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Dysmorphology of Chest Wall Deformities: Frequency Distribution of Subtypes of Typical Pectus Excavatum and Rare Subtypes Dismorfología de las deformidades de la pared torácica: Distribución de frecuencias de los subtipos de pectus excavatum típico y subtipos poco comunes
Indexado
Scopus SCOPUS_ID:85047698051
DOI 10.1016/J.ARBR.2013.03.001
Año 2013
Tipo

Citas Totales

Autores Afiliación Chile

Instituciones Chile

% Participación
Internacional

Autores
Afiliación Extranjera

Instituciones
Extranjeras


Abstract



Background/Purpose: More than forty percent of patients with pectus excavatum have a family history of chest deformity. However, no studies of the frequency of the different phenotypes of pectus excavatum have been published. Methods: A random sample of 300 non-syndromic pectus excavatum patients, from the chest wall deformities clinic at Children's Hospital of The King's Daughters in Norfolk, Va., was studied and classified according to a previously described classification system. Photographs and computed tomography (CT) scans were utilized. Results: Typical pectus excavatum. Photo data: localized deep depression (cup-shaped) deformity occurred in 67%; diffuse (saucer-shaped) 21%, trench-like (furrow-shaped) 10%, and Currarino-Silverman (mixed pectus excavatum/chondromanubrial carinatum) 1%. The deepest point was to the right of midline in 80%, left in 10% and central in 10%. By photo, the deepest point was in the lower sternum in 75%. When asymmetric, the deepest point of the deformity was to the right of midline in 90%. CT data: the average Haller index was 4.9. Severe sternal torsion (>. 30 degrees) was associated with greater Haller index (6.3) than mild torsion (4.5). The deepest point of the depression was at the mid- or lower sternum in more than 99%. It proved impossible to estimate width or length of the depression because of poorly defined borders. Conclusions: Typical PE is cup-shaped in 67% of cases, to the right of the midline in 80%, and involving the mid-to-lower sternum in 99%. However, other phenotypes, like the saucer and long trench, comprised one-third. Definition of the deformity is more reliable by CT scan. © 2012 SEPAR.

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Disciplinas de Investigación



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Respiratory System
Scopus
Pulmonary And Respiratory Medicine
SciELO
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Publicaciones WoS (Ediciones: ISSHP, ISTP, AHCI, SSCI, SCI), Scopus, SciELO Chile.

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Autores - Afiliación



Ord. Autor Género Institución - País
1 Kelly, Robert E. Hombre Children's Hospital of The King's Daughters Health System - Estados Unidos
2 Quinn, Amy - Children's Hospital of The King's Daughters Health System - Estados Unidos
3 VARELA-BALBONTIN, PATRICIO JOSE Hombre Universidad de Chile - Chile
Clínica Las Condes - Chile
4 Redlinger, Richard E. Hombre Children's Hospital of The King's Daughters Health System - Estados Unidos
5 Nuss, Donald Hombre Children's Hospital of The King's Daughters Health System - Estados Unidos

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