**4.3. Bronchopulmonary sequestration**

#### *4.3.1. Pregnancy management*

• Cyst aspiration should decompress a large macrocyst and reverse the mediastinal shift.

• Thoracoamniotic shunt provides a therapeutic option for select fetuses with large macrocystic lung lesions or pleural effusion at risk for hydrops and/or pulmonary hypoplasia. Survival following shunting depends on gestational age at birth, reduction in mass size, and hydrops resolution [66]. Complications include displacement or malfunction of the catheter, thrombus occlusion of the catheter, fatal fetal hemorrhage, procedure-related ab-

For solid or mixed solid/cystic CPAM with a large solid component, in-utero open resection has been successfully performed. Following resection, hydrops resolves over 1 to 2 weeks with reversal of the mediastinal shift over 3 weeks [61]. Maternal-fetal surgery requiring hysterotomy appears to be associated with an increased risk of premature labor, premature rupture of membranes, and subsequent pregnancy (uterine dehiscence or rupture) [61]. Percutaneous laser ablation of solid CPAM has been reported in only a few case reports and further research

A single study described fetal sclerotherapy in three patients under 26 weeks with CPAM and hydrops, severe mediastinal shift, and polyhydramnios [67]. Sclerotherapy was performed with percutaneous injection of Ethamolin (ethanolamine oleate) or Polidocanol (aethoxysklerol) into the mass under ultrasound guidance using a 22-gauge needle [61]. Resolution of hydrops and of the mass effect was observed in all cases. The patients were delivered at term without complications. Further studies are indicated to assess the risks and benefits of

If the lung mass has resolved or is small with no mediastinal shift or hydrops, CPAM itself is not an indication for early delivery or cesarean delivery [61]. Neonatal respiratory problems would be unlikely, but the delivery should be recommended in a tertiary care center. For fetuses with large masses that cause mediastinal shift and/or hydrops, delivery should be planned for a tertiary care center with an intensive care nursery capable of resuscitation of a neonate with respiratory difficulties, including capability of extracorporeal membrane oxygenation (ECMO), and with pediatric surgeons experienced in care of these infants [61]. If hydrops develops after 32 weeks of gestation, early delivery is recommended, possibly with the use of EXIT [61]. In EXIT, the fetus is partially delivered and intubated without clamping the umbilical cord. Uteroplacental blood flow and gas exchange are maintained by using inhalational agents to provide uterine relaxation and amnioinfusion to maintain uterine volume. This provides time for resection of the lung mass prior to complete delivery of the infant

Although fluid reaccumulation is common and limits its usefulness [61].

ruptio placentae, premature rupture of membranes, and preterm labor [61].

*4.2.3. Surgical resection*

486 Congenital Anomalies - From the Embryo to the Neonate

is warranted [61].

*4.2.4. Sclerotherapy*

this innovative technique [67].

*4.2.5. Delivery management*

Bronchopulmonary sequestration (BPS) is usually a small lesion and decreases in size in late gestation in about 75% of cases [68].

• Assessment of additional anomalies and genetic evaluation. When a lung mass is first identified, thorough assessment for additional anomalies is necessary. Intralobar sequestration is not associated with an increased risk of additional anomalies [68]. Extralobar BPS is associated with anomalies in up to two-thirds of cases [68]. These anomalies include chest wall and vertebral anomalies hindgut duplications, diaphragmatic hernia, congenital heart disease, and renal and intracranial abnormalities [68]. The incidence of chromosomal abnormalities is not increased above baseline in fetuses with BPS alone [68]. Karyotype analysis is recommended prior to initiating fetal therapy.

Parents should be counseled about the possible course of the BPS during pregnancy. At initial presentation in the early midtrimester, it is difficult to accurately predict what the outcome will be for an individual fetus, but some predictions are possible, e.g., a large BPS with hydrops in the second trimester is likely to do poorly [68].

• Follow-up assessment. All patients should have serial prenatal follow-up examinations to assess change in size of the lung mass and development of hydrops [68]. The frequency depends on the size of the lesion. The larger lesions should be followed more closely. The presence of hydrops is a sign of impending fetal demise and an indication for fetal intervention [68]. This recommendation is based on results from small case series showing higher survival rates if hydrops resolves [68]. Because hydrops is uncommon, fetal intervention is rarely required and is warranted only in cases where the fetus is severely compromised and remote from term. For fetuses greater than 32–34 weeks of age, early delivery with immediate postnatal resection is a reasonable option [68].

For gestations between 20 and 32 weeks, several interventions with the goal of improving fetal hemodynamics and preventing lung hypoplasia have been described and appear to improve survival [69]. These interventions should only be undertaken at centers experienced in fetal surgery. Prenatal intervention requires extensive counseling to the parents on the potential risks versus benefits of surgery.

• Fetal intervention. If the BPS is solid with a large pleural effusion, thoracentesis to prevent pulmonary hypoplasia is possible, but rapid reaccumulation of fluid limits its usefulness. It can be used as a temporizing maneuver to provide prognostic information about the possible result from placement of a thoracoamniotic shunt [68]. The fluid should be sent for cell count to exclude an infectious etiology [68]. Complications of shunts include displacement or malfunction of the catheter, thrombus occlusion of the catheter, fatal fetal hemorrhage, procedure-related abruptio placentae, premature rupture of membranes, and preterm labor [68]. There is also a risk of trauma to the fetal chest wall, especially if the procedure is performed before 20 weeks [68].

**5. Congenital abdominal wall defects**

quality evidence to guide clinical practice [71].

There is wide variability in the antenatal management of gastroschisis due to a lack of high-

Management of Pregnancy and Delivery in Prenatally Diagnosed Congenital Anomalies

http://dx.doi.org/10.5772/intechopen.71802

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• Assessment of associated anomalies. Associated gastrointestinal anomalies and problems (e.g., malrotation, atresia, stenosis, perforation, necrosis, volvulus) occur in up to 25% of cases [72] and may be related to vascular disruption caused by herniated bowel. Disruption of the superior mesenteric artery, for example, may lead to volvulus or to "apple peel" jejunal-ileal lesions. Meckel's diverticulum and gallbladder atresia also occur, but are less common. Bladder herniation has been reported in 6% of cases, with bowel or urinary tract dilation [73]. Most cases have no extraintestinal abnormalities, approximately 10% of gastroschisis cases were associated with major unrelated defects, approximately 2% of cases were part of a recognized syndrome, and cardiac anomalies were detected in 2–3% of cases [74]. Oligohydramnios is the most common amniotic fluid abnormality, but polyhydramnios may occur, particularly in fetuses with reduced bowel motility or

• Genetic assessment. The prevalence of chromosomal abnormalities in fetuses with isolated gastroschisis is not increased above the baseline population risk, so invasive fetal genetic testing is not routinely offered. The fetal genetic evaluation is suggested if nongastrointestinal structural abnormalities are identified on ultrasound examination. Chromosome abnormalities were detected in 1.2% of the total cases, which included isolated and nonisolated, and the most frequent abnormalities were trisomy 18, trisomy 13, sex chromosome

• Follow-up assessment. The most common pregnancy complications associated with gastroschisis include development of growth restriction (30–60% of cases), intrauterine fetal demise (3–6%), spontaneous preterm birth (30%), and bowel dilation and wall thickening (common, frequency depends on diagnostic criteria) [73]. The mechanisms causing these adverse outcomes in gastroschisis are unclear. Therefore, pregnancy monitoring is empiric and typically includes serial ultrasound examinations for assessment of fetal growth and fetal bowel abnormality and standard tests for antepartum fetal

• Assessment of fetal growth and amniotic fluid volume—serial ultrasound examinations every 3 weeks for assessment of fetal growth and amniotic fluid volume (AFV). If growth arrest or oligohydramnios is diagnosed, umbilical artery Doppler flow is evaluated [73]. A systematic error of birth weight underestimation when using the Hadlock formulas in fetuses affected with gastroschisis was found [75]. Siemer and colleagues developed a specific formula for estimating fetal weight in fetuses with abdominal wall defects using

**5.1. Gastroschisis**

obstruction [73].

surveillance [73].

anomalies, and trisomy 21 [73].

*5.1.1. Pregnancy management*

• In-utero open resection, percutaneous laser ablation of the feeding vessel has been successfully performed in several small studies [68]. Percutaneous ultrasound-guided fetal sclerotherapy has also been described [68]. Sometimes two procedures were necessary.
