keyword
https://read.qxmd.com/read/38245430/perioperative-management-of-primary-classic-bladder-exstrophy-a-single-institutional-pathway-to-success
#21
JOURNAL ARTICLE
Ahmad Haffar, Alexander Hirsch, Christian Morrill, Thomas G W Harris, Chad Crigger, Adelaide Garcia, Victoria Maxon, Heather N Di Carlo, Constance Monitto, John P Gearhart, Joann B Hunsberger
PURPOSE: Appropriate perioperative management is crucial in patients undergoing classic bladder exstrophy closure (CBE). Therefore, the authors sought to review their intra and postoperative management of patients with CBE undergoing primary closure and examine the impact of this pathway on patient outcomes. METHOD: A prospectively maintained institutional approved exstrophy-epispadias complex database was reviewed for patients with CBE who had undergone primary closure between 2016 and 2022 and whose closure was performed within one year of age...
January 11, 2024: Journal of Pediatric Urology
https://read.qxmd.com/read/38189741/the-true-pelvic-volume-change-with-various-corrective-osteotomy-techniques-for-exstrophy-epispadias-complex-spectrum-the-value-of-computer-assisted-virtual-surgery
#22
JOURNAL ARTICLE
Mohamed Kenawey, Emmanouil Morakis, Raimondo Cervellione, David Keene, Simon P Kelley
Pelvic osteotomies are essential to approximate widened symphysis pubis in the exstrophy-epispadias complex, yet it is unknown which osteotomy type has the greatest effect on pelvic volume. We therefore used virtual surgery to study pelvic volume change with anterior, oblique, and posterior iliac osteotomies. Preoperative CT scans of two cloacal and one classic bladder exstrophy patients were used. Simulations were free-hand or constrained to keep minimal strain in the sacrospinous SSL and sacrotuberous STL ligaments...
December 14, 2023: Journal of Pediatric Orthopedics. Part B
https://read.qxmd.com/read/38085295/-epidemiology-diagnosis-treatment-and-follow-up-of-the-bladder-exstrophy-epispadias-complex
#23
REVIEW
Anne-Karoline Ebert, Franziska Wolff, Julia Lackner
The development of the new S3 guideline "Epidemiology, diagnosis, treatment and follow-up of the bladder exstrophy-epispadias complex" was funded by the German Innovation Fund of the Federal Joint Committee (G-BA). Despite the relatively low level of evidence of the identified literature, a systematic approach and consistent evaluation of the literature enabled the formulation of a large number of evidence-based recommendations across a variety of topics. In addition, a patient guideline is under development in order to disseminate the guideline recommendations and to enhance self-management and understanding among patients and their relatives...
December 12, 2023: Urologie
https://read.qxmd.com/read/38082334/de-novo-variants-identified-by-trio-whole-exome-sequencing-of-bladder-exstrophy-epispadias-complex
#24
JOURNAL ARTICLE
Angie C Jelin, Elizabeth Wohler, Renan Martin, Heather Di Carlo, William Isaacs, Joan Ko, Jason Michaud, Karin Blakemore, David Valle, Nara Sobreira, John Gearhart
Bladder exstrophy epispadias complex (BEEC) encompasses a spectrum of conditions ranging from mild epispadias to the most severe form: omphalocele-bladder exstrophy-imperforate anus-spinal defects (OEIS). BEEC involves abnormalities related to anatomical structures that are proposed to have a similar underlying etiology and pathogenesis. In general, BEEC, is considered to arise from a sequence of events in embryonic development and is believed to be a multi-etiological disease with contributions from genetic and environmental factors...
December 11, 2023: American Journal of Medical Genetics. Part A
https://read.qxmd.com/read/38046258/bladder-triplication-associated-with-exstrophy-variant
#25
Monica Salgado-Diaz, Valerie Hogues, Anne-Marie Houle, Julie Franc-Guimond, Diego Barrieras
A one-day old full-term female neonate presented with a duplicate bladder and exstrophy variant including a patch of exstrophic or ectopic mucosa, duplicate vagina, uterus, and two complete bladders. We report on the surgical management performed in this case and functional urinary results based on a synchronous urodynamic study of the duplicate bladders. To our knowledge, the occurrence of duplicate bladder exstrophy variant with complete urinary continence has not been previously reported.
November 2023: Urology Case Reports
https://read.qxmd.com/read/37972897/lumbar-syndrome-and-bladder-exstrophy%C3%A2-a-case-report
#26
Catherine Nguyen, Niccolo Passoni
LUMBAR syndrome is rare with a multitude of features that requires a high index of suspicion for timely diagnosis and appropriate management. We present on a newborn female whose untreated segmental infantile hemangioma (IH) lead to poor healing of her bladder exstrophy closure. The objective of this report is to describe bladder exstrophy as a urogenital anomaly in patients with LUMBAR syndrome and the importance of balancing management of IH and time to surgery.
November 14, 2023: Urology
https://read.qxmd.com/read/37965120/unrepaired-cloacal-exstrophy-in-an-adult-medical-and-ethical-considerations
#27
Amelia Vu, Kelly T Harris, Lea Wehrli, Andrea Bischoff, Dan Wood
Cloacal exstrophy, also known as OEIS complex, is a rare condition, comprised of severe congenital anomalies. This case report describes a 24-year-old 46,XY individual who had repair of the omphalocele at birth, but has remained with an unrepaired bladder exstrophy. This case highlights the intersections between medical decision-making, individualized management of complex patients, and ethical considerations for adults lacking capacity.
November 2023: Urology Case Reports
https://read.qxmd.com/read/37941331/superior-vesical-fissure-as-an-incomplete-form-of-bladder-exstrophy-a-case-report-with-clinico-pathological-correlation-and-a-comprehensive-literature-review
#28
JOURNAL ARTICLE
Javier Arredondo Montero, Mónica Bronte Anaut, Ada Yessenia Molina Caballero, Elena Carracedo Vega, Rosa Guarch Troyas, Alberto Pérez-Martínez
Multiple variants of classic bladder exstrophy have been described, all of them infrequent. Superior vesical fissure is a mild variant of this pathology in which genital involvement is scarce or absent. To date, there are only isolated reports of this entity. We report a full-term female patient of Arabian descent with a clinical and radiological diagnosis of superior vesical fissure that was surgically corrected in our center with a favorable evolution. Histological study, supported by immunohistochemical techniques, showed squamous and transitional epithelium and discrete chronic inflammation...
November 8, 2023: International Journal of Surgical Pathology
https://read.qxmd.com/read/37933714/validation-of-icd-9-cm-codes-for-major-genitourinary-birth-defects-in-military-health-system-administrative-data-2006-2014
#29
JOURNAL ARTICLE
Celeste J Romano, Sandra M Magallon, Clinton Hall, Anna T Bukowinski, Gia R Gumbs, Ava Marie S Conlin
BACKGROUND: The Department of Defense Birth and Infant Health Research program is dedicated to birth defects research and surveillance among military families. Here, we assess and refine the validity of International Classification of Diseases, Ninth Revision, Clinical Modification (ICD-9-CM) codes for selected genitourinary birth defects in the Military Health System (MHS). We additionally outline methods for the calculation of positive predictive value (PPV) and negative predictive value (NPV), sensitivity, and specificity using a stratified sampling design...
November 7, 2023: Birth Defects Research
https://read.qxmd.com/read/37932196/novel-pelvic-immobilization-method-following-bladder-exstrophy-repair
#30
JOURNAL ARTICLE
Jacqueline P Morin, Molly Fuchs, Kevin Klingele, Rama Jayanthi
We introduce a novel method of pelvic immobilization following bladder exstrophy repair involving a custom-made brace that offers adequate pelvic immobilization with the added benefits of no added operative time, easy evaluation of surgical sites, simplified wound care including sponge bathing, and overall less cumbersome management for the family given its more streamlined size.
October 20, 2023: Journal of Pediatric Urology
https://read.qxmd.com/read/37928513/open-retropubic-prostatectomy-in-a-patient-with-bladder-exstrophy-for-management-of-hematuria-and-urethral-discharge-a-case-report
#31
Juan Sebastian Arroyave, Daniel Wang, Christopher Connors, Francisca Larenas, Fernando Ferrer, Michael Palese
Bladder exstrophy (BE) is a rare congenital disorder causing bladder and urethral malformation due to an abdominal wall embryological defect. Traditionally, BE had a poor life expectancy, but advances now offer a normal lifespan. A 57-year-old male with BE history and ureterosigmoidostomy repair presented with intractable hematuria, urethral discharge, and recurrent prostatic infections. He underwent retropubic subtotal prostatectomy without major complications, resolving urinary symptoms. Advances in BE management extend patient lifespans...
November 2023: Urology Case Reports
https://read.qxmd.com/read/37905290/a-rare-case-of-isolated-male-epispadias
#32
Dawood Shehzad, Mustafa Shehzad, Abdul Mutaal
Epispadias is a congenital malformation marked by the failure of the urethral bulb to tubularize dorsally. This results in a wide-open urethral plate dorsally. Epispadias is frequently associated with bladder exstrophy-epispadias complex.  We report the case of a five-year-old patient who presented in the outpatient department with isolated epispadias without any other associated abnormality. This report aims to document this rare case of isolated male epispadias with incontinence and the success of using the modified Cantwell-Ransley technique for its treatment...
September 2023: Curēus
https://read.qxmd.com/read/37856541/an-observational-study-on-the-sexual-genital-and-fertility-outcomes-in-bladder-exstrophy-and-epispadias-patients
#33
JOURNAL ARTICLE
Emma Van den Eede, Mira Sterckx, Kato Vangelabbeek, Charlotte Dunford, Anthony Noah, Dan Wood, Gunter De Win
INTRODUCTION: Bladder exstrophy and epispadias complex (BEEC) is a spectrum of congenital malformations ranging from an isolated epispadias to a full exstrophy. It is an uncommon disease and little is known on how patients cope with its implications later in life. OBJECTIVE: The goal of this study is to assess the sexual, continence and fertility outcomes of BEEC patients, who had reconstructive bladder surgery during childhood. Considering the sensitive nature of these topics, they are not easily spoken about in the doctor's office...
February 2023: Journal of Pediatric Urology
https://read.qxmd.com/read/37777362/multi-staged-vs-single-staged-pelvic-osteotomy-in-the-modern-treatment-of-cloacal-exstrophy-bridging-the-gap
#34
JOURNAL ARTICLE
Ahmad Haffar, Alexander M Hirsch, Chad B Crigger, Thomas G W Harris, Nora M Haney, Logan B Galansky, Isam W Nasr, Paul D Sponseller, John P Gearhart
PURPOSE: Staged pelvic osteotomy has been shown in the past to be an effective tool in the closure of the extreme pubic diastasis of cloacal exstrophy. The authors sought to compare orthopedic complications between non-staged pelvic osteotomies and staged pelvic osteotomies in cloacal exstrophy. METHODS: A prospectively maintained exstrophy-epispadias complex database of 1510 patients was reviewed for cloacal exstrophy bladder closure events performed with osteotomy at the authors' institution...
September 9, 2023: Journal of Pediatric Surgery
https://read.qxmd.com/read/37704529/urological-outcome-with-the-use-of-salvage-continent-vesicostomy-in-pediatric-reconstructive-surgery-a-single-tertiary-center-experience
#35
JOURNAL ARTICLE
Javier Ruiz, Felicitas Lopez Imizcoz, Santiago Weller, María Tatiana Szklarz, Catalina Tessi, Nicolas Rosiere, Luciana Diaz Zabala, Otilia Blain, Yesica Gomez, Esteban Gallino, Carol Maria Burek, Cristian Sager, Juan Pablo Corbetta
PURPOSE: To report our experience and results in terms of complications, reoperation rate and urinary continence with the Salvage Continent Vesicostomy (SCV) technique in pediatric patients with history or need of enterocystoplasty and absent appendix. METHODS: Retrospective review of all patients with a history of a continent catheterizable channel surgery performed in our institution between June 2016 and January 2022. Only patients with a SCV surgery with a minimum 6-month post operative follow up were included in this cohort and divided in group 1 (history of previous bladder augmentation) and group 2 (simultaneous bladder augmentation and SCV)...
September 1, 2023: Journal of Pediatric Urology
https://read.qxmd.com/read/37696307/classic-bladder-exstrophy-closure-without-osteotomy-or-immobilization-an-exercise-in-futility
#36
JOURNAL ARTICLE
Ahmad Haffar, Alexander Hirsch, Christian Morrill, Chad Crigger, Paul D Sponseller, John P Gearhart
OBJECTIVE: To review the outcomes of classic bladder exstrophy closure without the use of osteotomy or lower extremity/pelvic immobilization. STUDY DESIGN: A prospectively maintained institutional approved exstrophy-epispadias complex database of 1487 patients was reviewed for patients with CBE who had undergone closure without osteotomy nor immobilization. All patients were referred to the authors' institution for reconstruction later in life or for failed closure...
September 9, 2023: Urology
https://read.qxmd.com/read/37667897/presentations-of-bladder-exstrophy-in-a-resource-limited-setting-and-the-role-of-mainz-ii-continent-diversion-for-late-referrals-or-failed-primary-closures-a-multicentric-report
#37
JOURNAL ARTICLE
Alessandro Calisti, Kibreab Belay, Andrea Mombo, Faisal Abdelgalil Nugud, Diaaeldinn Yaseen Salman, Pierluigi Lelli Chiesa
Primary closure techniques that have been updated and longterm follow-up for CBE (classic bladder exstrophy) may be out of reach for many patients living in resource-limited settings. Late referrals to medical care and primary closures that lack the necessary skills and facilities for comprehensive treatment are still common. Alternative and long-term surgical solutions may improve the lives of these unfortunate patients. During surgical outreach missions, patients with CBE, either non-operated or with a previous unsuccessful bladder closure, who were referred from vast under-resourced rural areas to three Eastern African hospitals, were studied...
August 29, 2023: La Pediatria Medica e Chirurgica: Medical and Surgical Pediatrics
https://read.qxmd.com/read/37666328/prenatal-presentation-of-a-covered-cloacal-exstrophy-variant-early-diagnostic-challenges-within-the-broad-spectrum-of-the-exstrophy-epispadias-complex
#38
Maya R Overland, Edward R Oliver, Susan J Back, Thomas F Kolon, Aseem R Shukla, Dana A Weiss
There is a broad range of variant phenotypes that can occur within the bladder exstrophy and epispadias complex spectrum. Accurate prenatal detection helps prepare families and to coordinate subspecialty resources. Here, we present the case of a patient with prenatally diagnosed patient with covered cloacal exstrophy variant along with four additional cases illustrating the nonlinear spectrum from isolated epispadias to cloacal exstrophy. Given the rarity of these variants overall and of each subtype within the spectrum, there is a need for long-term multi-institutional outcomes data to improve detection, characterization, and prognostication for these patients...
September 2, 2023: Urology
https://read.qxmd.com/read/37649128/analyzing-the-factors-that-contribute-to-the-development-of-embryological-classical-type-of-bladder-exstrophy
#39
JOURNAL ARTICLE
Ria Margiana, Widya Juwita, Khoirul Ima, Zakiyatul Faizah, Supardi Supardi
Bladder exstrophy is a rare congenital condition of the pelvis, bladder, and lower abdomen that opens the bladder against the abdominal wall, produces aberrant growth, short penis, upward curvature during erection, wide penis, and undescended testes. Exstrophy affects 1/30,000 newborns. The bladder opens against the abdominal wall in bladder exstrophy, a rare genitourinary condition. This study is vital to provide appropriate therapy choices as a basis to improve patient outcomes. This study may explain bladder exstrophy and provide treatment...
August 31, 2023: Anatomy & Cell Biology
https://read.qxmd.com/read/37642679/initial-experience-with-act%C3%A2-periurethral-adjustable-balloons-to-treat-urinary-incontinence-due-to-intrinsic-sphincter-deficiency-in-the-pediatric-population
#40
JOURNAL ARTICLE
Alice Faure, Mirna Haddad, Jessica Pinol, Thierry Merrot, Jean-Michel Guys, Floriane Michel, Gilles Karsenty
PURPOSE: To present our initial experience with periurethral adjustable continence therapy (ACT™) for urinary incontinence due to intrinsic sphincter deficiency (ISD) in children. METHODS: This is an approved prospective non-randomized pilot study (NCT03351634) aiming to treat children born with spinal dysraphism (SD) or exstrophy epispadias complex (EEC) with ACT™. Endpoints were patient-reported changes in daily pad count, 24-h Pad test and complications...
August 29, 2023: World Journal of Urology
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