Page:EB1911 - Volume 18.djvu/773

 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