**9.4 Treatment of hydrocephalus**

*Spina Bifida and Craniosynostosis - New Perspectives and Clinical Applications*

for postnatal surgical hydrocephalus treatment [45].

by means of fetoscope.

choice [23].

**9.2 Postnatal surgery**

and wound healing complications [23].

**9.3 Treatment of complications**

patients require repeated surgeries.

children and renal disease in later life.

of multidisciplinary decision. Fetal surgery should be performed only in centres experienced with fetal surgery equipped by neonatology intensive care unit. It requires cooperation of more specialists: pediatric neurosurgeon, gynecologist, fetal surgeon, fetal cardiologist and neonatologist. During this procedure uterus needs to be surgically exposed, opened and spinal cord and meninges of the fetus are closed and covered by skin. In some cases this procedure can be performed less invasively

Prenatal surgery of fetuses with open spina bifida – myelomeningocele before 26th weeks of gestation leads to lower incidence of postnatal presence of Chiari II malformation or its lower severity and less amount of infants with myelomeningocele requiring ventriculo-peritoneal drainage for hydrocephalus. Other abnormalities accompanying Chiari II malformation – such as kinking of brainstem and dislocation of forth ventricle are less frequent in infants who underwent prenatal repair of myelomeningocele [30]. Children after fetal surgery are less likely to need mobility aids, such as crutches. But fetal surgery still remains a risky procedure with possible pregnancy complications such as preterm rupture of membranes and premature delivery before 34th week of gestation in almost half of the cases that underwent fetal surgery [30]. Persistence of Chiari II malformation with descendent herniation on MR imaging 6 weeks after prenatal repair is a predictor of need

Since prenatal surgery might bring more favorable results but carries risk of premature birth, parents facing decision about prenatal versus postnatal repair, need to get reliable and up-to-date information to make this difficult and necessary

Myelomeningicele and meningicele if not treated prenataly, need to be surgically treated as soon as possible within the first days after delivery [46]. Early surgery gives better chances for good outcome. Another reason for early surgery is diminishing the risk of infection of exposed neural structures. During surgical procedure surgeon closes spinal cord a creates a meningeal layer around spinal cord and covers defect with muscular and skin layer. In case of newborn with symptoms of intracranial hypertension, ventriculoperitoneal shunt can be immediately implanted to treat hydrocephalus. A meta-analysis of two prospective studies showed no significant difference in neurological outcome between prenatal and postnatal surgical repair, but suggested improved independent ambulation at 30 months of age in children following prenatal repair [47]. Surgical complications are cerebro-spinal fluid leakage, infection

As the irreparable nerve damage has already occurred during abnormal development all treatment modalities aim to minimize resulting neurological deficit and to treat complications and to exclude them form having impact on life quality and overall survival. Treatment of complications usually begins right after birth. Some

Although advances in treatment of spinal defect, such as fetal surgery and active screening and early treatment of complications have rapidly changed survival of these patients, complications are main cause of mortality of myelomeningocele patients. Most severe complications with possible impact on survival are brain stem dysfunction, hydrocephalus, shunt related complications and ventriculitis in

**40**

Hydrocephalus may be present at birth with symptoms of active hydrocephalus leading to raised intracranial pressure. In such conditions hydrocephalus needs to be treated immediately. In cases without acute symptomatic hydrocephalus could be initial decision of observation. In case of rising symptoms of hydrocephalus it needs to be treated. Typical procedure is insertion of ventriculoperitoneal shunt, in which cerebrospinal fluid is being drained to peritoneal cavity where it is resorbed. This shunt system consists of ventricular catheter, ventil with chamber and distal peritoneal catether. This type of drainage is most common. If there is a contraindication for drainage of cerebrospinal fluid to peritoneal cavity, it can be rarely drained to pleural cavity, right atrium of heart through venous system and very occasionally to gall bladder. Newer method is endoscopical procedure – third ventriculostomy in cases with stenosis of Sylvian aqueduct with cauterisation of choroid plexus to minimize production of cerebrospinal fluid. Possible complications of shunt systems, such as malfunction or infection usually require shunt revision. During growth children usually require revision with prolongation at least once [23].
