Ortho

Question 1

Clubfoot / Congenital talipes equinovarus

• Tx: Ponseti method 3 stages
  
  • 1st Stretching and casting. This consists of repositioning and recasting once weekly for 6-8 weeks.
  • 2. If unsuccessful, surgery may be needed
  • 3. Long-term bracing to maintain corrected position

Answer 1

Clubfoot / Congenital talipes equinovarus

• Tx: Ponseti method 3 stages
  
  • 1st Stretching and casting. This consists of repositioning and recasting once weekly for 6-8 weeks.
  • 2. If unsuccessful, surgery may be needed
  • 3. Long-term bracing to maintain corrected position

Question 2

Metatarsus adductus

• Usually noticed in infancy (less than ___yo)
• Tx:
  
  • Resolves spontaneously within the 1st year, almost always by ___yr. Can try manipulative stretching several times daily, but might not hasten condition.
  • Casting/splinting may be useful if persists beyond 6mo

Answer 2

Metatarsus adductus

• Usually noticed in infancy (<1yo)
• Tx:
  
  • Resolves spontaneously within the 1st year, almost always by 3-4yr. Can try manipulative stretching several times daily, but might not hasten condition.
  • Casting/splinting may be useful if persists beyond 6mo

Question 3

Internal tibial torsion

• Most common cause of intoeing age ___yo.
• Medial rotation of the tibia
• Tx:
  
  • Reassurance. Typically resolves spontaneously by _____yo without intervention.
  • Reserve surgery for severe cases causing functional or cosmetic deformity that persist past ___yo.

Answer 3

Internal tibial torsion

• Most common cause of intoeing age 1-3yo. Presents in 2nd year when child starts walking
• Medial rotation of the tibia
• Tx:
  
  • Reassurance. Typically resolves spontaneously by 8-10yo without intervention.
  • Reserve surgery for severe cases causing functional or cosmetic deformity that persist past 8yo.

Question 4

Internal femoral torsion / Femoral Anteversion

• Present in pre-school children ____yo
• Pt:
  
  • More internal rotation
  • ____ run pattern where legs “flip” outward in the swing phase
  • Sit in a “___” position. Find it difficult to sit cross-legged
• Tx:
  
  • Reassurance that is a common normal variant. Most cases resolve by ___yo.
  • Surgical correction considered in rare cases when children are at or near skeletal maturity experience pain or significant gait disturbance

Answer 4

Internal femoral torsion / Femoral Anteversion

• Present in pre-school children 3-6yo
• Pt:
  
  • More internal rotation
  • “Egg beater” run pattern where legs “flip” outward in the swing phase
  • Sit in a “W” position. Find it difficult to sit cross-legged
• Tx:
  
  • Reassurance that is a common normal variant. Most cases resolve by 11yo.
  • Surgical correction considered in rare cases when children are at or near skeletal maturity (>11yo) experience pain or significant gait disturbance

Question 5

Congenital Scoliosis

• Scoliosis that results from presents of 1 or more congenital vertebral malformations (CVMs)
  
  • Ex: Hemivertebrae, butterfly vertebrae, congenital vertebral fusions
• When presents with pt with congenital scoliosis, think _____ syndrome or ____ association
• Management
  
  • Refer to ortho.
  • If signs of CNS dysfunction or midline cutaneous lesions, _____
  • it s recommended that these children undergo screening with _____

Answer 5

Congenital Scoliosis

• Scoliosis that results from presents of 1 or more congenital vertebral malformations (CVMs)
  
  • Ex: Hemivertebrae, butterfly vertebrae, congenital vertebral fusions
• When presents with pt with congenital scoliosis, think Klippel-Feil syndrome or VACTERL association
• Management
  
  • Refer to ortho.
  • If signs of CNS dysfunction or midline cutaneous lesions, spinal imaging
  • Because renal abnormalities are present in 1/3 of children with CVM, it s recommended that these children undergo screening with renal US

Question 6

Idiopathic Scoliosis

• Screening: Scoliometer
  
  • > __ (BMI 85%) or >__ (BMI <85%) degrees is considered positive and should be evaluated further with standing radiographs of spine.
    • __-__ degrees warrants reexamination in months
    • > __ degrees: Radiologic evaluation for Cobb angle
• Physical exam: Visual inspection of back and Adam’s forward bending test.
  
  • Assess scapular asymmetry, prominence of rib cage, asymmetry of hips
• Dx: Cobb angle >___ degrees
• Tx:
  
  • Mild cases Cobb angle ___ degrees of 1st visit with curvature >___ degrees
    
    • ___ -___ degrees: Bracing
    • ___-___ degrees: Bracing or surgery
      
      • Bracing generally ineffective for >__ degrees
    • > ___ degrees: Surgery

Answer 6

Idiopathic Scoliosis

• Screening: Scoliometer
  
  • > 5 (BMI 85%) or >7 (BMI <85%) degrees is considered positive and should be evaluated further with standing radiographs of spine.
    • 5-9 degrees warrants reexamination in months
    • > 10 degrees: Radiologic evaluation for Cobb angle
• Physical exam: Visual inspection of back and Adam’s forward bending test.
  
  • Assess scapular asymmetry, prominence of rib cage, asymmetry of hips
• Dx: Cobb angle >10 degrees
• Tx:
  
  • Mild cases Cobb angle <20: serial radiographs every 6 months
  • Progression of curvature >25 degrees of 1st visit with curvature >30 degrees
    
    • 30 -39 degrees: Bracing
    • 40-49 degrees: Bracing or surgery
      
      • Bracing generally ineffective for >45 degrees
    • > 50 degrees: Surgery

Question 7

Kyphosis

• Postural kyphosis:
  
  • Up to _____ degrees of thoracic kyphosis using Cobb angle measurement on lateral radiographs is considered normal
  • Tx: Reassurance. Bracing, PT, NSAIDs. For severe cases (>80 degrees), surgery is often indicated

Answer 7

Kyphosis

• Postural kyphosis:
  
  • Up to 45 degrees of thoracic kyphosis using Cobb angle measurement on lateral radiographs is considered normal
  • Tx: Reassurance. Bracing, PT, NSAIDs. For severe cases (>80 degrees), surgery is often indicated

Question 8

Scheuermann disease

- Kyphosis caused by >=\_\_\_ degree anterior wedging of >=\_\_\_\_ consecutive thoracic vertebrae 
- Tx:
    - Initial management is conservative: avoiding pain triggers, NSAIDS, exercises to increase strength and flexibility
    - Curves >\_\_\_ degrees in skeletally immature may be improved with bracing, and curves >\_\_\_ degrees that are uncontrolled by bracing sometimes require surgery.

Answer 8

Scheuermann disease

- Kyphosis caused by >5 degree anterior wedging of >3 consecutive thoracic vertebrae 
- Tx:
    - Initial management is conservative: avoiding pain triggers, NSAIDS, exercises to increase strength and flexibility
    - Curves >60 degrees in skeletally immature may be improved with bracing, and curves >80 degrees that are uncontrolled by bracing sometimes require surgery.

Question 9

Pectus Excavatum

• Depression of midsternum
• Pt: By itself, rarely causes respiratory or cardiac problems through some complain of exercise intolerance

Pectus Carinatum (Pigeon chest/breast)

• Anterior protrusion of the sternum with lateral depression of the costal cartilages.
• Pt: Rarely symptomatic

Answer 9

Pectus Excavatum

• Depression of midsternum
• Pt: By itself, rarely causes respiratory or cardiac problems through some complain of exercise intolerance

Pectus Carinatum (Pigeon chest/breast)

• Anterior protrusion of the sternum with lateral depression of the costal cartilages.
• Pt: Rarely symptomatic

Question 10

Asphyxiating Thoracic Dystrophy (____ Syndrome)

• Inheritance?____
• Pt: _____, ___, ______ disease
• Tx: Standard approach is to provide long-term ventilation, if needed, early in life. Those less severely affected may only need ventilator support with respiratory infections.

Answer 10

Asphyxiating Thoracic Dystrophy (Jeune Syndrome)

• AR
• Pt: Short ribs, small rib cage, renal disease
• Tx: Standard approach is to provide long-term ventilation, if needed, early in life. Those less severely affected may only need ventilator support with respiratory infections.

Question 11

Developmental Dysplasia of Hip

• Screening: Screen until walking normally (~2yo)
  
  • For less than __ mo: ___
  • For ___mo: ____
• Dx:
  
  • less than __mo: ___
  • ___mo: ___
• Tx: Refer to Ortho
  
  • less than __mo: ____
  • __mo: ____

Answer 11

Developmental Dysplasia of Hip

• Screening: Screen until walking normally (~2yo)
  
  • For <3 mo (8-10 weeks): Barlow and Ortolani.
  • For >8-10 weeks: Galeazzi test
• Dx:
  
  • <6mo: Hip US at 4-6 weeks of life (closer to 6 weeks)
  • > 6mo: XR frog leg of hip
• Tx: Refer to Ortho
  
  • <6mo: Pavlick’s Harness for approx 3 mo
  • 6mo-2yo: Spica splint to attempt closed reduction. If these procedures are unsuccessful, surgery is indicated

Question 12

Leg length discrepancy

• Path:
  
  • Fractures of the femur have the highest risk of overgrowth
  • Overgrowth syndromes
  • Idiopathic
• Management
  
  • Discrepancies <2-2.5cm: Small shoe lifts
  • Discrepancies 2-5cm: Candidate for epiphysiodesis
  • For larger discrepancies, more complicated surgeries

Answer 12

Leg length discrepancy

• Path:
  
  • Fractures of the femur have the highest risk of overgrowth
  • Overgrowth syndromes
  • Idiopathic
• Management
  
  • Discrepancies <2-2.5cm: Small shoe lifts
  • Discrepancies 2-5cm: Candidate for epiphysiodesis
  • For larger discrepancies, more complicated surgeries

Question 13

Unilateral Coxa Vara

• When angle between neck and shaft of femur is less than ___
• Pt:
  
  • Trendelenburg limp: Abnormal but painless gait pattern
  • Limb length discrepancy, prominent greater trochanter, limitation of abduction and internal rotation of the hip

Answer 13

Unilateral Coxa Vara

• When angle between neck and shaft of femur is <110-120
• Pt:
  
  • Trendelenburg limp: Abnormal but painless gait pattern
  • Limb length discrepancy, prominent greater trochanter, limitation of abduction and internal rotation of the hip

Question 14

Coxa Valga

• Femoral neck shaft angle is >___ degrees
• Pt:
  
  • Leg lengthening (rather than shortening) of the affected limb, which leads to circumduction and limited ROM in _____

Answer 14

Coxa Valga

• Femoral neck shaft angle is >139 degrees
• Pt:
  
  • Leg lengthening (rather than shortening) of the affected limb, which leads to circumduction and limited ROM in adduction (other than abduction)

Question 15

Transient Synovitis

- Joint aspiration is the reference standard in differentiating toxic synovitis from septic arthritis.  - Tx: 
- After ruling out a disorder requiring specific tx and intervention, NSAIDs and relative rest are mainstays of tx. Full recovery within 1-4 weeks

Answer 15

Transient Synovitis

- Joint aspiration is the reference standard in differentiating toxic synovitis from septic arthritis.  - Tx: 
- After ruling out a disorder requiring specific tx and intervention, NSAIDs and relative rest are mainstays of tx. Full recovery within 1-4 weeks

Question 16

Septic (Pyogenic) Arthritis
- Dx: Joint aspiration is the best test for confirming the diagnosis
Kocher criteria to distinguish septic joint from transient synovitis
1) ____
2)____
3) ____
4) ____
CRP >___ (not part of the original Kocher criteria)
1= 3% probability of septic arthritis, 2= 40%, 3= 93%, 4= 99%
Rec: draw CBC, ESR, and CRP in all pts with clinical concern for septic arthritis or transient synovitis

Answer 16

Septic (Pyogenic) Arthritis
- Dx: Joint aspiration is the best test for confirming the diagnosis
Kocher criteria to distinguish septic joint from transient synovitis
1) Refusal to bear weight
2) Fever, Temp >38.5/101.3
3) ESR >40
4) WBC>12,000
CRP >25 (not part of the original Kocher criteria)
1= 3% probability of septic arthritis, 2= 40%, 3= 93%, 4= 99%
Rec: draw CBC, ESR, and CRP in all pts with clinical concern for septic arthritis or transient synovitis

Question 17

Legg Calve Perthes Disease

• ______ of the femoral head
• Ddx: Secondary to an underlying disease - corticosteroid use, sickle cell disease
• Pt:
  
  • Hip pain and a limp (90% unilateral). Pain is insidious and worsens with activity
• Providers should consider the potential for interarticular hip pathology in any pt presenting with a limp and painful, restricted hip ROM. There should be a low threshold for obtaining radiographs in these pts, which should include an AP view of the pelvis and a “frog leg” lateral view of the hip. It is important to remember that hip pathology may present as knee pain, and the hip should always be examined in pts presenting with knee pain.
• Tx:
  
  • Patients should be made ____
  • Supportive care with PT and anti-inflammatory medications are the best treatment choice. Complete recovery typically takes ____.

Answer 17

Legg Calve Perthes Disease

• Avascular necrosis (osteonecrosis) of the femoral head
• Ddx: Secondary to an underlying disease - corticosteroid use, sickle cell disease
• Pt:
  
  • Hip pain and a limp (90% unilateral). Pain is insidious and worsens with activity
• Providers should consider the potential for interarticular hip pathology in any pt presenting with a limp and painful, restricted hip ROM. There should be a low threshold for obtaining radiographs in these pts, which should include an AP view of the pelvis and a “frog leg” lateral view of the hip. It is important to remember that hip pathology may present as knee pain, and the hip should always be examined in pts presenting with knee pain.
• Tx:
  
  • Patients should promptly be made non-weight bearing of the affected limb and instructions for activity restriction to reduce strain across the femoral head. Prompt referral to an orthopedist.
  • Supportive care with PT and anti-inflammatory medications are the best treatment choice. Complete recovery typically takes 4-5 years.

Question 18

Slipped Capital Femoral Epiphysis
- Path: Posterior and inferior displacement of capital femoral epiphysis from femoral neck.

• RF: Obese child during early adolescence and near time of peak linear growth, boys, African Americans, those with endocrine dysfunction
  
  • Associated with ____ and _____ deficiency
• Dx:
  
  • In displaced SCFE (majority of cases): Capital femoral epiphysis slips posteriorly and medially relative to the femoral metaphysis (ice cream slipping off cone)
  • A common radiographic finding is lack of extension of the epiphysis past the Kline line (a line along the superior edge of the femoral neck)
• Tx:
  
  • Immediate nonweight bearing status, emergent ortho referral, surgical repair.

Answer 18

Slipped Capital Femoral Epiphysis
- Path: Posterior and inferior displacement of capital femoral epiphysis from femoral neck.

• RF: Obese child during early adolescence and near time of peak linear growth, boys, African Americans, those with endocrine dysfunction
  
  • Associated with hypothyroidism and growth hormone deficiency
• Dx:
  
  • In displaced SCFE (majority of cases): Capital femoral epiphysis slips posteriorly and medially relative to the femoral metaphysis (ice cream slipping off cone)
  • A common radiographic finding is lack of extension of the epiphysis past the Kline line (a line along the superior edge of the femoral neck)
• Tx:
  
  • Immediate nonweight bearing status, emergent ortho referral, surgical repair.

Question 19

Normal physiologic progression
- Genu varum up to ____yo followed by gene valgum at approx ____yo. By ___yo, most children return to normal physiologic vagus.

Answer 19

Normal physiologic progression

- Genu varum up to 2yo followed by gene valgum at approx 3yo. By 7yo, most children return to normal physiologic vagus.

Question 20

Blount Disease

• Pathologic ______ malformation caused by abnormal growth of the medial portion of the proximal tibial physis
• Infantile Blount
  
  • Diagnosed <4yo
  • Distinguish from physiologic varus
• Adolescent Blount: >4yo
• If exam demonstrates significant or asymmetric genu varus, radiographic exam must be performed.
• Plain radiographs show metaphysesal breaking (most common in the infantile form) and the tibial metaphysesal-diaphyseal angle is >16 degrees.
• Tx:
  
  • Infantile Blount: Bracing for approx 2 years. If unsuccessful or angle is >20 degrees, surgery is indicated
  • Surgical intervention usually needed for adolescent form.

Answer 20

Blount Disease

• Pathologic varus malformation caused by abnormal growth of the medial portion of the proximal tibial physis
• Infantile Blount
  
  • Diagnosed <4yo
  • Distinguish from physiologic varus
• Adolescent Blount: >4yo
• If exam demonstrates significant or asymmetric genu varus, radiographic exam must be performed.
• Plain radiographs show metaphysesal breaking (most common in the infantile form) and the tibial metaphysesal-diaphyseal angle is >16 degrees.
• Tx:
  
  • Infantile Blount: Bracing for approx 2 years. If unsuccessful or angle is >20 degrees, surgery is indicated
  • Surgical intervention usually needed for adolescent form.

Question 21

Genu varum (bowlegs)
- Increases as children start to walk, but typically resolves by 2yo, at which point the knee should transition to valgus alignment (genu valgum) 
Genu valgus (knock knee)
- Physiologic knee valgus peaks at 4yo and then decreases until final alignment is reached at about 7yo 

• Radiography for genu valgum is indicated for children
  
  • > ___yo with intermalleolar distances >__cm
  • Intermalleolar measurements outside 2 STDs from the mean for age.
  • With other indicators described earlier that increase the risk of associated pathology.

Answer 21

Genu varum (bowlegs)
- Increases as children start to walk, but typically resolves by 2yo, at which point the knee should transition to valgus alignment (genu valgum) 
Genu valgus (knock knee)
- Physiologic knee valgus peaks at 4yo and then decreases until final alignment is reached at about 7yo 

• Radiography for genu valgum is indicated for children
  
  • > 7yo with intermalleolar distances >8cm
  • Intermalleolar measurements outside 2 STDs from the mean for age.
  • With other indicators described earlier that increase the risk of associated pathology.

Question 22

Growing Pains

• Benign limb pains of unknown etiology that are not due to growing
• Pt: Typically bilateral deep sharp aching pain in the muscles of the legs. Occurs usually late in the day or during the night. There is no joint involvement, and inflammation is absent.
• Beware: The other diagnosis that causes nighttime bone pain (since it is difficult for most pts to differentiate bw bone and muscle pain) is malignancy.
• Unilateral symptoms require further evaluation.
• Children who should be evaluated for other conditions such as arthritis or infection: Activity-related pain, increasing pain intensity, joint swelling, limp, or constitutional symptoms (eg fever, malaise, or a decrease in activity)
• Tx: Reassurance. Generally disappear by _____yo.
  
  • _______ and OTC analgesics are often helpful for accelerating pain relief

Answer 22

Growing Pains

• Benign limb pains of unknown etiology that are not due to growing
• Pt: Typically bilateral deep sharp aching pain in the muscles of the legs. Occurs usually late in the day or during the night. There is no joint involvement, and inflammation is absent.
• Beware: The other diagnosis that causes nighttime bone pain (since it is difficult for most pts to differentiate bw bone and muscle pain) is malignancy.
• Unilateral symptoms require further evaluation.
• Children who should be evaluated for other conditions such as arthritis or infection: Activity-related pain, increasing pain intensity, joint swelling, limp, or constitutional symptoms (eg fever, malaise, or a decrease in activity)
• Tx: Reassurance. Generally disappear by 12-13yo.
  
  • Massage and OTC analgesics are often helpful for accelerating pain relief

Question 23

AMPLIFIED MUSCULOSKELETAL PAIN SYNDROMES

• Syndromes that cause noninflammatory musculoskeletal pain in children.
• Syndromes include juvenile fibromyalgia, complex regional pain syndrome, localized pain without autonomic changes, and intermittent pain.
• Path: AMP episodes are the result of amplified pain signs. The 3 primary causes include injury, illness, and psychological stressors.
• Pt:
  
  • Pain experience is real
  • Can present with one affected limb but can anywhere on the body and can also be diffuse.

Answer 23

AMPLIFIED MUSCULOSKELETAL PAIN SYNDROMES

• Syndromes that cause noninflammatory musculoskeletal pain in children.
• Syndromes include juvenile fibromyalgia, complex regional pain syndrome, localized pain without autonomic changes, and intermittent pain.
• Path: AMP episodes are the result of amplified pain signs. The 3 primary causes include injury, illness, and psychological stressors.
• Pt:
  
  • Pain experience is real
  • Can present with one affected limb but can anywhere on the body and can also be diffuse.

Question 24

Juvenile Fibromyalgia

• Hypersensitivity syndrome. ___ body areas of pain for at least ___ months
• Pt:
  
  • Diffuse musculoskeletal pain with many points on tenderness on exam.
  • Do NOT have evidence of articular swelling, loss of motion, or muscle weakness if these are present, look for another diagnosis
• Dx: Clinical
• Tx: OTC pain medications. Amitriptyline or cyclobenzaprine. Psychosocial intervention. No narcotics.

Answer 24

Juvenile Fibromyalgia

• Hypersensitivity syndrome. 3 body areas of pain for at least 3 months
• Pt:
  
  • Diffuse musculoskeletal pain with many points on tenderness on exam.
  • Do NOT have evidence of articular swelling, loss of motion, or muscle weakness if these are present, look for another diagnosis
• Dx: Clinical
• Tx: OTC pain medications. Amitriptyline or cyclobenzaprine. Psychosocial intervention. No narcotics.

Question 25

Complex regional pain syndrome (CRPS) - Pt: - Allodynia (pain aggravated by light touch) or hyperalgesia. - Have localized autonomic dysfunction with edema, coolness or excess warmth, mottling, and/or sweatiness. - Rule out _____ disorders - Tx: - PT/OT - School absenteeism can be a big problem in pts with AMP syndromes. Be sure to ask about attendance

Answer 25

Complex regional pain syndrome (CRPS) - Pt: - Allodynia (pain aggravated by light touch) or hyperalgesia. - Have localized autonomic dysfunction with edema, coolness or excess warmth, mottling, and/or sweatiness. - Because fatigue and pain can be seen in some pts with an autoimmune disorder, such as SLE, Sjogren, and JIA, be sure to check appropriate laboratory tests (CBC, ESR, RF, ANA, anti-SSA and anti-SSB, CPK, thyroid function tests) to rule out these conditions. - Tx: - PT/OT - School absenteeism can be a big problem in pts with AMP syndromes. Be sure to ask about attendance

Question 26

``` NON-NEOPLASTIC BONE LESIONS Nonossifying fibroma (NOF) - Pt: Small well defined radiolucent cortical lesion with surrounding rim of sclerosis. ``` - Management - Small NOFs ________ - Nonossifying firms ____ carry a risk of pathologic fracture and should be followed every 6-12mo with radiographs

Answer 26

``` NON-NEOPLASTIC BONE LESIONS Nonossifying fibroma (NOF) - Pt: Small well defined radiolucent cortical lesion with surrounding rim of sclerosis. ``` - Management - Small NOFs resolve spontaneously and do not require follow-up, reassurance for no tx - Nonossifying firms >50% of bone’s diameter carry a risk of pathologic fracture and should be followed every 6-12mo with radiographs

Question 27

NON-NEOPLASTIC BONE LESIONS Unicameral ‘simple’ bone cysts (UBCs) - Fluid-filled cysts surrounded by a thin rim of bone - Tx: - Observation with serial plain films and, for larger lesions (>50% of the bone), ______ - Children with UBCs ______, should be referred to an ortho surgeon for tx.

Answer 27

NON-NEOPLASTIC BONE LESIONS Unicameral ‘simple’ bone cysts (UBCs) - Fluid-filled cysts surrounded by a thin rim of bone - Tx: - Observation with serial plain films and, for larger lesions (>50% of the bone), activity restriction to prevent fracture - Children with UBCs in location where a fracture is likely to lead to surgery, such as the femoral neck, should be referred to an ortho surgeon for tx.

Question 28

NON-NEOPLASTIC BONE LESIONS Traumatic Myositis Ossificans - Extraskeletal ossification following blunt soft tissue trauma - Pt: - Mass is usually located away from a joint, is rounded, and is characterized on XR by mature peripheral ossification with a distinct margin surrounding a radiolucent center of immature osteoid and primitive mesenchymal tissue. This peripheral maturation is the reverse of that seen with a neoplasm. - Also in contrast to a neoplasm, the bony mass is ALWAYS slightly separated from the long shaft of the bone. - Tx: Rest, muscle stretching, anti inflammatory agents (eg indomethacin). - Surgical excision is rarely warranted - and only after a period of 6-12 months and with a negative bone scan. Earlier removal will likely result in return of new bone formation within the muscle. Therefore, myositis ossificans is surgically excised only if ______

Answer 28

NON-NEOPLASTIC BONE LESIONS Traumatic Myositis Ossificans - Extraskeletal ossification following blunt soft tissue trauma - Pt: - Mass is usually located away from a joint, is rounded, and is characterized on XR by mature peripheral ossification with a distinct margin surrounding a radiolucent center of immature osteoid and primitive mesenchymal tissue. This peripheral maturation is the reverse of that seen with a neoplasm. - Also in contrast to a neoplasm, the bony mass is ALWAYS slightly separated from the long shaft of the bone. - Tx: Rest, muscle stretching, anti inflammatory agents (eg indomethacin). - Surgical excision is rarely warranted - and only after a period of 6-12 months and with a negative bone scan. Earlier removal will likely result in return of new bone formation within the muscle. Therefore, myositis ossificans is surgically excised only if it interferes with joint mobility or is irritating a nerve.

Question 29

MALIGNANT BONE TUMORS Osteosarcoma (Osteogenic sarcoma) - Most common primary malignant tumor in children. - All show highly malignant and pleomorphic _____ cells in biopsy - Often affects the ____ bones, commonly the _____. Typically affect the areas around the ____ (distal femur > proximal tibia) - Radiographs: - Often reveal hallmark sclerotic, bone-forming lesion with mineralization extending into the soft tissues. - Characteristic____ pattern on plain film - _____ can also be seen on XR - The most common site for metastasis is the ____.

Answer 29

MALIGNANT BONE TUMORS Osteosarcoma (Osteogenic sarcoma) - Most common primary malignant tumor in children. - All show highly malignant and pleomorphic spindle cells in biopsy - Often affects the long bones, commonly the metaphysis. Typically affect the areas around the knee (distal femur > proximal tibia) - Radiographs: - Often reveal hallmark sclerotic, bone-forming lesion with mineralization extending into the soft tissues. - Characteristic “sunburst” pattern on plain film - Codman triangle can also be seen on XR - The most common site for metastasis is the lungs.

Question 30

MALIGNANT BONE TUMORS Ewing sarcoma - It is in a group of small, _____-cell, undifferentiated tumors of ____ origin. - Majority have a t(_____) translocation - Pt: - More likely to have systemic findings such as fever and weight loss - ____ bones (ribs, pelvis) and the _____ of the long bones are more commonly affected. - X-ray with “______” periosteal reaction or “____” - Dx: Confirm with tissue biopsy

Answer 30

MALIGNANT BONE TUMORS Ewing sarcoma - It is in a group of small, round-cell, undifferentiated tumors of neural crest origin. - Majority have a t(11;22) translocation - Pt: - More likely to have systemic findings such as fever and weight loss - Flat bones (ribs, pelvis) and the diaphyses of the long bones are more commonly affected. - X-ray with “onion skin” periosteal reaction or “hair-on-end pattern” - Dx: Confirm with tissue biopsy

Question 31

BENIGN BONE TUMORS Osteochondroma - Very common benign bone tumors in children that arises in cartilage. - Pt: Most occur in the _____ of long bones - Most asymptomatic and never recognized - On XR, appear as ______ from surface of the bone. Usually there is a cartilage “____” which can be as thick as 1cm.

Answer 31

BENIGN BONE TUMORS Osteochondroma - Very common benign bone tumors in children that arises in cartilage. - Pt: Most occur in the metaphysis of long bones - Most asymptomatic and never recognized - On XR, appear as stalks or projections from surface of the bone. Usually there is a cartilage “cap” which can be as thick as 1cm.

Question 32

BENIGN BONE TUMORS Enchondroma - Benign solitary lesion of hyaline cartilage that occurs centrally in the bone. - Hands are typically affected

Answer 32

BENIGN BONE TUMORS Enchondroma - Benign solitary lesion of hyaline cartilage that occurs centrally in the bone. - Hands are typically affected

Question 33

BENIGN BONE TUMORS Chondroblastoma - Rare, benign cartilage-forming lesion of the epiphysis of long bones. - Tx: Can be cured if tx with curettage and bone grafting before joint destruction occurs.

Answer 33

BENIGN BONE TUMORS Chondroblastoma - Rare, benign cartilage-forming lesion of the epiphysis of long bones. - Tx: Can be cured if tx with curettage and bone grafting before joint destruction occurs.

Question 34

BENIGN BONE TUMORS Osteoblastoma - Benign bone-forming tumor that causes local destruction of bone and grows over time. - Most commonly affects the vertebrae.

Answer 34

BENIGN BONE TUMORS Osteoblastoma - Benign bone-forming tumor that causes local destruction of bone and grows over time. - Most commonly affects the vertebrae.

Question 35

BENIGN BONE TUMORS Osteoid Osteoma - Benign lesion that produces ______. - Typically during ____ decade of life - Pt: - Often presents with severe nighttime pain that responds to ____- but not ____ - Imaging shows round/oval lucent nidus surrounded by sclerotic bone margins. - Tx: Provide reassurance and arrange for outpatient follow-up with ortho - Mildly symptomatic lesions can be controlled using ____, with serial exams and XRs every 4-6mo - Moderately-severely symptomatic lesions: Surgical resection

Answer 35

BENIGN BONE TUMORS Osteoid Osteoma - Benign lesion that produces prostaglandins. - Typically during 2nd decade of life - Pt: - Often presents with severe nighttime pain that responds to salicylate/aspirin and NSAIDs (contrast to osteosarcoma) - but not acetaminophen - Imaging shows round/oval lucent nidus surrounded by sclerotic bone margins. - Tx: Provide reassurance and arrange for outpatient follow-up with ortho - Mildly symptomatic lesions can be controlled using NSAIDs, with serial exams and XRs every 4-6mo - Moderately-severely symptomatic lesions: Surgical resection

Question 36

Sever Disease (Calcaneal apophysitis) - Common cause of heel pain - Inflammation of the ______ (apophysis) where the Achilles’ tendon inserts - Dx: ______ - Tx: - Decreased participation in the activity until the pt feels symptom relief, ice, stretching, heel cups, and NSAIDs - 1) _____ - 2) Application of _____ - 3) Activity modification ______

Answer 36

Sever Disease (Calcaneal apophysitis) - Common cause of heel pain - Inflammation of the calcaneal growth plate (apophysis) where the Achilles’ tendon inserts - Dx: Clinically when palpating the calcanea apophysis causes pain. Radiographs are generally not indicated in the initial presentation - Tx: - Decreased participation in the activity until the pt feels symptom relief, ice, stretching, heel cups, and NSAIDs - 1) Trial of heel cushions - 2) Application of ice several times/day, use o OTC analgesics - 3) Activity modification ONLY if there is significant pain or alteration in gait despite use of symptomatic treatment

Question 37

Osgood-Schlatter (Osteochondrosis) - Path: Overuse at the point of patellar tendon insertion - Pt: - On exam, affected individuals have tender and prominent_____. Knee pain worsens with activity and improves with rest. Pain with ____ of the knee against resistance or stressing quads. - Dx: Clinically by inspection and palpation of painful “bump” over the tibial tubercle, pain with resisted knee extension, or pain with full knee flexion. - Tx: - NSAIDs, ice, ____, PT, activity modification

Answer 37

Osgood-Schlatter (Osteochondrosis) - Path: Overuse at the point of patellar tendon insertion - Pt: - On exam, affected individuals have tender and prominent tibial tuberosity. Knee pain worsens with activity and improves with rest. Pain with extension of the knee against resistance or stressing quads. - Dx: Clinically by inspection and palpation of painful “bump” over the tibial tubercle, pain with resisted knee extension, or pain with full knee flexion. - Tx: - NSAIDs, ice, patellar tendon strap, PT, activity modification

Question 38

Sinding-Larsen-Johansson Syndrome (Jumper’s Knee) - Apophysitis of the _____ - Pt: Tenderness and activity-related pain - Tx: Self-resolves, stretching, conservative therapy

Answer 38

Sinding-Larsen-Johansson Syndrome (Jumper’s Knee) - Apophysitis of the inferior pole of the patella. - Pt: Tenderness and activity-related pain over the inferior pole of the patella - Tx: Self-resolves, stretching, conservative therapy

Question 39

Patellofemoral pain syndrome (PFPS) - One of the most common causes of knee pain in adolescents, particularly females - Pt: Pain with prolonged sitting, activity, and climbing or descending stairs - Exam: Tenderness over medial patellar facet, pain with patellar compression, and mild swelling - Dx: Physical exam findings to clarify diagnosis: - Positive patellofemoral compression test (with the leg extended, pain with compression of the patella) - Patella facet tenderness (while leg is extended, pain with manipulation of the patella and palpation of the facets) - Management: - Activity modification, ice, and NSAIDs.

Answer 39

Patellofemoral pain syndrome (PFPS) - One of the most common causes of knee pain in adolescents, particularly females - Pt: Pain with prolonged sitting, activity, and climbing or descending stairs - Exam: Tenderness over medial patellar facet, pain with patellar compression, and mild swelling - Dx: Physical exam findings to clarify diagnosis: - Positive patellofemoral compression test (with the leg extended, pain with compression of the patella) - Patella facet tenderness (while leg is extended, pain with manipulation of the patella and palpation of the facets) - Management: - Activity modification, ice, and NSAIDs.

Question 40

Iliotibial band bursitis - Pt: - Pain, sometimes described as “stinging,” is generally reported only during activity and is localized over the _______ - On physical exam, reproduction of pain occurs when palpating over the lateral femoral epicondyle with the patient in the lateral decubitus position, the hip flexed to 45, and the knee flexed to 30 degrees. - Tx: - Symptomatic treatment with rest, NSAIDs, knee sleeve, and icing resolves pain in most cases

Answer 40

Iliotibial band bursitis - Pt: - Pain, sometimes described as “stinging,” is generally reported only during activity and is localized over the lateral femoral epicondyle, just proximal to the lateral knee joint. - On physical exam, reproduction of pain occurs when palpating over the lateral femoral epicondyle with the patient in the lateral decubitus position, the hip flexed to 45, and the knee flexed to 30 degrees. - Tx: - Symptomatic treatment with rest, NSAIDs, knee sleeve, and icing resolves pain in most cases

Question 41

Patellar Dislocation - Pt: Physical exam shows an effusion and a positive apprehension test (fear with lateral displacement of the patella). - Tx: Initially closed reduction

Answer 41

Patellar Dislocation - Pt: Physical exam shows an effusion and a positive apprehension test (fear with lateral displacement of the patella). - Tx: Initially closed reduction

Question 42

Patellar subluxation - Excessive movement of the patella. - Exam may reveal effusion, a laterally displaced patella, localized tenderness on the adductor tubercle, and lateral facets of the patella. There may be abnormal tracking of the patella during extension of the knee, which is described as lateral movement of the patella at the end of knee extension.

Answer 42

Patellar subluxation - Excessive movement of the patella. - Exam may reveal effusion, a laterally displaced patella, localized tenderness on the adductor tubercle, and lateral facets of the patella. There may be abnormal tracking of the patella during extension of the knee, which is described as lateral movement of the patella at the end of knee extension.

Question 43

ACL tear - Most sensitive and specific test for ACL tear is Lachlan test, which evaluates the anterior translation of the tibia on the femur.

Answer 43

ACL tear - Most sensitive and specific test for ACL tear is Lachlan test, which evaluates the anterior translation of the tibia on the femur.

Question 44

Meniscal tears - Pt: - During the duck walk test, the pt is asked to squat down and walk like a duck. A positive test is one that causes reproducible pain in the joint line with the maneuver. - Repeated passive knee flexion and extension with tibial internal and external rotation produces pain and possible “clicking.” - Tx: - Isolated tears without associated ligamentous injury - or a mechanical block to full extension of the knee - often require only symptomatic tx with ice, compression, NSAIDs, and rest, followed by strengthening exercises and gradual return to activity as tolerated. - Surgery is needed in patients who have large tears, persistent effusions, or continued symptoms that interfere with activity.

Answer 44

Meniscal tears - Pt: - During the duck walk test, the pt is asked to squat down and walk like a duck. A positive test is one that causes reproducible pain in the joint line with the maneuver. - Repeated passive knee flexion and extension with tibial internal and external rotation produces pain and possible “clicking.” - Tx: - Isolated tears without associated ligamentous injury - or a mechanical block to full extension of the knee - often require only symptomatic tx with ice, compression, NSAIDs, and rest, followed by strengthening exercises and gradual return to activity as tolerated. - Surgery is needed in patients who have large tears, persistent effusions, or continued symptoms that interfere with activity.

Question 45

Prepatellar Bursitis - Inflammation of the bursa that is anterior to the kneecap. - Pt: In chronic cases, bursal wall thickening can be demonstrated by palpation of a fluid-filled mass anterior to the patella or the patellar tendon - Tx: Rest, ice, elevation, and NSAIDs. If there is no improvement with time and treatment, needle aspiration of the bursa is sometimes needed.

Answer 45

Prepatellar Bursitis - Inflammation of the bursa that is anterior to the kneecap. - Pt: In chronic cases, bursal wall thickening can be demonstrated by palpation of a fluid-filled mass anterior to the patella or the patellar tendon - Tx: Rest, ice, elevation, and NSAIDs. If there is no improvement with time and treatment, needle aspiration of the bursa is sometimes needed.

Question 46

Elbow pain - There should be a low threshold for obtaining radiographs in throwing athletes with elbow pain. Radiographs should include AP, lateral, and oblique views with comparison views of the normal elbow to identify subtle changes in the growth plates of the elbow. Bilateral elbow radiographs are often needed for detection of physeal injuries in the elbow.

Answer 46

Elbow pain - There should be a low threshold for obtaining radiographs in throwing athletes with elbow pain. Radiographs should include AP, lateral, and oblique views with comparison views of the normal elbow to identify subtle changes in the growth plates of the elbow. Bilateral elbow radiographs are often needed for detection of physeal injuries in the elbow.

Question 47

Medial Epicondyle Epiphysis / Medial Elbow Apophysitis (Little League elbow or youth baseball elbow) - Path: Overuse injury due to repetitive overhead motions (pitching, tennis). Results from valgus stress placed on the elbow during overhead throwing. - Pt: - ______ epicondyle pain with overhead throwing. - Pain can be elicited by palpation of the epicondyle and with valgus maneuvers of the elbow. - Tx: - Rest for 4-6 weeks from throwing, ice, NSAIDs, and physical therapy for scapular stabilization and proper throwing mechanics.

Answer 47

Medial Epicondyle Epiphysis / Medial Elbow Apophysitis (Little League elbow or youth baseball elbow) - Path: Overuse injury due to repetitive overhead motions (pitching, tennis). Results from valgus stress placed on the elbow during overhead throwing. - Pt: - Medial epicondyle pain with overhead throwing. - Pain can be elicited by palpation of the medial epicondyle and with valgus maneuvers of the elbow. - Tx: - Rest for 4-6 weeks from throwing, ice, NSAIDs, and physical therapy for scapular stabilization and proper throwing mechanics.

Question 48

``` Lateral Epicondylitis (Tennis Elbow) - Overuse injury causing inflammation and pain around the _____ epicondyle of the humerus at the origin of the wrist extensors and supination. ``` - Tx: Symptomatically with rest, NSAIDs, counterforce strap, and icing.

Answer 48

``` Lateral Epicondylitis (Tennis Elbow) - Overuse injury causing inflammation and pain around the lateral epicondyle of the humerus at the origin of the wrist extensors and supination. ``` - Tx: Symptomatically with rest, NSAIDs, counterforce strap, and icing.

Question 49

Osteochondritis Dissecans - Condition in which necrosis of subchondral bone causes a piece of bone or cartilage to break off into the joint, with resultant pain and mechanical symptoms. - Initial symptoms usually include vague anterior knee pain that worsens with activity. However, the presence of an effusion and joint locking should prompt the provider to obtain imaging studies that include AP, lateral, patellar, and tunnel view radiographs. - Tx: - If the physis is still open, tx of OCD is usually conservative and consists of rest, ice, NSAIDs, bracing, and physical therapy. - If there are loose bodies, closed physes, or a lack of improvement with conservative therapy after 4-6 months, surgery is often warranted.

Answer 49

Osteochondritis Dissecans - Condition in which necrosis of subchondral bone causes a piece of bone or cartilage to break off into the joint, with resultant pain and mechanical symptoms. - Initial symptoms usually include vague anterior knee pain that worsens with activity. However, the presence of an effusion and joint locking should prompt the provider to obtain imaging studies that include AP, lateral, patellar, and tunnel view radiographs. - Tx: - If the physis is still open, tx of OCD is usually conservative and consists of rest, ice, NSAIDs, bracing, and physical therapy. - If there are loose bodies, closed physes, or a lack of improvement with conservative therapy after 4-6 months, surgery is often warranted.

Question 50

Stress fracture - Tiny crack in bone that typically results from overuse in weight-bearing bones of the lower leg and foot - Path: Conditions increase the risk, including female athlete triad - Pt: Pain with activity that is relieved with rest. Exam reveals bony point tenderness and pain with hop or fulcrum testing. - Tx: - For low-risk stress fractures (eg metatarsal, femoral shaft, and most tibial/fibular fractures), _____ - For high-risk stress fractures (eg femoral neck/head, patella, anterior tibia, tarsal navicular, and Jones fractures of the 5th metatarsal), tx includes ____ - Surgical consultation is necessary ONLY for pts with high risk stress fracture or for whom a lengthy rehabilitation process would be impossible (eg a professional athlete or a laborer who needs a timely return to work).

Answer 50

Stress fracture - Tiny crack in bone that typically results from overuse in weight-bearing bones of the lower leg and foot - Path: Conditions increase the risk, including female athlete triad - Pt: Pain with activity that is relieved with rest. Exam reveals bony point tenderness and pain with hop or fulcrum testing. - Tx: - For low-risk stress fractures (eg metatarsal, femoral shaft, and most tibial/fibular fractures), conservative tx and avoidance of return to play until all symptoms resolve. - Rest from the offending activity - For high-risk stress fractures (eg femoral neck/head, patella, anterior tibia, tarsal navicular, and Jones fractures of the 5th metatarsal), tx includes strict non weightbearing. - Surgical consultation is necessary ONLY for pts with high risk stress fracture or for whom a lengthy rehabilitation process would be impossible (eg a professional athlete or a laborer who needs a timely return to work).

Question 51

Back pain - Back pain in children is not normal. Unlike in adults, it should always be investigated. - For back pack that persists for >___ weeks in the general population or >___ weeks in an athlete despite conservative tx, should complete initial evaluation - Radiography is the imaging modality of choice in the initial eval of back pain

Answer 51

Back pain - Back pain in children is not normal. Unlike in adults, it should always be investigated. - For back pack that persists for >6 weeks in the general population or >3 weeks in an athlete despite conservative tx, should complete initial evaluation - Radiography is the imaging modality of choice in the initial eval of back pain

Question 52

AAP clinical report “Optimizing Bone Health in Children and Adolescents” recommends_____ screening for those with conditions associated with low bone mass or those with multiple low-impact fractures. - In otherwise healthy children with fractures, the AAP currently recommends bone densitometry for children who have sustained clinically significant fractures (>=__ long bone fractures before age __ years, >=__ long bone fractures before ___ years, or a ____ fracture occurring without significant trauma or local disease). The specific role of stress fractures in determining the need for densitometry is not clearly delineated in the AAP report.

Answer 52

AAP clinical report “Optimizing Bone Health in Children and Adolescents” recommends vitamin D screening for those with conditions associated with low bone mass or those with multiple low-impact fractures. - In otherwise healthy children with fractures, the AAP currently recommends bone densitometry for children who have sustained clinically significant fractures (>2 long bone fractures before age 10 years, >3 long bone fractures before 19 years, or a vertebral fracture occurring without significant trauma or local disease). The specific role of stress fractures in determining the need for densitometry is not clearly delineated in the AAP report.

Question 53

Spondylolysis - Unilateral or bilateral vertebral injury involving fracture of the pars interarticularis due to repetitive stress. - Dx: - Radiography is the preferred initial study for suspected spondylolysis. - Sometimes evident on plain film, where the loss of bony continuity is esp visible on the oblique view of a lumbar X-ray appearing as a “____” - Tx_____

Answer 53

Spondylolysis - Unilateral or bilateral vertebral injury involving fracture of the pars interarticularis due to repetitive stress. - Dx: - Radiography is the preferred initial study for suspected spondylolysis. - Sometimes evident on plain film, where the loss of bony continuity is esp visible on the oblique view of a lumbar X-ray appearing as a “collar” (the break) on the neck of a “Scotty” dog. - Tx: Initial is conservative and consists of rest from offending activity until pain free, ice, heat, and NSAIDs, PT. - If pain does not improve, the pt has worsening slippage, or severe neurologic involvement (eg cauda equina syndrome), refer for surgical consultation.

Question 54

Spondylolisthesis - Spontaneous subluxation (usually forward) of one vertebra over another, most commonly ___ on ___. - Tx: _____

Answer 54

Spondylolisthesis - Spontaneous subluxation (usually forward) of one vertebra over another, most commonly L5 on S1. - Tx: Initial is conservative and consists of rest from offending activity until pain free, ice, heat, and NSAIDs, PT. - If pain does not improve, the pt has worsening slippage, or severe neurologic involvement (eg cauda equina syndrome), refer for surgical consultation.

Question 55

HYPERMOBILITY SYNDROMES Benign hypermobility joint syndrome - Thought to be familial and inherited in ____ fashion. - Pt: - Demonstrate hypermobility by having the child attempt 5 tasks: - 1) Extend the wrist and metacarpophalangeal joints so that the fingers are parallel to the dorsum of the forearm (bilateral) - 2) Passively oppose the thumb to the flexor aspect of the forearm (bilateral) - 3) Hyperextend the elbows 10 degrees of more (bilateral) - 4) Hyperextend the knees 10 degrees or more (bilateral) - Ability to perform the above tasks in >=_____ locations (a point for each side of the body plus flexing the trunk) indicates hypermobility on the Breighton scale. - Tx: _______

Answer 55

HYPERMOBILITY SYNDROMES Benign hypermobility joint syndrome - Thought to be familial and inherited in AD fashion. - Pt: - Demonstrate hypermobility by having the child attempt 5 tasks: - 1) Extend the wrist and metacarpophalangeal joints so that the fingers are parallel to the dorsum of the forearm (bilateral) - 2) Passively oppose the thumb to the flexor aspect of the forearm (bilateral) - 3) Hyperextend the elbows 10 degrees of more (bilateral) - 4) Hyperextend the knees 10 degrees or more (bilateral) - Ability to perform the above tasks in >5 locations (a point for each side of the body plus flexing the trunk) indicates hypermobility on the Breighton scale. - Tx: NSAIDs or acetaminophen. Lifestyle alterations (physical therapy, joint strengthening, stretching, increase in light exercise, avoidance of activities that put stress on the affected joints) - Swimming and other low or no-impact sports are good recommendations

Question 56

HYPERMOBILITY SYNDROMES HYPERMOBILITY SYNDROMES Marfan syndrome - Inheritance??__ - Path: - 75% disorder with mutation in ____ gene - 25% de novo spontaneous mutation - Pt: Skeletal manifestations, cardiovascular system, eyes, skin, lungs, dura - Skeletal: - Tall stature (women >70in at skeletal maturity, men >75in) - Scoliosis/kyphosis - Pes planus (flat feet) or hindfoot deformity - Arachnodactyly (long fingers) - Pectus deformities/excavatum or carinatum - Joint/skin laxity - Long arms/tall for family - Increased arm span to height ratio >____ - Reduced upper-to-lower segment ratio - Facial features (Marfanoid appearance): Long and narrow face (dolichocephaly) with deep-set eyes (enophthalmos), downslanting palpebral fissures, malar hypoplasia, micrognathia/retrognathia - Cardiac anomalies: Most deaths occur due to cardiovascular complications - _____, ______ tricuspid valve prolapse, proximal pulmonary artery enlargement. - ______ is the most common cause of morbidity and mortality in Marfan pts - Ocular findings: - Myopia (near sightedness/elongation of globe) >3 diopters is most common - _____ lens dislocation (Ectopia lentis) - Iridodenesis (rapid contraction and dilation of iris) - Cataracts - Retinal detachment - Wrist or thumb signs. - Fold hands with thumb tucked inside. Thumb sticks out past knuckle - Hand wrapped around wrist. Thumb and pinky meet passing knuckle - ________ cognition and head circumference. - Dx: - Clinically diagnosed based on family hx and characteristic systemic findings known as ____ criteria - Diagnosis is made if there are any 2 of the following major criteria: - 1) ______ - 2)______ - 3) ________ - If there is only 1 major finding, then either a _____ or >___ (out of 20) systemic points are required to make the diagnosis. The systemic points include the wrist and thumb signs, chest wall deformity, scoliosis, myopia, pneumothorax, and striae. - Ectopia lentis and aortic aneurysm have high specificity and clinical significance for a diagnostic consideration of Marfan syndrome. - FBN1 sequencing - Always rule out _______, which has many features similar to Marfan syndrome but carries a significant risk of stroke and other embolic events and has a much different treatment strategy. Homocystinuria features intellectual disability and a downwardly dislocated lens (in Marfan syndrome the lens dislocates upward).

Answer 56

HYPERMOBILITY SYNDROMES Marfan syndrome - AD disorder with variable expressivity. - Path: - 75% AD disorder with mutation in fibrillin-1 FBN1 gene - 25% de novo spontaneous mutation - Pt: Skeletal manifestations, cardiovascular system, eyes, skin, lungs, dura - Skeletal: - Tall stature (women >70in at skeletal maturity, men >75in) - Scoliosis/kyphosis - Pes planus (flat feet) or hindfoot deformity - Arachnodactyly (long fingers) - Pectus deformities/excavatum or carinatum - Joint/skin laxity - Long arms/tall for family - Increased arm span to height ratio >1.05 - Reduced upper-to-lower segment ratio - Facial features (Marfanoid appearance): Long and narrow face (dolichocephaly) with deep-set eyes (enophthalmos), downslanting palpebral fissures, malar hypoplasia, micrognathia/retrognathia - Cardiac anomalies: Most deaths occur due to cardiovascular complications - Aortic root dilatation, mitral valve prolapse (MVP, mid-systolic click and late systolic murmur), tricuspid valve prolapse, proximal pulmonary artery enlargement. - Aortic root dilatation is the most common cause of morbidity and mortality in Marfan pts - Ocular findings: - Myopia (near sightedness/elongation of globe) >3 diopters is most common - UPWARD lens dislocation (Ectopia lentis) - Iridodenesis (rapid contraction and dilation of iris) - Cataracts - Retinal detachment - Wrist or thumb signs. - Fold hands with thumb tucked inside. Thumb sticks out past knuckle - Hand wrapped around wrist. Thumb and pinky meet passing knuckle - NORMAL cognition and head circumference. - Dx: - Clinically diagnosed based on family hx and characteristic systemic findings known as Ghent criteria - Diagnosis is made if there are any 2 of the following major criteria: - 1) Ectopia lentis - 2) Aortic dilation or dissection - 3) Family hx - If there is only 1 major finding, then either a mutation in the FBN1 gene (encodes the protein fibrillin-1) or >7 (out of 20) systemic points are required to make the diagnosis. The 7 systemic points include the wrist and thumb signs, chest wall deformity, scoliosis, myopia, pneumothorax, and striae. - Ectopia lentis and aortic aneurysm have high specificity and clinical significance for a diagnostic consideration of Marfan syndrome. - FBN1 sequencing - Always rule out homocystinuria, which has many features similar to Marfan syndrome but carries a significant risk of stroke and other embolic events and has a much different treatment strategy. Homocystinuria features intellectual disability and a downwardly dislocated lens (in Marfan syndrome the lens dislocates upward).

Question 57

HYPERMOBILITY SYNDROMES Ehlers Danlos Syndrome - ______inheritance? connective tissue disorders - Pt: - Skin: - Texture is “wet chamois,” “fine sponge,” or “doughy.” - Skin is very stretchy and returns to its normal configuration on release, much like a rubber band. - Increasing bruising - Wrinkled palms and soles are commo - Joints - ____ is common but does not occur in certain types of Ehlers-Danlos syndromes. - Mitral valve prolapse and proximal aortic dilation occur. - Screen for these with echo, CT, or MRI - Causes esophageal perforation - subcutaneous crepitus, neck pain, and dysphagia after forceful vomiting.

Answer 57

HYPERMOBILITY SYNDROMES Ehlers Danlos Syndrome - AD connective tissue disorders - Pt: - Skin: - Texture is “wet chamois,” “fine sponge,” or “doughy.” - Skin is very stretchy and returns to its normal configuration on release, much like a rubber band. - Increasing bruising - Wrinkled palms and soles are commo - Joints - Joint hypermobility is common but does not occur in certain types of Ehlers-Danlos syndromes. - Mitral valve prolapse and proximal aortic dilation occur. - Screen for these with echo, CT, or MRI - Causes esophageal perforation - subcutaneous crepitus, neck pain, and dysphagia after forceful vomiting.

Question 58

OSTEOGENESIS IMPERFECTA - Path: Results from genetic mutation in COL1A1 or COL1A2 - 4 most common recognized forms are caused by abnormal structure of Type 1 collagen - OI Type I: ______ - OI Type 2: ______ - OI Type 3:______ - OI Type 4: ______ - None cause retinal hemorrhage or subdural hematomas, which distinguishes OI from abuse - Management - _______can be seen in up to 50% of osteogenesis imperfecta (OI) pts. - _______ can decrease chronic bone pain as well as increase vertebral bone mineral mass and mobility. - ____ is integral in OI. - ______levels should be checked in children with OI to ensure optimal bone health.

Answer 58

OSTEOGENESIS IMPERFECTA - Path: Results from genetic mutation in COL1A1 or COL1A2 - 4 most common recognized forms are caused by abnormal structure of Type 1 collagen - OI Type I: Classic nondeforming OI with blue sclerae - OI Type 2: Perinatally lethal OI - OI Type 3: Progressively deforming OI - OI Type 4: Common variable OI with normal sclerae - None cause retinal hemorrhage or subdural hematomas, which distinguishes OI from abuse - Management - Hearing loss can be seen in up to 50% of osteogenesis imperfecta (OI) pts. - IV bisphosphonates can decrease chronic bone pain as well as increase vertebral bone mineral mass and mobility. - PT is integral in OI. - Vitamin D levels should be checked in children with OI to ensure optimal bone health.

Question 59

Osteogenesis Imperfecta Type 1 “Brittle bone disease” - ____Inheritance? - Pt: - Multiple fractures (most occur before puberty and mimic child abuse) - _______ sclerae

Answer 59

Osteogenesis Imperfecta Type 1 “Brittle bone disease” - AD - Pt: - Multiple fractures (most occur before puberty and mimic child abuse) - Blue sclerae

Question 60

Osteogenesis Imperfecta Type 2 - Most severe form - Pt: - Results in _____ - These children have numerous fractures and severe bone deformity - XR: Long bones with a “___,” and the ribs are ____

Answer 60

Osteogenesis Imperfecta Type 2 - Most severe form - Pt: - Results in death during the newborn period due to respiratory insufficiency - These children have numerous fractures and severe bone deformity - XR: Long bones with a “crumpled appearance,” and the ribs are beaded due to callus formation.

Question 61

Osteogenesis Imperfecta Type 3 “Progressively deforming” type - Pt: - Newborn with numerous fractures. - _____ sclerae occur at birth but lighten with age (unlike those in Type 1, which stay dark blue) - ______ complications are most common with Type 3, including hydrocephalus and basilar skull invagination.

Answer 61

Osteogenesis Imperfecta Type 3 “Progressively deforming” type - Pt: - Newborn with numerous fractures. - Blue sclerae occur at birth but lighten with age (unlike those in Type 1, which stay dark blue) - Neurologic complications are most common with Type 3, including hydrocephalus and basilar skull invagination.

Question 62

Osteogenesis Imperfecta Type 4 | - Pt: Sclerae are typically ____

Answer 62

Osteogenesis Imperfecta Type 4 | - Pt: Sclerae are typically white or near-white.

Question 63

TOE WALKING Idiopathic toe walking - Should be bilateral and symmetric - Most are able to stand with their heels in contact with the ground. - Toe-walking resolves in more than half of children by ____ yo. It may persist through___yo in healthy children. - Observation with re-evaluation in 6 months - If toe walking persists, it is important to re-consider whether a developmental delay or neurologic disease may be present; additional investigation or referral to a pediatric orthopedist may be pursued. - Treatment is controversial given lack of data. Pathologic toe walking - Children with pathologic (non-idiopathic) toe walking typically exhibit consistent toe walking and are unable to voluntarily heel strike with prompting. - Toe walking that appears later in life, is constant, or is unilateral would be more concerning, as would a hx of peripartum asphyxia, delayed milestones, or family hx of neurologic disorders and gait disorders.

Answer 63

TOE WALKING Idiopathic toe walking - Should be bilateral and symmetric - Most are able to stand with their heels in contact with the ground. - Toe-walking resolves in more than half of children by 5.5 yo. It may persist through 7yo in healthy children. - Observation with re-evaluation in 6 months - If toe walking persists, it is important to re-consider whether a developmental delay or neurologic disease may be present; additional investigation or referral to a pediatric orthopedist may be pursued. - Treatment is controversial given lack of data. Pathologic toe walking - Children with pathologic (non-idiopathic) toe walking typically exhibit consistent toe walking and are unable to voluntarily heel strike with prompting. - Toe walking that appears later in life, is constant, or is unilateral would be more concerning, as would a hx of peripartum asphyxia, delayed milestones, or family hx of neurologic disorders and gait disorders.

Question 64

``` Shin Splints (Medial tibial stress syndrome) - Path: Cause by repetitive activity on hard surfaces or the forceful, excessive use of foot flexures. ``` - Tx: - Rest and ice. It is essential that a pt allow the tibia to recover from high levels of stress while reducing inflammation. After 1-2 weeks, pts should initiate a gradual return to activity. - Strengthening of the hip muscles and lower leg muscles is used as adjunctive treatment. - Pts with shin splints should be able to tolerate single-leg hops as well as progressive distances without pain prior to return to play. - Shin splints frequently recur even with appropriate rehabilitation on the initial presentation. It is important to counsel the pt and family regarding this as they can become frustrated.

Answer 64

``` Shin Splints (Medial tibial stress syndrome) - Path: Cause by repetitive activity on hard surfaces or the forceful, excessive use of foot flexures. ``` - Tx: - Rest and ice. It is essential that a pt allow the tibia to recover from high levels of stress while reducing inflammation. After 1-2 weeks, pts should initiate a gradual return to activity. - Strengthening of the hip muscles and lower leg muscles is used as adjunctive treatment. - Pts with shin splints should be able to tolerate single-leg hops as well as progressive distances without pain prior to return to play. - Shin splints frequently recur even with appropriate rehabilitation on the initial presentation. It is important to counsel the pt and family regarding this as they can become frustrated.

Question 65

``` Pes Planus (Flat Feet) - Absence of the normal medial longitudinal arch of the foot. ``` - With toe-standing - Return of the arch when the pt stands on his/her toes means that the pt has “flexible” pes planus, which rarely requires tx. - If the arch does not appear with toe-standing, the pt should be evaluated for “fixed” forms of pes planus, such as tarsal coalition. - Flatfoot is generally considered pathologic when it leads to pain or functional deficits, which are far more likely to occur with rigid as opposed to flexible flatfoot. - Distinguishing flexible from rigid flatfoot is important, as flexible painless flatfoot rarely requires treatment. - Tx: - If pts are asymptomatic, _______. (normally the arch develops by ___ yo, but may take as long as ___yo). - If flatfoot is persistent, there is less likelihood that the flatfoot will resolve. This does not necessarily mean that the pt will have pain or any issues related to their flatfoot. Pain is uncommon for most pts with flatfoot, whether they use orthosis or not. - Symptomatic:_________

Answer 65

``` Pes Planus (Flat Feet) - Absence of the normal medial longitudinal arch of the foot. ``` - With toe-standing - Return of the arch when the pt stands on his/her toes means that the pt has “flexible” pes planus, which rarely requires tx. - If the arch does not appear with toe-standing, the pt should be evaluated for “fixed” forms of pes planus, such as tarsal coalition. - Flatfoot is generally considered pathologic when it leads to pain or functional deficits, which are far more likely to occur with rigid as opposed to flexible flatfoot. - Distinguishing flexible from rigid flatfoot is important, as flexible painless flatfoot rarely requires treatment. - Tx: - If pts are asymptomatic, there is no need to initiate tx. Observation is appropriate bc most of these children will develop a strong arch within the 1st decade of life (normally the arch develops by 4-6 yo, but may take as long as 10yo). - If flatfoot is persistent at 10yo, there is less likelihood that the flatfoot will resolve. This does not necessarily mean that the pt will have pain or any issues related to their flatfoot. Pain is uncommon for most pts with flatfoot, whether they use orthosis or not. - Symptomatic: Based on severity of tendon damage. If a child is having pain with flexible flatfeet, initial therapy is conservative, and includes rest, icing after activity, and a trial of anti-inflammatory medications. Referral to PT to stretch Achilles tendon. Supportive custom foot orthosis.

Question 66

Leg-Length discrepancy - Path: Likely secondary to ______, which is most common in children who suffer an injury between ages 4-7 years. - Tx: - LLD of _____cm place the child at higher risk for future difficulties and should be referred to an orthopedic surgeon for surgical consideration.

Answer 66

Leg-Length discrepancy - Path: Likely secondary to post-traumatic overgrowth, which is most common in children who suffer an injury between ages 4-7 years. - Tx: - LLD of <1cm are considered to be within normal limits. The average LLD in skeletally mature individuals is ~5mm. - LLDs of >2cm place the child at higher risk for future difficulties and should be referred to an orthopedic surgeon for surgical consideration.

Question 67

Dx of osteoporosis in children: Low bone density for age and gender plus a clinically significant fracture history: >2 long bone fractures by 10yo, >3 long bone fractures by 19yo, or a vertebral compression fracture. - - A Z score of less than___ at either site defines low bone density for age and gender. - Calcium and vitamin D supplementation, bisphosphonates, minimizing corticosteroid use. - Bisphosphonates are recommended for tx and prevention of osteoporosis - If pts are on corticosteroids for >3 months, unable to discontinue corticosteroid use, and/or the DEXA T-score is less than 1.5, ____ therapy should be considered.

Answer 67

Dx of osteoporosis in children: Low bone density for age and gender plus a clinically significant fracture history: >2 long bone fractures by 10yo, >3 long bone fractures by 19yo, or a vertebral compression fracture. - - A Z score of less than -2 at either site defines low bone density for age and gender. - Calcium and vitamin D supplementation, bisphosphonates, minimizing corticosteroid use. - Bisphosphonates are recommended for tx and prevention of osteoporosis - If pts are on corticosteroids for >3 months, unable to discontinue corticosteroid use, and/or the DEXA T-score is <1.5, bisphosphonate therapy should be considered.

Question 68

Benign acute childhood myositis - Path: Most commonly _____ or other virus - Labs: ______ levels are usually highly elevated, but decrease within 2-3 weeks of onset. - Tx: Supportive care and resolves without sequelae

Answer 68

Benign acute childhood myositis - Path: Most commonly influenza or other virus - Labs: Creatine kinase levels are usually highly elevated, but decrease within 2-3 weeks of onset. - Tx: Supportive care and resolves without sequelae

Question 69

Arthrogryposis - Condition in which multiple nonprogressive joint contractures develop before birth - It can be associated with multiple diagnoses that decrease fetal movement in utero, including CNS disorders, neuromuscular diseases, connective tissue or muscular disorders, and trisomies.

Answer 69

Arthrogryposis - Condition in which multiple nonprogressive joint contractures develop before birth - It can be associated with multiple diagnoses that decrease fetal movement in utero, including CNS disorders, neuromuscular diseases, connective tissue or muscular disorders, and trisomies.