Abstract
Intestinal atresia in both twins from the same pregnancy is very rare. Only seven
pairs of twins have been described. The authors report on two cases of monozygotic
twins with different types of intestinal atresia and clinical evolution. Both pairs
of observed twins turned out to be concordant for the presence of intestinal malformations
and for the absence of other linked malformations; nevertheless, the atresic lesions
were anatomically discordant in each pair of monozygotic twins. Therefore, the diagnostic
and therapeutic procedures have shown some differences in phenotypic expression between
the twins of both pairs. Possible etiologic factors and pathogenetic pathways are
discussed, and the importance of an accurate clinical and instrumental investigation
and a long-term follow-up is underlined. Very rare models, such as pairs of monozygotic
twins presenting intestinal atresia, represent an extraordinary resource to add new
clinical and laboratory information likely to be useful in future advancements to
understand the underlying etiology and pathogenesis.
Keywords
Intestinal atresia - monozygotic twins - apple peel - double-bubble sign - malformation
- concordance