Pediat. Radio1. 4, 43-46 (1975) 9 by Springer-Verlag 1975
Pre- and Postoperative Urographic Findings in Posterior Urethral Valves':' O. Ekl/Sf a n d H. R i n g e r t z Department of Pediatric Radiology, Karolinska Sjukhuset, Stockholm, Sweden Date of final acceptance: April 4, 1975
Abstract. In a series of 65 male infants and children, all with the diagnosis of posterior urethral valves, pre- and postoperative urographic findings were reviewed. In addition changes occurring in the bladder, and the implication of vesico-ureteral reflux were assessed. -- Preoperatively diagnosed impairment of kidney function and concommitant dilatation of the upper urinary tract, with some exceptions. remained fairly unchanged at postoperative examinations In the case of marked vesico-ureteraI reflux, permanent kidney function annihilation was significantly commoner
than with slight or no reflux. -- Although, as a rule, both the upper urinary tract and the bladder were affected, there were cases of posterior urethral valves with a normal appearing bladder. As the intravenous urography do riot exclude the urethral abnormality, voiding cysto-urethrography has to be included in the primary radiological exploration of all cases with urological problems. Key words: Intravenous urography, non-functioning kidney, upper urinary tract dilatation, voiding cysto-urethrography, posterior urethral valve, bladder outflow obstruction.
A c c o r d i n g to r e c e n t s t a t e m e n t s i n the literature, p o s t e r i o r u r e t h r a l valves m u s t b e r e g a r d e d as the m o s t serious l o w e r u r i n a r y tract a b n o r m a l i t y of t h e male i n f a n t a n d child. D e p e n d i n g u p o n age at p r e s e n t a t i o n p r i m a r y m o r t a l i t y rates of 17--50 % are r e p o r t e d [3, 5, 7, 8]. I n the past r a d i o l o g i s t s seem p r i n c i p a l l y to h a v e f o c u s e d t h e i r i n t e r e s t o n different aspects of the d i a g n o s t i c a l l y critical v o i d i n g c y s t o - u r e t h r o g r a p h y , m o r e or less l e a v i n g the f i n d i n g s disclosed at i n t r a v e n o u s u r o g r a p h y o u t of discussion. E v e n so the d e g r e e of i m p a i r e d k i d n e y f u n c t i o n to a great e x t e n t d e t e r m i n e s the l o n g t e r m p r o g n o s i s of the c o n d i t i o n . I t therefore a p p e a r e d i m p o r t a n t to analyze the i n f l u e n c e of t r e a t m e n t o n the e v o l u t i o n of k i d n e y f u n c t i o n as e v a l u a t e d o n the base of available i n t r a venous urograms. In addition, changes occurring i n b l a d d e r a p p e a r a n c e a n d the i m p l i c a t i o n of vesicou r e t e r a l reflux will be briefly discussed.
urethra were other constant features. At the level of the valve urethral lumen was restricted to a narrow dorsal slit. The size of this slit is one of the main factors determining urethral flow-. In advanced cases the obstructive mechanism frequently becomes accentuated from compression caused by the dilated prostatic urethra on the segment below the valve [1].
Material and Methods The series consisted of 65 boys with a mean age of 3.4 years at the first voiding study, the occasion at which the diagnosis was made. The median age was just above 1 year. Eligible for study were only cases with the characteristic finding of an obstructive sail just below the verumontanum (Figs. 1 a and b). Elongation and dilatation of the posterior
* Based upon a paper read at the 10th Meeting of the E. S. P.R. in Birmingham, Great Britain, April 11--13, 1973.
Treatment primary treatment consisted throughout the series of transurethral electroresection of the valve. In 13 cases the operation was repeated one or several times because of persisting obstructive valvular remnants. In 23 cases resection of the bladder neck was performed in the same session or secondarily because of bladder neck obstruction. As therapy consistently aimed at correction of the abnormality proper and not at relieving secondary symptoms, there was no need for nephrostomies or other diverging procedures. Correction of occurring electrolyte disturbances and treatment with antibiotics supplemented the operative measures.
Results of Treatment Two out of the 65 patients died in connection with the primary operation at 4 days and 8 months of age, respectively. All other patients recovered and were in a fair condition, generally in good health at later follow-ups.
Radiologica/ Techniques and Findings Intravenous urography and voiding cysto-urethrography were carried out in all patients regardless of their condition at admission. High doses of contrast medium at urography and prolongation of the examination [2], whenever necessary, were the most important isolated measures used in the efforts to achieve maximal information. The voiding cysto-urethrograms were performed by use of single full size films exposed in the true lateral and frontal
44
O. Ekl6f and H. Ringertz: Urographic Findings in Posterior Urethral Valves projections. In selected cases, oblique views provided further important information. Preoperative urograms revealed impaired kidney function in 31 o/o of the series. In a further 19 % of cases, one kidney was non-functioning. 46 o/o of all patients had a moderate dilatation of the upper urinary tract, 39 o/o a marked one (Table 1). In most cases parallel with the changes of the upper urinary tract, however, in 12% as an isolated occurrence, bladder alterations were noted. Reduced bladder capacity, increased tonus and thickness of the wall, an irregular mucosal membrane pattern, frequently together with trabeculation of the bladder or with diverticula formation, was registered alone or in different combinations (Figs. la, lb, 4a). Unilateral vesico-ureteral reflux was present in 29 % of patients, bilateral in 18~ The reflux was frequently marked (Fig. 5) and associated with significant dilatation of the affected upper urinary tract (Table 1). The radiological follow-up time was 4.3 years in average. Postoperatively the kidney function, as evaluated by excretory urography improved in quite a number of cases. Sometimes the improvement was rather remarkable (Figs. 2a, b and c). Nevertheless, permanently impaired kidney function was demonstrated in other patients (Table 1). Thus 17 o/o of cases had a severe renal demage with one kidney non-functioning. Although decreasing width occasionally could be noted, the upper urinary tract remained more or less dilated in 76 % of cases (Figs. 3 a and b). The vesico-ureteral reflux was hardly affected by treatment. 13 patients had obstructive valvular remnants (Figs. l c and d) and 19 secondary bladder neck obstruction calling for repeated operations.
Discussion
Fig. 1. Typical posterior urethral valve in a 3 day old boy with clinical urosepsis, a and b) Reduced bladder capacity, increased tonus, swollen bladder mucosa and moderate right sided reflux. The bladder neck and the elongated posterior urethra are wide. c and d) 3 months after electroresection of the valve. Minor, slightly obstructive valvular remnants are seen. Normalisation of the bladder's appearance
Table 1. Kidney function and upper urinary tract dilatation Preoperative
Postoperative
Kidney function Impaired One kidney silent
31% 19 %
19 % 17 %
Dilatation Moderate Marked
46 % 39 %
53 % 23 %
Analysis of radiological findings of p r o g n o s t i c significance, f o r the future k i d n e y function, revealed m a r k e d vesico-ureteral reflux to be of crucial i m p o r tance. I n 17 % of patients w i t h this feature c o m p l e t e and p e r m a n e n t abolition of k i d n e y f u n c t i o n was n o t e d o n the m o s t affected side. As a rule there was i m p a i r e d excretory c a p a d t y o n the contralateral side too. Conversely, n o n e o r o n l y m o d e r a t e reflux resulted as an a v e r a g e in less severe functional disturbances; n o single case of c o m p l e t e k i d n e y f u n c t i o n annihilation was e n c o u n t e r e d in these later patients (Fig. 5). O u r o b s e r v a t i o n diverges f r o m the c o n c l u s i o n m a d e b y Williams et al. (8) w h o considered reflux " a less serious c o m p l i c a t i o n " . T h e reflux, h o w e v e r , s t o p p e d s p o n t a n e o u s l y in o n l y 22 o u t of 86 of their refluxing ureters. T h e i r r e m a i n i n g patients w e r e either o p e r a t e d u p o n , h a d persistent reflux or w e r e n e v e r reexamined. J o h n s t o n eta/. (3) f o u n d spontaneous cure a n d persistent reflux i n r o u g h l y o n t h i r d each of their reflux cases. T h e r e m a i n i n g t h i r d of the series was never reassessed o n this point. Persistent dilatation of n o n - r e f l u x i n g ureters, a n o t h e r c o m m o n feature of the present material, is
a
b
c
Fig. 2. Same patient as in Fig. 1. a and b) Intravenous urography at the initial admission. Impaired kidney function with severe, bilateral hydronephrosis and hydroureters, c) IVP 3 months after primary treatment reveals remarkable improvement
a
b
Fig. 3. Four year old boy with posterior urethral valve, a) IVP at initial admission revealing impaired kidney function and bilateral upper urinary tract dilatation, b) IVP 3 years later shows improved kidney function but remaining, although decreased upper urinary tract dilatation
a
b
Fig. 4. Same patient as in Fig. 3. a) Voiding cysto-urethrography at primary admission revealing the valve and the markedly altered bladder, b) Voiding cysto-urethrography 3 years later with improvement of the bladder changes. The bladder, however~
46
O. Ek18f and H. Ringertz: Urographic Findings in Posterior Urethral Valves
PREOPERATIVE DEGREE OF VESICO-URETERAL REFLUX ~
MARKED
~
MODERATE
rq~
14
19
36
::::::::::::::::
:1:1:5i
I~ J0 N Fig. 5. Postoperative degree of kidney damage
probably of significance in the case of recurrent urinary infections. Williams [9] believes this finding to be caused by a failure of peristaltic function, the presence of obstruction at the uretero-vesical junction or possibly by the permanently altered, frequently massively hypertrophied bladder (Figs. 4a and b). In our experience a combination of the two last mentioned factors appears the likely explanation in most cases. In the case of a posterior urethral valve, the bladder and the upper urinary tract, as a rule, are both affected. This combined form of lesion was noted in 55 of our patients. In 8 cases, however, only the lower urinary tract was influenced while 2 patients revealed isolated upper urinary tract commitment. T h u s neither a completely normal upper Urinary tract nor an ordinary bladder appearance excludes a posterior urethral valve [7]. The occurrence and significance of radiologically diagnosed bladder neck obstruction in patients with posterior urethral valves is doubted by many authors [3, 9]. In our material, however, the radiological diagnosis of secondary bladder neck obstruction, in some cases supported by findings at pressureflow studies, invariably resulted in electroresection just as valvular remnants became fulgurated.
Although complete anatomical and functionai restoration of the kidneys and the bladder was achieved only exceptionally, the presented results of therapy are nontheless unique. So far neither dialysis nor kidney transplantation have been necessary in our series. They may, however, become considered later in some of the cases with severe bilateral kidney damage. T h e prerequisite of a successful treatment is of course an adequate radiological exploration consisting of an excretory urography and voiding cysto-urethrography as well. Subsequently to primary operations a sufficiently long radiological follow-up period is recommended as further surgical measures may appear indicated.
1. Ekl/Sf, O., Ringertz, H.: The development of obstruction in true valves and in functionally valvlike abnormalities of the male posterior urethra. Paper read at the 11th Meeting of the European Society of Pediatric Radiology, ttelsinki, Finland, May 29-June 1, 1974 2. Ekl/Sf, O. : Radiological aspects of benign renal mass
3. 4. 5. 6. 7. 8. 9.
lesions in infancy and early childhood. Pediat. Radiol. 1, 53 (I973) Johnston, J. H., Kulatilake, A. E. : The sequelae of posterior urethral valves. Brit. J. Urol. 43, 743 (1971) Kjellberg, S. R., Ericsson, N. O., Rudhe, U. : The lower urinary tract in childhood. Uppsala: Almqvist & Wiksell 1957 Kumar, M., Shuroff, N., Bhat, H. S. : Posterior urethral valve. Problems in management of the upper urinary tract. Brit. J. Urol. 44, 486 (1972) Morag, B., Herz, M. : Congenital valves os the posterior urethra: Radiological aspects. Clin. Radiol. 28, 445 (1974) Tsingglou, S., Dickson, J. A. S. : Lower urinary obstruction in infancy. A review of lesions and symptoms in 165 cases. Arch. Dis. Child. 47, 215 (1972) Williams, D. I., Whitaker, R. H., Barrat, T. M., Keeton, J. E. : Urethral valves. Brit. J. Urol. 48, 200 (1973) Williams, D. I. : In: Urology in childhood. Encyclopedia of urology, XV. Suppl. Berlin, Heidelberg, New York: Springer 1974 O. EklSf, M. D. Dept. of Pediatric Radiology Karolinska Sjukhuset S-104 01 Stockholm 60 Sweden
Pre- and Postoperative Urographic Findings in Posterior Urethral Valves':' O. Ekl/Sf a n d H. R i n g e r t z Department of Pediatric Radiology, Karolinska Sjukhuset, Stockholm, Sweden Date of final acceptance: April 4, 1975
Abstract. In a series of 65 male infants and children, all with the diagnosis of posterior urethral valves, pre- and postoperative urographic findings were reviewed. In addition changes occurring in the bladder, and the implication of vesico-ureteral reflux were assessed. -- Preoperatively diagnosed impairment of kidney function and concommitant dilatation of the upper urinary tract, with some exceptions. remained fairly unchanged at postoperative examinations In the case of marked vesico-ureteraI reflux, permanent kidney function annihilation was significantly commoner
than with slight or no reflux. -- Although, as a rule, both the upper urinary tract and the bladder were affected, there were cases of posterior urethral valves with a normal appearing bladder. As the intravenous urography do riot exclude the urethral abnormality, voiding cysto-urethrography has to be included in the primary radiological exploration of all cases with urological problems. Key words: Intravenous urography, non-functioning kidney, upper urinary tract dilatation, voiding cysto-urethrography, posterior urethral valve, bladder outflow obstruction.
A c c o r d i n g to r e c e n t s t a t e m e n t s i n the literature, p o s t e r i o r u r e t h r a l valves m u s t b e r e g a r d e d as the m o s t serious l o w e r u r i n a r y tract a b n o r m a l i t y of t h e male i n f a n t a n d child. D e p e n d i n g u p o n age at p r e s e n t a t i o n p r i m a r y m o r t a l i t y rates of 17--50 % are r e p o r t e d [3, 5, 7, 8]. I n the past r a d i o l o g i s t s seem p r i n c i p a l l y to h a v e f o c u s e d t h e i r i n t e r e s t o n different aspects of the d i a g n o s t i c a l l y critical v o i d i n g c y s t o - u r e t h r o g r a p h y , m o r e or less l e a v i n g the f i n d i n g s disclosed at i n t r a v e n o u s u r o g r a p h y o u t of discussion. E v e n so the d e g r e e of i m p a i r e d k i d n e y f u n c t i o n to a great e x t e n t d e t e r m i n e s the l o n g t e r m p r o g n o s i s of the c o n d i t i o n . I t therefore a p p e a r e d i m p o r t a n t to analyze the i n f l u e n c e of t r e a t m e n t o n the e v o l u t i o n of k i d n e y f u n c t i o n as e v a l u a t e d o n the base of available i n t r a venous urograms. In addition, changes occurring i n b l a d d e r a p p e a r a n c e a n d the i m p l i c a t i o n of vesicou r e t e r a l reflux will be briefly discussed.
urethra were other constant features. At the level of the valve urethral lumen was restricted to a narrow dorsal slit. The size of this slit is one of the main factors determining urethral flow-. In advanced cases the obstructive mechanism frequently becomes accentuated from compression caused by the dilated prostatic urethra on the segment below the valve [1].
Material and Methods The series consisted of 65 boys with a mean age of 3.4 years at the first voiding study, the occasion at which the diagnosis was made. The median age was just above 1 year. Eligible for study were only cases with the characteristic finding of an obstructive sail just below the verumontanum (Figs. 1 a and b). Elongation and dilatation of the posterior
* Based upon a paper read at the 10th Meeting of the E. S. P.R. in Birmingham, Great Britain, April 11--13, 1973.
Treatment primary treatment consisted throughout the series of transurethral electroresection of the valve. In 13 cases the operation was repeated one or several times because of persisting obstructive valvular remnants. In 23 cases resection of the bladder neck was performed in the same session or secondarily because of bladder neck obstruction. As therapy consistently aimed at correction of the abnormality proper and not at relieving secondary symptoms, there was no need for nephrostomies or other diverging procedures. Correction of occurring electrolyte disturbances and treatment with antibiotics supplemented the operative measures.
Results of Treatment Two out of the 65 patients died in connection with the primary operation at 4 days and 8 months of age, respectively. All other patients recovered and were in a fair condition, generally in good health at later follow-ups.
Radiologica/ Techniques and Findings Intravenous urography and voiding cysto-urethrography were carried out in all patients regardless of their condition at admission. High doses of contrast medium at urography and prolongation of the examination [2], whenever necessary, were the most important isolated measures used in the efforts to achieve maximal information. The voiding cysto-urethrograms were performed by use of single full size films exposed in the true lateral and frontal
44
O. Ekl6f and H. Ringertz: Urographic Findings in Posterior Urethral Valves projections. In selected cases, oblique views provided further important information. Preoperative urograms revealed impaired kidney function in 31 o/o of the series. In a further 19 % of cases, one kidney was non-functioning. 46 o/o of all patients had a moderate dilatation of the upper urinary tract, 39 o/o a marked one (Table 1). In most cases parallel with the changes of the upper urinary tract, however, in 12% as an isolated occurrence, bladder alterations were noted. Reduced bladder capacity, increased tonus and thickness of the wall, an irregular mucosal membrane pattern, frequently together with trabeculation of the bladder or with diverticula formation, was registered alone or in different combinations (Figs. la, lb, 4a). Unilateral vesico-ureteral reflux was present in 29 % of patients, bilateral in 18~ The reflux was frequently marked (Fig. 5) and associated with significant dilatation of the affected upper urinary tract (Table 1). The radiological follow-up time was 4.3 years in average. Postoperatively the kidney function, as evaluated by excretory urography improved in quite a number of cases. Sometimes the improvement was rather remarkable (Figs. 2a, b and c). Nevertheless, permanently impaired kidney function was demonstrated in other patients (Table 1). Thus 17 o/o of cases had a severe renal demage with one kidney non-functioning. Although decreasing width occasionally could be noted, the upper urinary tract remained more or less dilated in 76 % of cases (Figs. 3 a and b). The vesico-ureteral reflux was hardly affected by treatment. 13 patients had obstructive valvular remnants (Figs. l c and d) and 19 secondary bladder neck obstruction calling for repeated operations.
Discussion
Fig. 1. Typical posterior urethral valve in a 3 day old boy with clinical urosepsis, a and b) Reduced bladder capacity, increased tonus, swollen bladder mucosa and moderate right sided reflux. The bladder neck and the elongated posterior urethra are wide. c and d) 3 months after electroresection of the valve. Minor, slightly obstructive valvular remnants are seen. Normalisation of the bladder's appearance
Table 1. Kidney function and upper urinary tract dilatation Preoperative
Postoperative
Kidney function Impaired One kidney silent
31% 19 %
19 % 17 %
Dilatation Moderate Marked
46 % 39 %
53 % 23 %
Analysis of radiological findings of p r o g n o s t i c significance, f o r the future k i d n e y function, revealed m a r k e d vesico-ureteral reflux to be of crucial i m p o r tance. I n 17 % of patients w i t h this feature c o m p l e t e and p e r m a n e n t abolition of k i d n e y f u n c t i o n was n o t e d o n the m o s t affected side. As a rule there was i m p a i r e d excretory c a p a d t y o n the contralateral side too. Conversely, n o n e o r o n l y m o d e r a t e reflux resulted as an a v e r a g e in less severe functional disturbances; n o single case of c o m p l e t e k i d n e y f u n c t i o n annihilation was e n c o u n t e r e d in these later patients (Fig. 5). O u r o b s e r v a t i o n diverges f r o m the c o n c l u s i o n m a d e b y Williams et al. (8) w h o considered reflux " a less serious c o m p l i c a t i o n " . T h e reflux, h o w e v e r , s t o p p e d s p o n t a n e o u s l y in o n l y 22 o u t of 86 of their refluxing ureters. T h e i r r e m a i n i n g patients w e r e either o p e r a t e d u p o n , h a d persistent reflux or w e r e n e v e r reexamined. J o h n s t o n eta/. (3) f o u n d spontaneous cure a n d persistent reflux i n r o u g h l y o n t h i r d each of their reflux cases. T h e r e m a i n i n g t h i r d of the series was never reassessed o n this point. Persistent dilatation of n o n - r e f l u x i n g ureters, a n o t h e r c o m m o n feature of the present material, is
a
b
c
Fig. 2. Same patient as in Fig. 1. a and b) Intravenous urography at the initial admission. Impaired kidney function with severe, bilateral hydronephrosis and hydroureters, c) IVP 3 months after primary treatment reveals remarkable improvement
a
b
Fig. 3. Four year old boy with posterior urethral valve, a) IVP at initial admission revealing impaired kidney function and bilateral upper urinary tract dilatation, b) IVP 3 years later shows improved kidney function but remaining, although decreased upper urinary tract dilatation
a
b
Fig. 4. Same patient as in Fig. 3. a) Voiding cysto-urethrography at primary admission revealing the valve and the markedly altered bladder, b) Voiding cysto-urethrography 3 years later with improvement of the bladder changes. The bladder, however~
46
O. Ek18f and H. Ringertz: Urographic Findings in Posterior Urethral Valves
PREOPERATIVE DEGREE OF VESICO-URETERAL REFLUX ~
MARKED
~
MODERATE
rq~
14
19
36
::::::::::::::::
:1:1:5i
I~ J0 N Fig. 5. Postoperative degree of kidney damage
probably of significance in the case of recurrent urinary infections. Williams [9] believes this finding to be caused by a failure of peristaltic function, the presence of obstruction at the uretero-vesical junction or possibly by the permanently altered, frequently massively hypertrophied bladder (Figs. 4a and b). In our experience a combination of the two last mentioned factors appears the likely explanation in most cases. In the case of a posterior urethral valve, the bladder and the upper urinary tract, as a rule, are both affected. This combined form of lesion was noted in 55 of our patients. In 8 cases, however, only the lower urinary tract was influenced while 2 patients revealed isolated upper urinary tract commitment. T h u s neither a completely normal upper Urinary tract nor an ordinary bladder appearance excludes a posterior urethral valve [7]. The occurrence and significance of radiologically diagnosed bladder neck obstruction in patients with posterior urethral valves is doubted by many authors [3, 9]. In our material, however, the radiological diagnosis of secondary bladder neck obstruction, in some cases supported by findings at pressureflow studies, invariably resulted in electroresection just as valvular remnants became fulgurated.
Although complete anatomical and functionai restoration of the kidneys and the bladder was achieved only exceptionally, the presented results of therapy are nontheless unique. So far neither dialysis nor kidney transplantation have been necessary in our series. They may, however, become considered later in some of the cases with severe bilateral kidney damage. T h e prerequisite of a successful treatment is of course an adequate radiological exploration consisting of an excretory urography and voiding cysto-urethrography as well. Subsequently to primary operations a sufficiently long radiological follow-up period is recommended as further surgical measures may appear indicated.
1. Ekl/Sf, O., Ringertz, H.: The development of obstruction in true valves and in functionally valvlike abnormalities of the male posterior urethra. Paper read at the 11th Meeting of the European Society of Pediatric Radiology, ttelsinki, Finland, May 29-June 1, 1974 2. Ekl/Sf, O. : Radiological aspects of benign renal mass
3. 4. 5. 6. 7. 8. 9.
lesions in infancy and early childhood. Pediat. Radiol. 1, 53 (I973) Johnston, J. H., Kulatilake, A. E. : The sequelae of posterior urethral valves. Brit. J. Urol. 43, 743 (1971) Kjellberg, S. R., Ericsson, N. O., Rudhe, U. : The lower urinary tract in childhood. Uppsala: Almqvist & Wiksell 1957 Kumar, M., Shuroff, N., Bhat, H. S. : Posterior urethral valve. Problems in management of the upper urinary tract. Brit. J. Urol. 44, 486 (1972) Morag, B., Herz, M. : Congenital valves os the posterior urethra: Radiological aspects. Clin. Radiol. 28, 445 (1974) Tsingglou, S., Dickson, J. A. S. : Lower urinary obstruction in infancy. A review of lesions and symptoms in 165 cases. Arch. Dis. Child. 47, 215 (1972) Williams, D. I., Whitaker, R. H., Barrat, T. M., Keeton, J. E. : Urethral valves. Brit. J. Urol. 48, 200 (1973) Williams, D. I. : In: Urology in childhood. Encyclopedia of urology, XV. Suppl. Berlin, Heidelberg, New York: Springer 1974 O. EklSf, M. D. Dept. of Pediatric Radiology Karolinska Sjukhuset S-104 01 Stockholm 60 Sweden
