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Anterior urethral valves
CASE REPORTS
ANTERIOR MICHAEL
URETHRAL
P. SMALL,
LAWRENCE
VALVES
M.D.
SCHOENFELD,
M.D.
From the Department of Surgery, Division of Urology, Variety Children’s Hospital, and the Department of Urology, University of Miami, School of Medicine, Miami, Florida
ABSTRACT -A case of anterior urethral valves is presented. A diagnosis of anterior urethral valves can be suspected clinically by watching the patient void. Dribbling and a diminished face of the patient’s urinary stream are present in this condition. Conjk-mation is made with a voiding cystourethrogram and panendoscopy. Various modes of therapy are discussed.
Posterior urethral valves have been recognized as a relatively common cause of severe bladder outlet obstruction and have received much attention. In contrast, the reports of anterior urethral valves are rare. Baum, Kaminsky, and Wright’ stated that their cases were the thirteenth and fourteenth reported in the literature. Williams and Retik2 reported 17 cases of lesions of the anterior urethra with the most common lesion being a wide-mouthed diverticulum of the ventral wall of the urethra. However, they did not distinguish between congenital valves and diverticulum of the urethra. A review of the charts and x-ray films at Variety Children’s Hospital for the past twenty years revealed 29 patients with posterior urethral valves and 19 patients with congenital urethral diverticula. Only 1 case of anterior urethral valves was found. We herein present this case with a discussion of its identification and treatment because even though this is a rare condition, it may lead to severe degrees of obstruction. Case Report A six and one-half pound, white, male infant was admitted to the hospital with a diagnosis of anterior urethral valves. He had been transferred from the hospital of birth after a pediatrician had noted physical findings of dribbling upon urination, decreased size and force of the urinary stream, and ballooning of the entire
262
proximal urethra with voiding. He was the first child of healthy, young parents, and gestation and delivery had been uncomplicated. Physical examination results were normal except for the deformity on urination. Laboratory studies, including blood urea nitrogen, creatinine, and a urine culture were within normal limits. An intravenous pyelogram revealed bilateral hydronephrosis and delayed visualization. A cystogram and voiding cystourethrogram were performed (Fig. 1). No evidence of vesicourethral reflux was found, but the bladder did reveal heavy trabeculation with diverticula formation. The entire penile urethra was dilated to within approximately 1.5 cm. of the urethral meatus where it abruptly narrowed. Here a definite obstruction was seen with the total urethra markedly dilated. There was a suggestion of a urethral diverticulum just proximal to the obstruction. However, it was believed that this area was dilated secondary to the urethral obstruction and was not a true diverticulum. Urethral calibration was carried out from an 8 through 12 F without difficulty. Following this, panendoscopy was performed with an 8 F pediatric panendoscope. This revealed a dense diaphragmatic valve located approximately 2 cm. proximal to the urethral meatus. A portion of the valve had been broken, and panendoscopy through the remainder of urethra revealed dilation but no other areas of obstruction.
UROLOGY
/ MARCH 1978 / VOLUME
XI, NUMBER
3
FIGURE 1. (A) Cystogram revealing dilated posterior urethra and marked trabeculation of bladder. (B) Voiding cystourethrogram (a) anterior urethral valve and (b) dilated proximal urethra.
The bladder revealed marked trabeculation with cellule and diverticula formation. Using a small electrode, the valve was gently fulgurated circumferentially. An 8 F Foley catheter was left indwelling for five days. The patient was discharged on a regimen of ampicillin trihydrate. Three weeks later he was readmitted with urosepsis, dehydration, and a palpable right flank mass. His general condition improved with intravenous fluids and antibiotic therapy. Intravenous pyelogram demonstrated only a bilateral nephrogram at twenty minutes. High urinary diversion by bilateral loop cutaneous ureterostomies was accomplished. The postoperative course was complicated by severe hypertension. Blood pressures greater than 250/120 mm. Hg were recorded on several occasions. Urine output from the left ureterostomy was markedly less than from the right. Therapy included furosemide, hydralazine, and one course of diazoxide. His general condition improved. Intravenous pyelogram two weeks after the urinary diversion revealed considerable functional improvement. At five months of age, the intravenous pyelogram showed continued improvement although bilateral pyelocaliectasis was still present (Fig. 2A). The most recent films at eleven months revealed renal growth, and continued improvement in the pyelocaliectasis. On the voiding
UROLOGY
/ MARCH 1978
/ VOLUME
XI, NUMBER
3
cystourethrogram, vesicoureteral reflux was present but there was no further dilation or tortuosity of the ureters (Fig. 2B and C). The bladder, urethra, and ureters have shown marked resolution in their earlier changes, and the patient is now ready for closure of the ureterostomies. Comment The age at which a diagnosis of anterior urethral valves is made depends on the degree of obstruction present. Our patient was diagnosed at an early age suggesting the presence of an anterior urethral obstruction of marked severity. Malhoski and Frank3 cited 4 cases in which anterior urethral valves were diagnosed at six to eight years of age. All had relatively low-grade obstructing lesions. The presenting symptoms of older children may be enuresis, dribbling, daytime wetting, or blood staining of underclothes. Other presenting symptoms such as chronic renal failure, failure to thrive, and urinary infection depend on the severity and duration of the urethral obstruction. The diagnosis of anterior urethral obstruction can be suspected clinically on physical examination by watching the patient void. He will dribble and void with diminished force of his urinary stream. In severe cases there will be ballooning of the urethra proximal to the area of
263
FIGURE 2. lntravenous pyelograms (A) showing continued improvement in pyelocaliectasis and (B) at eleven months. (C) Voiding cystourethrogram showing (a) vesicoureteral reJux but no further dilation or tortuosity of ureters and (b) marked reduction in urethral dilation.
obstruction.
In fact, there may even be a frank urethral diverticulum. The most accurate test to diagnose anterior urethral valves is the voiding cystourethrogram. Panendoscopy will confirm the presence of a valve. A retrograde urethrogram may show a normal distal urethra, a suggestion of obstruction, and possibly even ballooning of the pendulous urethra. However, as Williams and Retik2 have noted, a retrograde urethrogram may fail to identify the lesion, particularly if the valve obstructs only the antegrade flow of urine from the bladder. Simple cystography may not only fail to reveal the etiology of the obstruction in the urethra, it might even suggest a primary bladder neck obstruction and lead to erroneous treatment for this entity. A catheter or infant feeding tube may be easily inserted for a cystogram even though a valve is present. This cystogram may reveal a normal
264
bladder; but if the obstruction is severe, there will be trabeculation, diverticula formation, and even varying degrees of hydronephrosis or decreased visualization of the kidneys depending on the degree of reflux or urethral obstruction. The latter changes may be seen in the intravenous pyelogram. During the voiding cystourethrogram the urethral valve may cause a decreased or diminished urinary stream and ballooning of the urethra proximally as we have shown. Management of anterior urethral valves varies. Urethral dilation with “cracking” of the valve is one method. Excision of the valvular portion of the urethra and primary anastomosis has also been used. Texter and Engel have advocated urethrotomy with excision of the valve only. In the case presented we elected to fulgurate the valve circumferentially and leave a small
UROLOGY
/ MARCH1978
/ VOLUMEXI,
NUMBER3
catheter in for several days to allow the fulgurated portion of the urethra to heal. This procedure also put the proximal urethra and bladder at rest. Blumberg and Maletta5 reported on a thirty-six-hour-old infant whom they treated with a first-stage urethroplasty and excision of the urethral valve. At two and one-half years of age, the second-stage urethroplasty was performed. Because of obstruction at the distal stoma they found it necessary to excise the stricture and shorten the urethra. Unfortunately, in the case presented, the child already had secondary damage to the proximal urinary tract. The bladder was thick and trabeculated causing relative obstruction at the ureterovesical junction. The ureters were dilated, tortuous, and emptied poorly. When it became evident that this patient had continuing renal deterioration after simple relief of the urethral obstruction, bilateral loop cutaneous ureterostomies were performed. We believe that this form of high proximal urinary diversion has an important place in the treatment of patients with tortuous, dilated, and atonic or hypotonic ureters. It affords a tubeless method of temporary upper urinary tract decompression while awaiting possible regression of proximal and distal pathology.
UROLOGY
/
MARCH
1978
/
VOLUME
XI, NUMBER
3
One other aspect of this child’s course bears comment. The development of severe hypertension following an episode of sepsis and dehydration, associated with the observed marked diminution in urinary output from the left kidney, suggests the diagnosis of acute, left renal vein thrombosis. This clinical diagnosis was further supported by the gradual resumption of normal urinary output from the left side corresponding to the return of blood pressure to normal. This was not verified by venography.
7413 Miami Lakes Drive Miami Lakes, Florida 33014 (DR. SMALL)
References 1. Baum N, Kaminsky R, and Wright DJ: Anterior urethral valves, Urology 6: 723 (1975). 2. Williams DI, and Retik AB: Congenital valve and diverticula of the anterior urethra, Br. J. Ural. 41: 228 (1969). 3. Malhoski WE, and Frank IN: Anterior urethral valves, Urology 2: 382 (1973). 4. Texter JH, and Engel RME: Anterior urethral valves as a cause of urinary obstruction: a case report, J. Ural. 107: 316 (1972). 5. Blumberg N, and Maletta T: Anterior urethral valve: complications and treatment, Urology 6: 723 (1975).
265
ANTERIOR MICHAEL
URETHRAL
P. SMALL,
LAWRENCE
VALVES
M.D.
SCHOENFELD,
M.D.
From the Department of Surgery, Division of Urology, Variety Children’s Hospital, and the Department of Urology, University of Miami, School of Medicine, Miami, Florida
ABSTRACT -A case of anterior urethral valves is presented. A diagnosis of anterior urethral valves can be suspected clinically by watching the patient void. Dribbling and a diminished face of the patient’s urinary stream are present in this condition. Conjk-mation is made with a voiding cystourethrogram and panendoscopy. Various modes of therapy are discussed.
Posterior urethral valves have been recognized as a relatively common cause of severe bladder outlet obstruction and have received much attention. In contrast, the reports of anterior urethral valves are rare. Baum, Kaminsky, and Wright’ stated that their cases were the thirteenth and fourteenth reported in the literature. Williams and Retik2 reported 17 cases of lesions of the anterior urethra with the most common lesion being a wide-mouthed diverticulum of the ventral wall of the urethra. However, they did not distinguish between congenital valves and diverticulum of the urethra. A review of the charts and x-ray films at Variety Children’s Hospital for the past twenty years revealed 29 patients with posterior urethral valves and 19 patients with congenital urethral diverticula. Only 1 case of anterior urethral valves was found. We herein present this case with a discussion of its identification and treatment because even though this is a rare condition, it may lead to severe degrees of obstruction. Case Report A six and one-half pound, white, male infant was admitted to the hospital with a diagnosis of anterior urethral valves. He had been transferred from the hospital of birth after a pediatrician had noted physical findings of dribbling upon urination, decreased size and force of the urinary stream, and ballooning of the entire
262
proximal urethra with voiding. He was the first child of healthy, young parents, and gestation and delivery had been uncomplicated. Physical examination results were normal except for the deformity on urination. Laboratory studies, including blood urea nitrogen, creatinine, and a urine culture were within normal limits. An intravenous pyelogram revealed bilateral hydronephrosis and delayed visualization. A cystogram and voiding cystourethrogram were performed (Fig. 1). No evidence of vesicourethral reflux was found, but the bladder did reveal heavy trabeculation with diverticula formation. The entire penile urethra was dilated to within approximately 1.5 cm. of the urethral meatus where it abruptly narrowed. Here a definite obstruction was seen with the total urethra markedly dilated. There was a suggestion of a urethral diverticulum just proximal to the obstruction. However, it was believed that this area was dilated secondary to the urethral obstruction and was not a true diverticulum. Urethral calibration was carried out from an 8 through 12 F without difficulty. Following this, panendoscopy was performed with an 8 F pediatric panendoscope. This revealed a dense diaphragmatic valve located approximately 2 cm. proximal to the urethral meatus. A portion of the valve had been broken, and panendoscopy through the remainder of urethra revealed dilation but no other areas of obstruction.
UROLOGY
/ MARCH 1978 / VOLUME
XI, NUMBER
3
FIGURE 1. (A) Cystogram revealing dilated posterior urethra and marked trabeculation of bladder. (B) Voiding cystourethrogram (a) anterior urethral valve and (b) dilated proximal urethra.
The bladder revealed marked trabeculation with cellule and diverticula formation. Using a small electrode, the valve was gently fulgurated circumferentially. An 8 F Foley catheter was left indwelling for five days. The patient was discharged on a regimen of ampicillin trihydrate. Three weeks later he was readmitted with urosepsis, dehydration, and a palpable right flank mass. His general condition improved with intravenous fluids and antibiotic therapy. Intravenous pyelogram demonstrated only a bilateral nephrogram at twenty minutes. High urinary diversion by bilateral loop cutaneous ureterostomies was accomplished. The postoperative course was complicated by severe hypertension. Blood pressures greater than 250/120 mm. Hg were recorded on several occasions. Urine output from the left ureterostomy was markedly less than from the right. Therapy included furosemide, hydralazine, and one course of diazoxide. His general condition improved. Intravenous pyelogram two weeks after the urinary diversion revealed considerable functional improvement. At five months of age, the intravenous pyelogram showed continued improvement although bilateral pyelocaliectasis was still present (Fig. 2A). The most recent films at eleven months revealed renal growth, and continued improvement in the pyelocaliectasis. On the voiding
UROLOGY
/ MARCH 1978
/ VOLUME
XI, NUMBER
3
cystourethrogram, vesicoureteral reflux was present but there was no further dilation or tortuosity of the ureters (Fig. 2B and C). The bladder, urethra, and ureters have shown marked resolution in their earlier changes, and the patient is now ready for closure of the ureterostomies. Comment The age at which a diagnosis of anterior urethral valves is made depends on the degree of obstruction present. Our patient was diagnosed at an early age suggesting the presence of an anterior urethral obstruction of marked severity. Malhoski and Frank3 cited 4 cases in which anterior urethral valves were diagnosed at six to eight years of age. All had relatively low-grade obstructing lesions. The presenting symptoms of older children may be enuresis, dribbling, daytime wetting, or blood staining of underclothes. Other presenting symptoms such as chronic renal failure, failure to thrive, and urinary infection depend on the severity and duration of the urethral obstruction. The diagnosis of anterior urethral obstruction can be suspected clinically on physical examination by watching the patient void. He will dribble and void with diminished force of his urinary stream. In severe cases there will be ballooning of the urethra proximal to the area of
263
FIGURE 2. lntravenous pyelograms (A) showing continued improvement in pyelocaliectasis and (B) at eleven months. (C) Voiding cystourethrogram showing (a) vesicoureteral reJux but no further dilation or tortuosity of ureters and (b) marked reduction in urethral dilation.
obstruction.
In fact, there may even be a frank urethral diverticulum. The most accurate test to diagnose anterior urethral valves is the voiding cystourethrogram. Panendoscopy will confirm the presence of a valve. A retrograde urethrogram may show a normal distal urethra, a suggestion of obstruction, and possibly even ballooning of the pendulous urethra. However, as Williams and Retik2 have noted, a retrograde urethrogram may fail to identify the lesion, particularly if the valve obstructs only the antegrade flow of urine from the bladder. Simple cystography may not only fail to reveal the etiology of the obstruction in the urethra, it might even suggest a primary bladder neck obstruction and lead to erroneous treatment for this entity. A catheter or infant feeding tube may be easily inserted for a cystogram even though a valve is present. This cystogram may reveal a normal
264
bladder; but if the obstruction is severe, there will be trabeculation, diverticula formation, and even varying degrees of hydronephrosis or decreased visualization of the kidneys depending on the degree of reflux or urethral obstruction. The latter changes may be seen in the intravenous pyelogram. During the voiding cystourethrogram the urethral valve may cause a decreased or diminished urinary stream and ballooning of the urethra proximally as we have shown. Management of anterior urethral valves varies. Urethral dilation with “cracking” of the valve is one method. Excision of the valvular portion of the urethra and primary anastomosis has also been used. Texter and Engel have advocated urethrotomy with excision of the valve only. In the case presented we elected to fulgurate the valve circumferentially and leave a small
UROLOGY
/ MARCH1978
/ VOLUMEXI,
NUMBER3
catheter in for several days to allow the fulgurated portion of the urethra to heal. This procedure also put the proximal urethra and bladder at rest. Blumberg and Maletta5 reported on a thirty-six-hour-old infant whom they treated with a first-stage urethroplasty and excision of the urethral valve. At two and one-half years of age, the second-stage urethroplasty was performed. Because of obstruction at the distal stoma they found it necessary to excise the stricture and shorten the urethra. Unfortunately, in the case presented, the child already had secondary damage to the proximal urinary tract. The bladder was thick and trabeculated causing relative obstruction at the ureterovesical junction. The ureters were dilated, tortuous, and emptied poorly. When it became evident that this patient had continuing renal deterioration after simple relief of the urethral obstruction, bilateral loop cutaneous ureterostomies were performed. We believe that this form of high proximal urinary diversion has an important place in the treatment of patients with tortuous, dilated, and atonic or hypotonic ureters. It affords a tubeless method of temporary upper urinary tract decompression while awaiting possible regression of proximal and distal pathology.
UROLOGY
/
MARCH
1978
/
VOLUME
XI, NUMBER
3
One other aspect of this child’s course bears comment. The development of severe hypertension following an episode of sepsis and dehydration, associated with the observed marked diminution in urinary output from the left kidney, suggests the diagnosis of acute, left renal vein thrombosis. This clinical diagnosis was further supported by the gradual resumption of normal urinary output from the left side corresponding to the return of blood pressure to normal. This was not verified by venography.
7413 Miami Lakes Drive Miami Lakes, Florida 33014 (DR. SMALL)
References 1. Baum N, Kaminsky R, and Wright DJ: Anterior urethral valves, Urology 6: 723 (1975). 2. Williams DI, and Retik AB: Congenital valve and diverticula of the anterior urethra, Br. J. Ural. 41: 228 (1969). 3. Malhoski WE, and Frank IN: Anterior urethral valves, Urology 2: 382 (1973). 4. Texter JH, and Engel RME: Anterior urethral valves as a cause of urinary obstruction: a case report, J. Ural. 107: 316 (1972). 5. Blumberg N, and Maletta T: Anterior urethral valve: complications and treatment, Urology 6: 723 (1975).
265