Wiedemann-Beckwith syndrome in one of monozygotic twins

Sir, The article by Bose and his colleagues' deserves some comment. It is certainly unusual to find an exomphalos in one of monozygotic twins, although it has been described before. To label a baby as Beckwith-Wiedemann syndrome simply on the basis of the presence of an exomphalos and a large tongue is surely, however, not justified. The essence of the syndrome is the combination of exomphalos, macroglossia, and gigantism, manifest as both somatic and visceromegaly, together with the sporadic occurrence of other features such as indented ear lobes and naevus flammeus. The definitive histological diagnosis is based on the finding of cytomegaly of the adrenal cortex. In the absence of some corroborative findings it would be more reasonable to consider this baby as suffering from exomphalos, complicated by the incidental finding of macroglossia. I am also interested in the authors' use of the term Wiedemann-Beckwith syndrome. Since the syndrome was described by Beckwith in 1963 (Beckwith JB. Extreme cytomegaly of the adrenal fetal cortex, omphalocele, hyperplasia of the kidneys and pancreas, and Leydig cell hyperplasia-another syndrome? Presented at annual meeting of Western Society for Pediatric Research, Los Angeles, California, 1963.) and by Weidemann in 1964,2 then for both chronological and alphabetical reasons it would seem reasonable to use the well established and recognised terminology of Beckwith-Wiedemann syndrome. Perhaps, however, the authors can explain the justification for this change.