Vol. 96, Nov. Printed in U.S.A.
THE JOURN
Copyright © 1966 by The Williams & Wilkins Co.
SEGMENTAL ATROPHIC PYELONEPHRITIS IN RENAL REDUPLICATION: PRESERVATION OF SMALLER UPPER POLE SEGlVIENT BY PARTIAL NEPHRECTOMY B. MARVIN HARVARD From Section of Urology, Department of Surgery, Yale University School of Medicine, New Haven, Connecticut
In reviewing data pertinent to this subject since the first publications appeared in the late nineteenth century1 one is struck by the paucity of reports concerning salvage of the smaller upper segment in reduplicated kidneys. Possibly it is because, as pointed out by Young,2 about 80 per cent of pathological conditions are thought to cccur in the upper segment making it the most obvious target for surgical treatment. Perhaps Campbell's estimate is nearer the truth. 3 He reported that in more than 53 children requiring ureteroheminephrectomy for disease in a segment of a reduplicated kidney only three required removal of the lower segment. In past years complications associated with segmental resection of a kidney may have made some surgeons feel that attempts to salvage the smaller segment nullified any benefit which might be gained by attempting to preserve it. With improved diagnostic and surgical techniques, 4 - 9 Accepted for publication November 2, 1965. Read at annual meeting of New England Section, American Urological Association, Inc., Hamilton, Bermuda, October 12--17, 1965. 1 Czerny, V.: Quoted by Von Herzel, E.: Ueber nierenextirpation. Aus der Heidelberger Chirurgischen Klinik des Prof. Dr. Czerny. Beitz. z. klin. Chir., 6: 511, 1890. 2 Young, H. H. and Davis, E.G.: Double ureter and kidney with calculous pyonephrosis of one half: Cure by resection. Embryology and surgery of double ureter and kidney. J. Ural., 1: 17-32, 1917. 3 Campbell, M. F: Anomalies of the urogenital tract. In: Urology, edited by Campbell, 2nd edit. Philadelphia and London: W. B. Saunders Co., 1963, vol. 2, chapt. 33, p. 1634. 4 Hess, E., Roth, R. B., Kaminsky, A. F. and Swick, H. V.: Surgery for the conservation of renal parenchyma. J. Ural., 64: 175-187, 1950. 5 Spence, H. M.: Nephro-ureterectomyandheminephro-ureterectomy in infancy and childhood. J. Urol., 71: 171-182, 1954. 6 Murphy, J. J. and Best, R.: The healing of renal wounds: 1. Partial nephrectomy. J. Urol., 78: 504-509, 1957. 7 Parry, W. L. and Finelli, J. F.: Some considerations in the technique of partial nephrectomy. J. Ural., 82: 562-565, 1959. 8 Hamm, F. C. and Finkelstein, P.: Partial nephrectomy. J. Ural., 82: 625-629, 1959.
including vascular techniques, perhaps more effort will be exerted toward salvage of "normal" upper segments. Generally speaking, partial nephrectomy has been used in the past to eradicate or control tumors, pyohydronephrosis, stones, and specific infections such as tuberculosis. No histories of patients with atrophic pyelonephritis secondary to reflux10 in one segment are recorded as having been treated by partial nephrectomy. Undoubtedly some patients have been so treated, and more will be discovered with the now widespread use of voiding cystography. If preservation of renal function is of prime concern then one must consider the fact that preserving the smaller upper segment may represent salvage of some 16 per cent of total renal mass. That hypertrophy of the unaffected, upper segment does take place is unquestionably demonstrated in the 2 patients whose histories are presented here. Lodge and Williams have documented similar observations in their radiographic analysis of compensatory renal structural and functional changes.11 While laboratory studies have repeatedly demonstrated that animals can survive massive loss of renal tissue (75 per cent in dogs to 85 per cent in goats), 12 • 13 the amount a human can lose and still.. survive is not known precisely. It has been variously estimated through a combination of clinical observatimLand animal studies that loss of more than 75 per cent of. reflll mass is not compatible with human life. In report9 Poutasse, E. F.: Partial nephrectomy: New techniques, approach, operative indications and review of 51 cases. J. Ural., 88: 153-159, 1962. 10 Hinman, F., Jr. and Hutch, J. A.: Atrophic pyelonephritis from ureteral reflux without obstructive signs (reflux pyelonephritis). J. Ural., 87: 230-242, 1962. 11 Lodge, T. and Williams, J. L.: Compensatory structural and functional changes in the kidney. Brit. J. Urol., 37: 192-200, 1965. 12 Finkle, A. L., Karg, S. J., McGonigle, D. J. and Smith, D. R.: Renal functional capacities following one-stage nephrectomy and heminephrectomy in dogs. J. Ural., 89: 126-128, 1963. 13 Goldstein, A. E. and Abeshouse, B. S.: Partial resections of the kidney. J. Ural., 38: 15-42, 1937.
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ing on a series of 100 patients who underwent segmental nephrectomy for a variety of reasons, Culp cites a most illuminating case with a direct bearing upon this subject. 14 His patient had bilateral reduplication with stones in each of the lower segments. The lower right segment was removed leaving a functionally good upper segment. The asymptomatic left kidney was not treated at that time. Six years later the patient had a left perinephric abscess which was incised and drained. After 6 months of drainage segmental nephrectomy was performed "under most disagreeable circumstances." The flank healed, but the remaining upper portion of the kidney atrophied and became functionless. Noteworthy is the fact that blood urea measures only 52 mg. per 100 cc. Here, then, is a patient who appears to be surviving on less than 25 per cent of renal mass and represents the ultimate argument for exerting every effort toward salvage of these small, normally functioning renal segments. Responding to my inquiry about this patient, Culp15 replied in a letter dated September 23 1 1965, that: "The patient ... is still alive and was last seen here on the 10th of this month. His blood urea (not BUN) was 47. Our upper limit of normal is 40. It is true that he has done astonishingly well on a fraction of one kidney. Except for bronchial asthma, one severe episode of acute pyelonephritis, and blood pressure of 182/98 he has been remarkably well. This man is now 70 years old. His right kidney was operated on in 1950 and the left in 1956." CASE REPORTS
Case 1. A. L. G., Y-NHH 39-69-16, a 5-yearold girl, was first examined in 1953 during a third attack .in 6 months of chills, fever, right flank pain, burning and frequency of urination. The 2 previous attacks had responded promptly to antibacterials. Prior to her current illness her urologic history was normal. Growth and development were normal. Initial l).rologic survey revealed a small urethra, normal appearing bladder neck and bladder except for reduplication of the right ureteral orifice. Su1;>1:l~quent retrograde pyelogram showed a normal left kidney and ureter and a "normal" 14 Gulp, 0. S.: Heminephro-ureterectomy: Comparison of one-stage and two-stage operations. J. Urol., 83: 369-375, 1Q60. .. . . 15 Culp, 0. S.: Personal commumcat1on, September 23, 1965.
complete right reduplication (fig. 1, A). Phenolsulphonphthalein (PSP) excretion was 15 per cent from the left kidney in 15 minutes, while on the right 5 per cent was excreted from the upper segment and 10 per cent from the lower segment in the same period. Escherichia coli organisms were found in the right upper segment and bladder. The diagnosis was urethral stenosis and right upper segment acute pyelonephritis. The patient was treated by a single urethral dilation and antibacterials with satisfactory clinical response. In 1954, 1955, and 1958 she had isolated recurrences of sin1ilar attacks with similar findings, treatment, and response. In 1963, now an attractive, normally developed girl of 15, she returned with another attack of right loin pain, frequent painful urination, and E. coli in the urine. Excretory urography revealed a normal left upper tract, atrophy of the right lower segment, and normal architecture of the right upper collecting system (fig. 1, B). Voiding cystourethrography demonstrated right lower segment reflux (fig. 1, C). On cystourethroscopy a large, smooth bladder was found in the presence of a thickened, edematous, contracted bladder outlet as well as trigonitis. Phenolsulphonphthalein excretion from the left kidney was 20 per cent in 15 minutes. No excretion was obtained from the right lower segment. The right upper segment could not be catheterized after several attempts. The diagnosis was atrophic pyelonephritis of the right lower pole segment secondary to bladder neck contracture with reflux. Right lower pole partial nephrectomy, ureterectomy, anterior revision of the bladder neck, and revision of the external urinary meatus were performed during a single anesthetic period. Convalescence was completely smooth and the patient was dismissed from the hospital on the fourteenth postoperative day with sound wounds and urinating normally. Excretory urography demonstrated a satisfactorily functioning right upper segment (fig. 2, A). Sulfisoxizole was continued for a month after which the urine became sterile. No further medication was needed. Eleven months after operation the patient experienced acute right flank pain. Re-examination urologically showed a well healed, wide bladder neck, normal bladder, normal remaining right upper segment (fig. 2, B), and sterile urine. Phenolsulphonphthalein excretion was found to
°FIG. L Case LA, reirograde pyelogram, 1953. Initial study. Right complete reduplicatiotL cretory nrogram (5 mi1t.) December ID6:3. Right lower segment atrophy. Right upper segment hypertrophy. C, voiding cystourethrngram, December 1963. Right lower segment reflux and defined bladder neck.
FIG. 2. Case 1. A, excretory urogram (5 min.) January 1964, two weeks after right partial nephrec-· tomy. B, right retrograde pyeloureterogram, 1964, eleven months after right partial nephrectomy.
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FIG. 3. Case 2. A, excretory urogram, October 1960. Bilateral reduplication complete on right; partial on left. Initial study. B, voiding cystourethrogram, April 1961. Reflnx iu both right segments.
be 20 per cent in 15 n1inutes from the left kidney and 10 per cent from the right in the same period. The acute right flank pain was apparently caused by vigorous gymnastics. She has remained free of symptoms and infection. There have been no abnormal blood pressure changes. Case 2. C ..J., Y-NHH 45-78-06, a girl, born November 1956, with congenital heart disease had a patent ductus arteriosus divided and an anomalous right subclavian artery transsected the follmving year. There remained evidence of a small interventricular septa! defect without evidence of pulmonary hypertension and no further corrective cardiovascular surgery has been deemed necessary. Early in 1960 the patient began having enuresis and was found to haYe a urinary tract infection which responded promptly to antibacterials. After two recurrences of the infection an excretory urogram (fig. 3, A) was n1ade which revealed bilateral reduplication and the patient was referred for conrnltation in November 1960. Initial studies showed right complete reduplication with the ureters opening in a common sinus in the bladder wall. Reduplication on the left was partial
with the ureters joining about in the middle third of their course. The urethra was small, edematous, and thickened particularly in the distal third. The bladder and bladder neck appeared normal to inspection. l:rine was normal and sterile. Renal function as measured by PSP excretion and blood urea nitrogen determination was within normal limits. She was treated by urethral dilation and chemical cautery of the urethral mucosa. During the ensuing months several brief recurrences of infection responded promptly to antibacterials. However, in April 1961, she returned with fe,"er and painful, frequent urination. Re-examination revealed right reflux in both segments (fig. 3, B) and bladder neck thickening. A right ureteroneocystostomy (both ureters contained in one mucosal tunnel) and anterior revision of the bladder neck were performed without difficulty and convalescence vrns smooth. However, during the next 3,1,-i years the patient persisted in having intermittent transitory episodes of infection but with longer intervals between them. Each responded promptly to appropriate antibacterial drugs.
SEGMEi\i"TAL ATROPHIC PYELONEPHRITIS I:\' RENAL HEDUPLICATI0"i
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. ,-~IG .. 4. Case 2. A, ~xcr:etory urog;ram (5 min.) June 19G4. Right lo,ver segment atrophy. B C}Stollleth1ogram, 1%4. J\farker porntH to nght reflux. C, excretory urogram (5 min.) April
months after nght lower segment partial nephrectomy. Thus in 1964 further was made and of the right lower pole (fig. 4, A) as well as reflux into the lower segment (fig. 4, B) was demonstrated. The right upper pole appeared hypertrophied but otherwise normal and there was no reflux to the upper segment. The left side appeared unchanged. The urine was sterile, but the patient had recent]~· been taking an antibiotic. In October 1964, right partial nephrectomy and partial ureterectomy were performed. Demarcation between the affected lower twothirds and a hypertrophied but normal-appearing upper third was clear. During amputation of the lm1·er segment the renal vein ,vas found to extend into the hilum as a large sinus which required division and reconstruction to provide adequate venous drainage of the remaining kidney substance. Arterial supply 1rns not involved in the anomaly. Convalescence was smooth and the patient left the hospital on the fourteenth postoperative day urinating normally and with sterile urine. No urinary antiseptics ,rnre prescribed. She continued to recover rapidly and became obviously clinically imprnved and appeared to be a gay, frisky little girl. Six months after partial nephrectomy her urine was sterile, she was free of symptoms, and had gained 10 pounds over her
preoperative weight. An excretory urogram revealed excellent function (fig. 4, No1v, a year later, the patient continues to grow norma11y and has no further urological difficulties. COMMENT
If these 2 patients may be taken as rcprescmtative one must conclude that in children with this anomaly, coupled with bladder outlet or urethral anomalies, who become infected, the chance of secondary atrophic pyelonephritis in a single segment which continues to harbor the infection is a definite possibility. l<'urther, should the smaller of the two segments be unaffected it is certainly advisable to save it if at all possible. Culp's patient surviving with only one segment i, a case in point. Technical difficulties associated with partial nephrectomy are few and these are chieflv related to blood supply. With current techniques of vascular surgery appropriately adapted even these become fewer in number as management of the large venous sinus in the second patient demonstrates. Treatment of the whole problem, which is basically one of obstruction with superimposed infection, is necessary.
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SUMMARY
Partial resection of the kidneys is an old technique but apparently infrequently employed to salvage small upper pole segments in problems associated with reduplication. Clinical and laboratory data amply justify efforts to save these small masses of good kidney. Detailed case histories have been presented of 2 girls with atrophic pyelonephriti,, in the larger, lower segment of reno-ureteral reduplica-
tion and chronic, recurrent infection who were treated by segmental resection of the affected lower pole as well as appropriate correction of bladder neck and urethral obstruction with complete relief. Technical problems associated with the operation in the past have largely been conquered. Newer vascular techniques appropriately adapted further reduce the limiting factors to the procedure.