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.

• Surgical Procedure

  Penopubic epispadias repair in adulthood: Surgical and functional outcome with modified Cantwell-Ransley procedure. Sub-Saharan Afr J Med 2015;2:179-83 How to cite this URL: Atim T, Mustapha A. [ssajm.org]

  Salvage surgical procedures after failed reconstruction for an extrophy-epispadias complex are extremely challenging. [ncbi.nlm.nih.gov]

  Renal function: This is done to evaluate the renal function of the patient prior to any reconstructive surgical procedure of the urinary tract. [symptoma.com]

  Treatment of Epispadias The only available treatment of epispadias, aside from treating the symptoms it causes, is a surgical procedure to correct the position of the urethra. [ucipediatricurology.com]

• Infertility

  However, it remains unclear whether this finding may be due to assisted reproduction per se and/or underlying infertility/subfertility etiology or parent characteristics. Copyright © 2013 American Urological Association Education and Research, Inc. [ncbi.nlm.nih.gov]

  Upper urinary tract (ureter and kidney) damage and infertility may occur. References Gearhart JP, Mathews R. Exstrophy-epispadias complex. In: Wein AJ, ed. Campbell-Walsh Urology. 9th ed. Philadelphia, Pa: Saunders Elsevier; 2007:chap 119. Elder JS. [hopkinsmedicine.org]

  Ureter and kidney damage and infertility may occur. Call your health care provider if you have any questions about the appearance or function of your child's genitals or urinary tract. Congenital defect - epispadias Elder JS. [nlm.nih.gov]

  Sometimes the opening is right back near the root, causing difficulties with urination and infertility. [medical-dictionary.thefreedictionary.com]

• Weakness

  The disorder may be mild to severe and is often associated with wide pubic bones, abnormally rotated legs and feet, abnormal/weak abdominal muscles with an umbilical hernia where the umbilicus (belly button) sits above the bladder. [nicklauschildrens.org]

  Following the Kelly procedure, if the ring of muscle around the base of the bladder is too weak, urine could dribble out all the time. This also happens if the bladder cannot hold enough urine. [gosh.nhs.uk]

  […] visibility of the membrane of the bladder that is usually bright pink Abnormally-shaped abdominal muscles Displacement of the umbilicus (belly button), usually above the defect Umbilical hernia may be present (section of intestine protrudes through a weakness [stanfordchildrens.org]

  Bulging of intestine through a weak spot in the abdominal muscles (umbilical hernia) How is exstrophy of the bladder and epispadias diagnosed in a child? Exstrophy of the bladder can be diagnosed by fetal ultrasound before a baby is born. [lluch.org]

• Inguinal Hernia

  Associated anomalies include deformities of the external genitalia, inguinal hernia, diastasis of the public symphysis, deficiency of the urinary continence mechanism, vesicoureteral reflux, ectopia, and renal agenesis. [xpertdox.com]

  PURPOSE: Due to separated pubic bone and patent processus vaginalis, males with exstrophy-epispadias complex often present with inguinal hernia during infancy. [ncbi.nlm.nih.gov]

  Associated abnormalities: cryptorchidism and inguinal hernia. advertisement advertisement [auanet.org]

  Associated anomalies include deformities of the external genitalia, diastasis of the public symphysis, inguinal hernia, vesicoureteral reflux, deficiency of the urinary continence mechanism, renal agenesis and ectopia. [caps.nationwidechildrens.org]

• Pain

  Advanced Search: • Use “ “ for phrases o [ “pediatric abdominal pain” ] • Use – to remove results with certain terms o [ “abdominal pain” –pediatric ] • Use OR to account for alternate terms o [teenager OR adolescent ] [msdmanuals.com]

  The child will need to have regular pain relief after the operation. Initially, pain relief will be given through an epidural. [gosh.nhs.uk]

  But when it is painful or the bend is significant, it can lead to erectile dysfunction and may even make sexual intercourse impossible. [beaumont.org]

• Incontinence

  In contrast to other cases of epispadias, where urinary incontinence based on bladder neck and sphincter incompetence often is an important issue, bladder neck insufficiency was seen in only 1 case in our series without persisting incontinence. [jpurol.com]

  Three achieved continence; five had mild urinary incontinence; and 1 had moderate urinary incontinence and was awaiting an endoscopic injection. [ncbi.nlm.nih.gov]

  Female epispadias is often associated with urinary incontinence as in the presented case. The incontinence is varying from continuous dribbling of urine without bladder filling to single episodes of day-time stress-incontinence. [afrjpaedsurg.org]

  In this group surgery was followed by only slight improvement to a state of stress incontinence in one who was previously totally incontinent, total incontinence in another who had been incontinent prior to surgery, and continence in 3, one of whom now [pubs.rsna.org]

• Urinary Incontinence

  Three achieved continence; five had mild urinary incontinence; and 1 had moderate urinary incontinence and was awaiting an endoscopic injection. [ncbi.nlm.nih.gov]

  They may have trouble controlling urination (urinary incontinence). [nlm.nih.gov]

• Chordee

  Following disassembly, the three components are reassembled such that the urethra is in the most functional and normal position and dorsal chordee is corrected. [brandywineuc.com]

  Mobilization of urethral plate and proximal urethra up to bladder neck helps correction of chordee as well as torsion. [ncbi.nlm.nih.gov]

  Surgical technique Repair of epispadias aimed to reconstruct the epispadiac urethra and glans, and straighten the penis with correction of dorsal chordee. [jpurol.com]

  Types of Congenital Penile Anomalies Among Newborns, 1997-2000 Hypospadias 68.3% Chordee 8.6% Hypospadias plus chordee 5.0% Epispadias 1.9% Micropenis 1.8% Hidden penis 0.4% Other specific diagnostic combinations 0.1% Hypospadias plus micropenis 0.1% [freethesaurus.com]

  All forms of epispadias are associated with chordee. The extent of chordee varies. In females, epispadias consists of bifid clitoris with diastases of the corpora cavernosa, flattening of the mons, and separation of the labia. [emedicine.medscape.com]

• Cryptorchidism

  Associated abnormalities: cryptorchidism and inguinal hernia. advertisement advertisement [auanet.org]

  Management Journals Chemical Engineering Journals Chemistry Journals Clinical Journals Material Sciences Journals Medical Journals View more Relevant Topics Abortion Adenocarcinomas Azoospermia Balantis Benign Prostate Hyperplasia Cervical Dilation Cryptorchidism [omicsonline.org]

  In two patients who had cryptorchidism, orchiopexy was simultaneously performed. Figure 1. Penopubic epispadias. (A) Preoperative appearance of the patient with penopubic epispadias. (B) Complete penile disassembly. Glans with neurovascular bundles. [parjournal.net]

• Kidney Failure

  Depending on the severity of the VUR, sometimes the urine can flow backwards as far as the kidneys. This can damage the kidney and eventually lead to kidney failure. [gosh.nhs.uk]

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].

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

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].

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].

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.

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].

There are no guidelines for prevention of epispadias.

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].

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.

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.