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Hemihypertrophy

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Topic updated on 06/05/15 1:44pm
Introduction
  • Asymmetry between the right and left sides of the body to a greater degree than can be attributed to normal variation 
    • abnormal asymmetry defined as a 5% or greater difference in length and/or circumference
  • Epidemiology
    • incidence
      • varies from 1:14,000 to 1:86,000
    • demographics
      • male:female = 2:3
    • location
      • slightly more common on the right side
      • may also involve head, trunk and internal organs
  • Pathophysiology
    • most common causes are
      • idiopathic (non-syndromic)
      • syndromic
        • neurofibromatosis  
        • Beckwith-Wiedemann syndrome
        • Klippel-Trenauney syndrome
        • Proteus syndrome
  • Associated conditions
    • orthopaedic manifestations
      • scoliosis (compensatory)
      • peripheral nerve entrapment (rare)
    • medical conditions & comorbidities
      • malignant intra-abdominal tumors
        • Wilm's tumor (most common)
          • perform serial abdominal ultrasounds (every 3 months) until age 7 to rule out Wilm's tumor
          • then physical exam every 6 months until skeletal maturity
        • adrenal carcinoma
        • hepatoblastoma
      • genitourinary abnormalities
        • medullary sponge kidneys
        • polycystic kidney
        • inguinal hernias
Classification                                                                                                                                     
  • Congenital classification
    • congenital
      • total
        • involvement of all organ systems 
      • limited
        • only muscular, vascular, skeletal, or neurologic involvement
          • classic (ipsilateral upper and lower limbs)
          • segmental (a single limb)
          • facial 
          • crossed
    • acquired
      • very rare
      • can result from injury, infection, radiation or inflammation 
  • Syndromic classification
    • non-syndromic (isolated)
    • syndromic (part of a clinical syndrome)
Presentation                                                                                                                                       
  • Physical exam
    • findings rarely apparent at birth, manifest during growth
    • skin often thicker on involved side
    • more hair on corresponding side of the head
    • limb circumference asymmetric
    • leg-length discrepancy (LLD)
Imaging
  • Radiographs
    • AP + lateral of affected limb may demonstrate enlargement of bone and soft tissue 
      • osseous maturation may be seen
  • Abdominal ultrasound
    • indications
      • perform serial abdominal ultrasounds (every 3 months) until age 7 to rule out Wilm's tumor
Studies                                                                                                                                        
  • Renal function tests
  • Serum alpha-fetoprotein
    • screen for embryonal tumors
Treatment
  • Nonoperative
    • observation, shoelift, corrective shoes
      • indications
        • < 2 cm projected LLD at maturity
  • Operative
    • surgical correction
      • indications
        • based on principles of leg length discrepancies  

 

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