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Congenital surgical malformation

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https://www.readbyqxmd.com/read/27882508/regional-early-development-and-eruption-of-permanent-teeth-case-report
#1
A M Al Mullahi, A Bakathir, S Al Jahdhami
BACKGROUND: Early development and eruption of permanent teeth are rarely reported in scientific literature. Early eruption of permanent teeth has been reported to occur due to local factors such as trauma or dental abscesses in primary teeth, and in systemic conditions. Congenital diffuse infiltrating facial lipomatosis (CDIFL) is a rare condition that belongs to a group of lipomatosis tumours. In this disorder, the mature adipocytes invade adjacent soft and hard tissues in the facial region...
November 23, 2016: European Archives of Paediatric Dentistry: Official Journal of the European Academy of Paediatric Dentistry
https://www.readbyqxmd.com/read/27878457/poorly-understood-and-often-miscategorized-congenital-umbilical-cord-hernia-an-alternative-repair-method
#2
E İnce, A Temiz, S S Ezer, H Ö Gezer, A Hiçsönmez
PURPOSE: Umbilical cord hernia is poorly understood and often miscategorized as "omphalocele minor". Careless clamping of the cord leads to iatrogenic gut injury in the situation of umbilical cord hernia. This study aimed to determine the characteristics and outcomes of umbilical cord hernias. We also highlight an alternative repair method for umbilical cord hernias. METHODS: We recorded 15 cases of umbilical cord hernias over 10 years. The patients' data were retrospectively reviewed, and preoperative preparation of the newborn, gestational age, birth weight, other associated malformations, surgical technique used, enteral nutrition, and length of hospitalization were recorded...
November 22, 2016: Hernia: the Journal of Hernias and Abdominal Wall Surgery
https://www.readbyqxmd.com/read/27863630/comparison-of-treatment-outcomes-between-intracapsular-and-total-tonsillectomy-for-pediatric-obstructive-sleep-apnea
#3
David T Chang, Allison Zemek, Peter J Koltai
BACKGROUND: Intracapsular tonsillectomy (IT) has been advocated as a treatment for pediatric obstructive sleep apnea (OSA). However, evidence in the literature utilizing polysomnography (PSG) is limited. OBJECTIVE: To examine the experience at a tertiary children's hospital to evaluate the effectiveness and risks of intracapsular tonsillectomy compared to total tonsillectomy (TT) for treating pediatric OSA. METHODS: A retrospective study was undertaken of pediatric tonsillectomy cases performed for OSA at a tertiary children's hospital from 2005 to 2010...
December 2016: International Journal of Pediatric Otorhinolaryngology
https://www.readbyqxmd.com/read/27861710/endometriosis-and-uterine-malformations-infertility-may-increase-severity-of-endometriosis
#4
Jeremy Boujenah, Eleonora Salakos, Mélodie Pinto, Joanna Shore, Christophe Sifer, Christophe Poncelet, Alexandre Bricou
INTRODUCTION: The aim of our study was to compare the stage and severity of endometriosis in fertile and infertile women with congenital uterine malformations. MATERIAL AND METHODS: We performed an observational study from September 2007 to December 2015 in a tertiary care university hospital and assisted reproductive technology center. A total of 52 patients with surgically proven uterine malformations were included. We compared 41 infertile patients with uterine malformations with 11 fertile patients with uterine malformation...
October 13, 2016: Acta Obstetricia et Gynecologica Scandinavica
https://www.readbyqxmd.com/read/27861502/abnormal-profiles-of-local-functional-connectivity-proximal-to-focal-cortical-dysplasias
#5
René M H Besseling, Jacobus F A Jansen, Anton J A de Louw, Mariëlle C G Vlooswijk, M Christianne Hoeberigs, Albert P Aldenkamp, Walter H Backes, Paul A M Hofman
INTRODUCTION: Focal cortical dysplasia (FCD) is a congenital malformation of cortical development that often leads to medically refractory epilepsy. Focal resection can be an effective treatment, but is challenging as the surgically relevant abnormality may exceed the MR-visible lesion. The aim of the current study is to develop methodology to characterize the profile of functional connectivity around FCDs using resting-state functional MRI and in the individual patient. The detection of aberrant connectivity may provide a means to more completely delineate the clinically relevant lesion...
2016: PloS One
https://www.readbyqxmd.com/read/27859296/slide-tracheoplasty-outcomes-in-children-with-congenital-pulmonary-malformations
#6
Michael A DeMarcantonio, Catherine K Hart, Christina J Yang, Meredith Tabangin, Michael J Rutter, Roosevelt Bryant, Peter B Manning, Alessandro de Alarcón
OBJECTIVES/HYPOTHESIS: Evaluate and compare surgical outcomes of slide tracheoplasty for the treatment of congenital tracheal stenosis in children with and without pulmonary malformations. STUDY DESIGN: Retrospective chart review at a tertiary care pediatric medical center. METHODS: We identified patients with tracheal stenosis who underwent slide tracheoplasty from 2001 to 2014, and a subset of these patients who were diagnosed with congenital pulmonary malformations...
November 15, 2016: Laryngoscope
https://www.readbyqxmd.com/read/27834764/vascular-ring-diagnosis-and-management-notable-trends-over-25-years
#7
William N Evans, Ruben J Acherman, Michael L Ciccolo, Sergio A Carrillo, Gary A Mayman, Carlos F Luna, Robert C Rollins, William J Castillo, Humberto Restrepo
BACKGROUND: Vascular rings (VRs) are recognized as uncommon but not rare cardiovascular malformations. METHODS: We analyzed data from all patients born in Southern Nevada, who underwent diagnosis and management of VR from 1990 to 2015, RESULTS: From 1990 to 2015, a total of 92 patients were diagnosed prenatally and postnatally. Of the 92 patients, 73 (79%) had right aortic arch and aberrant left subclavian artery (RAA-ALS) with a left ductus arteriosus or ligamentum, 17 (19%) had a double aortic arch (DAA), and 2 (2%) had a pulmonary artery sling...
November 2016: World Journal for Pediatric & Congenital Heart Surgery
https://www.readbyqxmd.com/read/27833903/virtual-3d-modeling-of-airways-in-congenital-heart-defects
#8
Simone Speggiorin, Saravanan Durairaj, Branko Mimic, Antonio F Corno
The involvement of the airway is not uncommon in the presence of complex cardiovascular malformations. In these cases, a careful inspection of the relationship between the airway and the vasculature is paramount to plan the surgical procedure. Three-dimensional printing enhanced the visualization of the cardiovascular structure. Unfortunately, IT does not allow to remove selected anatomy to improve the visualization of the surrounding ones. Computerized modeling has the potential to fill this gap by allowing a dynamic handling of different anatomies, increasing the exposure of vessels or bronchi to show their relationship...
2016: Frontiers in Pediatrics
https://www.readbyqxmd.com/read/27800095/-antenatal-diagnosis-and-management-of-ileal-atresia
#9
Hanane Dhibou, Ahlam Bassir, Nadia Sami, Lahcen Boukhanni, Bouchra Fakhir, Hamid Asmouki, Abderraouf Soummani
Ileal atresia is a rare congenital malformation which affects a small percentage of the population, with an incidence of 1 in 5000 cases. It may be suspected and diagnosed by ultrasound at the end of the second and third trimester. Obstetrical and surgical consultation is the key to success here. Eliminating a systemic disease with poor prognosis, fighting to reduce premature births and immediately entrusting the child to the surgeon are the main objectives to achieve. During surgery, the surgeon will determine the type of atresia, its location, single or multiple areas of occlusion and its length; thus surgery depends on etiology...
2016: Pan African Medical Journal
https://www.readbyqxmd.com/read/27785331/congenital-esophageal-duplication-cyst-a-rare-cause-of-dysphagia-in-an-adult
#10
Nikhil Sonthalia, Samit S Jain, Ravindra G Surude, Ashok R Mohite, Pravin M Rathi
Esophageal duplication cyst is a rare congenital embryonal gastrointestinal (GI) malformation which is diagnosed most commonly in childhood. In adults, they can present with a variety of symptoms ranging from dysphagia, chest pain, epigastric discomfort, and vomiting to more serious complications including infections, hemorrhage, and ulcerations. A 30-year-old male presented with gradually progressive dysphagia to solids for 4 months without significant weight loss. Clinical examination and routine laboratory examination were unrevealing...
October 2016: Gastroenterology Research
https://www.readbyqxmd.com/read/27775296/-preaxial-polydactyly-of-the-hand-15-years-of-experience
#11
Y D Pacheco, C Lorca-García, B Berenguer, E De Tomás
INTRODUCTION: Preaxial polydactyly is one of the most common congenital malformations of the hand. The treatment is surgical and should be done early, between 6 and 12 months old. The purpose of this paper is to review our experience in terms of casuistry, treatment and functional and aesthetic results of duplicity of thumb, since 2000 until today. MATERIAL AND METHODS: A retrospective study of patients with diagnosis of preaxial polydactyly from 2000 to january 2016 was performed...
October 10, 2015: Cirugía Pediátrica: Organo Oficial de la Sociedad Española de Cirugía Pediátrica
https://www.readbyqxmd.com/read/27752213/comprehensive-treatment-of-upper-lip-arteriovenous-malformation
#12
Mohammad Jafarian, Nima Dehghani, Shahin Shams, Mohammad Esmaeelinejad, Farzad Aghdashi
Arteriovenous malformations are uncommon congenital disorders in vascular development. They frequently involve craniofacial structures and result in a morphogenic abnormality with ominous arteriovenous shunting. We present a huge AVM of the upper lip in an 18-year-old patient who was successfully treated by the combination method of presurgical endovascular embolization and complete resection of the lesion. Subsequent surgical defect in upper lip, which involved more than two-third of the lip length, was reconstructed via Webster's modification of cheek advancement flap...
September 2016: Journal of Maxillofacial and Oral Surgery
https://www.readbyqxmd.com/read/27751833/perineal-ultrasound-offers-useful-information-in-girls-with-congenital-adrenal-hyperplasia
#13
J Lindert, O Hiort, L Tüshaus, K Tafazzoli-Lari, L Wünsch
A variable spectrum of urogenital malformations exists in girls with congenital adrenal hyperplasia (CAH). The vagina may enter the urethra at a variable level, and relations to the sphincter complex vary accordingly. Furthermore, an enlarged clitoris and variations in the bladder sphincter anatomy can be found. Endoscopy, genitography or magnetic resonance imaging (MRI) are commonly used for the assessment of these anomalies, and to provide information for counselling and treatment. When surgery is planned, introitoplasty cosmetical reduction of the clitoris and labioplasty are discussed with the families; introitoplasty is the most demanding aspect...
September 10, 2016: Journal of Pediatric Urology
https://www.readbyqxmd.com/read/27750489/isolated-cleft-palate-requires-different-surgical-protocols-depending-on-cleft-type
#14
Anna Elander, Christina Persson, Jan Lilja, Hans Mark
A staged protocol for isolated cleft palate (CPO), comprising the early repair of the soft palate at 6 months and delayed repair of the eventual cleft in the hard palate until 4 years, designed to improve maxillary growth, was introduced. CPO is frequently associated with additional congenital conditions. The study evaluates this surgical protocol for clefts in the soft palate (CPS) and for clefts in the hard and soft palate (CPH), with or without additional malformation, regarding primary and secondary surgical interventions needed for cleft closure and for correction of velopharyngeal insufficiency until 10 years of age...
October 18, 2016: Journal of Plastic Surgery and Hand Surgery
https://www.readbyqxmd.com/read/27748336/clinical-characteristics-of-patients-who-underwent-surgery-for-genital-tract-malformations-at-peking-union-medical-college-hospital-across-31-years
#15
Guang-Han Wang, Lan Zhu, Ai-Ming Liu, Tao Xu, Jing-He Lang
BACKGROUND: Female genital malformations represent miscellaneous deviations from normal anatomy. This study aimed to explore the clinical characteristics of patients who underwent surgery for genital tract malformations at Peking Union Medical College Hospital (PUMCH) during a 31-year period. METHODS: We retrospectively reviewed surgical cases of congenital malformation of the female genital tract at PUMCH for a 31-year period, analyzed the clinical characteristics of 1634 hospitalized patients, and investigated their general condition, diagnosis, and treatment process...
2016: Chinese Medical Journal
https://www.readbyqxmd.com/read/27747125/gallbladder-volvulus-in-a-patient-with-type-i-choledochal-cyst-a-case-report-and-review-of-the-literature
#16
George Younan, Max Schumm, Fadwa Ali, Kathleen K Christians
Introduction. Gallbladder volvulus is a rare, potentially fatal condition unless diagnosed and treated early. Choledochal cysts are rare congenital malformations of the biliary tree predisposing to different pathologies and posing the risk of degradation into cholangiocarcinoma and gallbladder cancer. Dealing with both diseases at once has not been published yet in the literature. Presentation of Case. We report a case of gallbladder volvulus in an elderly female who happened to have a concomitant type I choledochal cyst...
2016: Case Reports in Surgery
https://www.readbyqxmd.com/read/27747032/unilateral-absence-of-pulmonary-artery-associated-with-contralateral-lung-cancer
#17
Liang-Ze Zhang, Wei-Guo Ma, Shu-Geng Gao, Jie He
Unilateral absence of a pulmonary artery (UAPA) is a rare congenital cardiac malformation that is often associated with other cardiovascular deformities. Surgical repair of this rare condition is usually performed only on the abnormal lung. The occurrence of lung cancer in association with UAPA is even rarer and clinical experience is very limited. This report aims to describe a case of unilateral absence of right pulmonary artery that was complicated by primary carcinoma of the contralateral lung. A left lower lobectomy was performed despite the absence of the right pulmonary artery and repeated decreases in the arterial oxygen saturation (SaO2) were encountered intraoperatively...
September 2016: Journal of Thoracic Disease
https://www.readbyqxmd.com/read/27742239/the-significance-of-the-interleaflet-triangles-in-determining-the-morphology-of-congenitally-abnormal-aortic-valves-implications-for-noninvasive-imaging-and-surgical-management
#18
Justin T Tretter, Diane E Spicer, Shumpei Mori, Sathish Chikkabyrappa, Andrew N Redington, Robert H Anderson
A comprehensive understanding of the normal and abnormal aortic root is paramount if we are to improve not only our assessment of the aortic root and its components but also the surgical approach to reconstructing this complex structure when congenitally malformed. Most anatomic and imaging-based classifications of the normal root recognize and describe the basic components, which include the shape and size of the three aortic sinuses and their three valvar leaflets, as well as the sinutubular junction and proximal ascending aorta...
October 11, 2016: Journal of the American Society of Echocardiography
https://www.readbyqxmd.com/read/27737328/long-term-follow-up-of-a-female-with-congenital-adrenal-hyperplasia-due-to-p450-oxidoreductase-deficiency
#19
Beatriz D S F Bonamichi, Stella L M Santiago, Débora R Bertola, Chong A Kim, Nivaldo Alonso, Berenice B Mendonca, Tania A S S Bachega, Larissa G Gomes
P450 oxidoreductase deficiency (PORD) is a variant of congenital adrenal hyperplasia that is caused by POR gene mutations. The POR gene encodes a flavor protein that transfers electrons from nicotinamide adenine dinucleotide phosphate (NADPH) to all microsomal cytochrome P450 type II (including 21-hydroxylase, 17α-hydroxylase 17,20 lyase and aromatase), which is fundamental for their enzymatic activity. POR mutations cause variable impairments in steroidogenic enzyme activities that result in wide phenotypic variability ranging from 46,XX or 46,XY disorders of sexual differentiation, glucocorticoid deficiency, with or without skeletal malformations similar to Antley-Bixler syndrome to asymptomatic newborns diagnosed during neonatal screening test...
October 2016: Archives of Endocrinology and Metabolism
https://www.readbyqxmd.com/read/27729302/unusual-association-between-spontaneous-lateral-sphenoid-encephalocele-and-chiari-malformation-type-i-endoscopic-repair-through-a-transpterygoid-approach
#20
Daniele Starnoni, Roy Thomas Daniel, Mercy George, Mahmoud Messerer
BACKGROUND: Spontaneous meningoencephaloceles of the lateral sphenoid sinus are rare entities and their peculiar location represent a surgical challenge due to the importance of a wide exposure and skull base reconstruction. They are thought to arise from congenital base defect of the lateral sphenoid or in some cases have been postulated to represent a rare manifestation of altered CSF dynamics. We report the first case in literature of a Chiari malformation type I and a lateral sphenoid encephaloceles revising the theoretical etiology and surgical technique of endoscopic repair...
October 8, 2016: World Neurosurgery
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