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SURGERY FOR SURGERY FOR CONGENITAL CONGENITAL LUNGLUNG MALFORMATIONS: MALFORMATIONS: ROLE OF THE AROLE OF THE ANTENATAL NTENATAL

DIAGNOSISDIAGNOSIS

Ognyan Brankov Ognyan Brankov

University Department of Pediatric Surgery University Department of Pediatric Surgery

Hospital “Pirogov” – Sofia, BulgariaHospital “Pirogov” – Sofia, Bulgaria

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Background Background

Disturbances Disturbances in in fetal lung growth fetal lung growth may give rise to a may give rise to a group of structural lesionsgroup of structural lesions which which include congenital include congenital cystic adenomatoid malformation (CCAM), cystic adenomatoid malformation (CCAM), sequestrationsequestration, bronchogenic cyst , bronchogenic cyst and lobar emphysema.and lobar emphysema.

TheseThese congenital congenital lung lung malformations malformations (CLM) (CLM) are usually are usually diagnosed antenatallydiagnosed antenatally or or in the newborn period, in the newborn period, but but some of them are presented clinically some of them are presented clinically in infancy or in in infancy or in late late childhood. childhood.

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Our research is based on the widely used Our 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 bronchogenic pulmonary 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

We We review review retrospectively retrospectively 27 children27 children with CLM with CLM treated treated in our Departmentin our Department between between 20032003 and 200 and 20099. .

Congenital cystic adenomatoid malformation (CCAM) Congenital cystic adenomatoid malformation (CCAM) n = 15n = 15

Bronchogenic cystBronchogenic cyst n = 6 n = 6

Congenital lobar emphysema (CLE) n = 4Congenital lobar emphysema (CLE) n = 4

Lung sequestrationLung sequestration n = 2 n = 2

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CCAM n = 15CCAM n = 15

EightEight cases with cases 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 terminated pregnancies were terminated and were not included and were not included

in the researchin the research

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CCAMCCAM is is usually usually classifiedclassified into types I to III, depending on cyst into types I to III, depending on cyst

diameter anddiameter and the dominant cellsthe dominant cells histologicallyhistologically..

Type IType I is the most is the most common variety and common variety and consists of single consists of single or multiple largeor multiple large cystscysts lined by ciliated, pseudostratified lined by ciliated, pseudostratified columnar epitheliumcolumnar epithelium with elastic tissue and smooth with elastic tissue and smooth muscle in the walls ofmuscle in the walls of the cysts. the cysts.

Type IIType II consists of consists of multiple small cystsmultiple small cysts lined by lined by cuboidal cuboidal to columnar epithelium and no cartilage orto columnar epithelium and no cartilage or mucous cells. mucous cells.

Type IIIType III is a is a large, bulky, noncystic lesionlarge, bulky, noncystic lesion producing producing mediastinal shift and containingmediastinal shift and containing bronchiolelikebronchiolelike structures structures lined by cuboidal epitheliumlined by cuboidal epithelium

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8 prenatal ultrasonographic examination8 prenatal ultrasonographic examination

RRegression egression or even or even resolvresolvinging of the lesion antenatally of the lesion antenatally was not observed owas not observed on prenatal ultrasoundn prenatal ultrasound

Two Two babies werebabies were symptomatic in the early neonatal symptomatic in the early neonatal period, and three developed symptoms shortly period, and three developed symptoms shortly afterwardsafterwards

ThreeThree babies were asymptomatic, babies were asymptomatic, butbut had abnormalities had abnormalities on chest radiograph or on chest radiograph or CTCT scan and had elective surgery scan and had elective surgery

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Inacurrate prenatal USGInacurrate prenatal USG

Of the seven initially Of the seven initially asymptomatic infants, asymptomatic infants, 33 were were operated on operated on between the 1,7 and 2,9 year of age between the 1,7 and 2,9 year of age because of because of CCAM infectionsCCAM infections; t; the remaining he remaining 44 children children 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, pyothorax empyema, pyothorax or or pneumothorax) and pneumothorax) and consequently consequently underwent resection. underwent resection.

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Nina, 3,4 years oldNina, 3,4 years oldReferred to us with destructive pneumonia. Antenatal workup not available. CT Referred to us with destructive pneumonia. Antenatal workup not available. CT revealed cystic lesions of the left upper lobe, encapsulated empyema at the revealed 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

1111

Mateja, 7 days oldMateja, 7 days old12.01.10

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 veins Anomaly 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 20023 day old neonate is referred to us for progressively increasing respiratory 3 day old neonate is referred to us for progressively increasing respiratory distress. Antenatal workup was not available. The radiological picture mimicked distress. Antenatal workup was not available. The radiological picture mimicked congenital diaphragmatic hernia. Normal abdominal anatomy and intact congenital 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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CCAM CCAM Type IType I 14 cases 14 cases Type IIType II 1 case 1 case

LocalisationLocalisation

Right lung (n = 11):Right lung (n = 11):upper lobe - 6upper lobe - 6lower lobe - 5 lower lobe - 5

Left lung (n = 4): Left lung (n = 4): upper lobe - 2upper lobe - 2lower lobe - 1 lower 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 pulmonary Surgery 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 bronchogenic ronchogenic 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 - 1 lower lobe - 1

Left lung (n = 2): Left lung (n = 2): upper lobe - 1upper lobe - 1lower lobe - 1 lower lobe - 1

Mediastinal bronchogenic cyst n = 1Mediastinal bronchogenic cyst n = 1

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Bronchogenic Bronchogenic ccysts of the ysts of the llungung

Bronchogenic cysts are lesions of congenital origin Bronchogenic cysts are lesions of congenital origin derived from the primitive foregut derived 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 from complications result from compression of adjacent structurescompression of adjacent structures or added i or 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 month Parents 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 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.

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Results BCResults BC

Mortality 0 %Mortality 0 %

Prolonged recovery – 1 Prolonged 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 ageRecurrent pulmonary infections, antibiotic treatment, no X-ray exam

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DiscussionDiscussion

Nomenclature and classificationNomenclature and classification

Antenatal diagnosisAntenatal diagnosis

Malign degeneration Malign 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

Congenital Congenital bbronchopulmonary ronchopulmonary vvascular ascular 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 cysts bronchogenic 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) 11–17 J 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 of Cystic 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 systems Previously 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, M Pumberger, M.. Hörmann, J Hörmann, J.. Deutinger, G Deutinger, G.. Bernaschek, Bernaschek, EE..BistrickyBistricky, , EE.. Horcher Horcher Longitudinal observation of Longitudinal observation of antenatally detected congenital lung malformationsantenatally detected congenital lung malformations (CLM)(CLM) Eur J Cardiothorac Surg Eur J Cardiothorac Surg 2003;24:703-7112003;24:703-711

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The nomenclature of congenital lung disease was never very The nomenclature of congenital lung disease was never very

clear, with terms clear, with terms ….…. being used to describe often overlapping being used to describe often overlapping abnormalities. abnormalities. Thus, a complete reappraisal of the Thus, 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 of Am 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 clear Congenital 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 how Congenital 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 different Furthermore, 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 and Congenital Pulmonary Airway Malformation : a new name for and an expanded classification of congenital cystic adenomatoid malformation of an 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

An An in-utero regression noted onin-utero regression noted on antenatal USG may not antenatal USG may not represent represent ““genuinegenuine”” regression regression ( (PC ChowPC Chow, , SL LeeSL Lee, 2007, 2007))

IIt should be givent should be given attention that prenatal diagnosis of CLM attention that prenatal diagnosis of CLM does notdoes not automatically mean that an infant should be subjected automatically mean that an infant should be subjected toto pre- and/or postnatal intervention which might be pre- and/or postnatal intervention which might be inappropriateinappropriate or unnecessary. or unnecessary. ((WW.. Pumberger Pumberger a.al, 2003 a.al, 2003) )

Congenital lung malformations are increasingly diagnosed Congenital lung malformations are increasingly diagnosed antenatallyantenatally. . All infants with a prenatal diagnosis require All infants with a prenatal diagnosis require postnatal evaluation postnatal evaluation and and sometimes necessitating emergent sometimes 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, late early 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, we After confirmation of CCAM location by postnatal chest CT scan, we recommend elective resection at 1 month of age or older. This age recommend elective resection at 1 month of age or older. This age has been chosen because anesthetic risk in babies decreases after has 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.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

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Malign degeneration?Malign degeneration?

Some authors find out Some authors find out associationassociation of of pleuro-pulmonary pleuro-pulmonary blastoma or blastoma 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 I III PPBII PPB.. Louis P. DehnerLouis P. Dehner Beware of ‘‘degenerating’’ congenital pulmonary Beware 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 the surgical 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 present atients who present wwithith asymptomatic CCAM asymptomatic CCAM

will subsequently become symptomatic.will subsequently become symptomatic. In the case of postpartumIn the case of postpartum confirmed diagnosis early confirmed diagnosis early

treatment treatment is intended to preventis intended to prevent unexpected deterioration or unexpected deterioration or recurrent infections whichrecurrent infections which could make surgical procedures could make surgical procedures difficult.difficult.

Early surgical referral and intervention may Early surgical referral and intervention may bbee beneficial to avoid the development of complications.beneficial 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-term Long-term

outcome of asymptomatic patients with congenitaloutcome of asymptomatic patients with congenital cystic adenomatoid cystic adenomatoid

malformationmalformation Ped Surg Int (2009) 25:479–48Ped Surg Int (2009) 25:479–485 5

WW.. Pumberger, M Pumberger, M.. Hörmann, J Hörmann, J.. Deutinger, G Deutinger, G.. Bernaschek, E Bernaschek, E..BistrickyBistricky, , EE..

HorcherHorcher Longitudinal observation of antenatally detected congenital Longitudinal observation of antenatally detected congenital lung lung

malformationsmalformations (CLM)(CLM) Eur J Cardiothorac Surg Eur J Cardiothorac Surg 2003;24:703-7112003;24:703-711

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CONCLUSIONSCONCLUSIONS

1. 1. All All congenital lung lesions congenital 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 of Waiting and observe carry the risk of infection which will complicate the surgery and prolong the time infection 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 6 For patients diagnosed prenatally, we recommend surgery at 3 to 6 months of life at the latest, so that compensatory lung growth can months of life at the latest, so that compensatory lung growth can occur. occur.

J-MJ-M.. Laberge, P Laberge, P.. Puligandla, H Puligandla, H.. Flageole Flageole Asymptomatic Asymptomatic congenital lung malformationscongenital lung malformations Seminars in Pediatric Seminars 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 to And 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 !