Exstrophy-epispadias complex (EEC) represents a spectrum of genitourinary malformations ranging in severity from epispadias (E) to classical bladder exstrophy (CEB) and exstrophy of the cloaca (EC). Depending on severity, EEC may involve the urinary system, musculoskeletal system, pelvis, pelvic floor, abdominal wall, genitalia, and sometimes the spine and anus. Prevalence at birth for the whol...

We report a case of a woman affected by covered exstrophy, uterus didelphys and external genital malformation presenting with advanced bladder cancer. After neoadjuvant therapy and anterior pelvic exenteration, the abdominal wall was reconstructed with a pedicled myocutaneous muscle-sparing vastus lateralis flap.

We report the case of a neonate who underwent bladder exstrophy repair on the second day of life. Bilateral ureteral stents were placed intra-operatively. Postoperatively azotemia developed secondary to kinking of the proximal ureter bilaterally. Issues in diagnosis and management are discussed.

Split notochord syndrome with dorsal enteric fistula is an extremely rare congenital anomaly that may be associated with meningomyelocele or meningocele, and genitourinary anomalies. This case presented with an additional finding of bladder exstrophy, raising the possibility of a relationship between this syndrome and the OEIS complex.

Omphalocele-exstrophy of the bladder-imperforate anus-spinal defect (OEIS) complex is a rare constellation of clinical abnormalities with wide phenotypic presentation. We describe a case of a preterm neonate with OEIS complex with acute renal failure, and the challenges in diagnosis and management of this patient as renal failure can be a multifactorial process when encountered with this rare c...

Genetic males who have cloacal exstrophy, a rare, severe pelvic field defect that leads to aphallia, traditionally have been socially, legally, and surgically sex-assigned female at birth and reared female, despite a male karyotype and a typical male prenatal hormonal milieu. Psychosexual development in such children previously has been unknown. Studies of 29 children revealed that despite the ...

Urea kinetics were measured in a child with congenital absence of the colon on days 15, 19, and 23 of age. Urea salvage was 5% of urea production in the first study, increasing to 79% by the third. This provides evidence that the colonic microflora play a more active part in urea salvage than the mucosa and that the establishment of an active lower ileal microflora takes over some of the metabo...

Conditions impairing bladder function in children and adults, such as myelomeningocele, posterior urethral valves, bladder exstrophy or spinal cord injury, often need urinary diversion or augmentation cystoplasty as when untreated they may cause severe bladder dysfunction and kidney failure. Currently, the gold standard therapy of end-stage bladder disease refractory to conservative management ...