**3.5. Exencephaly**

Exencephaly has been detected as early as the 10th postmenstrual week. In the second trimester, the usual appearance of the cranium encasing the brain is lost. The exposed brain has 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 levels are highly elevated.

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

Management of Pregnancy and Delivery in Prenatally Diagnosed Congenital Anomalies

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Iniencephaly is a rare, lethal developmental anomaly. Associated malformations occur in up to 84% of cases and include hydrocephaly, microcephaly, ventricular atresia, holoprosencephaly, polymicrogyria, agenesis of the cerebellar vermis, occipital encephalocele, diaphragmatic hernia, thoracic cage deformities, urinary tract anomalies, cleft lip and palate, omphalocele, and polyhydramnios [20]. The sonographic diagnosis has been made as early as 12.5–13 postmenstrual weeks. Detailed sonography or MRI should be performed to verify the diagnosis and to search for associated anomalies. If the iniencephaly is diagnosed at less than 22–24 weeks of gestation, the termination of the pregnancy can be offered to the

The presence of the hyperextended fetal head might cause dystocia. There is no indication for

Assessment for other abnormalities should be performed by the detailed sonography. Associated brain abnormalities include hydrocephaly, relative microcephaly, agenesis of the corpus callosum, and diastematomyelia. Non-CNS anomalies consist of congenital scoliosis or kyphosis and hip deformities [20]. There is a high prevalence of genetic abnormalities among fetuses with NTDs, especially in the presence of other congenital anomalies so microarray should be offered. The diagnostic sensitivity of prenatal sonography for detection of myelomeningocele in a high risk population is about 97–98% with 100% specificity [27]. Determining the site and extent of the spinal lesion is important because these features correlate with neurologic outcome; more severe neurologic dysfunction is associated with higher and larger lesions. Sonographic diagnosis of open spina bifida typically occurs during the

When the diagnosis of NTD is confirmed, the parents should be offered the opportunity to discuss the long-term prognosis for a child with multidisciplinary team (neonatologist,

postoperative CSF leaks.

*3.7.1. Pregnancy management*

*3.7.2. Delivery management*

*3.8.1. Pregnancy management*

second trimester of the pregnancy.

aggressive resuscitation of neonates [16].

**3.8. Spinal dysraphism and the Arnold-Chiari malformation**

**3.7. Iniencephaly**

parents.
