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Chest wall malformations include excavated deformities or pectus excavatum (PEX) and carinated deformities or pectus carinatum (PC). These deformities can be mixed defects and they may also be a part of a congenital syndrome such as the Currarino Silverman syndrome. The development in the field of chest wall malformations has been steep in the three last decades and both adult and pediatric surgeons have become specialized in the subject pushing even forward the baggage of knowledge. We aim to introduce the reader in the different aspects related to pectus deformities.

Objective To review standardized Nuss correction of pectus excavatum and vacuum bell treatment over the last 10 years. Summary of background data In 2010, we reported 21 years of the Nuss procedure in 1215 patients. Methods Over the last 10 years, 2008-2018, we evaluated 1885 pectus excavatum patients. Surgery was indicated for well-defined objective criteria. A consistent operation was performed by 8 surgeons in 1034 patients, median 15 years, (range 6-46); 996 were primary, and 38 redo operations. Surgical patients' mean computed tomography index was 5.46. Mitral valve prolapse was present in 5.4%, Marfan syndrome in 1.1% and scoliosis in 29%. Vacuum bell treatment was introduced for 218 patients who did not meet surgical criteria or were averse to surgery. Results At primary operation, 1 bar was placed in 49.8%; 2 bars, 49.4%; and 3 bars, 0.7%. There were no deaths. Cardiac perforation occurred in 1 patient who had undergone previous cardiac surgery. Paraplegia after epidural catheter occurred once. Reoperation for bar displacement occurred in 1.8%, hemothorax in 0.3%, and wound infection in 2.9%; 1.4% required surgical drainage. Allergy to stainless steel was identified in 13.7%. A good anatomic outcome was always achieved at bar removal. Recurrence requiring reoperation occurred in 3 primary surgical patients. Two patients developed carinate overcorrection requiring reoperation. Vacuum bell treatment produced better results in younger and less severe cases. Conclusions A standardized Nuss procedure was performed by multiple surgeons in 1034 patients with good overall safety and results in primary repairs. Vacuum bell treatment is useful.

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.

Historically, pectus excavatum (PE) was reported to be congenital, but in our experience only 22% are noticed in the first decade of life. Thus far, genetic studies support an autosomal recessive heritability, which coincides with only 40% of our patients having some positive family history, but is also contradictory given a constant sex ratio of 4:1 in favor of males. This inconsistency may be explained by the effect of more than one pectus disease-associated allele. Once the deformity is noticed, it tends to progress slowly until puberty, when rapid progression is often seen. We recommend surgical repair at around 12–14 years of age since the chest wall is still typically flexible and because this allows us to keep the bar in place as the patient progresses through puberty which may help decrease growth-related recurrences. Patients with mild to moderate PE are treated with therapeutic deep breathing, posturing, and aerobic exercises, and in appropriately selected patients, the vacuum bell may also be offered. Patients that have severe symptomatic PE are offered Minimally Invasive Repair of Pectus Excavatum (MIRPE). The surgical technique in children is similar to that of adults, except for the higher forces involved that often necessitate sternal elevation and more involved stabilization strategies. Postoperative management includes pain control, deep breathing, and early ambulation. Exercise restriction is mandatory for the first six weeks with slow resumption of normal activity after 12 weeks.

Repair of pectus excavatum began at the beginning of the 20 th century before endotracheal intubation was standard practice. Surgeons therefore developed techniques that corrected the deformity using an open procedure via the anterior chest wall. Initial techniques were unsatisfactory, but by the 1930s the partial rib resection and sternal osteotomy technique had been developed and was used in combination with external traction post-operatively to prevent the sternum from sinking back into the chest. In 1949, Ravitch recommended complete resection of the costal cartilages and complete mobilization of the sternum without external traction, and in 1961 Adkins and Blades introduced the concept of a substernal strut for sternal support. The wide resection resulted in a very rigid anterior chest wall, and in some instances, the development of asphyxiating chondrodystrophy. The primary care physicians therefore became reluctant to refer the patients for repair. In 1987, Nuss developed a minimally invasive technique that required no cartilage or sternal resection and relied only on internal bracing by means of a sub-sternal bar, which is inserted into the chest through two lateral thoracic incisions and guided across the mediastinum with the help of thoracoscopy. After publication of the procedure in 1998, it became widely accepted and a flood of new patients suddenly started to appear, which allowed for rapid improvements and modifications of the technique. New instruments were developed specifically for the procedure, complications were recognized, and the steps taken to prevent them included the development of a stabilizer and the use of pericostal sutures to prevent bar displacement. Various options were developed for sternal elevation prior to mediastinal dissection to prevent injury to the mediastinal structures, allergy testing was implemented, and pain management improved. The increased number of patients coming for repair permitted studies of cardiopulmonary function, which showed that patients with a severe degree of pectus excavatum have right- sided cardiac compression, decreased filling, and decreased stroke volume. The degree of pulmonary restriction and obstruction is related to the degree of deformity and degree of cardiac displacement into the left chest. The indications for surgical repair have been clearly outlined, the procedure has been standardized, and post-operative management protocols are now available. A review of our prospective database showed that 98% of patients have a good to excellent outcome. This review of the “Past” outlines the progression of the surgical techniques during the 20 th century, the review of the “Present” outlines the important modifications and results of the closed technique, and the review of the “Future” outlines the various new options that are becoming available for the treatment of pectus excavatum.

Background: Recently, technical improvement in the ability to measure lung function and the severity of chest deformity have enabled progress in understanding the mechanism of limitations of lung function in pectus excavatum. Methods: After establishing that most patients with pectus excavatum do have symptoms of exercise intolerance, easy fatigability, and shortness of breath with exertion, lung function has been evaluated by a variety of methods in different centers. Spirometry, plethysmography, exercise testing, oculo electronic plethysmography, and imaging methods have been used to assess lung function in pectus excavatum and its response to surgery. Results: Not all patients with pectus excavatum have subnormal static pulmonary function testing; some have above-average values. However, in more than 1500 adult and pediatric surgical patients with anatomically severe pectus excavatum at a single center, the bell curve of FVC, FEV1, and FEF 25-75 is shifted to significantly lower values in pectus excavatum. The curve is shifted to higher values after operation by approximately one standard deviation. Previous work has demonstrated that patients with more anatomically severe pectus excavatum are more likely to have diminished PFT’s. A mechanism for this effect is seen by oculo electronic plethysmography, which demonstrates that the depressed portion of the chest does not move on respiration. After Nuss procedure, the chest wall motion used to create suction to draw air into the lungs is indistinguishable from that of persons with a normal chest, and the intrathoracic volume is markedly increased. Conclusions: Pectus excavatum is accompanied in most patients by diminished static pulmonary function. Correction by Nuss procedure results in improvement in chest wall motion; this improvement in the thoracic bellows action is accompanied by improvement in pulmonary function testing.

Pectus excavatum is the most common congenital chest wall abnormality expressed in children, yet its inheritance is poorly understood. Here we present the first comprehensive assessment of the inheritance of this disorder. After evaluating 48 pedigrees and 56 clinical traits of probands and family members, we find strong evidence of autosomal recessive, genetic control for this disorder. Additionally there is likely more than one pectus disease-associated allele, as well as a relatively large number of disease allele carriers in the human population. Some clinical traits appear important and may serve as reliable indicators for predicting the likelihood of pectus excavatum in children before severe symptoms present. Quantifying sex-ratio bias in probands demonstrates a highly significant male bias associated with pectus excavatum. When combined with pedigree data, sex-bias is indicative of sex-linked, sex-limited, and/or epigenetic control such as X-inactivation, reiterating a point made with pedigrees alone, which is that more than one mutation is likely responsible for this disorder.

Recurrent pectus excavatum is more challenging to repair than primary pectus excavatum and requires significant knowledge of the risks and pitfalls in these complex repairs, as well as experience with the advantages and disadvantages of the various techniques available. This chapter will review types of recurrence and selection of open or closed technique in re-do repair, as well as preoperative preparation and technical considerations for repair of recurrent excavatum.

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.

Background/Purpose More than forty percent of patients with pectus excavatum have a family history of chest deformity. However, no studies of the frequency of the different phenotypes of pectus excavatum have been published. Methods A random sample of 300 non-syndromic pectus excavatum patients, from the chest wall deformities clinic at Children's Hospital of The King's Daughters in Norfolk, VA, was studied and classified according to a previously described classification system. Photographs and computed tomography (CT) scans were utilized. Results Typical pectus excavatum. Photo data: localized deep depression (cup-shaped) deformity occurred in 67%; diffuse (saucer-shaped) 21%, trench-like (furrow-shaped) 10%, and Currarino-Silverman (mixed pectus excavatum/chondromanubrial carinatum) 1%. The deepest point was to the right of midline in 80%, left in 10% and central in 10%. As per the photograph, the deepest point was in the lower sternum in 75%. When asymmetric, the deepest point of the deformity was to the right of midline in 90%. CT data: the average Haller index was 4.9. Severe sternal torsion (>30°) was associated with greater Haller index (6.3) than mild torsion (4.5). The deepest point of the depression was at the mid- or lower sternum in more than 99%. It proved impossible to estimate width or length of the depression because of poorly defined borders. Conclusions Typical PE is cup-shaped in 67% of cases, to the right of the midline in 80%, and involving the mid-to-lower sternum in 99%. However, other phenotypes, like the saucer and long trench, comprised one-third. Definition of the deformity is more reliable by CT scan.

Resumen Antecedentes/Objetivo Mas del 40% de los pacientes con pectus excavatum tienen antecedentes familiares de una deformidad toracica. Sin embargo, no se han publicado estudios de la frecuencia de los diferentes fenotipos de pectus excavatum. Metodos Se estudio una muestra aleatoria de 300 pacientes con pectus excavatum no sindromico de la clinica de deformidades de la pared toracica del Children's Hospital of the King's Daughters de Norfolk (Virginia, Estados Unidos) y se clasifico a los pacientes segun un sistema descrito con anterioridad. Se utilizaron para ello fotografias e imagenes de tomografia computarizada (TC). Resultados Pectus excavatum tipico . Datos fotograficos: se observo una deformidad con depresion profunda localizada (forma de taza) en el 67% de los casos, difusa (en forma de platillo) en el 21%, de tipo trinchera (en forma de surco) en el 10%, y de tipo Currarino-Silverman (deformidad mixta de pectus excavatum/pectus carinatum condromanubrial) en el 1%. El punto mas profundo se encontraba a la derecha de la linea media en el 80% de los casos, a la izquierda en el 10% y en el centro en el 10%. En las fotografias, el punto mas profundo se encontraba en la parte inferior del esternon en el 75% de los casos. Cuando habia asimetria, el punto mas profundo de la deformidad estaba a la derecha de la linea media en el 90% de los casos. Datos de TC: la media del indice de Haller fue de 4,9. La torsion esternal intensa (> 30 grados) se asocio a un indice de Haller mas alto (6,3) que el observado en la torsion leve (4,5). El punto mas profundo de la depresion se encontraba en la parte media o baja del esternon en mas del 99% de los casos. Resulto imposible estimar la anchura o la longitud de la depresion, ya que los limites estaban mal definidos. Conclusiones El pectus excavatum tipico tiene forma de copa en el 67% de los casos, se encuentra a la derecha de la linea media en el 80% y afecta a la parte media o baja del esternon en el 99%. Sin embargo, otros fenotipos, como el de forma de platillo y el de trinchera larga, constituyeron una tercera parte del total. La definicion de la deformidad es mas fiable mediante la TC.

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.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 chi(2) were applied for statistical analysis.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 (or =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).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.

Pectus excavatum can be corrected with minimally invasive techniques that do not require costal cartilage resection or sternal osteotomy. At our institution, 947 patients have been safely and effectively managed with excellent long-term results and low morbidity.

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.

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.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.Agreement was uniformly high for all comparisons (all Lin's concordance coefficients0.99 and all Cohen's kappa's0.85, all agreement on classification of patients95%) indicating almost perfect agreement. Disagreement on classification of patients using the Haller and asymmetry index was at the cut points for abnormality.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.

A very rare complication of minimally invasive repair of pectus excavatum, the development of pectus carinatum, was recently reported. This is an unexpected sequela that was never described when the method of Ravitch was used to repair pectus excavatum. We describe a similar case. However, in contrast to the symmetric pectus carinatum noted by Hebra et al., a very asymmetric hump of the anterior thorax, limited to the right side, developed in our patient, with extremely unappealing cosmetic consequences. The cause appears to have been an overly tight bar resulting from fast growth during puberty.

Purpose: The aim of this study is to review the new technical modifications and results of 303 patients who have had pectus excavatum repair utilizing the minimally invasive technique. Methods: A retrospective chart review was conducted of 303 patients undergoing minimally invasive pectus repair from 1987 through August 2000. Since 1997, a standardized treatment pathway was implemented, and 261 of the 303 patients have been treated on this pathway. Preoperative evaluation included computed tomography (CT) scan, pulmonary function tests (PFT), and cardiac evaluations with electrocardiogram (EKG) and echocardiogram. Indications for operation included at least 2 of the following: progression of the deformity, exercise intolerance or restrictive disease on PFT, Haller CT index greater than 3.2, mitral valve prolapse (MVP), or cardiac compression. Technical and design modifications since 1998 have included routine thoracoscopy, the use of an introducer/dissector for creating the substernal tunnel and elevating the sternum, and routine use of a wired lateral stabilizer to prevent bar displacement. The bar is removed as an outpatient procedure in 2 to 4 years. Results: In 303 patients undergoing minimally invasive pectus repairs, single bars were used in 87% and double in 13%. Lateral stabilizers were applied in 70% of patients and were wired for further stability in 65%. Bar shifts before the use of stabilizers were 15%, which decreased to 6% after stabilizers were placed and 5% with a wired stabilizer. Excellent results were noted in 85% with failure in only 1 patient. Complications included pneumothorax with spontaneous resolution in half of the patients and pericarditis in 7. Conclusions: The minimally invasive technique has evolved into an effective method of pectus excavatum repair. Modifications of the technique have reduced complications. Long-term results continue to be excellent.

The Nuss procedure has succeeded in minimizing incisions, blood loss, sternal fracturing, operating room time, recovery time, and length of hospital stay. Knowledge of the pre- and postoperative radiologic considerations is essential in providing appropriate imaging support to the surgeons performing this innovative, minimally invasive procedure.

We investigated polymorphisms in the variable number of tandem repeat polymorphisms (VNTR) regions of the ACAN gene in 154 patients with pectus excavatum that required surgery. To our knowledge, this is the first study to examine correlation of a functional VNTR genotype of cartilage with pectus excavatum. Patients, non-affected family members, and controls were genotyped by PCR and the number of VNTRs identified. We observed that patients had significantly more 27 repeats, and significantly less 25 repeats, than controls and parents. We examined VNTRs with severity of disorder and found no correlation between ACAN genotype, repeat numbers, and severity. Subgroups in the pectus population were observed. There were a number of unique differences in these subgroups, especially in patients with a Marfan clinical phenotype, where there was a correlation of increased number of VNTRs with phenotype. Conversely, in the affected females, there was an inverse correlation between ACAN VNTRs and gender. Females also tended to present with a more severe phenotype compared to males. This is the first description of genetic polymorphisms in a candidate gene for pectus excavatum. Overall, there are observed associations between the VNTR regions of ACAN, a potential candidate gene, with pectus excavatum.

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). VO2max during peak exercise increased by 10.1% (p = 0.015) and O2pulse 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 VO2max and O2pulse 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. © 2013 by the American College of Surgeons.

Background/Purpose: The purpose of this study is to discover whether a pediatric inguinal hernia surgical clinical pathway (CP) reduces the frequency of wound infections, return visits, times associated with surgical repair, or costs. Methods: A multidisciplinary team developed the inguinal hernia surgical clinical pathway. Healthy children greater than 50 weeks gestational age who required unilateral hernia repair were considered for the study. Two groups were formed: (1) an intervention group selected randomly (n = 46, CI=95%, power=.80) from patients enrolled from November 1996 through April 1997, and (2) a retrospective cohort control group (n = 46) matched to each intervention patient by age, gender, and medical history. Analysis of variance and χ 2 testing were used to test for significant differences between the 2 groups in postoperative wound infections, readmission and emergency department return visits within 72 hours, times associated with surgical repair, and costs. Results: There were no significant differences in postoperative wound infections, times associated with surgical repair, or readmission rates within 72 hours. Total cost significantly decreased, by 10% ( P ≤.05), for pathway patients ($982 v $880). Conclusion: These results show that the use of a pediatric inguinal hernia surgical clinical pathway is associated with reduced cost while maintaining quality of care. J Pediatr Surg 35:1045-1048. Copyright © 2000 by W.B. Saunders Company.

Minimally invasive repair of pectus excavatum is indicated for patients with a severe pectus excavatum deformity and associated physiologic impairment. Specific inclusion criteria include two or more of the following: Computed tomography (CT) index greater than 3.25, with associated cardiac or pulmonary compression Pulmonary function tests (PFTs) demonstrating restrictive and/or obstructive impairment Cardiology evaluation demonstrating cardiac compression, displacement, mitral valve prolapse, murmurs or conduction abnormalities Documentation of progression of the deformity with advancing age, in association with development or worsening of physiologic symptoms (i.e. shortness of breath, lack of endurance, exercise intolerance, palpitations or chest pain)

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.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).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).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.

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.

Surgical planners are used to achieve the optimal outcome for a surgery, especially in procedures where a positive aesthetic outcome is the primary goal, such as the Nuss procedure which is a minimally invasive surgery for correcting pectus excavatum (PE)--a congenital chest wall deformity. Although this procedure is routinely performed, the outcome depends mostly on the correct placement of the bar. It would be beneficial if a surgeon had a chance to practice and review possible strategies for placement of the corrective bar and the associated appearance of the chest. Therefore, we propose a strategy for the development and validation of a Nuss procedure surgical trainer and planner.

With the triumphal march of the surgical method (MIRPE) in the correction of pectus excavatum deformities, according to Donald Nuss [6] the method originally described by Ravitch in 1949 and 1958 [9, 10] was partially dislodged into the background and minor scope was left for special applications only. However the Ravitch technique is still widely used as a standard procedure in the correction of pectus carinatum deformities (Chapter 7.1) Although a recent study consisting of a systematic review and meta-analysis methodology confirmed that the complication rate in the MIRPE technique is higher than in the Ravitch technique, and the period of requirement of postoperative analgesics seems to be lower than in the MIRPE collectives, a clear difference concerning the aesthetic outcome could not be elaborated. It seems that particularly the parameters of pain and aesthetic result, being of paramount importance for the patient self, could not be studied comparatively due to too many biasing factors and lack of long-term comparability [1, 5]. Despite that the MIRPE technique offers a method requiring far shorter surgery time and represents an overall elegant method with however pleasing results lasting for many years. The elegancy and straightforward technique in experienced hands with relatively hidden scars supported its triumphal march so far, convincing patients and surgeons as well. However, these findings are predominantly true for children and adolescents, but must be regarded differentiated in adults. For the latter collective of patients no comparative data are yet available.

To review the technical improvements and changes in management that have occurred over 21 years, which have made the minimally invasive repair of pectus excavatum safer and more successful.In 1997, we reported our 10-year experience with a new minimally invasive technique for surgical correction of pectus excavatum in 42 children. Since then, we have treated an additional 1173 patients, and in this report, we summarize the technical modifications which have made the repair safer and more successful.From January 1987 to December 2008, we evaluated 2378 pectus excavatum patients. We established criteria for surgical intervention, and patients with a clinically and objectively severe deformity were offered surgical correction. The objective criteria used for surgical correction included computed tomography (CT) scans of the chest, resting pulmonary function studies (spirometry and/or plethysmography), and a cardiology evaluation which included echocardiogram and electrocardiogram. Surgery was indicated if the patients were symptomatic, had a severe pectus excavatum on a clinical basis and fulfilled two or more of the following: CT index greater than 3.25, evidence of cardiac or pulmonary compression on CT or echocardiogram, mitral valve prolapse, arrhythmia, or restrictive lung disease. Data regarding evaluation, treatment, and follow up have been prospectively recorded since 1994. Surgical repair was performed in 1215 (51%) of 2378 patients evaluated. Of these, 1123 were primary repairs, and 92 were redo operations. Bars have been removed from 854 patients; 790 after primary repair operations, and 64 after redo operations.The mean Haller CT index was 5.15 ± 2.32 (mean ± SD). Pulmonary function studies performed in 739 patients showed that FVC, FEV1, and FEF25-75 values were decreased by a mean of 15% below predicted value. Mitral valve prolapse was present in 18% (216) of 1215 patients and arrhythmias in 16% (194). Of patients who underwent surgery, 2.8% (35 patients) had genetically confirmed Marfan syndrome and an additional 17.8% (232 patients) had physical features suggestive of Marfan syndrome. Scoliosis was noted in 28% (340). At primary operation, 1 bar was placed in 69% (775 patients), 2 bars in 30% (338), and 3 bars in 0.4% (4). Complications decreased markedly over 21 years. In primary operation patients, the bar displacement rate requiring surgical repositioning decreased from 12% in the first decade to 1% in the second decade. Allergy to nickel was identified in 2.8% (35 patients) of whom 22 identified preoperatively received a titanium bar, 10 patients were treated successfully with prednisone and 3 required bar removal: 2 were switched to a titanium bar, and 1 required no further treatment. Wound infection occurred in 1.4% (17 patients), of whom 4 required surgical drainage (0.4% of the total). Hemothorax occurred in 0.6% (8 patients); 4 during the postoperative period and four occurred late. Postoperative pulmonary function testing has shown significant improvement. A good or excellent anatomic surgical outcome was achieved in 95.8% of patients at the time of bar removal. A fair result occurred in 1.4%, poor in 0.8%, and recurrence of sufficient severity to require reoperation occurred in 11 primary surgical patients (1.4%). Five patients (0.6%) had their bars removed elsewhere. In the 752 patients, more than 1 year post bar removal, the mean time from initial operation to last follow up was 1341 ± 28 days (SEM), and time from bar removal to last follow-up is 854 ± 51 days. Age at operation has shifted from a median age of 6 years (range 1-15) in the original report to 14 years (range 1-31). The minimally invasive procedure has been successfully performed in 253 adult patients aged 18 to 31 years of age.The minimally invasive repair of pectus excavatum has been performed safely and effectively in 1215 patients with a 95.8% good to excellent anatomic result in the primary repairs at our institution.

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.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.The percentages of patients with abnormal forced vital capacity (FVC), forced expiratory volume in 1 second (FEV(1)), 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 (FEV(1)/FVC67%), and 14.5% demonstrated a restrictive pattern (FVC and FEV(1)80% predicted; FEV(1)/FVC80%). Patients with a Haller index of 7 are4 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.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.

The Nuss procedure is a minimally invasive surgery for correcting pectus excavatum. Pectus excavatum (PE), also called sunken or funnel chest, is a congenital chest wall deformity which is characterized by a deep depression of the sternum. This condition affects primarily children and young adults and is responsible for about 90% of congenital chest wall abnormalities.

OBJECTIVE. This study evaluated changes in both physical and psychosocial quality of life reported by the parent and child after surgical repair of pectus excavatum. METHODS. As part of a multicenter study of pectus excavatum, a previously validated tool called the Pectus Excavatum Evaluation Questionnaire was administered by the research coordinator, via telephone, to parents and patients (8–21 years of age) before and 1 year after surgery. Eleven North American children's hospitals participated. From 2001 to 2006, 264 patients and 291 parents completed the initial questionnaire, and 247 patients and 274 parents completed the postoperative questionnaire. Responses used a Likert-type scale of 1 to 4, reflecting the extent or frequency of a particular experience, with higher values conveying less-desirable experience. RESULTS. Preoperative psychosocial functioning was unrelated to objective pectus excavatum severity (computed tomographic index). Patients and their parents reported significant positive postoperative changes. Improvements occurred in both physical and psychosocial functioning, including less social self-consciousness and a more-favorable body image. For children, the body image component improved from 2.30 ± 0.62 (mean ± SD) to 1.40 ± 0.42 after surgery and the physical difficulties component improved from 2.11 ± 0.82 to 1.37 ± 0.44. For the parent questionnaire, the child's emotional difficulties improved from 1.81 ± 0.70 to 1.24 ± 0.36, social self-consciousness improved from 2.86 ± 1.03 to 1.33 ± 0.68, and physical difficulties improved from 2.14 ± 0.75 to 1.32 ± 0.39. Ninety-seven percent of patients thought that surgery improved how their chest looked. CONCLUSIONS. Surgical repair of pectus excavatum can significantly improve the body image difficulties and limitations on physical activity experienced by patients. These results should prompt physicians to consider the physiologic and psychological implications of pectus excavatum just as they would any other physical deformity known to have such consequences.

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.

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.

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 (FEV 1 ), 89% of predicted; and forced expiratory flow during the middle half of the forced vital capacity (FEF 25% 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.

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.

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.