Beckwith-Wiedemann  Syndrome (BWS)

• 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
• Bechwith-Wiedemann Syndrome
• References: Ballock RT, JBJS 1997;79A:1731