Reactive pectus carinatum in patients treated for pectus excavatum

Purpose

The Ravitch and minimally invasive Nuss procedures have brought widespread relief to children with pectus excavatum, chest wall deformities, over the last half century. Generally accepted long-term complications of pectus excavatum repair are typically limited to recurrence of the excavatum deformity or persistent pain. This study examines the authors' experience with patients who develop a subsequent carinatum deformity within 1 year of pectus excavatum repair.

Methods

The authors retrospectively assessed the charts of all patients diagnosed as having a carinatum deformity subsequent to treatment for pectus excavatum at a tertiary urban hospital. We noted age at original correction of pectus excavatum, time from original correction to diagnosis of carinatum deformity, age at correction of carinatum deformity, complaints before correction, methods of repair, postoperative complications, and we reviewed relevant radiography.

Results

Three patients who underwent pectus excavatum repair between January 2000 and August 2007 developed a subsequent carinatum deformity. Two patients initially underwent minimally invasive Nuss correction of pectus excavatum; 1 patient underwent the Ravitch procedure. Within 1 year of original correction and despite intraoperative achievement of neutral sternal position, a protruding anterior chest deformity resembling de novo pectus carinatum emerged in each patient; we term this condition reactive pectus carinatum. The mean age of patients undergoing initial pectus excavatum repair was 13 years (range, 11-16 years). The pathophysiology of this reactive lesion is not well understood but is thought to originate from reactive fibroblastic stimulation as a result of sternal manipulation and bar placement. Patients who underwent Nuss correction initially were managed with early bar removal. Two of the patients eventually required surgical resection of the carinatum deformity at a time interval of 3 to 6 years after initial excavatum repair. In one patient, the carinatum deformity resolved spontaneously. Neutral chest position and absence of dyspenic symptoms were achieved in all patients.

Conclusions

Reactive pectus carinatum is functionally encumbering and a poor cosmetic complication of either the Ravitch or minimally invasive Nuss procedures. Our experience with reactive pectus carinatum introduces the importance of postoperative vigilance even in patients without underlying fibroelastic disease. Examination of the chest with attention to the possibility of an emerging carinatum deformity, particularly in the first 6 postoperative months, is paramount. A telephone call to the patient at 3 months may be a useful adjunct to clinic visits. An optimal long-term result may be achieved through a combination of early Nuss bar removal or postpubertal pectus carinatum repair.     

Simultaneous repair of pectus excavatum and congenital heart defect in adults by using the convex bar

Simultaneous repair of pectus excavatum and cardiac lesions remains technically difficult. In adults, most repairs of pectus deformity and heart lesions have been performed through long incisions, sternal splits, excision of deformed cartilages, and sternal turnover, which can result in poor cosmetic appearance because of sternal devascularization. We performed concomitant repair of pectus excavatum and an atrial septal defect through a short midline incision in an adult. The sternum was fixed by using absorbable plates and screws and was supported by a convex steel bar. The cosmetic appearance remained excellent after the operation. The technique and a review of the literature are included.     

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.     

Scoliosis in children with pectus excavatum and pectus carinatum

Between 1974 and 1985, 461 patients with pectus excavatum and 135 patients with pectus carinatum underwent operative repair of their anterior chest wall deformities. Twenty-one percent of patients with anterior chest wall deformity had mild scoliosis by clinical and radiographic examination. The average lateral spinal deformity was 15 degrees (range 6-78 degrees) for pectus excavatum patients and 16 degrees (range 5-57 degrees) for pectus carinatum patients. Eighteen percent of the pectus excavatum patients with scoliosis and 14% of the pectus carinatum patients with scoliosis required therapeutic intervention of bracing and/or arthrodesis.     

Repair of pectus excavatum and carinatum deformities in 116 adults

OBJECTIVE: To determine the feasibility of surgically correcting pectus excavatum and carinatum deformities in adult patients. SUMMARY BACKGROUND DATA: Although pectus chest deformities are common, many patients progress to adulthood without surgical repair and experience increasing symptoms. There are sparse published data regarding repair of pectus deformities in adults. METHODS: Since 1987, 116 patients over the age of 18 years with pectus excavatum (n = 104) or carinatum (n = 12) deformities underwent correction using a highly modified Ravitch repair, with a temporary internal support bar. The ages ranged from 19 to 53 years (mean 30.1). Eighty-six patients sought repair after reviewing information regarding pectus deformities available on the Internet. Each patient experienced dyspnea with mild exertion and decreased endurance; 84 had chest pain with activity; 75 had palpitations and/or tachycardia. Seven patients underwent repair for symptomatic recurrent deformities. The mean severity score (chest width divided by distance from sternum to spine) was 4.8. The sternal bar was removed from 101 patients 6 months after the repair without complications. RESULTS: Each of the patients with reduced endurance or dyspnea with mild exercise experienced marked improvement within 6 months. Chest discomfort was reduced in 82 of the 84 patients. Complications included pleural effusion (n = 7), pneumothorax (n = 2), pericarditis (n = 2), dislodged sternal bar (n = 3), and mildly hypertrophic scar (n = 12). Mean hospitalization was 2.9 days; mean blood loss was 122 mL. Pain was mild and of short duration (intravenous analgesics were used a mean of 2.1 days). There were no deaths. With a mean follow-up of 4.3 years, 109 of 113 respondents had a very good or excellent result. CONCLUSIONS: Although technically more difficult than in children, pectus deformities may be repaired in adults with low morbidity, short hospital stay, and very good physiologic and cosmetic results.     

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.     

Repair of pectus excavatum and carinatum in adults

BACKGROUND: There is sparse published information regarding the repair of pectus chest deformities in adults. This report summarizes our clinical experience with the surgical repair of pectus excavatum and carinatum deformities in 25 adults. METHODS: During the past 11 years, 25 patients 20 years of age or older (mean 31) with symptomatic pectus excavatum (23) or carinatum (2) deformities underwent surgical repair using a temporary internal sternal support bar. RESULTS: Each of the patients with decreased stamina and endurance or dyspnea with exercise experienced marked clinical improvement within 4 months postoperation. Exercise-induced asthma was improved in 6 of 7 patients; chest pain was reduced in each of 9 patients. Postoperative complications included pneumothorax (1), keloid (2), and discomfort from sternal bar (2). The sternal bar was removed 7 to 10 months postoperation in 19 patients; there has been no return of preoperative symptoms or recurrent depression in any patient with a mean follow-up of 4.8 years. CONCLUSIONS: For adults who have symptoms and activity limitations related to uncorrected pectus chest deformities, surgical repair can be performed with low morbidity, low cost, minimal limitation in activity, and a high frequency of symptomatic improvement. The operation in adults is more difficult than in children, although the results are similar.     

Surgical correction of pectus excavatum and carinatum.

This paper contains an analysis of the long-term results in 85 patients who had pectus excavatum or carinatum deformities repaired at the North Middlesex Hospital between 1951 and 1977. Seventy-seven patients had operations for correction of pectus excavatum and eight for pectus carinatum. A variety of surgical techniques was used. In the excavatum deformities the best results were obtained by the extensive resection of all deformed cartilages, the correction of the sternal deformity by a simple transverse wedge osteotomy, and by stabilising the chest with a stainless steel plate. For pectus carinatum, the involved cartilages were resected and an osteotomy of the sternum was performed. We preferred in most cases to stabilise the chest wall with a metal strut in this deformity as well. The best cosmetic results were achieved by the use of a stainless steel plate passed beneath the sternum and left for not more than six months.     

Technical considerations in the surgical management of pectus excavatum and carinatum

During the past 25 years, 650 operations have been performed on 608 patients for anatomically significant pectus excavatum or carinatum deformities of the anterior chest wall. There were no deaths in this series, and serious complications were very rare.We conclude that repair of pectus excavatum and carinatum deformities should include the following operative steps: (1) adequate mobilization of the sternum and correction of its abnormal angulation by transverse osteotomy; (2) adequate bilateral removal of the involved costal cartilage; and (3) securing the corrected position of the sternum with the patient's own living tissue, retaining its blood supply and using it as an internal support.Using these principles, new surgical procedures were developed for the correction of: symmetrical pectus excavatum, asymmetrical pectus excavatum, pectus carinatum with xiphoid angulation, pectus carinatum without xiphoid angulation, asymmetrical pectus carinatum, chondromanubrial prominence with chondrogladiolar depression, and recurrent pectus excavatum.We recommend surgical correction for patients in whom the deformity is significant and no contraindication exists. The ill effects of this condition should not be underestimated.     

Repair of the pectus deformity: results of the Ravitch approach in the current era

Background

Recent publications have advocated a minimally invasive approach to repair of the pectus deformity. Efforts to evaluate this new approach have been hampered by lack of comparative information regarding outcomes of the standard Ravitch approach. We use a modified Ravitch procedure, and present our series as a basis for comparison.

Methods

Records of 69 consecutive patients undergoing repair of the pectus deformity were retrospectively reviewed. Modifications included a minimal incision and a new technique to address sternal angulation. A patient satisfaction survey evaluated the patients' perception of the outcome.

Results

We found one wound infection (1.4%). Five patients (7.2%) had a seroma, and were treated as outpatients. Because the minimally invasive approach is used for pectus excavatum, we divided our series into excavatum and carinatum subsets. The subset of 44 pectus excavatum patients had a mean postoperative length of stay (LOS) of 2.9 days. The median patient satisfaction score was 4 on a scale of 1 to 5, at an average of 4.75 years after repair. The subset of 25 pectus carinatum patients had a mean LOS of 2.4 days and a median patient satisfaction score of 5.

Conclusions

The modified Ravitch procedure yields excellent results with low morbidity, hospital LOS, and cost, combined with high patient satisfaction. These current data will be useful for comparison as newer techniques for pectus repair continue to evolve.     

Dysmorphology of Chest Wall Deformities: Frequency Distribution of Subtypes of Typical Pectus Excavatum and Rare Subtypes

Background/PurposeMore 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.MethodsA 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.ResultsTypical 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.ConclusionsTypical 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.     

Minimally invasive repair of pectus excavatum in adult patients

Since 1996, the technique for minimally invasive repair of pectus excavatum (MIRPE) has gained increasing acceptance among pediatric patients. However, the feasibility of the operation and outcomes have not yet been evaluated in adult patients. This study was a retrospective analysis of the author's experience combined with a survey of members of the American Pediatric Surgical Association in treating adult patients with MIRPE. Thirty adults (age range, 18-32 years; mean, 23 years; 75% men) with severe pectus excavatum (chest index > 3.2) were treated with MIRPE. The main indication for surgery was cosmetic (80%). One 32-year-old female patient underwent simultaneous MIRPE and breast augmentation. In 60 per cent of cases, the operative time was 1 to 2 hours. Thoracic epidural was successfully used for postoperative pain management in 90 per cent of patients. Two pectus bars were necessary in 16 per cent of cases, and bilateral stabilizers were used in 53 per cent of patients. Complications included seroma (10%), bar displacement (6%), pneumothorax requiring tube thoracostomy (6%), superficial wound infection (3%), and stabilizer bar fracture (3%). Two patients required conversion to modified Ravitch repair. Patient satisfaction was rated as excellent (50%), good (36%), and fair (14%). Less than 50 per cent of patients achieved 100 per cent correction of their deformity. MIRPE can be used safely for repair of pectus excavatum in adult patients. The complication rate appears to be similar to previously reported series of pediatric patients. Although adult patients may have residual asymmetry of the chest postrepair, overall satisfaction with the repair was very good or excellent in 86 per cent of patients.     

Emergent pectus excavatum repair after aortic root replacement in Marfan patient

Chest wall deformities, including pectus excavatum, can complicate cardiac operations by impeding sternal entry and cardiac exposure and by affecting cardiac hemodynamic performance. We describe a patient with Marfan syndrome who underwent elective replacement of an aortic root aneurysm. The patient required a simultaneous, unplanned, emergent repair of her severe pectus excavatum because of hemodynamic instability after sternal closure. The success of this procedure suggests that emergent single-stage repair of pectus excavatum should be considered when median sternotomy closure precipitates hemodynamic instability in such patients.     

Surgical correction of pectus carinatum

Pectus carinatum is an infrequent but eminently correctable chest wall deformity. It is encountered much less frequently than pectus excavatum. In 12 years, from 1973 to 1985, 152 pectus carinatum (16.7%) and 758 pectus excavatum deformities (83.3%) were corrected. It occurs more frequently in boys (119 patients) than girls (33 patients). The majority, 89 cases, were symmetric, while 49 were asymmetric, and 14 were mixed deformities (ipsilateral carinatum, contralateral excavatum). In almost half the patients the deformity was not identified until after the 11th birthday. A family history of chest wall deformities was present in 26%, and of scoliosis in 12%. Associated musculoskeletal abnormalities were identified in 34 patients (scoliosis 23, Poland's syndrome 4, neurofibromatosis 2, Morquio's disease 2, vertebral anomalies 1, hyperlordosis 1, and kyphosis 1). Surgical correction required bilateral resection of the third through seventh costal cartilages in 143 patients, and unilateral resection in nine patients with an isolated abnormality. A single osteotomy was used in 88 patients and a double osteotomy in 53 patients. In 11 cases no osteotomy was required. Mixed deformity with posterior angulation of the sternum was managed by osteotomy and anterior displacement. The remaining cases had sternal osteotomy and fracture of the posterior cortex to correct anterior angulation. The operation was completed with a low complication rate 3.9% (pneumothorax 4, wound infection 1, atelectasis 1, and local tissue necrosis 1). Three patients required revision with additional unilateral lower cartilage resection for persistent malformation of the costal arch. All patients ultimately had a satisfactory result.     

Simultaneous repair of pectus excavatum and congenital heart disease over the past 30 years

Objective: Pectus excavatum may be present in patient requiring operations for cardiac defects. The study was undertaken in order to assess our simultaneous repairs of pectus excavatum and congenital heart disease over past 30 years. Methods: Between 1970 and 2000, 12 patients underwent simultaneous repair of pectus excavatum and congenital heart disease. Six of 12 patients had ventricular septal defects as cardiac malformations (subgroup A). Operative technique, after the intracardiac procedure using cardiopulmonary bypass, consists of total subperichondrial resection of deformed costal cartilages, transection of deformed portion of the sternum in 2–3 points, and fixation of the sternum in elevated position using two Kirschner wires and a bridge external traction. Postoperative catheterization was performed in five of 12 patients (subgroup B). We evaluated the operative data, the improvement of pectus deformity and right ventricular performance retrospectively. The operative data in subgroup A were compared with those in recent random patients with ventricular septal defects only or with pectus excavatum only (control groups). Results: There was no operative death and non-serious complications were seen in nine patients (atelectasis in six, superficial wound infection in two, chylothorax in one). Pectus deformities improved with the drop of vertebral index postoperatively. The mean total operative time and postoperative drainage in subgroup A were 128.4% and 123.7%, respectively of those in the ventricular septal defect control group. The mean perioperative bleeding in subgroup A was more than the sum of those in control groups. Right ventricular end-diastolic (RVEDVI), end-systolic (RVESVI), stroke (RVSVI) volume indices and ejection fraction (RVEF) in subgroup B tended to increase after surgery. In particular, there were significant increases of RVEDVI (35%, P<0.05) and RVSVI (77%, P<0.01). Conclusion: Simultaneous cardiac and pectus repairs were performed successfully without serious complications. Moreover, simultaneous repair resulted in an improvement of right ventricular performance with significant increases of RVEDVI and RVSVI.     

Cardiopulmonary resuscitation in patients with a Nuss bar--a case report and review of the literature

Pectus excavatum (PE) is a common chest wall deformity that may produce a variety of physiological and psychological effects in children and adolescents. In addition, some of these patients have associated cardiac diseases (ie, mitral valve prolapse and Marfan syndrome). Recently, a minimally invasive surgical repair of PE that requires sternal bar placement has become increasingly frequent to enhance patients' cardiopulmonary functioning as well as their self-esteem. However, despite this innovative technique, it is possible for such patients to have a cardiac arrest while their sternal bar is in place. Whether the presence of a metal bar on the underside of their sternum may hinder resuscitative chest compressions (cardiopulmonary resuscitation) is an issue that concerns us, our patients, and their families; the answer requires further investigation. We present a 21-year-old man with PE who underwent a minimally invasive pectus repair but had a fatal cardiac event before bar removal. Paramedics conducting cardiopulmonary resuscitation on the patient later reported that they were unable to deliver effective cardiac compressions and that the sternal bar may have contributed to this.     

Minimally invasive pectus surgery

The technique of minimally invasive repair of pectus excavatum is a new operation that allows for repair of this deformity without any cartilage resection or sternal osteotomy. The procedure has revolutionized the management of pectus excavatum. The innovative incorporation of thoracoscopic techniques and small but important modifications to the technique have made this operation very effective and safe.     

Thirty-year experience with repair of pectus deformities in adults

BACKGROUND: A plethora of studies have described repair of pectus deformities in children, but only few reports have described this repair in adults. The purpose of this study was to review our 30-year experience with surgical repair of pectus deformities in adults. METHODS: A retrospective review of all adult patients (> 16 years old) who underwent repair of congenital pectus deformities from 1971 through 2001. RESULTS: There were 77 patients, 64 men and 13 women. Sixty-eight patients underwent surgery for pectus excavatum and 9 for pectus carinatum; median age was 22 years old (range, 16 to 68 years old). Indication for repair was medical concerns in all patients. Preoperative symptoms were dyspnea on exertion in 43 patients, shortness of breath at rest in 22 patients, chest pain in 8 patients, and palpitations in 8 patients. Preoperative electrocardiogram findings included right bundle branch block in 9 patients, sinus bradycardia in 8 patients, left atrial enlargement in 6 patients, and right atrial dilatation in 5 patients. Patterns of the pectus defect were symmetric and localized in 29 patients, symmetric and diffuse in 21, asymmetric and localized in 18, and asymmetric and diffuse in 9 patients. Intraoperative classifications were severe in 38 patients, moderate in 33 patients, and mild in 6 patients. There were no operative deaths. Complications occurred in 11 patients (14.3%). Mean hospital stay was 4 days (range, 2 to 8 days). Mean follow-up was 12 +/- 7 years (range, 4 months old to 24 years old); 1 patient (1.3%) required reoperation for recurrent pectus excavatum. Patient satisfaction and relief of medical symptoms was excellent in 70 patients (90.9%), good in 6 patients, and fair in 1 patient. CONCLUSIONS: Repair of congenital defects of the sternum in adults can be performed safely with low morbidity and no mortality. Long-term results are excellent with requirement for reoperation rare.     

Simultaneous costal cartilage-sparing modified Ravitch procedure and latissimus dorsi transfer for chest wall deformity repair in Poland's syndrome

Poland's syndrome is a constellation of rare congenital anomalies that include hypoplasia of breast and underlying subcutaneous tissue, absence of the costosternal portion of the pectoralis major muscle, deformity or absence of ribs, absence of axillary hair, and syndactyly. Various surgical techniques have been described to repair such chest wall defects. We report a case of simultaneous Fonkalsrud procedure (costal cartilage-sparing version of the modified Ravitch procedure) and latissimus dorsi transfer in a 15-year-old boy with Poland's syndrome. The Fonkalsrud procedure has been used in the repair of pectus excavatum and pectus carinatum, and latissimus dorsi muscle transfer has been used in the repair of Poland's syndrome. In this report, we describe their combined use in an adolescent with severe pectus excavatum associated with Poland's syndrome. This combination of established operations resulted in a successful outcome.     

Chest wall deformities

BACKGROUND: Pectus deformities and atypical costal anomalies are congenital thoracic wall defects that can cause a marked cosmetic defect with attendant psychological trauma and limited physical performance. PATIENTS AND METHODS: We reviewed 43 patients with chest wall deformities, 24 (55.8%) were pectus excavatum, 13 (30.2%) pectus carinatum and 6 (14%) atypical costal anomalies, in the last sixteen years. There were nine female and 34 (79.1%) male patients. The mean age of the patients was 14.4 years (range, 5 to 23). Scoliosis (13.5%), Poland's syndrome (5.4%), Marfan's syndrome (5.4%), neurofibromatosis (2.7%), atrial septal defect (2.7%) and mitral valve prolapse (13.5%) were associated with pectus deformities. The modified Ravitch's technique was used in pectus cases. Concomitant surgery was performed in two patients with pectus carinatum. RESULTS: The complications of pectus deformity repair were pneumothorax (24.3%), wound infection (8.1%), and local tissue necrosis (2.7%). There was no major recurrence, while minor recurrence rate was 10.8%. There was no mortality. CONCLUSION: Timely surgical procedures for the treatment of pectus deformities result in an excellent cosmetic outcome and improve cardiorespiratory function, providing both physical and psychological benefits.