Conditions Caused by Defects in Physical Development

Initial Contact: The first indication that all is not well often occurs at the time of delivery. The atmosphere of happy anticipation suddenly changes to one laden with anxiety. Even when the mother is unable to see the infant, she may be terrifyingly aware of the heightened tension in the room, which conveys to her that something is seriously wrong. Health professionals, unprepared for this disturbing experience, find it difficult to cope with their own feelings and react with frustration and resentment toward a situation that they are powerless to change. As a result, they may forget about or retreat from the parents, who at this moment are suffering the most.

Most practitioners believe it is their responsibility to inform the parents of a congenital anomaly. At the time of delivery, unless a pediatrician or nurse practitioner is in attendance, there is a delay while the practitioner is involved with the mother’s care. During this period the mother, unable to see her child and feeling the tense atmosphere, will believe either that the child is normal but that others do not share her enthusiasm or that the child has a defect that is so terrible the professional people in the room are unable to talk about it. A nurse, the person who is most likely to be free to support the mother and who is familiar with most common congenital anomalies, can make truthful statements about the defect.

The manner in which nurses present the infant to the parents may well set the tone for the early parent-child relationship. It is probably best to explain briefly, in simple language, the nature of the defect and to reinforce and help clarify information given by the practitioner before the infant is shown to them. At this time they are more likely to “hear” what is said. Parents attach a great deal of meaning to the behavior of others during this critical period and will watch the facial expressions of others closely for signs of revulsion or rejection. Presenting the infant as something precious and emphasizing the well-formed aspects of the infant’s body provide some reassurance to parents during this crisis period.

It is important to allow time and opportunity for the parents to express their initial response to the situation. Many issues may surface, such as the importance placed on this particular infant or the cultural significance of one sex over the other. Encourage parents to ask questions and to receive honest, straightforward answers without undue optimism or pessimism.

Family Support: Parents need time to grieve for the loss of the expected child before they are able to form an emotional attachment to the child they have. It is a nursing responsibility to help parents with their grief work and to facilitate the formation of a satisfactory adjustment to the child with a defect. They need help to see their infant as a person, support in coping with their situation, and guidance in physical care of the child.

Nurses who understand the grief response will be prepared to support the parents through this necessary process. This is particularly important with the birth of a child with a defect, since the parents may not begin to invest any feeling for the child until they are able to talk about and work through their feelings of disappointment, resentment, guilt, and helplessness. The supportive nurse creates and maintains an atmosphere that encourages expression of feelings. Open expression is difficult for many people, and the parent(s) may hesitate to display intense feelings. Containing those feelings expends considerable energy that would be better used later on to develop a relationship with the infant. Nurses therefore need to listen closely for cues that indicate areas of discomfort or readiness to talk.

Parents may not be ready to talk about their feelings during the first few days after the birth. Their dream has vanished, and when others avoid them, they often interpret it as another abandonment. Staying near and available tells them that they are not alone and that someone cares about them and their feelings. What is said to them is also important. Clichés such as “You will be able to have more children” or “It could be a lot worse” are not a comfort to the parents. Such behavior implies that this infant is not important, and this behavior may destroy the parents’ trust. Parents may also attach inordinate significance to statements made by a health care worker about the prognosis of the infant; such statements may be recalled by the parent years later.

Initiating a discussion about matters that were of concern to others in a similar situation may help the parents to know that their feelings are natural. Parents need to be allowed silence and solitude if this is their wish. The parents are likely to be angry and often direct this anger at anyone nearby—physicians, nurses, friends, and families who have normal children. Directing their frustrations at a nonjudgmental target helps parents relieve some of their distress. Nurses must be prepared to accept any or all of the parental reactions and defenses—anger, hostility, rejection, dependency—without showing anger or withdrawing from the situation. If nurses make themselves available to the parents for support, they can often find nonthreatening ways to help and comfort. Most important, nurses need to promote communication and understanding within the family and help strengthen family interpersonal relationships.

Care of the Infant: Many parents are uneasy about handling their infant and require support and encouragement in their caregiving tasks. These parents need a longer period of dependency to muster their resources for coping. Although they should not feel forced to care for the infant until they are ready for the responsibility, they should be given opportunities to assume care as soon as possible to help them deal with the reality of the infant’s condition. Parents’ responses are highly individual and must be evaluated on this premise. However, all parents need sympathetic, patient, and understanding help to gain feelings of adequacy in the care of their child and to facilitate development of a positive relationship with the infant later on. As anxiety and the intensity of emotional responses decrease, parents begin to feel more comfortable with the infant and more confident in their ability to provide needed care.

Supplying Information: Parents need accurate, up-to-date information given to them early and in language they can understand. Because they do not hear everything the first time it is said, they need careful, repeated explanations about the child’s defect, the treatments outlined, and what will be expected of them. Parents often misinterpret information, another reason for repeated explanations. Often the nurse’s responsibility is to explain, interpret, and clarify and to answer questions about information that the practitioner has given. Following the basic concepts of informational needs assessment, the nurse determines what the parents know and proceeds from that point. One cannot assume that the parents’ failure to ask questions means they understand. Most parents have little or no knowledge of basic anatomy or physiology; therefore use pictures and other tangible visual aids to explain both normal and deviant structures.

Teaching the parents to provide the special care that is frequently required for an infant with a physical defect is an important nursing responsibility. Nurses need to explain and demonstrate special feeding, holding, and positioning techniques. Anticipatory guidance regarding problems that are unique to each condition reduces apprehension and stimulates the parents to institute preventive measures and to make alert observations.

Numerous agencies and organizations offer services to families of children with congenital defects. Some provide services for a variety of conditions; others are devoted to specific disorders. They help families with ongoing problems and with anticipating problems, including financial burdens, they will encounter in raising a child with a defect. Many have local support groups. All have unique and specialized services to support the family and aid parents in problem solving. Among those that include most types of defects and conditions are the Easter Seals,* the March of Dimes,† and Birth Defect Research for Children, Inc.,‡ most of which have branches in all major cities and communities. The Centers for Disease Control and Prevention, National Center on Birth Defects and Developmental Disabilities has a website with information on birth defects.§

Pain Management: During the postoperative period it is essential to assess and manage neonatal pain. This task is complicated by the variability with which neonates respond to painful stimuli and the lack of physiologic responses that may occur as a result of anesthesia. The use of muscle-paralyzing agents may further mask physiologic manifestations of pain in the postoperative period. It is often noted that the more preterm or physiologically immature the infant, the more difficult it becomes to measure pain responses, particularly when major surgery is involved. Because infants of any gestational age are capable of experiencing pain and being adversely affected by it during and after operative procedures, it is important to advocate for appropriate pharmacologic therapy to improve neonatal pain. Both pharmacologic and nonpharmacologic pain management therapies may be used in the postoperative period to effectively reduce neonatal pain. (See Pain in Neonates, Chapter 7.)

Prenatal Detection: It is possible to determine the presence of some major open NTDs prenatally. Ultrasonographic scanning of the uterus and elevated maternal concentrations of α-fetoprotein (AFP, or MS-AFP), a fetal-specific γ-1-globulin, in amniotic fluid may indicate the presence of anencephaly or myelomeningocele. (See Chapter 5.) The optimum time for performing these diagnostic tests is between 16 and 18 weeks of gestation, before AFP concentrations normally diminish and in sufficient time to permit a therapeutic abortion. It is recommended that such diagnostic procedures, as well as genetic counseling, be considered for all mothers who have borne an affected child, and testing is offered to all pregnant women (Kirkham, Harris, and Grzybowski, 2005). In addition, elective prelabor cesarean birth may result in less motor dysfunction. Chorionic villus sampling is also a method for prenatal diagnosis of NTDs; however, it carries certain risks (skeletal limb depletion) and is not recommended before 10 weeks of gestation.

Early surgical closure of the myelomeningocele sac through fetal surgery has been evaluated in relation to prevention of injury to the exposed spinal cord tissue and the improvement of neurologic and urologic outcomes in the affected child. Currently the Management of Myelomeningocele Study, a clinical trial supported by the National Institute of Health, is evaluating outcomes of fetal surgical correction of myelomeningocele at three sites in the United States; the results are expected to be published in 2011. The overall mortality rate from fetal surgery has been reported to be 4% to 6%, and complications include oligohydramnios, preterm delivery, and a smaller birth weight (Kaufman, 2004; Sutton, 2008).

Initial Care: Care of the newborn involves preventing infection; performing a neurologic assessment, including observation for associated anomalies; and dealing with the impact of the anomaly on the family. Although meningoceles are repaired early, especially if the sac is in danger of rupturing, the philosophy regarding skin closure of myelomeningocele varies. Most authorities believe that early closure, within the first 24 to 72 hours, offers the most favorable outcome. Surgical closure within the first 24 hours is recommended if the sac is leaking CSF (Kinsman and Johnston, 2007). Early closure, preferably in the first 12 to 18 hours, not only prevents local infection and trauma to the exposed tissues but also avoids stretching other nerve roots (which may occur as the meningeal sac expands during the first hours after birth), thus preventing further motor impairment. Broad-spectrum antibiotics are initiated, and neurotoxic substances such as povidone-iodine are avoided at the malformation.

A variety of neurosurgical and plastic surgical procedures are employed for skin closure without disturbing the neural elements or removing any portion of the sac. The objective is satisfactory skin coverage of the lesion and meticulous closure. Wide excision of the large membranous covering may damage functioning neural tissue.

Associated problems are assessed and managed by appropriate surgical and supportive measures. Shunt procedures provide relief from imminent or progressive hydrocephalus (see p. 409). When diagnosed, ventriculitis, meningitis, and urinary tract infection are treated with vigorous antibiotic therapy and supportive measures. Surgical intervention for Chiari II malformation is indicated only when the child is symptomatic (i.e., high-pitched crowing cry, stridor, respiratory difficulties, oral-motor difficulties, upper extremity spasticity).

Improved surgical techniques do not alter the major physical disability and deformity or chronic urinary tract infections that affect the quality of life for these children. Superimposed on these physical problems are the disorder’s effects on family life and finances and on school and hospital services.

Musculoskeletal Considerations: According to most orthopedists, musculoskeletal problems that will affect later locomotion should be evaluated early and treatment, where indicated, instituted without delay. Neurologic assessment determines the neurosegmental level of the lesion, spasticity and progressive paralysis, potential for deformity, and functional expectations. Orthopedic and musculoskeletal management includes preventing joint contractures, correcting the existing deformity, preventing or minimizing effects of motor and sensory deficits, preventing skin breakdown, and obtaining the best possible function of affected lower extremities. Common musculoskeletal problems requiring attention in SB include deformities of the knees, hips, feet, and spine; fractures and insensate skin further complicate orthopedic care. Other problems that may occur later include kyphosis and scoliosis (Lazzaretti and Pearson, 2010). Because children with this condition often have decreased sensitivity in lower extremities, preventive skin care is important. A high percentage (60%) of children seen in a wound clinic for skin breakdown had myelomeningocele at birth (Samaniego, 2003).

The status of the neurologic deficit remains the most important factor in determining the child’s ultimate functional abilities; however, many children with lumbar and sacral myelomeningocele are able to achieve functional ambulation (Kinsman and Johnston, 2007). With technologic advances, a variety of lightweight orthoses, including braces, special “walking” devices, and custom-built wheelchairs, are available to provide mobility to children with spinal cord lesions (see also Chapter 39). Early in infancy, intervention with passive range-of-motion exercises, positioning, and stretching exercises may help decrease the incidence of muscle contractures (Brown, 2001). Corrective surgical procedures, when indicated, are best initiated at an early age so that the child will not lag significantly behind age-mates in developmental progress. Where little hope exists for lower extremity function, surgery is seldom recommended unless it will improve sitting position in a wheelchair and function for activities of daily living and mobility.

Physical therapy and musculoskeletal management of children with myelomeningocele is a continual process to achieve optimum function and ambulation when possible. Problems such as type II Chiari malformation, hydrocephalus, and a tethered spinal cord can complicate expectations.

Management of Genitourinary Function: Myelomeningocele is one of the most common causes of neuropathic (neurogenic) bladder dysfunction among children. Myelomeningocele affects approximately 1 in 1000 infants born in the United States, and as many as 90% experience subsequent voiding dysfunction. In infants the goal of treatment is to preserve renal function. In older children the goal is to preserve renal function and achieve optimum urinary continence. Urinary incontinence is a chronic, often debilitating problem for the child. In addition, the neuropathic bladder may produce urinary system distress, characterized by symptomatic urinary tract infections, ureterohydronephrosis, vesicoureteral reflux, or renal insufficiency. The characteristics of bladder dysfunction in children vary according to the level of the neurologic lesion and the influence of bony growth and development of the spine. In addition, the presence of type II Chiari malformation and subsequent hydrocephalus has the potential to affect bladder function, although spinal influences predominate.

During infancy, urinary incontinence is normally physiologic, but urinary system distress may occur. Ongoing urologic monitoring is essential. Evidence is growing that early intervention, based on evaluation during the neonatal period and before complications occur, has the following benefits: (1) improves bladder function, (2) reduces the subsequent risk of urinary system distress, and (3) reduces the need for reconstructive surgery of the lower urinary tract. Ultrasonography of the bladder and ureters and routine urinalysis (and urine cultures when indicated) are used to detect urinary system distress before renal function is compromised. In addition, urodynamic testing is used to identify bladder dysfunction that predisposes the child to urinary system distress (Gray and Moore, 2009). These conditions include high pressure detrusor hyperreflexia (reflex contractions of the detrusor muscle) with vesicosphincter dyssynergia (incoordination of detrusor and sphincter muscles), low bladder wall compliance (poor distensibility of the bladder wall causing increased intravesical pressures during urine filling and storage), or detrusor areflexia (absence of detrusor contractions caused by the spinal defect).

Infants may have one of several predominant neuropathic bladder disorders. Detrusor contractions associated with vesicosphincter dyssynergia are particularly common. Some infants are able to empty the bladder efficiently despite incoordination between the sphincter mechanism and detrusor, but the majority experience chronic residual urine, urinary tract infections, or more serious types of urinary system distress. A minority of infants have poor detrusor contraction strength or detrusor areflexia. This condition is particularly damaging to the urinary system when it coexists with low bladder wall compliance and an elevated detrusor leak point pressure. Low bladder wall compliance occurs when collagen or fibrosis causes stiffening of the bladder wall. This stiffened bladder wall raises intravesical pressures, obstructing the bladder, ureters, and, ultimately, the nephron. The impact of low bladder wall compliance is directly related to the influence of the bladder outlet. Among children with myelodysplasia, the urethral muscles are typically weakened, and collagen replaces much of the muscle tissue. As a result, the sphincter is fixed, so that it neither closes efficiently to prevent urinary leakage nor opens well to allow urinary flow with a detrusor contraction. When the magnitude of the pressure required to drive urine across the abnormal sphincter is greater than 40 cm H₂O (the detrusor leak point pressure) and the compliance of the bladder wall is low (<10 cm H₂O), the risk of urinary system distress is high.

In contrast, a small number of infants experience effective detrusor contractions without vesicosphincter dyssynergia. Effective bladder evacuation is likely among this group, and the incidence of urinary system distress during the first year of life is low.

As the child grows, detrusor hyperreflexia is often replaced by deficient detrusor contraction strength and stress urinary incontinence (SUI) (leakage produced by physical exertion). The bladder wall is often poorly compliant (producing chronically elevated intravesical pressures), and the bladder outlet, while incompetent, obstructs the outflow of urine. When the detrusor leak point pressure exceeds 40 cm H₂O, the child is predisposed to chronic urinary leakage and urinary distress symptoms, including recurrent urinary tract infections and reflux. When the detrusor leak point pressure is lower than 40 cm H₂O, urinary leakage is more severe, although the risk of urinary system distress is lessened. Thus the child with more severe urinary incontinence is less predisposed than the “drier” child to serious urinary tract infections.

Infants with myelomeningocele and a neurogenic bladder who are not at risk for urinary system distress are managed by diaper containment and watchful waiting. The infant empties the bladder into a diaper, the urine is routinely monitored for infection, and the upper urinary tracts are monitored for evidence of urinary system distress (dilation of the ureters, renal pelves, or collecting systems) via serial ultrasonography.

In contrast, children with evidence of urinary system distress, or those considered at risk based on early urodynamic testing, are placed on clean intermittent catheterization (CIC), typically in combination with an antispasmodic medication such as oxybutynin or propantheline (Gray and Moore, 2009; de Jong, Chrzan, Klijn, et al, 2008). Anticholinergic medications are prescribed because they reduce detrusor muscle tone and reduce bladder pressures during both urine filling and storage and during micturition. CIC is not intended to prevent spontaneous voiding. Instead, it ensures routine, regular bladder evacuation, further preventing deleterious elevation of intravesical pressures. Usually, the parents learn to catheterize the infant every 4 hours during the day and once each night. Follow-up evaluation, consisting of serial ultrasonography and urinalysis, is completed every 3 to 6 months as indicated.

Infants with significant urinary system distress and hostile neuropathic bladder dysfunction at birth sometimes require temporary urinary diversion to ensure adequate urine outflow and prevent further damage to the upper urinary tracts. A vesicostomy is a relatively simple procedure wherein the anterior bladder wall is brought to the abdominal wall, creating a small stoma for urinary drainage. Urine is contained via a diaper, but double diapering or use of a larger diaper that can be placed higher on the abdomen is necessary for adequate urine containment. Meticulous skin care is necessary because the perineal skin is exposed to continuous urinary leakage.

Among older children the quest for continence typically begins with a CIC program. The parents learn the procedure, and teach the child to self-catheterize as soon as possible, usually by 6 years of age (Gray and Moore, 2009). The child with detrusor hyperreflexia and dyssynergia often responds well to antispasmodic medications and CIC. In contrast, the child with poor bladder wall compliance and SUI often requires a combination of antispasmodic medications to reduce intravesical filling pressures and an asympathetic agonist (such as imipramine, pseudoephedrine, or phenylpropanolamine) to enhance sphincter competence. Unfortunately, the combination of medications and CIC is typically only partially effective, and more aggressive interventions are often required to render the neuropathic bladder both continent and free from its predisposition toward producing urinary system distress.

When the child cannot attain continence by conservative measures, surgery is considered. Augmentation enterocystoplasty (or gastrocystoplasty) is a surgical procedure that increases bladder capacity, reverses or halts the negative effects of the poorly compliant bladder wall, and reduces harmfully high bladder pressures caused by detrusor hyperreflexia with vesicosphincter dyssynergia. A detubularized segment of large or small bowel or a wedge of the fundus of the stomach has been used to successfully augment bladder capacity. The choice of segment varies according to the surgeon’s preference and the status of the patient’s urinary and GI systems. Large and small bowel segments produce significant volumes of mucus that may clog catheters used for CIC. Augmentation with the stomach produces less mucus, and its acidic secretions may reduce the urinary system’s predisposition to infection. The bladder must be irrigated to decrease mucus within the bladder; this also decreases the possible complications of infection, stones, and bladder perforation.

Even though augmentation of the bladder may improve or resolve urinary leakage related to detrusor hyperreflexia or urinary system distress caused by low bladder wall compliance, the SUI produced by the abnormal sphincter mechanism typically persists. Several surgical procedures help correct this intrinsic sphincter deficiency. The Mitrofanoff procedure uses the appendix to provide an alternative route for intermittent catheterization. The appendix is removed from the colon and used to create a continent conduit between the abdominal wall and the bladder. The resulting stoma is relatively small and produces minimum mucus. The ureter may be used as an alternative to the appendix for some children. If the appendix is insufficient, a segment of tapered intestine, ileum, or colon may be used to create a conduit (Monti tube) (Gray and Moore, 2009; Mitrofanoff and Liard, 2001). CIC through the easily accessible abdominal route fosters greater independence in children, especially in those unable to transfer from wheelchair to toilet to perform CIC.

When intrinsic sphincter deficiency produces only mild stress urinary leakage, the construction of a Mitrofanoff route alone may be sufficient to achieve continence between catheterization episodes. However, when SUI is more severe, a suburethral sling or suburethral collagen injection is used to alleviate intrinsic sphincter deficiency.

The suburethral sling is a slip of fascia or synthetic material that is placed below the proximal third of the urethra. The sling may be placed in a fashion that uses only slight tension to obstruct the urethra and prevent SUI. The sling may be used for both boys and girls, and the procedure can be completed at the same time the augmentation enterocystoplasty is constructed. After augmentation enterocystoplasty and placement of a suburethral sling, the patient can expect to evacuate the bladder by CIC of the appendiceal Mitrofanoff route or the urethra if a Mitrofanoff route has not been constructed.

Suburethral injection of glutaraldehyde cross-linked (GAX) collagen also may be used to alleviate or prevent SUI caused by intrinsic sphincter deficiency. Collagen is used to bulk or expand the urethral tissue, promoting coaptation (approximation) of the mucosa. The collagen implant complements the urethra’s ability to form a watertight seal, rather than obstructing the urethral lumen. Collagen may be injected using different approaches. Transurethral collagen is injected through the working channel of a cystoscope. Transperineal collagen is directed underneath the urethra using a needle inserted through the perineal skin. In this case the location of the urethra is confirmed by simultaneous cystoscopic visualization of the urethra. The antegrade approach requires creation of a suprapubic cystostomy tract. A flexible cystoscope is then inserted through the cystostomy tract, and collagen is injected into the proximal urethra. Multiple injections may be required to achieve optimum continence. Subsequent injections may be required when the collagen is dissipated or resorbed by the body over a period of years.

The artificial urinary sphincter provides another alternative for the management of intrinsic sphincter deficiency in the child with myelomeningocele. The device consists of a urethral cuff, abdominal reservoir, and control pump. In the activated position, the cuff is filled, and the pressure of this cuff closes the urethral lumen. During micturition, the control pump is used to baffle fluid from the urethral cuff to the abdominal reservoir, opening the urethra for micturition or catheterization. However, because of the significant risk for infection, need for revision with growth, and mechanical failure, the popularity of the artificial urinary sphincter has declined.

Because of advances in neurogenic bladder management, adolescents and young adults with myelomeningocele and neurogenic bladders have been followed for up to 30 years without evidence of deterioration in renal function. Nevertheless, urinary and fecal incontinence are common, and these conditions lead to significant, and sometimes devastating, problems with growth and developmental tasks, including establishing independence and social and intimate relationships. This observation underscores the need to aggressively manage both continence and the threat of urinary system distress from an early age and to establish an expectation of social continence critical to providing these patients with the skills they need to thrive as adolescents and adults. Newborns with SB and normal urodynamics require close follow-up care during the first several years of life to prevent deterioration in urodynamic status as a result of neurologic deterioration.

Bowel Control: Some degree of fecal continence can be achieved in most children with myelomeningocele with diet modification, regular toilet habits, and prevention of constipation and impaction. It is frequently a lengthy process. Dietary fiber supplements (recommended 10 g/day), laxatives, suppositories, or enemas aid in producing regular evacuation. Older children and adolescents seeking more independence may attain bowel continence and higher quality of life after undergoing an antegrade continence enema procedure (Doolin, 2006). In a procedure similar to the Mitrofanoff, the appendix or ileum is used to create a catheterizable channel with attachment of the proximal end to the colon. The distal end of the channel exits through a small abdominal stoma. Every 1 or 2 days, a catheter is passed through the stoma, allowing enema solution to be instilled directly into the colon; this is called an antegrade colonic irrigation. After administration of the enema solution, the child sits on the toilet for 30 to 60 minutes as stool is flushed out through the rectum. Frequency of enemas and volume of solution used to completely evacuate the bowel vary among individuals.

Prognosis: The early prognosis for the child with myelomeningocele depends on the neurologic deficit present at birth, including motor ability, bladder innervation, and associated neurologic anomalies. Early surgical repair of the spinal defect, antibiotic therapy to reduce the incidence of meningitis and ventriculitis, prevention of urinary system dysfunction, and early detection and correction of hydrocephalus have significantly increased the survival rate and quality of life in such children. Many children with SB achieve partial independent living and gainful employment. Reports of survival rates vary, and many include adults who were born before medical advances and surgical techniques seen in the past 25 years. Coordinated care for adults with SB is essential; however, multidisciplinary adult care is often inadequate (Lazzaretti and Pearson, 2010). In children and adolescents with SB the achievement of urinary continence is associated with improved self-concept and esteem, especially among girls (Moore, Kogan, and Parekh, 2004). This chronic condition has an array of associated complications, including hydrocephalus and shunt malfunctions, scoliosis, bowel and bladder management issues, latex allergy, and epilepsy. However, based on current medical knowledge and ethical considerations, aggressive, early management is favored for the child with myelomeningocele.

Prevention: The Centers for Disease Control and Prevention (2009) continues to affirm that 50% to 70% of NTDs can be prevented by daily consumption of 0.4 mg of folic acid among women of childbearing age. The data indicate that serum folate concentrations among women of childbearing age decreased 16% from 2003 to 2004 in all ethnic groups studied. Lowest serum folate levels were seen in non-Hispanic Caucasians in 2003 to 2004; however, overall serum folate levels remained below recommended levels in non-Hispanic African-Americans during all three periods studied (Centers for Disease Control and Prevention, 2007). These results indicate that nurses and other health care workers have an important task in disseminating information that may decrease the incidence of birth defects in children by promoting maternal consumption of folic acid.*

To ensure adequate daily intake of the recommended amount of folic acid, women must take a folic acid supplement, eat a fortified breakfast cereal containing 100% of the recommended dietary allowance of folic acid (e.g., Kellogg’s Product 19, General Mills Total, Multigrain Cheerios Plus), or increase their consumption of fortified foods (cereal, bread, rice, grits, pasta) and foods naturally rich in folate (green, leafy vegetables and citrus fruits). For women who have had a previous pregnancy affected by NTDs, folic acid intake is increased to 4 mg under supervision of a practitioner beginning 1 month before a planned pregnancy and continuing through the first trimester. Supplementation of 4 mg of folate should not be given solely in multivitamin preparations because of the risk of overdose of other vitamins. The only population in which folic acid has not been effective in decreasing the incidence of NTDs is in women taking antiepileptic medications during pregnancy (Finnell, Gould, and Spiegelstein, 2003).

Care of the Myelomeningocele Sac: The infant is usually placed in an incubator or radiant warmer so that temperature can be maintained without clothing or covers that might irritate the CNS lesion. When an overhead warmer is used, the dressings over the defect require more frequent moistening because of the dehydrating effect of the radiant heat. Before surgical closure the myelomeningocele is kept from drying by the application of a sterile, moist, nonadherent dressing. The moistening solution is usually sterile normal saline. Dressings are changed frequently (every 2 to 4 hours), and the sac is closely inspected for leaks, abrasions, irritation, and signs of infection. The sac must be carefully cleansed if it becomes soiled or contaminated. Sometimes the sac ruptures during delivery or transport, and any opening in the sac greatly increases the risk of infection to the CNS.

Positioning: One of the most important and challenging aspects of early care of the infant with myelomeningocele is positioning. Before surgery the infant remains in the prone position to minimize tension on the sac and the risk of trauma. The prone position allows for optimum positioning of the legs, especially in cases of associated hip dysplasia. A variety of aids, including diaper rolls, pads, or specially designed frames and appliances, are available to maintain the desired position.

The prone position affects other aspects of the infant’s care. For example, in this position the infant is more difficult to keep clean, pressure areas are a constant threat, and feeding becomes a problem. The infant’s head is turned to one side for feeding. Fortunately, most defects are repaired early, and the infant can be held for feeding soon after surgery. Physical therapy consultation may be necessary for difficult positioning problems. Speech-language pathologist consultation may be needed for difficulty with oral-motor skills that may indicate complications caused by a Chiari malformation.

General Care: Diapering the infant may be contraindicated until the defect has been repaired and healing is well advanced or epithelialization has taken place. The padding beneath the diaper area is changed as needed to keep the skin dry and free of irritation. When the nurse detects urinary retention (the bladder is still an abdominal organ in early infancy), CIC is employed. Because the bowel sphincter is frequently affected, there may be continual passage of stool, often misinterpreted as diarrhea, which is a constant irritant to the skin and a source of infection to the spinal lesion.

Areas of sensory and motor impairment are subject to skin breakdown and therefore require meticulous care. The infant may be placed on a pressure-reducing mattress or mattress to prevent pressure on the knees and ankles. (See Skin Care, Chapter 10, and Maintaining Healthy Skin, Chapter 27.)

Gentle range-of-motion exercises are carried out to prevent contractures, and stretching of contractures is performed when indicated. However, these exercises may be restricted to the foot, ankle, and knee joint. When the hip joints are unstable, stretching against tight hip flexors or adductor muscles, which act much like bowstrings, may aggravate a tendency toward subluxation. A physical therapy consultation is often necessary to develop a multidisciplinary plan to prevent long-term complications.

Some infants with unrepaired myelomeningocele are unable to be held in the arms and cuddled as unaffected infants are, so their need for tactile stimulation is met by caressing, stroking, and other comfort measures. To facilitate handling and reduce parental anxiety, the infant can recline on a pillow placed in the parent’s lap. Black-and-white drawings or geometric shapes can be placed within the infant’s view, and other stimulation usually provided for infants is appropriate. All infants respond to pleasant sounds. (See Developmental Outcome, Chapter 10.)

Ophthalmic complications may occur in children with SB and hydrocephalus. The appearance of a squint, other ocular motility, or papilledema usually denotes hydrocephalus and is reported. Ophthalmologic follow-up care, particularly in children with shunts, is generally included in the multidisciplinary care plan.

Postoperative Care: Postoperative care for the infant with myelomeningocele involves the same basic care as for any postsurgical infant: monitoring vital signs, weight, and intake and output; maintaining body temperature; assessing and relieving pain; providing nourishment; and observing for signs of infection. The wound is managed according to the surgeon’s directions, and general care is continued as preoperatively.

The prone position is maintained after operative closure, although many neurosurgeons allow a side-lying or partial side-lying position unless it aggravates a coexisting hip dysplasia or permits undesirable hip flexion. This offers an opportunity for position changes, which reduces the risk of pressure sores and facilitates feeding. Once the effects of anesthesia have subsided and the infant is alert, feedings may resume unless there are other anomalies or associated complications.

Nursing assessments are carried out for implementation of comfort measures in the postoperative period. The infant can be held upright against the body, taking care to avoid pressure on the operative site. In the case of an unusually large defect, skin grafting may be required for wound closure; the infant must then be kept prone postoperatively with as little movement as possible to prevent tension on the skin graft.

The nurse can assist in determining the extent of neuromuscular involvement. Note movement of the extremities or skin response, especially an anal reflex, that might provide clues to the degree of motor or sensory status. Measure head circumference daily (see Chapter 6), and examine the fontanels for signs of tension or bulging. The nurse is also alert to early signs of infection, such as elevated or decreased temperature (axillary), irritability, and lethargy, and to signs of increased intracranial pressure (ICP). Urinary catheterization may be needed for urine retention. Although it may not have been a problem preoperatively, swelling around the operative site may cause transient urine retention, which resolves in 2 to 5 days.

Family Support and Home Care: As soon as the parents are able to cope with the infant’s condition, encourage them to become involved in care. They need to learn how to continue at home the care that has been initiated in the hospital: positioning, feeding, skin care, and range-of-motion exercises when appropriate. Parents also need to learn CIC technique when prescribed. The family needs to know the signs of complications and how to reach assistance when needed.

As the child grows and develops, parents need guidance to encourage and stimulate the infant to accomplish age-appropriate developmental tasks within the limits imposed by the disabilities. Upper limb movement can be stimulated early by placing the infant on the floor in a prone position with toys within reach. Activities that encourage body consciousness, such as rolling over and pulling to a sitting position, are encouraged at the appropriate times. Creeping and crawling help the child explore the environment. The parents may need help to modify appliances and activities normally expected of a growing child. A standing table, frame, or parapodium is helpful for a variety of activities, and it is best for the child to begin supported weight bearing and standing as close as possible to the expected time for standing to occur.

It is important for the family to understand the nature of sensory deficit in a child with a spinal defect. The child will be insensitive to pressure or other sources of tissue injury. Therefore the family must be alert to hot or cold items that could cause thermal injury to tissues and remember to inspect the skin regularly for signs of pressure, especially over bony prominences. Because of sensory impairment, the child is unaware of bladder discomfort. Therefore signs of urinary tract infections may go unnoticed. Urinary tract infection is often considered when the child becomes ill.

The long-range planning with and support of the parents and newborn begin in the hospital and extend throughout childhood and even into young adulthood. The life expectancy of children with SB extends well into adulthood; therefore planning should involve long-term goals and plans for optimum function as an adult. Long-range planning goals should include a discussion of achievement of functional mobility, urinary continence, and as much bowel continence as much physically possible. Discussion about aspects of adulthood such as having a mate, sexual relationships, and bearing and rearing children is important and should not be overlooked (Barker, Saulino, and Caristo, 2002; Rowe and Jadhav, 2008). The unique service needs of adolescents with SB as they attempt to gain independence from family and establish a life of their own has not been adequately addressed in the literature yet is slowly emerging (Buran, McDaniel, and Brei, 2002; Rowe and Jadhav, 2008). Advances in neurology, orthopedics, and urology have enabled adolescents to progress into adulthood with fewer deficits than observed in previous decades; one key factor is the recognition of subtle signs of neurologic deterioration and rapid intervention (Rowe and Jadhav, 2008).

Nurses assume an important role as a central member of the health team. As a coordinator, the nurse reviews information with the family, takes responsibility for family teaching, and acts as a liaison between inpatient and outpatient services. The child may require numerous hospitalizations over the years, and each one will be a source of stress to which the younger child is especially vulnerable.

Changes in functional ability, particularly in the lower extremities, bowel, or bladder, may indicate the presence of a tethered cord, one that is bound down or restricted in an abnormal position by scar tissue. These symptoms usually occur after a growth spurt and can best be detected with MRI. Tethering can be repaired surgically but, unfortunately, may recur. Hydromyelia, a dilation of the central canal of the spinal cord and elevated fluid accumulation, may occur with SB; common symptoms include rapidly developing scoliosis, upper extremity weakness, spasticity, and lower extremity ascending motor strength changes (Barker, Saulino, and Caristo, 2002).

Habilitation involves solving not only problems of self-help and locomotion but also the most distressing problem of incontinence, which threatens the child’s social acceptability. Assistance in preparing the child and the school regarding the special needs of children with disabilities helps the parents provide a better initial adjustment to broader social experiences. Numerous organizations and agencies offer assistance and support to children and families (see Family-Centered Care box). The Spina Bifida Association* provides services and support for families of children with spinal lesions.

The multiple aspects of care of the child with a disability are discussed in Chapter 22. Complex problems associated with partial or complete lower extremity paralysis are discussed in Chapters 39 and 40 and include bowel and bladder control; orthopedic appliances; and the observation and management of complications, especially urinary tract infections (see Chapter 30) and pressure necrosis (see Wounds, Chapter 18).

Ventricular Circulation: The fluid flows from the lateral ventricles through the foramen of Monro to the third ventricle, where it combines with fluid secreted into the third ventricle. From there CSF flows through the aqueduct of Sylvius into the fourth ventricle, where more fluid is formed; it then leaves the fourth ventricle by way of the lateral foramen of Luschka and the midline foramen of Magendie and flows into the cisterna magna. From there CSF flows to the cerebral and cerebellar subarachnoid spaces, where it is absorbed. A large portion is absorbed through the arachnoid villi, but the sinuses, veins, brain substance, and dura also participate in absorption.

Mechanisms of CSF Fluid Imbalance: The causes of hydrocephalus vary, but the result is either (1) impaired absorption of CSF fluid within the subarachnoid space, obliteration of the subarachnoid cisterns, or malfunction of the arachnoid villi (nonobstructive or communicating hydrocephalus); or (2) obstruction to the flow of CSF through the ventricular system (obstructive or noncommunicating hydrocephalus) (Kinsman and Johnston, 2007). The terms communicating and noncommunicating hydrocephalus traditionally referred to obstructive and nonobstructive types of hydrocephalus when pneumoencephalography was used to establish the diagnosis. Because other diagnostic methods are now used, the terms may be used only as a reference point in the diagnosis. Other authorities suggest that hydrocephalus be classified according to the cause as either congenital or acquired hydrocephalus (Rudy, 2005). Rarely, a tumor of the choroid plexus causes increased CSF secretion. Any imbalance of secretion and absorption causes an increased accumulation of CSF in the ventricles, which become dilated (ventriculomegaly) and compress the brain substance against the surrounding rigid bony cranium. When this occurs before fusion of the cranial sutures, it causes enlargement of the skull, as well as dilation of the ventricles. In children under 10 to 12 years of age, previously closed suture lines, especially the sagittal suture, may become diastatic, or opened. The cranial sutures do not permanently fuse until approximately the age of 12 years or later.

Most cases of hydrocephalus are a result of developmental malformations. Although the defect usually is apparent in early infancy, it may become evident at any time from the prenatal period to late childhood or early adulthood. Other causes include neoplasms, CNS infections (e.g., meningitis, encephalitis), and trauma (e.g., shaken baby syndrome). An obstruction to the normal flow can occur at any point in the CSF pathway to produce increased pressure and dilation of the pathways proximal to the site of obstruction. Table 11-3 describes the most frequent sites of obstruction and the consequences.

Developmental defects (e.g., Chiari malformation [see following discussion], aqueduct stenosis, aqueduct gliosis, and atresia of the foramina of Luschka and Magendie [Dandy-Walker malformation]) account for most cases of hydrocephalus from birth to 2 years of age. Dandy-Walker malformation involves a cystic expansion of the fourth ventricle and subsequent obstruction of CSF flow resulting in hydrocephalus (Kinsman and Johnston, 2007). Hydrocephalus is so often associated with myelomeningocele that all such infants should be observed for its development. In the remainder of cases there is a history of intrauterine infection (toxoplasmosis, cytomegalovirus), hemorrhage (posthemorrhagic hydrocephalus in preterm infant), and neonatal meningoencephalitis (bacterial or viral). In older children hydrocephalus is most often a result of intracranial masses (vascular anomalies, cysts, tumors), preexisting developmental defects, intracranial infections, trauma, or hemorrhage.

Chiari Malformations: A Chiari malformation is a brain defect involving posterior fossa contents. Table 11-3 describes the major types. The type II Chiari malformation, seen almost exclusively with myelomeningocele, is characterized by herniation of a small cerebellum, medulla, pons, and fourth ventricle into the cervical spinal canal through an enlarged foramen magnum. The resulting obstruction of CSF flow causes the hydrocephalus.

Infancy: In infants with hydrocephalus, the head grows at an abnormal rate, although the first signs may be bulging fontanels with or without head enlargement (Fig. 11-5). The anterior fontanel is tense, often bulging, and nonpulsatile. Scalp veins are dilated and markedly so when the infant cries. With the increase in intracranial volume, the bones of the skull become thin and the sutures become palpably separated to produce the cracked-pot sound (Macewen sign) on percussion of the skull. In severe cases there may be frontal protrusion, or frontal bossing, with depressed eyes, and the eyes may be rotated downward, producing a setting-sun sign, in which the sclera may be visible above the iris. Pupils are sluggish, with unequal response to light.

The infant is irritable and lethargic, feeds poorly, and may display changes in level of consciousness, opisthotonos (often extreme), and lower extremity spasticity. The infant cries when picked up or rocked and quiets when allowed to lie still. Early infantile reflexes may persist, and normally expected responses may not appear, indicating failure in the development of normal cortical inhibition.

Infants with Chiari malformation may exhibit behaviors that reflect cranial nerve dysfunction as a result of brainstem compression, including swallowing difficulties, stridor, apnea, aspiration, respiratory difficulties, and arm weakness.

The preterm infant with posthemorrhagic hydrocephalus may not exhibit any clinical signs and symptoms other than a gradual increase in head circumference. Alternatively, the nurse may note subtle seizure activity and alternating levels of consciousness. Assess ventricular size by ultrasonography or CT scanning in preterm infants at high risk for intraventricular hemorrhage.

If hydrocephalus is allowed to progress, development of lower brainstem functions is disrupted, as manifested by difficulty in sucking and feeding and a shrill, brief, high-pitched cry. Eventually the skull becomes enlarged, and the cortex is destroyed. If the hydrocephalus is rapidly progressive, the infant may display emesis, somnolence, seizures, and cardiopulmonary distress.

Childhood: The signs and symptoms in early to late childhood are caused by increased ICP, and specific manifestations are related to the focal lesion. Most commonly resulting from posterior fossa neoplasms and aqueduct stenosis, the clinical manifestations are primarily those associated with space-occupying lesions (i.e., headache on awakening with improvement following emesis or upright posture, papilledema, strabismus, and extrapyramidal tract signs such as ataxia [see Chapter 36]). As with infants, the child is irritable, lethargic, apathetic, confused, and often incoherent. In one of the congenital defects with later onset (by age 3 months), the Dandy-Walker syndrome, characteristic manifestations are a bulging occiput, nystagmus, ataxia, and cranial nerve palsies.

Manifestations of Chiari malformation in children over 3 years of age are related to spinal cord dysfunction rather than brainstem compression as observed in infants. Scoliosis proximal to the level of the myelomeningocele (usually associated with Chiari malformation) and development of upper extremity spasticity, which may progress to weakness and atrophy, are common. Cranial nerve deficits are rare.

Surgical Treatment: Improved neurosurgical techniques have established surgical treatment as the therapy of choice in almost all cases of hydrocephalus. This is accomplished by direct removal of an obstruction, such as resection of a neoplasm, cyst, or hematoma, or, in rare instances of fluid overproduction, by choroid plexus extirpation (plexectomy or electric coagulation). However, most children require a shunt procedure that provides primary drainage of the CSF from the ventricles to an extracranial compartment, usually the peritoneum.

Most shunt systems consist of a ventricular catheter, a flush pump, a unidirectional flow valve, and a distal catheter. All are radiopaque for easy visualization after placement, and all are tested for accuracy before insertion. A reservoir is frequently added to allow direct access to the ventricular system for administration of medications and removal of fluid. In all models the valves are designed to open at a predetermined intraventricular pressure and close when the pressure falls below that level, thus preventing backflow of fluid. Most shunts now in use have differential pressure and adjustable programmable valves with capability for changing the pressures with an external magnet, thus avoiding additional surgery (Chiafery, 2006; Kestle, 2003).

The standard procedure for many years has been the ventriculoperitoneal (VP) shunt, especially in neonates and young infants (Fig. 11-7). There is greater allowance for excess tubing, which minimizes the number of revisions needed as the child grows. Since it requires repeated lengthening, the ventriculoatrial (VA) shunt (ventricle to right atrium) is reserved for older children who have attained most of their somatic growth and children with abdominal pathologic conditions. The VA shunt is contraindicated in children with cardiopulmonary disease or elevated CSF protein.

Animation—Ventriculoperitoneal Shunt

Although placement of ventricular shunts (into the amniotic sac) in utero for ventricular enlargement is possible, results have not been as promising as shunting soon after birth (Golden and Bönnemann, 2007).

The initial shunt is placed when indicated on the basis of individual assessment. The timing of revisions varies widely. In most instances revisions are performed when physical signs indicate shunt malfunction (i.e., signs of elevated ICP). Sometimes revisions are planned for specific times during development. The initial success rate is relatively high. However, shunts are associated with complications that interfere with continued shunt function or that threaten the child’s life.

Endoscopic third ventriculostomy (ETV) is a procedure that has potential for allowing greater independence from VA or VP shunting in children with hydrocephalus. In this procedure a small opening is made in the floor of the third ventricle, allowing CSF to flow freely through the previously blocked ventricle, thus bypassing the aqueduct of Sylvius. Children with SB, anatomic ventricular malformations, posthemorrhagic hydrocephalus (preterm infants), and postinfectious hydrocephalus are reportedly poor candidates for this procedure (Drake, 2008), as are children with bleeding disorders and those who have had previous radiotherapy. Reports of the success of ETV in children vary (see Research Focus box). Complications include CSF leak, hemorrhage from a perforated basilar artery, meningitis, cranial nerve injury, obstruction, and hypothalamic injury (Drake, 2008).

Complications: The major complications of VP shunts are infection and malfunction. All shunts are subject to mechanical difficulties, such as kinking, plugging, or separation and migration of tubing. Malfunction is most often caused by mechanical obstruction either within the ventricles from particulate matter (tissue or exudate) or at the distal end from thrombosis or displacement as a result of growth. Functional obstruction of a shunt’s antisiphon device remains a common complication. Shunt malfunctions are reported to be 40% at 1 year and 50% at 2 years (Drake, 2008; Kestle, 2003). In a large study occurring over 13 years, at least one shunt revision was required in 58.5% of children; the median time to shunt failure or malfunction was 88 days (Mittler, 2005). The child with a shunt obstruction often is seen in an emergency visit with clinical manifestations of increased ICP, frequently accompanied by worsening neurologic status.

The most serious complication, shunt infection, can occur at any time, but the period of greatest risk is 1 to 2 months after placement. Shunt infection rates are reported to be approximately 10% (Drake, 2008). The infection may be a result of intercurrent infections at the time of shunt placement. Infections include sepsis, bacterial endocarditis, wound infection, shunt nephritis, meningitis, and ventriculitis. Brain abscess associated with colonic perforation and infection with a gram-negative enteric organism suggests an ascending shunt infection in a child who has a VP shunt. Meningitis and ventriculitis are of greatest concern because any complicating CNS infection is a significant predictor of subnormal intellectual outcome. Infection is treated with antibiotics administered intravenously or intrathecally for a minimum of 7 to 10 days. Antibiotic-impregnated shunts may decrease infection rates (Drake, 2008). A persistent infection may require removal of the shunt until the infection is controlled; however, this practice has been recently challenged (Drake, 2008). External ventricular drainage (EVD), or external ventriculostomy, is used until CSF is sterile. EVD allows removal of CSF from a tube placed in the child’s ventricle that flows by gravity into a collection device.

The primary reasons for inserting an EVD include unstable status, increased ICP that is difficult to stabilize, or infection from an existing VP shunt. The EVD may drain CSF intermittently or continuously according to need. The EVD is a closed system made up of transparent pliable tubing, a collection bag, and, at times, a drip chamber between the tubing and the collection bag. The EVD is placed at the level of the child’s external auditory meatus with the head at a 20- to 30-degree elevation, depending on physician preference. Elevating the EVD above this level decreases the flow of CSF, and placing the device below the level of the external meatus increases the flow. Ambulation or sitting up in bed or chair usually requires that the tubing be clamped to prevent imbalance in CSF drainage. In addition, the EVD is a closed sterile system and should be handled as such in relation to emptying the device or changing the scalp dressing. Accurate and frequent documentation of the incision site; amount, color, and consistency of drainage into the device; and the child’s vital and neurologic signs are an important part of the nursing care.

Complications related to an EVD include infection, meningitis, hemorrhage, obstruction, malfunction (Ngo, Ranger, Singh, et al, 2009), and, in some cases, tentorial herniation as a result of imbalance in CSF drainage (Pope, 1998). In preterm infants with intraventricular hemorrhage requiring CSF drainage, a ventricular access drain may be temporarily inserted. This system is similar to the EVD but has an access port for frequent taps and can be used to administer antibiotics and thrombolytic agents such as urokinase.

Another serious shunt-related complication is subdural hematoma caused by too rapid reduction of ICP and size. This usually can be averted by careful assessment of ICP before insertion of the shunt and use of correct valvular pressure. Other complications that may occur include peritonitis, abdominal abscesses, perforation of abdominal organs by catheter or trocar (at the time of insertion), fistulas, hernias, and ileus. Children often need shunt lengthening as body growth occurs. This procedure usually involves replacing the distal catheter below the valve during the toddler period and again before the growth acceleration of puberty.

Prognosis: The prognosis of children with treated hydrocephalus depends largely on the cause of the dilated ventricles before shunt placement and the amount of irreversible brain damage before shunting (Kinsman and Johnston, 2007). For example, malignant tumors have a high mortality rate regardless of other complicating factors.

Survivors have a high incidence of subnormal intellectual capacity, and a large majority have major physical and disabling neurologic handicaps such as ataxia, spastic diplegia, poor fine motor coordination, and perceptual deficits. Some children demonstrate aggressive or delinquent behavior, and those with myelomeningocele are likely to have accelerated pubertal development as a result of increased gonadotropin secretion with increased ICP (Kinsman and Johnston, 2007). Depression requiring treatment occurred in 45% of children in one survey; in the same survey 54% of the children with hydrocephalus required four or more shunt revisions (Gupta, Park, Solomon, et al, 2007).

Surgically treated hydrocephalus in patients with little or no evidence of irreversible brain damage has a survival rate of about 90%, with most deaths occurring within the first year of treatment. Those with poor outcomes included children shunted for posthemorrhagic hydrocephalus or meningitis, with 40% and 30%, respectively, showing cognitive impairment (Kestle, 2003). Of the surviving children, approximately two thirds are intellectually normal. The presence of additional medical problems in infancy, including ocular defects, is the most significant variable associated with a high likelihood of neurocognitive impairment. Most children who require shunting must depend on the shunt for the remainder of their life.

Postoperative Care: In addition to routine postoperative care and observation, the infant or child is positioned carefully on the unoperated side to prevent pressure on the shunt valve. The child remains flat to help avert complications resulting from too rapid reduction of intracranial fluid. The surgeon indicates the position to be maintained and the extent of activity allowed.

The nurse continues observation for signs of increased ICP, which indicate obstruction of the shunt. Neurologic assessment includes pupil dilation (pressure causes compression or stretching of the oculomotor nerve, producing dilation on the same side as the pressure) and blood pressure (hypoxia to the brainstem causes variability in these vital signs). The nurse also observes for abdominal distention because CSF may cause peritonitis or a postoperative ileus as a complication of distal catheter placement.

Because infection is the greatest hazard of the postoperative period, nurses are continually on the alert for the usual manifestations of CSF infection, including elevated temperature, poor feeding, vomiting, decreased responsiveness, and seizure activity. There may be signs of local inflammation at the operative sites and along the shunt tract. Antibiotics are administered by the IV route as ordered, and the nurse may also need to assist with intraventricular instillation. Inspect the incision site for leakage, and test any suspected drainage for glucose, an indication of CSF.

Family Support: Specific needs and concerns of parents during periods of hospitalization are related to the reason for the child’s hospitalization (shunt revision, infection, diagnosis) and the diagnostic and surgical procedures to which the child must be subjected. Parents may have little understanding of anatomy; therefore they need further explanation and reinforcement of information that was given to them by the physician and neurosurgeon, including information about what to expect. They are especially frightened of any procedure that involves the brain, and the fear of disability or brain damage is real and pervasive. Nurses can calm their anxiety with explanations of the rationale underlying the various nursing and medical activities such as positioning or testing and by simply being available and willing to listen to their concerns.

To prepare for the child’s discharge and home care, instruct the parents on how to recognize signs that indicate shunt malfunction or infection. Active children may have injuries, such as a fall, that can damage the shunt, and the tubing may pull out of the distal insertion site or become disconnected during normal growth. Contact sports should be avoided, but swimming or tennis is appropriate. A helmet may be worn to protect the head in case of a fall or injury when outside play is vigorous (Chiafery, 2006). It is also important for the nurse to encourage families to enroll infants and toddlers with hydrocephalus into an early childhood development program. Depending on the degree of initial damage and the underlying cause, many children have normal intellectual development.

The management of hydrocephalus in a child is a demanding task for both the family and health professionals, and helping the family cope with the child’s difficulties is an important nursing responsibility. Children with hydrocephalus have lifelong special health care needs. The nurse can provide optimum primary health care, including advice on immunizations, treatments for common infectious conditions, or child care and school precautions. The overall aim is to establish realistic goals and an appropriate educational program that will assist the child in achieving the maximum potential. Families can be referred to community agencies for support and guidance. The National Hydrocephalus Foundation (NHF)* and the Hydrocephalus Association† provide information on the condition for families, and the NHF assists interested groups in establishing local organizations.

Anticipatory guidance will prepare parents for possible problems and help them avoid being overprotective of the child. Few restrictions need be placed on the child’s activities (mainly contact sports), and the child is encouraged to live as would any other youngster of the same age and abilities. Parents need support and encouragement in coping with the child and problems the child may encounter in relationships with peers and others. Reactions of other children when the child has a noticeably enlarged head or requires shaving at times of revision are stressful for both the child and the parents. (See Chapter 22 for problems and coping with a child with a disability.)

Newborn to 6 Months: The hip joint is maintained by dynamic splinting in a safe position with the proximal femur centered in the acetabulum in an attitude of flexion. A variety of abduction devices are available for maintaining the femur in the acetabulum. Of these, the Pavlik harness is the most widely used device, and with time, motion, and gravity the hip works into a more abducted, reduced position (Fig. 11-12). The rate of reduction is about 95% effective when a Pavlik harness is used on a full-time basis for 6 weeks (Hosalkar, Horn, Friedman, et al, 2007). The harness is a dynamic splint that is worn continuously until the hip is clinically and radiographically stable, usually about 3 to 5 months. It is highly effective when the device is well constructed, follow-up care is adequate, and the parents follow instructions in its use. The Pavlik harness does not rigidly immobilize the hip but acts to prevent hip extension or adduction. Because of the infant’s rapid growth rate, the straps should be checked every 1 to 2 weeks for adjustments. Improper positioning may cause vascular or nerve damage.

When adduction contracture is present, other devices (such as skin traction) are employed to slowly and gently stretch the hip to full abduction, after which wide abduction is maintained until stability is attained. When maintaining stable reduction is difficult, a hip spica cast is applied and changed periodically to accommodate the child’s growth. After 3 to 6 months, sufficient stability is acquired to allow transfer to a removable protective abduction brace. The duration of treatment depends on development of the acetabulum but usually lasts less than a year.

Six to 18 Months: In this age-group the dislocation may not be recognized until the child begins to stand and walk, when attendant shortening of the limb and contractures of hip adductor and flexor muscles become apparent. Gradual reduction by traction may be used for approximately 3 weeks. An alternative to traction is the closed surgical reduction with casting. An individualized home traction program may be developed for the child preoperatively to decrease the length of hospitalization and keep the child in the home environment. Written directions should be provided to increase compliance with preoperative care.

The child who has initial traction treatment then undergoes an attempted closed reduction of the hip under general anesthesia; an arthrogram is used to confirm reduction. If the hip is not reducible, an open operative reduction is performed. After reduction, the child is placed in a hip spica cast for 2 to 4 months until the hip is stable, at which time a flexion-abduction brace is applied.

Older Child: Correction of the hip deformity in the older child is inherently more difficult than in the preceding age-groups because secondary adaptive changes and other etiologic factors (such as rheumatoid arthritis or nonambulatory cerebral palsy) complicate the condition. Operative reduction, which may involve preoperative traction, tenotomy of contracted muscles, and any one of several innominate osteotomy procedures designed to construct an acetabular roof, is usually required. After cast removal and before weight bearing is permitted, range-of-motion exercises help restore movement. Other rehabilitative measures may include muscle strengthening, a period of crutch walking, and gait training. Successful reduction and reconstruction become increasingly difficult after the age of 4 years and are usually impossible or inadvisable after age 6 because of severe shortening and contracture of muscles and deformity of the femoral and acetabular structures.

Hip dislocation is often observed in older children with SB and reportedly does not significantly interfere with ambulation. Reduction of the affected hip is not worthwhile in most of these children unless the dislocation is bilateral or there is significant flexion contracture (Rowe and Jadhav, 2008).

Care of the Child: The primary nursing goal is teaching parents to apply and maintain the reduction device. The Pavlik harness allows for easy handling of the infant and usually produces less apprehension in the parent than heavy braces and casts. It is important that parents understand the correct use of the appliance, which may or may not allow for its removal during bathing. If the infant has a harness that is not removed, a sponge bath is recommended.

The following instructions for preventing skin breakdown with the harness are stressed:

The parents are permitted to pad shoulder straps at pressure points if desired, but unbuckling or removal is determined on the basis of the family’s level of understanding and the degree of hip deformity. In general, parents are not encouraged to adjust the harness without supervision. The practitioner should examine the child before any adjustment is attempted to ascertain that the hips are in correct position before the harness is resecured. Problems reported by parents included difficulty applying the harness after the bath, carrying the child, skin-crease dermatitis, feet slipping, and difficulty in clothing the child (Hassan, 2009). These findings highlight the importance of close follow up within the first few weeks of treatment and written take-home instructions for the parents to follow in managing the Pavlik harness.

The major challenges in the care of an infant or child in a cast or other device are related to maintaining the device and adapting nurturing activities to meet the patient’s needs. Generally, treatment and follow-up care of these children are carried out in a clinic, practitioner’s office, or outpatient unit. Hospitalization may be necessary for cast application or brace fitting but seldom exceeds 24 to 48 hours. Longer hospitalization is required for open reduction. (See also The Child in a Cast, Chapter 39.)

Casts offer more challenging nursing problems, since they cannot be removed for routine care. Care of an infant or small child with a cast requires nursing innovation to reduce irritation and to maintain cleanliness of both the child and the cast, particularly in the perineum and sacrum. The importance of spica cast care should be emphasized when providing instructions. The life of the cast should be prolonged to hold the legs and hips in proper position postoperatively and prevent an unnecessary cast change.

Chapter 39 discusses cast care and observation, so these are not elaborated on here. However, since DDH is almost the only reason for application of casts in early infancy, some of the problems specific to that age-group are mentioned.

Parents need to learn the proper care of the cast (or brace) and need help to devise means for maintaining cleanliness. A disposable newborn diaper is tucked beneath the entire perineal opening of the cast. A larger (toddler-size) diaper can be applied and fastened over the small diaper and cast. The goal is to absorb the urine while avoiding urine saturation or soiling of the cast.

For tightly fitting casts, transparent film dressing can be cut into strips as for petaling (see Chapter 39), and one edge can be applied to the cast edge and the other directly to the perineum; this forms a continuous waterproof bridge between the perineum and the cast to prevent leakage. An additional advantage to using this dressing material is that it keeps both the skin and the cast dry while allowing observation of the skin beneath the dressing.

Older infants and small children may stuff bits of food, small toys, or other items under the cast. Alert parents to this possibility so they can initiate suitable preventive measures, such as placing clothing over the cast.

Feeding the infant in a hip spica cast or brace offers problems of positioning. Very young infants can be fed in the supine position with the head elevated, and, with the infant’s hips and legs supported on a pillow at the side, the parent can cuddle the infant during feeding. A somewhat similar position can be used for breast-feeding (i.e., with the infant supported on pillows or held in a “football” hold facing the mother with the legs behind her). An alternate position is to hold the infant upright on the mother’s lap with the infant’s legs astride the mother’s legs.

Infants who are able to sit up can be fed in a feeding table or modified high chair. A padded tilt board with an adjustable chair may be adapted for the child in a hip spica cast. The table or chair provides an excellent place for the child to play in an upright position. The child’s transportation in a safe car seat is also a vital consideration. Many hospitals have a child passenger safety program. A loan program for the appropriate automotive safety restraint or referral to an agency that provides this service may be offered by the discharge coordinator nurse or social worker.*

Family Support and Home Care: It is important for nurses, parents, and other caregivers to understand that children with DDH need to be involved in all the activities of any child in the same age-group. Toys are chosen that can be used in a prone position on the floor or in the seats devised for feeding and other activities. Confinement in a cast or appliance should not exclude children from family (or unit) activities. They can be held astride a lap for comfort and transported to areas of activity. The child may be allowed to walk in a cast or brace. An adapted wheelchair or stroller can offer mobility to the older infant or child. (See Chapter 39 for further discussion of care of a child in a spica cast.)

Prognosis: Some feet respond to treatment readily; some respond only to prolonged, vigorous, and sustained efforts; and the improvement in others remains disappointing even with maximum effort. Parents should realize that outcomes are not always predictable and depend on the severity of the deformity; age of the child at initial intervention; compliance with treatment protocols; and development of bones, muscles, and nerves. Surgical intervention may not restore the ankle to an entirely normal state, with the affected foot and leg remaining smaller and thinner than the unaffected side. Ankle range of motion after surgery may be even less than it was preoperatively. Many children with surgically corrected clubfoot, however, are able to walk without a limp and run and play.