Holoacardius Acephalus

Aside from a mention by Schinz et al. (5), the radiological literature fails to reveal any discussion of holoacardius acephalus, a rare type of fetal monstrosity in which the heart and head are completely absent. This entity can be determined radiographically if the possibility is considered, and it is noteworthy that in the few cases where prenatal roentgen reports are available, a diagnosis of anencephaly was made, as in the case to be presented. Case Reports B. R., a 28-year-old white female, entered the hospital in labor at term, with ruptured membranes. Her first pregnancy had resulted in a normal male infant 6 lb. 7 oz. in weight after forty weeks gestation, a labor of thirty-six hours, and forceps delivery. A second pregnancy had terminated at six months with normal spontaneous delivery of twins after a labor of seven hours; both infants died within twenty-four hours. The present pregnancy, her third, had progressed normally, with a 19-1b. gain in weight. On admission to the hospital, all findings were normal except for polyhydramnios of a degree which prevented clinical determination of fetal position and presentation. An abdominal roentgenogram (Fig. 1) revealed a twin pregnancy, with one of the twins appearing normal in the left occiput trans verse position, in vertex presentation. The second twin was markedly smaller, in a transverse lie across the upper fundus anteriorly, and no skull could be visualized. The radiographic diagnosis was twin pregnancy, with one normal fetus and one anencephalic monster. The patient delivered spontaneously after six hours of labor. A normal male infant was delivered first, followed in about ten minutes by the dead monster. A single placenta was delivered by Crede maneuver and revealed a double cord which confirmed the fact of identical asymmetrical twins. It is unfortunate that the blood supply to the umbilical cords was not studied, as this probably holds the clue to the development of the acardiac monster. The hospital course was normal and the mother was discharged six days after delivery. Discussion The subject of holoacardius is reviewed in the recent paper of Gillim and Hendricks (1) from the clinical and obstetrical point of view, and the pathology is discussed by Edith Potter (4). The reader is referred to these works for a more complete consideration of the subject. Prenatal radiographic studies are rare in this fetal abnormality and, when done (Kappelman, 2; Schinz et al., 5), have led to a diagnosis of anencephaly, as in the present instance. The incidence of holoacardius as calculated by Gillim and Hendricks from the literature is 0.028 per 1,000 births. Anencephaly has been estimated by Ru-Kan Lin and Plenk (3) as having a rate of 0.72 per 1,000 births; it is said to be much less frequent in twin pregnancies, but the percentage incidence is not given.