Epispadias

A birth defect due to malformation of the URETHRA in which the urethral opening is above its normal location. In the male, the malformed urethra generally opens on the top or the side of the PENIS, but the urethra can also be open the entire length of the penis. In the female, the malformed urethral opening is often between the CLITORIS and the labia, or in the ABDOMEN.

Also Known As:

Networked: 144 relevant articles (6 outcomes, 5 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1.	Urinary Incontinence
2.	Bladder Exstrophy (Exstrophy, Bladder)
3.	Hypospadias
4.	Fistula
5.	Pathologic Constriction (Stenosis)

Experts

1.	Gearhart, John P: 9 articles (12/2014 - 03/2003)
2.	Reutter, Heiko: 8 articles (10/2014 - 09/2008)
3.	Gearhart, J P: 7 articles (04/2008 - 09/2000)
4.	Nöthen, Markus M: 4 articles (06/2014 - 07/2010)
5.	Draaken, Markus: 4 articles (06/2014 - 07/2010)
6.	Bartels, Enrika: 4 articles (06/2014 - 07/2010)
7.	Ebert, Anne-Karoline: 4 articles (06/2014 - 11/2011)
8.	Ludwig, Michael: 4 articles (06/2014 - 07/2010)
9.	Rösch, Wolfgang H: 4 articles (11/2011 - 09/2008)
10.	Kajbafzadeh, Abdol-Mohammad: 4 articles (11/2011 - 05/2007)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Epispadias:

1.	CosmeticsIBA 07/01/2008 - "This novel method of one-stage urogenital reconstruction in adult males with total epispadia and extrophy of the urinary bladder provided satisfactory cosmetic results in all the patients and functional results in 68% patients, improved quality of life, social and sexual adaptation in patients with the above serious conditions. " 11/01/2015 - "To describe a technique for a reliable and cosmetic skin cover for epispadias repair. " 09/01/2014 - "The modified penile disassembly technique can be used in epispadias and complete primary repair of exstrophy with excellent cosmetic results. " 07/01/2012 - "Three patients underwent epispadias repair, four patients had chordee correction with cosmetic excision of skin tags and seven patients underwent chordee correction with penile lengthening. " 11/01/2011 - "The anatomic correction of penile epispadias can recover the normal anatomic structure, resulting good cosmetic and functional results."
2.	baysilon (polydimethylsiloxane)IBA 10/01/2006 - "To present our experience with the use of injectable polydimethylsiloxane (Macroplastique, Uroplasty, Minneapolis, MI, USA) for treating incontinence in children with the exstrophy-epispadias complex (EEC), as incontinence continues to be a challenging problem in such children, and although the primary management of EEC has developed over the last few decades, with early closure and reconstruction of the penis, achieving satisfactory continence status remains elusive. " 10/01/2006 - "Injectable polydimethylsiloxane for treating incontinence in children with the exstrophy-epispadias complex: long-term results." 02/01/1998 - "To evaluate the efficacy of the endoscopic insertion of polydimethylsiloxane particles (Macroplastique, Uroplasty Inc) (MPQ) into the posterior urethral region of boys with urinary incontinence and primary epispadias. " 10/01/2006 - "Patients with epispadias are more likely to benefit from an injection with Macroplastique than those with bladder exstrophy. " 10/01/2006 - "We retrospectively reviewed the hospital records of 52 patients (41 boys and 11 girls, mean age at first injection 6.6 years, range 3.6-16.7) with EEC who had injections with Macroplastique between January 1991 and February 2004; 34 had bladder exstrophy and 18 primary epispadias. "
3.	Fibrin Tissue Adhesive (Fibrin Sealant)IBA 08/01/2011 - "Single-donor fibrin sealant for repair of urethrocutaneous fistulae following multiple hypospadias and epispadias repairs." 08/01/2011 - "To evaluate the efficacy of fibrin sealant for repair of urethrocutaneous fistula after multiple failed hypospadias and epispadias surgeries. " 08/01/2011 - "Single-donor fibrin glue could be a useful adjunct to surgical management of patients after multiple failed attempts at hypospadias or epispadias fistula repair. "
4.	pamidronate (APD)FDA LinkGeneric 07/01/1999 - "The APD was half the normal value in an incontinent patient with failed epispadias repair. " 07/01/1999 - "We believe that in patients with failed exstrophy and epispadias repair, APD seems to be a predictive criterion for continence, and results of the reconstructive surgery with osteotomy should be improved by distraction of the anterior segment of the pelvic ring."
5.	AntigensIBA 05/01/2012 - "This is the first study to our knowledge to characterize the urothelium from infants with bladder exstrophy-epispadias complex for the expression of urothelial differentiation associated antigens. "
6.	CollagenIBA 08/01/1996 - "Patients with adequate capacity and compliant neurogenic bladders, and those with the exstrophy-epispadias complex and failed bladder neck plasty seem to be optimal candidates for collagen injection." 04/01/1995 - "Five more children with exstrophy-epispadias complex (aged 1-3 years) underwent periurethral collagen injection to stimulate bladder enhancement and allow subsequent bladder neck reconstruction. " 08/01/1993 - "We injected endoscopically 0.75 to 2.5 cc of cross-linked bovine collagen at the 3, 9 and 12 o'clock positions of the urethra or bladder neck of 9 children with epispadias and 7 with exstrophy. " 03/01/1993 - "Female genito-urethroplasty and submucosal periurethral collagen injection as adjunctive procedures for continence in the exstrophy-epispadias complex. " 08/01/1996 - "In bladder exstrophy and to a lesser extent in epispadias the muscular tissue in the bladder wall may often be replaced by collagen. "
7.	Anorectal atresiaIBA 10/01/2011 - "The aim of the German Network for Congenital Uro-REctal Malformations is to collect data of affected patients with anorectal malformation (ARM) or extrophy-epispadias complex, and to investigate molecular causes, clinical implications, and psychosocial outcome. " 10/01/2013 - "Most common cause for VUR was Primary (60%), followed by posterior urethral valve (PUV) (19%), bladder exstrophy (5%), anorectal malformation (ARM), epispadias, and duplex system. " 10/01/2006 - "Patients with bladder exstrophy and many previous bladder procedures are more exposed to complications such as erosion compared with patients with epispadias or anorectal malformation. " 10/01/2006 - "Instead there were bladder exstrophy in 12 patients, anorectal malformation with a rectourethral or vesical fistula in 7 and epispadias in 4. A bladder neck cuff between 5.5 and 7.5 cm, and a 61-70 balloon were used in all patients. " 01/01/1990 - "Report of the observation of a female newborn presenting a complex malformative set: distal anorectal malformation without fistula; bladder exstrophy without epispadias, with cutaneous bridge; congenital urethrovaginal fistula; malformative sexual ambiguity with bulky genital tubercle, whole urethra, urethral meatus on the apex of the genital tubercle, associated with a mid labial genital joint and a high genital orifice; unilateral multicystic kidney; vertebral abnormalities with supernumerary thoracolumbar vertebrae; no karyotype abnormalities and no familial pathological history; no endocrine abnormalities. "
8.	Sacral agenesisIBA 08/01/1993 - "The diagnoses were epispadias/exstrophy complex (19 patients), myelodysplasia (11), cloacal exstrophy (6), posterior urethral valves (6), Hinman syndrome (4), sacral agenesis (3) and other (8). " 06/01/1997 - "The etiology of incontinence included myelodysplasia in 12 patients, exstrophy/epispadias in 4, and epidural abscess, sacral agenesis, imperforate anus and posterior urethral valves in 1 each. " 12/01/1984 - "The majority (79%) had neurologic dysfunction of the bladder because of spinal malformation (myelodysplasia, sacral agenesis, or trauma) and the remaining were a mixed group including exstrophy/epispadias, urethral valves, pelvic fractures, etc. The patients were grouped in six categories. " 11/01/2003 - "Seven patients had exstrophy-epispadias, seven spinal dysraphism, two sacral agenesis, and one each cerebral palsy, epispadias, imperforate anus and small bladder with vesico-ureteric reflux and congenital incontinence. " 08/01/1989 - "Incontinence resulted from myelodysplasia in 16 patients, sacral agenesis in 3, spinal cord injury in 6, posterior urethral valves in 1, bilateral ectopic ureters in 1 and epispadias-exstrophy in 3. Erosion occurred in 2 patients (7 per cent): 1 female patient who underwent simultaneous sphincter implantation and enterocystoplasty and who had undergone previously many bladder neck reconstructive procedures, including polytetrafluoroethylene (Teflon) injection, and 1 female patient in whom the augmented bladder was entered at artificial urinary sphincter implantation. "
9.	Penis agenesisIBA 09/01/2014 - "We retrospectively reviewed notes on the 16 patients with bladder/cloacal exstrophy and micropenis-epispadias complex treated with total phallic construction using the radial artery based forearm free flap between January 2003 and October 2013. " 09/01/2014 - "We report our experience with total phallic construction using the radial artery forearm free flap in patients with bladder/cloacal exstrophy and micropenis-epispadias complex. " 08/01/2006 - "Indications were small penis after failed epispadias repair (4), micropenis (3) and intersexuality (1). " 01/01/1997 - "simple penile curvature, chordee with and without hypospadias, epispadias, micropenis, cripple penis, etc. The dose injected intracavernosally was dependent on the patient's age, penile size and response, and ranged from 0.5 to 15 micrograms. " 08/01/1995 - "Specific indications were female transsexualism in 4 patients, penile amputation in 2 and a small disabled penis in 18 (the exstrophy-epispadias complex, intersex and micropenis). "
10.	Testosterone (Sustanon)FDA Link 05/01/2001 - "Epispadias repair can usually be performed at the same time and is facilitated by prior testosterone administration. " 06/01/2001 - "The patient in whom the pubis was separated and the urethra was lost underwent reclosure, epispadias repair and combined osteotomy under testosterone stimulation. " 06/01/2001 - "The patient with minor prolapse was treated with reclosure with bilateral innominate and vertical iliac osteotomy, and reclosure with epispadias repair under testosterone stimulation. " 10/01/1976 - "The testosterone plasma level was determined in 5 groups: 1. in 69 normal juveniles and 85 fertile males at the age of 11 to 45 years, 2. in 42 patients with hypospadia or epispadia aged 11 to 25 years, 3. in 72 males with unilateral cryptorchidism at the age of 11 to 45 years, 4. in 83 males with bilateral cryptorchidism aged 11 to 45 years and 5. in 106 patients with Klinefelter's syndrome at the age of 16 to 45 years. "

Therapies and Procedures

1.	Osteotomy 08/01/2015 - "The present study is a retrospective review of patients that underwent pelvic osteotomies with spica casting at the time of CPRE or proximal epispadias repair from November 2006 to March 2013. " 10/01/2015 - "Surgical repair stages include initial closure of the bladder and abdominal wall with or without osteotomy, followed by epispadias repair at 6 to 12 months, and bladder neck repair around 5 years of life. " 12/01/2014 - "A prospectively maintained database of 1208 exstrophy-epispadias complex patients was reviewed for CE patients treated with pelvic osteotomies. " 10/01/2014 - "From April 2000 to March 2014, four patients aged 16 days to 7 years and 5 months underwent complex exstrophy-epispadias repair with pelvic osteotomies. " 10/01/2010 - "Exstrophy closure combined with epispadias repair can be followed by ischemic penile injury, particularly when osteotomy is not performed. "
2.	Urinary Diversion (Ileal Conduit) 11/01/1992 - "This series supports the efficacy of CUR as an alternative procedure to traditional forms of urinary diversion in the management of selected patients with exstrophy/epispadias complex." 05/01/2011 - "Continent urinary diversion in the epispadias-exstrophy complex." 09/01/2008 - "Of the 21 patients, 17 had undergone single-stage reconstruction, 1 had undergone a staged approach, 2 had primarily undergone urinary diversion, and 1 had only undergone external genital reconstruction of epispadias. " 03/01/2003 - "Continent urinary diversion and the exstrophy-epispadias complex." 12/01/2000 - "Urinary diversion in children with mainly exstrophy and epispadias: alternative to primary bladder closure."
3.	Transplantation (Transplant Recipients) 01/01/1924 - "Case of Transplantation of the Rectus for Incontinence due to Epispadias." 12/01/1895 - "I. Operative Treatment of Epispadias by Transplantation of the Urethra." 11/01/2011 - "The 4-year outcomes demonstrate that autologous myoblast transplantation for urinary incontinence in children with bladder exstrophy-epispadias complex is relatively reliable, reproducible, safe and effective with minimal morbidity. " 11/01/2011 - "Children with bladder exstrophy-epispadias complex undergoing endourethral autologous myoblast transplantation to treat urinary incontinence were evaluated at 4 years of followup regarding the safety, efficacy and durability of the procedure, and health related quality of life. " 11/01/2011 - "Safety, efficacy and health related quality of life of autologous myoblast transplantation for treatment of urinary incontinence in children with bladder exstrophy-epispadias complex."
4.	Transplants (Transplant) 10/01/2005 - "A urethral buccal graft was used in 1 patient, FTSG was used in 2 at a later operation and all 3 underwent C-R epispadias repair. " 10/01/2005 - "Urethral replacement was performed with full thickness skin graft (FTSG) in 3 and with buccal mucosa in 3. Five patients underwent a modified Cantwell-Ransley (C-R) epispadias repair after placement of skin expanders, and 1 awaits repair. " 01/01/1977 - "Use of paraexstrophy skin pedicle grafts for correction of exstrophy and epispadias repair." 06/01/2001 - "The 2 patients with complete dehiscence underwent reclosure with bilateral innominate and vertical iliac osteotomy with replacement of the urethral plate with a skin graft and await epispadias repair. " 08/01/2007 - "Hypospadias/epispadias recipient site success was significantly associated with the type of graft used (tube vs onlay, p <0.001), and by the site of oral mucosa harvest (labial vs buccal, p <0.001). "
5.	Artificial Urinary Sphincter 05/01/1995 - "The Tanagho bladder neck reconstruction is a useful addition to the procedures that may be used by the reconstructive urological surgeon in the treatment of carefully selected patients with complex incontinence, particularly in women with epispadias who for various reasons may wish to avoid the long-term potential complications of an artificial urinary sphincter." 01/01/1986 - "The patient with epispadias later had an artificial urinary sphincter because of persisting incontinence. " 04/01/1983 - "The AS792 artificial urinary sphincter was implanted in 11 incontinent patients with the epispadias-exstrophy complex. " 04/01/1983 - "Treatment of the epispadias-exstrophy complex with the AS792 artificial urinary sphincter." 08/01/1989 - "Incontinence resulted from myelodysplasia in 16 patients, sacral agenesis in 3, spinal cord injury in 6, posterior urethral valves in 1, bilateral ectopic ureters in 1 and epispadias-exstrophy in 3. Erosion occurred in 2 patients (7 per cent): 1 female patient who underwent simultaneous sphincter implantation and enterocystoplasty and who had undergone previously many bladder neck reconstructive procedures, including polytetrafluoroethylene (Teflon) injection, and 1 female patient in whom the augmented bladder was entered at artificial urinary sphincter implantation. "