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Fetal Chest
指導 洪正修主任 楊明智主任主講 陳志堯醫師
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Chest Development
Congenital Diaphragmatic Hernia
Cystic Adenomatoid Malformation
Bronchopulmonary Sequestration
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Chest Development
Congenital Diaphragmatic Hernia
Cystic Adenomatoid Malformation
Bronchopulmonary Sequestration
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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
LungsHomogenous intermediate echo
Echo G.A
Right side > left side
TC/AC > 0.8
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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.)
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Chest Development
Congenital Diaphragmatic Hernia (CDH)
Cystic Adenomatoid Malformation
Bronchopulmonary Sequestration
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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%
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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)
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CDHLeft side
Cystic massCystic mass
Absence of fluid-filled Absence of fluid-filled stomachstomach
Heart to rightHeart to right
PolyhydramniosPolyhydramnios
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 May be confused for chest massmass
Contain liver & intestine Contain liver & intestine (use Doppler showing (use Doppler showing portal vein)portal vein)
Stomach below diaphragm
Gallbladder often herniated
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CDH ~ ultrasonographic findings left side (1)
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CDH ~ ultrasonographic findings left side (2)
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CDH ~ ultrasonographic findings left side (3)
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CDH ~ ultrasonographic findings right side (1)
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CDH ~ ultrasonographic findings right side (2)
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CDH ~ ultrasonographic findings right side (2)
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CDH ~ ultrasonographic findings right side (3)
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CDH ~ PathologyPulmonary hypoplasia
Up to 50% associated an abnormality30% CNS malformation
20% cardiac anomalies
Renal & spinal
Chromosomal abnormalities common16~37%
Trisomy 18 , 13 , 21 , 9
Epidemiology : 1 : 2000~5000 births
Embryology : failure of fusion of posterior pleuroperitoneal membranes
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MRI of CDH (left side) Intestinal loop (red) left lobe of liver (white)Intestinal loop (red) left lobe of liver (white)
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MRI of CDH (right side) RT whiteRT white :: liverliver ,, redred :: stomach stomach LT white LT white :: normal right lungnormal right lung ,, redred ::
intestineintestine
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CDH ~ Clinical Issues65% survival if isolated
Factors which worsen prognosisOther abnormalities
Liver in chest (liver up : 57% mortality ; liver down :7%)
Diagnosed before 24 wks GA
Large size
Right or bilateral
Polyhydramnios
TreatmentIn utero repair : not useful
Tracheal occlusion
Postpartum surgery
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Chest Development
Congenital Diaphragmatic Hernia (CDH)
Cystic Adenomatoid Malformation
Bronchopulmonary Sequestration
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CCAM
Lung hamartoma with proliferation of terminal bronchioles and lack of normal alveoli.
The other classification is based on the size of the cystsa. Microcystic (cysts less than 5 mm in diameter)
b. macrocystic (cysts equal to or greater than 5 mm in diameter)
c. mixed
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CCAM
Type of development
Gestational age DescriptionCCAM
development
Pseudoglandular 5-17 weeks Bronchiolar division; differentiation into air conducting system
Type III
Canalicular 16-25 weeks Beginning terminal sacs development (primitive alveoli);
vascularization of lung
Type II
Terminal sac 24 weeks – birth Proliferation of terminal sacs; marked thinning
of the epithelium; bulging of capillaries
into sacs
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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)
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CCAM (1)
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CCAM (2)
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CCAM (3)
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CCAM (4) CCAM-video.swf
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CCAM (5)
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CCAM (6)
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CCAM ~ Image Recommendation
Use Doppler to identify feeding vessels
Monitor closely : every 1~2 wks
Calculate CCAM volume and the ratio to lung
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CCAM ~ Pathology
• Genetics : sporadic inheritance , no recurrence risk
• Most common fetal lung lesion (75%)
• Associated anomalies : 3~12%
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CCAM ~ Clinical IssuesUsually accidently diagnosed
Large for date or polyhydramnios
PrognosisMajority 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)
TreatmentNone unless hydrops
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Chest Development
Congenital Diaphragmatic Hernia (CDH)
Cystic Adenomatoid Malformation
Bronchopulmonary Sequestration
(To Be Continued)
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For God hath not given us the spirit For God hath not given us the spirit of fear; but of power, and of love, of fear; but of power, and of love, and of a sound mind.and of a sound mind.
2 Timothy 1 :7
Thanks for ListeningThanks for Listening