S E M I N A K S I N NEUK0IX)C;Y-VOL,UME

1 1, N O . 1

MARCH 1991

HISTORICAL NOTES

Do you think I would apply a song title like the one that heads this essay to the subject of anoxia o r anoxemia in the neonate, infant, and child? If you know me, of course you do. But it's not something I made up: I was in the audience when the Class of 1954, the class ahead of mine in medical school at Western Keserve University, said goodbye to their teachers. In a skit called "Is my Baby Blue Tonight?" which made fun of Dr. B. . . , the obstetrician who had brought me into the world, he was portrayed performing a delivery. 'l'he parturient patient was being maintained under anesthesia by a nurse with a watering can filled with ether. After the baby had finally been extracted, with high forceps, the nurse reported to the doctor that there was still some ether left in the can, and asked what she should d o with it. "Finish it off," said the doctor as he strode from the stage. T h e days of "soaking" mothers and babies with ether are over, and you may think we are better off for their passing, but I am not so sure. After all, in my own case, although I was delivered in that manner and, additionally, had fetal distress with bradycardia that forced Dr. B. . . to perform a "version and footling," dragging me forth to end a prolonged labor, things turned out pretty well. I wrote an essay a few years ago to prove the postulate that, at any age, head trauma may have a beneficial efftct; further, I contended, Normal maturation of'the human lwruous system depends upon head truuma in childhood.' In the article, I cited my own case of head injury in childhood and my brother's

more dramatic one, and I pointed out that we had both become professors but that he is also a department chairman. Now I have reached the realization that it is not only a knock on the head that rnakes a baby rnature successfully; some oxygen deprivation is needed, too. Please allow me to prove it. When 1 attended that class show in 1954, the Apgar score for rating the fitness of babies for their new lift had been available for a couple of years, and newborns were losing a point for being cyanotic (as they still d o today). It would be another year before Dr. Apgar would confess that "in the immediate postnatal period cyanosis does not necessarily parallel oxygen saturation as measured."' By then, "blue is not beautiful" had been ingrained in o u r minds. Some recent observations-if I pick and choose to support my thesis-make it clear that asphyxia, by itself, is not necessarily bad for newborns: Mujsce and coworkers report that "hypoxicischemic brain swelling in the newborn human infant is not associated with any major disruption of the cerebral circulation so long as systemic blood pressure is maintained.":' In their baby-rat model, in which one common carotid artery is ligated, 3 hours of hypoxia (8% oxygen at 37°C) produces neuronal necrosis, infarction, o r both, only in the cerebral hemisphere on the same side as the ligated vessel; "hypoxia alone produces no damage."" Even severe asphyxia may prove not to be as injurious to the newborn as we have been led to expect:

Departtiierlt of Neurology, Utiiversity of' Kochestel-, School of Medicine ant1 llentistry, Rocllestc.1-, New York Rcprilit requests: Dr. 1)avitl (;olclblatt, Box 673, Ut~ivel-sityof Rochester Metlical Center, Rochestet-, NY 1464'2 Copyright 0 1991 by ~ f h i e m ebleclical Publishers, Inc., 381 I'ark Avenue Soutll, New York, NY 100 16. All rights reserved.

Downloaded by: Universite Laval. Copyrighted material.

Is My Baby Blue?

SEMINARS IN NEUROLOGY

happened the others in the series, you have to read the article.) Scott studied intrapartum asphyxia of varying severity and duration.-she concluded that "acute periods of more complete asphyxia were not necessarily harmful."' Among 48 newborn infants, all of whom had Apgar scores of 1 and 2 at 1 minute, 23 survived; one child in later years had a developmental/intelligence quotient above 130." Now we begin to move beyond the idea that oxygen deprivation is not necessarily bad to the thesis that it may be good. After all, in how many classrooms does 1 child in 23 have an IQ above 130? A score that high or higher on the WechslerBellevue I is achieved by 1 in 100 and on the Wechsler Adult Intelligence Scale by 1 in 40, as nearly as I can judge from the bar graphs in Dr. Wechsler's book." Bullough described "an infant, the first of twins, [who] endured 20 minutes' intrauterine asphyxia, and 10 minutes' neonatal asphyxia . . . his first attempt to breathe was 40 minutes after- delivery . . . The child appears normal at the age of 1 year, having won a baby competition at 9 months."' Again, the suggestion of an advantage to the asphyxiated. Consider next the series of affectionate observations by Helen Taussig, during the 1970s, on what became of the blue babies for whom she and Alfred Blalock had devised a new life.' It is probably important for me to state this: I do understand, with Blalock and Taussig, that "it is not the cyanosis, per se, which does harm . . . cyanosis is a striking manifestation of the underlying anoxemia and the compensatory p ~ l ~ c y t h e m i a And . " ~ I am certainly not contending that the operation did no good: that it did was apparent from the first. In the original report, Blalock and Taussig said of one of their first 3 children, "His disposition has changed from that of a miserable whining child to a happy smiling boy."8 For that one case alone, and for the thousand that followed by 1950, they deserved to have their pictures taken by Yousef Karsh." T h e longterm outcome for the blue babies was not only very good; it was too good. In Dr. 1-aussig's first follow-up report (in which she reminds the reader that "patients who had had a Blalock-Taussig anastomosis do not have full saturation of the arterial blood"), 180 replies to a questionnaire were received.

76

Undoubtedly those who were doing well were proud to advise us of their achievement. Nevertheless, it

is extremely gratifying that 53 persons had reached the top professional bracket of doctors, lawyers, ministers, teachers. or nurses, by December 31, 1966. Moreover, two thirds of these individuals were living on their first anastomosis when they attained their top status. Among them was a young man who received his appointment as a full professor of law before his thirtieth birthday."'

Intelligence, as well as attainment, must be considered in trying to find out how well cyanotic newborns get along: Apgar et a12reported that "no significant correlation was found between levels of blood oxygen content measured in the first three hours after birth and intelligence as gauged by Stanford-Binet testing in early childhood." Usdin and Weill' reviewed the cases of 41 children who were apneic for three or more minutes at birth: Evaluation of intelligence by Stanford-Binet tests revealed a normal distribution for the intelligence of the apneic group around a mean quotient of 96.2, whereas that of the control group was normally distributed around a mean of 93.0. T h e range of intelligence quotients of the apneic group was from 71 to 130, whereas that of the controls was from 69 (with the exception of a 58 which was substantially separated from the other scores) to 129. Particularly noteworthy is the fact that one child who had a 10 minute apneic period at birth had an intelligence quotient of 105 fourteen years later. Two children who had experienced five minute periods of neonatal apnea had the highest intelligence quotients (130) of all the children studied.

Shampaine and his colleagues, studying tetralogy of Fallot (TOF), recently investigated the hypothesis that, in children with TOF, "surgical correction of the cardiac lesion and elimination of the cyanosis should lead to an improvement in physical, psychological, and cognitive performance on follow-up testing some 22 years postoperatively."" They concluded that "child and adult I Q scores remained relatively stable" over that span of more than a score of years-but to derive their conclusion, they had to do some fancy footwork with the statistics: T h e full-scale I Q scores of the children had a mean of 97.5; the comparable scores when they became adults had a mean of 93.4. The difference was statistically significant, but "was not considered a meaningful difference," for reasons you can read in their article if you are not swept along by the force of my argument. Further support for the idea that "blue is beneficial" came from comparing the TOF subjects with their families: they "had educational and occupational accomplishments similar to those of their same-sex parents," according to the report, but "11 subjects (52.4%) had the same educational degree, seven (33.3%) had a higher degree, and three (14.3%) had a lower degree. Among the same-sex siblings, 18 (60.0%) attained the same educational degree as the subjects,

Downloaded by: Universite Laval. Copyrighted material.

one baby who had a 1 minute Apgar score of 0 and a 5 minute score of 1, in a study by Clancy et al,l was normal at the age of 4 years. ( T learn ~ what

VOLUME 11, NUMBER 1 MARCH I991

stroke. Despite all that (or, I say, because of it), he is a talented, well-loved clinician with a national reputation. T h e biologic advantage of living with cyanosis is probably small, but it may prove increasingly important in a deteriorating environment. Adverse atmospheric conditions may make us all a little smarter in years to come. I certainly hope so.

REFERENCES Goldblatt D. Mrs. Gage and Mrs. J o e : T h e case for head injury. Semin Neurol 1985;5:265-7 Apgar V, Ghirdany BK, McIntosh R, Taylor HC. Neonatal anoxia. I. A study of the relation of oxygenation at birth to intellectual development. Pediatrics 1955; 15:653-61 Mujsce DJ, Christensen MA, Vanucci KC. Cerebral blood flow a n d edema in perinatal hypoxic-ischemic brain damage. Pediatr Res 1990;27:450-3 Clancy R, Legido A, Newell R, et al. Cor~tinuousintracranial pressure monitoring and serial electroencephalographic recordings in severely asphyxiated term neonates. Am J Dis Child 1988; 142:740-7 Scott H. Outcome of very severe birth asphyxia. Arch Dis Child 1976;5 1:712-6 Wechsler D. T h e measurement and appraisal of adult intelligence, 4th ed. Baltimore: Williams and Wilkins, 1958, p 107 Bullough J . Protracted foetal and neonatal asphyxia. Lancet 1958; 1:999-1000 Blalock A, Taussig HB. T h e surgical treatment of malformations of the heart in which there is pulmonary stenosis o r pulmonary atresia. JAMA 1945; 128: 189202 Suss RA. Blalock's first blue baby? (letter) Hopkins Medical News 1990; 14(2):5 Taussig HK, Crocetti A, Eshaghpour E, et al. Long-term observations on the Blalock-Taussig operation. I. Results of first operation. lohns Hopkins Med [ 1971; 129:243-89 11. Usdin GL, Weil ML. Effect of apnea neonatorum on intellectual development. Pediatrics 1952;9:387-94 12. Shampaine EL, Nadelman L, Rosenthal A, Behrendt D, Sloan H. Longitudinal psychological assessment in tetralogy of Fallot. Pediatr Cardiol 1990; 10: 155-40 13. Hayden MR. Huntington's chorea. Berlin, Heidelberg, New York: Springer-Verlag, 1981: 118

Downloaded by: Universite Laval. Copyrighted material.

five (16.7%) attained a higher degree, and seven (23.3%) attained a lower degree." If you look closely, it's "advantage: cyanotics." And note that, because this was a long-term study (that is, these were the children of the early years), the average age at surgical repair for those children was 8.7 years. (Nowadays, most T O F patients undergo repair prior to the age of 4, and even in infancy.) In other words, they had had a healthy dose of blueness. A small, selective biologic difference is likely to get the attention only of Darwinians and basketball recruiters. A medical example that comes to mind is the reproductive fitness of persons with Huntington's disease: although most would predict that "without preventive measures and notwithstanding mutations, the frequency of the disease will remain constant," some investigators, whose studies were summarized by Hayden,'" have concluded that "the gene for Huntington's chorea enhances patients' fertility relative to the general population and that consequently, in the absence of adequate control measures, this disorder will become more common." It is not statistical arguments but personal observations that most persuade me to believe in the value of asphyxia and cyanosis: In my lifetime, I have been friends with only two people who were blue babies. One underwent a Blalock operation late in childhood; definitive correction was put off until he was in his twenties, and only after he had developed an embolic abscess of the left parietal lobe that ruptured into the lateral ventricle and very nearly killed him. That man holds two Ph.D.s and is a violinist who is the concertmaster of an orchestra. My second friend (who was once my teacher in medical school) endured one-flight dyspnea with severe cyanosis, without any surgical intervention at the advanced age of 37 years. Prior to surgery, he sustained an embolic brainstem

Is my baby blue?

S E M I N A K S I N NEUK0IX)C;Y-VOL,UME 1 1, N O . 1 MARCH 1991 HISTORICAL NOTES Do you think I would apply a song title like the one that heads t...
275KB Sizes 0 Downloads 0 Views