“Chance favors only the prepared mind.” The purpose of this note is to draw the attention of radiologists to a rare fetal anomaly, iniencephaly, which can be rationally diagnosed in utero radiologically. Incidence: Iniencephaly is said to comprise a little over 1 per cent of all fetal abnormalities (5). The incidence has been estimated to be between 1 in 900 (4) and 1 in 2,200 deliveries (5). Only about 70 cases have been reported. Occurrence of the anomaly, however, is unquestionably greater than indicated by the literature: depending on local interest in teratology, some iniencephalic monsters may be discarded, or assigned to a specimen jar, in either case unreported. Too, there is a reluctance (or inertia) in many quarters to make single case reports. Pathology: The abnormalities of iniencephaly (inion, nape of neck) have been repeatedly detailed throughout the scanty literature, and only the salient findings need be given here: the fetal head is hyperextended, with a much enlarged foramen magnum opening directly into the upper cervical reaches of the spinal canal via defective neural arches and widened pedicles, making essentially a common cavity between the brain and much of the spinal cord. The vertebrae, especially the cervical, are fused, reduced in number, and of bizarre shape. The ventricular system may be dilated, with thinning of the cortex; all degrees of anencephaly may be present (2); the spinal cord is usually defective. There may be a meningocele. The soft parts of the anterior chest pass directly onto the permanently star-gazing face, by-passing a neck, resulting in a characteristic appearance. Associated anomalies of other structures are common, especially talipes and defective diaphragm. Iniencephaly has been regarded as an especially severe form of the Klippel-Feil syndrome (1). Most of these monsters are female, and most are stillborn; survival beyond the first day is rare. Antenatal Diagnosis: As with other fetal anomalies, polyhydramnios is frequent. There is some evidence that the mothers are not young and that there has been a rather long interval since the preceding pregnancy (4). This is doubted by some (5). Breech presentations are common. Delivery may be surprisingly easy, especially in view of the extension of the head, or may be difficult (5). Roentgen examination is the only way by which a positive antenatal diagnosis may be made. Although many of the cases in the literature were so examined, usually for polyhydramnios, never to our knowledge has the specific diagnosis been offered; only that the fetus was abnormal. Nor was the specific diagnosis made in the present case. Case Report Mrs. M. H., white, twenty-eight years old, patient of Drs. Gordon Jones and Lee Earnhardt, had been delivered four years earlier of a normal child who died of “pneumonia” at twenty days. Except for this and early abortions three and two years before, the past history and family history were normal.
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