Systemic Problems

Musculoskeletal presentation in children and adolescents can be a feature of potentially life-threatening conditions such as sepsis, malignancy or nonaccidental injury, and chronic pediatric conditions such as inflammatory bowel disease, cystic fibrosis, and juvenile idiopathic arthritis.

Systemic problems can include chronic conditions such as hemophilia, sickle cell disease, and arthritic diseases; neurologic problems such as cerebral palsy; and various cancers, including osteosarcomas and leukemias. Children with metabolic problems, such as vitamin D–resistant rickets, have bony deformities. Acute systemic problems can also affect the musculoskeletal system. For example, viruses and bacteria can infect joints and bones. In developing countries, tubercular infections of bones are common and devastating. Thus the pediatric provider must assess patients from a broad perspective, asking questions about other body systems and ordering appropriate laboratory studies that might identify systemic problems.

Genetic Problems

Many genetic problems have an orthopedic component. Osteogenesis imperfecta (OI) is a genetic disorder characterized by decreased levels of collagen, the major protein of the body’s connective tissue. Children with OI have bones that break easily, even from minor trauma. Down syndrome is a consequence of a genetic anomaly of chromosome 21. The main orthopedic pathology is hypotonia and the possibility of loose capsule and ligaments. Children with Down syndrome have a higher incidence of scoliosis, dislocation of the hip, Legg-Calvé-Perthes disease (LCPD), instability of the patella, and pes planus (flat feet). Children with Marfan syndrome may have long spider-like fingers, low muscle tone, and lax joints that are prone to dislocate. Severe scoliosis may develop in children with neurofibromatosis, Turner syndrome, and Noonan syndrome. Girls with Turner syndrome may present with webbed neck, short stature, valgus deformity of the elbow, and short fourth metacarpal deformity. Children with Noonan syndrome can present with webbed neck, clumsiness, poor coordination, and motor delay.

Many orthopedic problems have a multifactorial inheritance pattern. Thus if one child in a family has a dislocated hip or scoliosis, the risks increase for other children. The pediatric provider needs to understand the genetic disorder to monitor related orthopedic problems, consider the genetic implications, and provide families with appropriate genetic information or refer them for genetic counseling (see Chapter 40).

Uterine Packing Deformations

The developing fetus moves its body parts frequently, and this movement influences musculoskeletal development. When the baby fills the uterine space, movements are restricted and body parts begin to assume a shape in which they are fixed. With in utero positioning, joints and muscle contractions can develop and are considered generally physiologic in nature. Orthopedic deformities of uterine compression include clubfoot, torticollis, hip dislocation, metatarsus adductus, equinovarus foot, calcaneovalgus foot, tibial bowing, hyperflexed hips, hyperextended knees, contractures, and internal tibial torsion (Wynshaw-Boris and Biesecker 2007). Fetal movement is required for proper development of the musculoskeletal system, and anything that restricts fetal movement can cause deformation from intrauterine molding. Two major intrinsic causes for deformations are neuromuscular disorders and oligohydramnios. Extrinsic causes are related to fetal crowding that restricts fetal movement. For a fetus in the breech position the incidence of deformations is increased 10-fold (Wynshaw-Boris and Biesecker, 2007). Infants with deformations caused by extrinsic causes have an excellent prognosis with corrections occurring spontaneously. Torsional and angular alignment issues can affect the long bones, most commonly those of the lower extremities. Because much of the bony structure is cartilaginous, molding occurs with relative ease. Thus in normal newborns, the tibias are normally bowed, and the hips have a 20- to 30-degree flexion (Hosalkar and Wells, 2007). Occasionally, a foot may be turned awkwardly, the legs might be fixed straight up with the feet near the ears, or the neck may be tipped to one side. Such positioning issues are outside the range of normal. The outcomes are deformities in various degrees. The longer the position is maintained, the more severe the problems are. In general, there is a tendency for bowing and late deformations to straighten; however, the effects related to in utero positioning may not fully abate until the child is 3 to 4 years old. More severe deformities (i.e., those significantly outside the range of normal) need to be referred to orthopedists for treatment as soon as they are found because a softer skeleton is easier to realign in a positive direction.

Injuries

There are approximately 2.6 million emergency department visits annually in the U.S. for sports-related injuries in children ages 5 to 24 years, with the highest percentage occurring in the 5- to 14-year-old male. This does not account for the children who present to their primary care physician for evaluation and treatment (Soprano and Fuchs, 2007). The unique differences in the pediatric skeletal system predispose children to injuries unlike those seen in adults. The important differences are the presence of periosseous cartilage, physes, and a thicker, stronger, more osteogenic periosteum that produces new bone called callus more rapidly and in great amounts. Joint injuries, dislocations, and ligament disruptions are infrequent in children (Gholve et al, 2007). Physeal fractures in preadolescent children are the most common musculoskeletal injuries seen. Clavicular fractures are seen at all ages ranging from a newborn birth injury to trauma in adolescence. They can occur as a result of direct or indirect injury and are most commonly associated with a fall (Benjamin and Hang, 2007).Tendinoses are seen infrequently in children but may occur in the young athlete in the rotator cuff from throwing motions and swimming, in the iliopsoas in dancers, and in the ankle of dancers, gymnasts, and figure skaters. Shoulder injuries can be acute or result from chronic overuse. Overuse injuries are common chronic injuries in children that are related to repetitive stress on the musculoskeletal system without sufficient time to recover. The repetitive use overwhelms normal reparative processes (Soprano and Fuchs, 2007). Management of traumatic injuries is discussed in Chapter 39.

The possibility of child abuse should always be considered when orthopedic injuries, especially fractures, are present. The rule of thumb is that the injury history should match the appearance of the problem and be consistent with the child’s developmental capabilities. An unexplained fracture in a child younger than 2 years of age should be carefully evaluated. Certain fractures in younger children such as metaphyseal fractures, spiral fractures, posterior rib fractures, and fractures of the long bones in the nonambulatory child may be accidental but are highly suspicious for abusive trauma (Legano et al, 2009).

Fracture Healing

One of the major differences between the adult and the pediatric bone is that the periosteum in children is very thick. The major reason for increased healing speed of children’s fractures is the periosteum, which contributes to the largest part of new bone formation around a fracture. Children have significantly greater osteoblastic activity in this area because bone is already being formed beneath the periosteum as part of normal growth. This already active process is readily accelerated after a fracture. Periosteal callus bridges a fracture in children long before the underlying hematoma forms a cartilage anlagen that goes on to ossify. Once cellular organization from the hematoma has passed through the inflammatory process, repair of the bone begins in the area of the fracture. In most children, by 10 days to 2 weeks after fracture, a rubber-like bone forms around the fracture and makes it difficult to manipulate. As part of the reparative phase, cartilage formed as the hematoma organizes is eventually replaced by bone through the process of endochondral bone formation. The remodeling phase of fracture healing may continue for some time and is accelerated by motion of the adjacent joints and use of the extremity (Green and Swiontkowski, 2008).

Growth Plate Fractures

Fractures of the long bones can produce permanent deformities in children if the fracture occurs through the growth plate. The outcomes depend on the fracture location and type, the age of the child, the status of the blood supply to the physis, and the treatment. The Salter-Harris classification is based on the mechanism of injury, the relationship of the fracture line to the layers of physis, and the prognosis with respect to subsequent growth disturbance. There are five classifications (Fig. 37-2). Type I is the most frequent type of fracture that involves a fracture through the zone of hypertrophic cells of the physis with no fracture of the surrounding bone. Type II fractures are similar to type I except that a metaphyseal fragment is present on the compression side of the fracture. Growth disturbance in types I and II is rare.

FIGURE 37-2 The types of growth plate injury as classified by Salter and Harris.

(From Salter RB, Harris WR: Injuries involving the epiphyseal plate, J Bone Joint Surg Am 45:587, 1963.)

Type III fracture involves physeal separation with fracture through the epiphysis into the joint. The fracture requires anatomic reduction, occasionally through an open approach. Type IV fracture involves the metaphysis, physis, and epiphysis. Type V fracture involves a compression or crushing injury to the physis. Type V fractures are rare and are difficult to diagnose initially due to the lack of radiologic signs. Types IV and V require anatomic reduction to prevent articular incongruity and osseous bridging across the physis. There are additional rare types of Salter-Harris fractures, types VI through IX. Type VI is an injury to the perichondral ring of LaCroix, a fibrous band that is continuous with the periosteum and a potential reservoir for growth-plate germ cells. Type VI fracture is seen in injuries about the medial malleolus and often requires later reconstructive surgery (Green and Swiontkowski, 2008).

Although 30% of Salter-Harris fractures result in growth disturbances, only 2% result in significant functional disturbance (Moore and Smith, 2009).

Range-of-Motion Examination

Range of motion is the normal range, flexion, extension, and rotation of a joint. Joint hypermobility is the ability of the joint to move beyond its normal range. Hypermobility of joints generally does not cause problems, although there is a slight increase in dislocation and sprain of the involved joint. The normal values of joint motion are age related, which must be kept in mind (e.g., external hip rotation is greatest in early infancy). Passive range of motion, in which the examiner moves the joint, provides information about joint mobility and stability. It can also provide information about the limits of tendons and muscles that are contracted. Active range of motion, in which the child moves the joint, provides information about both muscle and bony structures working together for functional movement.

Limited range of motion can be the result of mechanical problems, swelling, muscle spasticity, pain, infection, injury or arthritis. Note pain, stiffness, limitations or deviations, and rigidity.

Gait Examination

A normal gait cycle consists of the stance phase, during which the foot is in contact with the ground, and the swing phase, during which the foot is in the air. The stance phase is further divided into three major periods: the initial double-limb support, followed by the single-limb stance, then another period of double-limb support. The gait undergoes developmental changes. Walking velocity, step length, and duration of the single-limb stance increase with age, whereas the number of steps taken per minute decreases. A mature gait pattern is well established by 3 years of age. By 7 years of age the gait is that of an adult (Sawyer and Kapoor, 2009).

Observe the child walking without shoes and minimal covering. The stance and swing phases should be compared in both legs, and the range of motion of each joint should be evaluated. Inspect from the front, side, and back as the child walks normally, on his or her toes, and then on the heels. The gait should be smooth, rhythmic, and efficient. Ankle, knee, and hip movements should be symmetric and full with little side-to-side movement of the trunk.

Limping is a disturbance in gait. Abnormal gait can be antalgic or non-antalgic. An antalgic gait is characterized by a shortening of the stance phase to prevent pain in the affected leg. Painful or antalgic gaits serve to reduce stress or pain at the affected area. The trunk shifts to the opposite side to keep balance and reduce stress; the stance phase and stride length are shortened as compensatory mechanisms. Causes of a painful gait include infection, trauma, or acquired disorders. A Trendelenburg gait in which the trunk tips over the affected hip indicates hip disease and might or might not be painful because it also involves muscle weakness around the hip joint.

Posture

To assess posture adequately, the child should be examined undressed to his or her underwear. The examiner needs to look at the child from the front, side, and back.

Galeazzi Maneuver

The Galeazzi sign can signal conditions that cause leg length discrepancies. The Galeazzi maneuver includes flexing the hips and knees while the infant or child lies supine, placing the soles of the feet on the table near the buttocks, and then looking at the knee heights for equality (Fig. 37-3, A). The Galeazzi sign is positive if the knee heights are unequal. However, it is not reliable in children with dislocatable but not dislocated hips or in children with bilateral dislocation.

FIGURE 37-3 Physical findings in congenital hip dislocation. A, Leg length inequality is a sign of unilateral hip dislocation (Galeazzi sign). B, Limitation of hip abduction is often present in older infants with hip dislocation. Abduction of greater than 60 degrees is usually possible in infants. Restriction or asymmetry indicates the need for careful radiologic examination. C, Trendelenburg sign. In single-leg stance the abductor muscles of the normal hip support the pelvis. Dislocation of the hip functionally shortens and weakens these muscles. When the child attempts to stand on the dislocated hip, the opposite side of the pelvis drops. D, Thigh-fold asymmetry is often present in infants with unilateral hip dislocation. An extra fold can be seen on the abnormal side. The finding is not diagnostic, however. It may be found in normal infants and may be absent in children with hip dislocation or dislocatability.

(From Scoles P: Pediatric orthopedics in clinical practice, ed 2, St Louis, 1988, Mosby.)

Barlow Maneuver

The Barlow maneuver dislocates an unstable or dislocatable hip posteriorly (Fig. 37-4, A). The infant is placed in the supine position with knees flexed. The hip is flexed, and the thigh is brought into an adducted position applying downward pressure. With hip instability the femoral head slips/drops out of the acetabulum or can be gently pushed out of the socket; this is termed a positive Barlow. The dislocation should be palpable as this maneuver is performed. The maneuver needs to be done gently in a noncrying neonate to keep from damaging the femoral head. The hips should be examined one at a time. The hip generally spontaneously relocates after release of the posterior force.

FIGURE 37-4 A, Barlow (dislocation) test. The “stabilizing hand” is positioned with the thumb on the symphysis and the fingers on the sacrum. The thumb of the abducting hand is placed on the inner aspect of the thigh and gives lateral pressure to the adductor region while the hand (wrapped around the knee with the index finger on the lateral side of the thigh) provides gentle downward pressure. If there is hip instability, dislocation is palpable as the femoral head slips out of the acetabulum. Diagnosis is confirmed with the Ortolani test. B, Ortolani (reduction) test. With the infant relaxed on a firm surface, the hips and knees are flexed to 90 degrees. The hips are examined one at a time. Grasp the infant’s thigh with the middle finger over the greater trochanter and lift the thigh to bring the femoral head from its dislocated posterior position to opposite the acetabulum. Simultaneously the thigh is gently abducted, reducing the femoral head in the acetabulum. In a positive finding, the examiner senses reduction by a palpable, nearly audible “clunk.” Test one hip at a time for both of these tests.

(From Marcdante KJ, Kliegman RM, Jenson HB et al, editors: Nelson essentials of pediatrics, ed 6, Philadelphia, 2011, Saunders.)

Ortolani Maneuver

The Ortolani maneuver can be done after the Barlow maneuver or separately (see Fig. 37-4, B). The Ortolani maneuver reduces a posteriorly dislocated hip. It is done to reduce a recently dislocated hip and is not done forcefully. The infant is in the supine position with both knees flexed and supported by the thumb and forefinger of the examiner. The thumb is placed near the lesser trochanter, and the pad of the second finger is positioned on the bony prominence of the greater trochanter. The leg is flexed at the hip and then abducted while pushing up with the fingers located over the trochanter posteriorly. The femoral head is lifted anteriorly into the acetabulum. A clunk and a palpable jerk are obtained as the femoral head is relocated. A mild clicking sound is not a positive Ortolani sign. These are common and normal sounds radiating from the knee or ankle that are fine, of short duration, and high pitched (Duderstadt and Schapiro, 2006). Of note, the hip may be dislocated easily only during the first month or two of life. The Ortolani maneuver is most likely to be positive in infants 1 to 2 months old. The examiner should not still be charting “no hip click” on examinations at 6 months old. Dislocation can occur late in infancy. However, if this occurs, the provider will note limited abduction on the side of the dislocation (Fig. 37-5).

FIGURE 37-5 Hip abduction test. The child is placed supine, and the hips are flexed 90 degrees and fully abducted. Although the normal abduction range is quite broad, one can suspect hip disease in any patient who lacks more than 35 to 45 degrees of abduction.

(From Chung SMK: Hip disorders in infants and children, Philadelphia, 1981, Lea & Febiger.)

Klisic Test

The Klisic test provides an observational sign of hip placement. The examiner places the tip of the third finger of one hand over the greater trochanter and the index finger of the same hand on the anterior superior iliac spine. An imaginary line is then drawn between the index and third fingers. Normally, the imaginary line points to the umbilicus. If the hip is dislocated, the imaginary line points halfway between the umbilicus and the pubis (i.e., the line points below the umbilicus). The Klisic sign is another physical assessment marker of dislocation (Hosalkar et al, 2007b) (Fig. 37-6).

FIGURE 37-6 Klisic test.

(From Kliegman RM, Stanton BF, St. Geme JW et al, editors: Nelson textbook of pediatrics, ed 19, Philadelphia, 2011, Saunders.)

Trendelenburg Sign

The Trendelenburg test can be used to identify conditions that cause weakness in the hip abductors. The Trendelenburg sign is elicited by having the child stand and then raise one leg off the ground. If the pelvis (iliac crest) drops on the raised leg side, the sign is positive and indicates weak hip abductor muscles on the side that is bearing the weight. Normally the muscles around a stable hip are strong enough to maintain a level pelvis if one leg is raised (see Fig. 37-3, C). With bilaterally dislocated hips, a wide-based Trendelenburg limp is noted.

Medial (Internal) and Lateral (External) Rotations

The child is placed prone, and the knees are flexed 90 degrees. Medial rotation is measured as the legs are allowed to fall apart as far as possible, using gravity alone or with light pressure. The angle between vertical (0 degree) and the leg position is the medial rotation. It is measured for each leg (Fig. 37-7, A). Asymmetric hip rotation is abnormal. Lateral rotation is measured by allowing the legs to cross while the child is still prone. The angle between vertical and the leg position is measured for each leg (see Fig. 37-7, B). Again, asymmetric hip rotation is abnormal. By 1 year old, a normal child has approximately 45 degrees of internal and external hip rotation.

FIGURE 37-7 Hip rotation in extension. This is measured with the child in prone position and the knee flexed 90 degrees. The lower leg is vertically oriented. This is considered the neutral position. On outward rotation (A), the leg produces internal hip rotation, and on inward rotation (B), the leg produces external hip rotation.

(From Thompson GH: Gait disturbances. In Kliegman RM, editor: Practical strategies in pediatric diagnosis and therapy, Philadelphia, 1996, Saunders.)

Adams Test or the Adams Forward Bend Position

The Adams forward bend test (Adams test) looks for asymmetry of the posterior chest wall on forward bending. This position allows for evaluation of structural scoliosis. The child bends at the waist to a position of 90 degrees back flexion with straight legs, ankles together, and arms hanging freely or with palms together (in a diving position) but not touching the toes or floor (Fig. 37-8). The back is inspected for asymmetry of the height of the curves on the two sides or rib hump; the provider inspects the child’s back by looking at it from the rear and side positions. The examiner should be seated in front of the child to best visually scan each level of the spine. If a rib hump is present, a scoliometer, if available, can be used to measure the angular tilt of the trunk. A spinal rotation greater than 5 degrees measured by placing the scoliometer at the peak of the curvature indicates the need for further evaluation (Duderstadt and Schapiro, 2006). Other characteristics of scoliosis to look for include unequal scapula heights, unequal waist angles, unequal iliac crests or shoulders, asymmetry of the elbow to flank distance, and some deviation of the spine from a straight head-to-toe line. Looking primarily at the straightness of the spine, however, can be misleading because scoliosis involves both rotation and misalignment of the vertebrae. The Adams forward bending position accentuates the rotational deformity of scoliosis.

FIGURE 37-8 Adams position with rib hump of structural scoliosis. Lateral curvature of thoracic and lumbar segments of the spine, usually with some rotation of involved vertebral bodies. Functional scoliosis is flexible; it is apparent with standing and disappears with forward bending. It may be compensatory for other abnormalities, such as leg length discrepancy. Structural scoliosis is fixed; the curvature is visible both on standing and on bending forward. Note rib hump with forward flexions. At greatest risk are females 10 years old through adolescence.

(From Delp MH, Manning RT: Major’s physical diagnosis: an introduction to the clinical process, ed 9, Philadelphia, 1981, Saunders.)

Brachial Plexus Injuries

Epidemiology

Obstetric brachial plexus palsy results from injury to the cervical roots C5-C8 and thoracic root T1. Most injuries are transient, with full return of function occurring in 70% to 92% of cases and prolonged and permanent disability in a small percentage (Zafeirious and Psychogious, 2008). Risk factors for brachial plexus palsies may be divided into three categories: neonatal, maternal, and labor-related factors. Maternal characteristics include diabetes, obesity, maternal age (more than 35 years), and maternal pelvic anatomy. Vacuum extraction or direct compression of the fetal neck during delivery by forceps can cause stretching of the cervical nerve roots and eventually brachial plexus injury. Other causes may include induction of labor, epidural anesthesia, and postdate gestation (Zafeirious and Psychogious, 2008).

Clinical Findings

Differential Diagnosis

The differential diagnosis of upper extremity paralysis in a newborn includes epiphyseal separation of the humeral head, fracture of the clavicle or humerus, septic arthritis of the upper extremity, spinal cord injury, cervical cord lesions, and congenital varicella of the upper limb (Zafeirious and Psychogious, 2008).

Management

A multidisciplinary team approach is ideal with referral to health professionals who specialize in treating brachial plexus injuries. Referral should be made in the first week of life. Therapy is initially conservative; 95% of infants born with obstetric brachial plexus palsy recover complete function with physical therapy only (Zafeirious and Psychogious, 2008). The initial goal of therapy is to maintain passive range of motion, supple joints, and muscle strength. Radiographs should be taken twice yearly for persistent upper plexus palsy. Indications for surgical exploration and reconstruction of the brachial plexus include failure of recovery of elbow flexion and shoulder abduction from the third to the sixth month of life. The spectrum of nerve surgery includes neurolysis neuroma resection, nerve grafting, and nerve transfers.

Complications

Late sequelae include internal rotation contractures, hypoplasia of the arm, altered sensibility, flexion contractures of the elbow, dislocations of the radial head, and psychological and social consequences.

Prognosis

Complete recovery may take up to 2 years. Infants should be examined regularly until either total recovery or an absence of improvement for 2 to 3 years is documented (Zafeirious and Psychogious, 2008).

Clavicle Fracture

Clinical Findings