POSTERIOR

URETHRAL

H. W. SCHOENBERG,

VALVES

M.D.

K. MIYAI, M.D. J. G. GREGORY,

M.D.

From the Department of Surgery, Section of Urology, St. Louis University School of Medicine, St. Louis, Missouri

ABSTRACT - Twenty cases of posterior urethral valves are reviewed with the presentation of several illustrative cases. Review of the current literature regarding management of patients is discussed.

The diagnosis and management of posterior urethral valves have been simplified by improvements in cystourethrography and more recently by development of improved pediatric endoscopic instruments. Recently much has been written concerning the techniques of endoscopic destruction of valves, but from our experience the more complicated aspect of the management of patients with urethral valves remains the management of vesical and ureteral dysfunction and renal deterioration associated with many such lesions. It is necessary to be alert to the possibility of these problems which may become manifest after any step in the rehabilitation of patients with urethral valves. This article reviews our experience with posterior urethral valves and illustrates some of the problems encountered. Twenty boys with posterior urethral valves have been treated at St. Louis University (Cardinal Glennon Memorial Hospital) between September, 1963, and June, 1974. More than half of these patients have been treated since July, 1972. Half of the patients were less than one year old when first seen and 75 per cent were under three years old (Table 1). Boys less than one year of age usually presented with urinary retention (33 per cent) and/or an abdominal mass, or failure to thrive. Older children had more specific urinary symptoms including poor urinary stream, straining on urination, and urinary infection. Half of the patients were azotemic, and electrolyte abnormalities were noted in 85 per cent of patients

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under one year of age. The urine was infected in 45 per cent of patients. Diagnosis was made in all cases by voiding cystourethrography and confirmed by endoscopic examination. Associated radiographic findings included vesicoureteral reflux in I2 patients and varying degrees of hydronephrosis in 16. A number of patients had small and large vesical diverticula. Only 4 patients had normal excretory urograms at the time of diagnosis. Therapy for 14 patients consisted of a primary attack on the valve by the transurethral route. These constitute the most recent cases. Three had primary open procedures to destroy the valve (transvesical excision of the valve with YV-plasty of the bladder neck). Of the remaining 3 patients, an ileal conduit was made in 1 and 2 are waiting for transurethral resection, 1 with ureterostomy and the other with vesicotomy. TABLE

I.

Age of patient at presentation

Age

Number

Newborn to 1 month 1 month to 1 year 1 to 2 years 2 to 3 years 3 to 4 years 4 to 5 years 5 to 6 years 6 to 7 years 12 years

6 3 3 2 1 1 2 1 1

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FIGURE 1. Voiding cystourethrograms. and (D) postoperative.

Case 1: (A) preoperative and(B) postoperative. Case 2: (C) preoperative

Eight patients were treated by preliminary drainage procedures prior to resection of the valve: loop cutaneous ureterostomy in 4; suprapubic cystostomy (emergency) in 3; and cutaneous vesicotomy in 1. Three patients required urinary diversion because of progressive upper tract deterioration after removal of the valve. One patient early in the series required permanent diversion by ureteroileostomy. There were no deaths in this series. The following selected cases illustrate the varied presentations and complications encountered. Case Reports Case 1 An eleven-month-old white male was seen with fever and urinary retention. A distended bladder was palpable. Blood urea nitrogen was 85 mg. per 100 ml. and potassium 7.2 mEq. per liter. On voiding cystourethrography a trabeculated bladder and posterior urethral valve were noted without vesicoureteral reflux (Fig. 1A). Excretory urogram was normal except for decreased concentration related to azotemia. After several days of catheter drainage blood urea nitrogen fell to normal. Transurethral resection of the valve was done. Postoperatively the urethra appeared to be normal on voiding radiographs. One year later the urine is sterile, voiding is normal, and the bladder empties almost completely (Fig. 1B).

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Case 2

A twelve-year-old white male was admitted with a four-day history of gross hematuria after wrestling. There were no previous urologic problems. Findings on physical examination were unremarkable. Blood urea nitrogen was 20 mg. per 100 ml. The urine was bloody and infected with alpha streptococci. Voiding cystourethrogram showed a posterior urethral valve and a large bladder diverticulum with left vesicoureteral reflux (Fig. 1C). Excretory urogram was normal. The urethral valve was resected via the urethra. Postoperatively the patient stated that he voided without straining for the first time in his life, and a postoperative voiding cystourethrogram showed an improved urethra (Fig. 1D). Subsequently, vesical diverticulectomy and left ureteroneocystostomy were done. Follow-up studies done in another city are reported to be satisfactory. Case 3 A fourteen-month-old white male was admitted for evaluation of an abdominal mass. A large soft mass was palpable in the left upper quadrant and another in the right upper quadrant. Blood urea nitrogen was 53 mg. per 100 ml,, sodium 128 mEq. and potassium 6.4 mEq. per liter. Urine was sterile. Excretory urogram and voiding cystourethrogram showed marked bilateral hydronephrosis with only faint visualization of the left

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FIGURE 2. Case 3: (A) preoperatiue excretory urogram; (B) preoperatiue and (C) postoperative cystourethrograms. Case 4: voiding cystourethrograms (D and E) preoperative and (F) postoperatiue.

kidney and a posterior urethral valve with massive reflux into the left kidney (Fig. 2A and B). Transurethral resection of the valve was performed. Postoperatively the urethra had a normal appearance, but on voiding much of the bladder contents refluxed into the nonfunctioning left kidney (Fig. 2C). Therefore, a left nephroureterectomy was performed. Subsequently, increasing right hydronephrosis was noted associated with increasing bladder residual. This was thought to be related to bladder decompensation. A right loop cutaneous ureterostomy was performed with subsequent improvement in hydronephrosis. Serial studies after a year demonstrated improvement in bladder function with a normal urethra. The ureterostomy was closed and bladder function is now satisfactory. Closure of the ureterostomy did not

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uoiding

produce evidence of obstruction or deterioration of the remaining kidney. Case 4

A two-week-old black child was admitted with history of failure to thrive and abdominal distention. The child was extremely ill with evidence of dehydration, electrolyte imbalance, azotemia, acidosis, and sepsis. Potassium was 10.5 mEq. and carbon dioxide 11.1 mEq. per liter; blood urea nitrogen was 124 mg. per 100 ml. The urine was infected with pseudomonas. Voiding cystourethrogram showed marked trabeculation of the bladder, reflux with massive bilateral hydronephrosis, and a dilated posterior urethra typical of urethral valve (Fig. 2D and E). The patient

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FIGURE 3. Case 5: preoperative (A)

voiding cystourethrograms

showing extravasation and (B) suggesting urethral valve. (C) Perforation of kidney. (D) Voiding cystourethrogram after transurethral resection of valve.

underwent left loop cutaneous ureterostomy under local anesthesia. A dramatic improvement in both kidneys was seen. The left loop cutaneous ureterostomy decompressed both kidneys because of free bilateral vesicoureteral reflux. When the patient was age three, the loop ureterostomy was closed with a satisfactory result after transurethral resection of the valve (Fig. 2F). Minimal reflux persists which may ultimately require correction. Case 5

A nine-day-old black child entered the hospital with a history of anuria of twelve hours and marked abdominal distention. Physical examination revealed ascites. No distention of the bladder was noted. Findings on urinalysis were negative. The child had respiratory difficulty due to abdominal distention. Blood urea nitrogen was 40 mg. per 100 ml. and potassium 7.6 mEq. per liter. Voiding cystourethrogram demonstrated right vesicoureteral reflux with evidence of extravasation of contrast medium through the right calyceal system (Fig. 3A). Although preoperative voiding studies were not entirely satisfactory, they suggested presence of a valve (Fig. 3B). At exploration a perforation of the right renal parenchyma was found with efflux of urine (Fig. 3C). This was sutured and the area drained. Because of

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evidence of urethral obstruction, vesicotomy was done. Follow-up urograms were normal except for minimal blunting of the right pelviocalyceal system. When the child was twenty-two months of age, transurethral resection of the valve was done and the ureterostomy closed with a satisfactory result (Fig. 3D). Case 6

A newborn black child was admitted with sepsis, azotemia, and electrolyte imbalance. Excretory studies showed marked hydronephrosis on the right and almost no function on the left (Fig. 4A). Voiding study demonstrated a valve (Fig. 4B). After correction of electrolyte problems transurethral resection was carried out (Fig. 4C). Three months later the child is clinically well; excretory study shows slow improvement but is far from perfect. Comment Improvements in pediatric endoscopic instruments have enabled us to attack urethral valves via the urethra even in the neonatal period. While many authors1-6 recommend transurethral resection or other forms of endoscopic destruction of the valves as the primary treatment in patients with this problem, preliminary temporary urinary

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diversion is useful in some severely ill patients.‘** Patients with normal upper urinary tracts are usually well managed by transurethral resection alone as demonstrated in Case 1. When the upper urinary tracts have been spared because of large vesical diverticula, resection and diverticulectomy will suffice as illustrated in Case 2. Many children with moderately advanced hydroureteronephrosis will show marked improvement after simple destruction of the valve. It may require months for improvement to occur as illustrated in Case 6. If, however, after removal of the valve azotemia increases, infection cannot be controlled, or evidence of increasing deterioration of the upper tracts is noted secondary to bladder failure, vesical or supravesical diversion may be necessary as illustrated in Case 3.‘,‘-12 Resection of valves in the neonate is technically more difficult, and for the urologist who deals with urethral valves only occasionally complications such as incontinence may be avoided if a preliminary diversion is used to allow the child to grow making resection easier. In infants the technique of fulguration of the valve by a wire passed through a ureteral catheter or by an insulated diathermy hook has been used by several authors with success. 1*3,4~13 In severe upper urinary tract deterioration some forms of diversion may often be necessary temporarily as seen in Case 4. Nephrostomy,

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cutaneous pyelostomy, cutaneous ureterostomy, vesicotomy, and cystostomy have all been used in this situation, and all have their advocates. We have used vesicotomy and loop ureterostomy most often. It must be noted that in this clinical situation Hendren4$12 might recommend destruction of the valve with primary reconstruction of the upper urinary tract. Case 5 illustrates the problem of neonatal ascites, one of the unusual complications of valve disease. 14-16 Since vesicoureteral reflux associated with valves will sometimes disappear after removal of the valve, most authors do not employ antireflux procedures early in the management of valves although later correction of the reflux may be necessary. 1,3~13Antireflux procedures should be avoided in conjunction with closure of loop ureterostomy because of the possibility of impairing the blood supply to the lower ureteral segment. 1325 South Grand Boulevard St. Louis, Missouri 63104 (DR. SCHOENBERG) References

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urethral

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2. BUESHEN, A. J., GARRETT, R. A., and NEWMAN, D. M.: Posterior urethral valves: management, J. Urol. 110:682 (1973). 3. WILLIAMS, D. I., WHITAKER, R. H., BARRATT,T. M., Urethral valves, Br. J. Urol. 45: and KEETON, J. E.: 200 (1973). 4. HENDREN, W. H. : Posterior urethral valves in boys, a broad clinical spectrum, J. Urol. 110:682 (1973). 5. JOHNSTON, J. H., and KULATILAKE, A. E.: The sequelae ofposterior urethral valves, Br. J. Urol. 43: 743 (1971). 6. WATERHOUSE,K., and HAMM,F. C. : The importance of urethral valve as a cause of vesical neck obstruction in children, J. Urol. 87: 464 (1962). 7. WALDBAUM,R. S., and MARSHALL,V. F.: Posterior urethral valves: evaluation and surgical management, ibid. 103: 801 (1970). 8. RAPER, F. P.: The recognition and treatment of congenital urethral valves, Br. J. Urol. 25: 136 (1953). 9. LOME, L. G., and WILLIAMS, D. I.: Urinary recon-

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struction following temporary cutaneous ureterostomy diversion in childhood, J. Urol. 108: 162 (1972). 10. KUMAR,M., SHROFF, N., and BHAT, H. S.: Posterior urethral valve: problems in management of the upper urinary tract, Br. J. Urol. 44: 486 (1972). 11. JOHNSTON, J. H.: Temporary cutaneous uretherostomy in the management ofadvanced congenital urinary obstruction, Arch. Dis. Child. 38: 161 (1963). 12. HENDREN, W. H.: Operative repair of megaureter in children, J. Urol. 101:491 (1969). 13. CASS, A. S., and STEPHENS, D.: Posterior urethral valves: diagnosis and management, ibid. 112:519(1974). 14. EVANS, R. J., and BROWN, H. E.: Ascites in newborn infant, Urology 1: 386 (1973). 15. THOMPSON,I. M., and BURNS, T. N. C.: Neonatal ascites: a reflection of obstructive disease, J. Urol. 107: 509 (1972). 16. PARKER,R. M.: Neonatal urinary ascites: a potentially favorable sign in bladder outlet obstruction, Urology 3: 589 (1974).

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Posterior urethral valves.

POSTERIOR URETHRAL H. W. SCHOENBERG, VALVES M.D. K. MIYAI, M.D. J. G. GREGORY, M.D. From the Department of Surgery, Section of Urology, St. Lou...
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