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Congenital surgical malformation

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https://www.readbyqxmd.com/read/28212403/cerebrospinal-fluid-biomarkers-of-infantile-congenital-hydrocephalus
#1
David D Limbrick, Brandon Baksh, Clinton D Morgan, Gakwaya Habiyaremye, James P McAllister, Terrie E Inder, Deanna Mercer, David M Holtzman, Jennifer Strahle, Michael J Wallendorf, Diego M Morales
INTRODUCTION: Hydrocephalus is a complex neurological disorder with a pervasive impact on the central nervous system. Previous work has demonstrated derangements in the biochemical profile of cerebrospinal fluid (CSF) in hydrocephalus, particularly in infants and children, in whom neurodevelopment is progressing in parallel with concomitant neurological injury. The objective of this study was to examine the CSF of children with congenital hydrocephalus (CHC) to gain insight into the pathophysiology of hydrocephalus and identify candidate biomarkers of CHC with potential diagnostic and therapeutic value...
2017: PloS One
https://www.readbyqxmd.com/read/28209955/-the-complications-of-intestinal-stoma-in-children
#2
S V Minaev, N I Bykov, A V Isaeva, A V Kachanov, E A Tovkan, N V Filip'yeva, I N Gerasimenko
AIM: To investigate the complications of intestinal stoma in children and to develop measures for decrease of their incidence. MATERIAL AND METHODS: The study included 152 children with congenital and acquired gastrointestinal pathology requiring surgical treatment with the imposition of intestinal stoma. Atresia of intestinal tube was observed in 28 (18.4%) children, meconium ileus - in 10 (6.6%) cases, Hirschsprung's disease - in 11 (7.2%)cases, anorectal malformations - in 39 (25...
2017: Khirurgiia
https://www.readbyqxmd.com/read/28208971/a-rare-association-of-pentalogy-of-fallot-with-situs-inversus-totalis-complicated-by-brain-abscess-in-an-adolescent-case-report
#3
Muhammed Basheer, Sunil Kumar Agarwalla
Tetralogy of Fallot (TOF) is the most frequently diagnosed congenital cyanotic heart disease. It is often associated with additional findings, such as atrial septal defect (i.e., pentalogy of Fallot) or right sided aortic arch. Association of this pentalogy of Fallot with situs inversus totalis is rarely reported in paediatric literature and it can cause technical challenges to intracardiac repair. We report the case of pentalogy of Fallot with dextrocardia and situs inversus presenting as parieto-occipital abscess in a 12-year-old child...
December 2016: Journal of Clinical and Diagnostic Research: JCDR
https://www.readbyqxmd.com/read/28204920/a-review-of-congenital-lung-malformations-with-a-simplified-classification-system-for-clinical-and-research-use
#4
REVIEW
Michael Seear, Jennifer Townsend, Amy Hoepker, Douglas Jamieson, Deborah McFadden, Patrick Daigneault, William Glomb
PURPOSE: Congenital lung abnormalities are rare malformations increasingly detected early by prenatal ultrasound. Whether management of these frequently asymptomatic lesions should be surgical or conservative is an unresolved issue. The necessary prospective studies are limited by the absence of a widely accepted practical classification system. Our aim was to develop a simple, clinically relevant system for classifying and studying congenital lung abnormalities. MATERIALS AND METHODS: We based our proposed grouping on a detailed analysis of clinical, radiological, and histological data from well-documented cases, plus an extensive review of the literature...
February 15, 2017: Pediatric Surgery International
https://www.readbyqxmd.com/read/28201848/new-advances-in-polycystic-liver-diseases
#5
A Santos-Laso, L Izquierdo-Sánchez, P Y Lee-Law, M J Perugorria, M Marzioni, J J G Marin, L Bujanda, J M Banales
Polycystic liver diseases (PLDs) include a heterogeneous group of congenital disorders inherited as dominant or recessive genetic traits; they are manifested alone or in association with polycystic kidney disease. Ductal plate malformation during embryogenesis and the loss of heterozygosity linked to second-hit mutations may promote the dilatation and/or development of a large number (> 20) of biliary cysts, which are the main cause of morbidity in these patients. Surgical procedures aimed to eliminate symptomatic cysts show short-term beneficial effects, but are not able to block the disease progression...
February 2017: Seminars in Liver Disease
https://www.readbyqxmd.com/read/28195886/pedicled-sensate-composite-calcaneal-flap-in-children-with-congenital-tibial-pseudoarthrosis
#6
Mauricio L D Mongon, Fernando C Ribera, Antonio M A de Souza, Aurelio L Sposito, William D Belangero, Bruno Livani
BACKGROUND: The preservation and functionality of a limb affected by a malformation (such as congenital pseudoarthrosis of the tibia) or a severely mangled lower limb in children, despite modern reconstructive techniques, remains challenging, often eventually requiring amputation to achieve a better outcome. The classical Syme and Boyd procedures are functionally better than transtibial (TT) amputation, but are not feasible for congenital tibial pseudoarthrosis. TT amputation delivers an excellent, effective, and functional stump that usually leads, after prosthetization, to a functional gait...
February 10, 2017: Journal of Pediatric Orthopedics
https://www.readbyqxmd.com/read/28192262/intra-operative-ultrasonography-for-definition-of-less-invasive-surgical-technique-in-patients-with-chiari-type-i-malformation
#7
Roger Schmidt Brock, Mario Augusto Taricco, Matheus Fernandes de Oliveira, Marcelo de Lima Oliveira, Manoel Jacobsen Teixeira, Edson Bor-Seng-Shu
INTRODUCTION: Chiari malformation Type I (CM) is the main congenital malformation disease of the craniovertebral junction. The ideal surgical treatment is still controversial. Invasive procedures inside CSF space and associated with dural repair are considered the gold standard, however a less invasive surgery with isolated bone decompression without dural opening may be possible in selected patients.. Our study evaluates the efficacy of intra-operative CSF flow measurement with ultrasonography (USG) as a determining parameter in the selection of these patients...
February 9, 2017: World Neurosurgery
https://www.readbyqxmd.com/read/28181314/congenital-uni-leaflet-mitral-valve-with-severe-stenosis-a-case-report-with-literature-review
#8
Weixin Zhang, Yonghuai Wang, Chunyan Ma, Zhiwei Zhang, Jun Yang
Numerical abnormalities of mitral leaflets is a special entity in congenital mitral malformations. Previously reported cases of uni-leaflet mitral valve were primarily related to absence or dysplasia of certain leaflets. We present a case here with mitral leaflets that were not divided into anterior and posterior as usual, but developed as an integral structure instead, which is different from previously documented cases of uni-leaflet mitral valves. Real time three-dimensional echocardiography (RT3DE) provides a visual presentation of the abnormal mitral structure which was confirmed by surgical operation...
February 9, 2017: Echocardiography
https://www.readbyqxmd.com/read/28180018/case-report-of-four-different-primary-mesenteric-neoplasms-and-review-of-literature
#9
Yusuf Yagmur, Sami Akbulut, Serdar Gumus, Mehmet Babur, Mehmet Ali Can
INTRODUCTION: It is rare for primary tumors to arise from the mesentery. Lymphangiomas appear as congenital malformations of the lymphatic system or benign neoplasms as a large, thin-walled, often multilocular cyst. Mesenteric infiltration is common and during surgical treatment, adjustment of structures such as the bowel and resection of the spleen may be required. Cystic mesotheliomas are rare, benign tumors that originate from the peritoneal mesothelium and are more common in women...
October 2016: Iranian Red Crescent Medical Journal
https://www.readbyqxmd.com/read/28178121/a-case-report-of-congenital-umbilical-arteriovenous-malformation-complicated-with-liver-failure-after-surgical-excision
#10
Ji-Won Han, Hyun-Young Kim, Sung-Eun Jung
RATIONALE: Few case reports of umbilical arteriovenous malformation (AVM) have been reported. Herein, we report a neonatal case of umbilical AVM who underwent liver failure after surgical excision. PATIENT CONCERNS: The patient was a girl delivered at a gestational age of 39+5 weeks showing cyanosis and heart murmur. DIAGNOSES: Cardiac echography, abdominal ultrasonography (USG), and computed tomography revealed suspecting the umbilical AVM...
February 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28178103/bronchogenic-cyst-in-the-head-and-neck-region
#11
Dong Hoon Lee, Tae Mi Yoon, Joon Kyoo Lee, Sang Chul Lim
OBJECTIVE: The objective of this study was to review the clinical characteristics and surgical treatment outcomes of patients with bronchogenic cysts in the head and neck region. METHODS: A retrospective chart review was performed at Chonnam National University Hwasun Hospital from January 2006 to May 2016. RESULTS: Eight patients with a final diagnosis of bronchogenic cyst in the head and neck region were identified based on their medical records...
February 7, 2017: Journal of Craniofacial Surgery
https://www.readbyqxmd.com/read/28169477/localised-intravascular-coagulation-complicating-venous-malformations-in-children-associations-and-therapeutic-options
#12
REVIEW
Kevin Y Zhuo, Susan Russell, Orli Wargon, Susan Adams
Venous malformations are slow-flow congenital vascular malformations that enlarge as the child ages and may be associated with localised intravascular coagulation, a consumptive coagulopathy characterised by elevated D-dimer and decreased fibrinogen levels. The authors review the known correlations between localised intravascular coagulation and venous malformation number, size and planes involved, and call attention to the concept of the progression of localised intravascular coagulopathy as the child ages and their venous malformations enlarge...
February 7, 2017: Journal of Paediatrics and Child Health
https://www.readbyqxmd.com/read/28168619/pure-endoscopic-management-of-epileptogenic-hypothalamic-hamartomas
#13
S Chibbaro, H Cebula, J Scholly, J Todeschi, I Ollivier, A Timofeev, M Ganau, P Di Emidio, M P Valenti, A M Staack, T Bast, B J Steinhoff, E Hirsch, P Kehrli, F Proust
Hypothalamic hamartomas (HH) are rare congenital malformations located in the region of the tuber cinereum and third ventricle. Their usual clinical presentation is characterized by gelastic/dacrystic seizures which often become pharmaco-resistant and progress to secondary focal/generalized intractable epilepsy causing mostly in children cognitive and behavioral problems (particularly in cases of progressive epileptic encephalopathy) and precocious puberty. Whereas gelastic seizures can be surgically controlled either by resection of the lesion or disconnection (tissue-destructive) procedures, aimed at functionally prevent the spreading of the epileptic burst; generalized seizures tend to respond better to HH excision rather than isolated neocortical resections, which generally fail to control them...
February 7, 2017: Neurosurgical Review
https://www.readbyqxmd.com/read/28165443/video-assisted-thoracoscopic-lobectomy-for-congenital-cystic-adenomatoid-malformations
#14
Amina Selimović, Edo Hasanbegović, Ermina Mujičić, Selma Milišić, Emir Haxhija, Kenan Karavdić, Alen Pilav
Aim The aim was to show rare cases of congenital cystic adenomatoid malformation (CCAM) and the manner of its surgical treatment with video-assisted thoracoscopic surgery (VATS). Methods Two male and one female child, 7, 4 and 3 years of age were treated for symptoms of cough and high temperature in district hospitals. In all three children laboratory blood tests and chest radiography were done. Auscultatory findings showed the presence of pneumonia. Children were treated with appropriate doses of antibiotics...
February 1, 2017: Medicinski Glasnik
https://www.readbyqxmd.com/read/28157255/current-views-on-fetal-surgical-treatment-of-myelomeningocele-the-management-of-myelomeningocele-study-moms-trial-and-polish-clinical-experience
#15
Jacek Zamłyński, Ewa Horzelska, Mateusz Zamłyński, Katarzyna Olszak-Wąsik, Leszek Nowak, Piotr Bodzek, Tomasz Horzelski, Rafał Bablok, Anita Olejek
Myelomeningocele (MMC) is the most frequent congenital defect of the central nervous system for which there is no satisfactory alternative to postnatal treatment. On the contrary prenatal MMC surgery is conducting before birth and is aimed at protecting from Chiari II malformation. The main goal of fetal MMC repair is to improve development and life quality of children with Chiari II malformation. Management of Myelomeningocele Study (MOMS) which was published in 2011 clearly confirmed effectiveness of prenatal surgery...
2017: Ginekologia Polska
https://www.readbyqxmd.com/read/28151866/acute-arterial-embolism-of-left-lower-extremity-caused-by-paradoxical-embolism-in-ebstein-s-anomaly-a-case-report
#16
Jun-Sheng Li, Jie Ma, Zi-Xing Yan, Dong-Ming Cheng, Liang Chang, Hai-Chun Zhang, Jiang-Yan Liu
INTRODUCTION: Ebstein's anomaly is a benign and stable congenital heart disease for asymptomatic patients. Despite a low incidence of Ebstein's anomaly (EA), patients' quality of life can be badly affected by EA without positive surgical intervention. Especially EA is associated with other congenital heart disease, such as the atrial septal defect, patent foramen ovale, and arterial embolism exclude other reasons, it is often considered to be the consequence of paradoxical embolism, and surgical intervention must be conducted...
February 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28144070/perpetual-dilemma-pleural-or-parenchymal-congenital-or-acquired-solitary-cystic-lesion-with-fluid-level
#17
Deepak Talwar, Onkar Jha, Rahul Kumar Sharma, Rajat Saxena
Congenital cystic adenomatoid malformations (CCAMs) are rare congenital, nonhereditary developmental anomalies of the lung with unknown etiology. CCAM is predominantly a disorder of infancy with the majority of the cases being diagnosed within the first 2 years of life. When CCAM presents in adults, it represents a diagnostic dilemma and requires careful evaluation. We here report a case of large solitary congenital pulmonary cystic adenomatoid malformation with infection and hemorrhage, which was diagnosed as encysted hydropneumothorax on computerized tomography scans but turned out to be infected pulmonary cystic adenomatoid malformation after surgical excision...
January 2017: Lung India: Official Organ of Indian Chest Society
https://www.readbyqxmd.com/read/28134642/popliteal-pterygium-syndrome-with-syngnathia
#18
Andrea Sisti, Nicola Freda, Alessandro Giacomina, Gian Luca Gatti
Popliteal pterygium syndrome is a condition characterized by skin webs of skin on the popliteal fossa, which may impair mobility unless surgically repaired. Affected individuals may also have syndactyly on the fingers and/or toes. Most people with this disorder present cleft lip and cleft palate and they can have syngnathia, which is a congenital adhesion between maxilla and mandible by fibrous bands, which affects the opening of the mouth. The case that we report is about a 2-month-old male, who presented skin webs bilaterally on the popliteal fossa, syndactyly between the IV and the V toe of the right foot and between the III and the IV toe of the left foot, and genital malformations...
January 27, 2017: Journal of Craniofacial Surgery
https://www.readbyqxmd.com/read/28133602/the-dorsal-mesenchymal-protrusion-and-the-pathogenesis-of-atrioventricular-septal-defects
#19
Tara Burns, Yanping Yang, Emilye Hiriart, Andy Wessels
Congenital heart malformations are the most common type of defects found at birth. About 1% of infants are born with one or more heart defect on a yearly basis. Congenital Heart Disease (CHD) causes more deaths in the first year of life than any other congenital abnormality, and each year, nearly twice as many children die in the United States from CHD as from all forms of childhood cancers combined. Atrioventricular septal defects (AVSD) are congenital heart malformations affecting approximately 1 in 2000 live births...
December 2016: Journal of Cardiovascular Development and Disease
https://www.readbyqxmd.com/read/28132084/pediatric-airway-surgery
#20
REVIEW
Kosaku Maeda
Pediatric airway surgery is a challenging field in pediatric surgery. Laryngotracheal stenosis has a variety of congenital and acquired conditions that require precise assessment and tailored treatment for each individual patient. About 90% of acquired conditions are represented by subglottic stenosis (SGS) resulting as a complication of tracheal intubation. Congenital tracheal stenosis (CTS) is a rare and life-threatening malformation, usually associated with complete tracheal rings along a variable length of the trachea...
January 28, 2017: Pediatric Surgery International
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