Complete ureteral triplication with ectopia.

A case of complete ureteral triplication is reported and a brief review of the literature is presented.

Comparison of the ileal conduit and clean intermittent catheterization for myelomeningocele.

Clean intermittent catheterization has been shown to be a safe and effective means of regular vesical emptying in children with neurogenic bladders secondary to myelomeningocele. The major benefits appear to be the protection of the upper urinary tract, and in many children, improved urinary control. In contrast, the ileal conduit has been shown to produce significant long-term complications; the major one is deterioration of the upper urinary tract. Most children with myelomeningocele begin life with normal kidneys. Our goal of therapy, therefore, is a continent independent child with normal renal function. With proper patient selection, clean intermittent catheterization offers an excellent means of achieving this result in many children; the ileal conduit for permanent urinary diversion in children is outmoded.

Urethral reconstruction in the young female using a perineal pedicle flap.

Five young female patients with a functionally short or absent urethra are described. Two patients had an abnormal urethra related to a congenital malformation (1 female hypospadias, 1 urogenital sinus) and three patients had an absent urethra related to previous surgery. Four of the five patients were totally incontinent of urine. Urethral reconstruction was achieved by tubularizing the mucosa of the vaginal vault and swinging a full thickness pedicle skin flap from the posterior perineum and buttock to cover the urethral repair. In two patients previously diverted, undiversion was made possible. Urinary continence has been achieved in all five patients.

Urethral strictures following transurethral resection of posterior urethral valves.

Dense urethral strictures developed in 3 of 36 patients (8 per cent) following transurethral fulguration of posterior urethral valves. Common potential etiologies include the use of a loop resectoscope, large size of the valves and immediate supravesical diversion. The latter results in a "dry" urethra and the strictures that resulted may be analogous to the stenosis seen following a "dry" ureteral reimplant. Techniques to maintain flow through the urethra after resection and the avoidance of an extensive resection may be helpful in preventing urethral strictures.

Results in children managed by cutaneous ureterostomy.

A review of 59 children with severe hydronephrosis managed by cutaneous ureterostomy reveals that the procedure is safe, quick, and effective in draining the kidney. Although chronic bacteriuria is common, pyelonephritis is rare. The major drawback of this technique for temporary urinary diversion in children is that the subsequent urinary reconstruction is formidable and more difficult than primary repair. The complications of urinary diversion using this technique are low, however, and it may remain the safest form of diversion available for long-term use in children with dilated ureters.

The use of antirefluxing intestinal segments in pediatric urinary reconstruction.

Because of inadequate ureteral length or disease preventing direct ureterovesical anastomoses, antirefluxing intestinal segments were used in eight children during reconstruction of their urinary tracts. Previous urinary diversions by either ileal conduit or cutaneous ureterostomy had been done in six of the eight. In five of the children, bladder augmentation was also required because of nondistensible fibrotic bladders secondary to disuse or prior obstruction. The intestinal segments used were ileal (4), ileocecal (4), and sigmoid (1). The ileal segments were tapered along the antimesenteric border and reimplanted into the bladder with long submucosal tunnels to prevent reflux. Reflux was prevented in the ileocecal segments by plication of the normal ileocecal valve. Reflux has not developed postoperatively in any of these patients. In one patient in whom an ileal segment was used. In one patient in whom an ileal segment was used, partial obstruction occurred at the new bladder hiatus. The serum creatinine rose from 2.8 mg/dl to 3.5 mg/dl necessitating a secondary repair. Renal function and serum electrolytes have improved or remained stable in all other patients. For children who have undergone multiple previous procedures resulting in inadequate ureteral length and/or abnormal bladders, these techniques offer excellent alternatives to permanent urinary diversion.

The protean aspects of posterior urethral valves.

Thirty-six patients were evaluated and treated for posterior urethral valves. Of these patients 16 were mild valvular obstruction presented with voiding dysfunction, including 14 (88 per cent) whose symptoms resolved following transurethral valve fulguration. The excretory urograms were normal. The 10 patients with moderate sized valves had symptoms of urinary retention or urinary tract infections. Ureteral surgery was necessary in 4 patients. Following valve resection 6 of the 10 patients with severe valves were managed by diverting loop cutaneous ureterostomies. They did well clinically but dense urethral strictures developed in 3 patients. No other complications of valve resection were seen. Posterior urethral valves have protean aspects requiring individualized treatment.

Pediatric nephrectomy.

A retrospective revue of 90 consecutive nephrectomies revealed 25 instances in which the nephrectomy was done for poor or diminished function only (28 per cent). The pathology was ureteropelvic obstruction (18 cases), reflux (4 cases) and obstructive megaureter (3 cases). All conditions are reparable. Although some kidneys in children are worthless and best removed, most have significant potential function if the anatomical defect is repaired. Poor or diminished renal function in children should not be an absolute indication for nephrectomy.

Clean intermittent catheterization for home management in children with myelomeningocele.

Sixty-two children with myelomeningocele and neurogenic bladders have been managed with clean intermittent catheterization (CIC) at home for periods of 6 to 42 months. Abnormal upper urinary tracts improved or stabilized in 83 per cent of the children. There was no renal deterioration in children who began with normal upper tracts while on CIC. Bacteruria was common, but clinically not a problem, unless ureterovesical reflux was present. Urinary control was improved in 66 per cent of the children. CIC is an effective and safe method to regularly empty the urinary bladder and is an alternative to supravesical urinary diversion in many children with neurogenic bladders.

Experimental ureteral lengthening in dogs.

The right ureters of eight dogs were divided and the end of the proximal segments were attached to a spring generating a constant tension force of 26 to 30 mm Hg. After 2 weeks, the segments had elongated by an average of 33%; one segment did not elongate because of a spring failure. Six of the seven elongated segments were viable. Microscopically, the segments revealed muscular hypertrophy, increased collagen and elastic tissue, and periureteral inflammation and edema. This study indicates that a dog ureter may be mechanically elongated and its tissue viability maintained by applying a constant tension external stretching force.

Tubed free skin graft for construction of male urethra.

A tubed, free skin graft was used to construct a urethra in 49 young male subjects. Indications included 1) multiple previous hypospadias operations, 2) new severe hypospadias cases, 3) chordee without hypospadias but with a short urethra, 4) epispadias, 5) distal urethral stricture and 6) a small number of patients undergoing an initial operation for hypospadias by the Horton-Devine technique. No fistulas or leaks occurred. The over-all complication rate was low. Based on our own experience and that reported by others we believe that a free tubed graft is an excellent method for making a substitute urethra in a variety of urethral problems in children and young adults. It is important that the graft is of full or nearly full thickness because thin grafts tend to contract.

Giant hydronephrosis in children.

Twenty pediatric patients with giant hydronephrosis were reviewed. This was defined as a kidney that occupied a hemiabdomen, met or crossed the midline, and was at least 5 vertebrae in length. The majority (16) were cases with ureteropelvic junction obstruction. In 2 cases, the primary pathology was obstructive megaureter. In 2 cases, the infants had severe urethral valves with massive upper tract dilatation. In 6 patients with a normal contralateral kidney, nephrectomy was performed. In one patient with bilateral pathology, one nonfunctioning kidney was removed later. A reconstructive operation was undertaken in 14 of the 20 patients. There was one late death from septicemia in an infant male with urethral valves and bilateral dysplastic kidneys.

Ileal conduit hemorrhage secondary to portal hypertension.

The clinical features and management of 3 patients who presented with the triad of massive hemorrhage from the ileal conduit, portal hypertension due to liver disease, and portosystemic varices related to the conduits are described. One patient, a class C cirrhotic, was treated conservatively and died of blood loss and hepatic coma. Two patients were managed with splenorenal shunts initially, followed by creation of colon conduits, and are currently doing well. Surgical approximation of areas draining in the portal and systemic circulation with subsequent development or adhesion-related varices probably explains the predilection for involvement of the ileal conduit and may explain the presence of varices in mild to moderate portal hypertension before other signs of hepatic decompensation are evident. Superior mesenteric angiography with special attention directed at the venous phase is necessary to document this entity.

Urologic manifestations of Wegener granulomatosis.

Wegener granulomatosis is a disease characterized by necrotizing granulomatous angiitis involving the upper and lower respiratory tracts and the kidneys. The introduction of cyclophosphamide in the treatment of Wegener granulomatosis has dramatically altered the rapidly fatal course of the disease and has altered our thinking regarding its management. Recently we have cared for 3 patients who demonstrated a dramatic spectrum of urinary tract pathology related to Wegener granulomatosis. The cases presented offer three points for consideration by the urologist: (1) the manifestations of the disease in the genitourinary tract can be varied and can present in a dramatic fashion; (2) the rapid progression of renal failure and the nearly uniform mortality associated with the disease have changed largely because of the use of cyclophosphamide; and (3) our approach toward patients with renal failure on the basis of Wegener granulomatosis can be altered, and renal transplantation is certainly feasible with the increased longevity afforded these patients by cyclophosphamide.