Paper - Congenital strictures and spiral twists of the ureters (1917)
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Eisendrath DN. Congenital strictures and spiral twists of the ureters. (1917) Ann Surg. 65(5): 552–559. PMID17863705
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Congenital Strictures and Spiral Twists of the Ureters
By Dantiel N. Eisendrath, M.D. or Cuicaco, I.
- Read at the March 15, 1917, Meeting of the Chicago Urological Society.
In my first and second papers? upon this subject, I reported four cases of congenital strictures of the ureter, and in a third communication? a fifth case was added. Of the first four cases, the stricture had not given rise to any clinical symptoms in one case, the specimen being obtained at the autopsy of a ten-days-old infant, who had died of icterus neonatorum. A second case of the first four occurred in onehalf of a horseshoe kidney, and it was not clearly established at the time of operation whether the hydronephrosis was the result of a stricture at the ureteropelvic junction or of a faulty insertion of the ureter. In view of these facts, I have eliminated the two cases from my list and shall only consider as true congenital strictures the remaining three of my former cases which caused clinical symptoms, and can now add a fourth recent case to those previously reported.
A brief résumé of the most important facts in regard to my first three cases may be of interest before the details of the fourh case are given.
Case I. — Boy of twelve. Referred on account of gradually increasing size of abdomen for past six years. Latter greatly enlarged, especially in left half, by soft elastic tumor lying behind inflated colon. No clinical symptoms except those of increased intra-abdominal pressure. At operation left kidney was found converted into an enormous hydronephrotic sac. Cause of obstruction was stricture of the ureter close to renal pelvis, which did not even admit a filiform bougie (A in Fig. 1). Uneventful recovery after nephrectomy.
Case II. — Boy of fourteen, previously well, had sudden attack of left-sided abdominal pain with fever, localized rigidity and tenderness. Under deep anesthesia, spindle-shaped swelling felt behind left rectus muscle. Upon opening abdomen, ureter found size of small intestine, very thin-walled and containing turbid urine. Local evidences of peri-ureteral infection. Ureter showed two points of narrowing, one close to renal pelvis of moderate degree (B in Fig. 1) and a second one just above bladder. At the latter point the ureter suddenly changed (B in Fig. 1) in size and became a fibrous cord for a distance of one inch. Through this stricture a very fine probe could still be passed. The kidney showed a moderate degree of hydronephrosis, but in the cortex
Fig. 1. — A, exterior and interior of kidney from Case I, showing location of stricture at ureteropelvic junction (1). The narrow opening of the ureter into the x renal pelvis is shown at X near 3 in lower illustration of A. 2 represents are inserted through the stricture into dilated renal pelvis. B, diagrammatic view of i, showing slight nar rowing of the greatly dilated left ureter (2), at ureteropelvic junction (2), and almost complete obliteration just above bladder (3). there were a number of small abscesses. Recovery after nephrectomy and ureterectomy.
Case III. — Girl of sixteen with stigmata of cretinism was admitted to the hospital on account of severe pains in abdomen accompanied by vomiting. These symptoms had appeared suddenly about two weeks before admission. At the first examination an enlargement in the suprapubic region was felt and an ovarian cyst with twisted pedicle suspected, but the tumor disappeared to a great extent shortly after a catheter was introduced and a large amount of turbid urine obtained. A suprapubic swelling still remained however. A cystoscopic examination was considered in
Fig. 2. — C, diagrammatic view of Case III. The'cystic dilatation of the vesical end of the left ureter with a narrowing as it passed through wall of the bladder is shown at 1. The spiral twist of the same ureter is shown at 2, and the narrow portion of the ureter at the ureteropelvic junction is at 3. The greatly dilated right ureter (4) was moderately stenosed above bladder, the chief cause of obstruction on right side being pressure of the intravesical cystic dilatation of the left ureter upon the right yreteral orifice. D, Case IV (see also Fig. 3) with impermeable stticture at 1 and narrowings at 2 and 3. advisable, so abdomen was opened in median line. Both ureters and kidneys were found much enlarged (C in Fig. 2). The left ureter ended in a blind sac within the bladder and was dilated as far up as its junction with the renal pelvis, where a marked spiral twist of the ureter acted as a second cause of obstruction (Fig. 3).
Fig. 3. — Ureter of Case III, showing spiral twist at junction of upper and middle thirds, with resultant obstruction of renal pelvis.
Fig. 4. — Photograph of kidney and ureter of Case IV. Note advanced degree of hydronephrosis with constriction of ureter of moderate degree close to renal pelvis and complete obliteration of lumen above the bladder. The ureter just above this impermeable stricture was the size of the small intestine.
Fig. 5. — Two specimens showing congenital strictures of the ureter obtained at autopsies of infants by Dr. H. O. Mertz of La Porte, Ind. Specimen on left shows stricture near bladder and just below renal pelvis in left ureter. Specimen on right shows marked dilatation of entire left ureter and renal pelvis with closed lower end of ureter, as in author’s Case III. A plastic operation was first performed on the closed vesical end of the ureter, and later a ureteronephrectomy on this side. Patient died of urzemia five months after the second operation. The right ureter showed, at autopsy, a narrowing just above the bladder with marked dilatation of the remainder of the ureter and of the renal pelvis.
A fourth case has been recently observed which I desire to add to the three preceding ones.
Case IV. — Male, aged twenty-six, referred by Dr. I. B. Diamond of Chicago, with diagnosis of left-sided renal infection. History of recurrent attacks of left-sided abdominal pain since age of fourteen. At first, the attacks would recur at intervals of six to eight months, the pain being of a dull aching character, radiating from a point a little to the left of the umbilicus towards the left kidney region and lasting about six to seven hours. During these attacks he noticed considerable left-sided abdominal enlargement with vomiting and marked constipation. He had never observed any disturbances of urination. He had only two attacks during the five years preceding the one for which he was sent to hospital. One of these two attacks had continued for nearly two months,
Upon admission to my service at the Michael Reese Hospital on February 14, 1917, he stated that the present attack of left-sided abdominal pain had begun suddenly about two weeks before. Examination revealed a soft elastic enlargement on the left side of the abdomen, extending from the level of the anterior superior spine of the ilium below to the costal arch above and disappearing under the latter. The ability to palpate the rounded lower and inner borders of the soft elastic enlargement and its location behind the inflated colon led me to conclude that a very large hydronephrotic sac was present, due to some form of ureteral obstruction. There was a moderate degree of leucocytosis and rise of temperature. Radiography of the urinary tract was negative. Cystoscopic examination showed the bladder to be normal except for a slightly prominent left ureteral orifice. Catheterization of the right ureter was easily carried out and showed a normal, functionating kidney with an excretion of 24 per cent. phthalein in the first hour. On the left side there was complete obstruction of the lumen of the ureter at a point just above the bladder. Even a filiform ureteral catheter could not be passed through the strictured portion ; so all attempts to make a ureterogram were abandoned. The diagnosis was made of a congenital stricture of the ureter in its pelvic portion with secondary hydronephrosis.
At operation (February 18, 1917) we exposed the kidney through the usual incision. The kidney was represented by a hydronephrotic sac which extended from the brim of the true pelvis to the diaphragm and forward to the median line. The fluid within this enormous kidney was under great tension and had to be evacuated (as in Case II) before the thin-walled sac could be brought into the incision. The ureter was distended to the size of the small gut down to a point about one inch above the bladder, where it terminated abruptly in an impervious fibrous cord (Fig. 4).
The kidney and entire ureter were removed and the wound closed in usual manner. No accessory arteries were found. The kidney after removal represented an extreme degree of hydronephrosis, the cortex being so thin that it would have ruptured spontaneously unless operation had been done. The walls of the greatly dilated ureter were relatively thick. There were two points of slight narrowing above the seat of stricture, one of these being close to the renal pelvis and a second where the ureter crossed the iliac vessels. At the lower end, however, the lumen ended abruptly, and the fibrous cord-like portion which represented the stricture had no lumen. There were no valves in the lumen and no spiral twist in the portion above the stricture as in Case III.
Review of chief points of interest in author's four cases:
1. Location of stricture. In Case I, there was a slight one close to renal pelvis and a second one, more marked but permeable, just above the bladder. In Case II the stricture was impermeable and located at the ureteropelvic junction. In Case III there was an intravesical pouch formed by the obliterated lower end of the ureter and a second obstruction in the form of a spiral twist just below the kidney. Case IV greatly resembles the second case, the stricture being located in the juxtavesical portion of the ureter, but being impermeable.
2. Effect on ureter and kidney. Except in Case II, where the stricture was near the kidney, the ureter was greatly dilated in all. The hydronephrosis was most marked in Cases II and IV, where the strictures were either impermeable or nearly so.
3. Clinical symptoms and pre-operative diagnosis. The first symptoms appeared at puberty in all of the cases and were due to infection. Abdominal pain, sudden in onset, dominated the clinical picture of infection except in Case III, where the suprapubic enlargement was a very striking finding. A diagnosis before operation of congenital stricture of the ureter with secondary hydronephrosis was only made in Case IV. In Case II, the diagnosis was hydronephrosis, but the ureteral stricture was not recognized until operation.
4. Effects on bladder. No bladder symptoms except in Case III, where the intravesical cystic dilatation of the left ureter gave rise to retention and also to pressure on the right ureteral orifice and favored ascending renal infection on that side.
Review of Literature. The most complete article upon this subject is that of Bottomley * in which fifty-six cases reported up to I910 are collected. Including the fifty-six cases collected by Bottomley, four of my own and seven published since 1910, a total of 67 cases are now on record. One of the most interesting of the cases which have caused clinical symptoms is that of Lower.* His patient was a boy of eighteen with frequent attacks since infancy of left-sided abdominal pain. The last attack was accompanied by severe pain, chills and fever. Radiography showed the shadow of a calculus in pelvic portion of left ureter. It was extremely difficult to find the calculus at operation because it migrated easily within the lumen of a widely dilated ureter. This dilatation had taken place between two congenital constrictions of the ureter, one close to the bladder and a second one near the renal pelvis.
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, Caspar, 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. Fig. 5 shows where these various conditions most frequently occur. The points of narrowing are as follows:
(1) Just before the ureter enters the bladder wall; (2) where the ureter crosses the pelvic brim; (3) close to the pelvis of the kidney.
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 fcetus. The most marked widening is situated in the middle third of the ureter, i.e., proximal to the narrowing which occurs at the pelvic brim or between it and the bladder wall.
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 my Case III. 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 rdéle 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.
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. Teyssedre ® collected reports of eleven such cases. The 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 Lower’s cases showed a stricture both at the upper and at the lower end of the same ureter. A single 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. The protrusions range in size from that of a small pea to one completely filling the bladder lumen. 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.
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.
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.
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 III. 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 sequele of retention of urine result. Both the right ureteral orifice and the internal meatus urethre were blocked by the cystiform protrusion in Case III and the bladder walls greatly hypertrophied.
Symptoms and Diagnosis —— 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. (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. (4) Those cases in which disturbances of micturition are the principal symptom. This consists either of increased frequéncy or retention.
The examination of the urine is of little value, unless infection has supervened, and even then there is nothing to be found. Ureterography and pyelography and the other methods used in the diagnosis of ureteral strictures and of hydronephrosis are of the greatest value when the stricture is permeable.
Treatment. — The treatment depends, first upon the degree of constriction ; 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.
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ANNALS OF SURGERY, I912, lv, 571.
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Amer. Journ. Urology, 1913, ix, 27.
Beitrage zur Geburtshilfe u Gynakologie, vol. xiii.
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Journal Medical Research, 1902, iii, 125.
Archiv fur klinische Chirurgie, 1915, cv, 251.
Thése de Paris, 1892. 559
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