**3.7. Iniencephaly**

a heterogeneous appearance and is not covered by the cranium. Although the cranial vault is absent, the fetal facial bones can be clearly visualized. Maternal serum alpha-fetoprotein

Exencephaly is a lethal condition, the termination of pregnancy should be recommended for parents. Typically, exencephaly is not associated with chromosomal abnormalities, but, because of the severity of the defect, a chromosome analysis should be performed to permit

The cesarean delivery should be considered only for maternal indications. There are no indi-

Encephalocele can be diagnosed at 11–14 weeks during sonographic screening for aneuploidy.

• Genetic assessment. Cephalocele usually occurs as an isolated lesion, but may be a part of a syndrome such as Meckel (or Meckel-Gruber) or Walker-Warburg syndrome in a small

• Assessment for associated anomalies. Detailed sonography or MRI should be performed to

Obstetrical management depends on the size of defect, the gestational age at diagnosis, and the presence or absence of associated anomalies. Prognosis depends on (1) the presence and amount of brain in the herniated sac (this is the most important consideration) and (2) the presence or absence of hydrocephalus, microcephaly, and other anomalies. If the encephalocele is diagnosed at less than 22–24 weeks of gestation, the termination of the pregnancy can be offered to the parents. If the pregnancy is not terminated, the consultations of neurosur-

Fetuses with neural tube defects or central nervous system abnormalities typically remain active; however, the quality of fetal movement is often different from that in normal fetuses [20]. The fetus with an encephalocele did not respond to repeated vibroacoustic stimulation

When diagnosed prenatally, vaginal delivery may be safe if the lesion is relatively small. Large encephaloceles require cesarean section. Neonates with encephalocele should be delivered at

percentage of cases. Both syndromes are autosomal recessive.

verify the diagnosis and to search for associated anomalies.

geon, neonatologist, and medical genetics are recommended [16].

(VAS) with a movement or fetal heart rate (FHR) acceleration [25].

levels are highly elevated.

476 Congenital Anomalies - From the Embryo to the Neonate

*3.5.1. Pregnancy management*

accurate genetic counseling [16].

cations for resuscitation of the newborn.

*3.5.2. Delivery management*

*3.6.1. Pregnancy management*

*3.6.2. Delivery management*

**3.6. Encephalocele**
