The first page of the original Little paper.
By W. J. Little, MD
Senior-Physician to the London Hospital; Founder of the Royal Orthopaedic Hospital; Visiting-Physician to Asylum for Idiots, Earlswood; etc.
Transactions of the Obstetrical Society of London, 3:243-344, 1861
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The first page of the original Little paper. |
Pathology has gradually taught that the foetus in utero is subject to similar diseases to those which afflict the economy at later periods of gestation. This is especially true if we turn to the study of the special class of abnormal conditions, which are termed deformities. We are acquainted, for example, with abundant instances of deformities arising after birth from disorders of the nervous system -- disorders of nutrition, affecting the muscular and osseous structures, -- disorders from malposition and violence. Each of these classes of deformity has its representative amongst the deformities which originate before birth, viz., congenital clubfoot, congenital rickets, congenital degenerations of muscles, amputations in utero from strangulation by umbilical cord or adventitious bands, intra-uterine fractures, &c.
There is, however, an epoch of existence, viz., the period of birth, during which, at first sight, we might consider that the foetal organism is subjected to conditions so different to those of its earlier and of its prospective later existence, that any untoward influences applied at this important juncture would affect the economy in a manner different to the influences at work during the periods ordinarily characterized as those of before birth and after birth.
The object of this communication is to show that the act of birth does occasionally imprint upon the nervous and muscular systems of the nascent infantile organism very serious and peculiar evils. When we investigate the evils in question, and their causative influences, we find that the same laws of pathology apply to diseases incidental to the acts of birth as those which originate before and after birth. We are, in fact, afforded another illustration that there exists no such thing as exceptional or special pathology.
Thirty-five years ago the pathology of deformities, if not invested with fable, was wrapped in obscurity; it was then scarcely perceived that the materials for intensive inductive observation existed.
Nearly twenty years ago, in a course of lectures published in the "Lancet," and more fully in a "Treatise on Deformities," published in 1853, I showed that premature birth, difficult labours, mechanical injuries during parturition to head and neck, where life had been saved, convulsions following the act of birth, were apt to be succeeded by a determinate affection of the limbs of the child, which I designated spastic rigidity of the limbs of new-born children, spastic rigidity from asphyxia neonatorum, and assimilated it to the trismus nascentium and the universal spastic rigidity sometimes produced at later periods of existence.
Dugès, Cruveilhier, Smellie, Davis, Evory Kennedy, Doherty, Weber and Hecker, who have described the condition of stillborn children, suspended animation, asphyxia neonatorum and apoplexy of new-born children, are almost entirely silent respecting the after consequences to the infant, when not fatal. The first named is the only one who distinctly enunciates that hemiplegia and idiocy may follow injury received at birth. The others seem quite unaware that abnormal parturition, besides ending in death or recovery, not infrequently has another termination, i.e. in the language of medical writers, has a third termination "in other diseases." My friends, Drs. West, Tyler Smith, and Barnes, have informed me that instances of such a termination of abnormal labour have not fallen under their notice. Dr. Ramsbotham says he can remember two instances. It is obvious that the great majority of apparently stillborn infants, whose lives are saved by the attendant accoucheur, recover unharmed from that condition. I have, however, witnessed so many cases of deformity, mental and physical, traceable to causes operative at birth, that I consider the subject worthy the notice of the Obstetrical Society. In orthopaedic practice alone, during about twenty years, I have met with probably two hundred cases of spastic rigidity from this cause. I omit reckoning the subjects of idiot and other asylums, in which probably such cases abound, but of which I have been able to obtain no history. I revert to the subject at the present moment because I believe I am now enabled to form an opinion of the nature of the anatomical lesions and the particular abnormal event at birth on which the symptoms depend. Moreover, as the study of the proximate cause of the affections which I shall describe requires the light of such facts as the members of this Society have peculiar opportunities of supplying, I shall make no further apology for occupying the Society's time.
Before I describe the mental and physical derangements of the infant which can be referred to the effects of abnormal parturition and asphyxia at birth, I may be permitted to dwell upon the physical phenomena which occur in the foetal organism immediately before, during, and immediately after, the act of normal parturition.
The foetus, during the long nine months of its abode in the uterus, has been justly regarded as being plunged into a deep sleep, giving no signs of existence as a semi-independent organism but by an occasional strictly reflex or convulsive moment of its limbs, and the pulsation of its heart during the later months of gestation. The materials for its nutrition, oxygenation of its blood, and the carrying off of the carbon, and probably of other residues of the metamorphosis of its tissues, being provided for by the intercourse which takes place between its blood and that of the mother at the placenta. With the commencement of normal parturition the long repose of the foetus is broken in upon. The foetus, during the uterine contractions, especially after evacuation of liquor amnii, is subjected, together with the placenta and umbilical cord, to a gradually increasing amount of pressure, through which it may reasonably be conjectured that the circulatory system, and consequently the capillary system, as of the lungs and nervous centres, are gradually prepared for the altered offices which are about to devolve upon them. This pressure is at first intermittent, the duration of the period of repose at first greatly exceeding the period of disturbance; as the final exit approaches, the pressure simply remits, until at length it is so considerable that prompt escape from the mother alone prevents mischievous results to the nascent organism. During the uterine contractions a certain amount of impediment to placental respiration or to placental interchange of material is unavoidable, so much of uncarbonized or deteriorated blood is contained in the foetal tissues -- among other tissues, in those of the excitor of respiratory acts, the medulla oblongata -- as suffices to give notice to the medulla oblongata of the need of inspiratory movements and of the admission of air into the lungs. Hence is explained the first-observed phenomenon of normal, independent, extra-uterine existence, the effectual act of inspiration, accompanied with the welcomed, characteristic, expressive cry of the new-born child. The normal impediment to placental interchange reaches its maximum at the moment of birth. Should any departure from the normal act of birth take place, should the act of normal respiration not be established at the moment of birth, the child presents itself in a state either of manifest death (still-born), apparently still-born, or in a state of more or less suspended animation, and does not utter the characteristic expressive cry of the new-born child.
The new-born child that has not yet attained to thoroughly independent existence tolerates a longer duration of suspended animation than the child in which pulmonary respiration has been thoroughly established or than the adult; yet reflection on the nature of a delay of only a few moments in the substitution of pulmonary for the ceased placental respiration would lead to the apprehension that even the want of a few breathings, if not fatal to the economy, may imprint a lasting injury upon it. The observations I have recorded of the direct connection between suspended animation at birth and mental and physical impairment of the individual, prove that the proportion of entire recoveries from the effects of asphyxia neonatorum is smaller than has hitherto been supposed.
It will be acknowledged that the state of things in the foetus at the moment of birth, at the moment of entire withdrawal of placental or maternal circulatory influence, is one of imminent failure in decarbonization of the blood. If pulmonary respiration be not immediately established, the state of suspended animation -- asphyxia neonatorum -- takes place. From analogy with other forms of suffocation in later life, as from drowning, when the air-passages are suddenly and forcibly obstructed, suffocation also from inhalation of certain gases which exclude oxygen from the lungs, we may infer that the want of respiration in the new-born child is followed by stagnation of blood in all the large venous channels. We may direct our thoughts to the necessary consequences of blood stagnation in the sinuses of the brain, the venous plexuses surrounding the spinal cord, the venae cavae, the right side of the heart, and the pulmonary system. We can apprehend the inevitable congestions of the capillary system of the brain and spinal cord, and a prompt result in death, if the mischievous circle of affairs is not relieved by suitable respiration.
The forms of abnormal parturition which I have observed to precede certain mental and physical derangements of the infant consisted of difficult labours, i.e. unnatural presentations, tedious labours from rigidity of maternal passages or apertures, instrumental labours, labours in which turning was had recourse too, breech presentations, premature labours, and cases in which the umbilical cord had been entangled around the infant's neck or had fallen down before the head. To these abnormal forms of labour I believe cases LII and LIII justify me in adding labours in which, from want of due attention immediately after birth or after expulsion from the mother, the child has been partially suffocated in the maternal secretions or under her clothes.
Doubtless in some of the instances I have recorded sufficient mechanical injury to head and neck was inflicted to account for whatever unfavourable consequences, whether these were fatal or not, may have ensued, but the more the facts I shall adduce are studied the more apparent, in my opinion, it will be that a larger proportion of infants, either dead, stillborn, apopleptic, or asphyxiated at birth, have been rendered so by interruption of the proper placental relation of the foetus to the mother, and non-substitution of pulmonary respiration, than from direct mechanical injury to the brain and spinal cord.
Until quite recently the morbid anatomy of children dead at birth or shortly afterwards had been little recorded. Jadelot ('Traité des Maladies des Enfants de Michael Underwood', 1823, p. 67) says -- "We never find effusion of blood, but only very considerable engorgement." Cruveilhier ('Anat. Pathol. sur l'Apoplexie des Nouveaux-nés') and Dr. Evory Kennedy ("On Cerebral Apoplexy of New-born Infants," 'Dublin Journal,' vol. x, p. 425) agree that effusion of blood takes place commonly on the surface or base of brain, never into the substance itself, and that in the majority even of fatal cases only intense turgescence of sinuses and veins, with extreme congestion of the capillary system, are found. Cruveilhier found in all cases the dura mater of the spinal canal distended with fluid blood. The fullest contributions to the morbid anatomy of stillborn children have recently been made by C. Hecker, of Berlin, and F. Weber, of Kiel. Hecker ('Verhandlungen der Gesellshaft von Geburtskunde,' Berlin, 1853), after quoting Schmidt, Ritgen, Litzmann, and Krahmer, to the effect that children dying at birth or subsequently asphyxiated present numerous dotted, petechiae-like ecchymoses on the surface of the lungs and diaphragm, gives a large number of his own dissections, proving beyond a doubt that punctiform ecchymoses are present, as a rule, on the serous surfaces of chest and abdomen, sometimes on the skin, besides intense congestion of viscera of chest and abdomen, blood extravasations between pericranium and cranium, the vessels and sinuses of brain gorged with blood, in children born dead, whether from interruption of placental or insufficient pulmonary respiration, caused by pressure on umbilical cord, premature separation of placenta, and uterine hemorrhage. Hecker also found several times in prematurely born children, who had lived a longer or shorter time after birth, similar ecchymoses on surfaces of lungs and heart.
F. Weber ('Beiträge zur Pathologischen Anatomie der Neugebornen,' Kiel, 1851-54) found laceration of dura mater and effusion of blood between it and the bones, rupture of longitudinal and transverse sinuses of brain and considerable hemorrhage on the surface and base of brain, sometimes sufficient to envelop cerebellum and oblongata in cases in which mechanical injury to bones of the head had occurred, whether or no instruments had been used to complete the delivery. But Weber found pretty generally the same tendency to punctated, capillary apoplexies, especially on the serous membranes of lungs, heart, brain, and spinal cord, as were first described by Hecker as a cause of death of newborn infants. The class of lesser injuries uniformly met with in death of child from abnormal labour was, according to Weber, great congestion of all the large veins and sinuses, intense congestion of surfaces of brain and spinal cord. In the spinal cord the small extravasations of blood and the congestion of pia mater were always greatest in the cervical and lumbar portions. Weber rationally accounts for the comparative unfrequency of capillary apoplexy of the brain in these dissections, on the ground that capillary apoplexy is usually recovered from, whilst extravasated blood oftener kills. The sequel will show that capillary apoplexies of the nervous centres are probably the cause of, at least, one form of persistent deformity of limbs -- general spastic rigidity, and that although capillary apoplexy may not commonly destroy life, its consequences seriously impair the organism.
Weber describes an autopsy similar to some of those of Hecker, in which the results of great congestion, apoplexy of the pleura, congestion of brain, bloody serum in ventricles, the cerebellum and medulla oblongata swimming in it, in a case of death through descent of umbilical cord, no pelvic obstruction having existed.
The detailed autopsies of Hecker and Weber, with the carefully appended histories of the nature of the fatal impediment at birth, have greatly facilitated an explanation of the spastic rigidity and paralysis of limbs, which appeared from my observations to be produced by so many different forms of unnatural parturition. The dissections of these obstetricians show the important fact that mechanical injury of the foetal head, neck, or trunk, is not necessary for the production of intense congestion and blood extravasation of serous surfaces of chest, brain, and spinal cord. The other phenomenon commonly observed in difficult and abnormal parturition is that of interruption of placental respiration and circulation with non-substitution of pulmonary breathing and circulation. To this phenomenon alone, when mechanical injury or impediment has not existed, can we attribute the internal congestions, capillary extravasations, serous effusions with correspond with, or produce the symptoms of asphyxia, suspended animation, apoplexy, torpidity, tetanic spasms, convulsions of new-born children, and the spastic rigidity, paralysis, and idiocy subsequently witnessed. I am justified in regarding the dissections of Hecker and Weber as confirmatory of the opinion emitted by me, that asphyxia neonatorum, through resulting injury to nervous centres, is the cause of the commonest contractions that originate at the moment of birth, namely, more or less general spastic rigidity, and sometimes of paralytic contraction.
The former class of affections may be described as impairment of volition, with tonic rigidity and ultimately structural shortening, in varying degrees, of a few or many of the muscles of the body. Both lower extremities are more or less generally involved. (See plate.) Sometimes the affection of one limb only is observed by the parent, but examination usually shows a smaller degree of affection in the limb supposed to be sound. The contraction in the hips, knees, and ankles, is often considerable. The flexors and adductors of thighs, the flexors of knees, and the gastrocnemii, preponderate. In most cases, after a time, owing to structural shortening of the limbs and of the articular ligaments, and perhaps to some form of change of articular surfaces, the thighs cannot be completely abducted or extended, the knees cannot be straightened, nor can the heels be properly applied to the ground. The upper extremities are sometimes held down by preponderating action of pectorals, teres major and teres minor, and latissimus dorsi; the elbows are semi-flexed, the wrists partially flexed, pronated, and the fingers incapable of perfect voluntary direction. Sometimes the upper extremities appear unaffected with spasm or want of volition, sometimes a mere awkwardness in using them exists. Not infrequently the parent reports that the hands were formerly affected. Participation of the muscles of trunk is sometimes shown by the shortened, flattened aspect of pectoral and abdominal surfaces, as compared with the more elongated and rounded form of the back. The prominence of back partially disappears on recumbency, but the greater weakness of muscles on dorsal aspect of trunk is obvious when the individual again attempts to sit upright. The muscles feel harder than natural to the age. Micturition is sometimes observed to be rare, and the bowels usually confined, either from deficient exercise of voluntary expulsive power or from implication of the sphincters. The muscles of speech are commonly involved, varying in degree from inability to utter correctly particular letters up to entire loss of articulating power. Sometimes articulation is only slow and difficult, like other acts of volition, the child or adult reminding us of a tardigrade animal. Sometimes the speech is nervous, impulsive, or stuttering. Often during the earliest months of life deglutition is impaired, and the power of carrying saliva into the fauces is not acquired until late. The intellectual functions are sometimes quite unaffected, but in the majority of cases the intellect suffers -- from the slightest impairment which the parent unwilling acknowledges or fails to perceive up to complete imbecility. The functions of organic life are unquestionably performed, except, perhaps, that of development of caloric, although the depression of temperature in later life is probably dependent more upon the want of proper exercise. The frame is often lean and wiry, but not wasted. On the contrary, it is generally well nourished. The appetite is good, the child is often described as the healthiest of the family. These subjects often lead a more precarious existence during the first weeks after birth, at first even vegetative existence languishes, sometimes, perhaps, because premature birth or difficult labour, by impairing the maternal supply of nutriment, renders more difficult the infant's recovery from the shock its system received at birth. However, in the majority of instances, after restoration of the vegetative functions, a gradual but slow amelioration of all the functions of animal life is perceived. Some cases present distinct convulsive twitchings of face or limbs during first days after birth, open or suppressed convusions, opisthotonos, or laryngismus. In some instances the persistent rigidity of muscles commences or is observed shortly after birth, in others it escapes observation until the lapse of some weeks or months. The child's limbs are sometimes reported to have been simply weaker, to have shared in the general debility, the question of viability having alone occupied the attention of the attendants during the first month. Occasionally the weakness of the limbs has been recognised as a genuine paralysis in the first instance, of which the rigidity of muscles has been the sequel. Before the age of three or four months, though sometimes in slight cases not until ordinary time for walking arrives, the nurse perceives that the infant never thoroughly straightens the knees, that these cannot be properly depressed or separated, that she is unable to wash and dress the infant with ordinary facility, that the hands are not properly used. The upper extremities recover before the lower limbs. Sometimes the trunk is habitually stiffened, so that the infant is turned over in the lap "all of a piece," as the nurse expresses it. Occasionally the head is habitually retracted. Where the symptom of convulsions or "inward convulsions" exists, the rigidity is attributed to the convulsions. In many cases convulsions have been absent. As the child approaches the period at which the first attempts at standing and progression should be made, it is observed to make no use of the limbs, or he is incapable of standing except on the toes, or the feet are disposed to cross each other. Even children slightly affected rarely "go alone" before three or four years of age, many are unable to raise themselves from the ground at that age, and others do not walk, even indifferently, at puberty. On examination, the surgeon finds that the soles of the feet are not properly applied to the ground, that the knees always incline inwardly, and continue bent. When locomotion is accomplished, the movements are characterised by inability to stand still and balance the body in erect attitude. In the best recoveries from general spastic rigidity, even in the adult, the gait is shuffling, stiff; each knee, by forcible spastic rubbing against its fellow, obstructs progression.
The external form of the cranium occasionally exhibits departure from the normal or average type, such as general smallness of skull, depression of frontal or occipital region only, sometimes one lateral half of skull, sometimes of one half of occiput, or forehead only. In slight cases the head has been well developed.
In cases with even great inertia as to exercise of volition in any part of the body, common sensibility appears little, if at all, deficient. The child often, indeed, manifests uncommon sensitiveness to external impressions, even when approaching adolescence he is alarmed at trifling noises. The sleep after the first weeks of life is light, easily disturbed. Often there is extreme sensitivity to touch, the whole condition reminding the observer of tetanus. In a few cases a distinct resemblance to severe chorea is perceptible. It is probably that some of the cases designated by authors congenital chorea have been cases of the affection I have described.
Amongst the more uncommon consequences of difficult or premature labour and asphyxia, I may refer to Cases XLVIII and XLIX, in which wry-neck apparently resulted from one or another of these causes, and Cases VII, X, XX, and others, in which a distinct hemiplegic contraction resulted. I have occasionally met with the slightest amount of single spastic talipes equinus referable to this cause. Such a case has commonly been attributed to dentition, a fit or illness during infancy, the first link in the pathological chain of nervous susceptibility caused by the asphyxia having been disregarded or overlooked.
A survey of the history of forty-seven cases, appended, shows that one fact is common to all the cases of persistent spastic rigidity of new-born children, namely, that some abnormal circumstance attended the act of parturition, or rather, the several processes concerned in separating the foetus from the parent and its establishment in the world as an independent being. I cannot recall positively to mind, or find recorded in my journals, more than a single case in which this persistent spastic rigidity affected a numerous series of muscles of the trunk and extremities which could be unequivocally referred to any illness subsequent to the establishment of proper pulmonary respiration as its starting-point. Often it has been found that convulsions in infancy had occurred, to which the disease had been attributed. Spastic contraction of a single set of muscles, as the gastrocnemii of one or both limbs, commonly of one limb only, or of the muscles of the forearm and calf on one side, is certainly an everyday occurrence after infantile convulsions, convulsions during dentition, and during early childhood without convulsions or other marked illnesses. But general spastic rigidity I have, with one exception, found to have been preceded by some abnormal act connected with mode of birth. Occasionally several causes, either of which may be competent to produce cerebro-spinal disorder and deformity, may exist. Thus, in Case XLIII uterine haemorrhage occurred two months before labour; labour was tedious, accoucheur was absent at birth, the child was born with navel-string around neck and legs, and did not cry for an hour afterwards, and a large, hard, substance, as large as another child -- possibly a blasted twin conception -- was discharged with the afterbirth. I may remark that asphyxia neonatorum, from whatever amount of disturbance in separation of foetus from the uterus it may have resulted, is, as might be surmised, very apt to be accompanied with, and to be succeeded by, convulsions at variable periods after birth. It will be borne in mind that convulsions at birth, or subsequently to it, are but a symptom of lesion of nervous centre, and that we cannot refer one symptom of disorder of the nervous system to another symptom of the kind. The convulsions may doubtless react upon the nervous centres, upon the lungs and heart, and probably aggravate the disorder. North ('Practical Observations on the Convulsions of Infants,' 1826, p. 52) -- says, "It cannot be doubted that convulsions occasionally arise from excessive and long-continued pressure of the head during protracted labour ... They generally pass off in a very short time after birth." But he adds, speaking of asphyxia, "If such an infant only partially recover, and convulsions succeed, the death of the child almost inevitably follows." Cases II, IV, XVII, XXII, XXXIV, and XLVI, in Appendix, are instances of recovery from such convulsions, and of production of subsequent spastic rigidity. North quotes Dr. Clark, to the effect that such children, if saved from immediate death, are liable to die suddenly in a fit of convulsions. I have witnessed several confirmations of this statement. Case III is an example of the kind. Baume ('Convulsions dans l'Enfance,', 1805, p. 69) makes a similar observation; and Smellie ('Midwifery,' 1772, vol. i., p. 230) alludes to convulsions before or soon after delivery from compression of head, to the danger, and ofttimes the destruction, of the child. Billard ('Diseases of Children,' translation of third French edition, 1839, p. 472), speaking of the morbid anatomy of diseases of cerebro-spinal apparatus developed after birth, says -- "The length of the labour, the necessary tractions in certain manoeuvres, the difficulty with which respiration is established, the changes which the circulation undegoes, explain why the cerebro-spinal system is so often the seat of sanguineous congestions, varying from simple injection of the meninges to true apoplexy." At page 477 he remarks that in two thirds of the cases of convulsions in new-born children, examined post mortem, spinal meningitis was found; myelitis was less frequently met with. Billard was not aware that cerebro-spinal congestions and apoplexy occur in infants where the labour had not been difficult, tedious, or involved mechanical aid -- after descent of and pressure upon umbilical cord only, for example. The Appendix shows that Brachet ('Traité des Convulsions des Enfans,' 1837, p. 97) is too absolute in the statement that "all the infants who are born after difficult and protracted labours are all, without exception, doomed to frequent convulsions."
It is impossible not to connect the persistent affections of the intellect, of volition, and of organic life, with the injury the several nervous centres suffered in some instances before the foetus had reached the maternal pelvis, in other whilst in transit through it; and in a third set of cases, where the foetus was exposed to neither of these kinds of injury, it suffered from asphyxia neonatorum, suspended animation, and its concomitant congestions, effusions, capillary apoplexies of brain, medulla oblongata, and spinal cord. Hitherto I have been afforded only one opportunity of learning the post-mortem condition of any of the cases of spastic rigidity which I have referred to asphyxia at birth, viz., Case LX, kindly furnished by my colleague, Dr. Down. It is certain that if, examined after death, after living many years, and such cases I find may live at least past the meridian of life, an anatomical condition very different from that present at or soon after birth may be found. Without going so far as Weber, as to assert that capillary apoplexies are necessarily absorbed when immediate death does not result from them, we may conclude that although the effused blood-particles may be absorbed, permanent lesion -- atrophy of the nervous tissue -- results (see Case LX). Possibly a state of chronic meningitis, with effusion, or of chronic meningeal hyperaemia or congestion, or a certain amount of chronic myelitis, may maintain the spastic excitable tetanoid, sometimes choreal contractions, with rigidity of the trunk and extremities. My experience as Physician to the London Hospital has afforded me some facts which support the idea that spinal meningitic and myelitic affections may play a considerable part in the phenomena of spastic rigidity. Thus the only case of persistent general spastic rigidity of upper and lower extremities, commencing after adult age, which I had the opportunity of seeing at intervals during twenty years, and the general appearance of which appeared to me similar in many respects to spastic rigidity from asphyxia neonatorum, was found by me after death to have depended upon chronic spinal meningitis and myelitis. A case related by Cruveilhier, of pus found in medulla spinalis, in a case of death of infant on the fifth day, after difficult labour, supports this view.
The greater or smaller impairment of intellect may safely be attributed to the greater or less mischief inflicted upon the cerebrum. As already observed, the considerable extravasations of blood on the surface of the brain are usually fatal. The autopsy, Case LX, showing cicatrized apoplexies on surface and interior of brain, is an exception. The only fatal instance of partially stillborn infant, which I have had the opportunity of post-mortem examination, was one which came rapidly into the world, preceded by uterine haemorrhage, nearly at full time, owing to fright to which the mother was exposed. Death of child ensued seventy hours after birth. In this case considerable effusion of blood was discovered in both ventricles of brain -- a true apoplexy in the new-born child without mechanical injury. The autopsy, Case XLI, illustrates congestive apoplexy, no pelvic obstruction having existed.
I formerly found much difficulty in the analysis of various symptoms met with in different cases of spastic rigidity traceable to something abnormal in the act of birth. It soon became apparent that the symptoms, of the living at least, attributed to mechanical injury of the head were a minority of the whole. This is consistent with the remark of Ollivier ('Traité sur les Maladies de la Moelle Epinière,' vol. i, p. 152), that whilst at natural birth the spinal cord is perfectly developed, the brain is still in a very rudimentary state, and consequently able to bear considerable disturbance without ultimate injury to its functions. In fact, in the new-born child brain-life is entirely absent; any injury it may have received at birth is at that period unaccompanied with special brain-symptoms, and, if not too severe, the child may entirely recover. Ollivier says (p. 244) "the brain of the new-born child is often found softened and destroyed without any external sign having permitted the practitioner to suspect it during life." In the present day, with the experience we now possess of the causes of death at or shortly after birth, the accoucheur will suspect the existence of some form of apoplexy in every case.
The severe lesions caused by mechanical compression and laceration, and extensive haemorrhages within the skull, when they do not destroy life, give rise to permanent deformity of cranium, to atrophy of injured portions of brain, and are the cause of many cases erroneously described as congenital idiocy. Dr. J. Crichton Browne ("Psychical Diseases of Early Life," 'Journal of Mental Science,' April, 1860) is one of the few observers who have traced idiocy to difficult labours (see also Dr. Howe, 'Causes of Idiocy,' Edinburgh, 1858). But in addition to the undoubted instances in which cranial injury and some imperfect development of intellect stand in the relation of cause and effect, the Appendix shows impaired intellect in Cases IV and VIII, in which no mechanical injury had taken place, but in which suspended animation, asphyxia neonatorum, and probably its consequent general and capillary congestion and ecchymoses -- capillary apoplexies of the brain as well as of the spinal cord -- perhaps even a moderate amount of larger apoplectic extravasation, had taken place, and had been imperfectly recovered from. I have observed that in impaired intellect from abnormal birth the degree of impairment met with in private practice often does not exceed feebleness of intellect; it varies much in degree, as elsewhere mentioned; it is often not sufficient to exclude the individual from family society. The individual may acquire a fair knowledge of music, the memory is good, the constructive tendency may exist, a fair capacity for arithmetic and languages may be displayed, but there commonly exists a great want of application, a slowness of intellect similar to the slowness of volition. In other cases, where intellectual powers are good, a preternatural impulsive nervous condition of mind exists, combined with an agitated, eager, anxious mode of performing acts of volition. Making every allowance for family peculiarities, there undoubtedly exists a considerable pathological resemblance, even in intellectual character and physiognomical expression, in these subjects of more or less general spastic rigidity. The occurrence of this feeble intellect in those who have not been exposed to mechanical injury of head, but in whom premature birth or pressure on umbilical cord has been recorded, appears explicable only on the supposition that the asphyxia and feebleness at birth had been followed by the usual capillary or larger haemorrhage or effusions in brain, and their transformations and consequences to the nervous tissue; and the degree and variety of impaired function of brain may be due to the degree and variety of situation of these haemorrhages.
The affections of the functions of organic life, the protracted inability to suck and swallow in a natural way, often observed during the first few weeks of life, the liability to "choking noises in the throat" and other signs of what may be classed under the name laryngismus stridulus, and the affections of speech dependent upon impaired innervation of glottis, pharynx, tongue, and lips, and consequent arrested development of some of these parts (larynx), may be referred to injury at base of brain and medulla oblongata. An occasional choke and gasp for breath, succeeded by a sign, was described by the nurse of Miss N. (Case XXX) as having continued to the date of the report, when the patient was twelve years old. Occasionally the injury to base of brain or medulla oblongata may have resulted from mechanical displacement of part of the occipital bone, as in some cases described by Dr. J. Marion Sims ('American Journal of Medical Science,' April, 1846) under the title of Tetanus of Newborn Children; in Case LV of my own in Appendix the injury was consequent upon violent traction exercised to extricate head in breech presentation. But as in the explanation I have given of the causes of impaired intellect in relation to supposed injury to brain at birth, so I can show by reference to Cases IV, VI, XI, XII, and others, in Appendix, that mechanical injury to base of cranium and neck in those who survive is only exceptionally the cause of difficult deglutition, respiration, and speech, but that these important symptoms occur in practice oftenest, in cases when suspended animation or asphyxia at birth took place without previous violence to head and neck rendering it probable that capillary apoplexy, serous or sanguineous effusion towards base of brain and in around medulla oblongata, resulted from the general blood-stasis accompanying the asphyxia. Joerg ('Kinderkrankheiten,' p. 387) says that immediate death, although the heart continues to pulsate for several minutes after birth, follows mechanical injury, such as stretching or twisting, of cervical vertebrae. Case LV, with which I was favoured by Dr. M'Intyre, of Odiham, and probably Cases XLVIII, by Mr. Brown, of Camberwell, and XLIX, show that children recover from the immediate consequences of considerable injury in this situation. Dr. Marion Sims (opus cit.) describes, under the head of Trismus nascentium, well-marked cases of spastic rigidity of new-born children. One case was that of a negro, a twin, the second born; labour was tedious, the child still-born, several minutes having elapsed before respiration was established. Tetanic symptoms were discovered on the sixth day, succeeded by death in ninety-six hours. At the autopsy "coagulum of blood was found occupying the whole length of spine, perfectly enveloping the medulla spinalis, thicker as it approached the brain. Spinal veins full of black blood." Dr. Sims attaches no importance to the tedious labour nor to the asphyxia at birth. Of six cases of trismus of new-born children reported by him incidental mention is made of two of them having ensued after difficult labours. In some later cases of trismus nascentium published by Dr. Sims (opus cit., 1848), either inability to suck or stridulous breathing were observed soon after birth. It appears probable that the usually fatal disorder denominated trismus nascentium is often induced by the same causes -- asphyxia at birth, and when recovered from has constituted the early stage of the condition which I have so often met with in older children, and have denominated spastic rigidity from asphyxia at birth. Abercrombie ('Diseases of Children,' sect. iv, Case 150) describes a case of spinal apoplexy of an infant who had been unable to suck and died with trismus and convulsions on the eleventh day. At Case 147 he speaks of hematorachis causing tetanus of new-born child. Weber (opus cit.), in death from trismus nascentium, always found the principle morbid appearances in spinal cord. Dr. Evory Kennedy (On Cerebral Apoplexy of New-born Infants, 'Dublin Journal,' vol. x, p. 429) relates a case of an infant which, after protracted birth and difficulty in establishment of its respiration, was seized on the second day after birth with general convulsions, hands clenched, screaming, abdominal muscles tense, respiration diaphragmatic, death on the third day. At the autopsy, the vessels on hemispheres were much loaded, serous fluid abounded in spinal canal. The veins and membranes of medulla oblongata were excessively turgid and congested. Among some interesting cases reported by Dr. Doherty ('Dublin Journal,' vol. xxv) of asphyxia of new-born children (a title to which he objects because the individuals have never breathed), are several which I recognise as belonging to the more numerous class of recovered asphyxia cases which present themselves in later life. Thus Dr. Doherty's Case 19, asphyxia of two hours' duration, resulted from prolapse of funis. Fifteen hours after birth convulsions set in; death on the fifth day. At autopsy -- infiltration of blood into cellular tissue about dura mater of the cord, dura mater congested, vessels in spinal canal gorged, serous effusion in theca; sinuses of brain distended. Blood between dura mater and parietal bones. Dr. Doherty relates another case (Case 4) in which labour lasted three hours, funis expelled before head. Asphyxia reported, "followed by general tendency to spasm," said to have gradually recovered. The case, however, reappears as Case 28 a year afterwards, the child "never having been able since birth to hold up head." Dr. Doherty, however, doubts whether the symptoms were connected with the original transient apoplexy, as he properly designates the primary state. It was doubtless a case of asphyxia from descent of cord before head, accompanied with capillary or more extensive apoplexy or other effusion in nervous centres, followed by debility, paralysis, and spastic rigidity, similar to several of the cases I have appended. (Cases XIX, XLIII, XLV.)
Brachet, the author of the most complete work on the convulsions of children, relates a case of what he designates hereditary convulsions, overlooking the fact, which he incidentally mentions, that the child was semi-asphyxiated for half an hour after birth. He adds (p. 102), "I confess I could find no exciting cause of the convulsions unless it were that M. Montain, the accoucheur, had been obliged to give the child some slaps on the buttocks to recall it to life." He adds, "Quelque peu de confiance que j'ajoute à ce cause, elle est la seule probable, surtout chez un infant qui y etait disposé par sa constitution." Brachet also relates (p. 106) the case of a female child coming into the world after the mother had suffered two or three frights. The child's weakness was so great that the child did not cry for a fortnight, and swallowed with difficulty. This was succeeded by "convulsions neophytes de sauvages." She recovered, but he says, "pour la moindre cause elle tressaille et parait menacée de convulsions."
It will be remembered that early in this paper I described this great susceptibility to impressions, almost tetanic, as a common accompaniment of spastic rigidity from asphyxia neonatorum.
Reference to more than fifty cases of injury of mind or body from abnormal parturition which are appended, will show that whilst in many cases the subsequent symptoms indicated that the brain and medulla oblongata had permanently suffered, the only one of the nervous centres which invariably presented symptoms of lesion was the medulla spinalis.
If -- from analogy with the contractions of limbs observed to follow well-known diseases of spinal cord in later life, and from the fact of capillary apoplexy, larger blood-extravasations, and serous effusions being met with after death in spinal cord of infants who have died still-born from premature birth, descent of funis before head, &c., without mechanical injury to head and neck -- I am justified in referring the spastic rigidity which follows asphyxia at birth to lesion of spinal cord, and not to lesion of brain or medulla oblongata, that from some cause this nervous centre suffers most often from the asphyxia, or least frequently recovers its integrity. It seems almost superfluous to add, as a further proof of non-dependence of spastic rigidity of limbs upon mechanical injury at birth, that the lower extremities are oftenest affected and are the slowest to recover, although they derive their nerve-power from the lower part of the spinal column, which is assuredly the part of the cerebro-spinal axis least obnoxious to mechanical injury.
When we consider the intimate pathological connection between spasm and paralysis it is remarkable that these cases of spastic rigidity from asphyxia at birth do not offer a decided combination of spasm and paralysis, such as is observed after ordinary cerebro-spinal disease in childhood. It is common, after such diseases, to find a child with one limb affected with paralysis or paralytic contraction and the opposite limb with spasmodic contraction.
As additional evidence of the dependence of the several states of brain, medulla oblongata, and medulla spinalis upon the asphyxia which so often attends abnormal parturition, I may recall to mind that recovery from asphyxia from choke-damp, asphyxia from suspension, are apt to be followed by cerebro-spinal disease; and I may add that at several autopsies after the asphyxia of Asian cholera, I have witnessed small blood-extravasations on serous surfaces of lungs and heart. Experiments of submersion of animals show internal congestion and ecchymoses of serous surfaces as a consequence of that form of suffocation.
Joerg ('Kinderkrankheiten,' 1828. pp. 402-438) is the only author I have met with who distinctly enunciates that too early and unripe-born foetuses present a state of weakness, persisting in the muscles until puberty or later. He says it interrupts use of muscles during first and second periods of life, as well in limbs as in carriage of head and trunk, often thereby causing curvatures of the spine and legs. Ollivier was aware of the liability of the spinal cord to suffer after difficult labour, for he says (opus cit., p. 240), "The greater influence of the spinal cord at birth appears to continue during the first period of extra-uterine existence, for affections of the spinal cord and its membranes sont assez communs dans les enfans naissans." Ollivier also distinctly attributes the marked injection of membranes of spinal cord in new-born children examined by him to the embarrassment which respiration and circulation undergo at this period of life.
It will be observed that I have in this paper often employed the term asphyxia neonatorum nosologically, in its widest sense, embracing in it all the conditions of suspended animation in the new-born infant which have for their result to prevent the immediate establishment of proper respiration and circulation, whether or no the colour of the infant be pale or dark. It is probably, from analogy with the asphyxias of later life, that the dark colour of the surface is a measure of the embarrassment of the pulmonic and cardiac functions, the pallor indicating greater prostration and greater tendency to cessation of nerve-life and death. D. D. Davis ('Principles and Practice of Obstetric Medicine,' vol. ii, p. 1212) endeavours in vain to establish a difference in the aspect of the infant, according to whether the state of suspended animation arises from asphyxia, asthenia, or apoplexy. C. A. Struve ('On Physical Education of Children,' translated by Willich, 1800) makes two kinds of apparently still-born, the adynamic and apopleptic. Joerg (opus cit., p. 402) recognizes that the suspended animation is sometimes composed of two states, injury (mechanical) to head and want of air. It is apparent that the phenomena of suspended animation of infants after birth will permit of more extended observation by the members of the Obstetrical Society. Davis's view, that three states occur, asphyxia, asthenia, and apoplexy, is doubtless correct. It is, however, evident from that which I have already stated, that it is not yet possible before death to point out upon which of these three conditions the suspended animation depends. The want of breathing is manifest in all cases, asthenia may be present as a complication, and if the suspended animation terminates in death or in cerebro-spinal disorder, we may infer the existence of sanguineous congestions and apoplexy in the nervous centres.
This is the class of cases which, during the "Sensation" times of the promulgation of subcutaneous tenotomy, furnished opportunity to an able French orthopaedic surgeon triumphantly to divide sixty or more muscles at one sitting. Happily, Stromeyer's operation of subcutaneous tenotomy rested upon a more secure foundation than could be overthrown by so great an abuse of it.
I trust the views of the pathology of the lesions of mind referable to the influence of the act of birth upon the child, which I hope to have somewhat unravelled, will promote the beneficial treatment of the disorders when detected in the early stages. In the later stages, the general principles of orthopaedy, and mental training when the intellect is affected, are successfully applicable in the inverse proportion to the extent of the permanent disorganization of the nervous centres and of peripheral structures. The length to which this paper has already extended prevents my dwelling upon the subject of treatment. I have had many of these cases under observation from one to twenty years, and may mention as an encouragement to other practitioners that treatment based upon physiology and rational therapeutics effects an amelioration surprising to those who have not watched such cases. Many of the most helpless have been restored to considerable activity and enjoyment of life. Even cases which exhibit impaired intellect may be benefited in mind and body to an unexpected extent.
When we reflect on the frequency of pulmonary engorgement and ecchymosis as well in the interior of the lungs as upon their surface, also the distension of venae cavae, the right side of heart, and the ecchymoses on pericardium, in the bodies of still-born children, it will appear not improbable that since one of the members of the tripod of life -- the cerebro-spinal system -- manifests defects in after-life referable to injury received at birth or to asphyxia neonatorum, in like manner partially still-born infants who recover with atelectasia pulmonum or with strained and injured hearts, may in after-life present anomalous affections of, or be prone to, pulmonary or cardiac disorder.
I would therefore suggest for inquiry, whether, for example, some cases of "congenital" cyanosis may not be induced at birth through impediment at this period to the normal substitution of infantile for the foetal circulatory route, causing, for example, interruption of development and non-closure of foramen ovale. I am indebted to Mr. Curling for the following case. A youth, aet. 12 years, the second child born of parents not liable to asthmatic or other pulmonary complaints, did not cry immediately at birth, but received several vigorous slaps from the hand of the accoucheur before respiration was established. The child has from an early period of infancy been subject to considerable difficulty of breathing, and to attacks of acute dyspnoea on slightest cause. His asthmatic condition has puzzled several distinguished physicians who have seen him. Refuge has been taken in "congenital asthma." May not the starting point of the complaint have been injury to the capillary system or larger vessels of heart and lungs at the moment of birth?
The researches of Weber and Hecker into the morbid anatomy of still-born children testify also that the vascular system of the abdominal viscera undergoes disturbance, causing ecchymosis in those organs from the accidents attending birth.
It is further suggested, therefore, whether some of the ailments in these viscera, occasionally presenting themselves in the earliest periods of life, may not be due to causes similar to those which I have shown undoubtedly influence the cerebro-spinal system and its dependent organs. And lastly, as the general capillary system cannot be independent of that which affects the circulation of the brain, chest, and abdomen, there remains for consideration whether the nutrition and development of the muscles and peripheral nerves are not directly affected, independently of the influence of the nervous centres upon them, by the proved abnormal congestions sometimes accompanying the act of birth.
The first column in Appendix of Cases contains the No. by which the case is alluded to in this communication.
The second column contains the initials of the case, the sex, sometimes the date and name of physician or surgeon with whom the case was seen in consultation, and the number under which the case is recorded in my journal of similar and allied cases.
The third column records the age in years when I first saw the case.
The fourth column contains a literal transcript of the description of the case as entered in my journal at the time the case was first seen.
The fifth column contains the history, mainly in the words of the informant. It is, of course, impossible to vouch for the accuracy of informant as to child being six months' child, &c., to a week or two.
Occasionally the report is brought by author to the present time. This has not generally been done, in order to save space. The complete progress of the case was not required to be shown when treating of pathogeny.
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General spastic contraction of the lower extremities. Premature birth. Asphyxia neonatorum of thirty-six hours duration. Hands unaffected. See Case XLVII. |
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Contracture of adductors and flexors of lower extremities. Left hand weak. Both hands awkward. More paralytic than spastic. Born with navel-string around neck. Asphyxia neonatorum one hour. See Case XLIII. |
Dr. Barnes said, that although not able, from his own observations, to produce any facts in confirmation or negation of Dr. Little's theory, this might be due to his not having studied the subsequent history of children in connexion with the phenomena attending their birth. He was now, however, able to look back upon a considerable number of children who had been born semi-asphyxiated, in consequence of difficulty involving resort to forceps, or turning. Many of these children he knew were healthy, and did not appear to bear any trace of the difficulties that attended their birth. He had, like most obstetricians, observed that occasionally children born with difficulty were liable to convulsions for a short time; but if these survived, they commonly did well. The difficulty there appeared to be in discussing this excellent paper, arose, no doubt, from the entire novelty and originality of the subject. Dr. Little had brought before the obstetric world new matter for inquiry of the highest interest. It was closely related to the question of the causes of still-birth -- a subject, also, of which little was known, at least in this country. One reason was, that there existed no large lying-in hospitals in England, for there could be no doubt that the lying-in hospitals of the Continent lent greater opportunities for investigations of this kind than existed here. Hence Dr. Little has been obliged to look for German authors for information. He (Dr. Barnes), however, hoped that the study of the causes of still-birth would be more closely prosecuted by post-mortem examinations in this country. There was a case of which Dr. Little might be glad to avail himself. It is recorded of Samuel Johnson that "he was born almost dead, and did not cry for some time." The name of Samuel Johnson was almost synonymous with intellectual grandeur, but he was well known to be affected with certain nervous disorders which Dr. Little could better interpret than the speaker.
Dr. Tyler Smith expressed the great obligation of the society to Dr. Little for his valuable paper. There could be no doubt the author had directed attention to an original field of observation in pointing out the injuries to which the nervous system was liable during, and immediately after, birth. Cases of early paralysis and contraction had not fallen under his (Dr. Smith's) observation, but he quite agreed with the possibility of their occurrence from the causes stated. In cases of spasm of the limbs, especially the lower extremities, shortly after birth, he had attributed the condition of the limbs to an excess of the tonic contraction of the muscles natural to the foetus in utero, and which gradually disappeared under the influence of volition and the use of the limbs. He thought dentition the great source of paraplegia and hemiplegia in young children. The irritation of teething sometimes caused paralysis by exciting convulsions, during which the nervous centres were damaged. At other times reflex paraplegia ensued, without fits, during dentition. These forms of disease were very commonly met with, especially in hospital practice, in children from six months to two years of age. The great point was to prevent these seizures by relieving the irritation of dentition, by timely scarification of the gums, and attention to the secretions.
Dr. Gibb said he was reminded of an instance that came under his observation some years ago, but which, perhaps, hardly came within the same category as those described by the author of the paper. After a lingering labour, a child was born with spastic rigidity of all the muscles on one side of the body; in fact, it was an instance of conjunctive hemiplegia. Suspecting that the cause existed in the brain, he was allowed to make an examination of the body of the infant, and found a clot in the substance of the brain on the side opposite to that on which the hemiplegia existed. The vessels generally were very much congested about the head, and, no doubt, had the child lived, it would have remained palsied. The case was recorded at the time in one of the medical journals. ['Lancet,' Nov. 13th, 1858.]
Dr. Little said he quite agreed with the President that the majority of infantile spastic and paralytic contractions arose between the ages of six months and two years from cerebro-spinal disorders, and that, perhaps, for one that depended on abnormal or premature labour there were twenty or more from other causes incidental to later life. He mentioned that, not having found any reference to the affections consequent on abnormal and premature parturition in the works of English medical writers, he had referred, with some confidence, to Shakspeare, to ascertain whether any notations on the subject were contained in his works. He said, the description of the physical character of Richard III was exactly that of an individual afflicted with one kind of deformity originating at birth.
"I that am curtailed of this fair proportion,
Cheated of feature by dissembling Nature,
Deform'd, unfinish'd, sent before my time
Into this breathing world, scarce half made up,
And that so lamely and unfashionable
That dogs bark at me as I halt by them."
In the following lines Shakspeare has used more poetic license. The great dramatist has here probably intensified some popular notions on the subject:
"If ever he have child, abortive be it;
Prodigious and untimely brought to light,
Whose ugly and unnatural aspect
May fright the hopeful mother at the view;
And that be heir to his unhappiness."
He was convinced Shakspeare had drawn the first picture from an individual who had suffered through asphyxia at birth. He, probably, was aware of the fact mentioned by Sir Thomas More, that "the Duchess of Gloster had much ado in her travail, he (Richard III) being born the feet forward."
