[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.

Pectus carinatum.

Among the deformities of the thoracic wall,pectus carinatum has not received the same attention as has pectus excavatum. Few pulmonologists, pediatricians, and thoracic surgeons are aware of the approaches to treating this condition. As a consequence, patients with pectus carinatum are not referred for treatment. This deformity, with an incidence of 1:1000 teenagers, is oligosymptomatic. However, for aesthetic and emotional reasons, it accounts for a large number of medical appointments. Such patients are introverted and do not engage in physical activities, since they are unwilling to expose their chest, which also discourages them from going to the beach or to swimming pools. The diagnosis is clinical and visual, and details are obtained through chest X-rays and computed tomography. The treatment is based on a well-known organogram that summarizes orthopedic and surgical procedures. Dynamic compression, combined with physical exercises, is indicated for teenagers with flexible thorax in inferior and lateral pectus carinatum, with limited indication for those with superior pectus carinatum. For individuals of any age with rigid thorax, surgery is indicated for aesthetic reasons. Among the techniques described, the modified sternum chondroplasty stands out due to the excellent aesthetic results achieved.

Pectus carinatum / Pectus carinatum

Dentre as deformidades da parede torácica, o pectus carinatum não tem recebido o mesmo grau de interesse que o pectus excavatum, sendo sua abordagem desconhecida por grande parcela dos pneumologistas, pediatras e cirurgiões torácicos. Isto faz com que estes pacientes não sejam encaminhados para tratamento. Trata-se de deformidade com incidência de 1:1000 adolescentes, oligosintomática, mas que leva a consultas médicas por implicações de ordem estética e emocional, sendo seus portadores introvertidos, não praticantes de exercícios físicos e não freqüentadores de praias ou piscinas para não expor o tórax. O diagnóstico é clínico e visual, e detalhes são obtidos com a radiografia do tórax e a tomografia computadorizada. O tratamento é baseado em organograma bem conhecido, que resume as condutas ortopédicas e cirúrgicas. O compressor dinâmico do tórax, associado a exercícios físicos, é indicado nos adolescentes com tórax flexível no pectus carinatum inferior e pectus carinatum lateral, tendo indicação limitada no pectus carinatum superior. A indicação cirúrgica é feita pela presença da deformidade e com motivação estética, em adolescentes com tórax não flexível, em jovens, e em adultos. Dentre as técnicas relatadas, destaca-se a esternocondroplastia modificada, pelos excelentes resultados estéticos alcançados.

Among the deformities of the thoracic wall,pectus carinatum has not received the same attention as has pectus excavatum. Few pulmonologists, pediatricians, and thoracic surgeons are aware of the approaches to treating this condition. As a consequence, patients with pectus carinatum are not referred for treatment. This deformity, with an incidence of 1:1000 teenagers, is oligosymptomatic. However, for aesthetic and emotional reasons, it accounts for a large number of medical appointments. Such patients are introverted and do not engage in physical activities, since they are unwilling to expose their chest, which also discourages them from going to the beach or to swimming pools. The diagnosis is clinical and visual, and details are obtained through chest X-rays and computed tomography. The treatment is based on a well-known organogram that summarizes orthopedic and surgical procedures. Dynamic compression, combined with physical exercises, is indicated for teenagers with flexible thorax in inferior and lateral pectus carinatum, with limited indication for those with superior pectus carinatum. For individuals of any age with rigid thorax, surgery is indicated for aesthetic reasons. Among the techniques described, the modified sternum chondroplasty stands out due to the excellent aesthetic results achieved.

Videotoracoscopia en el tratamiento del pectus excavatum / Videothoracoscopy in the treatment of pectus excavatum

El Pectus Excavatum o tórax de zapatero es la malformación más frecuente de la cara anterior del tórax. Desde que el Profesor de Cirugía Sauerbruch en 1920 intervino por primera vez un paciente adulto-joven, la técnica quirúrgica abierta con tracción postoperatoria se continuó aplicando; hasta que; Ravitch en 1947 publica su experiencia eliminando la tracción toráxica post operatoria. Desde entonces se suceden variaciones en la técnica manteniendo el principio básico de resección de cartílagos y remodelaje de la posición esternal. El gran cambio se produce con la innovación de la cirugía toráxica guiada por fibra óptica: la Videotoracoscopia (VTC).

Growth disturbance of the sternum and pectus deformities: imaging studies and clinical correlation.

BACKGROUND: Radiologic reports of "normal" chest are not uncommon when there clearly are irregularities of sternal ossification and maturation. Analysis of imaging studies of sternal deformities for growth disturbances is not common in the literature and is addressed in this manuscript. OBJECTIVE: To determine the influence of sternal growth on development of pectus deformities and correlate imaging studies with clinical aspects of different types of these deformities. MATERIAL AND METHODS: One hundred forty-one children and adolescents with pectus deformities were evaluated. Sternal growth was estimated through the development of radiographic indices that were available for 57 patients with pectus deformities and for 71 controls. Magnetic resonance imaging of the sternum was performed in two patients to correlate with radiographic information. RESULTS: Radiographic indices of the sternum suggested growth disturbances in three basic types of pectus carinatum deformities: superior, inferior and lateral, and in the localized type of pectus excavatum. CONCLUSION: Sternal growth seems to have an important influence on the development of carinatum superior; partial influence on carinatum inferior, carinatum lateral, and excavatum localized; and no influence on excavatum wide pectus deformities. The endochondral growth of the sternum and costal arches is an important concept that aids in the interpretation of imaging studies and the orthopedic approach to management of these deformities in children and adolescents.

Deformidades "pectus": novos conceitos e abordagem ortopédica na infância e adolescência: 1ª parte / Pectus deformities: new concepts and orthopedic approach in children and adolescents: 1st part

As deformidades da parede torácica anterior ou deformidades pectus em crianças e adolescentes têm sido tratadas por especialistas que nao estao acostumados a lidar com a fisiologia do crescimento ósseo e cartilagens, e que leva a equívocos e resultados questionáveis. Estudos sobre a etiologia, patogeina e os resultados obtidos com um tratamento ortótico sao descritos pelo autor, emnfatizando que as deformidades pectus dizem respeito à ortopedia.

Pectus carinatum: enfoque atual / Pectus carinatum: current approach

Desde 1977 os autores vêm analisando e tratando crianças e adolescentes portadores de deformidades em protrusäo da parede torácica anterior (pectus carinatum). Demonstram, através da análise radiográfica de tomografias do esterno, que o desenvolvimento deste osso tem importante papel na gênese dessas deformidades. Baseados nos estudos clínicos e radiológicos feitos ao longo dos últimos onze anos, apresentaram também uma classificaçäo própria e atualizada dessas deformidades. Demostraram ainda ser o tratamento conservador, através da utilizaçäo de órtese apropriada, viável e passível de bons resultados, conforme o tipo de deformidade e a idade do paciente