Academic literature on the topic 'Epispadias'

Purpose. To study the clinical picture, diagnosis and treatment of epispadias in girls. Material and methods. 22 girls with epispadias of various forms were under supervision for 50 years. During diagnostics the following issues were used: anamnesis, examination, catheterization and uroflowmetry, cystoscopy of the bladder, ultrasound and X-ray examination. Results and discussion. Out of 22 girls with epispadias, clitoral epispadia (partial ) was in 10 patients; sub-symphisal (subtotal) - in 4; symphisal (total) - in 8 girls. In 10 girls with the clitoral form, urological examination was made because of changes in the urine. Girls with sub-symphisal epispadia complained of irritation and itching in the vulva. All had vulvitis, cystitis, chronic pyelonephritis. In 2 patients, renal doubling was diagnosed; in other 2 patients ureterohydronephrosis and kidney dystopia were diagnosed. Uroflowmetry revealed hyperactive bladder in all. 8 girls with the total (symphisial) form of epispadia and urinary incontinence were operated by the Derzhavin technique; after the surgery the function of bladder sphincter was restored and the patients could have a normal quality of life. Conclusion. Epispadia in girls is a rare case . There are clitoral, sub-symphisal and symphisal (total) forms of epispadias. Cluster and sub-symphisial forms do not require surgical treatment. While in the symphisial (total) form, plastic surgery on the bladder neck by the Derzhavin technique is recommended. It is a good option for recovery.

Epispadias is a rare congenital anomaly in which the urethral opening is on the dorsal surface of penis. Its incidence is 1 in 117000 newborn males and 1 in 484000 in newborn females. Its etiology is considered as because of failure of medial migration of mesenchyme between the ectodermal and endodermal layers of cloacal membrane due to premature rupture of cloacal membrane. Epispadias often presents as exstrophy epispadias complex, a wide spectrum of abnormalities that consist of classic bladder exstrophy, Epispadias and cloacal exstrophy. This case was not a part of exstrophy epispadias complex and it was an isolated continent epispadias which is extremely rare and it was managed with Cantwell Ransley epispadias repair technique.

Introduction. Epispadias is an extremely rare disease. Epispadias in women is 5–6 times less common than in men. The incidence of female epispadias ranges from 1 in 160,000 to 480,000 live births. Epispadias can be diagnosed by a thorough examination of the genitals. Treatment of epispadias is only surgical, which is quite difficult and requires experience. The literature that discusses female epispadias is very limited. In this article, we would like to report on the surgical treatment of isolated epispadias in a 3-year-old girl at the Almazov National Medical Research Center.Clinical case. A 3-year-old girl presented with the main complaint of urinary incontinence since birth, during the day and at night. Urinary incontinence was not caused by physical activity and was not aggravated by eating/drinking. Physical examination showed that the external genital organs of the patient have underdeveloped labia minora, abnormal urethra, bifurcated clitoris. Laboratory results were within the normal range. Voiding cystourethrography revealed urine leakage during the filling phase. The bladder wall was normal, there was no vesicoureteral reflux. The patient underwent simultaneous surgical interventions, consisting in plastic surgery of the urethra and reconstruction of the bladder neck, plastic surgery of the clitoris and labia minora. There were no intraoperative and postoperative complications. After 1 week and 6 months of observation, the patient achieved urinary retention, the surgical wound healed primarily.Discussion. Epispadias is a rare condition that can vary in severity from mild to severe. Severely, splitting of the entire urethra occurs, involving the bladder neck, causing permanent urinary incontinence in the patient. Cases of epispadias are quite difficult to diagnose. The doctor should dilute the labia majora and carefully conduct a physical examination. The objective goals of the surgical treatment of epispadias are to achieve urinary retention, restore the anatomy, function and cosmetic appearance of the urethra and genitals. One-stage reconstruction is a modern surgical technique used to treat isolated epispadias in women.Conclusion. Epispadias in women is a rare congenital anomaly that is often not detected in childhood. For isolated epispadias in a girl, one-stage surgical correction is currently preferred.

Epispadias is part of the bladder exstrophy-epispadias complex; it has a defective dorsal wall of the urethra with potential incompetence of the urinary continence mechanism. Isolated male epispadias is due to failure of the urethral plate to tubularize on the dorsum of the penis. It is rare with an incidence rate of 1 in 117,000 live births in males. The severity depends on the position of the urethral opening and ranges from peno-pubic to penile and glandular. Epispadias is usually repaired within the first year of life though some patients have presented in their second decade. Epispadias is classically associated with bladder exstrophy in over 90% of the cases while isolated epispadias with continence is very rare constituting less than 10% of cases. Separation of pelvic bones is seen in 70% of peno-pubic epispadias affecting the bladder neck and external sphincter leading to incontinence and stress urinary dribbling. The goals of repair include; achieving a cosmetically acceptable and functional penis, which is straight and adequate in length, enabling penetrative sexual intercourse and urinary continence. There have been reports of isolated epispadias in adults but none was as old as 40 years. This patient has been married for 20 years and has been unable to impregnate his spouse. He had modified Cantwell-Ransley procedure after a detailed clinical and psychological evaluation to achieve the goals of repair. Adults presenting with isolated continent peno-pubic epispadias are rare. Presenting at this age and after having been married for two decades could have a derogatory effect on body image, self-confidence, psycho-sexual and reproductive life. Surgical correction of this anomaly as we did has the potential to lead to acceptable outcomes as demonstrated in this index case.

Epispadias as an isolated embryologic defect is a rare anomaly, with an incidence of 1 in 117.000 males. Isolated epispadias with continence is a very rare condition comprising less than 10% of all epispadias cases. Epispadias is a closure defect of the dorsal wall of the urethra. The biggest challenges for epispadias treatment are creating adequate penile length, straightening the severely curved penis, and creating a penopubic angle for allowing comfortable penetration of the penis. An 11-year-old boy underwent surgery for his continent penile epispadias in Dr. Soetomo General Academic Hospital. The surgical technique chosen for this patient was Modified Cantwell-Ransley repair. The dressing was opened on the 3rd day after the operation. The urethral catheter was removed on the 10th day. The patients underwent regular follow-up examinations for 6 months and were evaluated for subjective and objective outcomes. There was no post-operative fistula or urethral narrowing requiring urethral dilatation. The Uroflowmetry and post voiding residual ultrasound result was good. The cosmetic appearance of the penis was satisfying. Outcomes related to sexual activity were not evaluated because the patient had no sexual activity yet.

Epispadias with an intact prepuce (Concealed Epispadias) is an uncommon anomaly. The specific clinical signs in this situation are broad, spade-like glans with a dorsally directed preputial opening, gap between the corpora cavernosa may be palpable, dorsal chordee and abnormalities of the penile raphae. We report the case of a 3 year old male child with Concealed Epispadias who underwent a modified Cantwell-Ransley operation with good outcome. The relevant anatomy, embryology and surgical options are reviewed.

Introduction and purpose Bladder exstrophy (BE) is a rare malformation that involves not only the bladder, urethra and genital organs but also all of the pelvic floor and bones. Its prevalence is about 1/25.000-40.000 born in Italy and about 15 new cases are estimated each year. The disease affects males and females with a ratio of 2:1. The etiology is unknown. The effect of this malformation, affecting the urogenital complex, can be reflected on urinary continence and sexual life. These aspects can influence strongly the social life of patients. We evaluated both psychosexual and psychosocial aspects and sexual dysfunctional in adult with BE using a validated instrument. Materials and methods We conducted a survey using SESAMO (Sexuality Evaluation Schedule Assessment Monitoring). We evaluated a group of patients (cases) and a group of controls. All are invited to complete the male or female, single or partnership version of the instrument. The questionnaires are loaded into the program SESAME-Win and processed by the software SESAME-R. Both cases and the controls are analyzed in general and by sex and by emotional situation (single or partnership). We investigated the Areas found to be significant in the group of cases and not in the corresponding group of controls; in these Areas we analyzed individually every Items, highlighting the percentage of significant responses. We also assessed the state of continence about patients. Results This questionnaire is being distributed to 45 patients with BE, including 30 men (M) and 15 women (W) and 40 controls, 20 M and 20 W. 26 cases (17 M and 9 W), with a response rate of 57.7%, and 35 controls (18 M and 17 W) with a response rate of 87.5%, were finally included in analysis. With regard to the cases 16 (11 M and 5 W), completed the questionnaire as situation of “partnership” and 10 (6 M and 4 W) as "single". In group of controls 26 (14 M and 12 W) completed the questionnaire as situation of "partnership" and 9 (4 M and 5 W) as "single". Median age was 28 years in the Cases group and 30.5 in Controls. Environmental psychology and Lived Body Areas were significant in all groups of Cases (except in Females Total about Lived Body) and never in groups of controls. Desire Area is significant only in females Cases. Pleasure Areas are significant only in the groups of Controls and never in Cases. Remote Masturbation is significant, both in the group of Cases that Controls Females. Previous Experiential and the Sexual and Medical History are significant in all groups (except in the group of females controls about this last Area). Patients had a continence rate of 80, 7%. Conclusions Patients with bladder exstrophy have a tendency toward obsessive-compulsive disorder towards sexuality. They are dissatisfied about their free time, but they reached a satisfactory social position. They have a critical relationship with their parents and especially with mother, certainly more present in the clinical pathway. Especially with female patients, there is a lack of communication about sexuality. They present a difficulty to express and share their experiences, particularly sexuality, which they define like frustration experience. The body perception was very critical, more critical in single cases than in partnership. The areas of the body affected by the malformation are hated by themselves. Sexual dysfunction affects a high percentage of both sexes and the women seem to have more difficulty in having a satisfying sexual relationship. Sexuality appear a pathological experience in both single and partnership group. Singles are not satisfied with their emotional situation and even the frequency of sexual intercourse. Patients who live in a partnership use frequently masturbation not for his own pleasure but as act that substitute a complete sexual intercourse.
Introduzione e scopo dello studio L’Estrofia Vescicale-Epispadia (EV) è una rara patologia congenita caratterizzata da uno sviluppo incompleto della vescica e dell’uretra. La sua prevalenza varia da 1/25.000 a 1/40.000 nati ed in Italia si stima un'incidenza di circa 15 nuovi casi ogni anno. La patologia colpisce maschi e femmine con un rapporto di 2:1. L’etiologia non è nota. La patologia, colpendo l’apparato uro-genitale si riflette su elementi di primaria importanza, vale a dire la continenza urinaria e la vita sessuale. Tali aspetti possono condizionare fortemente la vita di relazione dei soggetti affetti. Scopo dello studio è stato quello di identificare sia gli aspetti psicosessuali e psicosociali che gli aspetti disfunzionali sessuali in soggetti affetti da EV. Materiali e metodi Abbiamo eseguito un’indagine strutturata mediante il questionario SESAMO (Sexuality Evaluation Schedule Assessment Monitoring - Boccadoro, Perillo 1996). Sono stati individuati due gruppi d’indagine: un gruppo di pazienti (casi) e uno di soggetti non portatori della patologia (controlli). Tutti i soggetti sono stati invitati a rispondere alla versione maschile o femminile, single o di coppia del questionario. I questionari sono stati caricati nel programma SESAMO-Win ed elaborati dal software SESAMO-R. Sia i Casi che i controlli sono stati analizzati sia in generale che divisi per sesso e situazione affettiva single/coppia. Sono state indagate le Aree risultate significative nel gruppo dei Casi e non nel corrispettivo gruppo dei Controlli; di tali Aree sono stati analizzati singolarmente i vari Item che le componevano, evidenziando la percentuale di risposte significative. Abbiamo inoltre valutato lo stato di continenza dei pazienti. Risultati Il questionario è stato consegnato a 45 pazienti affetti da EV di cui 30 maschi (M) e 15 femmine (F) e 40 controlli, di cui 20 M e 20 F. Hanno aderito allo studio 26 casi (17 M e 9 F), con un tasso di risposta di 57,7%, e 35 controlli (18 M e 17 F) con un tasso di risposta di 87,5%. In particolare per quanto riguarda i casi, 16 (61,5%) di cui 11 M e 5 F, hanno compilato il questionario come situazione affettiva di “coppia” e 10 (38,5%) di cui 6 M e 4 F come “single”; riguardo i controlli 26 (74,5%) (14 M e 12 F) hanno compilato il questionario come situazione affettiva di “coppia” e 9 (25,5) (4 M e 5 F) come “single”. L’età mediana è stata di 28 anni nel gruppo dei casi e 30,5 nei controlli. Le Aree Relati psicoambientali e Vissuto corporeo sono risultate significative in tutti i gruppi dei Casi (eccetto nei Casi Femmine totali per quanto riguarda il vissuto corporeo) e mai nei gruppi dei Controlli. L’Area Desiderio è significativa solo nei Casi Femmine. Gli Ambiti del piacere sono significativi solo nei gruppi dei Controlli e mai nei Casi. La Masturbazione remota è significativa sia nel gruppo dei Casi che dei Controlli Femmine. L’Esperienziale pregresso e l’ Anamnesi Medica e Sessuale sono significative in tutti i gruppi (eccetto nel gruppo dei Controlli Femmine per quest’ultima Area). I pazienti hanno presentato un tasso di continenza dell’80,7%. Conclusioni I pazienti affetti da estrofia vescicale presentano una spiccata tendenza all’ossessività che si manifesta sia nelle pratiche quotidiane che nei confronti della sessualità. Sono insoddisfatti del loro tempo libero ma circa la metà di essi ha raggiunto una condizione sociale soddisfacente. Presentano un rapporto critico con i propri genitori e in particolar modo con la madre, figura sicuramente più presente nel percorso clinico; specialmente nelle pazienti di sesso femminile si riscontra una carente comunicazione intrafamiliare riguardo alla sessualità. E’ emersa inoltre una difficoltà ad esprimere e raccontare le loro esperienze, in particolare quelle sessuali che definiscono frustranti. L’attenzione rivolta al “se’ corporeo” è risultata molto critica, più nei casi in situazione di coppia che nei single; le zone interessate dalla malattia e di conseguenza coinvolte negli interventi chirurgici sono le più odiate da essi. Le disfunzioni sessuali colpiscono in alta percentuale entrambi i sessi e nel complesso le donne sembrano avere più difficoltà ad avere un rapporto sessuale soddisfacente. La sessualità è vissuta in maniera patologica sia nel gruppo dei pazienti “single” che “coppie”. I pazienti single non sono soddisfatti della loro situazione affettiva e neppure della frequenza dei rapporti sessuali. I pazienti che vivono una situazione di coppia sono spinti ad attuare con maggiore frequenza la masturbazione non per proprio piacere ma come atto sostitutivo al rapporto sessuale completo.

This doctoral thesis explores how people with exstrophy-epispadias complex (EEC) conditions (including bladder exstrophy) experience intimate relationships. It includes a literature review, a research paper, a critical review, and an ethics section. The literature review takes a critical look at the current conceptualisation of sexuality for people with EEC through critique of the current EEC literature. As such, it argues that this current conceptualisation lacks sufficient consideration for psychological and social factors. Relevant findings from within related health psychology research are used to demonstrate the need for these factors to be considered. Furthermore, it is suggested that a new conceptualisation of sexual health should be adopted in order to incorporate these factors into both research and clinical practice. Finally, the implications of this potential change are discussed with consideration for how potential barriers may be overcome. The research paper leads on from this argument by adopting a qualitative approach to exploring an important psychological element of sexual health. Previous findings have suggested that people with bladder exstrophy and other EEC conditions have difficulties establishing and maintaining intimate relationships with friends or romantic partners. An interpretative phenomenological analysis was conducted with data from six participants with bladder exstrophy. Findings were described within three themes; 'exploring intimacy from a position of safety', 'the relationship between intimacy with the self and intimacy with others', and 'the magical value of intimacy: sharing the full story of bladder exstrophy'. Findings are discussed in relation to previous research and theory regarding concealment, body shame, and the development of identity, and clinical implications are presented. Finally, the critical review reflects on some of the key issues of relevance to carrying out this research. These include methodological issues related to analytical approach, homogeneity and data collection, and further reflection on the process of analysis.

Bladder exstrophy epispadias complex (BEEC) is a rare congenital malformation affecting both male and female with an incidence of 1/35,000 livebirths. The malformation involves the bladder, urethra, external genitalia and pelvis. The newborn presents with a lower abdominal defect, through which an open bladder template herniates. The malformation requires a staged surgical treatment but even after successful repair patients can experience psychosexual difficulties throughout their lives.This project investigates the presence or absence of a depressive disorder with sadness, emptiness, irritability, accompanied by somatic and cognitive changes which can significantly impact on the overall performance of the individual. The degree of alexithymia, a difficulty in verbalizing emotions, as well as overall quality of life of affected people is examined by means of several tests and specific interviews, created during the study. Aim of the study To evaluate the presence of alexithymia, depressive traits, difficulties in sexual assertiveness and/or in sex-lives and overall quality of life in people with BEEC compared with a group of unaffected peers. Materials and methods This case-control observational study included a group of BEEC cases compared with a group of control subjects unaffected by any urogenital malformation. Inclusion criteria for BEEC group were: proved diagnosis of BEEC; age> 16 years; sex M and F; compilation of informed consent and privacy forms. Inclusion criteria for control cases were: absence of any urogenital malformation; age> 16; sex M and F; compilation of informed consent and privacy forms. The following questionnaires were administered to fifty cases and fifty controls: the Beck Depression Interventory (BDI-II), the Toronto Alexithymia Scale (TAS-20), the Quality of Life, (SF-36), The Sexual Awareness Questionnaire (SAQ) and the Sexual History Form (SHF). The draft of a structured interview was administered to five cases. Data were analyzed by open source Rstudio and Kruskal-Wallis test. Results There were no differences between cases and controls as far as the presence of alexithymia and depressive traits are concerned. The QoL cases scored lower in physical activities and general health compared to controls; sex life experiences of the case group significantly differ from controls in terms of sexual monitoring (SAQ subscale). Women in the BEEC group had significantly lower scores in the Sexual History Form compared to controls. Conclusion Our findings demonstrate that the emotional burden suffered by the female affected population exceed that of the male population, therefore female would need an implementation of interventions to improve their sexual experiences. Further research on psycolosexual aspects and gender differences are needed to validate these data.

Anatomical and functional reconstruction of the bladder exstrophy/epispadias complex of anomalies presents paediatric urologists with one of their greatest challenges. Nevertheless, very considerable advances have been made during the last 30 years. Children with classic bladder exstrophy now have an excellent prospect of becoming fully continent—either by primary reconstruction or with a catheterizeable stoma. Achieving continence and normal voiding in children with primary epispadias can be difficult because of the dysplastic nature of their sphincteric and bladder neck tissues. Urachal anomalies may present symptomatically in childhood or incidentally during ultrasound examination of the lower urinary tract. Small bladder diverticula which are secondary to outflow obstruction or bladder dysfunction tend to resolve following treatment of the underlying cause, whereas surgical excision is usually required for larger, symptomatic diverticula.