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Piotr Cierpikowski, Adam Rzechonek, Piotr Błasiak, Hanna Lisowska, Grzegorz Pniewski, Patric Le Pivert
Pectus excavatum is the most common congenital deformity of the chest. The Nuss procedure is minimally invasive surgical correction of this defect, using retrosternal metal bars. The purpose of the present study was to describe a 15-year experience with the Nuss surgery, and to evaluate the long-term clinical results of the procedure. We retrospectively evaluated 239 patients, aged 14-34, who underwent the Nuss surgery in the years 2002-2016. Postoperative complications were observed in 40/236 (16.9%) patients...
March 11, 2018: Advances in Experimental Medicine and Biology
Rana Muhammad Kamran Shabbir, Gökhan Nalbant, Nafees Ahmad, Sajid Malik, Aslıhan Tolun
BACKGROUND: Carbohydrate sulfotransferase 11 (CHST11) is a membrane protein of Golgi that catalyses the transfer of sulfate to position 4 of the N-acetylgalactosamine residues of chondroitin. Chondroitin sulfate is the predominant proteoglycan in cartilage, and its sulfation is important in the developing growth plate of cartilage. A homozygous deletion encompassing part of the gene and the embedded miRNA MIR3922 had been detected in a woman with hand/foot malformation and malignant lymphoproliferative disease...
March 7, 2018: Journal of Medical Genetics
Robin Baudouin, Eric Levesque, René Jancovici
Pectus excavatum can lead to right ventricle compression. Although extremely rare, congestive hepatopathy should be considered when patients with severe pectus excavatum present with cardiovascular involvement.
March 1, 2018: European Journal of Cardio-thoracic Surgery
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...
March 2, 2018: World Journal of Pediatrics: WJP
Mariela Dore, Paloma Triana Junco, Carlos De La Torre, Alejandra Vilanova-Sánchez, Monserrat Bret, Gaspar Gonzalez, Vanesa Nuñez Cerezo, Javier Jimenez Gomez, Jose Luis Encinas, Francisco Hernandez, Leopoldo Martínez Martínez, Manuel Lopez Santamaria
Introduction  Minimally invasive repair for pectus excavatum (MIRPE) is controversial in extremely severe cases of pectus excavatum (PE) and an open repair is usually favored. Our aim is to describe a case of a patient with an extremely severe PE that underwent a minimally invasive approach. Case report  An 8-year-old girl with severe sternum depression was assessed. She had a history of exercise intolerance, nocturnal dyspnea, fatigue, and shortness of breath. Chest computed tomography showed that sternum depression was posterior to the anterior vertebral column; therefore, Haller and correction index could not be measured...
January 2018: European Journal of Pediatric Surgery Reports
M Sébastien, B Ange-Line, A Mirna, C Martin, S Elisabeth, G Cyril, G Anne-Marie, C Aude, C Perrine, H Delphine, F Anne, H Nada, V Antonio, T-M-T Frédéric, P Christophe, D Yannis, T-R Christel, F Laurence
Marfanoid habitus (MH) combined with intellectual disability (ID) is a genetically and clinically heterogeneous group of overlapping disorders. We performed exome sequencing in 33 trios and 31 single probands to identify novel genes specific to MH-ID. After the search for variants in OMIM genes and non-OMIM genes with classical approaches, we searched for variants in non-disease-causing genes whose pLI was above 0.9 (ExAC Consortium data), in which truncating variants were found in at least 3 unrelated patients, in order to identity novel MH-ID genes...
February 20, 2018: Clinical Genetics
MennatAllah M Ewais, Shivani Chaparala, Rebecca Uhl, Dawn E Jaroszewski
Pectus excavatum (PEx) is one of the most common congenital chest wall deformities. Depending on the severity, presentation of PEx may range from minor cosmetic issues to disabling cardiopulmonary symptoms. The effect of PEx on adult patients has not been extensively studied. Symptoms may not occur until the patient ages, and they may worsen over the years. More recent publications have implied that PEx may have significant cardiopulmonary implications and repair is of medical benefit. Adults presenting for PEx repair can undergo a successful repair with a minimally invasive "Nuss" approach...
2018: Patient related Outcome Measures
W P Zuidema, J W A Oosterhuis, G W Zijp, S M van der Heide, A F W van der Steeg, L W E van Heurn
BACKGROUND: An early observation after chest wall correction is direct inspection from the PE patient of their "new" thorax. Changes in self-perception may give raise to other psychological adaptations. The aim of this study was to evaluate the early changes in the fields of self-esteem, body image and QoL. METHODS: Prospective observational longitudinal multicenter cohort study. Self-esteem, emotional limitations and general health were assessed using the Child Health Questionnaire (CHQ) in patients under 18 and the World Health Organization Quality of Life Questionnaire-bref (WHOQOL-bref) was used for body image, psychological domain and overall QoL in patients over 16 years of age...
February 6, 2018: World Journal of Surgery
Tyler C Friske, Richard Sola, Yangyang R Yu, Abdur R Jamal, Eric Rosenfeld, Huirong Zhu, Mark V Mazziotti, Shawn D St Peter, Sohail R Shah
BACKGROUND: High narcotic requirements after minimally invasive repair of pectus excavatum (MIRPE) can increase the risk of urinary retention. Placement of intraoperative Foley catheters to minimize this risk is variable. This study determines the rate of urinary retention in this population to guide future practice. MATERIALS AND METHODS: We reviewed retrospectively all patients who underwent MIRPE from January 2012 to July 2016 at 2 academic children's hospitals...
January 31, 2018: Surgery
Xicheng Deng, Peng Huang, Liwen Yi, Xiaohui Yang, Cheng He
RATIONALE: Pectus excavatum (PE) in the setting of congenital heart disease is not uncommon. The surgical strategy has evolved over the last 20 years from a staged approach to simultaneous repair of both defects. PATIENT CONCERNS: A 3-year-old boy was admitted for elective repair of PE and atrial septal defect (ASD). DIAGNOSES: Clinically, there were obvious features of PE and a grade 2 systolic murmur heard loudest at the 2nd intercostal space abutting the left sternal border...
December 2017: Medicine (Baltimore)
Ekaterini Christina Tampaki, Athanasios Tampakis, Didier Lardinois
No abstract text is available yet for this article.
February 1, 2018: Interactive Cardiovascular and Thoracic Surgery
Kenji Inoue, Tomohiro Kumada, Ikuko Hiejima, Tatsuya Fujii
The severity of X-linked myotubular myopathy (XLMTM) ranges from mild to severe, depending on the level of ventilatory support required. Patients with the severe form of XLMTM usually die within the first year of life due to respiratory failure. Most survivors need tracheostomies, and there has only been one report about the use of non-invasive positive pressure ventilation (NPPV) in patients with the severe form of XLMTM because of the severity of the associated respiratory failure. We successfully applied NPPV with high-span positive inspiratory pressure (PIP) in a patient with the severe form of XLMTM, who also had secondary pectus excavatum...
January 14, 2018: Brain & Development
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...
January 9, 2018: Annals of Thoracic Surgery
Katherine M Huber, Amanda Zimmerman, Deniz Dayicioglu
Mastectomies for both cancer resection and risk reduction are becoming more common. Existing chest wall irregularities are found in these women presenting for breast reconstruction after mastectomy and can pose reconstructive challenges. Women who desired breast reconstruction after mastectomy were evaluated preoperatively for existing chest wall irregularities. Case reports were selected to highlight common irregularities and methods for improving cosmetic outcome concurrently with breast reconstruction procedures...
January 2018: Cancer Control: Journal of the Moffitt Cancer Center
Junke Wang, Quan Wang, Zhengxia Pan
PURPOSE: The present study was undertaken to summarize our experience with the concurrent repair of pectus excavatum (PE) and congenital heart defects in young children over the past 15 years. METHODS: A retrospective study was conducted that included patients who underwent combined repair of PE and a congenital heart defect in a large clinical center between 2002 and 2017. Intraoperative and postoperative patient characteristics, postoperative complications and surgical outcomes were recorded...
January 5, 2018: Pediatric Surgery International
Tariq Abu-Tair, Salmai Turial, Marike Hess, Christiane M Wiethoff, Gundula Staatz, André Lollert, Christoph Kampmann
BACKGROUND: Pectus excavatum is classified using the Haller Index (HI) or the Correction Index. However, no correlation between the HI and CI and cardiopulmonary impairment has been described in detail. METHODS: This prospective cohort study included 99 otherwise healthy patients with pectus excavatum who underwent cardiopulmonary exercise testing and magnetic resonance imaging at inspiration and expiration to correlate cardiopulmonary function with the grade of thoracic dysmorphia...
February 2018: Annals of Thoracic Surgery
H Tilliet Le Dentu, U Lancien, O Sellal, F Duteille, P Perrot
INTRODUCTION: Pectus excavatum is the most common congenital chest malformation and is a common reason for consultation in plastic surgery. Our attitude is most often a filling of the depression with a custom-made silicone prosthesis. The objective of this work was to evaluate the interest of computer-aided design (CAD) of implants compared to the conventional plaster molds method. PATIENTS AND METHODS: We have collected all the cases of custom-made silicone implants to treat funnel chests in our plastic surgery department...
December 12, 2017: Annales de Chirurgie Plastique et Esthétique
Joana Pimenta, António Vieira, Tiago Henriques-Coelho
Pectus excavatum is generally considered a benign problem with a wide spectrum of impact in body image. Some articles had highlighted that this deformity can be associated with important clinical problems. We report a case of a 14-year-old boy with a severe pectus excavatum associated with palpitations who was diagnosed with polymorphic ventricular arrhythmia. The arrhythmia was resolved after minimally invasive surgical correction of the pectus excavatum.
December 12, 2017: Interactive Cardiovascular and Thoracic Surgery
Vien T Truong, Candice Y Li, Rebeccah L Brown, Ryan A Moore, Victor F Garcia, Eric J Crotty, Michael D Taylor, Tam M N Ngo, Wojciech Mazur
AIMS: To investigate the right ventricular (RV) strain in pectus excavatum (PE) patients using cardiac magnetic resonance tissue tracking (CMR TT). MATERIALS AND METHODS: Fifty consecutive pectus excavatum patients, 10 to 32 years of age (mean age 15 ± 4 years), underwent routine cardiac magnetic resonance imaging (CMR) including standard measures of chest geometry and cardiac size and function. The control group consisted of 20 healthy patients with a mean age of 17 ± 5 years...
2017: PloS One
Awais Ashfaq, Staci Beamer, MennatAllah M Ewais, Jesse Lackey, Dawn Jaroszewski
BACKGROUND: Minimally invasive repair of pectus excavatum (MIRPE) has been extended to repair of defects in adults, with reported higher complication rates and outcome failures. The optimal revision for a prior failed MIRPE in an adult has not been identified. We review our experience for this adult population. METHODS: A retrospective review was performed of 129 patients (age ≥18 years) who underwent revision after a failed pectus excavatum (PE) repair from December 2010 through December 2016...
February 2018: Annals of Thoracic Surgery
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