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a congenital abnormality in males in which the urethra is on the upper surface of the penis

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出典(authority):フリー百科事典『ウィキペディア（Wikipedia）』「2015/07/03 05:54:17」(JST)

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An epispadias is a rare type of malformation of the penis in which the urethra ends in an opening on the upper aspect (the dorsum) of the penis. It can also develop in females when the urethra develops too far anteriorly. It occurs in around 1 in 120,000 male and 1 in 500,000 female births.^{[citation needed]}

Causes

Epispadias is an uncommon and partial form of a spectrum of failures of abdominal and pelvic fusion in the first months of embryogenesis known as the exstrophy - epispadias complex. While epispadias is inherent in all cases of exstrophy it can also, much less frequently, appear in isolation as the least severe form of the complex spectrum. It occurs as a result of defective migration of the genital tubercle primordii to the cloacal membrane, and so malformation of the genital tubercle, at about the 5th week of gestation.^{[citation needed]}

Presentation

Most cases involve a small and bifid penis, which requires surgical closure soon after birth, often including a reconstruction of the urethra. Where it is part of a larger exstrophy, not only the urethra but also the bladder (bladder exstrophy) or the entire perineum (cloacal exstrophy) are open and exposed on birth, requiring closure.^{[citation needed]}

Relationship to other conditions

Despite the similarity of name, an epispadias is not a type of hypospadias, and involves a problem with a different set of embryologic processes.^{[citation needed]}

Women can also have this type of congenital malformation. Epispadias of the female may occur when the urethra develops too far anteriorly, exiting in the clitoris or even more forward. For females, this may not cause difficulty in urination but may cause problems with sexual satisfaction. Frequently, the clitoris is bifurcated at the site of urethral exit, and therefore clitoral sensation is less intense during sexual intercourse due to frequent stimulation during urination. However, with proper stimulation, using either manual or positional techniques, clitoral orgasm is definitely possible.^{[citation needed]}

Treatment

The main treatment for isolated epispadias is a comprehensive surgical repair of the genito-urinary area usually during the first 7 years of life, including reconstruction of the urethra, closure of the penile shaft and mobilisation of the corpora. The most popular and successful technique is known as the modified Cantwell-Ransley approach. In recent decades however increasing success has been achieved with the complete penile disassembly technique despite its association with greater and more serious risk of damage.^{[citation needed]}

Prognosis

Even with successful surgery, patients may have long-term problems with:^{[citation needed]}

incontinence, where serious usually treated with some form of continent urinary diversion such as the Mitrofanoff
depression and psycho-social complications
sexual dysfunction

References

UpToDate Contents

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1. 膀胱外反症を有する幼児の臨床症状および初期マネージメント clinical manifestations and initial management of infants with bladder exstrophy
2. 新生児の評価 assessment of the newborn infant
3. 小児における身体的診察：会陰 the pediatric physical examination the perineum
4. 膀胱外反症を有する小児の外科的マネージメントおよび術後経過 surgical management and postoperative outcome of children with bladder exstrophy
5. 新生児の包皮環状切除の手技 techniques for neonatal circumcision

English Journal

Treatment for failed epispadias repair presenting in adults.

Djordjevic ML, Bizic MR, Martins F, Kojovic V, Krstic Z.SourceSchool of Medicine, University of Belgrade, Belgrade, Serbia. Electronic address: djordjevic@uromiros.com.
The Journal of urology.J Urol.2013 Jul;190(1):165-70. doi: 10.1016/j.juro.2013.01.027. Epub 2013 Jan 16.
PURPOSE: We evaluated the results of 1-stage and multistage penile reconstruction in adults with complications after multiple failed epispadias repairs.MATERIALS AND METHODS: A total of 23 adults underwent penile disassembly for repeat epispadias repair from February 2006 to June 2011. Median age at
PMID 23333794

New insights into the pathogenesis of bladder exstrophy-epispadias complex.

Mahfuz I, Darling T, Wilkins S, White S, Cheng W.SourceMonash Institute of Medical Research, Faculty of Medicine, Nursing and Health Sciences, Monash University, Australia.
Journal of pediatric urology.J Pediatr Urol.2013 Jun 3. pii: S1477-5131(13)00111-3. doi: 10.1016/j.jpurol.2013.05.001. [Epub ahead of print]
Bladder exstrophy-epispadias complex (BEEC) is a complex and debilitating congenital disease. Familial and twin studies suggest a possible genetic component in BEEC pathogenesis. Bladder mesenchyme (detrusor) development requires induction by a signal from bladder urothelium, and we and others have
PMID 23743131

Lower urinary tract symptoms (LUTS) in patients in adulthood with bladder exstrophy and epispadias.

Taskinen S, Suominen JS.SourceDepartment of Paediatric Surgery, Children's Hospital, University of Helsinki, Helsinki, Finland.
BJU international.BJU Int.2013 Jun;111(7):1124-9. doi: 10.1111/j.1464-410X.2012.11756.x. Epub 2013 Jan 25.
WHAT'S KNOWN ON THE SUBJECT AND WHAT DOES THE STUDY ADD : Many patients with bladder exstrophy or epispadias (BEE) have bladder augmentation or use a catheterisation programme. Of the remainder only some achieve so called '3-h urinary continence'. In addition, some of the patients develop urinary t
PMID 23350915

Japanese Journal

三期に分けた stage operation により完治し得た尿道上裂の1例

平賀紀行,鯉川弥須宏,妹尾康平 [他],山口孝則,中村繁,原岡正志
西日本泌尿器科 71(12), 665-669, 2009-12-20
NAID 10026433424

Investigation of FGF10 as a candidate gene in patients with anorectal malformations and exstrophy of the cloaca

KRUGER Victoria,KHOSHVAGHTI Mercedeh,REUTTER Heiko,VOGT Hannes,BOEMERS Thomas M.,LUDWIG Michael
Pediatric surgery international 24(8), 893-897, 2008-08-01
NAID 10027863208

Bladder exstrophy and epispadias complex in sibling : case report and review of literature

KAJBAFZADEH Abdol-Mohammad,TAJIK Parvin,PAYABVASH Seyedmehdi,FARZAN Shima,SOLHPOUR Amir Reza
Pediatric surgery international 22(9), 767-770, 2006-09-01
NAID 10027481956