NONDUPLICATION OF UPPER URINARY TRACT ASSOCIATED WITH HORSESHOE KIDNEY AND BILATERAL URETERAL ECTOPIA MAJ. RICHARD H. BARD (MC) USAF MAJ. HUNTER WELLES (MC) USAF From the Urology Service, and the Radiology Department, United States Air Force Medical Center, Scott Air Force Base, Illinois
ABSTRACT - A three-month-old white baby girl wasjound to have marked hydroureteronephrosis assodated with ectopic, nonduplicated ureters and horseshoe kidney. Ha- clinical and radiographic course are discussed.
The association of bilateral ureteral ectopia, solitary upper collecting systems, and horseshoe kidney is exceedingly rare. There are, however, sparse reports in the literature of ectopic ureters in nonduplicated systems.1-3 A persistent finding in al1 cases to date is a deficit in the development of the bladder neck resulting in urinary incontinence. As of this time, there are no reported cases of horseshoe kidney associated with bilateral nonduplicated ectopic ureters. Embryologically, a horseshoe kidney represents the abnormal fusion of the two nephrogenic blastemas between the fourth and eighth week of embryogenesis. This fusion usually retards the ascent of the kidneys and interferes with renal rotation so that the ureters exit fi-om the kidney anteriorly. In ureteral ectopia failure of the ureter to separate fï-om the mesonephric duet causes it to be carried caudally with the lower end of the duet. Since the mesonephric duet is the precursor of the posterior urethra, it is not unusual to find ectopic ureters entering at this point. An interesting case combining al1 of these urinary tract anomalies wil1 be discussed. Case Report The patient, a three-month-old baby girl, was w& until she presented, at the U.S. Air
784
Force Regional Hospita1 (Minot) with fever, irritability, and decreased appetite. She had been the product of a full-term gestation delivered by cesarean section because of cephalopelvic disproportion and premature rupture of membranes. Physical examination revealed a normally developed febrile three-month-old female infant. Her cardiac apex was noted to be on the right, and a grade IWI systolic murmur was auscultated along the right sternal border. Lumbar puncture was normal; urinalysis revealed 2 plus proteinuria and 60 to 80 white blood cells per high-power field. Subsequent urine cultures grew out greater than 100,000colonies of Escherichia coli sensitive to ampicillin. Chest x-ray film revealed situs solitus with dextroversion. The patient’s urosepsis responded to intravenous administration of ampicillin, A voiding cystourethrogram demonstrated a smoothcontoured bladder without evidente of vesicoureteral reflux. Intravenous pyelography revealed bilateral class IV hydroureteronephrosis (Fig. 1A). The patient was transferred to U.S. Air Force Medical Center Scott for further evaluation in August, 1973. On admission, the patient was afebrile and comfortable. Physical examination was within normal limits and corroborated the findings at
UROIAIGY / JUNElW5
/ VOLUMEV.NUMBER6
FIGUFW 1. (A) Delayed jîlm jì-om preoperative intravenous pyelogram demonstrating marked bilateral hydroureteronephroszS. (B) lntravenous pyelogt-am twenty days post biiúteral ureteral reimplantation revealing increased hydroureteronephrosi; suprapubic cystostomy tube is present. (C) Intravenous pyelogram, October 23, 1973, demonstrating decrease in dilatation of collecting systems; note media1 orientation of lower pole collecting systems suggesting horseshoe kidney. (D) lntravenous pyelogram eight months postoperatively indicating further reduction in dilutation of upper tracts. (E) Voiding cystourethrogram with refux into left ureteral stump; note entrance of distal stump into urethra distal to bladder neck and internal vesical sphincter. (F) Zntravenous pyelogram thirteen nwnths postoperatively; ureters now appear normul in caláber, and upper tracts are markedly improved particularly on right.
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/ JUNE 1975 / VOLUME
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Minot Regional Hospital. Significant laboratory values were a bloed urea nitrogen of 27 mg. per 100 ml. and a hematocrit of 30. Creatinine clearante corrected for body surface was 15 cc. per minute; three initial urine cultures were sterile. On August 7, 1973, panendoscopic examination of the patient’s lower urinary tract revealed normal vesical urothelium, but complete absence of the trigone and ureteral orifices. On antegrade examination of the urethra, the ureteral orifices were seen entering the urethra just distal to the bladder neck. On August 10, 1973, at the age of four months, the patient underwent bilateral ureteroneocystostomies; postoperative course was uneventful. A fellow-up intravenous pyelogram on August 30th indicated some increase in the hydroureteronephrosis (Fig. 1B). The patient was discharged on chronic antibiosis and suprapubic drainage. At time of discharge, her blood urea nitrogen was 23 mg. and serum creatinine 0.6 mg. per 100 ml. The patient was readmitted three weeks later because of a pseudomonas urinary tract infection and sepsis. Only after removal of the suprapubic tube did urine cultures become sterile. Intravenous pyelogram on October 23, 1973, demonstrated a decrease in the dilatation of the upper urinary tract (Fig. 1C). At this time, the possibility of a horseshoe kidney was first entertained. On November 13, 1973, voiding cystourethrogram revealed class 111 high-pressure vesicoureteral reflux on the left. She was discharged on chronic sulfisoxazole (Gantrisin) antibiosis with a creatinine clearance of 63 cc. per minute. In April, 1974, while on vacation with her family, the patient was treated at the Children’s Hospital, Birmingham, Alabama, for an E. coli urinary tract infection. Intravenous pyelogram revealed further decrease in the dilatation of the calyceal systems of both kidneys (Fig. 1D). A voiding cystourethrogram revealed reflux into the left ureteral stump, but no reflux into the reimplanted ureters (Fig. 1E). A goed sphincteric impression was also noted at the vesical neck on the voiding cystourethrogram. Intravenous pyelogram in September, 1974, revealed further decrease in the caliber of the collecting systems with obvious medial orientation of the lower pole calyces, strongly suggesting horseshoe kidney (Fig. 1F).
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At the present time, the patient is somewhat smal1 in stature although she appears to have reached her motor and cerebral milestones at the appropriate times. She is in the process of being toilet trained and appears to have normal vesical sphincter control. NO further surgery is planned unless chronic infection in the left ureteral stump necessitates removal. The patient is maintained on nitroíùrantoin (Furadantin) suspension, 25 mg. daily, for urinary tract prophylaxis. Comment Published case reports of solitary ureteral ectopia indicate a high incidence of postreimplantation incontinence. 3 The embryologie deficits in trigonal development places the reimplanted ureter at substantial risk. Of the 23 reported cases of single and 10 cases of bilateral nonduplicated ureteral ectopia, progressive upper tract decompensation ultimately resulted in urinary diversion in several instances. lm3Although preservation of renal function must be of paramount concern,4 the decision to divert or reconstruct must be made on an individual basis. This case report is of considerable importante to pediatrie urologists for several reasons: (1) contrary to previous published reports, this patient was born with a normal bladder neck and sphincteric mechanism; (2) a normal bladder capacity is evident; (3) she is continent of urine; (4) her ureteral ectopia is associated with a horseshoe kidney; and (5) she was “cured” by ureteral reimplantation and did not require an antiincontinence procedure or urinary diversion. In light of this unusual case, it also may be necessary to reevaluate our present concept of the embryologie development of the bladder neck. Montefiore Hospital and Medical Center 111 East 210th Street Bronx, New York 10467 (DR. BARD) References Cox, C. E., and HUTCH, J. A.: Bilateral single ectopic ureter: a report of 2 cases and review of the literature, J, Urol. 95: 493 (1966). JOHNSON,J. H., and DAVENPORT,T. J.: The single ectopic ureter, Br. J. Urol. 41: 428 (1969). WILLIAMS, D. I., and LIGHTWOOD, R. C.: Bilateral single ectopic ureters, ibid. 44: 267 (1972). BARD, R. H.: Early high diversion, Urology 3: 369 (1974).
UROLOGY / JUNE 1975 /
VOLUME V, NUMBER 6
ABSTRACT - A three-month-old white baby girl wasjound to have marked hydroureteronephrosis assodated with ectopic, nonduplicated ureters and horseshoe kidney. Ha- clinical and radiographic course are discussed.
The association of bilateral ureteral ectopia, solitary upper collecting systems, and horseshoe kidney is exceedingly rare. There are, however, sparse reports in the literature of ectopic ureters in nonduplicated systems.1-3 A persistent finding in al1 cases to date is a deficit in the development of the bladder neck resulting in urinary incontinence. As of this time, there are no reported cases of horseshoe kidney associated with bilateral nonduplicated ectopic ureters. Embryologically, a horseshoe kidney represents the abnormal fusion of the two nephrogenic blastemas between the fourth and eighth week of embryogenesis. This fusion usually retards the ascent of the kidneys and interferes with renal rotation so that the ureters exit fi-om the kidney anteriorly. In ureteral ectopia failure of the ureter to separate fï-om the mesonephric duet causes it to be carried caudally with the lower end of the duet. Since the mesonephric duet is the precursor of the posterior urethra, it is not unusual to find ectopic ureters entering at this point. An interesting case combining al1 of these urinary tract anomalies wil1 be discussed. Case Report The patient, a three-month-old baby girl, was w& until she presented, at the U.S. Air
784
Force Regional Hospita1 (Minot) with fever, irritability, and decreased appetite. She had been the product of a full-term gestation delivered by cesarean section because of cephalopelvic disproportion and premature rupture of membranes. Physical examination revealed a normally developed febrile three-month-old female infant. Her cardiac apex was noted to be on the right, and a grade IWI systolic murmur was auscultated along the right sternal border. Lumbar puncture was normal; urinalysis revealed 2 plus proteinuria and 60 to 80 white blood cells per high-power field. Subsequent urine cultures grew out greater than 100,000colonies of Escherichia coli sensitive to ampicillin. Chest x-ray film revealed situs solitus with dextroversion. The patient’s urosepsis responded to intravenous administration of ampicillin, A voiding cystourethrogram demonstrated a smoothcontoured bladder without evidente of vesicoureteral reflux. Intravenous pyelography revealed bilateral class IV hydroureteronephrosis (Fig. 1A). The patient was transferred to U.S. Air Force Medical Center Scott for further evaluation in August, 1973. On admission, the patient was afebrile and comfortable. Physical examination was within normal limits and corroborated the findings at
UROIAIGY / JUNElW5
/ VOLUMEV.NUMBER6
FIGUFW 1. (A) Delayed jîlm jì-om preoperative intravenous pyelogram demonstrating marked bilateral hydroureteronephroszS. (B) lntravenous pyelogt-am twenty days post biiúteral ureteral reimplantation revealing increased hydroureteronephrosi; suprapubic cystostomy tube is present. (C) Intravenous pyelogram, October 23, 1973, demonstrating decrease in dilatation of collecting systems; note media1 orientation of lower pole collecting systems suggesting horseshoe kidney. (D) lntravenous pyelogram eight months postoperatively indicating further reduction in dilutation of upper tracts. (E) Voiding cystourethrogram with refux into left ureteral stump; note entrance of distal stump into urethra distal to bladder neck and internal vesical sphincter. (F) Zntravenous pyelogram thirteen nwnths postoperatively; ureters now appear normul in caláber, and upper tracts are markedly improved particularly on right.
UROLOGY
/ JUNE 1975 / VOLUME
V, NUMBER
6
785
Minot Regional Hospital. Significant laboratory values were a bloed urea nitrogen of 27 mg. per 100 ml. and a hematocrit of 30. Creatinine clearante corrected for body surface was 15 cc. per minute; three initial urine cultures were sterile. On August 7, 1973, panendoscopic examination of the patient’s lower urinary tract revealed normal vesical urothelium, but complete absence of the trigone and ureteral orifices. On antegrade examination of the urethra, the ureteral orifices were seen entering the urethra just distal to the bladder neck. On August 10, 1973, at the age of four months, the patient underwent bilateral ureteroneocystostomies; postoperative course was uneventful. A fellow-up intravenous pyelogram on August 30th indicated some increase in the hydroureteronephrosis (Fig. 1B). The patient was discharged on chronic antibiosis and suprapubic drainage. At time of discharge, her blood urea nitrogen was 23 mg. and serum creatinine 0.6 mg. per 100 ml. The patient was readmitted three weeks later because of a pseudomonas urinary tract infection and sepsis. Only after removal of the suprapubic tube did urine cultures become sterile. Intravenous pyelogram on October 23, 1973, demonstrated a decrease in the dilatation of the upper urinary tract (Fig. 1C). At this time, the possibility of a horseshoe kidney was first entertained. On November 13, 1973, voiding cystourethrogram revealed class 111 high-pressure vesicoureteral reflux on the left. She was discharged on chronic sulfisoxazole (Gantrisin) antibiosis with a creatinine clearance of 63 cc. per minute. In April, 1974, while on vacation with her family, the patient was treated at the Children’s Hospital, Birmingham, Alabama, for an E. coli urinary tract infection. Intravenous pyelogram revealed further decrease in the dilatation of the calyceal systems of both kidneys (Fig. 1D). A voiding cystourethrogram revealed reflux into the left ureteral stump, but no reflux into the reimplanted ureters (Fig. 1E). A goed sphincteric impression was also noted at the vesical neck on the voiding cystourethrogram. Intravenous pyelogram in September, 1974, revealed further decrease in the caliber of the collecting systems with obvious medial orientation of the lower pole calyces, strongly suggesting horseshoe kidney (Fig. 1F).
786
At the present time, the patient is somewhat smal1 in stature although she appears to have reached her motor and cerebral milestones at the appropriate times. She is in the process of being toilet trained and appears to have normal vesical sphincter control. NO further surgery is planned unless chronic infection in the left ureteral stump necessitates removal. The patient is maintained on nitroíùrantoin (Furadantin) suspension, 25 mg. daily, for urinary tract prophylaxis. Comment Published case reports of solitary ureteral ectopia indicate a high incidence of postreimplantation incontinence. 3 The embryologie deficits in trigonal development places the reimplanted ureter at substantial risk. Of the 23 reported cases of single and 10 cases of bilateral nonduplicated ureteral ectopia, progressive upper tract decompensation ultimately resulted in urinary diversion in several instances. lm3Although preservation of renal function must be of paramount concern,4 the decision to divert or reconstruct must be made on an individual basis. This case report is of considerable importante to pediatrie urologists for several reasons: (1) contrary to previous published reports, this patient was born with a normal bladder neck and sphincteric mechanism; (2) a normal bladder capacity is evident; (3) she is continent of urine; (4) her ureteral ectopia is associated with a horseshoe kidney; and (5) she was “cured” by ureteral reimplantation and did not require an antiincontinence procedure or urinary diversion. In light of this unusual case, it also may be necessary to reevaluate our present concept of the embryologie development of the bladder neck. Montefiore Hospital and Medical Center 111 East 210th Street Bronx, New York 10467 (DR. BARD) References Cox, C. E., and HUTCH, J. A.: Bilateral single ectopic ureter: a report of 2 cases and review of the literature, J, Urol. 95: 493 (1966). JOHNSON,J. H., and DAVENPORT,T. J.: The single ectopic ureter, Br. J. Urol. 41: 428 (1969). WILLIAMS, D. I., and LIGHTWOOD, R. C.: Bilateral single ectopic ureters, ibid. 44: 267 (1972). BARD, R. H.: Early high diversion, Urology 3: 369 (1974).
UROLOGY / JUNE 1975 /
VOLUME V, NUMBER 6
