*3.5.1. Pregnancy management*

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 accurate genetic counseling [16].

#### *3.5.2. Delivery management*

The cesarean delivery should be considered only for maternal indications. There are no indications for resuscitation of the newborn.

#### **3.6. Encephalocele**

#### *3.6.1. Pregnancy management*

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


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

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

#### *3.6.2. Delivery management*

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 a facility with a level III NICU. Surgical treatment is appropriate in most cases unless the encephalocele is massive and there is severe microcephaly or other lethal anomalies. The procedure basically consists of removing the overlying sac and closing the defect including the dural defect [26]. In patients with basal encephaloceles or cerebrospinal fluid (CSF) leakage, prompt closure is important to reduce the risk of infection. Patients with hydrocephalus usually undergo ventriculoperitoneal shunt placement prior to encephalocele repair to prevent postoperative CSF leaks.
