Musculoskeletal Pain in Adolescents - Part 1: 'Growing Pains' or Something Else?

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March 1, 2000

Consultant

Musculoskeletal Pain in Adolescents: 'Growing Pains' or Something Else?

By Ilona S. Szer

The history and physical examination are the most important

elements of your evaluation of adolescents with musculoskeletal complaints.

Look specifically for cutaneous signs of rheumatic illnesses, such as

swelling and rash, focus on identifying arrhythmias, rubs, and murmurs, and

examine all joints. Laboratory screening--including complete blood cell

count with differential and liver and renal function tests--is warranted

when physical findings are abnormal but the cause of pain is undetermined;

imaging studies may be required as well. Benign hypermobility syndrome

affects girls and causes chronic, diffuse joint pain and frequently, skin

laxity. Inflammatory conditions include spondyloarthropathies that afflict

boys; they are the most common cause of chronic arthritis in teens. The

Schober test to assess back flexion is helpful in the evaluation of

adolescents with seronegative enthesopathy and arthropathy syndrome and

other spondyloarthropathies.

Musculoskeletal pain is among the most frequent complaints of adolescents.

It is a common cause of school absenteeism and withdrawal from normal

activities. Arthralgia is the single most common reason for referral to the

pediatric rheumatologist. [1]

Noteworthy, however, is the fact that roughly half of all children and

adolescents who are referred do not have a rheumatic condition. [1] During

the past decade, we have seen an increase in the number of adolescents and

preadolescents who experience musculoskeletal symptoms that appear to arise

as a defense against emotional stress. This stress frequently can be traced

to the pressure to achieve at academics or sports.

Coexisting organic and psychological disturbances can complicate the

clinical picture. It is easy to misdiagnose a psychogenic illness that has

physical manifestations and a physical illness that presents with

psychogenic symptoms--particularly if the clinician believes that all of the

patient's complaints can be explained by a unifying diagnosis.

This is the first in a three-part series on musculoskeletal pain in

adolescents. Here, the emphasis is on common organic syndromes.

Specifically, I describe the general work-up and then discuss benign

hypermobility syndrome, night cramps, shin splints, and inflammatory

conditions.

In a second article, I will address knee problems commonly seen in

adolescents. Finally, in part 3, I will shift the focus to psychogenic

causes of musculoskeletal pain.

EVALUATION

History of present illness. The evaluation of an adolescent with unexplained

musculoskeletal pain begins when you first meet with the patient and his or

her family. The initial interaction communicates your interest in the

patient and his problem. A skillful interview, with a thorough, organized,

and analytic approach, indicates that the patient is in good hands and

encourages cooperation.

Elicit a detailed description of the present illness--nothing is irrelevant.

Pay special attention to signs and symptoms of rheumatic disorders,

including joint pain, swelling, limitation of motion, stiffness, and

weakness. Inquire about rashes, eye manifestations, and genitourinary and

bowel symptoms.

Injury or illness that predated symptoms is important--particularly when you

suspect reflex sympathetic dystrophy. (I will discuss this syndrome in

detail in part 3 of this series.) A travel history can provide clues to

certain endemic conditions, such as Lyme disease. Family and social

histories also may supply important information.

School absences are a key clue to the presence of a somatoform, or

psychogenic, disorder. A somatoform disorder is characterized by a degree of

disability that is out of proportion to the physical findings.

The history often reveals a constellation of symptoms that may seem

mysterious but, in fact, represent a recognizable pattern. An optimal

history generally leads to the diagnostic hypothesis that will be confirmed

by the physical examination and laboratory and imaging studies.

Physical examination. A thorough examination is the most important aspect of

the evaluation of patients with musculoskeletal complaints. Generally,

adolescents with systemic illness appear ill, whereas those with somatoform

or localized causes of pain look well.

General examination. Pay particular attention to the patient's nutritional

status and height and weight. Check for diffuse lymphadenopathy or

multiple-organ involvement, either of which suggests systemic disease.

Abdominal evaluation may disclose other clues to systemic illness, such as

organomegaly, other masses that represent tumor or abscess, and localized or

diffuse tenderness.

Skin evaluation. Look for rashes and search for cutaneous manifestations of

rheumatic illnesses; these include dermatographism, livedo reticularis,

subcutaneous nodules or swelling, changes in dermal thickness, tightening or

contractures, and pigmentation changes. In addition, search for ungual or

dermal telangiectases, nail changes, and alopecia with broken frontal hairs.

When your patient has Raynaud's phenomenon, evaluate the fingertips for

signs of skin thinning, ulcerations, or slow capillary refill. Examine

mucous membranes for aphthous stomatitis, which is seen frequently in young

people with spondyloarthritis, systemic lupus erythematosus (SLE), mixed

connective tissue disease, and inflammatory bowel disease (IBD). Search for

genital ulcers that may indicate Behcet's syndrome or Crohn's disease.

Heart and lungs. Focus on identifying arrhythmias rubs, and murmurs.

Decreased breath sounds may signify a pleural effusion.

Muscle strength. To assess your patient's muscle strength quickly, have him

lie supine and:

* Lift his head.

* Sit up without assistance.

* Assume a squatting position.

* Rise unassisted from the squat.

The inability to perform these maneuvers independently calls for a directed

manual muscle evaluation. The muscle-strength grading system provides a

guideline for assessing disease severity (Table 1). A rating of 5 reflects

normal strength, 3 indicates just enough strength to resist gravity, and 1

implies trace muscle strength. Identification of specific, involved muscle

groups can aid in diagnosis. For example, children with dermatomyositis

early on will exhibit weakness that is limited to the neck muscles or the

proximal muscles of the shoulder or pelvic girdles. If the disease is

undiagnosed, profound muscle weakness will develop.

Joint assessment. Examine all joints: do not limit your evaluation to the

area of complaint. Often, you will discover decreased range of motion in

joints that are affected by inflammation but are asymptomatic, or you will

find diffuse hypermobility in a patient who has a localized complaint.

Observe the patient's gait and watch for any sign of a limp or bizarre

posture. If you discern a limp, measure both legs to document muscle atrophy

and leg-length discrepancy.

Evaluate each joint for swelling, warmth, redness, and range of motion. If

you are not certain whether the range of motion is normal, compare the

patient's range with your own.

Back examination. A thorough evaluation includes palpation of the spine and

the sacroiliac joints as well as assessment of low back flexion,

particularly in adolescent boys who may have spondyloarthritis. Palpate the

back and extremities for the presence of " tender " points (Table 2), which

could suggest fibromyalgia, and enthesopathy (pain of the entheses--the

insertion sites of tendons and ligaments into bone). Fibromyalgia will be

discussed in detail in the third article of this series.

Laboratory work-up. Tailor the laboratory evaluation to the intensity and

duration of symptoms. When the physical examination yields abnormal

findings, the cause of the complaint may be obvious and laboratory studies

may not be warranted. If the diagnosis is not clear from the examination

alone, screening studies need to be performed. Order the following tests:

* Complete blood cell count with a differential.

* Chemistry panel with liver and renal function tests.

* Routine urinalysis.

You may need to evaluate thyroid function and test for chronic viral

infection (such as Epstein-Barr virus infection) in adolescents whose

primary complaint is fatigue. Order antinuclear antibody (ANA) testing when

constitutional symptoms and multiorgan involvement are evident in the

initial screening tests. An isolated positive ANA result in an adolescent

who has fatigue and no other abnormal objective symptoms or findings does

not correlate with future development of SLE.[2] There is generally no need

to refer such patients for extensive evaluations.

Imaging studies. Further evaluation, including radiography, is appropriate

for patients with isolated or objective laboratory findings. Do not reorder

the same tests that produced negative results; repeated tests seldom yield

useful information.

Once the work-up has been completed, initiate treatment without delay. This

is of particular importance for patients with somatoform disorders, which

must be treated promptly and confidently.

When to refer? Referral to a consultant is dictated by the intensity of

symptoms and the presence of associated disability. Promptly refer

adolescents who stay out of school despite your intervention. These patients

may require rehabilitation directed by a rheumatologist.

COMMON ORGANIC DISORDERS

Benign hypermobility syndrome. A disorder common in childhood and

adolescence, benign hypermobility syndrome (BHS) is one of the heritable

diseases of the connective tissues. Marfan and Ehlers-Danlos syndromes

represent the more severe end of the spectrum. [3]

Presentation. Those typically affected are preadolescent and adolescent

girls who excel at gymnastics. They present with chronic, diffuse joint pain

that usually worsens at the end of the day or after strenuous activity.

These patients do not withdraw from activity or school. Symptoms may be

erroneously attributed to psychogenic pain, because there are no abnormal

physical findings and no evidence of systemic illness.

Work-up. Laboratory studies are normal, but physical examination confirms

joint hypermobility that is usually associated with skin laxity. Diagnostic

criteria for BUS include arthralgia and four of nine sites of laxity that

can be measured by the following:

* Passive bilateral knee and elbow extension beyond 180 degrees.

* Apposition of thumb to the forearm while the wrist is flexed.

* Hyperextension of fifth metacarpophalangeal past 90 degrees.

* Ability to place the palms flat on the floor while bending forward with

knees extended. [4]

Most authors stipulate that a minimum of 10 degrees of hyperextension at the

elbows and knees needs to be evident as well. Occasionally, laxity is

complicated by recurrent sprains, dislocations, and chronic overuse.

Management. Offer counseling and supportive care. Adolescents need not

curtail their activities unless they choose to do so. The prognosis for most

patients is excellent; hypermobility decreases and patients become less

flexible as they get older.

Night cramps. Many teens complain of leg and feet cramps that are unrelated

to exertion and often interrupt sleep. A recent study of more than 2,500

healthy children revealed that 7.3% experienced nocturnal leg cramps. The

cramps occurred most commonly in youths older than 12 years of age, and a

majority of those affected suffered these episodes one to four times a year.

[5]

The cause of night cramps is unknown. Symptoms may be exacerbated by walking

on cement floors or sitting for prolonged periods.

Work-up. The diagnosis rests on an appropriate history, pain pattern, and

demonstration of cramped muscles.

Management. Teach your patient to relieve the cramp by entirely extending

the affected muscle. The prognosis for teens with nocturnal lower extremity

cramps is excellent.

Shin splints. This condition is characterized by localized pain in the lower

extremities that is associated with prolonged walking or jogging. In mild

cases, the pain may be relieved by rest; in more severe cases, pain can be

sustained. [6]

Work-up. Palpation of the anterior aspect of the leg will reproduce the

symptom. The syndrome reportedly is caused by small tears at the origin of

involved muscles. Stress fractures or compartment syndromes must be

excluded.

Management. Shin splints may be prevented or minimized with proper footwear

or orthotics. Advice your patient to avoid the activities that commonly

cause the condition and to refrain from running on hard surfaces, which can

aggravate symptoms.

INFLAMMATORY CONDITIONS

Inflammatory conditions with musculoskeletal symptoms usually produce

objective findings of joint swelling and limitation of motion with pain.

However, spondyloarthropathy syndromes, conditions that most often affect

adolescent boys, may present only with enthesopathy.

Spondyloarthropathies. These conditions are related to ankylosing

spondylitis; they share a familial predisposition and a predilection for

causing low back pain and inflammation of the synovial lining of joints,

tendons, and entheses. The spondyloarthropathies are the most common cause

of chronic arthritis in adolescents. [7]

The characteristic pattern of joint involvement in spondyloarthritis is

asymmetric lower extremity joint swelling, which may follow a febrile or

diarrheal illness (reactive arthritis) or significant skeletal trauma. The

most frequently affected joint is the knee, followed by the hip; first

metatarsophalangeal joint swelling and sacroiliac pain with late radiologic

changes (ankylosing spondylitis) develop in 10% to 20% of patients.

Fever is not usually present during flares of arthritis or enthesitis except

in boys who have conjunctivitis and urethritis as well (Reiter's syndrome).

These patients may appear quite ill with symptoms that resemble

systemic-onset juvenile rheumatoid arthritis (JRA). Low-grade fever weight

loss, decreased linear growth, and anemia highlight the need to search for

underlying IBD even in the absence of overt gastrointestinal symptoms

(arthritis associated with IBD). Last, arthritis or inflammation of soft

tissues in the same pattern as spondyloarthritis (psoriatic arthritis) may

develop in patients who have psoriasis or a family history of psoriasis.

Most adolescents with spondyloarthritis do not have symptoms that are

consistent with one of the spondyloarthropathy syndromes outlined above.

Rather, they have asymmetric peripheral arthritis, with or without

enthesopathy, and a family history of morning low back pain and are HLA-B27

antigen-positive. Boys with this history were once thought to have

pauciarticular-onset JRA type 2. Young girls with asymmetric arthritis of

the lower extremities, positive ANA results, and iritis were classified as

having pauciarticular-onset JRA type 1. It is now widely accepted that

adolescent boys do not have pauciarticular JRA; the term seronegative

enthesopathy and arthropathy (SEA) syndrome has been adopted to describe

their illness.

Seronegative enthesopathy and arthropathy syndrome. In addition to the

typical pattern of arthritis--with a particular predilection for the hips

and lumbosacral spine--cardiovascular lesions have been reported in youths

with SEA. Proliferation of small blood vessels of the aortic wall that lead

to a subvalvular aortic ridge is seen most commonly.

Thorough evaluation includes the Schober test to assess low back flexion,

which is often decreased in patients with spondyloarthritis (Box). An

increase in the back span of at least 6 cm (15 cm to 21 cm) during the test

indicates normal low back flexion.

SEA syndrome typically is marked by recurrent flares of synovitis,

periostitis, enthesopathy, and low back pain. The overall prognosis is

excellent and the risk of future disability relatively low; however,

progressive hip disease may develop in some persons, who may eventually need

joint replacement. The risk of eventual ankylosis of the spine (ankylosing

spondylitis) appears to be low; exact data are not available because of the

relatively recent realization that this group of disorders is indeed

different from JRA. [8] The identification of HLA-B27 antigen as a

susceptibility marker for the spondyloarthropathy syndromes has led to the

recognition of these disorders as distinct and relatively common causes of

inflammatory, musculoskeletal pain in adolescence.

Treatment consists of pain control with NSAIDs and physical therapy to

maintain strength and range of motion and to prevent contractures and low

back limitation.

Dr Szer is professor of clinical pediatrics at the University of California,

San Diego, School of Medicine. She is also director of pediatric

rheumatology and codirector of the Rehabilitation Institute at Childrens

Hospital San Diego.

Editor's note: In a coming issue, Dr Szer will address the diagnosis and

management of 1ocalized causes of knee disorders in adolescents.

REFERENCES:

(1.) Denardo BA, Tucker LB, JC, et al. Demography of a regional

pediatric rheumatology patient population. J Rheumatol. 1994;21:1553-1561.

(2.) Deane PM, Liard G, Siegel DM, Baum J. The outcome of children referred

to a pediatric rheumatology clinic with a positive antinuclear antibody test

but without an autoimmune disorder. Pediatrics. 1995;95:892-895.

(3.) Child AH. Joint hypermobility syndrome: inherited disorder of collagen

synthesis. J Rheumatol. 1986;13:239-243.

(4.) Grahame R, Child A. A proposed set of diagnostic criteria for the

benign joint hypermobility syndrome. Br J Rheumatol. 1992;31:205.

(5.) Leung AK Wong BE, Chan PY, Cho HY. Nocturnal leg cramps in children:

incidence and clinical characteristics J Natl Med Assoc. 1999;91:329-332.

(6.) Baugher WU, et al. injuries of the musculoskeletal system in runners.

Contemp Orthop. 1979;1:46.

(7.) s JC, Berdon WE, ston AD. HLA-B27-associated spondyloarthritis

and enthesopathy in childhood: clinical, pathologic, and radiographic

observations in 58 patients. J Pediatr. 1982;100:521-528.

(8.) Cabral DA, Oen KG, Petty RE. SEA syndrome revisited; a long-term

follow-up of children with a syndrome of seronegative enthesopathy and

arthropathy. J Rheumatol. 1992;19:1282-1285.

(9.) The Industrial Medical Council. Guidelines for Evaluation of

Neuromusculoskeletal Disability. San Francisco: 1994. Available at:

http://www.dir.ca.gov/imc/nms.html. Accessed February 25,2000.

(10.) The American College of Rheumatology. 1990 Criteria for the

Classification of Fibromyalgia. Available at:

http://www.rheumatology.org/research/classification/fibro.html. Accessed

February 2, 2000.

Muscle strength grading chart

Muscle gradations Description

5 - Normal Complete range of motion against

gravity with full resistance

4 - Good Complete range of motion against

gravity with some resistance

3 - Fair Complete range of motion

against gravity

2 - Poor Complete range of motion

with gravity eliminated

1 - Trace Evidence of slight contractility

No joint motion

0 - zero No evidence of contractility

Adapted from The Industrial Medical Council. [9]

Examination of tender points [*]

Location Palpation site(s)

Occiput Bilateral; at the suboccipital muscle insertions

Low cervical Bilateral; at the anterior aspects of the

intertransverse spaces at C5 C7

Trapezius Bilateral; at the midpoint of the upper border

Supraspinatus Bilateral; at origins, above the scapula

spine and near the medial border

Second rib Bilateral; at the second costochondral junctions,

just lateral to the junctions of upper surfaces

Lateral epicondyle Bilateral; 2 cm distal to the epicondyle

Gluteal Bilateral; in upper outer quadrants of

buttocks in anterior fold of muscle

Greater trochanter Bilateral; posterior to the trochanteric prominence

Knee Bilateral; at the medial fat pad proximal

to the joint line

(*.)Perform digital palpation with approximately 4 kg of force. Pain at 11

of 18 sites suggests a diagnosis of fibromyalgia.

Adapted from American College of Rheumatology. [10]

Schober Test

Purpose:

To measure the amount of lumbar spine flexion.

Procedure:

Have the patient stand with his or her back to you.

Mark a reference point on the lower spine that corresponds to the dimples of

Venus. Measure a 15-cm span: 10 cm above and 5 cm below the reference mark.

Instruct the patient to bend forward and reach for his toes with his knees

extended. Remeasure the 15-cm span.

Result:

The difference between the two measurements indicates the amount of flexion

in the lumbar spine.

An increase of at least 6 cm (15 cm to 21 cm) indicates normal low back

flexion.

CLINICAL HIGHLIGHTS

* In the history of an adolescent with unexplained musculoskeletal pain, no

detail about the present illness is irrelevant. Pay special attention to

signs and symptoms of rheumatic disorders, including joint pain, swelling,

limitation of motion, stiffness, and weakness. Inquire about rashes, eye

manifestations, and genitourinary and bowel symptoms.

* Search the skin for signs of rheumatic diseases, such as dermatographism,

livedo reticularis, subcutaneous nodules or swelling, changes in dermal

thickness, tightening or contractures, and pigmentation changes; examine the

mucous membranes; and focus on identifying arrhythmias, rubs, and murmurs.

Assess muscle strength, check joint range of motion, and look for joint

laxity; swelling, and redness.

* Laboratory studies, including a complete blood cell count with

differential, chemistry panel with liver and renal function tests, and

urinalysis are indicated when the history and physical examination do not

yield a diagnosis. Test for thyroid dysfunction and viral diseases, such as

Epstein-Barr virus infection, if fatigue is the major complaint. When

multiorgan involvement is suspected, order ANA testing.

* Benign hypermobility syndrome (BHS), a heritable disorder that affects

girls, is akin to the more serious Ehlers-Danlos syndrome. Laboratory

findings in BHS are normal; however, joint hypermobility is evident and skin

laxity may be present. Diagnostic criteria for BHS include arthralgia and

four of nine sites of laxity.

* Boys are affected by spondyloarthropathy syndromes, Reiter's syndrome

(arthritis, conjunctivitis, and urethritis), and seronegative enthesopathy

and arthropathy syndrome (SEA). SEA may be associated with aortic lesions,

hip and lumbosacral spine arthritis, and enthesopathy, which also may be the

only symptom of spondyloarthropathy syndromes. The HLA-B27 antigen is a

susceptibility marker for these syndromes.