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Background: Exstrophy-epispadias complex (EEC) is a complex malformation of the lower abdominal wall, bladder, and pelvic floor, which necessitates multiple successive reconstruction procedures. Surgical and infectious complications are frequent. Our aim was to evaluate kidney function in these patients. Methods: This cross-sectional study included patients with EEC, followed since birth in a pediatric urology clinic, who underwent nephrological evaluation (blood pressure (BP) measurement and blood and urine chemistries) and imaging studies (urinary tract ultrasound and DMSA kidney scan) during 2017–2020. Results: Forty-three patients (29 males), median age 9 years (interquartile range 6–19), were included. Eleven (26%) used clean intermittent catheterization (CIC) for bladder drainage. At least one sign of kidney injury was identified in 32 (74%) patients; elevated BP, decreased kidney function (estimated glomerular filtration rate (eGFR) < 90 ml/min/1.73 m2), and proteinuria/albuminuria were detected in 29%, 12%, and 36% of patients, respectively. Urinary tract dilatation (UTD) was found in 13 (37%) ultrasound examinations. Parenchymal kidney defects were suspected in 46% and 61% of ultrasound and DMSA scintigraphy, respectively. UTD was significantly associated with DMSA-proven kidney defects (p = 0.043) and with elevated BP, 39% vs. 20% in those without UTD. Decreased eGFR and elevated BP were less frequent among patients on CIC than among patients who voided spontaneously: 10% vs. 14% and 18% vs. 36%, respectively. Recurrent UTIs/bacteriuria and nephro/cystolithiasis were reported by 44% and 29% patients, respectively. Conclusion: The high rate of signs of kidney injury in pediatric patients with EEC dictates early-onset long-term kidney function monitoring by joint pediatric urological and nephrological teams. A higher resolution version of the Graphical abstract is available as Supplementary information [ABSTRACT FROM AUTHOR]

Purpose of Review: Adult males with exstrophy-epispadias complex (EEC) are commonly dissatisfied with their genitalia and desire greater sexual normality. These patients have typically had several previous genital/pelvic procedures and there are specific considerations that must be taken into account if operative intervention is being considered. Genital reconstruction in the form of phalloplasty presents an option for improving sexual function. Recent Findings: The majority of studies have focused on the radial free forearm flap (RFFF) phalloplasty with subsequent insertion of penile prosthesis (PP). Long-term cosmetic and functional satisfaction is high. Early urethral complications are common and PP-related complications are higher than in other patient populations. Summary: Genital reconstruction is a safe procedure for EEC males with good long-term functional and cosmetic satisfaction. RFFF phalloplasty represents the most complete surgical option and should be considered in carefully selected patients. [ABSTRACT FROM AUTHOR]

Bladder exstrophy is a complex congenital birth defect ranging from complete epispadias to complete bladder exstrophy. Though not included in the exstrophy-epispadias complex (EEC) disease spectrum, cloacal exstrophy is a more severe form which includes bowel segments in the defect and has many of the same defects as the EEC. Initial care of these complex patients focuses on the primary repair of the defect, a repair which is the most important of the many operations these children will have in their lifetime. A failure of primary closure is associated with worse outcomes across the board. The most essential goal in treatment of the exstrophy patient is renal protection. Once that can be assured, social continence then becomes the most important goal. Given the complex nature of this disorder, these children should be managed at speciality centers with orthopedic, urologic, and nursing experience with these patients. [ABSTRACT FROM AUTHOR]

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Bladder exstrophy is a rare malformation characterized by an infra-umbilical abdominal wall defect, incomplete closure of the bladder with mucosa continuous with the abdominal wall, epispadias, and alterations in the pelvic bones and muscles. It is part of the exstrophy-epispadias complex, with cloacal exstrophy on the severe and epispadias on the mild ends of the spectrum. Bladder exstrophy is the most common of these entities and is more common in boys. The goal of this paper is to describe common methods of repair and to provide an imaging review of the postoperative appearances. [ABSTRACT FROM AUTHOR]

The exstrophy-epispadias complex is a rare congenital malformation of the genitourinary system, abdominal wall musculature, and pelvic bones. Historically, surgical outcomes in patients with classic bladder exstrophy, the most common presentation of the exstrophy-epispadias complex, were poor. However, modern techniques have increased the success of achieving urinary continence, satisfactory cosmesis, and improved quality of life. Still, recent studies recognize complications that may occur during management of these patients. This review provides readers with an overview of the exstrophy-epispadias complex, the modern management of bladder exstrophy, and potential surgical complications. [ABSTRACT FROM AUTHOR]

We report a new method of repair of exstrophy-epispadias complex (EEC) at a second stage by using flaps from the skin between the penis and scrotum (the clitoris and labia majora in females) used in seven patients. The non-pigmented skin between the penis and scrotum in males or a band of skin between the clitoris and labia majora in female is mobilized in two flaps, which are rotated superiorly. Five patients were treated primarily by this method and two patients at the time of secondary reconstruction. There were six boys and one girl. Two patients were post-pubertal, one male and one female. Follow-up (6–12 months) revealed good cosmetic and functional results. If EEC is repaired in a single stage, the entire pelvic diaphragm is visualized from inside and the sphincters can be reconstructed around the bladder neck. This procedure gives better exposure of attachments of the corporal bodies to the pubic rami, allowing easier mobilization. The bulbospongiosus muscle can be reconstructed. A normal penoscrotal relation is achieved in male patients. The root of the scrotum, which is splayed out and wide, narrows. Rotation of the flaps superiorly normalizes the symphyseal area. In female patients the appearance of the mons pubis is satisfactory. In post-pubertal children the pubic hair distribution appears normal. [ABSTRACT FROM AUTHOR]

Purpose of Review: The exstrophy-epispadias complex (EEC) represents a group of congenitally acquired malformations involving the musculoskeletal, gastrointestinal, and genitourinary systems. Classic bladder exstrophy (CBE) is the most common and best studied entity within the EEC. In this review, imaging features of CBE anatomy will be presented with surgical correlation. Recent Findings: Magnetic resonance imaging (MRI) has emerged as a useful modality for pre- and postnatal assessment of the abdominal wall, pelvic floor, and gastrointestinal and genitourinary systems of children with CBE. The authors' experience supports use of preoperative MRI, in conjunction with navigational software, as a method for identifying complex CBE anatomy. Summary: Imaging facilitates surgical approach and improves visualization of complex anatomy, potentially helping to avoid complications. Continued investigation of imaging guidance in CBE repair is needed as surgical techniques improve. [ABSTRACT FROM AUTHOR]