Effect of cryoablation in Nuss bar placement on opioid utilization and length of stay.

BACKGROUND: Our institution recently transitioned from paravertebral nerve blocks (PVBs) to intercostal nerve cryoablation (INC) for pain control following minimally invasive repair of pectus excavatum (MIRPE). This study aimed to determine how INC affected the operative time, length of stay, complication rates, inpatient opioid use, and outpatient prescription of opioids at a single center. METHODS: A retrospective review was performed at a single pediatric referral center of all patients who underwent MIRPE between 2018 and 2023. Patient demographics, operative details, and perioperative course were collected. The use of INC versus PVB was recorded. Univariate analyses were performed using Wilcoxon rank sum tests for continuous variables and chi-squared tests for categorical variables. RESULTS: 255 patients were included with a median age of 15 years, median BMI of 18.50 kg/m2, and median Haller index of 4.40. INC was utilized in 41% (105/255), and 59% (150/255) received PVB. The two groups did not differ significantly in BMI, Haller index, or complications, though the INC patients were older by 1 year (15.0 vs. 16.0, p = 0.034). INC was associated with an increased operative time (INC: 92 min vs. PVB: 67 min, p < 0.001), decreased length of stay (3 vs. 4 days, p = < 0.001), more than twofold decrease in inpatient opioids per day (INC: 16 MME vs. PVB: 41 MME, p < 0.001), and a fourfold decrease in the amount of opioids prescribed at discharge (INC: 90 MME vs. PVB: 390 MME, p < 0.001). CONCLUSION: INC after MIRPE significantly decreased both the inpatient opioid utilization and our outpatient prescribing practices while also decreasing our overall length of stay without increasing complications. LEVEL OF EVIDENCE: Level III.

Erector Spinae Plane Block Provided Comparable Analgesia as Thoracic Paravertebral Block Post Pediatric Nuss Procedure for Pectus Excavatum: A Randomized Controlled Trial.

BACKGROUND: Thoracic paravertebral block (TPVB) is frequently used to treat pain following a pediatric Nuss procedure but is associated with various undesirable risks. The erector spinae plane block (ESPB) also provides postoperative analgesia, which is purported to be easier to administer and has a favorable safety profile. However, it remains unknown whether ESPB provides analgesia comparable to the TPVB technique post  pediatric Nuss procedure. OBJECTIVE: This study aimed to compare the analgesic effects of ultrasound-guided ESPB and TPVB in children undergoing the Nuss procedure. STUDY DESIGN: A prospective, randomized, noninferiority trial. SETTING: A university hospital in the People's Republic of China. METHODS: A total of 68 children aged 4 to 18 scheduled for the Nuss procedure were enrolled in the study. They were randomly assigned to receive a single-injection ultrasound-guided bilateral T5-level ESPB or TPVB with 0.5 mL/kg of 0.25% ropivacaine post anesthesia induction. All patients received postprocedure multimodal analgesia. The primary outcomes were pain scores at rest and 24 hours postprocedure. The secondary outcomes included total rescue morphine milligram equivalents, emergence agitation, chronic postprocedure pain, and side effects. RESULTS: The median difference in pain scores at rest 24 hours postprocedure  was 0 (95% CI, 0 to 1), demonstrating the noninferiority of ESPB to TPVB. In addition, the difference in oral morphine milligram equivalents at 24 hours postprocedure was -4.9 (95% CI, -16.7 to 7.9) with the ESPB group consuming median (interquartile range) 37.7 mg (12-53.2) vs 36.9 mg (23.9-58.1) for the TPVB group. We concluded that the non-inferiority of ESPB with regard to opioid consumption as the 95% CI upper limit of 7.9, which was within the predefined margin of 10. We found no significant differences in pain scores at rest or during coughing, incidences of chronic postoperative pain, emergence agitation, or side effects. LIMITATIONS: We did not evaluate the effect of analgesic protocols on patient-centric outcomes, such as resuming functional status and emotional wellbeing. Also, the sample size is small to some extent. CONCLUSIONS: Preoperative ESPB, when combined with multimodal analgesia, was noninferior in analgesic effect compared with TPVB in terms of pain scores and opioid consumption in pediatric patients undergoing the Nuss procedure.

Severe stress cardiomyopathy following spinal corrective surgery for scoliosis complicated with pectus excavatum: a case report.

BACKGROUND: Stress cardiomyopathy (SCM) is an acute heart failure syndrome characterized by transient, usually reversible left ventricular systolic dysfunction with normal or enhanced basal compensatory wall motion abnormalities involving the left ventricular anterior septum and apex, resulting in a "ballooning" appearance. However, it has rarely been reported in patients undergoing spinal surgery. CASE PRESENTATION: We report a case of severe stress cardiomyopathy in a scoliosis patient with pectus excavatum who underwent spinal corrective surgery. During the wake-up period, circulatory collapse occurred. After multidisciplinary consultation, the patient was diagnosed with stress cardiomyopathy. At last, she had a good prognosis after a series of treatments including ECMO. CONCLUSION: Stress cardiomyopathy is a reversible but uncommon condition. It can cause death if it is not diagnosed in time. Consequently, this report should improve the awareness of orthopedists and anesthesiologists for timely identification and management. For patients with potential risk factors, timely preoperative intervention should be performed to reduce the occurrence of stress cardiomyopathy.

The role of cytokines in acute and chronic postsurgical pain after major musculoskeletal surgeries in a quaternary pediatric center.

STUDY OBJECTIVE: To determine if baseline cytokines/chemokines and their changes over postoperative days 0-2 (POD0-2) predict acute and chronic postsurgical pain (CPSP) after major surgery. DESIGN: Prospective, observational, longitudinal nested study. SETTING: University-affiliated quaternary children's hospital. PATIENTS: Subjects (≥8 years old) with idiopathic scoliosis undergoing spine fusion or pectus excavatum undergoing Nuss procedure. MEASUREMENTS: Demographics, surgical, psychosocial measures, pain scores, and opioid use over POD0-2 were collected. Cytokine concentrations were analyzed in serial blood samples collected before and up to two weeks after surgery, using Luminex bead arrays. After data preparation, relationships between pre- and post-surgical cytokine concentrations with acute (% time in moderate-severe pain over POD0-2) and chronic (pain score > 3/10 beyond 3 months post-surgery) post-surgical pain were analyzed using univariable and multivariable regression analyses with adjustment for covariates and mixed effects models were used to associate longitudinal cytokine concentrations with pain outcomes. MAIN RESULTS: Analyses included 3,164 repeated measures of 16 cytokines/chemokines from 112 subjects (median age 15.3, IQR 13.5-17.0, 54.5 % female, 59.8 % pectus). Acute postsurgical pain was associated with higher baseline concentrations of GM-CSF (ß = 0.95, SE 0.31; p = 0.003), IL-1ß (ß = 0.84, SE 0.36; p = 0.02), IL-2 (ß = 0.78, SE 0.34; p = 0.03), and IL-12 p70 (ß = 0.88, SE 0.40; p = 0.03) and longitudinal postoperative elevations in GM-CSF (ß = 1.38, SE 0.57; p = 0.03), IFNγ (ß = 1.36, SE 0.6; p = 0.03), IL-1ß (ß = 1.25, SE 0.59; p = 0.03), IL-7 (ß = 1.65, SE 0.7; p = 0.02), and IL-12 p70 (ß = 1.17, SE 0.58; p = 0.04). In contrast, CPSP was associated with lower baseline concentration of IL-8 (ß = -0.39, SE 0.17; p = 0.02), and the risk of developing CPSP was elevated in patients with lower longitudinal postoperative concentrations of IL-6 (ß = -0.57, SE 0.26; p = 0.03), IL-8 (ß = -0.68, SE 0.24; p = 0.006), and IL-13 (ß = -0.48, SE 0.22; p = 0.03). Covariates female (vs. male) sex and surgery type (pectus surgery vs. spine) were associated with higher odds for CPSP in baseline adjusted cytokine-CPSP association models for IL-2, IL-4, IL-5, IL-6, IL-8, IL-10, TNFα, and IL-8, IL-10, respectively. CONCLUSION: We identified pro-inflammatory cytokine profiles associated with higher risk of acute postoperative pain. Interestingly, pleiotropic cytokine IL-6, chemokine IL-8 (which promotes neutrophil infiltration and monocyte differentiation), and monocyte-released anti-inflammatory cytokine IL-13, were associated with lower CPSP risk. Our results suggest heterogenous outcomes of cytokine/chemokine signaling that can both promote and protect against post-surgical pain. These may serve as predictive and prognostic biomarkers of pain outcomes following surgery.

Single-stage Open Lobectomy and Modified Ravitch Procedure in an Infant with Coexisting Severe Pectus Excavatum and Congenital Cystic Adenomatous Malformation.

ABSTRACT: Coexisting congenital cystic adenomatous malformation of the lungs and severe pectus excavatum (PE) is an uncommon presentation that poses significant management challenges. Conventionally managed in a staged manner, there are increasing reports of superior outcomes with single-staged concurrent repair with minimally invasive techniques (video-assisted thoracoscopic surgery [VATS] and minimally invasive repair of PE [MIRPE]). The outcome of a single-stage open repair for both anomalies has not been previously reported to the best of our search. We report the successful single-stage management of a 9-month-old infant with both anomalies who had an open lobectomy and modified Ravitch procedure. We aim to report the feasibility and safety of a single-stage concurrent repair of both conditions using open techniques, as VATS and MIRPE are not readily available in our environment.

Minimally invasive mitral valve repair in a patient with severe pectus excavatum: a case report.

Pectus excavatum presents challenges in cardiac operations due to anatomical abnormalities and limited exposure. We report a case of a 58-year-old male with severe pectus excavatum and significant mitral regurgitation successfully treated with minimally invasive mitral valve repair. Preoperative imaging revealed leftward heart displacement and a Haller index of 3.8. Surgical intervention involved adaptations in atrial retraction and valve repair technique. Postoperative outcomes were favourable, with normal mitral function and a short hospital stay. Despite technical challenges, minimally invasive approaches offer benefits in pectus excavatum patients. This case underscores the feasibility and safety of minimally invasive surgery in patients with extensive pectus excavatum, emphasizing its potential as a preferred approach.

Effect of medial stabilizer chest position on pectus bar dislocation.

PURPOSE: The current standard method for pectus excavatum (PE) repair is the Nuss procedure. One major postoperative complication is the displacement of the implanted metal bar, which is used to remodel the chest wall. Blocking the possible ways that the bar can be displaced with the use of stabilizers and peri/intracostal sutures has reduced the incidence of bar displacement. Despite the modifications, bar dislocation is often reported. We adopted the medial position stabilizer placement method and imposed no postoperative restrictions. In this study, we analyzed the bar dislocation rate with this modification and concurrent postoperative full activity. METHODS: Nuss procedure modification where stabilizers are placed bilaterally in the medial location was done on patients irrespective of age and Haller index greater than 3.25. A single bar was used for all patients. Cryoanalgesia was performed on every patient. No postoperative restrictions were imposed on the patients. Full immediate activities, including sports, were allowed. RESULTS: 114 patients (103 male, 11 female) were analyzed from 2016 to 2023. The median age was 15 years old. There was zero incidence of bar displacement. The combined incidence of other postoperative complications was 4%: 2 wound infections and 2 hematoma formations, both needing incision and drainage. CONCLUSION: Bilateral medial stabilizer placement resulted in no incidence of bar dislocation. Return to immediate full activities after the Nuss procedure did not appear to increase the incidence of bar displacement if stabilizers were placed medially.

The pectus care guidelines: best practice consensus guidelines from the joint specialist societies SCTS/MF/CWIG/BOA/BAPS for the treatment of patients with pectus abnormalities.

Pectus defects are a group of congenital conditions found in approximately 1 in 250 people, where the sternum is depressed back towards the spine (excavatum), protrudes forwards (carinatum) or more rarely is a mixture of both (arcuatum or mixed defects). For the majority of patients, it is well tolerated, but some patients are affected psychologically, physiologically or both. The deformity becomes apparent at a young age due to the growth of the ribs and the cartilage that links them to the sternum. The majority of defects are mild and are well tolerated, i.e. they do not affect activity and do not cause psychological harm. However, some young people develop lower self-esteem and depression, causing them to withdraw from activities (such as swimming, dancing) and from interactions that might 'expose' them (such as sleepovers, dating, going to the beach and wearing fashionable clothes). This psychological harm occurs at a crucial time during their physical and social development. A small number of patients have more extreme depression of their sternum that impedes their physiological reserve, which can occur when engaging in strenuous exercise (such as running) but can also limit moderate activity such as walking and climbing stairs. The effects can be so extreme that symptoms occur at rest or cause life-threatening compression of the major blood vessels and organs. The group of patients with physiological impairment usually also suffer from low self-esteem and depression. This paper summarizes the current evidence for the different treatment strategies for this condition, including supportive care, psychological support and non-surgical techniques including bracing and vacuum bell therapy. We also consider surgical techniques including the Ravitch procedure, the Nuss procedure (minimally invasive repair of pectus excavatum), pectus implants and other rare procedures such as Pectus Up. For the majority of patients, supportive care is sufficient, but for a minority, a combination of the other techniques may be considered. This paper also outlines best practice guidance for the delivery of such therapies, including standardized assessment, consent to treatment, audit, quality assurance and long-term support. All the interventions have risks and benefits that the patient, parents and clinicians need to carefully consider and discuss when deciding on the most appropriate course. We hope this evidence review of 'Best Practice for Pectus' will make a significant contribution to those considerations and help all involved, from patients to national policy makers, to deliver the best possible care.

Cardiac Perforation Caused By Pectus Excavatum Metal Bar Stabilizer: A Case Report.

This paper reports the case of a female patient who underwent minimally invasive repair of pectus excavatum (MIRPE) in another service that evolved with bar rotation and cardiac perforation caused by the left stabilizer. The unique and frightening aspect of the case is that despite having the stabilizer inside the ventricle, the patient was oligosymptomatic: occasional chest pain and respiratory discomfort. Preoperative imaging showed rotation of the bar with stabilizers within the thoracic cavity. During surgery, intense ossification was observed around the prosthesis and it was noted that the left stabilizer had perforated the patient's left ventricle. Cardiac repair required a Clamshell incision and cardiopulmonary bypass. This case reinforces the validity of late radiological follow-up after MIRPE in an attempt to avoid this type of event, and the need to reevaluate the use of stabilizers perpendicular to the bar since they are not safe to prevent rotation of these implants.

Three-dimensional modelling and printing in anterior chest wall reconstruction and stabilisation.

Anterior chest wall instability as a result of sternocostal non-union is a rare complication but can give rise to invalidating pain and cardiac arrhythmias. A woman in her 40s was referred to us with anterior chest wall pain and instability after a modified Ravitch procedure. Sternocostal pseudoarthrosis was seen for which multiple operations were performed which were complicated by low-grade infections. A patient-specific three-dimensional modelled and printed prostheses was used in an operation to both lift the sternum for pectus correction and to reconnect the sternum and the sternal costal junction to regain anterior chest wall stability.

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.

Thoracic and vertebral deformities in lung transplantation: perioperative complications and long-term prognoses.

BACKGROUND: Lung transplantation (LTx) is a crucial therapeutic strategy for patients suffering from end-stage respiratory diseases, necessitating precise donor-recipient size matching to ensure optimal graft function. While standard allocation protocols rely on predicted lung capacity based on factors such as sex, age, and height, a subset of patients with respiratory diseases presents an additional challenge - thoracic or vertebral deformities. These deformities can complicate accurate volume predictions and may impact the success of lung transplantation. METHODS: In this retrospective cohort study of patients who underwent LTx at Tohoku University Hospital between January 2007 and April 2022, with follow-up until October 2022, the primary objective was to assess the influence of thoracic and vertebral deformities on perioperative complications, emphasizing interventions, such as volume reduction surgery. The secondary objective aimed to identify any noticeable impact on long-term prognoses in recipients with these deformities. RESULTS: Of 129 LTx recipients analyzed, 17.8% exhibited thoracic deformities, characterized by pectus excavatum, while 16.3% had vertebral deformities. Perioperative complications, requiring delayed chest closure, tracheostomy, and volume reduction surgery, were more prevalent in the deformity group. Thoracic deformities were notably associated with the need for volume reduction surgery. However, long-term prognoses did not differ significantly between patients with deformities and those without. Vertebral deformities did not appear to significantly impact perioperative or long-term outcomes. CONCLUSIONS: This study highlights the prevalence of thoracic deformities in LTx recipients, correlating with increased perioperative complications, particularly the potential need for volume reduction surgery. Importantly, these deformities do not exert a significant impact on long-term prognoses. Additionally, patients with vertebral deformities, such as scoliosis and kyphosis, appear to be manageable in the context of LTx.

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.

Experiences of living with funnel chest prior to corrective surgery: An interview study.

OBJECTIVES: Pectus excavatum, or funnel chest, causes both physical and psychosocial issues, affecting health-related quality of life. However, the literature on how funnel chest affects daily living prior to corrective surgery is sparse. Therefore, the study aimed to describe the experiences of living with funnel chest prior to correctional surgery. MATERIALS AND METHODS: The study had a qualitative exploratory design. Consecutive sampling was applied in which all individuals from a single cardiothoracic department scheduled for the minimally invasive repair of pectus excavatum were asked to participate. Nineteen participants, 17 men and two women, participated in the study. Individual telephone interviews were conducted from February 2020 until April 2021. The interviews were analyzed with qualitative content analysis using an inductive approach. RESULTS: The overall theme "To have or not to have a cavity in my chest, it could make a difference" was interpreted as the latent meaning of the participants' experiences. The theme included two subthemes with three categories each. The subtheme "The funnel chest puts a weight on my shoulders" describes the heavy burden the funnel chest places on the participants. The second subtheme, "This is me, but I want to change my future", describes that participants see the funnel chest as a part of themselves; nevertheless, they look forward to surgery and a life without it. CONCLUSION: The results emphasize the heavy burden funnel chest causes and the great limitations it places on the individual. It also highlights the importance of surgery and the hope for a better future for individuals with funnel chest.

Quantitative CT lung volumetry and densitometry in pediatric pectus excavatum.

The purpose of this study was to evaluate the quantitative computed tomography (CT) volumetry and densitometry and in pediatric patients with pectus excavatum (PE). We measured pectus index (PI) and separated inspiratory and expiratory lung volumes and densities. We obtained the total lung volume (TLV) and mean lung density (MLD) during inspiration and expiration, and the ratio of end expiratory to inspiratory volume (E/I volume) and MLD (E/I density) were calculated. The difference between inspiratory and end expiratory volume (I-E volume) and MLD (I-E density) were also calculated. A total of 199 patients, including 164 PE patients and 35 controls, were included in this study. The result shows that the PE group had lower inspiratory TLV (mean, 2670.76±1364.22 ml) than the control group (3219.57±1313.87 ml; p = 0.027). In the PE group, the inspiratory (-787.21±52.27 HU vs. -804.94±63.3 HU) and expiratory MLD (-704.51±55.41 HU vs. -675.83±64.62 HU) were significantly lower than the indices obtained from the control group (p = 0.006). In addition, significantly lower values of TLV and MLD difference and higher value of TLV and MLD ratio were found in the PE group (p <0.0001). PE patients were divided into severe vs. mild groups based on the PI cutoff value of 3.5. The inspiratory MLD and TLV ratio in the severe PE group were lower than those in the mild PE group, respectively (p <0.05). In conclusion, quantitative pulmonary evaluation through CT in pediatric PE patients may provide further information in assessing the functional changes in lung parenchyma as a result of chest wall deformity.

Enhanced Recovery After Surgery 2.0: Optimizing Pain Management in Nuss Procedure: Cryoablation and Nerve Block Strategies for Reduced Opioid Use.

INTRODUCTION: Our study assesses the association between cryoablation, with and without nerve block supplementation, post-Nuss procedure pain, and opioid use in pectus excavatum (PE) patients. METHODS: We conducted a retrospective cohort study at a single center for PE patients who underwent the Nuss procedure from 2017 to 2022. Outcomes included postoperative opioid use (measured in oral morphine milligram equivalent per kilogram [OME/kg]), average pain score (scale 0-10), and length of stay (LOS). RESULTS: One hundred sixty-four patients (146 males and 18 females) were included, with 79 (48.2%) receiving neither cryoablation nor nerve block, 60 (36.6%) receiving intraoperative cryoablation alone, and 25 (15.2%) receiving both cryoablation and nerve block. The median age was 16 y. Nerve block recipients consumed fewer opioids during hospitalization than cryoablation alone and nonintervention groups (1.5 versus 2.3 versus 5.8 OME/kg, respectively, P < 0.0001). Average pain scores over the total LOS were lower in nerve block recipients (3.5 versus 3.8 versus 4.2, P = 0.03), particularly on postoperative day 0 (P = 0.002). Nerve block recipients had a shorter LOS than cryoablation alone and nonintervention groups (43.4 versus 54.7 versus 66.2 h, P < 0.0001). On multivariate analysis, cryoablation alone resulted in significantly less opioid use compared to no intervention (3.32 OME/kg reduction, 95% confidence interval -4.16 to -2.47, P < 0.0001). Addition of nerve block further reduced opioid use by 1.10 OME/kg (95% confidence interval -2.07 to -0.14, P = 0.04). CONCLUSIONS: Cryoablation with nerve block supplementation is associated with reduced pain, opioid use, and LOS post-Nuss for PE repair compared to cases without cryoablation or with cryoablation only. Cryoablation with regional nerve blocks should be considered for Nuss repair under the enhanced recovery after surgery pathway.

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.

Ultrasound-guided erector spinae plane block versus thoracic epidural block for postoperative analgesia in pediatric Nuss surgery: a randomized noninferiority trial.

PURPOSE: Thoracic epidural anesthesia (TEA) is often used for analgesia after thoracic surgery. Erector spinae plane block (ESPB) has been proposed to provide adequate analgesia. We hypothesized that ESPB would be noninferior to TEA as a part of multimodal analgesia in pediatric patients undergoing the Nuss procedure. METHODS: Patients aged 7-18 years and scheduled for the Nuss procedure were randomly allocated to receive bilateral single-shot ESPB or TEA and a multimodal analgesic regimen including parent-controlled intravenous analgesia (PCIA). At 6 h, 12 h, 18 h, and 24 h postoperatively, pain was evaluated using the numeric rating scale (NRS) and opioid consumption was assessed by counting the number of PCIA boluses. The joint primary outcomes were the average pain score and opioid consumption at 24 h after surgery. The secondary outcomes were the NRS scores and the number of opioid boluses administered at different postoperative time points, adverse events, and recovery quality. RESULTS: Three hundred patients underwent randomization, and 286 received ESPB (147 patients) or TEA (139 patients). At 24 h postoperatively, ESPB was noninferior to TEA in terms of the average NRS score (mean difference, - 0.1, 95% confidence interval [CI], - 0.3-0.1, margin = 1, P for noninferiority < 0.001) and the number of opioid boluses administered (mean difference, - 1.1, 95% CI, - 2.8-0.6, margin = 7, P for noninferiority < 0.001). Adverse events and patient recovery were comparable between groups. CONCLUSIONS: The results demonstrate that combined with a multimodal analgesia, ESPB provides noninferior analgesia compared to TEA with respect to pain score and opioid consumption among pediatric patients undergoing the Nuss procedure.