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CHAPTER 36 The Fetal Chest 1255

central nervous system. At times, CHAOS is in association with

a tracheoesophageal istula. his istula acts as an alternative

pathway for the accumulated luid, leading to a decrease in lung

volume, reversal of diaphragmatic eversion, and resolution of

ascites and polyhydramnios. 108

If untreated, laryngeal and tracheal atresia can lead to death

either in utero from hydrops or within minutes ater birth from

respiratory compromise. Neonatal survival is possible if the

delivery is performed with the ex utero intrapartum treatment

(EXIT) procedure. 121-124 he EXIT procedure involves tracheostomy

placement below the level of the obstruction while maternal

placental circulation is maintained.

BRONCHOGENIC CYST

Bronchogenic cysts (Table 36.4) are rare anomalies that result

from abnormal budding or branching of the tracheobronchial

tree. hey are most oten located in the mediastinum in the

subcarinal region. 125 However, 15% of bronchogenic cysts occur

in the lungs, pleura, and diaphragm. hey account for 11% to

18% of mediastinal masses in infants and children. 126,127 he cyst

is lined by ciliated mucus-secreting bronchial epithelium and

may be mucus-illed. Although the mediastinal cysts do not

communicate with the bronchopulmonary tree, intrapulmonary

cysts usually do.

Bronchogenic cysts range in size from a few millimeters to

more than 5 cm. On prenatal ultrasound, they usually appear

as anechoic unilocular intrathoracic cysts (Fig. 36.9), at times

with layering echogenic material. 128,129 If the cyst does not cause

mass efect, it typically will not cause a problem in utero. However,

if the cyst compresses the airway, it can lead to airway obstruction

at birth. 130 he main diferential diagnosis is CPAM. However,

CPAM usually has more than one cyst and an associated echogenic

mass with mass efect. If a bronchogenic cyst causes obstruction,

it may lead to the distal lung accumulating luid, masquerading

as an echogenic lung lesion such as CPAM or sequestration.

he cyst can be mediastinal or parenchymal. Mediastinal

lesions are typically unilocular, located midline in the subcarinal

region (75%). Parenchymal lesions are usually simple unilocular

cysts located along the tracheobronchial tree. Septations may be

seen. At times the cyst can cause bronchial compression and

result in distal overinlation.

Fetal MRI can be helpful in cases with an unclear diagnosis.

he cyst exhibits high signal intensity on T2-weighted imaging.

When the cyst causes obstruction, there will be distal lung

overinlation, and the lung will become hyperintense as well. 130

Surgical resection for bronchogenic cysts is performed postnatally

TABLE 36.4 Differential

Diagnosis of Fetal Thoracic

Cystic Lesion

Congenital pulmonary

airway malformation

(CPAM)

Congenital diaphragmatic

hernia (CDH)

Teratoma

Neurenteric cyst

Bronchogenic cyst

Esophageal duplication

Lymphangioma

Distinguishing Features

Associated with echogenic

lung mass, typically

multiple cysts

Peristalsis of bowel in chest

Stomach above diaphragm

Absence of part of the

diaphragm

Mass does not obey lobar

boundaries; may have

calciications

Adjacent to spine

Typically single cyst, may

obstruct and have distal

hyperinlated lung

Adjacent to esophagus

Crosses anatomic boundaries

A

B

FIG. 36.9 Bronchogenic Cyst at 23 Weeks. (A) Axial gray-scale and (B) sagittal color Doppler ultrasound images of the chest demonstrate a

simple cyst (arrow) posterior to the heart.

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