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Cloacal malformations

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https://www.readbyqxmd.com/read/29223670/cystourethroscopy-versus-contrast-studies-in-urogenital-sinus-and-cloacal-anomalies-in-children
#1
Khaled Ashour, Sameh Shehata, Ahmed Osheba
BACKGROUND: Cloacal malformation is a spectrum of diseases affecting females, resulting in abnormal confluence of the urinary system, genital system, and/or gastrointestinal system. Proper reconstruction depends mainly on accurate preoperative illustration of the abnormal anatomy. Among the various modalities to delineate the urogenital sinus and the distances to confluence, lies the radiological contrast study, as well as the preoperative diagnostic cystourethroscopy. AIM OF THE STUDY: The aim of this study was to compare the accuracy of the contrast study and diagnostic cystourethroscopy in the demonstration of the exact changes in anatomy resulting from urogenital sinus / cloacal abnormalities...
November 14, 2017: Journal of Pediatric Surgery
https://www.readbyqxmd.com/read/29132797/cloaca-reconstruction-a-new-algorithm-which-considers-the-role-of-urethral-length-in-determining-surgical-planning
#2
Richard J Wood, Carlos A Reck-Burneo, Daniel Dajusta, Christina Ching, Rama Jayanthi, D Gregory Bates, Molly E Fuchs, Katherine McCracken, Geri Hewitt, Marc A Levitt
BACKGROUND: Cloacal malformations represent a uniquely complex challenge for surgeons. The surgical approach to date has been based on the common channel (CC) length with two patient groups considered: less than or greater than 3cm, which we believe is an oversimplification. We reviewed 19 patients, referred after surgery done elsewhere. Eight had postoperative urinary complications, 3 had constant urinary leakage and had been left after surgery with a urethra <1cm, .5 with an original 3 to 5cm common channel, who had undergone total urogenital mobilization (TUM), experienced peri-operative urethral loss needing a vesicostomy, and later, a Mitrofanoff...
October 12, 2017: Journal of Pediatric Surgery
https://www.readbyqxmd.com/read/29033614/fusion-of-lower-limbs-with-severe-urogenital-malformation-in-a-newborn-a-rare-congenital-clinical-syndrome-case-report
#3
Fatemah Al Hadhoud, Abeer H Kamal, Abdulmohsen Al Anjari, Michael Fe Diejomaoh
BACKGROUND: Fused lower limbs combined with severe urogenital malformation, also known as sirenomelia, is a rare congenital clinical syndrome. The etiology is unknown, and the outcome for the affected fetus is rather uncertain. CASE REPORT: Mrs RB, a Kuwaiti woman primigravida, married to a non-consanguineous husband, had uneventful antenatal care in a private health service, until she was admitted to the Maternity Hospital, Kuwait, at 31 weeks of gestation with a 3-hour history of ruptured membranes...
2017: International Medical Case Reports Journal
https://www.readbyqxmd.com/read/28960693/clinical-and-risk-factor-analysis-of-cloacal-defects-in-the-national-birth-defects-prevention-study
#4
Kim M Keppler-Noreuil, Kristin M Conway, Dereck Shen, Anthony J Rhoads, John C Carey, Paul A Romitti
Cloacal exstrophy (CE) and persistent cloaca (PC) (alternatively termed urorectal septum malformation sequence [URSMS]), represent two major cloacal defects (CDs). Clinical characteristics and risk factors often are studied for both defects combined, rather than exploring if these defects have different etiologies. We enumerated clinical features for 47 CE and 54 PC (inclusive of URSMS) cases from the National Birth Defects Prevention Study. Thirty-three CE cases were classified as isolated and 14 as multiple (presence of unassociated major defects); respective totals for PC cases were 26 and 28...
November 2017: American Journal of Medical Genetics. Part A
https://www.readbyqxmd.com/read/28924534/image-of-the-month-clinical-features-in-a-newborn-with-covered-cloacal-exstrophy
#5
Alejandra Vilanova-Sánchez, Christina B Ching, Alessandra C Gasior, Karen Diefenbach, Richard J Wood, Marc Levitt
Cloacal exstrophy is the most severe type of anorectal malformations that belongs to the bladder-exstrophy-epispadias complex of genitourinary malformations. Interestingly, its variant, the covered cloacal exstrophy, is often missed. The clinical findings of this variant may include an imperforate anus, low lying umbilicus, thick pubic bone, and pubic diastasis but with an intact abdominal wall. We present an interesting case of covered cloacal exstrophy with a side-by-side duplicated bladder and discuss important considerations for the time of colostomy creation in the newborn period...
January 2017: European Journal of Pediatric Surgery Reports
https://www.readbyqxmd.com/read/28890277/-prenatal-diagnosis-of-abdominal-wall-defects
#6
H El Mhabrech, H Ben Hmida, H Charfi, A Zrig, C Hafsa
Anterior abdominal wall defects (AAWD) correspond to a wide spectrum of congenital defects affecting 6.3/10,000 pregnancies. They have in common a closure defect of the anterior abdominal wall and can be fatal or expose the fetus and the neonate (NN) to many complications. This study was based on a retrospective study of 22 cases of AAWD collected between May 2009 and December 2014. Its purpose was to specify the importance of prenatal ultrasonography in the diagnosis and prognosis assessment of these defects...
October 2017: Archives de Pédiatrie: Organe Officiel de la Sociéte Française de Pédiatrie
https://www.readbyqxmd.com/read/28888815/neurogenic-bladder-in-a-girl-after-surgery-for-cloacal-malformation-the-importance-of-informing-patients-and-parents-of-all-various-options
#7
Candace F Granberg, Patricio C Gargollo
Management of neurogenic bladder in pediatric patients is complex. Treatment options must be individualized to not only address the clinical parameters but also take into account the social factors that directly impact the ability to achieve a successful outcome.
April 2017: European Urology Focus
https://www.readbyqxmd.com/read/28852767/european-society-of-paediatric-radiology-abdominal-imaging-task-force-recommendations-in-paediatric-uroradiology-part-ix-imaging-in-anorectal-and-cloacal-malformation-imaging-in-childhood-ovarian-torsion-and-efforts-in-standardising-paediatric-uroradiology
#8
REVIEW
Michael Riccabona, Maria-Luisa Lobo, Lil-Sofie Ording-Muller, A Thomas Augdal, E Fred Avni, Johan Blickman, Constanza Bruno, Beatrice Damasio, Kassa Darge, Akaterina Ntoulia, Frederica Papadopoulou, Pierre-Hugues Vivier
At the occasion of the European Society of Paediatric Radiology (ESPR) annual meeting 2015 in Graz, Austria, the newly termed ESPR abdominal (gastrointestinal and genitourinary) imaging task force set out to complete the suggestions for paediatric urogenital imaging and procedural recommendations. Some of the last missing topics were addressed and proposals on imaging of children with anorectal and cloacal malformations and suspected ovarian torsion were issued after intense discussions and a consensus finding process that considered all evidence...
September 2017: Pediatric Radiology
https://www.readbyqxmd.com/read/28810634/misdiagnosis-of-a-cloacal-exstrophy-variant-as-urorectal-septum-malformation-in-a-fetus-by-ultrasound-a-case-report
#9
Yang-Qing Xu, Xiao-Hong Yang, Xin-Lin Chen, Xiu-Qiin Ji, Sheng Zhao
Cloacal exstrophy variants are comprised of a wide range of characteristics, of which there are four primary features, including omphalocele, bladder exstrophy, an imperforate anus and spina bifida. The existing literature regarding the differential diagnosis from alternative urinary diseases prenatally are limited. If the bladder is present, defects in the ventral wall may not be visualized with prenatal ultrasound in certain conditions, including oligohydramnios, and differential diagnosis from urorectal septum malformation sequence is a challenge...
August 2017: Experimental and Therapeutic Medicine
https://www.readbyqxmd.com/read/28797517/gynecologic-anatomic-abnormalities-following-anorectal-malformations-repair
#10
Alejandra Vilanova-Sanchez, Carlos A Reck, Kate A McCracken, Victoria A Lane, Alessandra C Gasior, Richard J Wood, Marc A Levitt, Geri D Hewitt
BACKGROUND/AIM: Patients may present with gynecologic concerns after previous posterior sagittal anorectoplasty (PSARP) for repair of an anorectal malformation (ARM). Common findings include an inadequate or shortened perineal body, as well as introital stenosis, retained vaginal septum, and remnant rectovestibular fistula. An inadequate or shortened perineal body may impact fecal continence, sexual function and recommendations regarding obstetrical mode of delivery. We describe our experience with female patients referred to our center for evaluation of their previously repaired ARM, with a specific focus on perineal body anatomy and concomitant gynecologic abnormalities...
July 24, 2017: Journal of Pediatric Surgery
https://www.readbyqxmd.com/read/28753873/comment-on-neurogenic-bladder-in-a-girl-after-surgery-for-cloacal-malformation
#11
Raimund Stein, Nina Huck
In children with a neurogenic bladder and bowel, all options for continent and incontinent diversions have to be discussed in detail including all advantages and disadvantages with the patient, parents, caregivers and all disciplines involved.
July 5, 2017: European Urology Focus
https://www.readbyqxmd.com/read/28753841/case-presentation-neurogenic-bladder-in-a-girl-after-surgery-for-cloacal-malformation
#12
Goedele M A Beckers, R Jeroen A van Moorselaar
A girl born in 2006 has been under close surveillance in our pediatric urology unit since birth. Besides cloacal malformation, she has associated spinal cord dysraphism, uterus didelphys, and a vaginal septum.
July 4, 2017: European Urology Focus
https://www.readbyqxmd.com/read/28739627/reconstruction-of-urethra-using-appendix-in-a-patient-with-cloacal-malformation
#13
Sandeep Gupta, Barun Kumar, Sandip Basu, Dilip Kumar Pal
We report a case of 21-year-old young woman with congenital cloacal malformation. She was operated at the age of 1.5 years separating the rectum from common opening as a two-stage repair. She was incontinent in the earlier part of her life but she became continent to some extent later in early adulthood. She presented with urinary stress incontinence following delivery of dead fetus of 6 months. She underwent multiple investigations revealing common opening of bladder neck and vagina. A multidisciplinary evaluation was done and she underwent closure of common channel and neourethra reconstruction using pedicled appendix...
July 24, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/28696998/evaluation-and-management-of-vaginoplasty-complications
#14
Anne-Marie Amies Oelschlager, Anna Kirby, Lesley Breech
PURPOSE OF REVIEW: Indications for vaginoplasty include congenital conditions such as adrenal hyperplasia, cloacal malformations, and Müllerian agenesis, acquired conditions including stenosis from radiation or surgical resection for malignancy, and gender affirmation. All vaginoplasty techniques carry significant risk of both immediate and long-term complications. RECENT FINDINGS: The purpose of this study is to provide a review of the evaluation and management of the neovagina, addressing management of human papilloma virus infections and complications including stenosis, fistula, prolapse, and neovaginal colitis...
October 2017: Current Opinion in Obstetrics & Gynecology
https://www.readbyqxmd.com/read/28673795/use-of-3d-reconstruction-cloacagrams-and-3d-printing-in-cloacal-malformations
#15
Jennifer J Ahn, Margarett Shnorhavorian, Anne-Marie E Amies Oelschlager, Beth Ripley, Giridhar M Shivaram, Jeffrey R Avansino, Paul A Merguerian
INTRODUCTION: Cloacal anomalies are complex to manage, and the anatomy affects prognosis and management. Assessment historically includes examination under anesthesia, and genitography is often performed, but these do not consistently capture three-dimensional (3D) detail or spatial relationships of the anatomic structures. Three-dimensional reconstruction cloacagrams can provide a high level of detail including channel measurements and the level of the cloaca (<3 cm vs. >3 cm), which typically determines the approach for surgical reconstruction and can impact long-term prognosis...
August 2017: Journal of Pediatric Urology
https://www.readbyqxmd.com/read/28671963/effects-of-selective-and-combined-activation-of-estrogen-receptor-%C3%AE-and-%C3%AE-on-reproductive-organ-development-and-sexual-behaviour-in-japanese-quail-coturnix-japonica
#16
Anna Mattsson, Björn Brunström
Excess estrogen exposure of avian embryos perturbs reproductive organ development in both sexes and demasculinizes the reproductive behaviors of adult males. We have previously shown that these characteristic effects on the reproductive organs also can be induced by exposure of Japanese quail (Coturnix japonica) embryos to selective agonists of estrogen receptor alpha (ERα). In contrast, the male copulatory behavior is only weakly affected by developmental exposure to an ERα agonist. To further elucidate the respective roles of ERα and ERβ in estrogen-induced disruption of sexual differentiation, we exposed Japanese quail embryos in ovo to the selective ERα agonist 16α-lactone-estradiol (16αLE2), the selective ERβ agonist WAY-200070, or both substances in combination...
2017: PloS One
https://www.readbyqxmd.com/read/28616723/post-operative-strictures-in-anorectal-malformation-trends-over-15%C3%A2-years
#17
Charlotte Holbrook, Devesh Misra, Indre Zaparackaite, Stewart Cleeve
AIM: For decades, paediatric surgeons have employed the standard posterior sagittal anorectoplasty (PSARP) approach to deal with patients with anorectal malformations (ARM). In recent years, we noted an apparent increase in the incidence of anal stricture after surgical repair of ARM following the introduction of laparoscopic pull-through and techniques aiming to preserve the internal sphincter-the internal sphincter sparing approach (ISSA). We decided to analyse our data to find out if these new trends had added to the problem of post-operative strictures...
August 2017: Pediatric Surgery International
https://www.readbyqxmd.com/read/28601900/embryological-and-clinical-implications-of-the-association-between-anorectal-malformations-and-spinal-dysraphisms
#18
Giorgia Totonelli, Raffaella Messina, Francesco Morini, Giovanni Mosiello, Paolo Palma, Marianna Scuglia, Barbara D Iacobelli, Pietro Bagolan
PURPOSE: To describe the association of anorectal malformation (ARM) and spinal dysraphism (SD) in terms of impact on the management of SD and embryogenetic implications. METHODS: Patients with SD associated with (A) or without (B) ARM were included. The two groups were further divided into operated on (A1/B1) or not (A2/B2) for SD. Groups A and B were compared for type of SD (embryogenetic classification) and prevalence of neurosurgery; Groups A1 vs. A2 for type of ARM (Wingspread classification); Groups A1 vs...
August 2017: Pediatric Surgery International
https://www.readbyqxmd.com/read/28254239/psychosexual-development-management-of-bladder-exstrophy-epispadias-in-complex-patients
#19
M Di Grazia, S Pellizzoni, L G Tonegatti, W Rigamonti
INTRODUCTION: Bladder-exstrophy-epispadias complex (BEEC) represents a spectrum of urogenital step-wise malformations: epispadias, complete exstrophy, and cloacal exstrophy. Psychosexual development in adolescent patients with BEEC may become especially problematic. At present, there are few contributions in the literature investigating the validity of psychosexual treatment in order to tackle this particularly emotional and personal development phase. OBJECTIVE: The study aimed at verifying the efficacy of an intervention methodology for psychosexual support of a group of adolescents with BEEC...
April 2017: Journal of Pediatric Urology
https://www.readbyqxmd.com/read/28191911/mutations-of-myh14-are-associated-to-anorectal-malformations-with-recto-perineal-fistulas-in-a-small-subset-of-chinese-population
#20
Zhongxian Zhu, Lei Peng, Guanglin Chen, Weiwei Jiang, Ziyang Shen, Chunxia Du, Rujin Zang, Yang Su, Hua Xie, Hongxing Li, Yankai Xia, Weibing Tang
BACKGROUND: Anorectal malformations (ARMs) are among the most commonly congenital abnormalities of distal hindgut development, ranging from anal stenosis to anal atresia with or without fistulas and persistent cloaca. The etiology remains elusive for most ARM cases and the majority of genetic studies on ARMs were based on a candidate gene approach. MATERIALS AND METHODS: In all eight family members of a non-consanguineous Chinese family, we performed whole-exome sequencing...
February 13, 2017: Clinical Genetics
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