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