyoftheresectedsegmentshowedlungparenchymawithatelectaticchangesand
emphysematousdilatationofalveolarspacesinthesurroundingzone.(Fig3,,4
4).

Fig 3
Gross specimen of the resected emphysematous left upper lobe.

Fig 4
Microscopy showing distended alveolar spaces.
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Discussion
Controversystillexistsconcerningthediagnosisandtreatmentofcongenitallobaremphysema(CLE).
Althoughsurgicalremovaloftheaffectedlobeisthemostcommonlyacceptedformoftreatment,there
isasmallplaceforconservativetherapyinpatientswhoarenotclinicallyinrespiratorydistressand
abletofeedandgrow.Maintainingventilatorypressuresandvolumeaslowaspossibleavoids
producingventilatorrelatedhyperexpansionoftheaffectedlobe.Managementbymoreconservative,
gentleventilationtechniqueifsuccessfulwillresultinfeweremergencysurgerieswithCLE.Operative
mortalityrateis3to7%whereaswithconservativetherapiesitis50to75%.Henceconservative
managementshouldbereservedonlyforpatientswithmildersymptomsornodistressatall. 6
Monitoringofthevitalparameters,duringneonatalsurgeryisamust.Duringthoracotomy,thebabyis
atgreatrisk.Oninductionofanaesthesiaifpositivepressureventilationisappliedbeforeopeningof
thechest,itmaycauserapidinflationofemphysematouslobeorcystwithsuddenmediastinalshiftand
cardiacarrest7.Therefore,inductionofanaesthesiashouldprovideadequatespontaneousventilation
withminimalairwaypressure.Occasionalgentleassistanceisnecessary.Oncethechestisopenedand
theaffectedlobeisdelivered,thepatientcanbeparalyzedandthelungsventilatedbycontrolled
ventilation8.Hyperinflationoftheemphysematouslobeorcystcanbepreventedbyavoidingtheuse
ofnitrousoxidebeforethedeliveryoftheaffectedlobe,asitdiffusesfasterinaclosedcavityand
expandsthecavity,leadingtofurthercompressionofnormallungandmoremediastinalshift 9.Inour
patientnitrousoxidewasstartedonlyaftertheaffectedlobewasresected.10,11
Isolatedcasereportsofendobronchialintubationusingsinglelumenendotrachealtubewithgentle
ventilationasanalternativetospontaneousbilaterallungventilationarealsodescribed. 12However
endobronchialintubationofthenormalsideleadstotemporarycollapseoftheaffectedlobewith

eliminationofventilationtothenonperfusedlungsegmentonthediseasedsideisaneverpresentrisk.
Lackofdoublelumentubesinthisagegroupmakesthingsdifficult.Pediatricfibreopticbronchoscope
toconfirmproperplacementofthebronchialblockerwasnotavailableinourinstitute.
Thetechniqueofcaudalthoracicepiduralcatheterizationprovidesastablecardiovascularprofileand
excellentanalgesiawithoutdepressingrespiration.Howeverkinikinganddoublingbackofcatheter
maypreventthecatheterfromreachingthemidthoracicsegments.13

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References
1. Irving IM. Malformations and acquired lesions of lungs, pleura and
mediastinum. In: Lister J, Irving IM, editors. Neonatal Surgery.
London: Butterworth & Co Ltd; 1990. pp. 265271.
2. Al-Salem AH, Gyamfi YA, Grant CS. Congenital lobar emphysema.
Can J Anaesth. 1990;37:3779. [PubMed]
3. Monin I', Didier F, Vert I', et al. Giant lobar emphysema: neonatal
diagnosis. Pediatr Radiol. 1979;8:25960. [PubMed]
4. Berlinger NT, Porto DP, Thompson TR. Infantile lobar emphysema.
Ann Otol Rhinol Laryngol. 1987;96:10611. [PubMed]
5. Raynor AC, Cap MI', Scaly WC. Lobar emphysema of infancy:
diagnosis, treatment and etiological aspects. Ann Thorac Surg.
1967;4:37485. [PubMed]
6. Holli MA, Segar WE. The maintenance need for water inparenteral
fluidtherapy. Pediatrics. 1957;19:823832. [PubMed]
7. Tander B, Yalin Y, Yilmaz B, Ali Karadao C, Bulu M. Congenital
lobar emphysema: a clinicopathologic evaluation of 14 cases. Eur J
Peciatr Surg. 2003;13:10811. [PubMed]
8. Hatch DJ, Summer E. Anesthesia - Specific conditions. In: Feldman
SA, Scurr CF, editors. Current Topics in Anesthesia No 5, Neonatal
Anesthesia. 1st ed. London: Edward Arnold Publishers Pvt Ltd; 1981.
p. 112.
9. Raynor AC, Cap MP, Scaly WC. Lobar emphysema of infancy:
diagnosis, treatment and etiology aspects. Ann Thorac Surg.
1967;4:374375. [PubMed]
10. Brown TCK, Fisk GC. Anesthesia for children. 2nd ed. Oxford:
Blackwell Scientific Publications; 1992. Anesthesia for thoracic
surgery; p. 177.
11. Morray J. Anesthesia for thoracic surgery. In: Gregory G, editor.
Pediatric Anesthesia. vol 2. New York: Churchill Livingstone; 1983. p.
662.
12. Gupta R, Singhal SK, Rattan KN, Chhabra B. Management of
congenital lobar emphysema with endobronchial intubation and
controlled ventilation. Anesth Analg. 1998;86:713. [PubMed]
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three cases. Anesth Analg. 2001;93:34850. [PubMed]

Congenital Lobar Emphysema

Definition
Congenital lobar emphysema is a chronic disease that causes
respiratory distress in infants.
Description
Congenital lobar emphysema, also called infantile lobar
emphysema, is a respiratory disease that occurs in infants when air
enters the lungs but cannot leave easily. The lungs become overinflated, causing respiratory function to decrease and air to leak out
into the space around the lungs.
Half of the cases of congenital lobar emphysema occur in the first
four weeks of life, and three-quarters occur in infants less than six
months old. Congenital lobar emphysema is more common in boys
than in girls.
Each person has two lungs, right and left. The right lung is divided
into three sections, called lobes, and the left lung into two lobes.
Congenital lobar emphysema usually affects only one lobe, and this
is usually an upper lobe. It occurs most frequently in the left upper
lobe, followed by the right middle lobe.
Causes and symptoms
The cause of congenital lobar emphysema often cannot be
identified. The airway may be obstructed or the infant's lungs may
not have developed properly. Congenital lobar emphysema is almost
never of genetic origin.
Symptoms of congenital lobar emphysema include:

shortness of breath

wheezing

lips and fingernail beds that have a bluish tinge

Diagnosis
Congenital lobar emphysema is usually identified within the first two
weeks of the infant's life. It is diagnosed by respiratory symptoms
and a chest x ray, which shows the over-inflation of the affected
lobe and may show a blocked air passage.
Treatment
For infants with no, mild, or intermittent symptoms, no treatment is
necessary. For more serious cases of congenital lobar emphysema,

surgery is necessary, usually a lobectomy to remove the affected

lung lobe.
Alternative treatment
Alternative treatments that may be helpful for congenital lobar
emphysema are aimed at supporting and strengthening the
patient's respiratory function. Vitamin and mineral supplementation
may be recommended as may herbal remedies such as lobelia
(Lobelia inflata) that strengthen the lungs and enhance their
elasticity. Homeopathic constitutional care may also be beneficial for
this condition.
Key terms
Congenital A disease or condition that is present at birth.
Emphysema A condition in which the air sacs in the lungs
become overinflated, causing a decrease in respiratory function.
Lobar Relating to a lobe, a rounded projecting part of the lungs.
Prognosis
Surgery for congenital lobar emphysema has excellent results.
Prevention
Congenital lobar emphysema cannot be prevented.
Resources
Organizations
American Lung Association. 1740 Broadway, New York, NY 10019.
(800) 586-4872. http://www.lungusa.org.
National Heart, Lung and Blood Institute. P.O. Box 30105, Bethesda,
MD 20824-0105. (301) 251-1222. http://www.nhlbi.nih.gov.
National Jewish Center for Immunology and Respiratory Medicine.
1400 Jackson St., Denver, CO 80206. (800) 222-5864.
http://www.nationaljewish.org/main.html.
Gale Encyclopedia of Medicine. Copyright 2008 The Gale Group, Inc.
All rights reserved.
congenital lobar emphysema
Etymology: L, congenitus, born with; Gk, lobos, lobe; Gk, en, in +
physema, a blowing
a condition characterized by overinflation, commonly affecting one
of the upper lobes and causing respiratory distress in early life. Also
called congenital lobar overinflation.
Mosby's Medical Dictionary, 8th edition. 2009, Elsevier.
lobar
pertaining to a lobe.
congenital lobar emphysema
emphysema of one or more lung lobes usually the result of bronchial
dysplasia or agenesis in the neonate.
lobar pneumonia

pneumonia affecting one or more lobes of the lungs. See also lobar
pneumonia.
Saunders Comprehensive Veterinary Dictionary, 3 ed. 2007
Elsevier, Inc. All rights reserved
congenital lobar emphysema
[MIM*130710]
common cause of neonatal respiratory distress which usually
involves the left upper lobe.
Farlex Partner Medical Dictionary Farlex 2012