How is Beckwith-Wiedemann syndrome differentiated from Wilms tumor on imaging?

Updated: Mar 04, 2019
  • Author: Ali Nawaz Khan, MBBS, FRCS, FRCP, FRCR; Chief Editor: Eugene C Lin, MD  more...
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Answer

Beckwith-Wiedemann syndrome may be associated with omphalocele (12% of cases) and organomegaly. The incidence of hemihypertrophy, renal anomalies, Wilms tumor, and hepatoblastoma is increased. Antenatal diagnosis is possible.

Heredofamilial features of cystic dysplasia

Cystic renal dysplasia is a component of several familial syndromes. Renal cysts have been described in association with more than 50 syndromes. These include the trisomies, von Hippel-Lindau disease, Jeune syndrome (asphyxiating thoracic dystrophy), Meckel-Gruber syndrome, short rib–polydactyly syndrome, Beckwith-Wiedemann syndrome, and holoprosencephaly.


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