Ogundiran T O, Aghahowa M E, Brown B J, Irabor D O
Department of Surgery, University College Hospital, Ibadan, PMB 5116, Ibadan, Nigeria.
West Afr J Med. 2003 Jan-Mar;22(1):101-2.
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 individuals. It constitutes a wide spectrum of clinicopathologic entity with varied combinations of congenital and time dependent abnormalities that often make diagnosis and management tasking. 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.
贝克威思-维德曼综合征(BWS),也被称为EMG(脐膨出、巨舌、巨人症)综合征,于1963年由贝克威思独立发现,并于1964年由维德曼独立发现,目前已成为一个公认的实体,已有两百多人的病例报告。它构成了一个广泛的临床病理实体,具有先天性和时间依赖性异常的多种组合,这常常使诊断和管理变得困难。来自发展中世界的关于该实体的文献报道很少。我们介绍了最近在尼日利亚伊巴丹大学学院医院(UCH)看到的一个病例,以便进一步提高认识,并强调在我们这样的环境中可能适用的管理特点。