Major congenital neurologic malformations. A 17-year survey
T. E. Wiswell, D. J. Tuttle, R. S. Northam and G. R. Simonds
Neonatology Service, Walter Reed Army Medical Center, Washington, DC.
We present characteristics of four major congenital neurologic
malformations--anencephaly, spina bifida, encephaloceles, and
hydrocephalus--from a population of 763, 364 live-born and still-born
infants born from 1971 through 1987. During the 17-year study period, 275
infants had anencephaly (0.36 per 1000 total births), 526 had spina bifida
(0.69 per 1000 total births), 112 had encephaloceles (0.15 per 1000 total
births), and 370 had hydrocephalus (0.48 per 1000 total births). There was
a female preponderance of infants with anencephaly, spina bifida, and
encephaloceles, while males predominated among those with hydrocephalus. We
found declining incidences of anencephaly, spina bifida, and encephaloceles
only among white females. Black infants were significantly less likely than
white infants or infants of other races to have spina bifida. Twenty
percent of infants with anencephaly had congenital anomalies unrelated to
the primary defect, as did 40% with encephaloceles, 37% with hydrocephalus,
and 22% with spina bifida. Because the racial background of the patient
population closely resembles that of the United States as a whole, the
features of the malformations described may reflect those of the country.
