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Pectus carinatum

J Do Cao, A Wiedemann, T Quinaux, S F Battaglia-Hsu, L Mainard, R Froissart, C Bonnemains, S Ragot, B Leheup, P Journeau, F Feillet
: Patients under 5 years were not evaluated in the phase-3 study for enzyme replacement therapy (ERT) in MPS IV A. Here we describe the evolution of a severe Morquio A pediatric patient who was diagnosed at 19 months old and treated by ERT at 21 months old for the next 30 months. Applying the standard ERT protocol on this very young patient appeared to reduce his urinary excretion of glycosaminoglycans (GAGs); the improvements in both the 6 minute-walk test (6MWT) and the stair climb test, however, were no different than those reported in the nature history study...
December 2016: Molecular Genetics and Metabolism Reports
Hyung Joo Park, Kyung Soo Kim
BACKGROUND: Simple external compression of pectus carinatum seems to have its limitations, particularly the repair of asymmetric pectus carinatum or excavatum/carinatum complex. We devised the sandwich technique (press-molding) to remodel the entire chest wall. The purpose of this study is to introduce the sandwich technique and appraise the early results. METHODS: Between January 2007 and January 2016, 523 consecutive patients with pectus carinatum and its variants were analyzed retrospectively...
September 2016: Annals of Cardiothoracic Surgery
Y-X Kang, Y-J Wang, Q Zhang, X-H Pang, W Gu
Kearns-Sayre syndrome (KSS) is a disorder caused by mutations in mitochondrial DNA. Here, we report an unusual case of Kearns-Sayre syndrome accompanied by hypopituitarism (deficiencies in reproductive and growth hormones). A 20-year-old male presented with growth retardation for the last 8 years, as well as the following findings: short stature, delayed puberty, myasthenia, an extraocular movement deficit, drooping eyelids, pectus carinatum and scoliosis. Cerebral enhanced magnetic resonance imaging revealed dysplasias of the pituitary, white matter and cerebellum...
October 6, 2016: Andrologia
Johan van der Merwe, Filip Casselman, Bernard Stockman, Yvette Vermeulen, Ivan Degrieck, Frank Van Praet
OBJECTIVES: This study presents the first report on in-hospital and long-term outcomes of endoscopic port access atrioventricular valve surgery (EPAAVVS) in adult patients with uncorrected congenital chest wall deformities (CCWDs). METHODS: Our current surgical team performed EPAAVVS in 7 consecutive adult patients (mean age 51.3 ± 16.4 years, 14.3% female, 50% older than 60 years, mean EuroSCORE II 0.8 ± 0.1%) with uncorrected CCWDs between 1 November 2009 and 30 November 2015...
August 19, 2016: Interactive Cardiovascular and Thoracic Surgery
Theofano Tikka, Maninder S Kalkat, Ehab Bishay, Richard S Steyn, Pala B Rajesh, Babu Naidu
OBJECTIVES: Only a few studies report the long-term outcomes following repair of pectus excavatum (PE) and pectus carinatum (PC). Predictors of recurrence after surgery are important in this group of young patients. The purpose of this study was to assess the short- and long-term outcomes of both PE and PC and identify factors associated with postoperative complications and pectus recurrence. METHODS: This was a retrospective observational study that included all patients who underwent primary or recurrent repair of PC and PE in a regional thoracic centre over 20 years...
August 11, 2016: Interactive Cardiovascular and Thoracic Surgery
Barbara Del Frari, Stephan Sigl, Anton H Schwabegger
BACKGROUND: Various methods of corrective thoracoplasty for pectus carinatum deformity have been described, but to date no studies describe a review of complications and how to manage them. Complications are dependent not only on the technique used and the patient's age, but also on the experience of the treating surgeon. The authors present their 15 years' experience with surgical correction of pectus carinatum and the complications that have occurred. A literature review regarding complications with pectus carinatum surgery is performed...
August 2016: Plastic and Reconstructive Surgery
P Faglin, É Nectoux, A Belkhou, P Guerreschi, V Duquennoy-Martinot
Anterior chest wall deformity are mostly represented by pectus excavatum, which is a depression of the chondrosternal plastron from the 3rd to the 7th pairs of the costal cartilages, then by pectus carinatum which conversely represents a protrusion of this plastron. The major esthetic and psychosocial impact is not to be demonstrated anymore whereas the cardiopulmonary functional impact remains still highly debated. Regarding the management, curative surgical techniques such as Wurtz's sub-perichondrial simplified sternochondroplasty or Nuss' minimaly invasive technique are opposed to palliative filling technique such as customized silicone implant, lipostructure and flaps...
July 13, 2016: Annales de Chirurgie Plastique et Esthétique
Betül Kozanhan, Betül Başaran, Feride Aygın, İbrahim Akkoyun, Sadık Özmen
Laparoscopic repair has several advantages with a minimally invasive surgical option for children with Morgagni hernias; however, a number of physiological sequelae results from pneumoperitoneum and insufflation. These physiological changes may be more significant in patients with a congenital heart disease. Perioperative detailed evaluation, meticulous monitorization and cooperation with a surgical team are important in cases with patent foramen ovale for the possible risk of the paradoxical gas embolism. We present the anaesthetic management of a patient with patent foramen ovale, Down syndrome and pectus carinatus who successfully underwent laparoscopic Morgagni hernia repair...
February 2016: Turkish Journal of Anaesthesiology and Reanimation
Murat Kara, Ahmet Gokhan Gundogdu, Salih Zeki Kadioglu, Ertug Can Cayirci, Necati Taskin
BACKGROUND: The Ravitch procedure is a well-established surgical procedure for correction of chest wall deformities. Sternal wedge osteotomy is an important part of this procedure. We studied the incidence of wedge osteotomy with respect to the type of chest wall deformity in patients undergoing surgical correction with the use of a recently developed chest wall stabilization system. METHODS: A total of 47 patients, 39 (83%) male and 8 (17%) female with a mean age of 14...
September 2016: Asian Cardiovascular & Thoracic Annals
Maria Rita Araújo, Céline Marques, Sara Freitas, Rita Santa-Bárbara, Joana Alves, Célia Xavier
BACKGROUND: Marfan's Syndrome (MFS) is a disorder of connective tissue, mainly involving the cardiovascular, musculoskeletal, and ocular systems. The most severe problems include aortic root dilatation and dissection. Anesthetic management is vital for the improvement on perioperative morbidity. CASE REPORT: 61-year-old male with MFS, presenting mainly with pectus carinatum, scoliosis, ectopia lens, previous spontaneous pneumothorax and aortal aneurysm and dissection submitted to thoracoabdominal aortic prosthesis placement...
July 2016: Brazilian Journal of Anesthesiology
Franziska Ewert, Julia Syed, Sonja Kern, Manuel Besendörfer, Roman T Carbon, Stefan Schulz-Drost
Background The literature is silent on the relationship between symptoms and the Haller index. Nor is there a classification of the severity of the physical complaints. Materials and Methods Retrospectively, data from 128 patients (102 funnel, 25 pigeon chest patients, and 1 mixed type) were evaluated. To objectify the symptoms, we developed a score to describe the level of physical ailments. This score includes 10 different symptoms as well as the situation or frequency in which they occur and an impact factor...
June 24, 2016: Thoracic and Cardiovascular Surgeon
Katherine W Gonzalez, Brian G A Dalton, Ashwini S Poola, E Marty Knott, Brendan Kurtz, Charles L Snyder, Shawn D St Peter, George W Holcomb
PURPOSE: In 2011, we established a dedicated center for patients with chest wall deformities. Here, we evaluate the center's effect on patient volume and management. METHODS: A retrospective review of 699 patients with chest wall anomalies was performed. Patients were compared, based on the date of initial consultation, before the pectus center opened (July 2009-June 2011, Group 1) versus after (July 2011-June 2013, Group 2). Analysis was performed utilizing Chi-square and Mann-Whitney U tests...
July 2016: Pediatric Surgery International
Maria Rita Araújo, Céline Marques, Sara Freitas, Rita Santa-Bárbara, Joana Alves, Célia Xavier
BACKGROUND: Marfan's Syndrome (MFS) is a disorder of connective tissue, mainly involving the cardiovascular, musculoskeletal, and ocular systems. The most severe problems include aortic root dilatation and dissection. Anesthetic management is vital for the improvement on perioperative morbidity. CASE REPORT: 61-year-old male with MFS, presenting mainly with pectus carinatum, scoliosis, ectopia lens, previous spontaneous pneumothorax and aortal aneurysm and dissection submitted to thoracoabdominal aortic prosthesis placement...
July 2016: Revista Brasileira de Anestesiologia
Jee-Won Suh, Seok Joo, Geun Dong Lee, Seok Jin Haam, Sungsoo Lee
BACKGROUND: We used an Abramson technique for minimally invasive repair of pectus carinatum in patients who preferred surgery to brace therapy, had been unsuccessfully treated via brace therapy, or were unsuitable for brace therapy because of a rigid chest wall. METHODS: Between July 2011 and May 2015, 16 patients with pectus carinatum underwent minimally invasive surgery. RESULTS: The mean age of the patients was 24.35±13.20 years (range, 14-57 years), and all patients were male...
April 2016: Korean Journal of Thoracic and Cardiovascular Surgery
Mohammad F Obeid, Nahom Kidane, Krzysztof J Rechowicz, Salim Chemlal, Robert E Kelly, Frederic D Mckenzie
Depending on the severity of the condition and associated risk, surgical intervention may not always be the first choice. This is true for treating chest wall deformities such as pectus excavatum and pectus carinatum. For both conditions, novel non-surgical treatments have been developed to gradually alleviate the malformation making use of the elastic nature of the costal cartilages at an early age of the patient. To quantify the performance of such treatments, this paper introduces and discusses the development of a software-based instrument that utilizes 3D chest optical images (surface scans) as input and uses registration techniques to produce an objective gauge of a patient's physical improvement after undergoing treatments...
2016: Studies in Health Technology and Informatics
Madan Mohan Maddali, John Valliattu, Pranav Subbaraya Kandachar, Eapen Thomas, Arora Ram Nishant
During the surgical repair of a truncus arteriosus with aortic arch interruption and pulmonary artery origin stenosis, a Contegra conduit was implanted as part of reconstruction of the pulmonary artery in a small infant. There was a mismatch between the conduit size and the patient that resulted in protrusion of the conduit between the sternal edges. To accommodate the conduit inside the thoracic cavity, traction was applied to the sternum that was gradually released over a period of time guided by transesophageal echocardiography-derived cardiac output data, as well as continuous hemodynamic parameters...
May 2016: Journal of Cardiac Surgery
A Sangsin, C Srichomthong, M Pongpanich, K Suphapeetiporn, V Shotelersuk
Skeletal dysplasia is a group of disorders with more than 450 entities, many of which cannot be differentiated, especially during infancy, but could lead to different clinical courses and prognoses. In this study, we have described a case of a Thai infant with short stature, flat face, pectus carinatum, indirect inguinal hernia, platyspondyly, and generalized delayed endochondral ossification. Using whole-exome sequencing (WES), we successfully identified a de novo heterozygous mutation, c.2024G>A (p.G675D), in the COL2A1 gene, which, to our knowledge, has not been previously reported...
2016: Genetics and Molecular Research: GMR
Iskander S Al-Githmi
BACKGROUND: Pectus carinatum is a congenital chest wall deformity characterized by protrusion of the sternum and adjacent costal cartilages. Multiple treatment options are available for correction of pectus carinatum. OBJECTIVE: We report our initial experience with first-line treatment using a custom fitted dynamic compression orthosis. DESIGN: Prospective evaluation of all patients seen between November 2013 and December 2014. SETTING: University hospital...
January 2016: Annals of Saudi Medicine
Pinar Kuru, Aylin Cakiroglu, Aynur Er, Hincal Ozbakir, Ali Emin Cinel, Busra Cangut, Merve Iris, Berkay Canbaz, Ebru Pıçak, Mustafa Yuksel
BACKGROUND: Pectus excavatum (PE) and pectus carinatum (PC) are the most common chest wall deformities. In this study, we aimed to characterize how patients obtained information about these deformities, as well as patients' family history, associated medical problems, and postoperative satisfaction after the Nuss and Abramson procedures. METHODS: This cross-sectional retrospective study included patients who were operated by a single surgeon between 2006 and 2013...
February 2016: Korean Journal of Thoracic and Cardiovascular Surgery
Yucel Akkas, Bulent Kocer, Neslihan Gulay Peri
We modified the Onen method for pectus carinatum repair and used a vertical incision instead of a transverse incision. The most important advantage gained by the vertical incision is that we can switch to the Ravitch method without needing an additional incision, by elongating the existing incision in patients in whom a pectus bar cannot be placed. We successfully performed the modified Onen technique in a 16-year-old boy with a mixed-type pectus carinatum deformity.
February 2016: Asian Cardiovascular & Thoracic Annals
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