Purpose: We sought to determine the incidence of urolithiasis in patients with the exstrophy-epispadias complex, associated risk factors and guidelines for the proper clinical management of this problem.
Materials And Methods: We retrospectively reviewed the chart and x-rays of 530 patients with the exstrophy-epispadias complex treated at our institution.
Results: Stones formed in 77 of the 530 patients (15%), including 16% of those with classic exstrophy, 25% of those with cloacal exstrophy and 3% of those with epispadias. White and male individuals were most commonly affected. Most calculi formed in the bladder, whether native or augmented by enterocystoplasty. Standard techniques were successful for stone removal. Most calculi consisted of calcium apatite, calcium oxalate monohydrate or magnesium ammonium phosphate, usually in combination with at least 1 other element. The risk of stone formation was associated with augmentation cystoplasty (p < 0.001) and a bladder neck procedure to increase outlet resistance (p < 0.001). Other risk factors included urinary tract infection, foreign bodies, vesicoureteral reflux and urinary stasis but not acidosis or immobilization. Stones recurred in 30 patients (39%), including equal numbers of those treated with open and closed techniques, and recurrence was associated with urinary tract infection or struvite composition (p < 0.05). Serum calcium abnormalities were not present in any patient, including those with metabolic acidosis or prolonged immobilization. Urinary chemistry studies were incomplete or unavailable.
Conclusions: These data suggest that urolithiasis in the exstrophy-epispadias complex is related to risk factors associated with surgical reconstruction of this condition. The role of metabolic abnormalities that may predispose to urolithiasis is unknown but under investigation. Standard treatment is effective but stone recurrence remains a significant problem. Urine chemistry data may provide information to help minimize stone development in this patient population.
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http://dx.doi.org/10.1097/00005392-199709000-00175 | DOI Listing |
Urol J
January 2025
Pediatric Urology and Regenerative Medicine Research Center, Gene, Cell and Tissue Research Institute, Children's Medical Center, Tehran University of Medical Sciences, Tehran, Iran.
Purpose: To evaluate the effectiveness of the Complete Primary Repair of Exstrophy (CPRE) technique for bladder exstrophy-epispadias complex (BEEC) reconstruction and its comparison with the Modern Staged Repair of Exstrophy (MSRE) technique.
Materials And Methods: A comprehensive literature review of CPRE and MSRE was conducted, focusing on factors such as continence rates, postoperative outcomes, and complications. Various studies on pelvic biometry, surgical approaches, and long-term evaluations of renal function and continence were analyzed.
Afr Urol
June 2024
Division of Urology, Department of Surgery, University of Utah School of Medicine, United States of America.
Bladder exstrophy-epispadias-cloacal exstrophy complex (BEEC) is a spectrum of congenital urologic anomalies that involve the bladder, urethra, genitalia, and pelvic musculoskeletal system, and can affect urinary continence, sexual health, and fertility. BEEC includes a wide spectrum of anatomical abnormalities with different levels of severity: epispadias represents the mildest phenotype, classic bladder exstrophy (CBE) is the most common defect, and cloacal exstrophy (CE) - often referred to as omphalocele, exstrophy, imperforate anus, and spinal defects (OEIS) complex - is the most severe form. BEEC disorders cause significant health problems and affect the health-related quality of life (QoL) of affected individuals.
View Article and Find Full Text PDFJ Pediatr Orthop
December 2024
Paediatric Urology, Royal Manchester Children's Hospital, Manchester University NHS Foundation Trust, Manchester, UK.
Cells
November 2024
Akron Nephrology Associates, Cleveland Clinic Akron General Medical Center, Akron, OH 44307, USA.
Congenital anomalies of the kidney and urinary tract (CAKUT) represent a broad range of diseases with differing mechanisms, clinical presentations, and prognoses. With an estimated prevalence of between 4 and 60 per 10,000 births, CAKUT represents a sizable number of patients for pediatric and adult nephrologists as therapies have progressed, allowing longer life spans. Many CAKUT disorders are associated with genetic mutations, and with advances in genomic sequencing, these genes are being identified at an increasing rate.
View Article and Find Full Text PDFJ Pediatr Urol
November 2024
Department of Urology, School of Medicine, University of California San Francisco, 400 Parnassus Ave, San Francisco, CA, 94143, USA; Department of Surgery, San Francisco Veterans Affairs Medical Center, 4150 Clement St, San Francisco, CA, 94121, USA.
Background: Exstrophy-epispadias complex (EEC) classically presents as bladder exstrophy (BE) and requires lifetime urologic care. As men and women with BE age, there is an often difficult period of transition to adulthood in terms of addressing urologic and general health challenges. BE can lead to many urinary and sexual health issues as these patients age, which is often made more complex given their past surgical history and anatomy.
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