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Pectus carinatum

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https://www.readbyqxmd.com/read/29628596/subarachnoid-block-a-safe-choice-for-cesarean-section-in-neurofibromatosis-patient-with-severe-kyphoscoliosis
#1
Ibrahim Demir, Ebru Tarıkçı Kılıç, Mehmet Salim Akdemir
Neurofibromatosis type 1 (NF1) is an autosomal-dominant neuroectodermal hereditary disorder, in which spinal skeletal deformities are one of the manifestations. Pectus carinatum, temporomandibular joint dysfunction, and kyphoscoliosis can be seen with this genetic disorder which can lead to cardiorespiratory system problems and can cause difficulty in airway management. We present the anesthetic management of a NF1 patient with a sharp thoracolumbar dystrophic kyphoscoliosis who have been posted for emergency cesarean section as a result of fetal distress...
January 2018: Anesthesia, Essays and Researches
https://www.readbyqxmd.com/read/29627275/growth-impairment-in-mucopolysaccharidoses
#2
REVIEW
Melodie Melbouci, Robert W Mason, Yasuyuki Suzuki, Toshiyuki Fukao, Tadao Orii, Shunji Tomatsu
Mucopolysaccharidoses (MPS) are a group of lysosomal storage disorders that affect regulation of glycosaminoglycan (GAG) processing. In MPS, the lysosomes cannot efficiently break down GAGs, and the specific GAGs accumulated depend on the type of MPS. The level of impairment of breakdown varies between patients, making this one of the many factors that lead to a range of clinical presentations even in the same type of MPS. These clinical presentations usually involve skeletal dysplasia, in which the most common feature is bone growth impairment and successive short stature...
March 16, 2018: Molecular Genetics and Metabolism
https://www.readbyqxmd.com/read/29525272/bracing-of-pectus-carinatum-a-quantitative-analysis
#3
Tomasz Bugajski, Kartikeya Murari, Steven Lopushinsky, Marc Schneider, Janet Ronsky
BACKGROUND/PURPOSE: Primary treatment of pectus carinatum (PC) is performed with an external brace that compresses the protrusion. Patients are 'prescribed' a brace tightening force. However, no visual guides exist to display this force magnitude. The purpose of this study was to determine the repeatability of patients in applying their prescribed force over time and to determine whether the protrusion stiffness influences the patient-applied forces and the protrusion correction rate...
February 13, 2018: Journal of Pediatric Surgery
https://www.readbyqxmd.com/read/29500788/pigeon-chest-comparative-analysis-of-surgical-techniques-in-minimal-access-repair-of-pectus-carinatum-marpc
#4
REVIEW
Ancuta Muntean, Ionica Stoica, Amulya K Saxena
BACKGROUND: After minimally invasive repair for pectus excavatum (MIRPE), similar procedures for pectus carinatum were developed. This study aimed to analyse the various published techniques of minimal access repair for pectus carinatum (MARPC) and compare the outcomes. DATA SOURCES: Literature was reviewed on PubMed with the terms "pectus carinatum", "minimal access repair", "thoracoscopy" and "children". RESULTS: Twelve MARPC techniques that included 13 articles and 140 patients with mean age 15...
February 2018: World Journal of Pediatrics: WJP
https://www.readbyqxmd.com/read/29357992/force-required-for-correcting-the-deformity-of-pectus-carinatum-and-related-multivariate-analysis
#5
Chenghao Chen, Qi Zeng, Zhongzhi Li, Na Zhang, Jie Yu
OBJECTIVE: To measure the force required for correcting pectus carinatum to the desired position and investigate the correlations of the required force with patients' gender, age, deformity type, severity and body mass index (BMI). METHODS: A total of 125 patients with pectus carinatum were enrolled in the study from August 2013 to August 2016. Their gender, age, deformity type, severity and BMI were recorded. A chest wall compressor was used to measure the force required for correcting the chest wall deformity...
December 24, 2017: Journal of Pediatric Surgery
https://www.readbyqxmd.com/read/29326877/natural-history-of-morquio-a-patient-with-tracheal-obstruction-from-birth-to-death
#6
Caitlin Doherty, Lauren W Averill, Mary Theroux, William G Mackenzie, Christian Pizarro, Robert W Mason, Shunji Tomatsu
Morquio A syndrome (mucopolysaccharidosis IVA, MPS IVA) is a lysosomal storage disease caused by a deficiency of N -acetylgalactosamine-6-sulfate sulfatase, resulting in systemic accumulation of the partially degraded glycosaminoglycans (GAGs), keratan sulfate and chondroitin-6-sulfate. The accumulation of these GAGs leads to distinguishing features as skeletal dysplasia with disproportionate dwarfism, short neck, kyphoscoliosis, pectus carinatum, tracheal obstruction, coxa valga, genu valgum, and joint laxity...
March 2018: Molecular Genetics and Metabolism Reports
https://www.readbyqxmd.com/read/29325984/minimally-invasive-repair-of-pectus-carinatum
#7
Mustafa Yuksel, Tunc Lacin, Nezih Onur Ermerak, Esra Yamansavci Sirzai, Bihter Sayan
BACKGROUND: The second most common deformity of the anterior chest wall, pectus carinatum, is a diverse deformity that has been largely managed using open techniques. This study reviews clinical experience with a newly designed bar for minimally invasive repair of pectus carinatum. METHODS: We reviewed the records of all patients recorded in our Chest Wall Deformities Clinical Database. Between January 2006 and November 2016, minimally invasive repair of pectus carinatum was performed in 172 patients...
March 2018: Annals of Thoracic Surgery
https://www.readbyqxmd.com/read/29092017/the-effect-on-cardiopulmonary-function-after-thoracoplasty-in-pectus-carinatum-a-systematic-literature-review
#8
Stephan Sigl, Barbara Del Frari, Carina Harasser, Anton H Schwabegger
OBJECTIVES: Creating an aesthetically appealing result using thoracoplasty, especially when correcting extensive deformities, but only causing low morbidity, is challenging. The frequency of thoracoplasties in cases of pectus carinatum (PC) has increased due to improved experience and modified surgical techniques, resulting in low morbidity and low complication rates. The indications for surgical treatment are still controversial and, in most cases, remain aesthetic or psychological rather than physiological...
October 30, 2017: Interactive Cardiovascular and Thoracic Surgery
https://www.readbyqxmd.com/read/29027531/-costal-cartilage-structural-and-functional-changes-in-children-with-a-funnel-or-keeled-chest
#9
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/28946165/a-single-center-experience-with-dynamic-compression-bracing-for-children-with-pectus-carinatum
#10
Ashwini Suresh Poola, Amy L Pierce, Beth A Orrick, Shawn David St Peter, Charles L Snyder, David Juang, Pablo Aguayo, Jason D Fraser, George W Holcomb
OBJECTIVE:  Bracing for pectus carinatum (PC) has emerged as an alternative to surgical correction. However, predictive factors for bracing remain poorly understood, as much of the data have been reported from small series. MATERIALS AND METHODS:  We reviewed a prospective dataset in patients with PC who underwent dynamic compression bracing (DCB) from July 2011 to July 2016. Bracing was initiated in patients > 10 years of age with a significant PC and desire for bracing...
February 2018: European Journal of Pediatric Surgery
https://www.readbyqxmd.com/read/28893434/biallelic-mutations-in-szt2-cause-a-discernible-clinical-entity-with-epilepsy-developmental-delay-macrocephaly-and-a-dysmorphic-corpus-callosum
#11
Yuji Nakamura, Yasuko Togawa, Yusuke Okuno, Hideki Muramatsu, Kazuhiko Nakabayashi, Yoko Kuroki, Daisuke Ieda, Ikumi Hori, Yutaka Negishi, Takao Togawa, Ayako Hattori, Seiji Kojima, Shinji Saitoh
Mutations in SZT2 were first reported in 2013 as a cause of early-onset epileptic encephalopathy. Because only five reports have been published to date, the clinical features associated with SZT2 remain unclear. We herein report an additional patient with biallelic mutations in SZT2. The proband, a four-year-old girl, showed developmental delay and seizures from two years of age. Her seizures were not intractable and readily controlled by valproate. She showed mildly dysmorphic facies with macrocephaly, high forehead, and hypertelorism, and also had pectus carinatum...
February 2018: Brain & Development
https://www.readbyqxmd.com/read/28893382/pain-and-sensory-disturbances-following-surgical-repair-of-pectus-carinatum
#12
Marie Veje Knudsen, Hans K Pilegaard, Kasper Grosen
BACKGROUND/PURPOSE: The purpose of this study was to assess the characteristics of persistent postoperative pain and sensory disturbances following surgical repair of pectus carinatum. METHODS: Using a prospective observational design, 28 patients were assessed before, 6 weeks and 6 months after a modified Ravitch operation for pectus carinatum. Postoperative pain was assessed using the Short Form McGill Pain Questionnaire. Sensory testing was conducted to detect brush-evoked allodynia and pinprick hyperalgesia...
August 25, 2017: Journal of Pediatric Surgery
https://www.readbyqxmd.com/read/28857530/-surgery-in-times-of-crisis-conservative-treatment-of-pectus-carinatum-by-static-corset
#13
J V Redondo Sedano, M D Delgado Muñoz, M E Martí Carrera, A Gómez Fraile
INTRODUCTION: Dynamic compression system is the elective treatment for chondrogladiolar pectus carinatum. Nevertheless, its high cost poses a problem for its prescription in places where it is not subsidized. This article analyzes the experience of the Paediatric Plastic Surgery Service at a third grade hospital in the treatment of this deformity with a static compression system. MATERIALS AND METHODS: The study presents a descriptive, retrospective analysis of 30 patients with pectus carinatum treated with a static compression system...
April 20, 2017: Cirugía Pediátrica: Organo Oficial de la Sociedad Española de Cirugía Pediátrica
https://www.readbyqxmd.com/read/28757834/anesthesia-for-minimally-invasive-chest-wall-reconstructive-surgeries-our-experience-and-review-of-literature
#14
REVIEW
Shagun Bhatia Shah, Uma Hariharan, Ajay Kumar Bhargava, Laleng M Darlong
Minimal access procedures have revolutionized the field of surgery and opened newer challenges for the anesthesiologists. Pectus carinatum or pigeon chest is an uncommon chest wall deformity characterized by a protruding breast bone (sternum) and ribs caused by an overgrowth of the costal cartilages. It can cause a multitude of problems, including severe pain from an intercostal neuropathy, respiratory dysfunction, and psychologic issues from the cosmetic disfigurement. Pulmonary function indices, namely, forced expiratory volume over 1 s, forced vital capacity, vital capacity, and total lung capacity are markedly compromised in pectus excavatum...
July 2017: Saudi Journal of Anaesthesia
https://www.readbyqxmd.com/read/28738883/recurrent-c-g1636a-p-g546s-mutation-of-col2a1-in-a-chinese-family-with-skeletal-dysplasia-and-different-metaphyseal-changes-a-case-report
#15
Jing Chen, Xiaomin Ma, Yulin Zhou, Guimei Li, Qiwei Guo
BACKGROUND: Mutations in the COL2A1 gene cause type II collagenopathies characterized by skeletal dysplasia with a wide spectrum of phenotypic severity. Most COL2A1 mutations located in the triple-helical region, and the glycine to bulky amino acid substitutions (e.g., glycine to serine) in the Gly-X-Y repeat were identified frequently. However, the same COL2A1 mutations are associated with different phenotypes and the genotype-phenotype relationship is still poorly understood. Therefore, the studies of more patients about the recurrent mutations in COL2A1 will be needed for further research to provide more comprehensive clinical and genetic data...
July 24, 2017: BMC Pediatrics
https://www.readbyqxmd.com/read/28714634/-pectus-excavatumn-and-carinatum-in-children-and-adolescents-what-to-say-what-to-do
#16
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/28618768/psychosocial-functioning-in-pediatric-patients-with-pectus-excavatum-and-pectus-carinatum
#17
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/28499711/does-an-external-chest-wall-measurement-correlate-with-a-ct-based-measurement-in-patients-with-chest-wall-deformities
#18
Franziska Ewert, Julia Syed, Sonja Wagner, Manuel Besendoerfer, Roman T Carbon, Stefan Schulz-Drost
BACKGROUND: Measurements in chest wall deformities are typically conducted using a thorax caliper or a CT scan of the chest wall. This paper focuses on the possible correlation between these two methods to validate the reliability of the thorax caliper, minimize radiation exposure, and limit the usage of expensive imaging techniques. METHODS: We evaluated 95 consecutive patients (77 pectus excavatum (PE), 17 pectus carinatum (PC), 1 mixed deformity) who received surgical correction of the anterior chest wall...
April 27, 2017: Journal of Pediatric Surgery
https://www.readbyqxmd.com/read/28363366/minimal-invasive-repair-of-pectus-excavatum-and-carinatum
#19
REVIEW
Hans Pilegaard, Peter Bjørn Licht
Minimal invasive surgery has become the gold standard for surgical repair of pectus excavatum. The procedure can be performed as fast-track surgery and cosmetic results are excellent. In addition, cardiac performance improves after correction. With increased awareness on the Internet, the number of patients who seek help continues to rise, primarily for cosmetic reasons. Pectus carinatum is much less frequent than pectus excavatum. Over the past decade surgery has largely been replaced by compression techniques that use a brace, and cosmetic results are good...
May 2017: Thoracic Surgery Clinics
https://www.readbyqxmd.com/read/28274516/the-dynamic-compression-brace-for-pectus-carinatum-intermediate-results-in-286-patients
#20
Sjoerd A de Beer, Martin Gritter, Justin R de Jong, Ernst L W van Heurn
BACKGROUND: Dynamic brace compression is a novel treatment for patients with pectus carinatum. The dynamic compression system contains a device to measure the flexibility of the thoracic wall and regulate the pressure of the brace. METHODS: Patients referred to our pediatric surgical center were screened for treatment with the dynamic compression brace. Patients with a pressure of initial correction (PIC) of 10.0 pounds per square inch or less were offered treatment with the brace...
June 2017: Annals of Thoracic Surgery
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