Fetal Chest 指導 洪正修主任 楊明智主任 主講 陳志堯醫師

Chest Development Congenital Diaphragmatic Hernia Cystic Adenomatoid Malformation Bronchopulmonary Sequestration

Chest Development Congenital Diaphragmatic Hernia Cystic Adenomatoid Malformation Bronchopulmonary Sequestration

Chest Development (1)  Heart most obvious finding in chest ¼ to 1/3 of thoracic cavity Apex direct to left Cardiac axis approximately 45% Normal axis excludes significant chest mass  Lungs Homogenous intermediate echo Echo  G.A Right side > left side TC/AC > 0.8

Chest Development (2) Clinical Importance  Pulmonary hypoplasia ： single most important factor determining survival for many conditions.  Oligohydramnios ： important etiology component of pulmonary hypoplasia. (oligo as short as 6 days may cause P.H.)

Chest Development Congenital Diaphragmatic Hernia (CDH) Cystic Adenomatoid Malformation Bronchopulmonary Sequestration

CDH The commonest contents of a left-sided hernia are: If the defect is right-sided the usual intrathoracic organs are: 80~90% 10% stomach liver bowel gallbladder spleen bilateral < 5%

CDH  Abnormal AC  Most prenatal diagnosed CDH are large  Hydrops uncommon (unless associated malformations)  Small CDHs are easily missed (ex. stomach not herniated ； note cardiac axis)

CDH Left side Cystic mass Absence of fluid-filled stomach Heart to right Polyhydramnios Up to 85% contain herniated liver (liver up) Use Doppler to follow portal vein (to left) Right side More difficult May be confused for chest mass Contain liver & intestine (use Doppler showing portal vein) Stomach below diaphragm Gallbladder often herniated

CDH ~ ultrasonographic findings left side (1)

CDH ~ ultrasonographic findings left side (2)

CDH ~ ultrasonographic findings left side (3)

CDH ~ ultrasonographic findings right side (1)

CDH ~ ultrasonographic findings right side (2)

CDH ~ ultrasonographic findings right side (3)

CDH ~ Pathology Pulmonary hypoplasia Up to 50% associated an abnormality 30% CNS malformation 20% cardiac anomalies Renal & spinal Chromosomal abnormalities common 16~37% Trisomy 18 ， 13 ， 21 ， 9 Epidemiology ： 1 ： 2000~5000 births Embryology ： failure of fusion of posterior pleuroperitoneal membranes

Intestinal loop (red) left lobe of liver (white) MRI of CDH (left side) Intestinal loop (red) left lobe of liver (white)

RT white ： liver ， red ： stomach LT white ： normal right lung ， red ： intestine MRI of CDH (right side) RT white ： liver ， red ： stomach LT white ： normal right lung ， red ： intestine

CDH ~ Clinical Issues 65% survival if isolated Factors which worsen prognosis Other abnormalities Liver in chest (liver up ： 57% mortality ； liver down ： 7%) Diagnosed before 24 wks GA Large size Right or bilateral Polyhydramnios Treatment In utero repair ： not useful Tracheal occlusion Postpartum surgery

Chest Development Congenital Diaphragmatic Hernia (CDH) Cystic Adenomatoid Malformation Bronchopulmonary Sequestration

CCAM  Lung hamartoma with proliferation of terminal bronchioles and lack of normal alveoli.  The other classification is based on the size of the cysts a.Microcystic (cysts less than 5 mm in diameter) b.macrocystic (cysts equal to or greater than 5 mm in diameter) c.mixed

CCAM Type of development Gestational ageDescription CCAM development Pseudoglandular5-17 weeksBronchiolar division; differentiation into air conducting system Type III Canalicular16-25 weeksBeginning terminal sacs development (primitive alveoli); vascularization of lung Type II Terminal sac24 weeks – birthProliferation of terminal sacs; marked thinning of the epithelium; bulging of capillaries into sacs

CCAM ~ Ultrasonographic Findigs Best diagnostic clue ： solid or cystic lung mass with arterial supply from P.A. Size ： variable ， usually contained with one lobe 95% unilateral and one lobe Right = Left May spontaneously regress Hydrops ： most important prognostic factor (< 10%) Color Doppler ： vascular supply from P.A. (D/D with sequestration)

CCAM (1)

CCAM (2)

CCAM (3)

CCAM (4)

CCAM (5)

CCAM (6)

CCAM ~ Image Recommendation Use Doppler to identify feeding vessels Monitor closely ： every 1~2 wks Calculate CCAM volume and the ratio to lung

CCAM ~ Pathology Genetics ： sporadic inheritance ， no recurrence risk Most common fetal lung lesion (75%) Associated anomalies ： 3~12%

CCAM ~ Clinical Issues Usually accidently diagnosed Large for date or polyhydramnios Prognosis Majority remain stable or regress in utero Near 100% mortality with hydrops Dominant large cyst and CVR > 1.6 ： indicate poor prognosis (CVR = CCAM vol./HC) Treatment None unless hydrops

Chest Development Congenital Diaphragmatic Hernia (CDH) Cystic Adenomatoid Malformation Bronchopulmonary Sequestration (To Be Continued)

For God hath not given us the spirit of fear; but of power, and of love, and of a sound mind. For God hath not given us the spirit of fear; but of power, and of love, and of a sound mind. 2 Timothy 1 ： 7 Thanks for Listening