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Prune-Belly syndrome is a disorder characterized by the following triad of symptoms: deficiency of the abdominal muscles, malformations of the urinary tract and bilateral cryptorchidism. This study included a total of 16 patients. The findings included clinical characteristics, diagnostics, therapy and long-term clinical outcomes. All patients were asked to complete a questionnaire and, in some cases, were given further examination. All patients were diagnosed with congenital aplasia of the abdominal wall and a variety of urogenital malformations. Cryptorchidism was present in 11 patients (68.8%), malformations of the prostate in 3 (18.8%), urethral malformations in 8 (50%) and mega-ureter in 14 patients (87.5%). A mega-bladder was observed in 13 patients (81.3%). Distinctive renal malformations, such as renal dysplasia, in 3 patients (18.8%) and hydronephrosis in 9 patients (56.3%), respectively. Abdominoplasty was performed on 4 patients (25%). Urethral surgery was performed in 10 patients (62.5%). Seven patients (43.8%) required ureter surgery, most of which involved re-implantation of the ureter and, in some cases, additional ureter modeling. Renal surgery was performed on 5 patients. Four patients with non-functioning kidneys with hydronephrosis underwent a nephrectomy and one patient pyeloplasty. We demonstrate that successful treatment is possible even in cases of serious and complex malformations, such as those of the Prune-Belly syndrome. Treatment must be tailored to the individual patient. The severity of the renal dysplasia is the main prognostic factor.

Purpose: For cutaneous fixation of the continent catheterizable vesicostomy we present a new technique, in which the umbilicus is rotated caudally by 180° and the catheterizable channel is sutured to the umbilical cone. This procedure reduces the distance between the stoma entrance and the bladder., Materials and Methods: Between 2001 and 2008, 67 patients underwent construction of a continent catheterizable vesicostomy. In 82% (n = 55), this was an appendicovesicostomy according to the Mitrofanoff principle. For 21 of these patients, later in the series (2005 onwards), the procedure involved rotation of the umbilicus. We evaluated cosmetic outcome and channel-related complication rates and compared the results between the two groups, with and without umbilical rotation., Results: The overall complication rate was 22.4% (n = 15); stoma stenosis occurred in eight cases. In the majority of cases with complications, endoscopic treatment was sufficient. The complication rate was significantly lower in the group with umbilical rotation compared to the other group (14.3% vs 26.1%, P < 0.02)., Conclusions: Umbilical rotation always enables cutaneous implantation of the vesicostomy in the umbilical pit and is a safe and straightforward procedure., (Copyright © 2010 Journal of Pediatric Urology Company. Published by Elsevier Ltd. All rights reserved.)

A 12-year-old girl presented with recurrent gross hematuria over 3 months and regular findings in sonography of the urogenital tract. The first suspected diagnosis of familial IgA glomerulonephritis was excluded by kidney biopsy. After a further episode of gross hematuria leading to vesical tamponade, the patient received magnetic resonance imaging with angiography and urography, which was suspicious for a renal tumor. Consecutively, unilateral nephrectomy was performed and a nephroblastoma was diagnosed. This case demonstrates that even in grossly normal renal ultrasound, relapsing episodes of gross hematuria can be caused by renal tumor, and therefore in unclear situations, further diagnostic is necessary., (Copyright © 2011 Elsevier Inc. All rights reserved.)

Introduction: Sexual dysfunction is a frequent complication of visceral surgery after rectal resections as a result of carcinoma of the rectum., Aim: The purpose of our study is to assess the incidence and form of sexual dysfunction in our own population of patients., Methods: The study comprised all patients who had undergone surgery for carcinoma of the rectum at the Erlangen Surgery University Hospital, Germany, in the period 2000-04. All male patients were retrospectively surveyed and asked to complete standardized (International Index of Erectile Function 15) questionnaires regarding their pre- and postsurgical sexual function. One hundred and forty-five questionnaires could be analyzed. The statistical evaluation was conducted with aid of the SPSS statistics program. The univariate analysis was carried out with the chi-square test and the U-test (Mann-Whitney Test)., Main Outcome Measures: Erectile dysfunction, libido, and ability to have and sustain ejaculation and orgasm (both before and after surgery in each case) were among the dependent variables when compiling the data. The impact various surgical procedures and radiochemotherapy had on the severity of the sexual dysfunctions was analyzed. The scope of the postoperative urological care given was also assessed., Results: Erectile dysfunction was confirmed in N=112 patients (77.3%) after surgery (P-value<0.001). Other parameters such as orgasm capacity (4.1% vs. 16.5%), ejaculation ability (1.4% vs. 12.4%) and libido (3.4% vs. 22%) also showed a marked deterioration postoperatively. Postoperative erectile dysfunction was present in 77% of the patients with a colostomy and in 88.5% of the patients who had received neoadjuvant radiation., Conclusions: Male erectile dysfunction is a frequent complication after rectal resection as a result of carcinoma of the rectum. The high incidence of sexual dysfunctions results from the radical nature of the procedure and from additional radiation or colostomy therapy. These patients need accompanying urological care for treatment of their sexual dysfunction., (© 2010 International Society for Sexual Medicine.)

Background: Granulosa cell tumor of the testis is a rare intermediate stromal cell tumor that can be distinguished in the adult and juvenile type. The juvenile type is the most common reason for scrotal swelling in newborns under the age of six months. Less than fifty cases of this disease entity have been reported in the literature., Patients and Methods: In the following article, two newborn patients with scrotal swelling and a histological confirmation of juvenile granulosa cell tumor of the testis will be presented., Results: Case 1: A newborn patient presented with massive scrotal swelling. Sonography of the testicle exhibited a multiple septic and cystic enlargement of the testicle without distinction of the testicular parenchyma being possible. The laboratory findings demonstrated normal testosterone levels, beta-HCG and inhibin-B levels as well as an increased alpha-fetoprotein level of 35.350 ng/dl. Due to clinical and sonographic findings, an inguinal exploration and later, due to the impossibility of distinction of the testicular parenchyma, an inguinal orchiectomy of the right testicle was performed. Case 2: The clinical and sonographic examination of a newborn patient demonstrated a suspicious process of the left testicle. Sonography exhibited an enlarged testicle with cystic formations with the distinction of the testicular parenchyma not being possible. The laboratory findings demonstrated normal testosterone levels, beta-HCG and inhibin-B levels as well as an increased alpha-fetoprotein level of 9.038 ng/dl and LDH of 768 U/I. An inguinal orchiectomy of the left testicle was performed. In both cases, a histological diagnosis of juvenile granulosa cell tumor of the testis was made., Conclusion: These two aforementioned cases demonstrate that juvenile granulosa cell tumor of the testis is a benign disease encountered in newborns, which exhibits an excellent prognosis. Inguinal orchiectomy is the therapy of choice. After surgical removal of the involved testicle is performed no further management is required.

Background: Although the NWTSG and SIOP studies have included the largest number of patients, several individual institutions have made likewise important contributions to the optimization of Wilms' tumor therapy. The purpose of this study is to present our personal experience obtained in the last 42 years by treating Wilms' tumor in childhood., Patients and Methods: Throughout the period 1965-2006, 65 children with histological confirmation of Wilms' tumour were treated in the Department of Urology, University of Erlangen Medical Centre. The records of all patients presenting to our institution with Wilms' tumour were examined., Results: The results obtained by this study group indicate that prognosis according to age demonstrated no significance, but prognosis according to tumor size, lymph node involvement and distant metastasis was significant. After a 10-year follow-up, our data revealed an 89.4% survival rate. Furthermore, the statistical evaluation performed in order to evaluate the significance of surgical complications following neoadjuvant therapy in comparison to non adjuvant therapy, indicates that there is a significant increase in complications in patients who are not treated with neoadjuvant therapy., Discussion: Although clinicians in the U.S.A. and Europe have different philosophies on preoperative chemotherapy, most patients with Wilms' tumor survive long term, regardless of the sequence of therapeutic interventions. In the absence of a clear choice between up-front nephrectomy and preoperative chemotherapy, it is reasonable to base the timing of resection on factors such as tumor size, the patient's clinical condition, and the experience of the surgeon.

Aim: Functional disorders of micturition after rectal resection were investigated on an in-house patient collective. Because the neuro-anatomy of the small pelvis is very complex, the aim of this study was to evaluate the incidence and form of disorders in micturition., Materials and Methods: A total of 536 patients were operated on for a rectal carcinoma during the period 2000 to 2004 at the Surgical University Clinic in Erlangen. Patients with a recurrent tumor and patients who died during the study were excluded (140 patients). All patients were retrospectively questioned on the pre-operative and postoperative bladder function using a standard questionnaire., Results: The patient collective consisted of 167 males and 111 females with an average age of 63 years and a mean follow-up time of 2.6 years. Age (<65 years versus >65 years) was found not to play a significant role with respect to micturition (p>0.05). Of the patients 20 already had a pollakisuria before the operation and 63 patients after the operation. Also 9 patients already had a nycturia before the operation and 55 after the operation. An imperative urgency to urinate was experienced by 9 patients before and 47 after the operation. Stress incontinence grade I was present in 21 patients before the operation, grade II in 4 patients and grade III in 1 patient. Of the patients 43 complained of stress incontinence grade I after the operation, 20 patients of a grade II and 15 of a grade III. The postoperative quality of life was assessed as very limited by 57 patients and moderately limited by 85., Conclusions: Functional disorders of micturition after rectal resection in rectal cancer patients are commonly occurring complications which have received relatively little attention in the literature considering the clinical significance. Only few affected patients have received a urological treatment. Further studies are necessary, also experimental in nature, to understand the neuro-anatomy of the small pelvis in order to avoid intra-operative neurolesions as far as possible.

Female circumcision (genital mutilation) is a criminal violation of human rights under German law. Even with consent of the person to be circumcised and/or her legal representative this procedure must not be carried out since a consent to female circumcision is unethical and therefore void. As much consent as there is on female circumcision the legal situation with ritual male circumcision is very unclear. In practice and unnoticed by the public male circumcision is carried out - be it for medical or ritual reasons - without deeper-going reflexions on the clearness of the medical indication or the legal situation with ritual circumcision. From the medical aspect there are big differences between female and male circumcision but also certain parallels. Various reasons, partly founded in prejudice and misinformation, make people refrain from regarding circumcision of boys also as illegal. Contrary to the prevailing opinion male circumcision also represents a bodily harm which a doctor can only carry out after a preoperative interview and with the consent of the affected person. Since ritual male circumcision does not serve the wellbeing of a child it is not possible for the parents to give their consent to the circumcision in lieu of the child. Male circumcision is only permitted if the child has given his consent and is thus only legally permitted if the child has reached an age at which he is mature enough to understand the meaning and extent of such an action which is hardly the case before he has completed his 16 (th) year., ((c) Georg Thieme Verlag KG Stuttgart. New York.)

Ganglioneuromas are considered to be part of the neuroblastoma series of tumors, which evolve from the sympathogonia of neural crest origin. As imaging techniques have become more widely practiced, the number of ganglioneuromas detected incidentally has increased. Preoperative diagnosis of retroperitoneal ganglioneuromas is often difficult and the diagnosis is usually based on histopathological findings after surgical excision of the tumor. We present two cases of this unique disease entity and discuss its clinical presentation, therapeutic approach, and clinical outcome.

Introduction: Urethral duplication is a rare anomaly that can occur in various forms. Urethral duplication must be diagnosed and adequately treated. Only if clinical complaints develop should surgical management be considered., Patients and Methods: The aim of this study was to retrospectively analyze patients with congenital urethral duplication: age at the time of diagnosis, type of urethral duplication, diagnostic and therapeutic measures as well as clinical aftercare were evaluated., Results: This homogeneous and selected cohort included nine male children. The average follow-up was 6.2 years. Seven children were symptomatic (77.7%). We found a double urethra with epispadias in four boys and with hypospadias in three boys and urethral duplication with perineal/anal meatus in one patient and normotopia in another patient. In two children without symptoms, the urethral duplication was an incidental finding during diagnostic work-up for other anomalies. Seven children had additional urological anomalies: one fusiform megalourethra, three urethral valves with hypospadias, and two epispadias. Two children without symptoms were managed conservatively. An open operation joining the urethras was performed in three children, and four boys underwent endoscopic treatment of the urethral duplication. Long-term results revealed normal continence in all of the children. One child exhibited recurrent urinary tract infections and late stricture, which was opened endoscopically in a second session., Conclusion: A surgical approach is only indicated in the presence of clinical complaints. There is no surgical gold standard. The decision is ultimately reached on an individual basis depending on the findings and anatomy in view of the rarity and variability of the clinical picture. The functional result is of foremost importance for the urethra that requires reconstruction, but the cosmetic aspect should be respected to the same extent. We present the results of our experience in treating children with this urological condition. At the same time larger (multicenter) studies are needed, which will make it possible to formulate guidelines for this rare anomaly.

Penoscrotal elephantiasis is a symptom that can be caused by local but also by systemic disorders. When the changes are reversible, conservative measures such as physical and antiphlogistic approaches lead to success. In cases of irreversible penoscrotal elephantiasis, excision and amputation of the affected penoscrotal areas are recommended to eliminate the functional disturbances. In all manifestations it is important to prevent and treat those diseases known to cause elephantiasis.

Introduction: Recent studies suggest that RCC detected in young adults is likely to be more symptomatic and potentially aggressive, since young patients are less likely to undergo radiologic examinations unless they have symptoms. The aim of this study is to identify the clinical, biological and histological entities of RCC in young patients, and to compare survival rates for symptomatic and asymptomatic patients., Patients and Methods: A review of 2,510 records of patients with RCC from 1965 to 2007 was conducted. Thirty-four patients under 35 years old were identified. The Kaplan-Meier system was used to calculate the cumulative survival rate at five and ten years post-surgery as well as the survival prognoses for patients who presented with symptoms and for patients for whom RCC was incidentally diagnosed., Results: The differences in cumulative survival rates at five and ten years between patients who presented with symptoms and patients whom RCC was incidentally diagnosed were not significant (P = 0.06). The Kaplan-Meier system used to calculate the cumulative survival rate at five and ten years postsurgery yielded survival rates of 79.4% at five years and 73.5% at ten years, respectively., Discussion: The biological and histological entities as well as the survival prognosis for RCC in young patients are similar to those for patients who present with RCC in the sixth and seventh decades of life. Additionally, young adults are likely to be more symptomatic, but the difference in survival between patients with symptoms and those without symptoms is not statistically significant.

Objective: In 4-10% of cases of renal cell carcinoma (RCC), involvement of the inferior vena cava (IVC) is present. IVC involvement may be due to either a pure thrombus or tumor extension. This study indicates that there is no significant difference in survival rates between patients with thrombus and those with infiltration of the IVC., Material and Methods: The records of 84 patients who presented to our institution with RCC and IVC involvement and received surgical treatment between July 1973 and June 2006 were examined. The postoperative observation period was 120 months. Statistical analysis was performed using the Kaplan-Meier method and the log-rank test., Results: Sixty-one patients demonstrated thrombus involvement of the IVC, while the remaining 23 exhibited infiltration. Over the observation period, an average survival time of 64.0 months was observed. The median survival time was 35.2 months. No significant difference in survival rates was observed between patients with thrombus and those with malignant infiltration of the IVC., Conclusions: It is stated in the contemporary literature that IVC infiltration is an important prognostic factor in cases of RCC. This study indicates that there is no significant difference in survival rates between patients with thrombus and those with infiltration of the IVC. Lymph node metastases and undifferentiated RCC remain the only important prognostic factors that have a significant influence on the general survival of a patient.

Extracorporeal shock wave lithotripsy (ESWL) is nowadays accepted as the treatment of choice for the majority of patients with renal or proximal ureteral calculi. Although, a relatively noninvasive modality with low morbidity, minor or major complications can be noted. A relative severe complication for the patient and confusing for the treating physician is the perirenal hematoma. With review the literature and an example of perirenal hematoma induced by ESWL in a patient treated in our department, we describe its therapeutic management.

Introduction: Pasqualini's syndrome is an isolated, incretory functional disorder of the testes with a secondary Leydig cell insufficiency but, unlike Klinefelder's syndrome, with normal spermiogenesis and fertility. This unusual form of hypogonadism is caused by an inherent LH secretion disorder of the pituitary gland. The result of this is the secondary Leydig cell insufficiency and a corresponding peripheral androgen deficiency. In this communication we present the case of a 67-year-old man with Pasqualini's syndrome and a histologically confirmed left seminoma. In view of the current lack of data and our experience, we describe the properties of this uncommon syndrome; therapy, prognosis and possible relationship with seminoma are discussed in more detail., Case Report: The 67-year-old male presented in our outpatient department with the urgent suspicion of a left testicular tumour as a painless swelling of the testis since about three months. In the case history we found a previously diagnosed hypogonadotropic hypogonadism, which is described in the literature as the so-called Pasqualini syndrome. On palpation, the left testis was about fist-size, scirrhous, mildly pressure sensitive and highly suspicious. Sonography of the testes revealed the enlarged left testis to be completely inhomogeneous with hypo- and hyperdense areas and large spatial requirements with unremarkable perfusion. On the basis of the clinically and sonographically supported diagnosis of a tumour of the left testis and inguinal exposure of the testes was performed. Intraoperative histology of a sample confirmed the diagnosis of a testicular tumour in the sense of a seminoma. A high left ablatio testis was carried out., Discussion: The occurrence of testicular tumours of the seminoma type in association with Pasqualini's syndrome has not previously been described in the literature. Thus, we assume that the seminoma had occurred at this age independently of the Pasqualini syndrome. For the treatment of Pasqualini's syndrome not only hCG but also testosterone have been employed with success. The patient must continue therapy for his whole life. The results of interruption tests confirm that it is not a passing ailment but rather a persisting, endogenous disease. PRACTICAL CONCLUSIONS: Since the molecular genetics and genesis of this disease have not been clarified unambiguously, further clinical and experimental studies are necessary in order to better understand the disease. An increased risk of testicular tumours has not yet been observed on account of the small number of cases of Pasqualini's syndrome.

Objectives: To report our experience with 10 cases of pediatric xanthogranulomatous pyelonephritis and review the literature regarding this relatively uncommon disease., Methods: The records of 10 patients presenting to our institution with xanthogranulomatous pyelonephritis between 1996 and 2006 were examined. The mode of presentation, clinical and radiologic findings, blood count, blood chemistry, urine culture results, operative findings, and histologic findings of the affected kidney were recorded., Results: Staging was performed according to the classification of Malek and Elder. Through radiologic and operative investigations our patients were classified as Stage 1 (n = 1), Stage 2 (n = 6), and Stage 3 (n = 3). Simple nephrectomy was performed in all 10 patients., Conclusions: Previously the diagnosis of xanthogranulomatous pyelonephritis was normally based on pathologic assessment, most commonly after nephrectomy. During the last decade, however, increasingly sensitive radiologic investigations (sonography, four-phase computed tomography, and magnetic resonance imaging) in combination with clinical suspicion have made its preoperative diagnosis possible. Surgical intervention still remains the cornerstone of treatment for this relatively uncommon disease.

This case report describes a female patient with bladder exstrophy and chronic compensated renal insufficiency as it relates to the topic of urinary bladder augmentation using the stomach and cutaneous vesicostomy with a Mitrofanoff continent stoma. Due to complete congenital bladder exstrophy the patient underwent functional reconstructive surgery for cystoschisis and several revision procedures. Because of a low-capacity, low-compliance bladder with bilateral urinary stasis in the kidneys, temporary percutaneous renal fistulas were created on both sides. After the patient had declined any follow-up during her pubertal years, advanced bilateral renal damage ensued. In a state of compensated renal insufficiency with creatinine levels >2 mg/dl, the patient was offered surgery for placement of an incontinent bladder substitute as an ileal or colon conduit because of the progressive kidney damage with obstructive uropathy. Since the patient rejected any form of wet stoma despite several consultations, after adequate preparations were met, a gastrocystoplasty and Mitrofanoff cutaneous vesicostomy with an umbilical stoma and artificial umbilicoplasty were performed. The patient catheterizes herself six times daily and is under close urological/nephrological supervision.In exceptional cases, gastrocystoplasty is an alternative to construction of a neobladder from bowel segments. It is particularly indicated for patients with compensated or already poor kidney function. The interventions are associated with several specific complications. There have as yet been no reports of malignant degeneration inside the gastric portion used for augmentation or the urothelium exposed to stomach acid. However, pathological and precancerous alterations of mucous membranes have been observed, which should be regularly monitored by endoscopy/biopsy and warrant further long-term studies.

Penoscrotal elephantiasis is not an uncommon clinical picture that may arise as a symptom of many diseases; it is usually a sequela of a recurring inflammatory process, eczema or malignancy. Elephantiasis often occurs after radical operations in the pelvic region. Displacement of lymphatic pathways leads to a local edema which over the course of time may lead to a considerable increase in volume of the patient's genitals or other affected parts. The diagnosis of elephantiasis is not difficult. It is much more difficult to determine which disease has caused the obstruction of the lymphatic pathways. If it is a reversible stage, the object of treatment is to remove the obstruction and reinstate the physiological lymph flow. Conservative measures such as administration of anti-inflammatory drugs and diuretics, physical measures such as baths, massage, elevation of the affected parts and treatment of the underlying disease may be considered. On progression to irreversible elephantiasis a chronic lymphatic edema occurs for which conservative measures will be unsuccessful. For cases where physical and anti-inflammatory measures are unsuccessful, excision and amputation of the affected penoscrotal region is recommended in order to eliminate the functional dysfunction. We report on two cases of penoscrotal elephantiasis, one in a child and the other in an adult man.

Bladder tumors rarely occur in the first two decades of life, and, in contrast to the cases in adults, 97% of transitional cell carcinomas of the bladder in childhood are low grade, superficial, and of good prognosis, with a progression rate of only 2% to 4%. This report presents a case of low-grade bladder cancer in a 17-year-old patient which behaved quite aggressively. The most striking features of this case included the histopathologic findings and rapid disease progression, despite treatment with agents known for their efficacy against tumors of epithelial and mesenchymal origin.

Renal cell carcinoma (RCC) in childhood is a unique disease entity, representing only 2.3-6.6% of all renal tumors in children. Most experience with pediatric RCC is limited to case reports or case series consisting of relatively small numbers of cases. The aim of this study is to present four additional cases of this unique disease entity. The records of four patients presenting to our institution with renal cell carcinoma between 1986 and 2006 were examined. Only patients younger than 10 years were included. The clinical data included age, sex, signs and symptoms, surgery performed and clinical outcome. Data recorded from the pathology reports included tumor size, histologic subtype, grade (WHO), and stage (TNM). There were three boys and one girl aged between 11 months and 10 years at presentation. The classic triad of flank pain, gross hematuria and palpable abdominal mass were not encountered. A histopathological diagnosis of RCC was confirmed in all four cases without any positive lymph node involvement. Due to the increased detection of tumors with the use of imaging techniques such as ultrasound and computerized tomography, an increased number of incidentally diagnosed RCCs are found. The primary choice of the treatment of any stage of RCC is surgical excision. However, the preoperative diagnosis of tumor in children is difficult and the effects of chemotherapy, including immunotherapy, are unclear.

Wilms' tumors develop from persistent, primitive metanephrogenic stem cells. Their biology and etiology in adults is still unknown even though remnants of primitive metanephrogenic tissue, which tends to malignancy, is suspected, and there are very few scientific studies on the biology of Wilms' tumors in adults. Such a tumor occurs at a rate of 0.2/million adults in Europe and the USA. In this article, we describe the course of the disease in two adults with histologically confirmed Wilms' tumors. Both patients underwent a radical nephrectomy followed by chemotherapy as indicated by the SIOP nephroblastoma study.

Background: Wilms' tumors are highly malignant tumors of the kidneys and are among the most frequent solid tumors of childhood, which are diagnosed for the most part before the 5th year. Various congenital anomalies are associated with a genetic predisposition and thus an increased risk of developing a Wilms' tumor., Patients and Methods: As part of a retrospective analysis, the records from 1965 to 2003 of 66 patients with histologically confirmed Wilms' tumor were reviewed. The aim of this study was to determine the incidence and type of different urogenital malformations as well as the genetic mutations and overall survival in this patient group., Results: The patient cohort comprised 66 patients with histologically confirmed Wilms' tumor: 35 male patients (53%) and 31 female patients (47%). The overall survival after 10 years was 89.4%. All patients underwent radical nephrectomy: transperitoneal approach in 63 and lumbar approach in 3 patients. Eleven (16.7%) patients had diverse urogenital anomalies. A cytogenetic investigation was performed in 38 patients that revealed no pathological findings. The results of molecular genetic testing of tumor specimens were available for seven patients: a genetic mutation was detected in one case (heterozygous mutation R394 in exon 9 of the WT1 gene) which had already been described in the literature in conjunction with Denys-Drash syndrome. Hemihypertrophy with Beckwith-Wiedemann syndrome was present in two (3%) patients., Conclusion: In patients with urogenital malformations and Wilms' tumor, it would be important to carry out further molecular genetic testing to identify possible WT1 gene mutations. Close interdisciplinary cooperation between urologists, specialists in human genetics, and pediatricians is imperative.

Background: Wilms' tumor is the most common renal tumor in childhood. Preoperative treatment is still under discussion. The aim of this study was to determine, using our own patient collective, the risk factors for and type of intraoperative complications which can occur. In addition, the influence of the surgical procedure and tumor size on the complications and survival rate was analyzed., Methods and Materials: A total of 66 patients with Wilms' tumor were retrospectively analyzed. Evaluation included histology, size of the primary tumor as well as neoadjuvant and adjuvant chemotherapy. The total survival rate over periods of 5 and 10 years postoperatively were analysed using Kaplan-Meier survival probabilities., Results: All patients underwent radical nephrectomy: 63 using the transperitoneal and three the lumbar approach. The tumors had a mean size of 9.8 cm (range 2.5-20.0). Twenty patients (30.3%) received neoadjuvant chemotherapy for tumor reduction, while 46 patients underwent surgery without preoperative chemotherapy. Complications occurred in eight patients (15.2%). In two, a the tumor ruptured under surgery, four patients developed an ileus and two suffered cardiac arrest. One patient had postoperative hypertonia and another an incisional hernia. All complications occurred with a tumor size >5 cm or in the patient group without neoadjuvant chemotherapy. The 10 year survival rate was 89.4%., Conclusions: The risk of complications is associated with the local size of the primary tumor. Through tumor reduction, neoadjuvant chemotherapy influences the expression of the such complications. Transperitoneal tumor nephrectomy is the method of choice in surgery for Wilms' tumors.

The impact factor, cited half-life, and immediacy index are three important methods that can be used to demonstrate how a journal's articles are cited over the course of a specific time span. These three factors thus represent instruments for measuring the importance and reputation of a scientific magazine. The goal of our study was to evaluate the constancy of journals specialized in the field of urology and to analyze those listed in the 2005 Journal Citation Report (JCR) using the standardized methods specified by the Institute of Scientific Information (ISI) and JCR. Primarily we wanted to examine the different time spans needed for publication of original papers submitted to certain journals; in more exact terms: the time span from submission of an article or original paper to its publication and from the time of acceptance of an original paper or article to its publication in a urological journal. Furthermore, we wanted to investigate the dynamics of urological journals regarding their impact factors for the years 2000-2005. The study was performed using the ISI JCR and the library as well as the online library of the Friedrich Alexander University in Erlangen. A very satisfactory development can be observed in the field of urology with the standardized methods of the (ISI) JCR.

Nephroblastoma is commonly a chance finding in a series of malformation syndromes. With a frequency of 4-8%, urogenital malformations are amongst the most common congenital anomalies in Wilms' tumor patients. For various congenital anomalies, there is a genetic predisposition and thus an increased risk of developing Wilms' tumor. The WT1 gene product (WT1 protein) usually regulates genes that play an important role in normal kidney development and in maintaining kidney function. If this gene has a functional change, then this role is no longer possible. The consequence is the development of nephrotic syndrome and possibly of a Wilms' tumor.

Urethral duplication is a rare deformity which can present in various forms, commonly together with other congenital malformations. The embryological genesis is unknown. The isolated deformity is most often found in young males, while for young females it is very rare. We report the case of a 1.5 year old male child having a duplicate urethra with penopubic epispadias and dorsal penis deviation. The child, with known penopubic epispadias grade II and dorsal penis deviation, presented for pediatric urological consultation involving additional diagnostics and therapy. Examination revealed a penopubic epispadias with an external urethral exit at the base of the penis. A preoperative micturating cystogram led to the diagnosis of an accessory central as well as an epispadic urethra. We then carried out urethral reconstruction with the surgical combination of both urethras into a single functioning unit. The penis deviation was corrected after Ransley in the same operation. The postoperative course and aftercare showed normal micturition with a normotopic urethral entry as well as a good urine stream without evidence of a stricture or residual urine. An duplicate urethra is an uncommon malformation which is, however, found more often in male patients. Surgery is based on the individual and must be planned dependent on the morphology present. In all cases, both functional and cosmetic aspects must be taken into account.

Introduction: The purpose of our analysis was to assess the performance of urological journals in terms of the standardized measures created by the Institute of Scientific Information (ISI) Journal Citation Report (JCR) in comparison to other medical and surgical fields. Additionally, an evaluation of all original articles, clinical or experimental, published in the year 2005, assessing the time interval needed from submission to publication, submission to acceptance, and acceptance to publication of a manuscript was conducted for all urologic journals., Materials and Methods: The study was conducted with the help of the ISI JCR and the University of Erlangen Medical Center's library (and on-line library)., Results: The field of urology demonstrates satisfactory measures created by the (ISI) JCR in comparison with various fields of medicine. In comparison with the surgical field, urological journals demonstrate outstanding results., Discussion: The impact factor is a measure of the importance of scientific journals. Urological journals stack up quite well against other medical and especially surgical journals.

Endometriosis is a benign growth of ectopic endometrial mucous membrane which has maintained the histological characteristics and biological reactions of uterine mucous membrane. In only 1-2% of cases does it occur in the urinary system, most commonly in the urinary bladder. Such an endometriosis is often diagnosed very late due both to its commonly asymptomatic course and its rarity. Individual therapy is dependent on the age of the patient, the wish for children and the extent of the growth. For endometriosis covering a large area, surgery is recommended. Methods of choice are laparoscopic bladder resection for the urinary bladder, ureterocystoneostomy using the Psoas hitch for the distal ureter, end to end anastomosis or endoscopic incision for short, proximal cases, and for extended areas, ileum cross-bridge attachment or kidney mobilization using nephropexy.

Introduction: Endometriosis is a benign proliferation of discarded or ectopic endometrial mucus membranes which retain the histological features and biological reactions of uterine mucus membranes. This tissue is not capable of independent proliferation but is subject to the influence of estrogen progesterone. In this report we describe the clinical course of two patients with histologically proven endometriosis with participation of the uteral region and uronephrosis. CASE REPORT 1: Left-sided uronephrosis was diagnosed in a 36-year-old female during a sonographic examination for hypertension. Our gynaecologists obtained histological proof of endometriosis by laparoscopy. Two days after the laparoscopic detection of foci of endometriosis in the intestine, Douglas' pouch, ovaries as well as in the region of the left terminal ureter, a left percutaneous renal fistula was created under sonographic and radiological control. After resection of the afflicted section of the ureter, implantation of a new left ureter was performed by means of a psoas-hitch plasty. CASE REPORT 2: A 30-year-old female presented with a months-long history of dyspareunia, pain on palpation of the Douglas' pouch, occasional pain on bowel movements and back pain. In addition she had been trying for years to become pregnant. On sonography renal congestion grade III was detected. After admission and appropriate preparation, one week later an open laparotomy with lysis of intestinal as well as uterine adhesions, salpingectomy and, by the urologist, partial left uteral resection with new implantation by the psoas-hitch technique., Conclusions: Endometriosis of the urinary tract is a rare occurrence affecting 1 - 2 % of all endometriosis patients with the urinary bladder being the most commonly affected site. Endometriosis with involvement of the ureter is often diagnosed very late because of the rareness of this situation and its asymptomatic course. An individual therapy plan depending first of all on the patient's age, desire for children, and the extent of the endometriosis foci should always be attempted.

Megalourethra is a rare malformation of the urethra caused by a lack of corpus sponigosum and in some cases corpora cavernosa in the region of the distal urethra. The absence of these structures causes a ballooning of the urethra despite there being no mechanical obstruction. A male child presented with so-called fusiform megalourethra, with absence of the corpora cavernosa and urethral duplication. A voiding cystourethrogram was used to diagnose a fusiform megalourethra with pronounced meatal stenosis and extreme stenosis of pendulous urethra. In addition, there was urethral duplication in the form of an accessory urethra stretching from the urethral colliculus to the perineum. Absence of the corpora cavernosa was also suspected in the distal urethra. The surgical procedure involved pendulous urethroplasty with an onlay technique using urethral duplication and penile reduction. This method of treating megalourethra has not been previously reported. The operative technique for fusiform megalourethra with genital malformation has to be tailored to each individual case, depending on the intraoperative and endoscopic findings.

This article reports on a newborn with a scrotal space-occupying lesion and histologically proven granulosa cell tumor of the testes. A massive scrotal space-occupying lesion was diagnosed in a full-term male neonate with a birth weight of 2,260 g and body length of 45 cm. Sonography of the right testicle revealed a mass 6x6x4 cm in size which was multiply septate and cystic. The testicular parenchyma was poorly defined with a Swiss cheese appearance. Chemical analysis of blood serum evidenced normal levels of testosterone, beta-HCG, and inhibin B as well as physiologically a clearly elevated alpha-fetoprotein level at 35,350 ng/ml. Based on the clinical and sonographic findings of the right testicle, inguinal surgical exposure of the testes was undertaken. Since there was hardly any testicular parenchyma, we decided to perform high inguinal ablation of the right testicle. This case report confirms that congenital granulosa cell tumors usually exhibit benign behavior during the neonatal period.

The occurrence of primary non-Hodgkin's lymphomas of the testes is described in just a few studies in the urological literature. The clinical symptomatology and especially the treatment concept for this relatively rare tumor entity are hardly discussed. Imaging diagnostics, e.g., with CT or MRI, play a decisive role in determining the diagnosis and whether a primary testicular disease is involved or a generalized systemic disease. In cases of primary B-cell lymphomas of the testes, a high inguinal orchiectomy should be performed for diagnostic and therapeutic purposes. The standard chemotherapy for aggressive non-Hodgkin's lymphomas is the CHOP regimen consisting of cyclophosphamide, doxorubicin, vincristine, and prednisone. This article presents two adults aged 67 and 75 years with histologically proven B-cell lymphoma of the testes and discusses the characteristics of this relatively rare clinical picture as well as treatment and prognosis.

Introduction: Frasier syndrom is an autosomal dominant, hereditary disease characterized by nephropathy, gonadal dysgenesis and risk of gonadal blastoma in early childhood. To date, in many patients with Frasier syndrome WT1 mutations have been found, occurring exclusively as germ-line mutations of the alternative splicing donor site in intron 9. A Wilms tumor is seen only rarely in this clinical entity. In the present paper we describe the clinical course of a patient with Frasier syndrome confirmed by molecular genetic analysis., Case Report: Our patient with Frasier syndrome as confirmed by molecular genetic analysis is now 19 years old. The patient became dependent on dialysis due to nethropathy in the form of focal sclerosing glomerulonephritis and terminal renal insufficiency. A kidney transplantation in the left iliac fossa together with new implantation of the ureter according to Dodson. For prophylactic reasons on account of the high risk of gonadal blastoma associated with the disease and sonographically detected microlithiasis in both testicles we performed one year later an inguinal castration. Histology revealed the picture of a severe tubular testicular atrophy with arrested spermatogenesis and focal intratubular germ-line neoplasia., Conclusions: This case report shows that, besides our already published series with Denys-Drash syndrome, WT1 mutations may also be associated with the so-called Frasier syndrome. For children with Frasier syndrome confirmed by molecular genetic analysis and loss of function of the testicles, we recommend performance of a prophylactic castration. We also suggest that phenotypical female patients with focal sclerosing glomerulonephritis be examined for WT1 mutations.

Cowper's syringocele is a cystic dilation of the male urethra which should be surgically treated when symptomatic. Two cases of syringocele, 1 adult and 1 juvenile, with obstructive micturition and microhematuria are reported. Both patients were treated using the transurethral surgical technique. The results are discussed with reference to the relevant literature.

Renal artery aneurysms require selective treatment, depending on size, localisation, and morphology as well as on singular or multiple locations. Spontaneous course without sequelae was reported in smaller series, however, there is an indication for treatment of aneurysms without calcification, women in childbearing age and in cases of unilateral kidney. Besides endovascular procedures there are different surgical techniques of in vivo or ex vivo repair. Treatment of aneurysms of side branches is more complicated and requires ex vivo treatment in selected cases. Ex vivo preparation of the kidney offers nearly identical surgical complications as the renal transplantation, limiting its indication. The clinical course and treatment options of a 53 year old female demonstrates that even nephrectomy might be the treatment of first choice in case of normal contralateral kidney.

Objective: With an incidence of up to 5% in the general population, genital malformations are a frequent clinical occurrence. However, using the existing published classifications of malformations, difficulties arise in classifying genital malformations appropriately. The aim of the present study was to produce a simple, systematic, and reproducible classification system., Design: A systematic arrangement of genital and associated malformaltions, using a structure similar to that in the TNM classification of oncological tumors, was developed and validated., Setting: Patients with genital malformations in a university hospital., Patient(s): Ninty-nine premenopausal patients with genital malformations., Intervention(s): Patients were diagnosed for genital malformation using laparoscopy or magnetic resonance imaging., Main Outcome Measure(s): A new classification (VCUAM) is presented to evaluate patients with different genital malformations., Result(s): The external and internal female genital organs were divided into the following subgroups in accordance with the anatomy: vagina (V), cervix (C), uterus (U), and adnexa (A). Associated malformations were assigned to a subgroup (M) relative to each specific organ. The classification was validated in a group of 99 patients with genital malformations., Conclusion(s): The VCUAM classification for the first time makes it possible to reflect even complex malformations in a precise and individual fashion, taking associated malformations into account. The classification makes it easier to provide appropriate clinical care for the affected patients.

Denys-Drash syndrome is a rare symptom complex associated with obligatory childhood nephrotic syndrome, male pseudohermaphroditism, and Wilms' tumor. The etiology of Denys-Drash syndrome is attributed to a mutation of the WT1 gene. We report on two cases of Deny-Drash syndrome confirmed by genetic testing. Rapidly evolving terminal renal insufficiency was detected in both patients necessitating bilateral nephrectomies with prophylactic intent. In one of the patients, a Wilms' tumor had already been verified in one kidney so that chemotherapy had to be initiated.The risk of Wilms' tumor is very high in patients with a WT1 mutation, which leads to the need for removal of both kidneys during or before transplantation. It would be important to perform a diagnostic work-up for WT1 gene mutation in children who develop renal failure in the 1st year of life.

The ganglioneuroma is a benign neuroblastal tumor. All neuroblastomas and ganglioneuromas derive from immature cells of the sympathetic nerve system. The ganglioneuroma is a very rare disease and effects newborns and infants more often than adolescents and adults. The benign tumors are relatively difficult to diagnose since they usually are asymptomatic. A 5-year old girl with persistent bladder wall thickening and prominent course of the prevesical ureter presents for diagnosis and therapy. Sonography demonstrated a space-occupying lesion of the left kidney. Subsequent MRI raises the suspicion of a retroperitoneal neuroblastoma. The tumor could be removed in two surgical sessions. The ganglioneuroma is a benign tumor with symptoms depending on location and with a relatively good prognosis. Its low incidence and resultant limited experience often leads to delayed diagnosis, potentially determining therapy and diagnosis.

Introduction: Xanthogranulomatous pyelonephritis is a morphologically and clinically unique manifestation of chronic pyelonephritis with the formation of pus or granulomas. The most frequent predisposing factors for the development of xanthogranulomatous pyelonephritis are urinary obstruction (e. g., stones, tumors, congenital anomalies and functional impairment) and infection of the collecting system., Case Report: We describe a 2-year-old female patient with unclear abdominal complaints, diarrhea, malaise, loss of appetite, weight loss, pale skin color, and recurrent and undulating fever in the presence of known left nephrolithiasis. Based on the clinical examination and imaging, above all, CT, the presumptive diagnosis of xanthogranulomatous pyelonephritis of the left kidney was made. A left lumbar nephrectomy was performed and histology confirmed the diagnosis., Conclusion: Xanthogranulomatous pyelonephritis is a relatively rare entity that is associated with obstruction (e. g., stones) and infection of the urinary tract. Its rarity and resultant unfamiliarity often delay diagnosis and therapy, which in turn affect the prognosis. Furthermore, this entity can be mistaken for renal tumors (renal cell carcinoma and Wilms tumor), but nowadays this should be mostly eliminated with the advances in the imaging methods.

We report the case of a patient who experienced anuric renal transplant failure for 44 days after living related kidney transplantation. Immunosuppressive and other therapies were carefully adapted to the findings of frequent renal transplant biopsies, which ultimately led to excellent graft function., (Copyright 2004 Elsevier Inc.)