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Epispadias

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https://www.readbyqxmd.com/read/28615813/modified-cantwell-ransley-repair-for-isolated-continent-epispadias-in-adult-our-experience
#1
Venkat Arjunrao Gite, Hitesh Mahendra Jain, Sachin M Bote, Jayant Vijay Nikose
PURPOSE: We evaluate here our experience with modified Cantwell-Ransley technique described by Gearhart for correction of isolated continent epispadias in adults with respect to its long-term functional outcome and complications. INTRODUCTION: Isolated male epispadias is a rare anomaly with a reported incidence of 1 in 11,700 males. Many surgical techniques with various modifications described to correct epispadias as treatment are debatable and challenging. The majority of the cases are treated at childhood as clinical presentation is striking at birth; hence, presentation in adulthood is extremely rare...
January 2017: Indian Journal of Plastic Surgery: Official Publication of the Association of Plastic Surgeons of India
https://www.readbyqxmd.com/read/28534165/-the-german-research-network-cure-net-a-benefit-for-patients-with-rare-diseases
#2
REVIEW
A-K Ebert
With the help of the media, there is growing public awareness for the problems associated with rare diseases and their impact on the lives of those affected and their families. Bladder exstrophy-epispadias complex (BEEC) is also a part of the group of rare diseases within the urological field. The German network CURE-Net was founded in 2009 to systematically collect data regarding the epidemiological and molecular causes, and clinical and psychosocial effects of congenital urorectal malformations. With the help of self-help groups a national registry could be established for systematic data retrieval...
May 22, 2017: Der Urologe. Ausg. A
https://www.readbyqxmd.com/read/28505946/re-clinical-pathway-for-early-discharge-after-complete-primary-repair-of-exstrophy-and-epispadias-by-using-a-spica-cast
#3
Douglas A Canning
No abstract text is available yet for this article.
June 2017: Journal of Urology
https://www.readbyqxmd.com/read/28401037/female-epispadias-presenting-as-urinary-incontinence
#4
Asmir Jonuzi, Nusret Popovic, Zlatan Zvizdic, Emir Milišic, Kenan Karavdic, Dewan Paddy
Isolated female epispadias without bladder exstrophy is a rare congenital anomaly affecting 1 in 484,000 females. The presenting features of female epispadias are urinary incontinence and abnormal anatomical features. A 6-year-old girl presented with primary urinary incontinence who on physical examination had a bifid clitoris and labia minora. The vagina and hymen were normal. Voiding cystourethrogram showed no reflux and a funnel shaped proximal urethra. With the diagnosis of isolated female epispadias, one-stage reconstruction of the urethra, bladder neck, labia minora and clitoris was performed...
March 2017: APSP Journal of Case Reports
https://www.readbyqxmd.com/read/28392008/long-term-follow-up-after-traditional-versus-modified-perineal-approach-in-the-management-of-female-epispadias
#5
F Alyami, N Fernandez, L Lee, P Metcalfe, A Lorenzo, J Pippi Salle
OBJECTIVE: Isolated female epispadias (IFE) is a rare congenital anomaly. The defect extends to the bladder neck, which is usually incompetent. The traditional surgical approach includes urethral and genital reconstruction in the first year, followed by bladder neck reconstruction (Young-Dees-Leadbetter cervicoplasty (YDL)) at the age of social continence. An alternative single-stage technique includes urethral, bladder neck and clitoris repair by a perineal approach. The aim of the present study was to describe long-term follow-up of patients who underwent the traditional vs alternative approach...
March 14, 2017: Journal of Pediatric Urology
https://www.readbyqxmd.com/read/28371167/long-term-sexual-health-outcomes-in-men-with-classic-bladder-exstrophy
#6
Timothy S Baumgartner, Kathy M Lue, Pokket Sirisreetreerux, Sarita Metzger, Ross G Everett, Sunil S Reddy, Ezekiel Young, Uzoma A Anele, Cameron E Alexander, Nilay M Gandhi, Heather N Di Carlo, John P Gearhart
OBJECTIVES: To identify the long-term sexual health outcomes and relationships in men born with classic bladder exstrophy (CBE). MATERIALS AND METHODS: A prospectively-maintained institutional database comprised of 1248 patients with exstrophy-epispadias was utilized. Male patients 18 years or older with CBE were included. A 42-question survey was designed utilizing a combination of demographic information and previously validated questionnaires. RESULTS: A total of 215 men inclusion criteria, of which 113 (53%) completed the questionnaire...
March 31, 2017: BJU International
https://www.readbyqxmd.com/read/28314702/short-term-outcomes-of-the-multi-institutional-bladder-exstrophy-consortium-successes-and-complications-in-the-first-two-years-of-collaboration
#7
J G Borer, E Vasquez, D A Canning, J V Kryger, A Bellows, D Weiss, T Groth, A Shukla, M P Kurtz, M E Mitchell
INTRODUCTION/BACKGROUND: Bladder exstrophy is a rare diagnosis that presents major reconstructive challenges. To increase experience and proficiency in the care of bladder exstrophy (BE), the Multi-Institutional BE Consortium (MIBEC) was formed, with a focus on refining technical aspects of complete primary repair of bladder exstrophy (CPRE) and subsequent care. OBJECTIVE: Outcome measures included successful CPRE (absence of dehiscence), complications, and integrated points of technique and care over the short-term...
February 20, 2017: Journal of Pediatric Urology
https://www.readbyqxmd.com/read/28277470/patient-reported-impact-of-pelvic-organ-prolapse-on-continence-and-sexual-function-in-women-with-exstrophy-epispadias-complex
#8
Ross G Everett, Kathy M Lue, Sunil S Reddy, Daniel A Friedlander, Cameron E Alexander, Ezekiel E Young, Mahmoud Abdelwahab, Nilay M Gandhi, Edward J Wright, John P Gearhart
OBJECTIVE: This study aimed to characterize long-term urogynecologic issues of women with a history of bladder exstrophy and pelvic organ prolapse (POP) and to assess the impact of POP repair on continence and sexual function. DESIGN: Patient demographics and surgical history related to exstrophy and POP were collected through chart review. Patient perceptions regarding sexual function, urinary continence, and quality of life were assessed through Web-based administration of validated questionnaires: International Consultation on Incontinence Questionnaire-Urinary Incontinence Short Form and POP-Urinary Incontinence Sexual Questionnaire...
March 9, 2017: Female Pelvic Medicine & Reconstructive Surgery
https://www.readbyqxmd.com/read/28254239/psychosexual-development-management-of-bladder-exstrophy-epispadias-in-complex-patients
#9
M Di Grazia, S Pellizzoni, L G Tonegatti, W Rigamonti
INTRODUCTION: Bladder-exstrophy-epispadias complex (BEEC) represents a spectrum of urogenital step-wise malformations: epispadias, complete exstrophy, and cloacal exstrophy. Psychosexual development in adolescent patients with BEEC may become especially problematic. At present, there are few contributions in the literature investigating the validity of psychosexual treatment in order to tackle this particularly emotional and personal development phase. OBJECTIVE: The study aimed at verifying the efficacy of an intervention methodology for psychosexual support of a group of adolescents with BEEC...
June 9, 2016: Journal of Pediatric Urology
https://www.readbyqxmd.com/read/28202184/high-prevalence-of-same-sex-twins-in-patients-with-cloacal-exstrophy-support-for-embryological-association-with-monozygotic-twinning
#10
Brenna S Fullerton, Eric A Sparks, Amber M Hall, Cristine S Velazco, Biren P Modi, Dennis P Lund, Tom Jaksic, W Hardy Hendren
PURPOSE: Previous studies have hypothesized that cloacal exstrophy may be caused by errors early in embryological development related to monozygotic twinning. This study reports the prevalence of twins in a large cohort of patients with cloacal exstrophy. METHODS: Patients with cloacal exstrophy treated 1974-2015 were reviewed for reports of multiple gestation or conjoined twinning. The genetic sex of the patient and their twin, and any mention of anomaly in the twin were recorded...
May 2017: Journal of Pediatric Surgery
https://www.readbyqxmd.com/read/28088311/prevalence-of-preoperative-penile-abnormalities-among-voluntary-male-medical-circumcision-patients-in-swaziland
#11
Anthony R Oddo, Elizabeth Ruedrich, Christopher Zust, Lindsey Marugg, Echo VanderWal, Harry VanderWal, Rebekah Sartori, Ronald Markert, Mary C McCarthy
BACKGROUND: Circumcision has been found to be an effective strategy for lowering the transmission of HIV in Africa. The Luke Commission, a mobile hospital outreach program, has used this information to decrease the rate of HIV in Swaziland by performing voluntary male medical circumcisions throughout the country. During many of these circumcisions, genital medical conditions and penile abnormalities are simultaneously discovered and corrected. PURPOSE: The goal of our study was to evaluate the prevalence of penile abnormalities discovered and treated during voluntary male medical circumcisions performed by The Luke Commission (TLC) throughout rural Swaziland...
January 3, 2017: Journal of Pediatric Surgery
https://www.readbyqxmd.com/read/28039050/low-dose-human-chorionic-gonadotropin-stimulation-test-as-a-prognostic-incontinent-indicator-in-boys-with-bladder-exstrophy-epispadias-complex
#12
Nastaran Sabetkish, Sahar Eftekharzadeh, Azadeh Elmi, Saman Shafaat Talab, Abdol-Mohammad Kajbafzadeh
OBJECTIVE: To investigate the effect of low-dose human chorionic gonadotropin (HCG) administration on structural changes in the lower urinary tract in boys with urinary incontinence in the setting of bladder exstrophy-epispadias complex (BEEC). PATIENTS AND METHODS: We prospectively evaluated 30 patients (median age 7.5 years) with BEEC and randomly divided them into 2 groups. Patients in the HCG group were administered 250 IU HCG intramuscularly 3 times per week during a 4-week period...
December 27, 2016: Urology
https://www.readbyqxmd.com/read/27989536/complications-of-bladder-closure-in-cloacal-exstrophy-do-osteotomy-and-reoperative-closure-factor-in
#13
Daniel A Friedlander, Heather N Di Carlo, Paul D Sponseller, John P Gearhart
BACKGROUND/PURPOSE: The aims of surgical management in cloacal exstrophy (CE) have shifted to optimizing outcomes and quality of life while minimizing morbidity. This report reviews the single-institution experience of complications of bladder closure in CE. METHODS: Patients with CE were identified from a prospectively-maintained bladder exstrophy-epispadias complex database. Operative and follow-up data were analyzed to compare complications and failure rates of bladder closure between closures performed with and without osteotomy and primary versus reoperative closures...
December 9, 2016: Journal of Pediatric Surgery
https://www.readbyqxmd.com/read/27989535/history-of-hypospadias-lost-in-translation
#14
Ahmed T Hadidi
The evolution of hypospadias surgery can be classified under 3 periods that were closely related to advances in surgical instruments, introduction of anesthesia, and newer suture materials. Stretching dominated the early period, tunneling during the Middle Ages, and flaps during the modern period. Suture materials have included at various time silver wires, horsehair, and stainless steel. Examination and translation of the original manuscripts showed that Galen recommended stretching and suturing of glanular hypospadias and not amputation or partial penectomy as has been currently reported...
February 2017: Journal of Pediatric Surgery
https://www.readbyqxmd.com/read/27941186/mixed-gonadal-dysgenesis-with-an-unusual-inverted-y-chromosome
#15
Deepa Makhija, Hemanshi Shah, Charu Tiwari, Shalika Jayaswal, Jayesh Desale
Mixed gonadal dysgenesis is a rare disorder of sex development associated with sex chromosome aneuploidy and mosaicism of the Y chromosome. It is characterized by a unilateral non-palpable (usually intra-abdominal) testis, a contralateral streak gonad and persistent mullerian structures. The clinical presentation can vary from a typical male to female phenotype including all degrees of cryptorchidism, labial fusion, clitoromegaly, epispadias and hypospadias. It is the second most common cause of ambiguous genitalia in the neonatal period...
2016: Developmental Period Medicine
https://www.readbyqxmd.com/read/27896576/comparing-the-bulking-effect-of-calcium-hydroxyapatite-and-deflux-injection-into-the-bladder-neck-for-improvement-of-urinary-incontinence-in-bladder-exstrophy-epispadias-complex
#16
Sahar Eftekharzadeh, Nastaran Sabetkish, Shabnam Sabetkish, Abdol-Mohammad Kajbafzadeh
OBJECTIVES: The aim of this study was to evaluate the efficacy of the endoscopic injection of calcium hydroxyapatite (CaHA) into the bladder neck (BN) region of patients with urinary incontinence and bladder exstrophy-epispadias complex (BEEC). PATIENTS AND METHODS: We designed a retrospective cohort study in which we retrospectively studied medical charts of female and male patients of BEEC who had undergone CaHA or Deflux injection for continence improvement between 2009 and 2014...
November 28, 2016: International Urology and Nephrology
https://www.readbyqxmd.com/read/27751835/qualitative-questionnaire-on-the-psychosocial-wellbeing-of-mothers-of%C3%A2-children-with-beec
#17
Massimo Di Grazia, Sandra Pellizzoni, Luca Giacomo Tonegatti, Waifro Rigamonti
INTRODUCTION: The bladder exstrophy-epispadias complex (BEEC) represents a spectrum of malformations that affect the anatomical and functional structure of the urogenital system. The parents of patients affected by this condition are subject to particularly stressful situations, such as worrying about their child's health, long hospital stays, concerns about the health and constant need for personal care for their children, that can profoundly compromise the quality of family life. OBJECTIVE: The objective of this explorative qualitative study is to evaluate the social situation and the psychological strategies implemented by the mothers of children between 6 and 10 years of age who are affected by BEEC...
February 2017: Journal of Pediatric Urology
https://www.readbyqxmd.com/read/27751834/concealed-epispadias-associated-with-a-buried-penis
#18
REVIEW
Ricardo Sol Melgar, Daniela Gorduza, Delphine Demède, Pierre Mouriquand
OBJECTIVE: The aim was to describe the clinical presentation and the surgical management of penile epispadias associated with a buried penis in five children. PATIENTS AND METHODS: This is a 5-year retrospective review of patients presenting with a buried penis, a congenital defect of the penile skin shaft associated with an unretractable foreskin for whom a penile epispadias was found at the time of surgery. All had undergone surgery combining a Cantwell-Ransley procedure and refashioning of the penile skin following the authors' technique...
December 2016: Journal of Pediatric Urology
https://www.readbyqxmd.com/read/27751490/re-the-tunica-vaginalis-flap-as-an-adjunct-to-epispadias-repair-a-preliminary-report
#19
Douglas A Canning
No abstract text is available yet for this article.
November 2016: Journal of Urology
https://www.readbyqxmd.com/read/27722172/isolated-male-epispadias-anatomic-functional-restoration-is-the-primary-goal
#20
Anne-Francoise Spinoit, Tom Claeys, Elke Bruneel, Achilles Ploumidis, Erik Van Laecke, Piet Hoebeke
Background. Isolated male epispadias (IME) is a rare congenital penile malformation, as often part of bladder-exstrophy-epispadias complex (BEEC). In its isolated presentation, it consists in a defect of the dorsal aspect of the penis, leaving the urethral plate open. Occurrence of urinary incontinence is related to the degree of dorsal displacement of the meatus and the underlying underdevelopment of the urethral sphincter. The technique for primary IME reconstruction, based on anatomic restoration of the urethra and bladder neck, is here illustrated...
2016: BioMed Research International
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