Non-surgical approaches to the management of chest wall deformities.

Chest wall deformities in children encompass a broad spectrum of disorders but pectus excavatum and carinatum are by far the most common. Treatment varies substantially by center, and depends on patient symptoms, severity of disease, and surgeon preference. Historically, surgical approaches were the mainstay of treatment for these disease processes but new advances in non-surgical approaches have demonstrated reasonable results in select patients. These non-surgical approaches include vacuum bell therapy, autologous fat grafting and hyaluronic acid injections for pectus excavatum, and orthotic brace therapy for pectus carinatum. There is debate with regards to optimal patient selection for these non-surgical approaches, as well as other barriers including reimbursement issues. This paper will review the current non-surgical approaches to chest wall deformities available, including optimal patient selection, treatment protocols, indications, contraindications, and outcomes.

Chest wall deformities and their possible associations with different genetic syndromes.

OBJECTIVE: Our primary objective was to identify discrete and syndromic cases of Pectus excavatum (PE) and Pectus carinatum (PC). We also intended to highlight the significance of further genetic exploration in clinically suspected syndromic cases of PC and PE. Pectus excavatum (PE) and Pectus carinatum (PC) are the most common morphological chest wall deformities. Although various hypotheses have been put forth, the pathogenesis of both entities is largely unknown. Clinicians often refer such cases for further genetic evaluation to exclude an associated underlying connective tissue disorder or a syndrome. Additionally, a detailed anamnesis with focused family history and thorough dysmorphological physical examination was done. PE and PC are considered isolated abnormalities if there is the absence of features of other syndromes, eliminating the need for further genetic evaluations. It is believed that the pattern of inheritance of these non-syndromic isolated PE and PC cases with positive family history could be multifactorial in nature. The recurrence risk of such isolated cases is thought to be low. Further diagnostic studies are indicated as PE and PC could be a part of a syndrome. Among the many syndromes, the most common monogenic syndromes associated with PE and PC are Marfan's and Noonan's. PATIENTS AND METHODS: After obtaining the consent, we compiled a database of the patients who presented with chest wall deformities during the period 2017-2019. We selected 70 cases with PC and PE deformities to identify the discrete and syndromic PC and PE cases. During the study, we perused the cytogenetic and/or molecular analyses, that had been conducted to confirm the clinically suspected syndromic cases. We also scrutinized for the presence of PC and PE cases that are associated with the rare syndrome (s). RESULTS: Various genetic abnormalities were identified in 28 (40%) of the 70 cases that had been diagnosed with chest wall abnormalities. Along with PE and PC, other thoracic wall abnormalities were also identified, such as the broad chest, bell-shaped thorax, and elongated or enlarged thorax. One case of a rare genetic disorder of Morquio syndrome associated with PC was also identified. Novel (previously unpublished) genomic variants are reported here. CONCLUSIONS: It is important to delve deeper when encountering cases of PE and PC by conducting a further genetic exploration of such cases to identify syndromic associations that cause other structural and functional disorders, diagnosis of which might be missed during the early developmental period. Early identification of such disorders may help us correcting the defects, slowing the progression of disease processes, and preparing better to deal with the potential outcome.

Combined surgical correction of pectus carinatum and juvenile kyphosis.

Pectus carinatum may rarely be associated with kyphosis. However, the correlation between both conditions is not well reported. Therefore, there are no reports for combined correction of both deformities in the same patient. Moreover, studies estimating the kyphosis prevalence in patients with pectus carinatum are lacking. To our knowledge, this is the first paper to present such a case. We report an 18-year-old boy with both pectus carinatum and kyphosis that were surgically corrected in a combined procedure. The indication of surgery is cosmetic, and the postoperative recovery included pneumothorax but was otherwise uneventful and satisfactory.

External Compressive Bracing With Initial Reduction of Pectus Carinatum: Compliance Is the Key.

BACKGROUND: To assess the impact of manipulation and a tailored program for compressive bracing on the quality of life of patients with flexible pectus carinatum. METHODS: Two hundred forty-nine sequential patients attending a clinic for assessment of pectus carinatum deformities underwent outpatient manipulation and then followed a prescribed schedule of continuous external compressive bracing but without significant progressive tightening. RESULTS: There was successful sustained reduction of the deformity in 244 patients with high reported rates of concordance (98%) and satisfaction (94%). Patients experienced a reduction in symptoms of anxiety and depression (P < .001) and had improved body satisfaction (P < .001). Mild skin irritation occurred in 18% of patients (n = 44), and there were 2 severe cases of skin irritation, 1 of which resulted in abandonment of bracing. CONCLUSIONS: Manipulation and nontightening compressive bracing was associated with complete concordance, high levels of successful bracing, improved confidence, and reduced psychological morbidity.

Long-Term Results of Compressive Brace Therapy for Pectus Carinatum.

BACKGROUND: Pectus carinatum (PC) is one of the most common types of congenital chest wall deformity. Recently, noninvasive compressive brace therapy has been more frequently used than invasive surgical correction to treat PC. Hence, the purpose of this study was to determine the long-term outcome of compressive brace therapy. METHODS: We retrospectively reviewed patients with PC who underwent compressive brace therapy between January 2014 and December 2016. All patients underwent a 2-week compression period, in which braces were worn for 20 hours per day, followed by a 6-month maintenance period, in which braces were worn for 12 hours per day. Patient satisfaction was investigated via telephone survey. RESULTS: A total of 320 patients were included in this study. The average age was 13 years, and 280 were males (87.5%). The median follow-up period was 42 months (13-68). Good compliance was observed in 286 patients (89.4%; compliance group). In this group, the initial Haller index significantly increased from 2.20 ± 0.31 to 2.59 ± 0.38 after the 6-month therapy period (p = 0.001). After the 6-month period, 255 patients (89.1%) and 31 patients (12.1%) in the compliance group were very satisfied and satisfied, respectively. Satisfaction at the last follow-up via telephone survey was very satisfied in 250 patients (87.4%) and satisfied in 36 (12.6%). In the compliance group, no patient needed compressive braces again after the therapy period. CONCLUSION: Given the findings presented in this study, compressive brace therapy appears to be a relatively simple and safe method with good long-term outcome in treating patients with PC.

Deformidad de la caja torácica / Chest wall deformity

Resumen Las deformidades de la caja torácica se pueden dividir en dos tipos, las que son productos del desarrollo anormal del pecho en el crecimiento y las congénitas que son las secundarias a una malformación estructural del pecho evidente en el nacimiento. Las malformaciones del desarrollo son las más comunes, como por ejemplo pectus excavatum o pectun carinatum. Las menos comunes son las de tipo congénito: síndrome de Poland, displasia espondilotorácica, displasia espondilocostal, síndrome de Jeune y los defectos de la costilla o el esternón. Las deformidades del pecho de tipo congénita se caracterizan por afectar la relación entre la columna vertebral, la caja torácica y los pulmones. La mayoría de estos pacientes desarrollan un disturbio respiratorio progresivo de tipo restrictivo conocido como Síndrome de Insuficiencia Torácica. Este síndrome se define como la deficiencia de la caja torácica para mantener una respiración normal y sostener el crecimiento fisiológico del pulmón. En este artículo discutiremos varias condiciones que afectan el desarrollo y función de la caja torácica.

Chest wall deformities are divided as an abnormal development during the growth or those secondary to a congenital malformation. The developmental type is the most common: pectus excavatum or pectus carinatum. The less common are the congenital types of chest wall abnormalities: Poland's syndrome, Jeune's syndrome, espondylothoracic dysplasia, espondylocostal dysplasia and defects of the ribs or sternum. The congenital type usually affects the relationship between the spine, rib cage and the lungs. Therefore, many of these patients will develop a progressive respiratory disturbance of restrictive type known as Thoracic Insufficiency Syndrome. Thoracic insufficiency syndrome is defining as a deficiency of the rib cage to maintain a normal respiration and to sustain the physiological growth of the lungs. In this article will discuss several conditions that will affect the development and function of the chest wall.

[Surgical treatment of pectus carinatum in children]. / Khirurgicheskoe lechenie kilevidnoi deformatsii grudnoi kletki u detei.

AIM: To improve the results of surgical treatment of pectus carinatum. MATERIAL AND METHODS: The work included 47 pectus carinatum patients aged 3-15 years for the period from 2000 to 2015. All patients were divided into 2 groups depending on surgical approach: control group - 23 (48.94%) children who underwent thoracoplasty by M.M. Ravitsh procedure with the use of device for pectus carinatum repair of our design; main group - 24 (51.06%) patients where our method of thoracoplasty based on changes of deformed ribs biomechanics was used. RESULTS: In control group good results were obtained in 15 (65.3%) patients, satisfactory - in 5 (21.7%) patients, recurrent deformation with all consequences was observed in 3 (13%) cases. Long-term results of surgical treatment by original method were followed-up in all 24 patients and good outcomes were obtained in 100% of children. CONCLUSION: 1) Intraoperative 'spring' - effect is a provoking risk factor of recurrent chest deformation. 2) The new surgical method based on breaking the ribs bodies in 'green branch' fashion provides good results and does not require external devices to hold sternal-rib complex in corrected position.

Pigeon chest: comparative analysis of surgical techniques in minimal access repair of pectus carinatum (MARPC).

BACKGROUND: After minimally invasive repair for pectus excavatum (MIRPE), similar procedures for pectus carinatum were developed. This study aimed to analyse the various published techniques of minimal access repair for pectus carinatum (MARPC) and compare the outcomes. DATA SOURCES: Literature was reviewed on PubMed with the terms "pectus carinatum", "minimal access repair", "thoracoscopy" and "children". RESULTS: Twelve MARPC techniques that included 13 articles and 140 patients with mean age 15.46 years met the inclusion criteria. Success rate of corrections was n = 125, about 89% in cumulative reports, with seven articles reporting 100%. The complication rate was 39.28%. Since the pectus bar is placed over the sternum and has a large contact area, skin irritation was the most frequent morbidity (n = 20, 14.28%). However, within the complication group (n = 55), wire breakage (n = 21, 38.18%) and bar displacement (n = 10, 18.18%) were the most frequent complications. Twenty-two (15.71%) patients required a second procedure. Recurrences have been reported in four of twelve techniques. There were no lethal outcomes. CONCLUSIONS: MARPC techniques are not standardized, as MIRPE are, so comparative analysis is difficult as the only common denominator is minimal access. Surgical morbidity is high in MARPC and affects > 2/3rd patients with about 15% requiring surgery for complication management.

Anomalías pulmonares congénitas y de la pared torácica / Congenital lung and chest wall malformations

El propósito de este artículo es evaluar las lesiones congénitas pulmonares y de la pared torácica que se pueden detectar desde la etapa fetal hasta alcanzar el desarrollo adulto. Nos hemos centrado en las patologías más prevalentes en ambos campos: malformaciones pulmonares congénitas aéreas (MPCA), secuestro broncopulmonar, enfisema lobar congénito, atresia bronquial y quiste broncogénico, en cuanto a las lesiones de parénquima pulmonar, y pectus excavatum, pectus carinatum, pectus arcuatum y síndrome de poland en el grupo de lesiones de pared torácica. Hemos evitado las descripciones casuísticas y las prolijas explicaciones quirúrgicas para centrarnos en la clínica y en el manejo inicial de estas malformaciones

The aim of this study was to evaluate congenital lung lesions and chest-wall deformities that can be diagnosed from the fetal period until the adulthood. We focused on the most prevalent lesions of each group: Congenital pulmonary airway Malformation (CpaM), Bronchopulmonary Sequestration, Congenital lobar emphysema, Bronchial atresia and Bronchogenic Cyst regarding to parenchymal lesions and pectus excavatum, Carinatum and poland's Syndrome in the group of wall defects. We tried to avoid series description or surgical details in order to highlight clinical issues and management decisions

Incidence and Classification of Chest Wall Deformities in Breast Augmentation Patients.

Although chest wall deformities (CWDs) are seen four times more often in males than in females, most patients who consult plastic surgery clinics in our country are females. Breast augmentation and augmentation mastopexy were performed with a total of 812 breast implants in 406 patients. Forty-three of these patients had various CWDs. The CWD patient ages ranged from 18 to 43 years. Patients were observed for 6 months to 8 years (mean of approximately 3 years). Pectus excavatum (PE) was present in 22 patients, pectus carinatum (PC) in five patients, Poland syndrome (PS) in two patients, sunken chest deformity (SCD) in three patients, barrel chest deformity (BCD) in five patients, body builder deformity (BBD) in three patients, and long upper chest wall (LCW) in three patients. None of the patients had functional or cardiac deformities. A total of 86 round, anatomically textured gel implants in various profiles were used within a range of implant volumes for all patients. There were no serious complications in the 43 CWD patients. The frequency of CWDs in the total population of 406 patients was approximately 10.6%. PE was present in 51% (22/43) of the patients with CWDs. The deformity rates among patients who underwent augmentation mammoplasty were as follows (%): PE, 5.4; PC, 1.23; PS, 0.5; SCD, 0.73; BCD, 1.23; BBD, 0.73; and LCW, 0.73. The three most common deformities observed in this study were PE, PC, and BCD. The least common deformity was PS. Patients with CWDs should be carefully evaluated preoperatively, and all the associated measurements and calculations must be meticulously performed. The implant selection may differ according to the deformity pattern. For example, in patients with PE, prostheses with larger base diameters cover the deformity better. Although high-profile prostheses are preferred on the affected side in cases of PS and SCD, low- or middle-profile prostheses are preferred on the opposite side. Despite this common consensus, there was an asymmetry of approximately 1 cm between the sides, as observed postoperatively. However, the patients did not express dissatisfaction with this situation. Low-profile prostheses should be preferred for PC and BCD protruding CWDs because asymmetry becomes more prominent and over-projection occurs more frequently in cases of high-profile prostheses. The BBD projection is also a problem. For this reason, high-profile prostheses should be preferred in these patients. It is possible to obtain satisfactory results by using appropriate breast implants in patients with CWDs. 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 .

[Pectus excavatumn and carinatum in children and adolescents : what to say, what to do ?] / Pectus excavatum et carinatum chez l'enfant et l'adolescent : que dire, que faire ?

In children presenting with a pectus excavatum (PE) or pectus carinatum (PC) an underlying syndrome including Marfan's syndrome needs to be excluded. In adolescents, severe chest wall deformities may cause cardiac or respiratory problems but most commonly they have a psychological impact. The conservative treatment is a Vacuum Bell for PE, and a Dynamic Compression System for PC ; they play an increasing role in young patients. These devices need to be worn multiple hours per day for several months for an optimal result. Surgery is usually reserved for adolescents. The Nuss procedure for PE, also known as minimal invasive repair of PE offers excellent results. Sterno-chondro-plasty with stabilisation of the sternum with struts clipped to the ribs offers the same good results for PC. In our opinion, a multidisciplinary approach is preferable.

En présence d'un thorax en entonnoir (pectus excavatum, PE) ou en carène (pectus carinatum, PC), une affection syndromique, dont la maladie de Marfan, doit être recherchée. La déformation, avant tout disgracieuse, peut générer des troubles psychologiques chez l'adolescent, et des désordres cardiorespiratoires dans les formes sévères. Les traitements conservateurs sont très utiles chez le jeune : cloche aspirante pour PE ou corset dynamique de compression pour PC. Ils sont portés quelques heures par jour pendant plusieurs mois selon un « contrat moral ¼ établi avec l'enfant. La chirurgie peut être négociée chez l'adolescent. La technique mini-invasive par thoracoscopie de Nuss offre des résultats satisfaisants dans les PE. Il en est de même des sternochondroplasties fixées par des attelles-agrafes pour les PC. Un avis pluridisciplinaire est utile.

Symptoms in Pectus Deformities: A Scoring System for Subjective Physical Complaints.

Background The literature is silent on the relationship between symptoms and the Haller index. Nor is there a classification of the severity of the physical complaints. Materials and Methods Retrospectively, data from 128 patients (102 funnel, 25 pigeon chest patients, and 1 mixed type) were evaluated. To objectify the symptoms, we developed a score to describe the level of physical ailments. This score includes 10 different symptoms as well as the situation or frequency in which they occur and an impact factor. This depends on how much they affect everyday life. Results Pectus excavatum patients express physical complaints more frequently than pectus carinatum patients who actually suffer more from psychological stress. We could not find a correlation between the Haller index and symptoms or levels of ailment. Conclusion Pectus deformities are likely to cause physical and psychological complaints. Since the subjective symptoms did not show any correlation to the chest severity index, they are supposed to be independent from the deformity's extent.

Induced Pectus Carinatum.

During the surgical repair of a truncus arteriosus with aortic arch interruption and pulmonary artery origin stenosis, a Contegra conduit was implanted as part of reconstruction of the pulmonary artery in a small infant. There was a mismatch between the conduit size and the patient that resulted in protrusion of the conduit between the sternal edges. To accommodate the conduit inside the thoracic cavity, traction was applied to the sternum that was gradually released over a period of time guided by transesophageal echocardiography-derived cardiac output data, as well as continuous hemodynamic parameters. doi: 10.1111/jocs.12734 (J Card Surg 2016;31:357-360).

A new technique for mixed-type pectus carinatum: modified Onen method.

We modified the Onen method for pectus carinatum repair and used a vertical incision instead of a transverse incision. The most important advantage gained by the vertical incision is that we can switch to the Ravitch method without needing an additional incision, by elongating the existing incision in patients in whom a pectus bar cannot be placed. We successfully performed the modified Onen technique in a 16-year-old boy with a mixed-type pectus carinatum deformity.

Evaluation of sternochondroplasty efficacy in treatment of pectus deformities.

PURPOSE: This study aimed to evaluate the efficacy of sternochondroplasty for repair of pectus deformities. METHODS: Thirty-three patients underwent repair of pectus deformity with a sternochondroplasty technique between 1989 and 2009. Studied variables were age, sex, symptoms, pulmonary function tests before and after surgery, Haller index, complications, recurrence, and cosmetic results. RESULTS: The median age at operation was 13.27 years. Twenty patients (13 male and 7 female) had pectus excavatum, and 13 (11 male and 2 female) had pectus carinatum. Congenital anomalies associated with pectus excavatum included a skeletal anomaly in 6 (30%) patients and a cardiac anomaly in 4 (20%); only 1 patient with pectus carinatum had cardiac anomaly. Inspiratory vital capacity and forced expiratory volume in 1 s were significantly different before and after surgery in patients with pectus excavatum, but there was no significant difference in functional residual capacity. Pulmonary function tests showed no significant differences before and after surgery in patients with pectus carinatum. Complications after surgery were seroma in 2 cases and 1 case each of pneumothorax and atelectasis. Recurrence was observed in 2 cases of pectus excavatum. The cosmetic result was excellent in 93.9% of cases. Hospital mortality was zero. CONCLUSION: Sternochondroplasty is a desirable procedure for repairing pectus deformities. This procedure has minimal complications and the cosmetic and physiologic results are excellent.

Factors affecting patient compliance with compressive brace therapy for pectus carinatum.

OBJECTIVES: The aim of this study was to identify factors affecting patient compliance with brace therapy for pectus carinatum. METHODS: Eighty-six pectus carinatum patients who started brace therapy from August 2008 to November 2011 were included in this study. Patients were divided into two groups: patients who wore the brace for ≥6 months (compliance group) or patients who wore the brace for <6 months (non-compliance group). Factors affecting patient compliance were assessed at the last day of follow-up with a multiple-choice questionnaire. The questionnaire comprised seven items: pain at compression site, skin problems on compression area, confidence in brace treatment, shame, discomfort, initial result of bracing treatment and total number of factors affecting patient compliance. RESULTS: Eighty-six patients completed the survey, including seven (8.1%) female patients and 79 (91.9%) male patients, with a mean age of 12.0 years at the time of treatment (range, 3-20 years). The initial result of the compression period (P <0.001) and total number of factors affecting patient compliance (P <0.05) were significant predictors of patient compliance. CONCLUSIONS: An initial successful result of the compression period may increase patient compliance during treatment for pectus carinatum. Additional efforts to decrease pain, skin problems, shame and discomfort, and to give confidence may be beneficial in increasing compliance with bracing treatment.

Evaluation of the treatment of pectus carinatum with compressive orthotic bracing using three dimensional body scans.

PURPOSE: The purpose of this study is to measure the effectiveness of compressive orthotic brace therapy for the treatment of pectus carinatum using an adjusted Haller Index (HI) measurement calculated from 3D body scan (BS) images. METHODS: Pediatric patients with pectus carinatum were treated with either compressive orthotic bracing or observation. An adjusted BS Haller index (HI) was calculated from serial 3D BS images obtained on all patients. Medical records were evaluated to determine treatment with bracing and brace compliance more than 12hours daily. Compliant patient measurements were compared to non-compliant and non-brace groups. RESULTS: Forty patients underwent compressive orthotic bracing, while ten were observed. Twenty-three patients were compliant with bracing, and seventeen patients were non-compliant. Compliant patients exhibited an 8.2% increase, non-compliant patients had a 1.5% increase, and non-brace patients exhibited a 2.5% increase in BS HI. The change in BS HI of compliant patients was significantly different compared to non-brace patients (p=0.004) and non-compliant patients (p<0.001). CONCLUSIONS: Three dimensional BS is an effective, radiation free, and objective means to evaluate patients treated with compressive orthotic bracing.

Chest wall reconstruction in Marfan syndrome following aortic root replacement.

Chest wall and spine deformities are common in Marfan syndrome, and often coexist with cardiac defects. Surgery is often needed to both the aortic root and the anterior chest wall, and early spinal surgery is not uncommon. We describe a case of severe thoracic cage deformity with aortic root dilatation, which was managed by a staged approach, with a very good final result.