Academic literature on the topic 'Epispadia'

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.

Diphallia or penile duplication is an extremely rare congenital anomaly. It occurs once in every 5.5 million live births. The extent of penile duplication and the number of associated anomalies vary greatly, ranging from a double glans from a penis with no associated anomaly up to complete penile duplication associated with multiple anomalies. Here, we report a 12-year-old boy with complete bifid diphallia associated with bifid scrotum, epispadia, and pubic symphysis diastasis along with a review of the articles pertaining to this anomaly.

AbstractThe bladder exstrophy-epispadia complex (BEEC) includes malformations with midline closing defects of the lower abdomen and external genitalia. Long-term consequences with urinary incontinence and sexual dysfunction, in spite of multiple surgical interventions, are common and expected to affect the patient's health-related quality of life (HRQOL). The extent and the predictive factors are, however, not known. New patient-reported outcome research is emerging, but valid and reliable condition-specific HRQOL are still missing. The aim of this review is to summarize and discuss the latest published reports (2015–2019) on HRQOL in patients with the BEEC and its relationship to incontinence and sexual factors.

AbstractPatient K.L., 32 years old. Menses started at thirteen years old, C 5/28. Patient had two vaginal deliveries in 2004 and 2010. Dysplasia cervicis uteri CIN2-3 was diagnosed in 2007, subsequently diathermoconisation cervicis uteri was performed. The woman was born with epispadia — extrophia of urethra to abdominal wall, without pubic bone. At the age of 8 she underwent an operation in Moscow. During the operation the neck of the bladder was formed as well as urethra, which opens in vulva, place of clitoris. When she was 8 years old, her bladder capacity was 30 ml, in teenage years — 90 ml. The patient also reported history of recurrent urinary tract infections. 2011.08.02 Patient took medical advice in out patient department Kaunas university hospital with a complaint of stress urinary incontinence: when going, coughing, sneezing, doing exercises, having sex and at rest of time. Also it was the sexual intercourse problems with orgasm. She was urinating 8 times per day but none at night. Gynecological examination: absence of pubic bone, vulva is abnormal: absence of labium major and clitoris. Urethra opens into the place of clitoris. Front and back walls of vagina are moving down (POP-Q II-III stage prolapsed). Cervix of uterus is short, epithelised (after diathermoconisation). Uterus is normal in size, in retro-versio-flexio position. — without pathology. Sonography: internal genital organs without pathology. Boney, Valsalva test are positive, Ulmstein test negative. Urodynamic study revealed a bladder capacity of 134 ml, voided volume 173 ml. Pressure of detrusor — 10cmH2O. Compliancenormal, max flow rate 13,8 ml/s, voiding time 24 s. Spontaneous contractions of detrusor were not observed. Surgical treatment: 2011.10.10 TOT (tension obturator tape). Anterior and posterior colporrhaphy and perineoplastic was performed. There were no complications during and after surgery. On the 2 day after operation patient was released from hospital.

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 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.

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.