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.

Pectus excavatum: the effect of tricuspid valve compression on cardiac function.

BACKGROUND: Pectus excavatum (PE) is a common congenital chest wall deformity with various associated health concerns, including psychosocial impacts, academic challenges, and potential cardiopulmonary effects. OBJECTIVE: This study aimed to investigate the cardiac consequences of right atrioventricular groove compression in PE using cardiac magnetic resonance imaging. MATERIALS AND METHODS: A retrospective analysis was conducted on 661 patients with PE referred for evaluation. Patients were categorized into three groups based on the degree of right atrioventricular groove compression (no compression (NC), partial compression (PC), and complete compression(CC)). Chest wall indices were measured: pectus index (PI), depression index (DI), correction index (CI), and sternal torsion. RESULTS: The study revealed significant differences in chest wall indices between the groups: PE, NC=4.15 ± 0.94, PC=4.93 ± 1.24, and CC=7.2 ± 4.01 (P<0.0001). Left ventricle ejection fraction (LVEF) showed no significant differences: LVEF, NC=58.72% ± 3.94, PC=58.49% ± 4.02, and CC=57.95% ± 3.92 (P=0.0984). Right ventricular ejection fraction (RVEF) demonstrated significant differences: RVEF, NC=55.2% ± 5.3, PC=53.8% ± 4.4, and CC=53.1% ± 4.8 (P≥0.0001). Notably, the tricuspid valve (TV) measurement on the four-chamber view decreased in patients with greater compression: NC=29.52 ± 4.6; PC=28.26 ± 4.8; and CC=24.74 ± 5.73 (P<0.0001). CONCLUSION: This study provides valuable insights into the cardiac consequences of right atrioventricular groove compression in PE and lends further evidence of mild cardiac changes due to PE.

The Association of a Single Nucleotide Variant in COL5A1 to Early Onset Keratoconus and Pectus Excavatum-Convergence of Extracellular Matrix Pathologies.

Keratoconus is a bilateral ocular condition characterized by irregularities and the thinning of the cornea. Decreased central corneal thickness is a hallmark of the condition, and numerous genes have played a role in altering corneal thickness and the subsequent development of keratoconus. Variants in the structural and regulatory genes of the extracellular matrix have been highly associated with keratoconus, as well as with pectus excavatum, a chest wall deformity commonly seen in connective tissue disorders. This report describes a patient with a c.1720-11T>A intronic variant in the collagen-encoding gene, COL5A1, who was diagnosed with early-onset keratoconus and demonstrated a significant pectus excavatum. This report associates a COL5A1 variant with these seemingly unrelated phenotypic associations, further advancing the literature on the topic.

Sternal cleft and pectus excavatum: an overlooked congenital association?

BACKGROUND: Sternal cleft (SC), a rare thoracic malformation, is associated with pectus excavatum (PE) in 2.6-5% of cases. It remains unclear if these conditions are congenitally linked or if SC repair triggers PE. To investigate the potential higher frequency of PE in SC cases, we conducted a retrospective study of our SC patients. METHODS: We assessed PE incidence, progression, and management in SC patients treated at our institute from 2006 to 2022. When available, we collected pre-SC repair CT scan data, calculating the Haller Index (HI) and Correction Index (CI) and compared them to a selected control group. RESULTS: Among 8 SC patients, 7 had concomitant PE (87.5%), varying in severity. PE management ranged from observation to thoracoplasty, depending on its degree. We observed a significant pre-operative CI difference between SC and control group patients (p < 0.00001). In the last two SC repair cases, we attempted concurrent PE prevention or treatment. CONCLUSION: Our findings suggest an underestimated association between PE and SC in the existing literature. SC patients may exhibit a predisposition to PE from birth, which may become more apparent with growth after SC repair. Consequently, PE prevention or treatment should be considered during SC repair procedures.

Complications after Nuss bar removal procedure for pectus excavatum. Analysis and proposal of a safety protocol. / Complicaciones tras retirada de barra de Nuss en pectus excavatum. Análisis y propuesta de un protocolo de seguridad.

OBJECTIVE: The Nuss bar removal procedure may bring about different complications. Some are mild while others can be life-threatening. An adequate surgery setup and the fulfilment of some security steps may reduce their incidence. This study aims to analyze our experience with the complications that occurred during bar removal and our safety protocol for the prevention and management of these complications. MATERIALS AND METHODS: Observational cohort study from a retrospective chart review of all patients who underwent Nuss bar removal from November 2013 to March 2022 at a University hospital. Variables analyzed include patients' demographics; presence of comorbidities; time elapsed from bar placement to removal, and the occurrence of operative and postoperative complications. Study written under the 'PROCESS Guideline'. RESULTS: Fourty (40) patients were included in the study; 37 were male. One bar was removed in 17 patients and two in 22 patients. Median age at surgery: 17.5 years (Percentile 25-75%: 16.75-19.25). Time elapsed from placement to removal: 26 months (Percentile 25-75%: 23.75-30.25). Complications: 10 in 9 patients (22.5%); 6 Clavien-Dindo class I (67%); 2 class II (22%); 1 class IIIb, 1 class IV. The hemorrhagic complication motivated the development of a safety protocol to reduce incidence of complications. CONCLUSION: Nuss bar removal is a safe procedure with usually scant complications. Nonetheless, these may be serious sometimes. To prevent them, a protocol for a safe procedure is important.

OBJETIVO: La retirada de la barra de Nuss puede provocar diversas complicaciones, algunas leves y otras potencialmente mortales. Su incidencia puede verse reducida con una preparación quirúrgica adecuada y siguiendo ciertos pasos de seguridad. El presente estudio tiene por objeto analizar nuestra experiencia con las complicaciones acontecidas durante la retirada de la barra, así como nuestro protocolo de seguridad para la prevención y el manejo de dichas complicaciones. MATERIAL Y METODOS: Estudio de cohortes observacional llevado a cabo a partir del análisis retrospectivo de todos los pacientes sometidos a cirugía de retirada de barra de Nuss entre noviembre de 2013 y marzo de 2022 en un hospital universitario. Se analizaron las siguientes variables: demografía de los pacientes, presencia de comorbilidades, tiempo desde la colocación de la barra hasta su retirada, y complicaciones operatorias y postoperatorias. El estudio se realizó conforme a las directrices de la PROCESS Guideline. RESULTADOS: Se incluyó a 40 pacientes, 37 de ellos varones. En 17 pacientes se retiró una barra, y en 22, dos. La edad media en el momento de la cirugía fue de 17,5 años (percentil 25-75%: 16,75 - 19,25). El tiempo transcurrido desde la colocación hasta la retirada fue de 26 meses (percentil 25-75%: 23,75 - 30,25). Se registraron 10 complicaciones en 9 pacientes (22,5%), 6 de clase I según la clasificación de Clavien-Dindo (67%), 2 de clase II (22%), 1 de clase IIIb y 1 de clase IV. La complicación hemorrágica motivó la creación de un protocolo de seguridad para disminuir la incidencia de las complicaciones. CONCLUSION: La retirada de la barra de Nuss es un procedimiento seguro, habitualmente con escasas complicaciones, aunque en ocasiones pueden ser graves. Para evitarlas, es importante contar con un protocolo que garantice la seguridad.

The relationship between mitral valve prolapse and thoracic skeletal abnormalities in clinical practice: a systematic review.

BACKGROUND: Literature data suggest high inter-study variability in mitral valve prolapse (MVP) prevalence among individuals with thoracic skeletal abnormalities (TSA). This systematic review aimed at estimating the overall prevalence of MVP in individuals with the most common TSA, including not only the oldest studies (before the year 2000) but also the most recent ones (after the year 2000). METHODS: PubMed and EMBASE databases were systematically reviewed in November 2023. Studies assessing the relationship between MVP and TSA and estimating the MVP prevalence in pectus excavatum (PE), pectus carinatum (PC), scoliosis, straight back syndrome (SBS) and Marfan syndrome (MS) were included. There was no limitation on time periods. RESULTS: Twenty-five studies with a total of 2800 patients (27.9 ±â€Š13.9 years, 48.2% females) were analyzed. The highest prevalence of MVP was observed among MS patients (47.3%), while the lowest was detected in PC individuals (23%). Prevalence of MVP was similar among PE (30.8%), scoliosis (26.3%) and SBS (25.5%) patients. When dividing the studies on the basis of temporal period, the average MVP prevalence was approximately two-fold higher in all studies conducted before the year 2000 in comparison with the most recent ones, regardless of TSA type. This discrepancy might be primarily ascribed to relevant differences in the echocardiographic criteria employed for MVP diagnosis before (less specific) and after (more specific) the year 2000, respectively. CONCLUSIONS: The estimated MVP prevalence in TSA individuals is significantly higher than that observed in the general population. Individuals with TSA should be screened for MVP presence on transthoracic echocardiography.

Progressive Keratoconus in a Patient With Severe Pectus Excavatum and a Cartilage Oligomeric Matrix Protein Gene Mutation: A Case Report.

INTRODUCTION: Keratoconus is a progressive ocular disorder associated with numerous systemic diseases, many of which affect the musculoskeletal system. Although the etiology and pathophysiology of the disorder remain elusive, recent studies suggest a significant role of genetic predisposition in the pathogenesis of keratoconus. This case report aims to elucidate a potential genetic association in a patient presenting with keratoconus, severe pectus excavatum, generalized muscular weakness, and skeletal deformities. CASE DESCRIPTION: A 31-year-old Iranian man presented with progressively diminishing vision in both eyes over the years, eventually diagnosed with keratoconus. The patient's history and further examination indicated generalized muscular weakness, skeletal deformities, and severe pectus excavatum with cardiac and large vessel displacement. Whole-exome sequencing identified two heterozygous gene variants: one in the Cartilage Oligomeric Matrix Protein (COMP) gene and another in the Regulating Synaptic Membrane Exocytosis 1 gene. The patient's systemic and ocular symptoms, combined with the gene variants identified, suggested a connective tissue systemic disorder, potentially within the clinical spectrum of COMPopathies. CONCLUSION: This is the first documented case of bilateral progressive keratoconus associated with severe pectus excavatum, generalized musculoskeletal dystrophy, and a COMP gene mutation. It highlights the necessity of continued search into the pathogenic genes of keratoconus, particularly in cases with coexisting systemic manifestations, to further our understanding of the etiology and pathogenesis of this complex disease.

The electrocardiographic manifestations of pectus excavatum before and after surgical correction.

BACKGROUND: Pectus excavatum (PEx) can cause cardiopulmonary limitations due to cardiac compression and displacement. There is limited data on electrocardiogram (ECG) alterations before and after PEx surgical repair, and ECG findings suggesting cardiopulmonary limitations have not been reported. The aim of this study is to explore ECG manifestations of PEx before and after surgery including associations with exercise capacity. METHODS: A retrospective review of PEx patients who underwent primary repair was performed. ECGs before and after surgical correction were evaluated and the associations between preoperative ECG abnormalities and cardiopulmonary function were investigated. RESULTS: In total, 310 patients were included (mean age 35.1 ± 11.6 years). Preoperative ECG findings included a predominant negative P wave morphology in V1, and this abnormal pattern significantly decreased from 86.9% to 57.4% (p < 0.001) postoperatively. The presence of abnormal P wave amplitude in lead II (>2.5 mm) significantly decreased from 7.1% to 1.6% postoperatively (p < 0.001). Right bundle branch block (RBBB) (9.4% versus 3.9%, p < 0.001), rsr' patterns (40.6% versus 12.9%, p < 0.001), and T wave inversion in leads V1-V3 (62.3% vs 37.7%, p < 0.001) were observed less frequently after surgery. Preoperative presence of RBBB (OR = 4.8; 95%CI 1.1-21.6) and T wave inversion in leads V1-3 (OR = 2.3; 95%CI 1.3-4.2) were associated with abnormal results in cardiopulmonary exercise testings. CONCLUSION: Electrocardiographic abnormalities in PEx are frequent and can revert to normal following surgery. Preoperative RBBB and T wave inversion in leads V1-3 suggested a reduction in exercise capacity, serving as a marker for the need for further cardiovascular evaluation of these patients.

Cardiac anomalies in pediatric patients with pectus excavatum.

OBJECTIVE: Pectus excavatum is the most prevalently encountered deformity of the thoracic wall. It can be accompanied by congenital anomalies. METHODS: The cardiac findings of 36 children who were diagnosed at the Thoracic surgery outpatient clinic of our university between 10 February 2021 and 1 October 2021 and 57 healthy children in a similar age group were analyzed. RESULTS: We determined that the pectus excavatum patients in our study had a higher risk of having mitral insufficiency, mitral valve prolapse, tricuspid valve prolapse, cardiac malposition, and congenital heart disease. CONCLUSION: Our study showed that the prevalence of cardiac pathologies was higher in pediatric pectus excavatum patients than in healthy children in the control group. Thus, we recommend clinicians to refer pediatric pectus excavatum patients to pediatric cardiology outpatient clinics for the early diagnosis of potential cardiac pathologies.

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.

Impact of Cryoanalgesia Use During Minimally Invasive Pectus Excavatum Repair on Hospital Days and Total Hospital Costs Among Pediatric Patients.

INTRODUCTION: Surgical repair of pectus excavatum is a painful procedure requiring multimodal pain control with historically prolonged hospital stay. This study aimed to evaluate the impact of cryoanalgesia during minimally invasive repair of pectus excavatum (MIRPE) on hospital days (HDs), total hospital costs (HCs), and complications. We hypothesized that cryoanalgesia would be associated with reduced HDs and total HCs with no increase in post-operative complications. METHODS: We conducted a retrospective review of pediatric patients who underwent MIRPE from 2011 to 2021. MIRPE details and post-operative outcomes within 90 days were abstracted. Total HDs included the index MIRPE admission and readmissions within 90 days. HCs were obtained from the hospital accounting system, retroactively adjusting for medical inflation. Bayesian generalized linear models with neutral prior assuming no effect were used. Differences between treatment groups were assessed using gamma distribution (HDs and HCs) and poisson (post-operative complications). All models used log link and controlled for age, gender, race, and Haller index. RESULTS: Forty-four patients underwent MIRPE during the study period. Cryoanalgesia was utilized in 29 (66%) patients. The probability of a reduction with cryoanalgesia vs. no cryoanalgesia was 99% for HDs (3.0 vs. 5.4 days; Bayesian RR: 0.6, 95% CrI: 0.5-0.8), 89% for HCs ($18,787 vs. $19,667; RR: 0.9, 95% CrI: 0.8-1.1), and 70% for postoperative complications (17% vs 33%; RR: 0.8, 95% CrI: 0.3-1.9). CONCLUSION: Cryoanalgesia use in MIRPE likely reduced HDs, HCs, and post-operative complications. Further research is warranted to confirm these findings in large prospective studies. LEVEL OF EVIDENCE: Level III.

Implementation of a Cryoablation-based Pain Management Protocol for Pectus Excavatum.

INTRODUCTION: The Nuss repair for pectus excavatum is associated with significant postoperative pain. Our institution developed protocols to standardize pain management for pectus excavatum patients in the immediate postoperative period. We present our experience with protocol implementation and patient outcomes. METHODS: We standardized regional anesthesia with a 0.25% bupivacaine incisional soaker catheter (post-implementation 1, PI1) before transitioning to intercostal nerve cryoablation (INC) (post-implementation 2, PI2). Patient outcomes were tracked using statistical process control charts in AdaptX™ OR Advisor and run charts in Tableau. Chi-squared tests assessed demographic differences between cohorts. RESULTS: 244 patients were included: 78 pre-implementation, 108 PI1, and 58 PI2. Average age was 15.9-16.5 years. Patients were majority male, non-Hispanic white, and English speaking. Hospital length of stay decreased 4.1-2.4 days. INC increased surgery time (99-125 min) but decreased PACU time (112-78 min). Maximum pain scores improved in PACU (7.7-6.0) and 0-24 h postoperatively (8.3-6.8) but were not different 24-48 h postoperatively (5.4-5.8). Average opioid dosing decreased 0-48 h from 1.9 to 0.8 mg/kg morphine milliequivalents and was associated with reduction in post-operative nausea and constipation. There were no 30-day readmissions. CONCLUSION: An institution-wide pain management protocol using INC for pectus excavatum patients was implemented. Intercostal nerve cryoablation was found to be superior to bupivacaine incisional soaker catheters and reduced hospital length of stay, immediate postoperative pain scores, morphine milliequivalent opioid dosing, postoperative nausea, and constipation. LEVEL OF EVIDENCE: Level IV.

Refractory Hypotension During Implantation of a 70 mL Total Artificial Heart in a Patient With Pectus Excavatum: A Case Report.

The Syncardia total artificial heart system is the only commercially approved durable device for treating biventricular heart failure patients awaiting heart transplantation. Conventionally, the Syncardia total artificial heart system is implanted based on the distance from the anterior aspect of the 10th thoracic vertebra to the sternum and the patient's body surface area. However, this criterion does not account for chest wall musculoskeletal deformities. This case report describes a patient with a pectus excavatum who developed compression of the inferior vena cava after Syncardia total artificial heart implantation and how transesophageal echocardiography guided chest wall surgery to accommodate the total artificial heart system.

Simultaneous minimally invasive LVAD implantation and extracorporeal RVAD placement by patient with pectus excavatum and biventricular acute heart failure.

The optimal surgical approach in patients with pectus excavatum (PEx) who need cardiac surgery remains uncertain. The challenge is even greater, if it is already foreseeable that the patient will be needed further procedure in the next future. We describe a novel sternotomy-sparing approach for minimal-invasive biventricular assist device (BiVAD) implantation in a patient with an acute heart failure (HF) due to dilated cardiomyopathy and severe PEx. Moreover, alternative approaches for ventricular assist device (VAD) implantation and timing of the repair of PEx will be discussed.

Retrospective study comparing outcomes of multimodal epidural and erector spinae catheter pain protocols after pectus surgery.

INTRODUCTION: There are no optimal postoperative analgesia regimens for Nuss procedures. We compared the effectiveness of thoracic epidurals (EPI) and novel ambulatory erector spinae plane (ESP) catheters as part of multimodal pain protocols after Nuss surgery. METHODS: Data on demographics, comorbidities, perioperative details, length of stay (LOS), in hospital and post discharge pain/opioid use, side effects, and emergency department (ED) visits were collected retrospectively in children who underwent Nuss repair with EPI (N = 114) and ESP protocols (N = 97). Association of the group with length of stay (LOS), in hospital opioid use (intravenous morphine equivalents (MEq)/kg over postoperative day (POD) 0-2), and oral opioid use beyond POD7 was analyzed using inverse probability of treatment weighting (IPTW) with propensity scores, followed by multivariable regression. RESULTS: Groups had similar demographics. Compared to EPI, ESP had longer block time and higher rate of ketamine and dexmedetomidine use. LOS for ESP was 2 days IQR (2, 2) compared to 3 days IQR (3, 4) for EPI (p < 0.01). Compared to EPI, ESP group had higher opioid use (in MEq/kg) intraoperatively (0.32 (IQR 0.27, 0.36) vs. 0.28 (0.24, 0.32); p < 0.01) but lower opioid use on POD 0 (0.09 (IQR 0.04, 0.17) vs. 0.11 (0.08, 0.17); p = 0.03) and POD2 (0.00 (IQR 0.00, 0.00) vs. 0.04 (0.00, 0.06) ; p < 0.01). ESP group also had lower total in hospital opioid use (0.57 (IQR 0.42, 0.73) vs.0.82 (0.71, 0.91); p < 0.01), and shorter duration of post discharge opioid use (6 days (IQR 5,8) vs. 9 days (IQR 7,12) (p < 0.01). After IPTW adjustment, ESP continued to be associated with shorter LOS (difference -1.20, 95% CI: -1.38, -1.01, p < 0.01) and decreased odds for opioid use beyond POD7 (OR 0.11, 95% CI: 0.05, 0.24); p < 0.01). However, total in hospital opioid use in MEq/kg (POD0-2) was now similar between groups (difference -0.02 (95% CI: -0.09, -0.04); p = 0.50). The EPI group had higher incidence of emesis (29% v 4%, p < 0.01), while ESP had higher catheter malfunction rates (23% v 0%; p < 0.01) but both groups had comparable ED visits/readmissions. DISCUSSION/CONCLUSION: Compared to EPI, multimodal ambulatory ESP protocol decreased LOS and postoperative opioid use, with comparable ED visits/readmissions. Disadvantages included higher postoperative pain scores, longer block times and higher catheter leakage/malfunction. LEVELS OF EVIDENCE: Level III.

Left thoracotomy approach for aortic root surgery.

Marfan syndrome is an autosomal dominant disease of connective tissue affecting approximately one in 5 000 people. However, the majority of patients (60-80%) with Marfan syndrome have some degree of aortic root dilation or thoracic aortic aneurysm that can lead to aortic dissection and rupture, which has long been recognised as the leading cause of death in Marfan syndrome. The funnel breast (pectus excavatum), often seen in patients with Marfan syndrome, radically complicates the access during aortic root or arch interventions, forcing cardiosurgery practitioners to seek alternative approaches. We present a series of clinical observations of two patients with Marfan syndrome and aneurysm of the aortic root and ascending aorta. A left-sided thoracotomy provides the possibility of successful implementation of extensive surgical interventions, decreasing the risks of intra-operative damage to the cardiac structures adjacent to the sternum in this complex cohort of patients.