Acardia Myelacephalus. Presentation of the malformation
and review of the literature, by the occasion of a rare case
Amplianitis I.,1 Hadjileontis
C.,1 Nepka H.,1
Madenis N.,2 Kafanas A.1
1Department of Pathology,
Serres General Hospital
2Department of Gynecology and
Obstetrics, Serres General Hospital
The term “acardia” is used to describe a rare congenital anomaly
that occurs during multiple, usually twin pregnancies. Acardia
concerns severe developmental and functional malformations of
the heart, leading to the parasitic sustenance in life of the
abnormal fetus through vascular anastomoses with the normal twin.
We present a case of acardia myelacephalus that was diagnosed
by echo scan during the third month of a twin monochorionic pregnancy.
The co-twin was growing normally and the pregnancy progressed
having the fetuses under ultrasonographic surveillance. The acardiac
twin was eventually delivered with the normal one during the thirteenth
week of gestation. In gross examination the abnormal fetus composed
of an oval headless mass enclosing a central cavity, within which
an intestinal formation was found presenting with blind ends.
Between the central cavity and the lower portion of the mass,
there was tissue of increased vascularity containing epithelial
structures, most probably representing the urogenital vestiges
of the fetus. By dissection no heart was found, while the spinal
cord was bifurcated and the origins of the lower limps were spotted
within the lower portion of the amorphous mass. On the occasion
of this presentation several aspects of acardia its etiology and
pathogenesis are being reviewed and discussed.
Key words: Acardia, myelacephalus, monoamniotic pregnancy,
monozygous twins.
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