Back, Joint, and Extremity Pain - Case 5-6: 9-Year-Old Boy
I. History of Present Illness
In the winter of his 9th year of life, a very active young boy presented to his
pediatrician with left ankle pain of approximately 5 days
' duration. The boy's mother reported that her son had complained of various muscle injuries over
the previous month. Three weeks earlier, he began limping, and explained that
he had hurt his right hip playing basketball. After a few days of
“taking it easy,” he reported complete resolution. Shortly thereafter, he complained of left
elbow pain, and his mother speculated that the injury occurred while the boy
was wrestling with his older brother. He received ibuprofen, and after a few
days of treatment he was able to play video games without discomfort. In the
last few days, the mother had observed the boy limping again, but he denied any
problems until the coach of his indoor soccer team sat him out for the first
time all season. The boy confessed that his left ankle had been hurting him,
but he had not wanted to miss any games. The coach had called the boy
's mother and told her that her son would not be allowed back to practice until
he had been checked by a doctor. The boy described the hip and elbow symptoms
as vague pains that worsened with movement of the specific limbs. He denied
swelling or redness of the elbow or hip when they were bothering him but
thought that his ankle was
“little puffy” now.
The mother reported that he has felt warm on occasion and had not been eating
well. She believed that he had lost weight, and she had found his sheets damp
on a few mornings after she had awakened him for school. The boy denied
headache, rash, sore throat, nausea, vomiting, diarrhea, palpitations, or
fatigue and asked if he could still make it to a practice scheduled for later
that day.
II. Past Medical History
The patient had received all required immunizations, and, aside from a broken
nose sustained from a batted softball 3 years earlier, he had no significant
past medical history. He did not require any regularly scheduled medications.
He had no known medication allergies.
His family history was significant for a mother and maternal grandmother with
migraine headaches and trisomy 21 in his youngest brother. There is no family
history of arthritis or malignancy. His recent travel had consisted of 2 weeks
at the New Jersey shore over the summer and 1 month of
“sleep-away” camp in northeastern Pennsylvania.
III. Physical Examination
T, 38.6°C; RR, 18/min; HR, 112 bpm; BP, 112/60 mm Hg
Weight, 60th percentile (down 3 kg from his preparticipation physical
examination 4 months earlier); height, 75th percentile (up by 1.0 cm from the
earlier measurements)
The patient was a cooperative boy in no acute distress. He was slender, and his
clothes hung loosely from his frame. Eyes, nose, ears, and oropharynx were not
inflamed. His tonsils were 3+ and symmetric without erythema or exudates. His
neck was supple with only shotty anterior cervical adenopathy. His thyroid was
not enlarged. His lungs were clear with good aeration. His heart had a regular
rhythm but was tachycardic, with a soft systolic murmur at the apex that was
audible throughout systole. His abdomen was soft, nontender, nondistended, and
without hepatosplenomegaly. The left ankle demonstrated a small effusion with
increased warmth and mild erythema. There was exquisite pain with active and
passive range of motion and with gentle palpation of the joint. All other
joints were normal on examination.
IV. Diagnostic Studies
A complete blood count revealed 12,200 WBCs/mm3 (74% neutrophils, 20% lymphocytes, 5% monocytes, and 1% eosinophils);
hemoglobin, 9.5 g/dL; and a platelet count of 556,000/mm
3. A basic metabolic panel was normal, but inflammatory markers were elevated,
with an ESR of 120 mm/hour and a CRP concentration of 8.3 mg/dL. A rapid
streptococcal test and culture of his throat were both negative. Radiographs of
both ankles were obtained and were normal.
V. Course of Illness
The patient was treated with doxycycline for presumed Lyme arthritis. He also
received regularly scheduled naproxen, with good symptomatic relief of his
joint pain. Several days later, the Lyme antibody titers by Western blot were
found to be negative. An electrocardiogram (ECG) suggested another possible
etiology (Fig. 5-7). Additional testing confirmed the diagnosis that mandated
definitive ongoing treatment.
Discussion: Case 5-6
I. Differential Diagnosis
This previously healthy young man presented with a 1-month history of joint
pains, with true arthritis of the ankle demonstrated at the office visit. The
arthritis involved relatively large joints affected in a nonsimultaneous
sequence. This pattern is referred to as a migratory pattern, one in which new
joint inflammation occurs after previous joint inflammation resolves. The
fever, weight loss, elevated inflammatory markers, and mild anemia were also
noteworthy and suggested an infectious, rheumatologic, or malignant process.
Joint or extremity pain, with fever and weight loss, can certainly raise the
suspicion of a malignancy. True arthritis (joint effusion, calor, rubor, and
pain with range of motion) is not a typical presentation of musculoskeletal
tumors but may be part of a paraneoplastic syndrome with reactive arthritis.
Disseminated malignancies, such as neuroblastoma or leukemia, may also involve
joints or the skeletal system through direct bony destruction in close
proximity to a joint. Abnormalities may or may not be detected by plain
radiographs of the involved limb.
Infectious etiologies of arthritis include primary infection of a joint space or
infection of bone or soft tissue in close proximity to a joint, with or without
direct communication of the infection with the joint. These infections are more
likely to involve a single site, but multifocal infections may be seen, with
ongoing or intermittent bacteremia leading to multiple, hematogenously seeded
sites. If more than one joint is involved, postinfectious arthritis should be
considered. These inflammatory joint reactions are sequelae of preceding
infections and are usually considered to be sterile and mediated through an
immune response. Epstein-Barr virus and parvovirus B19 are among the common
viral agents associated with this reaction, and meningococcus is a notorious
bacterial cause. Arthritis characterizes late-stage Lyme disease and develops 6
to 12 weeks after the bite of an infected tick. Large joints, especially the
knees, are involved and recurrence of arthritis mimics a migratory pattern.
Each bout of arthritis lasts 1 to 2 weeks but may become prolonged if left
untreated. The involved joint often appears remarkably swollen, with little or
no erythema, and ambulation is often maintained despite impressive effusion in
the involved lower-extremity joint. Carditis, presenting as heart block, can
also complicate the picture, but it is more common in the early disseminated
stage of the disease, which occurs a few weeks to months after infection.
The involvement of multiple joints and stigmata of acute inflammation make
rheumatologic conditions an important consideration. Systemic-onset juvenile
rheumatoid arthritis can manifest initially with or without arthritis, but
chronic arthritis of at least 6 weeks duration is required for diagnosis.
Systemic lupus erythematosus (SLE) most often manifests with joint involvement
with constitutional complaints of fatigue, weight loss, fever, and a typical
rash, and there is almost always an elevation in the antinuclear antibody (ANA)
titer. Progressive muscle weakness is characteristic of dermatomyositis;
patients may present with extremity complaints, and infrequently with true
arthritis. Back pain eventually develops in ankylosing spondylitis, which is
much more common in boys than in girls and is associated with the human
leukocyte antigen HLA-B27 in 90% of cases.
Mixed connective tissue diseases can have overlapping features of many of these
conditions, but the arthritis often involves small joints. Vasculitis syndromes
may also cause arthritis, with HSP being the most common vasculitis of
children. The characteristic palpable petechial or purpuric rash, especially
evident on the lower extremities, in the absence of thrombocytopenia, is key in
the diagnosis of this immunoglobulin A
–mediated vasculitis. Other inflammatory conditions, including Crohn's disease, ulcerative colitis, Reiter syndrome, Behçet's disease, and Sjögren syndrome, may also result in arthritis, constitutional symptoms, and
elevated ESR, but other features of the illnesses are usually present or
eventually manifest themselves.
II. Diagnosis
The ECG revealed a ventricular rate of 110 bpm, sinus arrhythmia, first-degree
atrioventricular block (P-R interval, 0.2 seconds), and a Mobitz II
atrioventricular block (occasional atrial beats not conducted to the ventricle)
(see Fig. 5-7). Echocardiography revealed mild aortic insufficiency and
moderate mitral regurgitation. The ANA titer was less than 1:40. The
anti-streptolysin O (ASO) and anti-DNase B titers were positive at 1:1955 and
1:680, respectively. The negative Lyme serology and the elevated titers to
streptococcal antigens made acute rheumatic fever (ARF) an important diagnostic
consideration in this case.
The Jones criteria are used to make the diagnosis of ARF (Table 5-6). The
presence of two major criteria, or one major plus two minor criteria, along
with evidence of preceding group A
β-hemolytic streptococcal (GABHS) infection make the diagnosis of ARF highly
probable. This young boy demonstrated several features of ARF, including
ausculatory evidence of valvular heart disease, a migratory polyarthritis (two
major criteria) and fever, prolongation of the PR-interval on ECG, and elevated
ESR and CRP (three minor criteria). Echocardiography revealed mild aortic
valvular insufficiency and moderate mitral valve regurgitation with thickening
of the mitral valve.
Response to aspirin and other NSAIDs is typical and is perhaps a diagnostic
clue. Children given aspirin for the arthritis of ARF have responded so well as
to go from bed-ridden to running down the halls within hours after the first
dose. Current liberal use of ibuprofen for fever or analgesia may obscure the
classic migratory pattern of arthritis in ARF due to the symptomatic relief
this agent may provide.
III. Incidence and Epidemiology
Although ARF was common in the United States until the 1960s, its incidence
decreased in the 1970s in developed nations. Regional outbreaks occurred
throughout the United States in the 1980s and 1990s, and this resurgence may be
related to the increased prevalence of strains of GABHS that are thought to be
more
“rheumatogenic.” Populations at greatest risk for ARF mirror the populations with increased
incidence of GABHS pharyngitis: children age 5 to 15 years, and older
individuals living in close quarters (e.g., military recruits). In developing
countries and in the United States before 1970, poorer socioeconomic
communities had higher rates of GABHS pharyngitis and ARF. However, over the
last two decades of the 20th century, outbreaks of ARF in the United States
have occurred predominantly in suburban and rural middle-class communities and
among military recruits.
IV. Clinical Presentation
Acute rheumatic fever is a nonsuppurative sequela of GABHS pharyngitis. The
symptoms begin approximately 1 to 3 weeks after the throat infection, but in
many cases a sore throat is not reported, even in retrospect. GABHS infections
that do not include pharyngitis are not initiators of ARF.
Approximately 80% of patients present with arthritis, and it typically is a
migratory polyarthritis with predilection for large joints. In contrast to Lyme
arthritis, the subjective pain of ARF arthritis often is much more severe than
the objective findings visible to the examiner. Analysis of fluid from an
acutely inflamed joint reveals an elevated WBC count in the range of 20,000 to
40,000 cells/mm
3 with a neutrophil predominance.
Carditis can involve any part of the heart but most typically is an endocardial
process with particular affinity for the mitral and aortic valves. Acutely the
valves demonstrate insufficiency, but the lesions progress to stenosis over
time. Involvement of the myocardium can be seen especially with the more severe
presentations of congestive heart failure. Pericarditis and epicarditis can
also complicate the picture, but they rarely occur in isolation. Carditis
develops in approximately half of patients, but it has been reported in up to
80% of patients in the more recent U. S. outbreaks. Clinical signs accepted for
evidence of carditis include appropriate murmurs, cardiomegaly, congestive
heart failure, or pericardial friction rub. The most recent update of the Jones
criteria (1992) did not consider echocardiographic evidence of valvulitis
without auscultatory findings to be sufficient to establish the presence of
carditis for ARF.
Erythema marginatum and subcutaneous nodules are seen infrequently. When
erythema marginatum is fully developed, it has an indistinct serpiginous red
border with central clearing and is nonpruritic. It is specific for ARF, but
its usefulness is limited by the fact that the rash is evanescent and is
present in fewer than 10% of patients. Subcutaneous nodules are usually a late
finding of ARF and may correlate with more severe or prolonged carditis. The
lesions are pea-sized and nontender, and they tend to be located over extensor
tendons at the elbows, knees, or Achilles.
Sydenham's chorea is a late manifestation of ARF; it can manifest after resolution of the
other features, or in isolation if the other features were never clinically
apparent. This movement disorder may start with subtle deteriorations of
handwriting before evolving into the involuntary, uncontrollable, and
purposeless choreiform movements. Due to the late onset of this feature, the
presence of Sydenham
's alone can be considered diagnostic of ARF if other causes of chorea have been
excluded.
Some of the minor criteria for ARF in some ways overlap with major criteria.
Arthralgias, painful joints without objective findings of arthritis, should be
considered only if arthritis is not used as a major criterion. However, PR
prolongation by ECG can be considered in addition to ausculatory evidence of
carditis. The ESR and CRP are significantly elevated with the acute illness,
with the ESR usually greater than 50 mm/hour and often approaching or exceeding
100 mm/hour. The fever has no characteristic pattern; it usually resolves
within 3 weeks, even without treatment.
V. Diagnostic Approach
Establishing the diagnosis of ARF involves assessing for the presence of major
and minor criteria, documenting a preceding GABHS infection, and excluding
disorders that mimic ARF. The four major criteria were discussed in the
preceding section. The minor criteria include clinical assessment (fever,
arthralgias), laboratory assessment (ESR, CRP), and evidence of
atrioventricular conduction delay by ECG (PR-interval prolongation). Evidence
of preceding GABHS infection can be obtained by any of the following:
1. Throat swab yielding a positive antigen (rapid test) or culture for GABHS at
the time of presentation or documented in the preceding weeks. Most patients do
not demonstrate a positive throat swab when presenting with ARF, having cleared
the infection during the latent period. Furthermore, a positive throat swab at
the time of ARF presentation my represent colonization, which does not
necessarily indicate a preceding GABHS pharyngitis.
2. History of scarlet fever in the preceding weeks. Scarlet fever does not occur
during the presentation of ARF, but it is sufficiently specific for GABHS
infection that it can serve as evidence of a preceding infection.
3. Elevated serum GABHS antibodies (ASO, anti-DNAase B, anti-hyaluronidase,
anti-streptokinase). A commercial agglutination assay that tests for several
streptococcal antigens is rapid and widely available, but it is less
standardized and less reproducible than quantitative titers of specific
antibodies. Quantitative ASO is positive in 80% of patients with ARF; when
three antibodies are tested quantitatively, at least one will be elevated in
95% of ARF patients.
Other clinical findings of ARF are less specific but are often part of the
clinical picture (Table 5-7).
If joint pain is the salient complaint, the differential diagnosis for ARF
includes septic arthritis, juvenile rheumatoid arthritis, SLE, Lyme disease,
postinfectious reactive arthritis, serum sickness, and malignancies. If
carditis is the main manifestation, infective endocarditis and viral
myocarditis/pericarditis should be considered.
VI. Treatment
There are three distinct aspects to the treatment of ARF: eradication of the
GABHS, secondary prophylaxis, and treatment of the ARF manifestations.
At the time of ARF diagnosis, patients require treatment for acute streptococcal
pharyngitis regardless of the results of a throat culture or rapid antigen
test. The treatment is not thought to alter the course of the active ARF
illness, but it removes the inciting agent. After completion of this
therapeutic regimen, secondary prophylaxis is begun.
Recurrence of ARF from a subsequent GABHS infection is a well-recognized
phenomenon, and the degree of cardiac involvement increases with each episode
of ARF. Both asymptomatic and symptomatic GABHS throat infections can cause
recurrences of ARF, so prevention of these infections is vital. For this
reason, continuous antibiotic prophylaxis is necessary for all patients with
ARF. The American Heart Association has provided guidelines regarding choice of
antibiotic, route and schedule of administration, and duration of prophylaxis.
The risk for recurrence is greatest within the first 5 years after the initial
attack, but in some cases lifelong prophylaxis is indicated.
After ARF, patients with valvular heart disease, require protection against
infective endocarditis in addition to their ongoing secondary prophylaxis. A
short course of additional antibiotics is required to protect the valves during
certain procedures, such as dental procedures, cystoscopy, and intestinal
surgery.
Antiinflammatory medications such as aspirin are effective treatment for the
carditis and arthritis symptoms of ARF. Corticosteroids are reserved for the
treatment of more severe carditis, where their use may more promptly suppress
the inflammation, which may be critical in patients with life-threatening
cardiac symptoms. Aspirin is highly effective for symptomatic treatment of the
arthritis of ARF, and failure to respond to this therapy should bring the
diagnosis of ARF into question. Congestive heart failure is managed with
diuretics and inotropic support as indicated by the severity of the symptoms.
VII. References
1. Special Writing Group of the Committee on Rheumatic Fever, Endocarditis, and
Kawasaki Disease of the Council on Cardiovascular Disease in the Young of the
American Heart Association. Guidelines for the diagnosis of rheumatic fever:
Jones criteria, 1992 update.
JAMA 1992;268:2069–2073.
2. Behrman RE, Kliegman RM, Jensen HB. Nelson's textbook of pediatrics, 16th ed. Philadelphia: WB Saunders, 2000.
3. Ruddy S, Harris ED, Sledge CB. Kelley's textbook of rheumatology, 6th ed. Philadelphia: WB Saunders, 2001.
4. Homer C, Shulman ST. Clinical aspects of acute rheumatic fever. J Rheumatol 1991;18[Suppl 29]:2–13.
Pictures
Book Source Details
- Book Title: Pediatric Complaints and Diagnostic Dilemmas
- Author(s): Samir S Shah MD; Stephen Ludwig MD
- Year of Publication: 2003
- Copyright Details: Pediatric Complaints and Diagnostic Dilemmas, Copyright © 2003 Lippincott Williams & Wilkins.
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Copyright Details: Pediatric Complaints and Diagnostic Dilemmas, Copyright © 2008 Williams & Wilkins.
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