Laparoscopic Ureteroureterostomy vs. Common Sheath Ureteral Reimplantation in Children With Duplex Kidney Anomalies

Purpose: Laparoscopic ureteroureterostomy (LUU) has been proposed as an alternative to common sheath ureteral reimplantation (CSUR) in children with symptomatic duplex kidneys. However, data is limited for LUU in the pediatric population. The aim of this study was to analyze our experience with LUU and to compare the results with those after CSUR to assess whether a less invasive surgical approach could be a valid alternative. Patients and methods: The data of all children with duplex kidneys who underwent either LUU or CSUR at our center from 2006 to 2018 were reviewed retrospectively. After parental counseling, the option of LUU was provided as an alternative to CSUR for unilateral procedures and in the absence of vesicoureteral reflux to the receiving ureter. Baseline characteristics, indication for surgery, hospitalization and operative times, and intraoperative, post-operative, and late complications were analyzed. Preoperative and 1-year post-operative sonographies were reviewed by a pediatric radiologist. Increasing renal pelvic diameter (Δ >5 mm) was regarded as a sign of ureteral obstruction. Results: Forty children were included in this study, with 16 children receiving LUU and 24 children receiving CSUR. The children had a mean age of 2.7 years (7 months-9.8 years) and were followed up in our outpatient clinic for an average of 3.9 years (3 months-10.6 years) after surgery. The median hospital stay was 2 days shorter after LUU. Initially, a considerably longer time was needed for LUU, but after more experience was gained, similar operative times were observed for both procedures. Complications were encountered in both groups. After LUU, two patients developed anastomotic leakage: one was managed conservatively, and one required temporary nephrostomy. In the CSUR group, one patient developed vesicoureteral obstruction during follow-up and required reoperation with LUU. The occurrence of post-operative urinary tract infections was similar in both groups. No complications related to the ureteral stump after LUU arose. Conclusion: LUU is a safe and efficacious treatment option for children with duplex kidney anomalies and can be used as an alternative to CSUR. All children receiving LUU showed a non-obstructive, patent anastomosis and no signs for stenotic compromise of the receiving ureter

Pre- and postnatal imaging of a girl with a cloacal variant

We describe the prenatal MR findings in a 29-week fetus with a cloacal variant (urogenital sinus and anterior placed anus) in combination with an enlarged clitoris and urethral duplication and correlate them with postnatal imaging. Fetal MR imaging permits the diagnosis and characterization of cloacal and urogenital sinus malformations in utero. This information may guide pre-, peri- and postnatal managemen

Tissue Engineering in Pediatric Bladder Reconstruction—The Road to Success

Several congenital disorders can cause end stage bladder disease and possibly renal damage in children. The current gold standard therapy is enterocystoplasty, a bladder augmentation using an intestinal segment. However, the use of bowel tissue is associated with numerous complications such as metabolic disturbance, stone formation, urine leakage, chronic infections, and malignancy. Urinary diversions using engineered bladder tissue would obviate the need for bowel for bladder reconstruction. Despite impressive progress in the field of bladder tissue engineering over the past decades, the successful transfer of the approach into clinical routine still represents a major challenge. In this review, we discuss major achievements and challenges in bladder tissue regeneration with a focus on different strategies to overcome the obstacles and to meet the need for living functional tissue replacements with a good growth potential and a long life span matching the pediatric population

Prenatal Megacystis - Is Prediction of Outcome and Renal Function Possible?

Abstract Objective To assess prenatal prognostic criteria for fetuses with megacystis in order to counsel parents. Methods In a retrospective observational study at a single tertiary referral center, we assessed the clinical course of 53 fetuses with megacystis cared for at the Department of Obstetrics of the University Hospital Zurich between 1995 and 2008 and followed them up for 2 to 12 years. We determined fetal karyotype, amniotic fluid volume and fetal urinary biochemistry as prenatal prognostic factors. The renal function of survivors was grouped according to age-related creatinine values. Using logistic regression analysis, gestational age-dependent discrimination curves and corresponding ROC curves for fetal urine, beta-2 microglobulin, osmolarity and chloride were calculated. Results 43 out of 53 fetuses underwent vesicocentesis, and spontaneous remission occurred in 3 fetuses. 15 fetuses survived, termination of pregnancy was requested in 23 cases, and 12 neonatal and 3 intrauterine deaths were observed. Reduced amniotic fluid volume showed a significant (p = 0.0027) increase of impaired renal function or perinatal death. Discrimination between survivors and non-survivors was complete for fetal urine beta-2 microglobulin with an area under the curve (AUC) of 1.0. For fetal urine osmolarity and fetal urinary chloride, the AUC was 0.81 and 0.76, respectively. Conclusion The assessment of prognosis for fetal megacystis should include fetal k aryotyping, determination of amniotic fluid and assessment of fetal urine biochemistry. Gestational age-dependent regression lines disclose clinically relevant discrimination and can be used as selection criteria for fetal interventions and parental counselling.</jats:p

A rare case of symptomatic hydrometrocolpos in a 5y old female

Late presentation of symptomatic hydrometrocolpos is uncommon. We present a 5 years old continent girl with prenatally diagnosed multicystic dysplastic left kidney and late-onset of lower abdominal pain. Investigations revealed a nonfunctioning left kidney with an ectopic ureter draining into the left hemivagina, and a vaginal duplication with an obstructed and urine-filled left hemivagina. Surgical therapy included resection of the vaginal septum and laparoscopic nephroureterectomy. Not only renal agenesis but also dysplasia or multicystic kidney may part of an OHVIRA syndrome. In girls with unilateral renal dysplasia, a duplication of the internal genitalia must always be considered

Urological Outcome after Fetal Spina Bifida Repair: Data from the Zurich Cohort

INTRODUCTION Neurogenic lower urinary tract dysfunction (NLUTD) represents a severe burden for patients with open spina bifida (OSB). The effect of fetal OSB repair on the urological outcome remains unclear, as controversial data exist. The aim of this study was to further increment existing outcome data and to demonstrate that our earlier published positive preliminary results are not erratic. METHODS Data from standardized urological follow-up appointments of patients with fetal OSB repair operated at our center were analyzed. Data were obtained from urodynamic studies (UDSs) and radiologic exams performed in the newborn (gestational age 37-39 weeks), at ages of 6, 12, 18, and 24 months, and then at yearly intervals. RESULTS Of 82 patients (mean age 2.6 years, range 6 months to 7 years), 26 (32%) had a normal bladder function as demonstrated by UDSs. Of the 56 (68%) patients with NLUTD, 29 (51%) patients showed initially a normal UDS, but developed NLUTD in the follow-up, 19 (66%) of them spontaneously and another 10 (34%) in association with growth and development, or surgery of inclusion cysts. Radiologic abnormalities (upper tract dilatation and vesico-uretero-renal reflux) were seen in 15%, mainly patients with NLUTD. CONCLUSION Our results add an important set of information to the existing body of evidence. The data reconfirm our earlier published favorable preliminary results and support other studies that show a possible benefit of prenatal OSB repair on the urological outcome, but they also demonstrate that the positive effect remains limited

[Living with bladder exstrophy - the patients' perspective]

Bladder exstrophy (BE) is a rare but severe urogenital malformation that requires complex clinical management. It can lead to physical restriction as well as to psychosocial and psychosexual malfunction. For the first time, this qualitative study describes the experiences of persons living with BE from childhood into adulthood. The primary goal was to explore the experiences of persons with BE in order to optimise treatment, care and counselling at the children hospital in Zurich. Semi-structured interviews were conducted with 4 women and 16 men (38 to 68 years). Using content analysis techniques, three major categories with two respectively four and five subcategories emerged from the data. The first category "having a good life despite BE" describes the perception of the affected persons to lead a life worth living despite BE. It was categorised by episodes and experiences that were repeated throughout the persons' life, such as "being in hospital", "living with symptoms", "being different", "showing oneself" and "gathering information". The second category "the affected ones" demonstrated that "parents" and "partners" were affected too and provided considerable support to the person with BE. The third category "the influencing factors" revealed that the perception of living with BE was influenced by individual and contextual factors. The results of this study illustrated that persons with BE can live a good life, despite repeated health-related challenges, if the support provided by their caregivers permits a positive attitude towards their illness. For this, patients with BE and their family members require continuous and highly competent treatment, care and education by an interdisciplinary team