1. Fetal Chest 指導 洪正修主任 楊明智主任 主講 陳志堯醫師

2. Chest Development Congenital Diaphragmatic Hernia Cystic Adenomatoid Malformation Bronchopulmonary Sequestration

3. Chest Development Congenital Diaphragmatic Hernia Cystic Adenomatoid Malformation Bronchopulmonary Sequestration

4. 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

6. 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.)

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

8. 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%

9. 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)

10. 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

11. CDH ~ ultrasonographic findings left side (1)

12. CDH ~ ultrasonographic findings left side (2)

13. CDH ~ ultrasonographic findings left side (3)

14. CDH ~ ultrasonographic findings right side (1)

15. CDH ~ ultrasonographic findings right side (2)

17. CDH ~ ultrasonographic findings right side (3)

18. 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

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

20. 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

21. 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

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

23. 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

24. 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

25. 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)

26. CCAM (1)

27. CCAM (2)

28. CCAM (3)

29. CCAM (4)

30. CCAM (5)

31. CCAM (6)

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

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

34. 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

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

36. 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