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Muscular dystrophy refers to a group of inherited conditions that result in progressive muscle weakness and wasting. The muscles affected are primarily the skeletal (voluntary) muscles. Nine types of muscular dystrophy exist. All include muscle weakness over the lifetime; it is progressive in all cases but more severe
in others. The various muscular dystrophies are most often diagnosed in childhood and affect a variety of muscle groups. The inheritance pattern for muscular dystrophy differs for each type but may be X-linked, autosomal dominant, or recessive. The genetic muta- tion in muscular dystrophy results in absence or decrease of a specific muscle protein that prevents normal function of the muscle. The skeletal muscle fibers are affected, yet there are no structural abnor- malities in the spinal cord or the peripheral nerves. Table 44.1 gives specifics related to the various types of muscular dystrophy.
Duchenne muscular dystrophy, the
most common neuromuscular disorder of childhood, is universally fatal (usually by age 20 to 25) (Sarnat, 2011; Zak & Chan, 2010). The incidence is about 1 in 3,600 live male births (Sarnat, 2011). For these reasons, this discussion will focus on Duchenne muscular dystrophy.
The gene mutation in Duchenne muscular dystrophy results in the absence of dystrophin, a protein that is critical for maintenance of muscle cells.
The gene is X-linked recessive, meaning that mainly boys are affected and they receive the gene from their mothers (women are carriers but have no symptoms). Absence of dystrophin leads to generalized weakness of voluntary muscles, and the weakness progresses over time. The hips, thighs, pelvis, and shoulders are affected initially; as the disease progresses, all voluntary muscles as well as cardiac and respiratory muscles are affected. Rarely, males with Duchenne muscular dystrophy may survive beyond the early 30s
Boys with Duchenne muscular dystrophy are often late in learning to walk. As toddlers, they may display pseudohypertrophy (enlarged appearance) of the calves. During the preschool years they fall often and are quite clumsy. The affected child has difficulty climbing stairs and running and cannot get up from the floor in the usual fashion.The school-age child walks on the toes or balls of the feet with a rolling or waddling gait. Balance
is disturbed significantly, and the child’s belly may stick out when the shoulders are pulled back to stay upright and keep from fall- ing over. During the school-age years it also becomes difficult for the child to raise his or her arms. Some- time between the ages of 7 and 12 years nearly all boys with Duchenne muscular dystrophy lose the abil- ity to ambulate, and by the teen years any activity of the arms, legs, or trunk requires assistance or support (Muscular Dystrophy Association, 2009b). Most boys with Duchenne muscular dystrophy have normal intel- ligence, but many may exhibit a specific learning dis- ability (Sarnat, 2011).
There is no cure for Duchenne muscular dystrophy. However, the use of corticosteroids may slow the pro- gression of the disease (Muscular Dystrophy Association, 2009b; Sarnat, 2011; Zak & Chan, 2010). It is thought that prednisone helps by
protecting muscle fibers from damage to the sarcolemma (defective in the absence of dystrophin). Numerous studies have shown that boys treated with prednisone demonstrate improved strength and function. The side effects of corticosteroids are many, including weight gain, osteoporosis, and mood changes (Palmieria & Sblendorio, 2007; Sarnat, 2011). Calcium supplements are prescribed to prevent osteopo- rosis, and antidepressants may be helpful when depres- sion occurs related to the chronicity of the disease and/ or as an effect of corticosteroid use (Muscular Dystrophy Association, 2009b); see Healthy People 2020. Braces or orthoses and mobility and positioning aids are necessary.
HEALTHY PEOPLE 2020
Reduce the proportion of children and adolescents with disabilities who are re- ported to be sad, unhappy, or depressed.
Use every encounter with the child or adolescent with a disability as an op- portunity to screen for mental health concerns.
• Refer these children and teens as ap- propriate for mental health assessment and
As the muscles deteriorate, joints may become fix- ated, resulting in contractures. Contractures restrict flexibility and mobility and cause discomfort. Sometimes contractures require surgical tendon release. Spinal cur- vatures result over time.
The boy with Duchenne mus- cular dystrophy who can still walk may develop lordosis. More frequently, scoliosis or kyphosis develops with this disorder. Surgical spinal fixation with rod implantation is often required by adolescence (Muscular Dystrophy Asso- ciation, 2009b). Additional complications include pulmo- nary, urinary, or systemic infections; depression; learning or behavioral disorders; aspiration pneumonia (as oro- pharyngeal muscles become affected); cardiac dysrhyth- mias; and, eventually, respiratory insufficiency and failure (as weakness of the chest muscles and diaphragm progresses).
Examine the health history for a family history of neu- romuscular disorders. Note pregnancy and delivery his- tory, as this information may be useful in ruling out a pregnancy problem or birth trauma as a cause for the motor dysfunction. Determine status of developmental milestone achievement. Boys with Duchenne muscular dystrophy learn to walk but
over time become unable to do so. If the child was previously diagnosed with muscu- lar dystrophy, determine progression of disease. Inquire about functional status and need for assistive or adap- tive equipment such as braces or wheelchairs. Determine skills related to activities of daily living. Note history of cough or frequent respiratory infections, which occur as the respiratory muscles weaken. While talking with the child and family, determine whether psychosocial issues such as decreased self-esteem, depression, alterations in socialization, or altered family processes might be present.
INSPECTION AND OBSERVATION
Observe the child’s ability to r
rise from the floor. A hall- mark finding of Duchenne muscular dystrophy is the presence of the Gowers sign: the child cannot rise from the floor in standard fashion because of increasing weak- ness (Fig. 44.6). Observe the child’s gait. Determine effectiveness of cough.
AUSCULTATION AND PALPATION
Auscultate the heart and lungs. Note
tachycardia, which develops as the heart muscle weakens. Note adequacy of breath sounds, which may diminish with decreasing respiratory function. Note muscle strength with resis- tance testing. Palpate muscle tone.
Laboratory and Diagnostic Tests muscular dystrophy
Electromyography (EMG) demonstrates that the problem lies in the muscles, not in the nerves. Serum creatine kinase levels are elevated early in the disorder, when significant muscle wasting is actively occurring. Muscle biopsy provides definitive diagnosis, demonstrating the absence of dystrophin. DNA testing reveals the presence of the gene.
Nursing management is aimed at promoting mobility, maintaining cardiopulmonary function, preventing com- plications, and maximizing quality of life. Interventions directed at maintaining mobility and cardiopulmonary function also help to prevent complications. Refer to Nursing Care Plan 44.1, and individualize nursing care based on the child’s and family’s response to the illness. Additional specifics related to care of the child with mus- cular dystrophy are discussed below.
Administer corticosteroids and calcium supplements as ordered. Encourage at least minimal weight bearing in a standing position to promote improved circulation, healthier bones, and a straight spine.
Boys with Duch- enne muscular dystrophy may use a standing walker or standing frame to maintain an upright position. Perform passive stretching or strengthening exercises as recommended by the physical therapist. These exercises preserve mobility and may help to prevent muscle atrophy. Use orthotic supports such as hand braces or ankle–foot orthoses (AFOs) to prevent contractures of joints. Schedule activities during the part of the day when the child has the most energy. Teach parents the use of positioning, exercises, ortho- ses, and adaptive equipment.
Maintaining Cardiopulmonary Function-MD
Assess respiratory rate, depth of respirations, and work of breathing. Auscultate the lungs to determine whether aeration is sufficient and to assess clarity of breath sounds. Position the child for maximum chest expansion, usually in the upright position. Teach the child and fam- ily deep-breathing exercises to strengthen or maintain respiratory muscles and encourage
coughing to clear the airways. Perform chest physical therapy or assist with chest percussion. Monitor the results of pulmonary func- tion testing. Use of intermittent positive-pressure venti- lation and mechanically assisted coughing will become necessary in the teen years for some boys, possibly later for others. Teach parents monitoring of respiratory status and use of these modalities in conjunction with the respiratory therapist. Monitor cardiac status closely to identify heart failure early. Assess for edema, weight gain, or crackles. Strictly monitor fluid intake and output.
Maximizing Quality of Life-MD
Long periods of bed rest may contribute to further weakness. Work with the family and child to develop a schedule for diversional activities that provide appropri- ate developmental stimulation but avoid overexertion or frustration (related to inability to perform the activity). Periods of adequate rest must be balanced with activi- ties.
Walking or riding a stationary bike is appropriate for the child who has upper extremity involvement. For the child with lower extremity involvement, a wheelchair may become necessary for mobility, and the child may participate in crafts, drawing, and computer activities. Participating in the Special Olympics may be appropri- ate for some children. Visitfor a link to the Special Olympics website. Do not place limits on the child but encourage activities he or she is interested in that can be modified as needed to fit his or her abilities.
Provide emotional support to the child with MD and family.
Long-term direct care is stressful for families and becomes more complex as the child gets older. Families often need respite from continual caregiving duties. When a child is hospitalized, the caregiver may feel comfortable allow- ing nurses and other health care professionals to assume more of the child’s daily care; this can be an opportunity for the caregiver to obtain respite from daily care. Respite care may also be offered in the home by various com- munity services, so explore these resources with families.
Assess the child’s educational status. Some children attend school; others may opt for home schooling. Admin- ister antidepressants as ordered; managing depression may
increase the child’s desire to participate in activities and self- care. Refer the child and family to the Muscular Dystrophy Association (a link to which is located on), which provides multidisciplinary care via clinics located throughout the United States. The association is also a clearinghouse for resources for individuals with muscular dystrophy. Ensure that families receive genetic counseling for family planning purposes as well as determining which family members may be carriers for muscular dystrophy.
Cerebral palsy is a term used to describe a range of
non- specific clinical symptoms characterized by abnormal motor pattern and postures caused by nonprogressive abnormal brain function. The majority of causes occur before delivery, but can also occur in the natal and post- natal periods (Johnston, 2011; Zak & Chan, 2010) (Box 44.1); see Healthy People 2020. Cerebral palsy is the most common movement disorder of childhood; it is a lifelong condition and one of the most common causes of physical disability in children (Johnston, 2011;). The incidence is about 3.6 in every 1,000 live births
Most affected children with CP will develop symptoms in infancy or early childhood. There is a large variation in symptoms and disability. For some children it may be as mild as a slight limp; for others it may result in severe motor and neurologic impairments (NINDS, 2011a).
Primary signs include motor impairments such as spasticity, muscle weakness, and ataxia, which is lack of coordina- tion of muscle movements during voluntary movements such as walking or picking up objects. Complications include mental impairments, seizures, growth problems, impaired vision or hearing, abnormal sensation or per- ception, and hydrocephalus. Most children can survive into adulthood, but function and quality of life can vary from near normal to substantial impairments (NINDS, 2011a).
Cerebral palsy is a disorder caused by abnormal devel- opment of, or damage to, the motor areas of the brain, resulting in a neurologic lesion. It is difficult to
establish an exact location of the neurologic lesion, but it causes a disruption in the brain’s ability to control movement and posture. The lesion itself does not change over time; thus, the disorder is considered nonprogressive since the brain injury does not progress. However, the clinical
manifestations of the lesion change as the child grows. Some children may improve, but many either plateau in their attainment of motor skills or demonstrate worsen- ing of motor abilities because it is difficult to maintain the ability to move over time.
Management of cerebral palsy involves multiple disciplines, including a primary physician, specialty physicians such as a neurologist and an orthopedic surgeon, nurses, physi- cal therapists, occupational therapists, speech therapists, dietitians, psychologists, cnslrs, teachers, and parents.
There is no standard trtmnt for all children. The overall focus of therapeutic management will be to assist the child to gain optimal development and functn within the limits of the disease. Treatmnt is mnly prvnttve, sypmtc, and supprtive. Sptcty management will be a pri- mary concern and will be determined by clinical findings. Medical management is focsed on promoting mobility through the use of theraptic modalities and medications. Surgical management is often required and is used to correct deformities related to spasticity.
Physical, Occupational, and Speech Therapy- CP
The use of therapeutic modalities such as physical ther- apy, occupational therapy, and speech therapy will be essential in promoting mobility and development in the child with cerebral palsy. The earlier the treatment begins, the better chance the child has of overcoming developmental disabilities (NINDS, 2011a).
Physical therapists work with children to assist in the development of gross motor movements such as walking and positioning, and they help the child develop inde- pendent movement. They also assist in preventing con- tractures, and they instruct children and caregivers in the use of assistive devices such as walkers and wheelchairs. Occupational therapists may be responsible for fashioning orthotics and splints. AFOs are the most common orthotic used by children with cerebral palsy (Cervasio, 2011) (Fig. 44.9).
AFOs help prevent deformity from conditions such as contractures and help reduce the effects of exist- ing deformities. They can help improve a child’s mobility by assisting in control of alignment and helping to increase the efficiency of the child’s gait. Spinal orthotics such as braces are used in young children with cerebral palsy to combat scoliosis that develops due to spasticity. These braces are used to delay surgical management of the sco- liosis until the child reaches skeletal maturity. Splinting is used to maintain muscle length. Serial casting may also be used to increase muscle and tendon length.
Occupational therapy also assists in the development of fine motor skills and will help the child w cp to perform optimal self-care by working on skills such as activities of daily living. Speech therapy assists in the development of receptive and expressive language and addresses the
use of appropriate feeding techniques in the child who has swallowing problems. Speech therapists may teach augmented communication strategies to children who are nonverbal or who have articulation problems. Many chil- dren may not communicate verbally but can use alterna- tive means such as communication books or boards and computers with voice synthesizers to make their desires known or to participate in conversation.
Various pharmacologic options are available to manage spasticity (see Drug Guide 44.1). Oral medications used to treat spasticity include baclofen, dantrolene sodium, and diazepam. Children with athetoid cerebral palsy may be given anticholinergics to help decrease abnor- mal movements.
Parenterally administered medications such as botulin toxins and baclofen are also used to manage spasticity. Botulinum toxin is injected into the spastic muscle to balance the muscle forces across joints and to decrease spasticity. It is useful in managing focal spasticity in which the spasticity is interfering with function, producing pain, or contributing to a progressive deformity. Botulin toxin injection is performed by the physician or nurse practitioner and can be done in the clinic or outpatient setting.
Intrathecal administration of baclofen has been shown to decrease tone, but it must be infused continu- ously due to its short half-life. Surgical placement of a baclofen pump will be considered in children with gen- eral spasticity that is limiting function, comfort, activities of daily living, and endurance. To test whether it is a suit- able option,
an intrathecal test dose of baclofen will be administered. If the trial is successful, a baclofen pump will be implanted. Once inserted, delivery of the drug can be individualized to meet the child’s unique needs. The pump needs to be replaced every 5 to 7 years and must be refilled with medication approximately every 3 months, depending on the type of pump. Complica- tions with baclofen pump placement include infection, rupture, dislodgement, or blockage of the catheter.
Medications are also used to treat seizure disorders in children with cerebral palsy (refer to Chapter 38 for information related to seizure management).
Many children will require surgical procedures to correct deformities related to spasticity. Multiple corrective sur- geries may be required; they usually are orthopedic or neurosurgical. Surgery may be used to correct contrac- tures that are severe enough to cause movement limita- tions.
Common orthopedic procedures include tendon lengthening procedures, correction of hip and adductor muscle spasticity, and fusion of unstable joints to help improve locomotion, correct bony deformities, decrease painful spasticity, and maintain, restore, or stabilize a spinal deformity. Neurosurgical interventions may include placmnt of a shunt in children who have dvlpd hydrcphls, or surgical interventions to decrease spasticity. Selective dorsal root rhizotomy is used to decrease spasticity in the lower extremities by reducing the amnt of stmltn tht rches the mscles via the nerves.
Elicit a description of the present illness and chief com- plaint. Obtain a detailed account of gestational and perinatal events (refer to Box 44.1). Common signs and symptoms reported during the health history of the undi- agnosed child might include:
Intrauterine infections • Prematurity with intracranial hemorrhage • Difficult, complicated, or prolonged labor and delivery • Multiple births • History of possible anoxia during prenatal life or birth. History of head trauma • Delayed attainment of developmental milestones • Muscle weakness or rigidity • Poor feeding • Hips and knees feel rigid and unbending when pulled to a sitting position • Seizure activity • Subnormal learning • Abnormal motor performance, scoots on back instead
of crawling on abdomen, walks or stands on toes
children known to have cerebral palsy are often admitted to the hospital for corrective surgeries or other complications of the disease, such as
aspiration pneumonia and urinary tract infections. The health history should include questions related to: • Respiratory status: Has a cough, sputum production, or increased work of breathing developed? • Motor function: Has there been a change in muscle tone or increase in spasticity? • Presence of fever • Feeding and weight loss • Any other changes in physical state or medication regimen
Observe general appearance. Pay close attention to the neurologic assessment and motor assessment. Assess for delayed development, size for age, and sensory altera- tions such as strabismus, vision problems, and speech disorders. Abnormal postures may be present. While lying supine, the infant may demonstrate scissor crossing of the legs with plantar flexion. In the prone position the infant may raise his or her head higher than normal due to arching of the back, or the opisthotonic position may be noted.
The infant may also abnormally flex the arms and legs under the trunk. Primitive reflexes may persist beyond the point at which they disappear in a healthy infant. Evolution of protective reflexes may be delayed. Watch the infant or child play, crawl, walk, or climb to determine motor function and capability. Note any movement disorder. Infants with cerebral palsy may demonstrate abnormal use of muscle groups such as scooting on their back instead of crawling or walking.
Assess active and passive range of motion. Pay par- ticular attention to muscle tone. Though an increased or decreased resistance may be noted with passive move- ments, hypertonicity is most often seen. Increased resistance to dorsiflexion and passive hip abduction are the most common early signs.
Sustained clonus may be present after forced dorsiflexion. Lift the child by placing your hands in the infant’s or child’s axillary area to assess shoulder girdle function and tone. Infants with cerebral palsy often demonstrate prolonged standing on their toes when supported in an upright standing position in this fashion. Lift the young child off the ground while the child holds your thumbs to test hand strength. Observe
for prsnce of limb defrmty, as decrsed use of an extrmty (hemiparesis) may result in shortening of the extremity compared to the other one.
Laboratory and Diagnostic Tests
A complete history, physical examination, and ancillary investigations are the primary modality for establishing a diagnosis of cerebral palsy. The following laboratory and diagnostic tests will help determine whether cere- bral palsy is the likely cause or whether another condi- tion may be the cause of the child’s symptoms.
These tests also will be important in evaluating the severity of the child’s physical disabilities. Common supplementary laboratory and diagnostic tests ordered for the diagnosis and assessment of cerebral palsy include:• Electroencephalogram: usually abnormal but the pat- tern is highly variable• Cranial radiographs or ultrasound: may show cerebral asymmetry• MRI or CT: may show area of damage or abnormal development but may be normal• Screening for metabolic defects and genetic testing may be performed to help determine the cause of ce- rebral palsy.
Mobility is critical to the development of the child with cerebral palsy. Treatment modalities to promote mobility include physiotherapy, pharmacologic management, and surgery. Surgical procedures are discussed above.
Physical or occupational therapy as well as medications may be used to address musculoskeletal abnormalities, to facilitate range of motion, to delay or prevent defor- mities such as contractures, to provide joint stability, to maximize activity, and to encourage the use of adap- tive devices. The nurse’s role in relation to the various therapies is to provide ongoing follow-through with pre- scribed exercises, positioning, or bracing.
cp-When casting, splinting, or orthotics are used, assess skin integrity frequently. Pain management may also be necessary.
Nursing management of children receiving botulin toxin focuses on assisting with the procedure and providing education and support to the child and family. Nursing interventions related to baclofen include assisting with the test dose and providing preoperative and postoperative care if a pump is placed, as well as providing support and education to the child and fam- ily. Teaching Guidelines 44.2 gives information related to baclofen pump insertion.
Teaching Guidelines 44.2
BACLOFEN PUMP: CHILD/FAMILY EDUCATION
Check the incisions daily for redness, drainage, or swelling.• Notify the physician or nurse practitioner if the child has a temperature greater than 101.5oF, or if the child has persistent incision pain.• Avoid tub baths for 2 weeks. • Do not allow the child to sleep on the stomach for 4 weeks after pump insertion. • Discourage twisting at the waist, reaching high over-head, stretching, or bending forward or backward for 4 weeks. • When the incisions have healed, normal activity may
be resumed. • Wear loose clothing to prevent irritation at the incision site. • Carry implntd devce idntfctn and emrgncy infrmtn cards at all times.
Children with cerebral palsy may have difficulty eat- ing and swallowing due to poor motor control of the mouth, tongue, and throat. This may lead to poor nutri- tion and problems with growth. The child may require a longer time to eat because of poor motor control.
Special diets, such as soft or puréed, may make swallowing easier. Proper positioning during feeding is essential to facilitate swallowing and reduce the risk of aspiration. Speech or occupational therapists can assist in working on strengthening swallowing muscles as well as assisting in developing accommodations to facilitate nutritional intake. Consult a dietitian to ensure adequate nutrition for children with cerebral palsy. In children with severe swallowing problems or malnutrition, a feeding tube such as a gastrostomy tube may be placed.
Providing Support and Education
Cerebral palsy is a lifelong disorder that can result in severe physical and cognitive disability. In some cases disability may require complete intensive daily care of the child. Adjusting to the demands of this multifaceted illness is difficult.
Children are frequently hospitalized and need numerous corrective surgeries, which places strain on the family and its finances. From the time of diagnosis, the family should be involved in the child’s care. It is important to include parents in the planning of interventions and care of this child. In most cases they are the primary cargvrs and will assist the child in dvlpmnt of functioning and skills as well as providing daily care. They will provide essential infor- mation to the health care team and will be advocates for their child throughout his or her life. It is important that nurses provide ongng edctn for the chld and fmly.
As the child grows, the needs of the family and child will change. Recognize and respect these needs. Providing daily intense care can be demanding and tir- ing. When a child with cerebral palsy is admitted to the hospital, this may serve as a time of respite for fam- ily and primary caregivers. Encourage respite care and provide support and encouragement.
Because cerebral palsy is a lifelong condition, children will need meaningful education programs that emphasize indepen- dence in the least restrictive educational environment. Refer caregivers to local resources, including education services and support groups. Links to United Cerebral Palsy, a national organization, and Easter Seals, an organization that helps children with disabilities and special needs and provides support to families, can be accessed on .
Refer children younger than age 3 years to the local early intervention service. Early intervention provides case management of developmental services for children with special needs.
Each state has a coordinator for early inter- vention. The office of the early intervention coordinator can then direct the health care professional to the local or district early intervention office. The website 4MyChild (a link to which is available on) provides a com- prehensive list of contact information based on the state you live in.
Additional links to resources for families of young children with special needs are available on. For additional reading, recommend the book Children With Cerebral Palsy: A Parent
Dermatomyositis is an autoimmune disease that results in inflammation of the muscles or associated tissues. It occurs more often in girls and is generally diagnosed between the ages of 5 and 14 years (Muscular Dystrophy Association, 2009a). The autoimmune response may be triggered by exposure to a virus or to certain medications (Muscular Dystrophy Association, 2009a). As with other autoimmune diseases, a genetic predisposition is pres- ent. The inflammatory cells of the immune system cause a vasculitis that affects the skin, muscles, kidneys, retinas, and gastrointestinal tract.
Therapeutic management involving the use of cor- ticosteroids or other immunosuppressants is necessary to prevent the complications of painful calcium deposits under the skin, as well as joint contractures. With appro- priate treatment, children may recover completely, though some children experience relapses (Muscular Dystrophy Association, 2009a).
fever, fatigue, and rash, uslly fllwd by muscle pain and weakness. Determine onset and progression of muscle weakness. Inspect the skin for presence of rash involving the upper eyelids and extensor surfaces of the knuckles, elbows, and knees. The rash is initially a reddish-purplish color, and then progresses to scaling with resulting roughness of the skin. Test muscle strength, noting particularly weakness in the pelvic and shlder girdles. Labrtry and diagnostic testing may include mscle enzyme levels, a pstve antinuclear antibody (ANA) test, and an electromyelogram to dstngh msclr wknss frm othr cses
Administer medications as ordered and teach families about their use; instruct them to monitor for side effects. Educate the family about the importance of maintain- ing the medication regimen in order to prevent calcino- sis (calcium deposits) and joint deformity in the future. Encourage compliance with physical therapy regimens. Ensure that children are excused from physical educa- tion classes while the disease is active.
Pectus excavatum and pectus carinatum are anterior chest wall deformities. Pectus excavatum, a funnel- shaped chest, accounts for greater than 90% of all con- genital chest wall deformities (Boas, 2011). A depres- sion that sinks inward is apparent at the xiphoid process (Fig. 45.10). Pectus carinatum, a protuberance of the chest wall, accounts for only 5% to 15% of anterior chest wall deformities (Boas, 2011). The remainder are mixed deformities
Therapeutic management of pectus excavatum is
based on the severity and physiologic compromise. Options include observation, use of physical therapy to work on muscu- loskeletal compromise, and surgical correction, preferably before puberty, when the skeleton is more pliable. Various surgical techniques may be used and generally involve either the placement of a surgical steel bar or using a piece of bone in the rib cage to lift the depression. This discussion will focus on care of the child who undergoes surgical steel bar placement for pectus correction.
Elicit the health history, noting progression of the defect and effects on the child’s cardiopulmonary function. Note shortness of breath, exercise intolerance, or chest pain. Observe the child’s chest for anterior wall deformity, not- ing depth and severity. Auscultate the lungs to determine the adequacy of aeration. Radiographs, computed tomog- raphy (CT), or magnetic resonance imaging (MRI) may be used to determine the extent of the anomaly and com- pression of inner structures
Prepare the child preoperatively by allowing a tour of the surgical area and the pediatric intensive care unit. Introduce the child to the pain scale that will be used in the postoperative period.
Postoperatively, nursing management focuses on assessment, protection of the surgical site, and pain management. Auscultate lung sounds frequently to determine the adequacy of aeration and to monitor for development of the complication of pneumothorax.
Assess for signs of wound infection that would necessitate removal of the curved bar. During the first few postoperative days pstnng is chllngng; do not allow the child to roll in bed, lie on either side, or rotate or flex the spine (these pstns may dsrpt the bar’s position). Administer analgesics as ndd either intravenously or via the epidural catheter. Teach families that the child will not be allowed to lie on his or her side at home for 4 weeks after the surgery to ensure tht the band does nt shft. Encrge aerobic activity at home after being cleared by the surgeon (this will increase the child’s vl capcty). The bar will be rmvd 2-4 yrs aftr the initl plcmnt
is a congenital anomaly that occurs in 1 of 1,000 live births (Hosalkar, Spiegel, et al., 2011). Clubfoot consists of:• Talipes varus (inversion of the heel) • Talipes equinus (plantarflexion of the foot; the heel is raised and would not strike the ground in a standing position) • Cavus (plantarflexion of the forefoot on the hindfoot) • Forefoot adduction with supination (the forefoot is inverted and turned slightly upward)The foot resembles the head of a golf club (Fig. 45.13). Half of all cases occur bilaterally. Males are affected more frequently than females. The exact etiology of clubfoot is unknown.
Clubfoot may be classified into four categories:
The goal of therapeutic management of clubfoot
is achvmnt of a fnctnl foot; tx starts as soon after birth as possible. Wkly mnpltn with serial cast changes is prfrmd; later, cast changes occur every 2 wks. Othr infnts require corrective shoes or bracing. In some infants surgical release of soft tissue may be necessary. Following surgery, the foot is immobilzd with a cast for up to 12 weeks, and then anklfoot orthoses or corrective shoes are used for several years. Complications of clubfoot and its tx include residual deformity, rocker-bottom foot, awkward gait, weight bearing on the ltrl prtn of the ft if uncrrctd, and disturbnce to the epiphyss
Note family history of foot deformities and obstetric his- tory of breech position. Inspect the foot for position at rest. Perform active range of motion, noting inability to move foot into normal positioning at midline. Radio- graphs are obtained to determine bony abnormality and note progress during treatment.
Note family history of foot deformities and obstetric his- tory of breech position. Inspect the foot for position at rest. Perform active range of motion, noting inability to move foot into normal positioning at midline. Radio- graphs are obtained to determine bony abnormality and note progress during treatment.
Osteogenesis imperfecta is a genetic bone disorder that results in low bone mass, increased fragility of the bones, and other connective tissue problems such as joint hypermobility, resulting in instability of the joints. All of these contribute to fracture occurrence. Dentin- ogenesis imperfecta may also occur. This is character- ized by the tooth enamel wearing easily and brittle and discolored teeth.
The disorder usually occurs as a result of a defect in the collagen type 1 gene,
usually through an autosomal dominant inheritance pattern but some types are inher- ited in a recessive manner (Gunta, 2009). The types of osteogenesis imperfecta range from mild to severe con- nective tissue and bone involvement (Table 45.2). Sub- types A and B exist depending on (A) the absence or (B) the presence of dentinogenesis imperfecta (Marini, 2011). In children with moderate to severe disease, frac- tures are more likely to occur, and short stature is com- mon. In addition to multiple fractures, additional com- plications include early hearing loss, acute and chronic pain, scoliosis, and respiratory problems.
The goal of medical and surgical management is to decrease the incidence of fractures and maintain mobility. Bisphosphonate administratn is used for moderate to severe disease. Fracture care is often required. Physi- cal therapy and occupational therapy prevent contrac- tures and maximize mobility. Standing with bracing is encouraged. Lightweight splints or braces may allow the child to bear weight earlier. Severe cases may require surgical insertion of rods into the long bones.
Elicit a health history, which may reveal a family his- tory of osteogenesis imperfecta, a pattern of frequent fractures, or screaming associated with routine care and handling of the newborn. Inspect the eyes for sclerae that have a blue, purple, or gray tint. Note abnormalities of the primary teeth. Inspect skin for bruising and note joint hypermobility with active range of motion. Labo- ratory tests may include a skin biopsy (which reveals abnormalities in type 1 collagen) or DNA testing (locat- ing the genetic mutation).
Developmental dysplasia of the hip (DDH) refers to abnormalities of the developing hip that include dislocation, subluxation, and dysplasia of the hip joint. In DDH, the femoral head has an abnormal relationship to the acetabulum. Frank dislocation of the hip may occur, in which there is no contact between the femoral head and acetabulum. Sblxtn is a partial dislocation, mnng that the acetabulum is not fully seated within the hip joint. Dysplasia refers to an acetabulum that is shallow or sloping instead of cup shaped. DDH may affect just one or both hips. The dysplastic hip may be provoked to subluxation or dislocated and then reduced again
While dislocation may occur during a growth period in utero, the laxity of the newborn’s hip allows dislocation and relocation of the hip to occur. The hip can develop normally only if the femoral head is appropriately and deeply seated within the acetabulum. If subluxation and periodic or continued dislocation occur, then structural changes in the hip’s anatomy occur. Continued dysplasia of the hip leads to limited abduction of the hip and contracture of muscles. DDH is more common in females, probably due to the greater susceptibility of the female
newborn to maternal hormones that contribute to laxity of the ligaments. Mechanical factors such as breech positioning or the presence of oligohydramnios also contribute to the develop- ment of DDH. Genetic factors also play a role: there is an increased incidence of DDH among persons of Native American and Eastern Europe descent, with very low rates among people of African or Chinese heritage. Complications of DDH include avascular necrosis of the femoral head, loss of range of motion, recurrently unstable hip, femoral nerve palsy, leg-length discrepancy, and early o-arthritis.
The goal is to maintain the hip joint in reduction so that the femoral head and acetabulum can develop properly. Trtmnt varies based on the child’s age and the severity of DDH. Infants younger than 6 months of age may be treated with a Pavlik harness, which reduces and stabilizes the hip by preventing hip extension and adduction and maintaining the hip in flexion and abduction The Pavlik harness is successful in the treatment of DDH in the majority of infants younger than 6 months of age if it is used on a full-time basis and applied properly.
Nursing assessment of children with DDH includes obtaining a health history and inspecting, observing, and palpating for findings common to DDH.
Assess the health history for risk factors such as: • Family history of DDH • Female genderOligohydramnios or breech birth • Native American or Eastern European descent • Associated lower limb deformity, metatarsus adductus,
hip asymmetry, torticollis, or other congenital muscu- loskeletal deformity
Previously undiagnosed older children may com- plain of hip pain.
The physical examination for DDH includes inspection, observation, and palpation. Since DDH is a developmental process, ongoing screening assessments are required throughout at least the first several months of the infant’s life iNsPeCTiON AND OBserVATiONEnsure that the infant is on a flat surface and is relaxed. Note asymmetry of thigh or gluteal folds with the infant in a prone position
. Document shortening of the affected femur observed as limb-length discrepancy. Older children may exhibit Trendelenburg gait.illustrates these assessments. PALPATiONNote limited hip abduction while performing passive range of motion. Abdctn shld ordinarily occur to 75 degrees and addctn to within 30 degrees with the infant’s pelvis stabilzd. Perfrm Barlow and Ortolani tests, feeling for, or noting, a palpable “clunk” as the femoral head dislocates (positive Barlow) or reduces (positive Ortolani) back into the acetabulum. Force is not necessary when performing the Barlow and Ortolani maneuvers
Ultrasound of the hip allows for visualization of the fem- oral head and the outer edge of the acetabulum. Plain hip radiographs may be used in the infant or child older than 6 months of age
Earlier recognition of hip dysplasia
Earlier recognition of hip dysplasia with earlier harness use results in better correction of the anomaly. Excellent assessment skills and reprting of any abnormal findings are critical. Initially, the infant will need to wear the Pavlik harness continuously (Fig. 45.16). The physician or nurse prcttner makes all appropriate adjstmnts the harness when applied so that the hips are held in the optimal position for appropriate development. Teach parents use of the harness and assessment of the baby’s skin. If started early, harness use usually continues for about 3 months. Breast- fding can continue throughout the harness treatment
Teaching Guidelines 45.4
caring For a child in a PaVlik harnessCall the doctor if: • Your baby’s feet are swollen or bluish. • The harness appears too small. • Skin is raw or a rash develops. • Your baby is unable to actively kick his or her legs.Do not adjust the straps without checking with the physician or nurse practitioner first.• Until your physician or nurse practitioner instructs you to take the harness off for a period of time each day, it must be used continuously (first week or longer).• Change your baby’s diaper while he or she is in the harness.
• Place your baby to sleep on his or her back. • Check skin folds, especially behind the knees and diaper area, for redness, irritation, or breakdown.Keep these areas clean and dry. Once the baby is permitted to be out of the harness for a short period, you may bathe your baby while the harness is off.• Long knee socks and an undershirt are recommended to prevent rubbing of the skin against the brace.• Note location of the markings on the straps for appropriate placement of the harness.• Wash the harness with mild detergent by hand and air dry. If using the dryer, use only the air fluffing setting (no heat)
For infants or children diagnosed later than 6 months of age or those who do not improve with harness use, surgical reduction may be performed after a period of traction (Sankar, Horn, Wells, & Dormans, 2011). Postop- erative casting followed by bracing or orthotic use is com- mon. Caring for the child in the postoperative period issimilar to care of any child in a cast. Pain management and monitoring for bleeding are priority activities. Teach families care of the cast at home.
Scoliosis is a lateral curvature of the spine that exceeds 10 degrees. It may be congenital, associated with other disorders, or idiopathic. Table 45.3 explains the types of scoliosis. Idiopathic scoliosis, with the majority of cases occurring during adolescence, is the most common scoliosis. Hence, this discussion will focus on adolescent idiopathic scoliosis. The etiol- ogy of idiopathic scoliosis is not known, but genetic factors, growth abnormalities, and bone, muscle, disc, or central nervous system disorders may contribute to its development. Early screening and detection of scoliosis result in improved outcomes.
In the rapidly growing adolescent, the involved vertebrae rotate around a vertical axis, resulting in lateral curvature. The vertebrae rotate to the convex side of the curve, with the spinous processes rotating toward the concave side. Wedge-shaped vertebral bodies and discs develop because growth is suppressed on the concave side of the curve (Grewal & Ahier, 2010). As the curve progresses, the shape of the thoracic cage changes and respiratory and cardiovascular compromise may occur (the main complications of severe scoliosis).
Treatment of scoliosis is aimed at preventing progression of the curve and decreasing the impact on pulmonary and cardiac function. Treatment is based on the age of the child, expected future growth, and severity of the curve. Observation with serial examinations and spine radiographs is used to monitor curve progression. For curves of 25 to 40 degrees, bracing may be sufficient to decrease progression of the curve. describes types of scoliosis braces and shows examples of braces. The choice of brace will depend on the location and severity of the curve. Some curves will progress despite appropriate bracing and compliance.
Surgical correction is often required for curves greater than 45 degrees; it is achieved with rod placement and bone grafting
Health History ScoliosisDetermine why the child is presenting for evaluation of scoliosis.
Commonly the child or adolescent will not report back pain; only mild discomfort is associated with idiopathic scoliosis until the curve becomes severe. Often the family recognizes asymmetry in the hips or shoulders or the child is screened for scoliosis at school and determined to be at risk. Explore the child’s current and past medical history for risk factors such as: • Family history of scoliosis • Recent growth spurt • Physical changes related to puberty. Determine the age of development of secondary sex charactrstcs and the age of menarche, as these signs of pubertal development indcte the expctd vlcty and lngth of remng grwth
The physical assessment of a child with possible or actual scoliosis involves mainly inspection and observation. Auscultate the heart and lungs to determine compromise related to severe curvature.Observe the child at rest, sitting, and standing for evi- dence of poor posture. Inspect the child’s back in a stand- ing position. Note asymmetries such as shoulder
elevation,prominence of one scapula, uneven curve at the waistline, or a rib hump on one side. Measure shoulder levels from the floor to the acromioclavicular joints. Note the difference between the height of the high and low shoulder in centimeters. Measure heights of anterior and posterior iliac spines and note the difference in centimeters. View the child from the side, noting abnormalities in the spinal curve. With the child bending forward, arms hanging freely, note asymmetry of the back Note leg-length discrepancy if present. balance, motor strength, sensation, and reflexes should all be normal.
Full-spine radiographs are necessary to determine the degree of curvature. The radiologist will determine the extent of the curve based on specific formulas and tech- niques of measurement
Bracing is intended to prevent progression of the curve but does not correct the current curve. Although modern braces display an improved appearance, with no visible neckpiece, and can be worn under clothes, many adolescents are not compliant with brace wear. The brace must be worn 23 hours per day to prevent curve progression. Many factors may contribute to noncompliance, including the discomfort associated with brace wear such as pain, heat, and poor fit. The family environment may not be conducive to compliance with brace wear, and teen- agers are very concerned about body image.
Inspect the skin for evidence of rubbing by the brace that may impair skin integrity.
Teach families appropriate skin care and recommend they check the brace daily for fit and breakage. Encourage the teen to shower during the 1 hour per day that the brace is off and to ensure that the skin is clean and dry before putting the brace back on. Wearing a cotton T-shirt under the brace may decrease some of the discomfort associated with brace wear. Exercises to strengthen back muscles may prevent muscle atrophy from prolonged bracing and maintain spine flexibility.
Encourage the teen to express his or her feelings or concerns about wearing the brace. Give the teen ways to explain scoliosis and its treatment to his or her peers. Wearing stylish baggy clothes may help the teen to conceal the brace if desired. Refer teens and their families to the National Scoliosis Foundation for additional support. A link to the National Scoliosis Foundation is provided on .
Providing Preoperative Care
If the curve progresses despite bracing
or causes pulmonary or cardiac compromise, surgical intervention will be warranted. In the preoperative period, teach the teen the importance of turning, coughing, and deep breathing in the postoperative period. Explain the tubes and lines that will be present immediately after the surgery. Review positioning guidelines: back flexion or extension will not be allowed. Introduce the child to the patient-controlled analgesia pump and explain pain scales. There is a high risk for significant blood loss with spinal fusion and instrumentation, so if possible arrange for preoperative autologous blood donation.
Providing Postoperative Care
The goal of nursing management in the postoperative period after spinal fusion with or without instrumentation is to avoid complications. Perform neurovascular checks with each set of vital signs. When turning the child, use the log-roll technique to avoid flexion of the back (Fig. 45.23).Provide proper pain management and medicate for pain before repositioning and ambulation
Administer prophylactic intravenous antibiotics if ordered. Assess for drainage from the operative site and for excess blood loss via the Hemovac or other drainage tube. Main- tain Foley patency, as the child will be confined to bed for the first couple of days. Maintain strict recording of fluid intake and output. Administer transfusions of packed red blood cells if ordered. Ambulation, once ordered, should be done slowly to avoid orthostatic hypotension. Assist the family with arrangements to continue the teen’s schoolwork while hospitalized and/or arrange for home tutoring during the several-week recovery period.
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