Classification of Pectus Excavatum According to Objective Parameters From Chest Computed Tomography.

BACKGROUND: Previous classification systems of pectus excavatum have been based on subjective morphologic characteristics. We sought to suggest a new classification system derived from objective variables. METHODS: Patients who underwent surgical repair of pectus excavatum without a history of previous chest operations were included. Objective morphologic variables were measured from chest computed tomography scan images, and classification was performed by hierarchical clustering of measured indexes. Clinical relevance of the suggested classification was also verified. RESULTS: Included were 230 patients who underwent operation for pectus excavatum from January 2001 to August 2013. These patients were classified into two major groups: typical (group I; 197 [85.7%]) and atypical (group II; 33 [14.3%]). Group I was further classified into three subgroups according to flatness and symmetry of the chest wall. Group II was further classified into four subgroups according to the severity of sternal torsion and sternal angulation. Two unique types of deformity were identified in group II: the double distortion subgroup (group IIa; 8 [3.5%]) and the reverse torsion subgroup (group IIc; 16 [7.0%]). Scoliosis was more frequently associated with group IIa (p = 0.008). CONCLUSIONS: Morphologic classification obtained from computed tomography indexes hierarchical clustering identified seven distinct subtypes of pectus excavatum.

Dysmorphology of chest wall deformities: frequency distribution of subtypes of typical pectus excavatum and rare subtypes.

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.

Magnetic mini-mover procedure for pectus excavatum III: safety and efficacy in a Food and Drug Administration-sponsored clinical trial.

PURPOSE: The magnetic mini-mover procedure (3MP) uses magnetic force to gradually remodel pectus excavatum deformity. A magnet is implanted on the sternum and coupled with an external magnetic brace. Under Investigational Device Exemption and Institutional Review Board approval, we performed a pilot study of safety, probable efficacy, and cost-effectiveness of this new treatment of an orphan disease using an implantable pediatric device. METHODS: Ten otherwise healthy patients, ages 8 to 14 years, with severe pectus excavatum (pectus severity index [PSI] > 3.5) underwent 3MP treatment (mean, 18.8 ± 2.5 months). Safety was assessed by postimplant and postexplant electrocardiograms and monthly chest x-rays. Efficacy was assessed by change in pectus severity index as measured using pretreatment and posttreatment computed tomographic scan. Cost of 3MP was compared with that of standard procedures. RESULTS: The 3MP device had no detectable ill effect. Device weld failure or malpositioning required revision in 5 patients. Average wear time was 16 h/d. Pectus severity index improved in patients in the early or mid puberty but not in patients with noncompliant chest walls. Average cost for 3MP was $46,859, compared with $81,206 and $81,022 for Nuss and Ravitch, respectively. CONCLUSION: The 3MP is a safe, cost-effective, outpatient alternative treatment for pectus excavatum that achieves good results for patients in early and midpuberty stages.

Chest-deformities: a proposal for a classification.

BACKGROUND: In this article we assess the significance of classifying chest-deformities based on morphological findings in type-related treatment and its results. DATA SOURCES: Recent publications on chest-deformities in children and youth were retrieved from PubMed and Medline and from our clinical and intraoperative findings. RESULTS: Chest-deformities are diagnosed by thorax-measurements using a flexible meter projected on a graph-paper by MR/CT investigations and color coded videorasterstereography. In addition an ultrasound guided mediastinal analysis is performed on the heart, the great vessels and mediastinal organs. These investigations could determine meticulously the morphology of the sternum, the sterno-costal segments and the costal arch, enabling to find different chest wall deformities, i.e., 11 different types. The clinical and surgical significance of such a classification can be shown by comparing postoperative results of non-classified chest-deformities with those of classified. Preoperatively non-classified chest-deformities often have postoperative asymmetric shapes, partial local recurrences, costal arch eversions and a platythorax. Such a classification can be used to analyze and predict socalled "secondary associated alterations" of the vertebral column or mediastinal organs. CONCLUSIONS: Determining the specific type of a thorax deformity could be considered a type-related physiotherapy as conservative treatment or vacuum treatment and if surgery is indicated a type-related surgical correction can be performed. A type-related and adapted surgical correction can prevent subsequent mitral valve prolapse, recurrent infections, vertebral disturbances caused by kypho-scoliosis and increasing psychological irritation. Typing chest-deformities are an additional and essential help for the surgeon to perform specific surgical procedures: detorsion of the sternum, correction of the sterno-costal region, the costal arch bow and the kind of chest wall immobilization by metal struts. It can also compare the postoperative results more accurately in similar types of chest-deformities.

Pectus less invasive extrapleural repair (PLIER).

PURPOSE OF THE STUDY: This aim of this study was to evaluate the initial experience of pectus deformity repair using the pectus less invasive extrapleural repair (PLIER) procedure. METHODS: PLIER is an open procedure which involves a small midline chest incision, with exposure of the xiphoid which is raised to create a retrosternal space. The costal cartilages are excised to free the sternum and metal struts are implanted to stabilise the reconstructed chest wall. MAIN FINDINGS: Corrections were performed on 35 patients [25 pectus excavatum (PE) and 10 pectus carinatum (PC)] with a median age of 15.2 years (range 13-19 years). The mean size of the incision was 7.5 cm (range 7-10.5 cm). The mean operation time was 85 min (75-115 min). There were no intraoperative complications. The follow-up period ranged from 6 months to 2 years. CONCLUSIONS: The initial experience with the PLIER procedure demonstrates that it is suitable for the correction of PE and PC. The advantage of PLIER procedure is that it can be performed through small incisions in a short time with minimal loss of blood. Excellent aesthetic results after the procedure indicate the need to offer this method of surgical correction to low risk children.

Valuable lessons from two decades of pectus repair with the Willital-Hegemann procedure.

OBJECTIVE: Pectus deformities are the most common congenital hereditary chest wall deformity. The aim of this study was to evaluate the efficacy of thoracic wall reconstruction using a uniform technique of internal stabilization with stainless-steel struts. METHODS: Hospital charts of patients with chest wall deformities managed with the Willital-Hegemann procedure between January 1984 and January 2004 were reviewed. RESULTS: Surgical corrections were performed in 1262 patients with pectus deformities (968 male and 294 female patients). The corrections were completed with successful repair in 1244 (98.6%) patients, along with a low complication rate of 5.7%. The median age of the patients was 14.9 years (range, 2-53 years). The follow-up period ranged from 2 to 12 years (mean, 5.4 years). Major recurrences were observed in 18 (1.4%) patients, and mild recurrences were observed in 46 (3.6%) patients. There was 1 death in this series. The struts were removed after a period of 24 to 36 months and were associated with a complication rate of 2.6% at the time of removal. CONCLUSION: Custom-tailored molding of the chest wall can be achieved by using this method, which is not possible with minimal-access techniques. Open repair is effective for all variations of chest wall deformities and in patients of all ages, causes only mild pain, and produces good physiologic and cosmetic results. Improvement of subjective complaints, satisfactory long-term results, and improvement in psychological problems indicate the need to offer this procedure among other surgical correction options for low-risk children.

Classification of the dysmorphology of pectus excavatum.

BACKGROUND/PURPOSE: To describe the dysmorphology of pectus excavatum, the most common congenital chest wall anomaly. METHODS: A stratified sample of 64 patients, representative of a patient population with pectus excavatum of the Children's Hospital of King's Daughters in Norfolk, VA, was described and classified. The sample was stratified by sex to represent a 4:1 male-to-female ratio. The sample was further stratified to represent categories of age (3-10, 11-16, and 17 years and older). Preoperative photos and baseline chest computed tomography scans were examined and categorized according to the chief criteria, including asymmetry/symmetry of the depression, localized vs diffuse morphology, sternal torsion, cause of asymmetric appearance, and the length of the depression. RESULTS: Useful morphologic distinctions in pectus excavatum are localized depressions vs diffuse depressions, short and long length, symmetry, sternal torsion, slope/position of absolute depth, and unique patterns such as the horns of steer depression. CONCLUSIONS: These classifications simplify the diagnosis of pectus excavatum, aid in corrective surgery, and should improve correlation of phenotype and genotype in future genetic analysis.

Indice antropométrico para classificação quantitativa do pectus excavatum / Anthropometric index for quantitative assessment of pectus excavatum

INTRODUÇAO: O pectus excavatum caracteriza-se por uma depressão do esterno e das cartilagens para-esternais inferiores. Medidas clínicas para classificar essas depressões são poucas e de difícil aplicação. OBJETIVO: Criar medidas clínicas para quantificar a deformidade e poder comparar os resultados entre os períodos pré e pós-operatório. MÉTODO: Dez pacientes portadores de pectus excavatum, foram operados utilizando-se a técnica de Robicsek modificada pelo grupo de Cirurgia Torácica do Hospital das Clínicas da Faculdade de Medicina da Universidade de São Paulo, sendo também medidos clínica e radiologicamente nos periodos pré e pós operatõrio. Dez pacientes controles que não apresentavam anormalidades torácicas clínicas e ou radiológicas foram medidos da mesma forma. O defeito foi avaliado no nível do manúbrio e da maior deformidade através do índice antropométrico e do índice de Haller. RESULTADOS: A análise multivariada para as médias do índice antropométrico mostrou diferenças significativas entre o pré operatório e o grupo controle e entre as médias do pré e do pós operatório, e diferença não significativa entre o pós operatório e o grupo controle. A mesma análise, aplicada às médias do índice de Haller, demonstrou os mesmos resultados. O estudo pareado entre as médias do pré e do pós operatório mostrou tratarem-se de grupos diferentes. A correlação canônica evidenciou que o índice antropométrico e o índice de Haller têm correlação de 86 por cento. CONCLUSAO: Pacientes portadores de pectus excavatum podem ter a deformidade quantificada através de medidas do índice antropométrico no pré e no pós operatório, as quais permitem uma avaliação objetiva e comparativa dos resultados, e são de fácil realização.

Surgical correction of pectus excavatum: the Münster experience.

OBJECTIVES: Pectus excavatum is the most common congenital hereditary chest-wall deformity. This study analyses a single-center experience of pectus excavatum-thoracic wall reconstruction using a uniform technique of internal stabilization employing stainless steel struts. METHODS: From June 1984 to December 1997, we performed correction operations on 777 patients with pectus excavatum. The condition occurred more frequently in boys (621 patients) than girls (156 patients). Surgical repair was performed using a standard method of double bilateral chondrotomy parasternally and at points of transition to normal ribs. This was followed by detorsion of the sternum, retrosternal mobilization and correction of the inverted ribs. The anteriorly displaced sternum was stabilized by one trans-sternal and two bilateral parasternal metal struts. RESULTS: The corrections were completed with successful repair in 765 patients (98.5%) with a low complication rate of 6.7%. The follow-up period ranged from 4 weeks to 12 years, mean 6.4 years. Major recurrences were observed in 12 patients (1.5%) and mild recurrence were observed in 35 patients (4.5%). CONCLUSION: Significant reduction in postoperative cardiorespiratory disorders, low lethality, improvement of subjective complaints, satisfactory long-term results and improvement in psychological problems indicate the need to offer this method of surgical correction to low-risk children.

[Indications for surgery and methods of surgical correction of infundibuliform abnormality of the chest in children]. / Pokazaniia k operatsii i metody khirurgicheskoi korrektsii voronkoobraznoi deformatsii grudnoi kletki u detei.

Based on the survey of 104 patients, a method has been developed for early diagnosis of progression of infundibuliform chest deformity (ICD), which defines indications for thoracoplasty in children over 2 years. A differential approach has been applied to the stabilization of the sternocostal complex, taking into account various ICD types. Experience in surgical management of 247 patients with simple and complex ICD types has been generalized. A procedure has been improved to stabilize the sternocostal complex with a metallic plate in critical ICD types. The sparing thoracoplasty variants have been developed for simple ICD types and Degree I progressive ICD ones, stabilizing the sternocostal complex with a niticolic brace and a CPK-22 apparatus in the modified resistant case.

[Techniques and experiences in funnel chest operations]. / Techniken und Erfahrungen mit Trichterbrustoperationen.

Hegemann's technique of funnel chest correction using transsternal metal bar stabilization was adapted from the method published by Sulamaa and coworkers in 1958. Until the end of 1991, about 2400 chest wall corrections were performed in our department, more than 2000 of them using modifications of the original method. The principles of external chest wall measurement, our classification method, and the current operative modifications are described. In long-term follow-up examinations less than 3% recurrences should be possible.

[Clinical classification and surgical correction of pectus excavatum].

We described the experience in clinical classification and surgical correction of 320 cases of pectus excavatum (1984-1990). The patients age ranged from 2 to 14 years. According to the configuration, the deformity was divided into types: extensive type (7.5%); common type (74.3%); localized type (12.5%); mixed type or irregular (5.6%). We found such as four characteristic signs shoulder anteversion, kyphosis, chest excavation and abdominal bulge. Rotation of sternum, costal margin eversion, chest asymmetry and flat chest were also encountered. All these made the correction very difficult. A reverse "V" shape osteotomy of the sternum and ribs was with internal fixation. Small hand saw, special periosteum elevator and related internal fixation device were designed for the operation. In severe cases, satisfactory result was obtained by the technique combined with other surgical procedure.

Pectus carinatum: enfoque atual / Pectus carinatum: current approach

Desde 1977 os autores vêm analisando e tratando crianças e adolescentes portadores de deformidades em protrusäo da parede torácica anterior (pectus carinatum). Demonstram, através da análise radiográfica de tomografias do esterno, que o desenvolvimento deste osso tem importante papel na gênese dessas deformidades. Baseados nos estudos clínicos e radiológicos feitos ao longo dos últimos onze anos, apresentaram também uma classificaçäo própria e atualizada dessas deformidades. Demostraram ainda ser o tratamento conservador, através da utilizaçäo de órtese apropriada, viável e passível de bons resultados, conforme o tipo de deformidade e a idade do paciente

[The asymmetry of funnel chest: assessment, incidence, consequences]. / Die Asymmetrie der Trichterbrust: Beurteilung, Häufigkeit, Konsequenzen.

Cross-section profile measurements of 154 primary chest wall deformities showed 86 percent punctum maximum deviations and 55 percent asymmetries of the anterolateral chest wall, influencing the frontal and sagittal heart position. Operative consequences of these morphological findings are: Adequate cranial and lateral rib mobilisation and the need of metal strut stabilisation.

Morphologic findings of chest deformities in children corresponding to the Willital-Hümmer classification.

The clinical classification of funnel and pigeon chest deformities can be subclassified by means of special measurements of the chest. This simple technique makes it possible to differentiate sternocranial, sternocaudal, infrasternal, symmetrical, and asymmetrical marginal and peripheral deformities. This further classification also includes deformities of the vertebral column and deviatory development. The exact analysis of the abnormal chest in three different planes can be performed by means of external chest-wall measurements.

[Classification and subclassification of funnel and pigeon chest]. / Klassifizierung und Subklassifizierung der Trichter- und Kielbrust.

The Willital's clinical classification of funnel- and pigeon-chest deformities can be subclassified by special measurements of the chest. This easy technique enables to differentiate between: Sternocranial, sternocaudal, infrasternal, symmetric and asymmetric marginal and parasternal deformities. This further classification includes deformities of the vertebral column and deviatory development as well. The exact analysis of the abnormal chest in three different planes can be performed by external chest wall measurements that have been developed in our hospital.