Amianthoid transformation of costal cartilage matrix in children with pectus excavatum and pectus carinatum.

BACKGROUND: It is unclear if amianthoid transformation (AT) of costal cartilage extracellular matrix (ECM) has an impact on the development of pectus excavatum (PE) and pectus carinatum (PC). METHODS: AT foci were examined in intrasurgical biopsy specimens of costal cartilages of children (8-17 years old) with PE (n = 12) and PC (n = 12) and in age-matching autopsy control samples (n = 10) using histological and immunohistochemical staining, atomic force and nonlinear optical microscopy, transmission and scanning electron microscopy, morphometry and statistics. RESULTS: AT areas were identified in the costal cartilage ECM in children with normal chest, PE and PC. Each type of the AT areas ("canonical", "intertwined", "fine-fibred" and "intralacunary") had a unique morphological pattern of thickness and alignment of amianthoid fibers (AFs). AFs were formed via lateral aggregation of collagen type II fibrils in the intact ECM. Foci of the AT were observed significantly more frequently in the PE and PC groups. The AT areas had unique quantitative features in each study group. CONCLUSION: AT is a structurally diverse form of ECM alteration present in healthy and pathological costal cartilage. PE and PC are associated with specific AT disorders.

Chondrotomy and sternotomy combined with the Nuss procedure for severe asymmetric pectus excavatum: how to do it.

The correction of severe asymmetric pectus excavatum is still challenging, especially for adults with a rigid thorax. For the repair of asymmetric cases, we introduce our surgical techniques added to the Nuss procedure. Chondrotomy of the depressed and deformed costal cartilage to elevate the depressed side was performed in a 42-year-old female patient. The depressed chest wall was directly elevated using pectus bars. Her sternal rotation angle improved from 27° to 15° after bar removal. In a 26-year-old male patient, oblique sternotomy and chondrotomy were performed. The sternal rotation angle improved from 26° to 9° postoperatively. These techniques were effective for correcting severe asymmetric pectus excavatum in adults.

Pediatric Myocardial T1 and T2 Value Associations with Age and Heart Rate at 1.5 T.

The objective of the study was to determine normal global left ventricular reference values for T1 and T2 in children. This is a retrospective study that included healthy subjects, age 5-19 years, who underwent CMR for the indication of pectus excavatum from 2018 to 2019. Linear regression models were used to determine associations of native T1 and T2 values to heart rate, age, and other CMR parameters. 102 patients with a mean age of 14.0 ± 2.4 years were included (range 5.4-18.8). 87 (85%) were males and 15 (15%) were females. The mean global T1 was 1018 ± 25 ms and the mean T2 was 53 ± 3 ms. T1 was negatively correlated with age (r = - 0.39, p < 0.001) and positively correlated with heart rate (r = 0.32, p < 0.001) by univariate analysis. Multivariable analysis showed that age and heart rate were independently associated with T1. T2 demonstrated a weak negative correlation with age (r = - 0.20, p = 0.047) and no correlation with heart rate. There was no difference in T1 (p = 0.23) or T2 (p = 0.52) between genders. This study reports normal pediatric T1 and T2 values at a 1.5 Tesla scanner. T1 was dependent on age and heart rate, while T2 was less dependent on age with no correlation with heart rate.

The effectiveness of double-bar correction for pectus excavatum: A comparison between the parallel bar and cross-bar techniques.

PURPOSE: To compare the effectiveness between parallel bar and cross-bar techniques for treating pectus excavatum. METHODS: A total of 80 patients who underwent parallel bar insertion (group 1) or cross-bar insertion (group 2) were evaluated retrospectively. From the pre- and post-operative chest CT images, vertebral-level-specific pectus indices were defined as the ratio of the maximum transverse diameter to the anteroposterior diameter of the thoracic cavity at a specific vertebral level and measured at 3 levels up (3Up-PI, 2Up-PI, 1Up-PI) and 1 vertebral level down (1Down-PI) from the narrowest point. The effectiveness of double-bar correction was compared between the 2 groups using postoperative vertebral level-specific pectus index changes. RESULTS: A total of 44 patients were enrolled in group 1, and 36 patients were enrolled in group 2. Preoperative pectus index values were not different between the 2 groups (4.5 ± 1.0 vs. 4.9 ± 1.5, P = 0.135). After double-bar correction, pectus index significantly decreased in both groups. There were no differences in postoperative pectus indices between the 2 groups (2.7 ± 0.4 vs. 2.6 ± 0.3, P = 0.197). Postoperative changes in 3Up-PI, 2Up-PI, and 1Up-PI were not significantly different between the 2 groups (P > 0.05). However, postoperative changes at the narrowest level and at 1Down-PI were significantly greater in group 2 than in group 1 (1.78 ± 0.85 vs. 2.32 ± 1.44, P = 0.009; 1.21 ± 0.70 vs. 1.70 ± 1.20, P = 0.009, respectively). CONCLUSIONS: Double-bar correction appears to be effective for treating pectus excavatum. The cross-bar insertion technique might be superior to the parallel bar insertion technique for correcting a wider range of deformities, especially at the lower part of the depression.

Sternal torsion in pectus excavatum is related to cardiac compression and chest malformation indexes.

BACKGROUND/PURPOSE: The role of sternal torsion (ST) in patients with pectus excavatum (PEX) is unknown. We evaluated the relationship between ST and both chest malformation and cardiac compression (CC) indexes. METHODS: We included consecutive patients with PEX who underwent chest computed tomography and cardiac magnetic resonance (CMR) to define surgical candidacy. Malformation indexes included the Haller index (HI), correction index (CI), and ST. CC and the tricuspid to mitral annulus width ratio were evaluated using CMR. RESULTS: One-hundred and sixteen patients were included, with a mean HI of 5.8 ±â€¯3.6 and a mean CI of 35.8 ±â€¯18.0%. ST was significantly related to malformation indexes, being patients with absence of ST those showing the lowest HI (p = 0.048) and CI (p = 0.002). Right-sided ST was significantly related to the CC classification (p = 0.0001), and the tricuspid/mitral annulus width ratio was significantly lower among these patients (absence 0.98 ±â€¯0.15, left-sided 0.91 ±â€¯0.10, right-sided 0.80 ±â€¯0.15, p < 0.0001). A significant inverse relationship between ST degrees and the tricuspid/mitral ratio was also identified (r = -0.47, p < 0.0001). CONCLUSIONS: We identified a significant relationship between ST and both chest malformation and CC indexes; the absence of ST being identified as a marker of an overall more benign phenotype. TYPE OF STUDY: Study of diagnostic test. LEVEL OF EVIDENCE: Level II.

[Reliability of the correction index in the surgical indication for pectus excavatum]. / Valor del índice de corrección en la indicación quirúrgica del pectus excavatum: correlación con el gold-standard.

OBJECTIVE: The Haller index (HI) is widely used to indicate surgical intervention in patients with pectus excavatum (PE). However, in patients with an atypical thoracic morphology, the severity of the defect can be incorrectly estimated. We propose comparing this index with the correction index (CI). MATERIAL AND METHODS: We analyzed clinical data and CT scans of 50 patients who consulted for PE in our center between 2010 and 2017. Haller index (HI), Correction index (CI) and ideal thoracic index (ITI) were calculated for each patient. The ITI allowed dividing the sample into two groups based on the thoracic morphology by excluding the PE component, therefore separating those with thorax too wide or too narrow from the standard patients. A standard group (36 patients) and a non-standard group (14 patients) were generated, among which the HI and the CI were correlated. RESULTS: The mean HI and CI of all patients were 3.99 and 27%, respectively. 31 of the 50 patients (62%) underwent intervention, 8 of them with an HI below 3.25. When comparing both groups, there was a moderate correlation between HI and CI in the standard group (Spearman r 0.799, p <0.01) and a greater correlation in the non-standard group (Spearman r 0.858, p <0.01).ween the scale and the presence of foreign body, except for SCORE 1, which was 57% what we attribute to an information bias. If the foreign body were not nuts, inorganic or bone, its aspiration was very unlikely, that is why we included it in the SCORE with -1. CONCLUSION: In our cohort, correlation of HI and CI was not different between both groups of patients. The CI did not prove its superiority when compared to HI in the surgical indication of patients with PE.

OBJETIVO: El índice de Haller (IH) se utiliza ampliamente para indicar la intervención quirúrgica en pacientes con pectus excavatum (PE). Sin embargo, en pacientes con una morfología torácica atípica, puede estimarse erróneamente la severidad del defecto. Planteamos comparar este índice con el índice de corrección (IC). MATERIAL Y METODOS: Analizamos datos clínicos y TC torácicos de 50 pacientes que consultan por PE en nuestro centro entre 2010 y 2017. Para cada paciente, se recalcula el índice de Haller (IH), el índice de corrección (IC) y el índice torácico ideal (ITI). El ITI permite formar dos grupos en base a la morfología torácica excluyendo el componente del PE, separando aquellos con tórax demasiado anchos o estrechos, de los pacientes estándar. Se genera un grupo estándar (36 pacientes) y un grupo no-estándar (14 pacientes), entre los que se correlacionan el IH y el IC. RESULTADOS: El IH y el IC medio de todos los pacientes fue de 3,99 y 27%, respectivamente. Se intervinieron 31 de los 50 pacientes (62%), 8 de ellos con un IH inferior a 3,25. Al comparar ambos grupos, existe una correlación moderada entre IH e IC en el grupo estándar (r Spearman 0,789; p<0,01) y una correlación mayor en el grupo no-estándar (r Spearman 0,858; p<0,01). CONCLUSION: En nuestra cohorte, no se ha probado que la correlación del IH y el IC sea diferente entre ambos grupos de pacientes. El IC no ha demostrado tener mayor validez que el IH en la indicación quirúrgica del PE.

Measuring the impact of surgical intervention on pediatric pectus excavatum using white light scanning.

BACKGROUND: Objective preoperative assessment of pectus excavatum (PE) deformity in patients is limited to preoperative measurement of severity using computed tomography (CT) or magnetic resonance imaging (MRI). Postoperative assessment is currently subjective as postoperative CT scans are not recommended in light of radiation exposure and high cost to families. White Light Scanning (WLS) is a novel 3D imaging modality that offers an alternative that is a quick, nonionizing, inexpensive, and safe strategy for measurement both pre- and postsurgery. Our prior investigation demonstrated the feasibility of using WLS to measure PE deformity and showed very strong correlation of a new WLS-derived PE severity index, the Hebal-Malas Index (HMI), with CT-derived HI. The purpose of this study was to demonstrate use of WLS to assess extent of correction of PE deformities after the Nuss procedure. METHODS: WLS scan data were gathered prospectively in pediatric patients with PE from 2015 to 2018. HMI was obtained from the preoperative and postoperative WLS scans. Analysis assessed the differences of preoperative and postoperative HMI. Preoperative CT-derived HI was collected from the medical record and estimated postoperative Haller Index was calculated from HMI and correlation of HMI and HI using historical data. RESULTS: A total of 71 patients received a preoperative CT scan and underwent surgery for PE. Of those, 63 (89%) received WLS preoperatively and 51 (72%) had complete preoperative and postoperative WLS data. The average postoperative decrease in the WLS-derived HMI was 0.35 (SD: 0.15) and 1.73 (SD: 1.03) in WLS-estimated HI. CONCLUSIONS: WLS is highly effective in objectively quantifying the extent of surgical correction in PE patients. LEVEL OF EVIDENCE: IV TYPE OF STUDY: Diagnostic Study.

Against the Overgrowth Hypothesis: Shorter Costal Cartilage Lengths in Pectus Excavatum.

BACKGROUND: Pectus excavatum is a common chest wall deformity with no known cause. A common hypothesis is that in patients with pectus excavatum, there is an overgrowth of costal cartilage relative to healthy individuals. MATERIALS AND METHODS: We obtained radiological curvilinear three-dimensional measurements of the fourth to eighth costal cartilage and associated ribs in 16 patients with pectus excavatum and 16 age- and gender-matched controls between the ages of 6 and 32 y. An analysis of variance was used to compare bone length, cartilage length, and their ratios between patients and controls. RESULTS: Relative to bone length, patients with pectus excavatum overall had shorter costal cartilage lengths (P < 0.001), especially on the left side (P < 0.05). We were unable to localize this observation to specific ribs during post hoc analysis. CONCLUSIONS: This is the first study to empirically test the overgrowth hypothesis of pectus excavatum for ribs 4 through 8. Although we and others have found no evidence to support this hypothesis, we surprisingly found the alternate hypothesis to be true: patients with pectus excavatum tend to have shorter costal cartilages. Future studies should expand on these results with larger sample sizes and consider volumetric measurements longitudinally during thoracic development.

Abnormal response of costal chondrocytes to acidosis in patients with chest wall deformity.

Costal cartilage is much understudied compared to the load bearing cartilages. Abnormally grown costal cartilages are associated with the inherited chest wall deformities pectus excavatum and pectus carinatum resulting in sunken or pigeon chest respectively. A lack of understanding of the ultrastructural and molecular biology properties of costal cartilage is a major confounder in predicting causes and outcomes of these disorders. Due to the avascular nature of cartilage, chondrocytes metabolize glycolytically, producing an acidic environment. During physical activity hydrogen ions move within cartilage driven by compressive forces, thus at any one time, chondrocytes experience transient changes in pH. A variety of ion channels on chondrocytes plasma membrane equip them to function in the rapidly changing conditions they experience. In this paper we describe reduced expression of the ASIC2 gene encoding the acid sensing ion channel isoform 2 (previously referred to as ACCN1 or ACCN) in patients with chest wall deformities. We hypothesized that chondrocytes from these patients cannot respond normally to changes in pH that are an integral part of the biology of this tissue. Activation of ASICs indirectly creates a cascade ultimately dependent on intracellular calcium transients. The objective of this paper was to compare internal calcium signaling in response to external pH changes in costal chondrocytes from patients with chest wall deformities and healthy individuals. Although the molecular mechanism through which chondrocytes are regulated by acidosis remains unknown, we observed reduced amplitudes of calcium rise in patient chondrocytes exposed to low pH that become further impaired upon repeat exposure.

Is pectus excavatum a risk factor for radiation-induced lung disease in patients undergoing radiation therapy following breast-conserving surgery?

BACKGROUND: The relationship between radiation dose to the ipsilateral lung and subsequent radiation-induced lung disease (RILD) in breast cancer patients with pectus excavatum (PE) undergoing radiation therapy (RT) to residual breast tissue after breast-conserving surgery has not yet been established. The incidence of RILD in such patients with PE, meaning that a large volume of the lung is within the radiation field, has not been determined. Therefore, the aim of this study was to determine the relationship between these factors. METHODS: The study cohort comprised 133 women who underwent three-dimensional conformal RT to residual breast tissue after breast-conserving surgery for breast cancer. Diagnoses of PE were based on Haller's, frontosagittal, and Monden's depression indices. Radiation doses to the ipsilateral lung were established from dose-volume histograms. RESULTS: Fifty of the 133 participants (37.6%) were diagnosed with RILD; all were asymptomatic. Multivariate analysis revealed a significant correlation between the incidence of RILD and the administration of > 30 Gy (V30). Surprisingly, although patients with PE received higher ipsilateral lung doses, they were less likely to develop RILD than those without PE. CONCLUSIONS: Our data indicate that the incidence of RILD is correlated with the administration of > 30 Gy (V30) and that PE is not a risk factor for RILD after RT to residual breast tissue after breast-conserving surgery for breast cancer. Surprisingly, individuals with PE may have a lower incidence of RILD than those without this condition.

[Costal cartilage changes in children with pectus excavatum and pectus carinatum]. / Izmeneniia rebernogo khriashcha pri voronkovidnoi i kilevidnoi deformatsii grudnoi kletki u detei.

Pectus excavatum (PE) and pectus carinatum (PC) in children are the most common congenital deformities that cause complications in the thoracic organs; however, the role of chondrocytes and cartilage canals in the pathogenesis of these conditions remains unexplored. OBJECTIVE: To investigate qualitative and quantitative changes of cartilage lacunae and canals in the costal cartilages in children with PE and PC compared to those with normal chests. SUBJECT AND METHODS: Costal cartilages were investigated in 10 children with normal chests (a control group), in 12 children with PE, and in 12 children with PC. Tissue fragments were fixed in 10% neutral formalin and embedded in compacted paraffin. Sections were stained with hematoxylin and eosin. Slides were examined by light microscopy. Cartilage lacunae, hyper- and hypolacunar zones, and cartilage canals were morphometrically examined, followed by statistical data analysis. RESULTS: There was a significant decrease in the number of cartilage lacunae and in the frequency of hyperlacunar zones and an increase in that of hypolacunar zones in the PE and PC groups. There were no significant differences in these parameters between the PE and PC groups; however, there was a tendency to the smallest number of cartilage lacunae and canals in the PC group and that to the preponderance of empty lacunae in the PE group. Only the PC group showed also negative correlations between the proportions of empty lacunae and the age of children. CONCLUSION: The pathogenesis of PE and PC in children is related to the impaired trophism of costal cartilages due to the smaller number of cartilage channels containing vessels and lacunae with chondrocytes. The development of PE and PC is associated with specific costal cartilage morphological changes that suggest that PE and PC are different manifestations of the same disease, namely connective tissue dysplasia.

Quantification of pectus excavatum: Anatomic indices.

Pectus excavatum is the most common chest wall deformity in children. The central portion of the chest is displaced posteriorly relative to the remainder of the anterior chest wall. Quantification of defect severity can be performed with multiple imaging modalities or external thoracic measures, but is most commonly quantified by the Haller Index (HI) or Pectus Correction Index (PCI). These two measures provide a measure of the chest based on cross sectional imaging, most commonly CT scans, allowing for standard comparison and definitions of pectus defects. The purpose of this article is to describe the creation, calculation, and limitations of the methods quantifying pectus defects.

Relationship between cardiac MR compression classification and CT chest wall indexes in patients with pectus excavatum.

BACKGROUND/PURPOSE: In pectus excavatum (PE) patients owing to the location of the heart in the chest cavity, the most affected site of compression by the depressed chest wall is the right heart, and surgical repair has shown to provide a significant relief in the RH cavities compression. Our aim was to explore the relationship between the site of right cardiac compression, chest wall indexes (CT-CWI) and the sternal torsion angle (STA) based on cardiac magnetic resonance (CMR) and computed tomography (CT) among PE patients. METHODS: We included PE patients with no previous surgical correction referred to CMR and chest CT imaging as presurgical evaluations. The following CT-CWI were calculated: Haller Index (HI), correction index (CI) and STA. A CMR compression classification (CMR-CC) was implemented based on the analysis (in the horizontal long axis plane at end of diastole) of the right cardiac compression site, caused by the thoracic cage (sternum/ribs): Type 0 (T0): absence of cardiac compression; Type 1 (T1): compression of the lateral wall of the right ventricle (RV) without involvement of the atrioventricular (AV) groove; Type 2 (T2): compression of the RV involving the AV groove. RESULTS: Sixty PE patients underwent CMR and chest CT. Fifty (81%) patients were male, and the median age was 17.5 (14.0; 23.0) years. T0, T1 and T2 were found in 14 (23%), 27 (45%) and 19 (32%) patients. There were significant differences between types with regard to the HI (T0 3.9 ±â€¯1.1, T1 4.8 ±â€¯2.0, T2 6.4 ±â€¯3.1, p < 0.009) and to the CI (T0 22.1 ±â€¯10.4%, T1 31.6 ±â€¯16.1%, T2 46.9 ±â€¯16.3% p < 0.0001) and STA (T0 9.1 ±â€¯7.9°, T1 12.7 ±â€¯10.3°, class T2 23.0 ±â€¯13.6°p = 0.001) respectively. CONCLUSION: In this study, we established a cardiac magnetic resonance compression classification of patients with pectus excavatum comprising a simple discrimination of cardiac compression sites, which were related to chest wall indexes. TYPE OF STUDY: Study of Diagnostic Test. LEVEL OF EVIDENCE: Level II.

Nonoperative management of pectus excavatum with vacuum bell therapy: A single center study.

PURPOSE: The purpose of this study was to determine variables predictive of an excellent correction using vacuum bell therapy for nonoperative treatment of pectus excavatum. METHODS: A single institution, retrospective evaluation (IRB 15-01-WC-0024) of variables associated with an excellent outcome in pectus excavatum patients treated with vacuum bell therapy was performed. An excellent correction was defined as a chest wall depth equal to the mean depth of a reference group of 30 male children without pectus excavatum. RESULTS: Over 4years (11/2012-11/2016) there were 180 patients enrolled with 115 available for analysis in the treatment group. The reference group had a mean chest wall depth of 0.51cm. An excellent correction (depth≤0.51cm) was achieved in 23 (20%) patients. Patient characteristics predictive of an excellent outcome included initial age≤11years (OR=3.3,p=.013), initial chest wall depth≤1.5cm (OR=4.6,p=.003), and chest wall flexibility (OR=14.8,p<.001). Patients that used the vacuum bell over 12 consecutive months were more likely to achieve an excellent correction (OR=3.1,p=.030). Follow-up was 4months to 4years (median 12months). CONCLUSION: Nonoperative management of pectus excavatum with vacuum bell therapy results in an excellent correction in a small percentage of patients. Variables predictive of an excellent outcome include age≤11years, chest wall depth≤1.5cm, chest wall flexibility, and vacuum bell use over 12 consecutive months. TYPE OF STUDY: Retrospective chart review. LEVEL OF EVIDENCE: Level III treatment study.

Evaluation of thoracic factors after scoliosis surgery in patients with both scoliosis and pectus excavatum.

PURPOSE: Pectus excavatum can negatively impact cardiac function during scoliosis surgery. Several authors reported severe hypotension associated with the prone position during scoliosis surgery in children that had both scoliosis and pectus excavatum. However, we could find no studies that evaluated the change in the thoracic factors, such as sternal tilt angle and Haller index after scoliosis surgery in patients with both scoliosis and pectus excavatum. The purpose of this study is to evaluate the change in thoracic factors after surgical treatment for scoliosis associated with pectus excavatum. METHODS: We performed a retrospective review on 20 patients (10 males and 10 females) who underwent surgical treatment for scoliosis associated with pectus excavatum from August 2004 to April 2014 in our hospital. We investigated the scoliosis diagnosis, preoperative and postoperative Cobb and thoracic kyphosis (TK) angles, the change in TK after surgery and thoracic factors, including the AP and transverse diameters of the chest, the sternal tilt angle, and Haller index. RESULTS: Patient mean age was 13.2 years old (4-27 years old) at surgery. Types of scoliosis were idiopathic in 8 patients, syndromic in 10, and neuromuscular in 2. The mean Cobb angles were 72.1° preoperatively and 19.0° postoperatively. Curve locations were thoracic in 13 patients, thoracolumbar in 4, and lumbar in 3. Surgical treatment of pectus excavatum was performed in 9 patients (45 %) before scoliosis treatment. Mean sternal tilt angles were 11.5° preoperatively and 11.1° postoperatively. Mean Haller indices were 4.8 preoperatively and 5.3 postoperatively. This was especially true for syndromic or neuromuscular scoliosis and thoracolumbar/lumbar curve type patients in which scoliosis surgery tended to worsen the Haller index. CONCLUSION: The Haller index increased postoperatively in 11 of 20 patients, which means sternal depression deteriorated after scoliosis surgery in about 50 % of patients. We suggest that surgeons fully assess the thoracic factors in patients with scoliosis and pectus excavatum prior to performing scoliosis surgery and carefully monitor their patient's general condition during surgery.

Does an external chest wall measurement correlate with a CT-based measurement in patients with chest wall deformities?

BACKGROUND: Measurements in chest wall deformities are typically conducted using a thorax caliper or a CT scan of the chest wall. This paper focuses on the possible correlation between these two methods to validate the reliability of the thorax caliper, minimize radiation exposure, and limit the usage of expensive imaging techniques. METHODS: We evaluated 95 consecutive patients (77 pectus excavatum (PE), 17 pectus carinatum (PC), 1 mixed deformity) who received surgical correction of the anterior chest wall. The results of the external chest wall measurements and the CT-based measurements were statistically compared. RESULTS: A significant correlation between the two measurements was observed in PE and PC at the highest point of the deformation. The strongest correlation was noted in PE. We also noted a correlation between the transverse diameter of the external measurement and the inner thoracic diameter of the CT scan but not for the sagittal diameters in the upper parts of the sternum. CONCLUSIONS: Thorax caliper measurements are suitable for determining the sagittal thoracic diameter at the maximum level of the deformity and the transverse diameter with an accuracy comparable to that of CT measurements. Since these values key, the thorax caliper is reliable for monitoring and documenting chest wall malformations. LEVEL OF EVIDENCE: Study of diagnostic test. Testing previously developed diagnostic criteria in a consecutive series of patients and a universally "gold" standard-Level I.

The modified percent depth: Another step toward quantifying severity of pectus excavatum without cross-sectional imaging.

INTRODUCTION: Current approaches to quantifying the severity of pectus excavatum require internal measurements based on cross-sectional imaging. This study evaluated the modified percent depth (MPD), a novel index of severity that can be obtained with external measurements, potentially avoiding the need for cross-sectional imaging. METHODS: Patients undergoing surgical repair of pectus excavatum (pectus group), and those undergoing cross-sectional imaging for unrelated reasons (control group), between 2010 and 2016 were included. The MPD of the deformity was calculated using external (i.e. skin surface to skin surface) measurements from the radiographic images. The same external measurements were taken using chest calipers on a subset of these patients in the outpatient clinic. The optimal threshold for MPD that defined severe pectus deformity was derived from receiver-operator characteristic (ROC) analysis. Sensitivity and specificity of the MPD was compared with that of the Haller Index (HI) and Correction Index (CI). RESULTS: There were 92 children (49 pectus, 43 controls) included. The median MPD was 20.2% and 4.2% for pectus and control patients, respectively (p<0.0001). An MPD cutoff of 10% optimally discriminated between severe pectus patients and controls by ROC analysis. An MPD of >10% had 98% sensitivity and 98% specificity for severe pectus deformity. Sensitivity and specificity were respectively 93% and 93% for HI >3.25, and 100% and 79% for CI >10. CONCLUSION: An MPD >10% performs slightly better than the HI and CI in distinguishing patients with severe pectus deformities. This novel measurement approach offers distinct advantages over existing indices, in that it does not require cross-sectional imaging and can be done using chest calipers in the office setting. Further studies with larger sample size are needed to verify reproducibility of the technique. LEVEL OF EVIDENCE: Level II, Study of Diagnostic Test.

Manifestations and Medicolegal Significance of Loeys-Dietz Syndrome.

Loeys-Dietz syndrome is a recently described autosomal dominant disorder with underlying vasculopathy characterized by aortic and other vascular aneurysmal dissection/rupture. A 61-year-old man is reported who died suddenly and unexpectedly and at autopsy was found to have a ruptured abdominal aortic aneurysm. Additional findings included dolichostenomelia, high-arched palate, and pectus excavatum. There was a strong family history of Loeys-Dietz syndrome, although the decedent had never been tested. Death was, therefore, due to a ruptured abdominal aortic aneurysm in a case of probable Loeys-Dietz syndrome. Although Loeys-Dietz syndrome shares common characteristics with Marfan and other connective tissue syndromes, it is a distinct entity with a much more aggressive clinical course. Lethal events may not occur until later adult life; however, the identification of the syndrome and differentiation from other connective tissue disorders at autopsy is important so that genetic investigation of close relatives can be undertaken with prophylactic surgical treatment if necessary.

Pectus excavatum e assimetrias mamárias: correção com mamoplastia de aumento / Pectus excavatum and breast asymmetry: correction with breast augmentation

Introdução: O pectus excavatum é definido com uma depressão aproximando o esterno e as cartilagens costais à coluna vertebral. Existem diversas teorias em relação à etiologia; a mais aceita consiste no crescimento exagerado das cartilagens costais, causando um deslocamento posterior do esterno e consequente depressão. Dentre as formas de tratamento, encontra-se a correção das assimetrias mamárias utilizando próteses mamárias de silicone em pacientes sem sintomas cardiopulmonares, apenas com queixa estética. Métodos: Foram revisados os registros em prontuários e registros fotográficos de oito casos de pacientes femininas com diagnóstico de pectus excavatum operadas em um hospital privado na região Sul do Brasil, que procuravam o serviço com queixas estéticas locais, e não apresentavam queixas cardiorrespiratórias. Resultados: Seis casos foram submetidos somente à inclusão de prótese mamária. Uma paciente havia colocado prótese havia 15 anos, sendo essa substituída por novo implante, no mesmo plano. Outra paciente já havia realizado cirurgia de correção de pectus descrito por Nuss, 10 anos antes, vindo a consulta com queixa de hipomastia e assimetria. O tipo anestésico preferido foi a anestesia geral, em cinco dos casos. O plano de inclusão de prótese na quase totalidade dos casos foi o subglandular. Somente uma paciente apresentou complicação (seroma). Conclusões: Evidenciamos, na nossa amostra, que a inclusão de prótese mamária em pacientes com pectus excavatum é capaz de trazer resultados estético harmônicos, atenuando e/ou mascarando o defeito torácico, com resultados estéticos satisfatórios para os pacientes.

Introduction: Pectus excavatum is defined as a depression approaching the sternum and costal cartilages to the spine. Several theories explain its etiology, the most accepted of which is the exaggerated growth of the costal cartilages, which causes a posterior displacement of the sternum and consequent depression. The treatment includes correction of breast asymmetries by using silicone breast implants in patients without cardiopulmonary symptoms, only with esthetic complaints. Methods: We reviewed the medical and photographic records of eight female patients diagnosed as having pectus excavatum, who underwent operation at a private hospital in the southern region of Brazil. These women sought consultation for local esthetic complaints and had no cardiorespiratory complaints. Results: Six patients submitted only for breast prosthesis placement. One patient had a prosthesis implanted 15 years before, which was replaced by a new implant in the same plane. Another patient had undergone pectus repair with Nuss surgery 10 years before, and the patient came to the hospital with a complaint of hypomasty and asymmetry. The preferred anesthesia was general anesthesia in five of the cases. The prosthesis inclusion plane in almost all the cases was subglandular. Only one patient had a complication (seroma). Conclusions: In our sample, the placement of breast prostheses in the patients with pectus excavatum brought harmonic esthetic results, attenuating and/or masking the chest defect, with satisfactory esthetic results for the patients.