Ultrasound Evaluation of Fetal Chest Embryology & Pathogenesis ••NormalNormal closure of pleuroperitonealpleuroperitonealfolds folds Mani Montazemi, RDMS – 4th -10th wks GA Director of Ultrasound Education & Quality Assurancee –4th -10th wks GA Baylor College of Medicine Division of Maternal-Fetal Medicine ••LungLung development Maternal Fetal Center Imaging Manager – 3rd -16th wks GA Texas Children’s Hospital, Pavilion for Women –3rd -16th wks GA Houston Texas & Clinical Instructor Thomas Jefferson University Hospital - Radiology Departmentartment Philadelphia, Pennsylvania

Fetal Chest Fetal Chest

Approach to the Fetal Chest

••ChestChest configuration ••PositionPosition of heart ••SizeSize or growth of chest ••PresencePresence of any masses ••PresencePresence of fluid

Fetal Chest Fetal Chest

Fetal Lung Masses: DDX Fetal Chest

••DevelopmentalDevelopmental Anomalies ••NomogramsforNomograms for thoracic circumference, length ––CysticCystic adenomatoidadenomatoidmalformation malformation (CCAM) ––CysticCystic pulmonary airway malformation (CPAM) ••ChestChest size can be compared to head, abd, heart ––BronchopulmonarysequestrationBronchopulmonary sequestration (BPS) ••IntralobarIntralobar •• Extralobar ––“Hybrid”lesions“Hybrid” lesions ––BronchialBronchial atresia ––BronchogeniccystBronchogenic cyst ––LobarLobar emphysema ••NeoplasmsNeoplasms ––MesenchymalneoplasmMesenchymal neoplasm ––PleuropulmonaryblastomaPleuropulmonary blastoma

TC / AC stable throughout pregnancy Fetal Chest Fetal Chest Normal > .80

1 Approach to the Fetal Chest Mass Congenital Diaphragmatic Hernia

••IsIs the chest normal in size? ••ThisThis is a developmental discontinuity of the ••IsIs the axis of the heart deviated? ••WhereWhere is the stomach? diaphragm that allows abdominal viscera to ••IsIs the mass cystic or solid? herniate into the chest, resulting in ••IfIf cystic, is it a simple cyst or a complex cystic mass? ––PulmonaryPulmonary hypoplasia ••IfIf solid, what does the Doppler show? ••WhereWhere is the mass? ––PulmonaryPulmonary hypotension ••DoesDoes the mass extend beyond the chest wall? ••AreAre there other anomalies? ••IsIs there hydrops? ••What’sWhat’s the likelihood of pulmonary hypoplasia?

Fetal Chest Fetal Chest

Diaphragm Sites of Herniation ““imperativeimperative to view entire diaphragmdiaphragm””

••ForamenForamen of Morgagni ––PartialPartial or complete absence of the central diaphragm ––anteromedialretrosternalanteromedial retrosternal portion of diaphragm ••ForamenForamen of Bochdalek ––PosterolateralPosterolateral corner of the diaphragm fails to close ––MoreMore common

Most CDHs are posterior so coronal view Fetal Chest Fetal Chest of anterior diaphragm may be normal

Diaphragm ““imperativeimperative to view entire diaphragmdiaphragm”” Congenital Diaphragmatic Hernia

••AllAll infants have postnatal respiratory distress related to pulmonary hypoplasia

••IfIf IUGR, 90% have other major anomaly

••20%20% have cardiac & 30% have CNS anomalies

••ChromosomalChromosomal abnormalities

Fetal Chest Fetal Chest

2 Congenital Diaphragmatic Hernia Congenital Diaphragmatic Hernia

••Left-sidedLeft-sided 85% ••MediastinalMediastinal shift ––Bowel,Bowel, stomach, spleen ••AbdominalAbdominal circumference small ••Right-sidedRight-sided 15% (< 5th percentile) ––Bowel,Bowel, liver, GB ––Liver,Liver, gallbladder, stomachstomach,, ••PulmonaryPulmonary hypoplasia bowel or spleen in chest ––VariableVariable ––MayMay see peristalsis in the chest ––IsIs worse for CDH than ••AFV:AFV: poly, oligo, normal other chest masses of comparable size

Fetal Chest Fetal Chest

Congenital Diaphragmatic Hernia Congenital Diaphragmatic Hernia

••HowHow does the residual lung looks like?

Clubbing of the hand and webbing at the wrist and elbow consistent with early onset of fetal akinesia

Fetal Chest Fetal Chest

Congenital Diaphragmatic Hernia CDH: Predictors of Outcome

•Lung areaareatoto head circumferencecircumferenceratioratio < 1 is ••LHR:LHR: Lung-to-head ratio associated with a high rate of neonatal death ––>1.2>1.2 = 79% survival (30/38)

due to pulmonary hypoplasia – 0.9-1.2 = 59% survival (13/24) Heart –0.9-1.2 = 59% survival (13/24) Bowell

Lung Liver ––<< 0.9 = 4% survival (1/24) ST ••MRIMRI volumetric assessment

Metkus AP, et al. Sonographic predictors of survival in fetal diaphragmatic hernia. J Pediatr Surg 31:148, 1996

Fetal Chest Fetal Chest

3 CDH: Predictors of Outcome

••LiverLiver herniationherniationas as predictor of outcome How to Detect ––No:No: 79% survival ––Yes:Yes: 41% survival a Congenital Walsh DS, et al. Am J Obstet Gynecol 18:1067, 2000 Diaphragmatic Hernia

Fetal Chest Fetal Chest

Congenital Diaphragmatic Hernia Congenital Diaphragmatic Hernia

••LeftLeft Sided Hernia ––CysticCystic or heterogenousheterogenousmass mass in left side of chest ––AbsenceAbsence of fluid-filled stomach ––DeviationDeviation of heart toward right ––PolyhydramniosPolyhydramnios ––HydropsuncommonHydrops uncommon unless associated malformation Left-sided hernias more frequent than right-sided ones Left-sided hernias more frequent than right-sided ones present

Fetal Chest Fetal Chest

Congenital Diaphragmatic Hernia Congenital Diaphragmatic Hernia

“If multiple ribs are seen, image is oblique” Fetal Chest Incorrect scan plane may result in erroneous diagnosis Fetal Chest

4 Congenital Diaphragmatic Hernia Congenital Diaphragmatic Hernia

• Right Sided Hernia •Right Sided Hernia ••CDHCDH with liver herniation ––MayMay be confused for chest mass ––ContainsContains liver and bowel ••AlwaysAlways use Doppler to evaluate for liver ––StomachStomach is below diaphragm ––GallbladderGallbladder often herniated ––DopplerDoppler will show portal & hepatic veins

Fetal Chest Fetal Chest

Congenital Diaphragmatic Hernia EExtraxtra C Corporealorporeal M Membraneembrane O Oxygenationxygenation

••FactorsFactors that has been associated with poor prognosis include: ––MarkedMarked mediastinalmediastinalshift shift associated with pulmonary hypoplasia ––IUGRIUGR ––PolyhydramniosPolyhydramnios ––DetectionDetection prior to 24 weeks ––SignificantSignificant liver involvement

Fetal Chest Fetal Chest

Congenital Diaphragmatic Hernia Congenital Diaphragmatic Hernia

••ObstructionObstruction of trachea results in ••Hysterotomy&Hysterotomy & fetal surgery ––ExpansionExpansion of the fetal lungs by retained ––HighHigh maternal morbidity & no improved fetal survivasurvivall pulmonary secretions & lung tissue stretch, is J Pediatr Surg 1997; 32: 1637-1642 associated with improved lung growth & ••Hysterotomy&Hysterotomy & endoscopically development ––NeckNeck dissection & clipping of the trachea ––HighHigh rate of preterm delivery ––IrreversabledamageIrreversable damage to the laryngeal nerve & tracheatrachea

Am J Obstet Gynecol 2000; 183: 1059-1066 J Pediatr Surg 2003; 38:1012-1020 Fetal Chest Fetal Chest

5 Tracheal Occlusion Congenital Diaphragmatic Hernia Fetoscopic endotracheal balloon

••FetoscopicFetoscopic EEndoluminalTrachealndoluminal Tracheal OOcclusioncclusion with balloon Ultrasound Obstet Gynecol 2004; 24:121-126 Deprest, Belgium; Gratacos, Spain; Nicolaides, England

Fetal Chest Fetal Chest

FFetoscopicetoscopic E Endoluminalndoluminal TTrachealracheal OOcclusioncclusion Severe CHD – 20 days post PLUG

••PerformedPerformed before 26-28 weeks gestational 20 days Post - PLUG age on fetuses with poor prognosis ––LiverLiver up and LHR < 1.0 ••ReverseReverse occlusion at 34 weeks by fetoscopy or US guided balloon puncture

••EXITEXIT ––Surgery Surgery ––ECMO ECMO (may be best strategy) Ex Utero IntrapartumTtreatment LHR 0.67 LHR 2.5

Fetal Chest Fetal Chest

Congenital Diaphragmatic Hernia Severe CHD – 20 days post PLUG ““DifferentialDifferential DiagnosisDiagnosis””

••CongenitalCongenital cystic adenomatoidadenomatoidmalformation malformation ••bronchopulmonarysequestrationbronchopulmonary sequestration ••BronchogeniccystBronchogenic cyst ••TeratomasTeratomas

Fetal Chest Fetal Chest

6 Space occupying lesion in the chest Pulmonary hypoplasia

Fetal Chest Fetal Chest

Terminology CCongenitalongenital CCysticystic AAdenomatoiddenomatoid M Malformationalformation

••CongenitalCongenital CCysticystic AAdenomatoidMalformationdenomatoid Malformation ••PulmonaryPulmonary lesion due to ––OlderOlder name that reflects cystic and adenomatous abnormal development of histologic components of theses masses lung tissue ••CongenitalCongenital PPulmonaryulmonary AAirwayirway MMalformationalformation ––CysticCystic dilatation of ––NewerNewer terminology reflects developmental disorder ooff abnormal bronchi & pulmonary airway morphogenesis maldevelopment of associated alveoli

Fetal Chest Fetal Chest

CCongenitalongenital CCysticystic AAdenomatoiddenomatoid M Malformationalformation

••ThisThis disorder is NOT GENETIC ••CanCan be accompanied by hydrops & mediastinal shift ••AlmostAlmost exclusively unilateral --left left ••SuppliedSupplied by the pulmonary artery

Complex Cystic Mass – macrocystic

Fetal Chest Fetal Chest

7 CCongenitalongenital CCysticystic AAdenomatoiddenomatoid M Malformationalformation

Macrocystic Microcystic

Solid Appearing Mass – microcystic Ostor & Fortune – Am Clin Pathol 1978; 70: 595-604 Bale – Am J Clin Pathol 1979; 71: 411-420 Fetal Chest Fetal Chest Adzick – J Pediatr Surg 1985; 20: 483-488 Bunduki – Prenat Diag 2000; 20: 459-464

Fetal Surgical Resection Fetal Surgical Resection

Fetal Chest Fetal Chest

CCongenitalongenital C Cysticystic AAdenomatoiddenomatoid M Malformationalformation CCongenitalongenital C Cysticystic AAdenomatoiddenomatoid M Malformationalformation

History: ••FirstFirst described by Chin & Tang (1949) ••ClassicClassic 3 types; ••TypeType 1: Large cysts 3-10 cm Macrocystic ••TypeType 2: Cysts 0.5-2 cm ••TypeType 3: Solid Microcystic Stocker 1977

Fetal Chest Fetal Chest

8 CCongenitalongenital C Cysticystic AAdenomatoiddenomatoid M Malformationalformation CCongenitalongenital C Cysticystic AAdenomatoiddenomatoid M Malformationalformation

Type I Type I ––Macrocystic:Macrocystic: cysts > 2cm ––Macrocystic:Macrocystic: cysts > 2cm ••GoodGood prognosis after resection ••GoodGood prognosis after resection ••TypeType II ––Medium-sizedMedium-sized cysts: cysts < .5-2cm ••HighHigh incidence of anomalies, poor prognosis

Fetal Chest Fetal Chest

CCongenitalongenital C Cysticystic AAdenomatoiddenomatoid M Malformationalformation CCAM vs. Normal Lung

Type I –Macrocystic: cysts > 2cm •Good prognosis after resection •Type II –Medium-sized cysts: cysts < .5-2 cm •High incidence of anomalies, poor prognosis ••TypeType III ––Microcystic:Microcystic: cysts < 0.5cm ••Hyperechoic,Hyperechoic, looks solid ••PoorPoor prognosis

Fetal Chest Fetal Chest

CCongenitalongenital C Cysticystic AAdenomatoiddenomatoid M Malformationalformation CCongenitalongenital CCysticystic AAdenomatoiddenomatoid M Malformationalformation

••MayMay coexist with sequestration (50% ELS) ••HeartHeart is displaced ••StomachStomach is normal location ••HydropsHydrops ––MostMost important predictor of outcome ––DismalDismal prognosis

Fetal Chest Fetal Chest

9 Lung Masses – Prognostic Indicators CCongenitalongenital CCysticystic AAdenomatoiddenomatoid M Malformationalformation

••CalculateCalculate CCCAMCAM VVolumeolume RRatioatio (CVR) ••LookLook at the size of CCAM ––CCAMCCAM volume calculated by measuring all 3 ••LookLook for evidence of heart failure dimensions x 0.52 ••LookLook for other anomalies --rare rare ––CCAMCCAM volume is then divided by HC ••CVRCVR > 1.6 indicates increased risk of developing hydrops and fetal demise

Crombleholme, et al. J Pediatr Surg 2002 37:331

Fetal Chest Fetal Chest

CCongenitalongenital CCysticystic AAdenomatoiddenomatoid M Malformationalformation ““DifferentialDifferential DiagnosisDiagnosis”” Bronchogenic Cyst

••PulmonaryPulmonary sequestration ••ResultResult from an abnormal ••BronchogeniccystBronchogenic cyst development in the budding or branching of ••DiaphragmaticDiaphragmatic hernia budding or branching of the tracheobronchial tree ••MediastinallesionsMediastinal lesions ––EnterogenouscystEnterogenous cyst ––NeurentericcystNeurenteric cyst ––CysticCystic teratoma

Fetal Chest Fetal Chest

Bronchogenic Cyst Pulmonary Sequestration

••AA singular cyst usually 1.5-2.0 cm in diameter ••NonfunctioningNonfunctioning mass of lung tissue that ••CanCan be intrapulmonary or lacks normal communication with the lie in the posterior mediastinum tracheobronchial tree ••TheThe cyst does not displace the heart ••MayMay have mass effect and Intralobar pulmonary sequestration – 75% compress esophagus ––polyhydramniospolyhydramnios Extralobar pulmonary sequestration – 25% ••RarelyRarely associated with hydrops

Fetal Chest Fetal Chest

10 Pulmonary Sequestration Pulmonary Sequestration

•Intralobar sequestration •Extralobar sequestration ––LocatedLocated within the ––HaveHave separate pleural covering substance of a lung lobe ––VenousVenous return to pulmonary veins ––ArterialArterial supply from thoracic or abdominal aorta *

Other anomalies 14% Other anomalies 60% Fetal Chest Fetal Chest

Pulmonary Sequestration Pulmonary Sequestration

•Extralobar sequestration ••AppearsAppears as a well circumscribed uniformly –90% supradiaphragmatic echogenic mass ••AlmostAlmost always Lt hemithorax ––TriangularTriangular or lobar shape –10% Subdiaphragmatic ••MediastinalshiftMediastinal shift often is noted ••LesionLesion regress during the course of gestation ••HydropsoccasionallyHydrops occasionally develops due to vascular compression

Fetal Chest Fetal Chest

Diagnostic Challenge Pulmonary Sequestration

••SuppliedSupplied by arteries arising from descending aorta

Pulmonary Sequestration Vs. Cystic Adenomatoid Malformation Systemic flow to sequestration

Fetal Chest Fetal Chest

11 Pulmonary Sequestration

Fetal Chest Fetal Chest

Pulmonary Sequestration Congenital Highigh Airwayirway Obstruction Sequence

••LargeLarge lung sequestration may act as an ••ObstructionObstruction of cervical airway ––HighHigh airway obstruction (tracheal or laryngeal) caucausedsed by arteriovenous fistula and cause high-output atresia, stenosis, or web heart failure and hydrops ––TeratomaTeratoma ••Echogenic,Echogenic, overinflated lungs; diaphragm flatteningflattening ••HydropsfindingsHydrops findings with ascitesascitesbut but cardiac function better preserved; can be tolerated for weeks ••PoorPoor prognosis ––LungLung dysfunction ––ChronicChronic tracheostomy; no voice

Fetal Chest Fetal Chest

Congenital Highigh Airwayirway Obstruction Sequence Congenital Highigh Airwayirway Obstruction Sequence

Tracheal Atresia Laryngeal Atresia

High airway obstruction (tracheal(tracheal oror laryngeal)laryngeal) causedcaused byby Dilated trachea & bronchi distal Diaphragm is inverted atresia, stenosis or web toto pointpoint ofof obstructionobstruction Concave shape of the hemidiaphragms towardtoward thethe liverliver secondarysecondary toto thethe enlargedenlarged lungslungs Fetal Chest Fetal Chest

12 Congenital Highigh Airwayirway Obstruction Sequence Congenital Highigh Airwayirway Obstruction Sequence

Fetal ascites is usually present

Bilaterally enlarged echogenic lungs Heart appears small & ML in position Fetal Chest Fetal Chest

Cystic Lymphangioma Cystic Lymphangioma

••AA localized collection of lymphatic vessels ••BenignBenign tumors of the lymphatic system resulting in a nodule or mass resulting in a nodule or mass ••Uni-orUni- or multilocularmultilocularcystic cystic masses ––CheekCheek & parotid ––AroundAround the neck ––75% 75% ––Axilla&Axilla & hemithorax ––20% 20% ––RetroperitoneumRetroperitoneum & abdominal viscera ––LambarregionLambar region ––LimbsLimbs

Fetal Chest Fetal Chest

Cystic Hygromas Cystic Lymphangioma

••DeterminationDetermination of the karyotypekaryotypeis is recommended in all cases ••SerialSerial sonograms ––AssessAssess the growth ––MonitoringMonitoring for the development of hydrops

Cystic hygromas can extend to the scalp, axilla or chest

Fetal Chest Fetal Chest

13 Pleural Effusions Pleural Effusions – Common Causes

••FluidFluid in the pleural cavity ••HydropsfetalisHydrops fetalis (Bilateral)(Bilateral) ••Chromosomal,Chromosomal, most often T21 ••UnilateralUnilateral or bilateral sonolucencysonolucencyin in fetal thoracithoracicc cage ––ImmuneImmune ••SporadicSporadic syndromes, as well as ••ShiftingShifting mediastinum or 4 chambered cardiac apex ––Non-immuneNon-immune some associated anomalies ••ShrunkenShrunken lung parenchyma & fluttering with cardiac rhythm ––PoorPoor prognosis

••ChylothoraxChylothorax (Unilateral)(Unilateral) ––AccumulationAccumulation of lymphatic fluid ––MalformationMalformation in the thoracic duct ••LeakageLeakage from lower portion ––ResultResult in rt. Pleural effusion ••LeakageLeakage from upper portion ––ResultResult in lt. Pleural effusion

Fetal Chest Fetal Chest

Pleural Effusions Pleural Effusions ““ProtocolProtocol AdviceAdvice””

Outcome: ••LookLook for hydrops ––SkinSkin edema ••DependsDepends on degree of pulmonary hypoplasia ––AscitesAscites underlying causes and chromosomal abnormalities ––PericardiaceffusionPericardiac effusion ••LookLook carefully at fetal heart ••IfIf polyhydramniospolyhydramniosdevelops, develops, the prognosis is poor ––StructuralStructural defects ––TachycardiaTachycardia ••LookLook for sign of fetal infection ––Brain,Brain, liver, spleen calcifications ––IntracranialIntracranial hemorrhage ––IUGRIUGR

Fetal Chest Fetal Chest

Thank You

Fetal Chest

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