Original Article
Open-surgery repair of congenital malformation of the chest: indications, technical tips and outcomes
Abstract
Background: Congenital malformations of the chest may influence significantly the life of the patients, because of functional or psychological impairment. The interest for these anomalies has dramatically increased during the last 20 years. In this period, most of the concepts and surgical techniques that had dominated the surgical approaches over the last century have been abandoned or have evolved, leaving more space to non-surgical or minimally invasive treatments, in particular in pediatric patients. In growing children, we prefer to avoid open surgical repair if other minimally or non-invasive methods are available. Nevertheless, in some congenital anomalies the open treatment is still indicated in the pediatric age, as it is the only effective option available. In this review, we will present the open surgical treatment of these less common congenital anomalies of the chest wall: Currarino-Silverman (CS) deformity, Poland syndrome (PS), sternal cleft and Jeune syndrome.
Methods: Based on the large experience of two national referral centers of pediatric and thoracic surgery, we present the surgical treatment of the aforementioned thoracic anomalies, including the indications, technical aspects and the outcomes.
Results: While pectus excavatum (PE) is usually repaired through a minimally invasive approach and pectus carinatum (PC) is treated with orthotic measures, CS anomaly requires an open sternocondroplasty. Cartilages are removed on both sides of the sternum and the sternal angle is resected. The correction of the lower half of the sternum is achieved either by inserting a retrosternal support or by filling the depression with cartilage tissue. The correction of the thoracic defect in PS is tailored according to the single patient. Removal of prominent cartilages, sternal osteotomies, bridging the costal agenesis with metallic bars or custom-made prosthesis may be necessary in severe cases. Sternal clefts are better repaired early with a primary closure or later on with prosthetic materials. Jeune syndrome in the asphyxiating fatal variant requires lateral thoracic expansion (LTE).
Conclusions: In conclusion, chest wall anomalies encompass a large spectrum of deformities of varying severity and functional or cosmetically important impact.
Methods: Based on the large experience of two national referral centers of pediatric and thoracic surgery, we present the surgical treatment of the aforementioned thoracic anomalies, including the indications, technical aspects and the outcomes.
Results: While pectus excavatum (PE) is usually repaired through a minimally invasive approach and pectus carinatum (PC) is treated with orthotic measures, CS anomaly requires an open sternocondroplasty. Cartilages are removed on both sides of the sternum and the sternal angle is resected. The correction of the lower half of the sternum is achieved either by inserting a retrosternal support or by filling the depression with cartilage tissue. The correction of the thoracic defect in PS is tailored according to the single patient. Removal of prominent cartilages, sternal osteotomies, bridging the costal agenesis with metallic bars or custom-made prosthesis may be necessary in severe cases. Sternal clefts are better repaired early with a primary closure or later on with prosthetic materials. Jeune syndrome in the asphyxiating fatal variant requires lateral thoracic expansion (LTE).
Conclusions: In conclusion, chest wall anomalies encompass a large spectrum of deformities of varying severity and functional or cosmetically important impact.
