3 OURNAL Copyrigt,t © 1977 by The \Nilli&ms & \ViikiYis Co.
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PELVIC HYDRONEPHROSIS IN CHILDREN: PERSONAL CASES
REVIEW OF 219
J. H. JOHNSTON, JOHN P. EVANS, KENNETH I. GLASSBERG
AND
STEPHEN R. SHAPIRO
From the Department of Urology, Alder Hey Children's Hospital, Liverpool, England
ABSTRACT
A series of 238 hydronephrotic kidneys in 219 children is reported. The condition was more common in the male than in the female subject and occurred more frequently on the left side. It was often bilateral, especially in infants, with an abdominal mass as the common presenting feature. Loin or abdominal pain was the most frequent complaint in older children. In some cases hydronephrosis presented as a ruptured kidney following trauma. Only 1 patient was hypertensive. The lesion was asymptomatic in 18 cases and the incidence of urinary infection was low. Dismembered pyelometeroplasty was the procedure of choice for reconstruction. Preliminary nephrostomy was used rarely and nephrectomy was done in 10 per cent of the kidneys. Of the 7 reoperations 4 were for persistent obstruction and 3 were because stones had formed after the pyeloplasty. The late results, assessed clinically and radiologically, have been entirely satisfactory. Many kidneys of initially doubtful value showed useful improvement after reconstructive operation and no secondary nephrectomies were performed. The only death in the series occurred 2 ½ years postoperatively and was unrelated to the urinary tract. There have been few reports on large series of cases of hydronephrosis caused by pelvioureteral junction obstruction in children, none of which has recorded the experience of an individual surgeon. The present retrospective review relates to 238 hydronephrotic kidneys in 219 children between the ages of 1 day and 15 years who were hospitalized under the care of the senior author (J. H. J.) during a period of 18 years. The results were assessed by the other authors. The series contains only cases of primary pelvioureteral junction obstruction. Examples of obstruction at this site secondary to lower ureteral or infravesical obstruction or to vesicoureteral reflux have been excluded.
nation in 1 boy revealed severe hypertension and investigation showed a large hydronephrosis in a solitary kidney. ASSOCIATED ANOMALIES
Associated abnormalties affecting the genitourinary tract are listed in table 3. The opposite kidney was absent in 5 children because of a congenital anomaly and in 1 boy who had had a contralateral nephrectomy performed elsewhere 5 years previously at which time the subsequently affected kidney was normal pyelographically. In 3 other cases the opposite kidney was dysplastic and virtually or actually afunctional. The hydronephrosis involved a pelvic kidney in 3 cases, 1 portion of a horseshoe kidney in 8 and the lower moiety of a double kidney in 3. Diseases and anomalies in other systems were uncommon, with the only conditions affecting more than an isolated case being congenital heart disease, spina bifida and Down's syndrome, each of which occurred in 2 children.
CASE MATERIAL
The sex incidence and laterality of the hydronephrosis m children less than and more than 1 year old are recorded m table L It is noteworthy that, particularly in children less than 1 year old, the condition was much more common in the male than in the female and occurred more often on the left in infants, being side. Bilateral disease was especially present in 11 of 38 cases.
LOCAL COMPLICATIONS
Rupture of various degrees of of the hydronephrotic kidney followed external trauma in 6 children. Eleven kidneys contained small and usually multiple calculi; 3 of the affected children had hematuria. in 1 girl with severe infection without stones,
CLINICAL FEATURES
The presenting symptoms and signs are listed in table 2. Routine examination of non-communicative infants revealed an abdominal mass as the most common mode of presentation. In older children loin pain or more vaguely localized abdominal pain was the most frequent complaint. Of the children in the total series 74 had a history of fever or disturbances of micturition suggestive of urinary infection but only 39 (17 per cent of the series) had significant bacilluria in a bladder urine specimen. Macroscopic hematuria occurred in 31 children; 6 had a history of loin trauma and clinical evidence of kidney injury and 3 had calculi in the hydronephrotic kidney. In the baby with neonatal urinary ascites leakage from the hyd~onephrosis affecting a solitary kidney was demonstrable radiologically. The hydronephrosis was asymptomatic in 18 children and was discovered on pyelography performed because of other congenital anomalies or enuresis. A routine school exami-
PATHOLOGY AT THE PELVIOURETERAL JUNCTION
The operative anatomical findings at the pelvioureteral junction are recorded in table 4. Accurate records were made in only 219 of the 238 kidneys.The most common findings were various kinks and angulations, which occurred in 74 per cent of the kidneys. Kinking of the ureter on the dilated pelvis was generally associated with a high ureteral insertion. With ureteroureteral kinks the pelvis retained its conical shape and dependent outlet, giving these cases an appearance of persisting fetal ureteral folds. 1 Obstructing aberrant lower polar vessels were involved in 59 cases and, with 1 exception, the vessels crossed the ureter anteriorly. Localized narrowing affecting the proximal centimeter or less of the ureter was recorded in 57 patients, 9 of whom also had a pelvioureteral kink. Whether the degree of relative narrowing was of pathogenic significance is
Accepted for publication May 14, 1976. 97
98
JOHNSTON AND ASSOCIATES TABLE
1. Sex incidence and side affected Sex
Side Affected
Age Male Less than 1 yr. (38 cases) More than 1 yr. (181 cases)
Left
Right
Bilateral
32
6
23
4
11
122
59
116
57
8
TABLE
2. Presenting clinical features
Clinical Feature Abdominal mass History suggestive of urinary infection Failure to thrive Loin or abdominal pain Spontaneous hematuria Post-traumatic hematuria Hypertension Neonatal urinary ascites Enuresis Pyelography for other anomaly
TABLE
Female
Less Than 1 Year (38 cases)
More Than 1 Year (181 cases)
18 20
11 54
1
5
106
20
5
6
1 1 12 2
4
3. Associated anomalies of the genitourinary tract (219 cases) 12
Contralateral renal lesion: Absent kidney, 6 Dysplastic kidney, 3 Vesicoureteral reflux, 3 Horseshoe kidney Pelvic kidney Duplex kidney Undescended testis Hypospadias Patent urachus Persistent cloaca
8 3 3 4
2
uncertain but in only 4 cases was it impossible to pass a probe through the narrow portion. In the 3 cases in which no obstruction was detected it is clear, in retrospect, that these were examples of megacalicosis and that the operation was in fact unnecessary. 2 There were no examples of ureteral polyp in the series but 1 boy had an obstructing papilloma, a condition which also has been described by Miranda and DeAssis. 3 OPERATIONS
The operations performed are listed in table 5. Preliminary nephrostomy was seldom required and was performed in only 2 cases. Nephrectomy was performed as a primary procedure in 24 kidneys that were severely and irreparably damaged. Of these 24 kidneys 4 were the site of gross cystic dysplasia. By far the most commonly performed reconstructive operation was the Anderson-Hynes type of dismembered pyeloureteroplasty. When anterior aberrant vessels were involved the anastomosis was placed in front of them. In nearly all cases splinting of the anastomosis and nephrostomy were done. The splints were removed after 8 days and the nephrostomy tube was removed a few days later. The suture material used in the majority of cases was 4 or 5-zero chromic catgut and in a few cases polyglycolic acid was used.
Delay in urine passage through the anastomosis, which was at first indicated by the persistent drainage of a large volume through the nephrostomy tube after the removal of the splint and subsequently confirmed in some cases by radiography, was encountered occasionally. In most cases the obstruction resolved following partial withdrawal of the nephrostomy tube or gentle irrigation. In others the anastomosis began to function promptly after retrograde passage of a ureteral catheter. It was presumed that the temporary holdup was owing to postoperative edema at the suture line, blood clot or, in some instances, to the nephrostomy tube occluding the pelvic outlet. In 4 cases reoperation was needed because of persistent obstruction. One kidney was found, 1 month after operation, to have complete occlusion of the anastomosis because of faulty suturing. In an infant with bilateral hydronephrosis there was pyelographic deterioration on 1 side 4 months postoperatively. At reoperation an obstructing aberrant vessel was found. Since the vessel was not evident at the initial pyeloureteroplasty it was believed that the alterations in kidney anatomy and position produced by the operation had displaced a lower pole vessel, causing partial obstruction of the anastomosis. Two other children underwent reoperation 4 and 5 years after pyeloureteroplasty because of radiologic deterioration, which was accompanied by pain in 1 and followed trauma to the kidney in the other. In neither case was any significant obstructing pathology found except for adhesions. In each of the 4 reoperated cases dismembered pyeloureteroplasty was repeated successfully. Three children required reoperation for pelvic calculi, which formed between 1 and 5 years after pyeloureteroplasty. In 1 child a stone had formed on a small fragment of catgut. In no case could any persisting pelvioureteral obstruction be identified. There have been no persisting urinary fistulas and no secondary nephrectomies have been performed.
TABLE
4. Pathology at the pelvioureteral junction (219 cases)
Pelvioureteral or ureteroureteral angulations: Without aberrant vessels, 95 With aberrant vessels, 59 With localized narrowing of ureter, 9 With extensive narrowing of ureter, 1 Ureteral narrowing without significant angulations Mucosa! flap across interior of pelvioureteral junction Ureteral papilloma No detectable obstruction
TABLE
164
48 3
1 3
5. Operations performed (238 kidneys)
Temporary nephrostomy N ephrectomy Dismembered pyeloureteroplasty Y-Vplasty Lysis of angulations Pyelopyelostomy: Ipsilateral in duplex kidney, 1 Contralateral in horseshoe kidney, 1 Intubated ureterotomy Spiral flap pyeloureteroplasty Additional procedures: Pyelolithotomy, 11 Hamilton Stewart nephropexy, 3 Partial nephrectomy, 1
2 24 195 12 3 2
1 1 15
POSTOPERATIVE COMPLICATIONS
There was no operative mortality and early postoperative complications were few. Wound hematoma, significant wound infection and wound breakdown occurred once. Pneumonia and Klebsiella septicemia developed in 2 children, 2 ½ years and 2 months old, shortly after pyeloureteroplasty. Both patients recovered but gentamicin therapy caused partial deafness in 1.
TABLE
6. Comparison of IVPs before and after reconstructive operation
(140 kidneys)
Calices Emptying Concentration
Improved
Unchanged
Deteriorated
82 115 70
53 24 67
5 1 3
OQ •.J J
PELV!C HYDRONEPHR03IS E~"/ CHiLDREN
Fi?. 1. Left hydronephrosis in 11-year-old girl. A and B, preoperative IVP shows pelviocaliceal dilatation and non-visualization of ureter. C, IVP 10 months after dismembered pyeloureteroplasty demonstrates marked improvement and pelvis emptying into ureter.
RESULTS
The duration of after operation or reoperation has ranged from 1 to 17 Clinical records were available on all patients. The only in the series was a with Prader-Willis syndrome who died of causes unrelated to the urinary tract 2 ½ years after unilateral pyelou.reteroplasty. AU other children remain clinically free of~'"-·~"--,--~ referable to the hydronephrosis and those have been completely dren in whom the conditio·n was bilateral or a was affected it is evident later. In were in other hospitals it ,r.,ras torily the incidence of infection nc.ou,...,,,PQ In the absence of clinical indications for earlier assessment excretory urography (IVP) was done routinely between 6 and 12 rnonths postoperatively and was repeated on l or more occasions in many cases. Owing to the habit of x-ray departments to destroy films after a period of about 5 years, preoperative and postoperative IVPs of only 140 kidneys were available for study out of the total of 214 reconstructive operations. The radiograms were compared with respect to 1) the degree of dilatation of the calices, 2) the emptying of the pelvis as judged by visualization of contrast material in the ureter and 3) the renal concentration of the contrast medium. The pyelographic results after operation or reoperation are shown in table 6. In some instances the findings were contradictory in that the kidneys showed deterioration in caliceal shape and in concentration yet emptying appeared satisfactory.
There are obvious fallacies in trying to draw inferences concerning changes in renal concentrating power and in pelvic emptying from comparing preoperative and postoperative pyelograms. Delayed or poor --,-~·-·-·", in the kidney preoperatively may be caused by dilution of contrast material in a dilated obstructed pelvis rather than impaired renal function. In a routine intravenous series the detection of contrast medium in the ureter is often a matter of chance, depending upon the was made. However, in contour, which occurred in 58 per cent of the can be taken to indicate enhanced 1). On the other lack may simply mean that irreversible parenchymal changes existed preoperaIn those in which v,as repeated several years nncr,,rno·ea the appearances were similar to those in the n""""''"M radiograms and in no case was further improvement evident. Mild or moderate dilatation of the ureter, mainly the lumbar segment, was noted in several postoperative pyelograms in cases in which the ureter had appeared to be of normal size in preoperative radiograms and at operation, No ureteral obstruction was evident and it was considered that the widening was possibly owing to diuresis as a consequence of impaired renal concentration (fig. 2). DISCUSSION
Pelvic hydronephrosis in children is mainly diagnosed from the IVP and delayed films are especially helpful. Retrograde urography is needed when there is complete absence of contrast concentration. In order to confirm that the obstruction is
100
JOHNSTON AND ASSOCIATES
FIG. 2. IVP in 4 ½-year-old boy shows dilatation of lumbar segment of ureter following left dismembered pyeloureteroplasty when patient was 1 week old.
primary and not secondary, vesicoureteral reflux must be excluded and if the ureter is not seen on the intravenous series its normality should be demonstrated by retrograde ureterography preoperatively. Our experiences support the conclusions of Uson and associates• who recorded 130 cases and Kelalis and associates' who studied 109 cases in that pelvic hydronephrosis in children is more common in the male than the female subject, occurs more frequently on the left side and is often bilateral, especially in infants. The last finding also was noted by Williams and Karlaftis who reported on 26 infants less than 6 months old. 6 The frequent coexistence of upper urinary tract anomalies in children with congenital abnormalities elsewhere, especially of the lower genitourinary tract, spine and heart, is well recognized and routine pyelography in such cases revealed several hydronephrotic kidneys in our series. It is noteworthy that asymptomatic hydronephrosis occurred in 12 children with enuresis. Most of these cases were referred by one pediatrician who routinely performed IVP in enuretics. Since the hydronephrosis was not responsible for the bedwetting and relief of the obstruction had little effect on the symptom, the occasional finding of hydronephrosis does not logically indicate the need for routine urinary tract investigation in enuretics and one could equally well make a case for routine pyelography in all children. The incidence of urinary infection in our cases, as in those of Kelalis and associates, 5 was low, with only 17 per cent having confirmed significant bacilluria. This incidence contrasts strikingly with cases of obstruction affecting the lower ureter when complicating infection is common. The operative findings at the pelvioureteral junction in our series contribute little to the perennial argument as to whether
pelvioureteral kinks are the primary cause of the obstruction or are secondary to pelvic distension consequent upon some form of functional interference with the transmission of peristalsis. Also, the significance of a localized relative narrowing of the upper ureter remains of uncertain pathogenic importance. In 36 cases included in this series and reported previously 1 pressure flow studies were done at operation. There was a residual functional obstruction to the flow of urine through the pelvioureteral junction in only 4 cases after anatomical obstructing factors had been eliminated. Nephrectomy was performed in 10 per cent (24) of our cases, which contrasts with a nephrectomy rate of 24 per cent in the series of Kelalis and associates 5 and 33 per cent in the series of Uson and associates. 4 The latter authors stated that 50 per cent of their patients had advanced lesions beyond the possibility of repair so that nephrectomy or permanent nephrostomy was needed in a large proportion. However, it must be noted that this series dated back to 1935. In our cases the decision whether to remove or preserve a kidney was made mainly at operation. A large palpable hydronephrotic mass does not necessarily imply a hopelessly damaged kidney, especially in an infant. Often the mass is caused by a distended extrarenal pelvis and a relatively well preserved kidney is perched on its periphery (fig. 3). 1 As previously mentioned the concentration of contrast material on IVP is of limited prognostic value. At operation an assessment was made of the over-all bulk of stretched out kidney parenchyma rather than its thickness and our policy has been to preserve a doubtful kidney rather than remove it. In no i1,stance was this conservative attitude subsequently regretted since many kidneys of initially uncertain quality later showed useful recovery and there have been no secondary nephrectomies in the series. Preliminary nephrostomy was performed in nearly 20 per cent of the hydronephrotic cases of Uson and associates• but we have used it only twice. In our opinion nephrostomy is needed only in the exceptional case with a tense hydronephrosis when definitive operation has to be deferred because of intercurrent disease or the need for operation on a better preserved opposite kidney. Permanent nephrostomy is unacceptable socially and is a cause of persistent infection, stone formation and progressive kidney damage. Dismembered pyeloureteroplasty was performed in the majority of our patients treated by reconstructive operation and the method is applicable to virtually all pelvic hydronephroses in children. Because of the small size of the ureter, especially in
Fm. 3. Large abdominal mass presenting clinically as hydronephrosis in 3-month-old male infant. Palpable tumor was severely dilated pelvis. Relatively well preserved kidney on periphery.
101
H'i.'DRONE?HR031S IN (:HlLDREN
young infants "Ne ha·ve used of the anastomosis an.d drainage in all cases and have not experienced any of the disadvantages or complications predicted by Anderson. 7 In some bilateral cases pyeloureteroplasty was performed on both sides at the same time through a wide, transverse incision, although we prefer to deal with each side individually, with an interval of about 2 weeks between operations. The clinical results of operation in our series have been entirely satisfactory in that the morbidity was negligible, relief of symptoms occurred in all cases and the solitary death was unrelated to the urinary tract. The difficulties involved in comparing renal function and pelvic emptying before and after operation have been noted. However, the pyelographic appearance improved or stabilized in the majority of cases. In 32 children in the series reported previously the results of pyeloureteroplasty were assessed by isotope renography with analogue computer simulation. 8 These studies showed that, even in the absence of pyelographic change, 23 operated kidneys had improved tubular function and 29 had increased rates of emptying postoperatively. In our series maximal pyelographic improvement was apparent on the first pyelogram between 6 and 12 months postoperatively. In those cases in which later pyelograms were performed no progressive improvement was noted. However, we believe that a more lengthy followup and repeat radiography are particularly required in the case in which a hydronephrotic kidney has been removed. In 1 of our patients the opposite kidney, normal at the time of nephrectomy, was severely hydronephrotic 5 years later, presumably because the pelvioure1
teral unable to cope ,;vith the in urinary flow rate.
increase
REFERENCES
1. Johnston, J. H.: The pathogenesis of hydronephrosis in children.
Brit. J. Urol., 41: 724, 1969. 2. Johnston, J. H.: Megacalicosis: a burnt-out obstruction? J. Urol., HO: 344, 1973. 3. Miranda, D. and DeAssis, A. S.: Transitional cell papilloma of ureter in young boy. Urology, 5: 559, 1975. 4. Uson, A. C., Cox, L. A. and Lattimer, J. K.: Hydronephrosis in infants and children. I. Some clinical and pathological aspects. J.A.M.A., 205: 323, 1968. 5. Kelalis, P. P., Culp, 0. S., Stickler, G. B. and Burke, E. C.: Ureteropelvic obstruction in children: experiences with 109 cases. J. Urol., 106: 418, 1971. 6. Williams, D. I. and Karlaftis, C. M.: Hydronephrosis due to pelviureteric obstruction in the newborn. Brit. J. Urol., 38: 138, 1966. 7. Anderson, J. C.: Hydronephrosis. Springfield, Illinois: Charles C Thomas, Publisher, p. 56, 1963. 8. Johnston, J. H. and Kathel, B. L.: The results of surgery for hydronephrosis as determined by renography with analogue computer simulation. Brit. J. Urol., 44: 320, 1972. COMMENT The 2 per cent complication rate achieved with splints and nephrostomy may be matched or even bettered by primary closure when the pelvis is not infected or inflamed but no comparable series yet really demonstrates this fact. I am inclined to use a nephrostomy briefly postoperatively in solitary kidneys, so I guess I subscribe to the authors' viewpoint. Interestingly, all congenital anomalies of paired organs are more common on the left side. L.R.K.
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PELVIC HYDRONEPHROSIS IN CHILDREN: PERSONAL CASES
REVIEW OF 219
J. H. JOHNSTON, JOHN P. EVANS, KENNETH I. GLASSBERG
AND
STEPHEN R. SHAPIRO
From the Department of Urology, Alder Hey Children's Hospital, Liverpool, England
ABSTRACT
A series of 238 hydronephrotic kidneys in 219 children is reported. The condition was more common in the male than in the female subject and occurred more frequently on the left side. It was often bilateral, especially in infants, with an abdominal mass as the common presenting feature. Loin or abdominal pain was the most frequent complaint in older children. In some cases hydronephrosis presented as a ruptured kidney following trauma. Only 1 patient was hypertensive. The lesion was asymptomatic in 18 cases and the incidence of urinary infection was low. Dismembered pyelometeroplasty was the procedure of choice for reconstruction. Preliminary nephrostomy was used rarely and nephrectomy was done in 10 per cent of the kidneys. Of the 7 reoperations 4 were for persistent obstruction and 3 were because stones had formed after the pyeloplasty. The late results, assessed clinically and radiologically, have been entirely satisfactory. Many kidneys of initially doubtful value showed useful improvement after reconstructive operation and no secondary nephrectomies were performed. The only death in the series occurred 2 ½ years postoperatively and was unrelated to the urinary tract. There have been few reports on large series of cases of hydronephrosis caused by pelvioureteral junction obstruction in children, none of which has recorded the experience of an individual surgeon. The present retrospective review relates to 238 hydronephrotic kidneys in 219 children between the ages of 1 day and 15 years who were hospitalized under the care of the senior author (J. H. J.) during a period of 18 years. The results were assessed by the other authors. The series contains only cases of primary pelvioureteral junction obstruction. Examples of obstruction at this site secondary to lower ureteral or infravesical obstruction or to vesicoureteral reflux have been excluded.
nation in 1 boy revealed severe hypertension and investigation showed a large hydronephrosis in a solitary kidney. ASSOCIATED ANOMALIES
Associated abnormalties affecting the genitourinary tract are listed in table 3. The opposite kidney was absent in 5 children because of a congenital anomaly and in 1 boy who had had a contralateral nephrectomy performed elsewhere 5 years previously at which time the subsequently affected kidney was normal pyelographically. In 3 other cases the opposite kidney was dysplastic and virtually or actually afunctional. The hydronephrosis involved a pelvic kidney in 3 cases, 1 portion of a horseshoe kidney in 8 and the lower moiety of a double kidney in 3. Diseases and anomalies in other systems were uncommon, with the only conditions affecting more than an isolated case being congenital heart disease, spina bifida and Down's syndrome, each of which occurred in 2 children.
CASE MATERIAL
The sex incidence and laterality of the hydronephrosis m children less than and more than 1 year old are recorded m table L It is noteworthy that, particularly in children less than 1 year old, the condition was much more common in the male than in the female and occurred more often on the left in infants, being side. Bilateral disease was especially present in 11 of 38 cases.
LOCAL COMPLICATIONS
Rupture of various degrees of of the hydronephrotic kidney followed external trauma in 6 children. Eleven kidneys contained small and usually multiple calculi; 3 of the affected children had hematuria. in 1 girl with severe infection without stones,
CLINICAL FEATURES
The presenting symptoms and signs are listed in table 2. Routine examination of non-communicative infants revealed an abdominal mass as the most common mode of presentation. In older children loin pain or more vaguely localized abdominal pain was the most frequent complaint. Of the children in the total series 74 had a history of fever or disturbances of micturition suggestive of urinary infection but only 39 (17 per cent of the series) had significant bacilluria in a bladder urine specimen. Macroscopic hematuria occurred in 31 children; 6 had a history of loin trauma and clinical evidence of kidney injury and 3 had calculi in the hydronephrotic kidney. In the baby with neonatal urinary ascites leakage from the hyd~onephrosis affecting a solitary kidney was demonstrable radiologically. The hydronephrosis was asymptomatic in 18 children and was discovered on pyelography performed because of other congenital anomalies or enuresis. A routine school exami-
PATHOLOGY AT THE PELVIOURETERAL JUNCTION
The operative anatomical findings at the pelvioureteral junction are recorded in table 4. Accurate records were made in only 219 of the 238 kidneys.The most common findings were various kinks and angulations, which occurred in 74 per cent of the kidneys. Kinking of the ureter on the dilated pelvis was generally associated with a high ureteral insertion. With ureteroureteral kinks the pelvis retained its conical shape and dependent outlet, giving these cases an appearance of persisting fetal ureteral folds. 1 Obstructing aberrant lower polar vessels were involved in 59 cases and, with 1 exception, the vessels crossed the ureter anteriorly. Localized narrowing affecting the proximal centimeter or less of the ureter was recorded in 57 patients, 9 of whom also had a pelvioureteral kink. Whether the degree of relative narrowing was of pathogenic significance is
Accepted for publication May 14, 1976. 97
98
JOHNSTON AND ASSOCIATES TABLE
1. Sex incidence and side affected Sex
Side Affected
Age Male Less than 1 yr. (38 cases) More than 1 yr. (181 cases)
Left
Right
Bilateral
32
6
23
4
11
122
59
116
57
8
TABLE
2. Presenting clinical features
Clinical Feature Abdominal mass History suggestive of urinary infection Failure to thrive Loin or abdominal pain Spontaneous hematuria Post-traumatic hematuria Hypertension Neonatal urinary ascites Enuresis Pyelography for other anomaly
TABLE
Female
Less Than 1 Year (38 cases)
More Than 1 Year (181 cases)
18 20
11 54
1
5
106
20
5
6
1 1 12 2
4
3. Associated anomalies of the genitourinary tract (219 cases) 12
Contralateral renal lesion: Absent kidney, 6 Dysplastic kidney, 3 Vesicoureteral reflux, 3 Horseshoe kidney Pelvic kidney Duplex kidney Undescended testis Hypospadias Patent urachus Persistent cloaca
8 3 3 4
2
uncertain but in only 4 cases was it impossible to pass a probe through the narrow portion. In the 3 cases in which no obstruction was detected it is clear, in retrospect, that these were examples of megacalicosis and that the operation was in fact unnecessary. 2 There were no examples of ureteral polyp in the series but 1 boy had an obstructing papilloma, a condition which also has been described by Miranda and DeAssis. 3 OPERATIONS
The operations performed are listed in table 5. Preliminary nephrostomy was seldom required and was performed in only 2 cases. Nephrectomy was performed as a primary procedure in 24 kidneys that were severely and irreparably damaged. Of these 24 kidneys 4 were the site of gross cystic dysplasia. By far the most commonly performed reconstructive operation was the Anderson-Hynes type of dismembered pyeloureteroplasty. When anterior aberrant vessels were involved the anastomosis was placed in front of them. In nearly all cases splinting of the anastomosis and nephrostomy were done. The splints were removed after 8 days and the nephrostomy tube was removed a few days later. The suture material used in the majority of cases was 4 or 5-zero chromic catgut and in a few cases polyglycolic acid was used.
Delay in urine passage through the anastomosis, which was at first indicated by the persistent drainage of a large volume through the nephrostomy tube after the removal of the splint and subsequently confirmed in some cases by radiography, was encountered occasionally. In most cases the obstruction resolved following partial withdrawal of the nephrostomy tube or gentle irrigation. In others the anastomosis began to function promptly after retrograde passage of a ureteral catheter. It was presumed that the temporary holdup was owing to postoperative edema at the suture line, blood clot or, in some instances, to the nephrostomy tube occluding the pelvic outlet. In 4 cases reoperation was needed because of persistent obstruction. One kidney was found, 1 month after operation, to have complete occlusion of the anastomosis because of faulty suturing. In an infant with bilateral hydronephrosis there was pyelographic deterioration on 1 side 4 months postoperatively. At reoperation an obstructing aberrant vessel was found. Since the vessel was not evident at the initial pyeloureteroplasty it was believed that the alterations in kidney anatomy and position produced by the operation had displaced a lower pole vessel, causing partial obstruction of the anastomosis. Two other children underwent reoperation 4 and 5 years after pyeloureteroplasty because of radiologic deterioration, which was accompanied by pain in 1 and followed trauma to the kidney in the other. In neither case was any significant obstructing pathology found except for adhesions. In each of the 4 reoperated cases dismembered pyeloureteroplasty was repeated successfully. Three children required reoperation for pelvic calculi, which formed between 1 and 5 years after pyeloureteroplasty. In 1 child a stone had formed on a small fragment of catgut. In no case could any persisting pelvioureteral obstruction be identified. There have been no persisting urinary fistulas and no secondary nephrectomies have been performed.
TABLE
4. Pathology at the pelvioureteral junction (219 cases)
Pelvioureteral or ureteroureteral angulations: Without aberrant vessels, 95 With aberrant vessels, 59 With localized narrowing of ureter, 9 With extensive narrowing of ureter, 1 Ureteral narrowing without significant angulations Mucosa! flap across interior of pelvioureteral junction Ureteral papilloma No detectable obstruction
TABLE
164
48 3
1 3
5. Operations performed (238 kidneys)
Temporary nephrostomy N ephrectomy Dismembered pyeloureteroplasty Y-Vplasty Lysis of angulations Pyelopyelostomy: Ipsilateral in duplex kidney, 1 Contralateral in horseshoe kidney, 1 Intubated ureterotomy Spiral flap pyeloureteroplasty Additional procedures: Pyelolithotomy, 11 Hamilton Stewart nephropexy, 3 Partial nephrectomy, 1
2 24 195 12 3 2
1 1 15
POSTOPERATIVE COMPLICATIONS
There was no operative mortality and early postoperative complications were few. Wound hematoma, significant wound infection and wound breakdown occurred once. Pneumonia and Klebsiella septicemia developed in 2 children, 2 ½ years and 2 months old, shortly after pyeloureteroplasty. Both patients recovered but gentamicin therapy caused partial deafness in 1.
TABLE
6. Comparison of IVPs before and after reconstructive operation
(140 kidneys)
Calices Emptying Concentration
Improved
Unchanged
Deteriorated
82 115 70
53 24 67
5 1 3
OQ •.J J
PELV!C HYDRONEPHR03IS E~"/ CHiLDREN
Fi?. 1. Left hydronephrosis in 11-year-old girl. A and B, preoperative IVP shows pelviocaliceal dilatation and non-visualization of ureter. C, IVP 10 months after dismembered pyeloureteroplasty demonstrates marked improvement and pelvis emptying into ureter.
RESULTS
The duration of after operation or reoperation has ranged from 1 to 17 Clinical records were available on all patients. The only in the series was a with Prader-Willis syndrome who died of causes unrelated to the urinary tract 2 ½ years after unilateral pyelou.reteroplasty. AU other children remain clinically free of~'"-·~"--,--~ referable to the hydronephrosis and those have been completely dren in whom the conditio·n was bilateral or a was affected it is evident later. In were in other hospitals it ,r.,ras torily the incidence of infection nc.ou,...,,,PQ In the absence of clinical indications for earlier assessment excretory urography (IVP) was done routinely between 6 and 12 rnonths postoperatively and was repeated on l or more occasions in many cases. Owing to the habit of x-ray departments to destroy films after a period of about 5 years, preoperative and postoperative IVPs of only 140 kidneys were available for study out of the total of 214 reconstructive operations. The radiograms were compared with respect to 1) the degree of dilatation of the calices, 2) the emptying of the pelvis as judged by visualization of contrast material in the ureter and 3) the renal concentration of the contrast medium. The pyelographic results after operation or reoperation are shown in table 6. In some instances the findings were contradictory in that the kidneys showed deterioration in caliceal shape and in concentration yet emptying appeared satisfactory.
There are obvious fallacies in trying to draw inferences concerning changes in renal concentrating power and in pelvic emptying from comparing preoperative and postoperative pyelograms. Delayed or poor --,-~·-·-·", in the kidney preoperatively may be caused by dilution of contrast material in a dilated obstructed pelvis rather than impaired renal function. In a routine intravenous series the detection of contrast medium in the ureter is often a matter of chance, depending upon the was made. However, in contour, which occurred in 58 per cent of the can be taken to indicate enhanced 1). On the other lack may simply mean that irreversible parenchymal changes existed preoperaIn those in which v,as repeated several years nncr,,rno·ea the appearances were similar to those in the n""""''"M radiograms and in no case was further improvement evident. Mild or moderate dilatation of the ureter, mainly the lumbar segment, was noted in several postoperative pyelograms in cases in which the ureter had appeared to be of normal size in preoperative radiograms and at operation, No ureteral obstruction was evident and it was considered that the widening was possibly owing to diuresis as a consequence of impaired renal concentration (fig. 2). DISCUSSION
Pelvic hydronephrosis in children is mainly diagnosed from the IVP and delayed films are especially helpful. Retrograde urography is needed when there is complete absence of contrast concentration. In order to confirm that the obstruction is
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JOHNSTON AND ASSOCIATES
FIG. 2. IVP in 4 ½-year-old boy shows dilatation of lumbar segment of ureter following left dismembered pyeloureteroplasty when patient was 1 week old.
primary and not secondary, vesicoureteral reflux must be excluded and if the ureter is not seen on the intravenous series its normality should be demonstrated by retrograde ureterography preoperatively. Our experiences support the conclusions of Uson and associates• who recorded 130 cases and Kelalis and associates' who studied 109 cases in that pelvic hydronephrosis in children is more common in the male than the female subject, occurs more frequently on the left side and is often bilateral, especially in infants. The last finding also was noted by Williams and Karlaftis who reported on 26 infants less than 6 months old. 6 The frequent coexistence of upper urinary tract anomalies in children with congenital abnormalities elsewhere, especially of the lower genitourinary tract, spine and heart, is well recognized and routine pyelography in such cases revealed several hydronephrotic kidneys in our series. It is noteworthy that asymptomatic hydronephrosis occurred in 12 children with enuresis. Most of these cases were referred by one pediatrician who routinely performed IVP in enuretics. Since the hydronephrosis was not responsible for the bedwetting and relief of the obstruction had little effect on the symptom, the occasional finding of hydronephrosis does not logically indicate the need for routine urinary tract investigation in enuretics and one could equally well make a case for routine pyelography in all children. The incidence of urinary infection in our cases, as in those of Kelalis and associates, 5 was low, with only 17 per cent having confirmed significant bacilluria. This incidence contrasts strikingly with cases of obstruction affecting the lower ureter when complicating infection is common. The operative findings at the pelvioureteral junction in our series contribute little to the perennial argument as to whether
pelvioureteral kinks are the primary cause of the obstruction or are secondary to pelvic distension consequent upon some form of functional interference with the transmission of peristalsis. Also, the significance of a localized relative narrowing of the upper ureter remains of uncertain pathogenic importance. In 36 cases included in this series and reported previously 1 pressure flow studies were done at operation. There was a residual functional obstruction to the flow of urine through the pelvioureteral junction in only 4 cases after anatomical obstructing factors had been eliminated. Nephrectomy was performed in 10 per cent (24) of our cases, which contrasts with a nephrectomy rate of 24 per cent in the series of Kelalis and associates 5 and 33 per cent in the series of Uson and associates. 4 The latter authors stated that 50 per cent of their patients had advanced lesions beyond the possibility of repair so that nephrectomy or permanent nephrostomy was needed in a large proportion. However, it must be noted that this series dated back to 1935. In our cases the decision whether to remove or preserve a kidney was made mainly at operation. A large palpable hydronephrotic mass does not necessarily imply a hopelessly damaged kidney, especially in an infant. Often the mass is caused by a distended extrarenal pelvis and a relatively well preserved kidney is perched on its periphery (fig. 3). 1 As previously mentioned the concentration of contrast material on IVP is of limited prognostic value. At operation an assessment was made of the over-all bulk of stretched out kidney parenchyma rather than its thickness and our policy has been to preserve a doubtful kidney rather than remove it. In no i1,stance was this conservative attitude subsequently regretted since many kidneys of initially uncertain quality later showed useful recovery and there have been no secondary nephrectomies in the series. Preliminary nephrostomy was performed in nearly 20 per cent of the hydronephrotic cases of Uson and associates• but we have used it only twice. In our opinion nephrostomy is needed only in the exceptional case with a tense hydronephrosis when definitive operation has to be deferred because of intercurrent disease or the need for operation on a better preserved opposite kidney. Permanent nephrostomy is unacceptable socially and is a cause of persistent infection, stone formation and progressive kidney damage. Dismembered pyeloureteroplasty was performed in the majority of our patients treated by reconstructive operation and the method is applicable to virtually all pelvic hydronephroses in children. Because of the small size of the ureter, especially in
Fm. 3. Large abdominal mass presenting clinically as hydronephrosis in 3-month-old male infant. Palpable tumor was severely dilated pelvis. Relatively well preserved kidney on periphery.
101
H'i.'DRONE?HR031S IN (:HlLDREN
young infants "Ne ha·ve used of the anastomosis an.d drainage in all cases and have not experienced any of the disadvantages or complications predicted by Anderson. 7 In some bilateral cases pyeloureteroplasty was performed on both sides at the same time through a wide, transverse incision, although we prefer to deal with each side individually, with an interval of about 2 weeks between operations. The clinical results of operation in our series have been entirely satisfactory in that the morbidity was negligible, relief of symptoms occurred in all cases and the solitary death was unrelated to the urinary tract. The difficulties involved in comparing renal function and pelvic emptying before and after operation have been noted. However, the pyelographic appearance improved or stabilized in the majority of cases. In 32 children in the series reported previously the results of pyeloureteroplasty were assessed by isotope renography with analogue computer simulation. 8 These studies showed that, even in the absence of pyelographic change, 23 operated kidneys had improved tubular function and 29 had increased rates of emptying postoperatively. In our series maximal pyelographic improvement was apparent on the first pyelogram between 6 and 12 months postoperatively. In those cases in which later pyelograms were performed no progressive improvement was noted. However, we believe that a more lengthy followup and repeat radiography are particularly required in the case in which a hydronephrotic kidney has been removed. In 1 of our patients the opposite kidney, normal at the time of nephrectomy, was severely hydronephrotic 5 years later, presumably because the pelvioure1
teral unable to cope ,;vith the in urinary flow rate.
increase
REFERENCES
1. Johnston, J. H.: The pathogenesis of hydronephrosis in children.
Brit. J. Urol., 41: 724, 1969. 2. Johnston, J. H.: Megacalicosis: a burnt-out obstruction? J. Urol., HO: 344, 1973. 3. Miranda, D. and DeAssis, A. S.: Transitional cell papilloma of ureter in young boy. Urology, 5: 559, 1975. 4. Uson, A. C., Cox, L. A. and Lattimer, J. K.: Hydronephrosis in infants and children. I. Some clinical and pathological aspects. J.A.M.A., 205: 323, 1968. 5. Kelalis, P. P., Culp, 0. S., Stickler, G. B. and Burke, E. C.: Ureteropelvic obstruction in children: experiences with 109 cases. J. Urol., 106: 418, 1971. 6. Williams, D. I. and Karlaftis, C. M.: Hydronephrosis due to pelviureteric obstruction in the newborn. Brit. J. Urol., 38: 138, 1966. 7. Anderson, J. C.: Hydronephrosis. Springfield, Illinois: Charles C Thomas, Publisher, p. 56, 1963. 8. Johnston, J. H. and Kathel, B. L.: The results of surgery for hydronephrosis as determined by renography with analogue computer simulation. Brit. J. Urol., 44: 320, 1972. COMMENT The 2 per cent complication rate achieved with splints and nephrostomy may be matched or even bettered by primary closure when the pelvis is not infected or inflamed but no comparable series yet really demonstrates this fact. I am inclined to use a nephrostomy briefly postoperatively in solitary kidneys, so I guess I subscribe to the authors' viewpoint. Interestingly, all congenital anomalies of paired organs are more common on the left side. L.R.K.
