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Beckwith-Wiedemann Syndrome

  • organomegaly, omphalocele, large tongue triad
  • exopthalmos, macroplassia, gigantism, visceromegaly, abdominal wall defects, neonatal hypoglycemia
  • hemihypertrophy(15%) with spastic CP
  • BWS patients with hemiphypertrophy have @40% chance of developing malignancies (Wilms tumor, hepatoblastoma) frequent abdominal ultrasounds is recommeneded until age 7. (DeBaun MR, J Pediatr 1998;132:398).
  • predisposed to Wilms Tumor(need screening)

Hemihypertrophy Differential Diagnosis

  • Neurofibromatosis
  • Proteus syndrome
  • Klippel-trenaunay syndrome
  • References: Ballock RT, JBJS 1997;79A:1731