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Epispadias

Epispadias is a congenital genitourinary malformation which occurs in males and females.


Presentation

In males, epispadias may involve only the penis or may be associated with exstrophy of other organs such as the bladder.

The opening of the urethra can be situated anywhere on the upper surface of the penis. Usually, the penis is of a small size. It is also frequently bifid.

In females, the opening of the urethra is present anteriorly. It frequently opens in the clitoris, in which case the clitoris may be bifid.

Crying
  • One patient experienced urine leakage while playing or crying in the initial postoperative period. However, this patient became continent with a dry period of more than 3 hours after 4 months of follow-up.[ncbi.nlm.nih.gov]
Thrombosis
  • In 1 patient the distal third of the phallus was lost due to acute thrombosis of the arterial anastomosis immediately postoperatively.[ncbi.nlm.nih.gov]
Secondary Amenorrhea
  • The patient was referred for evaluation of excessive weight gain, secondary amenorrhea, and abnormal external genitalia.[ncbi.nlm.nih.gov]
Distractibility
  • He underwent a supraacetabular osteotomy of the pelvis with progressive anterior distraction of the anterior segment of the pelvic ring. Four months later, hardware was removed, and the APD was near normal value.[ncbi.nlm.nih.gov]

Workup

In all patients with epispadias and exstrophy, a number of studies are necessary before reconstructive surgery can be performed.

  • Renal function: This is done to evaluate the renal function of the patient prior to any reconstructive surgical procedure of the urinary tract.
  • Electrolyte studies: In patients suffering from cloacal exstrophy, electrolyte studies need to be performed to exclude electrolyte abnormalities produced by losses of salts from the terminal ileum.
  • Renal ultrasonography: Renal ultrasonography is useful for identifying other associated congenital anomalies of the upper urinary tract.
  • Spinal ultrasonography: This is of importance in patients who have cloacal exstrophy for identifying abnormalities that can lead to spinal cord tethering.
  • Voiding cystourethrography: This is used to investigate the competence of the ureteric sphincters at their openings into the bladder. Most cases of bladder exstrophy are associated with bilateral vesicoureteral reflux [5].
Suppression
  • Vesicoureteral reflux appeared in 60% of patients; despite suppressive antibiotic therapy, 33.3% are awaiting reimplantation. Continence with volitional voiding with dry intervals of 3 h was achieved in 40% of patients.[ncbi.nlm.nih.gov]
  • The erection was suppressed in post-operative period for 10 days by giving tablet Calmpose 5 mg at bedtime. In all cases, suprapubic cystostomy was done as a proximal diversion.[ijps.org]
Ischemic Changes
  • A conical-shaped glans with the absence of any ischemic changes occurred in 94% of patients. A mild degree of penile dorsal tilt occurred in 11.7% of patients, urethrocutanous fistula in 17.6% and meatal stenosis 5.8%.[ncbi.nlm.nih.gov]
Squamous Metaplasia of the Bladder
  • The persistence of the squamous metaplasia in the bladder despite twenty years of "turn-in" is noteworthy. The original method of closure reveals no "secret" to account for the success. The method of repair of traumatic rupture is presented.[ncbi.nlm.nih.gov]

Treatment

Management is surgical, aiming for urinary continence and functional genital reconstruction [7] [8].

Prognosis

Isolated epispadias is relatively easy to correct by surgical means. Patients suffering from epispadias along with any form of exstrophy require complex reconstructive surgery [6].

Therefore, the prognosis after surgery varies depending from patient to patient. The post-surgical survival rates for all surgical cases are excellent.

In most cases, bladder function and urinary continence can be restored if proper surgery is performed [7] [8].

Etiology

Epispadias is almost purely a developmental disorder. No causal agents or risk factors have been identified. There is very little evidence to support familial occurrence [3].

Epidemiology

Isolated epispadias is a very rare condition. It is present in only 1 in around 117,000 live births in males and 1 in 484,000 live births in females.

Epispadias associated with any form of exstrophy is much more frequent. Epispadias associated with bladder exstrophy occurs in 1 in 30,000 live births. Cloacal exstrophy is much rarer and occurs in 1 in 200,000 to 400,000 live births [2].

Males are affected much more commonly; the male to female ratio being 2.3:1. Besides, whites are affected much more commonly as compared to blacks.

Sex distribution
Age distribution

Pathophysiology

Epispadias is basically a developmental disorder that arises during the first trimester. At that time, the primitive cloaca is supposed to divide into the urogenital sinus and the hindgut.

Parallel to this process, the anterior abdominal wall also undergoes maturation. Normally, the mesenchyme migrates between the ectoderm and endoderm of the lower abdominal wall. This provides stability to the wall and prevents any form of exstrophy from developing.

Failure of the mesenchyme to migrate between those layers causes the cloacal membrane to become unstable. If the cloacal membrane ruptures before it has fully descended into the caudal area of the embryo, a number of anomalies including cloacal and bladder exstrophy can develop.

In classic epispadias, there is no bladder or cloacal exstrophy but the external meatus of the urethra lies on the dorsum of the penis. This happens when the bladder has formed normally but the urethra has not completely tubularized [4].

Prevention

There are no guidelines for prevention of epispadias.

Summary

Epispadias is a partial form of a complex of malformations in which there is the failure of the development of the anterior wall of the lower urogenital tract.

It affects both males and females. In males, typically the external meatus of the urethra is situated at some point on the dorsum of the penis. Isolated epispadias however, is very rare. Exstrophy of the bladder is frequently present in association with epispadias.

In the females, epispadias is said to be present when the urethra is positioned very anteriorly, where it may open in the clitoris or even further ahead [1].

Patient Information

Epispadias is a condition in which the opening of the urinary outlet lies on the upper surface of the penis instead of the tip. It may be associated with other abnormalities in which the bladder or intestines protrude from the abdominal wall.

All of these abnormalities arise during fetal development inside the mother. Surgery is required to correct these abnormalities. The results of surgery are usually favorable.

References

Article

  1. Shetty MV, Bhaskaran A, Sen TK. Female epispadias. African journal of paediatric surgery : AJPS. May-Aug 2011;8(2):215-217.
  2. Jayachandran D, Bythell M, Platt MW, Rankin J. Register based study of bladder exstrophy-epispadias complex: prevalence, associated anomalies, prenatal diagnosis and survival. The Journal of urology. Nov 2011;186(5):2056-2060.
  3. Gambhir L, Holler T, Muller M, et al. Epidemiological survey of 214 families with bladder exstrophy-epispadias complex. The Journal of urology. Apr 2008;179(4):1539-1543.
  4. Patten BM, Barry A. The genesis of exstrophy of the bladder and epispadias. The American journal of anatomy. Jan 1952;90(1):35-57.
  5. Ambrose SS. Epispadias and vesicoureteral reflux. Southern medical journal. Oct 1970;63(10):1193-1194.
  6. Zhang F, Kang C, Wang X, Gu B, Liu J. Surgical treatment of epispadias. Zhonghua wai ke za zhi [Chinese journal of surgery]. Mar 1998;36(3):133-135.
  7. Wittmeyer V, Aubry E, Liard-Zmuda A, et al. Quality of life in adults with bladder exstrophy-epispadias complex. The Journal of urology. Dec 2010;184(6):2389-2394.
  8. Stefan H. Congenital epispadias with urinary incontinence: long-term results of reconstructive therapy. Rozhledy v chirurgii : mesicnik Ceskoslovenske chirurgicke spolecnosti. Oct 1993;72(7):319-323.
  9. Bastuba MD, Alper MM, Oates RD. Fertility and the use of assisted reproductive techniques in the adult male exstrophy/epispadias patient. Fertility and sterility. Oct 1993;60(4):733-736.
  10. Wood HM, Babineau D, Gearhart JP. In vitro fertilization and the cloacal/bladder exstrophy-epispadias complex: a continuing association. Journal of pediatric urology. Aug 2007;3(4):305-310.

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Last updated: 2017-08-09 17:48