Key result
Imaging reveals an absent heart and severe craniofacial and sirenomelic malformations in a 36-week holoacardius.
Case Report (n=1)
This case report details the severe anatomical anomalies of a holoacardius, including the complete absence of a heart, using multimodality imaging.
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Documents severe holoacardius anomalies via imaging; extends descriptive literature but leaves open etiology and management questions.
A holoacardius from a monozygotic, monochorionic twin pregnancy of 36 weeks is described. Trisomy-18 was diagnosed in the viable female cotwin. Computerized tomographic, radiologic, and ultrasonographic procedures were applied to the acardiac. Its three-vessel umbilical cord was velamentously attached to the single placenta. Grossly malformed and poorly developed craniofacial structures, absence of neck and upper extremities, sirenomelic lower extremity, absence of heart, and development of only a few skeletal elements were prominent features. A review of selected world literature emphasizes the limits of the estimates dealing with the total number of reported cases and the incidence of acardii.
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Bhatnagar et al. (1986) conducted a case report in Holoacardius (n=1). Holoacardius was evaluated. A 36-week holoacardius exhibited grossly malformed craniofacial structures, absence of heart, neck, and upper extremities, and a sirenomelic lower extremity upon imaging.
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