PubMed · 7338605
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B Le Marec. 1981. [Iniencephaly].. https://pubmed.ncbi.nlm.nih.gov/7338605/
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Acardiac twinning is the most extreme form of twin-twin transfusion syndrome occurring in monzygotic twin pregnancies with monochorionic placentation. A case of acardiac amorphous foetus, occurring in association with spontaneously conceived triplet pregnancy, diagnosed on ultrasound in early second trimester is described and its antenatal management and brief review of literature discussed.
Acardiac twinning is a very rare complication of multiple pregnancy. The authors present the neuropathological and anatomopathological description of the twins of the multiple pregnancy complicated by the acardiac foetus and terminated at 26 weeks of gestation. An anatomopathological examination of the "normal" twin showed hyaline membrane syndrome, cardiomegaly and hepatomegaly. Neuropathologically, numerous hypoxic-ischaemic lesions, most likely associated with haemodynamic disorders during pregnancy as well as less pronounced perinatal changes were revealed. The acardiac foetus, classified as acardius acephalus, demonstrated the presence of some abdominal organs and a histologically well-developed spinal cord. In view of the neuropathological changes, monitoring "normal" twins for discreet pathological central nervous system signs, which may be similar in character to those described, may play a significant role.
Cyclotocephaly is a very rare malformative lethal condition which associates otocephaly (extreme hypoplasia of the mandibular arch with agnathia) and cyclopy with proboscis. The head of a cyclotocephalic neonate from our Museum of Anatomy and Embryology was examined using computed tomography (CT). Cutaneous and osseous three-dimensional reformations were performed. Severe bony malformations were observed. A single orbital cavity was surrounded by a cartilaginous proboscis and a median fusion of maxillae, temporal and zygomatic bones. The single orbital cavity contained both paramedial eyeballs (synophthalmia). The external auditory meati and the ear pinnae were also parasagittal. No oral cavity and mandible were observed. Despite the poor conservation state of the brain, lobar holoprosencephaly was suspected. The mesencephalon and pituitary gland were absent. This exercise could lead to optimizing ultrasonographic prenatal diagnosis.