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https://www.readbyqxmd.com/read/27896167/imperforate-anus-with-jejunal-atresia-complicated-by-intestinal-volvulus-a-case-report
#1
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
#2
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
#3
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
#4
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
#5
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
#6
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
#7
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
#8
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
#9
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
#10
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
#11
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
#12
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
#13
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
https://www.readbyqxmd.com/read/27549440/comprehensive-review-of-the-duplication-3q-syndrome-and-report-of-a-patient-with-currarino-syndrome-and-de-novo-duplication-3q26-32-q27-2
#14
G C Dworschak, C Crétolle, A Hilger, H Engels, E Korsch, H Reutter, M Ludwig
Partial duplications of the long arm of chromosome 3, dup(3q), are a rare but well-described condition, sharing features of Cornelia de Lange syndrome. Around two thirds of cases are derived from unbalanced translocations, whereas pure dup(3q) have rarely been reported. Here, we provide an extensive review of the literature on dup(3q). This search revealed several patients with caudal malformations and anomalies, suggesting that caudal malformations or anomalies represent an inherent phenotypic feature of dup(3q)...
August 23, 2016: Clinical Genetics
https://www.readbyqxmd.com/read/27522307/the-importance-of-social-media-for-patients-and-families-affected-by-congenital-anomalies-a-facebook-cross-sectional-analysis-and-user-survey
#15
Robyn Jacobs, Leanne Boyd, Kirsty Brennan, C K Sinha, Stefano Giuliani
BACKGROUND: We aimed to define characteristics and needs of Facebook users in relation to congenital anomalies. METHODS: Cross-sectional analysis of Facebook related to four congenital anomalies: anorectal malformation (ARM), congenital diaphragmatic hernia (CDH), congenital heart disease (CHD) and hypospadias/epispadias (HS/ES). A keyword search was performed to identify relevant Groups/Pages. An anonymous survey was posted to obtain quantitative/qualitative data on users and their healthcare needs...
November 2016: Journal of Pediatric Surgery
https://www.readbyqxmd.com/read/27495810/syndromic-anorectal-malformation-associated-with-holt-oram-syndrome-microcephaly-and-bilateral-corneal-opacity-a-case-report
#16
Usang E Usang, Thomas U Agan, Akan W Inyang, John-Daniel C Emehute, Itam H Itam
BACKGROUND: The occurrence of an anorectal malformation with Holt-Oram syndrome, microcephaly, and bilateral corneal opacity is rare and to the best of our knowledge has not previously been reported in the literature. Hence, there is a need to document our experience in this case and learn as much as possible from it. CASE PRESENTATION: We present the case of a Nigerian female neonate with a postnatal diagnosis of syndromic anorectal malformation associated with Holt-Oram syndrome, microcephaly, and bilateral corneal opacity...
2016: Journal of Medical Case Reports
https://www.readbyqxmd.com/read/27448173/spinal-cord-anomalies-in-patients-with-anorectal-malformations-without-severe-sacral-abnormalities-or-meningomyelocele-outcomes-after-expectant-conservative-management
#17
Kristiina Kyrklund, Mikko P Pakarinen, Seppo Taskinen, Reetta Kivisaari, Risto J Rintala
OBJECTIVE The goal of this study was to determine the significance of spinal cord anomalies (SCAs) in patients with anorectal malformations (ARMs) by comparing the outcomes for bowel function, lower urinary tract symptoms (LUTS), and lower-limb neurological abnormalities to these outcomes in patients with similar ARMs and a normal spinal cord. METHODS The spinal cord MRI records of female patients treated for vestibular and perineal fistula (VF/PF) and male patients with rectourethral fistula (RUF) at a single center between 1983 and 2006 were reviewed...
December 2016: Journal of Neurosurgery. Spine
https://www.readbyqxmd.com/read/27433450/isolated-vaginal-agenesis-associated-with-multiple-gastrointestinal-anomalies-a-case-report
#18
R Angotti, F Molinaro, A L Bulotta, F Ferrara, M Sica, E Bindi, M Messina
More than 50% of infants with esophageal atresia have associated anomalies. We present a case report of a 46XX neonate with long-gap esophageal atresia and tracheoesophageal fistula (EA/TEF), anorectal malformation, bowel duplication and vaginal agenesis. This is an unusual association of abnormalities which had not yet described in literature.
July 2016: Journal of Neonatal Surgery
https://www.readbyqxmd.com/read/27396272/congenital-lateral-abdominal-wall-hernia
#19
Fernando Montes-Tapia, Idalia Cura-Esquivel, Susana Gutiérrez, Isaías Rodríguez-Balderrama, Manuel de la O-Cavazos
Congenital abdominal wall defects that are located outside of the anterior wall are extremely rare and difficult to classify because there are no well accepted guidelines. There are two regions outside of the anterior wall: the flank or lateral wall; and the lumbar region. We report the case of a patient with an oval 3 cm-diameter hernia defect located above the anterior axillary line, which affects all layers of the muscular wall. An anorectal malformation consisting of a recto-vestibular fistula was also identified, and chest X-ray showed dextrocardia...
August 2016: Pediatrics International: Official Journal of the Japan Pediatric Society
https://www.readbyqxmd.com/read/27372296/anorectal-malformations-associated-spinal-cord-anomalies
#20
Giorgia Totonelli, Francesco Morini, Vincenzo Davide Catania, Paolo Maria Schingo, Giovanni Mosiello, Paolo Palma, Barbara Daniela Iacobelli, Pietro Bagolan
PURPOSE: The present study aims to identify clinical and pathological factors that can predict the risk of spinal cord anomalies (SCA) in patients with anorectal malformations (ARM), the need for neurosurgery, and to define the impact of SCA on the outcome of patients with ARM. METHODS: A 16-year retrospective analysis of all patients treated at a single tertiary children's Hospital with diagnosis of ARM. Data were collected to assess the impact of defined clinical characteristics on prevalence of SCA (detected at MRI)...
August 2016: Pediatric Surgery International
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