- Email: [email protected]
Ureteral Duplication: Clinical Findings and Therapy in 46 Children
V oL 105, Mar.
THE JOURNAL OF UROLOGY
Copyright © 1971 by The Williams & Wilkins Co.
Printed in U.S.A.
Pediatric Urology URETERAL DUPLICATION: CLINICAL FINDINGS AND THERAPY IN 46 CHILDREN ROBERT P. TIMOTHY,* ALLAN DECTERt
AND
ALAN D. PERLMUTTER
From the Surgical Service, Children's Hospital Medical Center and the Department of Surgery, Harvard ll1edical School, Boston, Massachusetts
Duplication of the ureter and renal pelvis, the most common congenital malformation of the upper urinary tract, occurred in 0.6 per cent of 51,000 autopsy cases reviewed by Campbell.1 Clinically, however, the incidence was 2 to 4 per cent in 2 large reviews of excretory urograms (IVPs) performed for urinary tract symptoms. 2- 4 Ureteral duplication may be partial or total, representing 2 different abnormalities of fetal development. In partial duplication a bifid or Y ureter is formed by bifurcation of a single ureteral bud prior to reaching the nephrogenic mass. In total duplication 2 ureteral buds arise directly from the wolffian system and ascend individually to the nephrogenic mass (fig. 1). The normal or orthotopic ureter drains the lower pole pelvis and inserts normally in the trigone. This ureter arises from the more caudal of the 2 ureteral buds and is the first to be incorporated into the future bladder as the wolffian duct is adsorbed into the urogenital sinus. The upper pole pelvis is drained by the ectopic ureter which inserts a varying Accepted for publication April 27, 1970. Read at annual meeting of New England Section, American Urological Association, Portsmouth, New Hampshire, September 27-30, 1967. * Current address: 131 State Street, Portland, Maine 04101. t Current address: Urology Associates, 611 Medical Arts Building, Winnipeg 1, Manitoba, Canada. 1 Campbell, M. F.: Embryology and anomalies of the urogenital tract. In: Urology, 2nd ed. Philadelphia: W. B. Saunders Co., p. 1505, 1963. 2 Nordmark, B.: Double formations of the pelves of the kidneys and the ureters. Embryology, occurrence and clinical significance. Acta Radio!., 30: 267, 1948. 3 Nation, E. F.: Duplication of the kidney and ureter: a statistical study of 230 new cases. J. Urol., 51: 456, 1944. 4 Ambrose, S.S. and Nicolson, W. P.: Ureteral reflux in duplicated ureters. J. Urol., 92: 439, 1964.
distance distal to the orthotopic orifice into any structure of wolffian origin. 4 MATERIAL
The 46 children with urinary tract disease associated with ureteral duplication who were treated at Children's Hospital Medical Center between 1962 and 1966 were evaluated with regard to laterality and level of duplication, symptoms and signs which prompted urologic investigation, radiographic changes noted and etiologic mechanisms associated with these changes, modes of therapy and results. Of 46 patients, 24 had total duplication only (fig. 2, A), 6 had a total duplication on one side with a bifid ureter on the other (fig. 2, B) and 16 children had unilateral or bilateral partial duplication only (fig. 3). No case of bifid pelves and no duplication of the upper third of the ureter were seen. Infection and fever were the most common presenting symptoms and abnormal physical signs were uncommon (fig. 4). Radiographic findings in the 41 totally duplicated renoureteral units were correlated with possible etiologic mechanisms (fig. 5). In 14 instances the orthotopic (lower) collecting system was dilated and vesicoureteral reflux was demonstrated into the dilated system in 11. No dilatation was seen in 6 kidneys and three were incompletely studied. The upper, ectopic system was dilated in 12 instances; 8 of these had demonstrable obstruction, 5 from ureterocele. In 9 kidneys decompensation involved both upper and lower collecting systems; 4 were obstructed due to a large ureterocele, in 3 cases reflux was noted into both segments and in 2 instances reflux was demonstrated in the orthotopic segment while the ectopic segment was obstructed. 445
446
TIMOTHY, DECTER AND PERLMUTTER
The 23 kidneys with bifid ureters were similarly evaluated and correlated with possible etiologic mechanisms (fig. 6). The most common morphologic abnormality, present in 12 of the 23 kidneys, was dilatation of both the upper and lower collecting systems; in 8 of these the dilatation was associated with stasis resulting from ureter-
'Y,,<
to-ureter reprocessing of urine above the common stem. THERAPY
Various surgical procedures were performed on these duplex renal units, including removal of non-functioning or poorly functioning renal parenchyma or poorly draining renal segments,
WOLFFIAN D
"1,,,..
s.
.,,..-.,,
-z..... 0
u'
V'
_) BLAST EMA RECTUM
4 WEEKS
5 WEEKS
KIDNEY
KIDNEY
6 WEEKS
7-8 WEEKS
Fm. 1. Embryology of total ureteral duplication. From Ambrose and Nicolson with permission of authors and The Williams & Wilkins Co. 4
A TOTAL OUPL!CAT!ON (24)
0~
5
-19
Average Age- 4 yrs. ( 2mos-10yrs.)
B TOTAL ANO PARTIAL OUPL /CATION
(6)
0-0 ~-6 Average Age- 4 yrs. ( 3wks.-10yrs.)
Fm. 2
447
URETERAL DUPLICATION
IA PARTIAL LOWER DUPLICATION (/2)
0-2 ~-10 Average Age-4.Syrs. ( I - I 3yrs)
B PARTIAL MIDDLE
ci 1J
~
,
DUPLICATION
~
)
,. .Et,~.\'"
r,9
t;J
I
~~--~--~~-~--~--~-~~~~-_J FIG. 3
INFECTION
PRESENTING SYMPTOMS ABDOMINAL PAIN
PALPABLE KIDNEY
PHYSICAL SIGNS
PROLAPSED URETEROCELE ~-~-~-~--~-~__j___--~-~--~-~~
%
0
20
40
60
80
100
Fw.4
correction of reflux, relief of obstruction and elimination of ureter-to-ureter reflux in a bifid system. To evaluate therapy the kidneys were grouped according to morphologic lesions and results were evaluated by symptomatic, bacteriologic and radiographic criteria. In totally duplicated collecting systems in which only the orthotopic segment was dilated (usually due to reflux), partial nephrectomy had variable results (fig. 7). Ureteroneocystostomy, by reimplantation of the ureter (with or without reflux) as a single unit within its common sheath,
was successful on all occasions. The single renal unit in which reimplantation was unsuccessful had been treated by isolating and reimplanting the orthotopic ureter which resulted in reflux into the ectopic segment. For totally duplex kidneys with decompensation of the ectopic segment (usually due to obstruction), partial nephrectomy was commonly performed and was successful (fig. 8). Common sheath reimplantation resulted in improvement in the 1 renal unit on which this was performed. Several different surgical approaches were
448
TIMOTHY, DECTER AND PERLMUTTER
used for the 9 total duplications with dilatation of both upper and lower segments (fig. 9). Pyeloureterostomy and common sheath reimplantation gave excellent improvement. Other procedures, including partial nephrectomy, incision of ureterocele and bladder neck operations, had varied results.
Of the 12 kidneys with partial duplication and dilatation of upper and lower segments (fig. 10), 3 were removed. Three of 4 kidneys treated nonoperatively improved. U reterovesical reimplantation for reflux provided satisfactory results in 3 instances. One of the 2 kidneys treated by pyeloureterostomy improved; the other was un-
TOTAL DUPLICATION (41 KIDNEYS) RADIOGRAPHIC CHANGES AND ETIOLOGY 14
12
REFLUX+OBST
OBSTRUCTION
?CAUSE
No Oecompensotion -6 9
Fw.5
BIFID URETERS ( 23 KIDNEYS) RADIOGRAPHIC CHANGES AND ETIOLOGY 2
4
?CAUSE
REFLUX+OBST
No Oecompensolton-5 FIG. 6
449
URETERAL DUPLICATION
improved due to technical difficulties resulting from extravasation of urine at the anastomosis. Four of 6 kidneys with bifid ureters and dilatation of the upper or lower segment alone did well (fig. 11). No operation was necessary in 2 instances. In 1 patient ureteroneocystostomy of the common stem was complicated by distal ureteral obstruction necessitating long-term drainage. In another patient a pyeloureteral anastomosis was performed between the lower segment pelvis and
TOTAL D(JPL/CAT!ON
( 9 KIDNEYS J
---~f- -B
PARTIAL NEPH-X (7)
-
0 TOTAL DUPLICATIONS (14 KIDNEYS/
NEPH-X
OTHER SURGERY (31
NON-OP
( 5)
•••• -
0 0
/A/Pf/OVEO
@
0 0 0
0 0 0
/INCHANGEO
Q1t10/1SE
0
~
0 0 0
0 0
Q NO FOLL0//1 /IP
NON - OP
~
0 0
M!PHOVEO
0 0 0 0
@ /INCHANGEO
0
0 OwonsE
0 0 0
(3)
0 0 0
PARTIAL D(JPL/CAT/ON (/2 KIDNEYS)
Q NO FOLLOW /IP NEPH-X
( 12 KIONEYS}
B
( 81
( 2)
~-------
8 0 0 0 /AIPIIOVEO
--
0 0 0 0 @
/INCHANGEO
PYE LO-
OTHER SURGERY
NON - OP
• •••• •
PARTIA}PYELO- -UV NEPH-X URETEROSTOMY REIMPLANT
PARTIAL NEPH-X
( 3)
0 0 0
TOTAL O(JPl!CAT/0/il
•
OTHER SURGERY
Fm. 9. Total duplication with disease of both segments.
Fm. 7. Total duplication with lower segment disease. Empty circle indicates no patient in that category.
0 0 0 0
( 3)
• •••
0 0 0 0 •
PARTIAL PYE LOUV NEPH-X URETEROSTOMY REIMPLANT ( 31 (51
•
(I)
~
0 0 0
PYELOUV URETEROSTOMY REIMPLANT
(I)
0 0 0 0
/AIPHOVEO
~
~
0
0
0
0
@ /INCHANGEO @ I/IOHSE
0 0
0
(5)
0
0
Q NO FOLL0//1 /IP
Fm. 10. Partial duplication with disease of both segments.
OTHER SURGERY ( 1)
-
• 0 0
8 0 0 0
0 0 ® 0
Q 11'0/ISE Q NO FOlL0//1 /IP
Fm. 8. Total duplication with upper segment disease.
the upper ureter. The lower pelvis drained poorly and partial nephrectomy was ultimately performed. DISCUSSION
Ureteral duplication, although a common anatomical variant, is not usually symptomatic . Symptomatic disease in duplicated renal collecting systems is most often associated with urinary infection. In this clinical series of total
450
TIMOTHY, DECTER AND PERLMUTTER
PARTIAL OUPL!CAT/ON (4KIONEYSJ
NEPH-X
PARTIAL NEPH-X
0 0 0 0 •
PYE LOUV URETEROSTOMY REIMPLANT
• • (l)
(l)
0 0
0 0 0 INPHOVEO
0 @
IJNCHANGEO
OTH SURGERY NON:- E OP l
(2)
0
0
0 0 0 0
0 0 0 0
Q WORSE Q NO FOLLOW /JP
Fm. 11. Partial duplication with disease of lower segment or upper segment. and partial ureteral duplication, the female patient predominance was 85 per cent compared to a 55 to 62 per cent predominance in nonclinical reviews.3· 5 The increased number of female patients in our clinical series as compared to autopsy and pyelographic series correlates with the predominance of urinary infection in female individuals. The greater prevalence of ureteral anomalies in female subjects may be related to the wolffian origin of the ureter. The wolffian system in female subjects serves only as a precurser to urinary structures and its adsorption early in fetal life may predispose to greater variability in its derivative structures. In cases of total duplication the orthotopic ureter often has a short intramural course and is therefore prone to reflux. 6 • 7 The ureter to the upper renal segment inserts more caudally in the 5 Whitaker, J. and Danks, D. M.: A study of the inheritance of duplication of the kidneys and ureters. J. Urol., 95: 176, 1966. 6 Williams, D. I.: Ureteral duplications and ectopia. In: Paediatric Urology. London: Butterworth & Co., Ltd., p. 198, 1968. 7 Stephens, F. D.: Anatomical vagaries of double ureters. In: Congenital Malformations of the Rectum, Anus, and Genito-Urinary Tracts. Baltimore: The Williams & Wilkins Co., p. 172, 1963.
bladder or anywhere from the trigone to the perineum in female subjects and to the verumontanum in male subjects. Varying degrees of obstruction can result from the ectopic location of the orifice and from ectopic ureteroceles. Stasis in partially duplicated or bifid ureters commonly results from ureter-to-ureter reflux, the yo-yo phenomenon. In this series of symptomatic patients there were no bifid pelves or partial duplications involving the upper third of the ureter. Ureters duplicated at the mid or lower third allow for greater ureter-to-ureter reprocessing of urine and more urinary stasis; more significant damage to the collecting system and renal parenchyma can result. 8 Treatment of damaged renal units should be selective, depending upon the type and degree of disturbed function and morphology. Non-operative antibacterial therapy may be sufficient. N ephrectomy or partial nephrectomy may be necessary for severe parenchymal damage or deformity. Salvable parenchyma should not be sacrificed for the sake of expediency prior to attempts at correcting underlying dynamic abnormalities. Ureteral duplication is often bilateral, occurring in 18 of 46 children in our series, and in these patients a more conservative operation is imperative. In cases of total duplication with reflux into the orthotopic ureter, reimplantation of both ureters, preserving their common sheath, is simple and effective. 9 • 10 The ureters should be handled as a single unit. Satisfactory results can be expected, with no obstruction of the uninvolved ureter. In cases of bifid ureters with stasis, a ureteropyelostomy or high ureteroureterostomy converts the system into a bifid pelvis with improved dynamics of emptying. 11-13 The upper ureter or pelvis should be anastomosed to the lower system since connecting the lower pelvis to the upper system has often resulted in failure. 14 8 Kaplan, N. and Elkin, M.: Bifid renal pelves and ureters. Radiographic and cinefluorographic observations. Brit. J. Urol., 40: 235, 1968. 9 Amar, A. D.: Reflux in duplicated ureters. Brit. J. Urol., 40: 385, 1968. 10 Politano, V. A. and Leadbetter, W. F.: An operative technique for the correction of vesicoureteral reflux. J. Urol., 79: 932, 1958. 11 Gibson, T. E.: A new operation for ureteral ectopia: case report. J. Urol., 77: 414, 1957. 12 Swenson, 0. and Ratner, I. A.: Pyeloureterostomy for treatment of symptomatic ureteral duplications in children. J. Urol., 88: 184, 1962. 13 Pearson, H. H.: Results of pyelopyelostomy for bifid ureters. Brit. J. Urol., 40: 483, 1968. 14 Williams, D. I.: Personal communication.
URETERAL DUPLICATION SUMMARY
A retrospective study was made of 46 children with partial or total duplication of one or both ureters. The most significant abnormality of the upper renal segment associated with totally duplicated ureters was obstruction by ureterocele or by the ectopic position of the ureteral orifice; the most common cause of decompensation of the lower renal segment was vesicoureteral reflux into
451
the orthotopic ureter. In partially duplicated ureters, ureter-to-ureter reflux was the most important factor in upper tract decompensation. N ephrectomy or partial nephrectomy as preferred treatment should be avoided whenever salvable renal parenchyma is present. Common sheath reimplantation of totally duplicated ureters and pyeloureterostomy for bifid ureters have proved to be simple, effective procedures which may spare renal parenchyma.
THE JOURNAL OF UROLOGY
Copyright © 1971 by The Williams & Wilkins Co.
Printed in U.S.A.
Pediatric Urology URETERAL DUPLICATION: CLINICAL FINDINGS AND THERAPY IN 46 CHILDREN ROBERT P. TIMOTHY,* ALLAN DECTERt
AND
ALAN D. PERLMUTTER
From the Surgical Service, Children's Hospital Medical Center and the Department of Surgery, Harvard ll1edical School, Boston, Massachusetts
Duplication of the ureter and renal pelvis, the most common congenital malformation of the upper urinary tract, occurred in 0.6 per cent of 51,000 autopsy cases reviewed by Campbell.1 Clinically, however, the incidence was 2 to 4 per cent in 2 large reviews of excretory urograms (IVPs) performed for urinary tract symptoms. 2- 4 Ureteral duplication may be partial or total, representing 2 different abnormalities of fetal development. In partial duplication a bifid or Y ureter is formed by bifurcation of a single ureteral bud prior to reaching the nephrogenic mass. In total duplication 2 ureteral buds arise directly from the wolffian system and ascend individually to the nephrogenic mass (fig. 1). The normal or orthotopic ureter drains the lower pole pelvis and inserts normally in the trigone. This ureter arises from the more caudal of the 2 ureteral buds and is the first to be incorporated into the future bladder as the wolffian duct is adsorbed into the urogenital sinus. The upper pole pelvis is drained by the ectopic ureter which inserts a varying Accepted for publication April 27, 1970. Read at annual meeting of New England Section, American Urological Association, Portsmouth, New Hampshire, September 27-30, 1967. * Current address: 131 State Street, Portland, Maine 04101. t Current address: Urology Associates, 611 Medical Arts Building, Winnipeg 1, Manitoba, Canada. 1 Campbell, M. F.: Embryology and anomalies of the urogenital tract. In: Urology, 2nd ed. Philadelphia: W. B. Saunders Co., p. 1505, 1963. 2 Nordmark, B.: Double formations of the pelves of the kidneys and the ureters. Embryology, occurrence and clinical significance. Acta Radio!., 30: 267, 1948. 3 Nation, E. F.: Duplication of the kidney and ureter: a statistical study of 230 new cases. J. Urol., 51: 456, 1944. 4 Ambrose, S.S. and Nicolson, W. P.: Ureteral reflux in duplicated ureters. J. Urol., 92: 439, 1964.
distance distal to the orthotopic orifice into any structure of wolffian origin. 4 MATERIAL
The 46 children with urinary tract disease associated with ureteral duplication who were treated at Children's Hospital Medical Center between 1962 and 1966 were evaluated with regard to laterality and level of duplication, symptoms and signs which prompted urologic investigation, radiographic changes noted and etiologic mechanisms associated with these changes, modes of therapy and results. Of 46 patients, 24 had total duplication only (fig. 2, A), 6 had a total duplication on one side with a bifid ureter on the other (fig. 2, B) and 16 children had unilateral or bilateral partial duplication only (fig. 3). No case of bifid pelves and no duplication of the upper third of the ureter were seen. Infection and fever were the most common presenting symptoms and abnormal physical signs were uncommon (fig. 4). Radiographic findings in the 41 totally duplicated renoureteral units were correlated with possible etiologic mechanisms (fig. 5). In 14 instances the orthotopic (lower) collecting system was dilated and vesicoureteral reflux was demonstrated into the dilated system in 11. No dilatation was seen in 6 kidneys and three were incompletely studied. The upper, ectopic system was dilated in 12 instances; 8 of these had demonstrable obstruction, 5 from ureterocele. In 9 kidneys decompensation involved both upper and lower collecting systems; 4 were obstructed due to a large ureterocele, in 3 cases reflux was noted into both segments and in 2 instances reflux was demonstrated in the orthotopic segment while the ectopic segment was obstructed. 445
446
TIMOTHY, DECTER AND PERLMUTTER
The 23 kidneys with bifid ureters were similarly evaluated and correlated with possible etiologic mechanisms (fig. 6). The most common morphologic abnormality, present in 12 of the 23 kidneys, was dilatation of both the upper and lower collecting systems; in 8 of these the dilatation was associated with stasis resulting from ureter-
'Y,,<
to-ureter reprocessing of urine above the common stem. THERAPY
Various surgical procedures were performed on these duplex renal units, including removal of non-functioning or poorly functioning renal parenchyma or poorly draining renal segments,
WOLFFIAN D
"1,,,..
s.
.,,..-.,,
-z..... 0
u'
V'
_) BLAST EMA RECTUM
4 WEEKS
5 WEEKS
KIDNEY
KIDNEY
6 WEEKS
7-8 WEEKS
Fm. 1. Embryology of total ureteral duplication. From Ambrose and Nicolson with permission of authors and The Williams & Wilkins Co. 4
A TOTAL OUPL!CAT!ON (24)
0~
5
-19
Average Age- 4 yrs. ( 2mos-10yrs.)
B TOTAL ANO PARTIAL OUPL /CATION
(6)
0-0 ~-6 Average Age- 4 yrs. ( 3wks.-10yrs.)
Fm. 2
447
URETERAL DUPLICATION
IA PARTIAL LOWER DUPLICATION (/2)
0-2 ~-10 Average Age-4.Syrs. ( I - I 3yrs)
B PARTIAL MIDDLE
ci 1J
~
,
DUPLICATION
~
)
,. .Et,~.\'"
r,9
t;J
I
~~--~--~~-~--~--~-~~~~-_J FIG. 3
INFECTION
PRESENTING SYMPTOMS ABDOMINAL PAIN
PALPABLE KIDNEY
PHYSICAL SIGNS
PROLAPSED URETEROCELE ~-~-~-~--~-~__j___--~-~--~-~~
%
0
20
40
60
80
100
Fw.4
correction of reflux, relief of obstruction and elimination of ureter-to-ureter reflux in a bifid system. To evaluate therapy the kidneys were grouped according to morphologic lesions and results were evaluated by symptomatic, bacteriologic and radiographic criteria. In totally duplicated collecting systems in which only the orthotopic segment was dilated (usually due to reflux), partial nephrectomy had variable results (fig. 7). Ureteroneocystostomy, by reimplantation of the ureter (with or without reflux) as a single unit within its common sheath,
was successful on all occasions. The single renal unit in which reimplantation was unsuccessful had been treated by isolating and reimplanting the orthotopic ureter which resulted in reflux into the ectopic segment. For totally duplex kidneys with decompensation of the ectopic segment (usually due to obstruction), partial nephrectomy was commonly performed and was successful (fig. 8). Common sheath reimplantation resulted in improvement in the 1 renal unit on which this was performed. Several different surgical approaches were
448
TIMOTHY, DECTER AND PERLMUTTER
used for the 9 total duplications with dilatation of both upper and lower segments (fig. 9). Pyeloureterostomy and common sheath reimplantation gave excellent improvement. Other procedures, including partial nephrectomy, incision of ureterocele and bladder neck operations, had varied results.
Of the 12 kidneys with partial duplication and dilatation of upper and lower segments (fig. 10), 3 were removed. Three of 4 kidneys treated nonoperatively improved. U reterovesical reimplantation for reflux provided satisfactory results in 3 instances. One of the 2 kidneys treated by pyeloureterostomy improved; the other was un-
TOTAL DUPLICATION (41 KIDNEYS) RADIOGRAPHIC CHANGES AND ETIOLOGY 14
12
REFLUX+OBST
OBSTRUCTION
?CAUSE
No Oecompensotion -6 9
Fw.5
BIFID URETERS ( 23 KIDNEYS) RADIOGRAPHIC CHANGES AND ETIOLOGY 2
4
?CAUSE
REFLUX+OBST
No Oecompensolton-5 FIG. 6
449
URETERAL DUPLICATION
improved due to technical difficulties resulting from extravasation of urine at the anastomosis. Four of 6 kidneys with bifid ureters and dilatation of the upper or lower segment alone did well (fig. 11). No operation was necessary in 2 instances. In 1 patient ureteroneocystostomy of the common stem was complicated by distal ureteral obstruction necessitating long-term drainage. In another patient a pyeloureteral anastomosis was performed between the lower segment pelvis and
TOTAL D(JPL/CAT!ON
( 9 KIDNEYS J
---~f- -B
PARTIAL NEPH-X (7)
-
0 TOTAL DUPLICATIONS (14 KIDNEYS/
NEPH-X
OTHER SURGERY (31
NON-OP
( 5)
•••• -
0 0
/A/Pf/OVEO
@
0 0 0
0 0 0
/INCHANGEO
Q1t10/1SE
0
~
0 0 0
0 0
Q NO FOLL0//1 /IP
NON - OP
~
0 0
M!PHOVEO
0 0 0 0
@ /INCHANGEO
0
0 OwonsE
0 0 0
(3)
0 0 0
PARTIAL D(JPL/CAT/ON (/2 KIDNEYS)
Q NO FOLLOW /IP NEPH-X
( 12 KIONEYS}
B
( 81
( 2)
~-------
8 0 0 0 /AIPIIOVEO
--
0 0 0 0 @
/INCHANGEO
PYE LO-
OTHER SURGERY
NON - OP
• •••• •
PARTIA}PYELO- -UV NEPH-X URETEROSTOMY REIMPLANT
PARTIAL NEPH-X
( 3)
0 0 0
TOTAL O(JPl!CAT/0/il
•
OTHER SURGERY
Fm. 9. Total duplication with disease of both segments.
Fm. 7. Total duplication with lower segment disease. Empty circle indicates no patient in that category.
0 0 0 0
( 3)
• •••
0 0 0 0 •
PARTIAL PYE LOUV NEPH-X URETEROSTOMY REIMPLANT ( 31 (51
•
(I)
~
0 0 0
PYELOUV URETEROSTOMY REIMPLANT
(I)
0 0 0 0
/AIPHOVEO
~
~
0
0
0
0
@ /INCHANGEO @ I/IOHSE
0 0
0
(5)
0
0
Q NO FOLL0//1 /IP
Fm. 10. Partial duplication with disease of both segments.
OTHER SURGERY ( 1)
-
• 0 0
8 0 0 0
0 0 ® 0
Q 11'0/ISE Q NO FOlL0//1 /IP
Fm. 8. Total duplication with upper segment disease.
the upper ureter. The lower pelvis drained poorly and partial nephrectomy was ultimately performed. DISCUSSION
Ureteral duplication, although a common anatomical variant, is not usually symptomatic . Symptomatic disease in duplicated renal collecting systems is most often associated with urinary infection. In this clinical series of total
450
TIMOTHY, DECTER AND PERLMUTTER
PARTIAL OUPL!CAT/ON (4KIONEYSJ
NEPH-X
PARTIAL NEPH-X
0 0 0 0 •
PYE LOUV URETEROSTOMY REIMPLANT
• • (l)
(l)
0 0
0 0 0 INPHOVEO
0 @
IJNCHANGEO
OTH SURGERY NON:- E OP l
(2)
0
0
0 0 0 0
0 0 0 0
Q WORSE Q NO FOLLOW /JP
Fm. 11. Partial duplication with disease of lower segment or upper segment. and partial ureteral duplication, the female patient predominance was 85 per cent compared to a 55 to 62 per cent predominance in nonclinical reviews.3· 5 The increased number of female patients in our clinical series as compared to autopsy and pyelographic series correlates with the predominance of urinary infection in female individuals. The greater prevalence of ureteral anomalies in female subjects may be related to the wolffian origin of the ureter. The wolffian system in female subjects serves only as a precurser to urinary structures and its adsorption early in fetal life may predispose to greater variability in its derivative structures. In cases of total duplication the orthotopic ureter often has a short intramural course and is therefore prone to reflux. 6 • 7 The ureter to the upper renal segment inserts more caudally in the 5 Whitaker, J. and Danks, D. M.: A study of the inheritance of duplication of the kidneys and ureters. J. Urol., 95: 176, 1966. 6 Williams, D. I.: Ureteral duplications and ectopia. In: Paediatric Urology. London: Butterworth & Co., Ltd., p. 198, 1968. 7 Stephens, F. D.: Anatomical vagaries of double ureters. In: Congenital Malformations of the Rectum, Anus, and Genito-Urinary Tracts. Baltimore: The Williams & Wilkins Co., p. 172, 1963.
bladder or anywhere from the trigone to the perineum in female subjects and to the verumontanum in male subjects. Varying degrees of obstruction can result from the ectopic location of the orifice and from ectopic ureteroceles. Stasis in partially duplicated or bifid ureters commonly results from ureter-to-ureter reflux, the yo-yo phenomenon. In this series of symptomatic patients there were no bifid pelves or partial duplications involving the upper third of the ureter. Ureters duplicated at the mid or lower third allow for greater ureter-to-ureter reprocessing of urine and more urinary stasis; more significant damage to the collecting system and renal parenchyma can result. 8 Treatment of damaged renal units should be selective, depending upon the type and degree of disturbed function and morphology. Non-operative antibacterial therapy may be sufficient. N ephrectomy or partial nephrectomy may be necessary for severe parenchymal damage or deformity. Salvable parenchyma should not be sacrificed for the sake of expediency prior to attempts at correcting underlying dynamic abnormalities. Ureteral duplication is often bilateral, occurring in 18 of 46 children in our series, and in these patients a more conservative operation is imperative. In cases of total duplication with reflux into the orthotopic ureter, reimplantation of both ureters, preserving their common sheath, is simple and effective. 9 • 10 The ureters should be handled as a single unit. Satisfactory results can be expected, with no obstruction of the uninvolved ureter. In cases of bifid ureters with stasis, a ureteropyelostomy or high ureteroureterostomy converts the system into a bifid pelvis with improved dynamics of emptying. 11-13 The upper ureter or pelvis should be anastomosed to the lower system since connecting the lower pelvis to the upper system has often resulted in failure. 14 8 Kaplan, N. and Elkin, M.: Bifid renal pelves and ureters. Radiographic and cinefluorographic observations. Brit. J. Urol., 40: 235, 1968. 9 Amar, A. D.: Reflux in duplicated ureters. Brit. J. Urol., 40: 385, 1968. 10 Politano, V. A. and Leadbetter, W. F.: An operative technique for the correction of vesicoureteral reflux. J. Urol., 79: 932, 1958. 11 Gibson, T. E.: A new operation for ureteral ectopia: case report. J. Urol., 77: 414, 1957. 12 Swenson, 0. and Ratner, I. A.: Pyeloureterostomy for treatment of symptomatic ureteral duplications in children. J. Urol., 88: 184, 1962. 13 Pearson, H. H.: Results of pyelopyelostomy for bifid ureters. Brit. J. Urol., 40: 483, 1968. 14 Williams, D. I.: Personal communication.
URETERAL DUPLICATION SUMMARY
A retrospective study was made of 46 children with partial or total duplication of one or both ureters. The most significant abnormality of the upper renal segment associated with totally duplicated ureters was obstruction by ureterocele or by the ectopic position of the ureteral orifice; the most common cause of decompensation of the lower renal segment was vesicoureteral reflux into
451
the orthotopic ureter. In partially duplicated ureters, ureter-to-ureter reflux was the most important factor in upper tract decompensation. N ephrectomy or partial nephrectomy as preferred treatment should be avoided whenever salvable renal parenchyma is present. Common sheath reimplantation of totally duplicated ureters and pyeloureterostomy for bifid ureters have proved to be simple, effective procedures which may spare renal parenchyma.