REDUPLICATION OF THE RENAL PELVIS AND URETER HOUSTON S. EVERETT From the Department of Gynecology of the Johns Hop kins University and Hospital
In 1922 Harpster, Brown, and Delcher in reporting a case of double kidney and double ureter thoroughly reviewed the literature and tabulated 382 cases recorded up to that time. In the same year Braasch and Scholl reported 144 such cases encountered at the Mayo Clinic from 1907 to 1922. Mertz in 1920 had published a similar review dealing largely with cases of bilateral duplication. Subsequent contributions have for the most part dealt with special phases of the subject or consisted of reports of small groups or single cases. For instance, Eisendrath in 1923 reported 80 cases, mostly tabulated from the literature, all of whom had been operated upon. In the same year Geisinger reported 7 cases all presenting features of special pathologic interest, and in 1931 Crowell, Thompson et al. added 12 cases all presenting unusual features. Additional case reports have appeared by Eisendrath and Pfifer (1925), Walther (1925), Hennessey (1925), Geiringer and Campuzano (1925), McKinney and Cary (1926), Ormond (1926), Schumacher (1926), Anderson (1927), Hyman (1928), Harris (1929), Roberts and Linfield (1929), Lintz (1932), and McBee (1933), to mention only those found in the recent American literature. In addition to the above there have also recently appeared several reviews and case reports dealing with the subject of ectopic ureteral openings, but as I intend to deal with this topic in a separate article in the near future, I now mention these only in passing. I do not intend to crowd the literature further with a repetition of tabulations of all recorded cases, but it so happens that I have encountered recently several cases of duplicated ureter and pelvis, one of which presents features of such unusual nature and interest that it seems to me worthy of a detailed report. In addition I have collected from the records of the female Cystoscopic Clinic of the Johns Hopkins Hospital and the private records of Dr. Guy L. Runner and myself, 48 cases, for the most part heretofore unpublished which I shall add in tabulated form. As the more complete reviews were published before the days of routine differential function tests, this subject is conspicuously absent from these contributions. A record of such a test, however, is found in several of 1 THE JOURNAL OF UROLOGY, VOL.
36, NO. 1
2
HOUSTON S. EVERETT
the more recent case reports. In our cases a differential phenolsulphonephthalein determination has been recorded a sufficient number of times to be worthy of special note and I shall attempt therefore to analyze as many cases as possible in this regard. Case report. Mrs. E. B., white, aged 53. Diagnosis: functionless right kidney; double renal pelvis and complete double ureter, left; infected hydronephrosis, left lower pelvis; nephrolithiasis, left lower pelvis; stricture of all 3 ureters; stricture of urethra; chronic trigonitis; hypertension. The patient first consulted me on November 9, 1934, because of persistent pyuria. At this time there were no other symptoms referable to the urinary tract. However, she had been seen by her family physician in the early spring of the same year because of severe headaches located in both the frontal and occipital regions. At that time he had found a hypertension of 220 systolic and 110 diastolic, very badly infected tonsils, a trace of albumin and much pus in the urine. He had treated the patient for about a month with rest in bed, diet, fluids and urinary antiseptics, and had advised urologic consultation, but probably because of lack of subjective symptoms referable to the urinary tract, the patient did not consent to this until she finally consulted me in November. Meanwhile on June 6, 1934 four carious teeth had been extracted, and on June 30, the tonsils and adenoids had been removed, Beta Streptococci being grown from the tonsils. Because of the headaches, dizziness and some blurring of vision, which had been present since the spring, she also had consulted an ophthalmologist who found a refractive error which he corrected with glasses. The ophthalmoscopic examination showed deep physiological cupping, mild arteriosclerosis, and slight engorgement of the veins. In spite of all this treatment the headaches and disturbance of vision had continued. The patient had had the usual minor childhood diseases and also diphtheria. She had always been subject to attacks of tonsillitis, but she stated that aside from this she had always been quite healthy except for the following episodes: At the age of 27, following a miscarriage which terminated her only pregnancy, she underwent a pelvic operation with removal of both tubes and ovaries. Ten years ago she had several attacks of appendicitis with high fever, nausea, vomiting, right lower quadrant pain, and lassitude. Following an appendectomy for several months she continued to suffer from pain in the right flank, frequency, urgency, and nocturia 3 to 4 times per night. She was treated with large quantities of fluids and sodium bicarbonate by mouth, until the symptoms eventually subsided. There was no further trouble until about 2 years previous to the onset of the present illness when, following an automobile accident in which she sustained a rather hard blow upon the back, she suffered frequent attacks of severe right flank pain over a period of
3
REDUPLICATION OF RENAL PELVIS AND URETER
about a month. During this time there was again slight frequency and nocturia. Since that time there had been occasional slight backache, but no further bladder symptoms. In the past 8 months there had been a weight loss of 20 pounds. The patient was admitted to the Johns Hopkins Hospital for further study and on November 12, 1934, the general physical examination was essentially negative except for the following points: The blood pressure was 178 systolic and 100 diastolic, the abdomen was flat and soft with low midline and low right rectus scars. The liver edge was just palpable on inspiration and the lower pole of the left kidney could also be palpated but was not tender. No other organs or masses could be palpated and no tenderness could be elicited. Vaginal and rectal examinations showed essentially normal findings. The hemoglobin was 98 per cent (Sahli), the blood non-protein nitrogen was 40 mgm. per cent; and a catheterized urine specimen showed a trace of albumin, no sugar, and countless pus cells. Two hour intravenous phenolsulphonephthalein determination gave the following results: First half hour. Second half hour. Second hour .. Total for two hours.
200 cc. 150 cc. 350 cc.
40 per cent 15 per cent 15 per cent
700 cc.
70 per cent
At cystoscopic examination done by the Kelly method on November 12, the urethra was found to be greatly infiltrated and narrowed, only permitting the use of a 7½ Kelly cystoscope. Culture of the bladder urine showed bacillus proteus. The bladder mucous membrane was everywhere practically normal in appearance except for a severe chronic trigonitis obscuring the right ureteral orifice. A left orifice was easily located and catheterized with a number 7 renal catheter, bearing a 3j mm. wax bulb, with obstruction to the bulb over a rather diffuse area in the broad ligament region. A specimen of urine from the left kidney showed about 40 pus cells and 3 red blood cells per high power field. A half hour differential phenolsulphonephthalein test gave the following result. Through the left kidney catheter ... Through the bladder catheter ...
Appearance time
Amount
4 minutes S minutes
30 cc. 35 cc.
Per cent
3
35
A plain X-ray of the abdomen showed a large left kidney with a large collection of stones in the pelvis (fig. 1). A pyeloureterogram with 21 cc. of sodium iodide solution showed considerable dilatation of the pelvis and calyces, and a ureter which was dilated in its upper portion, kinked at about the level of the transverse process of the third lumbar vertebra, moderately dilated in the abdominal spindle, and definitely narrowed in the broad ligament region (fig. 2). On withdrawal of the catheter the 3j mm. bulb hung quite firmly in the broad ligament region.
FIG.
FIG. 2
1
FIG. 3 FrG. 4 Plain X-ray with opaque catheter introduced into the pelvis of the left kidney. There are several large calculi in the lower part of the kidney. Note the large amount of kidney substance appearing above the calculi. FIG. 2. Left pyelo-ureterogram with the catheter withdrawn until a 4 mm. wax bulb placed 10 cm. back of the catheter tip was hanging firmly in the broad ligament region. There was also a hang of the bulb in a higher area which corresponds with the area of kinking and narrowing which appears opposite the lower part of the fourth lumbar vertebra. Note the dilatation of the pelvis and calyces and also of the ureter between and above the stricture areas. A considerable mass of kidney substance can be seen above this pelvis. FIG. 3. X -ray taken with catheter in the right ureter after intravenous injection of neoiopax. A small accessory pelvis can he seen in the upper pole of the left kidney. None of the opaque solution appears in the left lower pelvis or on the right side. No shadow of a right kidney can be seen in this or in the plates illustrated in figures 1, 2, and 4. No urine ever drained from the catheter in the right ureter. FIG. 4. Retrograde pyelo-ureterogram showing complete reduplication of pelvis and ureter on the left side. A 4 mm. wax bulb hung in the ureter from the upper pelvis at about the same levels as those in which strictures were demonstrated in the lower ureter. T he urine from the upper pelvis was sterile and 35 per cent phenolsulphonephthalein was coll ected from this pelvis in a half hour, while only 5 per cent was collected from the lower pelvis and none from the bladder. FIG. 1.
4
5
REDUPLICATION OF RENAL PELVIS AND URETER
The trigonitis was then treated for 2 weeks by bladder instillations of silver nitrate 1: 1000 after which it was found possible to catheterize the right ureter. This was found to be densely strictured in its lower portion and also near the kidney. No urine drained from the right ureteral catheter on either of two occasions so intravenous pyelograms, using neoiopex, was resorted to on December 17. These showed no evidence of secretion of the dye on the right side, nor into the large hydronephrotic pelvis containing the stones on the left side, but did reveal above this region a small accessory pelvis drained by a separate ureter (fig. 3) . Following this lead the two left ureteral orifices were found and catheterized, and the urine from the upper pelvis was found to be free from pus and sterile while that from the lower pelvis was still heavily infected. A half hour intravenous phenolsulphonephthalein test gave the following result: Appearance time
Left upper pelvis. . . . . . . . . . . . . . . . . . . . . . . . . . . 3 minutes Left lower pelvis. . . . . . . . . . . . . . . . . . . . . . . . . . . 11 minutes Right kidney through bladder catheter . ..... . .
Amount
100 cc. 90 cc. 0
Per cent
30 5 0
Pyelograms of the double left kidney were then taken using 12.5 cc. of sodium iodide solution for the lower pelvis, and 2.5 cc. for the upper pelvis (fig. 4). On withdrawal of the catheter from the upper pelvis the 3} mm. bulb hung at 18 cm. and 10 cm. from the outside. A second non-protein nitrogen determination done on this date showed 50 mgm. per cent. The situation now seemed clarified. There was either congenital absence of the right kidney or more probably, in view of the history, the right kidney had undergone atrophy as a result of slowly increasing obstruction over a period of years, culminating in complete ureteral obstruction at the time of the patient's acute pain 2 years ago. On the left side there was a double pelvis and double ureter, the lower pelvis being a large hydronephrotic sac containing stones, heavily infected, and draining very little functioning kidney cortex. The upper pelvis was uninfected and the substance drained by this pelvis had undergone functional hypertrophy to the extent that its phenolsulphonephthalein excretion was 30 to 35 per cent in a half hour, and the total excretion in 2 hours was 70 per cent. The elevation of non-protein nitrogen, however, showed that in spite of the apparently normal output of phenolsulphonephthalein, the patient was on the borderline of renal insufficiency. The question of subsequent treatment now arose. Removal of the stones from the left lower pelvis was considered, but this seemed to present a grave hazard in the possibility of injury to the good upper segment. The patient had already shown considerable improvement following a single dilatation of the ureter from the left lower pelvis and was now suffering from very few headaches and no disturbance of vision. High and low strictures had been demonstrated in both left ureters by means of wax bulbs and X-rays so it
6
HOUSTON S. EVERETT
was decided to treat the patient by a conservative course of ureteral dilatations. Treatments were given at weekly intervals using the bulbs near the tips of the catheters in order to dilate more thoroughly the upper stricture areas. These areas proved unusually dense in both ureters and could not be passed with bulbs larger than 4i mm. in diameter. Any bulb larger than this would obstruct permanently in these areas and on withdrawal of the catheter the wax was found to be molded and constricted. The ureter from the lower pelvis was dilated four more times and that from the upper pelvis three times. In spite of these treatments the urine from the lower pelvis continued to show pus and bacilli but the patient felt entirely well, so on March 27, she was dismissed with instruction to return immediately upon recurrence of symptoms. She returned on June 12, complaining of a moderate return of headaches. Each ureter was dilated once and since then the patient has reported several times, the last time December 12, 1935. Each time she has stated that she felt quite well and had been carrying on her usual activities. This rather extraordinary case brings out strikingly several interesting and important points of general urologic value. In the first place it illustrates the possibility of great disproportion between urinary tract disease and urinary tract symptoms. Except for the attacks of acute right sided pain and slight bladder symptoms 2 years ago, and the less severe pain in the same.region, but more marked bladder symptoms 10 years ago following the appendectomy, the patient had never had pain or discomfort in either kidney region, or other subjective symptoms referable to the urinary tract. In view of the condition found in the left kidney the total absence of pain in this region seems most remarkable, but such has been my experience repeatedly in cases with major pathology of the upper tract. I have found that persistence of cystitis for a period of more than 3 to 4 weeks, in spite of appropriate medicinal and local bladder treatment, nearly always indicates disease of the upper tract, regardless of the presence or absence of subjective symptoms referable to the kidneys or ureters. Patients with renal tuberculosis, for instance, nearly always suffer some form of bladder distress, but at present I do not recall one such patient who had subjective symptoms referable to the involved kidney. This, I believe, is a point well worth the careful consideration of general practitioners. In any case of persistent cystitis the complete diagnosis cannot be made without a thorough urologic investigation. If this had been done 10 years ago in the case under consideration, whatever pathologic lesions existed at that time could have been recognized and treated and the patient's present rather precarious condition no doubt could have been prevented.
REDUPLICATION OF RENAL PELVIS AND URETER
7
In the second place the case illustrates the remarkable ability of kidney tissue to undergo compensatory functional hypertrophy when called upon to do so. In the third place the case affords an example of the value of extreme care and patience in the study of urologic patients. A tendency to haste might easily have resulted in a left nephrectomy upon the assumption that the 35 per cent phenolsulphonephthalein excretion obtained through the bladder catheter at the original examination was coming from the right kidney. The outcome in such an event, of course, would have been tragic. The 48 other cases herewith presented are recorded in the tables. Types of anomalies. From the tables it is seen that including the case reported in detail we have encountered 19 cases of complete unilateral reduplication and 23 cases of incomplete unilateral reduplication. Of the 382 cases collected by Harpster, Brown, and Delcher, 181 and 123 were of these types respectively, while of the 144 cases reported by Braasch and Scholl, 36 of the unilateral cases were complete and 99 incomplete. Of the bilateral anomalies recorded in table 3 the reduplication was complete on each side in 2 cases. This type of anomaly is rare. Harpster, Brown and Delcher, found 40 such cases and Braasch and Scholl reported 8 others. Subsequently single cases have been reported by eleven of the authors referred to early in the paper. These with our two make a total of 61 cases of bilateral complete reduplications. In one case the reduplication was complete on one side and incomplete on the other, while in 3 others both sides were incompletely reduplicated. Harpster, Brown, and Delcher tabulated 28 such cases of incomplete bilateral reduplications. Case 7 of table 3 deserves special comment. In this case there was incomplete reduplication of the usual type, pelvis and upper ureter, on the left side. On the right side, however, the pelvis was single and drained by a single ureter. In the bladder, however, medial by only 2 or 3 mm. to the orifice of this ureter was a second smaller orifice. A catheter would enter this orifice and pass for a distance of 6 or 7 cm. but no farther. There was no drainage from the catheter and no communication could be demonstrated between this apparently blindly ending ureteral bud and the functioning right ureter (fig. 5). I have found no other exactly similar case recorded, though Papin and Eisendrath mention such a possibility in their general classification of urinary tract anomalies. Harpster, Brown and Delcher record 2 cases with
T ABLE
CASE
AGE
YEAR FIRST SEEN
!.-Complete unilateral reduplication of pelvis and ureter, 18 cases
SYMPTOMS
- - --
DURA-
TION
SIDE
COMPLICATING PATHOLOGY
TREATMENT
RESULT
REMARKS
CXl
--Improved
1. B.M.
36
1919
Frequency and dysuria
17 yrs.
Right
Multiple strictures of all 3 ureters Ureteral dilatations
2. E . S.
29
1920
Pain in right flank, pyuria, 6 mos. former acute pyelitis
Right
Stricture of both right ureters. Colon bacillus pyelitis, lower right pelvis
Ureteral dilatations
Well
3. A. C.
25
1921
Repeated abortions, tenderness in lower abdominal quadrants
3 yrs.
Right
Ureteral strictures, all 3 ureters
Ureteral dilatations, one to each right ureter and 2 to the left ureter
Improved
4. A.P.
12
1923
Constant urinary leakage
Life
Left
Accessory left ureter opened on vulva and showed congenital stricture and dilatation above. Stricture of right ureter with slight hydronephrosis
Abdominal extraperitoneal ligation and resec-
Well
tion of accessory ureter
Died 1924, infected abortion
See table 4. Reported by Runner, G. L., in. "Practice of Surgery/' Lewis, Dean, VIII, ch. XI, pp. 2- 5
lJ:I
~H ~
Ul
5. M . G.
44
1924
Backache, frequency , nocturia, fever
Few
Left
Hydronephrosis and hydroureter left lower pelvis. Two hour 'phthalein 55 %
Patient refused treatment
Not treated
Ureteral dilatations
Well
mos.
6. C. M.
41
1923
Recurrent attacks of leftsided pyelitis
2 yrs.
Left
Stricture of all 3 ureters. Slight hydronephrosis (19 cc.) right pelvis and left lower pelvis. Colon bacillus pyelitis left lower pelvis
7. E.L.
47
1926
Bladder pain and frequency
Years
Right
Ureteral stricture, all 3 ureters
Ureteral dilatations
Improved
8. F . P.
17
1927
Pain in both kidney re-
2 mos.
Left
Ureteral stricture, all 3 ureters.
Ureteral dilatations
Improved
Ureteral dilatations
Improved
See table 4
Chronic colon bacillus pyelitis left lower pelvis. Chronic
gions
nephritis
9. M . M.
32
1928
Sterility, leucorrhea, soreness in lower abdominal quadrants
?
L eft
Ureteral stricture, all 3 ureters. Ptosis of right kidney
See table 4
; ~
10. E.H.
68
1929
Pain in right flank and right lower quadrant
Few mos.
Left
Ureteral strictures, all 3 ureters. Slight hydronephrosis right pelvis (20 cc.) and left lower pelvis {12 cc.)
Ureteral dilatations
Well
See table 4
11. E.H.
43
1929
Pain in left upper quadrant. Attack of frequency, dysuria, hernaturia, following first delivery at 23
3 mos.
Left
Ureteral strictures, all 3 ureters
Ureteral dilatations
Improved
See table 4
12. L. R.
40
1929
Bladder pain and incontinence
10 yrs.
Right
Ureteral stricture, all 3 ureters. Slight hydronephrosis and hydroureter left and right lower. Ureter from right upper pelvis opened on urethral sphincter
Ureteral dilatations
Improved
Ureteral stricture, all 3 ureters
Ureteral dilatations
13. A. M.
47
1930
Recurrent attacks of frequency and dysuria
1 yr.
Left
i ~
Improved
See table 4
14. P. F.
37
1931
Frequency, nocturia
Several years
Right
Ureteral stricture, all 3 ureters
Ureteral dilatations
Improved
15. H. D.
34
1931
Pyuria, albuminuria, occasional dysuria Backache
9yrs.
Left
Ureteral stricture, all -3 ureters. 'Chronic pyelitis, colon bacil!us and staph. aureus, both left pelves
Ureteral dilatations
Improved
See table 4
Pain in flanks and urinary incontinence following delivery
9 mos.
Left
Vesico-urethro-vaginal fistula. Transplantation of ureters Improved Chronic pyelitis (colon bacilinto sigmoid colon, Coffey technique. Patient !us, b. proteus) both left pelves. returned 1 year later Slight dilatation of right and left lower pelves with suprapubic abscess which was drained Well
See table 4
Intermittent pain in left upper quadrant, fre-
4 yrs.
Left
N ephrolithiasis left lower pelvis. Pyonephrosis both left pelves (colon bacillus and streptocococcus). Left perinephritic abscess. Chronic colon bacil!us pyelitis, right
Left nephrectomy Right ureteral dilatations
Well
See table 4
Right
Ectopic opening of ureter from upper right pelvis near vaginal orifice
Abdominal extraperitonealligation and partial resection of ectopic ureter
Well
To be reported in detail elsewhere
16. E.T.
17. M. V.
35
38
1932
1933
1 yr.
quency, dysuria, noc-
tu.ria Right flank pain. Patient admitted as emergency, acutely ill
18.
s. s.
10
1935
Constant urinary leakage with normal voiding
~
g
1 yr.
Life
~
~ t"'
I ~ ~
s '-0
......
TABL E
CASE
AGE
YEAR FIRST SEEN
SYMPTOMS
-- -1. M.E.
2.
J. s.
3. L.'.B.
D URATION
SIDE
COMPLICATING PATHOLOGY
TREATMENT
RESULT
1912
Dragging sensation in pel- 5 yrs. vis and right side. I ndigestion, dysmenorrhea Att acks of right sided 1 year pain, frequency, dysuria
Right
N ephroptosis, tuherculous salpingitis, tuberculous peritonit is
Nephropexy
Improved
48
1919
Pain in left flank
Several years
Left
Mult iple strictures of left ureter. Chronic staph. aureus pyelitis, left
Ureteral dilatations
Improved
2 yrs.
Left
Ureteral stricture, bilateral. Hydronephrosis, left (25 cc.), · elusive ulcer of bladder
Ureteral dilatations, topical applications to bladder ulcer
1921
REMARKS
- --
34
46
0
2.- Incomplete unilateral reduplication. (Double pelvis and double ureter joining before entering bladder)
Severe frequency and dysuria, pain above Poupart's ligaments
Anomaly discovered at operation
i:Q
~>-3
Improved
~ rn
4. E.~C.
24
1921
Pain in kidney regions
Several years
Left
Ureteral stricture, bilateral; myomas of the uterus; hydrosalpinx, bilateral; colon bacil!us pyelitis, left ; bydronephrosis, right (30 cc.)
Ureteral dilatations; bilateral salpingectomy; partial resection of left ovary; retrograde dilatation of lower left ureter
Well
Left ureter bifurca ted from broad ligament region to bladder. Pelvis and upper ureter single. Died 1934 of myeloid leukaemia
5. B. M.
35
1923
Attacks of renal colic, passing stones, frequency nocturia
2 yrs.
Left
Ureteral stricture, bilateral; nephrolithiasis, bilateral; tuberculosis left lower kidney; hydronephrosis left (48 cc. both pelves)
Ureteral dilatations and removal of ureteral stones. L eft heminephrectomy. Right nephrolithotomy
Improved
See table 5
6. E. C.
28
1923
Gastric distress; pain in abdomen and back
Several years
Right
Ureteral stricture, bila teral; colon bacillus pyelitis, right; hydronephrosis, right (15 cc.)
Ureteral dilatations
Improved
See table 5
M
;J ~
>-3 >-3
7. F.M.
35
1923
Frequency, dysu.ria, haematuria
1 year
Left
Ureteral stricture, bilateral; tuberculosis of right kidney and ureter
8. E. B.
31
1925
Pain in flanks and lower quadrants
1½ yrs.
Right
Ureteral stricture, bilateral. Ureteral dilatations Ureteral calculus left. Hydronephrosis, left (16 cc.)
Improved
9. A. 0.
20
1926
Enuresis
15 yrs.
Right
Ureteral stricture, bilateral
Ureteral dilatations
Unimproved
10. N. F.
43
1928
Draining left kidney sinus following removal of
3 yrs.
Left
Functionless right kidney with Ureteral dilatations nephrolithiasis. Ureteral stricture bilateral; persistent draining sinus from left kidney
Improved
stones; pain in left flank
N ephrectomy and partial Well, 1932 right. ureterectomy, Dilatations of left ureter Stone passed after second dilatation
@ ~ Pain relieved, sinus persisted. Died 1934 of uremia. See table 5
z ~
11. J. V.
27
1930
Left renal colic
2 wks.
Left
None
2 ureteral dilatations
Well
12. L. E.
30
1931
Pain in left flank; chills, fever
2 days
Left
Ureteral stricture and acute pye-
Medicinal treatment for acute pyelitis and typhoid carrier; later left ureteral dilatations
Well
Acute colon bacillus pyelitis, right. Congenital absence of left kidney
Medical treatment only
Not treated Diagnosis made by
Ureteral
Ureteral dilatations
Well
See table 5. Stone passed after first dilatation
litis, left; typhoid carrier
~
0
See table 5
~
~
t-<
>rj
13,'.R"K.
63
1931
Years frequency. Haematuria 2 wks.
Attacks of dysuria and
Right
and pyuria
14. C."R.
40
1932
Pain in right flank and
2 mos.
Right
right lower quad.rant; frequency, dysuria
15. B. N.
15
1932
Pain in left flank, fre-
29
1932
Pain in upper right quadrant; dysuria, fre-
bilateral;
raphy. Two hour 'phthalein 80 %
small stone in right ureter
8wks.
Left
Ureteral stricture, bilateral; Ureteral dilatations pyelitis subacute, left. (Staph. aureus)
Well
See table 5. Two hour 'phthalein 90%
5 mos.
Right
Ureteral stricture, bilateral; hydronephrosis, right (60 cc.)
Improved
See table 5
quency, dysuria
16. J.C.
stricture,
intravenous pyelog-
Ureteral dilatations
t,,J
~
r.n
~t;:I
~
t;l
i:d
quency, urgency, hae-
maturia
1--'
,-.,.
~
T ABLE YEi,.R
CASE
17. A. S.
18. S. M.
AGE
FIRST SEEN
--
--
49
1933,
52
1934
SYMPTOMS
DURATION
SIDE
2-Conclitded TREATMENT
COMPLICATING PATHOLOGY
RESULT
REMARKS
--- --Large right kidney felt on medical serv ice. No symptoms Pains in bladder and kidney regions
Right
Hydronephrosis, right (35 cc.)
None
Not treated Two hour 'phthalein 60%
Several years
Right
Ureteral stricture, bilateral; Ureteral dilatations chronic pyelitis, bilateral (colon bacillus); hydronephrosis, and hydroureter, right (25 cc.)
Improved
Stricture of common right ureter below bifurcation
Well
See table 5
i:Il
g U1
19. V. C.
20. V. M.
28
26
1934
1934
Pain in right lower quadrant radiating to t high and flank
3-4 yrs.
Attacks of bilateral !um-
2 yrs.
Right
1934
Pain in right flank and right lower quadrant
1935
Painful mass in right lower quadrant; discharge of pus from urethra on
~
Chronic pyelitis, bilateral (Staph. Ureteral dilatations albus) : ureteral stricture, bilateral; slight hydronephrosis, right (16 cc.)
Well. Cul- See table 5. Two hour 'phthalein tures
Right
Slight hydronephrosis right (21 cc. both pelves)
Ureteral catheterizations
Improved
Left
Ectopic right kidney with pyo-
Right nephrectomy
Well. 7
Left
quency
22
H
U1
bar pains; dysuria, fre-
21. J. T .
Ureteral dilatations
5 yrs.
negative
40%
8
mos.
nephrosis, and ectopic open-
See table 5
mos.
post-op-
ing of right ureter in urethra
pressing over mass
23. A. S.
53
1935
Haematuria, dy suria, frequency, pyuria, pain in
both flanks
erative
Few days
Left
Cystitis, acute; pyelitis acute
bilateral; (colon bacillus)
Conservative, fluids and urinary antiseptics
~
t,j
H H
--22. B . G.
~
Well
See table 5
REDUPLICATION OF RENAL PELVIS AND URETER
13
single pelves and single upper ureters which bifurcated in their lower portions and entered the bladder by two orifices. Both of these were originally reported by Kapsammer, "Nierenchirurgie," 1905. Case 4 of table 2 of this series is a similar case. There were ectopic openings of one of the reduplicated ureters in 3 cases with complete reduplication, and in one case with incomplete reduplication on the left side, the ureter from the right single, but ectopic and pyonephrotic kidney opened into the urethra. Symptoms. Pain either generalized abdominal, backache, or referred to the upper urinary tract was the most frequent symptom, occurring
FTG. S. Case 7, table 3. Pyelogram of right kidney with catheter also in a blindly ending ureteral bud opening into bladder by a separate orifice, situated medial to the orifice by about 2 mm.
34 times. Bladder symptoms, frequency, dysuria and nocturia, were complained of by 23 patients, and hematuria had been noted by 5. Pyuria was recorded as having been noted by the patient in only 4 instances. Other symptoms occasionally noted were incontinence and the passing of calculi. Urinary tract pathology other than the anomalies. Ordinary infection, colon bacillus, staphylococcus, etc., was present in some portion of the upper urinary tract in 19 cases. Pyonephrosis occurred twice. In one instance (table 1, case 17) the reduplicated kidney was pyonephrotic, while the opposite kidney showed a chronic colon bacillus pyelitis. The other case (table 2, case 22) was that of an infant, aged 8 months,
.......
~
TABLE
3.-Bilateral reduplication, 7 cases
.,z [;l ~
CASE
".:
..,,t'.l ·---1. R. G.
2. E.W.
3. E.M.
- -
TYPE OF REDUPLICATION SYMPTOMS
..,,
.,"><
z 0
.:..,,
"p "
Right
TREATMENT
Stricture of all 4 ureters. Colon bacillus pyelitis all 4 pelves
Ureteral dilatations and pelvic lavage
---
Complete
28 1929 Pain in right flank Pain in left flank
Complete
33 1927 Lumbar pain Frequency, dys-
5 yrs. 1 yr.
23 1927 Pain in left flank
RESULT
REMARKS
----
37 1915 Pain in left flank, Several left lower quadyears rant and hip; frequency, tenesmus
Complete
Improved
See table 4
~
0
q
r:n H
~
14 yrs. 1 yr.
Complete
2 yrs.
Incomplete
Complete
Incomplete
Stricture of all 4 ureters. Slight Ureteral dilatations. hydronephroses (18 cc.) both Heminephrectomy lower pelves. Two hour 'phthalright upper, 1929. ein 37% Nephrectomy right, 1932
Improved
Stricture of all ureters. Bilateral
Improved
See table 4
Ureteral stricture bilateral. N eph- Ureteral dilatations. Improved rolithiasis bilateral. Colon Bilateral nephrolibacillus and staph. aureus pyethotomy separately. litis, bilateral. Hydronephrosis, Left heminephrecbilateral (25 cc. each). Two tomy lower portion hour 'phthalein 45 %. Persistent sinus from left kidney with exclusion of lower pelvis
See table 5
nephrolithiasis. Hydronephrosis (16 cc.), right lower pelvis. Two hour 'phthalein 30 %
uria, haema turia
4. M. S.
COMPLICATING PATHOLOGY
Left
Incomplete
Ureteral dilatations. Nephrolithotomy, right and left separately
See table 4. Two hour 'phthalein 1932, 60%
r:n M
~ ~ H H
5. L. C.
34 1927 Acute pyelitis 3 yrs. 3 yrs. ago. Pain in right flank and right lower quad-
Incomplete
Incomplete
None. Two hour 'phthalein 65 %
Not treated
Not treated
Incomplete
Incomplete
N ephrolithiasis right lower. Pyelonephritis, chronic, right, staph. aureus
Ureteral dilatations. Right heminephrectomy
Well, 1932 See table 5
Ureteral stricture, bilateral
Ureteral dilatations
Improved
rant
6. S. H.
45 1931 Pain in right flank. Passed stone 8
~
10 yrs.
years ago
7. C. D.
32 1935 Pain in right upper quadrant, !requency
6 mos.
Lower end of ureter only
Pelvis and upper ureter
t::1
~
~ Still under treatment. See table 5
~
0
z 0
>zj
~
~
t-<
~
@ en
~
!t:;j fd
( .n
TABLE
4.-Dijferential Phenolsulphonephthalein Determination in Cases with Complete Reduplication f rom T ables I and III, and Some from Recent Literature Collection tJime 30 minutes, unless otherwise stated
., A
CASE
RIGHT KIDNEY
I NFECTION
LEFT KIDNEY
STONES
H
cJ
::1
.,"'A
:a
~
.." ~
u" - -" - - - -
t;!
E--<
.:!)
00
·::
,l A.
,l A.
"t,,
ii
ii
00
~A. "
Cf}
p
>-1
"'.s
per
per
per
per
.s
A. A.
Ile
0
-- - - - - - cent
-°'
-~
~A.
cent
cent
Cf}
A.
,l A.
ii
ii
p
per
" 0
>-1
-- --- - --
cent
[
URETERAL STRICTURES
-~
~
A. A.
HYDRONEPHROSIS
REMARKS
...,
--=
...,
~
J
~
J -
Per
cent
cent
~
...,
~
...,
>-1
1Z
~
1l
-
ii!
...,
.!,!>
1l
-- -
>-1
1
4
Left
20
Trace
20
-
-
-
-
+
U*
+
U*
1 1 1
6 9 10
Left Left Left
L*
-
-
-
-
U* &L* U* &L*
-
-
-
-
+ L* - L* + -
+ +
Left Left Left
+ + + +
U* &L* U* &L* U* &L* U* &L*
1
16
Left
-
?
?
1
17
Left
30 21 10 15 20 21 10 15 10 2 0
-
11 13 15
7 12 9 12 10 12 10 13 5 Trace 0
-
1 1 1
37 21 33 25 23 32 25 30 20 17 35
+
U* &L*
3 3 3
1 2 3
U* &L* U* &L* U* &L*
U* &L* U* &L*
-
U*
20 minute collection
-
15 15 15 15
Both Both Both, left incomplete
(Pre-operative) (Post-operative) 16 3 4 0
15 10 18 15
Both
2
14
Both Right Right Right
5 8 8 10
11 10 4 10
Both Both
9 0
6 5
Trace Trace 7 8 12 7
-
U* &L*
-
-
U* &J.,*
-
+
U* &ZL*
U* &L*
U*&:L*
- L* - + +
-
-
-
-
-
-
-
+ - - -
L*
L* L* L* -
Ectopic opening of left upper ureter congenital with stricture
Pyonephrosis, both segments left kidney
+
AUTHOR
Geiringer and Campuzano Geisinger Crowell et al. Hennessey Roberts and Linfield Walther Hepburn
4
9
U* &L*
U* &L*
5
6
-
+ -
5 12 15
-
L* U* &L* 5 5
7 5
L* U*
L*
-
.. U and L* denote upper and lower pelves or the ureters from these pelves respectively.
-
-
-
-
-
-
-
-
-
-
-
-
-
-
minute minute minute minute
collection collection collection collection
15 minute collection 15 minute collection. Pyonephrosis, right upper pelvis
17
REDUPLICATION OF RENAL PELVIS AND URETER
with a single, ectopic, pyonephrotic right kidney, and a partially reduplicated left tract which was not infected. Of the other 17 cases showing infection, 6 were in patients with complete reduplication and 11 in patients with incomplete anomalies. Of the former group, 3 showed infection of the lower of the reduplicated pelves alone, 2 of both pelves TABLE
CASE
5.-Dijferential phenolsiilphonephthalein determinations in cases with incomplete rediiplication from tables 2 and 3 Collection time one-half hour, unless otherwise stated SIDE REDUPLICATED
INFECTION RIGHT
STONES
LEFT
HYDRONEPRROSIS
URETERAL STRICTURE
REMARKS
Right Left Right Left Right Left Right Left
--
-- --- --- -- Table 2
per cent
--
5
11
9
10
Right Left
15 20 26 0
12 10 30 25
12 14 15
Left Right Left
25
15 20 35 17 50 18 30 35 40
10 10
16 18
Right Right
20 21
Left Right
23
Left
50 15 15 30 30 U* 16 L* 8 25
Both Both Both
21 15 35
-
-
-
per cent
Left
6
-
-
-
Tbc. L*
+ +
-
-
+
-
+ + +
25
+ + + + - - - - + + - - - + + - - - - + - + - + - + - + + + + + - -- + -- +? + + + - - - - -
6 25 15
+ + + + + + + + + - L* -- -- - + + - + + -
--
+ -
-
+ +
-
+ + + + + + + +
2 hour collection
Discrepancy due to reflux
-
-
Table 3
--
4 6 7
* U and L
refer to upper and lower pelves, respectively.
on the reduplicated side, and one of all pelves. It seems remarkable that the upper pelvis alone showed infection in no case, as this pelvis is usually the less well developed and normal of the two anatomically. In the latter group the infection was in the reduplicated side alone in 7 patients, while in 4 others it was bilateral. In one of the latter there was bilateral incomplete reduplication (case 4, table 3).
18
HOUSTON S. EVERETT
Eighteen patients showed evidence of dilatation in varying degree of one or more pelves and ureters. Eight of these were patients with complete reduplication and 10 those with incomplete division. In the latter group the dilatation was on the reduplicated side in 7 patients and on the opposite side in 3. In the former group 3 showed dilatation of the lower pelvis alone, 4 showed dilatation of the lower pelvis on the reduplicated side and the single pelvis on the opposite side, and in one the combination was the upper of the reduplicated pelves and the opposite side. Only 2 cases showed renal tuberculosis. These were both in patients with incomplete reduplications. In one case (table 2, case 5) only the lower segment of the reduplicated left kidney was involved and heminephrectomy was done. In the other (table 2, case 7) the non-reduplicated kidney was involved and nephrectomy was performed. Stones were present in 8 cases. In 3 of these the reduplication was bilateral. In 2 of these there were stones in both kidneys while in the other only the lower pelvis of the right kidney contained stones. In 4 cases there was incomplete unilateral reduplication. In 2 of these the stones were bilateral, and it was one of these that showed tuberculosis in the lower segment of the reduplicated side. In another case there was only a small stone in the ureter on the reduplicated side below the point of division, and in still another there was a small stone in the nonreduplicated ureter. The eighth case (table 1, case 17) showed complete reduplication on the left side with pyonephrosis of thi.s kidney and stones in the lower pelvis. In 14 of the cases there were none of the above types of pathologic lesions. In 2 of these there was no disease present at the time of examination, though one had suffered previously from acute pyelitis. In one patient there was ptosis of the reduplicated kidney and nephropexy was performed with resulting relief of symptoms. In the 11 remaining patients, the only urinary tract lesions found to account for symptoms aside from the anomalies, were ureteral strictures. These patients were all treated by ureteral dilatations, and with one exception, a case of enuresis, made notable symptomatic improvement. Function. In tables 4 and 5 are presented the results of differential phenolsulphonephthalein tests in 26 of our own cases and in 7 others collected from the literature. From table 4, which includes 12 cases of complete reduplication from our own series and 7 others from the litera-
REDUPLICATION OF RENAL PELVIS AND URETER
19
ture, it will be seen that, except in those cases where the renal function has been distorted by some serious pathologic lesion, the function of the lower segment of a reduplicated kidney usually exceeds that of the upper segment. The ratio of function in lower and upper segments tends to be about 2: 1 though this ratio is sometimes considerably exceeded. This finding is quite in accord with the appearance presented by pyelograms in such cases, which as a rule show the upper pelves to be much smaller, and to drain much smaller segments of renal parenchyma than the lower pelves. These facts tend to emphasize the remarkable ~ompensatory functional hypertrophy of the upper segment of the left kidney in the case reported above, for the pyelographic evidence even in this case tends to show that the upper pelvis and segment are relatively smaller as compared with the lower. Further study of tables 4 and 5 tends to show that, other factors being equal, there is apt to be very little difference between the combined function of the component parts of the reduplicated side and that of the opposite side. Treatment. Operative treatment was resorted to in this series in only 13 cases. In 2 of these ligation and resection of the accessory ureter was done because of ectopic opening of the ureter with resulting constant leakage. In a third case the ureters were transplanted into the bowel because of irreparable damage to the vesico-vaginal septum, and in a fourth case the ptosed double kidney was suspended. In still another case the necessity of a pelvic operation was taken advantage of to expose and dilate a densely strictured lower ureter. This leaves then only eight patients in whom operation was necessary because of serious intercurrent renal pathology, or slightly more than 16 per cent. Nephrectomy was done 4 times, twice for pyonephrosis (table 1, case 17, and table 2, case 22), once for tuberculosis (table 2, case 7), and once for removal of a functionless remaining segment in a kidney previously subjected to heminephrectomy (table 3, case 2). Heminephrectomy was performed 4 times, once for stones and tuberculosis in the lower segment (table 2, case 5), once for removal of a functionless upper segment blocked by a densely strictured ureter (table 3, case 2), once for removal of a lower segment which had previously been subjected to nephrolithotomy after which it had be~n excluded from ureteral drainage (table 3, case 4), and once for removal of a pyelonephritic lower segment containing stones (table 3, case 6). Nephrolithotomy was done
20
HOUSTON S. EVERETT
in 3 cases, once unilateral with heminephrectomy on the opposite side (table 2, case 5), and twice bilateral (table 3, cases 3 and 4). Of the patients not operated upon 3 received no treatment. In two of these the diagnosis was made incidentally and no treatment seemed indicated. The other patient refused treatment. Two patients with acute pyelitis were treated only with rest, fluids, and urinary antiseptics. All other patients were treated by ureteral dilatations with considerable improvement in most instances. Of the 144 patients reported by Braasch and Scholl 30 required operation and 24 others showed definite pathology but were not subjected to operation. In 29 of their patients there was doubtful evidence of pathologic lesions, and in 61 others no definite pathology was found, although it is stated that in the majority of the group with doubtful lesions symptoms were or had been present and the presence of these symptoms is attributed by the authors to possible periods of temporary obstruction due to the anomalies. Some of these patients are stated to have shown some distention of the calyces but no mention is made of the possibility of ureteral stricture. In our series ureteral strictures were demonstrated by the wax bulb test and by the X-rays in the majority of instances, and adequate dilatation of the ureters resulted in relief of symptoms and improvement in condition in most cases. It is perhaps also possible that the resort to better ureteral drainage accounts for the small percentage of our patients in whom major surgery upon the kidneys was found necessary. SUMMARY
A case of complete unilateral reduplication of the renal pelvis and ureter is reported in detail. Forty-eight other cases presenting similar anomalies are tabulated and symptomatology, complicating pathology, and treatment in such cases are discussed. A special study of the differential function of the various renal segments is made and the conclusion drawn that, in the absence of serious complicating pathologic lesions, the function of the two sides is usually about equal and on the reduplicated side the function of the lower segment usually exceeds that of the upper segment in the ratio of about 2: 1. Attention is called to the value of ureteral dilatations as a conservative measure in the treatment of such cases and to its possible roll in the reduction of the incidence of operative treatment.
1201 N. Calvert Street, Baltimore, Maryland.
REDUPLICATION OF RENAL PELVIS AND URETER
21
REFERENCES ANDERSON, C. F.: Complete bilateral duplication of ureters and renal pelves. Jour. Tenn. Med. Assoc., 19: 281-283, 1926-27. BRAASCH, w. F., AND SCHOLL, A. J., JR.: Pathologic complications with duplication of the renal pelvis and ureter. Jour. Urol., 8: 507-546, 1922. CROWELL, A. J., ;THOMPSON, R., ET AL.: Pathological conditions arising in duplication of the kidney pelvis and ureter: Report of twelve cases. South. Med. Jour., 24: 741-749, 1931. ErSENDRATH, D. N.: Double kidney. Ann. Surg., 77: 450-475 and 531-557, 1923. EISENDRATH, D. N., AND PFIFER, F. M.: Bilateral heminephrectomy in bilateral double kidney. Jour. Urol., 13: 525-535, 1925. · GEIRINGER, D., AND CAMPUZANO, J.: Complete bilateral duplication of ureters and renal pelves. Jour. Urol., 14: 193-197, 1925. GEISINGER, J. F.: Reduplication of the ureter. Ann. Surg., 77: 563-571, 1923. HARPSTER, C. M., BROWN, T. H., AND DELCHER, H. A.: Abnormalities of the kidney and ureter; a case of double kidney and double ureter with a review of the literature. Jour. Urol., 8: 459-490, 1922. HARRIS, A.: An interesting anomaly of complete duplication of ureters and renal pelves, with a review of the literature. Urol. and Cutan. Rev., 33: 300-302, 1929. HENNESSEY, R. A.: Duplication of the kidney pelvis and ureter with infection. Jour. Tenn. Med. Assoc., 17: 113-116, 1924-25. HYMAN, A.: Bilateral double pelves and double ureters; right nephrectomy and left heminephrectomy. Am. Jour. Surg., 4: 437, 1928. LINTZ, R. M.: Bilateral double kidney with duplication of ureters. Ann. Int. Med., 5: 924-931, 1932. McBEE, T. J.: Bilateral double kidney with duplication of ureters. West Va. Med. Jour., 29: 257-262, 1933. McKINNEY, J. T., AND CAREY, S. B.: Complete bilateral duplication of ureters and renal pelves. Am. Jour. Roentgenol., 15: 149, 1926. MERTZ, H. 0.: Bilateral duplication of the ureters with a compilation of recorded cases. Urol. and Cutan. Rev., 1920, 24: 636-642, 1920. ORMOND, J. K.: An unusual instance of reduplication of ureter and pelvis. Jour. Urol., 15: 397-401, 1926. PAPIN, E., AND EISENDRATH, D. N.: Classification of renal and ureteral anomalies. Ann. Surg., 85: 735-756, 1927. ROBERTS, A. L., AND LINFIELD, E. H.: A case of double ureter and double pelvis of the kidney. U. S. Veterans' Bur. Med. Bull., 1929, 5: 56, 1929. SCHUMACHER, F. L.: Observation on reduplication of kidney pelves and ureters, with a case report. Radiology, 7: 475-479, 1926. WALTHER, H. W. E.: Bilateral duplication of renal pelves and ureters. Ann. Surg., 82: 968-970, 1925.
THE JOURNAL OF UROLOGY, VOL.
36, NO l