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Conjoined twins and conjoined triplets: At the heart of the matter

Journal

BIRTH DEFECTS RESEARCH
Volume 114, Issue 12, Pages 596-610

Publisher

WILEY
DOI: 10.1002/bdr2.2066

Keywords

congenital anomaly; conjoined triplets; conjoined twins; embryonic disk model; embryology; heart; teratology

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Conjoined triplets are rare human malformations, and the number and mutual position of hearts are crucial for correct diagnoses.
Conjoined triplets are among the rarest of human malformations, as are asymmetric or parasitic conjoined twins. Based on a very modest corpus of recent literature, we applied the embryonic disk model of conjoined twinning to 10 previously reported cases involving asymmetric anatomical multiplications to determine whether they concerned conjoined twins or conjoined triplets. In spite of their phenotypic similarities, we diagnosed four of these cases as conjoined twins and three of them as conjoined triplets. In the remaining three cases, no definite diagnosis could be made, as essential information was lacking from the reports. We conclude that it is not necessarily the expected duplication or triplication of structures that points to the correct diagnosis in these cases, but the number and mutual position of the hearts they presented with. Considering their rarity we stress to thoroughly investigate and describe internal (dys)morphology in novel cases of (asymmetric) conjoined twins and triplets to further unravel their pathogenicity and come to the correct diagnoses.

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