THE JoGRNAL OF URoLoc-1

Copyright© 1976 by The YVillian1s & \iVilkins Co.

MANAGEMENT OF SEVERE HYDROURETERONEPHROSIS IN INFANTS AND YOUNG CHILDREN JAMES G. SAALFIELD, L. KEITH LLOYD

AND

BLACKWELL B. EVANS

From the Section of Urology, Department of Surgery, Tulane University School of Medicine, New Orleans, Louisiana

ABSTRACT

Loop cutaneous ureterostomy and cutaneous pyelostomy are safe and effective means of temporary diversion with few complications. Reconstructive operation is delayed until the upper urinary tract has shown maximum improvement in form and function and the patient's tolerance for elective operation has improved. Results of reconstruction may be less than optimal and serious complications may arise. However, the staged approach has been shown to be safe and effective. Experience with 29 patients has led to a reassessment of the approach to patients with posterior urethral valves and a stronger inclination to primary valve resection, thus avoiding many of the reconstructive procedures ultimately associated with temporary diversion. Patients with posterior urethral valves who have hydronephrotic upper urinary tracts, normal or near normal serum chemistry studies and are free of infection may receive initial valve ablation. All patients with massive hydroureteronephrosis require long-term followup to ensure preservation of renal function regardless of the approach used. Infants and young children with lower urinary tract obstruction and associated dilated upper urinary tracts represent difficult, time-consuming surgical and medical problems. The dilated, poorly draining upper urinary tract is particularly susceptible to infection, which may cause continued deterioration of renal function. Effective drainage must be established to conserve existing renal function. Although temporary diversion of the urine may be the procedure of choice in many instances a direct approach to relieve the lower urinary tract obstruction may be just as effective and require fewer surgical procedures. The type of temporary diversion is important. Drainage with a supra pubic cystostomy tube or urethral catheter is often poor because of ineffectual ureteral peristalsis or relative ureterovesical junction obstruction, and a tube or catheter almost certainly will introduce infection into the decompensated system. Nephrostomy tube drainage is adequate but introduces a foreign body and infection, and presents the inherent problems of managing such a tube in the child. Cutaneous vesicostomy is easily accomplished but may be associated with significant problems, such as bladder herniation or stomal stricture, and is dependent upon effective peristalsis. 1 Ureteroileal cutaneous anastomosis should be reserved for permanent diversion since the procedure is to a large degree irreversible. In 1963 Johnston first described temporary loop cutaneous ureterostomy.2 Since then, numerous papers have been written describing favorable experiences with the procedure, which provides direct drainage without the use of any tube and maximum opportunity for preservation of renal function. The procedure is easily and rapidly performed, accomplishing the over-all goal of preserving renal function and allowing time for the remaining portion of the upper urinary tract to regain its muscular tone, function and appearance. Herein we will discuss the use of temporary loop cutaneous ureterostomy and, in certain selected cases, cutaneous pyelostomy. In addition, initial transurethral resection of posterior urethral valves will be compared to those cases managed by temporary high urinary diversion. Accepted for publication September 12, 1975. Read at annual meeting of Southeastern Section, American Urological Association, Atlanta, Georgia, April 13-16, 1975. 587

CLINICAL MATERIAL

During a 5-year period 29 children were treated for massive hydroureteronephrosis. Of these patients 18 underwent temporary upper urinary tract diversion and 11 were treated by immediate relief of lower urinary tract obstruction. All of those who received temporary urinary diversion were male subjects except one. They were seen initially at ages ranging from 1 day to 3 years. Presenting symptoms and signs in order of frequency were abdominal or flank mass in 10 patients, fever in 7, urinary tract infection in 4, irritability in 2, neonatal ascites in 1 and straining to void in 1. Primary diagnoses were posterior urethral valves in 10 cases, prune belly syndrome in 3, vesical neck obstruction in 2, ureterovesical obstruction in 1, bilateral ectopic ureteroceles in 1 and undetermined in 1. Three patients had undergone some previous surgical procedure when referred. One patient with posterior urethral valves had undergone trans urethral resection of the valves, Y-V plasty of the bladder neck with bilateral lower ureteroplasties (ureteral tailoring) and reimplantation. A patient with bilateral ectopic ureteroceles had undergone right cutaneous loop ureterostomy and left upper pole nephrectomy for a non-functioning duplicated upper pole with loop cutaneous ureterostomy of the left lower pole ureter. A patient with bladder neck contracture had undergone a right ureteroneocystostomy and Y- V plasty of the bladder neck. All of the 11 patients treated by direct relief of lower urinary tract obstruction had prostatic urethral valves. Presenting signs and symptoms were in the same order of frequency as for the 18 patients receiving temporary diversion. TECHNIQUE

Various surgical techniques for loop cutaneous ureterostomy and cutaneous pyelostomy have been described previously. 3- 7 The technique we used when performing the loop cutaneous ureterostomies was essentially that described by Maloney and Smith, 3 and differed from the technique of Perlmutter and Patil• in that the external oblique fascia was sutured underneath the ureteral loop. A cutaneous pyelostomy was performed when indicated, 7 care being taken to avoid the area of the ureteropelvic junction. The 2 primary technical points considered at the time of operation were to ensure that there

588

SAALFIELD, LLOYD AND EVANS

was no proximal obstruction and that any redundancy of the ureter between the renal pelvis and skin was eliminated. The 18 children who underwent temporary diversion had a total of 27 cutaneous loop ureterostomies and 6 cutaneous pyelostomies. The urinary tracts have been reconstructed in 12 patients. The reconstructions consisted of 18 dismembered pyeloplasties with simultaneous placement of nephrostomy tubes. Five ureteroplasties (ureteral tailoring) were performed" and 15 ureteroneocystostomies were done. Of 8 units with reflux preoperatively 2 continued to have reflux following reimplantation. Lower urinary tract operations in these patients consisted of 7 transurethral resections of posterior urethral valves, 1 Y-V plasty of the bladder neck, 1 excision of bilateral ectopic ureteroceles and 1 transurethral resection of the bladder neck. No operation was required in 2. In the 11 patients treated by primary relief of lower urinary tract obstruction, posterior urethral valves were either fulgurated or resected under direct vision using a loop electrode. A perinea! urethrostomy was necessary in most cases. Renal function and urinary tract configuration were followed by serial blood urea nitrogen (BUN) and creatinine determinations, and conventional radiographic studies. In addition, renal quantitative scintillation camera studies using 131! hippuran proved to be reliable and helpful. The technique for assessing renal function and, to some extent, urinary tract morphology and function has been described by Schlegel and associates.•12 This procedure gives a rapid assessment of differential and total renal function with a reliable indication of drainage and an exact determination of residual urine after voiding. Normal parameters of renal function have been established for the quantitative scintillation camera study. A comparison of counts over the 2 kidneys during the 1 to 2-minute interval has correlated, in patient studies and in animal studies, with the relative renal blood flow and normally should not be separated by more than a 10 per cent difference. The peak accumulation of radioactivity should occur on the 2 sides at essentially the same time and within the first 5 minutes after injection. At least 67 per cent of the injected radiohippuran should be returned in the urine in 30 minutes and there should be no significant calculated residual urine. RESULTS

Initial temporary diversion. The results of treatment with temporary loop cutaneous ureterostomy have been satisfactory. Prior to diversion 7 of the patients had abnormal BUN values. On subsequent determinations 5 had returned to normal and 2 remained slightly elevated. Of 6 patients having elevated creatinine levels all but 3 returned to normal after temporary diversion. The excretory urograms (IVPs) on these patients showed improvement in 13, no change in 2 and improvement on 1 side and no change on the other in 3. Although 3 patients had documented episodes of pyelonephritis during the temporary diversion each episode responded well to the appropriate medication. Twelve patients either stayed in their same preoperative weight percentile or advanced. None significantly failed to thrive after diversion. The average time from temporary diversion until reconstructive repair was 24 months, ranging from 14 to 44 months. There were no serious postoperative complications resulting from temporary diversion and no subsequent stomal stenosis was encountered. No problems resulted from possible ureteral devascularization. A renal calculus of mixed composition developed in 1 patient. The calculus, a typical infection calculus, was removed at the time of upper urinary tract reconstruction. There has been no recurrence of any calculus formation in 3 years. Results with temporary diversion were similar to those of Flinn and associates. 13 The outcome was more favorable when the BUN and creatinine were normal or

only slightly elevated, and when the patient was operated upon relatively early in the course of the disease. Total reconstruction. A complete reconstructive operation on the upper and lower urinary tract was done in 12 patients; 2 other patients required permanent diversion and the remainder are presently undergoing reconstruction. One patient has been lost to followup after successful reconstruction of the urinary tract. All patients except 1 have normal BUN and creatinine values. Six children who have uninfected urine are off all medicine and 6 are uninfected but on suppressive medication. Recurrent pyelonephritis occurred in 3 patients, 2 of whom underwent unilateral nephrectomy. Secondary unilateral reimplantation was done in 2 patients because of distal ureteral stenosis. Of 3 nephrostomy tubes 2 had to be surgically replaced. Bulbar urethral strictures secondary to instrumentation developed in 2 children and each underwent urethroplasty with good results. Fibrous adhesions between the ureteral orifices developed in 1 patient following bilateral ureteroneocystostomy into a defunctionalized bladder. The fibrous band was successfully removed by transurethral resection. Two patients underwent permanent diversion despite extensive measures at reconstructing the urinary tracts. Each had a nephrectomy for chronic pyelonephritis and poor function prior to the permanent diversion. The kidneys that were removed demonstrated poor function initially, later dropping from 21 to 3 per cent of total renal function in 1 case and from 27 to 4 per cent in the other. A pyeloileocutaneous anastomosis was used in 1 case and a ureterojejunocutaneous anastomosis was used in the other for permanent diversion. One patient presently has a normal BUN and creatinine with a 30-minute 131 I hippuran return of 45 per cent (normal 67 per cent), while the other has a BUN of 57, creatinine of 2.6 and a total 30-minute return of 18 per cent. In addition to the 18 patients with varying diagnoses who were treated by temporary diversion, 11 with posterior urethral valves were treated with valve ablation only. At the time of the valve resection they :anged in age from 2 days to 12 years. Presently, all are doing well with normal BUN and creatinine values, and uninfected urine. There has been no further decrease in renal function in any of these patients and radiographically all are showing either gradual resolution of the upper tract dilatation or at least not showing any further decompensation. The following case illustrates a good result of a patient with massive hydroureteronephrosis treated initially by temporary diversion. It also demonstrates the number of surgical procedures that were used to reconstruct such a patient. CASE REPORT

A 1-year-old boy was referred because of a urinary tract infection and an abnormal IVP, which revealed severe bilateral hydroureteronephrosis with an incompletely duplicated right upper urinary tract (fig. 1, A). A voiding cystourethrogram was typical of posterior urethral valves (fig. 1, B). BUN and creatinine levels were normal. A left cutaneous pyelostomy and double right cutaneous loop ureterostomies were performed. The patient did well and a followup IVP showed marked improvement. Posterior urethral valves were resected transurethrally 15 months later. Reconstructive operation on the upper urinary tracts was started when the patient was 3 years old with excision of the lower right cutaneous loop ureterostomy, a ureteroureterostomy, a pyelopyelostomy and resection of the right upper pole ureter (fig. 2, A). A routine left dismembered pyeloplasty was done 2 months later with resection of the upper third ureter and straightening of the middle third (fig. 2, A). The lower two-thirds of the right ureter and lower one-third of the left ureter were straightened and shortened, and bilateral ureteroneocystostomy was done 9 months after the upper

MANAGEMENT OF SEVERE HYDROURETERONEPHROSIS

589

FIG. 2. A, schematic representation of surgical reconstruction. B, IVP 1 year after total reconstruction

urinary tract operation. After removal of nephrostomy tubes the patient had an episode of pyelonephritis. The infection responded to appropriate therapy and ultimately the patient was taken off medication. An IVP 1 year following total reconstruction demonstrated a good result (fig. 2, B). The present BUN and creatinine are 14 and 0.5, respectively. A recent renal quantitative scintillation camera study revealed a total radionuclide return of 54 per cent (normal 67 per cent), with 41 per cent of the renal function on the right side and 59 per cent function on the left side. The patient has no infection, voids without difficulty and empties the bladder. DISCUSSION

Since 1963 when Johnston 2 described loop cutaneous ureterostomy many authors have presented their experience with temporary urinary diversion. 3- 7 • 14 The use of loop cutaneous ureterostomy and cutaneous pyelostomy has proved to be an excellent procedure for temporary diversion. It affords the opportunity of evaluating the primary pathology electively without danger of further renal deterioration. The procedure for temporary diversion is well tolerated even in the azotemic

patient and it allows maximum recovery and stabilization of renal function. Loop cutaneous ureterostomy and cutaneous pyelostomy are applicable in nearly all cases of massive hydroureteronephrosis. Immediate benefits gained by the sick patient outweigh consideration of the problems with later reconstruction. The infant obtains an opportunity to stabilize and develop while being subjected to minimal risk. This view is reflected not only in our modest experience but in the experience of others. 1, 3-6, 1•-16 The end result desired from temporary loop cutaneous ureterostomy is the resolution of the severe hydroureteronephrosis, a disappearance or decrease in ureteral redundancy, the recompensation of effective ureteral muscular tone and the absence of any upper urinary tract infection. In our experience, despite the often improved morphological changes demonstrated on x-ray studies, the degree of ureterectasis found at the time of surgical reconstruction was still quite significant in most cases. These findings were also reported by Lome and, Williams. 1 • The persistence of significant ureterectasis in our patients necessitated 5 ureteroplasties in the 12 who have undergone total urinary tract reconstruction.

590

SAALFIELD, LLOYD AND EVANS

In 1963 Johnston advocated mobilization of the ureteral loop with simple closure.2 He believed that interrupting the ureteral continuity would impair effective peristalsis. We have routinely excised the exposed ureter because of the chronic inflammatory changes present in the mucosa and then performed a dismembered pyeloplasty. Any redundant upper ureter has been resected along with simultaneous placement of a nephrostomy tube. There has been no functional obstruction at this anastomotic site. After excision of the cutaneous loop ureterostomy in our patients ureteroneocystostomy (15 units) along with ureteral tailoring (5 units) was done when indicated. 8 This operation was technically difficult because of the thick-walled dilated ureter present and the abnormal bladder. Our major complication was distal ureteral stenosis as reported by Lome and Williams. 15 Lome and associates 17 and Tanagho 1 • have reported the development of permanently contracted bladders after a period of temporary defunctionalization. We have not encountered any markedly contracted bladders in our 12 reconstructed patients. Adequate bladder capacity has developed rather rapidly following reconstruction. In order to avoid the multiple reconstructive surgical procedures and possible attendant complications we have reassessed our criteria for use of temporary loop cutaneous ureterostomy in patients with posterior urethral valves. Consequently, we have found that temporary diversion and the ensuing reconstructive procedures may be avoided in many cases by initial resection of the valves. The advent of improved cystoscopic instruments has greatly aided the urologist in performing primary valve ablation. The selection of the proper approach in a given patient can be a difficult decision. Certainly any patients selected for primary valve resection must be followed carefully. The potential for serious complications remains after valve resection because of the persistent upper urinary tract dilatation. The surgeon must be prepared for a long, careful followup and remain ready to perform temporary diversion in the presence of intractable infection or decreasing renal function. The closed, poorly draining upper urinary tract is more susceptible to damage by infection than the well drained system that drains by loop cutaneous ureterostomies. To avoid the multiple staged reconstructive procedures, Dwoskin reported a 1-stage repair after temporary tubeless diversion. 19 He reports good success without major complications. None of our patients has had a 1-stage repair following temporary diversion since we have been cautious about mobilization of both ends of the ureter with simultaneous resection of all redundant ureter present. Hendren has reported impressive experience with immediate relief of lower urinary tract obstruction with simultaneous. upper tract reconstruction.• We have avoided this formidable approach by performing temporary upper tract diversion in those cases with biochemical imbalance or severe infection. Simple posterior urethral valve resection was done in those patients whose serum chemistry studies were normal when first seen or returned to normal shortly after drainage of the bladder by indwelling catheter, and who had no significant infection.

REFERENCES

1. Leape, L. L. and Holder, T. M.: Temporary tubeless urinary diversion in children. J. Pediat. Surg., 5: 288, 1970. 2. Johnston, J. H.: Temporary cutaneous ureterostomy in the management of advanced congenital urinary obstruction. Arch. Dis. Child., 38: 161, 1963. 3. Maloney, J. D. and Smith, J .. P.: Temporary cutaneous loop ureterostomy. J. Ural., 103: 790, 1970. 4. Perlmutter, A. D. and Patil, J.: Loop cutaneous ureterostomy in infants and young children: late results in 32 cases. J. Ural., 107: 655, 1972. 5. Sholem, S. L., Lattimer, J. K. and Uson, A. C.: Further experience with loop cutaneous ureterostomy to save badly damaged kidneys. J. Ural., 111: 827, 1974. 6. Perlmutter, A. D. and Tank, E. S.: Loop cutaneous ureterostomy in infancy. J. Ural., 99: 559, 1968. 7. Immergut, M. A., Jacobson, J. J., Culp, D. A. and Flocks, R.H.: Cutaneous pyelostomy. J. Ural., 101: 276, 1969. 8. Hendren, W. H.: A new approach to infants with severe obstructive uropathy: early complete reconstruction. J. Pediat. Surg., 5: 184, 1970. 9. Schlegel, J. U. and Bakule, P. T.: A diagnostic approach in detecting renal and urinary tract disease. J. Ural., 104: 2, 1970. 10. Schlegel. J. U. and Warlick, J. T., III: Experience in urologic diagnosis using a gamma scintillation camera system. J. Ural., 108: 15, 1972. 11. Bueschen, A. J., Evans, B. B. and Schlegel, J. U.: Renal scintillation camera studies in children. J. Ural., 111: 821, 1974. 12. Evans, B. B., Bueschen, A. J., Colfry, A. J., Jr. and Schlegel, J. U.: 131 I hippuran quantitative scintillation camera studies in the evaluation and management of vesicoureteral reflux. J. Ural., 113: 404, 1975. 13. Flinn, R. A., King, L. R., McDonald, J. H. and Clark, S. S.: Cutaneous ureterastomy: an alternative urinary diversion. J. Ural., 105: 358, 1971. 14. Williams, D. I. and Rabinovitch, H. H.: Cutaneous ureterostomy for the grossly dilated ureter of childhood. Brit. J. Ural., 39: 696, 1967. 15. Lome, L. G. and Williams, D. I.: Urinary reconstruction following temporary cutaneous ureterastomy diversion in children. J. Ural., 108: 162, 1972. 16. Wasserman, D. H. and Garrett, R. A.: Cutaneous ureterostomy: indications in children. J. Ural., 94: 380, 1965. 17. Lome, L. G., Howat, J.M. and Williams, D. I.: The temporarily defunctionalized bladder in children. J. Ural., 107: 469, 1972. 18. Tanagho, E. A.: Congenitally obstructed bladders: fate after prolonged defunctionalization. J. Ural., 111: 102, 1974. 19. Dwoskin, J. J.: Loop cutaneous ureterastomy and cutaneous pyelostomy: one-stage reconstruction. J. Urol., 112: 275, 1974.

COMMENT This paper addresses a "hot issue" and really seems to demonstrate that temporary diversion-reconstruction, extensive immediate reconstruction and valve resection alone all work fairly well in the management of boys, usually infants, with valves and severe hydronephrosis. I have had troubles with extensive reconstruction particularly and so now prefer to resect the valve only and see how the patient does. I would make a stronger case for perinea! urethrostomy to permit valve resection without urethral trauma and resultant stricture. If a boy so treated becomes infected or if hydronephrosis does not diminish, a high loop ureterostomy or cutaneous pyelostomy then becomes indicated to preserve renal function. L.R.K.

Management of severe hydroureteronephrosis in infants and young children.

Loop cutaneous ureterostomy and cutaneous pyelostomy are safe and effective means of temporary diversion with few complications. Reconstructive operat...
181KB Sizes 0 Downloads 0 Views