Updated: 6/15/2021

Local Gigantism

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  • summary
    • Local Gigantism is a rare congenital or acquired condition that can be caused by neurofibromatosis, proteus syndrome, amyloidosis, or tumor and presents with enlargement of a digit. 
    • Diagnosis is made clinically with relative enlargement of one or more of the toes. Radiographs are helpful for surgical planning. 
    • Treatment is generally reconstruction or amputation if symptomatic or if leading to functional deficits. 
  • Epidemiology
    • Incidence
      • rare
  • Etiology
    • Pathophysiology
      • congenital causes:
        • neurofibromatosis
        • proteus syndrome
        • klippel-Trenaunay-Weber
      • acquired causes:
        • amyloidosis
        • elephantiasis (filariasis)
        • av malformation
        • tumor
        • acromegaly
      • Physiology
        • unknown
  •  Anatomy
    • Growth plate consists of three principal layers:
      • resting zone
      • proliferative zone
      • hypertrophic zone
  • Presentation
    • Symptoms
      • pain
    • Physical exam
      • enlarged digit or limb
  • Imaging
    • Radiograhs
      • needed for reconstruction or amputation planning
  • Treatment
    • Nonoperative
      • observation
        • monitor growth of affected region
    • Operative
      • epiphysiodesis vs. bony and soft-tissue reduction procedures
        • indications
          • individualized based on etiology, location of affected area, skeletal growth left, and severity

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