Musculoskeletal Conditions in Children
Distinguish among the characteristics of metatarsus adductus, metatarsus varus, and clubfoot.
Develop treatment options for flexible metatarsus adductus and make appropriate referrals for inflexible metatarsus adductus.
Describe the Adam’s forward bend test for detection of scoliosis in adolescents.
Identify the degree of scoliosis curvature that warrants consideration of brace therapy.
Describe 2 common overuse injuries that occur in adolescent baseball pitchers.
Describe the typical presentation of tibial tuberosity apophysitis (Osgood-Schlatter disease).
Explain why juvenile idiopathic arthritis is now the current name for conditions that include what was formerly called juvenile rheumatoid arthritis.
Assess for need of uveitis screening in patients who have juvenile idiopathic arthritis.
Key Practice Recommendations
- Treat clubfoot by casting and bracing according to the Ponseti method.
- Base decisions about managing adolescent idiopathic scoliosis on future growth potential, curve severity, and curve pattern.
- Recommend cessation of all throwing activities for approximately 3 months in a young baseball pitcher diagnosed with a proximal
humeral physeal stress injury.
- Recognize that a young athlete with low back pain with extension and normal lumbar x-rays might have a spondylolysis.
- Screen for uveitis in patients who have juvenile idiopathic arthritis.
- Strength of evidence: SORT A
Source: McKee-Garrett TM. Lower extremity positional deformations. UpToDate; February 2010.
- Strength of evidence: SORT B
Source: Scherl SA. Treatment and prognosis of adolescent idiopathic scoliosis. UpToDate; May 2010.
- Strength of evidence: SORT C
Source: Carson WG, Gasser SI. Little leaguer’s shoulder—a report of 23 cases. Am J Sports Med. 1998;26(4):575-580.
- Strength of evidence: SORT C
Source: Nigrovic PA. Back pain in children and adolescents: Overview of causes. UpToDate; January 2010.
- Strength of evidence: SORT C
Source: Cassidy J, Kivlin J, Lindsley C, et al. Ophthalmologic examinations in children with juvenile rheumatoid arthritis. Pediatrics. 2006;117(5):1843-1845. National Guideline Clearinghouse
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If you are anything like me, many of the classic musculoskeletal conditions in children—such as metatarsus adductus and clubfoot, intoeing from tibial torsion or femoral anteversion, scoliosis, and juvenile arthritis—are topics you learned about in medical school but see only occasionally in practice. As a result, you may not feel fully up-to-date on those conditions and end up making referrals to subspecialists when patients in your practice present with them.
In this issue of FP Essentials™, a group of pediatric subspecialists and family physicians who focus their practices on dealing with these conditions provides us with an update on the newest approaches to their diagnosis and management, along with a review of the increasingly common issue of overuse athletic injuries in children. Whether they are discussing the newest imaging techniques, the current recommended treatments, or the newest disease classification systems (eg, the term juvenile idiopathic arthritis is now used instead of juvenile rheumatoid arthritis), I found that I learned something on every page of this FP Essentials™. I’m hopeful that you will find it similarly useful.
Barry D. Weiss, MD, FAAFP, Medical Editor
Professor of Family and Community Medicine
University of Arizona College of Medicine, Tucson
Musculoskeletal conditions in children are common presenting concerns in a family medicine practice. The National Center for Health Statistics 2003 survey reported that musculoskeletal conditions in children and adolescents account for approximately 500,000 hospitalizations and 12 million visits to US physicians each year.
With the growing epidemic of childhood obesity, it is critical that children and adolescents engage in regular physical activity. But as the medical community encourages more physical activities, some children will experience injuries and some will be limited by a variety of abnormalities of the bone and joints. The ability of physicians to diagnose and appropriately manage injuries and bone and joint conditions is essential for children’s proper growth and development, participation in physical activity and athletics, and psychosocial well-being. Furthermore, physician ability to differentiate these various conditions from normal variants is necessary to guide appropriate referrals to expedite care and to prevent unnecessary consultations.
This edition of FP Essentials™ focuses on common musculoskeletal conditions in children that family physicians will encounter in practice. The text focuses on deformities of the foot and leg that cause intoeing, adolescent idiopathic scoliosis, overuse injuries, and juvenile idiopathic arthritis.
Common Deformities of the Foot and Leg: Current Concepts
Case 1. Kathryn, a 1-day-old girl, is seen for a regular newborn examination in the hospital nursery. The prenatal course was normal, and Kathryn was delivered at term with no complications. Kathryn’s mother Janet is concerned Kathryn’s feet are turned in.
On examination, the only abnormality noted is a mild adduction of the left foot. The foot can be easily abducted beyond the midline and there is no equinus (plantar flexion of the foot relative to the ankle), heel varus, or valgus (inward or outward positioning of the heel). The neurologic examination is normal.
Metatarsus Adductus, Metatarsus Varus, and Clubfoot
Epidemiology and Etiology
Metatarsus adductus is the most commonly seen foot deformity in newborns, with an incidence of 1 in 100 to 1 in 5,000 live births., It is thought to be caused by intrauterine positioning.
Infants with metatarsus adductus typically are identified by a family physician during the newborn examination or by parents who note that the baby’s feet turn in as presented in Case 1 (Figure 1).
Infant Metatarsus Adductus
Physical examination reveals an adducted forefoot, which is turned toward the midline, and the lateral border of the foot is curved with the heel, or hindfoot in neutral position. If the foot can be abducted beyond midline on examination, the metatarsus adductus is termed flexible. If it can be abducted, but not beyond midline, it is considered partially flexible. If the foot does not move at all with corrective maneuvers, it is termed inflexible (Table 1).
|Finding||Metatarsus Adductus||Metatarsus Varus||Clubfoot|
In-turning of forefoot (adduction)
Out-turning of heel (heel valgus)
In-turning of heel (heel varus)
Down-pointing of ankle (equinus)
Plantar flexion of first metatarsal
Further evaluation, including routine x-rays or other imaging, is not indicated unless the diagnosis is in doubt. Such doubt occurs when there is concern about the possibility of metatarsus varus, which is discussed below.
Treatment depends on whether the deformity is flexible, and most metatarsus adductus deformities are flexible. Flexible metatarsus adductus is treated conservatively with gentle stretching of the foot with each diaper change or sometimes with no treatment at all. In a study of 335 children with flexible metatarsus adductus monitored in this way,all had improved by ages 3 to 4 years.
If the feet are not flexible or the diagnosis of metatarsus varus is suspected, referral to a pediatric orthopedic subspecialist should be made to determine if casting is appropriate. In a study of children with metatarsus adductus that was only partially flexible or inflexible, 95% of patients treated with serial casting for 6 to 8 weeks had normal, nonpainful feet in adulthood.
Metatarsus varus, also known as skewfoot, is similar to metatarsus adductus in that forefoot adduction is present. But with metatarsus varus there also is concomitant hindfoot valgus, or turning out of the heel.
Metatarsus varus is significantly less common than metatarsus adductus, but the true incidence is not known, in part because the amount of hindfoot valgus required to classify a foot as having true metatarsus varus, rather than simply metatarsus adductus, has not been strictly defined.In one large study of feet with an adducted forefoot, only 12 of 2,818 patients were considered to have metatarsus varus and the remainder had simple metatarsus adductus.
Intrauterine positioning can play a role in the etiology of metatarsus varus because it typically is present at birth but not seen in preterm infants.However, abnormal insertion of the tibialis anterior or increased muscle activation of the tibialis anterior, tibialis posterior, and adductor hallucis muscles compared with the peroneal muscles also can contribute to the condition.
As in metatarsus adductus, the parent or family physician typically notes the foot deformity of metatarsus varus shortly after birth, though parents typically only note the “turning in of the foot” and will not appreciate the hindfoot valgus.
Depending on the severity of the deformity, physical examination reveals varying degrees of forefoot adduction and hindfoot valgus. In mild deformities, the hindfoot valgus can be difficult to identify. In severe deformity, the foot can take on an S-shaped appearance, with marked incurving of the forefoot and outcurving of the heel, leading early investigators to refer to the deformity as serpentine foot.Table 1).As with metatarsus adductus, the examiner might note varying degrees of flexibility (
In contrast to simple metatarsus adductus, for which x-rays are not routinely indicated, x-rays should be obtained when a foot has metatarsus adductus and hindfoot valgus. Anteroposterior (AP) and lateral foot x-rays in the weight-bearing or simulated weight-bearing position demonstrate forefoot adduction, midfoot abduction, and hindfoot valgus. Ankle x-rays also are useful if the patient is of walking age, to evaluate for possible accompanying ankle deformities, such as ball-and-socket ankle joint.
The natural history and best management of metatarsus varus have not been clearly defined, but the condition typically seems to be more resistant to conservative management than metatarsus adductus. Patients with suspected metatarsus varus should be referred to a pediatric orthopedic subspecialist, who typically will undertake serial casting for cases in which the deformity is mild and nonrigid.If there is no response to serial casting within 6 to 8 weeks or the deformity progresses, surgical intervention can be considered.
Children who were not treated during infancy or who had unsuccessful treatment might have pain and difficulty with shoe wear when they are older. These children might require surgical intervention. In older patients with no symptoms, intervention is not recommended.
For children being considered for surgery, it is important to note that there is no agreement on the most effective surgical method and/or timing for surgery.Thus, referral should be made to a pediatric orthopedic subspecialist who is familiar with and experienced with the disorder.
Clubfoot (Figure 2) occurs in 1 to 2 of 1,000 live births in the United States, making it less common than metatarsus adductus. It occurs more frequently in boys than girls, and it is bilateral approximately half the time.
The cause of clubfoot is unknown and likely multifactorial. Twin studies show that genetics probably play a role. Clubfoot affects 32.5% of monozygotic twins but only 2.9% of dizygotic twins. Furthermore, 24.4% of all infants with clubfoot have an affected family member., Many other theories about clubfoot etiologies have been proposed including vascular anomalies, anomalous musculature, histologic abnormalities, and intrauterine infection. It also can be associated with other conditions, including spina bifida, cerebral palsy, Larsen syndrome (hypermobility, congenital dislocations, brachycephaly, and cleft palate), and arthrogryposis (multiple joint contractures).
Intrauterine factors also can play a role in clubfoot. One study reported an 11 times increase (1.1% vs 0.1%) in clubfoot rates in infants whose mothers underwent amniocentesis before 11 weeks’ gestation. If an amniotic fluid leak occurred at the time of amniocentesis, the risk increased to 15%, despite the fact that persistent oligohydramnios was not seen on any follow-up ultrasound.When amniocentesis is obtained after 15 weeks’ gestation, there is no increased incidence of clubfoot, suggesting a possible susceptible time period during embryonic development.
Clubfoot often is diagnosed prenatally on routine prenatal ultrasound examination, but false-positives occur. False-positives are rare when clubfoot is identified on ultrasound at 20 weeks’ gestation, but are as high as 40% on third trimester ultrasounds. Patients identified with clubfoot on ultrasound at 20 weeks also have a 50% to 70% association with abnormalities of the neurologic, cardiac, and/or urogenital systems.When a prenatal diagnosis has not been made, parents and medical staff discover the deformity at the time of birth.
On physical examination, clubfoot involves a constellation of findings, including equinus at the ankle (pointed down); varus at the heel (turned in); midfoot adduction; and plantar-flexed (down-pointing) first metatarsal, creating a high arch. Midfoot and heel creases often are noted (Table 1).
Clubfoot is identified clinically; x-rays typically are not needed to make the diagnosis. Reproducible x-ray images of clubfoot also are difficult to obtain because of the stiff, deformed nature of the foot, lack of ossification of the tarsal bones, and difficulty holding the foot in position.Nonetheless, AP and lateral x-rays might be useful, if the foot has unusual characteristics or surgical treatment is being considered, to further evaluate and assess the degree of deformity.
Many grading/classification systems have been introduced to aid in determining the severity of deformity and most appropriate treatments for clubfoot. The most commonly used systems are those developed by Pirani and Dimeglio (Table 2).
|Factors Evaluated||Pirani System (Severity Scored 0 to 1)||Dimeglio System (Severity Scored 0 to 4)|
Curvature of lateral foot border
Degree of supination/pronation
Forefoot adduction relative to hindfoot
Heel position in frontal plane
Medial foot crease
Palpable head of talus
Palpable calcaneal tuberosity
Information from Pirani S, Outerbridge HK, Sawatzky B, et al. A reliable method of clinically evaluating a virgin clubfoot. Brussels: 21st SICOT Congress; 1999; Dimeglio A, Bensahel H, Souchet P, et al. Classification of clubfoot. J Pediatr Orthop B. 1995;4(2):129-136; Flynn JM, Donohoe M, Mackenzie WG. An independent assessment of two clubfoot-classification systems. J Pediatr Orthop. 1998;18(3):323-327.
The Piranisystem gives a score of 0, 0.5, or 1.0 to 3 physical examination features of the hindfoot and 3 features of the forefoot. Each feature is assigned a score and the values are added with higher scores indicating more severe deformity. Higher scores increase the chance that the patient will need tenotomy of the Achilles tendon to relieve equinus.
The Dimeglioscore uses a 20-point scale. From 0 to 4 points are assigned (again with higher scores indicating more severe deformity) to each of the following 4 factors: amount of equinus, heel varus, supination/ pronation, and adduction, which are present after maximal reduction of the deformity. One additional point is added for presence of a posterior crease, a medial crease, a high arch (cavus), and/or poor muscle condition. A total score of 0 to 5 is grade 1, and feet in this category are felt to have positional deformities, not true clubfoot; they resolve with expectant management. A score of 5 to 10 is grade 2, which is considered a moderate deformity that is partially reducible and typically responds to casting alone. A score of 10 to 15 is grade 3, which is considered severe and typically treated with casting and tenotomy. Finally, a score of 15 to 20 is grade 4, which is severe and typically associated with another deformity, such as spinal dysraphism or tethered cord syndrome.
These classification systems have been evaluated by independent groups and were found to have good interobserver reliability after an initial learning phase of evaluating 15 feet.
The Ponsetimethod is the most well-studied and widely used form of nonsurgical therapy. Clubfoot should be treated with casting and bracing according to the Ponseti method. It involves serial casting, plus percutaneous Achilles tendon-lengthening (tenotomy) if needed for correction of equinus. Treatment should be started within 7 to 10 days of birth; some physicians start casting before the infant is released from the hospital. Cast changes typically are obtained once a week for 5 to 8 weeks.
The first cast aims to correct the cavus deformity by positioning the forefoot in proper alignment with the hindfoot.Subsequent casting corrects the adduction, varus, and equinus deformities. To fully correct equinus, an Achilles tenotomy is performed in most patients just before the last cast application. Tenotomy can be performed with local anesthesia in the office setting or general anesthesia in the operating room. The last cast should remain in place for 3 weeks.
After casting is complete, bracing is used to prevent recurrence of clubfoot. The brace is worn full time for 3 months, then at night and during naps until ages 3 to 4 years. Parents should be counseled regarding the importance of bracing, because recurrence is likely if the bracing regimen is not followed. The most common etiology for relapse of clubfoot is nonadherence to bracing. Only 6% of infants whose families are fully adherent to bracing experience relapse, compared with an 80% relapse rate in those who are nonadherent.When relapse occurs, casting should be repeated until the deformity is corrected. Repeat Achilles tenotomy occasionally is also needed.
As previously discussed, treatment is ideally begun within the first 7 to 10 days of life. However, untreated clubfoot in an older child might still respond to a casting regimen and tenotomy.
The long-term outlook for patients with clubfoot is optimistic. A 30-year follow-up study of patients treated with the Ponseti method revealed that 78% of patients reported good or excellent outcomes in terms of pain and functional limitation, compared with 85% of patients who did not have a congenital foot deformity.In patients with unilateral deformity, the calf size of the affected leg can be expected to be smaller than that of the unaffected leg. Also, the clubfoot typically is shorter than the normal foot.
Other conservative techniques, including different types of serial casting, splinting, stretching, and taping, also have been described. However, they have not had the reproducible success of the Ponseti method.The most well-known of these is the French physiotherapy method, which uses daily stretching, taping, and use of a continuous passive motion machine. Good results also have been reported with this method.
Surgical repair of clubfoot by soft tissue release also has been advocated, but has not led to good long-term results, with patients reporting foot pain, stiffness, muscle weakness, and impaired quality of life at 10 to 30 years follow-up., In fact, investigators report better long-term outcomes in patients treated with the Ponseti method compared with patients treated by the same orthopedic subspecialist with extensive soft-tissue release, though prospective trials comparing the 2 methods are not available. Nonsurgical treatment is now considered the standard of care for a newborn or young child with clubfoot.
Intoeing After the Neonatal Period
Internal tibial torsion (ITT) and increased femoral anteversion (FA) are common causes of intoeing in older children. ITT is more common in toddlers and FA typically is seen during early childhood.
Classically, lower extremity alignment changes from birth, when legs are in approximately 15° of varus (internal rotation), to mild valgus (external rotation) by age 2 years, and then maximum valgus of 8° to 10° noted by ages 3 to 4 years. Valgus then decreases to approximately 6° to 7° by ages 6 to 7 years.Although valgus should be seen after age 2 years, children with ITT and FA have a varus deformity (ie, intoeing).
Internal Tibial Torsion
Epidemiology and Etiology.
ITT is bilateral in two-thirds of affected children. It typically is attributed to intrauterine positioning, though some think that sleeping in the prone position after birth and sitting with the feet tucked under the buttocks also can contribute to the deformity.
Parents typically report that the child’s feet or legs turn in. They also might report that the child trips frequently. On physical examination, alignment of the thigh down to the foot shows a gradual internal rotation, whereas the normal thigh-foot angle is 5° to 10° of external rotation.
ITT typically resolves spontaneously and treatment, including bracing or casting, is not typically needed. However, parents should discourage children from sitting on their feet. Parents also should be reassured that functional limitations are unlikely, and any residual intoeing is associated with the ability to run faster.
Surgery (tibial osteotomy) to correct the deformity should only be considered for children who are older than 8 years, have functional issues (eg, frequent falling causing facial injury), or a thigh-foot angle more than 3 standard deviations above the mean, which corresponds to a thigh-foot angle greater than 15° of internal rotation.
Increased FA typically is bilateral and idiopathic. The etiology is unknown. A familial association can be identified in some patients.
The child and parent often present with concerns about the appearance of the legs or about clumsiness or awkward gait. On physical examination, increased internal rotation and decreased external rotation of the hips are noted.
When assessing gait during the physical examination, the patellae point inward and the child might appear uncoordinated. A neurologic examination should be obtained. Asymmetry in the range of motion, spasticity, hyperreflexia, or abnormal findings at other joints should raise concerns regarding a possible neuromuscular disorder, most commonly cerebral palsy.
The family should discourage the child from W-sitting. The family should be reassured that intoeing from FA typically increases until age 5 years and then typically resolves by age 8 years. Increased FA might be related to future reports of patellofemoral pain, but has not been associated with increased risk of degenerative joint disease.
Surgical intervention (femoral osteotomy) might be considered if the child is older than 8 years, has significant cosmetic or functional deformity, anteversion greater than 50°, and internal rotation of the hip exceeding 80°.
Case 1, cont’d. Kathryn has feet turned in, consistent with metatarsus adductus. Because the foot can be easily abducted past neutral, the deformity is flexible and should resolve without intervention. Thus, referral is not needed. Reassurance should be provided to the parents and reevaluation undertaken at subsequent examinations is appropriate to monitor correction. If the foot is stiff or hindfoot valgus is present, referral to a pediatric orthopedic subspecialist is warranted for possible serial casting or surgical intervention.
Update on Adolescent Idiopathic Scoliosis
Case 2. Tracy brings her 12-year-old daughter Lydia in for a well child examination. Tracy’s only concern is that Lydia’s back seems uneven. Lydia does not have back pain and is otherwise healthy. The examination is normal except for back asymmetry noted on a forward flexion test. X-rays reveal a 35° lateral curvature of the spine.
Scoliosis is defined as a lateral curvature of the spine, measured as more than 10° on x-ray, typically associated with rotation. The 3 major types of scoliosis are congenital, neuromuscular, and idiopathic. Congenital scoliosis results from an abnormality in vertebral development, such as hemivertebrae, in which the abnormal shape of the vertebral bodies leads to deformity. Neuromuscular scoliosis arises from abnormal muscle forces and might not have a rotational component.
Idiopathic scoliosis, the most common type, is divided into 3 subcategories based on age of onset. Infantile idiopathic scoliosis affects patients younger than 3 years, juvenile idiopathic scoliosis appears in children between ages 3 and 10 years, and adolescent idiopathic scoliosis (AIS) develops in skeletally immature patients older than 10 years.
Adolescent idiopathic scoliosis is the most common form of idiopathic scoliosis. Approximately 2% to 4% of adolescents (between ages 10 and 16 years) have some degree of spinal curvature,, whereas curves of larger magnitude are less common. The prevalence of curves greater than 30° is approximately 0.2% and is less than 0.1% for curves greater than 40°. Small-magnitude curves have equal prevalence between girls and boys, but for curves of larger magnitude, girls show clear predominance over boys by approximately 4:1.
The cause of AIS is thought to be multifactorial. Genetics is thought to play a role, but the specific mode of inheritance remains undetermined.Twin studies demonstrate a high concordance rate of approximately 73% in monozygous twins and 36% in dizygous twins. Current research is under way to identify a panel of genetic markers that can help predict risk of curve progression severe enough to require surgical intervention.
An understanding of the natural history of AIS (ie, risk for and rate of curve progression) is helpful for making treatment recommendations. Curve progression is related to a patient’s sex (more progression in girls), future growth potential, curvature severity, and curve pattern.,,,
Future Growth Potential
Curve progression is most likely to occur during periods of skeletal growth, so adolescents with more remaining growth potential are at greater risk of progression. Remaining growth potential is determined by a combination of factors, including menarchal status, age at onset, and, most importantly, Risser sign (Figure 3), which is a measure of skeletal maturity. Risser sign shows the amount of ossification of the iliac crest, which begins anterolaterally and progresses posteromedially with maturity. Risser grade is determined by dividing the iliac crest into quadrants: no ossification = Risser grade 0; 25% = Risser grade 1, 50% = Risser grade 2; 75% = Risser grade 3; 100% = Risser grade 4; and complete bony fusion = Risser grade 5.
Risser Sign Method of Skeletal Age Determination
As a general rule, patients with a Risser grade of 2 or less have 3 times the risk of curve progression compared with patients with Risser grade of 3 or more.Menstruation status also is a consideration, because the risk of curvature progression for a Risser grade 2 patient before menarche is 50%, whereas after menarche the risk is reduced to less than 20%.
Curve severity also predicts the risk of progression. For example, a patient with a curve of less than 30° at skeletal maturity has a low risk of continued progression into adulthood.,In contrast, a curve between 30° and 50° will progress an additional 10° to 15° during adulthood and curves greater than 50° progress 1° to 2° annually.
Severity of curvature combined with skeletal maturity can further predict the risk of future curve progression. For example, curves of 20° to 29° in skeletally immature patients (Risser grade less than 2) have 68% probability of progression, opposed to those closer to maturity (Risser 2 to 4) who have only a 23% chance of progression.
Thoracic curves and double curves have largest the risk of progression. The risk is lower in thoracolumbar curves, and lumbar curves have the lowest risk.
Routine screening for AIS is controversial. Many states mandate scoliosis screening in schools, but the benefit of these programs is unproven. In 1996, the United States Preventive Services Task Force (USPSTF) concluded there was insufficient evidence to make a recommendation for or against screening. Later, in 2004, the USPSTF recommended against screening of asymptomatic adolescents.
In 2008, the American Academy of Orthopaedic Surgeons, Scoliosis Research Society, Pediatric Orthopaedic Society of North America, and American Academy of Pediatrics convened a task force to review the issues related to scoliosis screening. They issued an information statement, concluding that, although screening has limitations, the potential benefits that patients with idiopathic scoliosis receive from early treatment of the deformities can be substantial.The task force recommended that girls be screened twice, at ages 10 and 12 years, and boys once at ages 13 or 14 years, whether conducted as part of a clinic visit or a school screening program.
The recommended screening method is the Adam’s forward bend test (Figure 4), although scoliometer measurements have been used as well. These methods are discussed in subsequent sections.
Adam’s Forward Bend Test
Patients might present for evaluation of scoliosis in different ways, including referrals from school-based screening programs; report of deformity noted by themselves, family, or friends; or an incidental finding during routine well-child examination. A focused history should include age of onset of curvature; family history of scoliosis; menarchal status; reports of pain; and neurologic changes, including bowel or bladder dysfunction and weakness.
Although back discomfort might be reported, significant back pain is not typical. If present, the patient should be evaluated to rule out other possible etiologies. In a study of 2,442 patients with idiopathic scoliosis, only 23% had back pain at presentation and an additional 9% during the observation period. Nine percent of scoliosis patients with back pain were found to have another underlying condition, such as spondylolysis, spondylolisthesis, Scheuermann kyphosis, syrinx, herniated disk, hydromyelia, tethered cord, or intraspinal tumor.
Physical examination should include the patient’s height, plotted on a growth curve chart standardized for age and sex. Skin should be evaluated for midline abnormalities, such as hemangiomas, hair tufts, and lumbosacral dimpling that can indicate an underlying spinal cord abnormality. Visual inspection of alignment includes observation for both obvious and subtle asymmetry of shoulder height, scapulae, flank creases, iliac crests, and breasts. If a leg length discrepancy is detected, a lift beneath the shorter leg can help correct any compensatory scoliosis. Tanner stage determination can be helpful because peak curve progression occurs during pubertal growth spurt (Tanner stages 2 to 3).
A thorough neurologic examination should be obtained to rule out underlying neurologic etiologies. Reflexes, including abdominal reflex, balance, and lower extremity strength, should be assessed. The abdominal reflex can be helpful to rule out subtle intraspinal pathology. The patient lies supine while the examiner gently strokes the skin on either side of the midline above, at, and below the level of the umbilicus. Contraction of the muscle toward the stimulated side is a normal response.
Adolescent idiopathic scoliosis is a diagnosis of exclusion and, therefore, secondary etiologies of scoliosis should be ruled out. In particular, spinal dysraphism, neuromuscular disorders, and many syndromes (eg, Marfan syndrome) can present with scoliosis, so further evaluation should be pursued for patients with findings suggestive of those conditions or who present before age 10 years.
The Adam’s forward bend test (Figure 4) is the most commonly used test in the office and as part of school-based screening. The patient bends forward at the waist until the spine is horizontal with the floor, palms are placed together and arms extended. The physician evaluates the patient from behind for presence of asymmetry in contour, noted as one side of the back positioned higher than the other.
A scoliometer or inclinometer, similar to a standard carpenter’s level, is another approach to screening. With the patient forward bending, the scoliometer is placed on the back, perpendicular to the spine at the site of maximum prominence. Scoliometer measurements can be helpful in determining when to obtain x-rays or refer a patient, especially if neither is easily accessible. Controversy exists regarding its accuracy and utility beyond Adam’s forward bending alone.
X-rays are not necessary in every patient with suspected scoliosis. Indications for x-ray studies are shown in Table 3. Standard images include upright standing posteroanterior and lateral views.
Obvious significant curvatures on examination
Scoliometer measurements of ≥ 7°
Monitoring for progression in patients previously diagnosed patients
Information from Bunnell WP. Outcome of spinal screening. Spine (Phila Pa 1976). 1993;18(12):1572-1580; Skaggs DL. Referrals from scoliosis screening. Am Fam Physician. 2001;64(1):32, 34-35.
X-rays should be viewed with the heart on the left, as if examining the patient from behind. Curves are then described by the direction of the convexity points and location of the apex vertebrae (the one most deviated from midline). The most common type of curve in AIS is a right thoracic, left lumbar double curve. Leftward curves are uncommon and need further attention because they can be associated with nonidiopathic etiologies. The Risser sign, as described previously, also should be determined from the appearance of the pelvis. X-rays also should be reviewed for other possible underlying etiologies, such as soft tissue masses, wedged or hemivertebrae, vertebral body lucencies, and widening of interpedicular space.
The Cobb angle (Figure 5) is used to quantify the degree of scoliosis curvature. The most tilted vertebrae above and below the apex on posteroanterior x-ray are identified. The angle between intersecting lines, drawn perpendicular to the superior endplate of the top vertebrae and inferior endplate of the bottom vertebrae, forms the Cobb angle. Intraand interobserver reliability is approximately 5°.
Cobb Angle Measurement
Additional imaging, such as magnetic resonance imaging study, is reserved for patients with atypical presentations of AIS. These include left thoracic curve, significant or unusual pain, abnormal neurologic findings, x-ray findings that need clarification, or other red flag signs and symptoms from history and physical examination.
The goal of scoliosis management is to prevent further progression of the curvature. Management decisions are based on curve severity at presentation, pattern and location of curvature, and growth potential of the patient (chronologic age, menarche status, Risser sign). The majority of adolescents will not require intervention, with fewer than 10% of screened individuals requiring active treatment. Management options include observation and nonsurgical or surgical treatment and are summarized in Figure 6.
Algorithm for Management of Adolescent Idiopathic Scoliosis
Skeletally immature patients have the greatest risk for curve progression. Patients in Risser grade 0 to 2 with curves less than 25° can be observed every 3 to 6 months until curvature progresses more than 5° over any time interval, or becomes greater than 25°. Curves between 25° and 40° should be considered for brace therapy, and patients with curves greater than 45° or 50° should be evaluated for possible surgery.
In patients approaching skeletal maturity (Risser grade 3, 4, 5), curves less than 25° can be monitored every 6 to 9 months. Patients should be monitored for at least 1 to 2 years past skeletal maturity. Curves less than 30° at skeletal maturity are not likely to progress at all, and those with curves less than 40° only minimally; these patients can be discharged from care. Those with curves greater than 50° have potential for progression into adulthood and should undergo continued monitoring.Treatment of patients with curves between 40° to 50° varies on a case-by-case basis.
Brace therapy is the most common form of nonsurgical treatment. Bracing typically is recommended for skeletally immature patients with curves between 25° and 40° or curve progression greater than 5° during periodic follow-up appointments. The goal of bracing is to prevent curve progression, not to correct curvature. It is not indicated in skeletally mature patients or in patients with Cobb angle greater than 50°.
Patients treated with a brace should be monitored approximately every 6 months. Bracing is continued until peak growth spurt has concluded (Risser grade 4 or greater or 2 years after menarche for girls, and Risser grade 5 or greater for boys). Bracing is considered successful when the curve does not progress more than 5°.
The underarm, thoracolumbosacral orthosis, known as the Boston brace, is most commonly used and well tolerated because it is easily camouflaged beneath clothing. The Milwaukee brace (cervicothoracolumbosacral orthosis), which includes cervical extension, is more difficult to hide, less tolerable, and typically reserved for patients who have thoracic curves with an apex above T8 or double thoracic curves.
Thoracolumbosacral orthosis braces typically are worn for 16 to 23 hours/day, with removal for athletic activities and bathing. Nighttime braces (eg, Charleston bending brace, Providence) provide a maximal side-bending corrective force, are worn 8 to 10 hours/night, and can be considered in skeletally immature patients with single major curves of 25° to 35° with an apex below T8.,
Bracing therapy is the subject of some controversy with some advocating routine use and others reporting no difference in outcomes compared with observation alone.,,,, Patient adherence likely plays a role in effectiveness of use and can explain differences in outcomes. Currently, the National Institute of Arthritis and Musculoskeletal and Skin Diseases, part of the National Institutes of Health, is sponsoring a multicenter prospective randomized trial, the Bracing in AIS Trial (BrAIST), which compares bracing and observation in skeletally immature patients with curvatures 20° to 40°.
Other nonsurgical treatments include electrical stimulation, physical therapy, chiropractic treatments, and biofeedback. These methods have not yet been proven effective.
The goals of surgical treatment are to prevent progression, achieve maximal permanent correction of deformity, improve appearance, and minimize short- and long-term complications. Surgery typically is indicated in patients with Cobb angles greater than 45°, but additional factors to consider in the decision to proceed with surgery are age, rate and degree of curve progression, and symptoms such as pulmonary compromise. Adolescents can choose to delay surgery until adulthood, but adults tend to have less flexible curves and higher complication rates.
Surgical approaches are evolving from the Harrington rod system widely used in the past. There is now increasing use of pedicle screw instrumentation techniques, which can minimize the number of levels fused. Spinal cord monitoring and motor-evoked potentials accompanying spinal instrumentation have decreased the rate of intraoperative neurologic injury.
Case 2, cont’d. You diagnose Lydia with scoliosis based on a lateral curvature that exceeds 10° on x-ray; her curvature measures 35°. After a thorough history and physical examination to rule out underlying etiologies, further information reveals menarche 2 months ago and Risser grade 2 on x-ray.
Although most adolescents with adolescent idiopathic scoliosis have small curves that only require observation, Lydia’s curve is 35°. This large degree of curvature, combined with her young age and low Risser grade that indicates future skeletal growth potential, predicts substantial risk for curve progression. After discussion, brace therapy is recommended and chosen by the family. You arrange a follow-up examination in 6 months for continued monitoring. Although surgical intervention is unlikely, the family is informed that if the curve increases to more than 45°, surgery might be considered.
Common Overuse Injuries in Child and Adolescent Athletes
Overuse injuries in young athletes are common and represent a growing concern as the number of children participating in organized sports has increased. Children begin athletic training at younger ages than in the past, are training at higher intensity and often year-round, and at times are playing on multiple teams in the same season. This increases the risk of developing overuse injuries, which are thought to occur from repetitive microtrauma that exceeds the capacity of tissue to recover. The young athlete can develop the same overuse injuries as adults, such as tendinopathies, but are additionally at risk of experiencing overuse injuries unique to the developing skeleton.
The skeleton of a young athlete is different from that of an adult, primarily because of the presence of growing bone. Specifically, the physis (growth plate) and the epiphyseal and apophyseal secondary ossification centers are vulnerable to acute and chronic injuries. Epiphyseal ossification centers add length and width to the bone and are found at the ends of the long bones. Apophyseal ossification centers add shape and contour to bones. Although they do not contribute to the final length of the bone, they are the site of muscle-tendon attachments.
Case 3. Riley, a 14-year-old boy, has elbow pain that began several months ago when he was a pitcher in a baseball league, and gradually increased throughout the season. He began pitching at age 11 years and throws 180 pitches/week. This season he has been working on his curve ball.
Proximal Humeral Physeal Stress Injury
A skeletally immature athlete can develop a stress injury of the proximal humeral physis from repetitive overhead arm movements. This condition often goes by the name Little Leaguer’s shoulder, because it typically occurs in adolescent baseball pitchers who compete at a high level. This injury also has been reported in tennis and volleyball players, swimmers, and gymnasts.
The exact mechanism of injury is unknown, but is thought to result from the concentration of forces on the proximal humeral physis during the acceleration phase of throwing. The repetitive nature of these forces leads to microtrauma.
The patient typically is an adolescent male baseball player, ages 11 to 16 years, who often is the best thrower on his team. He throws most days a week and frequently is the pitcher. He will report the gradual onset of upper arm pain during and/or after throwing. The patient might have had the pain for many months before seeking medical attention, with the average amount of time reported as almost 8 months in one case series.
The majority of patients (70%) have tenderness to palpation over the proximal lateral humerus at the level of the deltoid insertion, which is the site of the proximal humeral physis. This finding is typical and should raise suspicion for this injury. Shoulder range of motion (ROM) often is normal, as is strength, with the notable exception of the external rotators. Pain can be reproduced by simulating the throwing motion, ideally by having the patient throw a ball in the clinic.
Many athletes who excel at sports involving arm strength will have weakness of core (abdominal and back) musculature and the lower extremity muscles. These muscles (the so-called kinetic chain from the ground to the area of injury in the shoulder) should be evaluated and considered in the plans for subsequent physical therapy.,
The diagnosis made on physical examination can be confirmed by finding physeal widening on plain x-rays, with the lateral portion of the proximal humeral physis more commonly involved. Comparison views of the opposite shoulder can be helpful to directly compare the physes (Figure 7). Other associated x-ray findings include lateral fragmentation, sclerosis, and/or demineralization of the metaphysis.
X-Rays of Proximal Humeral Physeal Stress Injury
Magnetic resonance imaging (MRI) study is occasionally helpful. This imaging technique allows for actual visualization of the physis and can demonstrate evidence of stress injury, particularly when the plain x-rays are normal but clinical suspicion is high. ,
The initial recommended treatment is to rest the involved arm from all throwing activities until the symptoms resolve, which on average is reported to take 3 months., The athlete can still participate in athletic activities that do not involve throwing or reproduction of the pain. A sling or other restrictive devices can be prescribed if the patient has pain at rest, but this is typically unnecessary.
The athlete’s symptoms typically resolve before the resolution of the physeal widening, which can take several months to completely remodel. Gradual return to throwing activities can be initiated after the patient is pain-free and often is done under the guidance of a physical therapist knowledgeable about rehabilitation in the throwing athlete. Rehabilitation programs designed specifically for throwing athletes are available to assess and correct the patient’s throwing mechanics along with addressing any deficits in core muscle strength.
The majority of athletes diagnosed with Little Leaguer’s shoulder are able to return to competitive overhead athletics. However, it is critical that the treating physician has a discussion with the patient, parents, and coaches regarding the injuries that can result from excessive throwing from participating on multiple teams at the same time, year-round throwing, and extra throwing outside of practice. Pitch type and throwing volume guidelines, based on expert opinion, are available.,
Medial Epicondyle Apophysitis
In the immature skeleton, the medial epicondyle of the elbow is an apophysis: an ossification center at the end of a bone to which muscles and tendons attach. It is the site of yet another throwing-related overuse injury.
Young athletes can develop traction apophysitis at this site from the repetitive valgus stress associated with activities, such as baseball, softball, tennis, volleyball, and gymnastics. Baseball pitching is the most common; thus, medial epicondyle apophysitis often is called Little Leaguer’s elbow.
Typically, the patient is a 9- to 14-year-old male baseball pitcher who presents with progressive, atraumatic medial elbow pain with throwing activities. Often, there will be a history of excessive throwing and reports of decreased throwing speed and/or accuracy because of the pain. Pain might be noted during the early and late cocking phases of throwing.
Physical examination reveals tenderness to palpation over the medial epicondyle apophysis that can be reproduced by applying a valgus stress across the elbow. Resisted wrist flexion also can reproduce the medial elbow pain. ROM can be decreased. Although the patient might have localized soft tissue swelling, there should not be an elbow effusion. If there is, the patient might have intra-articular pathology, such as osteochondritis dissecans. It is important to assess the kinetic chain, including shoulder, torso, and lower extremities, for muscular weakness that can contribute to additional elbow joint stress.,
Bilateral elbow x-rays often are obtained to assess the integrity of the medial epicondyle apophysis compared with the nonaffected side. The medial epicondyle apophysitis of the throwing arm can reveal hypertrophy, widening, and fragmentation, although these findings also have been seen in x-rays of asymptomatic adolescent baseball players.
The 2 central concepts for successful treatment of medial epicondyle apophysitis are temporary limitation of throwing activity and correction of poor throwing technique. Throwing activity limitation can range from no throwing activities for an extended period to a reduction of throwing or avoidance of certain type of pitches.
Correcting poor throwing biomechanics and kinetic chain weaknesses is key to a successful and pain-free return to throwing. A motion analysis study in pitchers ages 9 to 13 years found that those with more correct pitching mechanics generate lower humeral internal rotation torque, lower elbow valgus load, and more efficiency compared with young pitchers with poor mechanics.
Referral to a physical therapist experienced in evaluating and correcting throwing mechanics along with addressing any deficits in the kinetic chain is a critical component of the treatment plan. The input and recommendations of the patient’s coach or a consultant throwing coach can be helpful as well.
Athletes can gradually resume throwing activities if they have no swelling and have attained pain-free full ROM and strength in the elbow extensors, flexors, pronators, and supinators of the affected elbow. Successful return to throwing activities will be more likely if they have corrected poor throwing mechanics. The same return-to-play guidelines discussed previously in relation to proximal humeral physeal stress provide recommendation for pitching activity after medial epicondyle apophysitis.,
Tibial Tuberosity Apophysitis
Tibial tuberosity apophysitis, also known as Osgood-Schlatter disease, is a common overuse injury seen in growing athletes. The etiology of this condition is not known with certainty, but mechanical, growth, or traumatic factors have all been postulated. In particular, there is thought that repetitive strain on the secondary ossification center of the tibial tuberosity, leading to an apophysitis, is the cause.An MRI study of patients with a clinical diagnosis of Osgood-Schlatter disease compared with an asymptomatic control group suggested patients with a more proximal and broader patellar tendon might be predisposed to Osgood-Schlatter disease.
The patient with Osgood-Schlatter disease is typically a boy between ages 12 and 15 years or a girl between ages 8 and 12 years who reports anterior knee pain with jumping and/or kneeling activities. They frequently are involved in a sport that involves jumping, such as basketball or volleyball, and might have noticed swelling or enlargement of the tibial tuberosity.
The tibial tuberosity is tender to palpation and might be swollen and/or enlarged. Reproduction of pain can occur during resisted knee extension or with jumping or hopping.
Osgood-Schlatter disease is a clinical diagnosis and x-rays do not aid in diagnosis but are only recommended for excluding other diagnoses, such as stress fractures or bone tumors. Musculoskeletal ultrasound might have a future role in confirming and assessing the severity of Osgood-Schlatter disease, but it is not yet routinely used.,
Osgood-Schlatter disease is self-limited in the majority of patients with only a small percentage continuing to experience adverse effects after the apophysis has fused. Indeed, most studies demonstrate that approximately 90% of patients do well with activity modification as needed, plus ice, physical therapy, and time. Patellar tendon straps have shown to be helpful in some patients.
Patients with a mature tibial tuberosity and persistent symptoms from a tibial tuberosity that does not fuse to the main portion of the tibia have good to excellent results after surgical excision. A 10-year follow-up study of surgical treatment for unresolved Osgood-Schlatter disease revealed that 87% of the patients reported no daily activity or work restrictions and 75% reported return-to-preoperative sports participation.
Because Osgood-Schlatter disease is self-limited, sports participation should be allowed as tolerated.
The disease typically has a waxing and waning course and during times of increased pain, the athlete might need to reduce his or her participation level, minimize offending activities, and maximize pain-free cross-training activities.
In a young athlete who presents with low back pain associated with back extension activities, spondylolysis is frequently the cause of the pain. Spondylolysis is defined as a stress fracture of the pars interarticularis and is thought to result from repetitive microtrauma that causes ischemia. It most commonly occurs at the L5 vertebra and is more prevalent in sports requiring repetitive hyperextension, such as gymnastics, diving, and football.
An athlete who experiences spondylolysis typically is ages 10 to 15 years and presents with insidious onset of low back pain occurring during hyperextension. Radicular or neurologic symptoms typically are absent.
Physical examination often reveals pain with lumbar extension and can increase with single-leg hyperextension, although a recent study showed no correlation between spondylolysis diagnosis and single-leg hyperextension.Hamstring flexibility often is diminished. Neurologic examination should be normal and if not, should raise concern for a high-grade spondylolisthesis (forward slippage of the affected vertebral body due to bilateral spondylolysis) or another diagnosis, such as a lumbar disk herniation.
In the early stages of spondylolysis, x-ray findings typically are normal. Controversy exists on the views to be obtained for initial x-ray evaluation when spondylolysis is suspected. A fracture of the pars interarticularis, in the minority of patients in which it is visible, is best seen on oblique views of the lumbar spine and often is described as “a collar on the neck of the Scottie dog” but these views add additional significant radiation exposure. Because findings of initial x-rays are typically normal, some physicians advocate obtaining only standing anteroposterior and lateral views for purposes of excluding other conditions that might be causing the pain.
To confirm the diagnosis when initial x-rays are negative, the next imaging test obtained can be a single-photon emission computed tomography (SPECT) scan or an MRI study. A SPECT scan, preferred over a nuclear medicine bone scan, is considered the gold standard for diagnosing spondylolysis and is highly sensitive for increased metabolic bone activity.Figure 8). This thin-section CT scan will confirm that spondylolysis is the cause of the increased metabolic bone activity.However, it lacks specificity, so if the SPECT scan reveals a location of increased metabolic activity, then a limited, thin-section computed tomography (CT) scan is obtained (only at the level of increased activity) to define the actual anatomic site involved (
Computed Tomography Scan of Lumbar Spondylolysis
Magnetic resonance imaging study also can reveal spondylolysis, but at times it can fail to identify the presence or severity of the injury. A study comparing SPECT and CT scans with standard MRI study revealed that MRI study diagnosed the spondylolysis only 80% of the time.Another comparison study with special MRI study sequences improved the diagnostic accuracy, but performed less well with grading severity of the spondylolysis. Although not considered the imaging study of choice, MRI study has the advantage of visualizing other soft tissue abnormalities (eg, disk pathology) and involves no radiation exposure.
Research has not identified the best treatment for an acute spondylolysis and, thus, the approach to treatment is controversial. Most investigators agree that the athlete should avoid lumbar hyperextension for approximately 6 to 12 weeks, or longer if symptoms persist. Antilordotic bracing (which prevents hyperextension) can play a role in the treatment of an acute spondylolysis, but whether to prescribe a brace, what type of brace, and how long to wear it is unclear.
Physical therapy is recommended to address core stability and kinetic chain deficits, in addition to reintroducing sports-specific activities in a supervised and controlled fashion.A systematic review of physical therapy in the treatment of spondylolysis/spondylolisthesis found only 2 studies with methodology that met the review’s inclusion criteria. These 2 studies demonstrated a positive treatment effect but investigators concluded that more prospective research trials are needed.
Like other bony stress injuries, spondylolysis can heal with a bony union, though a sizeable percentage heals with a fibrous nonunion. These types of healing have similar short-term outcomes and return to sports.
If spondylolysis appears chronic on x-ray or other imaging, then bony union is unlikely to occur. In this case, treatment involves reduction or cessation of the offending activity until the pain resolves, coupled with a course of physical therapy.
Previously painful sports-specific activities are gradually reintroduced after the athlete’s symptoms have resolved and/or healing is demonstrated on advanced imaging. Additional recommended criteria for returning to play include full, pain-free ROM and strength, sports-specific core stability, and the ability to properly perform sport-specific skills in physical therapy. Full return to sports, without restrictions, typically requires 2 to 4 months of rehabilitation after the initial 6 to 12 weeks of treatment.
Case 3, cont’d. Riley presents with a history consistent with an overuse injury of the elbow, and medial epicondyle apophysis is the most likely diagnosis. Other conditions that cause medial elbow pain include acute medial epicondyle apophyseal avulsion fracture, ulnar collateral ligament injury, ulnar neuritis, flexor-pronator tendinopathy or muscle strain, and intra-articular loose body. Asking about associated symptoms such as swelling, locking, catching, loss of range of motion, and numbness or tingling is important to help exclude those other diagnoses, while keeping in mind that the patient might be experiencing 1 or more conditions at the same time. Physical examination and imaging will help confirm the suspected diagnosis.
Riley’s treatment will include a trial of no throwing or reduced throwing activities along with a referral to physical therapy. Gradual return to throwing coupled with evaluation of his throwing technique can begin after his symptoms have abated. It is important to have a discussion regarding recommendations on pitch types appropriate for age and technical ability, pitch counts, and number of innings pitched in hopes of avoiding a recurrence of elbow pain. Specific advice for Riley would be to avoid throwing curve balls and sliders because these types of pitches are known to increase the risk of elbow injuries in young pitchers. He also should limit pitch counts to a maximum of 125/week.,
Juvenile Idiopathic Arthritis
Case 4. Teresa, a 16-year-old girl, is a new patient in your practice. Medical history reveals that she was diagnosed with juvenile arthritis when she had an episode of knee pain at age 8 years. She was subsequently treated by a rheumatology subspecialist until age 12 years, but has not had any follow-up care since. She reports no current joint pain and states that she no longer has arthritis.
Juvenile idiopathic arthritis (JIA) encompasses several forms of chronic arthritis. They have in common the following features: onset before age 16 years, presence of arthritis (joint swelling or decreased motion in the presence of warmth, pain, or tenderness), and symptom duration longer than 6 weeks.Because infection, malignancy, rheumatic fever, connective tissue diseases, and other inflammatory diseases can all present with joint pain, they must be excluded before a diagnosis of JIA can be made.
Juvenile idiopathic arthritis has an incidence of 1 to 22 per 100,000 children and a prevalence of 8 to 150 per 100,000 children.,, Although the etiology is unknown, it is widely believed that JIA is an inflammatory and/or immune process that occurs in genetically susceptible individuals.
Children with JIA are considered to be in remission when the disease signs and symptoms are inactive for at least 6 consecutive months. Remission subsequently can be defined by whether it is attained when a patient is taking drugs or not.
New Classification Scheme
In 1997 the International League of Associations for Rheumatology (ILAR) developed the newest classification scheme used to categorize JIA. The ILAR recommended changing the nomenclature from juvenile rheumatoid arthritis to JIA because rheumatoid suggested that these patients had a similar form of disease to adult rheumatoid arthritis, which typically is not the case. Terminology of the American College of Rheumatology and the European League Against Rheumatism classifications, which still refer to juvenile rheumatoid arthritis, preceded the development of ILAR and still is cited frequently in the literature., However, the correct current terminology is JIA.
The JIA terminology is a reflection of the overall goal of the ILAR to encompass all subtypes of chronic childhood arthritis under one umbrella term, yet separate chronic arthritis into distinct homogeneous groups. The ILAR defines 7 subgroups of JIA (Table 4). Categorization is determined based on clinical signs and symptoms that develop within the first 6 months of the disease. Though the categorization is imperfect, it provides a framework for categorizing patients based on clinical presentations. This helps delineate treatment, predict patient outcomes, and advance JIA clinical research.
Involves <5 joints, with knees and ankles commonly involved. Uveitis in 30% of patients
Polyarticular rheumatoid factor-negative
Involves ≥5 joints; uveitis also can occur
Rash and high-spiking fever, followed later by polyarticular involvement of large or small joints
Polyarticular rheumatoid factor-positive
Symmetrical involvement of small joints, subcutaneous nodules on hands, finger deformities. Rheumatoid factor-positive. Can be a childhood form of adult rheumatoid arthritis
Involves lower extremities and small joints of hands and feet. Sausage-shaped digits (dactylitis) is classic. Psoriatic rash usually develops later
Occurs in association with inflammatory bowel disease and juvenile ankylosing spondylitis
Includes arthritis that does not fall into any of the other categories
aListed from most common to least common.
Oligoarticular JIA is the most common subtype, making up 27% to 56% of all JIA patients. Girls are more frequently affected than boys. The onset peaks between ages 2 to 4 years.
Oligoarticular JIA is defined by involvement of fewer than 5 joints within the first 6 months of symptoms. Asymmetrically affected large joints, typically knee or ankle, are characteristic., Persistent and extended subtypes have been described. Patients with arthritis limited to 4 or fewer joints are categorized as persistent. Extended subtype is defined by active arthritis in 5 or more joints after the first 6 months. Remission occurs in only 12% of extended subtype patients, whereas it occurs in 75% of persistent subtype patients.
Oligoarticular JIA carries a high risk of chronic uveitis. It occurs in 30% of patients. The presence of a positive antinuclear antibody (ANA) test increases the likelihood of developing uveitis. The uveitis is asymptomatic; therefore, frequent slit lamp examinations are recommended.
Polyarticular rheumatoid factor (RF)-negative JIA is the next most common subtype, making up 20% to 30% of all JIA patients.Onset occurs in early childhood, and girls are predominantly affected. Polyarthritis, defined by arthritis in 5 or more joints, is characteristic and prognosis is variable. Patients who are ANA-positive have the greatest risk for developing a chronic uveitis. This subtype is thought to be the most heterogeneous of the ILAR subtypes.
Systemic onset JIA (SOJIA) is the third most common subtype, comprising 10% to 20% of all patients.,There is no association with age or sex, but genetic factors appear to be involved because a relationship recently has been found between JIA and HLA-DRB1 alleles in some populations.
Systemic onset JIA commonly presents with rash and a characteristic high-spiking fever that lasts 2 weeks. The fever is referred to as the double quotidian fever because of its characteristic spike that occurs 2 times/day. The rash, which is well circumscribed, appears as less than 1-cm macules that are salmon pink in color with an area of central clearing or surrounded by a pale ring. The trunk and proximal extremities are typical locations. The rash tends to occur with the fever. Other extra-articular manifestations include lymphadenopathy, hepatomegaly, and serositis.
Within 6 months of SOJIA diagnosis, polyarticular arthritis of small or large joints typically occurs. Common laboratory test findings include anemia, leukocytosis, thrombocytosis, elevated liver enzymes, and an elevated erythrocyte sedimentation rate and C-reactive protein level. ANA titers typically are negative.Although the risk for developing uveitis is low, ophthalmologic screening should occur every 12 months.
Approximately two-thirds of SOJIA patients experience remission, while the remainder of patients can develop chronic polyarthritis.SOJIA patients also are at risk of a life-threatening condition called macrophage activation syndrome, in which histiophagocytosis occurs in the bone marrow (macrophages engulf and destroy bone marrow cells).
Polyarticular RF-positive JIA makes up 5% to 10% of all cases.Onset occurs in late childhood and early adolescence, and girls are predominantly affected. Symmetrical small joint disease in 5 or more joints is present during the first 6 months, and erosion can be severe. The axial skeleton, as well as large joints can be affected, but small joint involvement typically is present simultaneously. Nontender subcutaneous nodules on the hands develop, and finger deformities are common. This might be an early form of adult seropositive rheumatoid, because HLA associations are the same.
Psoriatic arthritis accounts for 10% or less of JIA cases. It initially presents with an asymmetrical arthritis that predominantly affects the lower extremities and the small joints of the hands and feet. The classic sausage digit (dactylitis) is a result of a diffuse swelling that occurs within the tendon sheaths of the proximal and distal interphalangeal joints.
The rash of psoriasis typically develops much later than the arthritis, and is an ILAR criterion for diagnosis. If rash is not present, the diagnosis can be made only if there is a first-degree family member with psoriasis, dactylitis is present, or nail pitting is evident on examination.
Girls are more frequently affected and onset occurs with a biphasic distribution with an early peak at ages 2 to 4 years and a subsequent peak at ages 9 to 11 years.ANA is frequently positive. Psoriatic arthritis carries a significant risk for a chronic form of uveitis.
Enthesitis-related arthritis subtype occurs in 10% or fewer of cases. It includes the arthritis associated with inflammatory bowel disease and juvenile ankylosing spondylitis.
Enthesitis is defined as inflammation at the bony attachment sites of tendons. Planter fascia and Achilles tendon insertion are commonly involved, as are the tendons surrounding the hip joint. Boys are affected more often and age of onset is in late adolesence.
Individuals with inflammatory bowel disease might first present with lower extremity joint symptoms, rather than gastrointestinal (GI) symptoms, making the condition challenging to diagnose. Other common symptoms can include weight loss, growth delay, and mucocutaneous changes. In some individuals, the joint symptoms parallel the bowel disease and improve as the GI symptoms resolve. In others, they occur independently of one another.
Juvenile ankylosing spondylitis is associated with HLA-B27 and causes significant disease in the spine. Common symptoms include pain, stiffness, and eventual loss of motion within the spine. It is more common in boys, and typically is diagnosed after age 8 years. Lower extremity joint arthritis precedes symptoms within the spine and the characteristic sacroiliac joint disease is a late finding.
Individuals with this form of JIA are RF-negative and ANA-negative. Up to 27% of patients with enthesitis-related arthritis are at risk of developing acute uveitis that presents with a unilateral painful red eye, distinct from the chronic uveitis seen in other forms of JIA.
Undifferentiated forms of JIA make up 11% to 21%.This group encompasses all individuals who cannot be classified into a single subtype under the ILAR criteria. The ILAR criteria frequently are criticized for the large proportion of individuals within this category. Also, some arthritis can be classified into more than one subtype under the current ILAR criteria. This can cause significant problems when physicians attempt to use the ILAR criteria for diagnosis and anticipatory guidance, or for research purposes.
Managing the extra-articular manifestations of JIA often requires participation by many different healthcare professionals. Ophthalmology, endocrinology, and orthopedic subspecialists, physical therapists, and dieticians can all play a key role in screening and treating the common complications of JIA.
Uveitis is a common extra-articular manifestation in several types of JIA. Oligoarticular arthritis patients are at greatest risk of developing the chronic, nonpainful form. However, patients with RF-negative polyarticular and psoriatic arthritis also might be affected, especially if the ANA test is positive.
For most JIA patients, uveitis onset occurs within the first 5 to 7 years after diagnosis.Because complications of uveitis can be severe, including glaucoma, cataracts, band keratopathy, and visual impairment in up to 30% of patients, routine screening for early detection of uveitis is recommended. The recommended screening frequency is every 3, 6, or 12 months, depending on risk category. The risk category, in turn, depends on JIA subtype, ANA status, age at symptom onset, and disease duration. The mainstay of treatment for uveitis is topical steroids and mydriatics.
Nutritional impairment also is common in JIA patients. Suboptimal caloric intake and/or increased resting energy expenditure are common. The latter is especially true for patients with SOJIA, whose illness can involve prolonged, high fever.Drug side effects, temporomandibular joint pain with eating, and/or cachexia related to increased levels of tumor necrosis factor (TNF)-alpha and interleukin 1 can further contribute to malnutrition. The poor nutritional status of children with JIA can lead to disturbances of linear growth. Growth hormone might be indicated to treat severe growth retardation.
Abnormal Bone Health
Children with JIA also are at risk of abnormal bone development and subsequent fractures. Chronic steroid use, decreased physical activity, arthritic disease, decreased lean body mass, and nutritional impairment all increase the risk of low bone mass and future osteoporosis.However, no formal recommendations to undertake bone density assessments have been developed. Weight-bearing exercise in conjunction with age-specific calorie, calcium, and vitamin D intake recommendations should be reviewed with all patients.
The goals of JIA treatment are improved patient function through decreased pain and inflammation, promoting normal growth and development, preventing long-term consequences of the disease, and attainment of overall well-being. Treatment options primarily involve drugs, but physical and occupational therapy can play a critical role for patients with painful joint disease by improving muscle strength, joint range of motion, and modifying activities of daily living.
First-line drug choices include nonsteroidal anti-inflammatory drugs (NSAIDs) and corticosteroids. Naproxen, ibuprofen, tolmetin, and diclofenac are the commonly used NSAIDs and are well tolerated in children. Indomethacin is frequently used for patients with systemic onset and enthesitis-related JIA, but it is more commonly associated with GI symptoms, headaches, and concentration difficulty.
Intra-articular injection of corticosteroids is an effective way to treat localized symptoms with minimal systemic effects.Systemic glucocorticoids are used with caution because of the potential for serious GI, endocrine, ophthalmologic, musculoskeletal, and central nervous system complications. Systemic steroids are reserved for patients with severe SOJIA or as a bridge drug for patients with polyarticular JIA who are awaiting relief from other second-line drugs. Tapering systemic steroids, to the lowest dose that controls symptoms, is recommended.
Methotrexate and Sulfasalazine.
The most widely used second-line drugs are the disease-modifying anti-rheumatic drugs (DMARDs) methotrexate and sulfasalazine. Methotrexate is a folate antagonist that is commonly used for polyarticular, extended oligoarticular, and systemic onset JIA. GI side effects are the most common adverse effect, and these can be minimized by adding folic acid supplementation. Severe side effects include hepatotoxicity, immunosuppression, teratogenicity, and rarely, pulmonary complications.Sulfasalazine is commonly used for patients with oligoarticular JIA and spondyloarthropathies. Serious side effects include myelosuppression, GI toxicity, and hypoimmunoglobulinemia. Frequent screening blood tests, including blood counts and liver transaminases, are recommended for patients taking methotrexate and/or sulfasalazine.
One of the newer class of DMARDs is inhibitors of TNF-alpha. This drug class includes etanercept (Enbrel), infliximab (Remicade), and adalimumab (Humira). The rationale for use of these drugs in JIA patients was the discovery of elevated serum TNF-alpha and soluble TNF receptors, as well as increased synovial fluid levels of TNF-alpha, in JIA patients. All of these drugs, except infliximab, have been approved by the Food and Drug Administration (FDA) for treatment of JIA in children. These drugs are expensive, with annual costs totaling as much as $20,000.
The anti-TNF drugs are effective and can be used in combination with another DMARD or as monotherapy. Etanercept has shown to be effective and well tolerated in a double-blind, placebo-controlled study of patients with polyarticular JIA who did not benefit from methotrexate treatment. In this study, injection site reactions, upper respiratory tract infections, headaches, GI symptoms, rash, and rhinitis were cited as side effects.In another study, anti-TNF drugs were used as monotherapy or in combination with methotrexate to treat patients with systemic JIA. After an average treatment length of 26 months, 24% of these patients went into remission.
Severe side effects of the anti-TNF drugs include a potential for invasive fungal infections, reactivation of tuberculosis, and development of lymphoma and other malignancies. These severe side effects have caught the attention of the FDA in recent years, resulting in the issuance of a black box warning.
Abatacept (Orencia) is another new drug that is FDA-approved for JIA treatment. Sometimes used when there is an inadequate response to anti-TNF-alpha drugs, abatacept is a costimulation modulator that blocks T-cell activation, which is needed for cytokine production. This can decrease auto-antibody production and inflammatory responses.
Herbal therapies frequently are used for JIA patients, often in conjunction with regularly prescribed conventional drugs. Gamma-linolenic acid is one of the most commonly used substances. It is found in primrose oil, black currant seed oil, and borage seed oil. In a Cochrane review of herbal therapies, gamma-linolenic acid was found to be superior to placebo in 6 out of 7 studies. Reduction in pain and joint swelling and decreased morning stiffness were noted in randomized controlled trials. Although the findings are promising, these studies had small sample sizes and there was significant variability in results among the handful of prospective, controlled trials. Therefore, further research into herbal therapies is required to support their safety and effectiveness.
Exercise is another important form of treatment for JIA patients. Because JIA patients are less active than their peers and rarely meet recommended daily activity levels, it is imperative to discuss with them the importance of physical activity., Activities such as swimming, water aerobics, and cycling offer minimal stress on the joints. This is especially helpful for patients with lower extremity large joint disease that is severe enough to interfere with other physical activities. However, weight-bearing exercise (if well tolerated) also is beneficial and can help improve bone density in JIA patients.
Overall, exercise has been shown to be well tolerated by JIA patients. A 2008 Cochrane review compared 3 randomized controlled trials of JIA patients and various forms of exercise. Although no statistically significant findings emerged from the review, no significant detrimental outcomes were associated with exercise.There are no formal exercise recommendations for JIA patients, but it should be encouraged for overall health benefits and possible bone density benefits.
Studies on prognosis are somewhat limited by the recent change in disease classification systems. However, it is clear that all JIA subtypes have a risk of progression into adulthood. Long-term studies conducted before the ILAR classification system revealed that 31% to 55% of JIA patients had active disease for at least 10 years. Individuals with extended pauciarticular, polyarticular, and systemic onset disease were at greatest risk of active disease state at 26-year follow-up., Moderate to severe disability has been documented in 15% to 25% of JIA patients of 7 to 15 years duration. Long-term follow-up studies also have revealed that activities of daily living are limited in more than half of patients with JIA.
Case 4, cont’d. Teresa was diagnosed with monoarticular arthritis of the knee at age 8 years. This would be consistent with oligoarticular juvenile idiopathic arthritis (JIA). By age 12 years, she reportedly no longer required care by a rheumatology subspecialist. This would place her into the persistent subtype of oligoarticular JIA, the subtype most likely to enter into remission. Although she has a negative antinuclear antibody test, thus a relatively low risk of uveitis, she nonetheless should undergo annual screening for uveitis based on current recommendations. No other treatment is required because she is in the remission phase. Regular health maintenance is recommended along with nutrition and exercise guidance.
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Common Deformities of the Foot and Leg
Lincoln TL, Suen PW. Common rotational variations in children. J Am Acad Orthop Surg. 2003;11(5):312-320.
Kim HJ, Blanco JS, Widmann RF. Update on the management of idiopathic scoliosis. Curr Opin Pediatr. 2009;21(1):55-64.
Scherl SA. Clinical features; evaluation; and diagnosis of adolescent idiopathic scoliosis. UpToDate. 2010. Available by subscription at www.uptodate.com/online/content/topic.do?topicKey=ped_orth/4271&selectedTitle=1~3&source=search_result. Accessed October 2010.
Scherl SA. Treatment and prognosis of adolescent idiopathic scoliosis. UpToDate. 2010. Available by subscription at www.uptodate.com/online/content/topic.do?topicKey=ped_orth/6016&selectedTitle=1~3&source=search_result. Accessed October 2010.
Cassas KJ, Cassettari-Wayhs A. Childhood and adolescent sports-related overuse injuries. Am Fam Physician. 2006;73(6):1014-1022.
Fleisig GS, Weber A, Hassell N, et al. Prevention of elbow injuries in youth baseball pitchers. Curr Sports Med Rep. 2009;8(5):250-254.
Soprano JV. Musculoskeletal injuries in the pediatric and adolescent athlete. Curr Sports Med Rep. 2005;4(6):329-334.
Juvenile Idiopathic Arthritis
Ravelli A, Martini A. Juvenile idiopathic arthritis. Lancet. 2007; 369(9563):767-778.
Weiss JE, Ilowite NT. Juvenile idiopathic arthitis. Rheum Dis Clin North Am. 2007;33(3):441-470, vi.
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