Isolated female epispadias without bladder exstrophy is a rare congenital anomaly affecting 1 in 484,000 females. The presenting features of female epispadias are urinary incontinence and abnormal anatomical features. A 6-year-old girl presented with primary urinary incontinence who on physical examination had a bifid clitoris and labia minora. The vagina and hymen were normal. Voiding cystourethrogram showed no reflux and a funnel shaped proximal urethra. With the diagnosis of isolated female epispadias, one-stage reconstruction of the urethra, bladder neck, labia minora and clitoris was performed.

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http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5371683PMC
http://dx.doi.org/10.21699/ajcr.v8i2.548DOI Listing

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  • The case presented involves an 8-month-old female with an unusual urinary opening in the prepubic area, confirmed through various diagnostic tests, and successfully treated with surgery to remove the accessory urethra.
  • Accurate diagnosis through imaging is crucial, and treatment must be customized based on the specifics of the anomaly; the case emphasizes the need for careful management of urethral duplication.
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Article Synopsis
  • - The study investigates the effectiveness of tendon allograft reconstruction in maintaining pubic stability after iliac osteotomies in patients undergoing bladder exstrophy repair, a condition needing surgical intervention due to pelvic organ issues.
  • - Eleven patients, averaging 14 months old, underwent the procedure, followed by a period with external fixators, and their pelvic positions were monitored through scans and X-rays at various times, showing a successful urologic closure across the board.
  • - Results indicated that in 9 of the 11 patients, the distance between their pubic bones either stabilized or decreased over an average follow-up of 11 months, suggesting that the reconstruction method is promising for managing pubic symphysis issues post-surgery.
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Background: The exstrophy-epispadias complex (EEC) is a spectrum of rare congenital defects affecting the genitourinary system that includes epispadias, bladder exstrophy, and cloacal exstrophy. EEC patients may require several surgeries throughout their lifetime and experience a number of difficulties that may impact their quality of life (QoL), mental health, and psychosocial functioning.

Objective: The purpose of this scoping review is to systematically map existing literature that explores the mental health, psychosocial functioning, and QoL of children and adolescents with EEC.

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Introduction And Importance: Cloacal exstrophy (CE) is defined as a complex anomaly that affects the urogenital and intestinal tracts. It is the most serious form of anomaly that is described within the so-called exstrophy-epispadias complex. These malformations usually present a challenge in the management of particular conditions, as most of these forms require multiple surgeries, resulting in the use of multidisciplinary approaches, including reconstructive urologists, pediatric surgeons, orthopedic surgeons, endocrinologists, pediatricians, psychologists and nutritionists.

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