AUTHOR AND EDITOR INFORMATION

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• Follow-up
• Miscellaneous
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INTRODUCTION

Section 2 of 11  

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

Pathophysiology

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.

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 are 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

As this is a congenital abnormality, newborns are affected.

CLINICAL

Section 3 of 11  

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History

• Antenatal ultrasound findings suggestive of exstrophy-epispadias complex include the following:
• • Repeated failure to visualize the bladder on ultrasound
  • 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.
• Classic bladder exstrophy and cloacal exstrophy are obvious to all in the delivery room. Most exstrophy variants and epispadias also are 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 covered by only a thin membrane externally. 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 uniformly 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, and meningocele. These patients are at risk of neurologic deterioration, and they should be observed closely.

Causes

• No risk factors or causative agents are known.
• Offspring of patients with exstrophy-epispadias complex have a 1 in 70 risk (500 times that of the general population) of being affected.
• Exstrophy has been reported in twins. In no cases were fraternal twins both affected. While all sets of identical twin males were concordant for exstrophy, in none of the sets of female identical twins were both twins affected.
• A higher incidence of bladder exstrophy is observed in infants of younger mothers and in those with relatively high parity.
• Heredity of cloacal exstrophy has not been established, since no offspring have been reported.

DIFFERENTIALS

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Other Problems to be Considered

Patent urachus

WORKUP

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Lab Studies

• Basic metabolic panel
• • Information about baseline renal function is important before complex reconstruction of the urinary tract.
  • Losses from the terminal ileum short-gut physiology can result in significant electrolyte abnormalities in patients with cloacal exstrophy.

Imaging Studies

• Renal ultrasound
• • Baseline examination of the kidneys is recommended for all patients with exstrophy, since increased bladder pressure after bladder closure can lead to hydronephrosis and upper urinary tract deterioration.
  • Congenital upper urinary tract anomalies are uncommon with classic exstrophy and epispadias but are present in approximately one third of patients with cloacal exstrophy (eg, ectopic pelvic kidney, renal agenesis, hydronephrosis).
• Spinal ultrasound or radiographs
• • Myelodysplasia should be excluded in newborns with cloacal exstrophy. This can be accomplished through ultrasound studies early in life.
  • MRI is recommended in cloacal exstrophy to help identify occult abnormalities that may predispose to symptomatic spinal cord tethering.
• Voiding cystourethrogram
• • Bilateral vesicoureteral reflux is present in nearly all patients with classic bladder exstrophy.
  • This study is performed in early childhood to assess bladder capacity in preparation for continence reconstruction.

Histologic Findings

Histologic studies are not performed routinely. However, clinical investigations have revealed an increased ratio of extracellular matrix to muscle within the bladder wall. In particular, the amount of type III collagen is increased. This ratio seems to approach a more normal range (ie, relative increase in muscle) after successful bladder closure. Exstrophied bladders also have fewer myelinated nerve fibers than unaffected bladders.

TREATMENT

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Medical Care

• Initiate general supportive care appropriate for the neonate's overall condition and associated anomalies.
• Institute parenteral nutrition early for patients with cloacal exstrophy.
• Place clean plastic wrap over the bladder plate. Avoid moistened or impregnated gauze, which is irritating to the delicate bladder mucosa. Mist tents may be used to protect exposed tissue.
• Start antibiotic therapy after delivery and continue through the early postoperative period.
• • Daily prophylactic antibiotic therapy is continued after bladder closure.
  • Infections may be related to poor emptying and are to be prevented in light of the high incidence of vesicoureteral reflux. Institute latex precautions due to high incidence of latex sensitization in patients with exstrophy-epispadias complex.

Surgical Care

Reconstruction of exstrophy-epispadias complex remains one of the greatest challenges facing the pediatric urologist. Many modifications in surgical procedures have improved the outcome for these patients, but the optimal approach remains uncertain. Longitudional prospective assessment of both surgical approaches is critical to optimize functional and cosmetic outcomes.

Goals of therapy include restoration of urinary continence with preservation of renal function and reconstruction of functional and cosmetically acceptable genitalia. Creation of a neo-umbilicus is also important to many of these patients. Surgical techniques include the following:

• Staged functional closure for classic bladder exstrophy
• • Traditional approach comprises a series of operations.
  • Initial bladder closure is completed within 72 hours of birth. If delayed, pelvic osteotomies are required to facilitate successful closure of the abdominal wall and to allow the bladder to lie within a closed and supportive pelvic ring.
  • Epispadias repair with urethroplasty at age 12-18 months allows enough increase in bladder outlet resistance to improve the bladder capacity.
  • Bladder neck reconstruction at age 4 years (typically a modified Young-Dees-Leadbetter repair) allows continence and correction of vesicoureteral reflux. Multiple modifications have been proposed. The procedure is delayed until bladder capacity has reached 60 mL.
• Complete primary repair for classic bladder exstrophy
• • Newer approach typically is completed in a single stage.
  • Primary bladder closure, urethroplasty, and genital reconstruction are performed in a single stage in newborns. This procedure involves complete penile disassembly in males.
  • The goal is early bladder cycling. Preliminary results are encouraging, and some patients ultimately may not require additional reconstruction for continence.
• Urinary diversion for classic bladder exstrophy
  • This procedure was the original surgical treatment of choice.
  • Diversion may be performed in a patient with an extremely small bladder plate not suitable for functional closure.
  • In Europe, early diversion has been widely used, with success for most exstrophy patients.
• Closure for cloacal exstrophy
  • Treatment of myelodysplasia and GI anomalies has priority over management of urinary and genital anomalies.
  • Closure can be staged or performed in one stage, depending on the overall condition of the child and the severity of the abdominal wall defect. A large omphalocele may limit the success of closing the abdomen and the bladder in one stage.
  • First stage involves separation of the GI and genitourinary tracts, closure of the colony, creation of a terminal end colostomy, and closure of the omphalocele. The bladder plates are brought together in the midline.
  • The hindgut should be incorporated into the GI tract to maximize absorptive surface area, since virtually all of these patients have some element of short-gut syndrome. Ileostomy should be avoided because of the high incidence of recurrent hospitalizations for dehydration and severe electrolyte abnormalities.
  • Subsequent bladder closure is as in classic bladder exstrophy. Pelvic osteotomies are needed because of the major separation of the symphysis, which is usually greater than in classic exstrophy.
• Gender reassignment
  • Historically, all males with cloacal exstrophy underwent early gender conversion because of inadequate male genitalia.
  • Testicular histology is normal despite frequent cryptorchidism.
  • Evidence suggesting that testosterone in utero has a significant impact on the developing brain has led to a change in surgical philosophy, as has anecdotal evidence suggesting that raising a 46,XY cloacal exstrophy patient as female can result in significant gender dysphoria.
• Intraoperative concerns of latex sensitization and latex allergy
  • Multiple or lengthy surgical procedures with exposure to latex antigens increase the risk of latex sensitization or allergy. Approximately 30% of patients with bladder exstrophy have demonstrated symptoms of latex allergy, and 70% reveal sensitization (elevation of specific immunoglobulin E [IgE] antibody) to latex antigens.
  • Full latex precautions are recommended in the operating room, beginning with preparation for the first operative procedure. Potential latex-containing materials in the operating room include gloves, catheters, drains, masks, anesthesia materials, bandages, and thromboembolic stockings. Polyvinyl chloride and silicone are acceptable alternatives. Latex allergy should be considered seriously in the event of intraoperative anaphylaxis. The offending agent should be removed and the surgical procedure aborted if necessary.
  • Treatment includes cardiopulmonary resuscitation with fluids, epinephrine, steroids, and histamine blockade. In those with a known latex allergy, premedication with steroids and H1 and H2 blockers should be considered.

Consultations

• Neonatologist for the special needs of a premature newborn and the care of a child with multiple anomalies
• Pediatric gastroenterologist if needed for the patient with cloacal exstrophy to manage the short-gut physiology with parenteral and modified external feeding
• Pediatric urologist or surgeon with special interest and expertise in the management of exstrophy-epispadias complex
• Neurosurgeon for patients with cloacal exstrophy with myelodysplasia
• Orthopedic surgeon for lower-extremity anomalies and if pelvic osteotomies are required for bladder closure
• Child psychiatrist if gender conversion is considered

Diet

Some young patients with cloacal exstrophy are seriously affected by short-gut syndrome and may depend on long-term supplemental parenteral nutrition for growth and development.

Activity

• Patient is kept in modified Bryant traction for 3 weeks after exstrophy closure. Alternatively, spica casts, lower extremity wraps, and external pelvic fixators are used based upon performance of osteotomies and institutional protocol.
• In childhood, patient may have a waddling gait because of abnormal pelvic rotation, but activity is unrestricted and no long-term orthopedic sequelae occur.

MEDICATION

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Antibiotics are given to prevent urinary tract infections.

Drug Category: Broad-spectrum antibiotics

These agents are used for prevention of perioperative tissue infection due to preoperative colonization of urinary tract or multiple indwelling catheters.

Drug Category: Prophylactic antibiotics

These agents are used for prevention of urinary tract infection in light of vesicoureteral reflux and possible inefficient bladder emptying.

FOLLOW-UP

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Further Inpatient Care

• After surgery, patients with exstrophy remain in the hospital in modified Bryant traction (legs adducted and pelvis slightly elevated) for 3 weeks after bladder closure. Alternative techniques of immobilization may be used based upon osteotomies or institutional protocol.
• Bladder and kidneys are drained fully with multiple catheters during the first few weeks after closure.
• Nutritional support is mandatory for patients with cloacal exstrophy. Patients with classic bladder exstrophy also may have early difficulties feeding because of the body position in traction.

Further Outpatient Care

• Remaining urinary drainage catheters are removed as part of outpatient follow-up care. Prophylactic antibiotics are continued.
• Lifelong follow-up care with a urologist is important to monitor for deterioration in bladder function or damage to the kidneys and to direct appropriate surgical therapy.
• Patients with cloacal exstrophy require long-term follow-up care with a neurosurgeon, orthopedic surgeon, and general surgeon.

In/Out Patient Meds

• Maintain oral antibiotic prophylaxis as described in Medication.

Complications

• In the treatment of complex congenital anomalies, the distinction between technical complications and problems inherent to the anomaly is not always obvious.
• Failure of closure may occur.
• • If the bladder plate is adequate, reclosure with pelvic osteotomies is recommended. In this instance, bladder closure and epispadias repair are performed in one stage.
  • Urinary diversion is the alternative therapy.
• Injury to Genitalia - Loss of the hemiglans or corporal body has been reported as a result of complete primary repair.
• Upper urinary tract deterioration is a potential complication. Causes include excessive outlet resistance and high pressure in a small-capacity reservoir and persistent vesicoureteral reflux.
• Abnormal bladder function may result in poor emptying. Clinical problems related to poor emptying include recurrent febrile infections, epididymitis, bladder stones, acute urinary retention, and rupture of the native bladder.
• Bladder prolapse is a potential complication.
• • Posterior bladder wall may prolapse through the patulous bladder neck after primary closure.
  • Recurrent prolapse, congestion, ischemia of bladder mucosa, or failure of ureteral drainage warrants early surgical correction.
• Malignancy is a rare late complication of bladder exstrophy, more common in untreated patients whose bladders are left exstrophic for many years.
  • Adenocarcinoma is the most common of these malignancies, from the precursor cystitis glandularis, which is caused by chronic irritation and inflammation of exposed mucosa of the exstrophic bladder.
  • Squamous cell carcinoma and rhabdomyosarcoma also have been reported.
  • Adenocarcinoma may develop adjacent to the ureterointestinal anastomosis in patients with urinary diversions that mix the urinary and fecal streams. This malignancy was reported in more than 10% of patients in one series.
  • Patients younger than 25 years with ureterosigmoidostomy have a 7000-fold greater risk of adenocarcinoma of the colon than the general population (mean latency, 10 y).
• Complications of short-gut syndrome are as follows:
  • Paucity of hindgut and, in many cases, limited small intestine can result in electrolyte abnormalities in patients with cloacal exstrophy.
  • Dehydration is particularly a concern during an acute GI illness with diarrhea.
  • Nutritional supplementation may be required.

Prognosis

• Survival: Postsurgical rates are excellent.
• Bladder function and/or continence: Reports vary depending on the type of reconstruction.
• • Objective and subjective evidence indicates that many exstrophic bladders do not function normally after reconstruction and may deteriorate over time.
  • Continence rates of 75-90% have been reported after staged reconstruction in classic exstrophy, but more than one continence procedure may be required (eg, bladder neck reconstruction, bladder augmentation, bladder neck sling, artificial urinary sphincter). Many of these patients require clean intermittent catheterization (CIC) through the urethra or a continent stoma because they are unable to void spontaneously to completion. Less encouraging results also are reported.
  • Continence results after staged reconstruction are poor (<25%) in cloacal exstrophy because of abnormal bladder innervation in many patients.
  • Experience with rectal reservoirs (ureterosigmoidostomy and variants) for exstrophy continence demonstrates rates greater than 95%, but they present long-term malignancy risks.
  • Continent reconstruction with intestinal bladder augmentation and CIC has a success rate greater than 90%.
• Sexuality
• • Males are generally potent, but some report inadequate phallus or residual curvature.
  • Females report normal sexual function.
• Fertility and childbearing
  • Retrograde ejaculation or iatrogenic obstruction of the ejaculatory ducts or vas deferens after surgical reconstruction often results in abnormal semen analysis. However, fertilization, with viable pregnancy, has been achieved by male patients with exstrophy.
  • Females have had successful pregnancies. Cesarean section is recommended to avoid injury to continence mechanism. Postpartum uterine prolapse is common because of aggravation of preexisting abnormal pelvic support.
• Psychosocial
• • Education, employment, and social relationships generally are not affected substantially in adults with a history of bladder exstrophy and epispadias.
  • Age-appropriate adaptive behaviors may be delayed in children with chronic medical conditions. One study revealed below-average daily living skills and socialization but above-average self-esteem.
  • Children may need support in disclosing their condition to new peers.
  • Multiple anomalies associated with cloacal exstrophy can have a significant impact on daily life. Patients are affected by permanent colostomy, the need for CIC, and impaired ambulation.

Patient Education

• Because of the potential long-term implications of exstrophy, patient education is critical. Exstrophy support groups are established at several major medical centers.

MISCELLANEOUS

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Medical/Legal Pitfalls

• Exstrophy-epispadias syndrome is a complex anomaly that often requires several surgical procedures and requires lifelong medical follow-up care. Patients and families need to be counseled about the complexity of the anomaly, the need for multiple procedures, and long-term expectations for continence, sexual function, and fertility. The risks, benefits, and alternatives of urinary tract reconstruction must be explained carefully to the family, and they must accept responsibility for daily care of the patient (eg, CIC after bladder augmentation) before surgery can proceed.

Special Concerns

• Children with congenital abnormalities that require surgical attention within the first few days of life, such as bladder exstrophy and spina bifida, have an increased incidence of latex sensitization and symptomatic latex allergy. Atopic children may be even more at risk for symptoms. However, early and recurrent exposure to latex antigens can lead to sensitization, even in children with no history of atopy.
• Consider all patients with exstrophy-epispadias complex to be latex-sensitive.
• • Latex-free catheters and gloves should be used in daily care, both in the hospital and at home.
  • Balloons, bandages, waistbands of undergarments, and other common items may contain natural rubber latex. Offending items should be avoided.
• Those with documented allergic reaction to latex should wear a MedicAlert tag and carry an anaphylaxis kit.

MULTIMEDIA

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Media file 1:  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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Media file 2:  Male with classic bladder exstrophy. Note low-set umbilicus and short, broad, upturned phallus. Distance between phallus and scrotum is abnormal. Courtesy of Richard Rink, MD.
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Media file 3:  External view in female patient with epispadias. Labia are separated anteriorly. Courtesy of Richard Rink, MD.
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Media file 4:  Female patient with epispadias with labia retracted. The clitoris is bifid, and the urethra is open dorsally. Courtesy of Richard Rink, MD.
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Media file 5:  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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Media file 6:  46,XY newborn with cloacal exstrophy. A very large omphalocele is present, and the hemibladders flank the exstrophic cecal plate. The phallus is small and bifid, with the hemiglans and hemiscrotum located just distal to the bladder. Courtesy of Martin Kaefer, MD.
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Media file 7:  Lipomeningocele in the same newborn shown in Image 6, a 46,XY newborn with cloacal exstrophy. Courtesy of Martin Kaefer, MD.
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Media file 8:  Superior vesical fissure variant of exstrophy. Genitalia are normal. Patent urachus is considered in the differential diagnosis, although the opening is well below the umbilicus. Rectus abdominis and pubic abnormalities are found in the exstrophic variant but not with patent urachus. Courtesy of Richard Rink, MD.
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Media file 9:  Female with covered exstrophy. The umbilicus is very low, the pubic bones are widely separated, and the rectus muscles are divergent. The bladder is intact, but the patient has undergone bladder neck reconstruction to achieve urinary continence. The external genitalia are normal. Courtesy of Elizabeth Yerkes, MD.
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Media file 10:  Typical appearance of pelvis in patient with exstrophy-epispadias complex with wide separation of pubic symphysis.
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Media file 11:  Bilateral vesicoureteral reflux and small-capacity bladder after initial exstrophy closure. Contrast escapes readily through the incompetent bladder neck.
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Media type:  X-RAY

Media file 12:  Use of plastic wrap to protect the delicate bladder mucosa in a newborn with bladder exstrophy. Courtesy of Richard Rink, MD.
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Media file 13:  Subtotal prolapse of bladder wall through patulous bladder neck after bladder closure in female with cloacal exstrophy. The bladder mucosa appears healthy, and urine is draining freely. Courtesy of Richard Rink, MD.
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REFERENCES

Section 11 of 11

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• Introduction
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• Differentials
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• Cerniglia FR Jr, Roth DR, Gonzales ET Jr. Covered exstrophy and visceral sequestration in a male newborn: case report. J Urol. Apr 1989;141(4):903-4. [Medline].
• Connolly JA, Peppas DS, Jeffs RD, Gearhart JP. Prevalence and repair of inguinal hernias in children with bladder exstrophy. J Urol. Nov 1995;154(5):1900-1. [Medline].
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Article Last Updated: May 18, 2006