Antenatal diagnosis, classification and surgical tacktick

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SURGERY FORSURGERY FOR CONGENITALCONGENITAL
LUNGLUNG MALFORMATIONS:MALFORMATIONS:
ROLE OF THE AROLE OF THE ANTENATALNTENATAL
DIAGNOSISDIAGNOSIS
Ognyan BrankovOgnyan Brankov
University Department of Pediatric SurgeryUniversity Department of Pediatric Surgery
Hospital “Pirogov” – Sofia, BulgariaHospital “Pirogov” – Sofia, Bulgaria

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BackgroundBackground
 DisturbancesDisturbances inin fetal lung growthfetal lung growth may give rise to amay give rise to a
group of structural lesionsgroup of structural lesions whichwhich include congenitalinclude congenital
cystic adenomatoid malformation (CCAM),cystic adenomatoid malformation (CCAM),
sequestrationsequestration, bronchogenic cyst, bronchogenic cyst and lobar emphysema.and lobar emphysema.
 TheseThese congenitalcongenital lunglung malformationsmalformations (CLM)(CLM) are usuallyare usually
diagnosed antenatallydiagnosed antenatally oror in the newborn period,in the newborn period, butbut
some of them are presented clinicallysome of them are presented clinically in infancy or inin infancy or in latelate
childhood.childhood.

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Our research is based on the widely usedOur research is based on the widely used
classification of CLM:classification of CLM:
 BBronchopulmonary (lung bud) anomaliesronchopulmonary (lung bud) anomalies (hypoplasia,(hypoplasia,
bronchial atresiabronchial atresia,, CCAM,CCAM, pulmonary bronchogenicpulmonary bronchogenic
cystscysts,, LE)LE)
 Vascular anomalies (A – V shunts)Vascular anomalies (A – V shunts)
 Combined anomalies (sequestration)Combined anomalies (sequestration)

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Clinical dataClinical data
WeWe reviewreview retrospectivelyretrospectively 27 children27 children with CLMwith CLM treatedtreated
in our Departmentin our Department betweenbetween 20032003 and 200and 20099..
 Congenital cystic adenomatoid malformation (CCAM)Congenital cystic adenomatoid malformation (CCAM)
n = 15n = 15
 Bronchogenic cystBronchogenic cyst n = 6n = 6
 Congenital lobar emphysema (CLE) n = 4Congenital lobar emphysema (CLE) n = 4
 Lung sequestrationLung sequestration n = 2n = 2

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CCAM n = 15CCAM n = 15
 EightEight cases withcases with prenatal ultrasonographic examinationprenatal ultrasonographic examination
 Seven cases with unreliable or no data for prenatal USGSeven cases with unreliable or no data for prenatal USG
 TwoTwo pregnancies were terminatedpregnancies were terminated and were not includedand were not included
in the researchin the research

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CCAMCCAM isis usuallyusually classifiedclassified into types I to III, depending on cystinto types I to III, depending on cyst
diameter anddiameter and the dominant cellsthe dominant cells histologicallyhistologically..
 Type IType I is the mostis the most common variety andcommon variety and consists of singleconsists of single
or multiple largeor multiple large cystscysts lined by ciliated, pseudostratifiedlined by ciliated, pseudostratified
columnar epitheliumcolumnar epithelium with elastic tissue and smoothwith elastic tissue and smooth
muscle in the walls ofmuscle in the walls of the cysts.the cysts.
 Type IIType II consists ofconsists of multiple small cystsmultiple small cysts lined bylined by cuboidalcuboidal
to columnar epithelium and no cartilage orto columnar epithelium and no cartilage or mucous cells.mucous cells.
 Type IIIType III is ais a large, bulky, noncystic lesionlarge, bulky, noncystic lesion producingproducing
mediastinal shift and containingmediastinal shift and containing bronchiolelikebronchiolelike structuresstructures
lined by cuboidal epitheliumlined by cuboidal epithelium

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8 prenatal ultrasonographic examination8 prenatal ultrasonographic examination
 RRegressionegression or evenor even resolvresolvinging of the lesion antenatallyof the lesion antenatally
was not observed owas not observed on prenatal ultrasoundn prenatal ultrasound
 TwoTwo babies werebabies were symptomatic in the early neonatalsymptomatic in the early neonatal
period, and three developed symptoms shortlyperiod, and three developed symptoms shortly
afterwardsafterwards
 ThreeThree babies were asymptomatic,babies were asymptomatic, butbut had abnormalitieshad abnormalities
on chest radiograph oron chest radiograph or CTCT scan and had elective surgeryscan and had elective surgery

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Inacurrate prenatal USGInacurrate prenatal USG
Of the seven initiallyOf the seven initially asymptomatic infants,asymptomatic infants, 33 werewere
operated onoperated on between the 1,7 and 2,9 year of agebetween the 1,7 and 2,9 year of age
because ofbecause of CCAM infectionsCCAM infections; t; the remaininghe remaining 44 childrenchildren
with late diagnosis of CCAMwith late diagnosis of CCAM (3,4 – 9 years of age)(3,4 – 9 years of age)
presented with complications (presented with complications (empyema, pyothoraxempyema, pyothorax oror
pneumothorax) andpneumothorax) and consequentlyconsequently underwent resection.underwent resection.

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Nina, 3,4 years oldNina, 3,4 years old
Referred to us with destructive pneumonia. Antenatal workup not available. CTReferred to us with destructive pneumonia. Antenatal workup not available. CT
revealed cystic lesions of the left upper lobe, encapsulated empyema at therevealed cystic lesions of the left upper lobe, encapsulated empyema at the
lower lobe. Surgery – decortication, lobectomy.lower lobe. Surgery – decortication, lobectomy.

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Mateja, born 06 Jan 2010Mateja, born 06 Jan 2010
07 Oct 2009
12 Nov 2009

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Mateja, 7 days oldMateja, 7 days old
12.01.10
27.01.10
CT 28.01.10
Bilobectomy 29.01.2010

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Isusska, 10 months oldIsusska, 10 months old
 Premature with cardio-pulmonary retardationPremature with cardio-pulmonary retardation
 Anomaly of pulmonary veinsAnomaly of pulmonary veins
 Neglected child; recurrent pilmonary infectionNeglected child; recurrent pilmonary infection
SURGERY:
Right inferior lobectomy

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Veselin, born 25 Jan 2002Veselin, born 25 Jan 2002
3 day old neonate is referred to us for progressively increasing respiratory3 day old neonate is referred to us for progressively increasing respiratory
distress. Antenatal workup was not available. The radiological picture mimickeddistress. Antenatal workup was not available. The radiological picture mimicked
congenital diaphragmatic hernia. Normal abdominal anatomy and intactcongenital diaphragmatic hernia. Normal abdominal anatomy and intact
diaphragm by laparotomy. At the next day left thoracotomy was performeddiaphragm by laparotomy. At the next day left thoracotomy was performed
that revealed multiple large cysts in both lobes leading to left pulmonectomy.that revealed multiple large cysts in both lobes leading to left pulmonectomy.
03.04.2002 15.09.2006

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CCAMCCAM Type IType I 14 cases14 cases Type IIType II 1 case1 case
LocalisationLocalisation
 Right lung (n = 11):Right lung (n = 11):
upper lobe - 6upper lobe - 6
lower lobe - 5lower lobe - 5
 Left lung (n = 4):Left lung (n = 4):
upper lobe - 2upper lobe - 2
lower lobe - 1lower lobe - 1
whole lung - 1whole lung - 1

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Results CCAMResults CCAM
 Mortality 0 %Mortality 0 %
 Recurrence (economy resection) – 1 (5.9 %)Recurrence (economy resection) – 1 (5.9 %)
 Prolonged recovery – 2 (11.8 %)Prolonged recovery – 2 (11.8 %)
Surgery performed at 1,1 and 3 years of age due to repeated pulmonarySurgery performed at 1,1 and 3 years of age due to repeated pulmonary
infectioninfection
 Thoracic deformity - 1Thoracic deformity - 1

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Congenital bCongenital bronchogenicronchogenic ccystsysts
 Bronchogenic cysts of the lung n = 5Bronchogenic cysts of the lung n = 5
Right lung (n = 3):Right lung (n = 3):
upper lobe - 1upper lobe - 1
lower lobe - 1lower lobe - 1
Left lung (n = 2):Left lung (n = 2):
upper lobe - 1upper lobe - 1
lower lobe - 1lower lobe - 1
 Mediastinal bronchogenic cyst n = 1Mediastinal bronchogenic cyst n = 1

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BronchogenicBronchogenic ccysts of theysts of the llungung
 Bronchogenic cysts are lesions of congenital originBronchogenic cysts are lesions of congenital origin
derived from the primitive foregutderived from the primitive foregut
 Most bronchogenic cysts originate in the mediastinum,Most bronchogenic cysts originate in the mediastinum,
while 15% to 20% occur in the lung parenchymawhile 15% to 20% occur in the lung parenchyma
 MMost cysts are symptomaticost cysts are symptomatic;; complications result fromcomplications result from
compression of adjacent structurescompression of adjacent structures or added ior added infection,nfection,
especially in cysts with bronchial communicationsespecially in cysts with bronchial communications

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Teodor, 3 years oldTeodor, 3 years old
03.11.2007
18.12.2007
Incidentally detection of round opacity by X-ray following cough and fever.Incidentally detection of round opacity by X-ray following cough and fever.
Parents refused surgery. No prenatal USG performed. After a monthParents refused surgery. No prenatal USG performed. After a month
admitted with purulent complication. Surgery – lobectomy.admitted with purulent complication. Surgery – lobectomy.

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Muzgen, 5 months oldMuzgen, 5 months old
Antenatal diagnosed thoracic cyst. Observation for 5 months.Antenatal diagnosed thoracic cyst. Observation for 5 months.
Admitted with compression syndrome and right atelectasis.Admitted with compression syndrome and right atelectasis.
Surgery – sternotomy, extirpation of the cyst.Surgery – sternotomy, extirpation of the cyst.

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Results BCResults BC
 Mortality 0 %Mortality 0 %
 Prolonged recovery – 1Prolonged recovery – 1
Delayed surgery after recognition of the cystDelayed surgery after recognition of the cyst

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CLE 4 casesCLE 4 cases
 No prenatal sonography availableNo prenatal sonography available
 Early postnatal presentation – 2 casesEarly postnatal presentation – 2 cases
 Late (4 - 6 mo postnatal) – 2 casesLate (4 - 6 mo postnatal) – 2 cases
 Surgical procedure – lobectomySurgical procedure – lobectomy
 Outcome – uneventfulOutcome – uneventful

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Stefka, 6 months of ageStefka, 6 months of age
Recurrent pulmonary infections, antibiotic treatment, no X-ray exam

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DiscussionDiscussion
Nomenclature and classificationNomenclature and classification
Antenatal diagnosisAntenatal diagnosis
Malign degenerationMalign degeneration
Surgery or observationSurgery or observation

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So far many definitions are proposedSo far many definitions are proposed
 Congenital cystic lesions of the lungCongenital cystic lesions of the lung
 Congenital pulmonary airway malformation (CPAM)Congenital pulmonary airway malformation (CPAM)
 BBronchopulmonary foregut malformationsronchopulmonary foregut malformations
 CongenitalCongenital bbronchopulmonaryronchopulmonary vvascularascular mmalformationsalformations

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PulmonaryPulmonary sequestration, congenital cystic adenomatoidsequestration, congenital cystic adenomatoid
mmalformationalformation (CCAM), congenital lobar emphysema, and(CCAM), congenital lobar emphysema, and
bronchogenic pulmonary cystsbronchogenic pulmonary cysts are major four congenitalare major four congenital
cystic lesions in the lung, but share similar embryologiccystic lesions in the lung, but share similar embryologic
and clinical characteristics.and clinical characteristics.
Shin-ichi TakedaShin-ichi Takeda a.al.a.al. Clinical spectrum of congenital cystic disease of the lung in childrenClinical spectrum of congenital cystic disease of the lung in children
EuEurr J of Cardio-thoracic Surgery 15 (1999) 11–17J of Cardio-thoracic Surgery 15 (1999) 11–17
Yukihiro Imai, Eugene J. MarkYukihiro Imai, Eugene J. Mark Cystic Adenomatoid Change Is Common to Various Forms ofCystic Adenomatoid Change Is Common to Various Forms of
Cystic Lung Diseases of Children.Cystic Lung Diseases of Children. Arch of Pathology & Labor Med, 2002, 126, 8, 934-940.Arch of Pathology & Labor Med, 2002, 126, 8, 934-940.

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Previously applied classification systemsPreviously applied classification systems mix a number ofmix a number of
variable characteristicsvariable characteristics (e.g., gross appearance,(e.g., gross appearance,
histopathology,histopathology, sonographic features, radiography, CT,sonographic features, radiography, CT,
MRI,MRI, and surgical findings).and surgical findings).
CLM are shown to represent a continuum of anomalies ofCLM are shown to represent a continuum of anomalies of
fetal lung developmentfetal lung development..
WW.. Pumberger, MPumberger, M.. Hörmann, JHörmann, J.. Deutinger, GDeutinger, G.. Bernaschek,Bernaschek,
EE..BistrickyBistricky,, EE.. HorcherHorcher Longitudinal observation ofLongitudinal observation of
antenatally detected congenital lung malformationsantenatally detected congenital lung malformations
(CLM)(CLM) Eur J Cardiothorac SurgEur J Cardiothorac Surg 2003;24:703-7112003;24:703-711

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The nomenclature of congenital lung disease was never veryThe nomenclature of congenital lung disease was never very
clear, with termsclear, with terms ….…. being used to describe often overlappingbeing used to describe often overlapping
abnormalities.abnormalities. Thus, a complete reappraisal of theThus, a complete reappraisal of the
nomenclature of congenital lung disease is timelynomenclature of congenital lung disease is timely..
AA..G.Nicholson, AG.Nicholson, A..BushBush Classification of Diffuse Lung Disease in InfantsClassification of Diffuse Lung Disease in Infants Am J ofAm J of
Respiratory and Critical Care MedicineRespiratory and Critical Care Medicine 176. pp. 1060-1061, (2007)176. pp. 1060-1061, (2007)
Bush A.Bush A. Congenital lung disease: a plea for clear thinking and clearCongenital lung disease: a plea for clear thinking and clear
nomenclature.nomenclature.
Pediatr Pulmonol. 2001 Oct;32(4):328-37.Pediatr Pulmonol. 2001 Oct;32(4):328-37.
Congenital cysts in the lung are a good example of howCongenital cysts in the lung are a good example of how
opinion on current proposed classifications remains divided.opinion on current proposed classifications remains divided.
Furthermore, different specialties frequently use differentFurthermore, different specialties frequently use different
systems for the same diseases.systems for the same diseases.

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Stocker JT.Stocker JT. Congenital Pulmonary Airway Malformation : a new name for andCongenital Pulmonary Airway Malformation : a new name for and
an expanded classification of congenital cystic adenomatoid malformation ofan expanded classification of congenital cystic adenomatoid malformation of
the lung.the lung. Histopathology 2002 41;(supplement 2) 424-430.Histopathology 2002 41;(supplement 2) 424-430.
Stocker JT, Husain AN.Stocker JT, Husain AN. Cystic Lesions of the Lung in Children -Cystic Lesions of the Lung in Children -
Classifications and Controversies. European Respiratory Monograph 2007,Classifications and Controversies. European Respiratory Monograph 2007,
Volume 12, 1Volume 12, 1--20.20.
A new and expanded classification of congenital
pulmonary airway malformations (CPAM) of the
lung identifies lesions on the basis of the likely
site of origin and clinical and pathological
features.

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Antenatal diagnosisAntenatal diagnosis
 AnAn in-utero regression noted onin-utero regression noted on antenatal USG may notantenatal USG may not
representrepresent ““genuinegenuine”” regressionregression ((PC ChowPC Chow,, SL LeeSL Lee, 2007, 2007))
 IIt should be givent should be given attention that prenatal diagnosis of CLMattention that prenatal diagnosis of CLM
does notdoes not automatically mean that an infant should be subjectedautomatically mean that an infant should be subjected
toto pre- and/or postnatal intervention which might bepre- and/or postnatal intervention which might be
inappropriateinappropriate or unnecessary.or unnecessary. ((WW.. PumbergerPumberger a.al, 2003a.al, 2003))
 Congenital lung malformations are increasingly diagnosedCongenital lung malformations are increasingly diagnosed
antenatallyantenatally.. All infants with a prenatal diagnosis requireAll infants with a prenatal diagnosis require
postnatal evaluationpostnatal evaluation andand sometimes necessitating emergentsometimes necessitating emergent
surgical resection.surgical resection. ((J.C.Pollock, 2005J.C.Pollock, 2005))

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Surgery or observation? That is the question!Surgery or observation? That is the question!
Eber E.Eber E.
Antenatal diagnosis of congenital thoracic malformations:Antenatal diagnosis of congenital thoracic malformations: early surgery, lateearly surgery, late
surgery, or no surgery?surgery, or no surgery?
Semin Respir Crit Care Med. 2007 Jun;28(3):355-66.Semin Respir Crit Care Med. 2007 Jun;28(3):355-66.
After confirmation of CCAM location by postnatal chest CT scan, weAfter confirmation of CCAM location by postnatal chest CT scan, we
recommend elective resection at 1 month of age or older. This agerecommend elective resection at 1 month of age or older. This age
has been chosen because anesthetic risk in babies decreases afterhas been chosen because anesthetic risk in babies decreases after
4 weeks of age.4 weeks of age.
Adzick NSAdzick NS.. Management of fetal lung lesions.Management of fetal lung lesions. Clin Perinatol. 2003;30(3):481-92.Clin Perinatol. 2003;30(3):481-92.
AzizkhanAzizkhan R.G,R.G,TT..M.CrombleholmeM.Crombleholme
Congenital cystic lung disease: contemporary antenatalCongenital cystic lung disease: contemporary antenatal
and postnatal managementand postnatal management Pediatr Surg Int (2008) 24:643–657Pediatr Surg Int (2008) 24:643–657

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Malign degeneration?Malign degeneration?
 Some authors find outSome authors find out associationassociation ofof pleuro-pulmonarypleuro-pulmonary
blastoma orblastoma or bronchoalveolar carcinoma with CCAM.bronchoalveolar carcinoma with CCAM.
 CongenitalCongenital lung cysts do not ‘‘degenerate’’ to become PPBs;lung cysts do not ‘‘degenerate’’ to become PPBs;
rather,rather, the cystic or type I PPB progresses over a 3–4-yearthe cystic or type I PPB progresses over a 3–4-year
periodperiod to a type II or typeto a type II or type IIII PPBII PPB..
Louis P. DehnerLouis P. Dehner Beware of ‘‘degenerating’’ congenital pulmonaryBeware of ‘‘degenerating’’ congenital pulmonary
cystscysts
Ped Surg Int (2005) 21: 123–124Ped Surg Int (2005) 21: 123–124
 Therefore many authorsTherefore many authors recommend arecommend a surgical resection as thesurgical resection as the
‘‘treatment‘‘treatment of choiceof choice”” for all pulmonary cysts.for all pulmonary cysts.

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As summary:As summary:
 PPatients who presentatients who present wwithith asymptomatic CCAMasymptomatic CCAM
will subsequently become symptomatic.will subsequently become symptomatic.
 In the case of postpartumIn the case of postpartum confirmed diagnosis earlyconfirmed diagnosis early
treatmenttreatment is intended to preventis intended to prevent unexpected deterioration orunexpected deterioration or
recurrent infections whichrecurrent infections which could make surgical procedurescould make surgical procedures
difficult.difficult.
 Early surgical referral and intervention mayEarly surgical referral and intervention may bbee beneficialbeneficial
to avoid the development of complications.to avoid the development of complications.
WongWong,, D. VietenD. Vieten,, S. SinghS. Singh,, J. G. HarveyJ. G. Harvey,, Andrew J. A. HollandAndrew J. A. Holland Long-termLong-term
outcome of asymptomatic patients with congenitaloutcome of asymptomatic patients with congenital cystic adenomatoidcystic adenomatoid
malformationmalformation Ped Surg Int (2009) 25:479–48Ped Surg Int (2009) 25:479–4855
WW.. Pumberger, MPumberger, M.. Hörmann, JHörmann, J.. Deutinger, GDeutinger, G.. Bernaschek, EBernaschek, E..BistrickyBistricky,, EE..
HorcherHorcher Longitudinal observation of antenatally detected congenitalLongitudinal observation of antenatally detected congenital
lunglung
malformationsmalformations (CLM)(CLM) Eur J Cardiothorac SurgEur J Cardiothorac Surg 2003;24:703-7112003;24:703-711

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CONCLUSIONSCONCLUSIONS
1.1. AllAll congenital lung lesionscongenital lung lesions with large cystswith large cysts confirmed byconfirmed by
postnatal CXR or CT scan should be resected even inpostnatal CXR or CT scan should be resected even in
asymptomatic children.asymptomatic children.
2.2. Since most patients will become symptomatic sooner orSince most patients will become symptomatic sooner or
later and because of the small risk of malignancy, resectionlater and because of the small risk of malignancy, resection
should be done in infancy.should be done in infancy. Waiting and observe carry the risk ofWaiting and observe carry the risk of
infection which will complicate the surgery and prolong the timeinfection which will complicate the surgery and prolong the time
spent in hospitalspent in hospital
3.3. For patients diagnosed prenatally, we recommend surgery at 3 to 6For patients diagnosed prenatally, we recommend surgery at 3 to 6
months of life at the latest, so that compensatory lung growth canmonths of life at the latest, so that compensatory lung growth can
occur.occur.
J-MJ-M.. Laberge, PLaberge, P.. Puligandla, HPuligandla, H.. FlageoleFlageole AsymptomaticAsymptomatic
congenital lung malformationscongenital lung malformations Seminars in PediatricSeminars in Pediatric
Surgery (2005) 14, 16-33Surgery (2005) 14, 16-33

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And now, for a final relax, let's see what for jokes make toAnd now, for a final relax, let's see what for jokes make to
us Mother Natureus Mother Nature
UPUP
DOWNDOWN

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Daniela, 7 years oldDaniela, 7 years old
Small CDH with supradiaphragmatic liver sequester

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Victoria, 3 day oldVictoria, 3 day old
Large CDH with infradiaphragmatic extralobar sequestration

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Thank you !Thank you !