Recent Trends in the Management of Bladder Exstrophy: The Gordian Knot Has Not Yet Been Cut

Although enormous effort has been made to further improve the operative techniques worldwide, the management of bladder exstrophy (BE) remains one of the most significant challenges in pediatric urology. Today it is universally agreed that successful and gentle initial bladder closure is decisive for favorable long-term outcome with regard to bladder capacity, renal function and continence. Due to a number of reasons, including a lack of comparable multicenter studies, a range of concepts is currently used to achieve successful primary closure. We review the literature of the last 15 years on the current concepts of bladder exstrophy repair with regard to the time of primary closure (initial vs. delayed closure), the concepts of primary closure (single-stage vs. staged approach; without osteotomy vs. osteotomy) and their outcomes. There is a worldwide lack of multicenter outcome studies with adequate patient numbers and precisely defined outcome parameters, based on the use of validated instruments. The modern staged repair (MRSE) in different variations, the complete primary reconstruction of exstrophy (CPRE), and the radical soft-tissue mobilization (RSTM) had been the most extensively studied and reported procedures. These major concepts are obligatory stable now for more than 20 years. Nevertheless, there are still a lot of open-ended questions e.g., on the potential for development of the bladder template, on continence, on long-term orthopedic outcome, on sexuality and fertility and on quality of life. Management of BE remains difficult and controversial. Further, clinical research should focus on multi-institutional collaborative trials to determine the optimal approach

Expression of Low Affinity Nerve Growth Factor Receptor p75 in Classic Bladder Exstrophy

Successful primary closure of classic bladder exstrophy (BE) is crucial for development of bladder capacity and voided continence. It is universally agreed that an intensive pain management including the use of caudal epidural anesthesia is an essential cornerstone for the outcome of this complex surgery. Whether and to what extent pain is caused by structural or functional changes is not yet known. The nerve growth factor (NGF) is regarded as a marker for pain in different bladder disorders. This prospective study investigated the role of histological alterations and NGF in patients with BE including 34 patients with BE and 6 patients with congenital vesicoureterorenal reflux (VUR) who served as controls. Between January 2015 and April 2020 transmural bladder biopsies were taken from the posterior bladder wall during delayed primary bladder closure. The samples were stained for histological evaluation and subjected to immunohistochemistry to analyze NGFR p75. Differences in histological alterations were examined with Fisher's exact test, and Mann-Whitney-U-test was used to compare the NGFR p75 staining intensity between patients with BE and controls. Patients with BE showed significantly more often acute inflammation (p < 0.001), squamous metaplasia (p = 0.002), and cystitis glandularis (p = 0.005) as well as NGFR p75 in the urothelium (p = 0.003) than patients with VUR. A limitation of this study is the small number of participants due to the rare disease entity. Similar to other painful bladder disorders, pain transmission in BE after intitial closure may in part be facilitated by elevated NGF signaling through its receptor

Zinner syndrome and infertility─a literature review based on a clinical case

Zinner syndrome (ZS) is a rare congenital malformation associated with seminal vesicle cysts, ejaculatory duct obstruction, and ipsilateral renal agenesis. The main treatment focus so far has been on symptomatic patients. Therefore, surgery has been reserved for these patients, and surgical treatment is mainly aimed at pain relief. ZS seems to be frequently associated with infertility, but diagnosing is challenging, particularly during adolescence. This literature review of ZS and infertility is based on the medical report of one adolescent patient

Ureteroureterostomy in patients with duplex malformations: does a large diameter of the donor ureter affect the outcome?

Introduction Ureteroureterostomy is a commonly adopted, minimally invasive approach in the management of duplex anomalies requiring diversion, e.g., ectopic upper pole ureters. Objective The authors hypothesized that a large diameter of the donor ureter could affect the outcome of this procedure. Study design Forty-two patients from two centers were retrospectively reviewed. To compare patients with small (group 1) vs large donor ureters (group 2), they were split at the median of the sonographically measured diameter at the level of the future anastomosis (n = 20 = 1.2 cm, mean 1.75 cm; P = 1.2 cm is not factoring adversely into the occurrence of postoperative complications. Conclusion A donor ureter diameter >= 1.2 cm in ureteroureterostomy was not associated with a higher complication rate or worse outcome considering further fUTIs or reoperations. The postoperative reduction in hydronephrosis grade was more pronounced in patients with large donor ureters with disappearance of the preoperative significant difference between the two groups

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

OBJECTIVE To further investigate associated anomalies in exstrophy-epispadias complex (EEC) patients con- genital 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. MATERIALS AND METHODS 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 as well as from a physical examination during a physician's interview, classified according to the international statistical classification of diseases and related health problems and grouped with the London Dysmorphology Database. Descriptive statistical analyses were performed. RESULTS Majority of participants were male (68%) and expressed the classical bladder exstrophy phenotype (71%). Exstrophy variants occurred significantly more often in newborns (21%, P < .0001). Anomalies such as inguinal hernias, skeleton, and joint anomalies were equally present in both groups (P = .65 and P = .67). Heart defects were seen more often in newborns (6%) than in the cross-sectional group (1%; P = .033) and the general German population (1%). In total, 59% of the prospective and 48% of the cross-sectional patients had associated anomalies outside the spectrum (P = .16). CONCLUSION Phenomenological multicenter data confirmed the dimension of associated anomalies inside and outside the EEC spectrum. The detected anomalies are either important in preparing for the primary reconstruction or later in long-term follow-up. Associated anomalies of EEC should be spotlighted during routine check-up in all EEC patients. (C) 2018 Elsevier Inc