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

Thanks for ListeningThanks for Listening