Epispadias

BACKGROUND: Bladder exstrophy (BE) poses challenges both during the surgical repair and throughout follow-up. In 2013, a multi-institutional BE consortium was initiated, which included utilization of unified surgical principles for the complete primary repair of exstrophy (CPRE), real time coaching, ongoing video capture and review of video footage, prospective data collection, and routine patient data analysis, with the goal of optimizing the surgical procedure to minimize devastating complications such as glans ischemia and bladder dehiscence while maximizing the rate of volitional voiding with continence and long term protection of the upper tracts...

Epispadias is a malformation of the urethra in which the urethral opening ends on the dorsum of the penis. It is considered a rare anomaly, especially when associated with urethral duplication. Urethral duplication is another congenital anomaly that has different clinical manifestations and a separate classification. In this case, we present a 4-year-old boy who underwent epispadias repair and was found to have an accidently duplicated urethra of Effiman's Type ⅡA during the operation. Excision of the accessory urethra was performed, and the patient tolerated the procedure well...

In less than 10% of cases, males may have isolated epispadias, which is caused by failure in the urethral tubularization process, leading to dorsal urethral defect. This case report presents a unique instance where epispadias was associated with ambiguous genitalia. A 5-year-old boy diagnosed with epispadias. The penis resembled external female genitalia, with scrotal skin covering it. He underwent a two-stage operation without complications. The aim of the surgical techniques is to correct these anomalies and restore urinary continence and sexual function...

Palliative care in the field of urology has largely been limited to adult oncologic conditions. Although there is a plethora of established literature suggesting the advantageous impact of palliative care, there is limited integration of palliative care in adult urology. This underutilization is further exacerbated in pediatric urology, and palliative care in pediatric urology remains an underexplored area despite the prevalence of several life-limiting conditions in this patient population. This paper highlights the potential need for palliative care intervention in a variety of urologic conditions in the pediatric population, including congenital lower urinary tract obstruction, neurogenic bladder dysfunction, exstrophy-epispadias complex, and congenital bilateral renal agenesis...

PURPOSE: This study aims to evaluate the long-term outcome of the serous-lined extramural continent catheterizable outlet procedure (SLECCOP) associated with ileal bladder augmentation in children. METHODS: This was a monocentric and retrospective study (2002-2021) that included children (<18 years) undergoing the SLECCOP associated with W-shaped ileocystoplasty with a catheterizable channel (Abol-Enein and Ghoneim procedure). Patients who received other types of bladder augmentation or W-shaped ileocystoplasty without a catheterizable channel were excluded...

INTRODUCTION: Bladder exstrophy-epispadias complex is a rare condition that necessitates numerous surgical procedures during a patient's youth to achieve adequate urine storage and continence. This study aimed to identify the specific needs and functional challenges faced by adults who underwent pediatric bladder exstrophy reconstructions and assessing the management of these issues in an adult population. METHODS: A retrospective chart review was conducted for all bladder exstrophy complex patients who underwent surgery at a young age and were subsequently referred to our center between 2005 and 2020...

PURPOSE: An overexpression of nerve growth factor (NGF) in the urothelium is discussed to lead to neuronal hyperinnervation of the bladder detrusor. The aim was to assess the sensory and sympathetic innervation of the detrusor in unclosed exstrophic bladders patients with known overexpression of NGF in the urothelium. METHODS: Full-thickness bladder biopsies were prospectively obtained from 34 infants at delayed primary bladder closure between 01/2015 and 04/2020...

INTRODUCTION: The bladder exstrophy-epispadias complex is a rare congenital disease. Urothelial carcinomas rarely occur in patients with this disease, and there have been few reports on its treatment. CASE PRESENTATION: We report the case of a 44-year-old man with a hemorrhage from the external urethral meatus. He was diagnosed with bladder exstrophy-epispadias complex and underwent urinary diversion with substitution cystoplasty and Mitrofanoff appendicovesicostomy...

INTRODUCTION: Epispadias, which occurs on the more mild end of the Bladder Exstrophy Epispadias Complex (BEEC) spectrum, presents still with a wide range of severity in boys, from mild glanular epispadias to penopubic epispadias with severe urethral and bladder neck defects. Surgical management ranges from isolated epispadias repair to epispadias repair with bladder neck reconstruction (BNR) with or without pelvic osteotomies. OBJECTIVES: We aimed to evaluate outcomes in epispadias treated at three institutions prior to formation of a formal collaboration...

Bladder exstrophy is a rare congenital anomaly in which the urinary bladder develops outside the body. It may be part of the bladder epispadias-exstrophy-cloacal complex, which is a serious embryological defect where a spectrum of abnormalities involving the urinary tract, genital tract, musculoskeletal system, and intestinal tract is seen. Primary signet ring cell adenocarcinoma (PSRCA) arising in exstrophy of the urinary bladder is extremely rare with only a few cases reported in the literature. A 32-year-old man with congenital, untreated bladder exstrophy presented with a large fungating mass on the lower abdominal wall...

Penile torsion is the abnormal three-dimensional twisting of penile corporal bodies. It can be classified as mild, moderate, or severe, depending on the degree of torsion. Severe penile torsion (>90°) is a very rare condition, with an estimated incidence of 0.4%-1% among all penile torsion cases. Our patient was a 37-year-old man complaining of a 2-year history of lower urinary tract symptoms. These symptoms appeared after the patient sustained an iatrogenic injury during Foley catheter insertion. Physical examination incidentally revealed an obvious counterclockwise penile rotation of 180°...

BACKGROUND: The exstrophy-epispadias complex is a spectrum of ventral wall malformations including classic bladder exstrophy (CBE) and cloacal exstrophy (CE). Patients undergo multiple soft-tissues procedures to achieve urinary continence. If unsuccessful bladder neck closure (BNC) is performed, muscle flaps may be used to reinforce BNC or afterwards for fistula reconstruction. In this study, patients reconstructed using a rectus abdominis or gracilis muscle flap were reviewed. METHODS: A retrospective cohort study of exstrophy-epispadias complex patients who underwent BNC and had a muscle fap was performed...

Specialist facilities for children are still unavailable in some Sub-Saharan African contexts. It is the case of pediatric urology, whose recent advances are still largely unshared. Prenatal diagnosis of urinary abnormalities (CAKUT) is largely unknown. Early recognition and referral of Undescended testis (UDT), Hypospadia, bladder exstrophy epispadias complex, ambiguous genitalia, stone disease, and tumours are uncommon in rural areas. Missed diagnosis is not uncommon and delayed management is associated with poor outcomes...

PURPOSE: Appropriate perioperative management is crucial in patients undergoing classic bladder exstrophy closure (CBE). Therefore, the authors sought to review their intra and postoperative management of patients with CBE undergoing primary closure and examine the impact of this pathway on patient outcomes. METHOD: A prospectively maintained institutional approved exstrophy-epispadias complex database was reviewed for patients with CBE who had undergone primary closure between 2016 and 2022 and whose closure was performed within one year of age...

BACKGROUND: This study aims at investigating the continence outcome in primary epispadias patients treated at a tertiary center. The authors hypothesized that additional continence procedures following primary epispadias repair is not routinely needed. METHODS: Patients treated for primary epispadias at the authors' institution between 2007 and 2019 and toilet trained, were identified from a prospective maintained database. Males underwent chordee correction, urethroplasty and glanuloplasty...

Pelvic osteotomies are essential to approximate widened symphysis pubis in the exstrophy-epispadias complex, yet it is unknown which osteotomy type has the greatest effect on pelvic volume. We therefore used virtual surgery to study pelvic volume change with anterior, oblique, and posterior iliac osteotomies. Preoperative CT scans of two cloacal and one classic bladder exstrophy patients were used. Simulations were free-hand or constrained to keep minimal strain in the sacrospinous SSL and sacrotuberous STL ligaments...

The development of the new S3 guideline "Epidemiology, diagnosis, treatment and follow-up of the bladder exstrophy-epispadias complex" was funded by the German Innovation Fund of the Federal Joint Committee (G-BA). Despite the relatively low level of evidence of the identified literature, a systematic approach and consistent evaluation of the literature enabled the formulation of a large number of evidence-based recommendations across a variety of topics. In addition, a patient guideline is under development in order to disseminate the guideline recommendations and to enhance self-management and understanding among patients and their relatives...

Bladder exstrophy epispadias complex (BEEC) encompasses a spectrum of conditions ranging from mild epispadias to the most severe form: omphalocele-bladder exstrophy-imperforate anus-spinal defects (OEIS). BEEC involves abnormalities related to anatomical structures that are proposed to have a similar underlying etiology and pathogenesis. In general, BEEC, is considered to arise from a sequence of events in embryonic development and is believed to be a multi-etiological disease with contributions from genetic and environmental factors...

Epispadias is a congenital malformation marked by the failure of the urethral bulb to tubularize dorsally. This results in a wide-open urethral plate dorsally. Epispadias is frequently associated with bladder exstrophy-epispadias complex.  We report the case of a five-year-old patient who presented in the outpatient department with isolated epispadias without any other associated abnormality. This report aims to document this rare case of isolated male epispadias with incontinence and the success of using the modified Cantwell-Ransley technique for its treatment...

BACKGROUND: With improved operative techniques pregnancy rates have been rising in patients with anomalies of the extrophy-epispadias-complex, including also female patients with bladder extrophy. Specific risks around pregnancy need to be addressed sufficiently beforehand. CASE PRESENTATION: An unplanned pregnancy was detected at 34 weeks in a 39-year old White female patient with former complex bladder extrophy. Decades after her operation she had not received any follow-up medical care and believed to be unable to conceive due to her anomaly...