Chapter VI: Minor Terata (4)

(9) The remarkable grasping power of the hand at birth and for a few weeks thereafter, that permits young babies to suspend their whole weight on a cane for a period varying from half a minute to two minutes.

Horrocks ascribes to these anal tags a pathologic importance. He claims that they may be productive of fistula in ano, superficial ulcerations, fecal concretions, fissure in ano, and that they may hypertrophy and set up tenesmus and other troubles. The presence of human tails has given rise to discussion between friends and opponents of the Darwinian theory. By some it is considered a reversion to the lower species, while others deny this and claim it to be simply a pathologic appendix.

Anomalies of the Spinal Canal and Contents.--When there is a default in the spinal column, the vice of conformation is called spina bifida. This is of two classes: first, a simple opening in the vertebral canal, and, second, a large cleft sufficient to allow the egress of spinal membranes and substance. Figure 130 represents a large congenital sacral tumor.

Achard speaks of partial duplication of the central canal of the spinal cord. De Cecco reports a singular case of duplication of the lumbar segment of the spinal cord. Wagner speaks of duplication of a portion of the spinal cord.

Foot records a case of amyelia, or absence of the spinal cord, in a fetus with hernia cerebri and complete fissure of the spinal column. Nicoll and Arnold describe an anencephalous fetus with absence of spinal marrow; and Smith also records the birth of an amyelitic fetus.

In some persons there are exaggerated curvatures of the spine. The first of these curvatures is called kyphosis, in which the curvature is posterior; second, lordosis, in which the curvature is anterior; third, scoliosis, in which it is lateral, to the right or left.

Kyphosis is the most common of the deviations in man and is most often found in the dorsal region, although it may be in the lumbar region. Congenital kyphosis is very rare in man, is generally seen in monsters, and when it does exist is usually accompanied by lordosis or spine bifida. We sometimes observe a condition of anterior curvature of the lumbar and sacral regions, which might be taken for a congenital lordosis, but this is really a deformity produced after birth by the physiologic weight of the body. Figure 131 represents a case of lordosis caused by paralysis of the spinal muscles.

Analogous to this is what the accoucheurs call spondylolisthesis. Scoliosis may be a cervicodorsal, dorsolumbar, or lumbosacral curve, and the inclination of the vertebral column may be to the right or left. The pathologists divide scoliosis into a myopathic variety, in which the trouble is a physiologic antagonism of the muscles; or osteopathic, ordinarily associated with rachitis, which latter variety is generally accountable for congenital scoliosis. In some cases the diameter of the chest is shortened to an almost incredible degree, but may yet be compatible with life. Glover speaks of an extraordinary deformity of the chest with lateral curvature of the spine, in which the diameter from the pit of the stomach to the spinal integument was only 5 1/2 inches.

Supernumerary ribs are not at all uncommon in man, nearly every medical museum having some examples. Cervical ribs are not rare. Gordon describes a young man of seventeen in whom there was a pair of supernumerary ribs attached to the cervical vertebrae. Bernhardt mentions an instance in which cervical ribs caused motor and sensory disturbances. Dumerin of Lyons showed an infant of eight days which had an arrested development of the 2d, 3d, 4th, and 5th ribs. Cases of deficient ribs are occasionally met. Wistar in 1818 gives an account of a person in whom one side of the thorax was at rest while the other performed the movements of breathing in the usual manner.

In some cases we see fissure of the sternum, caused either by deficient union or absence of one of its constituent parts. In the most exaggerated cases these fissures permit the exit of the heart, and as a general rule ectopies of the heart are thus caused. Pavy has given a most remarkable case of sternal fissure in a young man of twenty-five, a native of Hamburg. He exhibited himself in one medical clinic after another all over Europe, and was always viewed with the greatest interest. In the median line, corresponding to the absence of sternum, was a longitudinal groove bounded on either side by a continuous hard ridge which articulated with the costal cartilages. The skin passed naturally over the chest from one side to another, but was raised at one part of the groove by a pulsatile swelling which occupied the position of the right auricle. The clavicle and the two margins of the sternum had no connections whatever, and below the groove was a hard substance corresponding to the ensiform cartilage, which, however, was very elastic, and allowed the patient, under the influence of the pectoral muscles, when the upper extremity was fixed, to open the groove to nearly the extent of three inches, which was more than twice its natural width. By approximating his arms he made the ends of his clavicles overlap. When he coughed, the right lung suddenly protruded from the chest through the groove and ascended a considerable distance above the clavicle into the neck. Between the clavicles another pulsatile swelling was easily felt but hardly seen, which was doubtless the arch of the aorta, as by putting the fingers on it one could feel a double shock, synchronous with distention and recoil of a vessel or opening and closing of the semilunar valves.

Madden pictures (Figs. 134 and 135) a Swede of forty with congenital absence of osseous structure in the middle line of the sternum, leaving a fissure 5 3/8 X 1 3/16 X 2 inches, the longest diameter being vertical. Madden also mentions several analogous instances on record. Groux's case was in a person of forty-five, and the fissure had the vertical length of four inches. Hodgen of St. Louis reports a case in which there was exstrophy of the heart through the fissure. Slocum reports the occurrence of a sternal fissure 3 X 1 1/2 inches in an Irishman of twenty-five. Madden also cites the case of Abbott in an adult negress and a mother. Obermeier mentions several cases. Gibson and Malet describe a presternal fissure uncovering the base of the heart. Ziemssen, Wrany, and Williams also record congenital fissures of the sternum.

Thomson has collected 86 cases of thoracic defects and summarizes his paper by saying that the structures deficient are generally the hair in the mammary and axillary regions, the subcutaneous fat over the muscles, nipples, and breasts, the pectorals and adjacent muscles, the costal cartilages and anterior ends of ribs, the hand and forearm; he also adds that there may be a hernia of the lung, not hereditary, but probably due to the pressure of the arm against the chest. De Marque gives a curious instance in which the chin and chest were congenitally fastened together. Muirhead cites an instance in which a firm, broad strip of cartilage resembling sternomastoid extended from below the left ear to the left upper corner of the sternum, being entirely separate from the jaw.

Some preliminary knowledge of embryology is essential to understand the formation of branchial fissures, and we refer the reader to any of the standard works on embryology for this information. Dzondi was one of the first to recognize and classify congenital fistulas of the neck. The proper classification is into lateral and median fissures. In a case studied by Fevrier the exploration of a lateral pharyngeal fistula produced by the introduction of the sound violent reflex phenomena, such as pallor of the face and irregular, violent beating of the heart. The rarest of the lateral class is the preauricular fissure, which has been observed by Fevrier, Le Dentu, Marchand, Peyrot, and Routier.

The median congenital fissures of the neck are probably caused by defective union of the branchial arches, although Arndt thinks that he sees in these median fistulas a persistence of the hypobranchial furrow which exists normally in the amphioxus. They are less frequent than the preceding variety.

The most typical form of malformation of the esophagus is imperforation or obliteration. Van Cuyck of Brussels in 1824 delivered a child which died on the third day from malnutrition. Postmortem it was found that the inferior extremity of the esophagus to the extent of about two inches was converted into a ligamentous cord. Porro describes a case of congenital obliteration of the esophagus which ended in a cecal pouch about one inch below the inferior portion of the glottidean aperture and from this point to the stomach only measured an inch; there was also tracheal communication. The child was noticed to take to the breast with avidity, but after a little suckling it would cough, become livid, and reject most of the milk through the nose, in this way almost suffocating at each paroxysm; it died on the third day.

In some cases the esophagus is divided, one portion opening into the bronchial or other thoracic organs. Brentano describes an infant dying ten days after birth whose esophagus was divided into two portions, one terminating in a culdesac, the other opening into the bronchi; the left kidney was also displaced downward. Blasius describes an anomalous case of duplication of the esophagus. Grashuys, and subsequently Vicq d'Azir, saw a dilatation of the esophagus resembling the crop of a bird.

Anomalies of the Lungs.--Carper describes a fetus of thirty-seven weeks in whose thorax he found a very voluminous thymus gland but no lungs. These organs were simply represented by two little oval bodies having no lobes, with the color of the tissue of the liver. The heart had only one cavity but all the other organs were perfectly formed. This case seems to be unique. Tichomiroff records the case of a woman of twenty-four who died of pneumonia in whom the left lung was entirely missing. No traces of a left bronchus existed. The subject was very poorly developed physically. Tichomiroff finds four other cases in literature, in all of which the left lung was absent. Theremin and Tyson record cases of the absence of the left lung.

Supplementary pulmonary lobes are occasionally seen in man and are taken by some authorities to be examples of retrogressive anomalies tending to prove that the derivation of the human race is from the quadrupeds which show analogous pulmonary malformation. Eckley reports an instance of supernumerary lobe of the right lung in close connection with the vena azygos major. Collins mentions a similar case. Bonnet and Edwards speak of instances of four lobes in the right lung. Testut and Marcondes report a description of a lung with six lobes.

Anomalies of the Diaphragm.--Diemerbroeck is said to have dissected a human subject in whom the diaphragm and mediastinum were apparently missing, but such cases must be very rare, although we frequently find marked deficiency of this organ. Bouchand reports an instance of absence of the right half of the diaphragm in an infant born at term. Lawrence mentions congenital deficiency of the muscular fibers of the left half of the diaphragm with displacement of the stomach. The patient died of double pneumonia. Carruthers, McClintock, Polaillon, and van Geison also record instances of congenital deficiency of part of the diaphragm. Recently Dittel reported unilateral defect in the diaphragm of an infant that died soon after birth. The stomach, small intestines, and part of the large omentum lay in the left pleural cavity; both the phrenic nerves were normal. Many similar cases of diaphragmatic hernia have been observed. In such cases the opening may be large enough to allow a great part of the visceral constituents to pass into the thorax, sometimes seriously interfering with respiration and circulation by the pressure which ensues. Alderson reports a fatal case of diaphragmatic hernia with symptoms of pneumothorax. The stomach, spleen, omentum, and transverse colon were found lying in the left pleura. Berchon mentions double perforation of the diaphragm with hernia of the epiploon. The most extensive paper on this subject was contributed by Bodwitch, who, besides reporting an instance in the Massachusetts General Hospital, gives a numerical analysis of all the cases of this affection found recorded in the writings of medical authors between the years 1610 and 1846. Hillier speaks of an instance of congenital diaphragmatic hernia in which nearly all the small intestines and two-thirds of the large passed into the right side of the thorax. Macnab reports an instance in which three years after the cure of empyema the whole stomach constituted the hernia. Recently Joly described congenital hernia of the stomach in a man of thirty-seven, who died from collapse following lymphangitis, persistent vomiting, and diarrhea. At the postmortem there was found a defect in the diaphragm on the left side, permitting herniation of the stomach and first part of the duodenum into the left pleural cavity. There was no history of traumatism to account for strangulation. Longworth cites an instance of inversion of the diaphragm in a human subject. Bartholinus mentions coalition of the diaphragm and liver; and similar cases are spoken of by Morgagni and the Ephemerides. Hoffman describes diaphragmatic junction with the lung.

Anomalies of the Stomach.--The Ephemerides contains the account of a dissection in which the stomach was found wanting, and also speaks of two instances of duplex stomach. Bartholinus, Heister, Hufeland, Morgagni, Riolan, and Sandifort cite examples of duplex stomach. Bonet speaks of a case of vomiting which was caused by a double stomach. Struthers reports two cases in which there were two cavities to the stomach. Struthers also mentions that Morgagni, Home, Monro, Palmer, Larry, Blasius, Hufeland, and Walther also record instances in which there was contraction in the middle of the stomach, accounting for their instances of duplex stomach. Musser reports an instance of hour-glass contraction of the stomach. Hart dissected the stomach of a woman of thirty which resembled the stomach of a predaceous bird, with patches of tendon on its surface. The right extremity instead of continuously contracting ended in a culdesac one-half as large as the greater end of the stomach. The duodenum proceeded from the depression marking the lesser arch of the organ midway between the cardiac orifice and the right extremity. Crooks speaks of a case in which the stomach of an infant terminated in a culdesac.

Hernia of the stomach is not uncommon, especially in diaphragmatic or umbilical deficiency. There are many cases on record, some terminating fatally from strangulation or exposure to traumatism. Paterson reports a case of congenital hernia of the stomach into the left portion of the thoracic cavity. It was covered with fat and occupied the whole left half of the thoracic cavity. The spleen, pancreas, and transverse colon were also superior to the diaphragm. Death was caused by a well-defined round perforation at the cardiac curvature the size of a sixpence.

Anomalies of the Intestines.--The Ephemerides contains the account of an example of double cecum, and Alexander speaks of a double colon, and there are other cases of duplication of the bowel recorded. There is an instance of coalition of the jejunum with the liver, and Treuner parallels this case. Aubery, Charrier Poelman, and others speak of congenital division of the intestinal canal. Congenital occlusion is quite frequently reported.

Dilatation of the colon frequently occurs as a transient affection, and by its action in pushing up the diaphragm may so seriously interfere with the action of the heart and lungs as to occasionally cause heart-failure. Fenwick has mentioned an instance of this nature. According to Osler there is a chronic form of dilatation of the colon in which the gut may reach an enormous size. The coats may be hypertrophied without evidence of any special organic change in the mucosa. The most remarkable instance has been reported by Formad. The patient, known as the "balloon-man," aged twenty-three at the time of his death, had had a distended abdomen from infancy. Postmortem the colon was found as large as that of an ox, the circumference ranging from 15 to 30 inches. The weight of the contents was 47 pounds. Cases are not uncommon in children. Osler reports three well-marked cases under his care. Chapman mentions a case in which the liver was displaced by dilatation of the sigmoid flexure. Mya reports two cases of congenital dilatation and hypertrophy of the colon (megacolon congenito). Hirsohsprung, Genersich, Faralli, Walker, and Griffiths all record similar instances, and in all these cases the clinical features were obstinate constipation and marked meteorismus.

Imperforate Anus.--Cases in which the anus is imperforate or the rectum ends in a blind pouch are occasionally seen. In some instances the rectum is entirely absent, the colon being the termination of the intestinal tract. There are cases on record in which the rectum communicated with the anus solely by a fibromuscular cord. Anorectal atresia is the ordinary imperforation of the anus, in which the rectum terminates in the middle of the sacral cavity. The rectum may be deficient from the superior third of the sacrum, and in this position is quite inaccessible for operation.

A compensatory coalition of the bowel with the bladder or urethra is sometimes present, and in these cases the feces are voided by the urinary passages. Huxham mentions the fusion of the rectum and colon with the bladder, and similar instances are reported by Dumas and Baillie. Zacutus Lusitanus describes an infant with an imperforate membrane over its anus who voided feces through the urethra for three months. After puncture of the membrane, the discharge came through the natural passage and the child lived; Morgagni mentions a somewhat similar case in a little girl living in Bologna, and other modern instances have been reported. The rectum may terminate in the vagina. Masters has seen a child who lived nine days in whom the sigmoid flexure of the colon terminated in the fundus of the bladder. Guinard pictures a case in which there was communication between the rectum and the bladder. In Figure 140 a represents the rectum; b the bladder; c the point of communication; g shows the cellular tissue of the scrotum.

There is a description of a girl of fourteen, otherwise well constituted and healthy, who had neither external genital organs nor anus. There was a plain dermal covering over the genital and anal region. She ate regularly, but every three days she experienced pain in the umbilicus and much intestinal irritation, followed by severe vomiting of stercoraceous matter; the pains then ceased and she cleansed her mouth with aromatic washes, remaining well until the following third day. Some of the urine was evacuated by the mammae. The examiners displayed much desire to see her after puberty to note the disposition of the menstrual flow, but no further observation of her case can be found.

Fournier narrates that he was called by three students, who had been trying to deliver a woman for five days. He found a well-constituted woman of twenty-two in horrible agony, who they said had not had a passage of the bowels for eight days, so he prescribed an enema. The student who was directed to give the enema found to his surprise that there was no anus, but by putting his finger in the vagina he could discern the floating end of the rectum, which was full of feces. There was an opening in this suspended rectum about the size of an undistended anus. Lavage was practiced by a cannula introduced through the opening, and a great number of cherry stones agglutinated with feces followed the water, and labor was soon terminated. The woman afterward confessed that she was perfectly aware of her deformity, but was ashamed to disclose it before. There was an analogue of this case found by Mercurialis in a child of a Jew called Teutonicus.

Gerster reports a rare form of imperforate anus, with malposition of the left ureter, obliteration of the ostia of both ureters, with consequent hydronephrosis of a confluent kidney. There was a minute opening into the bladder, which allowed the passage of meconium through the urethra. Burge mentions the case of what he calls "sexless child," in which there was an imperforate anus and no pubic arch; the ureters discharged upon a tumor the size of a teacup extending from the umbilicus to the pubes. A postmortem examination confirmed the diagnosis of sexless child.

The Liver.--The Ephemerides, Frankenau, von Home, Molinetti, Schenok, and others speak of deficient or absent liver. Zacutus Lusitanus says that he once found a mass of flesh in place of the liver. Lieutaud is quoted as describing a postmortem examination of an adult who had died of hydropsy, in whom the liver and spleen were entirely missing. The portal vein discharged immediately into the vena cava; this case is probably unique, as no authentic parallel could be found.

Laget reports an instance of supernumerary lobe in the liver. Van Buren describes a supernumerary liver. Sometimes there is rotation, real or apparent, caused by transposition of the characteristics of the liver. Handy mentions such a case. Kirmisson reports a singular anomaly of the liver which he calls double displacement by interversion and rotation on the vertical axis. Actual displacements of the liver as well as what is known as wandering liver are not uncommon. The operation for floating liver will be spoken of later.

Hawkins reports a case of congenital obliteration of the ductus communis choledochus in a male infant which died at the age of four and a half months. Jaundice appeared on the eighth day and lasted through the short life. The hepatic and cystic ducts were pervious and the hepatic duct obliterated. There were signs of hepatic cirrhosis and in addition an inguinal hernia.

The Gall-Bladder.--Harle mentions the case of a man of fifty, in whom he could find no gall-bladder; Patterson has seen a similar instance in a men of twenty-five. Purser describes a double gall-bladder.

The spleen has been found deficient or wanting by Lebby, Ramsay, and others, but more frequently it is seen doubled. Cabrolius, Morgagni, and others have found two spleens in one subject; Cheselden and Fallopius report three; Fantoni mentions four found in one subject; Guy-Patin has seen five, none as large as the ordinary organ; Hollerius, Kerckringius, and others have remarked on multiple spleens. There is a possibility that in some of the cases of multiple spleens reported the organ is really single but divided into several lobes. Albrecht mentions a case shown at a meeting of the Vienna Medical Society of a very large number of spleens found in the mesogastrium, peritoneum, on the mesentery and transverse mesocolon, in Douglas' pouch, etc. There was a spleen "the size of a walnut" in the usual position, with the splenic artery and vein in their normal position. Every one of these spleens had a capsule, was covered by peritoneum, and exhibited the histologic appearance of splenic tissue. According to the review of this article, Toldt explains the case by assuming that other parts of the celomic epithelium, besides that of the mesogastrium, are capable of forming splenic tissue. Jameson reports a case of double spleen and kidneys. Bainbrigge mentions a case of supernumerary spleen causing death from the patient being placed in the supine position in consequence of fracture of the thigh. Peevor mentions an instance of second spleen. Beclard and Guy-Patin have seen the spleen congenitally misplaced on the right side and the liver on the left; Borellus and Bartholinus with others have observed misplacement of the spleen.

The Pancreas.--Lieutaud has seen the pancreas missing and speaks of a double pancreatic duct that he found in a man who died from starvation; Bonet speaks of a case similar to this last.

There are several cases of complete transposition of the viscera on record. This bizarre anomaly was probably observed first in 1650 by Riolanus, but the most celebrated case was that of Morand in 1660, and Mery described the instance later which was the subject of the following quatrain:--

"La nature, peu sage et sans douse en debauche Placa le foie au cote gauche, Et de meme, vice versa Le coeur a le droite placa."

Young cites an example in a woman of eighty-five who died at Hammersmith, London. She was found dead in bed, and in a postmortem examination, ordered to discover if possible the cause of death, there was seen complete transposition of the viscera. The heart lay with its base toward the left, its apex toward the right, reaching the lower border of the 4th rib, under the right mamma. The vena cava was on the left side and passed into the pulmonary cavity of the heart, which was also on the left side, the aorta and systemic ventricle being on the right. The left splenic vein was lying on the superior vena cava, the liver under the left ribs, and the spleen on the right side underneath the heart. The esophagus was on the right of the aorta, and the location of the two ends of the stomach was reversed; the sigmoid flexure was on the right side. Davis describes a similar instance in a man.

Herrick mentions transposition of viscera in a man of twenty-five. Barbieux cites a case of transposition of viscera in a man who was wounded in a duel. The liver was to the left and the spleen and heart to the right etc. Albers, Baron, Beclard, Boyer, Bull, Mackensie, Hutchinson, Hunt, Murray, Dareste, Curran, Duchesne, Musser, Sabatier, Shrady, Vulpian, Wilson, and Wehn are among others reporting instances of transposition and inversion of the viscera.

Congenital extroversion or eventration is the result of some congenital deficiency in the abdominal wall; instances are not uncommon, and some patients live as long as do cases of umbilical hernia proper. Ramsey speaks of entire want of development of the abdominal parietes. Robertson, Rizzoli, Tait, Hamilton, Brodie, Denis, Dickie, Goyrand, and many others mention extroversion of viscera from parietal defects. The different forms of hernia will be considered in another chapter.

There seem to be no authentic cases of complete absence of the kidney except in the lowest grades of monstrosities. Becker, Blasius, Rhodius, Baillie, Portal, Sandifort, Meckel, Schenck, and Stoll are among the older writers who have observed the absence of one kidney. In a recent paper Ballowitz has collected 213 cases, from which the following extract has been made by the British Medical Journal:--

"Ballowitz (Virchow's Archiv, August 5, 1895) has collected as far as possible all the recorded cases of congenital absence of one kidney. Excluding cases of fused kidney and of partial atrophy of one kidney, he finds 213 cases of complete absence of one kidney, upon which he bases the following conclusions: Such deficiency occurs almost twice as often in males as in females, a fact, however, which may be partly accounted for by the greater frequency of necropsies on males. As to age, 23 occurred in the fetus or newly born, most having some other congenital deformity, especially imperforate anus; the rest were about evenly distributed up to seventy years of age, after which only seven cases occurred. Taking all cases together, the deficiency is more common on the left than on the right side; but while in males the left kidney is far more commonly absent than the right, in females the two sides show the defect equally. The renal vessels were generally absent, as also the ureter, on the abnormal side (the latter in all except 15 cases); the suprarenal was missing in 31 cases. The solitary kidney was almost always normal in shape and position, but much enlarged. Microscopically the enlargement would seem to be due rather to hyperplasia than to hypertrophy. The bladder, except for absence of the opening of one ureter, was generally normal. In a large number of cases there were associated deformities of the organs of generation, especially of the female organs, and these were almost invariably on the side of the renal defect; they affected the conducting portion much more than the glandular portion--that is, uterus, vagina, and Fallopian tubes in the female, and vas deferens or vesiculae seminales in the male, rather than the ovaries or testicles. Finally, he points out the practical bearing of the subject--for example, the probability of calculus causing sudden suppression of urine in such cases--and also the danger of surgical interference, and suggests the possibility of diagnosing the condition by ascertaining the absence of the opening of one ureter in the bladder by means of the cystoscope, and also the likelihood of its occurring where any abnormality of the genital organs is found, especially if this be unilateral."

Green reports the case of a female child in which the right kidney and right Fallopian tube and ovary were absent without any rudimentary structures in their place. Guiteras and Riesman have noted the absence of the right kidney, right ureter, and right adrenal in an old woman who had died of chronic nephritis. The left kidney although cirrhotic was very much enlarged.

Tompsett describes a necropsy made on a coolie child of nearly twelve months, in which it was seen that in the place of a kidney there were two left organs connected at the apices by a prolongation of the cortical substance of each; the child had died of neglected malarial fever. Sandifort speaks of a case of double kidneys and double ureters, and cases of supernumerary kidney are not uncommon, generally being segmentation of one of the normal kidneys. Rayer has seen three kidneys united and formed like a horseshoe. We are quite familiar with the ordinary "horseshoe kidney," in which two normal kidneys are connected.

There are several forms of displacement of the kidneys, the most common being the "floating kidney," which is sometimes successfully removed or fixed; Rayer has made an extensive study of this anomaly.

The kidney may be displaced to the pelvis, and Guinard quotes an instance in which the left kidney was situated in the pelvis, to the left of the rectum and back of the bladder. The ureter of the left side was very short. The left renal artery came from the bifurcation of the aorta and the primitive iliacs. The right kidney was situated normally, and received from the aorta two arteries, whose volume did not surpass the two arteries supplying the left suprarenal capsule, which was in its ordinary place. Displacements of the kidney anteriorly are very rare.

The ureters have been found multiple; Griffon reports the history of a male subject in whom the ureter on the left side was double throughout its whole length; there were two vesical orifices on the left side one above the other; and Morestin, in the same journal, mentions ureters double on both sides in a female subject. Molinetti speaks of six ureters in one person. Littre in 1705 described a case of coalition of the ureters. Allen describes an elongated kidney with two ureters. Coeyne mentions duplication of the ureters on both sides. Lediberder reports a case in which the ureter had double origin. Tyson cites an instance of four ureters in an infant. Penrose mentions the absence of the upper two-thirds of the left ureter, with a small cystic kidney, and there are parallel cases on record.

The ureters sometimes have anomalous terminations either in the rectum, vagina, or directly in the urethra. This latter disposition is realized normally in a number of animals and causes the incessant flow of urine, resulting in a serious inconvenience. Flajani speaks of the termination of the ureters in the pelvis; Nebel has seen them appear just beneath the umbilicus; and Lieutaud describes a man who died at thirty-five, from another cause, whose ureters, as large as intestines, terminated in the urethral canal, causing him to urinate frequently; the bladder was absent. In the early part of this century there was a young girl examined in New York whose ureters emptied into a reddish carnosity on the mons veneris. The urine dribbled continuously, and if the child cried or made any exertion it came in jets. The genital organs participated but little in the deformity, and with the exception that the umbilicus was low and the anus more anterior than natural, the child was well formed and its health good. Colzi reports a case in which the left ureter opened externally at the left side of the hymen a little below the normal meatus urinarius. There is a case described of a man who evidently suffered from a patent urachus, as the urine passed in jets as if controlled by a sphincter from his umbilicus. Littre mentions a patent urachus in a boy of eighteen. Congenital dilatation of the ureters is occasionally seen in the new-born. Shattuck describes a male fetus showing reptilian characters in the sexual ducts. There was ectopia vesicae and prolapse of the intestine at the umbilicus; the right kidney was elongated; the right vas deferens opened into the ureter. There was persistence in a separate condition of the two Mullerian ducts which opened externally inferiorly, and there were two ducts near the openings which represented anal pouches. Both testicles were in the abdomen. Ord describes a man in whom one of the Mullerian ducts was persistent.

Anomalies of the Bladder.--Blanchard, Blasius, Haller, Nebel, and Rhodius mention cases in which the bladder has been found absent and we have already mentioned some cases, but the instances in which the bladder has been duplex are much more frequent. Bourienne, Oberteuffer, Ruysch, Bartholinus, Morgagni, and Franck speak of vesical duplication. There is a description of a man who had two bladders, each receiving a ureter. Bussiere describes a triple bladder, and Scibelli of Naples mentions an instance in a subject who died at fifty-seven with symptoms of retention of urine. In the illustration, B represents the normal bladder, A and C the supplementary bladders, with D and E their respective points of entrance into B. As will be noticed, the ureters terminate in the supplementary bladders. Fantoni and Malgetti cite instances of quintuple bladders.

The Ephemerides speaks of a case of coalition of the bladder with the os pubis and another case of coalition with the omentum. Prochaska mentions vesical fusion with the uterus, and we have already described union with the rectum and intestine.

Exstrophy of the bladder is not rare, and is often associated with hypospadias, epispadias, and other malformations of the genitourinary tract. It consists of a deficiency of the abdominal wall in the hypogastric region, in which is seen the denuded bladder. It is remedied by many different and ingenious plastic operations.

In an occasional instance in which there is occlusion at the umbilicus and again at the neck of the bladder this organ becomes so distended as to produce a most curious deformity in the fetus. Figure 143 shows such a case.

The Heart.--Absence of the heart has never been recorded in human beings except in the case of monsters, as, for example, the omphalosites, although there was a case reported and firmly believed by the ancient authors,--a Roman soldier in whom Telasius said he could discover no vestige of a heart.

The absence of one ventricle has been recorded. Schenck has seen the left ventricle deficient, and the Ephemerides, Behr, and Kerckring speak of a single ventricle only in the heart. Riolan mentions a heart in which both ventricles were absent. Jurgens reported in Berlin, February 1, 1882, an autopsy on a child who had lived some days after birth, in which the left ventricle of the heart was found completely absent. Playfair showed the heart of a child which had lived nine months in which one ventricle was absent. In King's College Hospital in London there is a heart of a boy of thirteen in which the cavities consist of a single ventricle and a single auricle.

Duplication of the heart, notwithstanding the number of cases reported, has been admitted with the greatest reserve by Geoffroy-Saint-Hilaire and by a number of authors. Among the celebrated anatomists who describe duplex heart are Littre, Meckel, Collomb, Panum, Behr, Paullini, Rhodins, Winslow, and Zacutus Lusitanus.

The Ephemerides cites an instance of triple heart, and Johnston has seen a triple heart in a goose.

The phenomenon of "blue-disease," or congenital cyanosis, is due to the patency of the foremen ovale, which, instead of closing at birth, persists sometimes to adult life.

Perhaps the most unique collection of congenital malformations of the heart from persons who have reached the age of puberty was to be seen in London in 1895. In this collection there was an adult heart in which the foremen ovale remained open until the age of thirty-seven; there were but two pulmonary valves; there was another heart showing a large patent foramen ovale from a man of forty-six; and there was a septum ventriculorum of an adult heart from a woman of sixty-three, who died of carcinoma of the breast, in which the foremen ovale was still open and would admit the fore-finger. This woman had shown no symptoms of the malformation. There were also hearts in which the interventricular septum was deficient, the ductus arteriosus patent, or some valvular malformation present. All these persons had reached puberty.

Displacements of the heart are quite numerous. Deschamps of Laval made an autopsy on an old soldier which justified the expression, "He had a heart in his belly." This organ was found in the left lumbar region; it had, with its vessels, traversed an anomalous opening in the diaphragm. Franck observed in the Hospital of Colmar a woman with the heart in the epigastric region. Ramel and Vetter speak of the heart under the diaphragm.

Inversion of the heart is quite frequent, and we often find reports of cases of this anomaly. Fournier describes a soldier of thirty years, of middle height, well proportioned and healthy, who was killed in a duel by receiving a wound in the abdomen; postmortem, the heart was found in the position of the right lung; the two lungs were joined and occupied the left chest.

The anomalies of the vascular system are so numerous that we shall dismiss them with a slight mention. Malacarne in Torino in 1784 described a double aorta, and Hommelius mentions an analogous case. The following case is quite an interesting anatomic anomaly: A woman since infancy had difficulty in swallowing, which was augmented at the epoch of menstruation and after exercise; bleeding relieved her momentarily, but the difficulty always returned. At last deglutition became impossible and the patient died of malnutrition. A necropsy revealed the presence of the subclavicular artery passing between the tracheal artery and the esophagus, compressing this latter tube and opposing the passage of food.

Anomalies of the Breasts.--The first of the anomalies of the generative apparatus to be discussed, although not distinctly belonging under this head, will be those of the mammae.

Amazia, or complete absence of the breast, is seldom seen. Pilcher describes an individual who passed for a female, but who was really a male, in whom the breasts were absolutely wanting. Foerster, Froriep, and Ried cite instances associated with thoracic malformation. Greenhow reports a case in which the mammae were absent, although there were depressed rudimentary nipples and areolae. There were no ovaries and the uterus was congenitally imperfect.

There was a negress spoken of in 1842 in whom the right breast was missing, and there are cases of but one breast, mentioned by King, Paull, and others. Scanzoni has observed absence of the left mamma with absence of the left ovary.

Micromazia is not so rare, and is generally seen in females with associate genital troubles. Excessive development of the mammae, generally being a pathologic phenomenon, will be mentioned in another chapter. However, among some of the indigenous negroes the female breasts are naturally very large and pendulous. This is well shown in Figure 144, which represents a woman of the Bushman tribe nursing an infant. The breasts are sufficiently pendulous and loose to be easily thrown over the shoulder.

Polymazia is of much more frequent occurrence than is supposed. Julia, the mother of Alexander Severus, was surnamed "Mammea" because she had supernumerary breasts. Anne Boleyn, the unfortunate wife of Henry VIII of England, was reputed to have had six toes, six fingers, and three breasts. Lynceus says that in his time there existed a Roman woman with four mammae, very beautiful in contour, arranged in two lines, regularly, one above the other, and all giving milk in abundance. Rubens has pictured a woman with four breasts; the painting may be seen in the Louvre in Paris.

There was a young and wealthy heiress who addressed herself to the ancient faculty at Tubingen, asking, as she displayed four mammary, whether, should she marry, she would have three or four children at a birth. This was a belief with which some of her elder matron friends had inspired her, and which she held as a hindrance to marriage.

Leichtenstern, who has collected 70 cases of polymazia in females and 22 in males, thinks that accessory breasts or nipples are due to atavism, and that our most remote inferiorly organized ancestors had many breasts, but that by constantly bearing but one child, from being polymastic, females have gradually become bimastic. Some of the older philosophers contended that by the presence of two breasts woman was originally intended to bear two children.

Hirst says: "Supernumerary breasts and nipples are more common than is generally supposed. Bruce found 60 instances in 3956 persons examined (1.56 per cent). Leichtenstern places the frequency at one in 500. Both observers declare that men present the anomaly about twice as frequently as women. It is impossible to account for the accessory glands on the theory of reversion, as they occur with no regularity in situation, but may develop at odd places on the body. The most frequent position is on the pectoral surface below the true mammae and somewhat nearer the middle line, but an accessory gland has been observed on the left shoulder over the prominence of the deltoid, on the abdominal surface below the costal cartilages, above the umbilicus, in the axilla, in the groin, on the dorsal surface, on the labium majus, and on the outer aspect of the left thigh. Ahlfeld explains the presence of mammae on odd parts of the body by the theory that portions of the embryonal material entering into the composition of the mammary gland are carried to and implanted upon any portion of the exterior of the body by means of the amnion."

Possibly the greatest number of accessory mammae reported is that of Neugebauer in 1886, who found ten in one person. Peuch in 1876 collected 77 cases, and since then Hamy, Quinqusud, Whiteford, Engstrom, and Mitchell Bruce have collected cases. Polymazia must have been known in the olden times, and we still have before us the old images of Diana, in which this goddess is portrayed with numerous breasts, indicating her ability to look after the growing child. Figure 145 shows an ancient Oriental statue of Artemisia or Diana now at Naples.

Bartholinus has observed a Danish woman with three mammae, two ordinarily formed and a third forming a triangle with the others and resembling the breasts of a fat man. In the village of Phullendorf in Germany early in this century there was an old woman who sought alms from place to place, exhibiting to the curious four symmetrical breasts, arranged parallel. She was extremely ugly, and when on all fours, with her breasts pendulous, she resembled a beast. The authors have seen a man with six distinct nipples, arranged as regularly as those of a bitch or sow. The two lower were quite small. This man's body was covered with heavy, long hair, making him a very conspicuous object when seen naked during bathing. The hair was absent for a space of nearly an inch about the nipples. Borellus speaks of a woman with three mammae, two as ordinarily, the third to the left side, which gave milk, but not the same quantity as the others. Gardiner describes a mulatto woman who had four mammae, two of which were near the axillae, about four inches in circumference, with proportionate sized nipples. She became a mother at fourteen, and gave milk from all her breasts. In his "Dictionnaire Philosophique" Voltaire gives the history of a woman with four well-formed and symmetrically arranged breasts; she also exhibited an excrescence, covered with a nap-like hair, looking like a cow-tail. Percy thought the excrescence a prolongation of the coccyx, and said that similar instances were seen in savage men of Borneo.

Percy says that among some prisoners taken in Austria was found a woman of Valachia, near Roumania, exceedingly fatigued, and suffering intensely from the cold. It was January, and the ground was covered with three feet of snow. She had been exposed with her two infants, who had been born twenty days, to this freezing temperature, and died on the next day. An examination of her body revealed five mammae, of which four projected as ordinarily, while the fifth was about the size of that of a girl at puberty.

They all had an intense dark ring about them; the fifth was situated about five inches above the umbilicus. Percy injected the subject and dissected and described the mammary blood-supply. Hirst mentions a negress of nineteen who had nine mammae, all told, and as many nipples. The two normal glands were very large. Two accessory glands and nipples below them were small and did not excrete milk. All the other glands and nipples gave milk in large quantities. There were five nipples on the left and four on the right side. The patient's mother had an accessory mamma on the abdomen that secreted milk during the period of lactation.

Charpentier has observed in his clinic a woman with two supplementary axillary mammae with nipples. They gave milk as the ordinary mammae. Robert saw a woman who nourished an infant by a mamma on the thigh. Until the time of pregnancy this mamma was taken for an ordinary nevus, but with pregnancy it began to develop and acquired the size of a citron. Figure 147 is from an old wood-cut showing a child suckling at a supernumerary mamma on its mother's thigh while its brother is at the natural breast. Jenner speaks of a breast on the outer side of the thigh four inches below the great trochanter. Hare describes a woman of thirty-seven who secreted normal milk from her axillae. Lee mentions a woman of thirty-five with four mammae and four nipples; she suckled with the pectoral and not the axillary breasts. McGillicudy describes a pair of rudimentary abdominal mammae, and there is another similar case recorded. Hartung mentions a woman of thirty who while suckling had a mamma on the left labium majus. It was excised, and microscopic examination showed its structure to be that of a rudimentary nipple and mammary gland. Leichtenstern cites a case of a mamma on the left shoulder nearly under the insertion of the deltoid, and Klob speaks of an acromial accessory mamma situated on the shoulder over the greatest prominence of the deltoid. Hall reports the case of a functionally active supernumerary mamma over the costal cartilage of the 8th rib. Jussieu speaks of a woman who had three breasts, one of which was situated on the groin and with which she occasionally suckled; her mother had three breasts, but they were all situated on the chest. Saunois details an account of a female who had two supernumerary breasts on the back. Bartholinus (quoted by Meckel) and Manget also mention mammae on the back, but Geoffroy-Saint-Hilaire questions their existence. Martin gives a very clear illustration of a woman with a supernumerary breast below the natural organ. Sneddon, who has collected quite a number of cases of polymazia, quotes the case of a woman who had two swellings in each axilla in which gland-structure was made out, but with no external openings, and which had no anatomic connection with the mammary glands proper. Shortly after birth they varied in size and proportion, as the breasts were full or empty, and in five weeks all traces of them were lost. Her only married sister had similar enlargements at her third confinement.

Polymazia sometimes seems to be hereditary. Robert saw a daughter whose mother was polymastic, and Woodman saw a mother and eldest daughter who each had three nipples. Lousier mentions a woman wanting a mamma who transmitted this vice of conformation to her daughter. Handyside says he knew two brothers in both of whom breasts were wanting.

Supernumerary nipples alone are also seen, as many as five having been found on the same breast. Neugebauer reports eight supernumerary nipples in one case. Hollerus has seen a woman who had two nipples on the same breast which gave milk with the same regularity and the same abundance as the single nipple. The Ephemerides contains a description of a triple nipple. Barth describes "mamma erratica" on the face in front of the right ear which enlarged during menstruation.

Cases of deficiency of the nipples have been reported by the Ephemerides, Lentilius, Severinus, and Werckardus.

Cases of functional male mammae will be discussed in Chapter IX.

Complete absence of the hymen is very rare, if we may accept the statements of Devilliers, Tardieu, and Brouardel, as they have never seen an example in the numerous young girls they have examined from a medico-legal point of view.

Duplication or biperforation of the hymen is also a very rare anomaly of this membrane. In this instance the hymen generally presents two lateral orifices, more or less irregular and separated by a membranous band, which gives the appearance of duplicity. Roze reported from Strasburg in 1866 a case of this kind, and Delens has observed two examples of biperforate hymen, which show very well that this disposition of the membrane is due to a vice of conformation. The first was in a girl of eleven, in which the membrane was of the usual size and thickness, but was duplicated on either side. In her sister of nine the hymen was normally conformed. The second case was in a girl under treatment by Cornil in 1876 for vaginitis. Her brother had accused a young man of eighteen of having violated her, and on examination the hymen showed a biperforate conformation; there were two oval orifices, their greatest diameter being in the vertical plane; the openings were situated on each side of the median line, about five mm. apart; the dividing band did not appear to be cicatricial, but presented the same roseate coloration as the rest of the hymen. Since this report quite a number of cases have been recorded.

The different varieties of the hymen will be left to the works on obstetrics. As has already been observed, labor is frequently seriously complicated by a persistent and tough hymen.

Deficient vulva may be caused by the persistence of a thick hymen, by congenital occlusion, or by absolute absence in vulvar structure. Bartholinus, Borellus, Ephemerides, Julius, Vallisneri, and Baux are among the older writers who mention this anomaly, but as it is generally associated with congenital occlusion, or complete absence of the vagina, the two will be considered together.

Complete absence of the vagina is quite rare. Baux a reports a case of a girl of fourteen in whom "there was no trace of fundament or of genital organs." Oberteuffer speaks of a case of absent vagina. Vicq d'Azir is accredited with having seen two females who, not having a vagina, copulated all through life by the urethra, and Fournier sagely remarks that the extra large urethra may have been a special dispensation of nature. Bosquet describes a young girl of twenty with a triple vice of conformation--an obliterated vulva, closure of the vagina, and absence of the uterus. Menstrual hemorrhage took place from the gums. Clarke has studied a similar case which was authenticated by an autopsy.

O'Ferral of Dublin, Gooch, Davies, Boyd, Tyler Smith, Hancock, Coste, Klayskens, Debrou, Braid, Watson, and others are quoted by Churchill as having mentioned the absence of the vagina. Amussat observed a German girl who did not have a trace of a vagina and who menstruated regularly. Griffith describes a specimen in the Museum of St. Bartholomew's Hospital, London, in which the ovaries lay on the surface of the pelvic peritoneum and there was neither uterus nor vagina; the pelvis had some of the characteristics of the male type. Matthews Duncan has observed a somewhat similar case, the vagina not measuring more than an inch in length. Ferguson describes a prostitute of eighteen who had never menstruated. The labia were found well developed, but there was no vagina, uterus, or ovaries. Coitus had been through the urethra, which was considerably distended, though not causing incontinence of urine. Hulke reports a case of congenital atresia of the vagina in a brunette of twenty, menstruation occurring through the urethra. He also mentions the instance of congenital atresia of the vagina with hernia of both ovaries into the left groin in a servant of twenty, and the case of an imperforate vagina in a girl of nineteen with an undeveloped uterus.

Brodhurst reports an instance of absence of the vagina and uterus in a girl of sixteen who at four years of age showed signs of approaching puberty. At this early age the mons was covered with hair, and at ten the clitoris was three inches long and two inches in circumference. The mammae were well developed. The labia descended laterally and expanded into folds, resembling the scrotum.