**2.4. Prenatal management**

associated with liver herniation. Basically, the main distinguishing ultrasound element is that in BPS, vascularization originates from the systemic circulation, primarily the direct branch from the descending aorta, which can be easily demonstrated with Doppler color or HD-flow Color Doppler [9, 10]. The differentiation criteria between CCAM and BPS

**Figure 8.** Differential diagnosis CCAM versus CDH right: white line—heart shift.

Therefore, the identification of the systemic circulation for a lung tumor mass is pathognomonic for BPS. Recently, Cass described 6 cases of CCAM that also had systemic vasculature and specific histological elements for BPS and CCAM, so these lesions were called hybrid lesions [10]. CDH right with hernia of the liver determines a significant mediastinal shift and secondary pulmonary compression. The highly hyperechogenic aspect of the CCAM microcystic form requires the differentiation from neuroblastoma as well. The association of CCAM with extrapulmonary abnormalities ranges from 0 to 26%, renal agenesis or dysgenesia being

Location Any lobes Inferior left Vascularization Pulmonary Systemic Airway communication Yes No Cysts Yes No

**CCAM BPS**

are shown in **Table 1**.

the most common associations [11, 12].

170 Congenital Anomalies - From the Embryo to the Neonate

**Table 1.** Differential diagnosis CCAM versus BPS.

It is important to emphasize that the mass has an important potential growth between 20 and 26-week gestation, then there is a plateau, and afterwards the mass tends to regress. Thus, since the volume of a mass is not expected to increase after 26 weeks, and if there is no hydrops, then it is unlikely for the hydrops to appear after 26-week gestation. In cases of large masses, it is recommended to plan delivery in a tertiary center because of the risk of lung hypoplasia, cardiovascular decompensation and high mortality. The postnatal risk is high for large masses and low for small-medium masses (**Figure 9**).

Prenatal fetal therapy is indicated in cases that develop hydrops or cardiac failure.

**Figure 9.** CCAM microcystic intraoperator view.

Karyotyping is not an indication if other anomalies are not present. However, amniocentesis for karyotyping is appropriate if fetal treatment is balanced or when the parents request it [14]. The attitude in CCAM associated with hydrops depends on the CVR value. Thus, if the CVR is less than 1.6 and we do not have a dominant cyst, then weekly fetal monitoring is indicated to identify early signs of hydrops. If we are dealing with a dominant cyst, even if CVR is less than 1.6, the fetus has a major risk of developing hydrops and a thoracoamniotic shunt should be considered at first signs of hydrops appearance. If CVR is above 1.6, the likelihood of developing hydrops is very high and monitoring is required 2 times a week.

**3.2. Ultrasound diagnosis**

lowing elements:

fetal chest.

racic kidney.

neuroblastoma.

• hyperechoic mass

• small or moderate size

• the large size can cause fetal hydrops

nating from the aorta (**Figure 10**).

The prenatal ultrasound diagnosis of bronchopulmonary sequestration is based on the fol-

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• a mass with triangular shape, with a paraspinal localization, usually in the base of the left

• color Doppler confirm the origin of the tumoral vessels as belonging to systemic vessels

Typically, BPS vascularization, and more specifically EPS, is supplied by a single artery, origi-

The veins of the BPS drain in the azygos system and hemiazygos. At the opposite end, the venous drainage of the IPS is achieved through the pulmonary veins [17]. The hydrothorax can be associated with BPS, usually ipsilateral, and if it is important, it can cause a mediastinal shift. Differential diagnosis includes the following: CCAM, bronchial atresia, lobar emphysema, CDH-particularly when the liver or spleen is the only component of herniation, mediastinal teratoma, neuroblastoma, mesoblastic nephroma, segmental thoracic obstruction, and tho-

The differential diagnosis between CCAM and BPS, when no systemic feeding vessel is evident, is based on the echogenicity of the mass: the presence of cyst suggests CCAM, while the

For extrapulmonary BPS, the differential diagnosis includes: mesoblastic nephroma, and

presence of a hyperechogenic triangular mass suggests BPS (**Figure 11**).

**Figure 10.** Bronchopulmonary sequestration: systemic vascularization.

The fetal therapy available nowadays is as follows: corticotherapy, in utero fine needle aspiration of macrocysts or thoracoamniotic shunt, laser vascular ablation and, finally, sclerotherapy [15]. A fetus with hydrops below 32-week gestation with a macrocystic lesion of CCAM will benefit from thoracoamniotic shunt. Also, the surgical resection is an option.

Corticosteroid treatment can be followed by the regression of the mass and it is especially indicated in cases of microcystic lesions. If CVR is equal to 1.6, corticosteroid therapy is indicated. Either fine needle aspiration or thoracoamniotic shunt improve the outcome of fetuses with macrocystic CCAM complicated with hydrops/hydrothorax. Microcystic lesions resulting in fetal hydrops of CCAM may need laser ablation of the feeding vessel, to improve survival and with regression of the lesion. The sclerotherapy is also indicated in microcystic cases and it is used Ethanolamine. But it must be emphasized that in most cases fetal CCAM needs only serial fetal surveillance, every 2 or 3 weeks, to confirm regression in size or the remaining at the same size.
