Posterior Urethral Valves: Management

Posterior Urethral Valves: Management

Vol. llO, December THE JOURNAL OF UROLOG, Copyright © 197:3 by The Williams & Wilkins Co. Printed in U.S.A. POSTERIOR URETHRAL VALVES: MANAGEMENT ...

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Vol. llO, December

THE JOURNAL OF UROLOG,

Copyright © 197:3 by The Williams & Wilkins Co.

Printed in U.S.A.

POSTERIOR URETHRAL VALVES: MANAGEMENT ANTON ,J. BUESCHEN,* ROBERT A. GARRETT

AND

DANIEL M. NEWMAN

From the Department of Urology, Indiana University Medical Center and the Methodist Hospital Medical Center, Indianapolis, Indiana

tion and/or urinary ascites were recognized in 4 patients, all of whom were less than 2 months old.

Posterior urethral valves were initially described by Young and associates in 1919. 1 For the next 3 decades few cases were reported in the literature. The voiding cystourethrogram has caused this entity to be recognized more frequently. Treatment has remained controversial. We review herein cases of posterior urethral valves seen during the past 11 years in an attempt to improve future treatment.

TREATMENT AND RESULTS

MATERIAL

Forty-seven patients with posterior urethral valves were hospitalized between 1961 and 1971. All patients were children except for a 29-year-old man. A division of these patients into 2 groups according to age proved meaningful. Group 1 consists of 16 patients less than 3 months old and group 2 includes all patients more than 3 months old. The median age for group 2 was 4 years. Clinical presentation and treatment were different in group 1 than in the older group. CLINICAL PRESENTATION

The most common presenting symptom in group 1 was an abdominal mass (table 1). Several of these children had respiratory distress shortly after hospitalization although they did not manifest this finding initially. In group 2 urinary tract infections, poor urinary stream, enuresis, abdominal mass and failure to thrive were commonly noted. The excretory urogram (IVP) was abnormal in all patients in group 1 and in all but 8 of the older children (table 2). The initial urine culture was positive in only 4 of the 16 boys in group 1 and 11 of 31 in group 2. Many patients had infection shortly after the first introduction of a catheter or cystoscope despite careful technique and antibacterial treatment. Vesicoureteral reflux was present in 63 per cent of group 1 and 70 per cent of group 2 when first evaluated. The blood urea nitrogen (BUN) was significantly elevated in 11 of the children in group 1 and 14 of 31 in the older group. The BUN was more than 40 mg. per cent of all those elevated in the young group whereas in only 8 of those elevated in the older group. Perirenal extravasaAccepted for publication June 15, 1973. Read at annual meeting of Southeastern Section, American Urological Association, Palm Beach, Florida, April 15-18, 1973. * Current address: University of Alabama School of Medicine, Birmingham, Alabama 35294. 1 Young, H. H., Frontz, W. A. and Baldwin, J. C.: Congenital obstruction of the posterior urethra. J. Urol., 3: 289, 1919. 682

Suprapubic cystostomy was used as the initial treatment in 7 of group 1 and 8 of group 2 patients. All but 1 of these in group 1 were converted to supravesical diversion owing to persistence or progression of the hydronephrosis. Of these children 5 had vesicoureteral reflux prior to cystostomy. Reflux was bilateral in 3 infants. Four of 8 older patients required subsequent supravesical diversion after cystostomy. All 4 of these patients had reflux, 3 of them bilaterally: One of the 4 patients not requiring higher diversion has been treated successfully with fulguration of prostatic valves and subsequent removal of the suprapubic tube. One patient is incontinent after transurethral resection of valves. The other 2 patients who had dysplastic kidneys are continuing with supra pubic tube drainage. Transurethral resection or fulguration of valves was done as the initial treatment in 2 infants in group 1 and in 15 of the older patients. One of the boys in group 1 was 3 months old when fulguration was done. The remaining kidney has responded well (fig. 1). The other patient, who was less than 3 months old, had perforation of the urethra during transurethral resection. He had a temporary suprapubic cystostomy and the valves were destroyed transvesically during that procedure. He was lost to followup when he was 4 months old. Only 2 of the 15 patients in group 2 who underwent transurethral resection required subsequent urinary diversion. The remaining 13 are voiding, 9 are continent, 1 is not yet toilet-trained, 1 has total incontinence and 2 have nocturnal enuresis. In 2 of these patients who had unilateral reflux into a non-functional kidney subsequent nephroureterectomy was necessary. Of the 15 patients 6 had normal kidneys on IVP. An example of improvement following transurethral resection is shown in figure 2. Reconstruction of the urinary tract after initial loop ureterostomy has been accomplished in 5 patients, 3 of whom had loop ureterostomy in the neonatal period (figs. 3 and 4). Of these, 1 had perirenal extravasation of urine and another had urinary ascites. In each case of extravasation, the extravasating kidney proved to be the only functional kidney. Subsequent reconstruction after loop ureterostomy was initiated by transurethral fulguration of the valves. After a satisfactory

683 v.ras derc_onstrated the excision of tomy anastomosis with fine chromic catgut sutures in 2 Care was taken to avoid ureteral redundance and to the reanastomosed ureter in its proper in :3 of A significant urethral stricture TABLE

1. Presenting symptoms

-----

Group '.2i· Abdominal mass Urinary: infect ion Poor st ream ~:nuresis Failure to thrive Hematuria Seizure Fever Hypertension Elevated Bl:1' Rickets Vomiting

* Less than :l months old. "i- More than :3 months old.

patients i~ group L Tvvo had lesions and permanent cutaneous ureterostomy. The third patient underwent urethral dilatation with anesthesia before reconstruction of the urinary tract. This boy is well 9 months after closure of the cutaneous ureterostomy. There were 4 deaths in group 1 and 1 patient in group 2 is presumably dead. Of the patients who

1\)

6

1 2

10

9 6 5

2

TABLE

2. !VP Group l *

Normal kidneys Bilateral hydronephrosis Bilateral non-visualization Bilateral dysplastic kidney Hydronephrosis and non-visualization Dysplastic and normal kidney Dysplastic and hydronephrosis Bilateral pyelonephrit is Not done (elevated BUN J

Group

'.2t

8

8 3 l 4

l:J

4

* Less than :l months old.

t More than :J months old.

FIG. 1. Three-month-old hoy with valves who underwent transurethral resection initially. A, preoperative IVP shows non-function on left side and marked hydronephrosis on right side. B, 1 year later. C, 2 years after valve resection. Left nephrectomy has been done.

FIG. 2. Ten-year-old boy in whom valves were resected and non-functional left kidney was removed. A, preoperative IVP. B, IVP 9 months postoperatively. C, IVP 21 months postoperatively.

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BUESCHEN, GARRETT AND NEWMAN

FIG. 3. Eighteen-day-old boy with urinary ascites. A, initial IVP with left non-function and _moderate right hydronephrosis. B, resolution of hydronephrosis after loop cutaneous ureterostomy. C, IVP when patient was 4 years old after take down of right loop ureterostomy, fulguration of valves and left nephrectomy.

FIG. 4. Two-day-old boy with perirenal urinary extravasation. A, initial IVP shows right renal non-function and large left perirenal mass. B, resolution of extravasation after left loop cutaneous ureterostomy. C, IVP of left side after take down of left cutaneous loop ureterostomy and fulguration of prostatic valves when patient was 5 years old.

died 3 presented during the first week of life and 1 of these died after bilateral loop ureterostomy. Autopsy demonstrated bilateral dysplastic kidneys. Two other patients died of respiratory arrest prior to any surgical treatment. A fourth patient was hospitalized when he was 5 weeks old for failure to thrive. This infant was in poor condition, was treated with urethral catheter drainage for several days and died approximately 12 hours after bilateral loop ureterostomy. DISCUSSION

Owing to differences in clinical presentation and necessary subsequent treatment a division of these patients into groups according to age is meaningful. Generally the younger patient exhibits a more severe degree of renal damage. This is also demonstrated in this series by mortality rate, IVP, renal function studies and subsequent or current status of the urinary tract in the 2 age groups. The neonatal mortality rate of 25 per cent herein

reported compares favorably to that reported m other series. 2 - 5 One adult patient presenting at age 29 with hypertension and urographic changes of chronic pyelonephritis represents a distinct rarity. Only 4 other such patients have been described. 6 • 7 Vesicoureteral reflux was found in 67 per cent of our patients. This is a higher incidence than reported by other investigators 3 · 5 and probably 2 Williams, D. I. and Eckstein, H. B.: Obstructive valves in the posterior urethra. J. Urol., 93: 236, 1965. 'Johnston, J. H. and Kulatilake, A. E.: The sequelae of posterior urethral valves. Brit. J. Urol., 43: 743, 1971. 'Retief, P. ,J.: Urethral valve obstruction. S. Afr. Med. J., 44: 181, 1970. 'Ellis, D. G., Fonkalsrud, E.W. and Smith, J.P.: Congenital posterior urethral valves. J. Urol., 95: 549, 1966. 6 Waldbaum, R. S. and Marshall, V. F.: Posterior urethral valves: evaluation and surgical management. J. Urol., 103: 801, 1970. 7 Marsden, R. T. H.: Posterior urethral valves in adults. Brit. J. Urol., 41: 586, 1969.

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sterns froL1 our intensive and wide use of the voiding cine Reflux often stops spontaneously after relief of obstruction. Few antireflux operations were done in this series and are reportedly often unsuccessful. 2 · 8 Coexistence of fetal urinary obstruction and cystic kidneys has been intensively studied 9 • 1 2 and has been related to coexistent ipsilateral reflux. Urinary ascites or perirenal urinary extravasation was found in 4 of our neonatal patients. This has been previously reported and is thought not to be as rare as formerly considered. 2 • 13 · 14 Indeed in the neonate who has valves, this phenomenon may be expected to be found in a significant minority. Primary management of the obstructing valves has been recommended as the preferred treatment.2· 3 • •. 15 • 16 Other investigators suggest preliminary diversion as the treatment of choice. •-s. 17 There is no doubt that transurethral resection or preferably fulguration when clinically feasible is the easiest and best treatment for the patient. There are many occasions when resection can be used as the only treatment even with hydronephrosis and azotemia if infection is controlled. How8 Waterhouse, K. and Hamm, F. C.: The importance of urethral valves as a cause of vesical neck obstruction in children. ,J. Urol., 87: 404, 1962. 'Cussen, L. ,J.: Cystic kidneys in children with congenital urethral obstruction. J. Urol., 106: 939, 1971. 1 " Osathanondh, V. and Potter, E. L.: Pathogenesis of polycystic kidneys. Type 4 due to urethral obstruction. Arch. Path., 77: 502, 1964. 11 Beck, A. D.: The effect of intra-uterine urinary obstruction upon the development of the fetal kidney. J. Urol., 105: 784, 1971. 12 Pathak, I. G. and Williams, D. I.: Multicystic and cystic dysplastic kidneys. Brit. ,J. Urol.. 36: :,18, 1964. 13 Garrett, R. A. and Franken, E, A., ,Jr.: Neonatal ascites: perirenal urinary extravasation with bladder outlet obstruction. J. Urol., 102: 627, 1969. 11 Britt, D. B., Bucy, ,J. G. and Robison, .J_ R.: Urinary ascites in the neonate. Southern Med. J., 64: :399. 1971. 15 Anderson, K. N. and Ansell, ,J. S.: Congenital valves of the posterior urethra. J. Urol., 95: 78:3, 1966. 1 ' Hendren, W. H.: Posterior urethral valves in boys. A broad clinical spectrum. J. Urol., 106: 298, 1971. 17 Raper, F. P.: The recognition and treatment of congenital urethral valves. Brit. ,J. Urol., 25: 1:16, 195:l.

ever, transurethral resection for children presenting before 3 months of age is not recommended. Mortality rate in neonates is approximately 50 per cent in many series in which transurethral resection is the initial form of treatment. 2 · 4 Only 4 patients (25 per cent) in the younger age group are voiding with continence, 3 after preliminary cutaneous ureterostomy and reconstruction of the urinary tract. Neonates suspected of harboring posterior urethral valves should have prompt urologic evaluation, including complete blood count, BUN, serum creatinine, electrolytes, serum calcium, urinalysis and culture and IVP. If valves are still suspected after these studies a voiding cystourethrogram should be done, preferably with an intracath polyethylene tube introduced into the bladder suprapubically. Supravesical diversion is indicated if valves are demonstrated with severe hydronephrosis. Cystostomy is inadequate owing to subsequent development of ureterovesical obstruction after suprapubic drainage. Bilateral loop cutaneous ureterostomy offers good drainage, is easy to perform, can be managed in infancy much easier than nephrostomy and eliminates the need for a foreign body. In cases in which loop ureterostomy is unsuccessful in achieving good drainage, nephrostomy or cutaneous pyelostomy can be performed. Panendoscopy and rigid urethral instrumentation should be avoided if diagnosis can be established in lieu of its usage. The incidence of urethral stricture and inducement of infection can thus be held at a minimum. SUMMARY

The management of 47 patients with posterior urethral valves has been reviewed. The treatment of neonates should be carried out promptly with supravesical diversion recommended as the initial treatment. Transurethral fulguration of valves often can be used as the only treatment in children more than :-i months old. Older patients with advanced renal obstruction may also require supravesical diversion.