Pediatrics EOR ORTHOPEDICS/RHEUMATOLOGY
What is the first line therapy for Juvenile (Idiopathic) Rheumatoid Arthritis?
First-line therapy: NSAIDs
What dx test would you order for Legg-Calve-Perthes Disease?
Hip Xray Radiographs - Early: increased density of the femoral epiphysis, widening of the cartilage space - Advanced: deformity, (+) Crescent sign (microfractures with collapse of the bone)
Autoimmune mono or polyarthritis in children under 16 years old for 6+ weeks. Dx?
Juvenile (Idiopathic) Rheumatoid Arthritis
What is this diagnostic criteria for? Diagnosis: -Primarily clinical diagnosis -Increased ESR & CRP, (+) ANA in oligoarticular, inc. ferritin -Systemic (Still's) usually associated with a negative RF & ANA -(+) Rheumatoid factor in only 15%
Juvenile (Idiopathic) Rheumatoid Arthritis
6 year old boy presents with a painless limp due to avascular necrosis of the femoral head. Dx?
Legg-Calve-Perthes Disease
Avascular necrosis of femoral head causing problems with internal rotation/ abduction and pain with walking. Its usually males, 4-10 y/o, pre-puberty DX?
Legg-Calve-Perthes Disease
Managment for Osteochondroma?
Management: Observation if asymptomatic Marginal resection including cartilage cap if it becomes painful or if located in the pelvis (pelvis MC site of malignant transformation) - usually delayed until skeletal maturity
Management of Slipped Capital Femoral Epiphysis?
Management• Non-weight bearing with crutches followed by internal fixation with pinning
Malignant bone tumors that present with pain and swelling. No improvement is noted with conservative therapy.
Osteosarcoma and Ewing Sarcoma
Pathophysiology • Femoral head epiphysis slips posterior & inferior @ growth plate
Slipped Capital Femoral Epiphysis
Risk factors Children 8-16 years, obese, African Americans, males during adolescent growth spurt (due to weakness of growth plate & hormonal changes at puberty) If seen in children before puberty, suspect hormonal or systemic disorders (eg. hypothyroidism, hypopituitarism) Dx?
Slipped Capital Femoral Epiphysis
most frequent age group of nursemaids elbow
age 1-4 years
Managment of osteosarcoma?
amputation of affected extremity and intensive chemotherapy
What dx test of choice to dx congenital hip dysplasia in a baby over 4 month?
AP Xray
How do you screen for scoliosis?
Adams forward bend test: most sensitive physical finding - thoracic or lumbar prominence on one side is seen w/ scoliosis
Complications of Slipped Capital Femoral Epiphysis?
Avascular necrosis of the hip
A practitioner is following a 10-year-old girl with scoliosis to monitor the potential progression of the curvature. At what minimal degree of curve angulation should treatment begin? A. 10 B. 20 C. 30 D. 40
B. 20
What are the two tests used to examine for Congenital Hip Displasia?
Barlow maneuver and Ortolani maneuver
Definitive dx for Osteochondroma?
Biopsy: definitive
What is the definitive dx for Osteosarcoma?
Biopsy: definitive diagnosis - malignant osteoid within the tumor & malignant sarcomatous stroma
When would you need bracing for scoliosis?
Bracing may be needed to stop progression in patients with a flexible deformity & still skeletally immature: 1) if Cobb angle increases 5+ degrees over a 3-6 month period or 2) some patients with Cobb angle 30-39 degrees
2nd MC primary bone malignancy in children & young adults (after osteosarcoma) Due to translocation between chromosomes 11 & 22 MC in Caucasian males 5-25 years old Common sites for metastasis: bone, bone marrow, & lung (Lung is common cause of death) Location: • 50% found in **diaphysis of long bones - femur (most common), pelvis, tibia, fibula are common sites
Ewing Sarcoma
Localized bone pain & swelling that may be accompanied by systemic symptoms (fever, malaise, weight loss) Physical exam: may have a palpable mass, local tenderness, or joint swelling Diagnosis • Radiographs: layered periosteal reaction "onion skin" appearance (see photo to R), lytic lesions with a "moth-eaten" appearance Dx?
Ewing Sarcoma
A 15 year old boy presents complaining of night pain in the pelvis. Since you have no idea what to do, you order an x-ray. The report comes back with a description of a mass with an "onion skin appearance." What is the most likely diagnosis?
Ewing sarcoma
In the neonate, unequal thigh folds may indicate which of the following? A. Coxa vara B. Legg-Calve-Perthes disease C. Developmental hip dysplasia D. Slipped capital femoral epiphysis
Explanations (u) A. Coxa vara is a hip deformity that would present with a decrease in the hip angle and a shift of the femoral shaft medially. (u) B. Legg-Calve-Perthes disease presents with a painless limp in children ages 4-10 due to avascular necrosis of the femoral head. (c) C. A dislocated hip displaces proximally in developmental hip dysplasia, causing a shortening of the leg that may present as unequal thigh folds. (u) D. A slipped capital femoral epiphysis is primarily an adolescent disorder with decreased range of motion in abduction and internal rotation of the hip on physical examination.
Management of Osteoid Osteoma?
Management: NSAIDs w/ serial examinations or radiographs q6 months, untreated osteoid osteoma often spontaneously resolves over several years Surgical resection for symptomatic lesions not responsive to conservative treatment
Radial head sublaxation - radial head is wedged into the stretched annular ligament, MC in children 2-5 y/o Lifting, swinging or pulling a child (longitudinal traction) while the forearm is pronated & extended Dx?
Nursemaid Elbow
Pt has scoliosis and Cobbs angel is under 25 degree. What is the management?
Observation: Cobb angle under 25 degrees: & Risser grade 0 to 2 @ time of presentation - regular follow up to monitor progression every 6-9 months, bracing may be recommended if Cobb angle increases 5 degrees or more over a 3-6 month period
Apophysitis of the tibial tuberosity (inflammation of the patellar tendon @ the insertion of the tibial tubercle) due to overuse (repetitive stress microtrauma) or small avulsions from repetitive knee extension & quadriceps contraction. Pathophysiology: • The apophysis is a muscle-tendon-bone attachment that is subject to injury from repetitive stress or an acute avulsion injury Risk factors: • Most common in males, 10-15 years, during growth spurts, athletes Dx?
Osgood-Schlatter Disease
Caused by rupture of the growth plate at the tibial tuberosity, which causes stress on the patellar tendon. It most commonly occurs in rapidly growing adolescents (10-15-years-old) and is five times more common among those active in sports and up to three times more common in boys. Dx?
Osgood-Schlatter Disease
Clinical Manifestations: Activity-related anterior knee pain & swelling (running, jumping, kneeling) & relieved with rest Prominence, swelling & tenderness to the anterior tibial tubercle Dx?
Osgood-Schlatter Disease
This benign tumor typically presents as a painless mass and appears in plain film radiographs as a stalk or broad-based projection from the surface of the bone. Dx?
Osteochondroma
Cartilage-capped benign chondrogenic bony overgrowth arising on the external surface of a bone & areas of tendon insertion (proximal tibia, femur, and proximal hemurus) MC benign bone tumor, 10% may become chondrosarcomas MC between 10-20 years of age & in males, begins in childhood & grows until skeletal maturity Clinical Manifestations • Painless, palpable mass - may develop symptoms of neurovascular compression Dx?
Osteochondroma (Benign MSK Neoplasia)
Benign bone tumor characterized by a small radiolucent nidus (less than 1- 1.5 cm in diameter) Most commonly presents in second decade, M>F Locations: proximal femur MC, tibia, remainder of the femur, spine Pathophysiology • The nidus produces high levels of prostaglandins Clinical Manifestations Progressively increasing pain worse @ night & unrelated to activity - the pain is relieved within 20-25 minutes of administration of NSAIDs May develop a limp, localized tenderness, & limitation of range of motion. Dx?
Osteoid Osteoma
Clinical Manifestations: Localized bone pain (may occur after injury) and can be worse @ night, progressively worsening. Joint swelling without systemic symptoms Physical exam: palpable soft tissue mass (may be tender to palpation) Diagnosis • Radiographs: "hair on end" or "sunburst" appearance (see photo) d/t tumor spicules of calcified bone radiating in right angles is classic (not specific) Other findings: mixed sclerotic & lytic lesions Codman's triangle: ossification of raised periosteum (can be seen in Ewing sarcoma) Dx?
Osteosarcoma
Management for Congenital Hip Dysplasia < 6 months of age?
Pavlik harness
"Cobb's Angle"
Preferred method for measuring amount of scoliosis curvature. Radiographs: Cobb's angle > 10 degrees measured on AP & lateral films
Management of Osgood-Schlatter disease
RICE NSAIDs/tylenol rare cases- surgery referral
What test can you order to confirm scoliosis?
Radiographs: Cobb's angle > 10 degrees measured on AP & lateral films
Initial dx test for Osteochondroma?
Radiographs: often pedunculated (narrow stalk) that grows away from the growth plate & involves the medullary tis
Dx test for Osteoid Osteoma?
Radiographs: small round lucency (nidus) with a sclerotic margin - CT/MRI more sensitive
T/F: Risk Factors for Legg-Calve-Perthes Disease Children 4-10 years old, 4x M>F, obesity, coagulation abnormalities (Factor V Leiden)
True
What is the test of choice to dx congenital hip dysplasia in a baby under 4 months old?
Ultrasound
Management for Congenital Hip Dysplasia 6 months - 2 years of age?
closed reduction in the OR (may need athrogram)
physical exam of nursemaids pertinent negatives
no bony tenderness or swelling child also resists movement of the arm
Initially the child will cry and hold the affected arm close to their body with the elbow flexed and forearm pronated. Dx?
nursemaids elbow
A 14 year-old male athlete presents complaining of a painful "bump" on his right lower extremity just below his knee and associated pain during and after activity. Examination of the right leg reveals a prominent tender tibial tubercle. Which of the following is the most likely diagnosis? A. Osgood-Schlatter disease B. Osteochondritis dissecans C. Tibia vara D. Patellofemoral syndrome
A. Osgood-Schlatter disease
- Malignant tumor of osteoblastic proliferation, MC primary bone malignancy in children & young adults - 90% occur in the **metaphysis of the long bones (distal femur MC), then proximal tibia & proximal humerus - MC METs to the lung (MCC of death) - Gene is associated with familial retinoblastoma What type of malignant neoplasm? A. Osteosarcoma B. Ewing Sarcoma
A. Osteosarcoma
T/F: Nursemaid elbow is a clinical diagnosis (radiographs are normal)
True
T/F: Osteosarcoma is Bimodal • MC in adolescents, second peak 50-60 years especially if history of Paget disease of the bone or radiation therapy
True
A mother brings her 14 month-old son to your clinic. Earlier today she lifted her son by grabbing him by the wrists and pulling him up off the floor. The child is sitting in his mother's lap with his left forearm is extended and in pronation. He is refusing to move the left arm, forearm or wrist. The arm and joints appear normal with no noted deformities, edema or erythema. Distal pulses and capillary refill are normal and he can move his fingers. Which of the following is the most likely diagnosis? A. Nursemaid's elbow B. Fractured left wrist C. Osteochondritis dissecans D. Child abuse
(c) A. This clinical history is classic for radial head dislocation or nursemaid's elbow. (u) B. There is no edema, deformity or erythema to suggest a fractured wrist. (u) C. Osteochondritis dissecans is avascular necrosis of subchondral bone, most commonly seen during adolescence.(a) D. Although child abuse could be suspected the clinical history best describes nursemaid's elbow.
What is the 2nd line Tx or Tx for severe Juvenile (Idiopathic) Rheumatoid Arthritis
- Anakinra (interleukin-1 receptor inhibitor) - Methotrexate - Leflunomide
Management of Osgood-Schlatter Disease
Management: Conservative: mainstay of treatment - RICE, NSAIDs, quadriceps stretching, knee immobilization - most syx resolve within 12-24 months Surgery only in refractory cases (if done, usually performed after growth plate has closed)
A 12 year-old female presents for a routine sports physical. The physical exam reveals asymmetry of the posterior chest wall on forward bending. This is most consistent with which of the following? A. spondylolysis B. spondylolisthesis C. scoliosis D. herniated disc
C. Scoliosis
An obese 12 year-old male presents with a 1 month history of right thigh pain worsened with weight bearing. Examination is normal, however, radiographs reveal a posterior and medial displacement of the femoral head. Which of the following is the most likely diagnosis? A. Legg Calvé Perthes disease B. Developmental dysplasia of the hip C. Slipped capital femoral epiphysis D. Osgood-Schlatter disease
C. Slipped capital femoral epiphysis
Managment of nursemaid elbow?
CLOSED REDUCTION: place pressure on the radial head w/ supination of the elbow followed by flexion of the elbow (supination-flexion technique) Observe the child for normal function, if the child uses the arm after 15 minutes, no XR needed If no use after 15 minutes, consider XR to r/o fracture or reattempt reduction
Management of Ewing Sarcoma?
Chemotherapy followed by limb-sparing resection when possible Radiation therapy when complete excision is not possible
Abnormality in the shape &/ stability of the shape of the femoral head & acetabulum • Examination of the hip is performed during newborn assessment soon after birth & at every well-check visit until about 9 months of age &/ the child is walking independently What condition?
Congenital Hip Dysplasia
Physical ExaminationAssessed for hip instability, asymmetry, or limited abduction: Barlow maneuver: gentle adduction without downward pressure to feel for dislocatability, resulting in a click, clunk or jerk Ortolani maneuver: abduction & elevation to feel for reducibility, resulting in a click, clunk or jerk Other findings may include: asymmetry & restricted hip abduction (look @ skin folds, femur length, or gait) In infants 3+ months, the dislocation may become relatively fixed & the Galeazzi test can be used instead • Galeazzi's test: check if knees are @ unequal heights when the hips & knees are flexed - the dislocated side will be lower What condition?
Congenital Hip Dysplasia
A 13 year-old girl reports two weeks of worsening right knee pain with no history of antecendent injury or recent trauma. She reports frequent episodes of nighttime awakening with knee pain in the past two weeks. Examination of the knee reveals edema and a tender mass over the anterior proximal right tibia. Her knee exam is otherwise within normal limits. Radiographs of the right knee show a lytic mass with a multi-laminated periosteal reaction involving the proximal anterior tibia. What is the most likely diagnosis? A. Ewing sarcoma B. Osteochondroma C. Multiple myeloma D. Osteoid osteoma
Correct Answer: A. Ewing Sarcoma (c) A. The distinctive feature of Ewing sarcoma is the radiographic appearance of a periosteal "onion skin" reaction. (u) B. This benign tumor typically presents as a painless mass and appears in plain film radiographs as a stalk or broad-based projection from the surface of the bone. (u) C. The classic radiographic appearance of multiple myeloma is a lytic lesion but this is a condition that is seen in a much older population and is more likely to present with back pain. (u) D. Although the presentation may be similar to Ewing's sarcoma, the radiographs in osteoid osteoma typically show a round lucency surrounded by sclerotic bone.
An obese 15 year-old male presents with complaint of a limp and right knee pain for two weeks. He denies recent trauma or history of previous injury. Physical examination of the right knee is unremarkable. Examination of the right hip reveals pain with passive range of motion and limited internal rotation and abduction. Flexion of the hip results in external rotation of the thigh. Gait is antalgic with the right hip externally rotated. Which of the following radiographic findings supports the most likely diagnosis? A. Displacement of the femoral epiphysis B. Irregularity and fragmentation of the joint space C. Capsular swelling of the joint D. Dislocation of the hip
Correct answer: A. Displacement of the femoral epiphysis Explanations (c) A. This patient has slipped capital femoral epiphysis (SCFE) and the classic x-ray findings will demonstrate displacement of the femoral head rotation of the femoral neck anteriorly. (u) B. Irregularity and fragmentation of the joint space is associated with avascular necrosis of the femoral head as seen in Legg-Calve-Perthes disease. This typically occurs in a younger male population and is not associated with the classic externally rotated hip with ambulation seen in SCFE. (u) C. Capsular swelling of the joint may be seen in transient synovitis of the hip but is not associated with SCFE. (u) D. Hip dislocation at this age is associated with major trauma, such as that sustained in a fall from height or dashboard injury. SCFE does not lead to hip dislocation.
An examination of the hips of a newborn, a "clunk" is noted with abduction of the right hip to almost 90 degrees while lifting the greater trochanter. Which of the following is the most likely diagnosis? A. Ligamentum teres rupture B. Legg-Calve-Perthes disease C. Slipped capital femoral epiphysis D. Developemental dysplasia of the hip
D- the Ortolani test is positive in the newborn patient with developmental dysplasia of the hip. The test is performed by lifting the greater trochanter while abducting the hip to 90 degrees.
A 13 year-old patient presents to the orthopedic surgeon's office for a follow-up evaluation for scoliosis. The Cobb angle is measured at 65 degrees. Which of the following is indicated at this time? A. Observation for 4 months B. Physical therapy C. Leg length measurements D. Pulmonary function testing
D. Pulmonary function testing
A 14 year-old male who is overweight presents with complaints of left knee and anteromedial thigh pain for the past month. He states the pain gets better with rest and denies any known trauma. On examination of the gait, a slight limp is noted. X-ray films of the left knee are normal. The most likely diagnosis is A. genu valgum. B. Legg-Calve-Perthes disease. C. Osgood-Schlatter disease. D. slipped capital femoral epiphysis.
D. slipped capital femoral epiphysis.
How do you diagnose congenital hip dysplasia?
Diagnosis Clinical w/ confirmation with imaging Ultrasound: often used in children under 4 months of age AP Xray in older children (over 4 months)
Legg-Calve-Perthes disease (LCP) or Slipped Capital Femoral Epiphysis (SCFE)
Differential for atraumatic pediatric hip pain or limp Legg-Calve-Perthes Disease is a PAINLESS LIMP
A 12 year-old male presents with pain in his left leg that is worse at night. Aspirin relieves the pain and the patient denies injury. On examination, there is point tenderness over the tibia, and the patient has a slight limp that favors the left leg. Radiographs show a 1 cm radiolucent nidus surrounded by osteosclerosis. Which of the following is the most likely diagnosis? A. Osteosarcoma B. Legg-Calve-Perthes disease C. Osgood-Schlatter disease D. Osteoid osteoma
Explanations (u) A. Osteosarcoma and Ewing sarcoma are malignant bone tumors that present with pain and swelling. No improvement is noted with conservative therapy. (u) B. Legg-Calve-Perthes disease is avascular necrosis of the hip affecting boys ages 4-10. (u) C. Osgood-Schlatter disease is inflammation of the tibial tuberosity affecting mainly boys in the ages of 10-15. Commonly associated bilaterally and due to jumping. (c) D. Osteoid osteoma is a benign tumor in children age 5 to 20, presents with increasing pain, worse at night and relieved by aspirin.
If no response to NSAIDs with Juvenile (Idiopathic) Rheumatoid Arthritis, what is the next pharm tx?
Glucocorticoids if no response to NSAIDs. (also physical therapy)
Dx of Osgood-Schlatter Disease
Imaging usually not necessary in classic presentations Radiographs: elevation, heterotopic ossification &/or bone fragmentation of the tibial tuberosity
Most Common Signs and Symptoms: Joint inflammation-large joints first Joint contracture (shortening of joint)- bc of bone overgrowth and restructuring Joint damage Alteration in growth Stiffness Weakness in muscles near affected joints Some children have c/o pain Swollen lymph nodes-Systemic JRARash and fever-Systemic JRA Dx?
JRA
-Aka juvenile idiopathic arthritis -Causes joint inflammation and stiffness for more than six weeks in a child aged 16 or younger -50,000-300,000 children in the US Dx?
Juvenile (Idiopathic) Rheumatoid Arthritis
Cause in unknown but considered to be: -Autoimmune disorder-body mistakenly identifies some of its own cells and tissues as foreign -The immune system begins to attack healthy cells and tissues-belief that child had a genetic marker that is triggered by environmental factors such as a virus -Like other forms of arthritis, JRA is characterized by times when symptoms flare up and times when symptoms disappear
Juvenile (Idiopathic) Rheumatoid Arthritis
Avascular necrosis of the proximal femur. What condition?
Legg-Calve-Perthes Disease
Clinical Manifestations - Painless limping for weeks (worsen w/ continued activity especially @ the end of the day) - May have mild intermittent hip, thigh, knee or groin pain - may have an antalgic or Trendelenburg gait - Restricted ROM (loss of abduction & internal rotation) - may have atrophy of the thigh muscles, may lag in bone age & height Dx?
Legg-Calve-Perthes Disease (Avascular necrosis of the proximal femur)
Management of Legg-Calve-Perthes Disease?
Management • Observation: activity restriction (non-weight bearing initially) with orthopedic follow up is initial treatment in most cases (usually self-limiting w/ revascularization within 2 years) - May advocate for protected weight bearing during early stages until reossification is complete -Physical therapy or brace/cast, NSAIDs for pain management • Surgical: pelvic osteotomy may be indicated in some children >8 years of age, more advanced disease (lateral pillar B and B/C)
Lateral curvature of the spine Dx?
Scoliosis
Clinical Manifestations: Ipsilateral dull, achy hip, groin, thigh or knee pain with a painful limp worse with activity Physical examination: externally rotated leg on the affected side (limited internal rotation abduction & flexion on ROM of the hip), altered gait • Drehmann sign: while in supine position, hip externally rotates and abducts w/ passive hip flexion dx?
Slipped Capital Femoral Epiphysis
Diagnosis Slipped Capital Femoral Epiphysis • XR: posterior displacement of femoral epiphysis, similar to ice cream slipping off a cone - best seen on frog-leg lateral pelvis or lateral hip view • Widening of joint space, decrease in epiphyseal height & Steel sign - double density from superimposition of epiphysis & metaphysis Dx?
Slipped Capital Femoral Epiphysis
Displacement of the femoral head (epiphysis) from the femoral neck through the growth plate. Dx?
Slipped Capital Femoral Epiphysis
At what Cobb angel would you recommend surgical correction for scoliosis?
Surgical correction may be an alternative to bracing if 40+ degrees & Risser grade 0-2 (skeletally immature)