Raimund Stein

Conservative surgery of renal cell tumors in 140 patients: 21 years of experience.

Operative method, course and complications were analyzed retrospectively in 140 patients who underwent a conservative operation for renal tumor between June 1969 and December 1990. In 53 patients (20 women and 33 men, mean age 61.2 years, range 38 to 77 years, with 49 renal cell carcinomas and 4 benign renal tumors) there was an imperative indication for an organ preserving operation because nephrectomy would have made dialysis obligatory. In 87 patients (29 women and 58 men, mean age 53.7 years, range 27 to 74 years, with 72 renal cell carcinomas and 15 benign renal tumors) the tumor was conservatively resected in the presence of a normal contralateral unit (elective indication) and 68 of these patients (78%) were symptom-free. In the imperative group 32 of 49 patients (65.3%) with renal cell carcinoma had no evidence of disease after a mean followup of 4.6 years. Known metastases were present in 4 of 7 patients who died of the tumors in this group. In 3 patients with an imperative indication for conservative surgery a second tumor occurred in the kidney: 2 were treated with further parenchyma sparing operations, while in 1 with poor physical condition no further measures were possible. Of 72 patients with renal cell carcinoma who underwent an elective operation 68 (94.4%) had no signs of tumor progression after a mean followup of 3.3 years. One patient died of tumor metastases, and 2 (2.7%) had tumor recurrence in the kidney requiring nephrectomy and enucleation, respectively. The 5-year cause-specific survival rates for the imperative and elective groups were 84% and 96%, respectively. Patients with a local stage T3 tumor were characterized by a significantly worse survival curve than those with a stage T1 or T2 tumor but no significant difference was noted among the various grades of differentiation.

ICUD-EAU International Consultation on Bladder Cancer 2012: Urinary diversion.

CONTEXT A summary of the 2nd International Consultation on Bladder Cancer recommendations on the reconstructive options after radical cystectomy (RC), their outcomes, and their complications. OBJECTIVE To review the literature regarding indications, surgical details, postoperative care, complications, functional outcomes, as well as quality-of-life measures of patients with different forms of urinary diversion (UD). EVIDENCE ACQUISITION An English-language literature review of data published between 1970 and 2012 on patients with UD following RC for bladder cancer was undertaken. No randomized controlled studies comparing conduit diversion with neobladder or continent cutaneous diversion have been performed. Consequently, almost all studies used in this report are of level 3 evidence. Therefore, the recommendations given here are grade C only, meaning expert opinion delivered without a formal analysis. EVIDENCE SYNTHESIS Indications and patient selection criteria have significantly changed over the past 2 decades. Renal function impairment is primarily caused by obstruction. Complications such as stone formation, urine outflow, and obstruction at any level must be recognized early and treated. In patients with orthotopic bladder substitution, daytime and nocturnal continence is achieved in 85-90% and 60-80%, respectively. Continence is inferior in elderly patients with orthotopic reconstruction. Urinary retention remains significant in female patients, ranging from 7% to 50%. CONCLUSIONS RC and subsequent UD have been assessed as the most difficult surgical procedure in urology. Significant disparity on how the surgical complications were reported makes it impossible to compare postoperative morbidity results. Complications rates overall following RC and UD are significant, and when strict reporting criteria are incorporated, they are much higher than previously published. Fortunately, most complications are minor (Clavien grade 1 or 2). Complications can occur up to 20 yr after surgery, emphasizing the need for lifelong monitoring. Evidence suggests an association between surgical volume and outcome in RC; the challenge of optimum care for elderly patients with comorbidities is best mastered at high-volume hospitals by high-volume surgeons. Preoperative patient information, patient selection, surgical techniques, and careful postoperative follow-up are the cornerstones to achieve good long-term results.

EAU Guidelines on Vesicoureteral Reflux in Children

CONTEXT Primary vesicoureteral reflux (VUR) is a common congenital urinary tract abnormality in children. There is considerable controversy regarding its management. Preservation of kidney function is the main goal of treatment, which necessitates identification of patients requiring early intervention. OBJECTIVE To present a management approach for VUR based on early risk assessment. EVIDENCE ACQUISITION A literature search was performed and the data reviewed. From selected papers, data were extracted and analyzed with a focus on risk stratification. The authors recognize that there are limited high-level data on which to base unequivocal recommendations, necessitating a revisiting of this topic in the years to come. EVIDENCE SYNTHESIS There is no consensus on the optimal management of VUR or on its diagnostic procedures, treatment options, or most effective timing of treatment. By defining risk factors (family history, gender, laterality, age at presentation, presenting symptoms, VUR grade, duplication, and other voiding dysfunctions), early stratification should allow identification of patients at high potential risk of renal scarring and urinary tract infections (UTIs). Imaging is the basis for diagnosis and further management. Standard imaging tests comprise renal and bladder ultrasonography, voiding cystourethrography, and nuclear renal scanning. There is a well-documented link with lower urinary tract dysfunction (LUTD); patients with LUTD and febrile UTI are likely to present with VUR. Diagnosis can be confirmed through a video urodynamic study combined with a urodynamic investigation. Early screening of the siblings and offspring of reflux patients seems indicated. Conservative therapy includes watchful waiting, intermittent or continuous antibiotic prophylaxis, and bladder rehabilitation in patients with LUTD. The goal of the conservative approach is prevention of febrile UTI, since VUR will not damage the kidney when it is free of infection. Interventional therapies include injection of bulking agents and ureteral reimplantation. Reimplantation can be performed using a number of different surgical approaches, with a recent focus on minimally invasive techniques. CONCLUSIONS While it is important to avoid overtreatment, finding a balance between cases with clinically insignificant VUR and cases that require immediate intervention should be the guiding principle in the management of children presenting with VUR.

The Fate Of The Adult Exstrophy Patient

Between 1968 and 1993, 101 patients with bladder exstrophy or incontinent epispadias underwent surgery at our hospital. The standard procedure was ureterosigmoidostomy and additional genital reconstruction. Of the 56 patients who have reached adulthood 45 could be interviewed regarding social integration, sexuality and fertility. All patients have a functioning urinary diversion. Of the 45 patients questioned 41 have completed vocational training or are currently in training, 3 are unemployed and 1 lives at a therapeutic center. Among the patients 29 are married or have a steady partner. All women engage in sexual intercourse and 2 have delivered 3 children by cesarean section. All men achieve erection. Of the 28 men who underwent reconstruction of the external genitalia 11 have a penile deviation, which is distressing in only 2. Only 3 of the men are dissatisfied with the cosmetic result and 33% had epididymitis, necessitating 2 orchiectomies and 3 vasectomies. No patient with reconstruction of the external genitalia can ejaculate normally or has fathered children, whereas all 5 who did not undergo genital reconstruction had normal ejaculation and 2 have fathered children. Male patients with genital reconstruction and closure of the urethra have a high risk of infertility. Our patients demonstrate that the cosmetic results after genital reconstruction are satisfactory. However, in male patients, surgery is performed at the expense of fertility. Because this corrective procedure is usually performed during childhood, the parents must be informed of these consequences before surgical correction.

Urinary tract infections in children: EAU/ESPU guidelines

CONTEXT In 30% of children with urinary tract anomalies, urinary tract infection (UTI) can be the first sign. Failure to identify patients at risk can result in damage to the upper urinary tract. OBJECTIVE To provide recommendations for the diagnosis, treatment, and imaging of children presenting with UTI. EVIDENCE ACQUISITION The recommendations were developed after a review of the literature and a search of PubMed and Embase. A consensus decision was adopted when evidence was low. EVIDENCE SYNTHESIS UTIs are classified according to site, episode, symptoms, and complicating factors. For acute treatment, site and severity are the most important. Urine sampling by suprapubic aspiration or catheterisation has a low contamination rate and confirms UTI. Using a plastic bag to collect urine, a UTI can only be excluded if the dipstick is negative for both leukocyte esterase and nitrite or microscopic analysis is negative for both pyuria and bacteriuria. A clean voided midstream urine sample after cleaning the external genitalia has good diagnostic accuracy in toilet-trained children. In children with febrile UTI, antibiotic treatment should be initiated as soon as possible to eradicate infection, prevent bacteraemia, improve outcome, and reduce the likelihood of renal involvement. Ultrasound of the urinary tract is advised to exclude obstructive uropathy. Depending on sex, age, and clinical presentation, vesicoureteral reflux should be excluded. Antibacterial prophylaxis is beneficial. In toilet-trained children, bladder and bowel dysfunction needs to be excluded. CONCLUSIONS The level of evidence is high for the diagnosis of UTI and treatment in children but not for imaging to identify patients at risk for upper urinary tract damage. PATIENT SUMMARY In these guidelines, we looked at the diagnosis, treatment, and imaging of children with urinary tract infection. There are strong recommendations on diagnosis and treatment; we also advise exclusion of obstructive uropathy within 24h and later vesicoureteral reflux, if indicated.

Bladder augmentation and urinary diversion in patients with neurogenic bladder: Non-surgical considerations

Segments from almost all parts of the bowel have been used for urinary diversion. As a result, the available absorptive surface area of the bowel is reduced, and the incorporation of bowel segments into the urinary tract may have metabolic consequences. This is an area somewhat neglected in the literature. Metabolic complications are rare, but sub-clinical metabolic disturbances are quite common. Several studies have demonstrated that some of the absorbent and secreting properties of the bowel tissue are preserved after incorporation into the urinary tract. Hyperchloraemic metabolic acidosis can occur if ileal and/or colon segments are used, as well as malabsorption of vitamin B(12) and bile acid after the use of ileal segments. These metabolic effects are not as severe as may be suspected and can be prevented by prophylactic substitution. Secondary malignancies can develop as a long-term consequence of bladder augmentation. Using colonic segments, tumours are most likely to occur at the ureteral implantation site. To prevent metabolic complications, careful patient selection and meticulous and lifelong follow up, as well as prophylactic treatment, are mandatory. Endoscopy for early detection has been recommended, starting 10 years postoperatively for patients who underwent surgery for a benign condition.

Epidemiological Survey of 214 Families With Bladder Exstrophy-Epispadias Complex

PURPOSE We sought to identify causative nongenetic and genetic risk factors for the bladder exstrophy-epispadias complex. MATERIALS AND METHODS A total of 237 families with the bladder exstrophy-epispadias complex were invited to participate in the study, and information was obtained from 214 families, mainly from European countries. RESULTS Two families showed familial occurrence. Male predominance was found among all subgroups comprising epispadias, classic bladder exstrophy and cloacal exstrophy, with male-to-female ratios of 1.4:1, 2.8:1 and 2.0:1, respectively (p = 0.001). No association with parental age, maternal reproductive history or periconceptional maternal exposure to alcohol, drugs, chemical noxae, radiation or infections was found. However, periconceptional maternal exposure to smoking was significantly more common in patients with cloacal exstrophy than in the combined group of patients with epispadias/classic bladder exstrophy (p = 0.009). Only 16.8% of mothers followed the current recommendations of periconceptional folic acid supplementation, and 17.6% had started supplementation before 10 weeks of gestation. Interestingly, in the latter group mothers of patients with cloacal exstrophy were more compliant with folic acid supplementation than were mothers of the combined group of patients with epispadias/classic bladder exstrophy (p = 0.037). Furthermore, mothers of children with cloacal exstrophy knew significantly more often prenatally that their child would have a congenital malformation than did mothers of children with epispadias/classic bladder exstrophy (p <0.0001). CONCLUSIONS Our study corroborates the hypothesis that epispadias, classic bladder exstrophy and cloacal exstrophy are causally related, representing a spectrum of the same developmental defect, with a small risk of recurrence within families. Embryonic exposure to maternal smoking appears to enforce the severity, whereas periconceptional folic acid supplementation does not seem to alleviate it. There is a disproportional prenatal ultrasound detection rate between severe and mild phenotypes, possibly due to the neglect of imaging of full bladders with a focus on neural tube defects.

Long-Term Followup of the Intussuscepted Ileal Nipple and the In Situ, Submucosally Embedded Appendix as Continence Mechanisms of Continent Urinary Diversion With the Cutaneous Ileocecal Pouch (Mainz Pouch I)

PURPOSE We analyzed stoma related complications and continence rates in patients who underwent continent urinary diversion with the cutaneous ileocecal pouch (Mainz pouch I). We compared the intussuscepted ileal nipple and in situ, submucosally embedded appendix as continence mechanisms. MATERIALS AND METHODS A total of 401 patients were included in a retrospective followup study. Continence mechanisms were the intussuscepted ileal nipple in 205 patients and the in situ, submucosally embedded appendix in 196. RESULTS A total of 144 patients (36%) required intervention for a stomal complication. Of patients who received an intussuscepted ileal nipple 34 (17%) had stomal stenosis at a mean time to first stenosis of 43.8 months, 41 (20%) had stones at a mean interval to the first stone of 62.8 months, 12 underwent reoperation for stomal incontinence, including 1 because of nipple necrosis, and 82% were completely continent. Of patients who received an in situ, submucosally embedded appendix 63 (32%) had stomal stenosis at a mean time to first stenosis of 31.4 months, 20 (10%) had stones at a mean interval to the first stone of 47.5 months, 3 underwent reoperation for stomal incontinence, 4 had appendiceal necrosis and 92% were completely continent. CONCLUSIONS Of stomal complications 63% were treated endoscopically. The higher rate of stomal stenosis with the appendiceal stoma is most likely due to the smaller diameter of the appendix. The higher rate of stone formation in patients with the intussuscepted ileal nipple is related to metal staples. Continence rates of the 2 outlets are good with somewhat larger amounts of mucous secretion from the larger stoma of the intussuscepted ileal nipple.

Social integration, sexual behaviour and fertility in patients with bladder exstrophy- a long-term follow up

After primary bladder closure or urinary diversion, other factors apart from the reconstruction itself gain importance for individuals with the exstrophy-epispadias complex: social integration and, after reaching puberty, sexuality and fertility. Between 1968 and July 1994 115 patients with bladder exstrophy or incontinent epispadias underwent surgery at our institution. A total of 104 patients could be followed, 2 of whom died in the meantime. Of the remaining 102 patients 48 attend school, 4 are in college, 40 have completed or are currently undergoing vocational training, 3 are unemployed, 1 lives in a therapeutic centre and 6 are younger than 6 years of age. A total of 95% of the patients with continent urinary diversion are continent day and night, whereas only three of five patients with a sling plasty (incontinent epispadias) or with primary bladder closure followed by a Young-Dees procedure are continent. None of the patients showed deterioration of renal function. In 25 females the external genitalia were reconstructed. Fixation of the uterus was done in 13 to correct or prevent uterine prolapse. Of the 17 women older than 18 years of age with genital reconstruction, 16 are satisfied with the cosmetic result. All adults engage in sexual intercourse. Five women have delivered seven children by Caesarean section. Of the 35 male adults 32 underwent reconstruction of the external genitalia and 34 males achieve erection. One developed necrosis of the penis early in life following primary bladder closure performed at an outside hospital. Penile deviation was present in 11 of the 32 patients with genital reconstruction, which is distressing in only 2. Thirty patients are satisfied with the cosmetic result. After genital reconstruction 9 males developed epididymitis, necessitating two orchiectomies and three vasectomies. No patient with reconstruction of the external genitalia can ejaculate normally or has fathered children, whereas ejaculation was normal in the three men who did not undergo genital reconstruction and in two patients prior to post-pubertal reconstruction. Furthermore, two of these three men have fathered four children.

Rectosigmoid Pouch (Mainz Pouch II) in Children

PURPOSE Continent anal urinary diversion is a therapeutic option in bladder exstrophy. We report our long-term results with the rectosigmoid pouch (Mainz pouch II), a modification of the classic ureterosigmoidostomy. MATERIALS AND METHODS A total of 38 children with a mean age of 5 years (range 0.5 to 17) underwent a Mainz pouch II procedure between 1991 and 2004. Most patients (33) had bladder exstrophy or incontinent epispadias. In 14 children (37%) urinary diversion was performed after failed primary reconstruction. In 6 children conversion was performed from an incontinent type of urinary diversion. Renal function, continence and metabolic changes were analyzed. A total of 35 children were followed for a mean of 112 months (range 5 to 147). RESULTS All children were continent during the daytime but 3 (8.6%) suffered from nighttime incontinence requiring pads. With respect to the upper urinary tract, 6 children (15.8%) had development of pyelonephritis, mostly with stenosis of the ureterointestinal anastomosis. Reimplantation of the ureter was required in 10 of 69 RU (14.5%), of which 7 (10.1%) were due to ureterointestinal stenosis and 3 (4.3%) were due to reflux. Serum creatinine was within normal limits in all children. During followup acid-base balance was monitored, and early alkali supplementation was initiated in 24 of 35 children (69%) when the base excess was less than -2.5 mmol/l. One child had development of clinical acidosis requiring hospitalization. After followup of more than 10 years annual rectosigmoidoscopy was performed in 16 children/young adults without pathological findings. CONCLUSIONS The Mainz pouch II procedure for children with genitourinary anomalies promises excellent continence rates. However, periodic followup studies are important to check the upper urinary tract and prevent metabolic acidosis. Due to the risk of malignancy at the ureterointestinal anastomosis, endoscopy should be performed annually beginning at postoperative year 10. The Mainz pouch II procedure is safe in the long term. Without stoma, appliance or catheterization this type of continent urinary diversion is specifically suitable for children.