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[Complex anomalies of the anterior chest wall. Personalized surgical treatment].

Among the congenital anomalies of the anterior chest wall, the group of the asymmetrical deformities, mixed or complex, are the least frequent and the ones that require a difference surgical strategy for each patient according to their anatomical features. In this paper we present our experience and findings with this type of malformation. The fundamental aspects of the surgical indications and procedure are commented. The evolution and the results are very satisfactory. We stress the positive reaction of the patients and their families after the operation.

Adolescent↗

Surgical correction of pectus excavatum and carinatum.

The author presents three decades of experience in the management of anterior chest wall deformities. During this period more than 800 operations were performed on patients with pectus excavatum and carinatum. In this series, there was no death and serious complications were rare. The author believes that the principles on which surgical treatment of pectus excavatum should be based are as follows: (1) bilateral removal of the "culprit" costal cartilages, (2) adequate mobilization of the sternum and correction of the sternal positional deformity by transverse osteotomy, (3) stabilizing the corrected position of the sternum with a substernal "hammock" support. Using this technique the author developed new surgical techniques for the correction of different varieties of chest wall deformities: Pectus excavatum, asymmetric pectus excavatum, pectus carinatum with xiphoid angulation, horizontal pectus excavatum, asymmetric pectus carinatum, chondrosternal prominence with chondrogladiolar depression, and recurrent pectus excavatum. The present method applied for correction of pectus excavatum utilizes the above principles and a substernal Marlex mesh support with bilateral muscle coverage. For carinatum repair, the author routinely uses positional correction of the sternum and sternal shortening. Patients who have significant pectus deformities should undergo surgical repair, preferably between one and eight years of age.

Biocompatible Materials↗

Anterior chest wall deformities and congenital heart disease.

Pectus excavatum and pectus carinatum usually exist as isolated abnormalities. Only 19 cases of associated congenital heart defects have been reported. Significant complications related to uncorrected pectus excavatum have been described either during or after cardiac operations. Therefore we reviewed our experience with these coexisting lesions to assess the risk of surgical repair of chest wall deformities before and after correction of congenital cardiac anomalies. Among 20,860 infants and children with congenital heart disease seen at our institution, 36 (0.17%) had associated anterior thoracic deformities, 22 of whom underwent surgical correction of pectus excavatum or pectus carinatum. Ten of these 22 patients had pectus repair after a cardiac operation. Pleural or pericardial entry was avoided in all and none required a blood transfusion. Ten other patients had pectus repair either before cardiac repair (five patients) or without a subsequent cardiac operation. Another patient had a cardiac operation performed through a median sternotomy both before and after pectus repair, and the remaining patient, early in the series, had simultaneous banding of the main pulmonary artery and repair of pectus excavatum complicated by chest wall instability and a lethal intrathoracic hemorrhage. The experience indicates that congenital chest wall deformities can be safely and effectively repaired after early correction of congenital heart defects through a median sternotomy, although repair of the chest wall deformity after cardiac surgery also gives good results. However, in children who require an extracardiac conduit for repair of their congenital heart defect, we recommend initial repair of the pectus excavatum followed at 6 weeks or later by repair of the cardiac lesion to eliminate possible extrinsic compression of the conduit by the depressed sternum. We avoid simultaneous cardiac and pectus excavatum repair because of potential associated major complications.

Adolescent↗

Endoscopically assisted pectus excavatum repair.

We present an endoscopic approach for the reconstruction of pectus excavatum with a custom silicone implant. The procedure incorporates endoscopic techniques to facilitate dissection of an extensive subcutaneous pocket through a 6-cm Chevron skin incision 8 cm below the level of the xiphoid. The incision is designed based on the size, shape, and flexibility of the custom implant and the configuration of the chest wall deformity. A superiorly based rectus abdominis anterior fascial sheath flap then is elevated up to the caudal-most margin of the implant, creating a sling below the implant, thus stabilizing its position and preventing direct communication with the overlying skin incision. In our patient, the endoscope permitted insertion of the custom implant while minimizing the length of incision. The cosmetic result using a minimally invasive approach to assist with the dissection was acceptable, and the morbidity and scarring were minimized.

Adolescent↗

Customized silicone implants of the breast and chest.

Custom-fabricated silicone implants were used in 17 patients to reconstruct thoracic deformities associated with pectus excavatum, hypoplasia of the thorax, and radical mastectomy. The implants have been in place an average of 2 1/2 years. Custom implants have been permanently removed in only two of these patients. These implants can provide accurate, complex contour restoration without creating donor defects. However, problems of extrusion, capsule formation, slippage, and infection requiring implant removal are characteristic of nonautogenous materials. Because these are foreign bodies, well-vascularized soft-tissue coverage is especially essential to retain these implants.

Adolescent↗

Reliability of a standardized protocol to calculate cross-sectional chest area and severity indices to evaluate pectus excavatum.

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 kappa'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.

Adolescent↗

Preliminary study on the effects of bar placement on the thorax after the nuss procedure for pectus excavatum using bone scintigraphy.

AIM: Bone scintigraphy was performed to elucidate the effects of the Nuss procedure for pectus excavatum on the bony thorax. METHODS: Eight boys and 6 girls (5 - 24 years of age) underwent bone scintigraphy, using (99m)Tc-HMDP. Eleven patients were studied 5 to 21 days after the Nuss procedure; 6 were studied 20 to 24 months after the operation before bar removal. Three of 14 were studied twice after the Nuss procedure and before bar removal. RESULTS: In the early postoperative phase, RI accumulation was found at the sternum and ribs in only 1 of 6 patients under 9 years of age, whereas in all 5 older patients, RI had accumulated at the sternum. Scintigrams before bar removal revealed, regardless of age, hot spots at the lateral ribs in contact with the bar and at the costochondral junctions where the bar passed through the intercostal spaces. Furthermore, chest roentgenograms showed the deformed lateral ribs in contact with the bar. CONCLUSIONS: The Nuss procedure creates minute fractures at the sternum and the ribs, especially in older patients. The bar deforms the ribs and restrains the growth of the thorax. Furthermore, it constantly rubs against the ribs and can therefore cause late complications. Bone scintigraphy may determine the appropriate timing for bar removal.

Adolescent↗

The use of absorbable material in correction of pectus deformities.

Various techniques have been described for correction of the congenital anterior chest wall deformities. We described a new technique in which 33 patients were used in correction of those deformities. In this new technique we used the resorbable copolymer plaque and polymer screw for the fixation of the sternum. We have obtained excellent results in one-year follow up period. This technique was developed to avoid a second operation, which usually requires for removal of the metal support. Resorption of copolymer plaques and polymer screws are completed at the end of the first year.

Absorption↗

Airway compression in children with abnormal thoracic configuration.

PURPOSE: To assess the clinical and imaging findings in children with severe tracheal or bronchial compression caused by abnormal thoracic configuration. MATERIALS AND METHODS: Clinical and imaging findings, care, and outcome were reviewed in six patients with airway compression caused by abnormal thoracic configuration. Radiographs and magnetic resonance (MR) images were available in all patients. RESULTS: Mechanisms of airway compression included alteration of mediastinal anatomy and direct airway compression secondary to a narrow anteroposterior chest diameter. In three patients, the trachea was compressed at the level of the thoracic inlet. In the other three patients, the left main bronchus was compressed. Four patients underwent surgery for the thoracic deformity, with resolution of airway symptoms. In all four patients, the presence, location, and cause of airway obstruction determined at MR imaging were confirmed at surgery. CONCLUSION: Abnormal thoracic configuration, in addition to causing restrictive lung disease, can cause respiratory distress secondary to severe compression of the central airways.

Adolescent↗

Present tendencies in the surgical treatment of pectus excavatum.

Among the malformations of the chest wall, pectus excavatum is undoubtedly the most frequent. It may appear in various forms and may show varying degrees of severity. Surgical correction by operation is mostly sought by the patients for aesthetic reasons. After careful review of the methods proposed by various writers for the correction of this malformation, the present writers emphasize the advantages and disadvantages of these techniques, especially in relation to the methods of stabilising the reduction. Finally, they report on their own case material and present some significant examples.

Adolescent↗

Outcome of pectus excavatum in patients with Marfan syndrome and in the general population.

We reviewed the records of 28 patients with Marfan syndrome and 30 age-matched control patients with presumed isolated pectus excavatum to determine the outcome of surgical repair of the pectus deformity in Marfan syndrome. One third of the patients with Marfan syndrome underwent repair of the pectus excavatum before diagnosis. Of the 30 patients with "isolated" pectus excavatum, 17 had findings by history or physical examination, such as mitral valve prolapse, scoliosis, or a relative with pectus excavatum, suggestive of an underlying disorder of connective tissue. Pectus excavatum of more than moderate severity recurred in 11 of 28 patients with Marfan syndrome and was associated with young age at initial surgery and lack of temporary internal stabilization of the chest after surgery. Only two of the control patients had recurrence of the defect; one of these patients had findings suggestive of an underlying heritable disorder of connective tissue. We conclude that pectus excavatum may indicate the presence of an underlying heritable disorder of connective tissue such as the Marfan syndrome. In patients with Marfan syndrome, and possibly other inherited connective tissue disorders, surgical repair should be delayed if possible until skeletal maturity is nearly complete and should employ internal stabilization.

Adolescent↗

Reconstruction of congenital chest-wall deformities.

Pectus deformities and Poland's syndrome are two relatively common congenital deformities of the chest wall that are amenable to reconstruction. The extent of the structural deformity in pectus deformity and the degree of associated cardiopulmonary dysfunction are critical variables in preoperative assessment. The operative approaches range from more extensive sternal eversion to the more popular subperichondrial cartilage resection with or without internal fixation. In Poland's syndrome, the options for reconstruction include anterior transfer of the ipsilateral latissimus dorsi muscle through a transaxillary tunnel and attachment to the clavicle and sternum. Submuscular insertion of a mammary prosthesis can be added in the female patient.

Female↗

Pectus excavatum with inspiratory inferior vena cava compression: a new presentation of pulsus paradoxus.

A 29-year-old man with pectus excavatum presented with exercise intolerance, pulsus paradoxus, and paradoxically split S2. Chest computed tomography (CT) showed the heart shifted leftward and a pectus severity index of 7.18. Cardiopulmonary exercise study showed reduced VO2max, anaerobic threshold, and oxygen pulse. Echocardiography revealed a decline in mitral and tricuspid valve inflow, and stroke volume during inspiration. Cardiac extrinsic compression and anatomic cardiac abnormalities were not present. Dynamic magnetic resonance imaging (MRI) demonstrated inspiratory inferior vena cava (IVC) compression at the diaphragm. We discuss IVC compression by the diaphragm as a source of patient symptoms and as a mechanism for pulsus paradoxus associated with pectus excavatum.

Adult↗

Pectus excavatum.

One of the more common chest wall anomalies seen in children is pectus excavatum. Although some studies suggest a physiologic impact of this anomaly on cardiac function during exercise, this remains somewhat controversial. Regardless, a number of children are symptomatic from either the appearance of the deformity or from the standpoint of tolerance to exercise. Most are relieved of these symptoms with surgical repair. Several different operations have been utilized for repair of this anomaly, but two techniques now are the predominant methods in use today. These are the modified Ravitch procedure and the Nuss procedure. Both have been shown to provide satisfactory results.

Funnel Chest↗

A pilot study of the impact of surgical repair on disease-specific quality of life among patients with pectus excavatum.

BACKGROUND: This study was conducted to determine the ability of 2 questionnaires (ie, child and parent versions) to measure physical and psychosocial quality-of-life changes after surgical repair of pectus excavatum. METHODS: The authors administered these questionnaires by telephone interviews with 22 parents and 19 children (ages 8 to 18) before surgery and 6 to 12 months after repair by the Nuss procedure. RESULTS: The instruments had high test-retest reliability (Rho > 0.6 for all retained questions). Children reported significant improvements in exercise intolerance, shortness of breath, and tiredness. Of 9 questions asking the children how they feel or act about their bodies, all but one question showed significant improvement after surgery. Parents also reported significant improvements in their child's exercise tolerance, chest pain, shortness of breath, and tiredness and decreases in the frequency of the child being frustrated, sad, self-conscious, and isolated. CONCLUSIONS: These questionnaires appear to be more than adequate to measure disease-specific quality-of-life changes after surgery. These data confirm for the first time that surgical repair of pectus excavatum has a positive impact on both the physical and psychosocial well-being of the child.

Adolescent↗

The Nuss procedure for pectus excavatum correction.

Pectus excavatum is an anterior chest wall deformity that now can be corrected with a minimally invasive technique known as the Nuss procedure. Patient criteria and assessment for this new surgical procedure are defined clearly in advance to ensure the need for surgical intervention. A multidisciplinary team approach has been established at Children's Hospital of The King's Daughters, Norfolk, Va. Team members work cooperatively throughout the perioperative cycle, addressing not only the surgical procedure but also pain management and postoperative recovery. This dedicated team approach helps ensure a successful outcome for the patient.

Adolescent↗

[Spontaneous evolution of pectus excavatum deformity in children: a five-year prospective study].

UNLABELLED: The pathophysiologic effects of pectus excavatum (PE) are controversial. Impaired respiratory performance during exercise is minimal and several studies evaluating cardiovascular function have shown conflicting results. There is no evidence that impaired cardiopulmonary performance, if any, is improved by surgical correction of the deformity. Spontaneous improvement or even disappearance of the deformity have been observed, but not extensively studied. AIM OF THE STUDY: The aim of the present study was to evaluate the spontaneous evolution of PE during the growth period (age 11 to 15 years), as well as the aesthetic component of the deformity based upon the judgment of the subjects, in connection with the evolution of PE and its depth. METHOD: A prospective study included 52 boys who were followed-up on a yearly basis (the first examination at the age of 11, the last at the age of 15 years). Two sagittal diameters of the chest were measured: the first at the deepest point of PE, the second over the flexible metal ruler in order to determine the hypothetic chest diameter without depresion. Index of the deformity was calculated by dividing the diameters and multiplying the result by 100. All results are expressed as mean +/- SD. Comparison of initial and follow-up studies for the same individual were compared with the paired t-test. RESULTS: Statistically significant improvement of the deformity (p<0.05) was observed in 35 subjects and differences between yearly measurements were not significant. In 17 boys the depression deteriorated significantly after five years (p<0.02). The differences between yearly measurements were not significant, except between years 12 and 13. At the age of fifteen, 38.5 per cent of children were satisfied with the appearance of the chest and 61.5 per cent were unsatisfied. The self-perception was not correlated to either spontaneous improvement or deterioration, or the depth of depression. DISCUSSION: In spite of numerous studies, pathophysiologic effects of PE and postoperative results are still a matter of controversy. The others believe that psychologic problems are the only indication for surgery, but spontaneous evolution of PE and its psychologic effects have not been extensively studied. The present study demonstrated unpredictable evolution of the deformity ant its complex impact on the psychology of children in puberty, the facts to be considered when contemplating surgery.

Adolescent↗

Tracheal compression caused by straight back syndrome, chest wall deformity, and anterior spinal displacement: techniques for relief.

BACKGROUND: Straight back syndrome and other causes of extreme narrowing of the space between sternal notch and vertebrae can cause critical tracheal obstruction. Additional points of compression may result from the brachiocephalic artery and from anterior vertebral displacement. METHODS: Individualized surgical maneuvers are necessary to correct all points of obstruction. Techniques include sternoplasty, sternal division, reimplantation of brachiocephalic artery, correction of severe pectus excavatum, and posterior wall tracheoplasty. RESULTS: Four patients were successfully treated by individualized techniques with complete long-term relief of critical tracheal obstruction. CONCLUSIONS: Severe tracheal compression caused by straight back syndrome and other causes of narrowed sternospinal channel is surgically correctable.

Adult↗