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Fetal Chest

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.

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Fetal Chest

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  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

  5. 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.)

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

  7. CDH

  8. 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)

  9. CDH Left side Right side More difficult May be confused for chest mass Contain liver & intestine (use Doppler showing portal vein) Stomach below diaphragm Gallbladder often herniated • 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)

  10. CDH ~ ultrasonographic findings left side (1)

  11. CDH ~ ultrasonographic findings left side (2)

  12. CDH ~ ultrasonographic findings left side (3)

  13. CDH ~ ultrasonographic findings right side (1)

  14. CDH ~ ultrasonographic findings right side (2)

  15. CDH ~ ultrasonographic findings right side (2)

  16. CDH ~ ultrasonographic findings right side (3)

  17. 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

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

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

  20. 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

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

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

  23. CCAM

  24. 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)

  25. CCAM (1)

  26. CCAM (2)

  27. CCAM (3)

  28. CCAM (4)

  29. CCAM (5)

  30. CCAM (6)

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

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

  33. 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

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

  35. 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

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