This study verifies the hypothesis that bone/cartilage proportion in deformed ribs of male pectus excavatum patients varies according to their ages. Anatomical evaluation of the thoraces was performed for 79 male pectus excavatum patients, referring to their three-dimensional computer-tomographic images. The patients were divided into Child Group (5-9 years old: n = 35); Adolescent Group (12-15 years old: n = 15) and Adult Group (18+ years old: n = 29). For each patient, the most concave point of the sternum was identified and the pair of ribs closest to the point were defined as Key Ribs. On each Key Rib, the most ventral point was defined as Prominent Point (PP); the junction between the bone and cartilage was defined as Costo-Chondral Junction (CCJ). The distances of these points from the spine were defined as Distance of Prominent Point (DPP) and Distance of Costo-Chondral Junction (DCCJ), respectively. The horizontal length of the Key Rib was defined as Rib Length (RL). Inter- and intra-group comparisons were performed for DPP/RL and DCCJ/RL. Inter-Group Comparison: DCCJ/RL is significantly smaller and DPP/RL is significantly greater in Adult Group than in Child Group, meaning CCJs shift medially and PPs shift laterally as patients get older. Intra-Group Comparison: In Child Group, DCCJ/RL is significantly greater than DPP/RL, meaning CCJs exist lateral to PPs. Contrarily, in Adult Group, DCCJ/RL is significantly smaller than DPP/RL, meaning CCJs exists medial to PPs. Bone/cartilage proportion in the concave part of the chest shifts according to patients' ages. To perform the Nuss procedure effectively, this age-related anatomical change must be taken into consideration.
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http://dx.doi.org/10.1080/2000656X.2018.1554532 | DOI Listing |
Ann Ital Chir
December 2024
Department of Thoracic Surgery, Shanghai University of Medicine & Health Sciences Affiliated Zhoupu Hospital, 201318 Shanghai, China.
Aim: This study aims to compare the efficacy of modified single-incision surgery with that of traditional modified Ravitch surgery for the repair of pectus excavatum in pediatric patients.
Methods: In this retrospective study, we included patients who underwent surgical correction for sternal depression from January 2015 to December 2020 across four major medical centers. Patients were categorized into two specific groups on the basis of the surgical technique employed: the modified single-incision surgery group, which comprised patients treated using the novel single-incision approach, and the traditional modified Ravitch surgery group, which included patients who received the conventional Ravitch surgery with multiple incisions.
Skeletal Radiol
December 2024
Department of Clinical Genomics, Mayo Clinic, 13400 East Shea Blvd, Scottsdale, AZ, 85259, USA.
COL9A1 encodes the alpha-1 chain of type IX collagen heterotrimer, which is a vital component of collagen fibrils in hyaline cartilage. There are preliminary lines of evidence suggesting that COL9A1 mutations may be associated with autosomal dominant multiple epiphyseal dysplasia (MED), a disorder affecting the epiphysis of long bones. With only 2 reported cases (both from the same family) of MED in autosomal dominant COL9A1-related disorders (MIM 614135) in the clinical scientific literature hitherto, the phenotype is poorly understood at present.
View Article and Find Full Text PDFJ Clin Med
November 2024
Division of Thoracic Surgery, Erlangen University Hospital, 91054 Erlangen, Germany.
: Pectus excavatum, a deformity of the chest wall, can lead to significant emotional and social challenges, adversely affecting an individual's overall well-being. While the Nuss procedure is a well-established treatment, this study evaluates the impact of a modified, minimally invasive approach on patients' quality of life. : This retrospective study analyzed patients who underwent minimally invasive pectus excavatum repair (MIRPE) from 1 January 2010 to 31 January 2024.
View Article and Find Full Text PDFGenes (Basel)
November 2024
Department of Cardiovascular and Thoracic Surgery, Mayo Clinic, Phoenix, AZ 85054, USA.
: Pectus excavatum (PEx) is considered, at least partially, a familial disorder. A variety of inheritance patterns, associations with genetic syndromes, and pathogenic variants have been reported. However, the etiology of this condition is still not completely understood, and no known genes have been identified as definitive contributors.
View Article and Find Full Text PDFGenes (Basel)
October 2024
División de Genética, Centro de Investigación Biomédica de Occidente, Instituto Mexicano del Seguro Social, Guadalajara 44360, Jalisco, Mexico.
Noonan syndrome (NS) is a genetic multisystem disease characterized by distinctive facial features, short stature, chest deformity, and congenital heart defects. NS is caused by gene variants of the RAS/MAPK pathway, with accounting for about 50% of cases. This study aimed to identify pathogenic variants in Mexican patients with NS to enhance our understanding of the disease in this population.
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