Kinderurologie und Fehlbildungen

MP-07.13

Long Term Results of Bladder Exstrophy

After primary bladder closure or urinary diversion, other factors apart from the reconstruction gain importance for individuals with the exstrophy-epispadias complex: social integration and, after reaching puberty, sexuality and fertility.

SLC20A1 Is Involved in Urinary Tract and Urorectal Development

Previous studies in developing Xenopus and zebrafish reported that the phosphate transporter slc20a1a is expressed in pronephric kidneys. The recent identification of SLC20A1 as a monoallelic candidate gene for cloacal exstrophy further suggests its involvement in the urinary tract and urorectal development. However, little is known of the functional role of SLC20A1 in urinary tract development. Here, we investigated this using morpholino oligonucleotide knockdown of the zebrafish ortholog slc20a1a. This caused kidney cysts and malformations of the cloaca. Moreover, in morphants we demonstrated dysfunctional voiding and hindgut opening defects mimicking imperforate anus in human cloacal exs…

Murine expression and mutation analyses of the prostate androgen-regulated mucin-like protein 1 (Parm1) gene, a candidate for human epispadias.

Abstract Background Epispadias is the mildest phenotype of the human bladder exstrophy–epispadias complex (BEEC), and presents with varying degrees of severity. This urogenital birth defect results from a disturbance in the septation process, during which separate urogenital and anorectal components are formed through division of the cloaca. This process is reported to be influenced by androgen signaling. The human PARM1 gene encodes the prostate androgen-regulated mucin-like protein 1, which is expressed in heart, kidney, and placenta. Methods We performed whole mount in situ hybridization analysis of Parm1 expression in mouse embryos between gestational days (GD) 9.5 and 12.5, which are e…

UP-01.127 Management of Fournier's Gangrene: A Case-Series of 20 Patients

Long-term follow-up of submucosal tunnel and serosa-lined extramural tunnel ureter implantation in ileocaecal continent cutaneous urinary diversion (Mainz pouch I)

Authors from Mainz, Germany present the long-term follow-up of submucosal tunnel and serosa-lined extramural tunnel ureter implantation in the ileocaecal continent cutaneous urinary diversion first described in that department, called the Mainz-Pouch I. OBJECTIVE To assess upper urinary tract complications and renal function in patients with a submucosal tunnel and serosa-lined extramural tunnel ureter implantation during the long-term follow-up of ileocaecal continent cutaneous urinary diversion (Mainz pouch I). PATIENTS AND METHODS In all, 458 patients who had diversion with the ileocaecal pouch were analysed in a retrospective follow-up study. Uretero-intestinal implantation was done usi…

Continent diversion with the Mainz pouch.

From 1983 until July 1994, 561 patients in 2 urology departments (Mainz and Wuppertal) underwent a Mainz pouch 1 procedure. The Mainz pouch 1 was used for bladder augmentation in 60 patients, for orthotopic bladder substitution in 61 patients, and for continent cutaneous urinary diversion in 440 patients. In the group of continent cutaneous urinary diversion, the continence mechanism applied was an ileal intussusception nipple in 270 patients, an appendix stoma in 146 patients, a submucosal seromuscular bowel-flap tube in 14 patients, and a submucosal full-thickness bowel-flap tube in 10 patients. Indications for urinary diversion were bladder cancer in 339 patients, anatomical or functiona…

Metabolic Evaluation of 94 Patients 5 to 16 Years After Ileocecal Pouch (Mainz Pouch 1) Continent Urinary Diversion

PURPOSE In continent urinary diversion metabolic disturbances may be encountered in long-term followup. We evaluated metabolic consequences in patients with a minimum followup of 5 years after Mainz pouch 1 urinary diversion.At our institution continent urinary diversion using the ileocecal segment was performed between 1983 and 1995 in 458 patients. A total of 94 patients with an ileocecal pouch for a minimum of 5 years were reevaluated for metabolic changes. Median followup was 9.0 years. Routine laboratory parameters, blood gas analysis, vitamin B12, vitamin D25, cross-laps, bone specific alkaline phosphatase, osteocalcin and propeptide of type I collagen were obtained. Bone density was …

Rhabdomyosarcoma of the bladder, prostate or vagina: the role of surgery

Rhabdomyosarcoma of pelvic organs is not common enough for many people to develop large series. However, the authors from Mainz retrospectively analysed 107 children with this condition, and suggest that primary chemotherapy followed by radical surgery yields excellent cure rates. OBJECTIVE To retrospectively analyse the outcome of children with rhabdomyosarcoma (RMS) of the bladder, prostate or vagina who were treated with chemotherapy, with or without radical surgery or additional radiotherapy, at our institution since 1968. PATIENTS AND METHODS From a total of 107 children with RMS seen between 1968 and December 2001, 22 (mean age 5.9, range 0.5–18) had RMS of bladder/prostate or vagina.…

Sexual function in adult patients with classic bladder exstrophy: A multicenter study

Summary Background The bladder exstrophy–epispadias complex (BEEC) comprises a spectrum of congenital anomalies that represents the severe end of urorectal malformations, and has a profound impact on continence as well as sexual and renal functions. Objective The relation between severity of BEEC and its associated functional impairments, on one hand, and the resulting restrictions in quality of life and potential psychopathology determine the patients' outcome. It is important for improving further outcome to identify BEEC-related sources of distress in the long term. Genital function and sexuality becomes an important issue for adolescent and adult BEEC individuals. Hence, the present stu…

Microduplications At 22q11.21 are Associated with Classic Bladder Exstrophy

Purpose Classic exstrophy of the bladder (CBE) is part of the exstrophy-epispadias complex (EEC), a spectrum of urogenital anomalies in which part or all of the distal urinary tract fails to close. Familial occurrence has been observed, and previous studies have suggested an underlying multifactorial mode of inheritance. To date, no causative genetic or non-genetic factor has been unequivocally identified in humans. The present study aimed to identify microaberrations characterized by loss or gain of genomic material that contribute to the EEC at a genome-wide level. Material and Methods Molecular karyotyping, utilizing 549,839 single nucleotide polymorphisms (SNPs) with an average spacing …

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

Operative method, course and complications were analyzed retrospectively in 140 patients who underwent a conservative operation for renal tumor between June 1969 and December 1990. In 53 patients (20 women and 33 men, mean age 61.2 years, range 38 to 77 years, with 49 renal cell carcinomas and 4 benign renal tumors) there was an imperative indication for an organ preserving operation because nephrectomy would have made dialysis obligatory. In 87 patients (29 women and 58 men, mean age 53.7 years, range 27 to 74 years, with 72 renal cell carcinomas and 15 benign renal tumors) the tumor was conservatively resected in the presence of a normal contralateral unit (elective indication) and 68 of …

How to Name Papillary Tumors of the Bladder in Children: Transitional Cell Carcinoma or Papillary Urothelial Neoplasm of Low Malignant Potential?

Urinary bladder malignancies are uncommon in children. Approximately 80 children with papillary carcinoma have been described to date, presenting as papillary neoplasms of both low grade and low stage. On the basis of the 1973 World Health Organization classification, tumors were classified as transitional cell carcinoma of the urinary bladder (TCCB). Owing to more detailed histologic criteria, this term has been replaced by papillary urothelial neoplasm of low malignant potential and low-grade carcinoma of the urinary bladder in the World Health Organization-International Society of Urologic Pathology consensus classification system of urothelial neoplasms 2004. Nevertheless, TCCB still re…

Colonic Conduit in Children

Complication rates of up to 86.6% have been reported after creation of an ileal conduit. In contrast to others, we construct an antirefluxing isoperistaltic colonic for incontinent diversion in children.Between 1968 and 1989 a colonic conduit was created in 105 patients up to age 20 years for neurogenic bladder (76), bladder exstrophy (16) and other reasons (13). Long-term followup (minimum 5 years, mean 16.3 years, range 5 to 26) was possible in 84 patients (159 renal units).Early and late stenosis at the ureterocolic anastomosis, and stomal stenosis occurred in 7.6 and 15.5% of the cases, respectively. Renal calculi developed in 8.2% of the renal units. Eight kidneys without function afte…

Parenchyma-sparing surgery for renal tumors

Between 1969 und 1989 conservative surgery for kidney tumors was performed in 123 patients. In 49 patients there was an imperative indication for a parenchyma-sparing operation because nephrectomy would have made dialysis obligatory. Thirty-five of these 49 patients show no signs of tumor progression after a mean follow-up of 4.5 years. Known metastases were present in 3 of 6 patients who died as a result of their tumors. In two patients there was a recurrence after 1 and 5 years, respectively, requiring a second organ-preserving operation. In one further patient we suspect that multiple small tumor lesions may be present 2 years after the first operation. In 74 patients with a healthy cont…

Urinary tract infections in children: EAU/ESPU guidelines.

Context: In 30% of children with urinary tract anomalies, urinary tract infection (UTI) can be the first sign. Failure to identify patients at risk can result in damage to the upper urinary tract.Objective: To provide recommendations for the diagnosis, treatment, and imaging of children presenting with UTI.Evidence acquisition: The recommendations were developed after a review of the literature and a search of PubMed and Embase. A consensus decision was adopted when evidence was low.Evidence synthesis: UTIs are classified according to site, episode, symptoms, and complicating factors. For acute treatment, site and severity are the most important. Urine sampling by suprapubic aspiration or c…

Tapering of the Megaureter

Treatment strategies and outcome of the exstrophy–epispadias complex in germany: data from the german CURE-Net

Introduction: To evaluate the impact of reconstructive strategies and post-operative management on short- and long-term surgical outcome and complications of classical bladder exstrophy (CBE) patients' comprehensive data of the multicenter German-wide Network for Congenital Uro-Rectal malformations (CURE-Net) were analyzed. Methods: Descriptive analyses were performed between 34 prospectively collected CBE patients born since 2009, median 3 months old [interquartile range (IQR), 2–4 months], and 113 cross-sectional patients, median 12 years old (IQR, 6–21 years). Results: The majority of included individuals were males (67%). Sixty-eight percent of the prospectively observed and 53% of the …

Confocal Laser Endomicroscopy and Chromoendoscopy - A New Option of Surveillance in Patients with Ureterosigmoidostomy or Rectosigmoid Pouch

Purpose In patients with bladder exstrophy-epispadias-complex (BEEC) or in selected patients with bladder loss for other reasons, ureterosigmoidostomy (USIG) & rectosigmoid pouch (Mainz Pouch (MZP) II) has been used at our institution. This diversion carries a lifelong risk for secondary malignancies. Therefore annual colonoscopy is recommended. Identification of potentially malignant structures can be challenging, and biopsies are taken in doubt, with the risk of injuring the ureterocolic implantation site. Using confocal laser endomicroscopy (CLE) and chromoendoscopy, the uretero-intestinal anastomosis is better visualized compared to conventional colonoscopy, and target biopsies could be…

Urinary diversion and reconstruction.

Orthotopic bladder augmentation and substitution has been established as the standard procedure for urinary diversion in many institutions, with current studies reporting mainly on continence rates and procedure-associated complications, such as the risk of impairment of neobladder function by local tumor recurrences in the small pelvis. Similarly, in other types of continent diversion, such as continent cutaneous diversion and rectal reservoirs, current interest is primarily directed towards minimization of surgery-associated complications.

Microduplications at 22q11.21 are associated with non-syndromic classic bladder exstrophy

The exstrophy-epispadias complex (EEC) comprises a spectrum of urogenital anomalies in which part or all of the distal urinary tract fails to close. The present study aimed to identify microaberrations characterized by loss or gain of genomic material that contribute to the EEC at a genome-wide level. Molecular karyotyping, utilizing 549,839 single nucleotide polymorphisms (SNPs) with an average spacing of 5.7 kilobases, was performed to screen an initial cohort of 16 patients with non-syndromic EEC. A de novo microduplication involving chromosomal region 22q11.21 was identified in one patient with classic exstrophy of the bladder (CBE). Subsequent multiplex ligation-dependent probe amplifi…

Epidemiological survey of 214 families with bladder exstrophy-epispadias complex.

We sought to identify causative nongenetic and genetic risk factors for the bladder exstrophy-epispadias complex.A total of 237 families with the bladder exstrophy-epispadias complex were invited to participate in the study, and information was obtained from 214 families, mainly from European countries.Two families showed familial occurrence. Male predominance was found among all subgroups comprising epispadias, classic bladder exstrophy and cloacal exstrophy, with male-to-female ratios of 1.4:1, 2.8:1 and 2.0:1, respectively (p = 0.001). No association with parental age, maternal reproductive history or periconceptional maternal exposure to alcohol, drugs, chemical noxae, radiation or infe…

Association Between Exstrophy-epispadias Complex And Congenital Anomalies: A German Multicenter Study

To further investigate associated anomalies in exstrophy-epispadias complex (EEC) patients congenital uro-rectal malformations network (CURE-Net) database was systematically screened. In literature the EEC comprises a spectrum of anomalies, mainly occurring "isolated" without additional congenital defects. Nevertheless, previous epidemiological studies indicated a higher association with renal, anorectal, and lower neurotubular anomalies, which may originate from the same developmental morphogenetic fields.Seventy-three prospectively (born since 2009) and 162 cross-sectional recruited EEC patients (born 1948-2008) were analyzed. Associated anomalies were derived from patient's medical data …

Kinder und Jugendliche mit neurogener Blasenfunktionsst�rung

After the failure of conservative treatment in patients with neurogenic bladders, urinary diversion is a viable compromise between the urologist's concerns and the patient's desire, as the upper urinary tract can be protected in the long-term and high continence rates can be provided. This can be achieved with an acceptable complication rate. Our indications for orthotopic bladder augmentation or substitution involve patients with a good orthopedic condition and mobility, who find it easy to perform CISC through the urethra. A continent umbilical stoma offers a good alternative in obese, immobile or wheelchair bound patients. The serous lined extramural tunnel technique has proven to be sup…

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

Segments from almost all parts of the bowel have been used for urinary diversion. As a result, the available absorptive surface area of the bowel is reduced, and the incorporation of bowel segments into the urinary tract may have metabolic consequences. This is an area somewhat neglected in the literature. Metabolic complications are rare, but sub-clinical metabolic disturbances are quite common. Several studies have demonstrated that some of the absorbent and secreting properties of the bowel tissue are preserved after incorporation into the urinary tract. Hyperchloraemic metabolic acidosis can occur if ileal and/or colon segments are used, as well as malabsorption of vitamin B(12) and bil…

Vitamin metabolism in patients with a Mainz pouch I: long-term followup.

We assessed whether creation of the Mainz pouch I, which requires 24 to 36 cm. of terminal ileum and 12 cm. of ascending colon including the ileocecal valve, leads to metabolic disturbances.In 137 patients the levels of vitamins A, B1, B2, B6, B12, D and E, folic and bile acids, ammonia, and intracorpuscular vitamin B12 and folic acid were evaluated and a red blood count was performed. Patients were divided into children and adults, and into 3 groups according to number of years after surgery (2 or less, more than 2 to 4 and more than 4).In all patients the levels of vitamins A, B1, B2, B6, D and E, folic and bile acids, and ammonia as well as red blood count were within normal ranges. In t…

Surgical Atlas. Primary hypospadias repair with buccal mucosa

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

After primary bladder closure or urinary diversion, other factors apart from the reconstruction itself gain importance for individuals with the exstrophy-epispadias complex: social integration and, after reaching puberty, sexuality and fertility. Between 1968 and July 1994 115 patients with bladder exstrophy or incontinent epispadias underwent surgery at our institution. A total of 104 patients could be followed, 2 of whom died in the meantime. Of the remaining 102 patients 48 attend school, 4 are in college, 40 have completed or are currently undergoing vocational training, 3 are unemployed, 1 lives in a therapeutic centre and 6 are younger than 6 years of age. A total of 95% of the patien…

Vitamin B12 Deficiency in Children after Continent Urinary Diversion by Mainz Pouch Technique

Purpose MAINZ pouch I urinary diversion provides a high-capacity, low-pressure reservoir. The use of terminal ileum can cause a decrease of vitamin B12 levels. Symptoms caused by vitamin B12 deficiency are rare, but some are irreversible, therefore annual check-ups are recommended. In this study the vitamin B12 levels in the above patients were analyzed in retrospect. Material and Methods The ileocecal segment was used for urinary tract reconstruction in > 80 children ≤17 years of age. B12 levels were determined at various time points within up to 21 years after surgery in 63 patients (8 with ileocecal bladder substitution, 55 continent cutaneous diversion). B12 values below 200pg/ml were c…

Tissue engineered pre-vascularized buccal mucosa equivalents utilizing a primary triculture of epithelial cells, endothelial cells and fibroblasts

Artificial generated buccal mucosa equivalents are a promising approach for the reconstruction of urethral defects. Limiting in this approach is a poor blood vessel supply after transplantation, resulting in increased morbidity and necrosis. We generated a pre-vascularized buccal mucosa equivalent in a tri-culture of primary buccal epithelial cells, fibroblasts and microvascular endothelial cells, using a native collagen membrane as a scaffold. A successful pre-vascularization and dense formation of capillary-like structures at superficial areas was demonstrated. The lumen size of pre-formed blood vessels corresponded to the capillary size in vivo (10-30 μm). Comparing native with a highly …

Age-Dependent Presentation and Clinical Course of 1465 Patients Aged 0 to Less than 18 Years with Ovarian or Testicular Germ Cell Tumors; Data of the MAKEI 96 Protocol Revisited in the Light of Prenatal Germ Cell Biology

Objective: To evaluate prognostic factors in pediatric patients with gonadal germ cell tumors (GCT). Methods: Patients <18 years with ovarian and testicular GCT (respectively OGCT and TGCT) were prospectively registered according to the guidelines of MAKEI 96. After resection of the primary tumor, patients staged ≥II received risk-stratified cisplatin-based combination chemotherapy. Patients were analyzed in respect to age (six age groups divided into 3-year intervals), histology, stage, and therapy. The primary end point was overall survival. Results: Between January 1996 and March 2016, the following patients were registered: 1047 OGCT, of those, 630 had ovarian teratoma (OTER) and 417…

Orthotopic MAINZ pouch bladder substitution – long-term follow-up

Introduction After radical cystectomy, orthotopic neobladder is one surgical strategy for urinary diversion. To assess the usefulness of an operation, long-term data are essential. We examined long-term complications and continence rates of orthotopic ileocecal (MAINZ pouch) bladder substitution. Material and methods Between 1986 and 2011, 193 patients underwent orthotopic MAINZ pouch bladder substitution. Until July 2000, ureter implantation was performed into the ascending colon through a submucosal tunnel technique (Goodwin-Hohenfellner). After July 2000, ureters were implanted into the terminal Ileum using the ileocecal valve to prevent reflux: the left spatulated ureter by an end-to-en…

Long-term metabolic effects in patients with urinary diversion

The use of intestinal segments in genitourinary reconstruction could influence vitamin metabolism and affect the skeletal bone and its mineral content in the long term. In 137 patients, serum levels of the vitamins A, B1, B2, B6, B12, D, and E and of folic acid, bile acid, and ammonia as well as levels of intracorpuscular vitamin B12 and folic acid were examined and a red blood cell count was performed. The patients were divided into three groups (or = 2 years,2 toor = 4 years, and4 years after surgery) as well as into children and adults. In addition, bone mineral density (dual-photon absorptiometry) was measured in 25 patients. Of these, 16 patients were approximately 16.8 years s/p recta…

Role of antibiotic prophylaxis in antenatal hydronephrosis: A systematic review from the European Association of Urology/European Society for Paediatric Urology Guidelines Panel

Summary Background The benefits and harms of continuous antibiotic prophylaxis (CAP) versus observation in patients with antenatal hydronephrosis (ANH) are controversial. Objective The aim was to determine the effectiveness of CAP for ANH, and if beneficial to determine the best type and regimen of antibiotic and the most harmful to provide guidance for clinical practice. Methods A systematic literature search was performed in databases including Medline, Embase, and Cochrane in June 2015. The protocol was prospectively registered to PROSPERO (CRD42015024775). The search started from 1980, when maternal ultrasound was first introduced into clinical practice. Eligible studies were critically…

Urinary diversion in bladder exstrophy and incontinent epispadias: 25 years of experience.

To determine the optimal surgical approach in achieving complete urinary continence with preservation of the upper urinary tract in the exstrophy-epispadias complex we reviewed the records of patients treated at our institution.From 1968 to July 1994, 115 patients with bladder exstrophy/incontinent epispadias were treated of whom followup was available for 104 and 2 died of causes unrelated to urinary diversion. Mean followup is 16.7 years. In 43 of the 102 patients surgery was primarily performed at our institution (urinary diversion in 39, a modified Young-Dees procedure in 1 and sling plasty in 3). In another 59 patients urinary diversion was done secondarily after therapy elsewhere (bla…

Evaluation of sexual function in females with exstrophy-epispadias-complex: a survey of the multicenter German CURE-Net

Standardized knowledge about genital function in adult female individuals with exstrophy-epispadias complex (EEC) is scarce. The aim of this study was to investigate sexual function using the standardized Female Sexual Function Index (FSFI), and to assess the influence of bladder and vaginal reconstruction and the presence of incontinence on FSFI results.Sixty-one females (aged ≥18 years) recruited by the German multicenter network for congenital uro-rectal malformations (CURE-Net) were asked to complete the FSFI and a self-designed semi-structured questionnaire assessing comprehensive medical data, gynecological, and psychosocial items. Twenty-one eligible females (34%) returned both quest…

The Fate Of The Adult Exstrophy Patient

Between 1968 and 1993, 101 patients with bladder exstrophy or incontinent epispadias underwent surgery at our hospital. The standard procedure was ureterosigmoidostomy and additional genital reconstruction. Of the 56 patients who have reached adulthood 45 could be interviewed regarding social integration, sexuality and fertility. All patients have a functioning urinary diversion. Of the 45 patients questioned 41 have completed vocational training or are currently in training, 3 are unemployed and 1 lives at a therapeutic center. Among the patients 29 are married or have a steady partner. All women engage in sexual intercourse and 2 have delivered 3 children by cesarean section. All men achi…

Assisted reproductive techniques and risk of exstrophy-epispadias complex: a German case-control study.

We assessed the risk of exstrophy-epispadias complex in children conceived by in vitro fertilization or intracytoplasmic sperm injection.Data from the German Network for Congenital Uro-REctal malformations were compared to nationwide data from the German In Vitro Fertilization Register and the German Federal Statistical Office. Odds ratios (95% CI) were determined to quantify associations using logistic regression.A total of 123 patients with exstrophy-epispadias complex born in Germany between 1997 and 2011 were recruited through participating departments of pediatric urology and pediatric surgery throughout the country as well as the German self-help organizations Blasenekstrophie/Epispad…

URINARY DIVERSION AND ORTHOTOPIC BLADDER SUBSTITUTION IN CHILDREN AND YOUNG ADULTS WITH NEUROGENIC BLADDER: A SAFE OPTION FOR TREATMENT?

Combined pharmacotherapy, clean intermittent catheterization and infection prophylaxis is currently the gold standard of treatment for neurogenic bladder. However, as the adolescent gains independence from parental supervision, the intervals of clean intermittent catheterization compliance with medical treatment and regularity of followup examinations may decrease, and neurological and/or orthopedic status may change. This situation sometimes leads to failure of conservative treatment, resulting in incontinence and/or deterioration of the upper urinary tract. A multidisciplinary team was established at our institution 30 years ago to assess all aspects of care for patients with neurogenic b…

Operative Reconstruction of the External and Internal Genitalia in Female Patients with Bladder Exstrophy or Incontinent Epispadias

AbstractPurpose: Urologists and gynecologists rarely encounter questions on cohabitation and pregnancy in female patients with bladder exstrophy or incontinent epispadias.Materials and Methods: Until 1994, 41 female patients underwent surgery at our institution (vaginal cutback or vaginoplasty in 23, correction of the external genitalia in 25 and uterus fixation to correct or prevent prolapse in 13).Results: Followup was available in 37 patients (mean 16.8 years, with followup of 18 years or longer in 19). Of the patients 94 percent are satisfied with the cosmetic results. All adults engage in sexual intercourse, 4 delivered 6 children by cesarean section and 5 presently desire children.Con…

Bladder augmentation and urinary diversion in patients with neurogenic bladder: Surgical considerations

Segments from almost all parts of the bowel have been used for urinary diversion. As a result, the available absorptive surface area of the bowel is reduced, and the incorporation of bowel segments into the urinary tract may have metabolic consequences. This is an area somewhat neglected in the literature. Metabolic complications are rare, but sub-clinical metabolic disturbances are quite common. Several studies have demonstrated that some of the absorbent and secreting properties of the bowel tissue are preserved after incorporation into the urinary tract. Hyperchloraemic metabolic acidosis can occur if ileal and/or colon segments are used, as well as malabsorption of vitamin B(12) and bil…

Metabolic Consequences after Urinary Diversion

Metabolic disturbances are well-known, but sometimes neglected immediate consequences or late sequelae following urinary diversion (UD) using bowel segments. Whereas subclinical disturbances appear to be quite common, clinically relevant metabolic complications, however, are rare. Exclusion of bowel segments for UD results in loss of absorptive surface for its physiological function. Previous studies demonstrated that at least some of the absorbtive and secreting properties of the bowel are preserved when exposed to urine.For each bowel segment typical consequences and complications have been reported. The use of ileal and/or colonic segments may result in hyperchloremic metabolic acidosis …

Applications of Ureterosigmoidostomy in Bladder Exstrophy

Until 1994, 95 patients with bladder exstrophy and 20 with incontinent epispadias were operated upon at our department. During the last years more and more exstrophycripples were referred to our institution after primary treatment elsewhere. Thus a total of 56 of the 104 patients had a secondary treatment after failure or unsatisfactory treatment. In contrast to many other institutions, our standard procedure was primary ureterosigmoidostomy and, since 1990 it is completely replaced by a modification of it, the sigma rectum pouch (Mainz Pouch II). After failed primary bladder closure and in patients with short or pathological ureters or insufficient anal sphincter function, we prefer an ile…

Genome-wide Association Study and Meta-Analysis Identify ISL1 as Genome-wide Significant Susceptibility Gene for Bladder Exstrophy

The bladder exstrophy-epispadias complex (BEEC) represents the severe end of the uro-rectal malformation spectrum, and is thought to result from aberrant embryonic morphogenesis of the cloacal membrane and the urorectal septum. The most common form of BEEC is isolated classic bladder exstrophy (CBE). To identify susceptibility loci for CBE, we performed a genome-wide association study (GWAS) of 110 CBE patients and 1,177 controls of European origin. Here, an association was found with a region of approximately 220kb on chromosome 5q11.1. This region harbors the ISL1 (ISL LIM homeobox 1) gene. Multiple markers in this region showed evidence for association with CBE, including 84 markers with…

Expression ofDNMT3A transcripts and nucleolar localization of DNMT3A protein in human testicular and fibroblast cells suggest a role for de novo DNA methylation in nucleolar inactivation

Transcriptional silencing during differentiation of human male germ cells and serum starvation of human fibroblasts is controlled by epigenetic mechanisms that involve de novo DNA methylation. It is associated with high expression of different transcripts of the DNA methyltransferase 3A (DNMT3A) gene that encode two isoforms with de novo methyltransferase activity and one without catalytic activity. Western blots revealed that DNMT3A protein (with catalytic domain) is present at low levels in several tissues and at increased levels in testicular cells and growth-arrested fibroblasts. Immunofluorescence experiments localized DNMT3A to discrete nucleolar foci in B spermatogonia and resting fi…

EAU Guidelines on Vesicoureteral Reflux in Children

Context: Primary vesicoureteral reflux (VUR) is a common congenital urinary tract abnormality in children. There is considerable controversy regarding its management. Preservation of kidney function is the main goal of treatment, which necessitates identification of patients requiring early intervention.Objective: To present a management approach for VUR based on early risk assessment.Evidence acquisition: A literature search was performed and the data reviewed. From selected papers, data were extracted and analyzed with a focus on risk stratification. The authors recognize that there are limited high-level data on which to base unequivocal recommendations, necessitating a revisiting of thi…

The Giessen-Mainz-Frankfurt procedure: a new method for complex pelvic reconstruction for bladder exstrophy.

In bladder exstrophy primary reconstruction remains the gold standard worldwide. Despite various types of osteotomies the permanent correction of pubic diastasis remains a challenge. In maxillofacial surgery callus distraction is a routine treatment for hypoplastic mandibles. Originally described by Ilizarov, this method provides stable and true bone lengthening after gradual distraction of an osteotomy site as long as the periosteum remains intact. In cooperation with the departments of maxillofacial surgery and orthopedics we used this technique to correct pubic diastasis and facilitate phallic reconstruction in a 4 1/2-year-old boy with bladder exstrophy who had previously undergone cont…

Long-Term Followup Of 158 Young Adults Surgically Treated For Vesicoureteral Reflux In Childhood: The Ongoing Risk Of Urinary Tract Infections

We recorded urinary tract infections in the long term after surgical reflux correction.A total of 158 of 189 patients (160 females and 29 males) who were followed in 1985, an average of 10.8 years after reflux surgery were contacted again in 1995. At that time median patient age was 26 years (range 15.7 to 38.8) and the average period of observation was 20.3 years (range 13.4 to 26).In 82% of the patients febrile and in 18% afebrile symptomatic urinary tract infections had developed preoperatively. In the first 10-year period after operation 46% of patients continued to have symptomatic urinary tract infections compared with 52% in the second 10-year interval. In the 2 periods the incidence…

MP-07.07

Continent Anal Urinary Diversion in Classic Bladder Exstrophy: 45-Year Experience

Objective To evaluate the long-term outcomes in patients with classic bladder exstrophy and continent anal urinary diversion (CAD) for continence, upper urinary tract status, secondary malignancies, and sexual function. Patients and Methods The medical records of 82 exstrophy patients having undergone CAD in our department between 1970 and 2015 were reviewed. Patients were invited for follow-up examinations and asked to complete validated questionnaires relating to sexual function. Results Thirty-two of 57 eligible patients with a median follow-up of 23.9 years were included in the study. Ninety-seven percent of patients were fully continent during daytime. Upper urinary tract and renal fun…

Commentary to 'Early clean intermittent catheterization may not prevent dimercaptosuccinic acid renal scan abnormalities in children with spinal dysraphism'.

The authors aimed to address a clinically relevant issue: do we prevent new renal scarring by early administration of clean intermittent catheterization (CIC) in patients with spina bifida? In 2006, Peter Dik and co-workers presented their results of the concept of prophylactic initiation of CIC combined with antimuscarinic therapy in myelodysplastic newborns. Out of 144 children, five patients had pre-existing renal abnormalities, 69 had an overactive sphincter, 27 had reflux, and six had renal scarring. Five of the six patients with renal scarring were put on CIC and antimuscarinic therapy several months after birth. This study provided prima facie evidence that early initiation of CIC an…

Correction of vesicoureteral reflux: where do we stand?

Purpose of review Newer techniques of molecular biology allow us to gain a better understanding of the molecular mechanism of primary urinary reflux. In recent years, diagnostic tools and treatment options for vesicoureteric reflux have dramatically changed. In this review, we want to focus on genetics, molecular biology, histology, clinical findings, diagnostic tools and newer surgical techniques for the treatment of vesicoureteric reflux published between 2003 and March 2004. Recent findings Recent findings have demonstrated that the renin-angiotensin-aldosterone system does not seem to be responsible for the development of primary vesicoureteric reflux. Echo-enhanced ultrasound with cont…

Korporoplastik bei Induratio penis plastica

Mainz pouch continent cutaneous diversion

Sexual Function and Fertility of Women with Classic Bladder Exstrophy and Continent Urinary Diversion

We evaluated the outcome in female patients with classic bladder exstrophy and continent urinary diversion for sexual function and fertility.We reviewed the medical records of female exstrophy patients who underwent continent urinary diversion in our department between 1969 and 2014. Patients were invited for followup examination and asked to complete questionnaires relating to sexual function, social integration and maternity.Of 38 eligible patients 29 (response rate 76%) with a followup of 22.3 years were included in study. Primary continent urinary diversion was done in 62% of patients and 38% underwent secondary continent urinary diversion after failed reconstruction of the exstrophic b…

Retroperitoneal neuroblastomas - a single institution experience in 90 patients

Purpose Nowadays neuroblastomas are detected incidentally by ultrasound in early stages. Primary surgery, chemotherapy and/or radiotherapy are treatment options. However, the approach in babies is controversial. A surveillance strategy is propagated by some groups. In this retrospective study, we evaluated age and stage at diagnosis, therapy and outcome of patients treated at our institutions since 1970. Material and methods 90 patients with retroperitoneal neuroblastomas were treated. They were divided into 2 groups according to age at time of diagnosis:  1 year. Results 40 patients were  1 year (1- 18 years). Detection of an asymptomatic tumour:  1 year 10%. Tumor stage at diagnosis > 1 y…

Corrigendum to “Management of undescended testes: European Association of Urology/European Society for Paediatric Urology Guidelines” [J Pediatr Urol 12 (2016) 335–343]

MP-07.06

Long-Term Results of Buccal Mucosa Onlay Grafts in Hypospadias Repair

Purpose Buccal mucosa onlay grafts are widely used in complex Hypospadias surgery. In a retrospective study we evaluated the long-term results in patients with a minimum follow-up of 5 years. Material and Methods All patients in who underwent buccal mucosa grafting for Hypospadias repair in our institution received a questionnaire and were invited for a follow-up visit. The questionnaire included two visual analogue scales (VAS) concerning the functional and cosmetic result (0 = very dissatisfied; 50 = satisfied; 100 = very satisfied). Results A total of 85 patients with a median age at the operation of 7.4 years (1 – 60 years) could be followed for a median of 8 years (5-16 years). In 36/8…

Long-term outcome after urinary diversion using the ileocecal segment in children and adolescents: Complications of the efferent segment.

Long-term outcomes are of special concern in children after urinary diversion. In a single institution study we evaluated retrospectively the long-term outcomes of urinary diversion in children, in whom the ileocecal segment had been used, in respect to complications of the efferent segment.The Mainz pouch was used in 107 children for continent urinary diversion. Indications were neurogenic bladder (53%, 57/107), exstrophy-epispadias complex (25%, 27/107), malignancy (13%, 14/107), and others (9%, 9/107). Continent cutaneous diversion was performed in 95 patients, and 12 patients received bladder augmentation/substitution with a continent cutaneous stoma. As efferent segment, we used the in…

Long-term results of fascial sling procedure

In a retrospective study 108 patients (average age: 56.5 years) could be evaluated, who were treated with a fascial sling plasty because of pure genuine stress incontinence (n=90) or combined stress/urge incontinence (n=18). 72 patients (66.6%) had recurrent incontinence after previous surgery. The average follow-up was 5 years.

Bladder augmentation using bowel segments (enterocystoplasty)

Genome-Wide Linkage Scan in a Moroccan Family With Autosomal-Recessive Exstrophy of the Bladder Identifies a Novel Susceptibility Locus on Chromosome 3p25.3

Purpose Exstrophy of the bladder (EB) is part of the bladder exstrophy-epispadias complex (BEEC) representing a spectrum of urogenital anomalies in which part or all of the distal urinary tract fail to close and are exposed on the outer abdominal wall. Familial occurrence is rare and previous studies are suggestive of an underlying multifactorial mode of inheritance. However, no causally related genetic or non-genetic factor has been identified so far. In this study, we aim to identify potential risk/modifying loci that might contribute to EB. Material and Methods A genome-wide linkage scan was performed in a consanguineous kindred of Moroccan origin where three affected males showed the sa…

Intermittent hyperammonemic encephalopathy after ureterosigmoidostomy: spontaneous onset in the absence of hepatic failure

Intermittent hyperammonemic encephalopathy after ureterosigmoidostomy is a rare, but if unrecognized, potentially lethal condition. Ureterosigmoidostomy was performed in a male patient with bladder extrophy. After 35 years, he developed hyperammonemic encephalopathy. Diagnostic procedures did not reveal hepatic nor metabolic disorders. Despite administration of preventive medical treatment, several episodes recurred. A durable prevention was finally achieved by conversion into an ileal conduit. Intermittent hyperammonemic encephalopathy can occur decades after ureterosigmoidostomy. In the case of absence of metabolic disorders and resistance to medical treatment, conversion into a urinary d…

Urinary diversion in children and young adults using the Mainz Pouch I technique

Objectives To determine the late complications and consequences for renal function, vitamin and acid-base metabolism after application of the Mainz Pouch I (MZP-I) technique in children and young adults. Patients and methods To November 1994, the MZP-I procedure was carried out in 463 patients at our institution, 91 of whom were children and adolescents (≤20 years old) using bladder augmentation in 21 and a continent cutaneous stoma in 70. A minimum follow-up of 1 year was possible in 87 patients or 163 renal units (RUs) with a mean of 5.5 years (range 1–10.5). Results At the last examination, 23 of 55 (42%) pre-operatively dilated RUs had improved, 131 of the 163 RUs (80%) were stable and …

Phenotype severity in the bladder exstrophy-epispadias complex: analysis of genetic and nongenetic contributing factors in 441 families from North America and Europe.

Objective To identify genetic and nongenetic risk factors that contribute to the severity of the bladder exstrophy-epispadias complex (BEEC). Study design Patients with BEEC from North America (n = 167) and Europe (n = 274) were included. The following data were collected: associated anomalies, parental age at conception, mode of conception, periconceptional folic acid supplementation, maternal risk factors during pregnancy, and environmental risk factors. The patients were divided into 3 subgroups according to phenotype severity: (i) mild, epispadias (n = 43); (ii) intermediate, classic bladder exstrophy (n = 366); and (iii) severe, cloacal exstrophy (n = 31). These subgroups then were com…

Expression of somatic DNA repair genes in human testes

Meiosis is the key process for recombination and reduction of the diploid chromosome set to a haploid one. Many genes that have been found in yeast or mouse models to play a role in meiosis are also important for the repair of DNA damage in somatic cells. To study the DNA repair gene transcriptome during male germ cell development, we have developed a specialized cDNA microarray with 181 human genes which are involved in different somatic DNA repair pathways and/or cell cycle control and 45 control house-keeping genes. This DNA repair gene chip was used to quantify the mRNA expression levels in three human testes samples versus a fibroblast RNA pool. Two hundred twenty genes on the chip (in…

Isolated bladder exstrophy associated with a de novo 0.9 Mb microduplication on chromosome 19p13.12

BACKGROUND: The exstrophy-epispadias complex (BEEC) is a urogenital birth defect of varying severity. The causes of the BEEC are likely to be heterogeneous, with individual environmental or genetic risk factors still being largely unknown. In this study, we aimed to identify de novo causative copy number variations (CNVs) that contribute to the BEEC. METHODS: Array-based molecular karyotyping was performed to screen 110 individuals with BEEC. Promising CNVs were tested for de novo occurrence by investigating parental DNAs. Genes located in regions of rearrangements were prioritized through expression analysis in mice to be sequenced in the complete cohort, to identify high-penetrance mutati…

Sexual Function, Social Integration and Paternity of Males with Classic Bladder Exstrophy following Urinary Diversion.

We evaluated patients with classic bladder exstrophy and a history of urinary diversion for sexual function, social integration and paternity.We reviewed the medical records of males older than 18 years with exstrophy who had undergone urinary diversion at our department between 1969 and 2014. Patients were invited for structured followup examinations and were asked to complete questionnaires relating to sexual function, social integration and paternity.Of 79 eligible patients 39 (49%) with a mean followup of 23.8 years (range 2 to 45) were included in the study. Of the patients 41% had undergone primary urinary diversion and 59% had undergone secondary urinary diversion after failed recons…

Radical surgery and different types of urinary diversion in patients with rhabdomyosarcoma of bladder or prostate--a single institution experience.

Abstract Purpose In a retrospective study we analyzed the outcome of patients treated for rhabdomyosarcoma (RMS) of the bladder/prostate with special attention to radical surgery. Methods In 25 patients with genitourinary RMS (15 bladder/10 prostate) the median age at diagnosis was 4 years [1] , [2] , [3] , [4] , [5] , [6] , [7] , [8] , [9] , [10] , [11] , [12] , [13] , [14] , [15] , [16] , [17] , [18] , and 8 patients had a stage II RMS, 12 stage III and 5 stage IV. In 19/25 (12 bladder/7 prostate), radical surgery and urinary diversion were performed. Urinary diversion comprised 2 continent anal diversions, 11 continent cutaneous diversions, 4 colon conduits and 2 urethral diversions (2 +…

Classic bladder exstrophy: Frequent 22q11.21 duplications and definition of a 414 kb phenocritical region

Background: Classic bladder exstrophy (CBE) is the most common form of the bladder exstrophy and epispadias complex. Previously, we and others have identified four patients with a duplication of 22q11.21 among a total of 96 unrelated CBE patients. Methods: Here, we investigated whether this chromosomal aberration was commonly associated with CBE/bladder exstrophy and epispadias complex in an extended case-control sample. Multiplex ligation-dependent probe amplification and microarray-based analysis were used to identify 22q11.21 duplications in 244 unrelated bladder exstrophy and epispadias complex patients (including 217 CBE patients) and 665 healthy controls. Results: New duplications of …

Long-term followup of the intussuscepted ileal nipple and the in situ, submucosally embedded appendix as continence mechanisms of continent urinary diversion with the cutaneous ileocecal pouch (Mainz pouch I).

We analyzed stoma related complications and continence rates in patients who underwent continent urinary diversion with the cutaneous ileocecal pouch (Mainz pouch I). We compared the intussuscepted ileal nipple and in situ, submucosally embedded appendix as continence mechanisms.A total of 401 patients were included in a retrospective followup study. Continence mechanisms were the intussuscepted ileal nipple in 205 patients and the in situ, submucosally embedded appendix in 196.A total of 144 patients (36%) required intervention for a stomal complication. Of patients who received an intussuscepted ileal nipple 34 (17%) had stomal stenosis at a mean time to first stenosis of 43.8 months, 41 …

Genome-wide association study and mouse expression data identify a highly conserved 32 kb intergenic region between WNT3 and WNT9b as possible susceptibility locus for isolated classic exstrophy of the bladder

Item does not contain fulltext Bladder exstrophy-epispadias complex (BEEC), the severe end of the urorectal malformation spectrum, has a profound impact on continence as well as sexual and renal functions. It is widely accepted that for the majority of cases the genetic basis appears to be multifactorial. Here, we report the first study which utilizes genome-wide association methods to analyze a cohort comprising patients presenting the most common BEEC form, classic bladder exstrophy (CBE), to identify common variation associated with risk for isolated CBE. We employed discovery and follow-up samples comprising 218 cases/865 controls and 78 trios in total, all of European descent. Our disc…

Hypospadias and bladder exstrophy

Reconstructive surgery for patients with hypospadias, bladder exstrophy and cloacal exstrophy is challenging. Over the last few years, more and more data have become available concerning incidence and epidemiology, as well as short- and long-term results of surgical techniques. The tubularized incised plate urethroplasty for hypospadias repair continues to gain in popularity. However, the results of long-term studies of larger series supporting this enthusiasm are still missing. Buccal mucosa is more frequently used in complex hypospadias cases with good short- and long-term results. The one-stage approach of exstrophy repair seems to be promising. However, failures, which require very comp…

Treatment of Patients with Bladder Exstrophy or Incontinent Epispadias

Objective: To determine the late outcome concerning urinary continence, late complications, sexuality, and fertility in patients with the exstrophy-epispadias complex. Methods: Until July 1994, 115 patients underwent surgical treatment at our institution. The mean follow-up period in 102 patients is 16.7 years. Urinary diversion was performed in 88 patients, a modified Young-Dees procedure in 8, a sling plasty in 3, and genital reconstruction alone in 3 patients. Results: The present continence rates are 96% for rectal reservoirs, 97% for Mainz pouch I, and 67% for the modified Young-Dees procedure. The upper tracts have remained stable, and no bowel neoplasms have developed. 16 of 17 women…

Acid-Base Balance after Continent Urinary Diversion in Childhood – Risk of Imbalance Despite Medication

Purpose Continent urinary-intestinal diversion can lead to alterations of the acid-base balance, acutely and chronically, the latter possibly causing bone demineralization. Annual check-ups have therefore been recommended for decades. Material and Methods At our institution, 89 children underwent continent urinary diversion by ileocecal pouch or bladder substitution between 1984 and 2006. In 58 of these patients (age 3-17 y., median 13.9 y.), we performed a minimum of 3 (range 3-23, median 6.2) consecutive annual blood-gas analyses over a follow-up period of up to 23 years (median 11.0) after surgery. At a base excess (BE) (as marker of the acid-base-balance) value below -2.5 mmol/l, treatm…

Frühzeitig mit der Therapie beginnen

Der Maldeszensus testis ist die haufigste genitale Fehlbildung des Mannes. Unbehandelt kann er nicht nur die Fertilitat beeintrachtigen, sondern erhoht auch das Risiko fur die Entstehung eines Hodentumors. Um diese Gefahren zu minimieren, sollte die Therapie moglichst bis zum 12. Lebensmonat abgeschlossen sein.

Ossifying renal tumor of infancy

A renal ossifying tumor of infancy is a rare event with few cases having been published, and the etiology has not yet been established. We report on two new cases of this unusual neoplasm. A 2-year-old boy presented with intermittent painless gross hematuria. After several diagnostic procedures, an open pyelolithotomy was performed and the histological diagnosis of renal tumor of infancy was finally made. The history of the second case is very similar. An 8-week-old infant presented with gross hematuria. As in the first case, an open pyelolithotomy was performed and a tumor entirely covered with blood clots was found in the renal pelvis and completely removed. A histological diagnosis of re…

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

Context: A summary of the 2nd International Consultation on Bladder Cancer recommendations on the reconstructive options after radical cystectomy (RC), their outcomes, and their complications. Objective: To review the literature regarding indications, surgical details, postoperative care, complications, functional outcomes, as well as quality-of-life measures of patients with different forms of urinary diversion (UD). Evidence acquisition: An English-language literature review of data published between 1970 and 2012 on patients with UD following RC for bladder cancer was undertaken. No randomized controlled studies comparing conduit diversion with neobladder or continent cutaneous diversion…

Reflections on the 25th Congress of the European Society for Pediatric Urology Part 1

The 25th annual meeting of the European Society for Pediatric Urology (ESPU) took place in Innsbruck, Austria from the 7th to 10th of May 2014. The Specialty Chief Editor and nine Associate editors of Frontiers in Pediatrics, Pediatric Urology were in attendance. Each was asked to select the two presentations that they considered most significant. Here, we highlight some of the papers that we found of particular interest and that may impact our practices. This report reflects personal preferences of the authors and does not pretend to be comprehensive or to rank the quality of the presentations. We are aware that some important presentations may not be included. Our selections are presented…

Presentation, Management and Long-Term Outcome of Ureteropelvic Junction Obstruction in Duplex Kidneys.

Ureteropelvic junction obstruction in association with a duplex collecting system is a rare but challenging upper urinary tract pathology. We report our 21-year experience with this anomaly in terms of presentation, diagnostic evaluation and management.We retrospectively identified all patients with ureteropelvic junction obstruction in a duplex collecting system between 1991 and 2012. We reviewed each case for presenting symptoms, anatomy and management. Median followup was 10.8 years (range 2 to 22).Ureteropelvic junction obstruction in duplex kidneys was diagnosed in 21 patients. Ten patients presented with clinical symptoms such as flank pain and urinary tract infection but 11 were asym…

Genome-wide analysis for micro-aberrations in familial exstrophy of the bladder using array-based comparative genomic hybridization

OBJECTIVE: Exstrophy of the bladder (EB) is part of the bladder-exstrophy-epispadias complex (BEEC). Because familial occurrence of BEEC is rare, exogenous factors are thought to play a major role in the etiology of most BEEC cases. We aimed to investigate a possible genetic basis of BEEC in a consanguineous kindred of Moroccan origin with three members showing the same phenotypic expression of BEEC. PATIENTS AND METHODS: The three affected males (two cousins and their maternal uncle) all presenting with nonsyndromic classic EB, were born in Morocco or The Netherlands. One Moroccan patient had an open bladder surface for 22 years due to late surgical reconstruction, avoided upright posture …

Rectosigmoid Pouch (Mainz Pouch II) in Children

Continent anal urinary diversion is a therapeutic option in bladder exstrophy. We report our long-term results with the rectosigmoid pouch (Mainz pouch II), a modification of the classic ureterosigmoidostomy.A total of 38 children with a mean age of 5 years (range 0.5 to 17) underwent a Mainz pouch II procedure between 1991 and 2004. Most patients (33) had bladder exstrophy or incontinent epispadias. In 14 children (37%) urinary diversion was performed after failed primary reconstruction. In 6 children conversion was performed from an incontinent type of urinary diversion. Renal function, continence and metabolic changes were analyzed. A total of 35 children were followed for a mean of 112 …

Editorial Comment on: In Vitro Investigations of Tissue-Engineered Multilayered Urothelium Established from Bladder Washings

Pregnancy After Urinary Diversion at Young Ages-Risks and Outcome.

Objective To assess the urologic and obstetric outcomes during and after pregnancy following urinary diversion (UD) performed during childhood or adolescence. Materials and Methods From our UD database, we identified 25 women who became pregnant between 1981 and 2013. Reasons for UD were neurogenic bladder, exstrophy, trauma, sinus urogenitalis, and interstitial cystitis. Seventeen had continent cutaneous diversion, 4 had continent anal diversion, and 4 had colonic conduit. Results The average age at delivery was 27.8 (18-39) years. Thirty-seven pregnancies occurred; 1 patient decided for an induced abortion. Thirty-two healthy children were born. Five patients had a spontaneous abortion be…

Sexual Function and Quality of Life in Adult Male Individuals with Exstrophy-Epispadias Complex—a Survey of the German CURE-Network

Objective To investigate sexual function and quality of life (QoL) in adult male individuals with exstrophy-epispadias complex (EEC). Data from the German Network for Congenital Urorectal Malformations (CURE-Net) were used. Patients and Methods Fifty-one male participants (≥18 years) recruited by CURE-Net between 2009 and 2012 were re-contacted per mail and asked to fill out 4 questionnaires including International Index of Erectile Function (IIEF-5), Cologne Assessment of Erectile Dysfunction (KEED), the Short-Form 36 (SF-36), and one self-designed questionnaire about their medical history, current health status, and sexual experience. The SF-36 results were compared with general German po…

Psoas hitch and Boari flap ureteroneocystostomy

Influence of maternal diet on the development of malformation of the external genitalia – Are we ready to make some recommendations?

The influence of our environment on the development of malformation is well known and, again and again, discussed with great enthusiasm. For example, the supplementation of the diet with folic acid has been proven to prevent neural tube defects already in 1991 [1, 6]. However, despite this well–known fact, only 10.6% of women take folic acid at least four weeks before getting pregnant; although 60% know the increased risk. It was interesting to see, that there were no big differences between the social groups [7]. Some epidemiological studies suggest an increasing risk of disorders / malformation of the external genitalia in males. The influence of the hormonal environment, especially of an…

Conversion from colonic conduit into recto-sigmoid pouch (Mainz pouch II)

The Mainz Pouch II has now been used widely and found to be a valuable addition to the various types of urinary diversion. The authors from Mainz describe how a colonic conduit can be converted into a Mainz Pouch II, and the expected outcome from such a procedure. OBJECTIVE To report our long-term results of conversion from conduit conversion into a continent anal urinary diversion, as after conduit urinary diversion in childhood, some patients wish to have a later conversion to a continent diversion to avoid external appliances and to improve their quality of life. PATIENTS AND METHODS Between 1992 and 2003, 139 patients had a urinary diversion with a recto-sigmoid pouch (Mainz pouch II), …

Radical Surgery in Children and Adolescents with Rhabdomyosarcoma of the Lower Urinary Tract Using Orthotopic Bladder Substitution and Heterotopic Urinary Diversion – A Single Institution Experience

Abstract Purpose Children with rhabdomyosarcoma(RMS)of the bladder/prostate can be treated by radical or organ sparing surgery both in combination with chemotherapy ± radiation therapy. In this retrospective study we analyzed the outcome with special attention to radical surgery and urinary diversion (UD). Material and Methods 24/32 patients with RMS of the genitourinary tract had RMS of the bladder (n=14) or prostate (n=10). 8 patients were stage II, 11 stage III and 5 stage IV. In 18/24 with RMS of the bladder/prostate, radical surgery and urinary diversion was performed. 3 had chemotherapy only, 2 in combination with radiotherapy. One patient underwent partial cystectomy. For UD: uretero…

Strategies for reconstruction after unsuccessful or unsatisfactory primary treatment of patients with bladder exstrophy or incontinent epispadias.

Following unsuccessful or unsatisfactory primary treatment in patients with the epispadias/exstrophy complex, the options for a surgical solution to preserve the upper urinary tract, to achieve complete continence, and to reconstruct the external and female internal genitalia are limited. We reviewed the records of the patients treated at our institution to determine a surgical compromise between ingenious operative constructions and patient desires, both of which are secondary to stabilization of renal function.From 1967 to December 1997, 128 patients with bladder exstrophy/epispadias complex were treated, of 80 whom had received previous unsuccessful or unsatisfactory treatment. Of these …

MP-16.10

CONVERSION FROM COLONIC OR ILEAL CONDUIT TO CONTINENT CUTANEOUS URINARY DIVERSION

ABSTRACT Purpose: After ileal or colonic conduit diversion some patients, particularly adolescents, desire conversion to a continent diversion to improve quality of life. We report our long-term results on conversion from conduit diversion to continent cutaneous diversion. Materials and Methods: Between 1986 and 2001, 39 patients (mean age 24 years, range 6 to 49) underwent conversion from a colonic (21) or ileal conduit (18) to an ileocecal pouch (Mainz pouch I) with a mean followup of 102 months (range 18 to 192). Conversion was performed after a mean of 11 years (range 1 to 36) of conduit urinary diversion by incorporating the preexisting colonic/ ileal conduit and the ileocecal pouch. R…