The role of 3D printing in chest wall reconstruction.

Technology is advancing fast, and chest wall surgery finds particular benefit in the broader availability of three-dimensional (3D) reconstruction and printing. An increasing number of reports are being published on the use of these resources in virtual 3D reconstructions of chest walls in computed tomography (CT) scans, virtual surgeries, 3D printing of real-size models for surgical planning, practice, and education, and of note, the manufacture of customized 3D printed implants, changing the fundamental conception from a surgery that fits all, to a surgery for each patient. In this review, we explore the evidence published on simple chest wall reconstruction, including the use of 3D technology to assist in the improvement of the repair of the most frequent chest wall deformities: pectus excavatum and carinatum. Current studies are oriented to the automatization and customization of transthoracic implants, as well as education on real-size models. Next, we investigate the implementation of 3D printing in the repair of complex chest wall reconstruction, comprised of infrequent chest wall deformities such as pectus arcuatum and Poland syndrome. These malformations are very heterogeneous resulting in a high degree of improvisation during the surgical repair. In this setting, 3D technology plays a role in the standardization of a process that contemplates customization, concepts that may seem contradictory. Finally, 3D printing with biocompatible materials is rapidly becoming the first choice for the reconstruction of wide chest wall oncological resections. In this work, we review the first and most important current publications on the subject.

The The Sandwich Technique For Minimally Invasive Repair Of Pectus Carinatum.

INTRODUCTION: Minimally invasive repair of pectus carinatum (MIRPC) has been performed using the Abramson technique in which the bar that compresses the sternum is fixed with steel wires on the ribs. A 14-year-old patient underwent to a MIRPC using a sandwich technique in which two metallic bars fixed with bridges were implanted below the sternum under thoracoscopic vision, and another bar in a subcutaneous tunnel was implanted above. This technique has the potential to avoid specific problems related to the original technique like loosening of support for correction (broken wire), avoidance of induction of pectus excavatum or subcutaneous tissue adhesion.

STERNUM TOMOGRAPHIC EVALUATION IN PECTUS PATIENTS: ANALYSIS OF SAGITAL PARAMETERS.

Objective: To evaluate the sagittal tomographic reformatting of the sternum using unpublished radiographic parameters (indexes and angles), comparing them between the different types of pectus, and controls. Methods: 44 patients with pectus deformities and controls underwent chest CT for analysis. The types of pectus were classified into: inferior pectus carinatum (IPC), superior (SPC) and lateral (LPC), and broad (BPE) and localized pectus excavatum (LPE). The following tomographic parameters were created and measured: (1) spine-manubrium-sternum index (SMS); (2) column-sternum index (CSI); (3) manubrium-sternal angle (MSA); (4) inferior manubrium angle (IMA); and (5) inferior sternum angle (ISA). Statistical analysis was performed between the pectus and control groups, and between the different types of pectus. Results: There was a significant difference between: a) pectus excavatum and pectus carinatum when analyzing the SMS, CSI, MSA and ISA indexes. b) LPE and control group for SMS and ISA. c) LPC and LPE, and LPC and BPE for SMS; d) BPE and LPC for CSI; e) IPC and LPE, and IPC and BPE for ISA; f) SPC and LPE, and SPC and BPE for IMA. Conclusion: The radiographic indexes and angles created provided differentiation parameters between patients with different types of pectus, and between these and controls. Level of Evidence II, Prognostic Studies.

Objetivos: Avaliar a reformatação tomográfica sagital do esterno por meio de parâmetros radiográficos inéditos (índices e ângulos), comparando-os entre os diferentes tipos de pectus e controles. Métodos: 44 pacientes com deformidades pectus e controles foram submetidos à TC do tórax para análise. Os tipos de pectus foram classificados em: pectus carinatum inferior (PCI), superior (PCS) e lateral (PCL), e pectus excavatum amplo (PEA) e localizado (PEL). Foram criados e mensurados os seguintes parâmetros tomográficos: (1) índice coluna-manúbrio-esterno (CME); (2) índice coluna-esterno (CE); (3) ângulo manúbrio-esternal (AME); (4) ângulo inferior do manúbrio (AIM); e (5) ângulo inferior do esterno (AIE). Foi realizada análise estatística entre os grupos pectus e controle, e entre os diferentes tipos de pectus. Resultados: Houve diferença significativa entre: a) pectus excavatum e pectus carinatum quando analisados os índices CME, CE, AIM e AIE; b) PEL e grupo controle para CME e AIE; c) PCL e PEL, e PCL e PEA para o CME; d) PEA e PCL para CE; e) PCI e PEL, e PCI e PEA para AIE; f) PCS e PEL, e PCS e PEA para AIM. Conclusões: Os índices e ângulos radiográficos criados forneceram parâmetros de diferenciação entre pacientes com diferentes tipos de pectus, e entre estes e controles. Nível de Evidência II, Estudos Prognósticos.

Sternum tomographic evaluation in pectus patients: analysis of sagital parameters / Avaliação tomográfica de pacientes com pectus: novos índices e ângulos sagitais

ABSTRACT Objective: To evaluate the sagittal tomographic reformatting of the sternum using unpublished radiographic parameters (indexes and angles), comparing them between the different types of pectus, and controls. Methods: 44 patients with pectus deformities and controls underwent chest CT for analysis. The types of pectus were classified into: inferior pectus carinatum (IPC), superior (SPC) and lateral (LPC), and broad (BPE) and localized pectus excavatum (LPE). The following tomographic parameters were created and measured: (1) spine-manubrium-sternum index (SMS); (2) column-sternum index (CSI); (3) manubrium-sternal angle (MSA); (4) inferior manubrium angle (IMA); and (5) inferior sternum angle (ISA). Statistical analysis was performed between the pectus and control groups, and between the different types of pectus. Results: There was a significant difference between: a) pectus excavatum and pectus carinatum when analyzing the SMS, CSI, MSA and ISA indexes. b) LPE and control group for SMS and ISA. c) LPC and LPE, and LPC and BPE for SMS; d) BPE and LPC for CSI; e) IPC and LPE, and IPC and BPE for ISA; f) SPC and LPE, and SPC and BPE for IMA. Conclusion: The radiographic indexes and angles created provided differentiation parameters between patients with different types of pectus, and between these and controls. Level of Evidence II, Prognostic Studies.

RESUMO Objetivos: Avaliar a reformatação tomográfica sagital do esterno por meio de parâmetros radiográficos inéditos (índices e ângulos), comparando-os entre os diferentes tipos de pectus e controles. Métodos: 44 pacientes com deformidades pectus e controles foram submetidos à TC do tórax para análise. Os tipos de pectus foram classificados em: pectus carinatum inferior (PCI), superior (PCS) e lateral (PCL), e pectus excavatum amplo (PEA) e localizado (PEL). Foram criados e mensurados os seguintes parâmetros tomográficos: (1) índice coluna-manúbrio-esterno (CME); (2) índice coluna-esterno (CE); (3) ângulo manúbrio-esternal (AME); (4) ângulo inferior do manúbrio (AIM); e (5) ângulo inferior do esterno (AIE). Foi realizada análise estatística entre os grupos pectus e controle, e entre os diferentes tipos de pectus. Resultados: Houve diferença significativa entre: a) pectus excavatum e pectus carinatum quando analisados os índices CME, CE, AIM e AIE; b) PEL e grupo controle para CME e AIE; c) PCL e PEL, e PCL e PEA para o CME; d) PEA e PCL para CE; e) PCI e PEL, e PCI e PEA para AIE; f) PCS e PEL, e PCS e PEA para AIM. Conclusões: Os índices e ângulos radiográficos criados forneceram parâmetros de diferenciação entre pacientes com diferentes tipos de pectus, e entre estes e controles. Nível de Evidência II, Estudos Prognósticos.

ANALYSIS OF STERNAL CURVATURE PATTERNS IN PATIENTS WITH PECTUS AND CONTROL.

OBJECTIVE: To analyze reformatted sagittal sternal tomography images and classify sternal body curvature types, and compare different types of pectus populations with one another and with normal individuals. METHODS: In total, 50 controls and 167 pectus patients were selected for chest CT to analyze the median sagittal plane, of whom 89 had pectus carinatum (mean age, 12 ± 10 years) and 78 pectus excavatum (mean age, 14 ± 10 years). Clinical types of pectus were classified as inferior, superior, or lateral pectus carinatum, and localized or broad pectus excavatum. The following types of sternal patterns were defined: gradual vertical curve, gradual posterior curve, gradual anterior curve, proximal third curve, middle third curve, distal third curve, anterior rectilinear, vertical rectilinear, and posterior rectilinear. Statistical analyses were performed to compare the different types of pectus with one another and with the control group. RESULTS: Patients with different thoracic deformities, but with similar sternal curvature patterns, were observed. Some types of sternal curvature were significantly more frequent in certain types of pectus (p < 0,05). The gradual vertical curve and anterior rectilinear types prevailed in controls (p < 0,05). CONCLUSION: Some sternal curvature patterns were more frequent than the others in certain types of pectus and the controls. Level of Evidence II, Prognostic studies - investigating the effect of a patient characteristic on the outcome of disease.

OBJETIVO: Avaliar a reformatação sagital tomográfica do esterno por meio da análise de uma classificação do tipo de curvatura do corpo esternal nos diferentes tipos de pectus, comparando-os entre si e com indivíduos normais. MÉTODOS: 50 controles e 167 pacientes submetidos à TC do tórax para análise da reconstrução sagital no plano mediano, sendo 89 com pectus carinatum (idade média, 12 ± 10 anos) e 78 com pectus excavatum (idade média, 14 ± 10 years). Os tipos clínicos de pectus foram classificados em: pectus carinatum superior, inferior e lateral, e pectus excavatum amplo ou localizado. Foram definidos os seguintes tipos de padrões esternais: curvo gradativo vertical; curvo gradativo posterior; curvo gradativo anterior; curvo terço proximal; curvo terço médio; curvo terço distal; retilíneo anterior; retilíneo vertical; e retilíneo posterior. Foi realizada análise estatística entre o grupo pectus e controle, e entre diferentes tipos de pectus. RESULTADOS: Observamos pacientes com deformidades torácicas diferentes, mas com esternos com padrão de curvatura semelhante. Alguns tipos de curvatura esternal são significativamente mais frequentes em alguns tipos de pectus (p < 0,05). Em controles prevaleceram os tipos curvo gradativo vertical e retilíneo anterior (p < 0,05). CONCLUSÃO: Alguns tipos de curvatura esternal são mais frequentes que outras em determinados tipos de pectus e controles. Nível de Evidência II, Estudos prognósticos - investigação do efeito de característica de um paciente sobre o desfecho da doença.

Analysis of sternal curvature patterns in patients with pectus and control / Análise dos padrões de curvatura esternal em pacientes com pectus e controles

ABSTRACT Objective: To analyze reformatted sagittal sternal tomography images and classify sternal body curvature types, and compare different types of pectus populations with one another and with normal individuals. Methods: In total, 50 controls and 167 pectus patients were selected for chest CT to analyze the median sagittal plane, of whom 89 had pectus carinatum (mean age, 12 ± 10 years) and 78 pectus excavatum (mean age, 14 ± 10 years). Clinical types of pectus were classified as inferior, superior, or lateral pectus carinatum, and localized or broad pectus excavatum. The following types of sternal patterns were defined: gradual vertical curve, gradual posterior curve, gradual anterior curve, proximal third curve, middle third curve, distal third curve, anterior rectilinear, vertical rectilinear, and posterior rectilinear. Statistical analyses were performed to compare the different types of pectus with one another and with the control group. Results: Patients with different thoracic deformities, but with similar sternal curvature patterns, were observed. Some types of sternal curvature were significantly more frequent in certain types of pectus (p < 0,05). The gradual vertical curve and anterior rectilinear types prevailed in controls (p < 0,05). Conclusion: Some sternal curvature patterns were more frequent than the others in certain types of pectus and the controls. Level of Evidence II, Prognostic studies - investigating the effect of a patient characteristic on the outcome of disease.

RESUMO Objetivo: Avaliar a reformatação sagital tomográfica do esterno por meio da análise de uma classificação do tipo de curvatura do corpo esternal nos diferentes tipos de pectus, comparando-os entre si e com indivíduos normais. Métodos: 50 controles e 167 pacientes submetidos à TC do tórax para análise da reconstrução sagital no plano mediano, sendo 89 com pectus carinatum (idade média, 12 ± 10 anos) e 78 com pectus excavatum (idade média, 14 ± 10 years). Os tipos clínicos de pectus foram classificados em: pectus carinatum superior, inferior e lateral, e pectus excavatum amplo ou localizado. Foram definidos os seguintes tipos de padrões esternais: curvo gradativo vertical; curvo gradativo posterior; curvo gradativo anterior; curvo terço proximal; curvo terço médio; curvo terço distal; retilíneo anterior; retilíneo vertical; e retilíneo posterior. Foi realizada análise estatística entre o grupo pectus e controle, e entre diferentes tipos de pectus. Resultados: Observamos pacientes com deformidades torácicas diferentes, mas com esternos com padrão de curvatura semelhante. Alguns tipos de curvatura esternal são significativamente mais frequentes em alguns tipos de pectus (p < 0,05). Em controles prevaleceram os tipos curvo gradativo vertical e retilíneo anterior (p < 0,05). Conclusão: Alguns tipos de curvatura esternal são mais frequentes que outras em determinados tipos de pectus e controles. Nível de Evidência II, Estudos prognósticos - investigação do efeito de característica de um paciente sobre o desfecho da doença.

Localized pectus excavatum treated with brace and exercise: long term results of a brazilian technique / Tratamento do pectus excavatum localizado com órtese e exercícios: resultados de longo prazo de uma técnica brasileira

ABSTRACT Objective: Pectus excavatum is a deformity that affects aesthetics and causes emotional disorders. Surgical correction is well established, but conservative treatment is less common. We investigated the long-term results of using a brace and performing specific physical exercises to treat localized pectus excavatum, a type of deformity in which the depressed area is restricted to the midline region along the nipple line. Methods: We selected 115 patients (mean age 12.8 years), with a minimum follow-up of 36 months, who were evaluated more than one year after the end of treatment and skeletal maturity. Results were correlated with deformity flexibility, severity, regular use of the device, and performance of specific exercises. The chi-square (χ2) and the Cochran-Mantel-Haenszel tests were used for statistical analysis. Results: Treatment was successful in 58% of patients, however, when exercises were performed and the brace was used regularly by patients with flexible deformities, the rate increased to 83% (p = 0.005). Severity and adherence to treatment greatly impacted successful treatment (p = 0.009 and < 0.001, respectively). Conclusion: The proposed treatment method was effective for correction or partial correction of the deformity in motivated patients followed up until skeletal maturity, especially when started early in milder and more flexible deformities. Level of Evidence V, Expert opinion.

RESUMO Objetivo: O pectus excavatum é uma deformidade importante por comprometer a estética e causar distúrbios emocionais. A sua correção cirúrgica é bem estabelecida, mas o tratamento conservador é menos familiar. Investigamos os resultados de longo prazo do tratamento do pectus excavatum localizado (deformidade restrita a linha média e na linha mamilar) com uso de órtese e exercícios físicos específicos. Métodos: Selecionamos 115 pacientes (média de 12,8 anos) com seguimento mínimo de 36 meses, sendo avaliados mais de um ano após o término do tratamento e maturidade esquelética. Os resultados foram relacionados estatisticamente (qui-quadrado e Cochran-Mantel-Haenszel) com a flexibilidade da deformidade, a gravidade, o uso regular da órtese e a realização de exercícios específicos. Resultados: O tratamento foi bem-sucedido em 58% dos pacientes, mas quando o uso da órtese e os exercícios foram regulares em pacientes com deformidades flexíveis, essa taxa aumentou para 83% (p = 0,005). A gravidade e a adesão ao tratamento tiveram grande impacto no sucesso do tratamento (p = 0,009 e <0,001, respectivamente). Conclusão: O método de tratamento proposto foi eficaz para correção total ou parcial da deformidade em pacientes motivados acompanhados até a maturidade esquelética, principalmente quando a terapêutica foi iniciada precocemente em deformidades mais leves e flexíveis. Nível de Evidência V, Opinião do especialista.

LOCALIZED PECTUS EXCAVATUM TREATED WITH BRACE AND EXERCISE: LONG TERM RESULTS OF A BRAZILIAN TECHNIQUE.

OBJECTIVE: Pectus excavatum is a deformity that affects aesthetics and causes emotional disorders. Surgical correction is well established, but conservative treatment is less common. We investigated the long-term results of using a brace and performing specific physical exercises to treat localized pectus excavatum, a type of deformity in which the depressed area is restricted to the midline region along the nipple line. METHODS: We selected 115 patients (mean age 12.8 years), with a minimum follow-up of 36 months, who were evaluated more than one year after the end of treatment and skeletal maturity. Results were correlated with deformity flexibility, severity, regular use of the device, and performance of specific exercises. The chi-square (χ2) and the Cochran-Mantel-Haenszel tests were used for statistical analysis. RESULTS: Treatment was successful in 58% of patients, however, when exercises were performed and the brace was used regularly by patients with flexible deformities, the rate increased to 83% (p = 0.005). Severity and adherence to treatment greatly impacted successful treatment (p = 0.009 and < 0.001, respectively). CONCLUSION: The proposed treatment method was effective for correction or partial correction of the deformity in motivated patients followed up until skeletal maturity, especially when started early in milder and more flexible deformities. Level of Evidence V, Expert opinion.

OBJETIVO: O pectus excavatum é uma deformidade importante por comprometer a estética e causar distúrbios emocionais. A sua correção cirúrgica é bem estabelecida, mas o tratamento conservador é menos familiar. Investigamos os resultados de longo prazo do tratamento do pectus excavatum localizado (deformidade restrita a linha média e na linha mamilar) com uso de órtese e exercícios físicos específicos. Métodos: Selecionamos 115 pacientes (média de 12,8 anos) com seguimento mínimo de 36 meses, sendo avaliados mais de um ano após o término do tratamento e maturidade esquelética. Os resultados foram relacionados estatisticamente (qui-quadrado e Cochran-Mantel-Haenszel) com a flexibilidade da deformidade, a gravidade, o uso regular da órtese e a realização de exercícios específicos. RESULTADOS: O tratamento foi bem-sucedido em 58% dos pacientes, mas quando o uso da órtese e os exercícios foram regulares em pacientes com deformidades flexíveis, essa taxa aumentou para 83% (p = 0,005). A gravidade e a adesão ao tratamento tiveram grande impacto no sucesso do tratamento (p = 0,009 e <0,001, respectivamente). CONCLUSÃO: O método de tratamento proposto foi eficaz para correção total ou parcial da deformidade em pacientes motivados acompanhados até a maturidade esquelética, principalmente quando a terapêutica foi iniciada precocemente em deformidades mais leves e flexíveis. Nível de Evidência V, Opinião do especialista.

Pectus carinatum: When less is more.

Awareness of pectus carinatum has increased among the medical community over the last several decades, as innovative options for nonsurgical treatments have become more widely known. Management alternatives have shifted from open resective to minimally invasive strategies, and finally, to reshaping the chest using both surgical and non-surgical modalities. We aim to review the evolution of the diagnosis and treatment of pectus carinatum up to its current management.

[Pectus excavatum y carinatum en el síndrome de Marfan y síndromes similares: prevalencia e impacto clínico pulmonar y cardiovascular].

INTRODUCTION: Pectus excavatum (PE) and carinatum (PC) are common in Marfan syndrome (SM) and similar syndromes (SS). Patients can evolve without symptoms. In some there is depression, social adjustment disorders, pulmonary and cardiovascular symptoms in which there is controversy about their relationship with the structural damage of the thorax. OBJECTIVE: To assess the prevalence of the type of thoracic deformity in patients with MS and SS in a historical and current cohort and to analyze the clinical, pulmonary and cardiovascular impact. METHOD: Prospective study. Subjects who met the Ghent criteria and who had a complete clinical record, an echocardiogram and/or magnetic resonance imaging, computed tomography and respiratory function tests were included. RESULTS: Of a total of 338 patients with MS and SS, 112 cases with thoracic deformity were detected, the prevalence of PE and PC in SM 13.6 and 12.4, respectively, was lower in SS. There is compression and displacement of lung and right cardiac cavities by PE and the correlation between the Haller Index and the increased PASP is 44 (p = 0.009). CONCLUSIONS: The prevalence of PE and PC in SM and SS is high, which impacts on lung function and cardiovascular damage, requires corrective management of the thoracic deformity and not only implies for aesthetic purposes.

INTRODUCCIÓN: El pectus excavatum (PE) y el pectus carinatum (PC) son frecuentes en el síndrome de Marfan (SM) y en síndromes similares (SS). Los pacientes pueden evolucionar sin síntomas. En algunos hay depresión, trastornos de adaptación social, síntomas pulmonares y cardiovasculares, en los cuales hay controversia de su relación con el daño estructural del tórax. OBJETIVO: Evaluar la prevalencia del tipo de deformidad torácica en pacientes con SM y SS en una cohorte histórica y analizar el impacto clínico, pulmonar y cardiovascular. MÉTODO: Estudio prospectivo. Se incluyeron sujetos con criterios de Ghent y características específicas de cada síndrome, con expediente completo, ecocardiograma o resonancia magnética y tomografía computada, y pruebas de función respiratoria. RESULTADOS: De un total de 338 pacientes con SM y SS, se detectaron 112 casos con deformidad torácica. Prevalencia de PE y PC en SM: 13.6 y 12.4; fue menor en SS. Hay compresión y desplazamiento de pulmón y cavidades cardiacas derechas por PE. Hay correlación entre el Índice de Haller y la presión sistólica de la arteria pulmonar incrementada es de 44 (p = 0.009). CONCLUSIONES: La prevalencia de PE y PC en el SM y SS es alta, lo cual impacta en la función pulmonar y cardiovascular, en esas condiciones se requiere del manejo correctivo de la deformidad torácica y el objetivo no es estético.

[Result of 12 years of non-surgical treatment of pectus carinatum]. / Resultado de 12 años de tratamiento no quirúrgico del pectus carinatum.

OBJECTIVE: To report our experience in the treatment of pectus carinatum by using the dynamic compression system. MATERIAL AND METHODS: Retrospective study during the period from January 2005 to September 2017. Patients with typical condrogladiolar pectus carinatum and correction pressure (PC) ≤ 14 PSI (pound square inch) were included. Exclusion criteria: patients with previous thoracic surgery, mixed malformations and chondromanubrial pectus carinatum. For the treatment, the Dynamic Thoracic Compressor System (FMF) with pressure meter in PSI was used. The PC, the treatment pressure (PT), the correction time (TC) and the maintenance time (TM), recurrences and complications were analyzed. A qualitative scale was measured in three grades: where A is excellent or very good, B is regular and C is bad. RESULTS: We treated 104 patients under 18 years of age. The PT was 2.26. The average of the TC was 8.8 months. The TM was on average 8 months. 36.5% of the patients finished the treatment, 36.5% still continue in treatment and 26.9% of the patients lost the follow-up due to desertion. The qualitative assessment was positive in 95.5% of our patients, and unfavourable in 4.5%. CONCLUSION: The non-surgical treatment of pectus carinatum is efficient, non-invasive and of low morbidity. Regarding the high dropout rate, we must analyze the variables to be modified to reduce it. This treatment should be considered as the first option to correct pectus carinatum in patients with flexible thorax.

OBJETIVO: Describir nuestra experiencia en el tratamiento del pectus carinatum mediante el uso del sistema de compresión dinámico. MATERIALES Y METODOS: Estudio retrospectivo durante el período de enero de 2005 a septiembre de 2017. Se incluyeron pacientes con pectus carinatum condrogladiolar típico y presión de corrección (PC) ≤ 14 PSI (pound per square inch). Criterios de exclusión: pacientes con cirugía torácica previa, malformaciones mixtas y condromanubriales. Para el tratamiento se utilizó el sistema compresor torácico dinámico (FMF) con medidor de presión en PSI. Se analizaron la PC, la presión de tratamiento (PT), el tiempo de corrección (TC) y el tiempo de mantenimiento (TM), recidivas y complicaciones. Se realizó una escala cualitativa medida en tres grados: donde A es excelente o muy bueno, B regular y C malo. RESULTADOS: Tratamos 104 pacientes menores de 18 años. La PT fue de 2,26. El promedio del TC fue de 8,8 meses. El TM fue en promedio de 8 meses. El 36,5% de los pacientes finalizaron el tratamiento, 36,5% aún continúan en tratamiento y 26,9% de los pacientes se perdió el seguimiento por deserción del mismo. La valoración cualitativa fue positiva en el 95,5% de nuestros pacientes, y desfavorable en el 4,5%. CONCLUSION: El tratamiento no quirúrgico del pectus carinatum es eficiente, no invasivo y de baja morbilidad. Respecto a la alta tasa de deserción, debemos analizar las variables a modificar para disminuirla. Este tratamiento debe ser considerado una opción de primera elección, para corregir el pectus carinatum en pacientes con tórax flexible.

Síndrome de Morquio, enfermedad de interés para la odontopediatría: presentación de un caso / Morquio syndrome, a disease of interest for pediatric dentistry: case report

El Síndrome de Morquio es un trastorno de almacenamiento de mucopolisacáridos se caracteriza principalmente por estatura corta y afectación ósea grave, pero el coeficiente intelectual es normal. La prevalencia es rara se estima que afecta a uno de cada 200.000 nacimientos hombres y mujeres por igual. La MPS IV A y B son enfermedades autosómicas recesivas con esto queremos decir que ambos progenitores son portadores del mismo gen afectado el cual se encuentra alterado produciendo así una deficiencia en la producción de la enzima. Las manifestaciones esqueléticas en esta displasia son retardo en el crecimiento, hipoplasia del odontoides, cifosis toracolumbar, displasia de cadera, genu valgo, manchas cutáneas y laxitud articular, en cuando a cuestiones dentales tenemos: el esmalte es delgado, rugoso e hipoplásico afectando dientes deciduos como permanentes. Se presenta el caso de un paciente masculino de 8 años 3/12 presentando MPS el cual requiere un protocolo de rehabilitación lo cual se realiza en el área de odontopedriatría del Hospital del Niño DIF.

Morquio syndrome is a mucopolysaccharide storage disorder is mainly characterized by short stature, severe bone involvement, but IQ is normal. The prevalence is rare is estimated to affect one in every 200,000 births men and women alike. The MPS IV A and B are autosomal recessive diseases with this we mean that both parents are carriers of the same gene affected which is altered thus producing a deficiency in the production of the enzyme. The skeletal manifestations in this dysplasia are growth retardation, hypoplasia of the odontoid, thoracolumbar kyphosis, hip dysplasia, genu valgus, skin blemishes and joint laxity, then dental issues are: the enamel is thin rugged and hypoplastic affecting deciduous theeth as permanent. The case of a male patient presenting eight years 3/12 MPS which requires a rehabilitation protocol which is done in the dental area of Hospital del Niño DIF is presented.

Non-surgical treatment of pectus carinatum with the FMF® Dynamic Compressor System.

Pectus carinatum is a chest wall deformity, sometimes associated with physical signs and symptoms, but always associated to significant psychological distress. Surgical correction used to be the only solution, and was therefore only indicated for the most severe cases. Non-surgical approaches have been developed and improved during the last 15-20 years. A paradigm shift occured when the medical community realized that, despite the wall deformity, the chest wall was not completely rigid, but flexible and capable of remodeling. Several bracing devices and protocols are available as of today. This article will focus specifically in the FMF® Dynamic Compressor System (DCS), which was developed in Argentina in 2001 and is currently used worldwide.

Minimally invasive repair of pectus carinatum and how to deal with complications.

While less common than pectus excavatum, pectus carinatum is also a chest wall deformity affecting males in higher proportion than women. Patient requests for a solution of this disease occur especially during the growth spurt of puberty when this malformation becomes more obvious and difficult to conceal. Those people suffering from pectus carinatum are very likely subject to behavioral changes and negative personality impacts. By compressing the protruding anterior region of the chest wall we achieve correction of the chest contour and simultaneous lateral expansion of the depressed costochondral arches. This original technique and the procedure to apply it fit within the category of minimally invasive surgery. The compression system acts in a way similar to that of orthodontic braces. Two rectangular fixation plates are fixed to the compression strut with screws. The plates have threaded holes along a groove in the central portion, and two holes at both ends used to attach them to the ribs by means of steel wire suture. The compression strut has to be modified into a convex shape to adapt it to the particular characteristics of the patient's malformation. This molding is done using benders designed as part of the procedure.

Mediastinal lymph nodes and pulmonary nodules in children: MDCT findings in a cohort of healthy subjects.

OBJECTIVE: Existing data are very limited on incidentally detected pulmonary nodules or mediastinal lymph nodes in healthy children who undergo chest MDCT. We aimed to evaluate the prevalence, distribution, and average dimensions of these occasional findings in a cohort of otherwise healthy patients. MATERIALS AND METHODS: Two radiologists reviewed in consensus the scans of patients referred for chest MDCT during the preoperative workup for pectus carinatum or pectus excavatum treatments. Exclusion criteria included the presence of any documented malignancy (by date of MDCT or during the 2 years after the examination), history of recent infections, or trauma. Patients' records were assessed after 2 years for the development of any malignancy. RESULTS: A total of 99 individuals (63 boys, 36 girls; mean age, 13.5 years; range, 4-18 years) who fulfilled the study criteria were evaluated. The presence of at least one pulmonary nodule was observed in 75% of the patients, with a mean diameter of 2.8 mm. Of a total number of 225 pulmonary nodules, only 24 (10.7%) were calcified. Mediastinal lymph nodes were also identified in 81% of the cases, with a maximum diameter of 7 mm (smallest axis). CONCLUSION: The presence of pulmonary nodules or mediastinal lymph nodes on the basis of preoperative chest MDCT scans in healthy children is frequent. Given that 95% of the nodules and 100% of the lymph nodes measured less than 6 mm and 7 mm, respectively, we conclude that incidental findings under these limits are very unlikely to be pathologic.

Pectus carinatum in a dog / Pectus carinatum em um cão

Background: The deformities of the anterior chest wall are universally designated as pectus deformities. Two types of deformities are known, pectus excavatum and pectus carinatum. Pectus excavatum is a dorsal ventral narrowing of the chest, also known as funnel chest, while pectus carinatum is a ventral protrusion of the sternum, also known as pigeon breast. Both are diseases seen in children and adolescents, however, they are considered rare in small animals, especially pectus carinatum. Thus, the aim of this study is to report a case of pectus carinatum in a Shih-tzu puppy dog. Case: A male canine Shih-tzu, 2 months-old, was present at the veterinary hospital. The owner reported that in the first days of life, the animal felt difficulty breathing when she took him in her lap, and still noticed abnormal increase of the thoracic region. The patient was from a litter of eight pups, without consanguinity, and the parents showed no congenital abnormality. During the interview, it was reported that the patient was eating and drinking water normally, and presented an active temperament. Moreover, he had one dose of vaccine and a dose of anthelminthic. On clinical examination, all physiological parameters were normal. However, during palpation, it was observed swelling in the ventral thoracic region. For that, we suspected pectus carinatum. Chest radiograph was ...(AU)

Pectus carinatum in a dog / Pectus carinatum em um cão

Background: The deformities of the anterior chest wall are universally designated as pectus deformities. Two types of deformities are known, pectus excavatum and pectus carinatum. Pectus excavatum is a dorsal ventral narrowing of the chest, also known as funnel chest, while pectus carinatum is a ventral protrusion of the sternum, also known as pigeon breast. Both are diseases seen in children and adolescents, however, they are considered rare in small animals, especially pectus carinatum. Thus, the aim of this study is to report a case of pectus carinatum in a Shih-tzu puppy dog. Case: A male canine Shih-tzu, 2 months-old, was present at the veterinary hospital. The owner reported that in the first days of life, the animal felt difficulty breathing when she took him in her lap, and still noticed abnormal increase of the thoracic region. The patient was from a litter of eight pups, without consanguinity, and the parents showed no congenital abnormality. During the interview, it was reported that the patient was eating and drinking water normally, and presented an active temperament. Moreover, he had one dose of vaccine and a dose of anthelminthic. On clinical examination, all physiological parameters were normal. However, during palpation, it was observed swelling in the ventral thoracic region. For that, we suspected pectus carinatum. Chest radiograph was ...

Os principios da inclinação lateral da coluna e compressão dinâmica do tórax para tratamento ortótico concomitante da escoliose associada a deformidades pectus / Los principios de inclinación lateral de la columna vertebral y compresión dinámica del tórax para tratamiento ortótico concomitante de la escoliosis asociada a deformidades pectus / The spine lateral bending and the dynamic chest compression principles for concomitant orthotic treatment of scoliosis and pectus deformities

OBJECTIVE:To investigate a concomitant orthotic treatment for coexisting scoliosis and pectus deformities. No detailed study on such concomitancy was found in literature. METHODS: A spine bending brace for use day and night, and dynamic chest compressor orthoses for use four hours a day, along with one hour of exercises, were prescribed. From 638 adolescents, 25 met the inclusion criteria for a retrospective study. Two groups of patients were identified: A (15 compliant patients) and B (10 non-compliant patients). The mean follow-up was 27 months for group A and 21 months for group B. Pre and post- treatment clinical signs of scoliosis and pectus were photographically compared. The scoliosis had radiologic evaluation by Cobb angle and Nash-Moe classification for vertebral rotation. RESULTS: For both conditions, scoliosis and pectus deformities, the outcome was significantly better in the compliant group (p<0.001 for both). One highly compliant adolescent with a 52º scoliosis and pectus carinatum, showed a surprising improvement to 27º after 40 months of treatment, when chest braces were interrupted and the weaning from the spine brace has started. A 34º scoliosis was maintained after 60 months of follow-up, with vertebral rotation improvement. CONCLUSION: The concomitant orthotic treatment yielded preliminary positive results for compliant patients, warranting investigation continuity.

OBJETIVO: Investigar o tratamento concomitante com órteses para as escolioses e deformidades pectus coexistentes. Nenhum estudo detalhado sobre tal aspecto foi encontrado na literatura. MÉTODOS: Um colete inclinado para uso diuturno e órteses de compressão dinâmica do tórax para uso durante quatro horas diárias, sem serem retiradas para uma série diária de exercícios por uma hora, foram prescritas. De 638 adolescentes, 25 apresentaram critérios de inclusão para estudo retrospectivo. Foram identificados dois grupos de pacientes: A (15 pacientes colaboradores com o tratamento) e B (10 pacientes não-colaboradores). O tempo de seguimento médio foi de 27 meses para o grupo A e de 21 meses para o grupo B. Os sinais clínicos do pectus e da escoliose pré e pós-tratamento foram comparados fotograficamente. A escoliose foi avaliada radiograficamente através do ângulo de Cobb e do método de Nash-Moe para a rotação vertebral. RESULTADOS: Para ambos, escoliose e deformidades pectus, os resultados foram significativamente melhores no grupo A (p < 0.001 em ambas). Uma adolescente altamente colaboradora, com escoliose de 52º e pectus carinatum, apresentou melhora surpreendente para 27º após 40 meses de tratamento, quando o uso das órteses torácicas foi interrompido e o desmame do colete para escoliose foi iniciado. Uma curva de 34º manteve-se após 60 meses de acompanhamento, com melhora da rotação vertebral. CONCLUSÃO: O tratamento ortótico concomitante mostrou resultados preliminares positivos para pacientes colaboradores, sugerindo continuidade de investigação.

OBJETIVO: Investigar un tratamiento concomitante con ortesis para las escoliosis y deformidades pectus coexistentes. No se ha encontrado ningún estudio detallado sobre tal aspecto en la literatura. MÉTODOS: Se prescribió un chaleco inclinado para uso día y noche, y ortesis de compresión dinámica del tórax para utilización durante cuatro horas al día, sin ser retirados para una sesión diaria de una hora de ejercicios. De 638 adolescentes, 25 presentaron criterios de inclusión para estudio retrospectivo. Fueron identificados dos grupos de pacientes: A (15 pacientes colaboradores con el tratamiento) y B (10 pacientes no colaboradores). El tiempo de seguimiento promedio fue 27 meses para el grupo A y 21 meses para el grupo B. Los signos clínicos del pectus y de la escoliosis, pre y postratamiento, fueron comparados fotográficamente. La escoliosis fue evaluada radiográficamente mediante el ángulo de Cobb y el método de Nash-Moe para la rotación vertebral. RESULTADOS: Para ambas deformidades, escoliosis y deformidades pectus, los resultados fueron significativamente mejores en el grupo A (p<0,001 en los dos casos). Una adolescente muy colaboradora, con una escoliosis de 52º y pectus carinatum, presentó una mejoría sorprendente para 27º después de 40 meses de tratamiento, cuando se interrumpió el uso de las ortesis torácicas y se inició la retirada gradual del chaleco para escoliosis. Una curva de 34º se mantuvo después de 60 meses de seguimiento, con mejoría de la rotación vertebral. CONCLUSIÓN: El tratamiento ortótico concomitante mostró resultados preliminares positivos para pacientes colaboradores, sugiriendo que se continúe la investigación.

Experiencia con la construcción quirúrgica de las deformidades de la pared torácica / Surgical experience with reconstruction of chest wall deformities

Analizar las características clínicas, indización para realizar la intervención y los resultados de la cirugía de pacientes con deformidades de la pared torácica. Métodos: Con el fin de obtener la informaci¢ón, se analizaron 32 expedientes clínicos que pudieron ser ubicados y la información incluida en la base de datos del servicio de otros 13 pacientes operados en el servicio de cirugía de tórax del Hospital Rafael Angel Calderón Guardia por Pectus Excavatum o Pectus Carinatum, desde enero de 1998 a enero del 2010. Resultados: Se encontraron 29 pacientes operados por Pectus Excavatum y 16 por Pectus Carinatum, de los cuales 37 fueron varones y 8 mujeres. La edad osciló entre 13 y 24 años con un promedio de 16 años. En 28 pacientes la única indicación para la cirugía fue la afección sicológica causada por la deformidad y en 17 se presentó además algún síntoma como disnea con el ejercicio, dolor torácico o palpitaciones. En 26 de los 29 pacientes operados por Pectus Excavatum se utilizó una barra de metal para manetener el esternón reducido en posición normal mientras se consolidaban retirándola a los seis meses y en los últimos tres las reducción se efectuó con una malla de polipropileno que funcionó igual, pero con la ventaja que no requiere reintervención para retirarla. Los resultados se evaluaron en forma subjetiva de acuerdo a la satisfacción manifestada por el paciente y a la nota del médico; encontrando en un caso de Pectus Excavatum recidiva de la...

Pectus carinatum in a dog / Pectus carinatum em um cão

Descreve-se o atendimento de um cão macho de cinco meses de idade, apresentando diversas malformações congênitas, incluindo uma protrusão ventral da parte distal do externo, que foi diagnosticada como Pectus carinatum tendo por base os achados clínicos e radiográficos.(AU)