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The Biological Basis of Craniofacially Conjoined Twins

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Fundamentals of Craniofacial Malformations

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Abstract

Conjoined twins are suggested to result from aberrant embryogenesis. The incidence rate is 1 in 50,000 births; however, since about 60% of the cases are stillborn, the true incidence is approximated at 1 in 400,000. There is a higher predisposition towards female than male gender with a ratio of 3:1. Conjoined twinning, however, only arises when the twinning event occurs at about the primitive streak stage of development, at about 13–14 days after fertilization in the human, and is exclusively associated with the monoamniotic monochorionic type of placentation. In the craniofacial region, three types of twinning can be present: craniopagus, diprosopus (facial duplication), or parasites. When the skull is mainly involved, they are termed craniopagus, whereas if the face is mainly involved they are termed cephalopagus or facial duplication (diprosopus). If there is an asymmetrical twinning, such phenotypes are termed parasites. The two main theories proposed to explain the phenomena are fission and fusion. Whereas both theories could potentially be extrapolated from embryological adjustments exclusively seen in conjoined twins, these theories cannot be applied to the full range of conjunction possibilities and thus remain a matter of persistent inconclusiveness. Conjoined twins are classified based upon the site of attachment.

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Authors and Affiliations

  1. Craniofacial Center, Kieferklinik Münster, Münster, Germany

    Ulrich Meyer

  2. University of Düsseldorf, Westdeutsche Kieferklinik, Moorenstrasse, Düsseldorf, Germany

    Ulrich Meyer

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  1. Craniofacial Center, Kieferklinik Münster, Münster, Germany

    Ulrich Meyer

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Meyer, U. (2021). The Biological Basis of Craniofacially Conjoined Twins. In: Meyer, U. (eds) Fundamentals of Craniofacial Malformations. Springer, Cham. https://doi.org/10.1007/978-3-030-46024-2_11

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  • DOI: https://doi.org/10.1007/978-3-030-46024-2_11

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