**9.5 Bladder and bowel management**

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 need to be performed.

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

**Urological management** consists of medications, proper and sufficient urine 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 controlled voiding [24].

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

this risk increases with age, so higher rate of urinary stone removal in adults is not unexpected [24].

Bladder continence is reported to increase by age. Around one third of patients is continent in school age but almost half of the patients in adult age [24]. Though urinary incontinence affects life quality, it has lower impact than bowel incontinence [48].

**Bowel management** consists of oral medications, antiemetics, suppositories and sometimes also manual derivation of stools by digital stimulation and disimpaction of stools. Other management possibilities are also use of standard rectal enema, cone enema and mini enema [19]. Though rarely, some cases are treated with anal plug [23].

Dramatic improvement of life quality of patients with severe anal incontinence, severe constipation and no anal control can experience with antegrade continence enema – ACE, that is minimally invasive surgical procedure [23].

Other surgical procedures for treatment of neurogenic bowel dysfunction are: cecostomy button or tube, cecostomy button closure, colostomy and ileostomy [19].

Type of used bowel management tool may also differ by age. Use of oral medication is low or insufficient in all age groups. Timed evacuation and suppositories are more used in small children and their use decreases with age. Standard rectal enemas are more used in school children and adults and less by adolescents. Cone and mini enemas are more used in school children and less by adolescents and adults. Antegrade enemas through a surgically created chanel or a cecostomy button and tube are more used in adolescent age than in childhood and adulthood. Digital stimulation and disimpaction is used by small number of patients and mostly used in adults. Higher prevalence of colostomy is in adults. Ileostomy is less frequently performed [19]. Degree of continence and sufficiency of incontinence control has great impact on life quality of patients with spina bifida [19].

#### **9.6 Treatment of orthopedic complications**

The mail goal of orthopedic treatment in patients with spina bifida is to correct deformities that may prevent the patient from using orthoses to ambulate during childhood [11]. Orthopedic complications are common in patients with spinal defects, mostly congenital and acquired deformities. Frequent congenital defermities are kyphosis, hemivertebra, teratologic hip dislocation, clubfoot and vertical talus. Acquired developmental deformities are related to the level of defect and are caused by muscle imbalance, paralysis and lack of sensitivity in lower extremities, such as contractures, calcaneus and cavovarus. Orthopedical complications may also be a consequence of surgical intervention – for example in postoperative tethered cord syndrome [11].

Orthopedical surgical interventions are most common within first 5 years of life, especially within the first year of life – mostly due to presence of congenital deformities that require surgical correction [38].

Deformities of **hip joint** are mostly contracture, subluxation and dislocation and also rotational deformity of the lower extremity, such as internal or external torsion. This deformity is a result of muscle imbalance and paralysis of the supporting muscles. Untreated deformity could lead to pelvic obliquity and compensatory spinal deformity.

**Knee joint** can be affected by flexion or extension contracture, valgus deformity or late knee instability and pain. Contractures are more common in patients with higher location of spinal defect. Flexion contracture is a result of gradual contracture of the hamstrings with contracture of the posterior knee capsule due to prolonged sitting and quadriceps weakness or spasticity of the hamstrings and

**43**

ovarus [49].

medial cuneiform.

rior tibiotalar and talocalcaneal joints.

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

improve the ambulation.

adulthood.

quadriceps weakness due to tethered spinal cord. Flexion deformity can be present in ambulatory and also non-ambulatory patients. Surgical release of the hamstrings, gastrocnemius and posterior capsule can decrease the angle of contracture and

Extension contractures are much less common and occurs secondary to unopposed quadriceps function in weak hamstrings, extensive bracing in extension or surgical treatment for flexion contracture, but most of the cases are congenital and often also with teratogenic hip dislocation or clubfoot. Initial treatment is usually serial casting attempting to achieve satisfactory knee flexion that is around 90 degree. If the contraction interferes with gait and is persistent, usually VY plasty of quadriceps is performed with good results. In non-ambulatory patients in whom the contraction causes difficulties with sitting, tenotomy of patellar tendon is an option. Deformity of the knee joint is a result of static forces of positioning, fibrosis of the surrounding muscles, muscle imbalance around knee joint and fracture malunion. Valgus knee deformity is more common in patients with low-lumbar or sacral dysraphism and leads to instability, pain and acceleration of arthrosis in

Torsional deformities of femur and tibia are frequent in patients with severe spinal dysraphims and presents more severe problem for ambulatory patients. Femoral torsion is present normally in all newborns initially, but in children with severe spina bifida does not decrease with growth due to abnormal gait and activity. Tibial torsion is more common. Internal torsion is congenital and frequently associated with clubfoot and external torsion is acquired secondary to muscle imbalance. Initial treatment is usually ankle-foot orthosis with twister cables. If not successful, internal torsion of tibia can be treated by rotational osteotomy and severe cases of

**Foot and ankle** deformities lead to problems with effective ambulation, cause difficulties with bracing and shoe wear, affect cosmetic appearance of foot and can cause skin irritation leading to pressure sores [49]. Surgical intervention is usually tendor excision or osteotomy for bony deformity followed by use of ankle-foot orthosis during day and splint during night. Arthrodesis in these deformities should be strictly avoided because stiffness resulting from fusion in combination with

Clubfoot is the most common foot deformity in spina bifida patients [50]. Factors that contributes to development of clubfoot are spasticity, intrauterine positioning, contractures and muscle imbalance. Treatment methods that could be used are: serial manipulations and long-leg casting to gradually correct deformity and tenotomy of the Achilles tendon followed by foot abduction bracing for several years. If this treatment is ineffective there is an indication for double osteotomy – closing wedge osteotomy of the cuboid with an opening wedge osteotomy of the

Equinus is also frequent deformity. Spasticity probably plays the most important role in it etiology. In prevention regular routine of passive stretching with a night-time ankle-foot orthosis is used. Surgical treatment is indicated in patients with unbraceable foot with skin breakdowns. Mild deformities respond to Achilles tendon excision, severe contractures require radical posterior release in the poste-

Another contracures occuring in spina bifida patients are vertical talus, calcaneus or calcaneovalgus, ankle valgus, hindfoot valgus, cavus, varus and cav-

More severe cases may require surgical repair – correction of ankle or foot deformity, correction of congenital foot deformity, correction of equinus contracture, tendon excisions, correction of scoliosis, osteotomy for correction of bony

external torsion by internal rotational osteotomy of the tibia.

insensate foot can cause neuropathic skin changes [49].

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

enema – ACE, that is minimally invasive surgical procedure [23].

great impact on life quality of patients with spina bifida [19].

**9.6 Treatment of orthopedic complications**

tethered cord syndrome [11].

spinal deformity.

mities that require surgical correction [38].

unexpected [24].

incontinence [48].

with anal plug [23].

this risk increases with age, so higher rate of urinary stone removal in adults is not

**Bowel management** consists of oral medications, antiemetics, suppositories and sometimes also manual derivation of stools by digital stimulation and disimpaction of stools. Other management possibilities are also use of standard rectal enema, cone enema and mini enema [19]. Though rarely, some cases are treated

Dramatic improvement of life quality of patients with severe anal incontinence, severe constipation and no anal control can experience with antegrade continence

Other surgical procedures for treatment of neurogenic bowel dysfunction are: cecostomy button or tube, cecostomy button closure, colostomy and ileostomy [19]. Type of used bowel management tool may also differ by age. Use of oral medication is low or insufficient in all age groups. Timed evacuation and suppositories are more used in small children and their use decreases with age. Standard rectal enemas are more used in school children and adults and less by adolescents. Cone and mini enemas are more used in school children and less by adolescents and adults. Antegrade enemas through a surgically created chanel or a cecostomy button and tube are more used in adolescent age than in childhood and adulthood. Digital stimulation and disimpaction is used by small number of patients and mostly used in adults. Higher prevalence of colostomy is in adults. Ileostomy is less frequently performed [19]. Degree of continence and sufficiency of incontinence control has

The mail goal of orthopedic treatment in patients with spina bifida is to correct deformities that may prevent the patient from using orthoses to ambulate during childhood [11]. Orthopedic complications are common in patients with spinal defects, mostly congenital and acquired deformities. Frequent congenital defermities are kyphosis, hemivertebra, teratologic hip dislocation, clubfoot and vertical talus. Acquired developmental deformities are related to the level of defect and are caused by muscle imbalance, paralysis and lack of sensitivity in lower extremities, such as contractures, calcaneus and cavovarus. Orthopedical complications may also be a consequence of surgical intervention – for example in postoperative

Orthopedical surgical interventions are most common within first 5 years of life, especially within the first year of life – mostly due to presence of congenital defor-

Deformities of **hip joint** are mostly contracture, subluxation and dislocation and also rotational deformity of the lower extremity, such as internal or external torsion. This deformity is a result of muscle imbalance and paralysis of the supporting muscles. Untreated deformity could lead to pelvic obliquity and compensatory

**Knee joint** can be affected by flexion or extension contracture, valgus deformity or late knee instability and pain. Contractures are more common in patients with higher location of spinal defect. Flexion contracture is a result of gradual contracture of the hamstrings with contracture of the posterior knee capsule due to prolonged sitting and quadriceps weakness or spasticity of the hamstrings and

Bladder continence is reported to increase by age. Around one third of patients is continent in school age but almost half of the patients in adult age [24]. Though urinary incontinence affects life quality, it has lower impact than bowel

**42**

quadriceps weakness due to tethered spinal cord. Flexion deformity can be present in ambulatory and also non-ambulatory patients. Surgical release of the hamstrings, gastrocnemius and posterior capsule can decrease the angle of contracture and improve the ambulation.

Extension contractures are much less common and occurs secondary to unopposed quadriceps function in weak hamstrings, extensive bracing in extension or surgical treatment for flexion contracture, but most of the cases are congenital and often also with teratogenic hip dislocation or clubfoot. Initial treatment is usually serial casting attempting to achieve satisfactory knee flexion that is around 90 degree. If the contraction interferes with gait and is persistent, usually VY plasty of quadriceps is performed with good results. In non-ambulatory patients in whom the contraction causes difficulties with sitting, tenotomy of patellar tendon is an option.

Deformity of the knee joint is a result of static forces of positioning, fibrosis of the surrounding muscles, muscle imbalance around knee joint and fracture malunion. Valgus knee deformity is more common in patients with low-lumbar or sacral dysraphism and leads to instability, pain and acceleration of arthrosis in adulthood.

Torsional deformities of femur and tibia are frequent in patients with severe spinal dysraphims and presents more severe problem for ambulatory patients. Femoral torsion is present normally in all newborns initially, but in children with severe spina bifida does not decrease with growth due to abnormal gait and activity. Tibial torsion is more common. Internal torsion is congenital and frequently associated with clubfoot and external torsion is acquired secondary to muscle imbalance. Initial treatment is usually ankle-foot orthosis with twister cables. If not successful, internal torsion of tibia can be treated by rotational osteotomy and severe cases of external torsion by internal rotational osteotomy of the tibia.

**Foot and ankle** deformities lead to problems with effective ambulation, cause difficulties with bracing and shoe wear, affect cosmetic appearance of foot and can cause skin irritation leading to pressure sores [49]. Surgical intervention is usually tendor excision or osteotomy for bony deformity followed by use of ankle-foot orthosis during day and splint during night. Arthrodesis in these deformities should be strictly avoided because stiffness resulting from fusion in combination with insensate foot can cause neuropathic skin changes [49].

Clubfoot is the most common foot deformity in spina bifida patients [50]. Factors that contributes to development of clubfoot are spasticity, intrauterine positioning, contractures and muscle imbalance. Treatment methods that could be used are: serial manipulations and long-leg casting to gradually correct deformity and tenotomy of the Achilles tendon followed by foot abduction bracing for several years. If this treatment is ineffective there is an indication for double osteotomy – closing wedge osteotomy of the cuboid with an opening wedge osteotomy of the medial cuneiform.

Equinus is also frequent deformity. Spasticity probably plays the most important role in it etiology. In prevention regular routine of passive stretching with a night-time ankle-foot orthosis is used. Surgical treatment is indicated in patients with unbraceable foot with skin breakdowns. Mild deformities respond to Achilles tendon excision, severe contractures require radical posterior release in the posterior tibiotalar and talocalcaneal joints.

Another contracures occuring in spina bifida patients are vertical talus, calcaneus or calcaneovalgus, ankle valgus, hindfoot valgus, cavus, varus and cavovarus [49].

More severe cases may require surgical repair – correction of ankle or foot deformity, correction of congenital foot deformity, correction of equinus contracture, tendon excisions, correction of scoliosis, osteotomy for correction of bony

deformity, osteotomy of femur, pelvic osteotomy, reduction of hip dislocation, release of contracture of hip or knee joint, spinal fusion and corrective osteotomies for scoliosis. Spina bifida patients are at higher risk of postoperative complications such as wound infection, delayed union or malunion, skin breakdowns and post-immobilization fractires. For post-surgical imobilisation custom-molded total body splint should be used and spica total body cast should be avoided [11].

Measures of muscle strenght are key for surgical management of orthopedic patients [38]. Better functional effect in patients with spina bifida is a result of better understanding of impact of radiologically diagnosed deformities on functional effect within last decades and their earlier treatment as a prevention of deterioration [11].

#### **9.7 Complications of mobility**

Due to present paraparesis in almost all cases, patient usually need mobility aids, such as: crutches, braces, walkers or even wheelchair at least for some time during growth. These mobility aids together with regular physiotherapy can help the child to become more independent. For daily functioning some aids could be used such as walking frames, commode chairs and bath chairs.

According to functional mobility ambulatory outcome could be:

**Community ambulator** who walks indoors and outdoors for most activities and may need crutches or braces.

**Household ambulator** who walks only indoors with help of aids and may use a wheelchair for some activities.

**Therapeutic ambulator** who can walk only in therapy session, sometimes with help of appartuses.

**Nonambulator** who exclusively uses a wheelchair for most of his activities [38].

Various factors affect ability to ambulate, most important of those are: level of neurological impairment, hip deformity, scoliosis, foot and ankle deformity, age and presence of obesity [11].

Almost all patients with severe forms of spinal dysraphism with exception of some patients with low sacral lesions will require use of orthoses in ambulation from indications, such as maintanance of alignment, prevention of deformity, correction of flexible deformity, facilitation of independent mobility and protection of insensitive limb.

In children with defect in thoracic and high-lumbar regions around age of 12 months if child controls head and neck position, usually standing frame is prescribed, later hip-knee-ankle-foot orthosis and reciprocating gait orthosis.

Patients with low-lumbar and sacral spinal dysrahpism usually require solid ankle-foot orthosis as it substitues for weak or absent ankle plantar flexors and dorsiflexors. Use of forearm crutches should be considered as well, as these patients often display weakness of hip extensors and abductors. Patients with internal or external rotational deformity can benefit from ankle-foot orthosis with twister cables. Also knee-ankle-foot orthosis can be used in patients with excessive valgus stress at the knee joint who are too young for correctional osteotomy.

#### **9.8 Wound healing and prevention**

Skin problems are very common in patients suffering from spina bifida [50]. As the skin sensitivity is affected in children and adults born with open spina bifida they are more likely to develop various skin problems. Blisters, soles, calluses and burns on lower extremities are frequent. When found late this skin problems can lead to serious problems of complicated healing.

**45**

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

lenght of the extremity as possible [49].

wound healing is increasing with age.

and during early stages of pregnancy.

**9.10 Cardiometabolic dysfunction**

exercise can improve metabolic dysfunction [51].

wall is increased what predicts endothelial dysfunction [51].

hypertension and insulin resistance [55].

**9.11 Treatment of other complications**

**9.9 Sexual dysfunction**

The most common site of pressure sores are sacrum, ischial tuberosity, greater trochanter and feet. Severe complication of skin breakdown and pressure sores is osteomyelitis of the underlying bone. When wound does not heal with appropriate soft tissue care, underlying deep infection must be suspected and diagnosed by laboratory and radiograph findings. Antibiotic treatment is indicated, in more severe cases also surgical debridement. Last therapeutical opportunity in severe non-healing deep wounds causing sepsis is amputation with preservation as much

Parents and caregives need to prevent bed sores – avoid lying on one side too long, prevent sun burns and regularly control skin of back and lower extremities at least once a day. In case of new orthosis even more often. In older children it is necessary to control if the shoes fit properly. Barefoot walking should be avoided, especially on rough and hot surfaces. Self-adhesive foam pads can be used over pressure points [49]. Interventions of plastic surgeon within first year of life are mostly skin flaps and skin grafts associated with spinal closure. Need for surgery due to complications of

In case of more severe bad healing wounds there is sometimes necessary to perform surgical debridement of skin wound, incision and drainage of abscess, skin flap operations, flap grafts, skin grafting, reduction of callus and revisions of skin scars [38].

Because of nerve damage patients born with open spina bifida defect suffer from sexual dysfunction in adolescence and adulthood. This might affect their sexual life. But most of spina bifida patients are fertile and are able to have children. Women born with open spina bifida should plan their pregnancy and are recommended to take 4 mg of folic acid a day (normal dose is 400 mcg) a month prior to conception

Patients with spina bifida affecting mobility have higher risk of developing components of metabolic syndrome in younger age due to low physical activity. They often suffer from abdominal obesity, insulin resistance or dyslipidemia with its metabolic and vascular complications [51]. People with physical disabilities spend less time performing physical activities compared to their nondisabled peers [52]. Consequences of a sedentary lifestyle include physical deconditioning [53]. Physical

Overweight, obesity, high BMI, high waist circumference and percentage of body fat are more prevalent in patients with spina bifida and they also have reduced aerobic fitness and muscle strenght [54]. Metabolic syndrome is more prevalent in spina bifida patients compared to nondisabled controls. They often display high levels of VLDL and overall cholesterol, triglycerides and low HDL, suffer from

Arterial diameters in these patients are reduced and sheer stress on the vascular

Patients with spina bifida often have post-operative complications, mainly infectious complications – wound and urinary infections, that require antibiotic therapy. According to frequent latex allergy in these patients, they should be operated and treated only in latex-free gloves in order to avoid severe anaphylactic reaction [11].

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

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

of deterioration [11].

**9.7 Complications of mobility**

may need crutches or braces.

wheelchair for some activities.

and presence of obesity [11].

**9.8 Wound healing and prevention**

lead to serious problems of complicated healing.

help of appartuses.

of insensitive limb.

walking frames, commode chairs and bath chairs.

According to functional mobility ambulatory outcome could be:

deformity, osteotomy of femur, pelvic osteotomy, reduction of hip dislocation, release of contracture of hip or knee joint, spinal fusion and corrective osteotomies for scoliosis. Spina bifida patients are at higher risk of postoperative complications such as wound infection, delayed union or malunion, skin breakdowns and post-immobilization fractires. For post-surgical imobilisation custom-molded total body splint should be used and spica total body cast should be avoided [11]. Measures of muscle strenght are key for surgical management of orthopedic patients [38]. Better functional effect in patients with spina bifida is a result of better understanding of impact of radiologically diagnosed deformities on functional effect within last decades and their earlier treatment as a prevention

Due to present paraparesis in almost all cases, patient usually need mobility aids, such as: crutches, braces, walkers or even wheelchair at least for some time during growth. These mobility aids together with regular physiotherapy can help the child to become more independent. For daily functioning some aids could be used such as

**Community ambulator** who walks indoors and outdoors for most activities and

**Household ambulator** who walks only indoors with help of aids and may use a

**Therapeutic ambulator** who can walk only in therapy session, sometimes with

**Nonambulator** who exclusively uses a wheelchair for most of his activities [38]. Various factors affect ability to ambulate, most important of those are: level of neurological impairment, hip deformity, scoliosis, foot and ankle deformity, age

Almost all patients with severe forms of spinal dysraphism with exception of some patients with low sacral lesions will require use of orthoses in ambulation from indications, such as maintanance of alignment, prevention of deformity, correction of flexible deformity, facilitation of independent mobility and protection

In children with defect in thoracic and high-lumbar regions around age of 12 months if child controls head and neck position, usually standing frame is prescribed, later hip-knee-ankle-foot orthosis and reciprocating gait orthosis. Patients with low-lumbar and sacral spinal dysrahpism usually require solid ankle-foot orthosis as it substitues for weak or absent ankle plantar flexors and dorsiflexors. Use of forearm crutches should be considered as well, as these patients often display weakness of hip extensors and abductors. Patients with internal or external rotational deformity can benefit from ankle-foot orthosis with twister cables. Also knee-ankle-foot orthosis can be used in patients with excessive valgus

Skin problems are very common in patients suffering from spina bifida [50]. As the skin sensitivity is affected in children and adults born with open spina bifida they are more likely to develop various skin problems. Blisters, soles, calluses and burns on lower extremities are frequent. When found late this skin problems can

stress at the knee joint who are too young for correctional osteotomy.

**44**

The most common site of pressure sores are sacrum, ischial tuberosity, greater trochanter and feet. Severe complication of skin breakdown and pressure sores is osteomyelitis of the underlying bone. When wound does not heal with appropriate soft tissue care, underlying deep infection must be suspected and diagnosed by laboratory and radiograph findings. Antibiotic treatment is indicated, in more severe cases also surgical debridement. Last therapeutical opportunity in severe non-healing deep wounds causing sepsis is amputation with preservation as much lenght of the extremity as possible [49].

Parents and caregives need to prevent bed sores – avoid lying on one side too long, prevent sun burns and regularly control skin of back and lower extremities at least once a day. In case of new orthosis even more often. In older children it is necessary to control if the shoes fit properly. Barefoot walking should be avoided, especially on rough and hot surfaces. Self-adhesive foam pads can be used over pressure points [49].

Interventions of plastic surgeon within first year of life are mostly skin flaps and skin grafts associated with spinal closure. Need for surgery due to complications of wound healing is increasing with age.

In case of more severe bad healing wounds there is sometimes necessary to perform surgical debridement of skin wound, incision and drainage of abscess, skin flap operations, flap grafts, skin grafting, reduction of callus and revisions of skin scars [38].

#### **9.9 Sexual dysfunction**

Because of nerve damage patients born with open spina bifida defect suffer from sexual dysfunction in adolescence and adulthood. This might affect their sexual life. But most of spina bifida patients are fertile and are able to have children. Women born with open spina bifida should plan their pregnancy and are recommended to take 4 mg of folic acid a day (normal dose is 400 mcg) a month prior to conception and during early stages of pregnancy.

#### **9.10 Cardiometabolic dysfunction**

Patients with spina bifida affecting mobility have higher risk of developing components of metabolic syndrome in younger age due to low physical activity. They often suffer from abdominal obesity, insulin resistance or dyslipidemia with its metabolic and vascular complications [51]. People with physical disabilities spend less time performing physical activities compared to their nondisabled peers [52]. Consequences of a sedentary lifestyle include physical deconditioning [53]. Physical exercise can improve metabolic dysfunction [51].

Overweight, obesity, high BMI, high waist circumference and percentage of body fat are more prevalent in patients with spina bifida and they also have reduced aerobic fitness and muscle strenght [54]. Metabolic syndrome is more prevalent in spina bifida patients compared to nondisabled controls. They often display high levels of VLDL and overall cholesterol, triglycerides and low HDL, suffer from hypertension and insulin resistance [55].

Arterial diameters in these patients are reduced and sheer stress on the vascular wall is increased what predicts endothelial dysfunction [51].

#### **9.11 Treatment of other complications**

Patients with spina bifida often have post-operative complications, mainly infectious complications – wound and urinary infections, that require antibiotic therapy. According to frequent latex allergy in these patients, they should be operated and treated only in latex-free gloves in order to avoid severe anaphylactic reaction [11].

Special care must be taken to avoid pressure sores as due to lack of sensitivity and frequent hypomobility these patients are at higher risk of developing pressure sores and other wounds. All skin lesions in spina bifida have higher risk of getting infected.

Children with spina bifida often display precocious puberty and need to be examined by endocrinologist.

Due to frequent joint contractions, post-surgical immobilization and worse mobility these patients often suffer from osteoporosis and they are at higher risk of pathologic fractures. These fractures are more common in patients with higher level of neurological impairment due to more severe neurological deficit and this risk rises with age [11].

### **10. Prevention**

As the effect of folic acid intake is known for its role in decreasing incidence of neural tube defects it has a great potential in prevention. Mandatory folic acid fortification has significantly higher effect on increasing serum folate levels that just recommendation [56]. Supplementation of folic acid starting at least one month prior conception and continuing through the first trimester of gravidity reduces the risk of neural tube defects. Because of the fact that many women discover their pregnancy within second months, what is late for prevention it is recommended for women in childbearing age to have long time daily supplement of 400 mcg of folic acid. Some foods such as: enriched bread, rice, cereals and pasta are fortified with 400 mcg of folic acid per serving. Except this also consumption of foods that are naturally rich in folate is recommended, such as: milk, egg yolks, avocado, citrus fruits and juices, beans and peas and dark green vegetables, as broccoli and spinach are.

Because of only available prevention of neural tube defects, that is also cheap and accessible, planned pregnancy is the best option. It is advised for women who plan pregnancy or are likely to get pregnant to have daily intake of 400 to 800 mcg of folic acid.

Fetal absorption of folic acid from mothers intake is better than absorption of folate from food. Combined with fact that most of the people do not have recommended intake of folate from diet rationale for intake of synthetic folic acid is high. There is an evidence, that proper intake of folic acid could lead to diminishing risk of also other developmental disorders – such as cleft lip, cleft palate and also congenital cardial defects. Women who were born with neural tube defect themselves or have anamnesis of birth or pregnancy with fetus with neural tube defect should take higher doses of folic acid. The same applies for women with anti-seizure medication.

#### **11. Initial management**

Management of treatment of severe forms of spina bifida that require surgical intervention begins just after diagnose. Prenatally diagnosed cases need to be referred to tertiary unit for further management. Usually first more detailed imagings are performed, genetic examination and screening for other congenital anomalies. Mothers with fetuses with spina bifida are referred to multidisciplinary team for pregnancy follow up, choosing the therapeutical strategy and timing of treatment in consensus with decision of instructed parents [23].

#### **11.1 Delivery**

All pregnancies with fetuses with more severe form of spina bifida are followed up in tertiary centres. Birth is usually scheduled from 38th week of gestation [23].

**47**

**12. Follow up**

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

able and newborn can be immediately treated.

as evaluation of severity of spina bifida symptoms.

require shunt placement immediately.

mild to moderate ventriculomegally will be stable.

magnetic resonance imaging.

**11.2 Postpartal management**

dislocation) [11].

Babies with myelomeningocele tend to be in breech position. Breech position, cystic form of spinal defect or big sac are indications for ceasarian section. This type of delivery is also performed for its timing during the day when all specialists are avail-

Directly after the birth newborn with open spinal defect is admitted to newborn

Examination of the newborn should include identification of level of paralysis of each extremity, presence of visible signs of spinal defect (skin lesion, visible sac), deformities of extremities (such a clubfoot, hip or knee contraction or

If newborn with open spinal defect did not underwent fetal surgery, postnatal

Infants with stable slight ventricullomegaly with normal neurological status who

After stabile postsurgical course with stable slight ventriculomegally and physiological neurological status with no symptoms of active hydrocephalus and decompensated Chiari II malformation, infant can be discharged to home with indication for frequent outpatient multidisciplinary evaluations. Weekly, later biweekly evaluations by neurosurgeon are necessary with active detection of symptoms of active hydrocephalus and decompensation of Chiari malformation. In some cases growth of head circumference will stabilize and follow normal growth curve and

Criteria for later indication of ventriculo-peritoneal shunting are onset of symptoms of intracranial hypertension (irritability, headache, somnolency, troubles with feeding, vomiting, bulging fontanelle, fixed downward gaze, "sunsetting "eye movements, bradycardia and sudden progress of head circumference), radiological finding of rapidly enlarged ventricles (via ultrasonography or MR imaging) and also worsening of Chiari II symptoms (abnormal eye movements, swallowing problems, apnoea or stridor). If these symptoms are present, infant should be immediately indicated for ventriculo-peritoneal drainage [58].

Children with spina bifida, mostly open forms require very close follow up and observation for all possible problems. Most of the complications could be solved or at least properly managed to maintain adequate life quality. According to study with 50 years follow up 50% of patients born with myelomeningocele and surgically

are indicated for observation for hydrocephalus need to be routinely controlled. Clinical examination consists of evaluation of neurological status as well as regular frequent measurements of head circumference, palpation of fontanelle and cranial sutures. Also radiological controls are necessary. Best accessible is routine imaging of brain ventricles via head ultrasound and in special circumstances also use of

surgery is indicated as soon as possible – just after stabilization of vital functions within the first days after delivery [46, 57]. Usually closure of spinal defect is indicated first with subsequent monitoring of ventricle size and symptoms of intracranial hypertension or symptoms of decompensation of Chiari malformation. Newborns who present with severe symptoms of intracranial hypertension at birth

intensive care unit and strictly monitored and stabilized. Newborns need to be properly examined for presence of other congenital defects or birth trauma, as well Babies with myelomeningocele tend to be in breech position. Breech position, cystic form of spinal defect or big sac are indications for ceasarian section. This type of delivery is also performed for its timing during the day when all specialists are available and newborn can be immediately treated.

### **11.2 Postpartal management**

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

examined by endocrinologist.

**10. Prevention**

of folic acid.

**11. Initial management**

Special care must be taken to avoid pressure sores as due to lack of sensitivity and frequent hypomobility these patients are at higher risk of developing pressure sores and other wounds. All skin lesions in spina bifida have higher risk of getting infected. Children with spina bifida often display precocious puberty and need to be

Due to frequent joint contractions, post-surgical immobilization and worse mobility these patients often suffer from osteoporosis and they are at higher risk of pathologic fractures. These fractures are more common in patients with higher level of neurological impairment due to more severe neurological deficit and this risk rises with age [11].

As the effect of folic acid intake is known for its role in decreasing incidence of neural tube defects it has a great potential in prevention. Mandatory folic acid fortification has significantly higher effect on increasing serum folate levels that just recommendation [56]. Supplementation of folic acid starting at least one month prior conception and continuing through the first trimester of gravidity reduces the risk of neural tube defects. Because of the fact that many women discover their pregnancy within second months, what is late for prevention it is recommended for women in childbearing age to have long time daily supplement of 400 mcg of folic acid. Some foods such as: enriched bread, rice, cereals and pasta are fortified with 400 mcg of folic acid per serving. Except this also consumption of foods that are naturally rich in folate is recommended, such as: milk, egg yolks, avocado, citrus fruits and juices,

Because of only available prevention of neural tube defects, that is also cheap and accessible, planned pregnancy is the best option. It is advised for women who plan pregnancy or are likely to get pregnant to have daily intake of 400 to 800 mcg

Fetal absorption of folic acid from mothers intake is better than absorption of folate from food. Combined with fact that most of the people do not have recommended intake of folate from diet rationale for intake of synthetic folic acid is high. There is an evidence, that proper intake of folic acid could lead to diminishing risk of also other developmental disorders – such as cleft lip, cleft palate and also congenital cardial defects. Women who were born with neural tube defect themselves or have anamnesis of birth or pregnancy with fetus with neural tube defect should take higher doses of folic acid. The same applies for women with anti-seizure medication.

Management of treatment of severe forms of spina bifida that require surgical intervention begins just after diagnose. Prenatally diagnosed cases need to be referred to tertiary unit for further management. Usually first more detailed imagings are performed, genetic examination and screening for other congenital anomalies. Mothers with fetuses with spina bifida are referred to multidisciplinary team for pregnancy follow up, choosing the therapeutical strategy and timing of

All pregnancies with fetuses with more severe form of spina bifida are followed up in tertiary centres. Birth is usually scheduled from 38th week of gestation [23].

treatment in consensus with decision of instructed parents [23].

beans and peas and dark green vegetables, as broccoli and spinach are.

**46**

**11.1 Delivery**

Directly after the birth newborn with open spinal defect is admitted to newborn intensive care unit and strictly monitored and stabilized. Newborns need to be properly examined for presence of other congenital defects or birth trauma, as well as evaluation of severity of spina bifida symptoms.

Examination of the newborn should include identification of level of paralysis of each extremity, presence of visible signs of spinal defect (skin lesion, visible sac), deformities of extremities (such a clubfoot, hip or knee contraction or dislocation) [11].

If newborn with open spinal defect did not underwent fetal surgery, postnatal surgery is indicated as soon as possible – just after stabilization of vital functions within the first days after delivery [46, 57]. Usually closure of spinal defect is indicated first with subsequent monitoring of ventricle size and symptoms of intracranial hypertension or symptoms of decompensation of Chiari malformation. Newborns who present with severe symptoms of intracranial hypertension at birth require shunt placement immediately.

Infants with stable slight ventricullomegaly with normal neurological status who are indicated for observation for hydrocephalus need to be routinely controlled. Clinical examination consists of evaluation of neurological status as well as regular frequent measurements of head circumference, palpation of fontanelle and cranial sutures. Also radiological controls are necessary. Best accessible is routine imaging of brain ventricles via head ultrasound and in special circumstances also use of magnetic resonance imaging.

After stabile postsurgical course with stable slight ventriculomegally and physiological neurological status with no symptoms of active hydrocephalus and decompensated Chiari II malformation, infant can be discharged to home with indication for frequent outpatient multidisciplinary evaluations. Weekly, later biweekly evaluations by neurosurgeon are necessary with active detection of symptoms of active hydrocephalus and decompensation of Chiari malformation. In some cases growth of head circumference will stabilize and follow normal growth curve and mild to moderate ventriculomegally will be stable.

Criteria for later indication of ventriculo-peritoneal shunting are onset of symptoms of intracranial hypertension (irritability, headache, somnolency, troubles with feeding, vomiting, bulging fontanelle, fixed downward gaze, "sunsetting "eye movements, bradycardia and sudden progress of head circumference), radiological finding of rapidly enlarged ventricles (via ultrasonography or MR imaging) and also worsening of Chiari II symptoms (abnormal eye movements, swallowing problems, apnoea or stridor). If these symptoms are present, infant should be immediately indicated for ventriculo-peritoneal drainage [58].

## **12. Follow up**

Children with spina bifida, mostly open forms require very close follow up and observation for all possible problems. Most of the complications could be solved or at least properly managed to maintain adequate life quality. According to study with 50 years follow up 50% of patients born with myelomeningocele and surgically treated after birth were able to walk 50 m at the age of 9 years and 27% at the age of 50 years. Mobility decreases in time partially due to obesity and deterioration of general health conditions [22].

#### **12.1 General practitioner**

The role of **general practioner** – primary care doctor (pediatrician or family doctor) is very important. Doctor evaluates appropriate growth, indicates vaccination and general medical issues and coordinates medical care given by specialists. Patients and caregivers have to be properly instructed of the need of healthy life style, physical activity, precise monitoring of the skin status in order to prevent severe skin problems [59].

As most of patients with spina bifida thanks to advances in treatment reach adulthood there is a need for transition of health care to adult specialists [19]. Because the prevalence of obesity and metabolic syndrome is higher in these patients there is a need for careful monitoring of metabolic complications also in young patients [51].

Children and later also adult patients with diagnosis of open spina bifida after initial treatment need special treatment and follow up from various specialists:

#### **12.2 Developmental pediatrician**

This specialist should be part of the multidisciplinary team and survey the overall development of the child and indicate further examination of specialist when needed.

#### **12.3 Neurosurgical controls**

Child needs to be regularly controlled by neurosurgeon to evaluate proper function of ventriculoperitoneal drainage. Especially children with mild ventriculomegaly without symptoms of active hydrocephalus need regular controls so that in case symptoms develop could child immediately undergo surgery. Around half of the patients with open spinal defect undergoes other neurosurgical intervention in addition to initial spinal closure and half of these additional surgeries occur before 1 year of age – most common procedures are spinal closure, ventriculo-peritoneal shunt placement or shunt revision [38].

#### **12.4 Neurology**

All children after open neural tube defect with subsequent lasting neurological impairment need to be under supervision of experienced pediatric neurologist who controls motor functions, indexes progression of motor functions. In case of worsening of neurological functions there is a need for imaging – usually MR imaging for active screening of possible complications, such as tethered cord syndrome or decompensation of hydrocephalus, malfunction of drainage.

#### **12.5 Urological controls**

Each child after open spina bifida repair needs to be regularly evaluated by pediatric urologist. This evaluation consists of examination of kidney functions and urine derivation. Blood tests together with ultrasound and X-rays are often used, also other imaging techniques and urodynamic evaluation. Aim of this follow up is

**49**

necessary.

depression [52].

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

• Sexual health and education

• Gastroenterologist controls

treatment may prevent fixed bony deformity [49].

**12.7 Rehabilitation medicine and physical therapy**

**12.8 Psychologist and occupational therapy**

**12.6 Orthopedics and orthotics**

adult urologist [24].

skin [11].

active screening for urological complications to prevent further damage. Since the survival of spina bifida patients has changed during years and most of patients live into adulthood there is also a need for management and follow up subsequently by

The role of orthopedics is to monitor and treat deformities, follow up after early treatment, also monitor spinal balance and deformity and help in evaluation of motor function. The follow-up periodic orthopedical examination should include assessment of motor functions, sensitivity, range of motion, spinal deformity and integrity of skin. Early treatment of deformity by casting, bracing and surgical

Orthotics should periodically control the motor and mobility aids to ensure that orthoses are appropriate, in good shape and do not cause any pressure points on the

Special rehabilitations are necessary for children with open neural tube defect to increase movement, flexibility and muscle strenght. Skilled physical therapist plays an important role in the early detection of subtle muscle imbalance which could lead to severe deformity if left untreated. Therapist should perform serial manual

There is a need for close cooperation between physical therapist and parents and caregivers to teach them basics of rehabilitation to practice it at home. For older children summer camps and recreational facilities for disabled are possibility to improve their physical activity. Some patients are also able to participate successfully in sports and should do so [51]. Basic physical strategies focus on muscle strengthening exercises, orthopedic supports and assistive devices meant to aid ambulation and posture control. Lifting weights is recommended for adolescents and adults to maintain condition of muscles. As patients have limited use of lower extremities, exercise has to rely on upper extremities and trunk. There is a potential for physical therapeutist to cooperate with local sport clubs and fitness centres to develop activity programs and supervise them [51]. Physical activities offer possibility to prevent other problems, such as obesity, metabolic problems or

Spina bifida patients have to face a lot of barriers to lead conventional life. As a result of physical disability, life-style, their environment and combination of these factors these patients are at higher risk of depressive disorder [60]. Teenagers, adolescents and also adults might feel isolated from their peers and have low selfesteem. They might feel worried, stressed, anxious or sad. If the feelings last for long time they might lead to depression. Occupational therapy and psychotherapy might help, as well as physical exercise. Daily physical activity is positively correlated with quality of life [51]. In serious cases consultation of psychiatrist might be

muscle testing as a part of the routine at least annual examination [49].

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

active screening for urological complications to prevent further damage. Since the survival of spina bifida patients has changed during years and most of patients live into adulthood there is also a need for management and follow up subsequently by adult urologist [24].

• Sexual health and education

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

general health conditions [22].

**12.1 General practitioner**

severe skin problems [59].

**12.2 Developmental pediatrician**

**12.3 Neurosurgical controls**

shunt placement or shunt revision [38].

young patients [51].

specialists:

when needed.

**12.4 Neurology**

**12.5 Urological controls**

treated after birth were able to walk 50 m at the age of 9 years and 27% at the age of 50 years. Mobility decreases in time partially due to obesity and deterioration of

The role of **general practioner** – primary care doctor (pediatrician or family doctor) is very important. Doctor evaluates appropriate growth, indicates vaccination and general medical issues and coordinates medical care given by specialists. Patients and caregivers have to be properly instructed of the need of healthy life style, physical activity, precise monitoring of the skin status in order to prevent

As most of patients with spina bifida thanks to advances in treatment reach adulthood there is a need for transition of health care to adult specialists [19]. Because the prevalence of obesity and metabolic syndrome is higher in these patients there is a need for careful monitoring of metabolic complications also in

Children and later also adult patients with diagnosis of open spina bifida after initial treatment need special treatment and follow up from various

This specialist should be part of the multidisciplinary team and survey the overall development of the child and indicate further examination of specialist

Child needs to be regularly controlled by neurosurgeon to evaluate proper function of ventriculoperitoneal drainage. Especially children with mild ventriculomegaly without symptoms of active hydrocephalus need regular controls so that in case symptoms develop could child immediately undergo surgery. Around half of the patients with open spinal defect undergoes other neurosurgical intervention in addition to initial spinal closure and half of these additional surgeries occur before 1 year of age – most common procedures are spinal closure, ventriculo-peritoneal

All children after open neural tube defect with subsequent lasting neurological impairment need to be under supervision of experienced pediatric neurologist who controls motor functions, indexes progression of motor functions. In case of worsening of neurological functions there is a need for imaging – usually MR imaging for active screening of possible complications, such as tethered cord syndrome

Each child after open spina bifida repair needs to be regularly evaluated by pediatric urologist. This evaluation consists of examination of kidney functions and urine derivation. Blood tests together with ultrasound and X-rays are often used, also other imaging techniques and urodynamic evaluation. Aim of this follow up is

or decompensation of hydrocephalus, malfunction of drainage.

**48**

• Gastroenterologist controls

#### **12.6 Orthopedics and orthotics**

The role of orthopedics is to monitor and treat deformities, follow up after early treatment, also monitor spinal balance and deformity and help in evaluation of motor function. The follow-up periodic orthopedical examination should include assessment of motor functions, sensitivity, range of motion, spinal deformity and integrity of skin. Early treatment of deformity by casting, bracing and surgical treatment may prevent fixed bony deformity [49].

Orthotics should periodically control the motor and mobility aids to ensure that orthoses are appropriate, in good shape and do not cause any pressure points on the skin [11].

#### **12.7 Rehabilitation medicine and physical therapy**

Special rehabilitations are necessary for children with open neural tube defect to increase movement, flexibility and muscle strenght. Skilled physical therapist plays an important role in the early detection of subtle muscle imbalance which could lead to severe deformity if left untreated. Therapist should perform serial manual muscle testing as a part of the routine at least annual examination [49].

There is a need for close cooperation between physical therapist and parents and caregivers to teach them basics of rehabilitation to practice it at home. For older children summer camps and recreational facilities for disabled are possibility to improve their physical activity. Some patients are also able to participate successfully in sports and should do so [51]. Basic physical strategies focus on muscle strengthening exercises, orthopedic supports and assistive devices meant to aid ambulation and posture control. Lifting weights is recommended for adolescents and adults to maintain condition of muscles. As patients have limited use of lower extremities, exercise has to rely on upper extremities and trunk. There is a potential for physical therapeutist to cooperate with local sport clubs and fitness centres to develop activity programs and supervise them [51]. Physical activities offer possibility to prevent other problems, such as obesity, metabolic problems or depression [52].

#### **12.8 Psychologist and occupational therapy**

Spina bifida patients have to face a lot of barriers to lead conventional life. As a result of physical disability, life-style, their environment and combination of these factors these patients are at higher risk of depressive disorder [60]. Teenagers, adolescents and also adults might feel isolated from their peers and have low selfesteem. They might feel worried, stressed, anxious or sad. If the feelings last for long time they might lead to depression. Occupational therapy and psychotherapy might help, as well as physical exercise. Daily physical activity is positively correlated with quality of life [51]. In serious cases consultation of psychiatrist might be necessary.

#### **12.9 Neurocognitive rehabilitation**

Many of children affected by open spina bifida, mostly those with implanted shunts to treat hydrocephalus have problems with concentration, hyperactivity, work slowly.

#### **12.10 Special education teachers**

Many children born with open spina bifida do not have problems at school. Children that present with some neurocognitive problems and special educational problems such as dyslexia or dyscalculia might profit from special education. Cognitive problems are more common in children who have hydrocephalus. Children at school need individualized educational plan with assistance. This plan is a result of cooperation of parents, teachers, school psychologist, school nurse and physical education teacher.

Patients with less severe forms of spinal dysraphism and lower level of spinal defect tend to reach higher educational effect. This is partially due to better mobility of these patients but very important factor is also bowel continence degree. Patients with bowel continence or sufficient incontinence control usually reach higher educational degrees, have higher employment and insurance status and are more likely to be independent, as educational status and employment are major determinants of health insurance status in adults [19]. Urinary incontinence does not have that impact on educational status as bowel incontinence. Bladder incontinence (particularly low-volume) has usually lower impact on overall life quality, educational status and employment than bowel incontinence [24, 48].

#### **12.11 Social workers and social contact**

There is an urgent need for adequate social contact of these children. Participation in sports and physical activity with peers improves social contact and life quality. Families can also contact Intervention programs for patients with spina bifida that work in many countries. Children with spina bifida meet a lot of barriers in acces to various activities and have to rely on adults to organize and supervise activities [51].

#### **12.12 Dietitians**

Proper diet is important to avoid complications mainly according to bowel function. With a help of dietitian can parents and caregivers find the best dietetic tools for each individual child. Addition of dietary fiber can help to maintain regular stool. Enough water intake is important in prevention of obstipation and urinary infection. Proper dietary intake together with physical exercise helps to prevent overweight and obesity that could lead to metabolic syndrome and cause later metabolic and cardiovascular complications [51].

#### **13. Prognosis**

In the 1950s the survival rate of patients with most severe form of spina bifida – myelomeningocele was about 10% [61]. In the last decades, the multidisciplinary approach to care of spina bifida patients, has significantly improved patients outcomes. Recently, thanks to advances in medical and supporting treatment almost all children born with spina bifida survive into adulthood. There has been increase in

**51**

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

tissue to amniotic fluid [21].

been prescribed wheelchair early [11].

significantly less frequent [22].

back pain.

**14. Conclusion**

life expectancy and gain in the quality of life of spina bifida patients but also of their caregiver's life [43] But unfortunately, neurological outcomes of spina bifida patients did not change significantly over the years of medical progress [44]. That is mostly due to fact, that the neurological impairment is mostly caused by primary damage from congenital spinal abnormality and also secondary due to exposure of nervous

Important factor for prognosis is ability to attain early ambulation. It provides physiological and psychological benefits even if the child will later become a sitter. Also patients with high-level spina bifida who participated in walking program have lower risk of fractures, pressure sores are more independent than those who had

According to long term follow up study median survival of myelomeningocele patients was 50 years. 34% of patients had died before 5 years of age. Most common cause of death was cardiorespiratory, neurological and urological complications, then hydrocephalus and infections of central nervous system, other causes are

Most of the survivors had IQ over 80 points. Only one fifth was fully continent without need of incontinency aids. One third of patients recorded chronic

Percentage of patients living independently on their caregivers raises in time after age of 25. Independent living at the age of 50 years is more common in patients without anamnesis of intracranial hypertension and without cerebrospinal fluid shunt revisions [22]. Increase of independent living with age might be partially due

Spina bifida occulta is usually an incidental finding with no effect on life quality. On the other hand, the most severe form myelomeningocele is a complex problem. Due to many complications threatening patient with this complex health issue, there is an essential need for multidisciplinary approach to monitor, prevent and treat possible complications that have impact on functionality, life quality and survival. Parents and close caregivers have crucial role in whole mutidisciplinary team. They need to know how to manage child's situation and give child social and emotional support. Raising the child in caring environment could help to develop into young adult who is able to fight basic life issues: to care about his own health issues, going to school, working, finding and using transportation, living in their

to fact that longer survival is associated with less severe primary defect.

own and having a healthy relationship and family.

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

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

Many of children affected by open spina bifida, mostly those with implanted shunts to treat hydrocephalus have problems with concentration, hyperactivity,

Many children born with open spina bifida do not have problems at school. Children that present with some neurocognitive problems and special educational problems such as dyslexia or dyscalculia might profit from special education. Cognitive problems are more common in children who have hydrocephalus. Children at school need individualized educational plan with assistance. This plan is a result of cooperation of parents, teachers, school psychologist, school nurse and

Patients with less severe forms of spinal dysraphism and lower level of spinal defect tend to reach higher educational effect. This is partially due to better mobility of these patients but very important factor is also bowel continence degree. Patients with bowel continence or sufficient incontinence control usually reach higher educational degrees, have higher employment and insurance status and are more likely to be independent, as educational status and employment are major determinants of health insurance status in adults [19]. Urinary incontinence does not have that impact on educational status as bowel incontinence. Bladder incontinence (particularly low-volume) has usually lower impact on overall life quality, educational status

There is an urgent need for adequate social contact of these children.

Participation in sports and physical activity with peers improves social contact and life quality. Families can also contact Intervention programs for patients with spina bifida that work in many countries. Children with spina bifida meet a lot of barriers in acces to various activities and have to rely on adults to organize and supervise

Proper diet is important to avoid complications mainly according to bowel function. With a help of dietitian can parents and caregivers find the best dietetic tools for each individual child. Addition of dietary fiber can help to maintain regular stool. Enough water intake is important in prevention of obstipation and urinary infection. Proper dietary intake together with physical exercise helps to prevent overweight and obesity that could lead to metabolic syndrome and cause later

In the 1950s the survival rate of patients with most severe form of spina bifida – myelomeningocele was about 10% [61]. In the last decades, the multidisciplinary approach to care of spina bifida patients, has significantly improved patients outcomes. Recently, thanks to advances in medical and supporting treatment almost all children born with spina bifida survive into adulthood. There has been increase in

**12.9 Neurocognitive rehabilitation**

**12.10 Special education teachers**

physical education teacher.

activities [51].

**12.12 Dietitians**

**13. Prognosis**

and employment than bowel incontinence [24, 48].

metabolic and cardiovascular complications [51].

**12.11 Social workers and social contact**

work slowly.

**50**

life expectancy and gain in the quality of life of spina bifida patients but also of their caregiver's life [43] But unfortunately, neurological outcomes of spina bifida patients did not change significantly over the years of medical progress [44]. That is mostly due to fact, that the neurological impairment is mostly caused by primary damage from congenital spinal abnormality and also secondary due to exposure of nervous tissue to amniotic fluid [21].

Important factor for prognosis is ability to attain early ambulation. It provides physiological and psychological benefits even if the child will later become a sitter. Also patients with high-level spina bifida who participated in walking program have lower risk of fractures, pressure sores are more independent than those who had been prescribed wheelchair early [11].

According to long term follow up study median survival of myelomeningocele patients was 50 years. 34% of patients had died before 5 years of age. Most common cause of death was cardiorespiratory, neurological and urological complications, then hydrocephalus and infections of central nervous system, other causes are significantly less frequent [22].

Most of the survivors had IQ over 80 points. Only one fifth was fully continent without need of incontinency aids. One third of patients recorded chronic back pain.

Percentage of patients living independently on their caregivers raises in time after age of 25. Independent living at the age of 50 years is more common in patients without anamnesis of intracranial hypertension and without cerebrospinal fluid shunt revisions [22]. Increase of independent living with age might be partially due to fact that longer survival is associated with less severe primary defect.

## **14. Conclusion**

Spina bifida occulta is usually an incidental finding with no effect on life quality. On the other hand, the most severe form myelomeningocele is a complex problem. Due to many complications threatening patient with this complex health issue, there is an essential need for multidisciplinary approach to monitor, prevent and treat possible complications that have impact on functionality, life quality and survival. Parents and close caregivers have crucial role in whole mutidisciplinary team. They need to know how to manage child's situation and give child social and emotional support. Raising the child in caring environment could help to develop into young adult who is able to fight basic life issues: to care about his own health issues, going to school, working, finding and using transportation, living in their own and having a healthy relationship and family.

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