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Bladder exstrophy

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https://www.readbyqxmd.com/read/28102169/hip-joint-arthroplasty-in-pubic-symphysis-separation-in-the-course-of-congenital-bladder-exstrophy-study-of-two-cases
#1
Marek Drobniewski, Andrzej Borowski, Magdalena Krasmska, Marcin Sibmski, Marek Synder
Congenital bladder exstrophy is a severe congenital malformation identified in one out of 30,000-50,000 live newborns and seen more often in girls. Some patients develop osteoarthritis of the hip joints which requires total hip arthroplasty. Reports of such cases are very rare in the world literature, which prompted us to present two cases treated at our Department and analyse their early outcomes. Our experience so far has been rather modest but the results encourage considering total hip arthroplasty in the treatment of hip osteoarthritis in future patients with pubic symphysis separation in the course of congenital bladder exstrophy...
August 30, 2016: Ortopedia, Traumatologia, Rehabilitacja
https://www.readbyqxmd.com/read/28058485/the-current-profile-of-persistent-cloaca-and-cloacal-exstrophy-in-japan-the-results-of-a-nationwide-survey-in-2014-and-a-review-of-the-literature
#2
Masayuki Kubota
PURPOSE: The current profile of persistent cloaca (PC) and cloacal exstrophy (CE) in Japan was first examined in 2014. MATERIALS AND METHODS: Information was obtained by sending a questionnaire to 244 university hospitals and children's hospitals. RESULTS: Responses from 113 institutions reported 466 PC cases and 229 CE cases. The incidences of PC and CE from 1980 to 2012 were 0.97 and 0.49 per 100,000 live births, respectively. In the previous 5 years, antenatal abnormalities were found in 57...
January 5, 2017: Pediatric Surgery International
https://www.readbyqxmd.com/read/28050437/a-rare-case-of-an-adult-with-untreated-bladder-exstrophy-presenting-with-signet-ring-cell-adenocarcinoma-of-urinary-bladder
#3
Abhishek J Savalia, Vikash Kumar, Gaurav Kasat, Ajit Sawant
Untreated bladder exstrophy in an adult is rare, as the defect is obvious and primary reconstruction is usually done in infancy. There are less than 90 reported cases of primary adenocarcinoma in an untreated bladder exstrophy in literature and only two such case reports from India. Of these, only one case was of signet-ring cell type of mucinous adenocarcinoma. Here we report the second case of signet-ring cell adenocarcinoma in a 63 year old male with untreated bladder exstrophy (oldest patient in literature), to highlight the extreme rarity, yet distinct possibility and challenges faced in surgical management of such cases...
November 2016: Journal of Clinical and Diagnostic Research: JCDR
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
#4
Nastaran Sabetkish, Sahar Eftekharzadeh, Azadeh Elmi, Saman Shafaat Talab, Abdol-Mohammad Kajbafzadeh
OBJECTIVES: To investigate the effect of low-dose human chorionic gonadotropin (HCG) administration on structural changes of the lower urinary tract in boys with urinary incontinence in the setting of bladder exstrophy/epispadias complex (BEEC). METHODS: We prospectively evaluated 30 patients (median age 7.5 years) with BEEC randomly divided into two groups. Patients in 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
#5
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/27915476/translational-research-for-pediatric-lower-urinary-tract-dysfunction
#6
REVIEW
Akihiro Kanematsu
This review provides a comprehensive view of translational research aimed at elucidating the pathophysiology of pediatric lower urinary tract dysfunction (LUTD). A web search was conducted according to combinations of keywords, and the significance of each article was defined by the author. The dramatic evolution of the mass analysis method of genomes, transcripts, and proteins has enabled a comprehensive analysis of molecular events underlying diseases, and these methodologies have also been applied to pediatric LUTD...
November 2016: International Neurourology Journal
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
#7
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/27890681/continent-anal-urinary-diversion-in-classic-bladder-exstrophy-45-year-experience
#8
Peter C Rubenwolf, Christian Hampel, Frederik Roos, Raimund Stein, Sebastian Frees, Joachim W Thüroff, Christian Thomas
OBJECTIVE: To evaluate the long-term outcomes in patients with classic bladder exstrophy and continent anal urinary diversion (CAD) for continence, upper urinary tract status, secondary malignancies, and sexual function. PATIENTS AND METHODS: The medical records of 82 exstrophy patients having undergone CAD in our department between 1970 and 2015 were reviewed. Patients were invited for follow-up examinations and asked to complete validated questionnaires relating to sexual function...
November 24, 2016: Urology
https://www.readbyqxmd.com/read/27889222/long-term-follow-up-of-composite-bladder-augmentation-incorporating-stomach-in-a-multi-institutional-cohort-of-patients-with-cloacal-exstrophy
#9
J T Casey, K H Chan, Y Hasegawa, T Large, B Judge, M Kaefer, R Misseri, R C Rink, K Ueoka, M P Cain
INTRODUCTION: Composite bladder augmentation, incorporating gastric and bowel segments, has the theoretical advantage of metabolic neutrality while potentially avoiding the morbidities of gastrocystoplasty, such as hematuria-dysuria syndrome. The most common indication for this operation is a paucity of bowel, such as in cloacal exstrophy. Despite several early descriptive studies of this technique, there are no reports, to date, of long-term follow-up in this population. OBJECTIVE: To describe the outcomes of composite bladder augmentation utilizing stomach in a cohort of cloacal exstrophy patients...
October 28, 2016: Journal of Pediatric Urology
https://www.readbyqxmd.com/read/27883314/diagnostic-and-surgical-approach-to-prenatally-detected-urinary-tract-anomalies
#10
Mile Petrovski
Regular ultrasound examinations carried out in the second trimester of pregnancy help in detecting many anomalies in the fetal urinary tract. Their percentage ranges from 1% to 3% of all controlled pregnancies. There is a wide spectrum of anomalies that affect the urinary tract, but the most significant are: uretero/hydronephrosis (unilateral or bilateral), kidney agenesis, dysplastic kidney, polycystic and multicystic kidneys, anomalies of ascent, anomalies of kidney rotation or fusion, bladder exstrophy, posterior urethra valve etc...
November 1, 2016: Prilozi (Makedonska Akademija Na Naukite i Umetnostite. Oddelenie za Medicinski Nauki)
https://www.readbyqxmd.com/read/27802004/one-staged-reconstruction-of-bladder-exstrophy-in-male-patients-long-term-follow-up-outcomes
#11
Amilcar Martins Giron, Marcos Figueiredo Mello, Paulo Afonso Carvalho, Paulo Renato Marcelo Moscardi, Roberto Iglesias Lopes, Miguel Srougi
INTRODUCTION: The surgical correction of bladder exstrophy remains challenging. In our institution, the repair has evolved from a staged repair to one-stage reconstruction. The one-stage reconstruction includes; bladder closure, Cantwell-Ransley neourethroplasty and abdominoplasty using groin flaps, without the need of pelvic ostheotomies. Repair of urinary continence (UC) and vesicoureteral reflux (VUR) is done after development of the infant. OBJECTIVE: To present our experience of our modified one-stage reconstruction of bla¬dder exstrophy in male patients...
November 2, 2016: International Braz J Urol: Official Journal of the Brazilian Society of Urology
https://www.readbyqxmd.com/read/27781020/stem-cells-in-functional-bladder-engineering
#12
REVIEW
Jakub Smolar, Souzan Salemi, Maya Horst, Tullio Sulser, Daniel Eberli
Conditions impairing bladder function in children and adults, such as myelomeningocele, posterior urethral valves, bladder exstrophy or spinal cord injury, often need urinary diversion or augmentation cystoplasty as when untreated they may cause severe bladder dysfunction and kidney failure. Currently, the gold standard therapy of end-stage bladder disease refractory to conservative management is enterocystoplasty, a surgical enlargement of the bladder with intestinal tissue. Despite providing functional improvement, enterocystoplasty is associated with significant long-term complications, such as recurrent urinary tract infections, metabolic abnormalities, stone formation, and malignancies...
September 2016: Transfusion Medicine and Hemotherapy
https://www.readbyqxmd.com/read/27751835/qualitative-questionnaire-on-the-psychosocial-wellbeing-of-mothers-of%C3%A2-children-with-beec
#13
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
https://www.readbyqxmd.com/read/27751489/re-ureteral-reimplantation-before-bladder-neck-reconstruction-in-modern-staged-repair-of-exstrophy-patients-indications-and-outcomes
#14
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
#15
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
https://www.readbyqxmd.com/read/27720781/failed-primary-bladder-exstrophy-closure-with-osteotomy-multivariable-analysis-of-a-25-year-experience
#16
Pokket Sirisreetreerux, Kathy M Lue, Thammasin Ingviya, Daniel A Friedlander, Heather N Di Carlo, Paul D Sponseller, John P Gearhart
PURPOSE: 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. We identify factors that contribute to a failed primary exstrophy closure with osteotomy. MATERIALS AND METHODS: We reviewed a prospectively maintained institutional database for classic bladder exstrophy cases primarily closed with osteotomy at our institution or referred after primary closure between 1990 and 2015...
October 5, 2016: Journal of Urology
https://www.readbyqxmd.com/read/27707652/bladder-continent-catheterizable-conduit-the-mitrofanoff-procedure-long-term-issues-that-should-not-be-underestimated
#17
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
https://www.readbyqxmd.com/read/27705734/wire-in-the-hole-a-case-series-of-eroded-intrapubic-wire-sutures-causing-genitourinary-complications-in-the-bladder-exstrophy-complex
#18
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
https://www.readbyqxmd.com/read/27695206/the-long-term-outcomes-after-staged-repair-of-exstrophy-epispadias-complex
#19
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
https://www.readbyqxmd.com/read/27649475/epispadias-and-the-associated-embryopathies-genetic-and-developmental-basis
#20
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
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