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Avascular (Aseptic) Necrosis of the Femoral Head (Hip), Pediatric


Basics


Description


  • Avascular (aseptic) necrosis results from the interruption of the blood supply to bone (either traumatic or nontraumatic occlusion).
  • The femoral head is the most common site.
  • A particular type of self-limiting idiopathic avascular necrosis of the hip that occurs in children is known as Perthes disease (see the "Perthes Disease" chapter).

Risk Factors


Genetics
Variable, depending on cause  

Pathophysiology


  • Death and necrosis of bone with gradual return of blood supply
  • Necrotic bone gradually resorbed and replaced by new bone
  • During bone resorption, structural integrity of femoral head may be reduced, leading to collapse.

Etiology


  • Traumatic
    • Hip fracture
    • Hip dislocation
    • Slipped capital femoral epiphysis
    • Complication of casting, bracing, surgery
  • Nontraumatic
    • Idiopathic (older, after physeal closure); similar to adult avascular necrosis
    • Idiopathic (younger, before physeal closure, Perthes disease)
    • Caisson disease
    • Sickle cell disease
    • Septic arthritis
    • Steroids or chemotherapy
    • Malignancy (leukemia)
    • Gaucher disease
    • Viral infection (HIV, CMV)
    • Radiation therapy
    • Hypercoagulable states

Diagnosis


History


  • Onset (gradual or after traumatic event)
  • Association with the following:
    • Trauma
    • Medications (steroids or chemotherapy)
    • Casting, splinting, surgery (iatrogenic)
    • Pain, limping
    • Stiffness (decreased range of motion)
    • Perthes disease may occasionally be bilateral or occur in contralateral hip at a later time point.

Physical Exam


  • Gait
    • Limping
    • Antalgic ("against pain") gait (shortened stance phase relative to swing phase)
    • Trendelenburg gait
  • Note range of motion:
    • Flexion and extension
    • Abduction and adduction
    • Internal and external rotation
  • Hip joint irritability (short arc rotation)
  • Signs of other disease processes associated with avascular necrosis (e.g., sickle cell disease)
  • Physical examination pearl
    • Loss of internal rotation is usually the first and most affected loss of motion seen.

Diagnostic Tests & Interpretation


Lab
  • Laboratory examinations should be normal in most forms of avascular necrosis of the femoral head.
  • Exceptions:
    • Sickle cell disease
    • Septic arthritis
    • Chemotherapy

Imaging
  • Radiographic findings:
    • Sclerosis
    • Subchondral fracture
    • Collapse
    • Reossification
    • Repair
  • Magnetic resonance imaging (MRI)
    • Bone edema
    • If contrast medium used, area of reduce blood flow evident
  • Bone scan
    • Reduced signal in affected hip
  • Other potential findings:
    • Cysts
    • Physeal growth arrest (young)
    • Early osteoarthritis
    • Subluxation

Differential Diagnosis


  • Trauma
    • Osteochondral fracture
    • Impaction fracture
    • Epiphyseal/physeal fracture
  • Infection
    • Osteomyelitis
    • Septic arthritis
  • Neoplastic process: epiphyseal tumors (chondroblastoma, Trevor disease, etc.)
  • Rheumatologic processes
  • Skeletal dysplasia, particular if bilateral hip involvement

Treatment


Medication


  • NSAIDs may reduce pain by decreasing associated inflammation but may also reduce new bone formation.
  • If associated with corticosteroid use, discontinuation or elimination of steroids may be helpful if appropriate.
  • Bisphosphonate therapy may help preserve joint shape.

Additional Therapies


General Measures
  • Maintain range of motion (physical therapy, traction, continuous passive motion).
  • Contain the femoral head in the acetabulum (see treatment principles listed in "Perthes Disease" chapter).
  • Duration of therapy variable, depending on cause
  • Reduced weight bearing on affected hip may help prevent collapse.

Surgery/Other Procedures


Redirectional osteotomy  
  • Femoral or acetabular reorientation
  • Core decompression to stimulate new blood supply

Ongoing Care


Diet


  • Thought not to alter disease process
  • Recommend general balanced diet
  • During immobilization, excessive weight gain may occur.

Prognosis


  • Depends on extent of femoral head collapse
  • Good if mild involvement and patient is young
  • When to expect improvement: variable, depending on cause
  • Moderate to severe cases often have significant collapse and end up requiring a total hip replacement.

Complications


  • Joint collapse with decreased range of motion, pain, limping
  • Osteoarthritis
  • Physeal arrest with growth disturbance

Alert
Signs to watch for:  
  • Subluxation
  • Early osteoarthritis
  • Growth arrest

Additional Reading


  • Lahdes-Vasama  T, Lamminen  A, Merikanto  J, et al. The value of MRI in early Perthes' disease: an MRI study with a 2-year follow-up. Pediatr Radiol.  1997;27(6):517-522.  [View Abstract]
  • Mont  MA, Jones  LC, Hungerford  DS. Nontraumatic osteonecrosis of the femoral head: ten years later. J Bone Joint Surg Am.  2006;88(5):1117-1132.  [View Abstract]
  • Roposch  A, Mayr  J, Linhart  WE. Age at onset, extent of necrosis, and containment in Perthes disease. Results at maturity. Arch Orthop Trauma Surg.  2003;123(2):68-73.  [View Abstract]
  • Shipman  SA, Helfand  M, Moyer  VA, et al. Screening for developmental dysplasia of the hip: a systematic literature review for the US Preventive Services Task Force. Pediatrics.  2006;117(3):e557-e576.  [View Abstract]
  • Tokmakova  KP, Stanton  RP, Mason  DE. Factors influencing the development of osteonecrosis in patients treated for slipped capital femoral epiphysis. J Bone Joint Surg Am.  2003;85-A(5):798-801.  [View Abstract]

Codes


ICD09


  • 733.42 Aseptic necrosis of head and neck of femur
  • 732.1 Juvenile osteochondrosis of hip and pelvis

ICD10


  • M87.059 Idiopathic aseptic necrosis of unspecified femur
  • M91.10 Juvenile osteochondrosis of head of femur, unspecified leg
  • M87.052 Idiopathic aseptic necrosis of left femur
  • M87.051 Idiopathic aseptic necrosis of right femur
  • M91.11 Juvenile osteochondrosis of head of femur, right leg
  • M91.12 Juvenile osteochondrosis of head of femur, left leg

SNOMED


  • 444904004 aseptic necrosis of head of femur (disorder)
  • 111255008 Avascular necrosis of the capital femoral epiphysis (disorder)
  • 15739006 Juvenile osteochondrosis of hip AND/OR pelvis (disorder)

FAQ


  • Q: What type of medication is most often associated with avascular necrosis of the hip?
  • A: Corticosteroids
  • Q: For avascular necrosis in children (Perthes disease of the hip, for example), is younger or older age associated with a better prognosis?
  • A: Younger age (<8 years)
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