Chronic Lower Extremity Pain
Radiology Cases in Pediatric Emergency Medicine
Volume 6, Case 11
Maria Victoria Spurbeck, MD
Martin I. Herman, MD
LeBonheur Children's Medical Center
University of Tennessee School of Medicine
This is a nearly 5-year old female with an 18 month
history of difficulty walking. She presents to the ED
with her parents because of an acute episode (3-4
hours) of lower extremity pain and associated with her
legs "giving out". Her parents deny any history of
trauma, fever or recent infections. Mother reports that
she has complained of pain many times before. Usually
the complaints are worse in the morning and at night.
She cries when she tries to put shoes or socks on or
cries when her legs are touched during episodes of
pain. She now walks with a limp and has a stiff left
knee. Mother has also noticed firm nodules along her
hip and lower extremities for 12 months and a lifelong
history of poor weight gain despite a good appetite.
She was evaluated by a genetics consultant at which
time blood was taken and radiographs were obtained.
Her mother does not know the results of these tests.
PMH: She is the product of twin gestation via
spontaneous vaginal delivery. Birth weight 1.8 kg. She
remained hospitalized for 5 days because of jaundice
and a urinary tract infection. There are no other
hospitalizations or surgeries.
Her early development was normal. She walked at
about 11 months, but suffered a minor fall and refused
to walk again until 18 months of age. At about 30
months of age, her parents noted complaints of pain in
her lower extremities, refusal to walk, tenderness of
thighs and ankles, inability to climb stairs, and difficulty
getting up from bed. She has never been able to run.
Her language development has been good.
Family history: Her twin brother and other sibling
are healthy. Her father had corrective surgery of both
lower extremities as a child, but the reason for this is
not known. Her mother had a kidney tumor as a child,
but the type of tumor is not known. There is a history of
arthritis in paternal uncles. Mother specifically denies
any family history of sickle cell disease, lupus,
rheumatoid arthritis or muscular diseases.
Social History: She attends preschool and is able to
keep up intellectually, but not in physical activities.
Exam: VS T37.6, P110, R28, BP 105/56, Ht. 91 cm
(5th%ile for a 3 year old ), Wt. 11.8 kg (5th%ile for a 3
year old), HC 48 cm ( 5th%ile for a 30 month old ).
This is a 5 year old female child, appearing small for
age, who is awake and alert with facial dysmorphism.
She is thin and appears chronically ill. Her head is
atraumatic with hypoplastic midline facial features.
PERRL, EOMI. TM's normal. Oral mucosa clear, pink
and moist. Neck supple with full range of motion.
Heart regular without murmurs, rubs or gallops. Lungs
clear. Abdomen soft, non-tender, rounded without
distention. No hepatosplenomegaly or masses.
Extremities: Bilateral upper and lower extremity
muscle wasting. There are hypopigmented flat papules
overlying her interphalangeal joints. There are
non-tender, firm, irregularly shaped subcutaneous
nodules palpated over both lower extremities. There is
decreased range of motion of her left knee. Her other
joints have normal range of motion. Her skin is warm
with brisk capillary refill. There are irregularly shaped
hyperpigmented macules along her lower extremities
and abdomen with firm subcutaneous nodules scattered
along her lateral lower extremities and hips. There are
no bruises. No cranial nerve deficits are noted.
Sensation is intact. UE motor strength 5/5 bilaterally.
LE strength 3/5 in the proximal muscles and 4/5 in the
distal muscles. Plantars are downgoing bilaterally.
Cerebellar function is intact. DTR 2+ bilaterally and
Radiographs of her painful lower extremities are
What do these radiographs show?
A. Generalized subcutaneous calcifications
B. Vascular calcifications
C. Myositis ossificans
These radiographs demonstrate extensive soft
tissue calcifications. The calcifications do not follow any
particular vascular pattern and are not attached to
What diagnosis is most likely?
A. Juvenile Rheumatoid Arthritis
B. Systemic Lupus Erythematosis
C. Rheumatic Fever
D. Congenital Myositis Ossificans
E. Juvenile Dermatomyositis
Juvenile Dermatomyositis (JDMS) is a rare
rheumatoid disorder which typically involves
inflammation of the skin and striated muscles. The
pathophysiology of JDMS includes muscle lesions with
a mix of degeneration and regeneration of muscle
fibers, various muscle fiber sizes and perivascular
lymphocytic infiltrates. There is also vasculitis of small
arteries, venules, and capillaries of the skin, muscle and
GI tract. Although unsure of the etiology there is an
association with Coxsackie B viral infection. Patients
with JDMS pass through 4 phases: 1) Prodrome with
nonspecific aches and pains. 2) Progressive muscular
weakness. 3) Persistent active disease. 4) Recovery
with contractures and calcinosis.
The onset is usually insidious during phase 1 with
vague aches and pains of the limbs, edema of the
hands and feet, low grade fever and sometimes a
nonspecific rash. Stage 2 includes a violaceous
heliotropic rash along the eyelids, ruddy and atrophic
lesions along the extensor surfaces of the knees,
elbows and hands, and periungual erythema. The skin
findings may come before or after muscle weakness.
The muscular complaints usually are tenderness
and symmetrical weakness of the proximal muscles and
anterior neck flexors while sparing the face. The
disease can involve palatopharyngeal muscles which
lead to regurgitation, a nasal voice and aspiration.
When respiratory muscles are involved, patients often
have a weak cough and are at risk for aspiration
pneumonia, which may lead to respiratory failure. This
stage is also associated with a risk of GI hemorrhage
The third stage has a variable course. A small
percentage of patients have severe vasculitis and
progressive muscular weakness unresponsive to
therapy. Many have a limited course lasting 1-2 years.
Others like our patient, have a chronic pattern with
frequent exacerbations and remissions lasting 2-5 years
with progressive muscular atrophy and soft tissue
calcifications. The recovery phase is characterized by
muscle wasting, contractures, and pigmentary changes.
Swischuk L, Johns S. Bones and Soft Tissues
(chapter 4). In: Differential Diagnosis Pediatric
Radiology. Baltimore, MD, Williams and Wilkins, 1995,
Schaller JG. Dermatomyositis (chapter 153). In:
Nelson WE, Behrman RE, Kliegman RM, Arvin AM
(eds). Nelson Textbook of Pediatrics, 15th ed.
Philadelphia, PA, W.B. Saunders Company, 1996,
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