Spinal posture, mobility, and position sense in adolescents with chest wall deformities: a comparison of pectus excavatum, pectus carinatum and healthy peers.

PURPOSE: The study aimed to compare spinal posture, mobility, and position sense in adolescents with pectus excavatum (PE), pectus carinatum (PC), and healthy control (HC). METHODS: 22 with PE, 22 with PC, and 21 HC were included in the study. The spinal posture (thoracic kyphosis, lumbar lordosis, pelvic tilt, thoracic, lumbar, pelvic lateral tilt angles) and mobility (thoracic, lumbar, hip/sacral, and overall, in the sagittal and frontal plane) with the spinal mouse, and spinal position sense (repositing errors) with the inclinometer were assessed. RESULTS: The thoracic kyphosis angle of PE and PC was higher than in HC (p < 0.001; p = 0.001). Hip/sacral mobility in the sagittal plane was lower in the PE and PC than control, respectively (p < 0.001; p < 0.001). Overall sagittal spinal mobility (p:0.007) and hip/sacral mobility in the frontal plane (p:0.002) were lower in the PC than in HC. Overall frontal spinal mobility was lower in the PE and PC than in HC (p:0.002; p:0.014). The PE and PC repositing errors were higher (p < 0.001; p:0.014). CONCLUSION: The study found that adolescents with PE and PC had decreased spinal mobility, spinal alignment disorders, and a decline in spinal position sense. It is important not to overlook the spine during physical examinations of adolescents with chest wall deformities. In clinical practice, we suggest that adolescents with chest deformities should undergo a spine evaluation and be referred for physical therapy to manage spinal disorders.

Parental Health Mindset Is Related to Preoperative Perceptions of Chest Wall Anomalies.

INTRODUCTION: Mindsets of health have been linked to different outcomes for patients with both surgical and medical conditions. A "growth" mindset, in which health is defined as malleable and subject to improvement, is associated with improved attitudes and outcomes when compared to a "fixed" mindset, in which health is defined as unchangeable. In pediatric surgery, parental growth mindsets of health have been correlated with better postoperative outcomes, including lower scores of anxiety and pain perceptions, for children. This was particularly notable in a study of postoperative outcomes for patients with pectus excavatum. In our current study, we extend our investigations to explore how health mindsets are associated with perceptions of chest wall anomalies before correction is undertaken. METHODS: Seventy-six patients (71 males, mean age 14 y, and 43 excavatum) and 18 parents were surveyed during a routine chest wall clinic visit. Demographic and clinical information as well as the Health Mindset Scale and Pectus Excavatum Evaluation Questionnaire were administered and collected pretreatment. RESULTS: Parental mindsets of health were significantly correlated with parent assessments of their children's chest pain, physical activity, and concerns about the life-time effect of the condition. A parental growth mindset was linked to lower scores of chest pain, higher ratings of activity, and lower overall level of concern. Furthermore, parental health mindsets also significantly correlated with children's own perceptions of their chest pain, physical activity, shortness of breath, and fatigue. Growth mindset also was linked to more positive ratings. CONCLUSIONS: Parental growth mindset was associated with more positive assessments of children's symptoms and limitations due to pectus deformities than fixed mindsets. Health mindset has been linked to patient perceptions of, and outcomes for, diabetes, renal disease, allergies, scoliosis, and obesity. Further study into parental and patient mindset correlation may help elucidate factors for bracing compliance, and perhaps to better prepare children and parents for corrective surgical procedures.

Increased incidence of mitral valve prolapse in children with pectus chest wall deformity.

BACKGROUND: Pectus anomalies constitute 95% of chest anomalies. Pectus carinatum (PC) and excavatum (PE) are often asymptomatic in childhood. However, symptoms and signs such as chest pain, dyspnea, and mitral valve prolapse (MVP) can be seen in pectus anomalies. Demographic characteristics and accompanying cardiac signs in children with pectus deformity were investigated. METHODS: In this study, the clinical findings for children with pectus deformity, and the incidence of MVP and other concomitant heart diseases detected in echocardiographic examinations were evaluated. RESULTS: Eighty-two children with PE, 27 with PC, and 107 healthy children were included in this study. In the echocardiographic examination of PE, PC patients, and healthy children, MVP was detected with frequencies of 25%, 33%, and 2% respectively. CONCLUSIONS: The study showed that pectus anomalies were associated with an increased incidence of MVP. All patients with pectus deformity should therefore undergo a screening echocardiogram in adolescence to assess for the presence of MVP.

Remote Monitoring System of Dynamic Compression Bracing to Correct Pectus Carinatum.

Pectus carinatum (PC) is a chest deformity caused by disproportionate growth of the costal cartilages compared with the bony thoracic skeleton, pulling the sternum forwards and leading to its protrusion. Currently, the most common non-invasive treatment is external compressive bracing, by means of an orthosis. While this treatment is widely adopted, the correct magnitude of applied compressive forces remains unknown, leading to suboptimal results. Moreover, the current orthoses are not suitable to monitor the treatment. The purpose of this study is to design a force measuring system that could be directly embedded into an existing PC orthosis without relevant modifications in its construction. For that, inspired by the currently commercially available products where a solid silicone pad is used, three concepts for silicone-based sensors, two capacitive and one magnetic type, are presented and compared. Additionally, a concept of a full pipeline to capture and store the sensor data was researched. Compression tests were conducted on a calibration machine, with forces ranging from 0 N to 300 N. Local evaluation of sensors' response in different regions was also performed. The three sensors were tested and then compared with the results of a solid silicon pad. One of the capacitive sensors presented an identical response to the solid silicon while the other two either presented poor repeatability or were too stiff, raising concerns for patient comfort. Overall, the proposed system demonstrated its potential to measure and monitor orthosis's applied forces, corroborating its potential for clinical practice.

Basic Strategies of Augmentation Mammoplasty in Patients with Tendencies of Pectus Excavatum and Carinatum.

BACKGROUND: In this era of growing popularity of cosmetic surgeries, more women of various chest wall shapes are likely to receive augmentation mammoplasty. Pectus excavatum (PE) and pectus carinatum (PC) are the two most common chest wall deformities, and many asymptomatic patients visit the clinic. In this study, we presented various strategies for successfully performing breast augmentation in PE and PC patients without long-term complications such as symmastia. METHODS: From January of 2019 to December of 2021, a total of 132 patients with tendencies of PE and PC received augmentation mammoplasty in our institute. We retrospectively reviewed data on demographics, surgical procedure, outcomes, and complications. RESULTS: Among the 132 cases, 71.21% were done via inframammary approach, and 28.79% via transaxillary approach. The mean implant volume was 337.25 ± 51.46 ml, and the mean follow-up period was 16.48 ± 6.74 months. The Likert scale of outcome satisfaction scored 9.13 ± 0.48. No symmastia occurred. CONCLUSION: We presented our basic strategies of breast surgery in patients with various chest wall anomalies. Augmentation mammoplasty can benefit PE and PC patients physically as well as psychologically, to carry out healthy positive lives. LEVEL OF EVIDENCE IV: This journal requires that authors assign a level of evidence to each article. For a full description of these Evidence-Based Medicine ratings, please refer to the Table of Contents or the online Instructions to Authors www.springer.com/00266 .

A Stop-gain Variant c.220C>T (p.(Gln74*)) in FLNB Segregates with Spondylocarpotarsal Synostosis Syndrome in a Consanguineous Family.

Spondylocarpotarsal synostosis (SCT) syndrome is a very rare and severe form of skeletal dysplasia. The hallmark features of SCT are disproportionate short stature, scoliosis, fusion of carpal and tarsal bones, and clubfoot. Other common manifestations are cleft palate, conductive and sensorineural hearing loss, joint stiffness, and dental enamel hypoplasia. Homozygous variants in FLNB are known to cause SCT. This study was aimed to investigate the phenotypic and genetic basis of unique presentation of SCT syndrome segregating in a consanguineous Pakistani family. Three of the four affected siblings evaluated had severe short stature, short trunk, short neck, kyphoscoliosis, pectus carinatum, and winged scapula. The subjects had difficulty in walking and gait problems and complained of knee pain and backache. Roentgenographic examination of the eldest patient revealed gross anomalies in the axial skeleton including thoracolumbar and cervical fusion of ribs, severe kyphoscoliosis, thoracic and lumbar lordosis, coxa valga, fusion of certain carpals and tarsals, and clinodactyly. The patients had normal faces and lacked other typical features of SCT like cleft palate, conductive and sensorineural hearing loss, joint stiffness, and dental enamel hypoplasia. Whole exome sequencing (WES) of two affected siblings led to the discovery of a rare stop-gain variant c.220C>T (p.(Gln74*)) in exon 1 of the FLNB gene. The variant was homozygous and segregated with the malformation in this family. This study reports extensive phenotypic variability in SCT and expands the mutation spectrum of FLNB.

The psychometric properties of the Turkish version of the pectus carinatum body image quality of life questionnaire.

PURPOSE: The present study aimed to evaluate the psychometric properties of the Turkish version of the Pectus Carinatum Body Image Quality of Life Questionnaire (PeCBI-QOL), which was originally created in American English in patients with pectus carinatum (PC). METHODS: The study included 52 volunteer patients with PC, aged 10-18 years. Cronbach α was used to assess internal consistency. Intraclass correlation coefficient (ICC) was used to estimate the test-retest reliability. Construct validity was analyzed with the Pectus Excavatum Evaluation Questionnaire (PEEQ). RESULTS: The reliability of the Turkish version of the PeCBI-QOL was found to have acceptable to excellent internal consistency (Cronbach's α coefficient = 0.701-950). Test-retest reliability calculated for investigating the reliability of the questionnaire over time was found to be excellent (ICC = 0.817-0.983). Construct validity of the PeCBI-QOL (child and parent form) was found to be correlated with all subscales in the PEEQ questionnaire (r = - 0.425 to 897, p < 0.001) except the treatment motivation subscale (r = 0.033-0.111, p = 0.439-0.816). CONCLUSION: We determined that the Turkish version of the PeCBI-QOL questionnaire was valid and reliable for the assessment of body image and quality of life in Turkish patients with PC. PeCBI-QOL questionnaire, a self-assessment one, will assist Turkish clinicians who wish to assess body image and quality of life of patients with PC.

Musculoskeletal deformities in children with congenital thoracic malformations: a population-based cohort study.

PURPOSE: It is unclear if musculoskeletal deformities observed in patients with congenital diaphragmatic hernia (CDH), congenital lung lesion (CLL) and esophageal atresia/tracheoesophageal fistula (EA/TEF) are associated with the anomaly or are a result of the surgery required to treat the anomaly. This study compared the prevalence of musculoskeletal deformities for: (1) children with congenital thoracic anomalies to controls; (2) CLL to EA/TEF both repaired via thoracotomy; and (3) CLL and EA/TEF to CDH repaired via laparotomy. METHODS: We performed a retrospective study of children with CLL, CDH or EA/TEF between 1990 and 2016. Date-of-birth-matched control groups were generated from a population-based dataset. International Classification of Disease codes were used to identify scoliosis and pectus anomalies. We determined Hazard ratios (HR) for cases versus controls. RESULTS: We included 167 cases (CDH n = 82; CLL n = 29; EA/TEF n = 56) and 1670 controls. EA/TEF had a greater risk of scoliosis (HR 5.52, 95%CI 1.49,13.73) and pectus deformities (HR 4.07, 95%CI 1.96,8.45). CDH showed more scoliosis (HR 5.03, 95%CI 1.99,12.74) but not pectus anomalies. Musculoskeletal deformities were not more common in CLL. CONCLUSION: Children born with CDH or EA/TEF, but not CLL, had more musculoskeletal deformities than controls. The inconsistent association between musculoskeletal deformities and the surgical approach suggested a congenital predisposition.

Loeys-Dietz Syndrome.

Loeys-Dietz syndrome is an autosomal dominant aortic aneurysm syndrome characterized by multisystemic involvement. The most typical clinical triad includes hypertelorism, bifid uvula or cleft palate and aortic aneurysm with tortuosity. Natural history is significant for aortic dissection at smaller aortic diameter and arterial aneurysms throughout the arterial tree. The genetic cause is heterogeneous and includes mutations in genes encoding for components of the transforming growth factor beta (TGFß) signalling pathway: TGFBR1, TGFBR2, SMAD2, SMAD3, TGFB2 and TGFB3. Despite the loss of function nature of these mutations, the patient-derived aortic tissues show evidence of increased (rather than decreased) TGFß signalling. These insights offer new options for therapeutic interventions.

Use of cryoanalgesia as a postoperative pain management for open pectus carinatum repair.

PURPOSE: Cryoanalgesia has shown to have safety and efficacy as an adjunct post-operative pain management for Nuss procedure. One retrospective study reported its efficacy for analgesia with the Ravitch procedure, with improved pain scores and decreased length of stay versus thoracic epidural. We describe our initial experience with the use of cryoanalgesia for an open repair of pectus carinatum. METHODS: We retrospectively reviewed the medical records of all patients who received cryoanalgesia during an open repair of pectus carinatum from 2016 to 2019 at our institution. We recorded pain scores at immediate post-operative and at 1-week follow up after hospital discharge. Length of stay and mean follow up time were also recorded. RESULTS: Five pediatric patients underwent open repair of pectus carinatum with cryoanalgesia. The median postoperative length of stay (LOS) was 1 (range 1-2) day. Only one patient reported a non-zero pain score during their hospitalization, and this was a 3 out of 10 in the post-analgesia care unit. At 1-week postoperative visit, all patients had a pain score of 0. Median follow up was 1 (0.5-2) year. No patients developed neuralgia. CONCLUSION: Cryoanalgesia is a safe and effective pain management strategy for pediatric patients undergoing open pectus carinatum repair.

Comparison of physical functions and psychosocial conditions between adolescents with pectus excavatum, pectus carinatum and healthy controls.

PURPOSE: The present study aimed to compare the physical and psychosocial conditions of adolescents with pectus excavatum (PE) and pectus carinatum (PC), who had mild-severe deformities, with those of healthy controls (HC). METHODS: This study included 180 adolescents (aged 10-18 years) with pectus deformity [PE (n = 90) and PC (n = 90)] and 90 age-matched HC. The following parameters were evaluated for each participant: clinical parameters, perceived appearance of the chest area (PAC), physical functions (grip strength, flexibility, muscle strength, endurance, physical activity), posture, psychosocial conditions, and quality of life (QOL). RESULTS: Patients with PE and PC had a lower body weight, a worse, a poorer posture, lower scores for physical functions compared to the HC group (p < 0.001, all). The PE group had lower scores (p < 0.05) for some psychosocial conditions and quality of life subscales compared to the HC and PC (except for the QOL) group (p < 0.05). The PC group had a poorer posture compared to the PE group (p < 0.05). PAC was associated with physical functions and psychosocial status (r = 0.19-0.40, p < 0.05) but pectus severity was not associated with these parameters (r = 0.02-0.12, p > 0.05). CONCLUSION: Our results indicate that all adolescents with mild, moderate, or severe pectus deformity should undergo a biopsychosocial evaluation, receive psychosocial support, and be referred for physiotherapy.

Echocardiography Reveals Heart Abnormalities in Pediatric Pectus Carinatum.

BACKGROUND: Symptoms including chest pain and palpitations are commonly described by pediatric patients with pectus deformity. Cardiac anomalies are thought to be less common in patients with pectus carinatum (PC) than those in patients with pectus excavatum; however, no literature supports this presumption. Echocardiogram (echo) assesses heart structure and function. We hypothesized that a screening echo would 1) determine the relationship between symptoms and echo findings and 2) define the incidence of cardiac defects in patients with PC. MATERIALS AND METHODS: This is an institutional review board-approved retrospective review of all patients with PC who received an echo from 2015 to 2019 at a tertiary care children's hospital. Echo findings and patient-reported symptoms were collected from electronic health records. Descriptive statistics were used to assess correlation between findings. RESULTS: We identified 155 patients with PC who received an echo with complete data available for analysis. Of these, 44 (28.4%) reported chest pain and 13 (8.4%) reported palpitations. Echo results showed that five patients (3.2%) had mitral valve prolapse and 11 (7.1%) had aortic root dilation. Patient-reported symptoms were not significantly associated with abnormal echo findings. CONCLUSIONS: Chest pain and palpitations frequently occur in the PC population but may not be related to abnormal echo findings. We recommend screening echo in patients with PC regardless of symptoms.

Does physiotherapy applied in conjunction with compression brace treatment in patients with pectus carinatum have efficacy? A preliminary randomized-controlled study.

PURPOSE: Non-invasive treatment of pectus carinatum (PC) deformity includes the use of a compression brace and exercises. In this study, we aimed to examine the effect of a physiotherapy protocol applied as adjunct to compression brace treatment in patients with PC. METHODS: The study included 30 male patients between 11 and 18 years of age. Patients were randomly assigned into two groups: a brace treatment only group (Group 1) and a brace and physiotherapy group (Group 2). Patient demographics and disease-related properties, protrusion measurements, postural evaluations, deformity perceptions, life quality, and treatment satisfaction were evaluated. RESULTS: Although both groups showed improvements based on external chest measurements related to PC protrusion following treatment (p < 0.001), Group 2 had more benefit from the treatment (effect size > 0.36) and displayed greater improvement in maximum protrusion degree and lateral length values (p < 0.05). Additionally, we found that patient perception of deformity, posture, psychological life quality, and treatment satisfaction scores were significantly better in Group 2 (p < 0.05). CONCLUSION: Owing to the satisfaction and additional benefits observed in the physiotherapy group, we think that a proper cardiopulmonary and musculoskeletal exercise program should be applied concurrently with brace treatment for patients with PC deformity. Nevertheless, long-term outcomes need to be clarified in future studies.

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.

Acute anterior myocardial infarction with pectus carinatum.

We presented a case of acute anterior myocardial infarction caused by left anterior descending artery occlusion in a patient with pectus carinatum. The electrocardiogram (ECG) on admission showed counterclockwise rotation and T wave inversion only in leads V1-V2. Computed tomography revealed that this patient with pectus carinatum had greater septal angle. Electrocardiographic counterclockwise rotation due to greater septal angle in pectus carinatum led to atypical ECG findings of acute myocardial infraction.

[Minimally Invasive Technologies in Surgery of the Chest Wall in Children].

Today, in our country, as well as throughout the world, there is no single approach to the tactics of managing patients with deformations of the anterior chest wall. These patients constitute a large and heterogeneous group. The team acquired great experience in treating children with pectus excavatum and pectus carinatum, as well as a unique experience in the surgical treatment of pediatric thoracic outlet syndrome and sternum clefts. Now in the world there are a lot of surgical methods used for the correction of congenital deformities of the chest wall, however, the majority of them are extremely traumatic for the patient, and the postoperative scar often presents a minor cosmetic problem than the deformation itself. In addition, almost all methods require the use of expensive technologies, the patient's long hospital stay and the use of potent drugs. As for the children with thoracic outlet syndrome and sternum clefts, these patients are extremely difficult in terms of timely diagnosis and surgical care, due to the fact that pediatric surgeons and pediatricians are very little aware of this pathology. The team of authors developed a number of techniques, based on the collaborative work of surgeons, anesthesiology and resuscitation specialists and pediatric services. They resulted in successful surgical correction of these malformations and gave patients the opportunity to lead a normal life.

[Result of 12 years of non-surgical treatment of pectus carinatum]. / Resultado de 12 años de tratamiento no quirúrgico del pectus carinatum.

OBJECTIVE: To report our experience in the treatment of pectus carinatum by using the dynamic compression system. MATERIAL AND METHODS: Retrospective study during the period from January 2005 to September 2017. Patients with typical condrogladiolar pectus carinatum and correction pressure (PC) ≤ 14 PSI (pound square inch) were included. Exclusion criteria: patients with previous thoracic surgery, mixed malformations and chondromanubrial pectus carinatum. For the treatment, the Dynamic Thoracic Compressor System (FMF) with pressure meter in PSI was used. The PC, the treatment pressure (PT), the correction time (TC) and the maintenance time (TM), recurrences and complications were analyzed. A qualitative scale was measured in three grades: where A is excellent or very good, B is regular and C is bad. RESULTS: We treated 104 patients under 18 years of age. The PT was 2.26. The average of the TC was 8.8 months. The TM was on average 8 months. 36.5% of the patients finished the treatment, 36.5% still continue in treatment and 26.9% of the patients lost the follow-up due to desertion. The qualitative assessment was positive in 95.5% of our patients, and unfavourable in 4.5%. CONCLUSION: The non-surgical treatment of pectus carinatum is efficient, non-invasive and of low morbidity. Regarding the high dropout rate, we must analyze the variables to be modified to reduce it. This treatment should be considered as the first option to correct pectus carinatum in patients with flexible thorax.

OBJETIVO: Describir nuestra experiencia en el tratamiento del pectus carinatum mediante el uso del sistema de compresión dinámico. MATERIALES Y METODOS: Estudio retrospectivo durante el período de enero de 2005 a septiembre de 2017. Se incluyeron pacientes con pectus carinatum condrogladiolar típico y presión de corrección (PC) ≤ 14 PSI (pound per square inch). Criterios de exclusión: pacientes con cirugía torácica previa, malformaciones mixtas y condromanubriales. Para el tratamiento se utilizó el sistema compresor torácico dinámico (FMF) con medidor de presión en PSI. Se analizaron la PC, la presión de tratamiento (PT), el tiempo de corrección (TC) y el tiempo de mantenimiento (TM), recidivas y complicaciones. Se realizó una escala cualitativa medida en tres grados: donde A es excelente o muy bueno, B regular y C malo. RESULTADOS: Tratamos 104 pacientes menores de 18 años. La PT fue de 2,26. El promedio del TC fue de 8,8 meses. El TM fue en promedio de 8 meses. El 36,5% de los pacientes finalizaron el tratamiento, 36,5% aún continúan en tratamiento y 26,9% de los pacientes se perdió el seguimiento por deserción del mismo. La valoración cualitativa fue positiva en el 95,5% de nuestros pacientes, y desfavorable en el 4,5%. CONCLUSION: El tratamiento no quirúrgico del pectus carinatum es eficiente, no invasivo y de baja morbilidad. Respecto a la alta tasa de deserción, debemos analizar las variables a modificar para disminuirla. Este tratamiento debe ser considerado una opción de primera elección, para corregir el pectus carinatum en pacientes con tórax flexible.

[Pectus excavatum y carinatum en el síndrome de Marfan y síndromes similares: prevalencia e impacto clínico pulmonar y cardiovascular].

INTRODUCTION: Pectus excavatum (PE) and carinatum (PC) are common in Marfan syndrome (SM) and similar syndromes (SS). Patients can evolve without symptoms. In some there is depression, social adjustment disorders, pulmonary and cardiovascular symptoms in which there is controversy about their relationship with the structural damage of the thorax. OBJECTIVE: To assess the prevalence of the type of thoracic deformity in patients with MS and SS in a historical and current cohort and to analyze the clinical, pulmonary and cardiovascular impact. METHOD: Prospective study. Subjects who met the Ghent criteria and who had a complete clinical record, an echocardiogram and/or magnetic resonance imaging, computed tomography and respiratory function tests were included. RESULTS: Of a total of 338 patients with MS and SS, 112 cases with thoracic deformity were detected, the prevalence of PE and PC in SM 13.6 and 12.4, respectively, was lower in SS. There is compression and displacement of lung and right cardiac cavities by PE and the correlation between the Haller Index and the increased PASP is 44 (p = 0.009). CONCLUSIONS: The prevalence of PE and PC in SM and SS is high, which impacts on lung function and cardiovascular damage, requires corrective management of the thoracic deformity and not only implies for aesthetic purposes.

INTRODUCCIÓN: El pectus excavatum (PE) y el pectus carinatum (PC) son frecuentes en el síndrome de Marfan (SM) y en síndromes similares (SS). Los pacientes pueden evolucionar sin síntomas. En algunos hay depresión, trastornos de adaptación social, síntomas pulmonares y cardiovasculares, en los cuales hay controversia de su relación con el daño estructural del tórax. OBJETIVO: Evaluar la prevalencia del tipo de deformidad torácica en pacientes con SM y SS en una cohorte histórica y analizar el impacto clínico, pulmonar y cardiovascular. MÉTODO: Estudio prospectivo. Se incluyeron sujetos con criterios de Ghent y características específicas de cada síndrome, con expediente completo, ecocardiograma o resonancia magnética y tomografía computada, y pruebas de función respiratoria. RESULTADOS: De un total de 338 pacientes con SM y SS, se detectaron 112 casos con deformidad torácica. Prevalencia de PE y PC en SM: 13.6 y 12.4; fue menor en SS. Hay compresión y desplazamiento de pulmón y cavidades cardiacas derechas por PE. Hay correlación entre el Índice de Haller y la presión sistólica de la arteria pulmonar incrementada es de 44 (p = 0.009). CONCLUSIONES: La prevalencia de PE y PC en el SM y SS es alta, lo cual impacta en la función pulmonar y cardiovascular, en esas condiciones se requiere del manejo correctivo de la deformidad torácica y el objetivo no es estético.

[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.

[Automatic diagnosis of pectus carinatum for children based on the improved Haller index].

Pectus carinatum (PC) is one of the most common chest wall anomalies, which is characterized by the protrusion of the anterior chest wall including the sternum and adjacent costal cartilages. Mildly patients suffer from mental problems such as self-abasement, while severely suffering patients are disturbed by significant cardiopulmonary symptoms. The traditional Haller index, which is widely used clinically to evaluate the severity of PC, is deficient in diagnosis efficiency and classification. This paper presents an improved Haller index algorithm for PC: first, the contour of the patient chest in the axial computed tomography (CT) slice where the most convex thorax presents is extracted; and then a cubic B-spline curve is employed to fit the extracted contour followed by an eclipse fitting procedure; finally, the improved Haller index and the classification index are automatically calculated based on the analytic curves. The results of CT data analysis using 22 preoperative and postoperative patient CT datasets show that the proposed diagnostic index for PC can diagnose and classify PC patients correctly, which confirms the feasibility of the evaluation index. Furthermore, digital measurement techniques can be employed to improve the diagnostic efficiency of PC, achieving one small step towards the computer-aided intelligent diagnosis and treatment for pediatric chest wall malformations.