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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.
a.n.a.logous 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 a.s.sociated 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 const.i.tuent 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 pa.s.sed 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 a.n.a.logous 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. Sloc.u.m 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 b.r.e.a.s.t.s, 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 cla.s.sify congenital fistulas of the neck.
The proper cla.s.sification 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 cla.s.s 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 a.n.a.logous 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 const.i.tuents to pa.s.s 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 Ma.s.sachusetts General Hospital, gives a numerical a.n.a.lysis 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 pa.s.sed into the right side of the thorax. Macnab reports an instance in which three years after the cure of empyema the whole stomach const.i.tuted 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-gla.s.s 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 cec.u.m, 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 ca.n.a.l. Congenital occlusion is quite frequently reported.
Dilatation of the colon frequently occurs as a transient affection, and by its action in pus.h.i.+ng 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 circ.u.mference 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 a.n.u.s.--Cases in which the a.n.u.s is imperforate or the r.e.c.t.u.m ends in a blind pouch are occasionally seen. In some instances the r.e.c.t.u.m is entirely absent, the colon being the termination of the intestinal tract. There are cases on record in which the r.e.c.t.u.m communicated with the a.n.u.s solely by a fibromuscular cord. Anorectal atresia is the ordinary imperforation of the a.n.u.s, in which the r.e.c.t.u.m terminates in the middle of the sacral cavity. The r.e.c.t.u.m 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 pa.s.sages. Huxham mentions the fusion of the r.e.c.t.u.m and colon with the bladder, and similar instances are reported by Dumas and Baillie. Zacutus Lusita.n.u.s describes an infant with an imperforate membrane over its a.n.u.s who voided feces through the urethra for three months. After puncture of the membrane, the discharge came through the natural pa.s.sage 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 r.e.c.t.u.m may terminate in the v.a.g.i.n.a.
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 r.e.c.t.u.m and the bladder. In Figure 140 a represents the r.e.c.t.u.m; b the bladder; c the point of communication; g shows the cellular tissue of the s.c.r.o.t.u.m.
There is a description of a girl of fourteen, otherwise well const.i.tuted and healthy, who had neither external genital organs nor a.n.u.s. There was a plain dermal covering over the genital and a.n.a.l 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 p.u.b.erty 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-const.i.tuted woman of twenty-two in horrible agony, who they said had not had a pa.s.sage 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 a.n.u.s, but by putting his finger in the v.a.g.i.n.a he could discern the floating end of the r.e.c.t.u.m, which was full of feces. There was an opening in this suspended r.e.c.t.u.m about the size of an undistended a.n.u.s. 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 a.n.a.logue of this case found by Mercurialis in a child of a Jew called Teutonicus.
Gerster reports a rare form of imperforate a.n.u.s, 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 pa.s.sage of meconium through the urethra. Burge mentions the case of what he calls "s.e.xless child,"
in which there was an imperforate a.n.u.s and no pubic arch; the ureters discharged upon a tumor the size of a teacup extending from the umbilicus to the p.u.b.es. A postmortem examination confirmed the diagnosis of s.e.xless child.
The Liver.--The Ephemerides, Frankenau, von Home, Molinetti, Schenok, and others speak of deficient or absent liver. Zacutus Lusita.n.u.s says that he once found a ma.s.s 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 a.s.suming 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 Riola.n.u.s, 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 pa.s.sed 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, d.u.c.h.esne, 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, d.i.c.kie, 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 a.n.u.s; 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 a.s.sociated 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, v.a.g.i.n.a, and Fallopian tubes in the female, and vas deferens or vesiculae seminales in the male, rather than the ovaries or t.e.s.t.i.c.l.es. 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 r.e.c.t.u.m 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 surpa.s.s 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.