11SURGERY FORSURGERY FOR CONGENITALCONGENITALLUNGLUNG MALFORMATIONS:MALFORMATIONS:ROLE OF THE AROLE OF THE ANTENATALNTENATALDIAGNOSISDIAGNOSISOgnyan BrankovOgnyan BrankovUniversity Department of Pediatric SurgeryUniversity Department of Pediatric SurgeryHospital “Pirogov” – Sofia, BulgariaHospital “Pirogov” – Sofia, Bulgaria
22BackgroundBackground DisturbancesDisturbances inin fetal lung growthfetal lung growth may give rise to amay give rise to agroup of structural lesionsgroup of structural lesions whichwhich include congenitalinclude congenitalcystic 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 usuallydiagnosed antenatallydiagnosed antenatally oror in the newborn period,in the newborn period, butbutsome of them are presented clinicallysome of them are presented clinically in infancy or inin infancy or in latelatechildhood.childhood.
33Our research is based on the widely usedOur research is based on the widely usedclassification of CLM:classification of CLM: BBronchopulmonary (lung bud) anomaliesronchopulmonary (lung bud) anomalies (hypoplasia,(hypoplasia,bronchial atresiabronchial atresia,, CCAM,CCAM, pulmonary bronchogenicpulmonary bronchogeniccystscysts,, LE)LE) Vascular anomalies (A – V shunts)Vascular anomalies (A – V shunts) Combined anomalies (sequestration)Combined anomalies (sequestration)
44Clinical dataClinical dataWeWe reviewreview retrospectivelyretrospectively 27 children27 children with CLMwith CLM treatedtreatedin 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
55CCAM 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 includedin the researchin the research
66CCAMCCAM isis usuallyusually classifiedclassified into types I to III, depending on cystinto types I to III, depending on cystdiameter 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 singleor multiple largeor multiple large cystscysts lined by ciliated, pseudostratifiedlined by ciliated, pseudostratifiedcolumnar epitheliumcolumnar epithelium with elastic tissue and smoothwith elastic tissue and smoothmuscle 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 cuboidalcuboidalto 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 producingproducingmediastinal shift and containingmediastinal shift and containing bronchiolelikebronchiolelike structuresstructureslined by cuboidal epitheliumlined by cuboidal epithelium
778 prenatal ultrasonographic examination8 prenatal ultrasonographic examination RRegressionegression or evenor even resolvresolvinging of the lesion antenatallyof the lesion antenatallywas not observed owas not observed on prenatal ultrasoundn prenatal ultrasound TwoTwo babies werebabies were symptomatic in the early neonatalsymptomatic in the early neonatalperiod, and three developed symptoms shortlyperiod, and three developed symptoms shortlyafterwardsafterwards ThreeThree babies were asymptomatic,babies were asymptomatic, butbut had abnormalitieshad abnormalitieson chest radiograph oron chest radiograph or CTCT scan and had elective surgeryscan and had elective surgery
88Inacurrate prenatal USGInacurrate prenatal USGOf the seven initiallyOf the seven initially asymptomatic infants,asymptomatic infants, 33 werewereoperated onoperated on between the 1,7 and 2,9 year of agebetween the 1,7 and 2,9 year of agebecause ofbecause of CCAM infectionsCCAM infections; t; the remaininghe remaining 44 childrenchildrenwith 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 ororpneumothorax) andpneumothorax) and consequentlyconsequently underwent resection.underwent resection.
99Nina, 3,4 years oldNina, 3,4 years oldReferred to us with destructive pneumonia. Antenatal workup not available. CTReferred to us with destructive pneumonia. Antenatal workup not available. CTrevealed cystic lesions of the left upper lobe, encapsulated empyema at therevealed cystic lesions of the left upper lobe, encapsulated empyema at thelower lobe. Surgery – decortication, lobectomy.lower lobe. Surgery – decortication, lobectomy.
1010Mateja, born 06 Jan 2010Mateja, born 06 Jan 201007 Oct 200912 Nov 2009
1111Mateja, 7 days oldMateja, 7 days old12.01.1027.01.10CT 28.01.10Bilobectomy 29.01.2010
1212Isusska, 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 infectionSURGERY:Right inferior lobectomy
1313Veselin, born 25 Jan 2002Veselin, born 25 Jan 20023 day old neonate is referred to us for progressively increasing respiratory3 day old neonate is referred to us for progressively increasing respiratorydistress. Antenatal workup was not available. The radiological picture mimickeddistress. Antenatal workup was not available. The radiological picture mimickedcongenital diaphragmatic hernia. Normal abdominal anatomy and intactcongenital diaphragmatic hernia. Normal abdominal anatomy and intactdiaphragm by laparotomy. At the next day left thoracotomy was performeddiaphragm by laparotomy. At the next day left thoracotomy was performedthat 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
1515Results 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 pulmonaryinfectioninfection Thoracic deformity - 1Thoracic deformity - 1
1616Congenital bCongenital bronchogenicronchogenic ccystsysts Bronchogenic cysts of the lung n = 5Bronchogenic cysts of the lung n = 5Right lung (n = 3):Right lung (n = 3):upper lobe - 1upper lobe - 1lower lobe - 1lower lobe - 1Left lung (n = 2):Left lung (n = 2):upper lobe - 1upper lobe - 1lower lobe - 1lower lobe - 1 Mediastinal bronchogenic cyst n = 1Mediastinal bronchogenic cyst n = 1
1717BronchogenicBronchogenic ccysts of theysts of the llungung Bronchogenic cysts are lesions of congenital originBronchogenic cysts are lesions of congenital originderived 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 fromcompression of adjacent structurescompression of adjacent structures or added ior added infection,nfection,especially in cysts with bronchial communicationsespecially in cysts with bronchial communications
1818Teodor, 3 years oldTeodor, 3 years old03.11.200718.12.2007Incidentally 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 monthadmitted with purulent complication. Surgery – lobectomy.admitted with purulent complication. Surgery – lobectomy.
1919Muzgen, 5 months oldMuzgen, 5 months oldAntenatal 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.
2020Results BCResults BC Mortality 0 %Mortality 0 % Prolonged recovery – 1Prolonged recovery – 1Delayed surgery after recognition of the cystDelayed surgery after recognition of the cyst
2222Stefka, 6 months of ageStefka, 6 months of ageRecurrent pulmonary infections, antibiotic treatment, no X-ray exam
2323DiscussionDiscussionNomenclature and classificationNomenclature and classificationAntenatal diagnosisAntenatal diagnosisMalign degenerationMalign degenerationSurgery or observationSurgery or observation
2424So 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
2525PulmonaryPulmonary sequestration, congenital cystic adenomatoidsequestration, congenital cystic adenomatoidmmalformationalformation (CCAM), congenital lobar emphysema, and(CCAM), congenital lobar emphysema, andbronchogenic pulmonary cystsbronchogenic pulmonary cysts are major four congenitalare major four congenitalcystic lesions in the lung, but share similar embryologiccystic lesions in the lung, but share similar embryologicand 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 childrenEuEurr J of Cardio-thoracic Surgery 15 (1999) 11–17J of Cardio-thoracic Surgery 15 (1999) 11–17Yukihiro Imai, Eugene J. MarkYukihiro Imai, Eugene J. Mark Cystic Adenomatoid Change Is Common to Various Forms ofCystic Adenomatoid Change Is Common to Various Forms ofCystic 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.
2626Previously applied classification systemsPreviously applied classification systems mix a number ofmix a number ofvariable 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 offetal lung developmentfetal lung development..WW.. Pumberger, MPumberger, M.. Hörmann, JHörmann, J.. Deutinger, GDeutinger, G.. Bernaschek,Bernaschek,EE..BistrickyBistricky,, EE.. HorcherHorcher Longitudinal observation ofLongitudinal observation ofantenatally detected congenital lung malformationsantenatally detected congenital lung malformations(CLM)(CLM) Eur J Cardiothorac SurgEur J Cardiothorac Surg 2003;24:703-7112003;24:703-711
2727The nomenclature of congenital lung disease was never veryThe nomenclature of congenital lung disease was never veryclear, with termsclear, with terms ….…. being used to describe often overlappingbeing used to describe often overlappingabnormalities.abnormalities. Thus, a complete reappraisal of theThus, a complete reappraisal of thenomenclature 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 ofRespiratory 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 clearnomenclature.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 howopinion on current proposed classifications remains divided.opinion on current proposed classifications remains divided.Furthermore, different specialties frequently use differentFurthermore, different specialties frequently use differentsystems for the same diseases.systems for the same diseases.
2828Stocker JT.Stocker JT. Congenital Pulmonary Airway Malformation : a new name for andCongenital Pulmonary Airway Malformation : a new name for andan expanded classification of congenital cystic adenomatoid malformation ofan expanded classification of congenital cystic adenomatoid malformation ofthe 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 congenitalpulmonary airway malformations (CPAM) of thelung identifies lesions on the basis of the likelysite of origin and clinical and pathologicalfeatures.
2929Antenatal diagnosisAntenatal diagnosis AnAn in-utero regression noted onin-utero regression noted on antenatal USG may notantenatal USG may notrepresentrepresent ““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 CLMdoes notdoes not automatically mean that an infant should be subjectedautomatically mean that an infant should be subjectedtoto pre- and/or postnatal intervention which might bepre- and/or postnatal intervention which might beinappropriateinappropriate or unnecessary.or unnecessary. ((WW.. PumbergerPumberger a.al, 2003a.al, 2003)) Congenital lung malformations are increasingly diagnosedCongenital lung malformations are increasingly diagnosedantenatallyantenatally.. All infants with a prenatal diagnosis requireAll infants with a prenatal diagnosis requirepostnatal evaluationpostnatal evaluation andand sometimes necessitating emergentsometimes necessitating emergentsurgical resection.surgical resection. ((J.C.Pollock, 2005J.C.Pollock, 2005))
3030Surgery 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, latesurgery, 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, werecommend elective resection at 1 month of age or older. This agerecommend elective resection at 1 month of age or older. This agehas been chosen because anesthetic risk in babies decreases afterhas been chosen because anesthetic risk in babies decreases after4 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.CrombleholmeCongenital cystic lung disease: contemporary antenatalCongenital cystic lung disease: contemporary antenataland postnatal managementand postnatal management Pediatr Surg Int (2008) 24:643–657Pediatr Surg Int (2008) 24:643–657
3131Malign degeneration?Malign degeneration? Some authors find outSome authors find out associationassociation ofof pleuro-pulmonarypleuro-pulmonaryblastoma 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-yearperiodperiod 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 pulmonarycystscystsPed 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.
3232As summary:As summary: PPatients who presentatients who present wwithith asymptomatic CCAMasymptomatic CCAMwill subsequently become symptomatic.will subsequently become symptomatic. In the case of postpartumIn the case of postpartum confirmed diagnosis earlyconfirmed diagnosis earlytreatmenttreatment is intended to preventis intended to prevent unexpected deterioration orunexpected deterioration orrecurrent infections whichrecurrent infections which could make surgical procedurescould make surgical proceduresdifficult.difficult. Early surgical referral and intervention mayEarly surgical referral and intervention may bbee beneficialbeneficialto 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-termoutcome of asymptomatic patients with congenitaloutcome of asymptomatic patients with congenital cystic adenomatoidcystic adenomatoidmalformationmalformation Ped Surg Int (2009) 25:479–48Ped Surg Int (2009) 25:479–4855WW.. 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 congenitallunglungmalformationsmalformations (CLM)(CLM) Eur J Cardiothorac SurgEur J Cardiothorac Surg 2003;24:703-7112003;24:703-711
3333CONCLUSIONSCONCLUSIONS1.1. AllAll congenital lung lesionscongenital lung lesions with large cystswith large cysts confirmed byconfirmed bypostnatal CXR or CT scan should be resected even inpostnatal CXR or CT scan should be resected even inasymptomatic children.asymptomatic children.2.2. Since most patients will become symptomatic sooner orSince most patients will become symptomatic sooner orlater and because of the small risk of malignancy, resectionlater and because of the small risk of malignancy, resectionshould be done in infancy.should be done in infancy. Waiting and observe carry the risk ofWaiting and observe carry the risk ofinfection which will complicate the surgery and prolong the timeinfection which will complicate the surgery and prolong the timespent in hospitalspent in hospital3.3. For patients diagnosed prenatally, we recommend surgery at 3 to 6For patients diagnosed prenatally, we recommend surgery at 3 to 6months of life at the latest, so that compensatory lung growth canmonths of life at the latest, so that compensatory lung growth canoccur.occur.J-MJ-M.. Laberge, PLaberge, P.. Puligandla, HPuligandla, H.. FlageoleFlageole AsymptomaticAsymptomaticcongenital lung malformationscongenital lung malformations Seminars in PediatricSeminars in PediatricSurgery (2005) 14, 16-33Surgery (2005) 14, 16-33
3434And now, for a final relax, lets see what for jokes make toAnd now, for a final relax, lets see what for jokes make tous Mother Natureus Mother NatureUPUPDOWNDOWN
3535Daniela, 7 years oldDaniela, 7 years oldSmall CDH with supradiaphragmatic liver sequester
3636Victoria, 3 day oldVictoria, 3 day oldLarge CDH with infradiaphragmatic extralobar sequestration