Unidade Funcional de Cirurgia Pediátrica, Área da Mulher,da Criança e do Adolescente, Hospital Dona Estefânia, Centro Hospitalar Universitário de Lisboa Central, Lisboa
- Tese de Mestrado 2018 - Master on Minimally Invasive and Robotic Pediatric Surgery, Alma Mater Studiorum - Universidade de Bolonha
Resumo:
The sternum is a bone that only completes its ossification very late and for this reason can deform during its lifelong growth. The transformation of the cartilage by the osteoclasts of the sternal bars after fusion is not uniform and the distribution and number of ossification centers in the sternum segments (sternebra) have many individual variants. Errors in the development of the sternum, especially those related to premature fusion of the ossification centers, can lead to abnormalities and anatomical variations in the anterior chest wall with repercussions throughout the fetal, child, adolescent and young adult growth. Anomalies and variations of the sternum although rare, are possible. Pectus arcuatum is a congenital abnormality of the anterior chest wall with its main origin in the sternum bone. It is also named as Pouter Pigeon Breast, Currarino-Silverman syndrome, Pectus Carinatum type 2 or Chondro-Manubrial Deformity. It is a rare deformity, frequently associated with a congenital heart disease, and is probably caused by premature fusion of the sternal ossification centers and obliteration of the manubriosternal joint. The sternum becomes thick and short with marked anterior angulation at the site of the normal manubriosternal junction or a little lower. The adjacent costal cartilages, usually from second to fifth, also protrude. One third of the patients presented with a concomitant depression of the lower third of sternum (10) and that is why it can easily be mistaken for Pectus excavatum as both may have a similar external presentation, however it results from a mixed form of excavatum and carinatum features. Atypical presentations are also possible and described as anatomic variations with sharp angulation at a lower level than manubriosternal junction and with absence of congenital heart disease. Combining knowledge from embryology and anatomy of the thoracic wall with a bibliographical review and concepts of malleability and elasticity of anterior chest wall described by Donald Nuss since 1998, our aim is to simplify the surgical technique. Our suggestion is based on the opportunity to perform the surgical correction during adolescence, around the time of the growth spurt or just after it, allowing future reshaping of the thorax during the end of puberty.