A case of acardius holosomus holocranius is presented. Ultrasound examinations, feto-amniography, and analysis of the amniotic fluid and the cystic fluid were performed.In addition, 88 cases of acardiac anomalies born in Japan were collected by reviewing the autopsy records of major institutes and literatures. Classification, incidence, epidemiology, diagnosis, management and etiology of this condition are discussed.
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