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Posterior Urethral Valves Causing Urinary Retention in an Infant with Duplication of the Urethra
0022-534 7/84/1322-0353$02.00 /0
Vol. 132, August Printed in U.S.A.
THE JOURNAL OF UROLOGY
Copyright© 1984 by The Williams & Wilkins Co.
POSTERIOR URETHRAL VALVES CAUSING URINARY RETENTION IN AN INFANT WITH DUPLICATION OF THE URETHRA SANDRA K. FERNBACH
AND
MAX MAIZELS
From the Department of Radiology and Division of Urology, Children's Memorial Hospital and Northwestern University Medical School, Chicago, Illinois
ABSTRACT
We report the early management of an infant with partial urethral duplication and posterior urethral valves who presented in urinary retention. Duplication of the male urethra is a rare anomaly. Children with this anomaly usually present because of an abnormal stream, infected urine or incontinence. 1 • 2 Concomitant abnormalities of the urinary tract are uncommon. We report on an infant with a bifid urethra and posterior urethral valves, who presented in urinary retention. We wish to stress the usefulness of the radiological studies in the evaluation and treatment of this infant.
A year later, when continued lack of urine production on the right side correlated with renal nonfunction on a repeat nuclear scan, right nephrectomy was performed. Microscopic examination of the right kidney revealed many hyalinized glomeruli, atrophic tubules and interstitial fibrosis consistent with chronic pyelonephritis. Dysplastic elements were not identified. As part of the same surgical procedure the infant underwent closure of the left ureterostomy and transurethral resection of the posterior urethral valves. The bifid urethra was left intact (fig. 3).
CASE REPORT
C. B., CMH 302562, was known to have a duplicated urethral meatus with urinary flow through both meatus since birth. When he was 3 months old the patient presented to the emergency room in marked cardiovascular and respiratory distress. Vital signs included a blood pressure of 90/palpable mm. Hg, a respiratory rate of 60 per minute and a pulse of 160 per minute. The infant weighed 7 pounds, compared to 8 pounds 13 ounces recorded at birth. Serum sodium was 154 mEq./1., potassium 9.7 mEq./1. and pH 7.08. Hematocrit was 29.1 per cent. Blood urea nitrogen was 230 mg. per cent and serum creatinine was 5.4 mg. per cent. The specific gravity of urine was 1.013 and pH was 6. There was 2+ proteinuria but no hematuria or pyuria. Hyperkalemic, hypernatremic metabolic acidosis was treated with intravenous bicarbonate, sodium polystyrene sulfonate enemas, glucose and insulin, intravenous fluids and peritoneal dialysis. Real-time portable ultrasound demonstrated dilated tortuous ureters and small echogenic kidneys with dilated calices. The small bladder could not be differentiated from the ureters. A catheter was placed into the bladder through the dorsal (but normally located) urethral meatus and 50 cc of water-soluble contrast material were instilled into the bladder. The single radiogram obtained demonstrated grade V (international classification) right vesicoureteral reflux, a small markedly trabeculated bladder and dilatation of the posterior urethra secondary to urethral valves (fig. 1). In addition, a second patent urethral channel arising at the level of the valves filled with contrast material during voiding and emptied via an orifice on the ventral aspect of the distal penile shaft. Delayed studies showed poor drainage of abnormal-appearing right calices and ureter (fig. 2, A). Percutaneous nephrostomy tubes were placed to provide temporary renal drainage. However, these worked poorly and urine output was drained through a Foley catheter. Serum creatinine decreased to 3.3 mg. per cent. Two days later bilateral loop cutaneous ureterostomy was performed. At operation the right kidney was small but reniform. Postoperatively, the creatinine decreased to normal, and the renal scan showed good function of the left kidney and nonfunction of the right kidney. Repeat urethral studies confirmed the presence of a duplicated urethra and posterior urethral valves (fig. 2, B). Accepted for publication April 13, 1984. 353
DISCUSSION
Posterior urethral valves are the most common cause of lower urinary tract obstruction in early childhood. While most often posterior urethral valves occur as an isolated urethral lesion, there have been reports of concomitant urethral abnormalities, including Cowper's duct cyst, megalourethra and anterior urethral diverticulum. 3 ' 4 Anatomic and functional variants of urethral duplication are multiple and have been well described previously. 1 • 2 Duplications arising in the posterior urethra or bladder are the most commonly encountered. 1 Some authors would consider this anomaly to be a bifid urethra. 2 In most reported cases of urethral duplication the upper urinary tracts either have been normal or not studied radiographically. A few authors have documented associated unilateral renal agenesis. 5 - 8 Urinary retention was described in 3 children with urethral duplication but this rare presenting symptom was not attributable to posterior urethral valves. 8 A newborn with anomalies similar to our patient has been described previously. 9 That patient, with posterior urethral valves, complete urethral duplication and bilateral dysplastic kidneys, suffered from other multiple anomalies and died in the neonatal period. 9 The embryological origin of posterior urethral valves is uncertain. They are most commonly believed to be an exaggeration of normally present mucosa! folds but other theories abound. 10 Similarly, there is no unifying theory that can explain all of the variants of urethral duplication. A duplication arising in the posterior urethra, as in our case, may be attributed to an abnormal infolding of the genital folds. 2 It is interesting to speculate upon the events contributing to the simultaneous development of posterior urethral valves and urethral duplication, especially since the duplication arises at the site of the valves. The radiological studies were performed in an appropriate sequence. The immediately available ultrasound demonstrated the bilaterally small kidneys and large ureters. Infantile polycystic kidney disease was excluded. The portable cystogram further delineated the anatomy and indicated that multiple problems existed. The necessity to improve urinary drainage from the upper tracts became apparent and ureterostomies were performed. Continued lack of right renal function, clini-
354
FERNBACH AND MAIZELS
FIG. 1. A, small trabeculated bladder (B) merges with markedly dilated right ureter. Right renal pelvis and calices (white arrows) are dilated. Feeding tube in dorsal urethra (black arrows) passes through circular posterior urethral valves (crossed white arrows). Second urethral channel is seen below dorsal channel. B, enlargement of urethra.
FIG. 2. A, delayed film after drainage of bladder demonstrates abnormal calices (arrows), dilated proximal ureter with abrupt change in caliber in mid ureter and bladder diverticulum filled with contrast material at ureterovesical junction. B, before resection of posterior urethral valves (arrow) cystogram with catheter in ventral urethra shows patent and poorly filled dorsal urethral channel (arrowheads).
cally and by nuclear scan, culminated in nephrectomy 1 year after presentation, during which the posterior urethral valves also were corrected. It is apparent that at each stage in the course of our patient radiological studies provided valuable information and enabled the most appropriate urological procedures to be performed. ADDENDUM
When the patient was 2 years old the duplicated urethra was reconstructed to provide a single urinary stream that originates from the glans. A window was created between the dorsal and ventral urethras. The orifice of the ventral urethra on the distal shaft of the penis was closed in a manner analogous to closure of a urethrocutaneous fistula. Postoperatively, the patient voids with a full stream from the urethral meatus. REFERENCES
1. Effmann, E. L., Lebowitz, R. L. and Colodny, A. H.: Duplication
FIG. 3. After posterior urethral valves were resected cystogram with catheter in dorsal urethra demonstrates again separate ventral urethra.
of the urethra. Radiology, 119: 179, 1976. 2. Das, S. and Brosman, S. A.: Duplicaton of the male urethra. J. Urol., 117: 452, 1977. 3. Krueger, R. P. and Churchill, B. M.: Megalourethra with posterior
POSTERIOR URETHRAL VALVES 1N DUPLICATED URETHRA
urethral valves. 18: 279, 1981. 4. Drouin, G., LaPerriere, and Gregoire, A.: Urethral valves as a cause of dilated Cowper's glands and perinea! pain. J. Urol., 120: 634, 1978. 5. Mehan, D. J. and Gonzales, J. H.: Urethral duplication with associated agenesis of left kidney and right ureteral ectopia. Urology, 6: 476, 1975. 6. Durrani, K. M., Shah, P. I. and Kakalia, G. R.: Interurethral fenestration for a case of double urethra with hypospadias. J. Urol., 108: 586, 1972.
355
7. Lane, V.: Congenital patent urachus associated with complete (hypospadiac) duplication of the urethra and solitary crossed renal ectopia. J. Urol., 127: 990, 1982. 8. Fellows, G. J. and Johnston, J. H.: Incomplete urethral duplication and urinary retention. Brit. J. Urol., 46: 449, 1974. 9. Lorenzo, R. L., Turner, W. R., Bradford, B. F., Upshur, J. and Sexton, F. M.: Duplication of the male urethra with posterior urethral valves. Ped. Rad., 11: 39, 1981. 10. Williams, D. I. and Eckstein, H. B.: Obstructive valves in the posterior urethra. J. Urol., 93: 236, 1965.
Vol. 132, August Printed in U.S.A.
THE JOURNAL OF UROLOGY
Copyright© 1984 by The Williams & Wilkins Co.
POSTERIOR URETHRAL VALVES CAUSING URINARY RETENTION IN AN INFANT WITH DUPLICATION OF THE URETHRA SANDRA K. FERNBACH
AND
MAX MAIZELS
From the Department of Radiology and Division of Urology, Children's Memorial Hospital and Northwestern University Medical School, Chicago, Illinois
ABSTRACT
We report the early management of an infant with partial urethral duplication and posterior urethral valves who presented in urinary retention. Duplication of the male urethra is a rare anomaly. Children with this anomaly usually present because of an abnormal stream, infected urine or incontinence. 1 • 2 Concomitant abnormalities of the urinary tract are uncommon. We report on an infant with a bifid urethra and posterior urethral valves, who presented in urinary retention. We wish to stress the usefulness of the radiological studies in the evaluation and treatment of this infant.
A year later, when continued lack of urine production on the right side correlated with renal nonfunction on a repeat nuclear scan, right nephrectomy was performed. Microscopic examination of the right kidney revealed many hyalinized glomeruli, atrophic tubules and interstitial fibrosis consistent with chronic pyelonephritis. Dysplastic elements were not identified. As part of the same surgical procedure the infant underwent closure of the left ureterostomy and transurethral resection of the posterior urethral valves. The bifid urethra was left intact (fig. 3).
CASE REPORT
C. B., CMH 302562, was known to have a duplicated urethral meatus with urinary flow through both meatus since birth. When he was 3 months old the patient presented to the emergency room in marked cardiovascular and respiratory distress. Vital signs included a blood pressure of 90/palpable mm. Hg, a respiratory rate of 60 per minute and a pulse of 160 per minute. The infant weighed 7 pounds, compared to 8 pounds 13 ounces recorded at birth. Serum sodium was 154 mEq./1., potassium 9.7 mEq./1. and pH 7.08. Hematocrit was 29.1 per cent. Blood urea nitrogen was 230 mg. per cent and serum creatinine was 5.4 mg. per cent. The specific gravity of urine was 1.013 and pH was 6. There was 2+ proteinuria but no hematuria or pyuria. Hyperkalemic, hypernatremic metabolic acidosis was treated with intravenous bicarbonate, sodium polystyrene sulfonate enemas, glucose and insulin, intravenous fluids and peritoneal dialysis. Real-time portable ultrasound demonstrated dilated tortuous ureters and small echogenic kidneys with dilated calices. The small bladder could not be differentiated from the ureters. A catheter was placed into the bladder through the dorsal (but normally located) urethral meatus and 50 cc of water-soluble contrast material were instilled into the bladder. The single radiogram obtained demonstrated grade V (international classification) right vesicoureteral reflux, a small markedly trabeculated bladder and dilatation of the posterior urethra secondary to urethral valves (fig. 1). In addition, a second patent urethral channel arising at the level of the valves filled with contrast material during voiding and emptied via an orifice on the ventral aspect of the distal penile shaft. Delayed studies showed poor drainage of abnormal-appearing right calices and ureter (fig. 2, A). Percutaneous nephrostomy tubes were placed to provide temporary renal drainage. However, these worked poorly and urine output was drained through a Foley catheter. Serum creatinine decreased to 3.3 mg. per cent. Two days later bilateral loop cutaneous ureterostomy was performed. At operation the right kidney was small but reniform. Postoperatively, the creatinine decreased to normal, and the renal scan showed good function of the left kidney and nonfunction of the right kidney. Repeat urethral studies confirmed the presence of a duplicated urethra and posterior urethral valves (fig. 2, B). Accepted for publication April 13, 1984. 353
DISCUSSION
Posterior urethral valves are the most common cause of lower urinary tract obstruction in early childhood. While most often posterior urethral valves occur as an isolated urethral lesion, there have been reports of concomitant urethral abnormalities, including Cowper's duct cyst, megalourethra and anterior urethral diverticulum. 3 ' 4 Anatomic and functional variants of urethral duplication are multiple and have been well described previously. 1 • 2 Duplications arising in the posterior urethra or bladder are the most commonly encountered. 1 Some authors would consider this anomaly to be a bifid urethra. 2 In most reported cases of urethral duplication the upper urinary tracts either have been normal or not studied radiographically. A few authors have documented associated unilateral renal agenesis. 5 - 8 Urinary retention was described in 3 children with urethral duplication but this rare presenting symptom was not attributable to posterior urethral valves. 8 A newborn with anomalies similar to our patient has been described previously. 9 That patient, with posterior urethral valves, complete urethral duplication and bilateral dysplastic kidneys, suffered from other multiple anomalies and died in the neonatal period. 9 The embryological origin of posterior urethral valves is uncertain. They are most commonly believed to be an exaggeration of normally present mucosa! folds but other theories abound. 10 Similarly, there is no unifying theory that can explain all of the variants of urethral duplication. A duplication arising in the posterior urethra, as in our case, may be attributed to an abnormal infolding of the genital folds. 2 It is interesting to speculate upon the events contributing to the simultaneous development of posterior urethral valves and urethral duplication, especially since the duplication arises at the site of the valves. The radiological studies were performed in an appropriate sequence. The immediately available ultrasound demonstrated the bilaterally small kidneys and large ureters. Infantile polycystic kidney disease was excluded. The portable cystogram further delineated the anatomy and indicated that multiple problems existed. The necessity to improve urinary drainage from the upper tracts became apparent and ureterostomies were performed. Continued lack of right renal function, clini-
354
FERNBACH AND MAIZELS
FIG. 1. A, small trabeculated bladder (B) merges with markedly dilated right ureter. Right renal pelvis and calices (white arrows) are dilated. Feeding tube in dorsal urethra (black arrows) passes through circular posterior urethral valves (crossed white arrows). Second urethral channel is seen below dorsal channel. B, enlargement of urethra.
FIG. 2. A, delayed film after drainage of bladder demonstrates abnormal calices (arrows), dilated proximal ureter with abrupt change in caliber in mid ureter and bladder diverticulum filled with contrast material at ureterovesical junction. B, before resection of posterior urethral valves (arrow) cystogram with catheter in ventral urethra shows patent and poorly filled dorsal urethral channel (arrowheads).
cally and by nuclear scan, culminated in nephrectomy 1 year after presentation, during which the posterior urethral valves also were corrected. It is apparent that at each stage in the course of our patient radiological studies provided valuable information and enabled the most appropriate urological procedures to be performed. ADDENDUM
When the patient was 2 years old the duplicated urethra was reconstructed to provide a single urinary stream that originates from the glans. A window was created between the dorsal and ventral urethras. The orifice of the ventral urethra on the distal shaft of the penis was closed in a manner analogous to closure of a urethrocutaneous fistula. Postoperatively, the patient voids with a full stream from the urethral meatus. REFERENCES
1. Effmann, E. L., Lebowitz, R. L. and Colodny, A. H.: Duplication
FIG. 3. After posterior urethral valves were resected cystogram with catheter in dorsal urethra demonstrates again separate ventral urethra.
of the urethra. Radiology, 119: 179, 1976. 2. Das, S. and Brosman, S. A.: Duplicaton of the male urethra. J. Urol., 117: 452, 1977. 3. Krueger, R. P. and Churchill, B. M.: Megalourethra with posterior
POSTERIOR URETHRAL VALVES 1N DUPLICATED URETHRA
urethral valves. 18: 279, 1981. 4. Drouin, G., LaPerriere, and Gregoire, A.: Urethral valves as a cause of dilated Cowper's glands and perinea! pain. J. Urol., 120: 634, 1978. 5. Mehan, D. J. and Gonzales, J. H.: Urethral duplication with associated agenesis of left kidney and right ureteral ectopia. Urology, 6: 476, 1975. 6. Durrani, K. M., Shah, P. I. and Kakalia, G. R.: Interurethral fenestration for a case of double urethra with hypospadias. J. Urol., 108: 586, 1972.
355
7. Lane, V.: Congenital patent urachus associated with complete (hypospadiac) duplication of the urethra and solitary crossed renal ectopia. J. Urol., 127: 990, 1982. 8. Fellows, G. J. and Johnston, J. H.: Incomplete urethral duplication and urinary retention. Brit. J. Urol., 46: 449, 1974. 9. Lorenzo, R. L., Turner, W. R., Bradford, B. F., Upshur, J. and Sexton, F. M.: Duplication of the male urethra with posterior urethral valves. Ped. Rad., 11: 39, 1981. 10. Williams, D. I. and Eckstein, H. B.: Obstructive valves in the posterior urethra. J. Urol., 93: 236, 1965.