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Anterior urethral valves
ANTERIOR URETHRAL VALVES NEIL
BAUM,
ROBERT
M.D.
KAMINSKY,
J. DAVID WRIGHT,
M.D. M.D.
From the Roy and Lillie Cullen Department of Urologic Research, Division of Urology, Baylor College of Medicine, and the Urology Service, St. Luke’s Episcopal Hospital and Texas Children’s Hospital, Houston, Texas
ABSTRACT - Two cases of anterior urethral valves are reported, one occurring in a child with renal osteodystrophy and the second being thefirst recorded case of anterior urethral valves in an adult. These 2 cases represent the thirteenth and fourteenth cases of anterior urethral valves reported in the literature.
Herein we report 2 cases of anterior urethral valves found in private practice in a three-month period by one of the authors (J.D.W.). The first case occurred in a pediatric patient with a heretofore unreported complication of anterior urethral valves, namely, renal osteodystrophy. The second case is the first report of anterior urethral valves in an adult. Thus, we add the thirteenth and fourteenth cases to be reported in the literature. ‘-’ Case Reports Case 1 A six-year-old male was seen on April 9, 1974, with a three-month history of pain in the hip and leg. Pain initially involved both hips with progression to both knees and ankles, and three days prior to admission he was unable to bear weight because of this pain. His mother noted that the child had a poor urine stream, strained to void, had postmicturition dribbling, and incontinence of urine both day and night. The patient was also noted to have polyuria, polydypsia, and a craving for salt. There was no past history of renal problems nor family history of diabetes insipidus. Physical examination revealed the blood pressure to be 90/65 mm. Hg, temperature 98.6” F., height 41 inches, and weight 38 pounds, 8 ounces.
UROLOGY
/ DECEMBER
1975 / VOLUME
VI, NUMBER 6
His chest was clear, and his heart showed regular sinus rhythm. Examination of his genitalia revealed a normal prepubertal male with small external meatus. The extremities showed normal range of motion with adequate muscle mass and tone. There was no swelling, erythema, or joint tenderness. Neurologic examination was grossly within normal limits. Initial laboratory examination showed a hemoglobin of 12 Gm. and hematocrit of 34 with a normal differential. Serum sodium was 151 mEq., potassium 4.3 mEq., CO2 content 18 mEq., and serum chloride 190 mEq. per liter. Blood urea nitrogen was 104 mg., creatinine 3.3 mg., and calcium 10.9 mg. per 100 ml.; phosphate 8.4 mEq. per liter; alkaline phosphatase 275 KingArmstrong units; serum pH 7.33. Urinalysis showed a trace of protein, pH 6, specific gravity 1.007, 24 white blood cells per high-power field, few bacteria, and no red blood cells. Serum osmolality was 322 mOsm. and urine osmolality 320 mOsm. per liter; urine sodium was 33 mEq. per liter. A bone survey showed advanced rachitic changes involving all joints compatible with secondary hyperparathyroidism. Xerograms of the hand revealed subperiosteal demineralization of the phalanges typical of secondary hyperparathyroidism (Fig. 1). Intravenous pyelogram
723
and resected, and a 12 F Foley catheter was inserted and left indwelling until the urine was ascertained to be free of gross blood at which time the catheter was removed. The child then voided with a good stream. The patient was discharged on a low-protein, high-biologic value diet, aluminum hydroxide gel, oral calcium, and dihydrotachysterol (vitamin D) 0.5 mg. per day. At follow-up he reported a decrease in bone pain, improved urinary stream, no incontinence or enuresis, improved appetite and weight gain, and no polydypsia, polyuria, or salt craving. Blood urea nitrogen was 38 mg., creatinine 2.9 mg., and calcium 9.2 mg. per 100 ml.; phosphate was 3.3 mEq. per liter on May 6, 1974. Postoperative voiding cystourethrogram revealed a marked decrease in the dilatation of the bulbous urethra (Fig. 2E). However, there was persistent vesicoureteral reflux. On June 28, 1974, he underwent bilateral ureteroneocystostomy of the Politano-Leadbetter variety. His postoperative course was unremarkable. Voiding cinecystourethrogram obtained three months postoperatively did not reveal any vesicoureteral reflux, and the anterior urethra was normal. FIGURE 1. Xerogram of right hand periosteal resorption of distal phalanges.
Case 2
with sub-
on the day of admission demonstrated poor visualization of the contrast material bilaterally and grade II hydronephrosis (Fig. 2A). Voiding cinecystourethrogram on April 11, 1974, revealed bilateral vesicoureteral reflux with dilatation of both renal pelves and calyces (Fig. 2B). Voiding urethrogram demonstrated a dilated bulbous urethra with a normal distal urethra suggestive of an anterior valve (Fig. 2C). The child was placed on a 30-Gm. protein diet with aluminum hydroxide gel (Amphojel) to manage the azotemia and hyperphosphatemia. On April 12, 1974, panendoscopy revealed urethral depression at the penoscrotal junction with a solitary semilunar valve lying within the depression. Cystoscopy demonstrated a large capacity bladder without trabeculation. The ureteral orifices were slit-like in appearance with adequate submucosal tunnels. A 12 F pediatric resectoscope was inserted, and the valve was easily engaged with the loop of the resectoscope. As the valve was pulled distally, it was noted to obstruct the urethra (Fig. 2D). The valve was fulgurated
724
A twenty-seven-year-old male had a two-tothree-month history of recurrent urinary tract infection. The patient stated that he had never had a good urinary stream, and had experienced marked hesistancy, especially in the morning, and frequently had spraying of his stream. During episodes of infection he experienced dysuria, frequency, and urgency, but all were improved with antibiotic therapy. The patient had undergone urologic evaluation at age five and again in high school for recurrent infection including excretory urography and cystoscopy with no apparent abnormalities noted. He was admitted to St. Luke’s Hospital on May 12, 1974, for repeat urologic evaluation. Physical examination was within normal limits. Routine blood studies and urinalysis were normal, and the urine culture was negative. Intravenous pyelogram revealed normal upper tracts. A voiding cinecystourethrogram revealed an anterior urethral valve with dilated bulbous urethra and mild bilateral vesicoureteral reflux (Fig. 3A). The patient underwent cystoscopy and was found to have a cusp-like valve in the anterior urethra at the level of the penoscrotal junction extending from approximately the seven-o’clock to threeo’clock position. The urethra proximal to this
UROLOGY
/ DECEMBER1975
/ VOLUMEVI,
NUMBER6
Dilated cn-ethrcf
FIGURE 2. (A) lntravenous pyelogram with bilateral hydroureteronephrosiss; (B) cystogram with bilateral vesicoureteral re&x; (C) voiding cineurethrogram with dilated anterior urethra; (D) diagram revealing anterior urethral valve at penoscrotal junction; and (E) voiding cineurethrogram with normal anterior urethra.
valve was dilated and appeared to be chronically inflamed. Using the Iglesias resectoscope the valve was resected at the six-o’clock position. Postoperatively, the patient volunteered that his stream was markedly improved. A repeat voiding cinecystourethrogram demonstrated a normal anterior urethra and absence of reflux (Fig. 3B). Comment Although Young, Frontz, and Baldwin9 first described posterior urethral valves more than fifty years ago, the diagnosis of urethral valves in
UROLOGY
/ DECEMBER1975
/ VOLUMEVI,
NUMBER6
general has been considered rare in children. However, since the advent of the voiding cinecystography, this entity is being recognized more frequently. Campbell and Harrison” stated that “prostatic urethral valves are not rare; they are just rarely identified.” More important is the fact that prior to the time of pediatric endoscopic instruments, the diagnosis of valves was more often made at postmortem examinations, and only those children with mild degrees of obstruction were apt to survive. The literature is replete with cases of posterior urethral valves, but only a few cases of anterior
725
FIGURE 3. Voiding cinecystourethrogram anterior urethra (B).
with anterior urethral valve at penoscrotal junction (A), and normal
urethral valves have been reported. Anderson, Hodgkinson, and Pyrah” reported 2 cases of renal osteodystrophy, one of which was associated with congenital obstruction of the bladder neck aggravated by a diverticulum of the urethra. Waterhouse and Scordamaglia2 described a single case of anterior urethral valves with bilateral hydronephrosis, but no mention was made of renal osteodystrophy. This is the first reported case of renal rickets (osteomalacia) associated with anterior urethral valves. Our second case also represents the oldest reported patient with anterior urethral valves as the cause of outlet obstruction. We, therefore, recommend all male children who are seen with urinary tract infection and/or obstruction of the lower tract should have an evaluation with a voiding cinecystourethrogram. We also believe that any adult male with undiagnosed urinary tract infection or outlet obstruction should also have a voiding cinecystourethrogram as a part of his urologic evaluation. Baylor College of Medicine 1200 Moursund Avenue Houston, Texas 77025 (DR. BAIJM)
726
References 1. SCOTT, F. B., and CAFFARENA, E.: Diagnosis of anterior urethral valves, J. Urol. 110:261 (1973). 2. WATERHOUSE, K., and SCORDAMAGLIA,L. J.: Anterior urethral valve: a rare cause of bilateral hydronephrosis, ibid. 87: 556 (1962). Anterior urethral valves: a case report, CHANG, C-Y. : ibid. 100:29 (1968). COLABAWALLA,B. N.: Anterior urethral valve: a case report, ibid. 94: 58 (1965). SAWANISHI, K. : Congenital valve of anterior urethra in infant: report of a case, Acta Urol. Jap. 8: 419 (1962). TEXTER, J. H., and ENGEL, R. M. E.: Anterior urethral valve as a cause for urinary obstruction: a case report, J. Ural. 107: 316 (1972). in Ency7. WILLIAMS, D. : Urology in childhood, clopedia of Urology, Berlin, Springer Verlag, 1958, vol. 15, p. 85. 8. HOPE, J. W., JAMESON, P. J., and MICHIE, A. J.: Diagnosis of anterior urethral valve by voiding urethrography: report of two cases, Radiology 74: 798 (1960). 9. YOUNG, H. H., FRONTZ, W. A., and BALDWIN, J. : Congenital obstruction of the posterior urethra, J. Ural. 3: 289 (1919). 10. CAMPBELL, M. F., and HARRISON, J. H.: Urology, 3rd ed., Philadelphia, W. B. Saunders Co., 1970, p. 1614. 11. ANDERSON,C. K., HODGKINSON,A., and PYRAH, L. . Azotemic renal osteodystrophy, Br. J. Surg. 48: Z8 (1961).
UROLOGY I DECEMBER 1975 / VOLUME VI, NUMBER 6
BAUM,
ROBERT
M.D.
KAMINSKY,
J. DAVID WRIGHT,
M.D. M.D.
From the Roy and Lillie Cullen Department of Urologic Research, Division of Urology, Baylor College of Medicine, and the Urology Service, St. Luke’s Episcopal Hospital and Texas Children’s Hospital, Houston, Texas
ABSTRACT - Two cases of anterior urethral valves are reported, one occurring in a child with renal osteodystrophy and the second being thefirst recorded case of anterior urethral valves in an adult. These 2 cases represent the thirteenth and fourteenth cases of anterior urethral valves reported in the literature.
Herein we report 2 cases of anterior urethral valves found in private practice in a three-month period by one of the authors (J.D.W.). The first case occurred in a pediatric patient with a heretofore unreported complication of anterior urethral valves, namely, renal osteodystrophy. The second case is the first report of anterior urethral valves in an adult. Thus, we add the thirteenth and fourteenth cases to be reported in the literature. ‘-’ Case Reports Case 1 A six-year-old male was seen on April 9, 1974, with a three-month history of pain in the hip and leg. Pain initially involved both hips with progression to both knees and ankles, and three days prior to admission he was unable to bear weight because of this pain. His mother noted that the child had a poor urine stream, strained to void, had postmicturition dribbling, and incontinence of urine both day and night. The patient was also noted to have polyuria, polydypsia, and a craving for salt. There was no past history of renal problems nor family history of diabetes insipidus. Physical examination revealed the blood pressure to be 90/65 mm. Hg, temperature 98.6” F., height 41 inches, and weight 38 pounds, 8 ounces.
UROLOGY
/ DECEMBER
1975 / VOLUME
VI, NUMBER 6
His chest was clear, and his heart showed regular sinus rhythm. Examination of his genitalia revealed a normal prepubertal male with small external meatus. The extremities showed normal range of motion with adequate muscle mass and tone. There was no swelling, erythema, or joint tenderness. Neurologic examination was grossly within normal limits. Initial laboratory examination showed a hemoglobin of 12 Gm. and hematocrit of 34 with a normal differential. Serum sodium was 151 mEq., potassium 4.3 mEq., CO2 content 18 mEq., and serum chloride 190 mEq. per liter. Blood urea nitrogen was 104 mg., creatinine 3.3 mg., and calcium 10.9 mg. per 100 ml.; phosphate 8.4 mEq. per liter; alkaline phosphatase 275 KingArmstrong units; serum pH 7.33. Urinalysis showed a trace of protein, pH 6, specific gravity 1.007, 24 white blood cells per high-power field, few bacteria, and no red blood cells. Serum osmolality was 322 mOsm. and urine osmolality 320 mOsm. per liter; urine sodium was 33 mEq. per liter. A bone survey showed advanced rachitic changes involving all joints compatible with secondary hyperparathyroidism. Xerograms of the hand revealed subperiosteal demineralization of the phalanges typical of secondary hyperparathyroidism (Fig. 1). Intravenous pyelogram
723
and resected, and a 12 F Foley catheter was inserted and left indwelling until the urine was ascertained to be free of gross blood at which time the catheter was removed. The child then voided with a good stream. The patient was discharged on a low-protein, high-biologic value diet, aluminum hydroxide gel, oral calcium, and dihydrotachysterol (vitamin D) 0.5 mg. per day. At follow-up he reported a decrease in bone pain, improved urinary stream, no incontinence or enuresis, improved appetite and weight gain, and no polydypsia, polyuria, or salt craving. Blood urea nitrogen was 38 mg., creatinine 2.9 mg., and calcium 9.2 mg. per 100 ml.; phosphate was 3.3 mEq. per liter on May 6, 1974. Postoperative voiding cystourethrogram revealed a marked decrease in the dilatation of the bulbous urethra (Fig. 2E). However, there was persistent vesicoureteral reflux. On June 28, 1974, he underwent bilateral ureteroneocystostomy of the Politano-Leadbetter variety. His postoperative course was unremarkable. Voiding cinecystourethrogram obtained three months postoperatively did not reveal any vesicoureteral reflux, and the anterior urethra was normal. FIGURE 1. Xerogram of right hand periosteal resorption of distal phalanges.
Case 2
with sub-
on the day of admission demonstrated poor visualization of the contrast material bilaterally and grade II hydronephrosis (Fig. 2A). Voiding cinecystourethrogram on April 11, 1974, revealed bilateral vesicoureteral reflux with dilatation of both renal pelves and calyces (Fig. 2B). Voiding urethrogram demonstrated a dilated bulbous urethra with a normal distal urethra suggestive of an anterior valve (Fig. 2C). The child was placed on a 30-Gm. protein diet with aluminum hydroxide gel (Amphojel) to manage the azotemia and hyperphosphatemia. On April 12, 1974, panendoscopy revealed urethral depression at the penoscrotal junction with a solitary semilunar valve lying within the depression. Cystoscopy demonstrated a large capacity bladder without trabeculation. The ureteral orifices were slit-like in appearance with adequate submucosal tunnels. A 12 F pediatric resectoscope was inserted, and the valve was easily engaged with the loop of the resectoscope. As the valve was pulled distally, it was noted to obstruct the urethra (Fig. 2D). The valve was fulgurated
724
A twenty-seven-year-old male had a two-tothree-month history of recurrent urinary tract infection. The patient stated that he had never had a good urinary stream, and had experienced marked hesistancy, especially in the morning, and frequently had spraying of his stream. During episodes of infection he experienced dysuria, frequency, and urgency, but all were improved with antibiotic therapy. The patient had undergone urologic evaluation at age five and again in high school for recurrent infection including excretory urography and cystoscopy with no apparent abnormalities noted. He was admitted to St. Luke’s Hospital on May 12, 1974, for repeat urologic evaluation. Physical examination was within normal limits. Routine blood studies and urinalysis were normal, and the urine culture was negative. Intravenous pyelogram revealed normal upper tracts. A voiding cinecystourethrogram revealed an anterior urethral valve with dilated bulbous urethra and mild bilateral vesicoureteral reflux (Fig. 3A). The patient underwent cystoscopy and was found to have a cusp-like valve in the anterior urethra at the level of the penoscrotal junction extending from approximately the seven-o’clock to threeo’clock position. The urethra proximal to this
UROLOGY
/ DECEMBER1975
/ VOLUMEVI,
NUMBER6
Dilated cn-ethrcf
FIGURE 2. (A) lntravenous pyelogram with bilateral hydroureteronephrosiss; (B) cystogram with bilateral vesicoureteral re&x; (C) voiding cineurethrogram with dilated anterior urethra; (D) diagram revealing anterior urethral valve at penoscrotal junction; and (E) voiding cineurethrogram with normal anterior urethra.
valve was dilated and appeared to be chronically inflamed. Using the Iglesias resectoscope the valve was resected at the six-o’clock position. Postoperatively, the patient volunteered that his stream was markedly improved. A repeat voiding cinecystourethrogram demonstrated a normal anterior urethra and absence of reflux (Fig. 3B). Comment Although Young, Frontz, and Baldwin9 first described posterior urethral valves more than fifty years ago, the diagnosis of urethral valves in
UROLOGY
/ DECEMBER1975
/ VOLUMEVI,
NUMBER6
general has been considered rare in children. However, since the advent of the voiding cinecystography, this entity is being recognized more frequently. Campbell and Harrison” stated that “prostatic urethral valves are not rare; they are just rarely identified.” More important is the fact that prior to the time of pediatric endoscopic instruments, the diagnosis of valves was more often made at postmortem examinations, and only those children with mild degrees of obstruction were apt to survive. The literature is replete with cases of posterior urethral valves, but only a few cases of anterior
725
FIGURE 3. Voiding cinecystourethrogram anterior urethra (B).
with anterior urethral valve at penoscrotal junction (A), and normal
urethral valves have been reported. Anderson, Hodgkinson, and Pyrah” reported 2 cases of renal osteodystrophy, one of which was associated with congenital obstruction of the bladder neck aggravated by a diverticulum of the urethra. Waterhouse and Scordamaglia2 described a single case of anterior urethral valves with bilateral hydronephrosis, but no mention was made of renal osteodystrophy. This is the first reported case of renal rickets (osteomalacia) associated with anterior urethral valves. Our second case also represents the oldest reported patient with anterior urethral valves as the cause of outlet obstruction. We, therefore, recommend all male children who are seen with urinary tract infection and/or obstruction of the lower tract should have an evaluation with a voiding cinecystourethrogram. We also believe that any adult male with undiagnosed urinary tract infection or outlet obstruction should also have a voiding cinecystourethrogram as a part of his urologic evaluation. Baylor College of Medicine 1200 Moursund Avenue Houston, Texas 77025 (DR. BAIJM)
726
References 1. SCOTT, F. B., and CAFFARENA, E.: Diagnosis of anterior urethral valves, J. Urol. 110:261 (1973). 2. WATERHOUSE, K., and SCORDAMAGLIA,L. J.: Anterior urethral valve: a rare cause of bilateral hydronephrosis, ibid. 87: 556 (1962). Anterior urethral valves: a case report, CHANG, C-Y. : ibid. 100:29 (1968). COLABAWALLA,B. N.: Anterior urethral valve: a case report, ibid. 94: 58 (1965). SAWANISHI, K. : Congenital valve of anterior urethra in infant: report of a case, Acta Urol. Jap. 8: 419 (1962). TEXTER, J. H., and ENGEL, R. M. E.: Anterior urethral valve as a cause for urinary obstruction: a case report, J. Ural. 107: 316 (1972). in Ency7. WILLIAMS, D. : Urology in childhood, clopedia of Urology, Berlin, Springer Verlag, 1958, vol. 15, p. 85. 8. HOPE, J. W., JAMESON, P. J., and MICHIE, A. J.: Diagnosis of anterior urethral valve by voiding urethrography: report of two cases, Radiology 74: 798 (1960). 9. YOUNG, H. H., FRONTZ, W. A., and BALDWIN, J. : Congenital obstruction of the posterior urethra, J. Ural. 3: 289 (1919). 10. CAMPBELL, M. F., and HARRISON, J. H.: Urology, 3rd ed., Philadelphia, W. B. Saunders Co., 1970, p. 1614. 11. ANDERSON,C. K., HODGKINSON,A., and PYRAH, L. . Azotemic renal osteodystrophy, Br. J. Surg. 48: Z8 (1961).
UROLOGY I DECEMBER 1975 / VOLUME VI, NUMBER 6