Aspects concerning posterior urethral valves

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International Urology and Nephroloyy 21 (1), pp. 57--62 (1989)

Aspects Concerning Posterior Urethral Valves M. D. MELEKOS, H. W. ASBACH, S. GIANNOULIS, P. PERIMENIS, G. BARBALIAS Departments of Urology, Patras University School of Medicine, Rio-Patras, Greece; Luebeck e Kreiskrankenhaus, Luebecke, FRG (Received September 21, 1987)

Twenty-six boys were evaluated and treated for posterior urethral vanes. At the time the valves were diagnosed unilateral or bilateral vesicoureteral reflux was present in 58 ~ of the ureters and 69 ~o of the children, while dilatation of the upper urinary tract was present in 88 ~o and 92 Yo, respectively. There was a variety of symptoms and signs, but the most prominent in neonates and infants were vesical urine retention, palpable kidneys and failure to thrive, whereas in the older children voiding dysfunction, incontinence and urinary infection were the most common. Twenty-four out of 26 boys were managed by primary valve ablation and in 62.5 ~ of them this type of treatment was adequate in resolving or sufficiently improving clinical manifestations and roentgenologic findings, requiring no further management. Urinary diversion in the form of vesicostomy and cutaneous ureterostomy was preserved for those patients who did not improve with vane ablation alone. The current aspects concerning posterior urethral valves are also discussed.

Introduction

Few areas in paediatric urology demonstrate as much controversy as do congenital urethral valves which form the most common obstructive abnormality of the lower urinary tract in the male infant. These valves have a broad spectrum of presentation affecting variably the upper and the lower urinary tract [91. Since Young and associates described and classified this malformation in 1919 [21], a significant number of reports were published, and during the last two decades there has been much discussion on the management [3, 4, 9, 20]. Herein we describe our experience on the diagnosis, treatment and results of treatment in boys with posterior urethral valves and discuss the current aspects concerning them. Material and methods

The records of 26 boys presenting with posterior urethral valves from 1977 to 1984 were reviewed retrospectively. The age of the children at the time of diagnosis is given in Table 1. Nineteen boys (73 ~ ) presented when they were < 1 year old, while 4 of them were neonates. VSP, Utrecht Akaddmiai Kiad6, Budapest

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Melekos et al.: Posterior urethral valves

Table 1 Age at presentation Age (mouth)

Group

1 2 3 4 5

No. o f patients

< 1 (neonates) 1-4 5-12 1- 3 years 4- 9 years

7 (4) 5 7 4 3 Total

26

Table 2 Correlation of clinical manifestations with age (in per cent) Groups Symptoms

Voiding dysfunction Palpable bladder Urinary infection Palpable kidney Haematuria Failure to thrive Vomiting Enuresis Abdominal distention Respiratory distress Hypertension Urinary ascites

1

2

3

4

5

14 57 29 57 14 43 29 . 43 14 0 14

20 60 20 40 20 40 40

29 43 43 29 29 29 29 . 29 0 14 0

50 25 50 0 25 25 0

67 0 33 0 33 0 0 67 0 0 0 0

.

. 40 20 0 0

0 0 25 0

Total

31 42 35 31 23 31 23 27 8 8 4

Symptoms and signs are listed in Table 2. They varied with the age at presentation, and in the majority they occurred in combination. Coexistent congenital malformations were present in 2 boys (8 %): cryptorchidism in one and hypospadias in the other. Diagnosis was made by voiding cystourethrography (VCUG) under fluoroscopic vision during various phases o f micturition, and endoscopy with the bladder overdistended. Twenty-two patients had type I valves (according to Young's classification) and 4 had type IlL Vesicoureteral reflux (VUR) was present in 29 ureters (58 %), respectively 18 children (69%), o f whom it was bilateral in 11 (61%). O f the latter the left side was affected in 5 out o f 7. International Urology and Nephrotogy 21. 1989

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The upper urinary tract was initially examined in all cases by ultrasound and in most by intravenous urography (IVP). In some newborns and severely uraemic infants, radioisotope renal scanning using 99mTc-dimercaptosuccinic acid was performed and gave reliable serial estimations of differential renal function. Unilateral or bilateral upper tract dilatation was present in 46 ureters (88%), respectively 24 children (92 %). Rettuxes and upper tract dilatations of higher grades were seen mostly in neonates and infants < 4 months old. Plasma urea and creatinine concentrations, creatinine clearance values, plasma bicarbonate levels and radioisotope scannings satisfactorily demonstrated renal function. Ten boys (38 %) had biochemical evidence of moderate or severe renal failure and of these 8 (80 %) were diagnosed below the age of 4 months.

Management and results

The initial treatment of the valves depended on the general condition of the patients and on the degree of the upper tract damage. Of the 26 children, 16 received primary urinary drainage (with intraurethral or suprapubic catheters) and antibiotics, and half of the fluid and electrolyte replacement. In cases of renal failure and severe upper urinary tract dilatation drainage was continued until acidosis, urea and creatinine values were diminishing. During that period diagnosis was done by VCUG and endoscopy, while upper tracts were examined and re-estimated by ultrasound, IVP or radioisotope scanning. In 2 newborns the suprapubic drainage remained for a longer period before valvular destruction, since their urethrae could not accommodate the scopes. Thus, possible future urethral strictures were avoided. In 2 infants, azotaemia, infection and upper tract dilatation were so severe that prior to valve ablation a bilateral nephrostomy followed by unilateral ring cutaneous ureterostomy and contralateral nephrectomy (because of a functionless kidney) were performed in one, while in the other a bilateral loop cutaneous ureterostomy was required. The latter, however, died of azotaemia despite free urinary drainage and dialysis. The remaining 24 patients were surgically managed by primary valve ablation, either through the penis, or by perineal urethrostomy (2 cases). In 15 of these children (62.5 %) - including all 7 boys > 1 year old - valve ablation alone was considered adequate in resolving or sufficiently improving clinical manifestations and roentgenologic findings, and no further treatment was employed. In the remaining 9 additional surgical procedures were required. Three underwent unilateral left nephrectomy because of a non-functioning kidney associated with massive persistent reflux. These renal units were proved to be dysplastic. Of the remaining 6 patients, Blocksom's vesicostomy [5] and bilateral ring or loop cutaneous ureterostomy were performed in 3 cases each. These procedures were undertaken in order to obtain improvements in the persistent or deteriorating azotaemia, dilated upper tracts, infection, VUR, or a combination of the former factors. Cutaneous ureterostomies, however, were done in the most severe form International Urology and Nephrology 21, 1989

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Melekos et al. : Posterior urethral valves

of obstruction. In 2 out of 3 cases vesicostomies resolved or sufficiently improved the clinical signs and the roentgenologic changes, despite the fact that in 1 of these 2 patients ureteral tailoring and reimplantations were undertaken later. In the third child vesicostomy was insufficient to improve the former findings, thus a bilateral ring cutaneous ureterostomy was performed. In this boy and in 1 of the remaining 3 who underwent ureterostomies megaureter repair and reimplantation were required in 3 ureters. All ureterostomy diverted children underwent 7-21 months later ureteral reconstruction, and during that period bladder irrigations with saline through ureterostomies were frequently done in order to diminish the possible event of bladder contracture. Preoperative hypertension was monitored in 2 patients, but blood pressure was normalized in both instances after surgical intervention. Urinary incontinence persisted after valve ablation in 2 children, while urethral stricture developed in one.

Discussion

Our data and those of others [6, 9, 18, 20] suggest that the main presenting signs in neonates and infants with posterior urethral valves are bladder distention, palpable kidneys, abdominal distention and failure to thrive, whereas in older children voiding dysfunction (straining, urine dribbling, poor stream, frequency, daytime urgency), incontinence and infection are the predominant clinical features. The need to individualize treatment is clear. In the older child with more or less well preserved renal function diagnosis may be followed by endoscopic urethral valve ablation without any special preoperative preparation. But in the usually uraemic neonates and infants much more intensive preoperative care is required, and urethral or suprapubic catheter drainage may be maintained for longer periods. Based on the experience of this series and on that of others [2, 6, 16, 17] we believe that early resection of the valves alone can be successful in > 60 % of these patients. Even severe degrees of ureteral dilatation or/and reflux may respond to primary valve ablation, since these ureters may have still adequate musculature and retain the capacity for remarkable improvement with relief of the obstruction [8]. However, there are some children in whom renal function fails to improve, infection persists and radiology shows grossly dilated tortuous ureters. In these circumstances there may arise the need for instituting long-term bladder (vesicostomy) or upper tract drainage (ureterostomy). The decision to institute bladder or upper tract drainage is controversial. The judgement between these two types of management really depends on a careful assessment of the clinical and radiological state of the child. In the milder forms it seems likely that Blocksom's vesicostomy is the preferable way to assume continuous vesical emptiness [4, 5]. Two out of 3 children in the present study responded well to this management. International Urology and Nephrology 21, 1989

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Cutaneous ureterostomy is usually preserved for those children in whom azotaemia, severe upper tract dilatation and infection do not improve following catheter drainage, valve ablation or vesicostomy. This inability of improvement in some patients is based on the obstruction that may be above the level of the bladder secondary to kinks and/or significant atony of the ureter or fibrosis of the distal ureter. Ureterostomy has been traditionally used by Hendren [10], Johnston [14] and Stephens [18] and can remain for months to allow maximum renal improvement and shrinkage of the ureters until definitive treatment is completed. It is reported, however, that prolonged upper tract drainage may lead to a contracted fibrotic bladder [19], but we think that this happens infrequently, especially after using bladder irrigations through the ureterostomies. Reflux, which is more common in the younger and usually more obstructed boy [4, 6, 20], in association with a reasonably functioning kidney shows a marked tendency to cease spontaneously (44 ~o in this series) following valve resection [15]. Further improvement may be achieved after urinary diversion procedures and, in the absence of persistent infection or decreasing renal function, thepresence of reflux alone is not an indication for primary ureteral reimplantation. In patients with reflux reimplantation can be performed on the basis of indications similar to those used for patients with reflux who never had valves, and only when the bladder has returned to normal after valve ablation. The association of massive dilatation with severe persistent unilateral reflux is a particularly ominous sign for that renal unit, which usually is dysplastic [11, 13]. Four such cases were monitored in this series, 3 kidneys of which were dysplastic. It is reported that the syndrome: posterior urethral valves, unilateral reflux and renal dysplasia usually affects the left side and its incidence may range even up to 15-20~o of the cases [13]. It is believed that reflux and renal dysplasia are both sequelae of abnormal ureteric budding [12]. Several conclusions were reached by the data in our study: (1) Primary resection of the valves alone is successful in >60 ~oof the cases and in few of them the severely dilated and tortuous ureters usually improve strikingly with time. (2) Temporary urinary diversion (e.g. ureterostomy) has a place in the severely ill infant or a child who has not responded to valve ablation alone, but these cases are fewer today. (3) After a period of diversion ureteral dilatation is decreased so much that a tailored reimplant is needed in few cases only. (4) Reflux is not a major problem in the initial management plan, but massive persistent unilateral reflux is usually associated with a non-functioning and commonly dysplastic renal unit.

References 1. Bauer, S. B., Dieppa, R. A., Labib, K. K., Retik, A. B. : The bladder in boys with posterior urethral valves: an urodynamic assessment. J. Urol., 121,769 (1978). 2. Carpiniello, V., Duckett, J. W., Filmer, R. B.: Posterior urethral valves: a review of 57 cases. Proc. Kimbrough Urol. Sem., 11, 282 (1978). International Uroloqv and hrephrology 21, 1989

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3. Cass, A. S., Stephens, F. D.: Posterior urethral valves: Diagnosis and management. J. Urol., 112, 519 (1974). 4. Duckett, J. W. Jr.: Current management of posterior urethral valves. Urol. Clin. North Am., 1, 471 (1974). 5. Duckett, J. W. Jr. : Cutaneous vesicostomy in childhood: the Blocksom technique. Urol., Clin. North Am., 1, 485 (1974). 6. Egami, K., Smith, D. 9 A study of the sequelae of posterior urethral valves. J. Urol., 127, 84 (1982). 7. Ericsson, N. O." Posterior urethral valves. Birth Defects, 13, 53 (1977). 8. Hanna, M. K., Jeffs, R. D., Sturgess, J. M., Barkin, M. : Ureteral structure and ultrastruc= ture. Part III; Congenitally dilated ureter (megaureter). J. Urol., 117, 24 (1977). 9. Hendren, W. H.: Posterior urethral vanes in boys. A broad clinical spectrum..1. Urol., 106, 298 (1971). 10. Hendren, W. H. : Complications of urethral valve surgery. In: R. B. Smith, D. G. Skinner (eds): Complications of Urologic Surgery. Prevention and Management. Chapter 16. W. B. Saunders Co., Philadelphia 1976, p. 303. 11. Henkel, H. B., Samuel, D., Lascari, A. D., Collins, J. G., Texter, J. H.: Urethral valves and ureterovesical reflux associated with unsuspected findings in a non-functioning kidney. J. Urol, 128, 788 (1982). 12. Hennebery, M. O., Stephens, F. D,: Renal hypoplasia in infants with posterior urethral valves. J.Urol., 123, 912 (1980). 13. Hoover, D. L., Duckett, J. W. Jr.: Posterior urethral valves, unilateral reflux and renal dysplasia: a syndrome. J. Urol., 128, 994 (1982). 14. Johnston, J. H., Kulatilake, A. E. : Posterior urethral valves: Results and sequelae. In" J. H. Johnston, R. J. Scholtmeijer (eds) : Problems in Paediatric Urology. Chapter 8. E• Medica, Amsterdam 1972, p. 161. 15. Johnston, J. H.: Reflux secondary to severe urethral obstruction. In: Management of Vesicourethral Reflux. The Williams and Wilkins Co., Baltimore 1984. 16. Rabinowitz, R., Barkin, M., Schillinger; F. J., Jeffs, R. D., Cook, G. T.: Upper tract management when posterior urethral valve ablation is insufficient. J. Urol., 122, 370 (1979). 17. Scott, J. E. S. : Management of congenital posterior urethral valves. Br. J. Urol., 57, 71 (1985). 18. Stephens, F. D. : Congenital lesions of the posterior urethra. In: Congenital Malformations of the Urinary Tract. Praeger Publishers, New York 1983. 19. Tanagho, E.A.: Congenitally obstructed bladders: Fate after prolonged defunctionaliza= tion. J. Urol., 111, 102 (1974). 20. Williams, D. I., Whitaker, R. H., Barrett, T. M., Keeton, J. E. : Urethral valves. Br. J. UroL, 45, 200 (1973), 21. Young, H. H., Frontz, W. A., Baldwin, J. C. : Congenital obstruction of the posterior uretra. J. Urol., 3, 289 (1919).

International Urologyand Nephrolo#,v21, 1989

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