keyword
https://read.qxmd.com/read/37696307/classic-bladder-exstrophy-closure-without-osteotomy-or-immobilization-an-exercise-in-futility
#21
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/37666328/prenatal-presentation-of-a-covered-cloacal-exstrophy-variant-early-diagnostic-challenges-within-the-broad-spectrum-of-the-exstrophy-epispadias-complex
#22
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
#23
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
#24
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
https://read.qxmd.com/read/37607850/long-term-evaluation-of-continence-after-complete-primary-bladder-exstrophy-repair
#25
JOURNAL ARTICLE
Hisham M Hammouda, Ahmed A Shahat, Nariman Abol Oyoun, Ahmed S Safwat, Ahmed A Elderwy, Mohamed A Elgammal
INTRODUCTION: Continence after bladder exstrophy (BE) repair remains a major debatable challenge to pediatric urologists, together with the lack of standard definitions and long-term results in large series. OBJECTIVE: We assessed the long-term urinary continence in 142 toilet-trained cases after one (1-) stage of complete primary repair of bladder exstrophy (CPRE) and consequent procedures to achieve this goal in a single tertiary referral center. STUDY DESIGN: The current retrospective study included 123 boys and 19 girls with BE that were repaired by (1-) stage CPRE...
August 20, 2023: Journal of Pediatric Urology
https://read.qxmd.com/read/37509153/exome-survey-and-candidate-gene-re-sequencing-identifies-novel-exstrophy-candidate-genes-and-implicates-lztr1-in-disease-formation
#26
JOURNAL ARTICLE
Ricarda Köllges, Jil Stegmann, Sophia Schneider, Lea Waffenschmidt, Julia Fazaal, Katinka Breuer, Alina C Hilger, Gabriel C Dworschak, Enrico Mingardo, Wolfgang Rösch, Aybike Hofmann, Claudia Neissner, Anne-Karolin Ebert, Raimund Stein, Nina Younsi, Karin Hirsch-Koch, Eberhard Schmiedeke, Nadine Zwink, Ekkehart Jenetzky, Holger Thiele, Kerstin U Ludwig, Heiko Reutter
BACKGROUND: The bladder exstrophy-epispadias complex (BEEC) is a spectrum of congenital abnormalities that involves the abdominal wall, the bony pelvis, the urinary tract, the external genitalia, and, in severe cases, the gastrointestinal tract as well. METHODS: Herein, we performed an exome analysis of case-parent trios with cloacal exstrophy (CE), the most severe form of the BEEC. Furthermore, we surveyed the exome of a sib-pair presenting with classic bladder exstrophy (CBE) and epispadias (E) only...
July 13, 2023: Biomolecules
https://read.qxmd.com/read/37487788/assessment-of-anorectal-function-and-related-quality-of-life-of-27-patients-with-bladder-exstrophy-or-epispadias-after-kelly-radical-soft-tissue-mobilisation
#27
JOURNAL ARTICLE
Hortense Alliot, Sébastien Faraj, Thomas Loubersac, Guillaume Meurette, Stéphan De Napoli Cocci, Marc-David Leclair
INTRODUCTION: The Radical soft-tissue mobilisation (RSTM) described by J.H. Kelly for bladder exstrophy repair implies a detachment of levator ani muscle insertions from the pelvic wall. The aim of this controlled study was to evaluate the impact of this procedure on subsequent anorectal function. METHODS: Monocentric controlled study of prospectively collected data of children who underwent RSTM for BEEC from 2010 to 2017. Patients born after 2017 were not included, as they were below the theoretical age of continence acquisition at the time of the study...
June 16, 2023: Journal of Pediatric Surgery
https://read.qxmd.com/read/37473922/three-dimensional-computed-tomography-ct-scan-of-the-pelvic-bone-in-isolated-epispadias-analysis-before-surgical-correction
#28
JOURNAL ARTICLE
Hooman Kamran, Masoud Bitaraf, Roozbeh Tanhaeivash, Abdol-Mohammad Kajbafzadeh
OBJECTIVES: To determine intrapelvic angles and distances in pure epispadias patients and compare them to normal values. METHODS: Using three-dimensional computed tomography (3D-CT), 17 intrapelvic dimensions (eight angles and nine distances) were measured in 26 patients with pure epispadias (21 boys and five girls). These values were compared to normal values obtained from six patients (five boys and one girl) who underwent either pelvic or abdominopelvic CT for purposes unrelated to their bony pelvis...
July 18, 2023: Urology
https://read.qxmd.com/read/37470562/classical-bladder-exstrophy-in-an-adolescent-a-case-report-on-management-challenges-and-outcome
#29
Victor Ifeanyichukwu Modekwe, Okechukwu Hyginus Ekwunife, Jideofor Okechukwu Ugwu, Chuka Abunike Ugwunne, Chibuzo Uchechukwu Ndukwu, Henry Obinna Obiegbu, Afam Ben Obidike
Classical bladder exstrophy is a congenital anomaly whose management and outcome has advanced over years. Management and outcome are better when management starts at the newborn period. This was the management of a neglected bladder exstrophy in a male presenting at 16 years of age. We report our challenges, management and outcome to highlight the rarity of this presentation, and the adaptation to the usual protocol of care. The patient presented at 16 years of age with classic bladder exstrophy. The bladder plate was contracted and had cystitis...
2023: African Journal of Paediatric Surgery: AJPS
https://read.qxmd.com/read/37410166/ensuring-our-exstrophy-epispadias-complex-patients-and-families-thrive
#30
REVIEW
Emily Haddad, Lillian C Hayes, Diane Price, Christina G Vallery, Michael Somers, Joseph G Borer
Individuals with bladder exstrophy-epispadias complex (EEC) need long-term integrated medical/surgical and psychosocial care. These individuals are at risk for medical and surgical complications and experience social and psychological obstacles related to their genitourinary anomaly. This care needs to be accessible, comprehensive, and coordinated. Multiple surgical interventions, reoccurring hospitalizations, urinary and fecal incontinence, extensive treatment regimens for continent diversions, genital differences, and sexual health implications affect the quality of life for the EEC patient...
July 6, 2023: Pediatric Nephrology
https://read.qxmd.com/read/37385703/exstrophy-epispadias-complex
#31
REVIEW
Ted Lee, Joseph Borer
Exstrophy-epispadias complex encompasses a spectrum of disorders with lower abdominal midline malformations, including epispadias, bladder exstrophy, and cloacal exstrophy, also known as Omphalocele-Exstrophy-Imperforate Anus-Spinal Anomalies Complex. In this review, the authors discuss the epidemiology, embryologic cause, prenatal findings, phenotypic characteristics, and management strategies of these 3 conditions. The primary focus is to summarize outcomes pertaining to each condition.
August 2023: Urologic Clinics of North America
https://read.qxmd.com/read/37336177/surgical-correction-of-epispadias-associated-with-buried-penis
#32
Tarek Abdelazeem Sabra, Sarah Magdy Abdelmohsen, Ahmed Kamel Ali
INTRODUCTION AND IMPORTANCE: Isolated male epispadias is a rare entity with incidence of approximately 1 in 120,000 live births. Epispadias usually presents with a phimotic preputial orifice where glans is not visible and hence is also known as concealed epispadias. Buried penis in children is defined as a congenital insufficient penile skin with an unretractable foreskin that keeps the penis deep inside the pre-pubic fat. This congenital malformation of the penile envelopes is usually isolated...
June 16, 2023: International Journal of Surgery Case Reports
https://read.qxmd.com/read/37179209/a-single-institutional-experience-with-prenatal-diagnosis-of-cloacal-exstrophy-room-for-improvement
#33
JOURNAL ARTICLE
Christian C Morrill, Ahmad Haffar, Chad Crigger, Mara Black, Angie Jelin, Isam Nasr, John P Gearhart
INTRODUCTION: A single institutional study characterizes the rate of prenatal diagnosis of cloacal exstrophy (CE) and examines its role on successful primary closures. MATERIALS AND METHODS: An institutional database of 1485 exstrophy-epispadias patients was reviewed retrospectively for CE patients with confirmed presence/absence of prenatal diagnostics, primary exstrophy closure since 2000, institution of closure, and at least 1 year of follow up following closure...
April 20, 2023: Journal of Pediatric Surgery
https://read.qxmd.com/read/37164676/a-case-of-marked-metabolic-acidosis-due-to-a-transverse-stoma-after-urethroplasty-for-congenital-epispadias
#34
JOURNAL ARTICLE
Mariko Yamauchi, Sahoko Kamejima, Risa Ueda, Akio Nakashima, Fumihiko Urabe, Izumi Yamamoto, Ichiro Ohkido, Takashi Yokoo
A 58-year-old woman was admitted to our hospital. At 10 years old, she had undergone bilateral uretero-sigmoid anastomosis for congenital epispadias, and at 57 years old, she had received transverse colostomy. Biochemical tests showed marked metabolic acidosis. Computed tomography showed urine stagnation in the sigmoid colon, leading to a diagnosis of metabolic acidosis associated with transverse stoma after bilateral uretero-sigmoid anastomosis. Her bone mineral density was below normal, and the bone metabolic marker levels were high, indicating high-turnover osteoporosis...
May 10, 2023: Internal Medicine
https://read.qxmd.com/read/37150636/exstrophic-bladder-duplication-in-the-sagittal-plane-surgical-management-of-a-rare-case
#35
JOURNAL ARTICLE
Wietse Claeys, Caroline Jamaer, D Abramowitz, C Berquin, I Verbakel, A Ploumidis, P Hoebeke, E Van Laecke, A F Spinoit
INTRODUCTION: The bladder-Exstrophy-Epispadias complex (BEEC) contains a wide spectrum of congenital malformations. A treatment naïve refugee was referred to our center with what was identified as BEEC. MATERIALS AND METHODS: A 27-year-old female patient was referred for total incontinence since birth by the general practitioner from the refugee center. An exstrophic bladder with blind ending ureteral orifices and a second non-exstrophic bladder with two orthotopic ureters was identified, demonstrating the bladder duplication in the sagittal plane...
April 12, 2023: Journal of Pediatric Urology
https://read.qxmd.com/read/37149474/the-impact-of-repeated-bladder-surgery-on-successful-bladder-neck-closure-in-classic-bladder-exstrophy-the-role-of-mucosal-violations
#36
JOURNAL ARTICLE
Thomas G W Harris, Chad B Crigger, Tamir N Sholklapper, Ahmad Haffar, Robin Yang, Richard J Redett, John P Gearhart
INTRODUCTION: Restoration of genitourinary anatomy with functional urinary continence is the reconstruction aim is the exstrophy-epispadias complex (EEC). In patients who do not achieve urinary continence or those who are not a candidate for bladder neck reconstruction (BNR), bladder neck closure (BNC) is considered. Interposing layers including human acellular dermis (HAD) and pedicled adipose tissue are routinely placed between the transected bladder neck and distal urethral stump to reinforce the BNC and minimize failure due to fistula development from the bladder...
April 23, 2023: Journal of Pediatric Urology
https://read.qxmd.com/read/37120367/prevalence-of-opioid-and-benzodiazepine-use-in-adult-patients-with-the-exstrophy-epispadias-complex
#37
JOURNAL ARTICLE
Nora M Haney, Oscar Li, Pranjal Agrawal, Taylor P Kohn, Chad Crigger, Tamir Sholklapper, Heather DiCarlo, John P Gearhart
INTRODUCTION: Bladder exstrophy (BE), cloacal exstrophy (CE), and epispadias (E) are variants of the exstrophy-epispadias complex (EEC). These children require opioids and benzodiazepines to achieve pain management and immobilization for a lifetime of surgeries. It is hypothesized that these children would be sensitized to opiates and benzodiazepines as adults. The objective was to identify incidence of opiate and benzodiazepine use in adult EEC patients. METHODS: A US Health network, TriNetX Diamond was queried from 2009 to 2022...
April 5, 2023: Journal of Pediatric Urology
https://read.qxmd.com/read/37032596/bladder-exstrophy-epispadias-complex-related-litigation-a-legal-database-review
#38
JOURNAL ARTICLE
Christian C Morrill, Ahmad Haffar, Thomas Ditton, Heather N DiCarlo, John P Gearhart, Chad Crigger
OBJECTIVE: To review the nature and extent of bladder exstrophy-epispadias related malpractice litigation in the United States. METHODS: Two legal databases (Nexis Uni, WestLaw) were reviewed for state and federal cases using the terms "bladder exstrophy", "cloacal exstrophy", "epispadias", in combination with "medical malpractice", or "negligence", or "medical error", or "complication", or "malpractice", or "tort". Databases were queried from 1948 to 2022 and reviewed for medical and legal details...
April 9, 2023: Medico-legal Journal
https://read.qxmd.com/read/36977792/modelling-human-lower-urinary-tract-malformations-in-zebrafish
#39
REVIEW
Caroline M Kolvenbach, Gabriel C Dworschak, Johanna M Rieke, Adrian S Woolf, Heiko Reutter, Benjamin Odermatt, Alina C Hilger
Advances in molecular biology are improving our understanding of the genetic causes underlying human congenital lower urinary tract (i.e., bladder and urethral) malformations. This has recently led to the identification of the first disease-causing variants in the gene BNC2 for isolated lower urinary tract anatomical obstruction (LUTO), and of WNT3 and SLC20A1 as genes implicated in the pathogenesis of the group of conditions called bladder-exstrophy-epispadias complex (BEEC). Implicating candidate genes from human genetic data requires evidence of their influence on lower urinary tract development and evidence of the found genetic variants' pathogenicity...
March 29, 2023: Molecular and Cellular Pediatrics
https://read.qxmd.com/read/36959037/-we-the-be-an-educational-mobile-health-application-for-children-and-families-affected-by-bladder-exstrophy-epispadias-cloacal-exstrophy-complex
#40
JOURNAL ARTICLE
Scott Durham, Jason Odegaard, William Reiner, Ahmet Sancaktutar, Jake Klien, Eloise Kropp, Alican Kilicarslan, Bradley Kropp
GOALS: Despite the proliferation of over 45 000 smartphone mobile health applications (MHAs), as far as we know, there is no MHA for those living with rare diseases such as Bladder Exstrophy-Epispadias-Cloacal Exstrophy complex (BEEC). We hypothesized that an MHA could provide similar "on-demand" information and connectivity within health communities for patients with BEEC as they do for more common diseases. Thus, our primary goal was to create an MHA for patients and families affected by BEEC to provide them with important information about the condition and a format for them to connect with other affected patients and families...
February 28, 2023: Journal of Pediatric Urology
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