The Prune Belly syndrome: urological aspects and long-term outcomes of a rare disease.

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.

Umbilical rotation: a new technique for the cutaneous fixation of continent catheterizable vesicostomies.

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.

Recurrent gross hematuria with positive family history of IgA nephropathy and an unexpected diagnosis.

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.

Clinical and surgical experience with Wilms' tumor. Long-term results of a single institution.

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.

Congenital juvenile granulosa cell tumor of the testis in newborns.

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.

Sexual dysfunction after rectal surgery: a retrospective study of men without disease recurrence.

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.

Long-term follow-up of male patients after reconstruction of the bladder-exstrophy-epispadias complex: psychosocial status, continence, renal and genital function.

OBJECTIVE: There is a paucity of knowledge about long-term outcome issues in the bladder-exstrophy-epispadias complex (BEEC). Adult male BEEC patients were investigated in respect of bladder and renal function, fertility, genital function and psychosocial facts. PATIENTS AND METHODS: In a cross-sectional study, 17 adult male BEEC patients (mean age 23.4 years) from a single centre were evaluated with a questionnaire, renal and bladder ultrasound, blood tests, hormonal profile and semen analysis. RESULTS: Phenotypically one patient had complete epispadias and 16 had classical bladder exstrophy. Five patients underwent a one-stage functional reconstruction as a primary and 12 as a redo procedure. After a mean follow-up of 19.4 years, 15 bladders were preserved with 12 voiding per urethram and 3 performing intermittent catheterization; 2 were secondarily diverted. Significant residual urine was present in 10; kidneys were normal in 14 patients. Sixteen patients proved ejaculations, 3 had normospermia, 7 oligoasthenospermia and 6 azospermia. In patients with only one single bladder neck procedure normospermia was statistically significant. CONCLUSION: After functional BEEC reconstruction, long-term bladder function is preserved with mostly normal renal function. The number of bladder neck attempts has a significant influence on andrologic outcome. Detailed analysis may detect multifactorial pathogenesis from the impaired sperm quality in the BEEC.

Retroperitoneal ganglioneuroma in childhood--a presentation of two cases.

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.

Inferior vena cava involvement in renal cell carcinoma: comparison of survival rates between patients with thrombus and infiltration.

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.

Renal cell carcinoma under 35 years of age: comparison of survival rates for symptomatic and asymptomatic patients.

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.

Perirenal hematomas induced by extracorporeal shock wave lithotripsy (ESWL). Therapeutic management.

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.

Xanthogranulomatous pyelonephritis in childhood: a critical analysis of 10 cases and of the literature.

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.

Impact factors in urology. How well do our journals stack up against other medical and surgical journals?

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.

Renal cell carcinoma in children under 10 years old: a presentation of four cases.

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.

Cowper's syringocele--a rare malformation of the male urethra. Erlangen experience in two example cases.

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.

Femoral vein occlusion and spontaneous collateral ectasia presenting as recurrent hernia of the groin: a case report.

Femoral vein occlusion is not a common complication even after repeated hernia repair. We describe a case of a 14-year-old boy with a visible and soft, yet irreducible, mass below the inguinal ligament after 3 previous inguinal hernia repairs and heart catheterization in infancy. Further examination showed dilated venous collaterals, bypassing an occluded common femoral vein via the testicular sheaths and across the pelvic floor. We discuss etiology, diagnostic pitfalls, therapeutic options, and possible future complications, with a literature review.