Search

The prevalence and type of life-threatening complications related to the minimally invasive repair of pectus excavatum (MIRPE) and bar removal are unknown and underreported. The purpose of this communication is to make surgeons aware of the risk of these life threatening complications as well as the modifications which have been developed to prevent them. Methods Data related to life-threatening complications of Pectus Excavatum (PE) patients was obtained from four sources: 1. A survey of Chest Wall International Group (CWIG) surgeons who specialize in repairing congenital chest wall malformations, 2. Papers and case reports presented at CWIG meetings, 3. Review of medico-legal cases from the USA and 4. A systematic review of the literature related to major complications post MIRPE. Results From 1998 to 2016, we identified 27 published cases and 32 unreported life-threatening complications including: cardiac perforation, hemothorax, major vessel injury, lung injury, liver injury, gastrointestinal problems, and diaphragm injury. There were seven cases of major complications with bar removal (reported and non-reported) with two lethal outcomes. Mortality data with bar placement surgery: Four published death cases and seven unpublished death cases. The overall incidence of minor & major complications post MIRPE has been reported in the literature to be 2–20%. The true incidence of life-threatening complications and mortality is not known as we do not know the overall number of procedures performed worldwide. However, based on data extrapolated from survey information, the pectus bar manufacturer in the USA, literature reports, and data presented at CWIG meetings as to the number of cases performed we estimated that approximately fifty thousand cases have been performed and that the incidence of life-threatening complications is less than 0.1% with many occurring during the learning curve. Analysis of the cases identified in our survey revealed that previous chest surgery, pectus severity and inexperience were noted to be significant risk factors for mortality. Conclusions Published reports support the safety and efficacy of MIRPE; however major adverse outcomes are underreported. Although major complications with MIRPE and pectus bar removal surgery are very rare, awareness of the risk and mortality of life-threatening complications is essential to ensure optimal safety. Factors such as operative technique, patient age, pectus severity and asymmetry, previous chest surgery, and the surgeon's experience play a role in the overall incidence of such events. These preventable events can be avoided with proper training, mentoring, and the use of sternal elevation techniques. Type of study Treatment Study. Level of evidence Level IV. [ABSTRACT FROM AUTHOR]

Only patients with a severe pectus excavatum deformity should be candidates for surgical repair. Mild and moderate degrees of deformity should be treated with a deep breathing and exercise program and the vacuum bell. The best age for repair is during puberty, but successful repair in adult patients (up to 70 years of age) has been reported. Those scheduled for minimally invasive repair should be measured for bar length and screened for metal allergies. The minimally invasive technique for pectus excavatum repair is done thoracoscopically and requires no rib cartilage or sternal resection. Instead, it relies on bracing the anterior chest wall with a sub-sternal support bar placed in position under the sternum after first correcting the deformity with an introducer specially developed for the procedure. Two bars generally give better correction than one bar. It is essential that the bars be adequately stabilized to prevent bar displacement and recurrence of the deformity. Several techniques have been developed to elevate the sternum during mediastinal tunneling to improve thoracoscopic visibility in very severe and asymmetric deformities. Since 1987 we have evaluated 3836 patients in our chest wall deformities clinic, of whom 1921(50%) have undergone repair. 1738 patients were primary repairs and 1346 of these have had their bars removed two to three years after placement. The biggest challenge in the early years was bar displacement but that has now been reduced to just over 1% by use of a stabilizer on the left side and “0” PDS pericostal sutures on the right side. 88.9% of patients reported an excellent result, 9.6% a good result and 1.5% a poor or failed result. In conclusion: The minimally invasive repair of pectus excavatum is highly successful when carried out by experienced surgeons in a center dedicated to the management of chest wall malformations. [ABSTRACT FROM AUTHOR]

Abstract: Purpose: We previously demonstrated that patients with pectus excavatum (PE) have significantly decreased chest wall motion at the pectus defect compared with the rest of the chest vs unaffected individuals and use abdominal respiratory contributions to compensate for decreased upper chest wall motion. We hypothesize that PE repair will reverse chest wall motion dysfunction. Methods: A prospective, institutional review board–approved study compared patients with PE before and after Nuss repair. Informed consent was obtained before motion analysis. Sixty-four patients with uncorrected PE ages 10 to 21 years underwent optoelectronic plethysmography analysis. Repeat analysis was performed in 42 patients 6 months postoperative (PO). Results: Volume of the chest wall and its subdivisions were calculated. Total chest wall volume at rest was significantly increased after repair and in each thoracic compartment. PO patients developed increased midline marker excursion at the pectus defect and significantly decreased excursion at the level of the umbilicus. Conclusions: Optoelectronic plethysmography kinematic analysis demonstrates that chest wall remodeling during Nuss repair results in increased thoracic volume. Chest wall motion dysfunction at the pectus defect is reversed after Nuss repair. Abdominal respiratory contributions are also markedly decreased. These findings may help to explain why patients with PE report an improvement in endurance after the Nuss procedure. [Copyright &y& Elsevier]

Abstract: Purpose: Controversy exists as to the best operative approach to use in patients with failed pectus excavatum (PE) repair. We examined our institutional experience with redo minimally invasive PE repair along with the unique issues related to each technique. Methods: We conducted an institutional review board–approved review of a prospectively gathered database of all patients who underwent minimally invasive repair of PE. Results: From June 1987 to January 2010, 100 patients underwent minimally invasive repair for recurrent PE. Previous repairs included 42 Ravitch (RAV) procedures, 51 Nuss (NUS) procedures, 3 Leonard procedures, and 4 with previous NUS and RAV repairs. The median Haller index at reoperation was 4.99 (range, 2.4-20). Fifty-five percent of RAV patients and 25% of NUS patients required 2 or more bars (P = .01). Two RAV patients had intraoperative nonfatal cardiac arrest owing to thoracic chondrodystrophy—1 at insertion and 1 upon removal. Bar displacements occurred in 12% RAV and 7.8% NUS patients (P = .05). Overall reoperation for bar displacement is 9%. Conclusions: The minimally invasive NUS technique is safe and effective for the correction of recurrent PE. Patients with prior NUS repair can have extensive pleural adhesions necessitating decortication during secondary repair. Patients with a previous RAV repair may have acquired thoracic chondrodystrophy that may require a greater number of pectus bars to be placed at secondary repair and greater risk for complications. We have a greater than 95% success rate regardless of initial repair technique. [Copyright &y& Elsevier]

Abstract: Background: Paradoxical chest wall motion is recognized clinically in pectus excavatum (PE). We report chest wall volume and motion differences between PE patients and unaffected individuals. Methods: A prospective, institutional review board–approved study compared nonoperated PE patients with normal controls (C). Subjects had deep breathing maneuvers captured by infrared cameras. Chest wall volume and excursion were calculated using optoelectronic plethysmography marker reconstruction combined with proprietary software (BTS Bioengineering, Milan, Italy). Results: One hundred nineteen patients underwent optoelectronic plethysmography analysis (PE: 64, C: 5). Total chest wall volume at rest was similar in both groups (PE: 13.6 L, C: 14.1 L, P = .55). During maximal inspiration, PE patients had a significant increase in the volume within the abdominal rib cage compartment (PE: 0.77 L, C: 0.6 L, P < .01). Patients with PE had 51% less midline marker excursion at the angle of Louis (P < .01), a 46% decrease at the level of the nipples (P < .01), and 28% less excursion at the xiphoid process (P = .02). At the level of the umbilicus, PE patients had 147% increase in midline marker excursion compared with controls (P < .01). Conclusions: Optoelectronic plethysmography kinematic analysis allows for quantification of focal chest wall motion dysfunction. Patients with PE demonstrate significantly decreased chest wall motion at the area of the pectus defect and increased abdominal contributions to respiration compared with controls. This finding may help to explain exertional symptoms of easy fatigability or shortness of breath in PE. [Copyright &y& Elsevier]

Abstract: Purpose: The presence of a pectus excavatum (PE) requiring surgical repair is a major skeletal feature of Marfan syndrome. Marfanoid patients have phenotypic findings but do not meet all diagnostic criteria. We sought to examine the clinical and management differences between Marfan syndrome patients and those who are marfanoid compared with all other patients undergoing minimally invasive PE repair. Methods: A retrospective institutional review board–approved review was conducted of a prospectively gathered database of all patients who underwent minimally invasive repair of PE. Patients were grouped according to diagnosis of Marfan syndrome (MAR), Marfanoid appearance (OID), and all others (ALL). Patient demographics, preoperative imaging and testing, operative strategy, complications, and postoperative surveys were evaluated. Fisher''s Exact test and χ 2 were applied for statistical analysis. Results: From June 1987 to September 2008, 1192 patients underwent minimally invasive PE repair (MAR = 33, OID = 212, ALL = 947). There was a significantly higher proportion of females with either MAR or OID who underwent repair (21.5%vs 15.5%, P = .04). The MAR patients had significantly more severe PE determined by computed tomography index (MAR = 8.75, OID = 5.82, ALL = 4.94, P < .0001) and required multiple pectus bars (≥2) to be placed during operation (MAR = 58%, OID = 36%, ALL = 29%, P = .001). There was a trend toward higher wound infection rates in MAR patients (MAR = 6%, OID = 1.4%, ALL = 1.3%, P = .07). The recurrence rate was similar among all groups (MAR = 0%, OID = 2%, ALL = 0.7%, P = .12). Successful outcome from surgeon perspective in either MAR or OID patients was similar to ALL (98%vs 98%, P = .88) and correlated well with patient satisfaction after repair (96%vs 95%, P = .43). Conclusions: Minimally invasive PE repair is safe in patients with Marfan syndrome or marfanoid features with equally good results. Patients with Marfan syndrome have clinically more severe PE requiring multiple bars for chest repair and may have slightly higher wound infection rates. Patients are satisfied with minimally invasive repair despite a phenotypically more severe chest wall defect. [Copyright &y& Elsevier]

The minimally invasive repair of pectus excavatum has become widely accepted. The number of patients presenting for repair has increased dramatically. There have been many technical improvements over 20 years that have made the procedure much safer and more successful. The complications have been identified and preventative measures instituted. The long-term results have shown a 95% good to excellent outcome, and patient satisfaction studies have shown similar results. [Copyright &y& Elsevier]

Abstract: Purpose: Increasing use of implantable bars for minimally invasive pectus excavatum repair has introduced metal allergy (nickel and chromium) to pediatric surgeons. Metal allergy is a well-recognized entity in neurologic, orthopedic, and craniofacial surgery. This study was performed to evaluate metal allergy and its effects on treatment with the Nuss procedure in 862 patients. Methods: After institutional review board approval, we undertook a retrospective review of a prospectively gathered database of patients undergoing the Nuss procedure. Metal allergy was diagnosed either with the use of dermal patch or clinically, based on rash, fever, elevated erythrocyte sedimentation rate, cultures, and pathology specimens. Data collection included demographics, an allergy to jewelry, and history of atopy. Clinical outcomes including need for reoperation, removal of stainless steel bar, and replacement with titanium bar were evaluated. Results: Over an 18-year period (1987-2005), 862 patients underwent the Nuss procedure. Nineteen (2.2%) were diagnosed with metal allergy, with an average age of 14.7 years (9-23 years). Eighteen (95%) were males. A history of atopy was present in 9 (56%) patients. Ten (63%) patients presented with rash and erythema, 1 (6%) with granuloma, 5 (32%) with pleural effusion, and 3 (15%) were diagnosed on preoperative screening. Stainless steel bars were removed because of allergic skin breakdown in 3 patients, with 2 patients requiring replacement titanium bars. In all 3 of these patients, symptoms resolved after removal of stainless steel bars. Titanium bars were placed in the 3 patients who were diagnosed preoperatively with metal allergy, without event. Conclusions: Allergy symptoms often are misdiagnosed as infection, but require different treatment. If a history of metal allergy or atopy is suggested preoperatively, the patient should be tested for metal allergy, and if positive, a titanium bar used. Because the consequences of metal allergy may include the need to replace the bar, pediatric surgeons should be aware of this occurrence. [Copyright &y& Elsevier]

Abstract: Purpose: A nemesis of surgical implants is infection. We evaluated the various infectious complications after Nuss repair of pectus excavatum in 863 patients over 18 years. Methods: After institutional review board approval, a retrospective review of a prospectively gathered database of patients was performed who underwent minimally invasive repair of pectus excavatum and developed an infection. All patients received intravenous antibiotics before surgery continuing until discharge. Patients with a persistent fever after operation were discharged with oral antibiotics. Results: From January 1987 to September 2005, 863 patients underwent a minimally invasive pectus excavatum repair and 13 (1.5%) developed postoperative infections. These included 6 bar infections, 4 cases of cellulitis, and 3 stitch abscesses. Cellulitis was defined as erythema and warmth which responded to a single course of antibiotics. Bar infections were defined as an abscess in contact with the bar. Surgical drainage and long-term antibiotics resolved 3 of these abscesses, whereas 3 patients required early bar removal (1 after 3 months and 2 after 18 months). Cultures identified a single organism in each case and Staphylococcus aureus was the most common organism (83%) identified, and all being methicillin sensitive. All infections occurred on the side of the stabilizer if a stabilizer had been placed. Conclusions: Infectious complications after Nuss repair are uncommon and occurred in 1.5% of our patients. Published rates of postoperative infection range from 1.0% to 6.8%. Superficial infections responded to antibiotics alone. Bar infection occurred in only 0.7% and required surgical drainage and long-term antibiotics. Only 3 of these (50% of bar infections and 0.34% overall) required early bar removal at 3 and 18 months because of recurring infections. Early bar removal should be a rare morbidity with the Nuss repair. [Copyright &y& Elsevier]

Abstract: Background: The most common congenital deformity of the chest wall is pectus excavatum, a malformation that is present in between 1 in 400 and 1 in 1000 live births and causes the body of the sternum to be displaced, producing a depression. There are many different shapes of the pectus, and multiple factors probably contribute to the final form. The etiology of pectus excavatum is uncertain, but a familial tendency has been found in clinical experience, where it may be seen in more than one sibling. Pectus excavatum is commonly associated with connective tissue disorders such as Marfan and Ehlers Danlos syndromes. Extensive literature review failed to identify articles documenting families with multiple affected members. Purpose: The purpose of this study was to collect evidence that pectus excavatum is familial and may be an inherited disorder. Methods: Using the Children''s Surgical Specialty Group database at Children''s Hospital of The King''s Daughters, families with more than one affected individual were selected. With Institutional Review Board–approved informed consent, 34 families agreed to participate. Family histories were obtained, and a 4-generation pedigree was constructed for each family. Forty questions were asked about each individual''s medical history, and comprehensive systems review included features of connective tissue-related problems. Inheritance patterns for each family were determined by pedigree analysis. Results: A total of 14 families suggested autosomal dominant inheritance, 4 families suggested autosomal recessive inheritance, and 6 families suggested X-linked recessive inheritance. Ten families had complex inheritance patterns. Pectus excavatum occurred more frequently in males than in females (1.8:1). Long arms, legs, and fingers; high-arched palate; mitral valve prolapse; heart arrhythmia; scoliosis; double jointedness; flexibility; flat feet; childhood myopia; poor healing; and easy bruising were commonly associated with pectus excavatum. Conclusions: Pedigree analysis of 34 families provides evidence that pectus excavatum is an inherited disorder, possibly of connective tissue. Although some families demonstrate apparent Mendelian inheritance, most appear to be multifactorial. [Copyright &y& Elsevier]

Abstract: Background/Purpose: To describe the dysmorphology of pectus excavatum, the most common congenital chest wall anomaly. Methods: A stratified sample of 64 patients, representative of a patient population with pectus excavatum of the Children''s Hospital of King''s Daughters in Norfolk, Va, was described and classified. The sample was stratified by sex to represent a 4:1 male-to-female ratio. The sample was further stratified to represent categories of age (3-10, 11-16, and 17 years and older). Preoperative photos and baseline chest computed tomography scans were examined and categorized according to the chief criteria, including asymmetry/symmetry of the depression, localized vs diffuse morphology, sternal torsion, cause of asymmetric appearance, and the length of the depression. Results: Useful morphologic distinctions in pectus excavatum are localized depressions vs diffuse depressions, short and long length, symmetry, sternal torsion, slope/position of absolute depth, and unique patterns such as the horns of steer depression. Conclusions: These classifications simplify the diagnosis of pectus excavatum, aid in corrective surgery, and should improve correlation of phenotype and genotype in future genetic analysis. [Copyright &y& Elsevier]

Abstract: Purpose: In evaluating the impact of surgical repair of pectus excavatum, the Haller index developed for preoperative decision-making purposes may be inadequate to quantify postoperative changes in shape of the chest. Individual patients may also have chest characteristics that impact the success of repair, many of which would be unlikely to be measured by the Haller index alone. We have developed a protocol that measures the cross-sectional chest area and the asymmetry index along with the Haller index to more completely quantify the nature of the deformity. The purpose of this study was to determine the reliability of this protocol in the interpretation of chest computed tomography images from multiple sites. The protocol was developed as part of a multicenter study of clinical outcomes after surgical repair of pectus excavatum. Methods: Two radiologists independently selected 5 images from each of 32 computed tomography scans from multicenter study participants according to the protocol. A digitizer was used to measure the diameters and cross-sectional areas of the images selected; these results were used to calculate the Haller and asymmetry indices. The protocol was tested for intradigitizer and interradiologist reliability. Using the Haller and asymmetry indices, we also assessed agreement between radiologists classifying patients as abnormal. Results: Agreement was uniformly high for all comparisons (all Lin''s concordance coefficients >0.99 and all Cohen''s κ''s >0.85, all agreement on classification of patients >95%) indicating almost perfect agreement. Disagreement on classification of patients using the Haller and asymmetry index was at the cut points for abnormality. Conclusion: The protocol was found to be a highly reliable method for deriving the cross-sectional area of the chest and the Haller and asymmetry indices and for classifying patients for surgical eligibility. Borderline cases should be examined carefully to determine the appropriateness of surgical intervention. Cross-sectional area can be measured reliably using this protocol and thus may be useful in quantifying the success of surgical intervention. [Copyright &y& Elsevier]

Objective: To determine whether pulmonary function decreases as a function of severity of pectus excavatum, and whether reduced function is restrictive or obstructive in nature in a large multicenter study. Study design: We evaluated preoperative spirometry data in 310 patients and lung volumes in 218 patients aged 6 to 21 years at 11 North American centers. We modeled the impact of the severity of deformity (based on the Haller index) on pulmonary function. Results: The percentages of patients with abnormal forced vital capacity (FVC), forced expiratory volume in 1 second (FEV1), forced expiratory flow from 25% exhalation to 75% exhalation, and total lung capacity findings increased with increasing Haller index score. Less than 2% of patients demonstrated an obstructive pattern (FEV1/FVC <67%), and 14.5% demonstrated a restrictive pattern (FVC and FEV1 <80% predicted; FEV1/FVC >80%). Patients with a Haller index of 7 are >4 times more likely to have an FVC of ≤80% than those with a Haller index of 4, and are also 4 times more likely to exhibit a restrictive pulmonary pattern. Conclusions: Among patients presenting for surgical repair of pectus excavatum, those with more severe deformities have a much higher likelihood of decreased pulmonary function with a restrictive pulmonary pattern. [Copyright &y& Elsevier]

Background: A multicenter study of pectus excavatum was described previously. This report presents our final results. Study Design: Patients treated surgically at 11 centers were followed prospectively. Each underwent a preoperative evaluation with CT scan, pulmonary function tests, and body image survey. Data were collected about associated conditions, complications, and perioperative pain. One year after treatment, patients underwent repeat chest CT scan, pulmonary function tests, and body image survey. A subset of 50 underwent exercise pulmonary function testing. Results: Of 327 patients, 284 underwent Nuss procedure and 43 underwent open procedure without mortality. Of 182 patients with complete follow-up (56%), 18% had late complications, similarly distributed, including substernal bar displacement in 7% and wound infection in 2%. Mean initial CT scan index of 4.4 improved to 3.0 post operation (severe >3.2, normal = 2.5). Computed tomography index improved at the deepest point (xiphoid) and also upper and middle sternum. Pulmonary function tests improved (forced vital capacity from 88% to 93%, forced expiratory volume in 1 second from 87% to 90%, and total lung capacity from 94% to 100% of predicted (p < 0.001 for each). VO2 max during peak exercise increased by 10.1% (p = 0.015) and O2 pulse by 19% (p = 0.007) in 20 subjects who completed both pre- and postoperative exercise tests. Conclusions: There is significant improvement in lung function at rest and in VO2 max and O2 pulse after surgical correction of pectus excavatum, with CT index >3.2. Operative correction significantly reduces CT index and markedly improves the shape of the entire chest, and can be performed safely in a variety of centers. [Copyright &y& Elsevier]

Oxalacetate acetylhydrolase (OAH), a member of the phosphoenolpyruvate mutase/isocitrate lyase superfamily, catalyzes the hydrolysis of oxalacetate to oxalic acid and acetate. This study shows that knock-out of the oah gene in Cryphonectria parasitica, the chestnut blight fungus, reduces the ability of the fungus to form cankers on chestnut trees, suggesting that OAH plays a key role in virulence. OAH was produced in Escherichia coli and purified, and its catalytic rates were determined. Oxalacetate is the main OAH substrate, but the enzyme also acts as a lyase of (2R,3S)-dimethyl malate with ~1000-fold lower efficacy. The crystal structure of OAH was determined alone, in complex with a mechanism-based inhibitor, 3,3-difluorooxalacetate (DFOA), and in complex with the reaction product, oxalate, to a resolution limit of 1.30, 1.55, and 1.65 Å, respectively. OAH assembles into a dimer of dimers with each subunit exhibiting an (α/β)8 barrel fold and each pair swapping the 8th α-helix. An active site "gating loop" exhibits conformationai disorder in the ligand-free structure. To obtain the structures of the OAH⋅ligand complexes, the ligand-free OAH crystals were soaked briefly with DFOA or oxalacetate. DFOA binding leads to ordering of the gating loop in a conformation that sequesters the ligand from the solvent. DFOA binds in a gem-diol form analogous to the oxalacetate intermediate/transition state. Oxalate binds in a planar conformation, but the gating loop is largely disordered. Comparison between the OAH structure and that of the closely related enzyme, 2,3-dimethylmalate lyase, suggests potential determinants of substrate preference. [ABSTRACT FROM AUTHOR]

Background: Given widespread adoption of the Nuss procedure, prospective multicenter study of management of pectus excavatum by both the open and Nuss procedures was thought desirable. Although surgical repair has been performed for more than 50 years, there are no prospective multicenter studies of its management. Study Design: This observational study followed pectus excavatum patients treated surgically at 11 centers in North America, according to the method of choice of the patient and surgeon. Before operation, all underwent evaluation with CT scan, pulmonary function tests, and body image survey. Data were collected about associated conditions, hospital complications, and perioperative pain. One year after completion of treatment, patients will repeat the preoperative evaluations. This article addresses early results only. Results: Of 416 patients screened, 327 were enrolled; 284 underwent the Nuss procedure and 43 had the open procedure. Median preoperative CT index was 4.4. Pulmonary function testing before operation showed mean forced vital capacity of 90% of predicted values; forced expiratory volume in 1 second (FEV1), 89% of predicted; and forced expiratory flow during the middle half of the forced vital capacity (FEF25% to 75%), 85% of predicted. Early postcorrection results showed that operations were performed without mortality and with minimal morbidity at 30 days postoperatively. Median hospital stay was 4 days. Postoperative pain was a median of 3 on a scale of 10 at time of discharge; the worst pain experienced was the same as was expected by the patients (median 8), and by 30 days after correction or operation, the median pain score was 1. Because of disproportionate enrollment and similar early complication rates, statistical comparison between operation types was limited. Conclusions: Anatomically severe pectus excavatum is associated with abnormal pulmonary function. Initial operative correction performed at a variety of centers can be completed safely. Perioperative pain is successfully managed by current techniques. [Copyright &y& Elsevier]