Bible Encyclopedias
Monster

Montrosities from Defective Closure in the Middle Line

Under this head come some of the commonest congenital malformations, including slight deficiencies such as harelip, and serious defects such as a gap in the crown of the head with absence of the brain. The embryo is originally a circular flattened disk spread out on one pole of the yolk, and it is formed into a cylindrical body (with four appendages) by the free margins of the disk, or rather its ventral laminae, folding inwards to meet in the middle line and so close in the pelvic, abdominal, thoracic, pharyngeal and oral cavities. Meanwhile, and indeed rather earlier, two longitudinal parallel ridges on the top or along the back of the disk have grown up and united in the middle line to form the second barrel of the body - the neural canal - of small and uniform width in the lower three-fourths or spinal region, but expanding into a wide chamber for the brain. This division into neural (dorsal) and haemal (ventral) canals underlies all vertebrate development. Imperfect closure along either of those embryonic lines of junction may produce various degrees of monstrosity. The simplest and commonest form, hardly to be reckoned in the present category, is harelip with or without cleft palate, which results from defective closure of the ventral laminae at their extreme upper end. Another simple form, but of much more serious import, is a gap left in the neural canal at its lower end; usually the arches of the lumbar vertebrae are deficient, and the fluid that surrounds the spinal cord bulges out in its membranes, producing a soft tumour under the skin at the lower part of the back. This is the condition known as hydrorhachis, depending on the osseous defect known as spina bifida. More rarely the gap in the arches of the vertebrae is in the region of the neck. If it extend all along the back, it will probably involve the skull also. Deficiency of the crown of the head, and in the spine as well, may be not always traceable to want of formative power to close the canal in the middle line; an over-distended condition of the central canal of the cord and brain may prevent the closure of the bones, and ultimately lead to the disruption of the nervous organs themselves; and injuries to the mother, with inflammation set up in the foetus and its appendages, may be the more remote cause. But it is by defect in the middle line that the mischief manifests itself, and it is in that anatomical category that the malformations are included. The osseous deficiency at the crown of the head is usually accompanied by want of the scalp, as well as of the brain and membranes. The bones of the face may be well developed and the features regular, except that the eyeballs bulge forward under the closed lids; but there is an abrupt horizontal line above the orbits where the bones cease, the skin of the brow joining on to a spongy kind of tissue that occupies the sides and floor of the cranium. This is the commonest form of an anencephalous or brainless monster. There are generally mere traces of the brain, although, in some rare and curious instances, the hemispheres are developed in an exposed position on the back of the neck. The cranial nerves are usually perfect, with the exception sometimes of the optic (and retina). Vegetative existence is not impossible, and a brainless monster has been known to survive sixty-five days. The child is usually a very large one.

Closely allied, as we have seen, to the anencephalous condition is the condition of congenital hydrocephalus. The nervous system at its beginning is a neural canal, not only as regards its bony covering, but in its interior; a wide space lined by ciliated epithelium and filled with fluid extends along the axis of the spinal cord, and expands into a series of chambers in the brain. As development proceeds the walls thicken at the expense of the internal spaces, the original tubular or chambered plan of the central nervous system is departed from, and those organs assume the practically solid form in which we familiarly know them. If, however, the spaces persist in their embryonic proportions notwithstanding the thickening of the nervous substance forming their walls, there results an enormous brain which is more than half occupied inside with fluid, contained in spaces that correspond on the whole to the ventricles of the brain as normally bounded. A hydrocephalic foetus may survive its birth, and will be more apt to be affected in its nutrition than in its intelligence. In many cases the hydrocephalic condition does not come on till after the child is born.

Returning to the ventral middle line, there may be defects of closure below the lips and palate, as in the breast-bone (fissure of the sternum), at the navel (the last point to close in any case), and along the middle line of the abdomen generally. The commonest point for a gap in the middle line of the belly is at its lower part, an inch or two above the pubes. At that point in the embryo there issues the allantois, a balloon-like expansion from the ventral cavity, which carries on its outer surface bloodvessels from the embryo to interdigitate with those of the mother on the uterine surface. Having served its temporary purpose of carrying the bloodvessels across a space, the balloon-like allantois collapses, and rolls up into the rounded stem-like umbilical cord through most of its extent; but a portion of the sac within the body of the foetus is retained as the permanent urinary bladder. That economical adaptation of a portion of a vesicular organ, originally formed for purposes of communication between the embryo and the mother, appears to entail sometimes a defect in the wall of the abdomen just above the pubes, and a defect in the anterior wall of the bladder itself. This is the distressing congenital condition of fissure of the urinary bladder, in which its interior is exposed through an opening in the skin; the pubic bones are separated by an interval, and the reproductive organs are ill-formed; the urachus is wanting, and the umbilicus is always placed exactly at the upper end of the gap in the skin. A monstrosity recalling the cloacal arrangement of the bird is met with as a more extreme defect in the same parts.

Hermaphroditism

Although this anomalous condition does not fall under defective closure in the middle line, it may be said to be due to a similar failure of purpose, or to an uncertainty in the nisus formativus at a corresponding stage of development. Strictly speaking, a hermaphrodite is a creature containing ovaries and testes - the essential organs of each sex. Evidence accumulates, however, that at least in all the higher vertebrates, including man, the sex is predetermined in the fertilized ovum, and it is more than doubtful if true hermaphroditism occurs. On the other hand, if there be no such double sex in the essential organs (as in the majority of so-called hermaphrodites) there is a great deal of doubling and ambiguity entailed in the secondary or external organs and parts of generation. Those parts which are rudimentary or obsolete in the male but highly developed in the female, and those parts which are rudimentary in the female but highly developed in the male tend in the hermaphrodite to be developed equally, and all of them badly. Amongst human beings the greater number of so-called hermaphrodites are really females, in which there is an abnormal development of the clitoris, but it also happens that true males may be born with a small clitoris-like penis, with hypospadia - that is to say, with imperfect urethra, open on the ventral side, and with undescended testes. Failure of the development of the testes or ovary, or their removal in the adult condition induces an ambiguous condition of the body in which the secondary sexual characters approach those of the other sex. Experimental removal of the ovaries or testes, followed by implantation of organs of the other sex, has produced an inversion of the secondary sexual characters.

Cyclops, Siren, &c. - The same feebleness of the formative energy which gives rise to some at least of the cases of defective closure in the middle line, and to the cases of ambiguous sex, leads also to imperfect separation of symmetrical parts. The most remarkable case of the kind is the cyclops monster. At a point corresponding to the root of the nose there is found a single orbital cavity, sometimes of small size and with no eyeball in it, at other times of the usual size of the orbit and containing an eyeball more or less complete. In still other cases, which indicate the nature of the anomaly, the orbital cavity extends for some distance on each side of the middle line, and contains two eyeballs lying close together. The usual nose is wanting but above the single orbital cavity there is often a nasal process on the forehead, with which nasal bones may be articulated, and cartilages joined to the latter; these form the framework of a short fleshy protuberance like a small proboscis. The lower jaw is sometimes wanting in cyclopeans; the cheek-bones are apt to be small, and the mouth a small round hole, or altogether absent; the rest of the body may be well developed. The key to the cyclopean condition is found in the state of the brain. The olfactory nerves or lobes are frequently absent; the brain is very imperfectly divided into hemispheres, and appears as a somewhat pear-shaped sac with thick walls, the longitudinal partition of dura mater (falx cerebri) being wanting, the surface almost unconvoluted, the corpus callosum deficient, the basal ganglia rudimentary or fused. The optic chiasma and nerves are usually replaced by a single mesial nerve, but sometimes the chiasma and pair of nerves are present. The origin of this monstrosity dates back to an early period of development, to the time when the future hemispheres were being formed as protrusions from the anterior cerebral vesicle or fore-brain; it may be conceived that, instead of two distinct buds from that vesicle, there was only a single outgrowth with imperfect traces of cleavage. That initial defect would carry with it naturally the undivided state of the cerebrum, and with the latter there would be the absence of olfactory lobes and of a nose, and a single eyeball placed where the nose should have been. A cyclops has been known to live for several days. The monstrosity is not uncommon among the domestic animals, and is especially frequent in the pig.

Another curious result of defective separation of symmetrical parts is the siren form of foetus, in which the lower limbs occur as a single tapering prolongation of the trunk like the hinder part of a dolphin, at the end of which a foot (or both feet) may or may not be visible. The defects in the bones underlying this siren form are very various: in some cases there is only one limb (thigh and leg-bones) in the middle line; in others all the bones of each limb are present in more or less rudimentary condition, but adhering at prominent points of the adjacent surfaces. The pelvis and pelvic viscera share in the abnormality. A much more common and harmless case of unseparated symmetrical parts is where the hand or foot has two, three, or more digits fused together. This syndactylous anomaly runs in families.

Limbs Absent or Stunted

Allied to these fused or unseparated states of the extremities, or of parts of them, are the class of deformities in which whole limbs are absent, or represented only by stumps. The trunk (and head) may be well formed, and the individual healthy; all four extremities may be reduced to short stumps either wanting hands and feet entirely, or with the latter fairly well developed; or the legs only may be rudimentary or wanting, or the arms only, or one extremity only. Although some of these cases doubtless depend upon aberrant or deficient formative power in the particular directions, there are others of them referable to the effects of mechanical pressure, and even to direct amputation of parts within the uterus.

Acardiac and Acranial Monsters

It sometimes happens in a twin pregnancy that one of the embryos fails to develop a heart and a complete vascular system of its own, depending for its nourishment upon blood derived from the placenta of its well-formed twin by means of its umbilical vessels. It grows into a more or less shapeless mass, in which all traces of the human form may be lost. Other viscera besides the heart will be wanting, and no head distinguishable; the most likely parts to keep the line of development are the lumbar region (with the kidneys), the pelvis, and the lower limbs. The twin of this monster may be a healthy infant.

Reversed Position of the Viscera

This is. a developmental error associated with the retention of the right aortic arch as in birds, instead of the left as is usual in mammals. The position of all the unsymmetrical viscera is transposed, the spleen and cardiac end of the stomach going to the right side, the liver to the left, the caecum resting on the left iliac fossa, and the sigmoid flexure of the colon being attached to the right. This condition of situs inversus viscerum need cause no inconvenience; and it will probably remain undetected until the occasion should arise for a physical diagnosis or post-mortem inspection.

The causes of congenital anomalies are difficult to specify. There is no doubt that, in some cases, they are present in the sperm or germ of the parent; the same anomalies recur in several children of a family, and it has been found possible, through a variation of the circumstances, to trace the influence in some cases to the father alone, and in other cases to the mother alone. The remarkable thing in this parental influence is that the malformation in the child may not have been manifested in the body of either parent, or in the grandparents. More often the malformation is acquired by the embryo and foetus in the course of development and growth, either through the mother or in itself independently. Maternal impressions during pregnancy have often been alleged as a cause, and this causation has been discussed at great length by the best authorities. The general opinion seems to be that it is impossible to set aside the influence of subjective states of the mother altogether, but that there is no direct connexion between the cause of the subjective state and the resulting anomaly. The doctrine of maternal impressions has often been resorted to when any other explanation was either difficult or inconvenient; thus, Hippocrates is said to have saved the virtue of a woman who gave birth to a black child by pointing out that there was a picture of a negro on the wall of her chamber. Injuries to the mother during pregnancy have been unquestionably the cause of certain malformations, especially of congenital hydrocephalus. The embryo itself and its membranes may become the subject of inflammations, atrophies, hypertrophies, and the like; this causation is doubtless accountable for a good many of them. But a very large residue of malformations must still be referred to variation in the embryonic cells and cell-groups. The nisus formativus of the fertilized ovum is always subject to morphological laws, but, just as in extra-uterine life, there may be deviations from the beaten track; and even a slight deviation at an early stage will carry with it far-reaching consequences. This is particularly noticeable in double monsters.

2. Double Monsters

Twins are the physiological analogy of double monsters, and some of the latter have come very near to being two separate individuals. The Siamese twins, who died in 1874 at the age of sixty, were joined only by a thick fleshy ligament from the lower end of the breast-bone (xiphoid cartilage), having the common navel on its lower border; the anatomical examination showed, however, that a process of peritoneum extended through the ligament from one abdominal cavity to the other, and that the blood-vessels of the two livers were in free communication across the same bridge. There are one or two cases on record in which such a ligament has been cut at birth, one, at least, of the twins surviving. From the most intelligible form of double monstrosity, like the Siamese twins, there are all grades of fantastic fusion of two individuals into one down to the truly marvellous condition of a small body or fragment parasitic upon a well-grown infant - the condition known as foetus in foetu. These monstrosities are deviations, not from the usual kind of twin gestation, but from a certain rarer physiological type of dual development. In by far the majority of cases twins have separate uterine appendages, and have probably been developed from distinct ova; but in a small proportion of (recorded) cases there is evidence, in the placental and enclosing structures, that the twins had been developed from two rudiments arising side by side on a single blastoderm. It is to the latter physiological category that double monsters almost certainly belong; and there is some direct embryological evidence for this opinion. Allen Thomson observed in the blastoderm of a hen's egg at the sixteenth or eighteenth hour of incubation two "primitive traces" or rudiments of the backbone forming side by side; and in a goose's egg incubated five days he found on one blastoderm two embryos, each with the rudiments of upper and lower extremities, crossing or cohering in the region of the future neck, and with only one heart between them. A very large number of similar observations have been published and appear to be found in all cases where a large material is available. The developing ova of fish, available in large numbers in hatcheries, and the laboratory investigation of the chick and the frog have provided cases of almost every degree of blending. The perfect physiological type appears to be two rudiments on one blastoderm, whose entirely separate development produces twins (under their rarer circumstances), whose nearly separate development produces such double monsters as the Siamese twins, and whose less separate development produces the various grotesque forms of two individuals in one body. There can be no question of a literal fusion of two embryos; either the individuality of each was at no time complete, or, if there were two distinct primitive traces, the uni-axial type was approximately reverted to in the process of development, as in the formation of the abdominal and thoracic viscera, limbs, pelvis or head. Double monsters are divided in the first instance into those in which the doubling is symmetrical and equal on the two sides, and those in which a small or fragmentary foetus is attached to or enclosed in a foetus of average development - the latter class being the so-called cases of "parasitism." Symmetrical Double Monsters are subdivided according to the part or region of the body where the union or fusion exists - head, thorax, umbilicus or pelvis. One of the simplest cases is a Janus head upon a single body, or there may be two pairs of arms with the two faces. Again, there may be one head with two necks and two complete trunks and pairs of extremities. Two distinct heads (with more or less of neck) may surmount a single trunk, broad at the shoulders but with only one pair of arms. The fusion, again, may be from the middle of the thorax downwards, giving two heads and two pairs of shoulders and arms, but only one trunk and one pair of legs. In another variety, the body may be double down to the waist, but the pelvis and lower limbs single. The degree of union in the region of the head, abdomen or pelvis may be so slight as to permit of two distinct organs or sets of organs in the respective cavities, or so great as to have the viscera in common; and there is hardly ever an intermediate condition between those extremes. Thus, in the Janus head there may be two brains, or only one brain. The Siamese twins are an instance of union at the umbilical region, with the viscera distinct in every respect except a slight vascular anastomosis and a common process of peritoneum; but it is more usual for union in that region to be more extensive, and to entail a single set of abdominal and thoracic viscera. The pelvis is one of the commonest regions for double monsters to be joined at, and, as in the head and abdomen, the junction may be slight or total. The Hungarian sisters Helena and Judith (1701-1723) were joined at the sacrum, but had the pelvic cavity and pelvic organs separate; the same condition obtained in the South Carolina negresses Millie and Christina, known as the "two-headed nightingale," and in the Bohemian sisters Rosalie and Josepha. More usually the union in the pelvic region is complete, and produces the most fantastic shapes of two trunks (each with head and arms) joining below at various angles, and with three or four lower limbs extending from the region of fusion, sometimes in a lateral direction, so. metimes downwards. A very curious kind of double monster is produced by two otherwise distinct foetuses joining at the crown of the head and keeping the axis of their bodies in a line. It is only in rare instances that double monsters survive their birth, and the preserved specimens of them are mostly of foetal size.

Unequal Double Monsters, Foetus in Foetu. - There are some well-authenticated instances of this most curious of all anomalies. The most celebrated of these parasite-bearing monsters was a Genoese, Lazarus Johannes Baptista Colloredo, born in 1716, who was figured as a child by Licetus, and again by Bartholinus at the age of twenty-eight as a young man of average stature. The parasite adhered to the lower end of his breast-bone, and was a tolerably well-formed child, wanting only one leg; it breathed, slept at intervals, and moved its body, but it had no separate nutritive functions. The parasite is more apt to be a miniature acardiac and acephalous fragment, as in the case of the one borne in front of the abdomen of a Chinaman figured by I. Geoffroy St Hilaire. Sometimes the parasite is contained in a pouch under the skin of the abdominal wall, and in another class (of which there is a specimen in the Hunterian Museum) it has actually been included, by the closure of the ventral laminae, within the abdominal cavity of the foetus - a true foetus in foetu. Shapeless parasitic fragments containing masses of bone, cartilage and other tissue are found also in the space behind the breast-bone (mediastinal teratoma), or growing from the base of the skull and protruding through the mouth ("epignathous teratoma," appearing to be seated on the jaw), and, most frequently of all, attached to the sacrum. These last pass by a most interesting transition into common forms of congenital sacral tumours (which may be of enormous size), consisting mainly of one kind of tissue having its physiological type in the curious gland-like body (coccygeal gland) in which the middle sacral artery comes to an end. The congenital sacral tumours have a tendency to become cystic, and they are probably related to the more perfect congenital cysts of the neck region, where there is another minute gland-like body of the same nature as the coccygeal at the point of bifurcation of the common carotid artery. Other tumours of the body, especially certain of the sarcomatous class, may be regarded from the point of view of monstra per excessum; but such cases suggest not so much a question of aberrant development within the blastoderm as of the indwelling spontaneity of a single post-embryonic tissue. (See Tumour And Pathology.) Monstrosities in man and animals have attracted attention since the earliest times, and amongst primitive and uncivilized peoples have been regarded as of supernatural origin. Aristotle himself appears to have been the first to examine them as a naturalist, and to explain that although they were outside the usual course of nature they were in the strictest sense of natural origin. Pliny described many well-known forms, but did not distinguish between legendary and actual monstrosities. In the middle ages they were treated in the fullest spirit of superstition, and many relics from such a point of view still survive. The human monstrosities were regarded as having been engendered in women by the devil who had commerce with them either in his own form or in the guise of some animal. The belief still to be found amongst uneducated persons that unnatural union between women and male animals, or between men and female animals, may be fertile and produce monsters, is an attenuated form of the satanic legend. The scientific appreciation of monsters has grown with the study of embryology. William Harvey in Exercitationes de generatione animalium (1651) first referred monstrosities to their proper place as abnormalities in embryonic reproduction. The doctrine of pre-formation (see Heredity) obsessed biological science until 1759 when C. F. Wolff overthrew it, and Harvey's advance was not pursued, except that a number. of anatomists published careful studies and descriptions of monsters or monstrous organs. Those who believed that the normal process of development was an unrolling and expansion of a pre-formed miniature of the adult had to apply a similar theory to monsters, and Sylvain Regis, a contemporary of Malbranche, obtained acceptance of his view that monstrous germs as well as normal germs had been created at the beginning of the world. A discussion almost as memorable as that between E. G. St Hilaire and Cuvier on specific types was pursued in the French Academy from 1724 to 1 743, J. B. Winslow, who supported the current pre-formationist view, having the better of the argument with Louis Lemery, who was almost alone in a rational interpretation of monstrosities. From the time of Wolff it was accepted that normal and abnormal embryos alike developed by processes of epigenetic change. Wolff himself, however, and even J. F. Meckel at the beginning of the 19th century, did not recognize the influence of physiological causes in the production of abnormalities; they believed the latter to proceed certainly in an orderly and natural way, but from abnormal ova. E. G. St Hilaire was the first to attempt experimental teratology and to lay down that many monstrosities were the result of influences causing deviations from the normal course of embryonic development. I. G. St Hilaire, the son of E. G. St Hilaire, carried the experimental method little further, but published an elaborate descriptive treatise on anomalies (Paris, 1832-1837) which remains one of the most valuable records of the subject. A similar treatise with an incomparable atlas of illustrations was issued by W. Vrolik, the great Dutch anatomist, between 1840 and 1849, whilst A. Forster issued in 1861 a valuable textbook with a very large number of illustrations chiefly from preparations in the museum at Wiirzburg. The great museums devoted much attention to the collection and display of malformations, and no account of the subject can be adequate which does not include reference to the magnificent series in the Museum of the Royal College of Surgeons of England, with the descriptive catalogues of the animal malformations written by B. T. Lowne (1893) and of the vegetable malformations by M. T. Masters (1893).

The work hitherto referred to, as well as a vast bulk of scattered contributions to teratology throughout the 19th century, was chiefly descriptive, anatomical and embryological teratology, and carried the experimental side little beyond where it had been left by the St Hilaires. In 1891 Camille Dareste published his Recherches sur la production artificielle des monstruosites, ou essais de,te'ratogenie experimentale; his experiments, chiefly on the developing egg of the fowl, not only showed the probable cause of many of the most common abnormalities, but practically created a new branch of science, experimental embryology. Teratology has since become a side issue of the general study of the inter-relations between the inherited tendencies of the developing organism and the play of the circumambient media, and must be studied in relation to the work of O. Hertwig, W. Roux, H. Driesch, O. Baschli, J. Loeb and their school. Bland Sutton's popular Evolution and Disease (1890) puts in a cogent way the relation between comparative anatomy and common abnormalities, whilst W. Bateson in his Materials for the Study of Variation (1894) describes the acquisition of new symmetries by abnormal organs, and discusses the possible relation between abnormalities and the origin of species.

E. Schwalbe's Morphologie der Missbildungen (1906-1909) is a very complete study of the most modern developments of teratology, and contains a careful and elaborate list of authorities from the earliest times. (C. C.; P. C. M.)