Paper - Congenital malformations of the ureters (1912)

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Eisendrath DN. Congenital malformations of the ureters. (1912) Ann Surg. 55(4): 571–592. PMID17862830

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This 1912 paper by Eisendrath describes abnormal development of the ureters.

Also by this author: Eisendrath DN. Congenital strictures and spiral twists of the ureters. (1917) Ann Surg. 65(5): 552–559. PMID17863705

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Congenital Malformations of the Ureters

By Daniel N. Eisendrath, M.D., Of Chicago, Illinois.

  • Read before the Western Surgical Association, December 21, 1911.

Introduction

The object of the present paper is to add another case of congenital malformation of the ureter to the series of four similar cases reported by the writer in two previous articles, published during the past year.?

In order to permit of a survey of the entire five cases, and to bring out the various forms and clinical pictures shown by them, I will give a brief synopsis of the already published first four cases.

1 Surgery, Gynecology and Obstetrics, June, 1911, and Jour. A. M. A, July 8, 1911.

2 Annales des Mal. d. Organes Genito-Urin., vol. xxviii.


Case I. — Congenstal Hydronephrosis of a Horseshoe Kidney

Boy of six, with severe right abdominal pain, septic symptoms, and palpable transverse tumor (Fig. 1) in upper abdomen. Latter at operation seen to be a hydronephrotic horseshoe kidney. Ureter not enlarged. Operation consisted of draining sac. Existence of closure or narrowing of ureter at junction with renal pelvis to be assumed from absence of dilatation of ureters and from similar cases collected by Papin and Christian.2 They found that the cause of the hydronephrosis in fifteen cases of hydronephrotic horseshoe kidneys seen at autopsy was either congenital stricture or faulty insertion of the ureter. In this case the condition had remained latent until infection occurred.

Case II. — Congenital Hydronephrosis Due to Stricture at Ureteropelvic Junction

Boy of twelve, with enlargement of left side of abdomen, only noticed after sixth year (Figs. 2 and 3). No symptoms referable to urinary tract. Soft elastic retrocolic-tumor to be felt to left of median line, extending from costal arch to Poupart’s ligament, and having distinct notch. Urine very turbid and contained many pus corpuscles. No fever or other evidence of infection, only marked malnutrition. At operation an immense hydronephrotic kidney was found. The sac contained two quarts of turbid fluid, which showed no growth on the ordinary media. The pelvis and calyces. were enormously dilated at the expense of the parenchyma (Fig. 4.) The cause of the obstruction was found to be .a sudden narrowing of the ureter (Fig. 5) at its junction with the renal pelvis. It was impossible even to pass a filiform bougie through the ureteral lumen at the point of obstruction. After removal of the kidney, patient made an uneventful recovery, and has remained well. Fic. 2. Fic. 3.

Fig. 1. Outline of hydronephrotic horseshoe kidney on abdomen (Case I).



Front view of abdomen in congenital hydronephrosis (Case 11). Result of inflation of colon in Case I1. Fic. 4.


Front. view of kidney of Case II. This shows the enormous dilatation of the pelvis and the narrow margin of parenchyma with prominences due to dilated calyces. Note narrowness of ureter at junction with pelvis. Fic. s.


Interior view of kidney df Case II. Note narrow rim of kidney tissue, dilated calyces and pelvis, and small orifice of ureter (probe inserted through ureter).

Case III. — Boy of fourteen

Boy of fourteen, previously well, was suddenly seized with left-sided abdominal pain. Latter was most marked in the left iliocostal space and radiated toward front of abdomen. Temperature 101°. Left side of abdomen rigid. Urine contained a few pus corpuscles. Urinary retention was present, so that it was necessary to catheterize. No cystoscopic examination or collargol injection of the ureter was made. Diagnosis before operation was some left intraperitoneal infection, possibly appendix or diverticulitis of sigmoid. Under anzsthesia spindle Fig. 6.


Spindle-like tumor felt on outer side of left rectus {under anesthesia). It tapered toward pelvis. (Case III).

like tumor (Fig. 6) to be felt beneath left rectus muscle, tapering sharply toward true pelvis. When peritoneal cavity was opened, a translucent, elongated, fluctuating enlargement was found lying behind the peritoneum, to the inner side of the descending colon. Ureter exposed through usual oblique incision. Tissues around ureter found cedematous, as if the seat of a recent infection, and upon incision a slightly turbid serum escaped. No cultures were made. Ureter dilated to size of small gut, with walls extremely thin and easily torn. Dilation showed gradual narrowing at ureter; vessel.

IE, iliac art

Author’s case of congenital stenosis of left ureter (see text). B, bladder.

ery; constriction (S

(Semidiagrammat:

Fig. 7.


junction with renal pelvis above, and a quite sharp termination at lower end, just below brim of true pelvis (Fig. 7). From this point downward ureter felt hard and fibrous, but allowed an ordinary ureteral catheter to pass through into bladder. The ureter above this narrower pelvic portion was found to be of tissue-paper thinness, with markedly oedematous and reddened mucous membrane. At about the middle of the enlargement two distinct mucous valves were seen, which did not cause any obstruction. Ureter at first drained, but recurrence of fever, which became quite high, necessitated removal of dilated ureter and kidney. The ureter was distinctly narrowed at its junction with the renal pelvis (Fig. 7). There were. marked evidences of a recent pyelonephritis, many groups of small abscesses being scattered over the renal cortex. Patient made an uneventful recovery, and has remained well up to present time (eighteen months after operation).

Case IV. — Specimen of kidney

loaned by Dr. I. A. Abt.

Kidney, ureter, and bladder from a child, ten days old, who had died of icterus neonatorum. The left ureter (Fig. 8) showed two distinct points at which its lumen was impermeable, even to the very smallest bougie. The proximal of these two strictures of the left ureter is one-half inch below the renal pelvis. The distal stricture of the left ureter is one-eighth of an inch above the bladder. A similar proximal stricture was found in the right ureter, five-eighths of an inch below the ureteropelvic junction. There is no distal stricture of this left ureter. There is a marked spindle-like dilation of the ureter, and there is also marked dilation of the ureter and renal pelvis (moderate hydronephrosis) on both left and right sides above the proximal strictures.

The following case was observed since the publication of the preceding four:

Case V. — Girl of sixteen

Girl of sixteen, but with physical and mental development of child of six, was admitted to the service of Dr. I. A. Abt, in the Michael Reese Hospital. She had always been backward in intelligence and size, and showed all of the stigmata of cretinism. Her history was that of the sudden onset of severe pains in the abdomen, accompanied by frequent vomiting. This condition had lasted for about two weeks prior to her admission.


The abdomen was enlarged and soft. There was tenderness and a feeling of resistance, or of a mass over the right kidney, but nothing definite. No evidence of free fluid to be found. She was transferred to the surgical service of the writer, with the diagnosis of a probable right kidney tumor. My examination, in addition to confirming the presence of marked retardation of mental and physical development, showed the presence of a suprapubic soft enlargement, which entirely obscured the possibility of palpating the tumor previously thought as present by Dr. Abt. Owing to her irritability and the inability to reason with her, it was impossible to obtain any urine or to make a satisfactory abdominal examination until she was anesthetized. Upon inserting a catheter, a very large amount of turbid urine was evacuated and the greater part of the suprapubic enlargement disappeared. Our suspicion entertained before anesthesia, that the suprapubic enlargement was due to a greatly distended bladder, seemed at first to be confirmed. But in spite of suprapubic pressure, the catheter being left in the bladder, some enlargement remained, and I decided to open the abdomen in the median line and explore the peritoneal cavity. This was done and showed that both ureters were dilated, the left one far more so than the right. The left ureter could be seen to be about the dimension of the adult thumb. The enlargement seemed to be uniform, 1.e., it extended without any narrowing to the kidney above and bladder below. The corresponding (left) kidney was the size of the adult fist and markedly hydronephrotic, showing a number of translucent prominences corresponding to dilated calyces. The right ureter was about the size of a lead pencil, and much less translucent than the left one. The right kidney was only slightly hydronephrotic. No cause for the obstruction in the left ureter was found proximal to the bladder. The latter could still be felt to be considerably enlarged, so the first incision was closed and a second lower extraperitoneal opening made, just above the pubes, the bladder brought into wound, and opened. A very large quantity of turbid urine escaped. The walls of the bladder were greatly hypertrophied and a projection into the lumen was to be distinctly seen on the left side (Fig. 9). It looked like an elevation of the bladder wall covered by smooth mucosa, which seemed to fill up almost the entire bladder, projecting beyond the median line and Fig. 8


Specimen obtained at autopsy from Dr. Abt’s case of infant, 10 days old, who had died of icterus neonatorum. Note narrowing of ureter just above bladder and again just below renal pelvis. Proximal to each one of these narrowings or stenoses is a marked discoloration. (Case IV.) Fig. 9.


Appearance of bladder at operation. Cystiform protrusion seen on left side (retracted so as to show relation to right ureteral orifice and to internal meatus). (Case V.)

anteriorly, so that it was necessary to hold it back with a retractor to expose to view the right ureteral orifice and the internal meatus, both of which this prominence covered.

It looked not unlike the bulging of a rectocele, seen in a case of marked perineal tear. Examination of the right ureteral orifice and internal meatus showed that both were permeable in a normal manner. Most careful search was made for a left ureteral orifice, but none found. A diagnosis of cystiform protrusion of the lower end of a congenitally closed ureter was made, and a plastic operation begun, so as to create a permanent left ureteral opening into the bladder.

The mucous membrane covering the cystiform protrusion was incised and the greatly dilated ureter exposed. This was opened and divided transversely into a proximal and distal portion, the point of division being about one and a half inches above. the lower end of the dilatation. The new ureteral openings were formed by uniting the mucous membrane of the bladder, as shown in Fig. 10, with the wall of the divided ureter. Although a probe was shown to have a wide range of movement in the distal portion of the cystiform protrusion of the ureter, no communication with the bladder lumen was found. A case of Bostrom’s, reproduced in Fig. 16, will give one the best idea of the condition, as found by us at operation. The ureter opened widely into the protrusion, and I thought it advisable to establish a permanent means of communication (Fig. 10) between the distal portion and the bladder lumen, in order to permit of drainage of this distal pocket. It would, of course, have been ideal to make the new and only ureteral opening at the distal end of the dilatation, but it was impossible to find this end until we had made our incision through the posterior bladder wall and opened the ureter at a point a little higher up. This case, so far as I can learn, is the first one in which an intravesical ureterostomy for a congenital cystiform protrusion was performed. That the opening remained perfectly patent was shown by the fact that it was found to admit a No. 5 English soft rubber catheter at the autopsy, held nearly four months after this ureterostomy operation.

The only manner in which I can explain the retention of urine, resulting in the suprapubic tumor felt before this first operation, is that the cystiform protrusion, filled with urine, so filled up the bladder lumen as to obstruct, not only the internal meatus, but also the right ureteral orifice. Before we could expose the right ureteral orifice, in order to determine its permeability to a catheter after opening the bladder, we were obliged to strongly retract the cystiform protrusion to the left. It is my opinion that the urine from this left kidney probably passed by filtration into the bladder lumen or through some minute opening which we did not find. It would seem reasonable to expect a very much more marked hydronephrosis on the left side, if none of the urine had escaped during the sixteen years of her life.


Fig. 10. Plastic operation to correct cystiform protrusion. I, course of both ureters before ureterostomy (note dilated lower end of left ureter). II, method of suturing proximal and distal portions of ureter into bladder.


After this ureterostomy, on May 20, 1911, both laparotomy and suprapubic wounds healed very slowly. From May 20 until June 20, the temperature was practically normal, seldom rising as high as 100° F. Beginning June 20, the temperature began to rise, reaching its first high point on June 26 (102.5° F.).


From this date to July 20, there were four febrile periods, lasting on an average from two to four days, with intervals of two to three days. The diagnosis of ascending infection, from the bladder, of the left kidney was made. An exploratory operation to determine the condition of this kidney was decided upon. This was performed on July 25, 1911.

The kidney was brought into the abdominal wound. Its cortex showed a very large number of deep hemorrhagic spots, and at the upper pole there were a number of pin-point yellowish pus foci. Both of these findings confirmed the diagnosis of a pyelonephritis. It was at first thought possible to save the kidney by simply incising these pus foci, but owing to the spiral twist of the ureter described below, this was deemed inadvisable. It was found that the pelvis of the kidney was quite large compared to the size of the kidney itself. This enlargement extended down into the upper portion of the ureter, which was also greatly dilated down to a point about 4 cm. below the kidney. The form of the enlargement was like that of the bowl of a pipe. The enlargement stopped suddenly at a point where the ureter seemed obstructed by a complete spiral turst of over 180 degrees* (Fig. 11). There was a second spiral twist about 2 cm. below this one. On account of the infected condition of the kidney and the difficulty of performing a plastic operation to join the ureter at a point below its second spiral twist with the pelvis of the kidney, it was deemed best to perform a nephrectomy. Before performing this, search was made for the possibility of an accessory vessel which could cause an obstruction of the renal pelvis, but none was found. The description of the removed kidney and removed ureter is as follows:

Left kidney and ureter removed July 25, 1911.

Ten cm. long by 4.5 wide by 2 cm. thick. Fetal lobulations were well marked. The cortex showed innumerable petechize at the upper pole and there were a number of pin-point areas which, on section, were seen to be small pus foci in the cortex. Surface of cortex rough and somewhat nodulated. Section showed renal pelvis greatly dilated and a moderate degree of hydronephrosis.


Both the upper or major and the lower or minor twists were not the result of traction. They were demonstrated to a number of visitors, with the kidney in its normal position, without the least degree of torsion of the same (Fig. 11).

Pelvic mucous membrane smooth and shiny, except for a few punctate hemorrhages. Dilatation of pelvis extended into upper portion of ureter for a distance of nearly 4 cm.

The ureter showed an enlargement extending to about 4 cm. below kidney, resembling the bowl of a pipe, just distal to which was a peculiar twist in the ureter spiral, to the long axis of the ureter, so that when ureter was placed in the position occupied in the body there was an almost complete closure of ureter at this point, due to the spiral twist of nearly 180° and enlargement above it. There was a second spiral twist in the ureter, about 3 cm. below the one described above, and a tendency to a third a similar distance lower down. From this point toward the bladder, the ureter was quite large and showed no twists. The walls were thickened. Upon inserting a probe from the lower end of the ureter upward, one met with obstruction at the above-described twists, especially the one nearest to the renal pelvis.

Post-operative Notes—The kidney wound healed without complications. The patient, however, continued to look very pale, although passing a large quantity of urine, which, microscopically, showed a considerable number of pus corpuscles and traces of albumin. The microscopic examination of the tissue of the left kidney showed a high grade of chronic diffuse nephritis, with small focal areas of polymorphonuclear leucocytes. Patient continued to feel well, being up and about, and shown in clinic a number of times. She was discharged from the hospital on October 15, apparently greatly improved, but returned on October 21, with symptoms of laryngeal stenosis and pulmonary cedema, due to uremia, with resultant general anasarca, and died on October 25, 1911.

The autopsy showed an absence of the thyroid, thus confirming our clinical diagnosis of cretinism. I shall only add the report on the autopsy specimen of the right kidney, right ureter, and stump of the left ureter (Figs. 12 and 13).

The stump of the right ureter was two and a half inches long. From a point about one-quarter of an inch above its entrance into the bladder wall it became dilated. The convexity of the dilatation was toward the left; in other words, not symmetrical. The concave surface of the dilatation in this ureter faced the concave surface of the dilatation in the right ureter. The diameter of the dilated portion of this ureter at its widest point when filled with paraffin was almost one-half inch. Where it narFic. 11.


Kidney from Case V showing spiral twist of ureter. DL, dilated ureter above point of stenosis; ST, spiral twist in ureter, causing stenosis. Fig. 13.

Fig. 12.


Congenital stenosis of the ureter (Case V). Note Right kidney, ureter and bladder (removed at constrictions at A and C, and dilatation at B. E is autopsy of Case vi. Bladder opened to show patstump of left ureter; D, right kidney. ent ureterostomy (left) and dilated right ureter and kidney. Fig. 14.


Case showing protrusion into bladder (Johnson’s case). Right kidney enlarged and its pelvis distended. Right ureter dilated and curving. Double ureters on left side, both dilated and tortuous. Protrusion extends from left ureter into urethral canal (see text). Fig. 15.


Congenital closure of left ureter, with protrusion of closed vesical end into bladder. Enormous dilatation of left ureter. Marked atrophy of left kidney. (Bostrom’s case.) Fig. 16,


Cystiform projection of right ureter into bladder. Ureter gpens widely into protrusion. (Bostrom’s case.) (Type of Case V.) rowed just before entrance into the bladder wall, it was one inch in diameter. The description of the vesical orifice of this ureter will be found under the description of the bladder. The right kidney was two and three-quarter inches long, one and one-half inches in width, and threefourths inch in thickness.

The kidney showed no abnormalities in shape; the fetal lobulations were still quite marked. The true capsule was quite adherent, being almost impossible to strip. Distributed over the cortex of the kidney were a number of pin-point, yellowish areas, which upon section were seen to be small collections of pus. <A similar condition was found at the operation of July 25, 1911, in the left kidney which had been removed. In addition to these yellowish areas, the surface of the kidney showed a number of pale yellowish elevations. The surface resembled that of a chronic interstitial nephritis. On section of the parenchyma, considerable purulent fluid escaped from the pelvis and dilated ureter. The cut surface of the kidney showed that the pelvis of the kidney was greatly dilated at the expense of the parenchyma. The calyces were markedly dilated and contained a turbid yellowish fluid. The pelvic mucous membrane lining these dilated calyces showed intense injection of the blood-vessels, and in places there was a considerable velvety appearance of the mucosa, 4.e., the typical picture of a chronic pyelitis. The parenchyma on section seemed to be present only to a small degree, maximum width being one-quarter inch. The parenchyma, wherever present, showed marked glomerular injection. The right ureter showed a marked constriction close to the point of entrance to the bladder. This narrow point corresponded to that on the left side, about one-quarter inch in diameter, and extended about one-half inch in a vertical direction.

From this point upward, for a distance of nearly four inches, occurred the first spindle-like dilatation of the ureter. The concavity of the spindle was toward the median line. Just above this point the ureter again narrowed and there was an incomplete spiral twist at this point. This was followed by a second spindle-like dilatation, which was shorter and ended about one and a half inches below the ureteropelvic junction. At this point there was a second less complete spiral twist.

Bladder specimen: Internal meatus readily admitted a No. 5 soft rubber catheter. The right ureteral orifice was distinctly visible, and showed no abnormalities and easily admitted ordinary probe. The left ureteral orifice corresponded to the point where the previous plastic operation had been performed. This readily admitted a No. 5 soft rubber catheter. Dissection of the bladder wall for traces of a previous distal pouch corresponding to the lower end of the cystiform protrusion of the ureter was negative. This was, in all probability, due to the fact that between the date of operation of the ureter and the date of dissection of the specimen (six months), cicatricial contraction of this segment had caused complete obliteration of its lumen. There were absolutely no traces of any other ureteral orifice, which confirmed the observation made at the first operation, that no left ureteral orifice could be found, the orifice being simply represented by a little dimple covered with mucous membrane. The bladder walls showed marked hypertrophy, as though great difficulty had been encountered in evacuating the urine, due no doubt to obstruction of the internal meatus by the cystiform protrusion.

Review Of Literature

In my previous article I expressed my indebtedness to Dr. John T. Bottomley for the painstaking manner in which he collected all of the cases published up to the date of appearance of his article upon “‘ Congenital Strictures of the Ureter,”’ published in the ANNALS OF SuRGERY, November, 1910. In order to better understand the nature of these congenital lesions of the ureter, I shall briefly review some of the chief points of my first paper.

Etiology

This is not quite clear, but is best explained as either the result of the persistence of fetal conditions or a retardation of development of structures destined to perform important functions.

Persistence of fetal conditions:

Seitz, Byron Robinson, ‘Hamann, and others have shown by their studies of the normal ureter, in the foetus and at different periods of post-uterine life, that the ureter in all mammals possesses certain more or less constant points of narrowing and of dilatation. In addition, the occurrence of valves and of twists or tortuosities is not at all uncommon, especially in the fetal ureter. Figs. 17, 18, and 19 show where these various conditions most frequently occur. The points of narrowing are as follows:

  1. Just before the ureter enters the bladder wall (C in Fig. 17).
  2. Where the ureter crosses the pelvic brim (B in Fig. 17).
  3. Close to the pelvis of the kidney (4 in Fig. 17).


These three points of narrowing become more marked toward the end of fetal life, as Seitz has shown, and were constantly found in the normal adult ureter by Robinson.


The dilatations are always found on the proximal side of these narrowings in the normal human foetus. The most marked widening is situated in the middle third of the ureter, i.¢., proximal to the narrowing which occurs at the pelvic brim or between it and the bladder wall.


Fig. 17. Fetal ureters (Seitz) showing normal constrictions (A. B, and C) and normal dilatations on heir proximal side.

Fig. 18. Case of double ureters showing twists and dilatations (Byron Robinson).

The spiral twists occur at any point, but are most marked at the junction of the upper and middle thirds of the ureter, as in the writer’s case (Case V). The rdle which valves play in the production of stenoses is not quite clear. They are found in about 20 per cent. of cadavers of normal children, and contain mucous membrane, submucous and muscular tissue. In some cases the ureter is stenosed at the point where valves are found, and in all probability the valves play a rdle here.

Clinical Importance

The presence of the narrowings, twists, and valves, as found in the normal fetal and adult ureter, was formerly thought to be of interest to the anatomist and pathologist alone. Although a number of the cases collected by Bottomley were reports of specimens found at autopsy, yet in quite a large number the persistence of these congenital conditions gave rise to symptoms requiring operative measures. We shall see later that there are many cases which remain latent throughout life, and, on the other hand, quite a number in which infection or the presence of a tumor cause symptoms which draw the clinician’s attention to the case.

In several of the cases collected by Bottomley, a portion of the ureter was wholly fibrous, while in others the ureter was impermeable throughout and was represented only by a fibrous cord, a functionless ureter. Teyssédre* collected reports of eleven cases. Such a fibrous ureter may lead to absence of the kidney or of the whole or a portion of the ureter. In eight cases of Bottomley’s series the stricture was located in the upper third of the ureter, usually at or close to the opening into the renal pelvis.

In 36 of 56 cases the obstruction was in or very close to the bladder; 17 of Bottomley’s cases had vesical protrusions, 39 did not. Nichols’s and Abt’s (Fig. 8) cases showed a stricture both at the upper and at the lower end of the same ureter. The same ureter may present alternating portions with and without a lumen.

The most important, and, in their effects, the most far reaching modifications of form are seen in the obstructions at the lower end of the ureter. In these the ureter usually reaches the bladder, but in most instances ends there in a blind sac. This blind end may be (a) just beneath the mucous lining of the bladder, (b) it may lie in the muscular layer of the bladder wall, or (c) may just reach the outer wall. If the blind end is in the muscular layer and one examines the bladder from within, there will either be no trace whatever of a ureteral opening, or in its place will be seen a dimple, a shallow invagination of the mucous membrane.


  • Thése de Paris, 1892. Sagittal section of adult human ureter, showi formation (Byron Robinson).



Fig. 19.


The most interesting forms, however, have to.do with the ureters ending just beneath the vesical mucous membrane, as in my Case V. These produce a cyst-like protrusion of the mucous membrane into the vesical cavity. The protrusions range in size from that of a small pea to one completely filling the bladder lumen; the smaller are usually hemispherical in shape; the larger are either triangular or finger-shaped, sometimes reach the vesical opening of the urethra, may pass varying distances along the course of the latter, and in women may even appear at the external meatus. When empty, they are flat and flaccid; when full, they are finger-like, pearshaped, and tense, broader and thicker at the base, gradually tapering and thinning in the direction of the tip. They vary in length from 0.5 cm. to 6.5 cm. The ureteral opening into these pouches may be bristle-like or may be an aperture 2 cm. in diameter. Usually (in 12 out of 17 cases) the protrusions are blind cul-de-sacs without an opening into the vesical cavity. In five cases there was a communication between the protrusion and the vesical cavity, almost invariably by minute openings at the tip or on the side of the former. The contents of the protrusions is usually a clear fluid. It may vary considerably in color. Sometimes it is muddy or cloudy.


Effect on Ureter — When the obstruction is at the lower end, the ureter is almost invariably dilated, thin-walled, and tortuous. It varies in size from that of a pencil to that of the small intestine (Fig. 7). It becomes widened and lengthened. Occasionally it is lobulated, shows windings and twistings (Figs. 11, 12, and 13), the latter also causing obstruction as in my fifth case.

Effects on the Renal Pelvis and Kidney. — Either an enormous hydronephrosis or a most marked primary atrophy may result from ureteral obstructions of the same location and character. Hydronephrosis of varying degree is the rule. It may show itself simply as a slight or a moderate distention of the pelvis, as in Case III, or it may present as an enormous cyst filling the whole abdominal cavity, with only remnants of the true kidney tissue in the cyst wall, as in Case II. Infection produces its usual disastrous effects, and in some cases completely disorganized kidneys are found.

Effects on the Bladder. — The cystiform protrusions of the blind ending ureter into the bladder may cause marked secondary pathologic changes in the bladder, as well as in the other ureter, or in the other kidney, as in Case V. The protrusion may block the opening of the opposite ureter. In other cases it may wholly obstruct the vesical opening of the urethra, give rise to a distended hypertrophied bladder, and then the usual sequelz of retention of urine result. Both the right ureteral orifice and the internal meatus urethre were blocked by the cystiform protrusion in Case V and the bladder walls greatly hypertrophied.

Symptoms aNnp D1agnosis. — In Bottomley’s series, 19 cases gave either subjective or objective symptoms. Many cases occurred in subjects too young to make complaints. Hydronephrosis of great size and protrusions filling the bladder may exist for years without symptoms, unless infection occurs.


A survey of all the published cases of congenital anomalies of the ureter shows that clinically they belong to one of four classes: (1) Those in which the condition remains latent throughout life and is an accidental finding at autopsy. The majority of the published cases belong to this class. (2) Those in which the presence of an abdominal tumor, which may or may not be diagnosed before operation as being of renal origin, is found when the abdomen is opened. My first and second cases belong to this class. In the first case the boy was thought to have an appendiceal abscess, on account of the persistence of the septic symptoms and the prominence of a tumor in the right hypochondrium, which was found at operation to be a hydronephrosis of the right half of a horseshoe kidney. My second case was diagnosed before operation as being a renal tumor, and in all probability a hydronephrosis. The probable congenital origin of the hydronephrosis was also diagnosed before operation, on account of the age of the patient—twelve years. The operation verified this diagnosis. (3) Cases in which the predominant symptoms are those of an abdominal infection. The clinical picture may resemble that of an acute infection of one of the intraperitoneal viscera, or it may point more directly to the kidney and ureter as the source of the symptoms of infection. My third case resembled a case of Bottomley’s in presenting the picture of an intra-abdominal infection whose nature could not be accurately determined until operation. In my own case (Case III) the symptoms had begun very acutely, with pain and rigidity in the left half of the abdomen, and only under anzsthesia was it possible to palpate what would seem to be rather a characteristic finding in these cases when it is present. This was also present in Bottomley’s case. This is the palpation of a sausage-shaped tumor extending from the costal arch toward the pelvis and tapering somewhat from above downward. In cases where one suspects a dilated ureter and such a finding is present, one could easily confirm the diagnosis, if operative interference were not.urgent, by filling the ureter with collargol solution and then taking an X-ray. (4) Those cases in which disturbances of micturition are the principal symptom. This consists either of increased frequency or retention. My fifth case belongs to this class. The principal symptom was a suprapubic enlargement, which was deter590 DANIEL N. EISENDRATH.

mined to be only in part due to a distended bladder. The catheterization in this case showed that there was still a tumor present, in spite of the evacuation of a considerable quantity of urine. In such a case or wherever there is a history of increased frequency of micturition in children, not due in all probability to any other cause, one should always bear in mind the possibility of a protrusion of the closed or almost closed lower end of the ureter into the bladder—what has been spoken of frequently in this article as a cystiform protrusion.

The examination of the urine is of little value, unless infection has supervened, and even then there is nothing specific to be found. In the second and fifth cases the urine contained a considerable number of pus corpuscles, without any active evidences of infection.

I have noticed one striking feature, and that is that these children who have congenital lesions of the ureter and kidney seem not to be as well developed as other children of the same age.

In no case thus far published, with the exception of my second case, was a diagnosis of congenital lesion of the ureter made before operation. I do not doubt, however, that since the attention of surgeons has been directed to these malformations of the ureters by the articles of Bottomley and the writer, that the condition will be more frequently recognized in the future. No doubt many cases will be fecognized before operation, and at least the possibility of the presence of a congenital malformation be suspected, when a hydronephrosis is found in children. Cystoscopic examination will, of course, show the presence of cystiform protrusion, and it will be impossible in such cases to enter the ureter on the side of this protrusion, in many cases. When there is a ureteral opening present and the stenosis is higher up than the bladder, it may be possible in the future to distend the ureter with collargol and thus make a diagnosis from the radiographic examination.

Treatment

The treatment depends, first, upon the degree of stenosis; second, upon the damage to the kidneys due to the ureteral obstruction; and, third, upon whether infection is present or not. In the latter class of cases nephrectomy is, as a rule, the only method of treatment. If the kidney has not been too greatly damaged, it may be possible to perform a pyelo-ureteroplasty or implant the ureter into the bladder from a point above the stenosis. Some work has been recently done toward making artificial ureters, and I had intended in my second case to attempt the implantation of the Fallopian tube from a dog, in order to make an artificial ureter, but this was impossible, on account of the presence of pyelonephritis on the side of the stenosis. Cases have been operated on by Drew, Dudley Allen, Whipple, Mayo, Nichols, Bottomley, and myself. Of Drew’s cases, one was cured and the other relieved by pyelo-ureteroplasty, the congenital stricture being near the pelvis in both cases. It was my intention to perform an operation of this kind in my fifth case, but the infected condition of the kidney and the complete twist in the ureter seemed insurmountable obstacles. In cases operated on by Dudley Allen and Whipple, nephrectomy was performed in both cases, successfully in Whipple’s case, but in Allen’s case a pyelonephritis developed and the patient succumbed. Mayo’s, Nichols’s, Bottomley’s, and my first three cases are the only ones in which successful removal of both cause and effect by nephrectomy was accomplished. In my fifth case the patient seemed to recover from the ureterostomy, but nephrectomy was later necessary, on account of the infected condition of the kidney of the same side and the coincident twist of the ureter, shown in Fig. 11.

There are no records of any cases of operation in cystiform protrusion of the lower end of the ureter, and the ureterostomy performed in my fifth case is, so far as I can learn, the first report of an attempt to correct the protrusion of the lower end of the ureter in a congenital case. The autopsy in this case showed that the ureterostomy opening had remained patent and had it not been that the ureter was again obstructed by the twist close to the renal pelvis I have no doubt but that recovery would have been a permanent one. For the first four weeks after operation the patient seemed to be in excellent condition. During the second four weeks the symptoms of infection due to pyelonephritis of the kidney of this side presented. Following her nephrectomy, two months after the ureterostomy, she had apparently made an excellent recovery until the uremic symptoms suddenly appeared three months later.

Conclusions

  1. Congenital malformations of the ureters were formerly thought to be only of interest to the pathologist.
  2. The surgical importance of these malformations is just beginning to be appreciated.
  3. Four of my cases were found to give rise to clinical symptoms sufficient to require operation. The fifth case was a specimen obtained at autopsy.
  4. The clinical picture in these cases varies. In some the abdominal tumor is the most prominent symptom. These are the cases which are generally spoken of as congenital hydronephrosis, where the obstruction is close to the renal pelvis. A second clinical variety is that in which the sausage-shaped tumor extends from the costal arch downward. A third variety is where a protrusion of the lower end of the ureter into the bladder exists. A fourth variety is where the clinical picture is that of a renal infection. Some of these cases may be very difficult to diagnose on account of their resemblance to other varieties of intra-abdominal infection.
  5. The origin of all congenital malformations of the ureter is undoubtedly a persistence of fetal conditions. The principal varieties found at operation and autopsy are these: (1) no lumen for the entire length; (2) narrowing or localized absence of lumen at (a) ureteropelvic junction, (b) upper third alone, (c) both upper and lower thirds, (d) vesical end (cystiform protrusion); (3) spiral twists, usually one or more; (4) in none of the cases was the lesion due to any accessory renal vessels or any extra-ureteral inflammatory process,



Cite this page: Hill, M.A. (2021, May 12) Embryology Paper - Congenital malformations of the ureters (1912). Retrieved from https://embryology.med.unsw.edu.au/embryology/index.php/Paper_-_Congenital_malformations_of_the_ureters_(1912)

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