Aortic Arch Replacement via the L-Incision Approach for Stanford Type A Aortic Dissection with Pectus Excavatum / 日本心臓血管外科学会雑誌

A 65-year-old woman who had been diagnosed with a thoracic aneurysm was admitted to our hospital because of loss of consciousness. Brain CT revealed that the left corticomedullary junction is obscured. Contrast-enhanced CT demonstrated an acute type A aortic dissection with right internal carotid artery occlusion, left internal carotid artery stenosis, and severe pectus excavatum. Although the consciousness level at the time of admission was JCS200, it gradually improved and she regained spontaneous movement of the right side of her body. Repair of the acute type A dissection was indicated because her neurological deficit had improved. The surgery was performed via an L-shaped approach consisting of a median sternotomy and a left 5th intercostal thoracotomy with moderate hypothermic circulatory arrest and selective cerebral perfusion. An entry was found in the aortic arch between the origins of the brachiocephalic artery and the left common carotid artery, and a partial arch replacement was performed using a four-branched artificial graft. Although the right hemiparesis remained, she recovered well and was transferred to a rehabilitation hospital at 45 days postoperatively. The L-incision approach obtained a good surgical field in a patient with a type A dissection and severe pectus excavatum.

Cardiac anomalies in pediatric patients with pectus excavatum

SUMMARY OBJECTIVE: Pectus excavatum is the most prevalently encountered deformity of the thoracic wall. It can be accompanied by congenital anomalies. METHODS: The cardiac findings of 36 children who were diagnosed at the Thoracic surgery outpatient clinic of our university between 10 February 2021 and 1 October 2021 and 57 healthy children in a similar age group were analyzed. RESULTS: We determined that the pectus excavatum patients in our study had a higher risk of having mitral insufficiency, mitral valve prolapse, tricuspid valve prolapse, cardiac malposition, and congenital heart disease. CONCLUSION: Our study showed that the prevalence of cardiac pathologies was higher in pediatric pectus excavatum patients than in healthy children in the control group. Thus, we recommend clinicians to refer pediatric pectus excavatum patients to pediatric cardiology outpatient clinics for the early diagnosis of potential cardiac pathologies.

Clinical efficacy of thoracoscopy-assisted modified Nuss procedure in children with pectus excavatum: A retrospective analysis in a single center / 中国胸心血管外科临床杂志

@#Objective    To explore the clinical efficacy of thoracoscopy-assisted modified Nuss procedure for pectus excavatum (PE) in children. Methods    The clinical data of patients with PE who underwent thoracoscopy-assisted modified Nuss procedure from October 2013 to October 2020 in Daping Hospital were retrospectively analyzed. Results    A total of 86 patients were collected, including 79 males and 7 females with a mean age of 14.03±3.36 years. The operations were performed successfully in all patients without intraoperative cardiac vascular injury or perioperative death. The mean operation time was 87.30±33.45 min, bleeding volume was 19.94±14.60 mL, and the postoperative hospitalization stay time was 6.89±2.59 d. Early postoperative complications included 2 patients of pneumothorax, 2 patients of wound fat liquefaction and infection, 2 patients of bar flipping and displacement. One patient had bar displacement 1 year after the surgery. The total complication rate was 8.14%. All patients were followed up for 3-42 months. The bars were taken out about 36 months after the surgery. According to the evaluation criteria of orthopedic effect, 68 (79.07%) patients were excellent, 10 (11.63%) patients were good, 5 (5.81%) patients were moderate and 3 (3.49%) patients were poor. Conclusion    Minimally invasive and individualized shaping via the Nuss procedure for PE children is safe and convenient, with satisfied effect. It is worthy of popularization in the clinic.

Epidural Analgesia and Abnormal Coagulation in Patients Undergoing Minimal Invasive Repair of Pectus Excavatum

Background:Epidural analgesia (EA) is effective in patients undergoing minimal invasive repair of pectus excavatum(MIRPE) but is associated with major complications such as epidural hematomas. It is recommended to assess coagulation status in patients receiving anticoagulant therapy prior to EA, although no consensus exists in patients without a history of bleeding tendency or anticoagulant therapy. Thus, the aim of this paper was to assess 1) the prevalence of abnormal routine coagulation parameters, i.e., international normalized ratio (INR) and platelet count, and 2) the safety of EA in patients undergoing MIRPE. Methods: In this retrospective study, we identified 1,973 patients undergoing MIRPE at our center between 2001 and 2019. Complications related to EA were registered for all patients. Information on coagulation parameters was present in 929 patients. Patients with spontaneously elevated INR ?1.5 were referred for assessment of coagulation factor VII in order to assess the cause of the elevated INR. Results: Of 929 patients with coagulation information available, 18 patients had spontaneously elevated INR ?1.5 (1.9%). In patients with INR ?1.5, 12 patients underwent further assessment of factor VII, with all patients having a slightly reduced factor VII close to the lower reference range. The majority of the 1,973 patients undergoing MIRPE received EA (99.6%) with very low complication rates (0.2%) and no incidence of epidural hematomas. Conclusion: In patients undergoing MIRPE, coagulation screening prior to EA should not be mandatory as it revealed no clinically relevant consequences. EA is safe with very low complication rates

Abordagem de pectus carinatum na Atenção Primária e seu impacto na saúde emocional do paciente: relato de caso / Pectus carinatum approach in primary health care and its impact on the emotional health of the patient: case report / Abordaje del Pectus Carinatum en Atención Primaria y su impacto en la salud emocional del paciente: caso clínico

Introdução: O pectus carinatum (PC) é uma deformidade incomum da parede torácica, que geralmente surge na infância e se acentua na adolescência. Essa malformação pode levar a distúrbios estéticos e de autoestima, além de problemas respiratórios, que se devem à restrição da parede torácica. Existem poucos estudos brasileiros dedicados exclusivamente ao PC, e a maioria desses trabalhos direciona-se ao estudo do pectus excavatum (PE) ou de ambas as condições de forma conjunta. Apresentação do caso: Apresenta-se o caso de um paciente de 13 anos, masculino, que procura atendimento na Atenção Primária à Saúde (APS) por surgimento de tumefação em região paraesternal. Nega sintomas físicos, porém relata constrangimento e desconforto estético com a presença da proeminência. Conclusões: A abordagem multidisciplinar da deformidade torácica é fundamental na aceitação e no tratamento, tendo a APS um papel essencial Toda a equipe deve auxiliar e proporcionar, por meio do tratamento, melhor qualidade de vida estética, física e emocional ao paciente, fatores estes que nos impulsionaram a documentar este caso.

Introduction: Pectus carinatum (PC) is an unusual deformity of the chest wall that usually arises in childhood and becomes more pronounced in adolescence. This malformation can lead to aesthetic and self-esteem disorders, in addition to respiratory problems, due to the restriction of the chest wall. There are few Brazilian studies dedicated exclusively to PC, and most are directed at pectus excavatum or the investigation of both conditions jointly. Presentation of the case: In this case, a 13-year-old male sought medical attention in primary health care (PHC) because of the appearance of tumefaction in the parasternal region. He denied physical symptoms but reported embarrassment and aesthetic discomfort with the presence of the prominence. Conclusions: The multidisciplinary approach of thoracic deformity is fundamental in acceptance and treatment, with PHC playing an essential role, since all the team must assist and provide, through treatment, a better aesthetic, physical and emotional quality of life to the patient, and these factors led us to document this case.

Introducción: El pectus carinatum (PC) es una deformidad poco frecuente de la pared torácica, que suele surgir en la infancia y se acentúa en la adolescencia. Esta malformación puede provocar trastornos estéticos y de autoestima, así como problemas respiratorios, que se deben a la restricción de la pared torácica. Hay pocos estudios brasileños dedicados exclusivamente al CP, y la mayoría de los trabajos están dirigidos al estudio del Pectus Excavatum (PE) o al estudio de ambas condiciones conjuntamente. Presentación del caso: Presentamos el caso de un paciente masculino de 13 años que acude a Atención Primaria de Salud (APS) por la aparición de una tumefacción en la región paraesternal. Niega síntomas físicos, pero refiere malestar y vergüenza estética con la presencia de prominencia. Conclusiones: El abordaje multidisciplinar de la deformidad torácica es fundamental para su aceptación y tratamiento, teniendo la APS un papel fundamental, ya que todo equipo debe asistir y proporcionar, a través del tratamiento, una mejor calidad de vida estética, física y emocional al paciente, factores que nos han llevado a documentar este caso.

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.

Pectus excavatum em uma ninhada de Buldogue Francês: relato de caso / Pectus excavatum in a French Bulldog Litter: case report

RESUMO O pectus excavatum é considerado uma patologia da parede torácica, onde há convexidade no aspecto ventral do esterno, o que gera complicações secundárias, como alongamento ventrodorsal do tórax e aumento da pressão intratorácica, entre outras. No presente caso, o corpo de um cachorro Buldogue Francês nasceu com sinais prévios de decaimento e baixo consumo de leito materno, além de uma avaliação médica forense. De acordo com a avaliação patológica, foi encontrada uma fenda no peito esternal relacionada às esternas caudais próximas à cartilagem xifóide, conteúdo espumoso na cartilagem epiglótica da laringe e sinais graves de enfisema nos lobos caudal, craniano e médio pulmonar. Na avaliação cardiológica, foi observada assimetria morfológica invaginante no septo interventricular na mesma área em que a fenda esternal ocorre, por sua vez, na avaliação radiográfica, evidencia uma depressão dorsal do terço caudal do esterno com alterações consideráveis na silhueta cardíaca. Na Colômbia não há relatos de ninhadas completas que apresentem esta alteração, portanto, pretende-se que este seja o primeiro relatório anatomopatológico e de imagem que descreva a patologia em caninos.

ABSTRACT Pectus excavatum is considered a pathology of the chest wall where there is convexity in the ventral aspect of the sternum, which generates secondary complications, such as ventrodorsal stretching of the chest and increased intrathoracic pressure, among others. In the present case, the body of a French bulldog dog was born with previous signs of decay and low consumption of maternal waste, in addition to a forensic medical evaluation. According to the pathological evaluation, a crack was found in the sternal chest related to the caudal sternum close to the xiphoid cartilage, frothy content in the laryngeal epiglottis cartilage and severe signs of emphysema in the caudal, cranial and middle lung lobes. In the cardiological evaluation, an invaginating morphological asymmetry was observed in the interventricular septum in the same area in which the sternal cleft occurs, in turn, in the radiographic evaluation, it shows a dorsal depression of the caudal third of the sternum with considerable changes in the cardiac silhouette. In Colombia there are no reports of complete litters that present this alteration, so it is intended that this is the first anatomopathological and imaging report that describes the pathology in canines.

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.

Broad pectus excavatum treatment: long term results of a brazilian technique / Tratamento do pectus excavatum amplo: resultados a longo prazo de uma técnica brasileira

ABSTRACT Objective: This study aims the treatment results of broad pectus excavatum after a long-term follow-up and skeletal maturity. Methods: Eighty-four children and adolescents with broad-type pectus excavatum were selected for evaluation after treatment with a dynamic orthosis that applies compression to the lower rib projections and prescription of exercises. The broad pectus excavatum was defined as a deformity that the depressed area was greater and covered the area above and below the nipple line. All patients were evaluated for more than 1 year after the end of treatment and skeletal maturity. Post-treatment results were categorized as mild, moderate and severe. Statistic correlations between results and deformity flexibility, deformity severity, and adherence to treatment were assessed. Results: The mean age at the beginning of treatment was 13.3 years, and the follow-up duration was 25.7 months after suspension of orthosis use. Forty-eight percent of patients showed good results. With regular use of orthoses and performance of exercises, this rate increased to 70% (p < 0,001). Mild cases showed more success than severe cases (p = 0,007). Initial flexibility didn't influence the results (p = 0,63). Conclusion: Treatment of broad pectus excavatum with orthoses and exercises led to good definitive results in most resilient patients, especially in those with mild deformities. Level of Evidence V, Expert Opinion.

RESUMO Objetivo: Estudar os resultados de longo prazo e com seguimento até a maturidade esquelética do tratamento do pectus excavatum amplo. Métodos: 84 crianças e adolescentes foram tratados com uma órtese que aplicacompressão nas saliências costais inferiores, associada a exercícios específicos. A deformidade foi classificada como ampla quando a depressão tem maior extensão e abrange uma área acima e abaixo da linha mamilar. Os resultados foram categorizados em ruim, regular ou bom, sendo correlacionados estatisticamente com a flexibilidade, a gravidade da deformidade e a adesão ao tratamento, com avaliação um ano após o fim do tratamento e na maturidade esquelética. Resultados: A idade média no início do tratamento foi de 13,7 anos e o seguimento médio foi de 25.7 meses após a suspensão do uso da órtese. 48% dos casos apresentaram sucesso com o tratamento, mas quando os exercícios e o uso da órtese foram regulares, esta taxa aumentou para 70% (p < 0,001). Os casos mais leves tiveram maior sucesso que os de maior gravidade (p = 0,007), mas a flexibilidade inicial não influenciou os resultados (p = 0,63). Conclusão: O tratamento do pectus excavatum amplo com o uso de órtese e exercícios apresentou bons resultados definitivos na maioria dos pacientes resilientes, em especial nos casos mais leves. Nível de Evidência V, Opinião do Especialista.

A novel modified Nuss procedure without plate turnover for treatment of adult recurrent pectus excavatum / 中国胸心血管外科临床杂志

@#Objective    To summarize the experience of treating adult recurrent pectus excavatum without plate turnover. Methods    Twenty-seven patients with recurrent pectus excavatum treated by thoracoscopy-assisted placement without plate turnover from 2010 to 2019 in our hospital were enrolled. There were 23 males and 4 females with the age of 3-29 (12.81±7.79) years at the first operation, and 18-29 (21.74±3.56) years at this operation. Incision of 2-3 cm at bilateral axillary midline of the deepest point of pectus excavatum was made, and an auxiliary incision under xiphoid process was adopted according to the intraoperative situation. Results    All patients underwent thoracoscopy-assisted correction of pectus excavatum without bar turnover, and subxiphoid incision was performed in 11 patients. Twenty-five patients had one bar placed, and two patients required two bars. The operation time was 28-45 (33.00±6.44) min. Postoperative Haller index (2.95±0.40) was improved compared with preoperation (4.63±1.03). The postoperative hospital stay was 4-6 (4.00±0.32) day. All patients were followed up for 1-8 years. Complications included poor wound healing in 1 patient, and steel wire fracture and displacement in 1 patient. There was no plate rotation or bar displacement. Fourteen patients removed the bar 29-84 (40.36±13.93) months after the placement. Haller index was improved to 2.43-3.61 (2.86±0.35) during removal of steel plate. Untill June 2020, there was no recurrence of pectus excavatum. Conclusion    The treatment of adult recurrent pectus excavatum without plate turnover is satisfactory, and the protection of intercostal muscle and firm fixation is the key to ensure the success of operation and long-term effects.

Progress in surgical treatment of pectus excavatum / 中国胸心血管外科临床杂志

@#The surgical treatment of pectus excavatum has a history of more than 100 years, which has gone through from traditional open surgery to the current popular minimally invasive surgery. Nuss procedure, as the most classic minimally invasive operation, has been improved in accordance with the clinical needs since its inception to achieve fewer complications and better results, but there are still limitations that are difficult to break through, attracting a large number of scholars to make continuous innovation and develop updated devices and operation methods. This article reviews the history of funnel chest surgery, application and improvement of Nuss operation, double compression and complete fixation bar system and Wang procedure.

Pectus excavatum, manejo mínimamente invasivo con técnica de Nuss: experiencia Instituto Nacional del Tórax / Pectus excavatum, minimally invasive management with Nuss technique

Resumen Objetivo Comunicar la experiencia en el tratamiento de pectus excavatum en el Instituto Nacional del Tórax. Material y Método: Estudio descriptivo con seguimiento. Se recolectaron datos de las variables de interés, mediante la revisión de fichas clínicas. Se incluyeron pacientes operados entre marzo de 2007 y abril de 2018. Resultados Se incluyeron 86 pacientes operados con técnica mínimamente invasiva en el período descrito. De ellos, 74 pacientes de sexo masculino. El promedio de edad al momento de la cirugía fue de 17,8 años. El principal motivo de consulta fue por desmedro estético que correspondió al 41,8% de los pacientes. Salvo en 1 paciente, en todo el resto se ocupó una sola barra. En nuestra serie hubo 12 pacientes que presentaron alguna complicación operatoria y 1 paciente fallecido. Hasta el cierre del seguimiento se retiraron 61 barras, 2 de ellas previo al período estipulado de tratamiento, por morbilidad. Hay 20 barras in situ y 4 pacientes de los cuales no se tiene registro por abandono de controles. Discusión El pectus excavatum es la más frecuente de las deformidades de la pared torácica, es 4 a 6 veces más frecuente en hombres que en mujeres. En general los pacientes son asintomáticos, aunque algunos pueden presentar síntomas cardiopulmonares. La reparación con cirugía mínimamente invasiva con técnica de Nuss aparece hoy en día como el gold standard de manejo. Conclusión El manejo de los pacientes con pectus excavatum en nuestra Institución se asemeja a lo reportado en la literatura internacional. Nuestros esfuerzos deben apuntar a disminuir la morbimortalidad asociada.

Aim To communicate the experience in the treatment of pectus excavatum in the National Institute of Thorax. Materials and Method: Descriptive study with follow-up. Data of variables of interest were collected through the review of clinical records. Patients operated between March 2007 and April 2018 were included. Results 86 patients operated with the Nuss technique were included in the period described. Of them, 74 male patients. The average age at the time of surgery was 17.8 years. The main reason for consultation was due to cosmetic detriment that corresponded to 41.8% of the patients. Except in 1 patient, in all the rest a single bar was occupied. In our series, there were 12 patients who presented some operative complication and 1 patient died. Up to the end of the follow-up, 61 bars were removed, 2 of them in non-scheduled surgery. There are 20 bars in situ and 4 patients of which there is no registration due to abandonment of controls. Discussion Pectus excavatum is the most frequent of the deformities of the chest wall, it is 4 to 6 times more frequent in men than in women. In general, patients are asymptomatic, although some may have cardiopulmonary symptoms. The repair with minimally invasive surgery with Nuss technique appears today as the goldstandard of management. Conclusion The management of patients with pectus excavatum in our Institution is similar to that reported in the international literature. Our efforts should aim to reduce the associated morbidity and mortality.

Surgical design and novel modified Nuss procedure for pectus excavatum / 中国胸心血管外科临床杂志

@#Objective    To explore the surgical design and efficacy of novel modified Nuss procedure for pectus excavatum (PE). Methods    We retrospectively analyzed the clinical data of 903 patients with PE who were treated by the new kind of steel bar and the novel modified Nuss procedure. There were 716 males and 187 females at mean age of 2-45 (12.1±6.8) years. Preoperative chest CT scans Haller index (HI) was 3.1-15.2 (4.6±1.3), with 38 patients of mild PE (HI<3.2), 302 patients of moderate PE (HI 3.2-3.5), 521 patients of severe PE (HI 3.6-6.0), and 42 patients of extremely severe PE (HI>6.0). The operative time, operative blood loss, hospital stay time as well as postoperative complications were reviewed and analyzed. Results    All of the 903 patients successfully completed the surgery. The mean operative time was 20-45 (25.2±2.6) min for primary PE and that for special type of PE (including recurring PE, PE patients after heart disease operation and those corrected by 2 bars) was 48-150 (63.5±28.1) min. Blood loss was less than 10 mL for primary PE and 15-50 (23.5±5.5) mL for special type of PE. Postoperative hospital stay was 3-15 (4.5±1.6) d. A total of 845 patients (93.6%) required 1 steel bar insertion, 58 patients (6.4%) required 2 steel bars. Postoperative evaluation of the surgery outcomes revealed the following: excellent in 805 patients, good in 84 patients, fair in 14 patients and poor in 0. The good quality rate was 98.4%. Conclusion    Novel modified Nuss procedure can simplify and optimize the surgical design with good short and mid-term effects.

Advances in genetic etiology research of congenital chest wall deformity / 中国胸心血管外科临床杂志

@#Congenital chest deformity is caused by abnormal development of spine or ribs, resulting in sternal depression or protrusion. Pectus carinatum and pectus excavatum are the most common diseases in clinic, which can either be accompanied by other syndromes or exist alone. The genetic factors of congenital thoracic deformity can be related to single gene mutation, polygene mutation and chromosome aberration. Common clinical congenital thoracic deformity with syndromes, such as Marfan syndrome and Noonan syndrome, often have relatively fixed and clear pathogenic genes. The genetic pathogenesis of non-syndromic and independent congenital thoracic malformations is usually diverse, and treatments for syndromic and non-syndromic congenital thoracic deformity are different. Therefore, it is necessary for us to differentiate syndromic and non-syndromic congenital thoracic deformities in basic research, clinical diagnosis and treatment.

Early results of thoracic remodeling for patients with bar removal after pectus excavatum correction in minimal-procedure / 中华胸心血管外科杂志

Objective To study thoracic re-remodeling and therapeutic effect after the bar removal for pectus excavatum corrected by minimal-invasive technique.Methods 145 cases with pectus excavatum,male 115,femal 30;adults in 59,children 86;corrected by minimal-invasive technique improved and performed by the same group surgeon.Bar removed 12-82 months after the procedure,appraising index of curative effects include in chest appearance,thoracic index,thoracic computer tomography(CT) and the distance between the behind of sternum to the anterior border of thoracic spine in the sagittal view.Results The chest shape was good.Thoracic index:before bar removal 2.36 ± 0.32 in children,2.60 ± 0.45 in adults;after that,2.77 ± 0.44 in children,3.04 ± 0.56 in adults.There was all subsidence on the each point of the sternum,descent the maximum at the inferior end of the midsternum,(15.18 ±7.95)mm in children,(14.93 ± 8.81) mm in adults,comparing with bar removal before and after.There was statistical significance.Not the signs of compressing the heart on the CT view.The time interval of the bar removed 3-year in children,5-year in adults without affecting the development of the patients' thorax.Conclusion The sternum descended slightly after bar removal when pectus excavatum corrected to expecting effects.After that,thoracic remodeling again,the chest shape well.

A 3.06-Mb interstitial deletion on 12p11.22-12.1 caused brachydactyly type E combined with pectus carinatum / 中华医学杂志(英文版)

Background@#Brachydactyly, a developmental disorder, refers to shortening of hands/feet due to small or missing metacarpals/metatarsals and/or phalanges. Isolated brachydactyly type E (BDE), characterized by shortened metacarpals and/or metatarsals, consists in a small proportion of patients with Homeobox D13 (HOXD13) or parathyroid-hormone-like hormone (PTHLH) mutations. BDE is often accompanied by other anomalies that are parts of many congenital syndromes. In this study, we investigated a Chinese family presented with BDE combined with pectus carinatum and short stature.@*Methods@#A four-generation Chinese family was recruited in June 2016. After informed consent was obtained, venous blood was collected, and genomic DNA was extracted by standard procedures. Whole-exome sequencing was performed to screen pathogenic mutation, array comparative genomic hybridization (Array-CGH) analysis was used to analyze copy number variations, and quantitative real-time polymerase chain reaction (PCR), stride over breakpoint PCR (gap-PCR), and Sanger sequencing were performed to confirm the candidate variation.@*Results@#A 3.06-Mb deletion (chr12:25473650–28536747) was identified and segregated with the phenotype in this family. The deletion region encompasses 23 annotated genes, one of which is PTHLH which has been reported to be causative to the BDE. PTHLH is an important regulator of endochondral bone development. The affected individuals showed bilateral, severe, and generalized brachydactyly with short stature, pectus carinatum, and prematurely fusion of epiphyses. The feature of pectus carinatum has not been described in the PTHLH-related BDE patients previously.@*Conclusions@#The haploinsufficiency of PTHLH might be responsible for the disease in this family. This study has expanded the knowledge on the phenotypic presentation of PTHLH variation.

Sternal suspension technique in treatment of pectus excavatum after open repair of congenital heart disease / 中华胸心血管外科杂志

Objective@#To evaluate the efficacy of using sternal suspension technique in correcting pectus excavatum after open repair of congenital heart disease and improve the safety of this technique.@*Methods@#A retrospective study was conducted for 8 cases of pectus excavatum treated with sternal suspension procedure after open repair of congenital heart disease from October 2005 to October 2018 in our institution, 5 males and 3 females. The median age of patients was 5.75 years (range 3.75-12.33 years) and the median Haller index was 4.20(range 3.60-5.19). All patients underwent cardiac ultrasound and chest CT scan to evaluate the severity retrosternal adhesions.@*Results@#All patients underwent the sternal suspension procedure successfully with a median operation time 55 min (range 30-230 min) and a median blood loss of 2 ml (range 2-120 ml). There was one patient suffered pneumothorax after operation. The patients were followed up for 4 months to 13 years with an excellent and good outcome.@*Conclusion@#Sternal suspension technique is a safe and effective procedure for pectus excavatum. To improve the security and reduce the incidence of cardiac injury, sternal suspension technique is a promising alternative for pectus patients with severe adhesion after open repair of congenital heart disease.

A control study of measuring thoracic cage indexes in 3-17 years old healthy children and children with pectus excavatum / 中华胸心血管外科杂志

Objective@#To provide data references for Nuss procedure biomechanics research, and to understand morphological pathological changes in children with pectus excavatum.@*Methods@#A retrospective study was conducted for 434 cases of pectus excavatum from January 2009 to December 2015 in our institution. The anterior rib width(RW), anterior intercostal space width(IS) and thickness of the rib in the middle(RT) on the right side of 3-7 ribs had been measured in each patient. The widths were compared between the IS of children with pectus excavatum and the bar which currently used in clinic. The measurement indexes were compared between healthy children and children with pectus excavatum.@*Results@#The IS of prepubertal children with pectus excavatum was less than the width of bar which currently used in clinic (12.7 mm). Children with pectus excavatum compared with healthy children, the IS from 4 to 6 and the 5th RW of children with pectus excavatum were less than healthy children (P<0.05), the 3rd and 4th RT were greater than healthy children (P<0.05).@*Conclusion@#The measured values will be useful for the improvement of pectus bar and Nuss procedure biomechanics research. By morphology contrast, it reflects the pectus excavatum deformity has some restriction in thoracic development and the cardiopulmonary function.

Experience with Wang procedure for treatment of pectus excavatum in young children / 南方医科大学学报

OBJECTIVE@#To review the experience with Wang procedure for treatment of pectus excavatum in young children.@*METHODS@#The clinical data of 21 children with a mean age of 3.3 ± 1.1 years (ranging from 1.5-6 years) undergoing Wang procedure for pectus excavatum were analyzed. A longitudinal incision (1 to 2 cm) was made in the front of the xiphoid, and two tunnels were created using steel bars beneath the muscles on two sides of the chest wall. The fibrous tissue between the diaphragm and the sternum was dissociated, and the steel wires were sutured through the deformed chest wall. After the steel bar was placed in the tunnels, the wires were pulled and fixed in the middle of the bar, and the incision was sutured.@*RESULTS@#All the operations were performed using 3 wires and 1 steel bar. The operation time was 25 to 51 (38.1 ± 9.6) min with an intraoperative bleeding volume of 5 to 10 (7.1±1.5) mL. The time of hospitalization of the patients ranged from 6 to 10 days (mean 8.1±1.3 days). In all the patients, the incision healed smoothly without serious pain or obvious complications. All the patients were followed up for 1 to 13 months after the operation. During the follow- up, no recess recurred and no such complications as bar displacement or transposition occurred. According to the evaluation criteria after pectus excavatum operation, 13 cases had a total score of 9, and 8 had a total score of 8. The overall effect was satisfactory, and there were no cases rated as basically satisfactory or unsatisfactory.@*CONCLUSIONS@#Wang procedure is a good option for treatment of pectus excavatum in young children.