Table 3.

Classification of BWS clinical signs according to the Beckwith-Wiedemann spectrum (BWSp) scoring system [51].

Cardinal Features (2 Points for Feature)
Macroglossia
Exomphalos
Lateralized overgrowth
Multifocal and/or bilateral Wilms tumors or nephroblastomatosis
Hyperinsulinism (lasting >1 week and requiring medical treatment)
Pathology findings: adrenal cortex cytomegaly, placental mesenchymal dysplasia or pancreatic adenomatosis
Suggestive Features (1 Point for Feature)
Birthweight >2SDS above the mean
Facial nevus flammeus
Polyhydramnios and/or placentomegaly
Ear creases and/or pits
Hyperinsulinism (lasting <1 week)
Typical tumors: neuroblastoma, rhabdomyosarcoma, unilateral Wilms tumors, hepatoblastoma, adrenocortical carcinoma or pheochromocytoma
Nephromegaly and/or hepatomegaly Umbilical hernia and/or diastasis recti