*5.1.1. Pregnancy management*

There is wide variability in the antenatal management of gastroschisis due to a lack of highquality evidence to guide clinical practice [71].


the biparietal diameter, occipitofrontal diameter, and femur length measurements [76]. Oligohydramnios may be related to fetal growth restriction and is a risk factor for cord compression and its sequelae. Polyhydramnios is less common, but an important finding because it is often caused by dysfunction of the gastrointestinal tract due to bowel atresia [73].

with a geneticist [77]. There is a 10–20% risk of Beckwith-Wiedemann syndrome in fetuses

Management of Pregnancy and Delivery in Prenatally Diagnosed Congenital Anomalies

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• Assessment of associated anomalies. Associated abnormalities that occur with increased frequency in these fetuses include additional gastrointestinal abnormalities, cardiac defects (up to 50% of cases), genitourinary anomalies, orofacial clefts, neural tube defects, defects of the diaphragm, polyhydramnios, and growth restriction. Associated syndromes are best categorized by upper, middle, and lower midline omphalocele defects. Omphalocele has been associated with several syndromes, including Pentalogy of Cantrell (upper midline defect), amniotic band sequence, schisis association (at least two of the following defects: neural tube defect, oral cleft, omphalocele, diaphragmatic hernia); lower midline defects are associated with OEIS syndrome (omphalocele, exstrophy of the bladder, imperforate anus, spinal defects), Shprintzen syndrome, Carpenter syndrome, Goltz syndrome, Marshall-Smith syndrome, Meckel-Gruber syndrome, otopalatodigital type II syndrome, CHARGE (coloboma, heart defect, atresia choanae, retarded growth and development, genital abnormality, and ear abnormality) syndrome, and Beckwith-

Wiedemann syndrome (hallmark features: macroglossia, gigantism, omphalocele) [77].

predictive of an increased risk of adverse neonatal outcome [77].

Visceral trauma has also been reported after cesarean delivery [77].

*5.2.2. Delivery management*

**5.3. Bladder exstrophy**

• Follow-up obstetrical care. The serial ultrasound examination every 3–4 weeks to evaluate fetal growth is recommended. When growth is appropriate and amniotic fluid volume is normal, weekly nonstress testing or biophysical profile monitoring at 32 weeks of gestation to assess fetal well-being are recommended, as these pregnancies appear to be at increased risk of late fetal death [79]. Fetal growth restriction and preterm delivery are not uncommon in pregnancies complicated by an omphalocele, particularly with associated abnormalities [77]. Nonreassuring fetal testing and/or cessation of fetal growth at or near term is an indication for early delivery. A systematic error of birth weight underestimation in fetuses affected with omphalocele was found, the same situation as discussed in fetuses with gastroschisis. Intrauterine growth restriction in fetuses with abdominal wall defects is

Delivery should be planning at a tertiary care center. In the absence of standard indications for early delivery, it is reasonable to await spontaneous labor or achieve 39 weeks of gestation. Preterm birth offers no advantage to affected neonates and is associated with increased morbidity and mortality. There is no evidence that cesarean delivery improves outcome in uncomplicated omphalocele; surgery should be reserved for usual obstetric indications [77]. However, some pediatric surgeons have recommended cesarean delivery for fetuses with giant omphaloceles (defined as an omphalocele containing >75% of the liver and defect greater than 5 cm) in an attempt to avoid dystocia, rupture, infection, and hemorrhage [80].

Often the diagnosis of bladder exstrophy is made by prenatal ultrasound and, in some cases, may be confirmed by MRI. In the event that a prenatal diagnosis is not made, the diagnosis

with apparently isolated omphalocele on ultrasound.


#### *5.1.2. Delivery management*

Gastroschisis increases the risk of preterm delivery; delivery should occur in a facility with appropriate resources for caring for these neonates. Gastroschisis alone is not an indication for preterm intervention or cesarean delivery [73].

The decision on timing of delivery is based on a combination of factors, including gestational age, ultrasound findings (fetal growth profile, AFV, appearance of fetal bowel), and fetal testing results (NST, BPP, umbilical cord Doppler if fetal growth restriction is present). In the absence of standard obstetric indications for abdominal delivery, a trial of labor rather than scheduled cesarean birth for most patients is suggested. Cesarean delivery is reasonable if the liver is significantly herniated because of the theoretic risk of dystocia and trauma. Delivery of pregnancies complicated by fetal gastroschisis at 37 or 38 weeks of gestation is suggested to minimize neonatal morbidity and mortality and avoid the possibility of term (39–40 weeks) stillbirth; however, there is no consensus on the optimum timing of delivery of these pregnancies [73].
