[Disorders in urinary bladder micturition after rectal operations]. / Harnblasenentleerungsstörungen nach Rektumoperationen.

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.

[Legal aspects of ritual circumcision]. / Juristische Aspekte der rituellen Zirkumzision.

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.

[Urethral duplication: long-term results for a rare urethral anomaly]. / Urethralduplikatur : Langzeitergebnisse einer seltenen Urethralfehlbildung.

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: diagnostics and treatment options]. / Penoskrotale Elephantiasis : Diagnostik und Therapieoptionen.

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.

[Pasqualini's syndrome]. / Pasqualini-Syndrom.

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.

[Penoscrotal elephantiasis]. / Penoskrotale Elephantiasis.

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.

[Urinary bladder augmentation using the stomach in patients with compensated renal insufficiency]. / Harnblasenaugmentation mit Magen bei kompensierter Niereninsuffizienz.

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.

[Congenital urogenital malformations associated with nephroblastomas. Long-term results of a study in Erlangen]. / Kongenitale urogenitale Fehlbildungen bei Nephroblastomen. Erlanger Langzeitergebnisse.

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.

[Endometriosis involving the ureter. The Erlangen experience exemplified by two case reports]. / Endometriose mit Ureterbeteiligung. Erlanger Erfahrungen am Beispiel zweier Kasuistiken.

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.

[Risk factors for intra- and postoperative complications in Wilms' tumor surgery]. / Risikofaktoren intra- und postoperativer Komplikationen in der Chirurgie des Wilms-Tumors.

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.

[Wilms' tumors in adults]. / Wilms-Tumoren im Erwachsenenalter.

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.

[Urogenital malformations associated with Wilms' tumor. Molecular genetic and clinical aspects]. / Assoziierte urogenitale Fehlbildungen mit Wilms-Tumor. Molekulargenetische und klinische Aspekte.

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 in a male child. Accessory urethra with a normotopic opening in a penopubic epispadias with dorsal penis deviation]. / Urethraduplikatur beim männlichen Kind. Akzessorische Harnröhre mit normotoper Mündung bei penopubischer Epispadie mit dorsaler Penisdeviation.

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.

[Endometriosis of the ureter and urinary bladder]. / Endometriose von Ureter und Harnblase.

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.

[Impact factors and publication time spans in urological journals]. / Impact Factors und Publikationszeitspannen in urologischen Fachzeitschriften.

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.

[Congenital cystic juvenile granulosa cell tumor of the testes. Scrotal space-occupying lesion in a male neonate]. / Kongenitaler zystischer juveniler Granulosazelltumor des Hodens. Skrotale Raumforderung beim männlichen Neugeborenen.

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.

[B-cell lymphoma of the testes]. / B-Zell-Lymphome des Hodens.

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.

[Frasier syndrome: a rare syndrome with WT1 gene mutation in pediatric urology]. / Frasier-Syndrom: Ein seltenes Syndrom mit WT1-Genmutation in der Kinderurologie.

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.

A complex case of megalourethra and its repair.

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.