Post on 13-Jan-2016
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
LungsHomogenous 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)
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
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 (2)
CDH ~ ultrasonographic findings right side (3)
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
MRI of CDH (left side) Intestinal loop (red) left lobe of liver (white)Intestinal loop (red) left lobe of liver (white)
MRI of CDH (right side) RT whiteRT white :: liverliver ,, redred :: stomach stomach LT white LT white :: normal right lungnormal right lung ,, redred ::
intestineintestine
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
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 cystsa. 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 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
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-video.swf
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 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
Chest Development
Congenital Diaphragmatic Hernia (CDH)
Cystic Adenomatoid Malformation
Bronchopulmonary Sequestration
(To Be Continued)
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
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