**9. Treatment**

Treatment of spina bifida depends on severity of primary defect. Occult forms of spina bifida usually do not require any treatment. All other forms are indicated for surgical repair.

There has been a significant improval of patient outcomes in the past 50 years because of multidisciplinary approach with an increased life quality of patients and prolonged life expectancy [43]. But though survival has changed significantly within past decades, there has not been significant improval of neurological outcome [44]. In struggle to ameliorate the neurological outcome fetal surgery seems to be a promise. If fetal surgery is not indicated newborn needs to be operated soon after birth.

As the spina bifida is a complex problem, also treatment needs to be complex and include also treatment of complications.

#### **9.1 Fetal surgery**

Neurological deficit in patients with spina bifida is thought to be result of two circumstances – the primary insult is the congenital anomaly and the second is from direct exposure of spinal cord to amniotic fluid and intrauterine trauma. This hypothesis is the rationale for preventing secondary damage to spinal cord by fetal surgery [21].

Fetal surgery in fetus with open type of spina bifida, though risky, is a promising procedure in improval of neurological outome. This procedure has to take place before completed 26th week of gestation. Indication for fetal surgery is a result

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 by means of fetoscope.

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 for postnatal surgical hydrocephalus treatment [45].

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 choice [23].

#### **9.2 Postnatal surgery**

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 and wound healing complications [23].

#### **9.3 Treatment of complications**

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 patients require repeated surgeries.

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 children and renal disease in later life.

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*Management of Pediatric Patients with Spina Bifida DOI: http://dx.doi.org/10.5772/intechopen.97237*

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].

Patients with open spina bifida need to be regularly evaluated for bladder and bowel functions to minimize the risk of complications and organ damage. Regular blood tests, ultrasound, kidney function tests, urodynamic assessment and X-rays

Urinary and bowel complications are associated with diminished life quality of patients [25]. Degree of continence and management techniques differ by type of

**Urological management** consists of medications, proper and sufficient urine

Urinary management also changes with age of these patients. Chance for spontaneous voiding decreases with age. Daily use of antibiotics as a prophylaxis is rare, but more common in adults. Almost half of patients uses antimuscarinic medication and this is more frequent in children and younger age groups. Alpha adrenergic receptor agonist and antagonist medication to improve continence and voiding is rare [24]. One fourth of adults have undergone bladder augmentation. Creation of continent cathetrizable channel (Mitrofanoff or Monti), cutaneous vesicostomy and bladder outlet operation are more common in adults compared to school children and adults. History of surgery for urinary stones is more prevalent in adult age group. Use of CIC is less frequent in school children than in older age groups and is more common in patients with severe open spinal dysraphism. Management by indwelling catheter, condom catheter, Credé maneuver or urostomy bag (incontinent diversion) is overall less frequent but from age groups is more common in adults. Patients with spina bifida are more likely to develop urinary stones and

derivation – using catheters to empty urinary bladder if necessary and some surgical procedures. Patients with overactive bladder may require anticholinergic medical therapy. Antibiotic medication for treatment of urinary infection and in indicated cases also as a prophylaxis [23, 24]. Some patient also require clean intermittent catheterisation – CIC [23]. Surgical procedures used in these patients are: bladder augmentation, continent cathetrizable urinary channel, cutaneous vesicostomy, bladder outlet operation for continence and urinary stone removal. Used bladder management techniques are: indwelling catether, CIC, cutaneous vesicostomy, urostomy into external appliance, condom catheter and use of Credé maneuver. Some patients are left incontinent in diaper and some have spontaneous

**9.4 Treatment of hydrocephalus**

**9.5 Bladder and bowel management**

need to be performed.

controlled voiding [24].

spina bifida and age of patient.
