Advertisement

Surgical Management of Pectus Excavatum

      Pectus excavatum is a malformation of the anterior chest characterized by a posterior curvature of the lower two-thirds of the sternum, usually with the deepest point just above the xiphoid. The lower costal cartilages curve inward to form a broad symmetrical depression. The first and second ribs and costal cartilages, as well as the manubrium, are normal in most patients. Asymmetric deformities are common, with the concavity usually deeper on the right and the sternum rotated slightly, with the heart shifted into the left chest (Fig 1). Occasional patients may have protrusion of a few costal cartilages on the left. The chest wall characteristically has a decreased anteroposterior diameter. There is often an outward bowing of the lower costal cartilages over the abdomen, giving a “pot-bellied” appearance, particularly in younger children. An asthenic build and slumped posture are common.
      Figure thumbnail gr1
      1Transverse section of the midthorax showing displacement of the heart into the left chest by the mid and lower sternum in a child with a severe pectus excavatum deformity.
      The pathogenesis of pectus excavatum remains unclear, but it has no relationship to rickets.
      • Brodkin HA
      Congenital anterior chest wall deformities of diaphragmatic origin.
      An abnormality of the diaphragm or a band of tissue retracting the sternum posteriorly has been suggested; however, this is not supported by operative findings, or by the therapeutic ineffectiveness of simple retrosternal dissection. A recent theory is that the deformity results from an unbalanced growth in costochondral regions, further explaining the occasional asymmetric appearance, frequent association of other defects of osteogenesis and chondrogenesis, and the existence of the converse type of deformity, pectus carinatum. The involved cartilages are often fused, bizarrely deformed, or rotated. Resected cartilage segments occasionally show a disorderly arrangement of cartilage cells, perichondritis, and areas of aseptic necrosis. Abnormalities in the costal cartilages, with decreased levels of zinc and increased levels of magnesium and calcium, have been demonstrated.
      • Rupprecht H
      • et al.
      Pathogenesis of the chest wall deformities-Electron microscope studies and analysis of trace elements in the cartilage of the ribs.
      Pectus excavatum is inherited, although not clearly as a recessive trait. A member of the immediate family constellation with a chest wall deformity can be identified in approximately 40% of cases. The anomaly is believed to occur in as many as 1 in 400 births, but is uncommon in blacks and Hispanics (Personal communication, March 1995, March of Dimes Birth Defects Foundation, White Plains, NY). Other malformations may coexist, especially musculoskeletal anomalies, including scoliosis (16%), Marfan's syndrome, clubfoot, and others. The deformity is usually apparent soon after birth, progresses slowly during childhood, and becomes much more severe during the rapid skeletal growth of early adolescence. Deep inspiration characteristically accentuates the deformity. Spontaneous regression rarely, if ever, occurs. During the past few years, many adults have sought surgical repair of pectus excavatum deformities that were not repaired earlier and that caused persistent symptoms and limitations in activity.
      • Fonkalsrud EW
      • Bustorff-Silva J
      Repair of pectus excavatum and carinatum in adults.
      Symptoms are uncommon during early childhood, apart from an awareness of the abnormality and an unwillingness to expose the chest while swimming or engaging in other social or athletic activities. Easy fatigability and decreased stamina and endurance often become apparent during early adolescence when children become involved in competitive sports. In a severe deformity pulmonary expansion during inspiration is moderately confined in the relatively fixed thoracic compartment. Some very competitive children are able to compensate for the pectus-induced limitations in chest expansion in short-duration athletic activities with wider diaphragmatic excursions at a cost of greater energy expenditure. There is an appreciable incidence of chronic bronchitis, asthma, and occasional bronchiectasis, particularly in adolescents and adults. The xiphoid may be bifid, twisted, elongated, or displaced to one side.
      Most patients will be advised by well-meaning family physicians that the deformity will improve with age, that it will not effect cardiopulmonary performance, that it is primarily a cosmetic problem, and that surgical repair is dangerous, minimally effective, and unnecessary. Each of these views is incorrect, given our present knowledge of pectus excavatum deformities and the current techniques for surgical repair based on extensive clinical experience with long-term followup.
      • Fonkalsrud EW
      • Salman T
      • Guo W
      • et al.
      Repair of pectus deformities with sternal support.
      • Haller Jr, JA
      • Scherer LR
      • Turner CS
      • et al.
      Evolving management of pectus excavatum based on a single institutional experience of 664 patients.
      Several methods of quantifying the severity of anterior chest deformities have been proposed, although none has been widely accepted. Most methods include measuring the distance between the sternum and the spine as a primary consideration. Transverse and anteroposterior measurements obtained from computed axial tomograms of the chest are accurate; however, this method is relatively costly and in our experience is rarely necessary. Objective assessment of the severity of the deformity for patients of all ages can be obtained from chest radiographs (front and side views) to obtain the pectus severity score. The pectus severity score is determined by measuring the internal width of the lower chest and dividing it by the distance between the posterior surface of the sternum and the anterior surface of the spine. The normal chest has a score of 2.56, as noted by Haller and associates,
      • Haller JA
      • Kramer SS
      • Lietman SA
      Use of CT scans in selection of patients for pectus excavatum surgery. A preliminary report.
      and the mean value for patients undergoing pectus repair in our experience is 4.65.
      • Fonkalsrud EW
      • Dunn JCY
      • Atkinson JB
      Repair of pectus excavatum deformities: 30 years experience with 375 patients.
      The severity score also permits measurement of the improvement after surgical repair. Patients with the higher pectus severity scores commonly have the most severe symptoms. The declination index based on the angle of posterior displacement of the fifth costal cartilage was helpful in determining the severity in patients from the large clinical series reported by Welch.
      • Welch KJ
      Chest wall deformities.
      Moire phototopography may indicate the severity of the depression with striking contrast.
      Electrocardiographic abnormalities are common, often showing right axis deviation and depressed ST segments. Echocardiograms may show mitral valve prolapse, which is rarely of clinical concern. A functional systolic cardiac murmur is often present along the upper left sternal border, possibly due to partial pulmonary outflow compression. Angiocardiograms have shown compression of the right ventricular outflow tract. Pressure waves in patients with pectus excavation are similar to those in patients with constrictive pericarditis. Measurements of cardiac output using right ventricular catheterization have demonstrated diminished cardiac output and stroke volume in preoperative patients during upright exercise, which increased more than 35% following surgical repair. These findings are not demonstrable in patients tested supine without exercise, which accounts for some of the misleading reports of no physiologic impairment in these patients.
      • Beiser GD
      • Epstein SE
      • Stampfer M
      • et al.
      Impairment of cardiac function in patients with pectus excavatum, with improvement after operative correction.
      Similarly, conventional pulmonary tests with the patient at rest are almost always within normal limits or borderline in children with pectus excavatum, although it is difficult to obtain meaningful measurements in young children. Studies by Cahill and associates
      • Cahill JL
      • Lees GM
      • Robertson HT
      A summary of preoperative and postoperative cardiorespiratory performance in patients undergoing pectus excavatum and carinatum repair.
      using the cycle ergometer to evaluate exercise performance in children both before and after pectus repair have shown a significant improvement in maximal voluntary ventilation, maximal oxygen consumption, and total exercise time. After repair, patients exhibited a lower heart rate and higher minute ventilation compared to preoperative values. These observations support the hypothesis that both a restrictive cardiac stroke volume and the increased work of breathing described by many patients can be improved by operative repair of the anomaly. Thus, although the pectus excavatum deformity is cosmetically unattractive for almost all patients, the major indication for surgical repair is physiologic. There are no studies indicating that breathing exercises or weight lifting ameliorate the defect or its effects.

      Patient Selection

      Because almost all young children with pectus excavatum are asymptomatic, the selection of patients for surgical repair has been highly variable. Several surgeons recommend that children with moderate to severe sternal depression undergo repair in the preadolescent years when the operation is technically easier and patient recovery is more rapid and less confining than in adolescent years or later. Repair of pectus deformities can be performed successfully at any age, although the operation is technically more demanding and the hospitalization and recovery are somewhat longer in older adolescents and adults.

      Surgical Technique

      Figure thumbnail fx2
      2The repair of pectus excavatum that we have found to provide the most consistent, good, long-term results with the fewest complications in 385 consecutive patients is a modification of the original procedure described by Brown
      • Brown AL
      Pectus excavatum (funnel chest).
      and modified by Ravitch
      • Ravitch MM
      Operative technique of pectus excavatum repair.
      and Welch,
      • Welch KJ
      Satisfactory surgical correction of pectus excavatum deformity in childhood: a limited opportunity.
      and extensively detailed in previous reports.
      • Fonkalsrud EW
      Chest wall deformities.
      It begins with a transverse curvilinear incision midway between the nipples and the costal margin extending from the mid nipple line brilaterally. Limited skin flaps are elevated over the pectoralis muscles, using needlepoint electrocautery to minimize blood loss.
      Figure thumbnail fx3
      3The pectoralis muscles are reflected laterally over a short distance from attachments to the sternum and deformed costal cartilages (usually the lower 4 or 5). The abdominal muscles are mobilized from the lower costal cartilages.
      Figure thumbnail fx4
      4The perichondrium is incised on the mid anterior surface of the lower 4 or 5 costal cartilages bilaterally, extending from the costochondral junction to the sternum. Abnormal costal cartilages are resected carefully, preserving the intact perichondrium. The xiphoid is detached from the sternum.
      Figure thumbnail fx5
      5The intercostal muscles and perichondrial sheaths of the resected cartilages are detached from the lower sternum, and the space is mobilized. The pleura is incised on the right side, and a small chest tube is inserted.
      Figure thumbnail fx6
      6A transverse anterior wedge osteotomy of the sternum is made at the level where the sternum depresses posteriorly. The posterior table of the sternum is gently fractured without displacement and then elevated and twisted to the desired position.
      Figure thumbnail fx7
      7Nonabsorbable sutures are placed through the anterior table of the sternum across the osteotomy. A stainless steel strut is placed across the lower anterior chest to support the tip of the sternum and is wired to the appropriate rib on each side (fifth or sixth). The bar often will skewer through the perichondrium to prevent a localized depression. The xiphoid and perichondrial sheaths are sutured back to the sternum.
      Figure thumbnail fx8
      8The pectoralis and abdominal muscles are sutured together over the sternum. Thorough hemostasis is achieved with needlepoint electrocautery, and the wound is copiously irrigated with antibiotic solution. A small chest tube is placed into the right pleural space. The skin is closed with subcuticular absorbable sutures and steri strips or staples and a loose dry dressing is applied.

      Postoperative Care

      The endotracheal tube is removed in the recovery room within 2 hours. The chest tube is removed within 24 hours after the operation. Intravenous cefazolin (Ancef) is given for 3 days, and oral cephalexin (Keflex) is given for 4 additional days. Postoperative pain is remarkably mild and is controlled with intravenous analgesics for the first 2 postoperative days, and by oral medications thereafter. Epidural analgesia is not used.
      During the past 15 years, the mean duration of the operation has been 2.7 hours (2.2 hours for patients under age 11 years, and 3.3 hours for older patients). Only the infrequent patient with cardiac anomalies has been placed in an intensive care unit postoperation during the past 19 years. The total period of hospitalization rarely exceeded 3 days (mean = 3.1 days), with many children under age 11 years discharged within 48 hours. Blood loss rarely exceeded 100 mL (mean = 93 mL). The steel strut was removed on an outpatient basis approximately 6 months after repair with the patient under light general anesthesia (mean operating time = 21 minutes). The vast majority of patients returned to school or work within 2 weeks. Full physical activity, including strenuous exercise but no body contact sports, was resumed by almost all patients within 10 weeks.

      Results

      Each of the patients with preoperative limitation in stamina and endurance noted considerable improvement within 4 months after surgery, and most were able to participate in vigorous exercise within 5 months. A decrease in frequency and severity of pulmonary infections was noted by 96%; 92% of those with asthmatic symptoms showed improvement within 3 months. Almost all patients exhibited shifting of the heart from the left chest to the normal position immediately following operation (Figure 9).
      Figure thumbnail gr9
      9(A) View of the chest of a 17-year-old boy with symptomatic pectus excavatum. (B) View of the chest in the same patient 3 months postoperation.
      Postoperative complications included wound seroma (3.2%), pleural effusion (3.5%), atelectasis (3.2%), unintentional pneumothorax (0.8%), and pericardial effusion (0.8%). Recurrent depression occurred in 1.3%, all from the early period when a sternal bar was not used; 3 patients underwent a secondary repair. Protrusion of the second or third costal cartilages occurred in 21 patients during the adolescent years after earlier repair; 5 underwent resection of the cartilages. Hypertrophy of the scar occurred in 9.3%. We currently inject triamcinalone solution (10 mg/mL) into the scar if it shows evidence of hypertrophy. Only 7 patients indicated any discomfort from the sternal bar. There were no deaths within 12 months after operation.

      Comments

      During the past 13 years, we have been consulted by numerous patients over age 20 who have severe untreated pectus excavatum deformities with worsening symptoms and limitations and who desire surgical correction; 40 of these patients underwent successful repair.
      • Fonkalsrud EW
      • Salman T
      • Guo W
      • et al.
      Repair of pectus deformities with sternal support.
      Although the technical aspects of repair were more tedious than in children, the postoperative recovery and the long-term results have been similar.
      We have not observed the appearance of constricting asphyxiating thoracic dystrophy reported by Haller et al
      • Haller JA
      • Colombani P
      • Humphries C
      • et al.
      Chest wall constriction after too extensive and too early operations for pectus excavatum.
      in any of the operated patients. We have used the sternal support bar for adolescent patients for 30 years, and have used the bar for patients of all ages during the past 13 years. The sternal support bar eliminates postoperative flail chest and paradoxical respiration, which reduces pain and permits earlier ambulation and deeper respirations, ultimately reducing length of hospitalization and overall cost. The bar is easily removed and ensures good long-term results; recurrent depression is rare, regardless of the patient's age at the time of repair. Costal cartilage regeneration from the perichondrium is remarkably rapid, with the chest becoming very stable within 4 weeks.
      We are awaiting with enthusiasm the long-term results with the minimally invasive pectus excavatum repair recently described by Nuss et al.
      • Nuss D
      • Kelly Jr, RE
      • Croitoru DP
      • et al.
      A 10-year review of a minimally invasive technique for the correction of pectus excavatum.
      The current experience of these authors with a small number of patients who underwent bar removal 2 or more years postrepair with short follow-up is encouraging. It is apparent that pain is more severe and prolonged, the complications are more frequent and severe, the period of hospitalization is longer, and the limitation on physical activity is longer than for patients undergoing repair as described in this report. Pectus repair requires attention to several technical details with small alterations made on the basis of different anatomic features in each specific patient. The operative technique used for patients in the present report has provided excellent results in more than 97% of cases.

      References

        • Brodkin HA
        Congenital anterior chest wall deformities of diaphragmatic origin.
        Dis Chest. 1953; 24: 259
        • Rupprecht H
        • et al.
        Pathogenesis of the chest wall deformities-Electron microscope studies and analysis of trace elements in the cartilage of the ribs.
        Z Kinderchir. 1987; 42: 228
        • Fonkalsrud EW
        • Bustorff-Silva J
        Repair of pectus excavatum and carinatum in adults.
        Am J Surg. 1999; 177: 121-124
        • Fonkalsrud EW
        • Salman T
        • Guo W
        • et al.
        Repair of pectus deformities with sternal support.
        J Thorac Cardiovasc Surg. 1994; 107: 37-42
        • Haller Jr, JA
        • Scherer LR
        • Turner CS
        • et al.
        Evolving management of pectus excavatum based on a single institutional experience of 664 patients.
        Ann Surg. 1989; 209: 578-583
        • Haller JA
        • Kramer SS
        • Lietman SA
        Use of CT scans in selection of patients for pectus excavatum surgery. A preliminary report.
        J Pediatr Surg. 1987; 22: 904-906
        • Fonkalsrud EW
        • Dunn JCY
        • Atkinson JB
        Repair of pectus excavatum deformities: 30 years experience with 375 patients.
        Ann Surg. 2000; 231: 443-448
        • Welch KJ
        Chest wall deformities.
        in: Holder TH Ashcraft K Pediatric Surgery. W.B. Saunders, Philadelphia, PA1980: 162-182
        • Beiser GD
        • Epstein SE
        • Stampfer M
        • et al.
        Impairment of cardiac function in patients with pectus excavatum, with improvement after operative correction.
        N Engl J Med. 1972; 287: 267-272
        • Cahill JL
        • Lees GM
        • Robertson HT
        A summary of preoperative and postoperative cardiorespiratory performance in patients undergoing pectus excavatum and carinatum repair.
        J Pediatr Surg. 1984; 19: 430-433
        • Brown AL
        Pectus excavatum (funnel chest).
        J Thorac Surg. 1939; 9: 164-169
        • Ravitch MM
        Operative technique of pectus excavatum repair.
        Ann Surg. 1949; 129: 429-444
        • Welch KJ
        Satisfactory surgical correction of pectus excavatum deformity in childhood: a limited opportunity.
        J Thorac Surg. 1958; 36: 697-713
        • Fonkalsrud EW
        Chest wall deformities.
        in: Rowe MI O'Neill Jr, JA Grosfeld JL Fonkalsrud EW Coran AG Essentials of Pediatric Surgery. C.V. Mosby, St. Louis, MO1995: 383-391
        • Haller JA
        • Colombani P
        • Humphries C
        • et al.
        Chest wall constriction after too extensive and too early operations for pectus excavatum.
        Ann Thorac Surg. 1996; 61: 1618-1625
        • Nuss D
        • Kelly Jr, RE
        • Croitoru DP
        • et al.
        A 10-year review of a minimally invasive technique for the correction of pectus excavatum.
        J Pediatr Surg. 1998; 33: 545-552