Bladder exstrophy

Thomas E. Novak, John Phillip Gearhart

Research output: Chapter in Book/Report/Conference proceedingChapter

Abstract

The epispadias/exstrophy complex encompasses a spectrum of rare midline defects in the pelvis and genitalia. Classic bladder exstrophy is a severe congenital malformation affecting approximately 1 in 40000 live births. Although the exact etiology remains unknown, current evidence seems to implicate poor mesenchymal ingrowth with subsequent deleterious effects on the timing and position of rupture of the cloacal membrane. The birth of a child with bladder exstrophy marks the start of a lifelong journey. The severity of the defect promises an extensive surgical undertaking that starts most frequently during the first days of life and often extends through adolescence. Components of surgical correction include: Closure of the bladder, posterior urethra, pelvis and abdominal wall at birth, genital reconstruction, and some form of continence procedure. The approach pioneered at the senior author's institution addresses these components in a modern, staged fashion. Alternatively, there are also proponents of complete primary closure, which attempts to address all components in a single operation. However, recent data from the American Academy of Pediatrics reveals inferior continence rates when this technique is used. Longer follow-up is needed to ascertain the applicability of this repair, as the associated complications are not insignificant. With respect to outcomes, the goals of reconstruction in the exstrophy patient are urinary continence and satisfactory genital cosmesis and function. Although most children prefer to achieve continence with voiding per urethra, some will accomplish this same outcome with intermittent catheterization.

Original languageEnglish (US)
Title of host publicationPediatric Surgery and Urology: Long-Term Outcomes, Second Edition
PublisherCambridge University Press
Pages621-630
Number of pages10
ISBN (Print)9780511545757, 0521839025, 9780521839020
DOIs
StatePublished - Jan 1 2006

Fingerprint

Bladder Exstrophy
Urethra
Pelvis
Epispadias
Parturition
Genitalia
Live Birth
Abdominal Wall
Catheterization
Rupture
Urinary Bladder
Pediatrics
Membranes

ASJC Scopus subject areas

  • Medicine(all)

Cite this

Novak, T. E., & Gearhart, J. P. (2006). Bladder exstrophy. In Pediatric Surgery and Urology: Long-Term Outcomes, Second Edition (pp. 621-630). Cambridge University Press. https://doi.org/10.1017/CBO9780511545757.049

Bladder exstrophy. / Novak, Thomas E.; Gearhart, John Phillip.

Pediatric Surgery and Urology: Long-Term Outcomes, Second Edition. Cambridge University Press, 2006. p. 621-630.

Research output: Chapter in Book/Report/Conference proceedingChapter

Novak, TE & Gearhart, JP 2006, Bladder exstrophy. in Pediatric Surgery and Urology: Long-Term Outcomes, Second Edition. Cambridge University Press, pp. 621-630. https://doi.org/10.1017/CBO9780511545757.049
Novak TE, Gearhart JP. Bladder exstrophy. In Pediatric Surgery and Urology: Long-Term Outcomes, Second Edition. Cambridge University Press. 2006. p. 621-630 https://doi.org/10.1017/CBO9780511545757.049
Novak, Thomas E. ; Gearhart, John Phillip. / Bladder exstrophy. Pediatric Surgery and Urology: Long-Term Outcomes, Second Edition. Cambridge University Press, 2006. pp. 621-630
@inbook{e81dff99722544239cac60ccdfed4202,
title = "Bladder exstrophy",
abstract = "The epispadias/exstrophy complex encompasses a spectrum of rare midline defects in the pelvis and genitalia. Classic bladder exstrophy is a severe congenital malformation affecting approximately 1 in 40000 live births. Although the exact etiology remains unknown, current evidence seems to implicate poor mesenchymal ingrowth with subsequent deleterious effects on the timing and position of rupture of the cloacal membrane. The birth of a child with bladder exstrophy marks the start of a lifelong journey. The severity of the defect promises an extensive surgical undertaking that starts most frequently during the first days of life and often extends through adolescence. Components of surgical correction include: Closure of the bladder, posterior urethra, pelvis and abdominal wall at birth, genital reconstruction, and some form of continence procedure. The approach pioneered at the senior author's institution addresses these components in a modern, staged fashion. Alternatively, there are also proponents of complete primary closure, which attempts to address all components in a single operation. However, recent data from the American Academy of Pediatrics reveals inferior continence rates when this technique is used. Longer follow-up is needed to ascertain the applicability of this repair, as the associated complications are not insignificant. With respect to outcomes, the goals of reconstruction in the exstrophy patient are urinary continence and satisfactory genital cosmesis and function. Although most children prefer to achieve continence with voiding per urethra, some will accomplish this same outcome with intermittent catheterization.",
author = "Novak, {Thomas E.} and Gearhart, {John Phillip}",
year = "2006",
month = "1",
day = "1",
doi = "10.1017/CBO9780511545757.049",
language = "English (US)",
isbn = "9780511545757",
pages = "621--630",
booktitle = "Pediatric Surgery and Urology: Long-Term Outcomes, Second Edition",
publisher = "Cambridge University Press",

}

TY - CHAP

T1 - Bladder exstrophy

AU - Novak, Thomas E.

AU - Gearhart, John Phillip

PY - 2006/1/1

Y1 - 2006/1/1

N2 - The epispadias/exstrophy complex encompasses a spectrum of rare midline defects in the pelvis and genitalia. Classic bladder exstrophy is a severe congenital malformation affecting approximately 1 in 40000 live births. Although the exact etiology remains unknown, current evidence seems to implicate poor mesenchymal ingrowth with subsequent deleterious effects on the timing and position of rupture of the cloacal membrane. The birth of a child with bladder exstrophy marks the start of a lifelong journey. The severity of the defect promises an extensive surgical undertaking that starts most frequently during the first days of life and often extends through adolescence. Components of surgical correction include: Closure of the bladder, posterior urethra, pelvis and abdominal wall at birth, genital reconstruction, and some form of continence procedure. The approach pioneered at the senior author's institution addresses these components in a modern, staged fashion. Alternatively, there are also proponents of complete primary closure, which attempts to address all components in a single operation. However, recent data from the American Academy of Pediatrics reveals inferior continence rates when this technique is used. Longer follow-up is needed to ascertain the applicability of this repair, as the associated complications are not insignificant. With respect to outcomes, the goals of reconstruction in the exstrophy patient are urinary continence and satisfactory genital cosmesis and function. Although most children prefer to achieve continence with voiding per urethra, some will accomplish this same outcome with intermittent catheterization.

AB - The epispadias/exstrophy complex encompasses a spectrum of rare midline defects in the pelvis and genitalia. Classic bladder exstrophy is a severe congenital malformation affecting approximately 1 in 40000 live births. Although the exact etiology remains unknown, current evidence seems to implicate poor mesenchymal ingrowth with subsequent deleterious effects on the timing and position of rupture of the cloacal membrane. The birth of a child with bladder exstrophy marks the start of a lifelong journey. The severity of the defect promises an extensive surgical undertaking that starts most frequently during the first days of life and often extends through adolescence. Components of surgical correction include: Closure of the bladder, posterior urethra, pelvis and abdominal wall at birth, genital reconstruction, and some form of continence procedure. The approach pioneered at the senior author's institution addresses these components in a modern, staged fashion. Alternatively, there are also proponents of complete primary closure, which attempts to address all components in a single operation. However, recent data from the American Academy of Pediatrics reveals inferior continence rates when this technique is used. Longer follow-up is needed to ascertain the applicability of this repair, as the associated complications are not insignificant. With respect to outcomes, the goals of reconstruction in the exstrophy patient are urinary continence and satisfactory genital cosmesis and function. Although most children prefer to achieve continence with voiding per urethra, some will accomplish this same outcome with intermittent catheterization.

UR - http://www.scopus.com/inward/record.url?scp=84927054411&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=84927054411&partnerID=8YFLogxK

U2 - 10.1017/CBO9780511545757.049

DO - 10.1017/CBO9780511545757.049

M3 - Chapter

AN - SCOPUS:84927054411

SN - 9780511545757

SN - 0521839025

SN - 9780521839020

SP - 621

EP - 630

BT - Pediatric Surgery and Urology: Long-Term Outcomes, Second Edition

PB - Cambridge University Press

ER -