Anatomic findings in dicephalic conjoined twins: Implications for morphogenesis

Abstract

The morphogenesis of conjoined twins is incompletely understood. We therefore conducted a postmortem study of dicephalus dibrachii dipus conjoined twins. The twins were born without pertinent history or prenatal diagnosis at 38 weeks and lived for several hours. External genitalia were female and partly duplicated; a caudal appendage was present in the thoracolumbar region. The heart and liver were shared and exhibited major abnormalities in configuration. Four lungs, three kidneys and adrenal glands, and two spleens were identified; biliary and upper gastrointestinal tracts appeared as mirror images. From these findings, we postulate three major sets of consequences arising from the anatomical disposition of the twin notochords (“paleoaxes”). 1) The degree of convergence/divergence of craniocaudal paleoaxes is variable. Convergences are maximal in the upper thoracic and sacral regions, where duplication of organs is minimal because of interaction aplasia. 2) In the horizontal plane, paleoaxes are sufficiently divergent to produce a degree of twin expression posteriorly, whereas anteriorly they converge to form a single, anterior, midline “neoaxis.” Interposed between these zones of paleoaxial and neoaxial expression are areas of variable interaction aplasia. 3) The left twin was in situs solitus; the right twin was in situs inversus in a manner resembling polysplenia.