Sympathicotomy for isolated facial blushing: long-term follow-up of a randomized trial.

OBJECTIVES: Thoracoscopic sympathicotomy may be an effective treatment for disabling facial blushing in selected patients. Short- and mid-term results are good but very long-term results are scarce in the medical literature and there is no knowledge which extent of sympathicotomy is better long-term for isolated facial blushing. METHODS: We previously randomized 100 patients between a rib-oriented R2 or R2-R3 sympathicotomy for isolated facial blushing, and reported local effects, side effects and quality of life after 12 months. In the present study, we sent identical questionnaires to all patients after a median of 16 years (interquartile range 15-17 years). RESULTS: The response rate was 66%. Overall, 82% reported excellent or satisfactory results on facial blushing, with significant better local effect after R2 sympathicotomy compared with R2-R3 sympathicotomy. Patients who underwent R2 sympathicotomy were also significantly more satisfied with the operation. We found no significant difference between R2 and R2-R3 sympathicotomy in quality of life or rates of compensatory sweating (77%) and recurrence of blushing (41%) which was milder than preoperatively in most patients. CONCLUSIONS: R2 sympathicotomy should be the preferred approach for isolated facial blushing because of better local effect and higher satisfaction rates. Although this was a very long-term follow-up of the only randomized trial of its kind the response rate was limited leaving a risk of undetected bias.

Epidural analgesia and abnormal coagulation in patients undergoing minimal invasive repair of pectus excavatum.

Background: Epidural analgesia (EA) is effective in patients undergoing minimal invasive repair of pectus excavatum (MIRPE) but is associated with major complications such as epidural hematomas. It is recommended to assess coagulation status in patients receiving anticoagulant therapy prior to EA, although no consensus exists in patients without a history of bleeding tendency or anticoagulant therapy. Thus, the aim of this paper was to assess 1) the prevalence of abnormal routine coagulation parameters, i.e., international normalized ratio (INR) and platelet count, and 2) the safety of EA in patients undergoing MIRPE. Methods: In this retrospective study, we identified 1,973 patients undergoing MIRPE at our center between 2001 and 2019. Complications related to EA were registered for all patients. Information on coagulation parameters was present in 929 patients. Patients with spontaneously elevated INR ≥1.5 were referred for assessment of coagulation factor VII in order to assess the cause of the elevated INR. Results: Of 929 patients with coagulation information available, 18 patients had spontaneously elevated INR ≥1.5 (1.9%). In patients with INR ≥1.5, 12 patients underwent further assessment of factor VII, with all patients having a slightly reduced factor VII close to the lower reference range. The majority of the 1,973 patients undergoing MIRPE received EA (99.6%) with very low complication rates (0.2%) and no incidence of epidural hematomas. Conclusion: In patients undergoing MIRPE, coagulation screening prior to EA should not be mandatory as it revealed no clinically relevant consequences. EA is safe with very low complication rates.

Incidence and severity of surgical complications after pectus excavatum bar removal.

OBJECTIVES: Pectus bar removal is the final step of minimally invasive repair of pectus excavatum. Complication rates related to bar removal have been reported in 2-15% of patients and severe, near-fatal and fatal complications have been reported. No systematic assessment of complication severity or risk factors associated with bar removal has been reported in large study populations. The aim of this paper is to investigate the safety of the bar removal procedure with regard to complication rates and severities as well as assessment of risk factors. METHODS: Between 2003 and 2019, 1574 patients underwent the bar removal procedure. Medical records were assessed retrospectively and complications registered. Complications were categorized in infections, bleedings and other complications. The severity of the surgical complications was systematically classified using the validated Clavien-Dindo classification. Furthermore, risk factors associated with complications were assessed. RESULTS: The overall complication rate was 4.1% (Clavien-Dindo classification I-IV), mainly consisting of bleedings (1.3%) and infections (1.5%). Five cases of severe bleedings were registered (0.3%, Clavien-Dindo classification IV). Risk factors associated with complications during bar removal were greater age and removal of more than one bar. CONCLUSIONS: The bar removal procedure is a safe and effective procedure. Both age and number of bars inserted should be considered prior to surgical correction of pectus excavatum as these factors predict complications related to bar removal.

Prevalence of comorbidities in a surgical pectus excavatum population.

BACKGROUND: Pectus excavatum is the most common chest wall deformity and is associated to various connective tissue, cardiopulmonary, and skeletal abnormalities. Several conditions and syndromes have been associated to pectus excavatum, although the overall health implications of the pectus excavatum phenotype are unclear. Therefore, in this study we aimed to examine the health implications of the pectus excavatum phenotype by assessing all comorbidities and previous medical conditions in a cohort of patients undergoing pectus excavatum surgery. METHODS: This single-centre retrospective prevalence study included 1,046 patients undergoing minimal invasive repair of pectus excavatum from 2001 to 2012. Hospital medical charts were assessed and comorbidities and previous medical conditions were registered systematically and categorized according to the affected organ system. RESULTS: In our study population of 1,046 patients, we registered 623 conditions. The median age was 17 years and the majority of patients (56%) had no previous or present conditions. Notable prevalence of asthma (8.8%), allergies (12.3%), previous hernia surgery (5.2%), and psychiatric conditions (4.9%) were found. CONCLUSIONS: The majority of patients undergoing pectus excavatum surgery have no comorbidities or previous medical conditions. It seems that this patient category is comparable to the background population in this regard and our findings do not support screening this patient category for associated conditions.

Pectus excavatum requiring temporary pacemaker implantation before Nuss procedure: a case report.

Pectus excavatum is the most common chest wall deformity, and some patients also have it combined with cardiac arrhythmias. It is a rare occurrence for there to be a severe conduction block that requires a temporary pacemaker implantation before the surgical correction. Here we reported a case of pectus excavatum with a second-degree atrial-ventricular (AV) block (Mobitz II) who had temporary pacemaker implantation before the Nuss procedure. The young patient had a chest wall deformity for 6 years and it got worse with age. The Haller index was 4.21, and we evaluated that he should receive the Nuss procedure. An AV block was found during the preoperative electrocardiogram examination; furthermore, Holter monitor proved that he had first-degree AV block and a second-degree AV block (Mobitz II). After consultation with the anesthesiologist and cardiologist, we suggested that a temporary pacemaker placement should be performed under local anesthesia before the minimally invasive operation and removed as soon as the patient revived from general anesthesia. A postoperative Holter monitor was implemented, and the conduction defect disappeared shortly after the operation. However, the Holter monitor showed that the conduction defect was still existed during the follow-up period, which indicated that severe conduction defects should be originated from the conduction system itself, rather than the compression to the heart. The temporary pacemaker was essential to ensure the conducting of the operation went smoothly.

Combining Correction of Pectus Excavatum and Open Heart Surgery in a Single-Stage Procedure.

The presence of pectus excavatum in patients undergoing open heart surgery is rare, and no consensus has been reached regarding the surgical approach. Thus, this paper describes a single-stage procedure for correction of pectus excavatum with the modified Nuss procedure ad modum Pilegaard in 4 patients undergoing open heart surgery. Three of 4 patients successfully underwent the single-stage procedure. At follow-up after bar removal, all patients had no complications related to the Nuss bar, and all had excellent cosmetic results.

Cardiovascular magnetic resonance assessment of biventricular changes during vacuum bell correction of pectus excavatum.

BACKGROUND: Evidence of cardiac dysfunction in patients with pectus excavatum (PE) remains controversial. A growing number of studies report increased exercise tolerance following surgery. Nevertheless, many consider the correction of PE a cosmetic intervention, with post-operative changes ascribed to the concurrent growth of the young patient population. No studies have investigated non-invasively the immediate cardiac changes following relief of the deformity. The aim of this study was to assess cardiac function before and during temporary sternal elevation using the non-invasive vacuum bell (VB) device on young adults with PE. METHODS: Adult patients scheduled for surgical correction of PE underwent cardiac magnetic resonance imaging (CMRI) before and during the application of the VB. Steady-state free precession sequences were used for the evaluation of biventricular volume and function. Phase contrast sequences measured the aortic and pulmonary flow to calculate stroke index (SI). Scans were analyzed post hoc by the same investigator. A control group of healthy individuals was assessed in the same way. RESULTS: In total, 20 patients with PE (mean age 23±10 years) and 10 healthy individuals (mean age 25±6 years) underwent CMR before and during VB application. Before intervention, baseline cardiac volumes and function were similar between the groups, with patient-values in the low-to-normal range. Following VB application, PE patients revealed a 10% increase in biventricular SI. Furthermore, left ventricular end-diastolic volume index (LV EDVI) improved by 8% and right ventricular ejection fraction (RV EF) increased by 7%. These findings were not mirrored in the healthy individuals. No correlations were found between improved cardiac parameters and the baseline Haller index (HI) of PE patients. CONCLUSIONS: Non-invasive, momentary correction of PE is associated with an immediate improvement in SI, RV EF and LV EDVI, not observed in controls. The findings suggest that sternal depression in PE patients affects cardiac function.

Single-stage tracheal and cricotracheal segmental resection with end-to-end anastomosis: Outcome, complications, and risk factors.

OBJECTIVE: Management of tracheal stenosis remains controversial. Endoscopic interventions commonly provide immediate relief of symptoms, but are associated with high recurrence rates. In contrast, high success rates have been reported in patients undergoing tracheal resection. However, well-defined indications and contraindications for tracheal resection are lacking and previous studies commonly ignore patient-related outcomes (e.g. dyspnoea). We aimed to evaluate the outcome of tracheal resection at our institution and identify risk factors for complications. METHODS: All adult patients undergoing tracheal resection at Aarhus University Hospital between January 2009 and September 2016 were included RESULTS: Twenty-seven patients were included. The most frequent aetiologies for tracheal stenosis were previous tracheostomy (n=8), prolonged intubation (n=3), a combination of the two (n=5), and intraluminal tumour (n=7). Sixteen patients underwent high tracheal resection, seven patients low tracheal resection, and four patients partial cricotracheal resection. Surgical success (no dyspnoea and no need for additional intervention) was achieved in 74% of patients. Four of six patients undergoing resection because of malignant disease were recurrence-free during the follow-up period. Fifteen (56%) patients suffered complications (transient or permanent). Four (15%) patients had recurrent stenosis and underwent reintervention. Other permanent complications included idiopathic hoarseness (15%), unilateral recurrent nerve palsy (11%), and dysphagia (7%). Previous treatment (endoscopic and open surgery) (OR=5.5, p=0.06) and chronic diseases (OR=8.3, p=0.02) were associated with increased risk for complications. CONCLUSIONS: Tracheal resection was efficient treatment for the alleviation of dyspnoea in adults with tracheal stenosis. However, complications were frequent and careful preoperative patient selection and information are crucial.

Pain and sensory disturbances following surgical repair of pectus carinatum.

BACKGROUND/PURPOSE: The purpose of this study was to assess the characteristics of persistent postoperative pain and sensory disturbances following surgical repair of pectus carinatum. METHODS: Using a prospective observational design, 28 patients were assessed before, 6 weeks and 6 months after a modified Ravitch operation for pectus carinatum. Postoperative pain was assessed using the Short Form McGill Pain Questionnaire. Sensory testing was conducted to detect brush-evoked allodynia and pinprick hyperalgesia. Additionally, generic and disease-specific quality of life was assessed using the Short Form-36 Health Survey and the Nuss Questionnaire Modified for Adults before and after surgery. RESULTS: Six weeks after surgery, ten patients reported mild pain or discomfort. Six months after surgery, four patients reported only mild pain. Allodynia was detected in two patients 6 weeks and 6 months after surgery. Hyperalgesia was detected in eight patients 6 weeks after surgery, and in six patients 6 months after surgery. Generic quality of life was significantly improved over time. CONCLUSIONS: The study showed no significant pain problems, a tendency to reduced sensory disturbances and significant improvements in quality of life 6 months after surgical repair of pectus carinatum. Future studies should include a longer follow-up period to determine if these positive results are persistent. LEVELS OF EVIDENCE: 1 (Prognosis Study).

Evaluating interest in narrative therapy for decision making about pectus excavatum treatment.

OBJECTIVES: The decision to proceed with surgical treatment for pectus excavatum (PE) is rarely clear-cut. Patients interested in treatment are referred for evaluation by numerous different specialists, but psychosocial counselling is currently not included in this process. Our objective was to assess whether PE patients would be interested in formal assistance with the decision-making process surrounding PE surgery using narrative therapy principles. METHODS: Ninety-seven untreated PE patients at 5 different institutions in 4 countries completed a questionnaire consisting of 13 questions, with 3 questions specifically evaluating interest in narrative therapy. RESULTS: Eighty-two percent of participants were interested in narrative therapy to assist with the decision-making process surrounding PE surgery. Individuals most interested in narrative therapy tend to be more interested in correction (P < 0.05) to improve the way they feel about their body (P < 0.05). CONCLUSIONS: The majority of PE patients are interested in narrative therapy to aid the decision-making process about treatment. We propose that narrative therapy should be offered to PE patients during the treatment evaluation process to address the psychosocial difficulties associated with PE and the surgical decision-making process overall. Future studies should assess the effectiveness of this interdisciplinary model.

Pectus excavatum repair after sternotomy: the Chest Wall International Group experience with substernal Nuss bars.

OBJECTIVES: Patients with pectus excavatum (PE) after prior sternotomy for cardiac surgery present unique challenges for repair of PE. Open repairs have been recommended because of concerns about sternal adhesions and cardiac injury. We report a multi-institutional experience with repair utilizing substernal Nuss bars in this patient population. METHODS: Surgeons from the Chest Wall International Group were queried for experience and retrospective data on PE repair using sub-sternal Nuss bars in patients with a history of median sternotomy for cardiac surgery (November 2000 to August 2015). A descriptive analysis was performed. RESULTS: Data for 75 patients were available from 14 centres. The median age at PE repair was 9.5 years (interquartile range 10.9), and the median Haller index was 3.9 (interquartile range 1.43); 56% of the patients were men. The median time to PE repair was 6.4 years (interquartile range 7.886) after prior cardiac surgery. Twelve patients (16%) required resternotomy before support bar placement: 7 pre-emptively and 5 emergently. Sternal elevation before bar placement was used in 34 patients (45%) and thoracoscopy in 67 patients (89%). Standby with cardiopulmonary bypass was available at 9 centres (64%). Inadvertent cardiac injury occurred in 5 cases (7%) without mortality. CONCLUSIONS: Over a broad range of institutions, substernal Nuss bars were used in PE repair for patients with a history of sternotomy for cardiac surgery. Several technique modifications were reported and may have facilitated repair. Cardiac injury occurred in 7% of cases, and appropriate resources should be available in the event of complications. Prophylactic resternotomy was reported at a minority of centres.

Minimal Invasive Repair of Pectus Excavatum and Carinatum.

Minimal invasive surgery has become the gold standard for surgical repair of pectus excavatum. The procedure can be performed as fast-track surgery and cosmetic results are excellent. In addition, cardiac performance improves after correction. With increased awareness on the Internet, the number of patients who seek help continues to rise, primarily for cosmetic reasons. Pectus carinatum is much less frequent than pectus excavatum. Over the past decade surgery has largely been replaced by compression techniques that use a brace, and cosmetic results are good. Rare combinations of pectus excavatum and carinatum may be treated by newer surgical methods.

Single centre experience on short bar technique for pectus excavatum.

BACKGROUND: Pectus excavatum (PE) is the most frequent anomaly of the anterior chest wall. Before 2001, all patients in Denmark were referred to the plastic and reconstructive surgeon for implantation of a subcutaneous silicone prosthesis, because the modified Ravitch procedure was not used. Since 2001, all patients have been treated with a modified Nuss procedure, which today has become the gold standard for correction of PE. METHODS: From September 2001 to March 2016, 1,713 patients have been operated by a modified Nuss procedure using the short bar at Aarhus University Hospital with a male-female ratio 6:1. The median age was 16 years (range 7-58 years). All operations were done in general anesthesia with epidural analgesia and all patients were operated by the same surgeon. All patients were seen routinely 6 weeks after surgery and the bars were removed after 3 years. RESULTS: Patients were younger than 18 years in 1,109 cases (65%). The number of bars needed for optimal correction was one in 1,209 patients, two in 486 patients and three in 18 patients. The median length of bar changed from 11 inches to 10 inches during the study period. The annual number of procedures continued to rise during the study period [833 patients during the first 10 years and 880 patients in the last 6 years, though more patients received two bars in the later period, (34%) versus (24%)] and the proportion of patients older than 30 years increased from 7.7% to 10%. The average duration of the operation was 36 minutes (range 12-270 minutes) and did not change significantly during the study period, change in operation time which was around half an hour. The median postoperative hospital stay decreased over time from 6 to 2 days. There was no mortality. CONCLUSIONS: The modified Nuss procedure with the short bar technique is effective for the correction of PE. The results are stable with a low rate of bar malrotations, and in may most cases can be done in less than an hour in experienced hands.

A prospective study on quality of life in youths after pectus excavatum correction.

BACKGROUND: The impact of correction of pectus excavatum (PE) on adolescents' health-related quality of life (HRQL) has only been investigated in prospective designs using disease-specific measures and without controls. The aim of this prospective study was to evaluate the HRQL before and after surgical correction of PE using a generic HRQL measure, and to compare the reported level of HRQL before surgery with an age-comparable control group. METHODS: Patients (n=107) and one of their parents (n=106) completed the generic HRQL measure: the Child Health Questionnaire before, 3 months, and 6 months after correction for PE. A control group (n=183) consisting of school children comparable in age completed the same measure on one occasion. RESULTS: The patients' level of HRQL before surgery was comparable to the level of the controls except for physical functioning; here boys reported impaired function compared to controls (P<0.0001, d=0.72). Both patients and parents reported improved emotional wellbeing and self-esteem, as well as an increase in physical and social activities from pre- to post-surgery. These improvements were statistically significant (P≤0.001-0.03) and yielded moderate to high effect sizes (ƞ2=0.04-0.22). CONCLUSIONS: The improvement of physical and psychosocial HRQL reported by both patients and their parents as proxy indicates the psychological implications of the deformity. Patients reported impaired physical function compared to controls. Further, pre-surgery differences in HRQL between the patients and the controls were lacking. However, the improvement in the patients' HRQL following surgery may justify the correction of pectus excavatum. The effect of the deformity on patients wellbeing compared with controls' needs to be addressed in further studies.

Short Nuss bar procedure.

The Nuss procedure is now the preferred operation for surgical correction of pectus excavatum (PE). It is a minimally invasive technique, whereby one to three curved metal bars are inserted behind the sternum in order to push it into a normal position. The bars are left in situ for three years and then removed. This procedure significantly improves quality of life and, in most cases, also improves cardiac performance. Previously, the modified Ravitch procedure was used with resection of cartilage and the use of posterior support. This article details the new modified Nuss procedure, which requires the use of shorter bars than specified by the original technique. This technique facilitates the operation as the bar may be guided manually through the chest wall and no additional stabilizing sutures are necessary.

Mixed Adenoneuroendocrine Carcinoma Is a Rare but Important Tumour Found in the Oesophagus.

Mixed adenoneuroendocrine carcinoma (MANEC) is a rare tumour of the gastrointestinal tract that consists of a dual adenocarcinomatous and neuroendocrine differentiation, each component representing at least 30% of the tumour. We report a case of a 68-year-old man who presented with two-month history of postprandial pain and vomiting. Gastric endoscopy revealed a polypoid mass in the lower part of the oesophagus. In contrast to the majority of these tumours, this biopsy was immunohistochemically positive for chromogranin A, and synaptophysin and Ki-67 index was 50% and the tumour was diagnosed as poorly differentiated neuroendocrine carcinoma of the oesophagus. The patient underwent surgery and lower oesophagus resection was performed. Based on the histopathology and immunohistochemistry of the tumour in the oesophagogastrectomy specimen, a mixed adenoneuroendocrine carcinoma (MANEC) was diagnosed. The objective of this case report is to advocate for the focus on the MANEC diagnosis as such patients need to be referred to a centre of excellence with expertise in NET tumours, to have the correct diagnostic work-up, treatment, and secondary diagnostic procedures performed at progression, as this will have paramount influence of the choice of treatment.

Cardiac function in adults following minimally invasive repair of pectus excavatum.

OBJECTIVES: To study if minimally invasive repair of pectus excavatum (PE) in adult patients would improve cardiopulmonary function at rest and during exercise as we have found previously in young and adolescent patients with PE. METHODS: Nineteen adult patients (>21 year of age) were studied at rest and during bicycle exercise before surgery and 1 year postoperatively. Lung spirometry was performed at rest. Cardiac output, heart rate and aerobic exercise capacity were measured using a photo-acoustic gas-rebreathing technique during rest and exercise. Data are shown as mean ± standard deviation. RESULTS: Fifteen patients completed the 1-year follow-up. No significant differences were found in neither cardiac output (14.0 ± 0.9 l min at baseline vs 14.8 ± 1.1 l min after surgery; P = 0.2029), nor maximum oxygen uptake (30.4 ± 1.9 and 33.3 ± 1.6 ml/kg/min; P = 0.0940 postoperatively). The lung spirometry was also unchanged, with no difference in forced expiratory capacity during the first second. CONCLUSIONS: Correction of PE in adult patients does not improve the cardiopulmonary function 1 year after surgery as seen in children and adolescents.

Successful resection of oesophageal adenocarcinoma 16 years after heart transplantation-a case report.

INTRODUCTION: With improving results of heart transplantation and subsequently increasing survival, long-term complications such as neoplastic malignancies are more often being discovered. PRESENTATION OF CASE: In this report, we present a unique case of successful oesophagus resection with gastric pull-up, on a heart-transplanted patient diagnosed with oesophageal adenocarcinoma. DISCUSSION: With the growing number of long-term surviving heart transplanted patients, the number of neoplasia in this patient-group will subsequently grow. Since physical condition and quality of life in long-term surviving heart transplanted patients is comparable to the general population, and since surgical treatment remains the mainstay of treatment for localised oesophageal carcinoma in non-heart transplanted patients, a larger subgroup of heart transplanted patients will most likely be considered for cancer surgery. CONCLUSION: Our case demonstrates the possibility of operating on the heart transplanted patient-group, and supports the option, that well-managed heart transplantation not should be a limiting factor, when deciding whether a patient is a candidate for surgical intervention or not.