ESSENTIALS OF DIAGNOSIS & TREATMENT
Many represent normal physiologic growth patterns.
Key to diagnosis is recognition of abnormal patterns that deviate from normal development.
Treatment is varied depending on condition.
Metatarsus adductus, a common congenital foot deformity, is characterized by inward deviation of the forefoot. It is the most common foot abnormality, observed in newborns at a rate of 1–2 per 1000 live births. When the deformity is more rigid, it is characterized by a vertical crease in the medial aspect of the arch. Angulation occurs at the base of the fifth metatarsal causing prominence of this bone. Most flexible deformities are secondary to intrauterine positioning and usually resolve spontaneously. Several investigators have noticed that 10%–15% of children with metatarsus adductus have hip dysplasia; therefore, a careful hip examination is necessary. The etiology of rigid deformities is unknown.
Fully flexible deformity requires no treatment. If the deformity is rigid and cannot be manipulated past the midline, it is worthwhile to perform serial casting, with cast changes in 1- to 2-week intervals, to correct the deformity. Orthoses and corrective shoes do not improve symptoms; however, they can be used to maintain the correction obtained by casting.
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Clubfoot (Talipes Equinovarus)
Classic talipes equinovarus, or clubfoot, requires three features for diagnosis: (1) plantar flexion of the foot at the ankle joint (equinus), (2) inversion deformity of the heel (varus), and (3) medial deviation of the forefoot (adductus) (Figure 26–1). Clubfoot occurs in approximately 1 per 1000 live births. The three major categories of clubfoot are idiopathic, neurogenic, and those associated with syndromes such as arthrogryposis and Larsen syndrome. Infants with a clubfoot should be examined carefully for associated anomalies, especially of the spine. Idiopathic club feet may be hereditary.
Manipulation of the foot to stretch the contracted tissues on the medial and posterior aspects, followed by casting to hold the correction is the preferred treatment. Serial castings are typically performed on a weekly basis for 6–8 weeks. When instituted shortly after birth, correction is rapid. If treatment is delayed, the foot tends to become more rigid within a matter of days. Casting treatment requires patience and experience, but fewer patients require surgery when attention is paid to details of the Ponseti technique. If there is remaining equinus, surgery may be required in the form of a percutaneous Achilles tenotomy in order to achieve full correction. After full correction is obtained, a night brace is necessary for long-term maintenance of correction. Recent studies indicate that there is poor compliance with brace use following intervention with the Ponseti technique; however, many patients undergoing this treatment are able to independently ambulate only 2 months later than infants with no deformity. The French method is another nonsurgical method of treatment, which is commonly performed by physical therapists. The feet are stretched and manipulated several times per week and a combination of taping and a plastic splint are used to hold the correction after each session. If the foot is rigid and resistant to correction through the Ponseti or French techniques, extensive surgical release and correction are occasionally needed to improve the functional position of the foot. Approximately 15%–50% of patients require a surgical release.
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Flatfoot is normal in infants. If the heel cord is of normal length, full dorsiflexion is possible when the heel is in the neutral position. As long as the heel cord is of normal length and a longitudinal arch is noted when the child is sitting in a non–weight-bearing position, a normal arch will generally develop.
Younger children who are male, obese, and have excessive joint laxity are more likely to be flatfooted. Around 15% of flatfeet do not resolve spontaneously. There is usually a familial incidence of relaxed flatfeet in children who have no apparent arch. In any child with a shortened heel cord or stiffness of the foot, other causes of flatfoot such as tarsal coalition (congenital fusion of the tarsal bones) should be ruled out by a complete orthopedic examination, radiographs and advanced imaging.
For an ordinary correctable flatfoot, no active treatment is indicated unless calf or leg pain is present. In children who have leg pains attributable to flatfoot, a supportive shoe, such as a good-quality sports shoe, is useful. An orthotic that holds the heel in neutral position and supports the arch may relieve discomfort if more support is needed. An arch insert should not be prescribed unless passive correction of the arch is easily accomplished; otherwise, the skin over the medial side of the foot will be irritated. Surgical correction can be done; however, surgery has been found to only improve symptoms associated with shoe or brace wear such as pain, calluses or skin breakdown, with limited improvement of the planovalgus deformity.
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Talipes calcaneovalgus is characterized by excessive dorsiflexion at the ankle and eversion of the foot (Figure 26–2). This disorder can be associated with posteromedial bowing of the tibia and is due to intrauterine position and is often present at birth. The deformity occurs in 0.4-1–0 per 1000 live births.
Excessive dorsiflexion and eversion of the foot in talipes calcaneovalgus.
Treatment consists of passive exercises, such as stretching the foot into plantar flexion. With or without treatment, the deformity usually resolves by age 3–6 months. In rare instances, it may be necessary to use plaster casts to help with manipulation and positioning. Complete correction is the rule.
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Cavus foot consists of an unusually high longitudinal arch of the foot. It may be hereditary or associated with neurologic conditions such as poliomyelitis, hereditary sensory motor neuropathies, tethered spinal cord, and diastematomyelia (congenital splitting of the spinal cord). Typically there is an associated contracture of the toe extensors, producing a claw toe deformity in which the metatarsal phalangeal joints are hyperextended and the interphalangeal joints acutely flexed. Cavus foot presents with diffuse and localized pain in the lower legs and is commonly associated with an inflexible foot deformity. Any child presenting with progressive cavus feet should receive a careful neurologic examination as well as radiographs and magnetic resonance imaging (MRI) of the spine.
Conservative therapy, such as an orthotic to realign the foot, can be effective in milder cases. In symptomatic cases, surgery may be necessary to lengthen the contracted extensor and flexor tendons and to release the plantar fascia and other tight plantar structures, but tethered cord or other spinal anomalies should be addressed first. Associated varus heel deformities cause more problems than the high arch.
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With a prevalence of 23%–35%, hallux valgus is the most common forefoot deformity. The etiology is unknown. Adolescents may present with lateral deviation of the great toe associated with a prominence over the head of the first metatarsal. Around 60% of patients have a family history of this condition. The deformity is painful with shoe wear and almost always relieved by fitting shoes that are wide enough in the toe area. Since further growth tends to cause recurrence of the deformity, surgery should be avoided in the adolescent.
Therapeutic treatments are aimed at correcting the muscular and weight-bearing forces that act on the joint. While conservative treatment provides symptomatic relief, it does not reverse the natural history, as these deformities will typically continue to progress until corrected surgically. A high percentage of these patients ultimately have surgery in adulthood due to a continued progression of the deformity. Surgical treatment should be delayed until the patient is mature, due do the risk of remodel and recurrence of deformity. Surgery leads to satisfactory outcomes in 95% of patients.
Genu varum (bowleg) is normal from infancy through 3 years of age. The alignment then changes to genu valgum (knock-knee, Figure 26–3) until about age 8 years, at which time adult alignment of 5–9 degrees of anatomic valgus is attained. If bowing persists beyond age 2, increases rather than decreases, occurs in only one leg, or if a patient is knock-kneed in association with short stature, the patient should be referred to an orthopedist. Genu varum is usually secondary to tibial rotation (Blount disease, Figure 26–4), while genu valgum may be caused by skeletal dysplasia or rickets.
Blount’s disease with genu varum.
Individuals with genu varum may be at a greater risk for falling as the deformity increases the normal postural sway in the mediolateral direction. Bracing may be appropriate. An osteotomy may be necessary for severe problems such as occurs in Blount disease (proximal tibial epiphysial dysplasia).
TIBIAL TORSION & FEMORAL ANTEVERSION
“Toeing in” in small children is a common parental concern. Tibial torsion refers to rotation of the leg between the knee and the ankle. Internal rotation amounts to about 20 degrees at birth but decreases to neutral rotation by age 16 months. The deformity may be accentuated by laxity of the knee ligaments, which allows excessive internal rotation of the leg in small children. This condition is largely self-limiting and usually resolves spontaneously with further growth and development. Toeing in beyond age 2 or 3 years is usually secondary to femoral anteversion, characterized by more internal rotation of the hip than external rotation. This femoral alignment decreases toward neutral during growth.
Treatment for tibial torsion is focused on educating the families to the benign nature and expected resolution with observation. In older children suspected to have femoral anteversion, there is no long-term improvement with treatment with shoes or braces. Osteotomy for rotational correction is occasionally required when symptoms such as knee and hip pain are present. However, the vast majority go on to resolve spontaneously.
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DEVELOPMENTAL DYSPLASIA OF THE HIP JOINT
Developmental dysplasia of the hip (DDH) encompasses a spectrum of conditions where an abnormal relationship exists between the proximal femur and the acetabulum. In the most severe condition, the femoral head is not in contact with the acetabulum and is classified as a dislocated hip. In a dislocatable hip, the femoral head is within the acetabulum but can be dislocated with a provocative maneuver. A subluxatable hip is one in which the femoral head comes partially out of the joint with a provocative maneuver. Acetabular dysplasia is used to denote insufficient acetabular development and is a radiographic diagnosis. Congenital dislocation of the hip more commonly affects the left hip, occurring in approximately 1%–3% of newborns. At birth, both the acetabulum and femur are underdeveloped (Figure 26–5).
Radiographic evidence of underdeveloped acetabulum and femur in developmental dysplasia of the hip.
Clinical diagnosis of dislocations in newborns is dependent on demonstrating the instability of the joint by placing the infant on his or her back and obtaining complete relaxation. As these clinical signs can be subtle, they can be easily missed with a crying or upset infant. The examiner’s long finger is placed over the greater trochanter and the thumb over the inner side of the thigh. Both hips are flexed 90 degrees and then slowly abducted from the midline, one hip at a time. With gentle pressure, an attempt is made to lift the greater trochanter forward. A feeling of slipping as the head relocates is a sign of instability (Ortolani sign). When the joint is more stable, the deformity must be provoked by applying slight pressure with the thumb on the medial side of the thigh as the thigh is adducted, thus slipping the hip posteriorly and eliciting a palpable clunk as the hip dislocates (Barlow sign, Figure 26–6). Limited hip abduction of less than 60 degrees while the knee is in 90 degrees of flexion is believed to be the most sensitive sign for detecting a dysplastic hip. Asymmetrical skin folds are present in about 25% of normal newborns and therefore are not particularly helpful to diagnosing hip dislocation.
Ortolani/Barlow examination technique.
The signs of instability become less evident after the first month of life. If the knees are at unequal heights when the hips and knees are flexed, the dislocated hip will be on the side with the lower knee. This is called a Galeazzi sign. If dysplasia of the hip has not been diagnosed before the child begins to walk, there will be a painless limp and/or a lurch to the affected side. When the child stands on the affected leg, a dip of the pelvis will be evident on the opposite side, due to weakness of the gluteus medius muscle. This is called the Trendelenburg sign and accounts for the unusual swaying gait. As a child with bilateral dislocation of the hips begins to walk, the gait is waddling.
Clinical signs of instability are more reliable than radiographs for diagnosing developmental dislocation of the hip in the newborn. Ultrasonography is most useful in newborns, and can be helpful for screening high-risk infants, such as those with breech presentation or positive family history. Radiological examination becomes more valuable after the first 6 weeks of life, with lateral displacement of the femoral head being the most reliable sign. In children with incomplete abduction during the first few months of life, a radiograph of the pelvis is indicated.
Dysplasia is progressive with growth unless the instability is corrected. If the dislocation is corrected in the first few weeks of life, the dysplasia can be completely reversible and a normal hip will more likely develop. If the dislocation or subluxation persists with age, the deformity will worsen until it is not completely reversible, especially after the walking age. For this reason, it is important to diagnose the deformity and institute treatment early.
A Pavlik harness, which maintains reduction by placing the hip in a flexed and abducted position, can be easily used to treat dislocation or dysplasia diagnosed in the first few weeks or months of life (Figure 26–7). In order to be safely treated in a Pavlik harness, hips must be manually reducible with only gentle manipulation. Forced abduction, or reduction requiring extremes of motion for stability, can lead to avascular necrosis of the femoral head and is contraindicated. The use of double or triple diapers is ineffective. An orthopedic surgeon with experience managing the problem is best to supervise treatment.
A Pavlik harness used to treat developmental dysplasia of the hip.
If a hip cannot be reduced and have a stable reduction with Pavlik treatment, then a closed reduction with arthrogram is appropriate treatment. A hip spica cast is used after reduction. If the hip is not stable within a reasonable range of motion after closed reduction, open reduction is indicated.
Treatment is still possible in the first 2 years of life, but the results are not as good as with early treatment. In patients older than 2 years, more aggressive procedures like osteotomies and operations to correct the deformities of the acetabulum and femur, as well as open reduction, are often necessary to create a more normal orientation and shape of the hip joint. Older children, as well as children who are bilaterally affected, are more likely to experience complications from more extensive procedures.
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SLIPPED CAPITAL FEMORAL EPIPHYSIS
Slipped capital femoral epiphysis (SCFE) is caused by displacement of the proximal femoral epiphysis due to disruption of the growth plate (Figure 26–8). The head of the femur is usually displaced medially and posteriorly relative to the femoral neck. This condition is most commonly seen in adolescent, obese males. It occurs when stress increases across the proximal femoral physis (growth plate) or resistance to shear is reduced. Factors that can lead to this increase in stress or decrease in resistance include endocrine disorders, obesity, coxa profunda (a deep acetabular socket), and femoral or acetabular retroversion. Retroversion of the femur occurs when the proximal femoral segment is angled posteriorly relative to the shaft of the femur. Acetabular retroversion refers to when the alignment of the opening of the acetabulum does not face the normal anterolateral direction, but inclines more posterolaterally.
Radiographic evidence of slipped capital femoral epiphysis.
Clinically, SCFE is classified as stable or unstable. SCFE is considered stable if the child is able to bear weight on the affected extremity. In unstable SCFE, the child is unable to bear weight. An increased rate of avascular necrosis is correlated with the inability to bear weight.
Acute SCFE occasionally occurs following a fall or direct trauma to the hip. More commonly, vague symptoms occur over a protracted period in an otherwise healthy child who presents with pain and limp. The pain can be referred into the thigh or the medial side of the knee, making examination of the hip joint important in any obese child complaining of knee pain. Physical examination consistently reveals a limitation of internal rotation of the hip. The diagnosis may be clearly apparent only in the lateral radiographic view of the hip or frog lateral of the pelvis.
Initial management consists of making the patient non–weight bearing on crutches and immediate referral to an orthopedic surgeon. Treatment is based on the same principles that govern treatment of any fracture of the femoral neck: the head of the femur is internally fixated in situ to the neck of the femur and the physeal fracture allowed to heal. In situ fixation without closed reduction is considered the standard of care for SCFE treatment due to the high risk of AVN of the femoral head associated with attempted closed reduction. For a SCFE that is significant in terms of displacement, specialized centers are beginning to perform open reduction through a surgical dislocation of the hip, but due to the risk of avascular necrosis of the femoral head, this should only be employed by an orthopedic surgeon with experience in this procedure.
The long-term prognosis is guarded because most of these patients continue to be overweight and overstress their hip joints. Follow-up studies have shown a high incidence of premature degenerative arthritis, even in those who do not develop avascular necrosis. The development of avascular necrosis almost guarantees a poor prognosis, because new bone does not readily replace the dead bone at this late stage of skeletal development. About 30% of patients have bilateral involvement, which may occur as late as 1 or 2 years after the primary episode.
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