Von Hippel-Lindau syndrome with bilateral synchronous renal cell carcinoma.
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Biomedical subjects
Publications and source records attributed to A D Amar.
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During the last 12 years 18 patients with urinary calculi were found to have associated cystic renal disease. We herein describe the difficulties in diagnosing and in treating this group, which included patients with simple single renal cysts, multiple cysts of 1 or both kidneys and polycystic renal disease. Renal calculi may be caused by or perpetuated by renal cystic disease. After spontaneous passage or surgical removal renal calculi may recur as long as renal cysts continue to cause urinary stasis owing to obstruction and distortion of the renal calices. Superimposed renal infection may cause further difficulties in diagnosis and treatment. The timely removal of calculi and the relief of obstruction caused by the cysts and stones are emphasized. We further recommend the judicious use of antibiotics, correction of electrolyte disturbances and prolonged followup in an attempt to prevent future calculous disease.
Ureteral strictures occurring after ureterolithotomy may not be recognized until obstruction or recurrent stone impaction occurs in the area. We describe our treatment of this problem in 9 patients. Management varied from conservative treatment in patients having no discernible renal function on the affected side to various forms of reconstruction or reimplantation, depending upon the situation and site of the stricture. All patients were followed for a minimum of 2 years without complications. The etiopathogenesis and other methods of treatment available for this problem are discussed.
Recurrent urinary tract infection was seen in a 3-year-old girl with a ureterocele at the lower end of the upper segment ureter and reflux into the lower segment ureter of a duplicated kidney on the left side. We combined ipsilateral ureteroureterostomy (end-to-side anastomosis) with reimplantation of the host, single ureter into the bladder distal to the anastomosis to reduce dilatation, correct the reflux and keep the patient free of infection without medication.
In male patients with extravesical ureteral ectopia the ectopic orifice does not cause urinary incontinence and the diagnosis is often delayed. We describe our experience with 12 male patients who had extravesical ureteral ectopia. According to the embryologic phenomena leading to such anomalies we have classified them into mesonephric ductal ectopia and urogenital sinus ectopia. Distinctive clinical and radiologic features of these 2 types of ureteral ectopia are discussed. Guide lines to surgical therapy are based upon the type of ectopia, function of the affected kidney or renal segment and whether a single or duplicated ureteral system is affected.
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A case of ureteral ectopia into a cystic seminal vesicle with ipsilateral renal dysgenesis is presented. Related embryologic events, clinical manifestations, diagnosis and management are discussed. Associated ipsilateral absence of the testes with this syndrome has not been reported previously.
Roentgenographic visualization of a calculus in the distal ureter is often made difficult by gas or bowel contents in the region of the pelvis. Filling the bladder with sterile water raises the bladder dome and displaces the bowel upward. Any calculus in the lower 4 to 5 cm. of the distal ureter is then clearly demonstrated on roentgenograms taken against the water-filled bladder instead of against the bowel filled with gas and feces. This maneuver also aids in differentiation of a calculus in the distal ureter from a phlebolith in the bladder wall, and has improved visualization of distal ureteral calculus in 50 patients during the last six years.
For treatment of simple ureterocele in children an operative technique is presented which is designed to eliminate the ureterocele and prevent future vesicoureteral reflux. This technique combines an intravesical with an extravesical approach to the distal ureter. The ureterocele is excised and the ureter reimplanted in the bladder at a different site. The exposure of the distal ureter allows tailoring to reduce its caliber, if needed, before reimplantation. During the last eight years 7 children with ureterocele of a single ureter have been treated with this technique. In each of the 7 children the objectives of the operation have been achieved, as proved by postoperative follow-up.
Delayed recurrence of reflux was discovered in 4 children among 103 who were followed at least 5 years after ureteral reimplantation for treatment of vesicoureteral reflux. In each of the 103 children initial success in correction of reflux was proved by 2 cystographic series performed 3 months apart after the initial ureteral reimplantation. Reoperation was successful in correction of the recurrent reflux in 2 children, failed in 1 and has been attempted in 1. Prolonged postoperative followup (to at least 5 years) is recommended after ureteral reimplantation for treatment of vesicoureteral reflux.
Ipsilateral ureteroureterostomy near the bladder (end-to-side anastomosis) for treatment of single ureteral disease in patients with complete ureteral duplication has been performed in 16 patients during the last 8 years. The operative technique, indications, results, followup and complications are presented. Reflux in the lower segment ureter was treated in 13 patients and upper segment ectopia or ureterocele was managed in 3 patients with this operative procedure. This operative technique is simpler and safer than reimplantation of both ureters into the bladder. Because no dissection of the bladder wall is needed there is less risk of injury to the pelvic viscera and vasculature. The chance of success in correcting reflux appears better than with reimplantation of both ureters into the bladder. Long-term followup has shown few complications and no repeat operations were needed in this series. The small stump of the diseased ureter left behind near the bladder caused no serious problems.
Of 37 adult patients with ureteroceles, 13 also had calculous disease (35 per cent). Of these 13 cases 7 had single ureters and 6 had duplicated ones. None had a metabolic or urinary abnormality such as hypercalcemia, gout, hypercalciuria or hyperuricuria. Two of the patients were a mother and daugher--the first reported familial incidence of ureterocele with calculous disease. A surgical technique is described for removal of the calculus, excision of the ureterocele and reimplantation of the ureter. The procedure was used in 4 of the 7 patients with single ureters, while the stone passed spontaneously in 2 patients and was treated by ureterolithotomy in 1. A modification of the technique was used in 2 of the 6 patients with duplicated ureters but other surgical procedures were used in the remaining 4. Of 10 stones that were analyzed 2 were struvite and none contained cystine or uric acid. Long-term followup is a requisite to assure control of this clinical entity.
Congenital hydronephrosis of the lower segment in duplex kidney was treated in 12 patients. This uncommon abnormality carries the risk of calculous formation, chronic urinary tract infection, and functional failure of the involved segment. Diagnostic problems encountered included differentiation from tumor, obstruction by a calculus, and hydronephrosis due to reflux. Treatment was individualized according to severity of hydronephrosis and presence or absence of specific complications. Mild obstruction could be treated nonsurgically while the patient was closely observed for evidence of stone, infection, or worsening of obstruction. Available surgical procedures include end to side or side to side pyeloureteral anastomosis, plastic widening of a narrowed obstructive site, removal of calculus with relief of obstruction, and heminephrectomy.
Early recognition, adequate treatment, close observation, and systematic follow-up over a long period are essential to renal salvage in the child with vesicoureteral reflux. The decision whether and when to operate is made on a balance of factors by the pediatrician-urologist team. Among 236 patients with reflux diagnosed during childhood, reflux was stopped in 55 per cent of those treated medically and in 98 per cent of the more severe cases who were treated by ureteral reimplantation. Chronic pyelonephritis appeared or worsened during medical management in 12 per cent; no child showed new pyelonephritic scars or worsening of pyelonephritis after ureteral reimplantation.
Vesicoureteral reflux ceased after eradication of a reservoir of infection in 6 patients. In each case the reflux was mild, the ureter with reflux was not markedly dilated and no ureter had a golfhole orifice. A reservoir of infection should be sought for in adults with chronic or recurrent urinary tract infection and reflux. If it is found and eliminated ureteral reimplantation can be withheld while the patient is followed closely. However, if infection, reflux and associated pyelonephritis persist reimplantation may be necessary.
Of 250 children with vesicoureteral reflux, 23 had a renal caliceal diverticulum in one or both kidneys. In a third of these 23 children this was the only renal change noted and in the remaining two-thirds other changes of chronic pyelonephritis were seen. The presence of a caliceal diverticulum is evidence that renal damage of some degree is associated with reflux and urinary tract infection, and should be added to existing criteria for investigation of reflux in a child.
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