Congenital Exstrophy of the Urinary Bladder: Late Results of Treatment by the Coffey-Mayo Method of Uretero-Intestinal Anastomosis

Harvard, B. Marvin; Thompson, Gershom J.

doi:10.1016/s0022-5347(17)68478-7

Cited by 50 publications

(4 citation statements)

References 12 publications

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“…In this study, CBE showed a statistically significant downward linear trend by year, and these authors also identified summer conception and male sex as possible risk factors. For CBE, Nelson et al (2005) found an almost even male‐to‐female ratio and a similar ratio (1.32) was reported by Martinez‐Frias et al (2001), whereas it is 2.4:1 summarized from multiple surveys (Harvard and Thompson, 1951; Higgins, 1962; Bennett, 1973; Jeffs et al, 1982; Gambhir et al, 2008a). However, two series reported a 5:1 to 6:1 male‐to‐female ratio (Ives et al, 1980; Anonymous, 1987).…”

Section: Discussionsupporting

confidence: 58%

Bladder exstrophy‐epispadias complex

Ludwig

Ching

Reutter

et al. 2009

Birth Defects Research

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The bladder exstrophy-epispadias complex (BEEC) represents an anterior midline defect with variable expression comprising a spectrum of anomalies involving the abdominal wall, pelvis, urinary tract, genitalia, and occasionally the spine and anus. The vast majority of BEEC cases are classified as non-syndromic and the etiology of this malformation is still unknown. This review presents the current state of knowledge on this multifactorial disorder, including historical retrospect, phenotypic and anatomical characterization, epidemiology, proposed developmental mechanisms, existing animal models, and implicated genetic and environmental components. These published lines of evidence argue strongly that BEEC occurs as a result of strong genetic predisposition that is yet to be deciphered.

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Section: Discussionsupporting

confidence: 58%

Bladder exstrophy‐epispadias complex

Ludwig

Ching

Reutter

et al. 2009

Birth Defects Research

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“…The male‐to‐female ratio of EB derived from multiple series is 2.3:1 [Harvard and Thompson, 1951; Higgins, 1962; Bennett, 1973; Jeffs et al, 1982]. However, two series have reported a 5:1 to 6:1 male‐to‐female ratio [Ives et al, 1980; International Clearinghouse for Birth Defects Monitoring Systems, 1987].…”

Section: Discussionmentioning

confidence: 99%

Exstrophy of the cloaca and exstrophy of the bladder: Two different expressions of a primary developmental field defect

et al. 2001

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Exstrophy of the bladder (EB) and exstrophy of the cloaca (EC) are generally recognizable as distinct clinical entities. In patients with EB, the posterior bladder wall is exposed through a midline defect of the abdomen. The umbilicus is inferiorly displaced and located close to the superior margin of the exstrophic bladder. Genital abnormalities are common in boys and girls who may present epispadias and a small, split phallus or a split clitoris, a bifid uterus, and a duplicate or exstrophic vagina. In contrast to classic EB, EC is commonly associated with omphalocele, spinal defects, and incompletely formed external genitalia and is always associated with imperforate anus. Some authors state that EC and EB constitute two distinct disorders, but others consider them part of a "continuum," representing different levels of severity within the same spectrum. The use of the acronym OEIS to refer to the combination of omphalocele, exstrophy, imperforate anus, and spinal defects, in our opinion, has not helped to clarify the clinical definition, pathogenesis, or cause of this multiple congenital anomaly (MCA) pattern, mostly because the term makes no distinction between EC or EB. Here we present the epidemiological analysis of a group of characteristics in infants with EC and infants with EB to determine if they constitute two different entities. We also analyze if the different combinations of omphalocele, imperforate anus, and spinal defects are more frequent in infants with EC than in infants with MCA patterns other than EC and EB. The prevalence in our data for EC was 1:200,233 live births and 1:35,597 for EB. The clinical analysis indicated that the study defects (omphalocele, spine defects, spina bifida, and imperforate anus) tend to occur together in the same child with a higher frequency if the child has the EC defect than in infants with MCA patterns that did not include EC or EB. Our findings of low birth weight, twinning, single umbilical artery, and preferentially associated malformations suggest that EC is the result of damage occurring very early in development and that EC and EB are two different expressions of a primary polytopic developmental field defect.

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“…The results of this study demonstrated that CEB showed a statistically significant linear downward trend by year and summer conception, and male sex was also identified as a possible risk factor. Though Nelson et al [ 11 ] found an almost equal male-to-female ratio for CEB, multiple surveys summarized a ratio of 2.4:1 [ 4 , 7 , 13 - 15 ]. In rare cases only, male-to-female ratios as high as 5:1 to 6:1 have been reported [ 3 , 5 ].…”

Section: Epidemiologymentioning

confidence: 99%

The Exstrophy-epispadias complex

Ebert

Reutter

Ludwig

et al. 2009

Orphanet J Rare Dis

204

177

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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 whole spectrum is reported at 1/10,000, ranging from 1/30,000 for CEB to 1/200,000 for EC, with an overall greater proportion of affected males. EEC is characterized by a visible defect of the lower abdominal wall, either with an evaginated bladder plate (CEB), or with an open urethral plate in males or a cleft in females (E). In CE, two exstrophied hemibladders, as well as omphalocele, an imperforate anus and spinal defects, can be seen after birth. EEC results from mechanical disruption or enlargement of the cloacal membrane; the timing of the rupture determines the severity of the malformation. The underlying cause remains unknown: both genetic and environmental factors are likely to play a role in the etiology of EEC. Diagnosis at birth is made on the basis of the clinical presentation but EEC may be detected prenatally by ultrasound from repeated non-visualization of a normally filled fetal bladder. Counseling should be provided to parents but, due to a favorable outcome, termination of the pregnancy is no longer recommended. Management is primarily surgical, with the main aims of obtaining secure abdominal wall closure, achieving urinary continence with preservation of renal function, and, finally, adequate cosmetic and functional genital reconstruction. Several methods for bladder reconstruction with creation of an outlet resistance during the newborn period are favored worldwide. Removal of the bladder template with complete urinary diversion to a rectal reservoir can be an alternative. After reconstructive surgery of the bladder, continence rates of about 80% are expected during childhood. Additional surgery might be needed to optimize bladder storage and emptying function. In cases of final reconstruction failure, urinary diversion should be undertaken. In puberty, genital and reproductive function are important issues. Psychosocial and psychosexual outcome depend on long-term multidisciplinary care to facilitate an adequate quality of life.

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Congenital Exstrophy of the Urinary Bladder: Late Results of Treatment by the Coffey-Mayo Method of Uretero-Intestinal Anastomosis

Cited by 50 publications

References 12 publications

Bladder exstrophy‐epispadias complex

Bladder exstrophy‐epispadias complex

Exstrophy of the cloaca and exstrophy of the bladder: Two different expressions of a primary developmental field defect

The Exstrophy-epispadias complex

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