Updated: 6/14/2021

Hemihypertrophy

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  • summary
    • Hemihypertrophy is an idiopathic condition that can also be caused by a variety of syndromes, and presents with asymmetry between the right and left sides of the body to a greater degree than can be attributed to normal variation.
    • Diagnosis is made clinically with abnormal asymmetry defined as a 5% or greater difference in length and/or circumference.
    • Treatment is observation with or without shoe lifts for leg length differences < 2 cm at skeletal maturity. Surgical intervention is indicated for leg length differences > 2 cm, with different techniques depending on the discrepancy magnitude and remaining skeletal growth.
  • Epidemiology
    • Incidence
      • varies from 1:14,000 to 1:86,000
    • Demographics
      • male:female = 2:3
    • Anatomic location
      • slightly more common on the right side
      • may also involve head, trunk and internal organs
  • Etiology
    • 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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