Beckwith-Wiedemann syndrome

Beckwith-Wiedemann syndrome (BWS) is a congenital overgrowth disorder characterized by a unique set of features that can consist of:

The incidence is reported at ~1:13,000-37,000 live births.

The majority of cases are sporadic while a small proportion (~15%) is autosomal dominant with abnormality related to chromosome 11p15.5. The syndrome carries a well recognized increased risk for the development of neoplasia 4. Another 10-20 of cases show paternal disomy 11.

First described independently by JB Beckwith in 1963 and H-R Wiedemann in 1964 6,7.

Article information

rID: 7412
System: Paediatrics
Section: Syndromes
Tag: cases, cases
Synonyms or Alternate Spellings:
  • EMG syndrome
  • exomphalos-macroglossia-gigantism
  • BWS
  • Beckwith Wiedemann syndrome (BWS)

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Cases and figures

  • Case 1: chest radiograph
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  • Case 1: pelvic radiograph
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  • Case 1: scintigraphy
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  • Case 2
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  • Case 3: with bilateral Wilms tumors
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