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Congenital surgical malformation

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https://www.readbyqxmd.com/read/28650705/current-and-emerging-treatments-for-postsurgical-cleft-lip-scarring-effectiveness-and-mechanisms
#1
E Papathanasiou, C A Trotman, A R Scott, T E Van Dyke
Cleft lip with or without cleft palate is the most common congenital malformation of the head and the third-most common birth defect. Surgical repair of the lip is the only treatment and is usually performed during the first year of life. Hypertrophic scar (HTS) formation is a frequent postoperative complication that impairs soft tissue form, function, or movement. Multiple lip revision operations are often required throughout childhood, attempting to optimize aesthetics and function. The mechanisms guiding HTS formation are multifactorial and complex...
June 1, 2017: Journal of Dental Research
https://www.readbyqxmd.com/read/28645241/long-term-outcome-after-right-ventricle-to-pulmonary-artery-conduit-surgery-and-reintervention
#2
Kristofer Skoglund, Gunnar Svensson, Ulf Thilén, Mikael Dellborg, Peter Eriksson
BACKGROUND: Reconstruction of the right ventricular outflow tract with a conduit is an established surgical procedure in congenital heart disease and reinterventions are common. OBJECTIVE: An increasing number of patients have a conduit, but there are few population-based studies of long-term outcomes after conduit surgery, reoperations, and transcatheter pulmonary valve replacement. METHODS: In April 2015, all adult patients with a conduit were identified in the Swedish National Registry for Congenital Heart Disease (SWEDCON)...
June 23, 2017: Scandinavian Cardiovascular Journal: SCJ
https://www.readbyqxmd.com/read/28642639/foregut-duplication-cysts-in-children
#3
Keshawadhana Balakrishnan, Frances Fonacier, Shilpa Sood, Natasha Bamji, Howard Bostwick, Gustavo Stringel
BACKGROUND AND OBJECTIVES: Duplications of the alimentary tract are rare anomalies. We report our experience with foregut duplication cysts including their clinical presentation, diagnostic modalities, and surgical management. METHODS: We report a 20-year retrospective review of all foregut duplication cysts managed at our institution. RESULTS: Twelve patients with 13 foregut duplication cysts were identified. The ages of the children at the time of surgery ranged from infancy to adolescence, with a mean age of 7...
April 2017: JSLS: Journal of the Society of Laparoendoscopic Surgeons
https://www.readbyqxmd.com/read/28637939/surgery-of-thymic-tumor-with-persistent-left-superior-vena-cava
#4
Masahiro Yanagiya, Jun Matsumoto, Hirotsugu Hashimoto, Yoshio Suzuki, Hajime Horiuchi
Because a persistent left superior vena cava (PLSVC) is a rare congenital malformation in the thoracic venous system, surgery of the thymus in such patients has rarely been reported. We herein present a case involving a 68-year-old woman who was treated for a thymic tumor adhering to a PLSVC. She underwent complete resection of the thymic tumor through median sternotomy with left-sided video-assisted thoracic surgery. Although the tumor was close to the left phrenic nerve, this nerve was safely preserved. The pathological diagnosis was mucosa-associated lymphoid tissue (MALT) lymphoma of the thymus...
June 22, 2017: Annals of Thoracic and Cardiovascular Surgery
https://www.readbyqxmd.com/read/28637106/-a-case-of-hemorrhage-of-an-esophageal-duplication-cyst-improved-by-endoscopic-drainage
#5
In Sub Han, Gwang Ha Kim, Seong Jun Lee, Bong Eun Lee, Hoseok I, Yeong Dae Kim
Esophageal duplication cyst is a rare congenital gastrointestinal malformation. It is the second most common duplication cyst following small bowel duplication cyst in the gastrointestinal tract. Patients with an esophageal duplication cyst are generally asymptomatic; however, some patients may present the following symptoms: dysphagia, chest pain, stridor, unproductive cough, and epigastric discomfort by compression of the surrounding structures. Surgical removal is the treatment of choice in symptomatic cases and can be considered in asymptomatic cases if they are at risk for developing complications, such as ulceration or perforation...
June 25, 2017: Korean Journal of Gastroenterology, Taehan Sohwagi Hakhoe Chi
https://www.readbyqxmd.com/read/28637056/-urosepsis-in-children
#6
Josef Oswald
Urinary tract infections (UTI) are the most common infectious diseases in children. Urosepsis in childhood, though rare, is the most complicated possible variant. In newborns and infants, unspecific symptoms are a significant barrier to a fast and reliable diagnosis, which is crucial to successful treatment. In addition to urine and laboratory tests as well as non-invasive examinations (ultrasound), there may be an indication for invasive examinations of the kidneys (DMSA scans) in cases of a severe infection...
June 21, 2017: Aktuelle Urologie
https://www.readbyqxmd.com/read/28633269/stenting-and-reimplanting-disconnected-pulmonary-artery-in-tetralogy-of-fallot
#7
Harikrishnan K N Kurup, Giedrius Baliulis, Marcus P Haw, Joseph J Vettukattil
Tetralogy of Fallot with absent pulmonary valve syndrome (TOF/APV) is a rare congenital malformation. Although pulmonary artery (PA) anomalies have been observed in TOF, its association with disconnected PA is extremely rare. We report successful stenting of the disconnected left PA in a 3-year-old boy with TOF/APV followed by surgical reimplantation. The significance of this transcatheter intervention for guidance during surgery and the importance of visualizing a ductal stump on angiography as an indicator of disconnected PA are discussed...
July 2017: Annals of Thoracic Surgery
https://www.readbyqxmd.com/read/28631915/-multispiral-computer-tomography-in-differential-diagnosis-of-congenital-uretheroidhydronefrosis-in-children
#8
E B Khakkulov, Zh U Khusankhodzhaev
AIM: To identify the characteristic MSCT signs of pediatric ureterohydronephrosis allowing to assess the disease severity and differentiate between its various forms. MATERIAL AND METHODS: Sixty five children with III-IV grade ureterohydronephrosis underwent a comprehensive examination including MSCT urography. The study comprised 40 (61.5%) boys and 25 (38.5%) girls aged 3 months to 14 years (mean age 4.15+/-3.21 years). RESULTS: Obstructive disease was detected in 38 (58...
June 2017: Urologii︠a︡
https://www.readbyqxmd.com/read/28631675/-the-morphological-and-clinical-aspects-of-the-curvature-of-the-nasal-septum
#9
V N Krasnozhen, D A Shcherbakov, A V Volodeev, L A Musina, Yu A Garskova
The curvature of the nasal septum (NS) is one of the most widespread deformations of the facial skeleton. The objective of the present study was to substantiate the principles of and develop the rationale for the surgical correction and conservative treatment of this condition based on the morphological features of various types of deflection of the nasal septum. We have undertaken the morphological analysis of the osseous and cartilaginous structures determining the type and the shape of the curvature of the nasal septum together with the clinical analysis of different morphological variants of the deflection of the nasal septum making use of the R...
2017: Vestnik Otorinolaringologii
https://www.readbyqxmd.com/read/28614263/misdiagnosed-anomalous-left-coronary-artery-from-the-pulmonary-artery-as-endocardial-fibroelastosis-in-infancy-a-case-series
#10
Fan Ma, Kaiyu Zhou, Xiaoqing Shi, Xiaoqing Wang, Yi Zhang, Yifei Li, Yimin Hua, Chuan Wang
INTRODUCTION: Anomalous left coronary artery from the pulmonary artery (ALCAPA) is a rare but severe congenital cardiac malformation. The prognosis mainly depends on the early and accurate diagnosis and treatment. However, without a typical and specific clinical manifestation in early stage, ALCAPA has a higher rate of false initial diagnosis. DIAGNOSTIC AND THERAPEUTIC PROCEDURE: Three infants with impaired left ventricle (LV) function, LV enlargement, mitral valve regurgitation (MR), and LV endocardium thickness were initially diagnosed as endocardial fibroelastosis (EFE)...
June 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28607884/rigid-bronchoscopic-placement-of-fogarty-catheter-as-a-bronchial-blocker-for-one-lung-isolation-and-ventilation-in-infants-and-children-undergoing-thoracic-surgery-a-single-institution-experience-of-27-cases
#11
Sunil Kant Kamra, Ashwin Ashok Jaiswal, Amrish Kumar Garg, Manoj Kumar Mohanty
One-lung ventilation (OLV) is a challenging task in infants and children as few techniques are possible because of narrow anatomy. The aim of this study is to evaluate and experience lung isolation with Fogarty catheters as a bronchial blocker placed by rigid bronchoscope for OLV in infants and children with lung pathologies requiring surgical management in an industrial hospital. This study is a prospective study carried out in J.L.N. Hospital and Research Centre, Bhilai (CG), from January 2011 to December 2014...
June 2017: Indian Journal of Otolaryngology and Head and Neck Surgery
https://www.readbyqxmd.com/read/28598265/robot-assisted-endoscopic-third-ventriculostomy-institutional-experience-in-9-patients
#12
Reid Hoshide, Mark Calayag, Hal Meltzer, Michael L Levy, David Gonda
OBJECTIVE The endoscopic third ventriculostomy (ETV) is an established and effective treatment for obstructive hydrocephalus. In its most common application, surgeons plan their entry point and the endoscope trajectory for the procedure based on anatomical landmarks, then control the endoscope freehand. Recent studies report an incidence of neural injuries as high as 16.6% of all ETVs performed in North America. The authors have introduced the ROSA system to their ETV procedure to stereotactically optimize endoscope trajectories, to reduce risk of traction on neural structures by the endoscope, and to provide a stable mechanical holder of the endoscope...
June 9, 2017: Journal of Neurosurgery. Pediatrics
https://www.readbyqxmd.com/read/28584070/the-natural-history-of-prenatally-diagnosed-congenital-cystic-lung-lesions-long-term-follow-up-of-119-cases
#13
James Cook, Lyn S Chitty, Paolo De Coppi, Michael Ashworth, Colin Wallis
BACKGROUND: A paucity of evidence regarding the natural history of congenital pulmonary airway malformations (CPAMs) and pulmonary sequestration (PS) has resulted in a divergence in management strategy of asymptomatic cases. METHODS: We describe the long-term clinical course of 119 children diagnosed with these lesions treated at Great Ormond Street Hospital (GOSH). Cases were identified via the GOSH patient database. Study entry required the identification of a cystic lung lesion on prenatal ultrasound and confirmation of CPAM/PS on postnatal CT imaging...
June 5, 2017: Archives of Disease in Childhood
https://www.readbyqxmd.com/read/28582959/supernumerary-nostril-two-years-follow-up
#14
Amir Labib, Ahmed Elshahat
Supernumerary nostril is a very rare congenital anomaly of the nose. Since the first patient reported by Lindsay in 1906, few number of patients were reported in the literature. Various types had been described with different surgical modalities for correction. It can be isolated or associated with other malformations such as facial cleft, esophageal atresia, and imperforate anus. Most of the patients are unilateral, but it may be bilateral. It may have a communication with a normal nasal cavity or not.In this study, the authors present a case of a 1-year-old male with a positive perinatal history of teratogen exposure had isolated supernumerary left nostril with communication to the nasal cavity...
June 2017: Journal of Craniofacial Surgery
https://www.readbyqxmd.com/read/28566821/mid-term-outcomes-of-surgical-repair-for-anomalous-origin-of-the-left-coronary-artery-from-the-pulmonary-artery-in-infants-children-and-adults
#15
Maziar Gholampour Dehaki, Alwaleed Al-Dairy, Yousef Rezaei, Alireza Alizadeh Ghavidel, Gholamreza Omrani, Nader Givtaj, Reza Sadat Afjehi, Hassan Tatari, Amir Hossein Jalali, Mohammad Mahdavi
BACKGROUND: Anomalous origin of left coronary artery from the pulmonary artery (ALCAPA) is a rare congenital malformation. We sought to evaluate in-hospital and mid-term outcomes of patients with a diagnosis of ALCAPA who underwent surgical repair. OBJECTIVES: The objective of this study is to evaluate the mid-term outcomes of surgical repair of ALCAPA at our center and to analyze the surgical techniques used. MATERIALS AND METHODS: In a retrospective study, we analyzed early and mid-term clinical and echocardiographic data to determine the outcomes of patients who underwent surgical repair of ALCAPA in our institution between 2005 and 2015...
May 2017: Annals of Pediatric Cardiology
https://www.readbyqxmd.com/read/28566819/neonates-with-critical-congenital-heart-defects-impact-of-fetal-diagnosis-on-immediate-and-short-term-outcomes
#16
Sylvia Michael Colaco, Tanuja Karande, Prashant Raviprakash Bobhate, Rashmi Jiyani, Suresh G Rao, Snehal Kulkarni
BACKGROUND: Fetal echocardiography is being increasingly used for prenatal diagnosis of congenital cardiac malformations, but its impact on the neonatal outcomes in low- and middle-income countries is still unknown. AIMS: The objective of this study is to determine the impact of fetal echocardiography on immediate postnatal and short-term outcome in a tertiary pediatric cardiac center. STUDY DESIGN: This is a prospective study. MATERIALS AND METHODS: One hundred consecutive patients with critical congenital heart defects (CHD) requiring active medical or surgical interventions in the 1(st) month of life were included in the study...
May 2017: Annals of Pediatric Cardiology
https://www.readbyqxmd.com/read/28565802/continuous-type-splenogonadal-fusion-a-case-report
#17
Guizhen Huang, Yidong Huang, Li Zeng, Miao Yuan, Yang Wu, Lugang Huang
Splenogonadal fusion (SGF) is a rare congenital malformation. Since it lacks characteristic features, very few cases of SGF have been diagnosed preoperatively. Laparoscopy was effective in both diagnosing and surgically treating this condition. Herein, we reported left side SGF in a male patient who was diagnosed during laparoscopic exploration, and Fowler-Stephens orchidopexy was implemented at the same time. The patient was followed up for one year. At a 6-month follow-up, the left scrotum demonstrated swelling and the internal contents were hard...
May 2017: Experimental and Therapeutic Medicine
https://www.readbyqxmd.com/read/28564670/current-concepts-in-the-pathogenesis-diagnosis-and-management-of-type-i-chiari-malformations
#18
Cody A Doberstein, Radmehr Torabi, Petra M Klinge
Type 1 Chiari malformations (CMs) are a group of congenital or acquired disorders which include the abnormal presence of the cerebellar tonsils in the upper spinal canal, rather than the posterior fossa. The resulting anatomic abnormality causes crowding of the structures at the craniocervical junction and can impair the normal flow of cerebral spinal fluid (CSF) in this region. This impairment in CSF flow dynamics can led to the development of syringomyelia or hydrocephalus. Type 1 CMs have been associated with a wide array of symptoms resulting from either cerebellar and brainstem compression and distortion or disturbances in CSF dynamics, and can affect both children and adults...
June 1, 2017: Rhode Island Medical Journal
https://www.readbyqxmd.com/read/28559048/endometriosis-in-adolescents-and-young-girls-report-a-series-of-85-cases
#19
Michail Matalliotakis, George N Goulielmos, Charoula Matalliotaki, Alexandra Trivli, IoannisMatalliotakis, Aydin Arici
OBJECTIVE: Endometriosis is a common benign and chronic gynecologic disorder that is related to the presence of endometrial glands and stroma outside of their normal location. The aim of this retrospective study was to evaluate endometriosis in adolescent and young girls and further to review the menstrual, reproductive characteristics and risk factors including the obstructive mullerian anomalies and family history. STUDY DESIGN: We review the medical records of adolescent and young women with endometriosis from two different countries...
May 27, 2017: Journal of Pediatric and Adolescent Gynecology
https://www.readbyqxmd.com/read/28553382/klippel-feil-syndrome-associated-with-sacral-agenesis-low-lying-cord-lipomyelomeningocele-and-split-cord-malformation-presenting-with-tethered-cord-syndrome-pentads-neural-tube-defects-spread-along-whole-spinal-axis
#20
Guru Dutta Satyarthee, Amandeep Kumar
Neural tube defects are congenital development anomaly of the central nervous system and usually have relatively more predilection to affect at anterior and posterior neuropore embryological development sites, so usually one or two defects are commonly encountered. However, occurrence of simultaneous multiple neural tube defects is very rare, presence of constellation of five neural defects is extremely rare, and all defects add up together to produce gross neurological deficit. We present an interesting case of a 23-year-old male who presented with history of lower backache and noticed wasting and weakness of lower limbs associated with difficulty in walking for the last 2 years but had no associated sphincter disturbances...
January 2017: Journal of Pediatric Neurosciences
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