Atelencephalic microcephaly: craniofacial anatomy and morphologic comparisons with holoprosencephaly and anencephaly.

This is the first detailed report of the craniofacial anatomy of atelencephaly. Comparisons were made with a control specimen and others with holoprosencephaly, anencephaly, and anencephaly with holoprosencephalic facial features. In the atelencephalic fetus, severe microcephaly, flattened calvaria, and partial synostosis of the metopic suture were apparent.

Atelencephalic microcephaly in a 21-week human fetus.

Atelencephalic microcephaly, a rare and extreme disorder, is known morphologically by only six cases. Derivatives of the telencephalon are absent or dysplastic, while more caudal structures are normal or mildly deformed. A more extensive form, aprosencephaly, involves structures of the diencephalon and may be associated with holoprosencephalic facies.

Management of spina bifida and associated anomalies.

Myelomeningocele and its accompanying deformities are among the most complex and frequent of the malformations to which the developing nervous system is subject. a multidisciplinary approach to management of the patient with myelomeningocele is essential, and with aggressive and continuous care, the results may be extremely gratifying.

Arteriovenous malformations of the brain: a teratologic challenge.

Congenital arteriovenous malformations of the brain are lesions which are well-known to neurologists and neurosurgeons but are puzzling to teratologists because they occur sporadically and are unassociated with congenital malformations outside the central nervous system. They are recommended to teratologists as a field of etiologic and pathogenetic research.

Laissez-faire in research.

These were informal remarks in an after-dinner speech about nutritional and pediatric research as it was initiated 50 years ago when the Cincinnati Children's Hospital Research Foundation was opened. Although from the beginning there were "divisions" in the institute, the success of the organization was due to cooperation rather than division and to the freedom of the investigators to alter their directions according to their change of interests and to changes of needs and opportunities. A plea is made to let capable investigators choose their topics and their methods of research; they are most likely to have success when they are left alone and free of outside pressures.

Congenital malformations of the central nervous system and transplacental carcinogenesis: modification of ethylnitrosourea-induced brain tumors in rats by pretreatment with methylazoxymethanol.

X-irradiation of rat fetuses prior to exposing them transplacentally to the neurotropic carcinogen ethylnitrosourea (ENU) greatly reduces the frequency of offspring that develop neurogenic tumors. Since the tumor inhibition may have been related to the teratogenic effects of the irradiation of the fetal brain, it was of interest to learn whether another means of causing such brain damage would also interfere with the development of ENU-induced neurogenic tumors. For this purpose methylazoxymethanol (MAM), known to produce microencephaly, was used.

Experimental spina bifida and associated malformations.

Seven litters and 17 near-term rats of mothers treated with teratogens during gestation were analyzed for concordance and discordance of congenital malformations of the central nervous system. In animals with spina bifida a firm association with Arnold-Chiari malformation was found but only in fetuses near term. Associations with aqueduct stenosis and hydrocephalus were poor.

Dose-related reduction by prenatal x-irradiation of the transplacental neurocarcinogenicity of ethylnitrosourea in rats.

Pregnant Sprague-Dawley rats were X-irradiated on the 16th day of gestation with 5 to 250 rads and given i.p. injections 4 days later with 10 mg ethylnitrosourea per kg.

The medical profession and congenital malformations (1900-1979).

Physicians' interest in congenital malformations has varied greatly during the last two centuries. After an acme in the 19th century, teratology was of little inteerest to the medical profession during the first four decades of the 20th century. Since then a variety of events have again made birth defects important.

Oncogenic response of rats with x-ray-induced microcephaly to transplacental ethylnitrosourea.

The relation of congenital malformations to tumor development was examined. Pregnant Sprague-Dawley rats were given 200 rads of X-rays on the 15th or 16th day of gestation and injections of 10 mg ethylnitrosourea (ENU)/kg 1-4 days later, or they were irradiated or injected only. Surviving weanlings that had been irradiated had micrencephaly and other malformations.