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https://www.readbyqxmd.com/read/28081578/birth-prevalence-of-anorectal-malformations-for-the-western-cape-province-south-africa-2005-to-2012
#1
Andre Theron, Alp Numanoglu
Introduction Anorectal malformations (ARMs) are a major birth anomaly worldwide. South Africa has ethnically and geologically diverse populations. A recent publication indicated an increased birth prevalence of ARMs in the Witwatersrand referral area between 2005 and 2010. The purpose of this study was to determine the birth prevalence of ARM and its various subtypes in the Western Cape referral district over an 8-year period. Methods For an 8-year period from January 1, 2005, to December 31, 2012; retrospective data were collected from the Pediatric Surgical Departments of Red Cross War Memorial Children's Hospital, Tygerberg Children's Hospital, as well as the private sector health registries...
January 12, 2017: European Journal of Pediatric Surgery
https://www.readbyqxmd.com/read/28065719/the-currarino-triad-what-pediatric-surgeons-need-to-know
#2
Amr Abdelhamid AbouZeid, Shaimaa Abdelsattar Mohammad, Mohammad Abolfotoh, Ahmed Bassiouny Radwan, Mohamed Mohamed ElSayed Ismail, Tarek Ahmed Hassan
PURPOSE: We report our experience in managing a group of patients with Currarino syndrome, highlighting diagnostic challenges, surgical techniques, in addition to a review of current neurosurgical options. PATIENTS AND METHODS: The study included patients with Currarino syndrome who presented to our pediatric surgery department during the period 2010 through 2016. The 'sacral scimitar' in plain X-ray provided the clue for the diagnosis; while MRI examination was essential to define the nature of the presacral mass and associated spinal anomalies...
December 27, 2016: Journal of Pediatric Surgery
https://www.readbyqxmd.com/read/28057877/previous-miscarriages-and-gli2-are-associated-with-anorectal-malformations-in-offspring
#3
Romy van de Putte, Charlotte H W Wijers, Ivo de Blaauw, Carlo L M Marcelis, Cornelius E J Sloots, Alice S Brooks, Paul M A Broens, Nel Roeleveld, Loes F M van der Zanden, Iris A L M van Rooij
STUDY QUESTION: Are anorectal malformations (ARMs) associated with previous miscarriages or single nucleotide polymorphisms (SNPs) in the Bone Morphogenetic Protein 4 (BMP4) and GLI family zinc finger 2 (GLI2) genes? SUMMARY ANSWER: The SNP rs3738880 in GLI2 and miscarriages were associated with ARM, especially in patients with multiple congenital anomalies (MCA). WHAT IS KNOWN ALREADY: ARM are one of the most common birth defects of the gastrointestinal tract...
January 5, 2017: Human Reproduction
https://www.readbyqxmd.com/read/28051043/experience-with-neonatal-hydrometrocolpos-in-the-niger-delta-area-of-nigeria-upsurge-or-increased-recognition
#4
Philemon Ekemenye Okoro, C Obiorah, C E Enyindah
BACKGROUND: Hydrometrocolpos (HMC) is a clinical condition in which there is a cystic distension of the vagina (hydrocolpos), uterus and sometimes, fallopian tubes (hydrosalpinx) with fluid. This study described our experience with cases of HMC seen in our practice, and highlighted the flare in our practice. PATIENTS AND METHODS: A retrospective study of cases of HMC managed at the University of Port Harcourt Teaching Hospital between September 2010 and August 2012...
October 2016: African Journal of Paediatric Surgery: AJPS
https://www.readbyqxmd.com/read/28018799/caudal-duplication-syndrome-the-vital-role-of-a-multidisciplinary-approach-and-staged-correction
#5
Inbal Samuk, Marc Levitt, Elena Dlugy, Dragan Kravarusic, David Ben-Meir, Gustavo Rajz, Osnat Konen, Enrique Freud
Caudal duplication syndrome is a rare entity that describes the association between congenital anomalies involving caudal structures and may have a wide spectrum of clinical manifestations. A full-term male presented with combination of anomalies including anorectal malformation, duplication of the colon and lower urinary tract, split of the lower spine, and lipomyelomeningocele with tethering of the cord. We report this exceptional case of caudal duplication syndrome with special emphasis on surgical strategy and approach combining all disciplines involved...
December 2016: European Journal of Pediatric Surgery Reports
https://www.readbyqxmd.com/read/27928719/correlation-between-quality-of-life-and-functional-outcomes-in-operated-children-with-anorectal-malformations-using-the-krickenbeck-consensus
#6
V Shankar Raman, Sandeep Agarwala, Veereshwar Bhatnagar
OBJECTIVE: To correlate the functional outcomes in children operated for anorectal malformations (ARM) using the Krickenbeck consensus and their quality of life (QOL). METHODS: Thirty-three children operated at a tertiary care Pediatric surgery centre were studied 2 y after completion of all the surgeries and if more than 3 y of age. The functional stooling outcomes, type of anomalies and surgical procedures were tabulated using the Krickenbeck classification. The QOL questionnaire consisted of five parameters (Social habit, school attendance, daily activity, relation to peers and feeling) and the scoring ranged between 0 and 12: Good (8-12), Fair (5-7) and Poor (0-4)...
December 8, 2016: Indian Journal of Pediatrics
https://www.readbyqxmd.com/read/27915340/2q33-1q34-deletion-in-a-girl-with-brain-anomalies-and-anorectal-malformation
#7
Luisa Ronzoni, Antonio Novelli, Giulia Brisighelli, Angela Peron, Fabio Triulzi, Vera Bianchi, Ernesto Leva, Maria F Bedeschi
2q33 deletions are considered to constitute a distinct clinical entity (Glass syndrome or 2q33 microdeletion syndrome) with a characteristic phenotype. Most patients have moderate to severe developmental delay, speech delay, a particular behavioural phenotype, feeding problems, growth restriction, a typical facial appearance, thin and sparse hair, tooth abnormalities, and skeletal anomalies. Here, we report on a patient with a 2q33.1q34 deletion spanning 8.3 Mb of genomic DNA. Although her clinical features are very reminiscent of the 2q33 microdeletion syndrome, she also presented with brain and anorectal malformations...
2016: Cytogenetic and Genome Research
https://www.readbyqxmd.com/read/27896167/imperforate-anus-with-jejunal-atresia-complicated-by-intestinal-volvulus-a-case-report
#8
Hae Soo Joung, Alexandra Leon Guerrero, Sandra Tomita, Keith A Kuenzler
Anorectal malformations (ARMs) commonly co-occur with other congenital anomalies, particularly VACTERL (vertebral, anorectal, cardiac, tracheal, esophageal, renal, limb, and duodenal) associations. However, this collection of associations is not comprehensive, and other concurrent anomalies may exist that can be missed during the standard work-up of patients with ARMs. We present a rare case of a neonate with a low ARM with concurrent jejuno-ileal atresia that was diagnosed after the correction of the ARM when the patient developed segmental volvulus...
October 2016: Journal of Neonatal Surgery
https://www.readbyqxmd.com/read/27896163/varied-presentation-of-congenital-segmental-dilatation-of-the-intestine-in-neonates-report-of-three-cases
#9
Binod Kumar Rai, Bilal Mirza, Imran Hashim, Muhammad Saleem
Congenital segmental dilatation (CSD) of the intestine is a rare developmental anomaly characterized by sharply demarcated dilatation of a gastrointestinal segment and may present with intestinal obstruction. We report three cases of CSD of the intestine in neonates with varied presentation. First patient was mistaken as pneumoperitoneum on abdominal radiograph, which led to initial abdominal drain placement. The 2nd patient was a case of anorectal malformation associated with congenital pouch colon (CPC) and CSD of ileum; and the third case presented as neonatal intestinal obstruction and found to have CSD of ileum...
October 2016: Journal of Neonatal Surgery
https://www.readbyqxmd.com/read/27896158/congenital-duodenal-obstruction-in-neonates-over-13-years-experience-from-a-single-centre
#10
Parveen Kumar, Chiranjiv Kumar, Prince Raj Pandey, Yogesh Kumar Sarin
AIM: To study the prevalence of associated anomalies with neonatal duodenal obstruction and factors impacting short-term survival. MATERIAL AND METHODS: Records of 31 neonates with neonatal duodenal obstruction could be retrieved and analyzed for a 13.5-year-period (October 2003-May 2016). M:F ratio was 1.58:1. The mean birth weight was 2.15 kg; 12 patients were preterm. Etiologies included duodenal atresia (n=23), duodenal web (n=8) and malrotation of gut (n= 6)...
October 2016: Journal of Neonatal Surgery
https://www.readbyqxmd.com/read/27896151/alimentary-tract-atresias-associated-with-anorectal-malformations-10-years-experience
#11
Manoj Saha
Anorectal malformation (ARM) is one of the most common congenital anomaly that requires emergency surgery in the neonatal period. ARMs are frequently associated with other life threatening congenital anomalies. Commonly associated anomalies are genito-urinary, cardiovascular, gastro-intestinal, skeletal and spinal. Alimentary tract anomalies are frequently masked by the intestinal obstruction produced by the anorectal atresia. This retrospective study was carried out to find out the incidence of associated alimentary tract atresias with ARM...
October 2016: Journal of Neonatal Surgery
https://www.readbyqxmd.com/read/27887934/currarino-syndrome-in-a-fetus-infant-child-and-adolescent-spectrum%C3%A2-of-clinical-presentations-and-imaging-findings
#12
REVIEW
Pablo Caro-Domínguez, Juan Bass, Julie Hurteau-Miller
In 1981, Currarino et al described a triad of findings that consist of partial sacral dysgenesis, presacral mass (anterior meningocele, enteric cyst, or presacral teratoma) and anorectal malformation. Currarino syndrome exhibits variable expressivity and the clinical presentation tends to vary with the age of the subject such as spinal anomaly detected in the fetus, imperforate anus in the newborn, and intractable constipation or neurologic symptoms in the infant and older child. At any age, meningitis can be the presenting symptom and imaging is required for proper investigation...
November 22, 2016: Canadian Association of Radiologists Journal, Journal L'Association Canadienne des Radiologistes
https://www.readbyqxmd.com/read/27849232/long-term-evaluation-of-fecal-continence-and-quality-of-life-in-patients-operated-for-anorectal-malformations
#13
Ana Cristina Aoun Tannuri, Mariana Aparecida Elisei Ferreira, Arthur Loguetti Mathias, Uenis Tannuri
Introduction: Patients operated for correction of anorectal malformations (ARM) can develop fecal incontinence, constipation, and soiling, with loss in quality of life. Objective: To evaluate, through the use of questionnaires, fecal continence, and quality of life of children in the late postoperative follow-up of ARM correction, both high and low. In addition, the levels of fecal continence and quality of life were compared with those of a control group. Method: A Fecal Continence Index Questionnaire (ICF) and a Questionnaire for Assessment of Quality of Life Related to Fecal Continence in Children and Adolescents (QQVCFCA) were administered to 63 patients with ARM, aged from 7 to 19 years, whose surgical treatment had been completed for at least 6 months...
September 2016: Revista da Associação Médica Brasileira
https://www.readbyqxmd.com/read/27761181/currarino-syndrome-and-the-effect-of-a-large-anterior-sacral-meningocele-on-distal-colostogram-in-an-anorectal-malformation
#14
Jason K Lee, Alexander J Towbin
Currarino syndrome is a congenital disorder, consisting of a triad of anomalies including an anorectal malformation, sacral anomaly, and a presacral mass. Anterior sacral meningoceles are the most common presacral mass. A young child presented to our institution with an unrepaired anorectal malformation and a large anterior sacral meningocele. We describe how the anterior meningocele affected the imaging work-up.
June 2016: Journal of Radiology Case Reports
https://www.readbyqxmd.com/read/27759786/laparoscopically-assisted-anorectoplasty-and-the-use-of-the-bipolar-device-to-seal-the-rectal-urinary-fistula
#15
Robson Azevedo Dutra, Adriana Cartafina Perez Boscollo
Background: The anorectal anomalies consist in a complex group of birth defects. Laparoscopic-assisted anorectoplasty improved visualization of the rectal fistula and the ability to place the pull-through segment within the elevator muscle complex with minimal dissection. There is no consensus on how the fistula should be managed. Aim: To evaluate the laparoscopic-assisted anorectoplasty and the treatment of the rectal urinary fistula by a bipolar sealing device...
July 2016: Arquivos Brasileiros de Cirurgia Digestiva: ABCD, Brazilian Archives of Digestive Surgery
https://www.readbyqxmd.com/read/27710988/outcome-of-loop-versus-divided-colostomy-in-the-management-of-anorectal-malformations
#16
Osama Ibrahim Almosallam, Ali Aseeri, Saud Al Shanafey
BACKGROUND: Colostomy is a common part of the management of high anorectal malformation (ARM) in the pediatric population. OBJECTIVE: To evaluate whether the type of colostomy (loop vs divided) has an impact on outcome in patients with ARM. DESIGN: A retrospective study. SETTING: King Faisal Specialist Hospital and Research Center, a tertiary care center. PATIENTS AND METHODS: All patients who were managed with colostomy for ARM and had definitive repair during the period of January 2000 to December 2014...
September 2016: Annals of Saudi Medicine
https://www.readbyqxmd.com/read/27695999/diversities-of-h-type-anorectal-malformation-a-systematic-review-on-a-rare-variant-of-the-krickenbeck-classification
#17
REVIEW
Shilpa Sharma, Devendra K Gupta
Congenital H-type fistula is a rare congenital rectourogenital connection with an external anal opening in a normal or ectopic position. A systematic review was done to study the anatomical types of congenital H-type fistula, embryology, clinical presentation, relative gender distribution, associated anomalies, investigative modalities, and recent advances in treatment of these lesions. A PubMed search included H-type anorectal malformation; H-type anorectal malformations; H-type anorectal; and H-type congenital anorectal that gave 9;43;76;26 abstracts, respectively...
October 1, 2016: Pediatric Surgery International
https://www.readbyqxmd.com/read/27695213/currarino-syndrome-rare-clinical-variants
#18
Bindey Kumar, Amit Kumar Sinha, Prem Kumar, Anil Kumar
Currarino syndrome (CS) is a rare clinical condition. The classical presentation includes a triad of sacral anomaly, anorectal malformations, and presacral mass. This syndrome belongs to the group of persistent neuroenteric malformations. This article presents two cases of Currarino syndrome, where there was rare clinical variants such as rectal atresia in the first case and rectal stenosis in the second case. The clinical presentations were very deceptive as the first case presented as high anorectal malformation and the second case was simulating Hirschprung's disease...
October 2016: Journal of Indian Association of Pediatric Surgeons
https://www.readbyqxmd.com/read/27686585/congenital-anorectal-malformation-severity-does-not-predict-severity-of-congenital-heart-defects
#19
Jara E Jonker, Eryn T Liem, Nynke J Elzenga, Bouwe Molenbuur, Monika Trzpis, Paul M A Broens
OBJECTIVE: To determine the prevalence of congenital heart defects (CHDs) in patients with mild or severe congenital anorectal malformations (CARMs), and whether all patients with CARM need pediatric cardiology screening. STUDY DESIGN: We included 129 patients with CARM born between 2004 and 2013, and referred to University Medical Center Groningen. Recto-perineal and recto-vestibular fistulas were classified as mild CARMs, all others as severe. Significant patent foramen ovale, secundum atrial septal defect, and small ventricular septum defect were classified as minor CHDs, all others as major...
September 26, 2016: Journal of Pediatrics
https://www.readbyqxmd.com/read/27595629/-assessment-before-surgical-treatment-for-pelvic-organ-prolapse-clinical-practice-guidelines
#20
L Donon, S Warembourg, J-F Lapray, A Cortesse, J-F Hermieu, B Fatton, M Cayrac, X Deffieux, M Geraud, L Le Normand
INTRODUCTION: The issue addressed in this chapter of recommendations is: What is the clinical and para-clinical assessment to achieve in women with genital prolapse and for whom surgical treatment has been decided. What are the clinical elements of the examination that must be taken into account as a risk factor of failure or relapse after surgery, in order to anticipate and evaluate possible surgical difficulties, and to move towards a preferred surgical technique? MATERIAL AND METHODS: This work is based on a systematic review of the literature (PubMed, Medline, Cochrane Library, Cochrane Database of Systemactic Reviews, EMBASE) for meta-analyzes, randomized trials, registries, literature reviews, controlled studies and major not controlled studies, published on the subject...
July 2016: Progrès en Urologie
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