eMedicine Specialties > Pediatrics: Surgery > Urology

Exstrophy and Epispadias

Author: Elizabeth B Yerkes, MD, Assistant Professor of Urology, Northwestern University Feinberg School of Medicine; Attending Urologist, Division of Urology, Children's Memorial Hospital of Chicago
Coauthor(s): Richard C Rink, MD, Chief of Pediatric Urology, Robert a Garrett Professor of Pediatric Urologic Research, Department of Urology, Riley Hospital for Children, Indiana University School of Medicine
Contributor Information and Disclosures

Updated: Mar 6, 2009

Introduction

Background

The exstrophy-epispadias complex comprises a spectrum of congenital abnormalities that includes classic bladder exstrophy, epispadias, cloacal exstrophy, and several variants. Each of these anomalies is thought to result from the same embryologic defect. Separation of the primitive cloaca into the urogenital sinus and hindgut occurs during the first trimester at approximately the same time as maturation of the anterior abdominal wall. Failure of mesenchyme to migrate between the ectodermal and endodermal layers of the lower abdominal wall leads to instability of the cloacal membrane. Premature rupture of the membrane before its caudal translocation leads to this complex of infraumbilical anomalies. Rupture of the cloacal membrane after complete separation of the genitourinary and GI tracts results in classic bladder exstrophy.

Open bladder plate and urethra with bifid clitori...

Open bladder plate and urethra with bifid clitoris in female patient with classic bladder exstrophy. Note low-set umbilicus and anteriorly displaced anus. Courtesy of Richard Rink, MD.

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Open bladder plate and urethra with bifid clitori...

Open bladder plate and urethra with bifid clitoris in female patient with classic bladder exstrophy. Note low-set umbilicus and anteriorly displaced anus. Courtesy of Richard Rink, MD.


However, rupture prior to descent of the urorectal septum allows externalization of the lower urinary tract and the distal GI tract (cloacal exstrophy). 

Cloacal exstrophy must be distinguished from the condition of persistent cloaca or cloacal malformation. The latter terms refer to the most extreme form of anorectal malformation in female infants. This complex anomaly involves incomplete separation of the urinary tract, genital tract and hindgut. No abdominal wall defect is present in persistent cloaca. 

Epispadias is a variant that displays normal bladder formation but incomplete urethral tubularization from the bladder neck down.

External view in female patient with epispadias. ...

External view in female patient with epispadias. Labia are separated anteriorly. Courtesy of Richard Rink, MD.

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External view in female patient with epispadias. ...

External view in female patient with epispadias. Labia are separated anteriorly. Courtesy of Richard Rink, MD.


Penopubic epispadias in male patient. Typical spa...

Penopubic epispadias in male patient. Typical spadelike configuration of glans penis with incomplete foreskin, dorsal urethral plate, and open bladder neck. Courtesy of Richard Rink, MD.

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Penopubic epispadias in male patient. Typical spa...

Penopubic epispadias in male patient. Typical spadelike configuration of glans penis with incomplete foreskin, dorsal urethral plate, and open bladder neck. Courtesy of Richard Rink, MD.


Pathophysiology

The true cause of bladder exstrophy has not been elucidated. In classic bladder exstrophy, the lower urinary tract, genitalia, and musculoskeletal system are affected. Cloacal exstrophy is a much more severe abnormality, with significant involvement of the GI tract and CNS. In epispadias, only the urethra and external genitalia are involved.

Frequency

United States

Prevalence of classic bladder exstrophy is 3.3 per 100,000 births; male epispadias occurs in 1 in 117,000 births, female epispadias in 1 in 484,000 births, and cloacal exstrophy in 1 in 200,000-400,000 births.

International

Prevalence is the same as in the United States.

Mortality/Morbidity

Mortality with classic bladder exstrophy or epispadias is rare. Morbidity associated with underlying conditions or surgical therapy is discussed in Complications.

Cloacal exstrophy was associated historically with significant mortality. Reconstruction was not attempted until the 1970s. Advances in the care of critically ill neonates and recognition of the importance of early parenteral nutritional support have allowed successful reconstruction and survival of children with cloacal exstrophy.

Race

These conditions seem to be more common in whites than in other races.

Sex

For classic bladder exstrophy, the male-to-female ratio is 2.3:1 and as high as 6:1 in some series.

Age

Because this is a congenital abnormality, newborns are affected.

Clinical

History

  • Antenatal ultrasonography findings suggestive of exstrophy-epispadias complex include the following:
    • Repeated failure to visualize the bladder on ultrasonography
    • Lower abdominal wall mass
    • Low-set umbilical cord
    • Abnormal genitalia
    • Increased pelvic diameter
  • Additional antenatal ultrasound findings suggestive of cloacal exstrophy include omphalocele, limb abnormalities, myelomeningocele, and trunk sign from prolapsed intestine. Increased use of fetal MRI may further improve the accuracy of prenatal diagnosis, but this test is not necessary if suspicion is high given ultrasonography findings.
  • Classic bladder exstrophy and cloacal exstrophy are obvious to all in the delivery room. Most exstrophy variants and epispadias are also identifiable at birth.
  • Unrecognized female epispadias may present as persistent childhood incontinence.
  • Unrecognized split-symphysis variants of exstrophy may be identified in childhood only because of persistent incontinence or a waddling gait.

Physical

  • General
    • Classic bladder exstrophy or epispadias: Patients typically appear as term infants.
    • Cloacal exstrophy: Patients are often preterm. They may have respiratory embarrassment requiring mechanical ventilation.
  • Abdomen
    • Classic bladder exstrophy: The bladder is open on the lower abdomen, with mucosa fully exposed through a triangular fascial defect. The abdominal wall appears long because of a low-set umbilicus on the upper edge of the bladder plate. The distance between the umbilicus and anus is foreshortened. Rectus muscles diverge distally, attaching to the widely separated pubic bones. Indirect inguinal hernias are frequent (>80% of males, >10% of females) due to wide inguinal rings and the lack of an oblique inguinal canal.
    • Epispadias: The pubic symphysis is generally widened. The rectus muscles are divergent distally.
    • Cloacal exstrophy: Nearly all patients have an associated omphalocele. The bladder is open and separated into 2 halves, flanking the exposed interior of the cecum. Openings to the remainder of the hindgut and to 1 or 2 appendices are evident within the cecal plate. Terminal ileum may prolapse as a "trunk" of bowel onto the cecal plate.
    • Exstrophy variants: The pubic symphysis is widely separated, and rectus muscles diverge distally. The umbilicus is low or elongated. A small superior bladder opening or a patch of isolated bladder mucosa may be present. The intact bladder may be externally covered by only a thin membrane. Isolated ectopic bowel segments have been reported. Patent urachus is a differential diagnosis for the superior vesical fissure variant of exstrophy-epispadias. However, patent urachus lacks the typical musculoskeletal abnormalities of exstrophy and is open at the umbilicus. Superior vesical fissure is infraumbilical.
  • Genitalia (When describing the anatomy of the penis, the terms "dorsal" and "ventral" refer to a normal phallus in the erect state. The dorsal surface is in continuity with the abdominal wall, and the ventral surface is in continuity with the scrotum.)
    • Classic bladder exstrophy (male): The phallus is short and broad with upward curvature (dorsal chordee). The glans lies open and flat like a spade, and the dorsal component of the foreskin is absent. The urethral plate extends the length of the phallus without a roof. The bladder plate and urethral plate are in continuity, with the verumontanum and ejaculatory ducts visible within the prostatic urethral plate. The anus is anteriorly displaced with a normal sphincter mechanism.
    • Epispadias (male): The phallus is short and broad with upward curvature (dorsal chordee). The glans lies open and flat like a spade, and the dorsal component of the foreskin is absent. The urethral meatus is located on the dorsal penile shaft, anywhere between the penopubic angle and the proximal margin of the glans.
    • Classic bladder exstrophy (female): The clitoris is uniformly bifid with divergent labia superiorly. The open urethral plate is in continuity with the bladder plate. The vagina is anteriorly displaced. The anus is anteriorly displaced with a normal sphincter mechanism.
    • Epispadias (female): The clitoris is most often bifid with divergent labia superiorly. The dorsal aspect of the urethra is open distally. The urethra and bladder neck are patulous and may allow visualization of bladder. Bladder mucosa may prolapse through the bladder neck.
    • Cloacal exstrophy: The penis is generally quite small and bifid, with a hemiglans located just caudal to each hemibladder. Infrequently, the phallus may be intact in the midline. In females, the clitoris is bifid and 2 vaginas are present. The anus is absent.
    • Exstrophy variants: Genitalia generally are intact, though epispadias can occur.
  • Musculoskeletal
    • Classic bladder exstrophy: The pubic symphysis is widely separated. Divergent rectus muscles remain attached to the pubis. External rotation of the innominate bones results in a waddling gait in ambulatory patients but does not appear to result in orthopedic problems later in life.
    • Cloacal exstrophy: Examination is the same as for bladder exstrophy. As many as 65% of patients have a clubfoot or major deformity of a lower extremity. As many as 80% of patients have vertebral anomalies.
    • Exstrophy variants: In these split-symphysis variants of exstrophy, the pubic symphysis is widely separated, and the rectus muscles are divergent.
  • Neurologic: In cloacal exstrophy, as many as 95% of patients have myelodysplasia, which may include myelomeningocele, lipomeningocele, meningocele, or other forms of occult dysraphism. These patients are at risk of neurologic deterioration, and they should be observed closely. Early neurosurgical consultation is appropriate if a radiographic abnormality of the spinal cord or canal is observed.

Causes

  • No definitive risk factors or causative agents are known. Based on the known embryologic principles of cloacal development, any inciting event would occur early in pregnancy. 
  • Offspring of patients with exstrophy-epispadias complex have a 1 in 70 risk (500 times that of the general population) of being affected. Nevertheless, familial occurrence is uncommon in large series.1  
  • Exstrophy has been reported in twins. Concordance rates show strong evidence of genetic effects, but less than 100% concordance among identical twins suggests some role for environmental effect on development of exstrophy-epispadias.
  • A higher incidence of bladder exstrophy is observed in infants of younger mothers and in those with relatively high parity.
  • Maternal tobacco exposure is associated with more severe defects (cloacal vs classic exstrophy).
  • Growing evidence suggests an increased incidence of cloacal exstrophy and bladder exstrophy-epispadias in vitro fertilization (IVF) pregnancies.
  • Heredity of cloacal exstrophy has not been established because no offspring have been reported.

More on Exstrophy and Epispadias

Overview: Exstrophy and Epispadias
Differential Diagnoses & Workup: Exstrophy and Epispadias
Treatment & Medication: Exstrophy and Epispadias
Follow-up: Exstrophy and Epispadias
Multimedia: Exstrophy and Epispadias
References
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Further Reading

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Keywords

exstrophy-epispadias complex, exstrophy-epispadias syndrome, complete epispadias, incontinent epispadias, penopubic epispadias, superior vesical fissure, cloacal exstrophy, OEIS sequence, vesicointestinal fissure, short-gut syndrome, omphalocele, myelodysplasia, myelomeningocele, lipomeningocele, meningocele, chordee, rupture of the membranes, cloacal malformation, incontinence, inguinal hernia, patent urachus, clubfoot

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Contributor Information and Disclosures

Author

Elizabeth B Yerkes, MD, Assistant Professor of Urology, Northwestern University Feinberg School of Medicine; Attending Urologist, Division of Urology, Children's Memorial Hospital of Chicago
Elizabeth B Yerkes, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Pediatrics, American Urological Association, North American Society for Pediatric and Adolescent Gynecology, Phi Beta Kappa, Society for Fetal Urology, and Society for Pediatric Urology
Disclosure: Nothing to disclose.

Coauthor(s)

Richard C Rink, MD, Chief of Pediatric Urology, Robert a Garrett Professor of Pediatric Urologic Research, Department of Urology, Riley Hospital for Children, Indiana University School of Medicine
Richard C Rink, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Surgeons, American Urological Association, and Indiana State Medical Association
Disclosure: Nothing to disclose.

Medical Editor

Howard M Snyder III, MD, Professor, Department of Surgery, Division of Pediatric Urology, University of Pennsylvania School of Medicine
Howard M Snyder III, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Surgeons, American Medical Association, American Urological Association, and National Kidney Foundation
Disclosure: Nothing to disclose.

Pharmacy Editor

Mary L Windle, PharmD, Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy, Pharmacy Editor, eMedicine
Disclosure: Pfizer Inc Stock Investment from financial planner; Avanir Pharma Stock Investment from financial planner ; WebMD Salary and stock Employment and investment from financial planner

Managing Editor

Harry P Koo, MD, Chairman of Urology Division and Director of Pediatric Urology, Virginia Commonwealth University; Professor of Surgery, VCU School of Medicine, Medical College of Virginia; Director of Urology, Children's Hospital of Richmond
Harry P Koo, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Surgeons, and American Urological Association
Disclosure: Nothing to disclose.

CME Editor

Daniel Rauch, MD, FAAP, Director, Pediatric Hospitalist Program, Associate Professor, Department of Pediatrics, New York University School of Medicine
Daniel Rauch, MD, FAAP is a member of the following medical societies: Ambulatory Pediatric Association, American Academy of Pediatrics, and Society of Hospital Medicine
Disclosure: Baxter Honoraria Consulting

Chief Editor

Marc Cendron, MD, Associate Professor of Surgery, Harvard School of Medicine; Consulting Staff, Department of Urological Surgery, Children's Hospital Boston
Marc Cendron, MD is a member of the following medical societies: American Academy of Pediatrics, American Urological Association, European Society for Paediatric Urology, Johns Hopkins Medical and Surgical Association, New Hampshire Medical Society, Society for Fetal Urology, and Society for Pediatric Urology
Disclosure: Nothing to disclose.

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