• Cloacal exstrophy (EC) is a severe birth defect wherein much of the abdominal organs (the bladder and intestines) are exposed. (wikipedia.org)
  • Cloacal exstrophy is a rare birth defect, present in 1/200,000 pregnancies and 1/400,000 live births. (wikipedia.org)
  • Many individuals who are born with XY cloacal exstrophy are assigned female at birth due to the appearance of the external genitalia. (wikipedia.org)
  • The exstrophy-epispadias complex (EEC) comprises a spectrum of increasingly complex congenital anomalies, including epispadias, classic bladder exstrophy, and cloacal exstrophy. (medscape.com)
  • The timing and degree of premature rupture of this membrane result in the spectrum of conditions seen in EEC, with cloacal exstrophy the earliest defect and epispadias a later insult. (medscape.com)
  • Rupture of the cloacal membrane after complete separation of the genitourinary (GU) and gastrointestinal (GI) tracts results in classic bladder exstrophy. (medscape.com)
  • However, rupture prior to descent of the urorectal septum allows externalization of the lower urinary tract and the distal GI tract (cloacal exstrophy). (medscape.com)
  • Cloacal exstrophy must be distinguished from the condition of persistent cloaca or cloacal malformation . (medscape.com)
  • For patients with cloacal exstrophy, nutrition is important and is often started early. (medscape.com)
  • Cloacal exstrophy, however, is an early event and thus affects concomitant anomalies to the most severe degree, representing the most challenging end of the EEC spectrum. (medscape.com)
  • The exstrophy-epispadias complex is a severe and rare malformation syndrome of the bladder (exstrophy), the urethra (epispadias), and colon (cloacal exstrophy) with often lifelong consequences (Kiddoo et al. (urology-textbook.com)
  • The stage of development at the time of the rupture determines the extent of the malformation: bladder exstrophy, epispadias, and cloacal exstrophy. (urology-textbook.com)
  • Conquering cloacal exstrophy, nursing beyond the borders and All Children's Hospital's growing research enterprise in this issue. (hopkinsmedicine.org)
  • Bladder exstrophy-epispadias complex (BEEC) is a congenital anomaly of the genitourinary system with a spectrum of congenital malformations ranging from isolated epispadias, to classical bladder exstrophy, to cloacal exstrophy as the most severe-and rarest-presentation. (southsudanmedicaljournal.com)
  • Most (94%) older children and adults with cloacal exstrophy are managed by incontinent diversion or clean intermittent catheterization. (pediatricsnationwide.org)
  • The published literature about cloacal exstrophy can be misleading, and there is an impression that with surgical bladder reconstruction, normal bladder function and voiding can be restored. (pediatricsnationwide.org)
  • To evaluate real-world incontinence management in cloacal exstrophy, the Pediatric Urology Midwest Alliance (PUMA) group analyzed data from children and adults cared for at five institutions. (pediatricsnationwide.org)
  • Urinary and bowel management in cloacal exstrophy: A long-term multi-institutional cross-sectional study. (pediatricsnationwide.org)
  • This included 18 patients with classic bladder exstrophy, 5 with epispadias, and 1 with a cloacal exstrophy variant. (johnshopkins.edu)
  • The central claim in this question is addressed based on "Discordant Sexual Identity in Some Genetic Males with Cloacal Exstrophy Assigned to Female Sex at Birth" (2004) which isn't behind a paywall. (stackexchange.com)
  • Omphalocele, exstrophy of cloaca, imperforate anus, and spinal defects complex is a rare malformation complex that includes omphalocele, cloacal exstrophy, imperforate anus and spinal defects with the incidence of 1 in 200,000 to 400,000 pregnancies and is even rarer in twin gestation. (bvsalud.org)
  • We present a 4-day first twin child with underdeveloped ambiguous genitalia delivered via emergency lower section cesarean section at 32+3 weeks of gestation with giant liver containing omphalocele, cloacal exstrophy, imperforate anus and meningocele with severe pulmonary artery hypertension and non-visualization of right kidney and ureter, absence of uterus, fallopian tubes and right ovary. (bvsalud.org)
  • In addition, you may find more help and information at aBEc (Association for the Bladder Exstrophy Community) , including videos as well as links to online support groups. (180medical.com)
  • Bladder exstrophy-epispadias complex (BEEC) is a rare congenital malformation of the genitourinary system usually associated with other malformations. (southsudanmedicaljournal.com)
  • Associated with OEIS syndrome- omphalocele, exstrophy, imperforated anus and spinal abnormalities. (radiopaedia.org)
  • Apart from Husmann and Vandersteen [in: Gearhart JP, Matthews R (eds) The Epispadias-Exstrophy Complex. (nih.gov)
  • This larger group of congenital disabilities is known as the Bladder Exstrophy-Epispadias Complex. (180medical.com)
  • During one-stage delayed Kelly's Radical Soft Tissue Mobilization (RSTM) for Bladder Exstrophy Epispadias Complex (BEEC), children underwent a pelvic floor muscles detachment. (espu.org)
  • There is a high variance in the characteristics of the below-mentioned components of the exstrophy-epispadias complex. (urology-textbook.com)
  • Transfer the child to a center with experience in the surgical treatment of the exstrophy-epispadias complex. (urology-textbook.com)
  • Initial management of complex urological disorders: bladder exstrophy. (urology-textbook.com)
  • This genitourinary anomaly was consistent with the bladder exstrophy-epispadias complex (BEEC). (southsudanmedicaljournal.com)
  • Methods: The authors retrospectively reviewed an institutional database of patients with exstrophy-epispadias complex who underwent BNR-CS between 2000 and 2015. (johnshopkins.edu)
  • Results: A total of 24 patients with exstrophy-epispadias complex (15 male and 9 female) underwent BNR-CS at a median age of 8.9 years (range 5.4-17.4). (johnshopkins.edu)
  • In the most common presentation, classic bladder exstrophy, the lower urinary tract, genitalia, and the musculosketal system are usually affected. (medscape.com)
  • Since 1999, consecutive patients with classic bladder exstrophy were treated by new-born primary bladder closure and Kelly soft tissue reconstruction in their second year. (espu.org)
  • Methods: This prospective observational study was conducted at BSMMU on ten postoperative cases of Classic bladder Exstrophy who underwent one-stage repair from September 2018 to October 2021. (abcresearchalert.com)
  • Materials and Methods: Pelvic magnetic resonance imaging was performed on 10 men who underwent exstrophy reconstruction in childhood, and 10 age and race matched controls. (johnshopkins.edu)
  • Standing the test of time: long-term outcome of reconstruction of the exstrophy bladder. (urology-textbook.com)
  • Purpose: We attempted to determine whether the penis in adulthood after exstrophy reconstruction is short because of a congenital defect in the size of the corpora cavernosa. (johnshopkins.edu)
  • Results: The corpora cavernosa in men after exstrophy reconstruction were shorter than normal. (johnshopkins.edu)
  • Conclusions: After exstrophy reconstruction the penis is short in adulthood, at least partially due to a congenital deficiency of corporeal tissue. (johnshopkins.edu)
  • However, because the corpora cavernosa are short, after exstrophy reconstruction the penis will always be shorter than normal in adulthood. (johnshopkins.edu)
  • In the course of time, the repair of bladder exstrophy has evolved from a staged repair to a one-stage reconstruction with encouraging results in different countries. (abcresearchalert.com)
  • Conclusion: From the results found in this study, one-stage repair of bladder exstrophy achieved success in bladder, and abdominal wall reconstruction, as evidenced by the rate of wound dehiscence is comparably similar. (abcresearchalert.com)
  • Giron AM, Mello MF, Carvalho PA, Moscardi PR, Lopes RI, Srougi M. One - staged reconstruction of bladder exstrophy in male patients: Long - term follow-up outcomes. (abcresearchalert.com)
  • Bladder exstrophy is a deficient closure of the lower anterior abdominal wall: the bladder is open anteriorly, and the trigonum of the bladder is visible. (urology-textbook.com)
  • This page from Great Ormond Street Hospital (GOSH) explains the Kelly procedure used to construct and strengthen the sphincter at the bladder neck following the initial closure of bladder exstrophy and what to expect when your child is admitted to GOSH for the operation. (gosh.nhs.uk)
  • After the initial closure of the bladder exstrophy, there is no sphincter at the junction of the bladder and urethra. (gosh.nhs.uk)
  • Bladder exstrophy is a rare congenital anomaly of the genitourinary tract producing ventral abdominal wall defect. (abcresearchalert.com)
  • Rachel Althizer is traveling with A-BE-C for the 2023 International Exstrophy Workshop in Ahmedabad, India, as a social worker to assist children and families with exstrophy as they prepare for surgery. (bladderexstrophy.com)
  • Bladder exstrophy is an anatomical anomaly during fetal development where the bladder (and surrounding parts) develop outside of the body. (180medical.com)
  • MTHFR 677 TT genotype in a mother and her child with Down syndrome, atrioventricular canal and exstrophy of the bladder: implications of a mutual genetic risk factor? (nih.gov)
  • Kluwer, New York, pp 199-206, 1999], we report only the second case of Down syndrome (DS) associated with exstrophy of the bladder (EB). (nih.gov)
  • Bladder exstrophy (BE) is a rare, lower ventral midline defect with the bladder and part of the urethra exposed. (radygenomics.org)
  • Bladder exstrophy is a rare birth defect. (urologyhealth.org)
  • Children born with bladder exstrophy will have to undergo reconstructive surgery to repair and close the bladder. (180medical.com)
  • For neonates with exstrophy and epispadias, general supportive care appropriate for the overall condition and associated anomalies should be instituted. (medscape.com)
  • Bladder Exstrophy" is a descriptor in the National Library of Medicine's controlled vocabulary thesaurus, MeSH (Medical Subject Headings) . (jefferson.edu)
  • this study was conducted on one-stage repair of bladder exstrophy to observe early possible outcomes. (abcresearchalert.com)
  • If you're a parent with a child born with bladder exstrophy, you have more options for helpful resources as you navigate this new and unexpected journey. (180medical.com)
  • Catheritization or "cathing" is a fact of life for many bladder exstrophy patients. (bladderexstrophy.com)
  • Moreover, one-stage of repair of bladder exstrophy appears to be better enough for broader practice. (abcresearchalert.com)
  • While a child born with bladder exstrophy may face a difficult road ahead at times, as a parent or caregiver, you have options to help make living with this condition easier. (180medical.com)
  • In this article, you'll discover what bladder exstrophy is and its potential causes and treatment options. (180medical.com)
  • Do you have questions about what type of catheter to use for your child with bladder exstrophy? (180medical.com)