Radiology Cases in Pediatric Emergency Medicine Volume 6, Case 11
Maria Victoria Spurbeck, MD Martin I. Herman, MD

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.

Birth History: 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.

Developmental History: 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.

Examination: 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 symmetric. Radiographs of her painful lower extremities are obtained.

What do these radiographs show?
A. Generalized subcutaneous calcifications B. Vascular calcifications C. Myositis ossificans D. Artifacts These radiographs demonstrate extensive soft tissue calcifications. The calcifications do not follow any particular vascular pattern and are not attached to bone.

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 and perforation.

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

Copyrighted:-Radiology Cases in Pediatric Emergency Medicine Volume 6, Case 11 Loren Yamamoto, MD, MPH, Professosr of Pediatrics, University of Hawaii John A. Burns School of Medicine.Loreny@hawaii.edu