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Congenital deformity

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https://www.readbyqxmd.com/read/28094564/secondary-cleft-nasolabial-deformities-a-new-classification-system-for-evaluation-and-surgical-revision
#1
Ghassan Abu Sittah, Odette Abou Ghanem, Usama Hamdan, Paul Ramia, Elias Zgheib
INTRODUCTION:   Secondary or residual cleft lip and nasal deformities following primary unilateral or bilateral cleft lip repair are common. Many classification systems have been proposed to describe congenital cleft lip and palate deformities before repair. This article proposes a one-of-a-kind classification system for residual cleft deformities and describes its application to 136 cleft lip revision cases from cleft outreach missions worldwide. METHODS:   Patients' demographics and deformities were classified preoperatively, and a database of the classification was created...
January 17, 2017: Cleft Palate-craniofacial Journal
https://www.readbyqxmd.com/read/28090526/the-necessity-to-restore-the-anatomic-hip-centre-in-congenital-hip-disease
#2
COMMENT
George A Macheras, Panagiotis Lepetsos, Panagiotis P Anastasopoulos, Spyridon P Galanakos
Total hip replacement (THR) is the treatment of choice for the patient suffering from end-stage hip osteoarthritis. In the presence of deformities due to congenital hip disease (CHD), THR is, in most of the cases, a difficult task, since the technique of performing such an operation is demanding and the results could vary. We present our experience and preferred strategies focusing on challenges and surgical techniques associated with reconstructing the dysplastic hip.
December 2016: Annals of Translational Medicine
https://www.readbyqxmd.com/read/28087285/apert-syndrome-a-consensus-on-the-management-of-apert-hands
#3
David A Pettitt, Zeeshaan Arshad, Anuj Mishra, Paul McArthur
INTRODUCTION: Apert Syndrome is a congenital condition characterised by primary craniosynostosis, midfacial malformations and complex symmetrical malformations of the hands and feet. The hands demonstrate one of the most complex collections of congenital upper limb deformities, posing a significant challenge for the paediatric hand surgeon. This study examines the extant literature and current practice of the four UK specialist craniofacial units regarding the management of Apert hands in order to provide a basis for guideline development...
December 10, 2016: Journal of Cranio-maxillo-facial Surgery
https://www.readbyqxmd.com/read/28079273/abnormal-fetal-muscle-forces-result-in-defects-in-spinal-curvature-and-alterations-in-vertebral-segmentation-and-shape
#4
Rebecca A Rolfe, James H Bezer, Tyler Kim, Ahmed Z Zaidon, Michelle L Oyen, James C Iatridis, Niamh C Nowlan
The incidence of congenital spine deformities, including congenital scoliosis, kyphosis and lordosis, may be influenced by the in utero mechanical environment, and particularly by fetal movements at critical time-points. There is a limited understanding of the influence of fetal movements on spinal development, despite the fact that mechanical forces have been shown to play an essential role in skeletal development of the limb. This study investigates the effects of muscle forces on spinal curvature, vertebral segmentation and vertebral shape by inducing rigid or flaccid paralysis in the embryonic chick...
January 12, 2017: Journal of Orthopaedic Research: Official Publication of the Orthopaedic Research Society
https://www.readbyqxmd.com/read/28070867/analytical-modeling-for-computing-lead-stress-in-a-novel-epicardial-micropacemaker
#5
Li Zhou, Yaniv Bar-Cohen, Raymond A Peck, Giorgio V Chirikian, Brett Harwin, Ramen H Chmait, Jay D Pruetz, Michael J Silka, Gerald E Loeb
Implantation and maintenance of a permanent cardiac pacing system in children remains challenging due to small patient size, congenital heart defects and somatic growth. We are developing a novel epicardial micropacemaker for children that can be implanted on the epicardium within the pericardial space via a minimally-invasive technique. The key design configurations include a novel open-coiled lead in which living tissue replaces the usual polymeric support for the coiled conductor. To better understand and be able to predict the behavior of the implanted lead, we performed a radiographic image-based modeling study on a chronic animal test...
January 9, 2017: Cardiovascular Engineering and Technology
https://www.readbyqxmd.com/read/28070823/relationship-between-postoperative-complications-and-fibular-integrity-in-congenital-pseudarthrosis-of-the-tibia-in-children
#6
Yao-Xi Liu, Hai-Bo Mei, Guang-Hui Zhu, Rong-Guo He, Kun Liu, Jin Tang, Jiang-Yan Wu, Wei-Hua Ye, Xin Hu, Qian Tan, An Yan, Sheng-Xiang Huang, Xiao-Qian Tan, Ting Lei
BACKGROUND: This study aimed to investigate the relationship between postoperative complications and fibular integrity in congenital pseudarthrosis of the tibia (CPT) in children. METHODS: A retrospective study was performed in 59 patients with Crawford type IV CPT who were treated with combined surgical technique from 2007 to 2011. The patients were divided into two groups, the CPT with fibular pseudarthrosis (group A) and CPT with intact fibula groups (group B), on the basis of fibula status after the union of CPT...
December 23, 2016: World Journal of Pediatrics: WJP
https://www.readbyqxmd.com/read/28061881/moebius-sequence-a-multidisciplinary-clinical-approach
#7
Line Kjeldgaard Pedersen, Rikke Damkjær Maimburg, Jens Michael Hertz, Hans Gjørup, Thomas Klit Pedersen, Bjarne Møller-Madsen, John Rosendahl Østergaard
BACKGROUND: Moebius Sequence (MS) is a rare disorder defined by bilateral congenital paralysis of the abducens and facial nerves in combination with various odontological, craniofacial, ophthalmological and orthopaedic conditions. The aetiology is still unknown; but both genetic (de novo mutations) and vascular events in utero are reported. The purpose of present study was through a multidisciplinary clinical approach to examine children diagnosed with Moebius-like symptoms. Ten children underwent odontological, ophthalmological, obstetric, paediatric, orthopaedic, genetic, radiological and photographical evaluation...
January 6, 2017: Orphanet Journal of Rare Diseases
https://www.readbyqxmd.com/read/28050536/functional-and-clinical-results-achieved-in-congenital-clubfoot-patients-treated-by-ponseti-s-technique
#8
Pedro Augusto Jaqueto, Guilherme Salgado Martins, Fernando Saddi Mennucci, Cintia Kelly Bittar, José Luís Amim Zabeu
OBJECTIVES: To analyze and evaluate functional and clinical results in patients with congenital clubfoot treated with Ponseti's technique. METHODS: This study evaluated 31 patients diagnosed with 51 congenital clubfeet, treated between April 2006 and September 2011 with Ponseti's technique. The patients who did not achieve an equinus correction with manipulation were treated with Achilles tenotomy. An anterior tibial tendon transfer was performed in patients who maintained residual adduction...
November 2016: Revista Brasileira de Ortopedia
https://www.readbyqxmd.com/read/28050458/congenital-cystic-adenomatoid-malformation-a-tertiary-care-hospital-experience
#9
Asmita Anilkumar Mehta, Naveen Viswanathan, Anil Kumar Vasudevan, Roopa Paulose, Mohan Abraham
INTRODUCTION: Congenital Cystic Adenomatoid Malformation (CCAM) is an uncommon developmental deformity affecting the terminal respiratory structures. It is characterized by broncho pulmonary foregut malformations. The reason behind it is an arrest in lung development between 4(th) and 7(th) week of fetal life. AIM: The present study was conducted to assess the clinical and radiological profile and also to study the role of surgical intervention in patients with CCAM...
November 2016: Journal of Clinical and Diagnostic Research: JCDR
https://www.readbyqxmd.com/read/28045856/a-multiparametric-alarm-criterion-for-motor-evoked-potential-monitoring-during-spine-deformity-surgery
#10
Martín J Segura, María E Talarico, Mariano A Noel
PURPOSE: This is a pilot study to compare changes in the amplitude, area below the curve, number of phases, duration, and latency of the intraoperative transcranial motor evoked potentials (TcMEP) for early detection of impending spinal cord injury. An empirical ratio calculated by a combination of the above-mentioned parameters was also assessed. METHODS: Intraoperative TcMEP recordings from five patients presenting with neuromuscular kyphoscoliosis, idiopathic scoliosis, achondroplasia and lumbar kyphosis, congenital kyphosis, and achondroplasia with cervical instability were reviewed...
January 2017: Journal of Clinical Neurophysiology: Official Publication of the American Electroencephalographic Society
https://www.readbyqxmd.com/read/28041885/a-veil-in-the-oral-cavity-report-of-two-cases-of-oral-synechiae
#11
A Dongol, P Acharya, J N Prasad, M R Jaisani
An oral synechia is an adhesion between the maxilla and the mandible; these adhesions can be found at various locations in the oral cavity. The presence of oral synechiae associated with cleft palate represents a rare congenital deformity. Oral synechiae with cleft palate can have serious implications for airway management and feeding in babies, and hence requires early intervention to reduce morbidity. Two cases of congenital oral synechiae accompanied by cleft palate, in which the synechiae veiled the oral cavity restricting a detailed intraoral examination, are reported here...
December 29, 2016: International Journal of Oral and Maxillofacial Surgery
https://www.readbyqxmd.com/read/28034627/two-dimensional-right-ventricular-strain-by-speckle-tracking-for-assessment-of-longitudinal-right-ventricular-function-after-paediatric-congenital-heart-disease-surgery
#12
Clement Karsenty, Khaled Hadeed, Yves Dulac, Florent Semet, Xavier Alacoque, Sophie Breinig, Bertrand Leobon, Philippe Acar, Sebastien Hascoet
BACKGROUND: Right ventricular (RV) function is a prognostic marker of cardiac disease in children. Speckle tracking has been developed to assess RV longitudinal shortening, the dominant deformation during systole; little is known about its feasibility in children with congenital heart disease (CHD). AIMS: To evaluate the feasibility and reproducibility of RV two-dimensional (2D) strain assessed by speckle tracking in infants undergoing CHD surgery compared with conventional markers...
December 26, 2016: Archives of Cardiovascular Diseases
https://www.readbyqxmd.com/read/28032345/zygomatic-bone-shape-in-intentional-cranial-deformations-a-model-for-the-study-of-the-interactions-between-skull-growth-and-facial-morphology
#13
S Ketoff, F Girinon, S Schlager, M Friess, T Schouman, P Rouch, R H Khonsari
Intentional cranial deformations (ICD) were obtained by exerting external mechanical constraints on the skull vault during the first years of life to permanently modify head shape. The repercussions of ICD on the face are not well described in the midfacial region. Here we assessed the shape of the zygomatic bone in different types of ICDs. We considered 14 non-deformed skulls, 19 skulls with antero-posterior deformation, nine skulls with circumferential deformation and seven skulls with Toulouse deformation...
December 29, 2016: Journal of Anatomy
https://www.readbyqxmd.com/read/28032030/lessons-learned-from-study-of-congenital-hip-disease-in-adults
#14
REVIEW
George Hartofilakidis, Kalliopi Lampropoulou-Adamidou
Orthopaedic surgeons specialising in adult hip reconstruction surgery often face the problem of osteoarthritis secondary to congenital hip disease (CHD). To achieve better communication among physicians, better treatment planning and evaluation of the results of various treatment options, an agreed terminology is needed to describe the entire pathology. Furthermore, a generally accepted classification of the deformities is necessary. Herein, the authors propose the use of the term "congenital hip disease" and its classification as dysplasia, low dislocation and high dislocation...
December 18, 2016: World Journal of Orthopedics
https://www.readbyqxmd.com/read/28027255/evidence-based-medicine-cleft-palate
#15
Albert S Woo
LEARNING OBJECTIVES: After studying this article, the participant should be able to: 1. Describe the incidence of cleft palate and risk factors associated with development of an orofacial cleft. 2. Understand differences among several techniques to repair clefts of both the hard and soft palates. 3. Discuss risk factors for development of postoperative fistulas, velopharyngeal insufficiency, and facial growth problems. 4. Establish a treatment plan for individualized care of a cleft palate patient...
January 2017: Plastic and Reconstructive Surgery
https://www.readbyqxmd.com/read/28025728/semiautomatic-classification-of-acetabular-shape-from-three-dimensional-ultrasound-for-diagnosis-of-infant-hip-dysplasia-using-geometric-features
#16
Abhilash Rakkunedeth Hareendranathan, Dornoosh Zonoobi, Myles Mabee, Chad Diederichs, Kumaradevan Punithakumar, Michelle Noga, Jacob L Jaremko
PURPOSE: Developmental dysplasia of the hip (DDH) is a congenital deformity which in severe cases leads to hip dislocation and in milder cases to premature osteoarthritis. Image-aided diagnosis of DDH is partly based on Graf classification which quantifies the acetabular shape seen at two-dimensional ultrasound (2DUS), which leads to high inter-scan variance. 3D ultrasound (3DUS) is a promising alternative for more reliable DDH diagnosis. However, manual quantification of acetabular shape from 3DUS is cumbersome...
December 26, 2016: International Journal of Computer Assisted Radiology and Surgery
https://www.readbyqxmd.com/read/28012500/familial-diffuse-pulmonary-ossification-a-possible-genetic-disorder
#17
Yoshiaki Kinoshita, Ichiko Mizuguchi, Kouko Hidaka, Hiroshi Ishii, Kentaro Watanabe
Diffuse pulmonary ossification (DPO) is an uncommon disease that is characterized by the widespread formation of ectopic bone in the lungs. Herein, we describe two familial cases of DPO. The patients were a 47-year-old woman and her 76-year-old father. Both patients had a history of recurrent cough, and their chest images showed multiple nodules with ossification. No underlying diseases that might have caused DPO were evident; however, the female patient also had congenital finger hypoplasia and deformity. The present cases indicate the possibility that DPO might be partly associated with genetic disorders...
January 2017: Respiratory Investigation
https://www.readbyqxmd.com/read/28009797/what-s-new-in-congenital-scoliosis
#18
Joshua M Pahys, James T Guille
BACKGROUND: Congenital scoliosis is a failure of vertebral formation, segmentation, or a combination of the 2 arising from abnormal vertebral development during weeks 4 to 6 of gestation. The associated spinal deformity can be of varying severity and result in a stable or progressive deformity based on the type and location of the anomalous vertebra(e). Bracing for congenital scoliosis is rarely indicated, while recent reports have demonstrated the utility of serial derotational casting for longer curves with multiple anomalous vertebrae as an effective "time buying strategy" to delay the need for surgery...
December 22, 2016: Journal of Pediatric Orthopedics
https://www.readbyqxmd.com/read/28006008/osmotic-and-heat-stress-effects-on-segmentation
#19
Julian Weiss, Stephen H Devoto
During vertebrate embryonic development, early skin, muscle, and bone progenitor populations organize into segments known as somites. Defects in this conserved process of segmentation lead to skeletal and muscular deformities, such as congenital scoliosis, a curvature of the spine caused by vertebral defects. Environmental stresses such as hypoxia or heat shock produce segmentation defects, and significantly increase the penetrance and severity of vertebral defects in genetically susceptible individuals. Here we show that a brief exposure to a high osmolarity solution causes reproducible segmentation defects in developing zebrafish (Danio rerio) embryos...
2016: PloS One
https://www.readbyqxmd.com/read/28005737/nasoalveolar-molding-therapy-for-the-treatment-of-unilateral-cleft-lip-and-palate-improves-nasal-symmetry-and-maxillary-alveolar-dimensions
#20
María Gloria Ruíz-Escolano, Adoración Martínez-Plaza, Ricardo Fernández-Valadés, Rosario Cortés-Sánchez, María Angeles Muñoz-Miguelsanz, Eugenio Velasco-Ortega, María Bélen Perez-Ureña, Kamel Matar-Satuf, Antonio José España-López
OBJECTIVE: The aim of this study is to assess the esthetic and morphologic outcomes before surgery using nasoalveolar molding (NAM) therapy in children with unilateral cleft lip and palate. DESIGN: A prospective analysis was performed. SETTING: The study was carried out in the Congenital Malformations Craniofacial and Cleft Lip and Palate Unit, Hospital Virgen de las Nieves, Andalusian Health Service, Granada (Spain). PATIENTS: Twenty consecutively enrolled infants ranging in age from 7 to 30 days with nonsyndromic unilateral cleft lip and palate treated from 2008 to 2012...
November 2016: Journal of Craniofacial Surgery
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