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Ileal Replacement of the Ureter in Solitary Kidney
Symposium on Gastrointestinal Surgery
Ileal Replacement of the Ureter in Solitary Kidney
Joseph B. Dowd, M.D., and Fusen Chen, M.D.
The ingenuity, experience, and skill of the urologic surgeon are occasionally contested. One of the less common of these challenges occurs when the ureter may become damaged or be deficient at a level too high to permit reimplantation in the urinary bladder. This may occur as a result of an anomaly, such as agenesis (Case 1), a long aperistaltic distal segment, severe stenosis, ureteral valve, or dysplasia. More commonly, however, it is the result of injury, either from without, such as blunt or penetrating missiles, or from within. In this category are ureteral compromise as during or after surgery of the retroperitoneum or pelvis for tumors (bowel, cervix, uterus), the surgery for aneurysms, fistulas, stone manipulation, pyeloureterolithotomy (Case 3), or ureteral ischemia from purposeful (Case 2) or incidental ureterolysis. Long stricture may occur consequent to denuding as in lymphadenectomy or after pelvic packing or sepsis with extravasation, multiple pelvic operations, irradiation, or tuberculous ureteritis, with resultant destruction of the lumen at several levels. Occasionally the distal half or more of the ureter may be resected because of involvement by extrinsic tumor or even electively resected because of the presence of a low-grade primary ureteral tumor with evidence that it did not seed from the renal pelvis above. Rarely, the surgeon is taxed because of inadequate length of the ureter when it is desirable to transplant a ureter back into the bladder after prior diversion to either bowel or skin. Whatever the cause of ureteral deficiency or irreversible damage, the surgeon has the choice of ipsilateral nephrectomy, permanent nephrostomy, high cutaneous ureterostomy, autotransplantation, or substitution of the ureter. Ipsilateral nephrectomy in general is undesirable, violating as it does the urologist's basic principle of preserving renal parenchyma. At times, particularly in palliation when life span is limited, it may be warranted and justifiable. It is, however, not an alternative when the patient has a solitary kidney (Cases 1 and 3), or when there is inadequate contralateral renal tissue to sustain life (Case 2). Surgical Clinics of North America- Vol. 51, No.3, June 1971
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Nephrostomy, an immediate solution, is undesirable as a permanent diversion because of the threat to the patient in regard to infection, diminished function, difficulties in keeping the tube in place, keeping the patient dry, and adjusting to the inaccessible external drainage apparatus. Cutaneous ureterostomy is fraught with complications and commonly results in permanent intubation because of abdominal wall level strictures. Appliances for this uncommon form of adult diversion are notoriously inefficient in keeping the patient dry and relatively unlimited in activity. The same renal insult is present in regard to infection and loss of function as with permanent nephrostomies. Autotransplantation of the kidney to the pelvis has merit, although it is not applicable when the upper one third to one half of the ureter is not available. However, great redundancy of an extrarenal pelvis may permit connection to a bladder flap. Not every surgeon would undertake autotransplantation, especially in a patient with solitary kidney. Not infrequently in these patients (Case 3), the renal pedicle is fixed so as to preclude autotransplantation. Although veins and artificial substitutes for the ureter have been used, their application is experimental and not theoretically sound. Transureteroureterostomy is not applicable if the proximal half of the ureter is not available or in instances of congenital solitary kidney. Nor is it a certainty that this procedure does not compromise the contralateral side if present, even if technically possible in the presence of proximal ureteral damage. High ureterocolic anastomosis is mentioned only to condemn it as unwise, prone as it is to pyelonephritis with or without reflux, to hyperchloremic acidosis, and to potassium deficiency. Under any circumstances it is unwise in the presence of a solitary kidney. We are thus left with isolated segments of distal small bowel as substitute ureters.!. 6 They have much to commend them-accessibility, mobility, vascularity, and peristaltic activity. These segments are not ideal, however, because of problems of electrolyte absorption, dilatation and stasis, mucus formation, and reflux.
CASE REPORTS CASE 1. An 8 year old boy was referred to us on April 16, 1963, with a left nephrostomy tube (No. 14 French Foley catheter) that was increasingly difficult to replace, frequently becoming dislodged despite great limitation of the child's activity. He was having frequent attacks of chills, fever, and left flank pain despite maintenance antiseptic therapy. The patient was born after a normal gestation and delivery. He was anuric the first 24 hours, and the level of blood urea nitrogen the second day of life was more than 60 mg. per 100 ml. An attempted intravenous pyelogram revealed no excretion of dye or renal shadows. No visible external anomalies were noted. The right flank was explored and revealed absence of the right kidney as such, although a 1.0 cm. nubbin of embryonic fibrous tissue at the end of a blind-ending ureter was found at the level of the second lumbar vertebra. The ureter "seemed normal below that point." Following immediate left nephrostomy, the infant's condition promptly improved, and the level of blood urea nitrogen dropped to
ILEAL REPLACEMENT OF THE URETER IN SOLITARY KIDNEY
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normal limits. All subsequent pyelograms revealed massive hydronephrosis with a sizable extrarenal pelvis and adequate cortex but no descent of dye to a ureter. Cystoscopy at age 2 showed absence of the trigone and a normal right ureteral orifice. Retrograde ureterography showed the right ureter to end blindly at the level of the second lumbar vertebra. No left ureteral orifice was observed. Left flank exploration at age 3'12 failed to locate a left ureter. At this time the child was neither anemic nor azotemic and was well developed but small for his age. Culture from the nephrostomy tube revealed resistant Proteus vulgaris and Pseudomonas aeruginosa. A voiding cystourethrogram revealed a normal capacious bladder without reflux, trabeculation, or residuum. Although the bladder had never functioned as such, the child had normal urinary control. Cystometrography revealed normal findings; at subsequent cystoscopy, no obstruction was present and no left ureteral orifice was found. The right blind-ending ureter extended to the level of the second lumbar vertebra but was inadequate to reach the left renal pelvis. At transabdominal exploration, a fibrous strand without a lumen replaced the upper third of the ureter, a 5.0 cm. fusiform segment represented the midureter where it ended blindly, and no ureter was found from the iliac vessels to the bladder. Adequate renal cortex and a redundant extrarenal pelvis were present. An isolated segment of ureter, 20 cm., was prepared, and anterior ureteroureterostomy was accomplished. The proximal end of the prepared isolated segment was anastomosed to the redundant renal pelvis obliquely, and the shortest, straightest, direct course to the left posterior wall of the bladder was accomplished. The ileovesical anastomosis was accomplished end to side transvesically. A No. 18 Levin tube splint traversed ileopelvic and ileovesical anastomoses and was brought through a right abdominal stab wound. A No. 18 French nephrostomy tube and suprapubic tube were also placed. The anastomoses at kidney and bladder levels were extraperitoneal, but the main course of the ureter was intraperitoneal through generous mesenteric and parietal peritoneal windows. The postoperative course was uneventful. The transileal splint was removed on the twelfth postoperative day. The suprapubic tube on the fifteenth postoperative day, and the left nephrostomy tube was removed 1 month later in the office. The cystostomy and nephrostomy stab wounds promptly healed. Although there was occasional difficulty voiding mucus, with double voiding and administration of maintenance doses of nitrofurantoin (Furadantin), the clinical course was unremarkable. Interval checks revealed continued infection, increasing bladder residual urines, and dilatation followed by tortuosity of the ileal ureter. Levels of electrolytes and blood urea nitrogen remained normal. In July 1967, transurethral resection of hypertrophied bladder neck was performed through a perineal urethrostomy. Triple voiding was urged but rarely followed. Blood counts, including electrolytes, blood urea nitrogen, and serum creatinine have remained normal, but the ileal segment remains dilated and elongated. Enforced triple voiding shows improvement to less than 200 ml. of residual urine, but this is rarely performed by the patient. The ileal segment will be brought to the skin at the end of the current school year. There has been unusual physical development and athletic activity, including contact sports, since conversion from a permanent nephrostomy to an ileal substitute ureter (Fig. 1).
Comment. There were a number of interesting facets in the findings and management of this child. The right ureter unfortunately did not reach the left renal pelvis. At the age of 8, the first time fluid had been placed in his bladder, he demonstrated normal urinary control without residuum. Bladder hypertrophy developed with relative bladder neck obstruction that may have been the result of intermittent obstruction of the ileal mucus or the presence of a pseudodiverticulum, refluxing up the capacious left ureter, via the gaping ileovesicostomy. Voiding improved with the transurethral resection, but ileal decompensation and residual urine recurred. The ileal mucus has not diminished in 8
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JOSEPH B. DOWD AND FC5L.'-1 CHEN
Figure 1. A, Left ileal ureter, immediate postoperative view. B, View 51/2 years after operation.
years, although triple voiding is demonstrably helpful, with voidings of 800 ml. followed by 200 ml. in 3 to 5 minutes, followed by an additional 130 to 200 ml. in 3 to 5 minutes. A residual urine of 200 ml. remains. It took almost 3 years to sterilize the urinary tract with continual administration of nitrofurantoin (Furadantin). In recent years, orally administered nitrofurantoin macrocrystals (Macrodantin) maintenance therapy is well tolerated and has been continued because of the iliectasia of the substitute ureter and occasional low colony count of organisms on culture. CASE 2. A 49 year old extremely obese woman was found to have a hypoplastic left kidney and grade 1 right hydroureteronephrosis in April 1966 when a pyelogram was taken because of steady use of methysergide N.F. (Sansert). The patient was urged to discontinue methysergide N.F., and alternate medication for migraine headaches was prescribed. In December 1967 she was referred by a psychiatrist because of anemia, oliguria, and right flank pain. Infusion pyelography revealed bilateral hydroureteronephrosis. The patient had continued to use methysergide in increasing doses despite the warning 19 months earlier. A right nephrostomy was performed. Twelve days later a transabdominal bilateral ureterolysis was performed. On the tenth postoperative day, because of acute bacteremic shock, a right nephrostogram (Fig. 2A) revealed extravasation of dye at the midureter level. Transabdominal exploration revealed a foul intraperitoneal and retroperitoneal abscess in which the tip of the appendix was found free. Approximately 6.0 cm. of ureter was found ischemic, possibly denuded at the time of ureterolysis. Urinary extravasation was obvious. After a stormy course, including the use of large doses of antibiotics and steroids, the patient recovered and was discharged with a nephrostomy tube in place. She returned 3 months later, and on March 29, 1968, at transabdominal exploration an isolated segment of ileum was prepared, and posterior enteroenterostomy was carried out. The proximal end of the ileal segment was anas-
!
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\
Figure 2. A, Right nephrostogram with extravasation. B, Right ileal ureter 2'12 years later.
tomosed retroperitoneally to viable though dilated ureter, 2.0 cm. below an unobstructed ureteropelvic junction. The ureter was brought through a generous window in the right colic mesentery and posterior parietal peritoneum. The most direct intraperitoneal course of ileum was then directed to the bladder where it was anastomosed low on the posterior wall extraperitoneally by direct transvesical mucosa-to-mucosa anastomosis without a nipple. A No. 16 silas tic splint traversed the ileal ureter and was brought through a left suprapubic stab wound. A protective suprapubic cystotomy tube was placed, and the right nephrostomy tube was left in place. A generous window of bladder was excised at the ileovesical anastomotic site. The postoperative course was uneventful. The patient passed mucus with ease. The hydronephrosis resolved within a month after removing all splints. The patient has been followed up at 3-month to 6-month intervals and remains comfortable. The urine has been sterile since 5 months after operation. After 22 months mild hyperchloremic acidosis has appeared. Some improvement in the apparent function of the left kidney is noted pyelographically 21(2 years later (Fig.2B).
Comment. The relationship between retroperitoneal fibrosis and prolonged use of methysergide N.F. is well documented. 3 , 4 The improvement in the apparent function of the hypoplastic left kidney may be the result of ureterolysis, or, in terms of renal counterbalance, improved because of demand from diminished function of the hypertrophic right kidney. Despite the immense size of the patient and the complex intraperitoneal findings due to perforated appendix and ureteral ischemia from overzealous ureterolysis, the anastomosis both at the kidney and bladder levels was easily carried out by an adequate xiphoid-to-pubis incision. There has been no problem voiding the mucus, presumably because of the patient's normal bladder and unobstructed urethra, unlike
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the difficulties found in men. The ileal mucus shows no tendency to diminish to date. CASE 3. A 34 year old man was referred to us on July 7, 1969, with a No.5 French ureteral catheter in place as it had been for most of the preceding 9 months. The patient had increasing renal insufficiency and refractory urinary tract infections. At the age of 23 while in military service, he had a right nephrectomy for "infected kidney stones." The left upper ureter and small intrarenal pelvis had apparently become damaged as a result of two attempts to extract obstructing calculi from the small left intrarenal pelvis, resulting in destruction of the left intrarenal pelvis, ureteropelvic junction, and proximal 4.0 cm. of ureter (Fig. 3A). On July 16, 1969, autotransplantation or ileal substitute ureter was planned. Because the left renal pedicle was densely scarred and technically impossible to dissect, an isolated 25 cm. segment of ileum was prepared. All stones were removed from the intrarenal pelvis, and with the aid of a flap of renal capsule the defective intrarenal pelvis was closed. A rim of markedly thinned-out cortex of the lower pole the size of a quarter was removed permitting access to an immensely dilated lower collecting system to which the upper end of the isolated ileal segment was anastomosed by interrupted everting stitches. The lower segment was anastomosed low on the posterior wall of the bladder after removal of a generous portion of detrusor muscle. The nipple technique of ten Cate 2 to prevent reflux was used. The ileal anastomoses' at kidney and bladder levels were extraperitoneal, but the main course of the ileal segment was transperitoneal, entering and leaving through generous mesenteric and peritoneal windows. A suprapubic tube was left in place, and a No. 16 Silas tic transileal splint was brought out through the bladder. The postoperative course was uneventful. Levels of serum calcium and serum uric acid have remained normal, although the patient continues to have idiopathic hypercalciuria (380 mg. per 100 ml. average of three collections). The patient was discharged on the twenty-first postoperative day, the splint and tube had been removed, and electrolytes were within normal limits. He convalesced satisfactorily and returned to work for the first time in a year 2 months after operation. Six weeks later, chills, fever, nausea, vomiting, and weakness developed. He was readmitted and found to be markedly hypokalemic and dehydrated and to have hyperchloremic acidosis. The level of blood urea nitrogen was 36 mg. per 100 ml. An infusion pyelogram showed increased hydronephrosis with a dilated and tortuous ileal segment (Fig. 3B). Attempts to pass a catheter through the nipple ileostomy were unsuccessful. At open operation no catheter could be passed through the everted nipple intravesical ileostomy. This was disconnected, and the ureter straightened, shortened, and directly anastomosed to the same low posterior bladder site, extraperitoneally, without a nipple. A No. 22 Silas tic catheter was easily passed to the renal pelvis and left in place for 1 month. The patient was readmitted at the end of this time, at which time the ileal splint was removed, having served as a suprapubic cystotomy tube during this interval as well. The hyperchloremic acidosis had completely cleared, without the use of alkali, and has not recurred. Although the patient has had one bout of chills and fever, it promptly responded to oral medication. He still takes nitrofurantoin orally and has had no further hyperchloremic acidosis, anemia, or alteration of serum creatinine or blood urea nitrogen levels 1V2 years later (Fig. 3C).
Comment. Pyelolithotomy through a small intrarenal pelvis can be exceedingly difficult. We have found that osmotic diuresis, control of the renal pedicle, generous freeing up of the kidney, and development of the pelvis by the use of vein retractors on the overhanging parenchyma have been helpful. The use of a nipple ileostomy at the bladder level according to the method of ten Cate seemed justified and prevented reflux but caused significant obstruction, pyelonephritis, and severe
ILEAL REPLACEMENT OF THE URETER IN SOLITARY KIDNEY
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Figure 3. A , Preoperative ileal replacement of left ureter. Note necessary indwelling ureteral catheter. B, Left ileal ureter six weeks after operation. Note dilation, tortuosity, and retention. C, Left ileal ureter 1'/2years later.
hyperchloremic acidosis and had to be removed. A direct ileovesicostomy was performed. At the time of the reimplantation of the revised ileal segment and removal of the nipple ileovesicostomy, a wedge resection of the bladder neck was performed because of a pea-sized cellule in the right recess of the bladder that was present even before creation of the substitute ureter. The patient has had no apparent difficulty voiding ileal mucus, and has done well clinically over the past 18 months .
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DISCUSSION The accessibility, mobility, vascularity, and peristaltic activity of the ileum as a substitute ureter commend its use. However, absorption of electrolytes, dilatation and stasis, the formation of mucus, and vesicoileal reflux are disadvantages. The construction of a narrowed segment of ureter and submucosal reimplantation combined with scraping off (removal) of the mucous membrane may help to prevent these undesirable side effects. Our experience here with nipple intravesical ileostomy was not favorable, for although it prevented reflux, it created obstruction. With proper construction, fashioning is possible and it could be made to work. Transileal postoperative splints were used as a safety factor because of two anastomotic sites that may leak, particularly in patients with solitary kidneys. It is interesting that the ileal ureter dilates only to a degree and then elongates when there is distal anatomic or functional obstruction. Tapering the ureter, modifying the Swenson-Fisher technique> (Fig. 4) has much to commend it and would be used by us if confronted by these circumstances in the future. Detrusor hypertrophy and relative bladder neck obstruction seem to develop in men. Whether this is because of difficulties in passing the ileal mucus or from the gaping vesicoileal reflux is not certain. Women pass the mucus without difficulty. One cannot replace a segment of ureter with ileum in the upper or middle segments or both and anastomose it to the lower ureter because of mucus obstruction and ineffective peristaltic progression of the urine. We have not noted any diminution in the ileal mucus by changing the pH of the urine. It is important to remove an adequate segment of
Figure 4. Modification of the Swenson-Fisher technique with tapering of the ureter.
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detrusor muscle when implanting the ileum low in the posterior wall of the bladder. It is wise to do a transvesical anastomosis and be sure that the bladder is normal and unobstructed. No permanent suture material should be used when the segment will be involved in conduction of urine. There seems to be no difficulty with the intraperitoneal course of the ureter provided anastomoses at kidney and bladder levels are well extraperitonealized and their exit and entrance to the peritoneum are through generous mesenteric and peritoneal windows. In the construction of the ileal ureter it is important to avoid tension, torsion, and angulation. Although we did not have to resort to it, ileal replacement of the right ureter may require placement of the ileal segment in an antiperistaltic manner.
SUMMARY Three instances of ileal replacement of the ureter involving both right and left sides, male and female, child and adult, in patients with, in essence, a solitary kidney, are reviewed. Follow-up periods of 51f2, 21f2, and 11/2 years are available. The actual and theoretical pitfalls are reviewed and their avoidance discussed. This operation should be used only when simpler measures of ureteral repair have failed or are manifestly impracticable.
REFERENCES 1. Goodwin, W. E., Winter, C. C., and Turner, R. D.: Replacement of the ureter by small intestine: clinical application and results of the ileal ureter. J. Urol. 81 :406-418 (March) 1959. 2. Janknegt, R. A., and Cate, H. W., ten: The ileal nipple as a reflux-preventing mechanism in ileal replacement of the ureter. Arch. Chir. Neerl. 19:187-196, 1967. 3. Ormond, J. K.: Idiopathic retroperitoneal fibrosis: a discussion of the etiology. J. Urol. 94:385-390 (Oct.) 1965. 4. Suby, H. I., Kerr, W. S., Jr., Graham, J. R., et al.: Retroperitoneal fibrosis: a missing link in the chain. J. Urol. 93:144-152 (Feb.) 1965. 5. Swenson, 0., Fisher, J. H., and Cendron, J.: Megaloureter: investigation as to cause and report on results of newer forms of treatment. Surgery 40:223-233 (July) 1956. 6. Wells, C. A.: The use of the intestine in urology, omitting ureterocolic anastomosis. Brit. J. Urol. 28:335-350 (Dec.) 1956.
Ileal Replacement of the Ureter in Solitary Kidney
Joseph B. Dowd, M.D., and Fusen Chen, M.D.
The ingenuity, experience, and skill of the urologic surgeon are occasionally contested. One of the less common of these challenges occurs when the ureter may become damaged or be deficient at a level too high to permit reimplantation in the urinary bladder. This may occur as a result of an anomaly, such as agenesis (Case 1), a long aperistaltic distal segment, severe stenosis, ureteral valve, or dysplasia. More commonly, however, it is the result of injury, either from without, such as blunt or penetrating missiles, or from within. In this category are ureteral compromise as during or after surgery of the retroperitoneum or pelvis for tumors (bowel, cervix, uterus), the surgery for aneurysms, fistulas, stone manipulation, pyeloureterolithotomy (Case 3), or ureteral ischemia from purposeful (Case 2) or incidental ureterolysis. Long stricture may occur consequent to denuding as in lymphadenectomy or after pelvic packing or sepsis with extravasation, multiple pelvic operations, irradiation, or tuberculous ureteritis, with resultant destruction of the lumen at several levels. Occasionally the distal half or more of the ureter may be resected because of involvement by extrinsic tumor or even electively resected because of the presence of a low-grade primary ureteral tumor with evidence that it did not seed from the renal pelvis above. Rarely, the surgeon is taxed because of inadequate length of the ureter when it is desirable to transplant a ureter back into the bladder after prior diversion to either bowel or skin. Whatever the cause of ureteral deficiency or irreversible damage, the surgeon has the choice of ipsilateral nephrectomy, permanent nephrostomy, high cutaneous ureterostomy, autotransplantation, or substitution of the ureter. Ipsilateral nephrectomy in general is undesirable, violating as it does the urologist's basic principle of preserving renal parenchyma. At times, particularly in palliation when life span is limited, it may be warranted and justifiable. It is, however, not an alternative when the patient has a solitary kidney (Cases 1 and 3), or when there is inadequate contralateral renal tissue to sustain life (Case 2). Surgical Clinics of North America- Vol. 51, No.3, June 1971
739
740
JOSEPH B. DOWD AND FUSEN CHEN
Nephrostomy, an immediate solution, is undesirable as a permanent diversion because of the threat to the patient in regard to infection, diminished function, difficulties in keeping the tube in place, keeping the patient dry, and adjusting to the inaccessible external drainage apparatus. Cutaneous ureterostomy is fraught with complications and commonly results in permanent intubation because of abdominal wall level strictures. Appliances for this uncommon form of adult diversion are notoriously inefficient in keeping the patient dry and relatively unlimited in activity. The same renal insult is present in regard to infection and loss of function as with permanent nephrostomies. Autotransplantation of the kidney to the pelvis has merit, although it is not applicable when the upper one third to one half of the ureter is not available. However, great redundancy of an extrarenal pelvis may permit connection to a bladder flap. Not every surgeon would undertake autotransplantation, especially in a patient with solitary kidney. Not infrequently in these patients (Case 3), the renal pedicle is fixed so as to preclude autotransplantation. Although veins and artificial substitutes for the ureter have been used, their application is experimental and not theoretically sound. Transureteroureterostomy is not applicable if the proximal half of the ureter is not available or in instances of congenital solitary kidney. Nor is it a certainty that this procedure does not compromise the contralateral side if present, even if technically possible in the presence of proximal ureteral damage. High ureterocolic anastomosis is mentioned only to condemn it as unwise, prone as it is to pyelonephritis with or without reflux, to hyperchloremic acidosis, and to potassium deficiency. Under any circumstances it is unwise in the presence of a solitary kidney. We are thus left with isolated segments of distal small bowel as substitute ureters.!. 6 They have much to commend them-accessibility, mobility, vascularity, and peristaltic activity. These segments are not ideal, however, because of problems of electrolyte absorption, dilatation and stasis, mucus formation, and reflux.
CASE REPORTS CASE 1. An 8 year old boy was referred to us on April 16, 1963, with a left nephrostomy tube (No. 14 French Foley catheter) that was increasingly difficult to replace, frequently becoming dislodged despite great limitation of the child's activity. He was having frequent attacks of chills, fever, and left flank pain despite maintenance antiseptic therapy. The patient was born after a normal gestation and delivery. He was anuric the first 24 hours, and the level of blood urea nitrogen the second day of life was more than 60 mg. per 100 ml. An attempted intravenous pyelogram revealed no excretion of dye or renal shadows. No visible external anomalies were noted. The right flank was explored and revealed absence of the right kidney as such, although a 1.0 cm. nubbin of embryonic fibrous tissue at the end of a blind-ending ureter was found at the level of the second lumbar vertebra. The ureter "seemed normal below that point." Following immediate left nephrostomy, the infant's condition promptly improved, and the level of blood urea nitrogen dropped to
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normal limits. All subsequent pyelograms revealed massive hydronephrosis with a sizable extrarenal pelvis and adequate cortex but no descent of dye to a ureter. Cystoscopy at age 2 showed absence of the trigone and a normal right ureteral orifice. Retrograde ureterography showed the right ureter to end blindly at the level of the second lumbar vertebra. No left ureteral orifice was observed. Left flank exploration at age 3'12 failed to locate a left ureter. At this time the child was neither anemic nor azotemic and was well developed but small for his age. Culture from the nephrostomy tube revealed resistant Proteus vulgaris and Pseudomonas aeruginosa. A voiding cystourethrogram revealed a normal capacious bladder without reflux, trabeculation, or residuum. Although the bladder had never functioned as such, the child had normal urinary control. Cystometrography revealed normal findings; at subsequent cystoscopy, no obstruction was present and no left ureteral orifice was found. The right blind-ending ureter extended to the level of the second lumbar vertebra but was inadequate to reach the left renal pelvis. At transabdominal exploration, a fibrous strand without a lumen replaced the upper third of the ureter, a 5.0 cm. fusiform segment represented the midureter where it ended blindly, and no ureter was found from the iliac vessels to the bladder. Adequate renal cortex and a redundant extrarenal pelvis were present. An isolated segment of ureter, 20 cm., was prepared, and anterior ureteroureterostomy was accomplished. The proximal end of the prepared isolated segment was anastomosed to the redundant renal pelvis obliquely, and the shortest, straightest, direct course to the left posterior wall of the bladder was accomplished. The ileovesical anastomosis was accomplished end to side transvesically. A No. 18 Levin tube splint traversed ileopelvic and ileovesical anastomoses and was brought through a right abdominal stab wound. A No. 18 French nephrostomy tube and suprapubic tube were also placed. The anastomoses at kidney and bladder levels were extraperitoneal, but the main course of the ureter was intraperitoneal through generous mesenteric and parietal peritoneal windows. The postoperative course was uneventful. The transileal splint was removed on the twelfth postoperative day. The suprapubic tube on the fifteenth postoperative day, and the left nephrostomy tube was removed 1 month later in the office. The cystostomy and nephrostomy stab wounds promptly healed. Although there was occasional difficulty voiding mucus, with double voiding and administration of maintenance doses of nitrofurantoin (Furadantin), the clinical course was unremarkable. Interval checks revealed continued infection, increasing bladder residual urines, and dilatation followed by tortuosity of the ileal ureter. Levels of electrolytes and blood urea nitrogen remained normal. In July 1967, transurethral resection of hypertrophied bladder neck was performed through a perineal urethrostomy. Triple voiding was urged but rarely followed. Blood counts, including electrolytes, blood urea nitrogen, and serum creatinine have remained normal, but the ileal segment remains dilated and elongated. Enforced triple voiding shows improvement to less than 200 ml. of residual urine, but this is rarely performed by the patient. The ileal segment will be brought to the skin at the end of the current school year. There has been unusual physical development and athletic activity, including contact sports, since conversion from a permanent nephrostomy to an ileal substitute ureter (Fig. 1).
Comment. There were a number of interesting facets in the findings and management of this child. The right ureter unfortunately did not reach the left renal pelvis. At the age of 8, the first time fluid had been placed in his bladder, he demonstrated normal urinary control without residuum. Bladder hypertrophy developed with relative bladder neck obstruction that may have been the result of intermittent obstruction of the ileal mucus or the presence of a pseudodiverticulum, refluxing up the capacious left ureter, via the gaping ileovesicostomy. Voiding improved with the transurethral resection, but ileal decompensation and residual urine recurred. The ileal mucus has not diminished in 8
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JOSEPH B. DOWD AND FC5L.'-1 CHEN
Figure 1. A, Left ileal ureter, immediate postoperative view. B, View 51/2 years after operation.
years, although triple voiding is demonstrably helpful, with voidings of 800 ml. followed by 200 ml. in 3 to 5 minutes, followed by an additional 130 to 200 ml. in 3 to 5 minutes. A residual urine of 200 ml. remains. It took almost 3 years to sterilize the urinary tract with continual administration of nitrofurantoin (Furadantin). In recent years, orally administered nitrofurantoin macrocrystals (Macrodantin) maintenance therapy is well tolerated and has been continued because of the iliectasia of the substitute ureter and occasional low colony count of organisms on culture. CASE 2. A 49 year old extremely obese woman was found to have a hypoplastic left kidney and grade 1 right hydroureteronephrosis in April 1966 when a pyelogram was taken because of steady use of methysergide N.F. (Sansert). The patient was urged to discontinue methysergide N.F., and alternate medication for migraine headaches was prescribed. In December 1967 she was referred by a psychiatrist because of anemia, oliguria, and right flank pain. Infusion pyelography revealed bilateral hydroureteronephrosis. The patient had continued to use methysergide in increasing doses despite the warning 19 months earlier. A right nephrostomy was performed. Twelve days later a transabdominal bilateral ureterolysis was performed. On the tenth postoperative day, because of acute bacteremic shock, a right nephrostogram (Fig. 2A) revealed extravasation of dye at the midureter level. Transabdominal exploration revealed a foul intraperitoneal and retroperitoneal abscess in which the tip of the appendix was found free. Approximately 6.0 cm. of ureter was found ischemic, possibly denuded at the time of ureterolysis. Urinary extravasation was obvious. After a stormy course, including the use of large doses of antibiotics and steroids, the patient recovered and was discharged with a nephrostomy tube in place. She returned 3 months later, and on March 29, 1968, at transabdominal exploration an isolated segment of ileum was prepared, and posterior enteroenterostomy was carried out. The proximal end of the ileal segment was anas-
!
ILEAL REPLACEMENT OF THE URETER IN SOLITARY KIDNEY
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\
Figure 2. A, Right nephrostogram with extravasation. B, Right ileal ureter 2'12 years later.
tomosed retroperitoneally to viable though dilated ureter, 2.0 cm. below an unobstructed ureteropelvic junction. The ureter was brought through a generous window in the right colic mesentery and posterior parietal peritoneum. The most direct intraperitoneal course of ileum was then directed to the bladder where it was anastomosed low on the posterior wall extraperitoneally by direct transvesical mucosa-to-mucosa anastomosis without a nipple. A No. 16 silas tic splint traversed the ileal ureter and was brought through a left suprapubic stab wound. A protective suprapubic cystotomy tube was placed, and the right nephrostomy tube was left in place. A generous window of bladder was excised at the ileovesical anastomotic site. The postoperative course was uneventful. The patient passed mucus with ease. The hydronephrosis resolved within a month after removing all splints. The patient has been followed up at 3-month to 6-month intervals and remains comfortable. The urine has been sterile since 5 months after operation. After 22 months mild hyperchloremic acidosis has appeared. Some improvement in the apparent function of the left kidney is noted pyelographically 21(2 years later (Fig.2B).
Comment. The relationship between retroperitoneal fibrosis and prolonged use of methysergide N.F. is well documented. 3 , 4 The improvement in the apparent function of the hypoplastic left kidney may be the result of ureterolysis, or, in terms of renal counterbalance, improved because of demand from diminished function of the hypertrophic right kidney. Despite the immense size of the patient and the complex intraperitoneal findings due to perforated appendix and ureteral ischemia from overzealous ureterolysis, the anastomosis both at the kidney and bladder levels was easily carried out by an adequate xiphoid-to-pubis incision. There has been no problem voiding the mucus, presumably because of the patient's normal bladder and unobstructed urethra, unlike
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the difficulties found in men. The ileal mucus shows no tendency to diminish to date. CASE 3. A 34 year old man was referred to us on July 7, 1969, with a No.5 French ureteral catheter in place as it had been for most of the preceding 9 months. The patient had increasing renal insufficiency and refractory urinary tract infections. At the age of 23 while in military service, he had a right nephrectomy for "infected kidney stones." The left upper ureter and small intrarenal pelvis had apparently become damaged as a result of two attempts to extract obstructing calculi from the small left intrarenal pelvis, resulting in destruction of the left intrarenal pelvis, ureteropelvic junction, and proximal 4.0 cm. of ureter (Fig. 3A). On July 16, 1969, autotransplantation or ileal substitute ureter was planned. Because the left renal pedicle was densely scarred and technically impossible to dissect, an isolated 25 cm. segment of ileum was prepared. All stones were removed from the intrarenal pelvis, and with the aid of a flap of renal capsule the defective intrarenal pelvis was closed. A rim of markedly thinned-out cortex of the lower pole the size of a quarter was removed permitting access to an immensely dilated lower collecting system to which the upper end of the isolated ileal segment was anastomosed by interrupted everting stitches. The lower segment was anastomosed low on the posterior wall of the bladder after removal of a generous portion of detrusor muscle. The nipple technique of ten Cate 2 to prevent reflux was used. The ileal anastomoses' at kidney and bladder levels were extraperitoneal, but the main course of the ileal segment was transperitoneal, entering and leaving through generous mesenteric and peritoneal windows. A suprapubic tube was left in place, and a No. 16 Silas tic transileal splint was brought out through the bladder. The postoperative course was uneventful. Levels of serum calcium and serum uric acid have remained normal, although the patient continues to have idiopathic hypercalciuria (380 mg. per 100 ml. average of three collections). The patient was discharged on the twenty-first postoperative day, the splint and tube had been removed, and electrolytes were within normal limits. He convalesced satisfactorily and returned to work for the first time in a year 2 months after operation. Six weeks later, chills, fever, nausea, vomiting, and weakness developed. He was readmitted and found to be markedly hypokalemic and dehydrated and to have hyperchloremic acidosis. The level of blood urea nitrogen was 36 mg. per 100 ml. An infusion pyelogram showed increased hydronephrosis with a dilated and tortuous ileal segment (Fig. 3B). Attempts to pass a catheter through the nipple ileostomy were unsuccessful. At open operation no catheter could be passed through the everted nipple intravesical ileostomy. This was disconnected, and the ureter straightened, shortened, and directly anastomosed to the same low posterior bladder site, extraperitoneally, without a nipple. A No. 22 Silas tic catheter was easily passed to the renal pelvis and left in place for 1 month. The patient was readmitted at the end of this time, at which time the ileal splint was removed, having served as a suprapubic cystotomy tube during this interval as well. The hyperchloremic acidosis had completely cleared, without the use of alkali, and has not recurred. Although the patient has had one bout of chills and fever, it promptly responded to oral medication. He still takes nitrofurantoin orally and has had no further hyperchloremic acidosis, anemia, or alteration of serum creatinine or blood urea nitrogen levels 1V2 years later (Fig. 3C).
Comment. Pyelolithotomy through a small intrarenal pelvis can be exceedingly difficult. We have found that osmotic diuresis, control of the renal pedicle, generous freeing up of the kidney, and development of the pelvis by the use of vein retractors on the overhanging parenchyma have been helpful. The use of a nipple ileostomy at the bladder level according to the method of ten Cate seemed justified and prevented reflux but caused significant obstruction, pyelonephritis, and severe
ILEAL REPLACEMENT OF THE URETER IN SOLITARY KIDNEY
745
Figure 3. A , Preoperative ileal replacement of left ureter. Note necessary indwelling ureteral catheter. B, Left ileal ureter six weeks after operation. Note dilation, tortuosity, and retention. C, Left ileal ureter 1'/2years later.
hyperchloremic acidosis and had to be removed. A direct ileovesicostomy was performed. At the time of the reimplantation of the revised ileal segment and removal of the nipple ileovesicostomy, a wedge resection of the bladder neck was performed because of a pea-sized cellule in the right recess of the bladder that was present even before creation of the substitute ureter. The patient has had no apparent difficulty voiding ileal mucus, and has done well clinically over the past 18 months .
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DISCUSSION The accessibility, mobility, vascularity, and peristaltic activity of the ileum as a substitute ureter commend its use. However, absorption of electrolytes, dilatation and stasis, the formation of mucus, and vesicoileal reflux are disadvantages. The construction of a narrowed segment of ureter and submucosal reimplantation combined with scraping off (removal) of the mucous membrane may help to prevent these undesirable side effects. Our experience here with nipple intravesical ileostomy was not favorable, for although it prevented reflux, it created obstruction. With proper construction, fashioning is possible and it could be made to work. Transileal postoperative splints were used as a safety factor because of two anastomotic sites that may leak, particularly in patients with solitary kidneys. It is interesting that the ileal ureter dilates only to a degree and then elongates when there is distal anatomic or functional obstruction. Tapering the ureter, modifying the Swenson-Fisher technique> (Fig. 4) has much to commend it and would be used by us if confronted by these circumstances in the future. Detrusor hypertrophy and relative bladder neck obstruction seem to develop in men. Whether this is because of difficulties in passing the ileal mucus or from the gaping vesicoileal reflux is not certain. Women pass the mucus without difficulty. One cannot replace a segment of ureter with ileum in the upper or middle segments or both and anastomose it to the lower ureter because of mucus obstruction and ineffective peristaltic progression of the urine. We have not noted any diminution in the ileal mucus by changing the pH of the urine. It is important to remove an adequate segment of
Figure 4. Modification of the Swenson-Fisher technique with tapering of the ureter.
ILEAL REPLACEMENT OF THE URETER IN SOLITARY KIDNEY
747
detrusor muscle when implanting the ileum low in the posterior wall of the bladder. It is wise to do a transvesical anastomosis and be sure that the bladder is normal and unobstructed. No permanent suture material should be used when the segment will be involved in conduction of urine. There seems to be no difficulty with the intraperitoneal course of the ureter provided anastomoses at kidney and bladder levels are well extraperitonealized and their exit and entrance to the peritoneum are through generous mesenteric and peritoneal windows. In the construction of the ileal ureter it is important to avoid tension, torsion, and angulation. Although we did not have to resort to it, ileal replacement of the right ureter may require placement of the ileal segment in an antiperistaltic manner.
SUMMARY Three instances of ileal replacement of the ureter involving both right and left sides, male and female, child and adult, in patients with, in essence, a solitary kidney, are reviewed. Follow-up periods of 51f2, 21f2, and 11/2 years are available. The actual and theoretical pitfalls are reviewed and their avoidance discussed. This operation should be used only when simpler measures of ureteral repair have failed or are manifestly impracticable.
REFERENCES 1. Goodwin, W. E., Winter, C. C., and Turner, R. D.: Replacement of the ureter by small intestine: clinical application and results of the ileal ureter. J. Urol. 81 :406-418 (March) 1959. 2. Janknegt, R. A., and Cate, H. W., ten: The ileal nipple as a reflux-preventing mechanism in ileal replacement of the ureter. Arch. Chir. Neerl. 19:187-196, 1967. 3. Ormond, J. K.: Idiopathic retroperitoneal fibrosis: a discussion of the etiology. J. Urol. 94:385-390 (Oct.) 1965. 4. Suby, H. I., Kerr, W. S., Jr., Graham, J. R., et al.: Retroperitoneal fibrosis: a missing link in the chain. J. Urol. 93:144-152 (Feb.) 1965. 5. Swenson, 0., Fisher, J. H., and Cendron, J.: Megaloureter: investigation as to cause and report on results of newer forms of treatment. Surgery 40:223-233 (July) 1956. 6. Wells, C. A.: The use of the intestine in urology, omitting ureterocolic anastomosis. Brit. J. Urol. 28:335-350 (Dec.) 1956.