Beckwith-Wiedemann syndrome

• Facts:
  Overgrowth disorder that is present from birth, characterized by an increased risk for childhood cancers
• History / PE:
  Macrosomia, macroglossia
  Visceromegaly (liver, kidneys)
  Omphalocele
  Hyperplasia of pancreas
  Prominent eyes/occiput
  Ear creases
• Diagnosis:
  Hypoglycemia / hyperinsulinemia
• Complications:
  Wilms' tumor
  Hepatoblastoma
  Gonadoblastoma
• Associated With:
  Duplication of chromosome 11p (contains IGF-2 gene)
• Differential Diagnosis:
  Von-Gierke disease (fasting hypoglycemia)
  Denys-Drash syndrome (early onset renal failure)