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Pectus in children

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https://www.readbyqxmd.com/read/29175559/desmosterolosis-presenting-with-multiple-congenital-anomalies
#1
Mersedeh Rohanizadegan, Stephanie Sacharow
Desmosterolosis is a rare multiple congenital anomaly syndrome caused by defect in the enzyme 3-beta-hydroxysterol delta-24-reductase (DHCR24) in the cholesterol biosynthesis pathway. Defects in this enzyme cause increased level of the cholesterol precursor desmosterol while disrupting development of cholesterol, impacting embryogenesis. A total of 9 cases of desmosterolosis have been reported to date. We report a 20-month-old male from consanguineous parents with multiple congenital anomalies including corpus callosum hypoplasia, facial dysmorphism, cleft palate, pectus deformity, short, wide neck and distal contractures...
November 23, 2017: European Journal of Medical Genetics
https://www.readbyqxmd.com/read/29027531/-costal-cartilage-structural-and-functional-changes-in-children-with-a-funnel-or-keeled-chest
#2
A V Kurkov, A B Shekhter, V S Paukov
Congenital chest wall deformities (CCWDs) in children are severe diseases leading to cosmetic defects and diseases of the respiratory and cardiovascular systems. The most common of these deformities are funnel-shaped (pectus excavatum, FD) and keeled (pectus carinatum, KD) ones. The pathogenesis of CCWDs and the role of costal cartilage structural and functional changes in their pathogenesis have now been not well studied, which makes it difficult to elaborate pathogenetic approaches to correcting these diseases...
2017: Arkhiv Patologii
https://www.readbyqxmd.com/read/28857528/-benefits-of-magnetic-resonance-for-the-study-of-pectus-excavatum-in-children-initial-experience
#3
M Dore Reyes, C De La Torre, M Bret Zurita, P Triana Junco, J Jiménez Gómez, M Romo Muñoz, A Vilanova Sánchez, M Parrón Pajares, A Pérez Vigara, J L Encinas Hernández, L Martínez Martínez, F Hernández Oliveros, M López-Santamaría
INTRODUCTION/AIM OF THE STUDY: In patients with PE, cardiovascular alterations ensue as a result of the mediastinum compression caused by sternum impingement and is responsible of many of the symptoms. Anatomical and functional assessment is of the utmost importance for a comprehensive understanding of the disease and an adequate treatment plan. Our aim was to describe the use of magnetic resonance image (MRI) in the study of PE and whether it is comparable to imaging techniques. PATIENTS AND METHODS: A retrospective study of the first 10 patients in which an MRI was performed as part of PE assessment within august 2015-2016...
April 20, 2017: Cirugía Pediátrica: Organo Oficial de la Sociedad Española de Cirugía Pediátrica
https://www.readbyqxmd.com/read/28827049/the-effect-of-thoracoscopy-upon-the-repair-of-pectus-excavatum
#4
Nicole Mennie, Geoff Frawley, Joe Crameri, Sebastian K King
BACKGROUND/AIMS: The Nuss procedure is the most commonly performed operation to correct pectus excavatum (PE). Thoracoscopic assistance has been anecdotally noted to improve the safety of this operative approach. This study aimed to compare complications and clinical outcomes before and after the introduction of thoracoscopy in a single-center. METHODS: A retrospective review was performed of all patients who underwent the Nuss procedure at The Royal Children's Hospital over an 11-year period (2005-2015), collecting data on all intra-operative and post-operative outcomes...
July 31, 2017: Journal of Pediatric Surgery
https://www.readbyqxmd.com/read/28774473/hyperinflation-of-the-left-anterior-basal-segment-a-new-sign-of-pectus-excavatum-severity-on-ct
#5
S Singh, S B Greenberg
AIM: To describe a new finding in patients with pectus excavatum - left lower lobe anterior basal segment hyperinflation [LABSH]. A secondary objective is to determine the frequency of the new finding and association with pectus excavatum severity. MATERIAL AND METHODS: The study population included 52 children who underwent preoperative computed tomography (CT) for the evaluation of pectus excavatum. A control group of 50 children was obtained after evaluating 137 CT examinations performed for other reasons...
November 2017: Clinical Radiology
https://www.readbyqxmd.com/read/28759900/advantages-of-cardiac-magnetic-resonance-imaging-for-severe-pectus-excavatum-assessment-in-children
#6
Mariela Dore, Paloma Triana Junco, Monserrat Bret, Manuel Gómez Cervantes, Martha Muñoz Romo, Javier Jiménez Gómez, Ana Pérez Vigara, Manuel Parrón Pajares, José Luis Encinas, Francisco Hernandez, Leopoldo Martinez, Manuel López Santamaría, Carlos De La Torre
No abstract text is available yet for this article.
July 31, 2017: European Journal of Pediatric Surgery
https://www.readbyqxmd.com/read/28714634/-pectus-excavatumn-and-carinatum-in-children-and-adolescents-what-to-say-what-to-do
#7
Pierre Lascombes, Isabelle Ruchonnet-Métrailler, Maurice Beghetti, Armand Bottani, James Wilde
In children presenting with a pectus excavatum (PE) or pectus carinatum (PC) an underlying syndrome including Marfan's syndrome needs to be excluded. In adolescents, severe chest wall deformities may cause cardiac or respiratory problems but most commonly they have a psychological impact. The conservative treatment is a Vacuum Bell for PE, and a Dynamic Compression System for PC ; they play an increasing role in young patients. These devices need to be worn multiple hours per day for several months for an optimal result...
February 15, 2017: Revue Médicale Suisse
https://www.readbyqxmd.com/read/28624480/evaluation-of-thoracic-vertebrae-rotation-in-patients-with-pectus-excavatum
#8
Ryszard Tomaszewski, Łukasz Wiktor, Ludwina Machała
PURPOSE: The aim of our study was to evaluate thoracic vertebrae rotation in patients with pectus excavatum. Moreover, we wanted to assess the prevalence, the severity and relationship between pectus excavatum and adolescent idiopathic scoliosis (AIS). METHODS: We performed retrospective analysis of 82 preoperative chest CT in children with pectus excavatum performed between January 2008 and December 2011. For each patient Haller Index and Cobb angle was measured...
July 2017: Acta Orthopaedica et Traumatologica Turcica
https://www.readbyqxmd.com/read/28618768/psychosocial-functioning-in-pediatric-patients-with-pectus-excavatum-and-pectus-carinatum
#9
Aliye Tuğba Bahadir, Pınar Kuru Bektaşoğlu, Aylin Çakiroğlu Eser, Ceyda Afacan, Mustafa Yüksel
BACKGROUND/AIM: Pectus excavatum and pectus carinatum are the most commonly seen anterior chest wall deformities. Recent studies reveal that minimal invasive repair of pectus deformities improves the quality of life. Our aim is to assess the psychosocial functioning and sociodemographic characteristics of pediatric patients with pectus deformities and evaluate the differences between patients operated on with minimal invasive repair techniques and nonoperated patients. MATERIALS AND METHODS:  Thirty-two patients with pectus deformities who were operated on 6 months or more before and 31 nonoperated patients participated in the study...
June 12, 2017: Turkish Journal of Medical Sciences
https://www.readbyqxmd.com/read/28615652/development-of-a-computer-aided-design-and-finite-element-analysis-combined-method-for-customized-nuss-bar-in-pectus-excavatum-surgery
#10
Longhan Xie, Siqi Cai, Liang Xie, Gang Chen, Haiyu Zhou
Pectus excavatum (PEX) is the most common chest deformity in children, which is usually corrected by using the minimally invasive Nuss method. The orthopedic effect of the Nuss operation is mainly evaluated by both the Haller index and the appearance of the chest wall configuration, which is dependent on the operator's clinical experience to a great extent. To improve the orthopedic effect, we proposed a novel method to individually design and optimize the shape of the Nuss bar and to advise its location as well as the incisions...
June 14, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28602524/monitoring-the-effectiveness-of-the-vacuum-bell-during-pectus-excavatum-treatment-technical-innovation
#11
Sergio B Sesia, David Hradetzky, Frank-Martin Haecker
BACKGROUND: The vacuum bell (VB) is a valid and the only non-invasive treatment for pectus excavatum (PE). To elevate the sternum the patient himself creates a differential negative pressure inside the VB using a hand pump. A distance and differential pressure measuring device (DPMD) enables us for the first time to assess objectively those parameters. METHODS: After approval by the institutional review board, 53 patients recruited from our outpatient clinic were included in this retrospective study and distributed into three groups (group 1 aged 6 to 10years; group 2 aged 11 to 15years; group 3 aged 16 to 20years)...
May 31, 2017: Journal of Pediatric Surgery
https://www.readbyqxmd.com/read/28202768/respiratory-morbidity-in-infants-born-with-a-congenital-lung-malformation
#12
MULTICENTER STUDY
Celine Delestrain, Naziha Khen-Dunlop, Alice Hadchouel, Pierrick Cros, Héloïse Ducoin, Michael Fayon, Isabelle Gibertini, André Labbé, Géraldine Labouret, Marie-Noëlle Lebras, Guillaume Lezmi, Fouad Madhi, Guillaume Thouvenin, Caroline Thumerelle, Christophe Delacourt
BACKGROUND AND OBJECTIVES: The actual frequency of respiratory symptoms related to congenital pulmonary malformations (CPMs) remains undetermined. The goal of this study was to prospectively evaluate the respiratory symptoms occurring in infants with prenatally diagnosed CPMs, identify factors associated with the occurrence of these symptoms, and evaluate their resolution after surgery. METHODS: Infectious and noninfectious respiratory symptoms were prospectively collected in a French multicenter cohort of children with CPMs...
March 2017: Pediatrics
https://www.readbyqxmd.com/read/28087135/surgical-site-infection-related-to-use-of-elastomeric-pumps-in-pectus-excavatum-repair-lessons-learned-from-root-cause-analysis
#13
Nadja Apelt, Joshua Schaffzin, Christina Bates, Rebeccah L Brown, Marc Mecoli, Senthilkumar Sadhasivam, Victor Garcia
BACKGROUND: Pectus excavatum repair (PEX) is among the most painful thoracic procedures performed. Continuous peripheral nerve blockade (CPNB) is known to be efficacious in optimizing pain control while limiting narcotic use in adult thoracic procedures. It was introduced in May 2015 as a bridge to oral pain control in children undergoing PEX. Consequently, the surgical site infection (SSI) rate increased from 2.7% to 27.7%. METHODS: SSI surveillance followed national guidelines...
January 6, 2017: Journal of Pediatric Surgery
https://www.readbyqxmd.com/read/27847075/combined-nuss-procedure-and-cardiac-procedure-through-a-left-anterolateral-thoracotomy
#14
François Roubertie, Sowmya Ramanan, Frederic Lavrand, Jean-Benoit Thambo
Aortic root aneurysms associated with severe pectus excavatum caused by Marfan's syndrome can be challenging to correct in children. Recent reports suggest single-stage correction of both cardiac and chest wall deformities through a median sternotomy. We report a 9-year-old girl with Marfan's syndrome, an ascending aorta aneurysm, mitral regurgitation, and pectus excavatum, who underwent David's procedure with mitral valve repair and the Nuss procedure to correct the thoracic deformity. This single-stage combined procedure was performed through a left anterolateral thoracotomy...
December 2016: Annals of Thoracic Surgery
https://www.readbyqxmd.com/read/27823841/minimally-invasive-repair-of-pectus-excavatum-in-children-results-of-a-modified-nuss-procedure
#15
A Durry, C Gomes Ferreira, T Tricard, P Gicquel, F Becmeur
INTRODUCTION: Pectus excavatum (PE) is the most common deformity of the anterior thoracic wall. The Nuss technique allows the thorax to be reshaped with the aid of a retrosternal metallic bar. The aim of this study is to evaluate and compare the complication rate between the original Nuss technique and a lightly modified approach. MATERIAL AND METHOD: We performed a retrospective single-center observational study based on the medical files of patients operated for PE in the Pediatric Surgery Unit between July 2004 and July 2015...
November 4, 2016: Annales de Chirurgie Plastique et Esthétique
https://www.readbyqxmd.com/read/27747183/pectus-excavatum-from-a-pediatric-surgeon-s-perspective
#16
Donald Nuss, Robert J Obermeyer, Robert E Kelly
Historically, pectus excavatum (PE) was reported to be congenital, but in our experience only 22% are noticed in the first decade of life. Thus far, genetic studies support an autosomal recessive heritability, which coincides with only 40% of our patients having some positive family history, but is also contradictory given a constant sex ratio of 4:1 in favor of males. This inconsistency may be explained by the effect of more than one pectus disease-associated allele. Once the deformity is noticed, it tends to progress slowly until puberty, when rapid progression is often seen...
September 2016: Annals of Cardiothoracic Surgery
https://www.readbyqxmd.com/read/27747179/a-prospective-study-on-quality-of-life-in-youths-after-pectus-excavatum-correction
#17
Johanne Jeppesen Lomholt, Elisabeth Brammer Jacobsen, Mikael Thastum, Hans Pilegaard
BACKGROUND: The impact of correction of pectus excavatum (PE) on adolescents' health-related quality of life (HRQL) has only been investigated in prospective designs using disease-specific measures and without controls. The aim of this prospective study was to evaluate the HRQL before and after surgical correction of PE using a generic HRQL measure, and to compare the reported level of HRQL before surgery with an age-comparable control group. METHODS: Patients (n=107) and one of their parents (n=106) completed the generic HRQL measure: the Child Health Questionnaire before, 3 months, and 6 months after correction for PE...
September 2016: Annals of Cardiothoracic Surgery
https://www.readbyqxmd.com/read/27745867/intercostal-nerve-cryoablation-versus-thoracic-epidural-catheters-for-postoperative-analgesia-following-pectus-excavatum-repair-preliminary-outcomes-in-twenty-six-cryoablation-patients
#18
MULTICENTER STUDY
Benjamin A Keller, Sandra K Kabagambe, James C Becker, Y Julia Chen, Laura F Goodman, Julianna M Clark-Wronski, Kenneth Furukawa, Rebecca A Stark, Amy L Rahm, Shinjiro Hirose, Gary W Raff
BACKGROUND: Multimodal pain management strategies are used for analgesia following pectus excavatum repair. However, the optimal regimen has not been identified. We describe our early experience with intercostal cryoablation for pain management in children undergoing the Nuss procedure and compare early cryoablation outcomes to our prior outcomes using thoracic epidural analgesia. METHODS: A multi-institutional, retrospective review of fifty-two patients undergoing Nuss bar placement with either intercostal cryoablation (n=26) or thoracic epidural analgesia (n=26) from March 2013 to January 2016 was conducted...
December 2016: Journal of Pediatric Surgery
https://www.readbyqxmd.com/read/27568387/evaluation-of-thoracic-factors-after-scoliosis-surgery-in-patients-with-both-scoliosis-and-pectus-excavatum
#19
Ryoji Tauchi, Noriaki Kawakami, Taichi Tsuji, Tetsuya Ohara, Yoshitaka Suzuki, Toshiki Saito, Ayato Nohara
PURPOSE: Pectus excavatum can negatively impact cardiac function during scoliosis surgery. Several authors reported severe hypotension associated with the prone position during scoliosis surgery in children that had both scoliosis and pectus excavatum. However, we could find no studies that evaluated the change in the thoracic factors, such as sternal tilt angle and Haller index after scoliosis surgery in patients with both scoliosis and pectus excavatum. The purpose of this study is to evaluate the change in thoracic factors after surgical treatment for scoliosis associated with pectus excavatum...
August 27, 2016: European Spine Journal
https://www.readbyqxmd.com/read/27510834/a-review-of-anesthetic-techniques-and-outcomes-following-minimally-invasive-repair-of-pectus-excavatum-nuss-procedure
#20
Geoff Frawley, Jacinta Frawley, Joe Crameri
BACKGROUND: Pectus excavatum (PE) is the most common congenital chest wall deformity, occurring in 1 : 1000 children with a male to female ratio of 4 : 1. Several procedures have been described to manage this deformity, including cartilage resection with sternal osteotomy (the Ravitch procedure) and a minimally invasive repair technique (the Nuss procedure). While initially described as a nonthoracoscopic technique, the current surgical approach of the Royal Childrens Hospital involves thoracoscopic assistance...
November 2016: Paediatric Anaesthesia
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