Bi-valve braces for treatment of pectus carinatum in teenagers: impact on patients quality of life

Abstract

Background

Pectus carinatum may be a major issue for adolescent patients, as chest-wall malformations can have a negative impact on body image and induce self-esteem disorders. Bracing has become the first line treatment for adolescent patients whose anterior chest wall is still flexible. The main obstacle is the tolerability of the bracing device along with the patient’s compliance to the device. The aim of this study was to examine the quality of life of adolescent patients treated with bracing to correct pectus carinatum in our institution. From November 2021 to July 2023, 28 patients with pectus carinatum were treated with bracing therapy in our chest wall deformities unit. We used a bivalve brace, manufactured with computer-aided design. All patients were asked to answer a multiple-choice questionnaire designed to assess how bracing therapy impacts their daily life, treatment compliance, and overall satisfaction. Patients who wore the brace for at least 3 months, and at least 5 days per week and 10 h per day, are defined as compliant with the bracing protocol.

Results

The mean age of the patients was 14 ± 1.36 years. Patients wore the brace for an average of 10.1 ± 6.9 months in the compliance group (n = 18), and 2.2 ± 2.5 months in the non-compliance group (n = 10). Our results show that compliant patients are fairly satisfied of the comfort of the brace, with a limited impact of the device on their daily activities and social life. However, non-compliant patients report the following dissatisfactions regarding the brace: higher pain scores, difficulty in breathing, and issues sleeping with the brace. The compliant patients noted a significant improvement in the appearance of their chest, and were satisfied with the appearance of their chest. The non-compliant patients did not notice any changes.

Conclusions

Compliant patients treated with a bi-valve brace for pectus carinatum seem to have a good quality of life with a limited impact on their daily life and social activities. The bi-valve brace also seems to be an effective therapy in compliant patients.

Background

Pectus carinatum (PC) is the second most common chest wall deformity, with an incidence of 1 in 1000 adolescent patients, and results from an abnormal growth of costal cartilages leading to an anterior protrusion of the sternum with varying degrees of severity [1]. PC usually becomes noticeable in teenagers at the time of a growth spurt. PC may be a major issue for adolescent patients, as chest-wall malformations can have a negative impact on body image and induce self-esteem disorders [2]. Teenagers with PC may also avoid social interactions due to their chest wall deformity. The surgical treatment of PC is associated with significant postoperative pain and potential surgical morbidities such as bleeding, pleural effusions, or thoracic dystrophy [3]. The resultant scar may also impair the cosmetic result from the surgical correction of the chest wall deformity. The external compression of PC is a non-invasive alternative to surgery and has become the first-line treatment for adolescent patients whose anterior chest wall is still flexible, in guidelines published by the American Pediatric Surgical Association [4]. Bracing has been found to be effective to restore a normal thoracic shape by applying an external pressure to the anterior chest wall and to give satisfactory results in adolescents with no major complications [5,6,7,8]. The main limitation is the tolerability of the bracing device along with adherence and compliance. Some studies report high levels of treatment abandonment, leading to the failure of the bracing therapy [8,9,10]. The main reasons for non-compliance with bracing treatment are embarrassment of brace appearance, pain or discomfort, skin problems, and insufficient initial results of compression [11, 12]. However, in orthotic treatment of PC, compliance with bracing therapy is critical for success because the results are strongly related to the duration of compression [7] [13]. The quality of life for patients is therefore one of the key factors determining success of bracing, but few studies investigate the impact of bracing therapy on the daily life of young patients [14, 15]. We present herein the results of non-invasive treatment for PC with the use of a bi-valve brace which has not previously been described in the literature. The aim of this study was to examine the quality of life of adolescent patients treated with bracing to correct PC in our institution.

Methods

Our primary outcome with the impact of bracing on patients’ daily life, including the physical impact of bracing, the limitation of physical activities, and the social repercussions due to the brace. Our secondary endpoints were overall patient satisfaction towards bracing therapy and compliance with treatment.

Participants

From November 2021 to July 2023, 28 patients with PC were treated with bracing therapy in our chest wall deformities unit. Demographic characteristics, clinical features, and patients’ answers to the PEEQ (Pectus Excavatum Evaluation Questionnaire) at baseline were obtained from patients’ medical charts. All patients were asked during their follow-up visit to participate in the study by answering a multiple-choice questionnaire designed to assess how bracing therapy impacts their daily life, compliance with treatment, and satisfaction. Informed consent was obtained for each patient and their parents.

Bracing protocol

Clinical examination and PC diagnosis were performed by two thoracic surgeons in our outpatient clinic. An echocardiography was performed for every patient, once the diagnosis of PC was made, to detect any cardiac disorder associated with elastic tissue disease such as Marfan syndrome. Chest X-rays or chest computed tomography were not systematically performed in the absence of physical symptoms, such as chest pain, dyspnea, or signs of inflammation. The flexibility of costal cartilage was assessed by a manual compressive test, which includes compressing the sternal protrusion with one hand while fixing the back of the patient with the other hand. A bracing therapy was offered to each patient with a flexible deformity. All patients answered a questionnaire before beginning treatment to evaluate the psychological and physical impact of the pectus on their daily life. We used a French translation of PEEQ (Pectus Excavatum Evaluation Questionnaire) because there is no specific quality of life questionnaires for PC. A fitted chest compression brace was designed by our local orthotist for each patient (Fig. 1). The bi-valve brace is custom-made for every patient and is manufactured using a chest surface acquisition made with an optical scanner and a 3D computer-aided design approach. It is made of two polyethylene shells positioned on the anterior and the posterior chest. The shells are connected with an adjustable strap ratchet on each side, and a soft protective pad is added to the anterior shell to reduce friction on the skin. The patients were instructed to wear the brace over a t-shirt for skin protection for 20 h per day. They were allowed to remove the brace during sports, and they were also advised to continue their usual physical activities. A first follow-up visit was scheduled after 4 to 6 weeks with both the thoracic surgeon and the orthotist to assess the patient’s compliance, check the fitting of the brace, and detect any side effects. Patients were then evaluated at 3 months and every 3-to-6-month intervals. When complete correction was achieved, and if the patients were satisfied with the shape of their chest, they were instructed to wear the brace 10 to 12 h per day (during the day, or night, according to their preferences) for 6 months as a maintenance period. Weaning of the brace was then achieved progressively over another 3 to 6 months. Patients who wore the brace for at least 3 months, and at least 5 days per week and 10 h per day, are defined as compliant with the bracing protocol.

Fig. 1

Custom made bi-valve brace

Design of the questionnaire

Analysis of compliance

The first questions were designed to evaluate the patients’ compliance with bracing therapy.

Quality of life with the brace

To evaluate the impact of the use of bracing on patients’ quality of life, we used several questions from the questionnaire published by Pessanha et al. [15] (questionnaire used in a Portuguese center to assess factors that could influence compliance with bracing in patients treated for PC). The questions aimed to quantify the physical impact of bracing, the social repercussions, and the limitation of physical activities due to bracing. The questions related to their quality of life are summarized in Table 3 (see the results section).

Patient’s satisfaction

Due to the lack of a validated questionnaire specifically designed for patients treated with bracing for PC, we adapted and translated some questions from pectus excavatum quality of life questionnaires. Several questions from the PEEQ (Pectus Excavatum Evaluation Questionnaire) were used to evaluate patients’ satisfaction about the current shape of their thorax (the same questions had been asked before beginning bracing therapy, allowing comparison between baseline and the current self-assessment). Other questions were adapted from SSQ (Single Step Questionnaire) to assess the overall satisfaction with bracing therapy. PEEQ and SSQ have been validated [16, 17] and are also sometimes used for patients with PC [14]. Table 1 summarizes the questions used in our questionnaire to assess patients’ satisfaction with their chest, and it details how the satisfaction score was obtained. The patients were invited to give their opinion about the brace and to suggest areas of improvement for the device at the end of the questionnaire. The full questionnaire is available as an additional file.

Table 1 Patient’s satisfaction score

Data analysis

Patients were asked to answer the questionnaire during their routine follow-up visit. This is a retrospective study. Patients no longer being evaluated, or who were lost to follow-up, were contacted by email and by telephone. If the patient and their parents agreed to participate in this study, the questionnaire was sent to them by email with an explanatory letter. Continuous variables are presented as the means ± standard deviation and categorical variables as frequencies and percentage. Comparisons of continuous variables were performed using student’s t-test or non-parametric Mann–Whitney test when appropriate. For all statistical analysis, p < 0.05 was considered statistically significant. All statistical analyses were performed with JAMOVI (The jamovi project (2023). jamovi (Version 2.3) [Computer Software]. Retrieved from https://www.jamovi.org).

Results

Patients’ characteristics and compliance with bracing

Twenty-eight patients were included in this study, with a mean age of 14 ± 1.36 years at the start of bracing. The study population included 27 males (94.4%) and 1 female. Sixteen (57.1%) patients had a symmetric PC, and 12 (42.8%) had an asymmetric deformity. Only one patient had a family history of chest wall deformity (3.57%). Eighteen (64.3%) patients were considered compliant with treatment, and 10 (35.7%) of them were non-compliant. As expected, compliant patients wore the brace longer than non-compliant patients, and more frequently (see Table 1). Nine patients chose to wear the brace only during the day, 7 of them only at night, and 12 wore it night and day, without any difference between compliant and non-compliant patients (Table 2).

Table 2 Compliance with bracing protocol

Fifteen patients (53.6%) were still wearing the brace. Among the 13 (46.4%) patients who are no longer using the brace, 5 of them (38.5%) stopped treatment because they were satisfied with the look of their chest and no longer considered the need for bracing. The 8 other patients (61.5%) have interrupted bracing because they felt that the brace was uncomfortable and/or unaesthetic.

Quality of life with bracing

Overall, compliant patients consider that the brace is rather comfortable, whereas non-compliant patients think that the brace is uncomfortable. The non-compliant group has a significantly higher score on each variable evaluating the physical discomfort associated with the brace: They feel more pain with the brace, they have more difficulty in breathing, and they find that it is more difficult to fall asleep with the brace than compliant patients (Table 3). They also feel more impaired in their daily life because of the brace. Regarding the social impact of bracing, the non-compliant patients are also more frequently uncomfortable wearing the brace in front of others. Wearing the brace at school is also more problematic for them. The compliant patients most frequently answered “never” or “rarely” to all the questions assessing how the brace might negatively impact their quality of life, whereas non-compliant the most frequent answers of non-compliant patients were “sometimes” or “often.” Table 3 summarizes the mean score of each group of patients on the quality-of-life questionnaire.

Table 3 Quality of life in patients treated with bracing and self-reported factors affecting compliance with treatment

Patients’ satisfaction

The satisfaction score about the current look of their chest is significantly higher in the compliance group (Table 4). The compliant patients are fairly satisfied with the way their chests look now, whereas non-compliant patients express dissatisfaction. In the compliance group, patients consider that the look of their chest has been significantly improved since the start of bracing. Non-compliant patients felt that their chests have not changed, which led to a significantly higher score from the compliance group. The patients’ satisfaction score was obtained by adding the scores on the nine questions assessing the psychological impact of PC (Table 4). This score was calculated at baseline just before starting bracing (pre-treatment satisfaction score) and at the time of the study (post-treatment satisfaction score). The post-treatment satisfaction score is higher than the pre-treatment score, in both groups. Patients were also asked if they would recommend bracing therapy to a friend with PC: 83.3% of compliant patients and 50% of non-compliant patients answered “yes” (p = 0.071).

Table 4 Patients’ satisfaction about their chest

None of the patients included in this study, who considered themselves satisfied or dissatisfied with the look of their chest, considered surgery as an alternative treatment for their PC.

The patients suggested that improvement of the brace comfort (82.1% of them) and its aesthetic (32.1% of them) might help the compliance and adherence to treatment. Meeting other patients who had successfully completed the bracing protocol would be motivating for 52.6% of them, and talking to other patients who are wearing a similar brace would also be useful to improve compliance for 21.4% of them.

Discussion

Bracing therapy for PC was first described by Haje et al. in 1995 [18] and is now the first line treatment in adolescent patients [7, 8] [19]. Several studies demonstrate that bracing is an effective therapy in compliant patients [7,8,9,10] [19, 20], but few studies have investigated the quality of life of these young patients who are asked to wear a brace every day for several months. The quality of life during bracing is crucial because it is strongly related to the compliance of treatment, which is critical to the success of bracing [8]. We present the results of our bracing protocol with the use of a specific bi-valve brace, which has never been described for the treatment of PC. Our results show that compliant patients are fairly satisfied with the comfort of the brace and the limited impact on their daily activities and social life. Patients that we defined as non-compliant, who have failed to adapt to the brace, reported higher scores of pain, difficulty in breathing, and difficulty in sleeping with the brace. Our results indicated that, in addition to the physical symptoms, the psychosocial impact of bracing is important for non-compliant patients because they feel significantly uncomfortable in front of others when they wear the brace. This might suggest that both the discomfort of the brace and the negative psychosocial impact of wearing the device would contribute to non-compliance. Indeed, Moon et al. [11] showed that the main factors affecting compliance with bracing were embarrassment of brace appearance, pain, and discomfort. Other studies suggest that discomfort and shame are not correlated with compliance and that the main predictor of compliance is the initial result of compliance [12] [15]. Fraser et al. [10] point out that a rapid correction of the deformity provides motivation to carry on bracing. The temporality of the effectiveness of bracing was not evaluated in our study. We found that 64.3% of the patients were compliant with our bracing protocol. The compliance rates reported in the literature are inconsistent and may vary from less than half of the patients [12] to 90% of the study population [11]. Firstly, the definition of compliance varies among studies. For example, Moon et al. consider a patient as compliant if he had completed the first two-week compression period [11], whereas a compliant patient is one who has worn the brace at least 6 months in Kang et al.’s study [12]. Secondly, the bracing protocols and the follow-up are also different among studies. The compression phase is highly variable and may vary from a few weeks to several months. The first weeks of bracing are an adjustment period, during which the patient must accept the brace related constraints and to include the brace in his/her daily life. These first weeks may be particularly difficult for adolescent patients, and it is therefore important to perform early monitoring [10]. The reasons why some patients easily got used to wearing the brace, while others quickly lose motivation and give up bracing therapy, remains unclear. The psychological and emotional factors involved in a patient’s adherence and initial motivation should be taken into consideration when a bracing therapy is offered. The optimum age at which bracing should be started is also not consensual. The mean age at the start of bracing in our study (14 years) is similar to other studies [11] [14, 15]. Some authors described very good results in younger patients with a low recurrence rate [12]. Nevertheless, the age at which bracing can be started depends on when the patients are referred to us by general practitioners. These doctors are sometimes not aware that a non-invasive treatment may be offered to their patients with PC.

In our study, the compliant patients noted a significant improvement in the appearance of their chest and are rather satisfied with the current look of their chest. As expected, the non-compliant patients did not notice any change. It is noteworthy that the patient’s satisfaction score, assessing the current psychosocial impact of their PC, has shown improvement in both groups. This could suggest that non-compliant patients may have benefited from receiving treatment and dedicated follow-up appointments. This treatment plan may have also enabled them to better accept and adapt to their PC, even though the brace was not worn as prescribed.

In addition, there is no consensus about the best device to use for bracing therapy. In most studies, the brace is typically made of two bars positioned on the anterior and posterior chest with an anterior pad for compression of the PC [11,12,13,14,15]. Some authors also use a dynamic compression system in addition to the brace [19,20,21,22]. The use of dynamic compression allows to gradually reduce the sternal protrusion, to monitor the applied pressure, and is supposed to reduce discomfort and improve compliance with treatment. Numerous studies indicate favorable outcomes associated with dynamic compression systems [19,20,21,22]. However, the superiority of these devices over alternative braces in terms of effectiveness, compliance, or patient satisfaction remains undetermined. All these devices are not covered by the French Health insurance, and the cost of the devices are entirely borne by the patients’ parents. Traditional braces can cost several hundred of euros (from 500 to 800 euros), and dynamic compression systems can be charged up to 1500 to 2000 euros. The device used at our institution is a custom-made bi-valve brace composed of an anterior and a posterior shell. It is fully covered by the French national health insurance, ensuring that every patient can have access to this therapy. However, the financial coverage of the brace cost depends on the healthcare policy of each country.

Compared to other braces, the bi-valve brace covers more parts of the chest, which could be less comfortable for patients. However, the custom-made design of the bi-valve brace by the orthotist ensure that the compression is limited to the sternal protrusion. It would nevertheless be interesting to objectively compare the comfort of the bi-valve brace with other devices. To the best of our knowledge, this is the first study reporting on the use of a bi-valve brace for the correction of PC.

There are some limitations to this study. First, this is a single center study with a limited number of patients. Half of our patients are still on treatment, and we cannot present long-term results of our bracing protocol for now. Second, patients’ satisfaction is subjectively measured. We did not perform any imaging such as computed tomography to assess objectively the efficacy of our device. We chose not to perform systematic imaging to avoid unnecessary radiation exposure. We are convinced that utilizing external 3D scanning, a radiation-free and objective method for evaluating and monitoring chest wall deformities, is a valuable tool. It has the potential to keep patients motivated throughout their treatment journey. However, external 3D scanning is not yet available in our institution.

Conclusions

Compliant patients treated with a bi-valve brace for PC seem to have a pretty good quality of life with a limited impact of the brace on their daily life and social activities. The bi-valve brace also seems to be an effective therapy for PC in compliant patients. Continuously enhancing both the comfort and aesthetic appeal of the brace is essential in order to minimize the likelihood of non-compliance with the treatment. Frequent and careful follow-up appointments of these young patients is a key factor of the treatment’s success. Every action that may help the patients to adhere to the treatment and to remain motivated should be promoted, such as organizing meetings and dedicated discussion groups with collaborating patients.