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Ann Thorac Surg 1999;67:511-518
© 1999 The Society of Thoracic Surgeons
a Division of Cardiovascular-Thoracic Surgery, Department of Surgery, Childrens Memorial Hospital, Northwestern University Medical School, Chicago, Illinois, USA
Address reprint requests to Dr Backer, Childrens Memorial Hospital, 2300 Childrens Plaza, MC22, Chicago, IL 60614
e-mail: c-backer{at}nwu.edu
Presented at the Forty-fifth Annual Meeting of the Southern Thoracic Surgical Association, Orlando, FL, Nov 1214, 1998.
| Abstract |
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Methods. From 1972 through 1998, 120 children underwent pectus deformity repair. Operative technique used a vertical midline incision with subperichondrial resection of deformed cartilages and an anterior sternal osteotomy. Thirty-five patients had a temporary metal bar for retrosternal support for 6 months; 85 underwent repair without a bar. Patients and parents were asked to assess the outcome after pectus repair as poor, fair, good, or excellent.
Results. There were 94 male and 26 female patients (mean age, 8.4 years; range, 3 to 21 years). There were 111 cases of pectus excavatum and 9 of pectus carinatum. Fourteen children (11.5%) had an associated congenital heart defect; 9 patients had simultaneous pectus and intracardiac repair. One patient was referred for emergent open heart repair and pectus repair after attempted "Nuss" repair resulted in a perforated right atrium, perforated right ventricle, and partially disrupted tricuspid valve apparatus. There were no deaths and only one significant complication, which required a return to the operating room for bleeding. Morbidity was not higher in patients with simultaneous intracardiac repair. Long-term follow-up was established in 83% of patients. Results were classified as excellent in 64 patients (64%), good in 25 (25%), fair in 8 (8%), and poor in 3 (3%). Thirty (86%) of 35 patients with a sternal bar had excellent results versus 34 (52%) of 65 without a bar (p = 0.004); 97% of patients who underwent repair with a sternal bar classified the result as excellent or good.
Conclusions. Long-term results of pectus excavatum and carinatum repair through a vertical midline approach are excellent. Outcome with a temporary sternal bar is superior to outcome without a bar. Concomitant repair of congenital heart defects and pectus deformity may be performed successfully without additional morbidity.
| Introduction |
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| Patients and methods |
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Operative technique
Operative technique used a limited vertical midline incision directly over the defect (Fig 1a). Use of a vertical (rather than horizontal) incision has been used in our division since the time of Willis Potts [10]. Bilateral skin, pectoralis, and rectus muscle flaps are mobilized (Figs 1b, 1c). Bilateral subperichondrial resection of all deformed cartilages (usually cartilages 3 to 6 or 3 to 7) is then performed with a Freer elevator (Fig 2). The xyphoid is freed from the rectus attachments. The intercostal muscle bundles and perichondrial sheaths are dissected free of the sternum with electrocautery (Fig 3). The cephalad extension of the sternal dissection is usually to a point just inferior to the second cartilage, at the sternalmanubrial junction. For pectus excavatum deformities, an anterior sternal osteotomy is performed with resection of a triangular wedge of sternum just inferior to the last normal costal cartilage, leaving the posterior cortex intact (Fig 4a). The posterior table is fractured and angulated anteriorly, without displacement, to maintain an adequate blood supply (Fig 4b). Two heavy Ticron (Davis & Geck, Manati, PR) sutures are placed to close the wedge after the sternum has been elevated to the desired position (Fig 5a). A substernal bar (V. Mueller Co, Allegiance Healthcare Corp, Deerfield, IL) is now used in all cases of pectus excavatum and is placed at the fourth or fifth intercostal space. The bar extends from the right anterior axillary line to the left anterior axillary line anterior to the ribs and posterior to the sternum (Fig 5a). The ends are bent to conform to the contour of the ribs and are transfixed with absorbable sutures (0 Maxon [Davis & Geck]) to the rib in two sites. In pectus carinatum deformities, a bar is not routinely used, and a posterior sternal osteotomy is performed. After resection of a triangular wedge of the sternum, the sternum is fractured and angulated posteriorly without displacement to the desired position. Reattachment of the perichondrial sheaths to the sternal edges (Fig 5b), followed by reapproximation of the rectus and pectoralis muscles and skin flaps completes the operation (Fig 6). Pliable, closed suction drains (Hemovacs) are left beneath the pectoralis and skin flaps. The drains are removed when the output is 20 to 30 mL/day or less (usually postoperative day 3 or 4). A small chest tube is routinely placed in the right pleural space and removed on postoperative day 2. The sternal bar is removed as an outpatient procedure in the operating room 6 months later, except in children with Marfans syndrome, when it is removed 12 months later.
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After 1 year, patients are not routinely followed up; thus, long-term follow-up was established through telephone conversation with the patient or parents, or both. A rating scale similar to that published by Humphreys and Jaretzki [11] was used to judge the surgical outcomes. Results were deemed excellent when the chest contour was perceived as perfectly normal, with no postoperative sequelae (ie, an inconspicuous scar, no persistent pain or clicks, and no bony "bumps"). Results were coded as good if the chest contour was comparable to ones peers but maybe not quite normal and with only minor postoperative sequelae occurring. Results were regarded as fair if the chest had partially sunk back or remained somewhat protuberant (carinatum defect). Also termed fair were prominent scar, persistent pain or clicks, or bony "bumps." Results were classified as poor if the chest appeared as it had preoperatively or if the patient had had a revision operation. Of note, we did not do any reoperative pectus repairs in any of our own patients or in any patient operated on by other surgeons. One 21-year-old patient had been previously operated on by a plastic surgeon and had had subcutaneous placement of a silicone prosthesis at age 18 years to relieve the cosmetic deformity. This prosthesis was uncomfortable (felt "heavy"), and he had respiratory symptoms with exercise. The prosthesis (19 x 15 x 3 cm, 0.55 kg) was removed; standard pectus repair was performed; and his respiratory symptoms disappeared. One patient underwent reoperation to remove several prominent cartilage "tags," which were noticeable through the skin.
One child was emergently referred when an attempted noninvasive "Nuss" procedure [12] was complicated by perforation of the right atrium and right ventricle by the sternal bar, with both resultant hemorrhage and disruption of the tricuspid valve apparatus. Emergent standard median sternotomy incision was performed, and the right atrial and right ventricular perforations were repaired. Transesophageal echocardiography demonstrated severe tricuspid valve insufficiency secondary to the bar passing through the tricuspid valve, and cardiopulmonary bypass was initiated. The heart was arrested with cardioplegia, and the tricuspid valve, which was partially disrupted anteriorly, was repaired. The sternotomy incision was closed, and standard pectus repair was performed with subperichondrial cartilage resection and substernal bar.
| Results |
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One hundred of 120 patients were contacted, for a total follow-up of 83%. The results, as shown in Table 2, are grouped into 5-year intervals. Results were classified as excellent or good in 89 patients (89%). Results were described as poor in only 3 patients (3%). Selective use of a sternal support bar began in 1981, and the bar has been routinely used since 1994. These results were compared with those for repairs done without a sternal bar, which have follow-up data since 1972 (Table 3). Of patients undergoing repair with a sternal bar, 30 (86%) of 35 had an excellent outcome versus 34 (52%) of 65 undergoing repair without a sternal bar (p = 0.004,
2 analysis). No poor results were reported for patients who had a temporary sternal support, and only 1 patient had a fair result.
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Four male patients with Marfans syndrome (age range, 8 to 17 years) had repair with a sternal bar. Three had pectus excavatum, and 1 had pectus carinatum. The follow-up period ranges from 3 to 16 years, with excellent outcomes in 2 patients and good outcomes in the other 2. One patient with a good outcome was previously described. The second good outcome was in one of the patients who had migration of his sternal bar, requiring early removal at 1 month.
| Comment |
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Fonkalsrud and colleagues [14] described a 25-year experience with 252 children having repair of pectus deformities. Their technique is essentially identical to ours, except for the use of a transverse skin incision. The substernal bar was used chiefly for patients older than 5 years (136 patients [54%]). They reported no deaths and no significant complications, except for recurrent chest depression in 3 patients (1.2%). More than 98% of patients had improvements in exercise tolerance, endurance, respiratory symptoms, and cosmetic appearance; these improvements were considered excellent results.
The excellent outcomes in the clinically complex children with Marfans syndrome further support use of a sternal bar. A previous report recommended delaying the repair of an anterior chest wall deformity in children with Marfans syndrome until skeletal maturity was reached [16]. We believe that pectus repair can be done safely with a sternal bar, with a good cosmetic outcome, at a young age, preferably between 4 and 8 years old, before skeletal maturity is reached. One variation of our treatment in these patients is to allow the bar to remain in place a full 12 months.
Abnormal cardiac findings associated with pectus deformities range from benign functional murmurs to single-ventricle physiology. The flow murmurs may be innocent or may possibly be related to compression of the right ventricular outflow tract [17]. When the children with connective tissue disorders were excluded, the incidence of mitral valve prolapse in our study was 5%. In a larger study by Shamberger and Welch [18], mitral valve prolapse was found in 7.7% of 426 patients. The incidence of mitral valve prolapse in the general population is approximately 5% [19]. After repair of the pectus deformity, approximately in 40% of cases the mitral valve prolapse resolves [18]. The incidence of congenital heart disease was 11.6% in our review. However, we believe that this incidence is unusually high and is probably related to our referral pattern, which is that of a pediatric heart surgical center in a large urban setting. A review from Childrens Hospital in Boston documented a 0.17% incidence of pectus deformities in children with congenital heart defects [20]. Probably because this association is uncommon, there are only scattered case reports documenting the efficacy of doing combined repairs. Some surgeons suggest doing staged procedures [1, 20]. Historically, there have been concerns for complications, such as increased bleeding, increased sternal infections, or very extended operative times. We agree that operating time is prolonged, and if the heart defect is very complex and has high associated morbidity, for instance, a Fontan procedure, we will stage the procedure. In particular, for the Fontan operation, it is advantageous to have no compression whatsoever of the highly compressible lateral venous tunnel. Our incidence of complications in 9 patients with simultaneous combined pectus and congenital heart defect repairs was very low and was limited to atelectasis in 1 patient. Combined repairs may be safely accomplished without increased bleeding or sternal wound problems. Furthermore, chest wall instability, which could lead to pulmonary difficulties compounded by the cardiac procedure, is not a problem because we now use a sternal support bar in all cases.
Another problem frequently cited with simultaneous pectus and intracardiac repair is lack of exposure and inability to place a sternal retractor. Various techniques, including sternal turnover or horizontal or vertical sternal splits, have been proposed. Our technique is simple and provides adequate exposure for intracardiac defects. The midline incision provides the same access that cardiac surgeons are familiar with using a median sternotomy. If improved exposure is necessary for repair of the aortic arch or distal pulmonary arteries, we will perform a sternotomy after resecting the deformed cartilages, leaving the intercostal muscle bundles attached to the sternum. The long-term outcomes are excellent and similar to that of isolated pectus repairs. Further benefits are that two operations are performed under a single anesthetic, and only one hospital stay is required. Patients with connective tissue disorders such as Marfans syndrome are a special group. There is a definite association of cardiovascular anomalies with thoracic skeletal deformities in these patients [21]. Up to 60% of patients with Marfans syndrome may have mitral or aortic regurgitation. Again, if necessary, we believe a combined repair may be done without added morbidity.
The ideal age for pectus repair remains controversial. Our patients ranged in age from 3 to 21 years, with a mean age at operation of 8.4 years. Randolph and associates [22] reported on 50 children undergoing pectus excavatum repair at under 36 months of age and concluded that 90% had excellent results. A potential problem with this young age at repair has been raised independently by Haller [23] and Weber and Kurkchubasche [24]. Weber and Kurkchubasche [24] described 1 patient who had pectus repair at age 4 and required reoperation at age 14 because of restrictive thoracic dystrophy secondary to the extensive and early pectus repair. Haller [23] discussed 3 patients with severe chest wall restriction after pectus repair at ages 2, 3, and 4 years. He recommended not repairing pectus excavatum before 4 years of age, not removing the second cartilage, limiting the number of cartilages removed, and not suturing the perichondrial sheaths behind the sternum [23]. Use of a substernal bar avoids the need for suturing the perichondrial sheaths behind the sternum. We have not seen this complication in the relatively few patients less than 4 years old (6 patients) operated on in our series.
As noted earlier, one of the patients in our series was referred emergently when the right atrium, right ventricle, and tricuspid valve were perforated during an attempted "Nuss" repair [12]. The Nuss technique involves placement of a convex steel bar under the sternum through bilateral thoracic incisions. Cartilage resection is not performed. The steel bar is inserted with the convexity facing posteriorly, and when the bar is turned over, the deformity is "corrected." Average hospital stay was 4.3 days. The bar is left in place for 2 years, at which time remolding of the chest has occurred. Nuss and colleagues [12] reported on follow-up in 30 patients, with results classified as excellent in 22 patients (73%), good in 4 (13%), fair in 2 (7%), and poor in 2 (7%). Obviously it is difficult to compare two different series, but on superficial analysis, our technique using a bar was superior and resulted in excellent or good results in 97% of patients. The risk of cardiac perforation requiring emergent sternotomy and cardiopulmonary bypass needs to be considered in light of the patient emergently referred to our service.
In conclusion, our clinical review documents that there is long-term patient satisfaction with our described standard surgical pectus excavatum and carinatum repair. The technique uses a vertical midline incision with subperichondrial cartilage resection, anterior sternal osteotomy, and placement of a temporary metal bar for retrosternal support. There were no deaths, and the operative morbidity was minimal. The chest wall contour is maintained over time. The metal sternal support bar improves the long-term outcome and should be used routinely. Patients with congenital heart defects can undergo simultaneous cardiac and pectus deformity repair, with no added morbidity and excellent long-term results with regard to chest wall contour.
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