Beckwith–Wiedemann syndrome (BWS): A case report and literature review

  • TO Ogundiran Department of Surgery, University College Hospital, Ibadan PMB 5116, Ibadan, Nigeria
  • ME Aghahowa Department of Surgery, University College Hospital, Ibadan PMB 5116, Ibadan, Nigeria.
  • BJ Brown Department of Paediatrics, University College Hospital, Ibadan PMB 5116, Ibadan, Nigeria.
  • DO Irabor Department of Surgery, University College Hospital, Ibadan PMB 5116, Ibadan, Nigeria.
Keywords: Exomphalous, Macroglossia, Visceromegaly, Conservative management, Close follow-up

Abstract

Beckwith-Wiedemann Syndrome (BWS), also known as the EMG (Exomphalos, Macroglossia, Gigantism) syndrome was recognised independently by Beckwith in 1963 and Wiedemann in 1964 and is now a well established entity having been reported in more than two hundred individuals1,2,3. It constitutes a wide spectrum of clinicopathologic entity with varied combinations of congenital and time dependent abnormalities that often make diagnosis and management tasking3,4. There is paucity of report in the literature on this entity from the developing world. We present a case recently seen at the University College Hospital (UCH) Ibadan, Nigeria in order to create further awareness and highlight peculiarity of management as may be applicable in a setting as ours.

Keywords: Exomphalous, Macroglossia, Visceromegaly, Conservative management, Close follow-up.

West African Journal of Medicine Vol.22(1) 2003: 101-102
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