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Posterior Urethral Valves
THE
,J OUR!
OF UROLOGY
Vol. 112, August Printed in U.S.A.
Copyright© 1974 by The Williams & Wilkins Co.
POSTERIOR URETHRAL VALVES VICTOR E. AGUSTA AND STUARTS. HOWARDS From the Department of Urology, University of Virginia School of Medicine, Charlottesville, Virginia
Although the diagnosis and treatment of posterior urethral valves have been described frequently 1-• little has been reported on the long-term prognosis and the quality of life in patients with this anomaly.'· 6 Therefore, we reviewed our experience with posterior urethral valves, emphasizing the long-range results. The relationship among prognosis and age at presentation, delay in diagnosis, initial treatment and renal function at the time of diagnosis is explored. METHODS AND MATERIALS
All patients seen at our hospital between 1945 and 1972 with the definitive diagnosis of posterior urethral valves were studied. There were 31 patients with a mean followup of 8 years and a range of 1 to 27 years (10 seen before 1960 and 21 seen after 1960). Patients were usually evaluated during periodic clinic visits, although a few were contacted personally or through the family physician. All charts were reviewed and all x-rays from 1960 until the present were re-evaluated. · RESULTS
Presentation. The ages of patients at presentation are noted in figure 1. All 8 patients less than 1 year old were newborns. Some patients who were first seen at this hospital between the ages of 1 and 5 actually had been treated at other institutions for urethral valves by temporary diversion. These patients were then referred to us for complications of the disease process. Excluding the neonatal period the number of patients presenting during each 5-year period is similar until age 15. Duration of symptoms ranged from less than 1 day to more than 10 years. However, 17 of 31 patients had symptoms for less than 1 year and only 5 patients had symptoms for more than 5 years. The symptoms recorded were secondary to obstruction and infection. Three patients had hemaAccepted for publication February 8, 1974. Supported by the Mr. and Mrs.· Howell E. Jackson Urology Research Fund. 1 Williams, D. I. and Eckstein, H. B.: Obstructive valves in the posterior urethra. J. Urol. 93: 236 196.S. 2 Williams, D. I., Whitaker, R. H., B;rratt, T'. M. and Keeton, J. E.: Urethral valves. Brit. J. Urol. 45: 200 ' , 1973. 3 Hendren, W. H.: Recent advances in the management of lower urinary obstruction in the newborn. Prog. Pediat. Surg., 2: 11.S, 1971. 'Hendren, W. H.: Posterior urethral valves in boys. A broad clinical spectrum. J. Urol., 106: 298, 1971. 5 Waldbaum, R. S. and Marshall, V. F.: Posterior urethral valves: evaluation and surgical management. J. Urol., 103: 801, 1970. 6 Waterhouse, K.: Encyclopedia of Urology. New York: Springer-Verlag, band VII/1, p. 259, 1968.
turia only but no patient had nocturnal enuresis as the sole complaint. Several newborns had a palpable bladder. Urologic evaluation. Excretory urography (IVP): Only 9 of 29 initial IVPs were normal, which is defined as a study demonstrating normal calices and ureters without evidence of hydronephrosis (fig. 2). Eighteen patients (approximately 60 per cent) had grade 3 to 4 hydronephrosis or non-function of 1 or both kidneys. Of the 8 newborns 7 had an abnormal IVP with grade 3 to 4 hydronephrosis. An IVP was not made in 1 case of a neonate seen early in the series. Excluding newborns 3 of 8 IVPs were abnormal in patients with symptoms less than 1 year in duration, 5 of 8 IVPs were abnormal in patients with symptoms from 1 to 5 years in duration and 4 of 5 IVPs were abnormal in patients with symptoms more than 5 years in duration. Creatinine clearance: The patients were arbitrarily divided into 2 groups (fig. 3). In group 1 the estimated creatinine clearance was more than 25 per cent of normal for the patient's age and in group 2 it was less than 25 per cent of normal. Of the 30 patients in whom renal function was estimated 10 (33 per cent) had a creatinine clearance less than 25 per cent of normal. Six of the 10 patients were newborns. Cystoscopy: All patients who underwent cystoscopy had a trabeculated bladder. Cystogram: Of 29 patients who underwent cystography 14 had ureterovesical reflux on 1 or both sides. Roughly, a third of the ureters were normal, a third were obstructed and a third had reflux (table 1). If reflux and delayed drainage were noted in the same ureter the ureter was recorded only in the reflux category. Initial treatment. Primary therapy is difficult to evaluate since this is a retrospective review for a 25-year period and often initial treatment was performed at another institution (table 2). In 14 patients the valves and/or bladder neck was resected only. Of these 14 patients 4 required a repeat resection of the valves. Nine of these 14 patients had a normal IVP while 5 had hydronephrosis. Although 3 of the 5 patients with hydronephrosis required no further therapy 2 eventually required ureteral neocystostomy for reflux. Patients who underwent resection of the valves and/or bladder neck combined with temporary cystostomy or nephrostomy are grouped according to their diversionary procedure. Interestingly, only 1 of 4 patients who underwent open destruction of the valves is doing well presently. Survival and quality of survival. The over-all mortality rate in this series is 13 per cent (4 of 31 patients). Two patients died of sepsis, 1 committed
280
281
POSTERIOR URETHRAL VALVES
AGE OF PRESENTATION
6
4 2 6 to 10 y.o.
NE ONA TES l 10 5 y.o.
11 to 15
>15 y.o.
y.o.
FIG. DURATION Of SYMPTOMS versus !VP ON PR~SENTATION
~
No funclion of l or both kidneys and grade 4 hydronephrosis of other
fil]
Grode 3 lo 4 hydronephrosis
B
Grads 1 lo 2 hydronophrosi~
Ill
NormolIVP
* l peili<>nt no (Vf>
FrG. 2 DURATION Of SYMPTOMS versus REN.Al FUNCTION AT PRESENTATION Group I[
Group I •
<1 YEAR
* Renal
~ Creatinina clearance
Creatinine clearance greater than 25% of normal
~ less than 25% of normal
6 to 10 YEARS
1 lo 5 YEARS
>10 YEARS
fundion not done in l patient
FIG. :1
suicide at age 21 and 1 died of hypertension and uremia at age 46 (this patient had a normal IVP several years before death and, therefore, the death may be unrelated to the posterior urethral valves). Another 13 per cent have a guarded prognosis. One patient is awaiting renal transplantation with a completely rehabilitated bladder. Another patient has a creatinine clearance of only 7 cc per minute. Two other patients listed with a guarded prognosis are 21 and 27 years old and have had a stable serum creatinine of 3 for the last 10 years. They may do well despite renal impairment and permanent cutaneous ureterostomies. More than twothirds of the patients (22 of 31) are voiding well
with a creatinine clearance of more than 25 per cent of normal. Of the 21 patients with an abnormal IVP on admission 1:3 are fully reconstructed and voiding normally. Continence: We evaluated 20 patients for continence (table :3). Patients were considered continent if they were dry day and night. One patient 'Nho was continent but later underwent diversion to preserve renal function is considered in the continent group. Eleven patients were not evaluated because they were less than :3 years old and underwent diversion before evaluation or were lost to followup. No patient who underwent resection of the valves only was incontinent. Several patients
282
AGUSTA AND HOWARDS TABLE
1. Evaluation of ureterouesical junction Ureters No. (% )
Normal Reflux Obstruction >lot evaluated Totals TABLE
(29) (32) (32) (7) 4 - ~ - -62 (100)
18
20 20
2. Initial treatment of patients with posterior urethral values No. Cases
Resection of valves and bladder nec k Open destruct ion of va lves Urethral or su prapubic catheter Supra\·esical di\·ersion Total
14 4 6
7*
31
* Kephrostomy was done in 6 cases . TABLE
3. Evaluation of continence and surgical procedure No. Cases
Continent (18 pts.): Transurethral resection of valve only Transu rethral resection of rnh·e and bladder neck Open dest ruction of valve and Y -Y plastv· Incontinent (2 pts.): Transurethra l resection of va lve and bladder neck Open antegrade destruction of valves
12 5
initially had stress incontinence or nocturnal enuresis but this invariably disappeared with time. Fertility: Nine patients more than 21 years old are married, Fertility data could not be obtained in 3 cases but, of the remaining 6, 4 or 67 per cent have fathered children. One of the 2 men who did not father a child committed suicide when he was 21 years old. Growth a nd development: We obtained adequate data concerning growth and development in 16 cases. We measured the growth percentile at presentation versus that at followup. Only 1 patient shifted downward in the percentile growth from the fiftieth to less than the third percentile and this patient is awaiting a transplantation. Two patients remained at less than the third percentile in growth. One of these patients had end-stage renal disease when he was 7 years old, with a creatinine clearance of 7 cc per minute. He had been moribund when he was 2 years old , with severe uremia, renal tubular acidosis and renal rickets. The other patient in the third percentile level is 3 ½ years old and presented as a newborn in the third percentile. He has remained at this level despite reconstruction and a creatinin e clearance of 100 cc per minute. Quality of survival versus presentation. To simplify our discussion the patients were divided into 2 groups: group I-patients who underwent recon struction and are voiding per urethra and group
2-patients who died of the primary disease , have a guarded prognosis, are await ing transplantation or have permanent diversion at the time of followup (fig. 4). If 1 or both kidneys are non-functioning or exhibit marked hydronephrosis the possibility for total reconstruction is decreased. However, all patients with mild hydronephrosis or normal kidneys underwent reconstruction. The initial renal function was compared to the eventual reconstructability (fig. 5). The newborns were separated from the other patients in this study since we found that newborn patients are harder to evaluate and usually have a more severe form of the disease. All newborns had grade 4 hydronephrosis or non-function of 1 or both kidneys and only 50 per cent could undergo reconstruction. Two of the 4 patients with renal function less than 25 per cent of normal on admission died of sepsis. These were preventable deaths. If the renal function was more than 25 per cent of normal the outlook was better. Surprisingly, in older patients, if the renal function was adequate they could eventually undergo reconstruction regardless of the radiographic appearance of the kidneys. Quality of survival versus initial treatment. Best results were obtained following resection of the valves and/or bladder neck but these patients were pre-selected because of minimal renal damaue (table 4). On the other hand, 3 of these 13 patients had significant bilateral hydronephrosis (grade 3 to 4). The patients treated by supraves ic al diversion initially did poorly because they usually had the most severe renal damage . T he groups treated by open destruction of valves and initial bladder drainage will be considered together, since a suprapubic catheter was usually left in place in the former group. The poor results in this group demonstrate that bladder drainaue is inadequate in patients with complete obstruction of 1 or both ureters at the ureterovesical junction. Therefore, a greater number of supravesica l diversions should have been performed. DISCUSSION
L__i_~tle CO.!:felation wa~ noted between the length of symptoms ana the seventy of renal impairment. Of the 7 patients with impaired renal function and symptoms for less than a year 4 were newborns. he loss of functioning renal tissue se o be better corre a e w e severity of th.e_nb_s.t.r.uction than with the dela}c in_tliagnosis. - Contrary to other authors• we did not have any] patients with nocturnal enuresis as the only symp- · tom. Also, every patient who underwent cystos copy IIBd-hl r trabeculation. We aorneueve 1Fiatthe diagnosis of ostenor uret ra va ves can lie made wit out evidence of obstructive c_hgnges p'roximal to the les10n. 7 '°Our study reveals that patients have a surpris1 Forsythe, W. I. and McFadden, G.D. F.: Congenital postenor urethral valves: a study of 35 cases. Brit. J. Urol., 31: 63, 1959.
283
POSTERIOR URETHRAL VALVES
PIP ON PRESEMTAT!ON v,mus QUAUYY Of SUR\fiVAl
G,oup ]I
Group I •
iii
flotienh r®constn$d0d
NORMAL
GIHHliE 1-2
NP
Diveried o, dead ol disease
G~AD~ 3-4 1 OR 110TH KIDNEYS HYDRONEPHROS15 NON-FUNCTION AND
HYDROMEPHROSIS
OTHrn GRADE 4 HYDRONEPHROSIS 2 potierib unable
Hi
<1:1vahrnh1 -no !VP - ] deiad of sepsis
i no follow-up
FIG. 4
RrnAL Fl!NCllON ON PRESENTATION versus QUALITY OF SURVI\/Al
Group I •
<25% Of NORMAL Cereal
Group Il
~
R0construded
<25% Of NORMAL Cmwt
>25% Of
NORMAl Ccroat
>25% OF NORMAL C cr~cit
All OTHER PATIENTS
N[WBORNS ,;,
Divert'3d or dead of diseass
1 pati(>n/ no renal function performed
Frc. 5
TABLE 4.
Initial treatment L'ersus qua/itv of suruiL·al Group 1-"
Open destruct ion of vah-e:-; Suprapuhic or urethral catheter Supra\·esical di\·ersion Transurethral resection of \'akec-s
Group
:2+
:; ~
:,
1:1:j:
and/or bladder neck
* Patients who underwent reconstruct ion.
t Patients
who undenvent diversion or died of the disease.
+1 patient had no followup.
ingly good prognosis despite radiographic evidence of severe upper tract decompensation. Figures 4 and 5 suggest that the chemical determination of renal function is a better prognostic index than the pyelogram. Furthermore, once the lower tracts are
rehabilitated the patient can have a normal life. Only 2 patients were persistently incontinent after treatment. '.\;o patient who underwent resection of the valves only or fulguration was incontinent. previously stated some of these patients did hm e stress incontinence or nocturnal enuresis but this gradually disappeared as the patient matured and their bladder returned to normal. VVe agree v,ith Whitaker and associates who state that an opera tion on the bladder neck increases the of incontinence and that bladder neck changes will resolve after the valves are resected. 8 Poor resultc', are usually secondary to unresected valves rather 8 Whitaker, R. H., Keeton, J. E. and Posterior urethral valves: a studv of urinan· operation. ,J. Urol.. 108: 16,, 197:2. ·
D. I. after
284
AGUSTA AND HOWARDS
than b ladder neck obstruction. Indeed, 4 of our 14 patients required a second resection of the valves. This latter point is to be emphasized since our followup suggests that t h ese patients are capable of satisfactory sexual function and reproduction. Resection of t he bladder neck can result in retrograde ejaculation and infertility. Our series is too small and too varied to comment on the most appropriat e form of t reatment . However, no matter what form is chosen these patients need early and careful followup. Since a third of the ureters are obstructed at t he ureterovesical junction complete destruction of t he valves or bladder diversion will not immediately reverse the obstruction. A higher form of diversion is indicated if the patient becomes septic, renal function shows n o improvement or the upper tracts fail to improve. We prefer nephrostomy tubes which can be removed without another operative procedure since t h e need for diversion is usually temporary. We use supra pubic cystostomy for bladder diversion if the diversion is to be in place more than several days . A severe urethral stricture developed in 1 of our patients treated with a urethral catheter
I
r
for 2 mont hs , requiring further operations. We are not concerned a bout the potential infection related to a suprapubic or nephrostomy tube as long as t he upper tracts are adequately drained. In conclusion, althou gh posterior urethral valves can be a devastating disease, with time, patience and individually structured treatment a full, normal productive life is attainable for the majority of patients. SUMMARY
The followup of 31 patients treated between 1945 and 1972 for posterior urethral valves is reviewed . Emphasis is placed on t he long-term results. P-I:Qgn_2.s is correlates well with renal function at the time_ of presentation. Initial radiologic appearance is a less reliable p.r_o.gno.s.tic index All patients, but especially those with ureterovesical obstruction, should be carefully followed and supravesical diversion should be used when indicat ed. The majority of the patients were totally rehabilitated. N infil'y_per...cent--a-re-ent.i-re-l-j<-G0Btirnmt Eighty.....p_ru: cen t of J;!l!tients mare than 21 years aid and married have-fathered-ehildre.~ nly 1 patient experienced a reduced growth rate after diagnosis.
,J OUR!
OF UROLOGY
Vol. 112, August Printed in U.S.A.
Copyright© 1974 by The Williams & Wilkins Co.
POSTERIOR URETHRAL VALVES VICTOR E. AGUSTA AND STUARTS. HOWARDS From the Department of Urology, University of Virginia School of Medicine, Charlottesville, Virginia
Although the diagnosis and treatment of posterior urethral valves have been described frequently 1-• little has been reported on the long-term prognosis and the quality of life in patients with this anomaly.'· 6 Therefore, we reviewed our experience with posterior urethral valves, emphasizing the long-range results. The relationship among prognosis and age at presentation, delay in diagnosis, initial treatment and renal function at the time of diagnosis is explored. METHODS AND MATERIALS
All patients seen at our hospital between 1945 and 1972 with the definitive diagnosis of posterior urethral valves were studied. There were 31 patients with a mean followup of 8 years and a range of 1 to 27 years (10 seen before 1960 and 21 seen after 1960). Patients were usually evaluated during periodic clinic visits, although a few were contacted personally or through the family physician. All charts were reviewed and all x-rays from 1960 until the present were re-evaluated. · RESULTS
Presentation. The ages of patients at presentation are noted in figure 1. All 8 patients less than 1 year old were newborns. Some patients who were first seen at this hospital between the ages of 1 and 5 actually had been treated at other institutions for urethral valves by temporary diversion. These patients were then referred to us for complications of the disease process. Excluding the neonatal period the number of patients presenting during each 5-year period is similar until age 15. Duration of symptoms ranged from less than 1 day to more than 10 years. However, 17 of 31 patients had symptoms for less than 1 year and only 5 patients had symptoms for more than 5 years. The symptoms recorded were secondary to obstruction and infection. Three patients had hemaAccepted for publication February 8, 1974. Supported by the Mr. and Mrs.· Howell E. Jackson Urology Research Fund. 1 Williams, D. I. and Eckstein, H. B.: Obstructive valves in the posterior urethra. J. Urol. 93: 236 196.S. 2 Williams, D. I., Whitaker, R. H., B;rratt, T'. M. and Keeton, J. E.: Urethral valves. Brit. J. Urol. 45: 200 ' , 1973. 3 Hendren, W. H.: Recent advances in the management of lower urinary obstruction in the newborn. Prog. Pediat. Surg., 2: 11.S, 1971. 'Hendren, W. H.: Posterior urethral valves in boys. A broad clinical spectrum. J. Urol., 106: 298, 1971. 5 Waldbaum, R. S. and Marshall, V. F.: Posterior urethral valves: evaluation and surgical management. J. Urol., 103: 801, 1970. 6 Waterhouse, K.: Encyclopedia of Urology. New York: Springer-Verlag, band VII/1, p. 259, 1968.
turia only but no patient had nocturnal enuresis as the sole complaint. Several newborns had a palpable bladder. Urologic evaluation. Excretory urography (IVP): Only 9 of 29 initial IVPs were normal, which is defined as a study demonstrating normal calices and ureters without evidence of hydronephrosis (fig. 2). Eighteen patients (approximately 60 per cent) had grade 3 to 4 hydronephrosis or non-function of 1 or both kidneys. Of the 8 newborns 7 had an abnormal IVP with grade 3 to 4 hydronephrosis. An IVP was not made in 1 case of a neonate seen early in the series. Excluding newborns 3 of 8 IVPs were abnormal in patients with symptoms less than 1 year in duration, 5 of 8 IVPs were abnormal in patients with symptoms from 1 to 5 years in duration and 4 of 5 IVPs were abnormal in patients with symptoms more than 5 years in duration. Creatinine clearance: The patients were arbitrarily divided into 2 groups (fig. 3). In group 1 the estimated creatinine clearance was more than 25 per cent of normal for the patient's age and in group 2 it was less than 25 per cent of normal. Of the 30 patients in whom renal function was estimated 10 (33 per cent) had a creatinine clearance less than 25 per cent of normal. Six of the 10 patients were newborns. Cystoscopy: All patients who underwent cystoscopy had a trabeculated bladder. Cystogram: Of 29 patients who underwent cystography 14 had ureterovesical reflux on 1 or both sides. Roughly, a third of the ureters were normal, a third were obstructed and a third had reflux (table 1). If reflux and delayed drainage were noted in the same ureter the ureter was recorded only in the reflux category. Initial treatment. Primary therapy is difficult to evaluate since this is a retrospective review for a 25-year period and often initial treatment was performed at another institution (table 2). In 14 patients the valves and/or bladder neck was resected only. Of these 14 patients 4 required a repeat resection of the valves. Nine of these 14 patients had a normal IVP while 5 had hydronephrosis. Although 3 of the 5 patients with hydronephrosis required no further therapy 2 eventually required ureteral neocystostomy for reflux. Patients who underwent resection of the valves and/or bladder neck combined with temporary cystostomy or nephrostomy are grouped according to their diversionary procedure. Interestingly, only 1 of 4 patients who underwent open destruction of the valves is doing well presently. Survival and quality of survival. The over-all mortality rate in this series is 13 per cent (4 of 31 patients). Two patients died of sepsis, 1 committed
280
281
POSTERIOR URETHRAL VALVES
AGE OF PRESENTATION
6
4 2 6 to 10 y.o.
NE ONA TES l 10 5 y.o.
11 to 15
>15 y.o.
y.o.
FIG. DURATION Of SYMPTOMS versus !VP ON PR~SENTATION
~
No funclion of l or both kidneys and grade 4 hydronephrosis of other
fil]
Grode 3 lo 4 hydronephrosis
B
Grads 1 lo 2 hydronophrosi~
Ill
NormolIVP
* l peili<>nt no (Vf>
FrG. 2 DURATION Of SYMPTOMS versus REN.Al FUNCTION AT PRESENTATION Group I[
Group I •
<1 YEAR
* Renal
~ Creatinina clearance
Creatinine clearance greater than 25% of normal
~ less than 25% of normal
6 to 10 YEARS
1 lo 5 YEARS
>10 YEARS
fundion not done in l patient
FIG. :1
suicide at age 21 and 1 died of hypertension and uremia at age 46 (this patient had a normal IVP several years before death and, therefore, the death may be unrelated to the posterior urethral valves). Another 13 per cent have a guarded prognosis. One patient is awaiting renal transplantation with a completely rehabilitated bladder. Another patient has a creatinine clearance of only 7 cc per minute. Two other patients listed with a guarded prognosis are 21 and 27 years old and have had a stable serum creatinine of 3 for the last 10 years. They may do well despite renal impairment and permanent cutaneous ureterostomies. More than twothirds of the patients (22 of 31) are voiding well
with a creatinine clearance of more than 25 per cent of normal. Of the 21 patients with an abnormal IVP on admission 1:3 are fully reconstructed and voiding normally. Continence: We evaluated 20 patients for continence (table :3). Patients were considered continent if they were dry day and night. One patient 'Nho was continent but later underwent diversion to preserve renal function is considered in the continent group. Eleven patients were not evaluated because they were less than :3 years old and underwent diversion before evaluation or were lost to followup. No patient who underwent resection of the valves only was incontinent. Several patients
282
AGUSTA AND HOWARDS TABLE
1. Evaluation of ureterouesical junction Ureters No. (% )
Normal Reflux Obstruction >lot evaluated Totals TABLE
(29) (32) (32) (7) 4 - ~ - -62 (100)
18
20 20
2. Initial treatment of patients with posterior urethral values No. Cases
Resection of valves and bladder nec k Open destruct ion of va lves Urethral or su prapubic catheter Supra\·esical di\·ersion Total
14 4 6
7*
31
* Kephrostomy was done in 6 cases . TABLE
3. Evaluation of continence and surgical procedure No. Cases
Continent (18 pts.): Transurethral resection of valve only Transu rethral resection of rnh·e and bladder neck Open dest ruction of valve and Y -Y plastv· Incontinent (2 pts.): Transurethra l resection of va lve and bladder neck Open antegrade destruction of valves
12 5
initially had stress incontinence or nocturnal enuresis but this invariably disappeared with time. Fertility: Nine patients more than 21 years old are married, Fertility data could not be obtained in 3 cases but, of the remaining 6, 4 or 67 per cent have fathered children. One of the 2 men who did not father a child committed suicide when he was 21 years old. Growth a nd development: We obtained adequate data concerning growth and development in 16 cases. We measured the growth percentile at presentation versus that at followup. Only 1 patient shifted downward in the percentile growth from the fiftieth to less than the third percentile and this patient is awaiting a transplantation. Two patients remained at less than the third percentile in growth. One of these patients had end-stage renal disease when he was 7 years old, with a creatinine clearance of 7 cc per minute. He had been moribund when he was 2 years old , with severe uremia, renal tubular acidosis and renal rickets. The other patient in the third percentile level is 3 ½ years old and presented as a newborn in the third percentile. He has remained at this level despite reconstruction and a creatinin e clearance of 100 cc per minute. Quality of survival versus presentation. To simplify our discussion the patients were divided into 2 groups: group I-patients who underwent recon struction and are voiding per urethra and group
2-patients who died of the primary disease , have a guarded prognosis, are await ing transplantation or have permanent diversion at the time of followup (fig. 4). If 1 or both kidneys are non-functioning or exhibit marked hydronephrosis the possibility for total reconstruction is decreased. However, all patients with mild hydronephrosis or normal kidneys underwent reconstruction. The initial renal function was compared to the eventual reconstructability (fig. 5). The newborns were separated from the other patients in this study since we found that newborn patients are harder to evaluate and usually have a more severe form of the disease. All newborns had grade 4 hydronephrosis or non-function of 1 or both kidneys and only 50 per cent could undergo reconstruction. Two of the 4 patients with renal function less than 25 per cent of normal on admission died of sepsis. These were preventable deaths. If the renal function was more than 25 per cent of normal the outlook was better. Surprisingly, in older patients, if the renal function was adequate they could eventually undergo reconstruction regardless of the radiographic appearance of the kidneys. Quality of survival versus initial treatment. Best results were obtained following resection of the valves and/or bladder neck but these patients were pre-selected because of minimal renal damaue (table 4). On the other hand, 3 of these 13 patients had significant bilateral hydronephrosis (grade 3 to 4). The patients treated by supraves ic al diversion initially did poorly because they usually had the most severe renal damage . T he groups treated by open destruction of valves and initial bladder drainage will be considered together, since a suprapubic catheter was usually left in place in the former group. The poor results in this group demonstrate that bladder drainaue is inadequate in patients with complete obstruction of 1 or both ureters at the ureterovesical junction. Therefore, a greater number of supravesica l diversions should have been performed. DISCUSSION
L__i_~tle CO.!:felation wa~ noted between the length of symptoms ana the seventy of renal impairment. Of the 7 patients with impaired renal function and symptoms for less than a year 4 were newborns. he loss of functioning renal tissue se o be better corre a e w e severity of th.e_nb_s.t.r.uction than with the dela}c in_tliagnosis. - Contrary to other authors• we did not have any] patients with nocturnal enuresis as the only symp- · tom. Also, every patient who underwent cystos copy IIBd-hl r trabeculation. We aorneueve 1Fiatthe diagnosis of ostenor uret ra va ves can lie made wit out evidence of obstructive c_hgnges p'roximal to the les10n. 7 '°Our study reveals that patients have a surpris1 Forsythe, W. I. and McFadden, G.D. F.: Congenital postenor urethral valves: a study of 35 cases. Brit. J. Urol., 31: 63, 1959.
283
POSTERIOR URETHRAL VALVES
PIP ON PRESEMTAT!ON v,mus QUAUYY Of SUR\fiVAl
G,oup ]I
Group I •
iii
flotienh r®constn$d0d
NORMAL
GIHHliE 1-2
NP
Diveried o, dead ol disease
G~AD~ 3-4 1 OR 110TH KIDNEYS HYDRONEPHROS15 NON-FUNCTION AND
HYDROMEPHROSIS
OTHrn GRADE 4 HYDRONEPHROSIS 2 potierib unable
Hi
<1:1vahrnh1 -no !VP - ] deiad of sepsis
i no follow-up
FIG. 4
RrnAL Fl!NCllON ON PRESENTATION versus QUALITY OF SURVI\/Al
Group I •
<25% Of NORMAL Cereal
Group Il
~
R0construded
<25% Of NORMAL Cmwt
>25% Of
NORMAl Ccroat
>25% OF NORMAL C cr~cit
All OTHER PATIENTS
N[WBORNS ,;,
Divert'3d or dead of diseass
1 pati(>n/ no renal function performed
Frc. 5
TABLE 4.
Initial treatment L'ersus qua/itv of suruiL·al Group 1-"
Open destruct ion of vah-e:-; Suprapuhic or urethral catheter Supra\·esical di\·ersion Transurethral resection of \'akec-s
Group
:2+
:; ~
:,
1:1:j:
and/or bladder neck
* Patients who underwent reconstruct ion.
t Patients
who undenvent diversion or died of the disease.
+1 patient had no followup.
ingly good prognosis despite radiographic evidence of severe upper tract decompensation. Figures 4 and 5 suggest that the chemical determination of renal function is a better prognostic index than the pyelogram. Furthermore, once the lower tracts are
rehabilitated the patient can have a normal life. Only 2 patients were persistently incontinent after treatment. '.\;o patient who underwent resection of the valves only or fulguration was incontinent. previously stated some of these patients did hm e stress incontinence or nocturnal enuresis but this gradually disappeared as the patient matured and their bladder returned to normal. VVe agree v,ith Whitaker and associates who state that an opera tion on the bladder neck increases the of incontinence and that bladder neck changes will resolve after the valves are resected. 8 Poor resultc', are usually secondary to unresected valves rather 8 Whitaker, R. H., Keeton, J. E. and Posterior urethral valves: a studv of urinan· operation. ,J. Urol.. 108: 16,, 197:2. ·
D. I. after
284
AGUSTA AND HOWARDS
than b ladder neck obstruction. Indeed, 4 of our 14 patients required a second resection of the valves. This latter point is to be emphasized since our followup suggests that t h ese patients are capable of satisfactory sexual function and reproduction. Resection of t he bladder neck can result in retrograde ejaculation and infertility. Our series is too small and too varied to comment on the most appropriat e form of t reatment . However, no matter what form is chosen these patients need early and careful followup. Since a third of the ureters are obstructed at t he ureterovesical junction complete destruction of t he valves or bladder diversion will not immediately reverse the obstruction. A higher form of diversion is indicated if the patient becomes septic, renal function shows n o improvement or the upper tracts fail to improve. We prefer nephrostomy tubes which can be removed without another operative procedure since t h e need for diversion is usually temporary. We use supra pubic cystostomy for bladder diversion if the diversion is to be in place more than several days . A severe urethral stricture developed in 1 of our patients treated with a urethral catheter
I
r
for 2 mont hs , requiring further operations. We are not concerned a bout the potential infection related to a suprapubic or nephrostomy tube as long as t he upper tracts are adequately drained. In conclusion, althou gh posterior urethral valves can be a devastating disease, with time, patience and individually structured treatment a full, normal productive life is attainable for the majority of patients. SUMMARY
The followup of 31 patients treated between 1945 and 1972 for posterior urethral valves is reviewed . Emphasis is placed on t he long-term results. P-I:Qgn_2.s is correlates well with renal function at the time_ of presentation. Initial radiologic appearance is a less reliable p.r_o.gno.s.tic index All patients, but especially those with ureterovesical obstruction, should be carefully followed and supravesical diversion should be used when indicat ed. The majority of the patients were totally rehabilitated. N infil'y_per...cent--a-re-ent.i-re-l-j<-G0Btirnmt Eighty.....p_ru: cen t of J;!l!tients mare than 21 years aid and married have-fathered-ehildre.~ nly 1 patient experienced a reduced growth rate after diagnosis.