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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...
September 15, 2016: Journal of Pediatric Urology
Douglas A Canning
No abstract text is available yet for this article.
November 2016: Journal of Urology
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
Pokket Sirisreetreerux, Kathy M Lue, Thammasin Ingviya, Daniel A Friedlander, Heather N Di Carlo, Paul D Sponseller, John P Gearhart
PURPOSE: A successful primary bladder exstrophy closure provides the best opportunity for patients to achieve a functional closure and urinary continence regardless of the method of repair. Use of osteotomy during initial closure has significantly improved success rates, however failures can still occur. This study aimed to identify factors that contribute to a failed primary exstrophy closure with osteotomy. MATERIALS AND METHODS: A prospectively-maintained institutional database was reviewed for classic bladder exstrophy patients who were primarily closed with osteotomy at our institution or referred after primary closure from 1990 to 2015...
October 5, 2016: Journal of Urology
Alice Faure, Rebecca Cooksey, Aurore Bouty, Alan Woodward, John Hutson, Mike O'Brien, Yves Heloury
BACKGROUND: Effective bladder emptying by clean intermittent catheterization for children with severe bladder dysfunction is critical for renal preservation and social integration. Use of a continent catheterizable conduit (CCC) as urethral alternative procedure provides effective bladder drainage. However, it brings a substantive maintenance. METHODS: Retrospective review of the indications and long-term outcomes of 54 patients with a Mitrofanoff procedure in a single center over a 20-year period (1995-2015)...
September 23, 2016: Journal of Pediatric Surgery
Joan S Ko, Angela D Gupta, Heather N Di Carlo, Kathy Lue, John P Gearhart
Bladder exstrophy and cloacal exstrophy are rare congenital defects of the genitourinary tract that require complex surgical reconstruction. Malrotation of the bony pelvis causes a characteristic diastasis of the pubic symphysis, which is surgically reduced at the time of initial bladder closure. For a successful primary closure without tension such that the bladder can be placed deep within the pelvis, pelvic osteotomy is often used. However, alternative techniques have been utilized to bring the pubic rami into apposition...
October 2016: Canadian Journal of Urology
Sajni I Khemchandani
INTRODUCTION: Classic bladder exstrophy (BE) is a rare malformation of the genito-urinary tract affecting 1:50,000 to 1:100,000 live births. The surgical reconstruction of the BE-epispadias complex is challenging for the most experienced pediatric urologists, surgeons, and orthopedists. PURPOSE: To assess the success of staged reconstruction of the BE and long-term effects on the upper urinary tract, renal function, and continence. MATERIALS AND METHODS: This is retrospective study; between 1994 and 2013, 30 patients with BE have undergone stage 1 repair at the institute...
October 2016: Journal of Indian Association of Pediatric Surgeons
K Suzuki, D Matsumaru, S Matsushita, A Murashima, M Ludwig, H Reutter, G Yamada
The abnormalities in the urogenital organs are frequently observed as human developmental diseases. Among such diseases, the defects in the upper part of external genitalia are rather rare named epispadias. The cleft in the dorsal part of external genitalia often reaches to the urethra. In general, the urogenital abnormalities accompany defects in the adjacent tissues and organs. The ventral body wall and bladder can also be affected in the patients with dorsal defects of the external genitalia. Therefore, such multiple malformations are often classified as bladder exstrophy and epispadias complex (BEEC)...
September 20, 2016: Clinical Genetics
Luciano Alves Favorito, Ulisses Sobrinho, Rodrigo Galves Martins, Juliana Bezerra Bastos
No abstract text is available yet for this article.
September 19, 2016: International Urogynecology Journal
Usha D Nagaraj, Karin S Bierbrauer, Jose L Peiro, Beth M Kline-Fath
OBJECTIVE: The purpose of this study is to identify differences in findings between open and closed spinal dysraphisms seen on fetal MR images. MATERIALS AND METHODS: A single-institution retrospective analysis of fetal MR images for spinal dysraphism was performed. Postnatal images and clinical and operative reports were reviewed. RESULTS: Sixteen fetuses with postnatally confirmed closed spinal dysraphisms were included. Of these, 25% (4/16) had posterior fossa anomalies, 12...
September 9, 2016: AJR. American Journal of Roentgenology
Douglas A Canning
No abstract text is available yet for this article.
September 2016: Journal of Urology
P Mishra, S Rajendran, I Mushtaq
No abstract text is available yet for this article.
August 2016: Journal of Pediatric Urology
R Maximilian Cervellione
No abstract text is available yet for this article.
August 2016: Journal of Pediatric Urology
R Ragu, G Meurette, M Kim, L Le Normand, P A Lehur
Bladder exstrophy is a rare malformation. Ureteral diversion, such as ureterosigmoidostomy or a neorectal bladder, has been described. When the patients reach adulthood, cancer may arise in these reconstructions. Our aim was to perform a systematic review (all languages) of the published literature on neoplasia after urinary diversion and suggested management in cases of cancer. PubMed and Cochrane library were searched for relevant articles published within the last 20 years. All identified articles were reviewed for inclusion...
September 3, 2016: Techniques in Coloproctology
Carlo Pavone, Marco Vella, Dario Fontana, Cristina Scalici Gesolfo, Sebastiano Oieni, Francesca Toia, Adriana Cordova
We report a case of a woman affected by covered exstrophy, uterus didelphys and external genital malformation presenting with advanced bladder cancer. After neoadjuvant therapy and anterior pelvic exenteration, the abdominal wall was reconstructed with a pedicled myocutaneous muscle-sparing vastus lateralis flap.
2016: Case Reports in Plastic Surgery & Hand Surgery
Jose Arnaldo Shiomi da Cruz, Bruno de Mattos, Miguel Srougi, Hiep Nguyen, Rafael Bonan, Francisco Denes, Amilcar Giron, Carlo Passerotti
INTRODUCTION: Bladder exstrophy (BE) is a rare condition that requires complex surgical corrections to achieve the goals of bladder functionality, normal sexual function, continence, and finally cosmesis. The purpose of this study was to identify clinical parameters that predict better quality of life (QOL) scores using a validated questionnaire (SF-36) with young adults after completing surgical reconstruction. MATERIAL AND METHODS: Forty-three young adults (mean age 22...
2016: Central European Journal of Urology
Kristina D Suson
In recent years, there has been increasing interest in transitional urology, or how to best prepare patients with major congenital urologic diseases, such as bladder exstrophy and neuropathic bladder, to manage their own health care with adult urologists. However, common pediatric urologic conditions may be encountered by the adult urologist with more regularity. This review focuses on three relatively common conditions which may be identified in childhood, the consequences from which a patient may seek help from an adult urologist: cryptorchidism, varicocele, and Klinefelter syndrome...
October 2016: Current Urology Reports
G Reinfeldt Engberg, Ä Mantel, M Fossum, A Nordenskjöld
INTRODUCTION: Bladder exstrophy is a rare, congenital, complex malformation where the underlying cause is largely unknown. Both environmental and genetic mechanisms are thought to be involved. There are divergent results concerning the prevalence, birth descriptive data, and potential maternal risk factors for bladder exstrophy. Few previous studies have reflected nationwide populations, population registers, or spanned a longer period of time. OBJECTIVE: To describe and assess bladder exstrophy and the potential maternal risk factors, for a time period of four decades, by conducting a nationwide register study of bladder exstrophy in Sweden...
July 15, 2016: Journal of Pediatric Urology
Sarah Giutronich, Aurélien Scalabre, Thomas Blanc, Peter Borzi, Yves Aigrain, Mike O'Brien, Pierre D E Mouriquand, Yves Heloury
OBJECTIVE: Bladder perforation is not commonly described in bladder exstrophy patients without bladder augmentation. The goal of this study was to identify the risk factors of spontaneous perforation in non-augmented exstrophy bladders. METHODS: The study was a retrospective multi-institutional review of bladder perforation in seven male and two female patients with classic bladder exstrophy-epispadias (E-E). RESULTS: Correction of E-E was performed using Kelly repair in two and staged repair in seven (Table)...
July 16, 2016: Journal of Pediatric Urology
Yuval Bar-Yosef, Mario Sofer, Margaret P Ekstein, Yosef Binyamini, Jacob Ben-Chaim
OBJECTIVES: To evaluate our results of epispadias repair with a modified Cantwell-Ransley (MCR) technique. METHODS: A retrospective IRB-approved chart review of all patients who underwent an MCR epispadias repair was conducted between 1998-2015. Procedures were performed at birth or after the age of 8 months as part of the modern staged repair (MSR) of exstrophy-epispadias complex (EEC) in patients with bladder exstrophy treated since birth, at presentation for older patients, and after the age of 6 months in isolated epispadias patients...
July 20, 2016: Urology
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