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Urinary Tract Reconstruction Patient 5: Undiversion by Removing an Ileal Loop Done for Failed Ureteral Reimplants

This 13-year-old boy was referred in 1976 for reconstructive surgery of an ileal loop done after ureteral reimplantation had failed in two prior hospitals 5 years previously. As an infant, he had been treated successfully for HIrschsprung’s Disease by colon resection and pull through. It was presumed that he had a neuropathic bladder.

Preoperative evaluation showed a 100-ml bladder, with good sensation and ability to empty the bladder when it was filled with saline.

Several useful maneuvers are illustrated in this operation. First, is the use of transureteroureterostomy, which we have used extensively. Care must be taken to avoid wedging the crossing ureter beneath a mesenteric vessel, especially the left colic artery. The loop was tapered to substitute for loss of right ureter.

Comment from Dr. Hendren: Patients with small bowel in the urinary tract very commonly will develop one or more well defined strictures which may need dilatation or even cutting as he did. This will require careful long-term observation.

Anatomy (Select Image for High-quality Version). (Top) Before and (Bottom) after reconfiguring his urinary tract.

X-ray (Select Image for High-quality Version). Antegrade study via left kidney. Note discrete narrowing of tapered bowel loop just prior to its entry to bladder. He is allergic to I.V. contrast. Subsequent evaluations have been done by ultrasonography.

Urinary Tract Reconstruction Patient 4: Undiversion Using Gastric Augmentation

This 3-year-old girl was referred for reconstructive surgery in 1988. Born with severe bilateral obstructive megaureters with hydronephrosis, bilateral end ureterostomies had been performed at age 1 month. The ureterostomies did not drain well. Therefore, bilateral loop cutaneous ureterostomies were performed at age 1 year. Hydronephrosis improved; the ureterostomies were then closed, but severe hydronephrosis recurred.

Preoperative assessment showed a very small bladder volume of only 10 ml. When contrast was introduced through the end ureterostomy stomas, the ureters drained well, but contrast remained in the kidneys, indicating functional ureteropelvic junction obstruction bilaterally.

At reoperation the better ureter on the left was reimplanted with a long tunnel into her tiny bladder, together with a psoas hitch. The right side was drained by a transureteral ureterostomy to the left. Both upper ureters were obstructed from angulation at the site of the ureterostomy closures. Therefore, bilateral dismembering pyeloplasties were performed, maintaining adequate ureteral blood supply in the surrounding periureteral tissues. The bladder was augmented with a large segment of stomach based on the right gastric epiploic artery. This 13-hour operation was well tolerated. Bladder emptying was performed initially by intermittent catheterization by her mother. Gradually she learned to void completely, and catheterization was stopped.

Postoperative studies showed some reflux. Subureteric injection of Teflon paste beneath the single ureteral orifice stopped the reflux.

Comment from Dr. Hendren: Use of the stomach for bladder augmentation has some advantages over small bowel and colon. It is metabolically superior regarding chloride absorption and base loss, seen with segments of small bowel and colon. Those segments tend to lose bicarbonate and potassium and to a lesser extent sodium while resorbing chloride. If potassium loss is replaced as potassium chloride, bicarbonate loss can be accelerated, aggravating acidosis. Gastric mucosa secretes chloride and is therefore preferred in patients with poor renal function who cannot tolerate increased solute resorption with acidosis. The urine is usually sterile after gastrocystoplasty. The ureters are tunneled very easily into gastric mucosa which is durable and also compliant. The stomach may be the only reasonable means for reconstruction in certain patients with cloacal exstrophy who have little or no colon, and who have a short small bowel in particular. Its metabolic superiority probably makes it the augmentation of choice in patients with very poor renal function. These patients also do not have excess mucous in the urine, which is an added advantage because mucous provides a nidus for stones. Cutaneous ureterostomy was a common type of diversion in infants a few years ago. It can cause many complications however, as shown in this case.

Anatomy (Select Image for High-quality Version). (Top) Pre- and (Bottom) Postop anatomy.

X-rays (Select Image for High-quality Version). Preoperative x-ray studies. Both kidneys and ureters filled via end ureterostomies Small bladder filled via urethral catheter.(Left) Postoperative bladder studies after gastrocystoplasty. (Top Right) After filling. (Bottom Right) Voiding cystogram with good control.

X-rays (Select Image for High-quality Version). IVP 2 years postoperatively. Note: right ureter enters left just above bladder. Both ureters greatly improved in size and emptying.

Urinary Tract Reconstruction Patient 3: Male Infant with Horseshoe Kidney

This 1.5-year-old male baby was referred in February 1994 with multiple anatomic problems depicted below in the Before figure. Cutaneous vesicostomy had been performed elsewhere as an infant. Previous studies had shown a horseshoe kidney, the left side of which was dysplastic. The right ureter ended in a retrovesical “cyst.” Because of vesicoureteral reflux, he had been maintained since infancy on prophylactic antibiotics. Prolonged endoscopy, through duplex urethras, and the vesicostomy, plus appropriate radiology studies, disclosed the anatomy seen in the Before figure.

In a 10-hour operation the dysplastic right kidney was removed, together with the retro vesical “cyst” formed by the lower end of the right ureter, and the right seminal vesicle and vas. The right ureter was reimplanted, repairing the adjacent diverticulum as well. The perineal sinus tract connected with the prostatic urethra was excised. The septum between the two urethras was incised endoscopically.

At age 3 years, endoscopic cutting of the septum between his two penile urethras was performed, creating a single urethral meatus, and correcting his mild hypospadias.

Anatomy (Select Image for High-quality Version). (Top) Before undiversion. (Bottom) After undiversion.

Urinary Tract Reconstruction Patient 2: Severe Hydronephrosis in a Solitary Kidney

This 9-year-old girl was referred in February 1972. At age 5 weeks, an evaluation for failure to thrive showed a solitary right kidney with severe hydronephrosis literally filling the abdomen. Pyeloplasty was attempted, but failed. An inlying nephrostomy tube was placed and was being changed monthly.

Bladder volume was only 50 ml. Dilating it hydrostatically, although done gently, ruptured the dome. The dome was closed and pyeloplasty was performed, reducing the size of the renal pelvis. A nephrostogram 10 days later showed an easy passage of contrast to the bladder. Cystogram 4 months later showed increased bladder size, but massive reflux. The ureter was reimplanted curing the reflux. In Dec.1977, because she was small (4 feet, 3 inches tall), she was referred to an Endocrinologist who made the diagnosis of Turner’s Syndrome.

Scoliosis surgery was performed in 1980 at age 18 years. The patient has remained under close metabolic supervision; she has a stable creatinine level of 1.3.

X-rays (Select Image for High-quality Version). (Top Left) Delayed IVP as neonate showing huge right hydronephrosis. (Top Right) Nephrostogram and simultaneous retrograde pyelogram at age 9 years to assess anatomy. (Bottom Left) Nephrostogram 10 days after pyeloplasty. (Bottom Right) IVP age 15 years, note normal bladder size.

Anatomy (Select Image for High-quality Version). (Before) Dismembered pyeloplasty with reduction in size of renal pelvis. (After) Reimplantation of ureter for massive reflux 6 months later.

Urinary Tract Reconstruction Patient 1: Undiversion Case with Cloacal Anatomy

This 5-year-old girl was referred in Sept. 1980 for extensive pelvic surgery. More than half her life since birth she had been hospitalized elsewhere. She had been born with cloacal anatomy with high confluence of her bladder, two vaginas, and rectum. The right kidney had a nephrostomy tube in place because the reimplanted right ureter was obstructed. The left ureter also had an obstructed ureteral reimplant and drained via a loop ureterostomy. The bladder was severely scarred.

The first procedure was to create a urethra and bring the two vaginas to the perineum. The left ureterostomy was closed, its lower end was reimplanted, and a right to left transureteroureterostomy was done. Six months later the bladder, which did not enlarge with hydrostatic stretching, was augmented with ileum.

This case illustrates the trouble which can be created when the surgery gets started “in the wrong direction.” High vaginas require a pull thru procedure, disconnecting them and using the UG sinus as a urethra. Her early years were a nightmare existence and lost most of her renal function.

With a devoted mother to lead her, the child entered adolescence as a young woman with self assurance, doing well socially and psychologically. 

Anatomy (Select Image for High-quality Version). (Before) Preoperative anatomy when referred at age 5 years. Note prior reimplanted ureters, both obstructed. Right kidney drained by nephrostomy tube; left by loop ureterostomy. (After) Initial surgery to fashion a urethra from what had been the urogenital sinus and covered with flap from buttocks. After undiversion 6 months later by left long tunnel reimplant, and transureteroureterostomy; and ileal augmentation to increase capacity of scarred and contracted bladder.

Patient X-rays (Select Image for High-quality Version). (Top Left) Antegrade perfusion of kidneys initially. (Top Right) Showing reconstructed urethra. Malformation: Confluence-Hi (Bottom Left) and (Bottom Right) Augmentation.