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| Bladder exstrophy, epispadias, and cloacal exstrophy are a spectrum of anomalies involving the urinary tract, genital tract, intestinal tract, and musculoskeletal system ... a congenital anomaly (birth defect) where the bladder and related structures ... are "turned inside-out" ... exposed outside the abdominal wall. Epispadias is a mild form of bladder exstrophy ... urethra ... opens inappropriately to the external environment (usually the top of the penis) ... epispadias and bladder exstrophy occur twice as frequently in males ... Surgical repair is usually successful at achieving continence ... child may discover his bladder is too small ... cloacal extrophy is the most devastating of these three conditions ... protrusion through the abdominal walls of the abdominal organs. The bladder and rectum will communicate ... the bladder will be split in half around the rectum ... anus may not be formed or perforated and will communicate with the bladder. Spinal defects are also associated with this condition ... The cause of these three birth defects is unknown ... not been shown to have a genetic basis ... males suffering from cloacal extrophy undergo gender reassignment in many cases ... due to the inability to reconstruct male genitalia ... |
Bladder Exstrophy and Epispadias
Pediatric Urology Associates
 "... Comprehensive ... information for parents with affected children ... "
What Is Bladder Exstrophy?
Bladder Exstrophy is a congenital birth defect that is the malformation of the bladder and urethra, in which the bladder is turned "inside out" ... It is unknown what causes Bladder Exstrophy ... Bladder Exstrophy is noted in 1 of 30,000 to 50,000 live births! ... is more likely to occur in males than females by approximately 2 to 1 ... risk of having a 2nd child with bladder exstrophy is about 1 in 100 and 1 in 70 if ... Bladder Exstrophy requires surgical repair usually involving staged "reconstruction" ... 1st Stage - Closure of bladder and abdomen ( 24 - 48 hours of life ) 2nd Stage - Epispadius repair ( 2 - 3 yrs old ) 3rd Stage - Achieve urinary continence ( 4 - 5 yrs old ) ... In the majority of cases the children are healthy with normal intelligence ... The female patient usually presents with a normal uterus, fallopian tubes, and ovaries. The vagina may be slightly higher in placement and somewhat narrowed. The clitoris is separated into two parts, the labia and mons pubis (hair bearing skin) are spread apart ... will be able to have normal sexual intercourse. Almost all females are able to have children ...
Exstrophy of the Cloaca Sequence
Roy Erb, MD, et al., TheFetus.net, 1992
"... Comprehensive review of bladder exstrophy and epispadias for ... health care professionals ... Good pictures and radiographic images."
Synonyms: Exstrophia splanchnica, exstrophy - epispadias complex ... Prevalence: 0.25 - 0.5:10,000 1,2 Etiology: Unknown, ... Pathogenesis: Failure of two concomitant mesodermal migrations: first, the urorectal spetum fails to develop and divide the urogenital sinus from the rectum; second, the mesodermal proliferation forming the infraumbilical abdominal wall and genital tubercle fails to develop. The failure of these two events to occur results in exstrophy of both bladder and intestine. Associated anomalies: Commonly associated with other anomalies including cardiovascular, central nervous system, omphalocele (70 - 90 %), vertebral anomalies (46 %), upper urinary tract (42 %), malrotation (30 %), lower extremity anomalies (30 %), double appendix (30 %), absent appendix (21%), short small bowel (19%), small bowel arresia (5%), abdominal musculature deficiency (1%) 3 ... two hemibladders lateral to the hindgut. No anus or anal canal is formed. In males, ... two halves of the phallus are separated. In females, a bicormuate uterus and duplicated vagina ... The vagina enters the bladder ... Prior to 1950, cloacal exstrophy was considered uniformly fatal ... Risk of recurrence: Unknown for subsequent pregnancy ... sonogram at approximately 21 weeks EGA ... abnormal external genitalias and a soft tissue mass on the infraumbilical abdominal wall were noted ... The newborn underwent immediate operation for an ostomy ... Longitudinal scan of the lower abdomen. The cord insertion is visualized above the protruding mass that contains bladder and bowel loops ... The bladder clearly identified between both umbilical arteries, before their entrance into the cord ... The 27 - week fetus after delivery. Note the protruding mass from the anterior abdominal wall ... had an imperforated anus but normal - appearing bowel. The uterus and vagina were split ... very small bladder was noticed. A small occult spina bifida was detected ... Cloacal exstrophy is commonly associated with other anomalies ... OEIS syndrome
- Omphalocele
- Exstrophy of the cloaca
- Imperforated anus
- Spinal abnormalities
Vertebral anomalies (46%)
- Sacralization of L5
- Congenital scoliosis
- Sacral agenesis
- Interpedicular widening
Upper urinary tract (42%)
- Pelvic kidney
- Horseshoe kidney
- Hypoplastic kidney
- Solitary kidney
Gastrointestinal
- Malrotation (30%)
- Double appendix (30%)
- Absent appendix (21%)
- Short small bowel (19%)
- Small bowel atresai (5%)
... 70 - 90 % of patients with cloacal exstrophy have an omphalocele ...
Reiner & Gearhart's NEJM Study on Cloacal Exstrophy
Vernon Rosario, M.D., Ph.D., Intersex Society of North America, March 29, 2004
"... good description of ... current ideas concerning gender reassignment in those affected by cloacal exstrophy ... a necessary issue to address with parents ... May be too complex for general readers."
Association for Bladder Exstrophy Community
"Good site ... relevant to patients ...with bladder exstrophy, espadias, and cloacal exstrophy."
John Hopkins Exstrophy Center
Brady Urological Institute
... an informational resource from the Division of Pediatric Urology to the patients with extrophy, ...
Recién nacido con extrofia vesical y posible exposición intrauterina a finasterida
Annals de Pediatria, Lunes 1 Noviembre 2004. Volumen 61 - Número 05 p. 451 - 453
[Spanish]
Cartas al editor
... La extrofia vesical (EV) es un defecto grave del cierre de la pared abdominal fetal de patogenia desconocida. En España se ha registrado una prevalencia de 1:35.597 nacidos vivos , similar a la descrita en todo el mundo, siendo más frecuente en los varones. Forma parte de un espectro de defectos de cierre de la pared, que comprendería desde la epispadias como forma más leve hasta la extrofia de cloaca como forma más grave ...
Extrofia de vejiga: Material educativo para el paciente y la familia
Children's Hospital & Regional Medical Center, Seattle Washington
[Spanish]
¿Qué es la extrofia de vejiga?
La extrofia de vejiga es una vejiga que no está
formada correctamente. La vejiga y genitales están
partidos a la mitad, volteados de adentro hacia fuera
y situados afuera del cuerpo. Existen varias clases
de extrofias
Bladder Exstrophy
I.B.I.S. Birth Defects, May 26, 2003
[Ukrainian]
A fact sheet for parents in Ukrainian
What is bladder extrophy? ... Other anomalies associated with bladder extrophy ... Bladder extrophy in boys and girls ... Treatment ...
Cloacal Extrophy
I.B.I.S. Birth Defects, June 29, 2003
[Ukrainian]
A fact sheet for parents in Ukrainian
OEIS syndrome ... Life prognosis ... Sex determination problems ... Surgical treatment ...
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Last Updated: 2008/3/10
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