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The germinal matrix is the birthplace of neurons during embryonic development. If the neuroblasts (nerve cell precursors) born here fail to migrate normally to their proper location, all of the succeeding events of brain development are altered as well. Such an abnormal sequence of developmental events is thought to be responsible for schizencephaly, in which pathological clefts in the brain are among the abnormal findings. Schizencephalies, the term for these malformations, are always the result of abnormal development, and must be distinguished from other conditions in which developing brain tissue is destroyed. The authors review three cases of schizencephaly and contrast the neuropsychological deficits with the appearance of the brain on magnetic resonance imaging. Two of the patients were examined for seizure activity; the third was seen because of muscle weakness. Two of the three patients showed slow development as children, but all had graduated high school and lead normal lives. The magnetic resonance images revealed striking abnormalities of the lobes of the brain, including large clefts. A curious finding was that all three patients were left-handed. The patients did not have any difficulty with normal conversational language, but comprehensive assessment revealed some deficits in language skills. The results of this study are an example of how developmental brain damage which does not show up as global mental retardation or a decrease in IQ scores can nevertheless be identified on occasion by comprehensive neuropsychologic testing. (Consumer Summary produced by Reliance Medical Information, Inc.)

The major causes of cerebral palsy were researched using a massive database prepared by the United States National Institute of Neurological and Communicative Disorders and Stroke. Cerebral palsy was defined as a chronic neurological disorder involving abnormal control of movement or posture that occurs early in life and does not progress. This prospective study of 43,437 full-term infants identified 34 quadriplegic and 116 nonquadriplegic children who suffered from cerebral palsy. Significant events attending to the pregnancy, labor, delivery, and early postnatal period of the infants were recorded and developmental progress was evaluated up to seven years of age. Twenty-three infants were diagnosed with cerebral palsy before the age of one, and the remaining 127 children were diagnosed by the age of seven. The 150 cases of cerebral palsy ranged from mild to severe. The data suggest that 53 percent of the 34 quadriplegic children suffered from cerebral palsy as the result of congenital disorders, and that only 14 percent of the cases of quadriplegia were caused by asphyxia, or decreased oxygen supply, during the birthing process. In the less severely afflicted group, 35 percent of the cases were attributed to congenital problems and 6 percent to other disorders. Only 9 of the 150 cases of cerebral palsy were caused by birth asphyxia (6 percent). Congenital disorders were responsible for nearly four times as many cases of quadriplegic cerebral palsy than birth asphyxia. Contrary to popular opinion, these data suggest that most cases of cerebral palsy do not result from a reduced supply of oxygen during a difficult birth. To validate a correct diagnosis of birth asphyxia, the importance of accurate documentation of measurements and observations of newborns is stressed. (Consumer Summary produced by Reliance Medical Information, Inc.)

ABSTRACT: A study on the frequency of birth defects was conducted in the area around Seveso, Italy, which was contaminated by 2,3,7,8-tetrachlorodibenzo-p-dioxin in July 1976; this has been the largest population ever exposed to dioxin. From Jan 1, 1977, to Dec 31, 1982, a total of 15291 births (still and live) were examined, and malformations were reported to an ad hoc birth defects registry. In the most highly contaminated area, 26 births were observed. None of these infants had any major structural defect. Two infants had mild defects. The frequencies of major defects detected in the areas of low or very low contamination were 29.9/1000 and 22.1/1000, respectively. A frequency of 27.7/1000 was registered in the control area. Relative risks were calculated for specific categories of birth defects and for grouped malformations. Although the data collected failed to demonstrate any increased risk of birth defects associated with 2,3,7,8-tetrachlorodibenzo-p-dioxin, the number of exposed pregnancies was not big enough to show a low and specific teratogenic risk increase.

The congenital anomaly of isolation of the subclavian artery, on the left side, is associated with a right aortic arch. The subclavian artery loses its connection with the aorta and is connected to the left pulmonary artery via the ligamentum arteriosum. Subclavian steal syndrome accompanies this anomaly in a minority of Patients. We report 2 cases of adults with subclavian artery isolation, one of whom was symptomatic, and we discuss the importance of recognizing its presence.

The public and the legal system are conditioned to the notion that when brain damage occurs in newborns, and cerebral palsy results, someone must be blamed. Pediatricians remain at risk for suits which attempt to establish that they, or the obstetrician, erred in some way that caused this unfortunate outcome. The plaintiff's lawyer must show that medical care fell below some accepted standard and that, as a result, the child developed cerebral palsy. Cerebral palsy is defined as a nonprogressive motor problem caused by damage, or a lesion, within the nervous system. The exact location of the lesion and its time of production are responsible for the unique type of motor impairment seen in each case of cerebral palsy. A study presented in the October 1989 issue of the American Journal of Diseases of Children provides important evidence that cerebral palsy is usually not the result of birth trauma or other neonatal injuries, but is the consequence of intrauterine difficulties which are not preventable. There has been much confusion in the literature regarding the association of cerebral palsy with difficult birthing (such as occurs in breech birth). This analysis of 60,000 pregnancies indicates that difficult birthing may itself be a consequence of untoward events of intrauterine development. The pediatrician has a role in preventing the continuance of malpractice suits which are filed as a consequence of needing to blame someone for a child's cerebral palsy. Pediatricians should exercise caution by providing objective documentation of a newborn's health status. The pediatrician must actively aid the legal profession, other physicians, and the public-at-large to gain insight into the possibility that, when the outcome of pregnancy is not perfect, perhaps no individual is responsible. (Consumer Summary produced by Reliance Medical Information, Inc.)