scholarly journals Pectus updates and special considerations in Marfan syndrome

2018 ◽  
Vol 9 (4) ◽  
Author(s):  
Stephanie Fraser ◽  
Anne Child ◽  
Ian Hunt
Keyword(s):  
Psychological Distress ◽  
General Population ◽  
Chest Wall ◽  
Marfan Syndrome ◽  
Pectus Excavatum ◽  
Significant Proportion ◽  
Funnel Chest ◽  
Pectus Carinatum ◽  
Significant Morbidity ◽  
New Techniques

Congenital chest wall or pectus deformities including pectus excavatum (funnel chest) and pectus carinatum (pigeon chest) affect a significant proportion of the general population and up to 70% of patients with Marfan syndrome. Patients often experience significant morbidity and psychological distress, which can worsen with age. Here we discuss new techniques for both operative and non-operative treatment of pectus deformity, the importance of a welltimed intervention and special considerations in patients with Marfan syndrome.

Chest Wall Deformities

1989 ◽  
Vol 11 (5) ◽  
pp. 147-151
Author(s):  
Dick G. Ellis
Keyword(s):  
Chest Wall ◽  
Pectus Excavatum ◽  
Surgical Correction ◽  
Funnel Chest ◽  
Pectus Carinatum ◽  
Correction Surgery ◽  
Cosmetic Problem

Chest wall deformities are principally depression deformities, the most common being pectus excavatum (funnel chest). The most common protrusion deformity is pectus carinatum (pigeon breast). Pectus carinatum is a cosmetic problem, but its presence can be psychologically devastating to the patient. Pectus excavatum is definitely a physiologic problem, the effects of which may be reversible by surgical correction. Surgery is safe, complications are minimal, and results are usually satisfactory.

Current Options for the Treatment of Pectus Carinatum: When to Brace and When to Operate?

2018 ◽  
Vol 28 (04) ◽  
pp. 347-354 ◽  
Author(s):  
Sherif Emil
Keyword(s):  
Minimally Invasive ◽  
Chest Wall ◽  
Pectus Excavatum ◽  
Surgical Correction ◽  
Diagnosis And Treatment ◽  
Pectus Carinatum ◽  
The Past ◽  
Minimally Invasive Surgical

AbstractPectus carinatum has traditionally been described as a rare chest wall anomaly in comparison to pectus excavatum. However, recent data from chest wall anomaly clinics demonstrate that this deformity is probably much more frequent than once believed. In the past, invasive surgical correction by the Ravitch technique was essentially the only option for treatment of pectus carinatum. Major advances over the past two decades have provided additional options, including noninvasive chest wall bracing and minimally invasive surgical correction. This article will discuss current options for the treatment of pectus carinatum, and some of the factors that should be taken into account when choosing the options available. Diagnosis and treatment of the pectus arcuatum variant will also be described.

Deformities of the chest

2021 ◽  
pp. 991-1000
Author(s):  
Harvey Stern
Keyword(s):  
Heart Disease ◽  
Ischaemic Heart Disease ◽  
Chest Wall ◽  
Pectus Excavatum ◽  
Surgical Correction ◽  
Tumour Resection ◽  
Poland Syndrome ◽  
Pectus Carinatum ◽  
Full Spectrum ◽  
Reconstructive Techniques

Analysis and surgical management of chest wall deformities involves understanding complex congenital and acquired conditions and well as the full spectrum of reconstructive techniques. Congenital chest wall deformity includes pectus excavatum, pectus carinatum, and Poland syndrome. In surgical correction of these conditions, timing and the understanding of the growing child is important. Traumatic chest wall deformities present a different set of challenges, especially as the context is often a very unwell patient. As cardiothoracic surgeons push the boundaries of what is possible in patients with ischaemic heart disease, iatrogenic defects are not uncommon, and repair of such defects is described. Lastly, this chapter covers surgery for the coverage of major tumour resection defects in oncological surgery.

scholarly journals Radiological changes after Nuss operation for pectus excavatum

Medicina ◽  
2009 ◽  
Vol 45 (9) ◽  
pp. 699 ◽  
Author(s):  
Artūras Kilda ◽  
Saulius Lukoševičius ◽  
Vidmantas Barauskas ◽  
Živilė Jankauskaitė ◽  
Algidas Basevičius
Keyword(s):  
Chest Wall ◽  
Pectus Excavatum ◽  
First Year ◽  
Nuss Procedure ◽  
Funnel Chest ◽  
Haller Index ◽  
Wall Deformation ◽  
Lateral Chest ◽  
Radiological Changes ◽  
The Mean

The objective of this study was to evaluate sternovertebral distance and the chest wall deformation after Nuss procedure. Materials and methods. Anteroposterior and lateral chest radiographs were performed before Nuss procedure, 1, 6, and 12 months after operation and finally 1 month after bar removal. Sternovertebral distance and transversal chest dimension were measured on radiographs, as well as Haller and vertebral indexes were calculated. Results. A total of 84 children with funnel chest were operated on. Preoperative sternovertebral distance was 79.81±6.96 mm; 1 month after operation, 97.84±17.08 mm; 6 months, 110.55±13.85 mm; and 12 months, 113.6±14.61 mm. After removal of the bar, the distance was 105±11.95 mm. The mean increase in sternovertebral distance during the first month was 18 mm (P<0.0001); 1–6 months, 12.8 mm (P=0.0006); and 6-12 months, 3 mm (P=0.48). The mean decrease in sternovertebral distance after removal of the bar was 8.6 mm (P=0.47). The decrease in transversal chest dimension during the first month was significant (13.3±12.86 mm, P=0.012). Conclusions. The sternovertebral distance was significantly increased after Nuss operation. Restoration of deformation proceeds during all the first year after operation. The dynamics of deformation is better depicted by means of vertebral index rather than Haller index.

Outcome of pectus excavatum in patients with Marfan syndrome and in the general population

1989 ◽  
Vol 115 (6) ◽  
pp. 954-958 ◽  
Author(s):  
Pamela Hawks Arn ◽  
L.R. Scherer ◽  
J. Alex Haller ◽  
Reed E. Pyeritiz
Keyword(s):  
General Population ◽  
Marfan Syndrome ◽  
Pectus Excavatum

scholarly journals Aspectos gerais e bucais da Síndrome de Marfan

2020 ◽  
Vol 9 (5) ◽  
pp. 498-502
Author(s):  
Roberta Andréia Lammers ◽  
Letícia Stefenon ◽  
Paula Wietholter
Keyword(s):  
Marfan Syndrome ◽  
Pectus Excavatum ◽  
Root Resorption ◽  
Genetic Alterations ◽  
Cone Beam ◽  
Oral Manifestations ◽  
Pectus Carinatum ◽  
Review Of The Literature ◽  
Porto Alegre ◽  
Maxillary Constriction

Introdução: A Síndrome de Marfan é uma desordem genética que afeta o tecido conectivo. No contexto da Odontologia, poucos profissionais da área conhecem os sintomas da síndrome, bem como os cuidados necessários no atendimento ao paciente. Objetivo: O objetivo deste trabalho foi descrever as características anatômicas gerais e bucais de pessoas com Síndrome de Marfan. Material e método: Foram realizadas pesquisas nas bases de dados EBSCO, Bireme e Pubmed entre os anos de 2017 e 2018, sendo utilizados os seguintes descritores: Síndrome de Marfan AND Odontologia AND Manifestações bucais. Resultados: Foram localizados 13 artigos na base de dados BIREME, 23 no PubMed e cinco no EBSCO, totalizando 41 artigos. Desses, 10 foram selecionados para a realização desta pesquisa. As principais alterações gerais descritas na literatura incluem membros superiores e inferiores longos, pé chato, corpo fino com o segmento inferior maior que o segmento superior, aracnodactilia, peito plano com costelas proeminentes e escoliose, pectus carinatum, pectus excavatum, cifose, hiperextensibilidade, dolicostenomelia, alterações oculares e problemas cardíacos. As principais alterações bucais descritas incluem hipoplasia maxilar, retrognatia mandibular, macrostomia, dentição altamente apinhada com mordidas cruzadas anteriores e posteriores, palato de arco alto e relação molar classe II de Angle em ambos os lados e apresentam maior índice de doenças periodontais do que pacientes normais. Conclusões: Os principais cuidados que devem ser observados durante o tratamento odontológico relacionam-se a anamnese e ao exame clínico. O melhor entendimento dessa patologia poderá orientar decisões terapêuticas para prevenção e correção das desordens mencionadas neste trabalho. Descritores: Síndrome de Marfan; Odontologia; Manifestações Bucais. Referências Muñoz Sandoval J, Saldarriaga-Gil W, Isaza de Lourido C. Síndrome de Marfan, mutaciones nuevas y modificadoras del gen FBN1. 2014;27(2):206-15. García JLG, Cedeño LM, Medina JAG. Síndrome de Marfan. Medisan. 2007;11(4):1-5. Pfeiffer MET. Síndrome de Marfan em crianças e adolescentes: importância, critérios e limites para o exercício físico. Rev DERC. 2011;17(3):82-6. Lebreiro A, Martins E, Cruz C, Almeida J, Maciel MJ, Cardoso JC, et al. Síndrome de Marfan: manifestações clínicas, fisiopatologia e novas perspectivas da terapêutica farmacológica. Rev Port Cardiol. 2010; 29(6):1021-36. Velásquez C. Manejo odontológico integral en centro quirúrgico de un paciente con Sindrome de Marfan. Odontol Pediatr (Lima). 2015;14(1):80-5. Tsang AK, Taverne A, Holcombe T. Marfan syndrome: a review of the literature and case report. Spec Care Dentist. 2013;33(5):248-54. Bilodeau JE. Retreatment of a patient with Marfan syndrome and severe root resorption. Am J Orthod Dentofacial Orthop. 2010;137(1):123-34. Baraldi CEE, Paris MF, Robinson WM. A síndrome de Marfan e seus aspectos odontológicos: relato de caso e revisão da literatura. Rev Fac Odontol Porto Alegre. 2008;49(3):36-9. Sinha A, Kaur S, Raheel SA, Kaur K, Alshehri M, Kujan O. Oral manifestations of a rare variant of Marfan syndrome. Clin Case Rep. 2017;5(9):1429-34. Anuthama K, Prasad H, Ramani P, Premkumar P, Natesan A, Sherlin HJ. Genetic alterations in syndromes with oral manifestations. Dent Res J (Isfahan). 2013;10(6):713-22. Jain E, Pandrey RK. Marfan Syndrome. BMJ Case Rep. 2013;25(16):16-22. Staufenbiel I, Hauschild C, Kahl-Nieke B, Vahle-Hinz E, von Kodolitsch Y, Berner M, et al. Periodontal Conditions in patients with Marfan Syndrome: a multienter case conrol study. BMC Oral Health. 2013;13:59. Mallineni SK, Jayaraman J, Yiu CK, King NM. Concomitant occurrence of hypohyperdontia in a patient with Marfan syndrome: a review of the literature and report of a case. J Investig Clin Dent. 2012;3(4):253-57. Gott VL. Antoine Marfan and his syndrome: one hundred years later. Md Med J. 1998;47(5):247-52. Alves IC, Navarro F. Exercício fisico e sindrome de Marfan. Rev Bras Prescrição e Fisiologia do Exercício. 2008;2(8):149-57. Sivasankari T, Mathew P, Austin RD, Devi S. Marfan Syndrome. J Pharm Bioallied Sci. 2017;9(1):73-7. Sabbatini IF. Avaliação dos components anatômicos do sistema estomatognático de crianças com bruxismo, por meio de imagens obtidas por tomografia computadorizada cone beam [dissertação de Mestrado]. Ribeirão Preto: Universidade do Estado de São Paulo; 2012. Cistulli PA, Richards GN, Palmisano RG, Unger G, Berthon-Jones M, Sullivan CE. Influence of maxillary constriction on nasal resistance and sleep apnea severity in patients with Marfan's syndrome. Chest. 1996;110(5):1184-8.

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