[Beckwith-Wiedemann syndrom: a case report in Dakar].

Abstract

Background: Beckwith-Wiedemann syndrome is a congenital syndrome with variable phenotypic expression. It is less commonly described in Africa. We report a case in Dakar universitary hospital center.

Observation: This report is about a two month old child from Mauritania presenting an hemihypertrophy, macroglassia and an umbilical hernia. Glycemia was under normal level showing a mild hypoglycemia (0,6 g/dl). T3, T4 and TSH values were in normal range. Abdominal echography was normal. Our patient was stable at the first clinical examination.

Conclusion: we advocate for dietetic measures and rigorous clinical follow up, every 3 to 6 month, to screen for recurrent hypoglycaemia and the occurence of an eventual neoplasmic desorders.