**3.9. Cantrell pentalogy**

With an incidence of 5.5 cases per 1 million live births and a male predominance [107], pentalogy of Cantrell is characterized by a midline, supraumbilical AWD, with a defect of the lower sternum, deficiency of the anterior diaphragm, defect in the diaphragmatic pericardium and cardiac anomalies such as septal defects and tetralogy of Fallot [108]. The main event during embryogenesis, thought to be the cause of this rare anomaly, is an abnormal differentiation of the intraembryonic mesoderm, at approximately 14–18 days after conception [108]. Chromosomal anomalies, such as trisomy 13, 18 and Turner syndrome, are often associated, so the invasive diagnosis is mandatory. Other anomalies observed with pentalogy of Cantrell include craniofacial and vertebral anomalies. US diagnosis of Cantrell's pentalogy is possible early, at 10 weeks' gestation, using 2D and 3D scans [109]. The combination of omphalocele and ectopia cords highly indicates a case of pentalogy of Cantrell [110]. The pentalogy is "complete" if four or all five defects are present, and is "incomplete" when various combination of defects are observed, if a sternal abnormality is present [86]. The AWD may contain stomach, liver,


intrauterine growth restriction and oligohydramnios seem not to worsen the prognosis of fetuses with gastroschisis [116]. Fetal bowel features can be also evaluated, to estimate postnatal bowel complications. A cut-off of 1 cm for bowel diameter was considered a far-seeing marker for bowel damage [117, 118]. Overall, there is not yet a consensus regarding how and when fetal monitoring during pregnancy. Because of the associated risk, recommended attitude is a careful monitoring and a monthly interval control scheme, somewhat arbitrarily chosen. In the third trimester, repeated fetal monitoring is indicated [111]. Hospital admittance was proposed at 35 weeks of gestation, as many patients with fetal AWDs deliver pre-

Fetal Abdominal Wall Defects

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Even with recent progress in major medical and surgical specialties, the mode and time of delivery of fetuses with antenatal diagnosed abdominal wall defects remains a controversy. Fetal delivery by elective cesarean section is advocated by some centers [119–125], while others consider a vaginal delivery more suitable in cases with diagnosed fetal abdominal wall defect [126–130]. More so, there is no difference in fetal outcome regarding the mode of delivery [131–135]. In cases of omphalocele, delivery by cesarean section is recommended in cases with a large defect, to prevent the sac rupture and the liver damage during labor [136]. However, some researchers found that features such as the size or liver herniation have no importance in establishing the outcome of vaginal delivery [137]. The gestational age for induced delivery or elective cesarean section is another controversy (preterm versus term delivery). Some authors reported more complications and longer hospitalization in preterm deliveries [138, 139]. Others recommend a preterm delivery to optimize the toxic damage of the amniotic fluid to the herniated bowel in gastroschisis [120, 124, 129, 140]. The most recent study presented good results using a protocol for a preterm elective delivery, between 35 and 36 + 6 gestational age for fetuses with gastroschisis. Preterm delivery is not indicated in cases of omphalocele [26]. Still, most studies agree that in utero transport to a specialized pediatric center, where the defect can be corrected, offers an optimal fetal

Postpartum, fetuses with gastroschisis must benefit from intravenous fluid resuscitation and wrapped herniated loops in warm saline as there is an increased risk for water and heat losses by evaporation. Specialized management of gastroschisis includes repositioning of the herniated bowel into the abdominal cavity, with closure of the abdominal wall (primary reduction and repair). In such cases, there is a high risk of respiratory complication. The surgical procedure can be also postponed if the patient is unstable [18], but with subsequent longer time

maturely [112, 115].

outcome [126, 141].

**6.1. Gastroschisis**

**6. Postnatal prognosis and management**

**5. Mode and time of delivery**

**Figure 5.** Ultrasound findings in fetal abdominal wall defects [26].

bowel or total abdominal contents, evisceration. There is a pleural and pericardial effusion and the fetal heart is completely external or just partially. The prognosis is fatal and the survival is uncommon. Prenatal diagnosis is important as termination of pregnancy is the only option for the couple.

The ultrasound features that best characterize fetal AWDs are presented in **Figure 5** [26].
