The pectus care guidelines: best practice consensus guidelines from the joint specialist societies SCTS/MF/CWIG/BOA/BAPS for the treatment of patients with pectus abnormalities.

Pectus defects are a group of congenital conditions found in approximately 1 in 250 people, where the sternum is depressed back towards the spine (excavatum), protrudes forwards (carinatum) or more rarely is a mixture of both (arcuatum or mixed defects). For the majority of patients, it is well tolerated, but some patients are affected psychologically, physiologically or both. The deformity becomes apparent at a young age due to the growth of the ribs and the cartilage that links them to the sternum. The majority of defects are mild and are well tolerated, i.e. they do not affect activity and do not cause psychological harm. However, some young people develop lower self-esteem and depression, causing them to withdraw from activities (such as swimming, dancing) and from interactions that might 'expose' them (such as sleepovers, dating, going to the beach and wearing fashionable clothes). This psychological harm occurs at a crucial time during their physical and social development. A small number of patients have more extreme depression of their sternum that impedes their physiological reserve, which can occur when engaging in strenuous exercise (such as running) but can also limit moderate activity such as walking and climbing stairs. The effects can be so extreme that symptoms occur at rest or cause life-threatening compression of the major blood vessels and organs. The group of patients with physiological impairment usually also suffer from low self-esteem and depression. This paper summarizes the current evidence for the different treatment strategies for this condition, including supportive care, psychological support and non-surgical techniques including bracing and vacuum bell therapy. We also consider surgical techniques including the Ravitch procedure, the Nuss procedure (minimally invasive repair of pectus excavatum), pectus implants and other rare procedures such as Pectus Up. For the majority of patients, supportive care is sufficient, but for a minority, a combination of the other techniques may be considered. This paper also outlines best practice guidance for the delivery of such therapies, including standardized assessment, consent to treatment, audit, quality assurance and long-term support. All the interventions have risks and benefits that the patient, parents and clinicians need to carefully consider and discuss when deciding on the most appropriate course. We hope this evidence review of 'Best Practice for Pectus' will make a significant contribution to those considerations and help all involved, from patients to national policy makers, to deliver the best possible care.

Pectus Disorders: Excavatum, Carinatum and Arcuatum.

This article reviews pectus excavatum, carinatum, and arcuatum. Topics covered include etiology, epidemiology, associated syndromes, physiologic impact, workup, indications for treatment, surgical and nonsurgical therapy, results, complications, and emerging therapies. Pectus excavatum is an inward deformation of the sternum and/or anterior chest wall. Pectus carinatum is ether an outward protrusion or tilt of the sternum with potential psychological impact, but no demonstrated physiologic impact. Nonoperative compression bracing is successful in carinatum patients with chest wall flexibility who are compliant with a bracing program. Pectus arcuatum is an abnormally short, fully fused sternum with a high anterior protrusion.

A 1-Day-Old Girl With Infantile Hemangioma and Sternal Cleft.

CASE PRESENTATION: A newborn girl presented to the hospital on the first day of life because of respiratory failure. She was born at home at 37 weeks' gestation with minimal prenatal care and was found to be small for gestational age. The patient was found to have partial sternal agenesis and sternal cleft, cutis aplasia, left facial hemangioma, micrognathia, wide-spaced nipples, and low-set ears. The mother's and baby's urine toxicology screening were positive for amphetamines. Chest radiographs on admission showed bilateral hazy opacities. CT scan of the chest showed an absent sternum with midline chest wall concavity. The patient was monitored preoperatively in the cardiac ICU for risks of arrythmia, respiratory failure, altered cardiac output, and acute cardiopulmonary decompensation.

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.

A minimally invasive hybrid procedure to correct pectus arcuatum.

BACKGROUND: Pectus arcuatum, also known as horns of steer anomaly or Currarino-Silverman Syndrome, is a distinct chest wall anomaly characterized by severe manubriosternal angulation, a shortened sternum, and mild pectus excavatum. The anomaly is typically repaired using open techniques, employing orthopedic fixation devices. Here, we report the results of a minimally invasive hybrid procedure to repair pectus arcuatum. METHODS: The procedure combines a standard Nuss procedure to correct the depressed sternum with a short upper chest (in boys) or inter-mammary (in girls) incision for bilateral subperichondrial resection of the upper costal cartilages, osteotomy, and correction of the manubrial angulation. The medical records of all patients who underwent the procedure over the last 10 years were reviewed. RESULTS: Five patients, 3 boys and 2 girls, aged 14 to 17 years, underwent the procedure. Three patients had their pectus bars removed 3-4 years after repair. Follow-up after correction ranged from 6 months to 7 years. Good correction resulted in all patients achieving recovery without complications and recurrence. To date, all patients have been satisfied with their results. CONCLUSIONS: The minimally invasive hybrid procedure adequately corrects pectus arcuatum with minimal scarring and high satisfaction.

Sternal cleft and pectus excavatum: an overlooked congenital association?

BACKGROUND: Sternal cleft (SC), a rare thoracic malformation, is associated with pectus excavatum (PE) in 2.6-5% of cases. It remains unclear if these conditions are congenitally linked or if SC repair triggers PE. To investigate the potential higher frequency of PE in SC cases, we conducted a retrospective study of our SC patients. METHODS: We assessed PE incidence, progression, and management in SC patients treated at our institute from 2006 to 2022. When available, we collected pre-SC repair CT scan data, calculating the Haller Index (HI) and Correction Index (CI) and compared them to a selected control group. RESULTS: Among 8 SC patients, 7 had concomitant PE (87.5%), varying in severity. PE management ranged from observation to thoracoplasty, depending on its degree. We observed a significant pre-operative CI difference between SC and control group patients (p < 0.00001). In the last two SC repair cases, we attempted concurrent PE prevention or treatment. CONCLUSION: Our findings suggest an underestimated association between PE and SC in the existing literature. SC patients may exhibit a predisposition to PE from birth, which may become more apparent with growth after SC repair. Consequently, PE prevention or treatment should be considered during SC repair procedures.

An X across the Chest: A Rare Case of a Criss-crossed Sternalis Muscle.

OBJECTIVE: The present case study aims at drawing attention to a very rare presentation of the sternalis muscle noticed during routine dissection, and is intended to highlight the clinical significance and usefulness of this unique muscle in reconstructive surgeries, especially of the breast. CASE REPORT: Though many morphological variants of the muscle have been reported, we came across a unique bilateral sternalis muscle during routine dissection for undergraduate medical teaching, in an 80-year-old male cadaver. The muscle originates on both sides from the external oblique aponeurosis from the fleshy belly, and after becom-ing tendinous, converges in the midline to form a common tendon at the level of the sternal angle, and then splits again into two tendons which become continuous with the ipsilateral sternocleidomastoid. CONCLUSION: Notwithstanding the fact that the presence of a sternalis can be misdiagnosed as a wide range of anterior chest wall lesions and tumors, especially with misdiag-nosis of breast masses in routine mammograms, it has great use as a muscular flap for reconstructive surgeries of the anterior chest wall, head, neck and breast.

Asociación de malformación esternal y displasia vascular en un lactante / Association of sternal malformation and vascular dysplasia in an infant

Se describe el caso clínico de un lactante primogénito de 3 meses de edad, que había sido evaluado por un especialista de Genética Clínica en el Hospital Infantil Norte "Dr. Juan de la Cruz Martínez Maceira" de Santiago de Cuba, por presentar hendidura del tercio inferior del esternón. Posteriormente aparecieron hemangiomas en el labio inferior, el lóbulo de la oreja derecha y la parte lateral derecha del cuello, así como un rafe abdominal medio desde la hendidura esternal hasta el ombligo. Se practicaron los exámenes complementarios requeridos y se interconsultó con varias especialidades, con lo cual se concluyó que el paciente presentaba una asociación de malformación esternal y displasia vascular. Se trata del primer caso notificado en Cuba.

The clinical case of a 3 months first-born infant is described who had been evaluated by a specialist of Clinical Genetics in "Dr. Juan de la Cruz Martínez Maceira" Northern Pediatric Hospital in Santiago de Cuba, for presenting a fissure in the inferior third of the sternum. Later on hemangiomas emerged in the inferior lip, the lobe of the right ear and the lateral right part of the neck, as well as a mid abdominal raphe from the sternal fissure to the navel. The required complementary tests were carried out and she was scheduled for several specialties, with which it was concluded that the patient presented an association of sternal malformation and vascular dysplasia. It is the first case notified in Cuba.

High prevalence of sternal foramen: quantitative, anatomical analysis and its clinical implications in acupuncture practice / Alta prevalencia del foramen esternal: análisis cuantitativo y anatómico y sus implicancias clínicas en la práctica de la acupuntura

The aim of our study was to verify the prevalence of the sternal foramen in adult cadavers and dry bones, as well as to determine its exact localization and relation to acupuncture points. One hundred eighty sternums fixed and contained in 10 percent formaldehyde solution were examined, of which 100 were dry bones and 80 belonged to adult cadavers. The morphometric measures were expressed in millimeters when measuring the length: 1) from jugular incision to the foramen (JI-F); 2) from sternal angle to the foramen (EA-F); 3) from xiphoid process to the foramen (XP-F). Statistical analysis was performed to compare expected vs obtained foramina frequency, using Fisher's test (p0.05 was considered significant). The results showed 16.6 percent sternal foramina, significant higher than expected (p0.0248). Of the 30 foramina, 53.3 percent were found in cadavers, of which one was a woman, and 46.7 percent sternums were found in dry bones. Of the cadavers with sternal foramen, 10 foramina were at the 5th sternochondral articulation level and 6 foramina at the 4th sternochondral level. Of the 14 analyzed dry bones, 9 foramina were at the space between the 4th and the 5th costal pit level and 5 foramina were at the 5th costal pit level. The mean±SD (mm) total length of the sternums was 151.8+/-34 and the measures JI-F, EA-F, and XP-F were, respectively, 103.8+/-22.1mm, 65.6+/-15mm and 46.9 +/-15mm. Knowledge of this occurrence is important to avoid serious heart injury by needle insertion, especially as this area holds a commonly used acupuncture point and sternal puncture...

El objetivo del estudio fue verificar la prevalencia del foramen esternal en cadáveres adultos y huesos secos, así como, determinar su localización y su relación con los puntos de acupuntura. Fueron examinados 180 esternones fijados en solución de formol al 10 por ciento, 100 eran huesos secos y 80 pertenecían a cadáveres de individuos adultos. Las medidas morfométricas se expresaron en milímetros, efectuándose las siguientes mediciones: 1) desde la incisura yugular al foramen; 2) desde el ángulo esternal al foramen y 3) desde el proceso xifoides hasta al foramen. El análisis estadístico permitió comparar la frecuencia de los forámenes, usando el test de Fisher (p 0,05 ). Los resultados mostraron la presencia del foramen esternal en 16,6 por ciento de los casos, siendo significativamente mayor a lo esperado (p 0,0248). De los 30 forámenes, el 53,3 por ciento se encontró en cadáveres y en el 46,7 por ciento en los huesos secos. De los cadáveres con foramen esternal, 10 forámenes se encontraban a nivel de la 5 articulación esternocondral y 6 a nivel de la 4 articulación esternocondral. De los 14 forámenes de los huesos secos, 9 forámenes se encontraban entre entre el 4 y el 5 espacio intercostal y 5 se localizaban a nivel del 5 articulación. La longitud total del esternón fue 151,8 mm +/- 34 y las medidas desde la incisura yugular al foramen, desde el ángulo esternal al foramen y desde el proceso xifoides hasta al foramen fueron, 103,8 +/- 22,1 mm, 65,6 +/- 15 mm y 46,9 +/- 15 mm, respectivamente. El conocimiento de este hecho es importante para evitar la lesión cardiaca grave por la inserción de agujas, ya que esta zona tiene un punto de acupuntura de uso común en la punción esternal...

Pentalogía de Cantrell en un recién nacido expuesto en útero a misoprostol / Pentalogy of Cantrell in a newborn associated with prenatal exposure to misoprostol

El misoprostol es un análogo sintético de la prostaglandina E1 y es usada por sus efectos utero-tónicos como abortivo. En los países donde se utiliza como abortivo sin éxito durante el primer trimestre de gestación, se han informado un espectro de malformaciones congenitas asociadas con la exposición prenatal, que incluyen el síndrome de Moebius, defectos de la pared abdominal, artrogriposis y anomalías por reducción de los miembros, entre otros. La pentalogía de Cantrell (PC), con una prevalencia de 1 en 65.000 nacidos vivos, es un síndrome polimalformativo que consta de 5 anomalías: un defecto de la pared abdominal, defecto esternal inferior, defecto del diafragma anterior, defecto pericárdico diafragmático y anormalidades congenitas del corazón. Se reporta un caso de pentalogía de Cantrell asociado al uso de misoprostol en el primer trimestre de la gestación, siendo este reporte el primer registro de esta asociación.

Misoprostol is a synthetic analogue of prostaglandin E1 and is used for effects such as uterotonic aborti-facient. In countries where abortion is used as unsuccessful in the first trimester of gestation, reported a spectrum of congenital malformations associated with prenatal exposure, which include Moebius syndrome, abdominal wall defects, and anomalies as artrogriposis reduction members, among others. The pentalogy of Cantrell with a prevalence of 1 in 65,000 live births, is a syndrome consisting of 5 abnormalities: a defect in the abdominal wall, lower sternal defect, defect above the diaphragm, diaphragmatic pericardial defect, and congenital abnormalities of the heart. We report a case of Pentalogy of Cantrell associated with the use of misoprostol in the first trimester of pregnancy, this report is the first record of this association.

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.

Pentalogía de Cantrell. A propósito de un caso / Pentalogy of Cantrell. A case report

Ectopia cordis is an extremely rare cardiac anomaly. The heart is localized partially or totally outside the thorax cavity. This anomaly occurs as an isolated defect or combined with others midline defects. Cantrell and colleagues described, in 1958, a syndrome including defects of the abdominal wall, sternum, diaphragm, pericardium and heart. There are few successful surgical cases with this pentalogy. We describe a case with this Cantrell's pentalogy. The cardiac malformation was a univentricular heart with pulmonary stenosis. The patient underwent successful surgical palliation with a systemic-to-pulmonary anastomosis and uneventful recovering.

Hendidura esternal total en un recién nacido y parcial en una niña de 4 años / Cleft sternum: 2 clinical cases

La hendidura esternal es una malformación congénita de la pared torácica que se origina en una falla embrionaria de la fusión de las valvas esternales en la línea media. El defecto debe ser reparado precozmente al nacer, para reestablecer la protección ósea de las estructuras del mediastino, prevenir el movimiento paradojal de las vísceras en la respiración, eliminar la deformidad visible y permitir un desarrollo normal de la caja torácica. Objetivo: Notificar 2 pacientes portadores de esta infrecuente malformación y revisar sus características clínicas, permitiendo realizar un diagnostico preciso, orientar el estudio y definir un adecuado tratamiento. Casos clínicos: Recién nacido masculino con una hendidura esternal completa que provoca un "distress" respiratorio y una preescolar de 4 años, con una hendidura parcial del tercio superior esternal, asociada a una cardiopatía congénita operada. Los pacientes son sometidos a una reparación quirúrgica que permitió un alta precoz y una evolución clínica favorable. Conclusión: La Hendidura Esternal es una malformación de baja frecuencia que debe ser corregida precozmente para evitar el uso de técnicas de mayor complejidad con resultados variables.

Malformaciones de la pared torácica en el adolescente y el adulto: experiencia quirúrgica / Techniques and results in the thoracic wall malformations surgical treatment

Antecedentes: El tratamiento de las anomalías de la pared torácica fue encarado desde los comienzos de la cirugía torácica. Tratándose de una patología de presentación proteiforme, múltiples han sido los métodos que aquella ha propuesto para su resolución. La implicancia psicológica que esta malformación conlleva es por lo general el motivo debido al cual debe ser resuelta. Objetivo: Exposición de técnicas y resultados obtenidos en la resolución de las malformaciones de la pared torácica. Lugar: Hospital Francés de la ciudad de Buenos Aires, Argentina. Diseño: Retrospectivo y descriptivo. Población: 38 pacientes operados con variada patología de pared torácica, atendidos en un hospital de comunidad de Buenos Aires. Método: De acuerdo a los distintos tipos de patologías presentadas fueron utilizadas las osteocondrotomías con soportes metálicos retroesternales y fijación externa con materiales termoplásticos temporarios. En algunas oportunidades fue necesario la aplicación de prótesis acrílicas mamarias. Resultados: De 38 pacientes operados, la conformidad absoluta fue obtenida en el 52 por ciento de los casos, la conformidad en 15 por ciento y se encontraron disconformes en 7,9 por ciento de los casos. La mejoría de la capacidad respiratoria fue escasamente significativa en los casos tratados. Conclusiones: Las malformaciones de la pared torácica se presentan con escasa frecuencia. Deben ser resueltas por su implicancia psicológica. Es una patología proteiforme por lo que han de conocerse las diferentes técnicas propuestas. Las osteocondrotomías con sujeción transtorácica mediante clavos de acero y sujeción externa temporal con plancha termoplástica acrílica sujeta a las costillas subyacentes, dieron muy buenos resultados en la mayoría de los casos

Pectus excavatum: avances en el tratamiento / Pectus excavatum: advances in the treatment

Trabajo presentado en la Sesión Plenaria del Congreso del American College of Surgeons, Santiago, Chile, 2003. Premio al Mejor Trabajo Científico. El pectus excavatum, pecho excavado, hundido o en embudo es la malformación torácica más frecuente. Se presenta en uno de cada 1000 recién nacidos. La deformidad se caracteriza por la presencia de una profunda depresión esternal. En los casos sintomáticos, el manejo es fundamentalmente quirúrgico. El objetivo es reportar una experiencia clínica de 30 pacientes portadores de pectus excavatum tratados con una novedosa técnica quirúrgica mínimamente invasiva con apoyo de videotoracoscopía, denominada Técnica de Nuss, que consiste en el implante, temporal, de una barra metálica retroesternal.

Le corps complexe "Espinhela" au Brésil / The complex body "Espinhela" in Brazil

Aborder une maladie localisée au niveau du plexus solaire, appelée respectivement, prolapsus cartilaginis mucronatae par la médecine traditionnelle du Brésil, muçuaara par les Indiens Tupi, et espinhela caida par la médecine traditionnelle du Brésil, nous a amené à articuler des savoirs spécifiques dans la perspective d'une pensée complexe. Du point de vue ethnographique, nous pouvons distinguer les axes en termes de diagnostic, anatomie, étiologie, guérison magique et de dimensions vitale/fatale de la espinhela. Du point de vue historique, nous veillons à reconstituer la conjonction des composantes indiennes et portugaise, et leur apport à la genèse et au développement de la médecine traditionnelle du Brésil.(AU)

Disorders in the sternal growth and "pectus" deformities: an experimental model and clinical correlation

O crescimento da parede torácica anterior nao tem sido bem explorado. Controvérsia sobre esse assunto inclui a descriçao de suturas no esterno por alguns autores. Os objetivos deste estudo sao demonstrar que placas de crescimento cartilaginosas estao presentes entre os segmentos ósseos do esterno em crescimento e que lesao dessas placas pode causar deformidades pectus. A parede torácica anterior normal de ratos Sprague-Dawley e a de três crianças foram comparadas macroscópica e histologicamente. Foi desenvolvido um modelo experimental no qual a epifisiodese parcial das placas de crescimento esternais resultou em deformidades pectus. Um caso clínico no qual o distúrbio do crescimento do esterno, causado iatrogenicamente por cirurgia, resultou em deformidade pectus em uma criança foi correlacionado com o modelo animal. Placas de crescimento cartilaginosas existem entre os segmentos ósseos do esterno em crescimento. Lesao dessas placas de crescimento resulta em deformidade pectus. Deformidades da parede torácica anterior podem ser causadas por distúrbios de crescimento das placas de crescimento esternais. Lesoes dessas placas de crescimento em um modelo animal experimental e lesoes causadas iatrogenicamente em um paciente demonstram a formaçao de deformidades pectus.

Diagnóstico antenatal de pentalogía de Cantrell / Prenatal diagnosis of Cantrell's pentalogy

Presentamos un caso clínico de diagnóstico antenatal de Pentalogía de Cantrell. Se discute diagnóstico y manejo perinatal