Daniel P. Doody
Introduction
Pectus excavatum, depression of the sternum and parasternal cartilages, is the most common congenital chest wall abnormality, accounting for approximately 90% of chest wall defects. The second most common abnormality, pectus carinatum, accounts for 5% to 6% of chest wall anomalies where more complex abnormalities including Poland syndrome, Silverman–Currarino syndrome, sternal cleft, and asphyxiating thoracic chondrodystrophy (Jeune syndrome) are rare chest wall abnormalities.
Pectus excavatum occurs in approximately 1 in 300 to 400 births. There is a definite male predominance with a male to female ratio of 3:1 to 4:1. In addition, an ethnic predisposition is seen as Caucasian children and adolescents have a much higher incidence of pectus excavatum as compared to children of African American or East Asian descent.
In approximately half of the children who present for evaluation of pectus excavatum, some sternal depression may be noted in early childhood, with some parents dating the abnormality to infancy. However, many families will report the new appearance of the sternal depression in the preadolescent or early adolescent age period when somatic growth accelerates.
Approximately 25% to 35% of children who present for evaluation of the chest wall defect will identify other family members as having a chest wall abnormality, either pectus carinatum or pectus excavatum. Approximately 5% of children will have identifiable genetic or connective tissue disorders such as Noonan syndrome or Marfan syndrome. In particular, children with Marfan syndrome have a much higher incidence of failure of repair following surgical correction of the pectus excavatum defect, which needs to be considered when recommending repair to the family.
There is no question that the chest wall abnormality often affects the child’s perception of his/her body image and exercise capacity. Controversy exists regarding the physiologic consequences of pectus excavatum although more recent data support that there is a physiologic impairment because of abnormality in chest wall motion, particularly with exercise, as well as compression and impingement on the right side of the heart with more severe defects. Many children will have a mild restrictive defect on static pulmonary function tests and typically will fall in the low–normal range during exercise stress test. The incidence of mitral valve prolapse is much greater in patients with pectus excavatum as compared to the general population.
The Nuss repair can be used in repair of adults who present with pectus excavatum defect. The repair with a stiffer anterior chest wall may be associated with a greater degree of pain and discomfort in the postoperative period. Moreover, the repair in the adult patient often requires two or even three pectus bars to support and reshape the adult thorax. Although minor, some series report a higher incidence of bar displacement, likely related to the more rigid and less compliant chest wall.
Finally, the Nuss repair can be used successfully in reoperative pectus excavatum surgeries whether the initial surgery was the minimally invasive repair or the open repair as described by Ravitch and Welch. As with the adult repair, there does appear a higher incidence of bar displacement and complications. In addition, the current thought argues that a more severe defect, as measured by the Haller pectus index, is needed before reoperative surgery is recommended. While there appears to be consensus that a Haller index of greater than 3.25 with symptoms is an indication for surgical correction of a pectus excavatum defect, experienced authors have argued that a Haller index greater 3.7 should be seen and the patient should be suffering symptoms (dyspnea, chest wall pain) before recommending repair for retrusion following a previous pectus excavatum repair.
PREOPERATIVE PLANNING
A variety of symptoms may occur in the patient with the pectus excavatum defect, or the patient may have no symptoms but a clinically apparent anterior wall depression. In our practice, it is infrequent that preschool-aged or early school-aged children are evaluated. However, children in the early school years with a clinically apparent pectus excavatum may present for evaluation. These younger children frequently have few complaints in terms of exercise intolerance or chest wall pain.
The more typical patient is the preadolescent or a child in the early teenage years, frequently with the new appearance and progression of symptoms that may be associated with the chest wall abnormality. Most, but not all, patients will report some increasing dyspnea on exertion, particularly with low-intensity, long-duration exercise such as distance running, swimming, rowing, or bicycle riding as compared to anaerobic exercises where high-intensity, short-duration muscle activity is needed, such as sprinting or weightlifting. Often, the patient will report that they can sprint without problem but middle distance running will often lead to early fatigue and exhaustion. Shortness of breath at rest is very atypical in this age group and may need additional cardiac and pulmonary evaluation.
Chest wall pain is commonly noted, particularly in the area of the parasternal cartilages and sternum. Young women may note unilateral breast hypoplasia, particularly with asymmetrical chest wall defects.
Finally, body image issues in this particular teenage and young adult age group should not be ignored and the psychosocial pressures can be significant to the point that it is often life altering (“I won’t take off my shirt.”; “I am considering not having children.”; “I have stopped playing sports.”) to infrequent, but worrisomely life-threatening, concerns (“I can’t live like this.”).
A past history of known metal allergy or adverse reaction to jewelry should alert the clinician that a nickel allergy may be present. Nickel is a component of the stainless steel pectus bar and has been identified as an allergen. The potential adverse effects include an erythematous macular rash, the development of excessive granulation tissue at the surgical sites, wound separation, and pleural effusions. For patients with known nickel allergy, individualized preshaped titanium pectus bars can be manufactured for correction of the symptomatic chest wall defect but must be ordered before surgery can be scheduled. These titanium bars are more brittle and should not be reshaped at the time of the operation.
Physical examination often demonstrates typical findings including the posterior deflection of the sternum that may involve the lower sternum or involve the entire sternal body below the manubrium. Many times, the defect is asymmetrical, typically with the greater depression seen on the right side. There is often some costal flaring, which needs to be pointed out to the patient and family as that may become more prominent after surgical correction using the Nuss technique. The shoulders are frequently inturned. The surgeon should listen carefully for a systolic click, which may indicate mitral valve prolapse. Additional skeletal abnormalities including scoliosis or kyphosis should be noted. Other clinical signs including a high-arched palate, crowded teeth, poor vision, abnormal long arms, joint laxity, or striae without recent weight gain or loss with an associated chest wall defect may be seen with Marfan syndrome. Measurement from the midaxillary line to midaxillary line at the point of greatest chest depression should be recorded as that measurement helps to determine the length of the pectus bar used during the surgical correction. The pectus bar length is often 2 to 3 cm less than the midaxillary to midaxillary line measurement.
Figure 13.1 Axial CT imaging at point of greatest sternal depression to determine Haller index. The internal transverse measurement is divided by the internal anteroposterior measurement from the sternum or parasternal tissue to the anterior border of the vertebral body.
Imaging studies have proven the most helpful in determining whether surgical correction should be recommended. The Haller index, a CT-derived measure, is generally accepted as the most accurate measurement of the severity of the chest wall defect. This CT scan of the chest without contrast allows the surgeon and radiologist to determine the internal transverse diameter of the chest at the point of greatest depression as well as the anteroposterior distance from the retrosternal or parasternal point of greatest depression to the anterior vertebral body (Fig. 13.1). The general population without a chest wall defect will have a Haller index of 2.5. Although authors vary at the Haller index value where they would recommend surgical correction, an index greater than 3.25 is used in our institution. In the asymptomatic patient, an abnormal Haller index alone is not an indication for surgical repair. However, most patients are experiencing symptoms before presenting to the pediatric surgeon or thoracic surgeon for evaluation of the chest wall defect.
If the Haller index is greater than 3.25, an echocardiogram is performed to look for aortic root dilatation or mitral valve prolapse. The presence of mitral valve prolapse is an indication for surgery, as this valvular abnormality may disappear following correction. If aortic root dilatation is found, evaluation by cardiology and genetics is performed before we recommend surgical correction. Significant compression of the heart chambers by the pectus defect is also considered an indication for surgery.
Exercise pulmonary function tests are performed, which we feel more accurately reflect the cardiopulmonary effects associated with the pectus excavatum defect. The vast majority of children will show low, normal, or below normal values, particularly in oxygen delivery and cardiac output with exercise. Below normal values on the exercise stress test makes a strong argument that correction of the chest wall defect is indicated.
A similar preoperative evaluation will be performed for adult patients or patients with recurrent pectus excavatum defects who are being considered for repair.
Generally, we do not recommend repair of a pectus excavatum defect in the preschooler or elementary school-aged child. The classic open repair in this age group is contraindicated because of the potential of the abnormal chest wall scarring and secondary thoracic chondrodystrophy. While this abnormal scarring does not seem to be an issue in patients following the Nuss repair, our concern for retrusion during the adolescent growth spurt leads us to recommend repair at the beginning of the growth spurt (ages 8 to 12) with the expectation that correction and stabilization of the chest wall during the growth spurt would be ideal. However, a more typical patient who first presents to our practice would be a teenager who has completed his/her growth spurt and noted either new appearance of the pectus defect or significant worsening of the chest wall depression.
Figure 13.2 Silverman–Currarino defect with broad superior pectus carinatum defect often associated with a lower sternal pectus excavatum anomaly and congenital heart defects.
For some chest wall defects, the Nuss repair without removal of parasternal cartilages may result in unsatisfactory outcome. The Silverman–Currarino syndrome, or pouter pigeon chest wall deformity (Fig. 13.2), is a rare anomaly, often associated with congenital cardiac defects that requires an open correction of the superior pectus carinatum portion of the mixed chest wall defect while the Nuss repair can be used to support the lower sternal pectus excavatum defect. The etiologic cause of the sternal abnormality is believed to be related to premature fusion of the manubrial–sternal junction in addition to early fusion of other sternal elements that may result in a foreshortened sternum.
The severe, broad-based upper carinatum defect typically does not respond to orthotic bracing, and these young patients will not have significant chest wall remodeling with correction of the lower pectus excavatum defect alone. A combined repair to address the mixed pectus carinatum–pectus excavatum abnormality should be recommended. We have used the Nuss repair to correct the lower sternal pectus excavatum defect while removing the parasternal cartilages as one would with an open correction of the pectus carinatum defect. Often, several sternal osteotomies, or even wedge resection of the sternum with osteotomies, are necessary to address the severe anterior angulation of the upper sternum to have the best result.
SURGERY
Positioning
In most patients, an epidural catheter is placed for intraoperative and postoperative pain management. The patient is returned to the supine position and induced with general endotracheal anesthesia. While other centers prefer the patient in a simple shoulder abducted position on boards, our preferred position for a standard repair is with the arms elevated and flexed over the head, placed either in an arm cradle or attached to a padded ether screen.
Technique
After an appropriate patient position has been obtained, the chest and lateral chest walls are sterilely prepped and draped from above the sternal notch to below the costal margins. We use an iodophor-impregnated adhesive drape to cover this wide surgical field and hold the underlying sterile towels in place.
Before the procedure begins, the surgical team selects the intercostal spaces for the planned bar placement close to the point of greatest sternal depression. If a single bar is planned, 4- to 6-cm lateral chest wall incisions from the anterior axillary line to the midaxillary line are made simultaneously at the point of greatest depression. It has been our practice to use a two-team approach with surgeons working on both sides of the thorax simultaneously. This lateral chest wall dissection typically brings us directly onto the serratus anterior muscles while being anterior to the latissimus dorsi muscles. Subcutaneous pockets are created above the fascia of the serratus muscles, extending in a cephalocaudad (vertical) direction for the later placement of the stabilizing bars. Once the lateral pockets are of adequate size, we dissect anteriorly in the subcutaneous plane above the muscular fascia to the tip of the pectus ridge. In most cases, this dissection is at the inferior margins of the pectoralis major and pectoralis minor muscles.
Although we have used bilateral thoracoscopic guidance, particularly with severe pectus excavatum defects, in most cases we will place a single right-sided 5-mm Thoracoport for thoracoscopic visualization of the anterior mediastinal dissection and placement of the pectus bar. The right side port placement is chosen to direct the dissection as the depressed left sternal border is obscured by the heart and pericardium. This right-sided port is introduced inferior to the right-sided subcutaneous pocket, typically in the eighth intercostal space. A 5-mm 30-degree-angled thoracoscope placed into the right chest provides good field visualization for the procedure (Fig. 13.3).
We do use carbon dioxide insufflation during our dissection with the settings at 6 to 8 mm Hg and 2 L of flow. This additional intrathoracic positive pressure helps to prevent loss of visual field during the mediastinal dissection with expansion of the native lung from positive pressure ventilation with general anesthesia.
Under vision, the pectus introducer is placed into the right hemithorax. With the tip of the pectus introducer at the junction of the sternum and the anterior pleura, a cephalocaudad rubbing/abrasion is used to bluntly create a space in the right-sided pleura and begin the dissection in the anterior mediastinum. Once a small space is created, the surgeon uses the pectus introducer to displace the pericardium in a posterior direction and away from the sternum by moving the tip of the instrument in an anterior–posterior direction (Fig. 13.4). Moving slowly and cautiously across the sternum with the tip of the pectus introducer firmly pressed anteriorly against the posterior sternum, the surgeon enters through the contralateral left pleural space above the pericardium and into the left hemithorax.
Figure 13.3 Right-sided thoracoscopic view of pectus excavatum defect.
Figure 13.4 Retrosternal dissection using the pectus introducer with posterior deflection of the adjacent pericardium.
Once the tip of the pectus introducer is palpated at the previously marked left-sided intercostal space, the pectus introducer is forcibly directed anteriorly to exit the left hemithorax at the most superior portion of the left-sided pectus ridge. A small retractor is placed to lift the anterior margin of the left lateral chest wall incision, so that the left-sided surgical team can visualize the tip coming through the intercostal muscles (Fig. 13.5). The pectus introducer can most frequently be introduced bluntly through the intercostal muscles. In some instances, the tough pericardium will be caught by the tip of the pectus introducer and brought to the surface even through the intercostal muscles. The surgical team should be aware that if this white fibrous pericardial tissue is seen, the pectus introducer needs to be withdrawn and placed again after extending the dissection into the left hemithorax and displacing the pericardium posteriorly.
If the depression is so severe that the surgeon cannot keep the tip of the pectus introducer in view during the retrosternal dissection, a second bar should be placed one to two intercostal spaces superior to the point of greatest depression. Elevating the more superior sternum almost invariably improves the sight lines for the lower bar to be placed safely.
Figure 13.5 View from the head of the operating table: Pectus introducer exiting the left chest at the pectus ridge just lateral to the retractor.
Figure 13.6 Orange aluminum templates contoured to the patient’s chest wall for planned shaping of pectus bar. The template helps to determine the bar length that would be appropriate for the patient. Cotton cloth cord has been divided and separated for the planned bar placement.
Once the pectus introducer has been placed across the anterior mediastinum and brought out onto the anterior surface of the left chest, the pectus introducer is forcibly lifted in an anterior direction, often several times, almost to the point that the pectus excavatum defect appears and corrected by the pectus introducer. This maneuver may help to prevent intercostal muscle injury when the bar is flipped.
A long length of cloth cord tape (approximately 100 to 110 cm) is introduced to the eyelet at the tip of the pectus introducer, and then the pectus introducer is withdrawn under vision from the left hemithorax, through the anterior mediastinum, and back out through the right-sided chest wall incision. The cloth cord tape is divided at its midpoint, with one cord to guide the pectus bar through the dissection plane while the other cord is reserved as a safety measure in the event there is a problem with the introduction of the pectus bar, and the initial cloth cord tape breaks or unties from the pectus bar.
We use the orange aluminum template to determine the approximate length and shape of our planned correction (Fig. 13.6). As with the pectus bars, the templates are serially sized from lengths of 7 to 17 in in 1-in increments.
Using the tabletop bar bender and the hand-held bar bender, the pectus bar is individually shaped to the patient’s “corrected” chest wall contour. Once shaped, one of the cloth cord tapes is tied through the opening at the end of the pectus bar. The bar with the convex side directed posteriorly is then introduced from the left hemithorax, underneath the sternum through the anterior mediastinum (Fig. 13.7), and out through the right hemithorax under vision. When first placed, the convexity of the pectus bar is facing posteriorly toward the heart, and the ends of the bar will be facing anteriorly and above the anterior chest (Fig. 13.8).
Figure 13.7 Thoracoscopic view from the right chest showing pectus bar exiting the mediastinum.
Figure 13.8 Pectus bar after placement with convex side positioned posteriorly.
The thoracoscope is removed from the chest as the rotation of the bar may catch the telescope and break it. The bar flippers are then placed at the end of the bars and rotated simultaneously (Fig. 13.9). The bars may be rotated in a superior–inferior direction or in an inferior–superior direction. Occasionally, if we are concerned that after the initial rotation the bar placement is not stable on the anterior chest when we check thoracoscopically (the bar appears angled rather than lying flat against the sternum), we will rotate the bar in the opposite direction to see if the bar appears more stable and flat against the anterior musculoskeletal wall.
If the correction does not appear to be sufficient, the bar may need to be withdrawn and reshaped using the bar benders. With an inadequate correction, we first check to determine if there has been posterior displacement of the bar, typically seen by tears in the intercostal muscle posteriorly. This intercostal tearing is a particular problem for the adult patient and in those patients who are having repair of a recurrent pectus excavatum defect. If the intercostal muscles cannot support the bars, the use of an additional pectus bar is almost certainly needed. If there is no tear in the intercostal muscles, the bar is removed and can be reshaped to ensure an adequate correction.
Once satisfied with the correction, the stabilizing bars are placed over the ends of the bar and secured with a 22-gauge wire to prevent pectus bar rotation. The stabilizing bars and the pectus bar itself are then secured to the muscular chest wall using a series of interrupted 0 Vicryl sutures. We prefer the UR-6 needle as the 5/8 needle curve passes easily from the muscular chest wall and through the eyelet of the stabilizing bars and pectus bar.
Figure 13.9 Pectus bar flipped 180 degrees into position with immediate correction of the chest wall deformity.
While the surgical teams are securing the pectus bar and stabilizing bars to the muscular chest wall, the thoracoscope is removed and a 16-French chest tube is passed through the Thoracoport and directed superiorly. The Thoracoport is slid backward over the chest tube and removed from the right hemithorax. The chest tube is then placed to Pleur-evac drainage at −20 cm of water during the closure. We previously used red rubber catheters passed through the Thoracoport, aspirating the pleural spaces with 60 mL irrigation syringes but found that several patients had moderate, often bilateral, pneumothoraces identified on their postoperative x-rays. The use of the chest tube and Pleur-evac suction during closure has essentially eliminated that problem. After the surgical sites are closed, the patient is placed in Trendelenburg position and airplaned with the left side down, and several large, positive pressure inspiratory breaths are given by the anesthesia team to help evacuate any residual intrathoracic carbon dioxide. Once the Pleur-evac shows that there is no additional carbon dioxide to be evacuated, a U-suture is placed at the chest tube site, and the chest tube is removed.
If two bars are needed for the repair, we typically make separate incisions for each bar placement. The superior bar is also positioned over the serratus anterior muscle, but we will then tunnel the superior bar under the pectoralis major and minor muscles when we create our tunnel for bar placement. If there is definite need for two bar placements because of the length of the pectus excavatum defect or because it is a second repair, placing and turning the initial bar at the less severe portion of the defect often facilitates instrumentation and bar placement at the more severely depressed portion.
POSTOPERATIVE MANAGEMENT
A postoperative chest x-ray is obtained. As mentioned in techniques, the placement of a chest tube through the Thoracoport site placed to negative suction drainage at the end of the operative procedure has minimized the risk of postoperative pneumothoraces of significance. If there is a persistent air leak seen in the operating room (almost only seen in repair of a recurrent chest wall defect), the chest tube will remain in place and to suction until the air leak is no longer seen.
Most postoperative care is primarily directed at pain management, and it can be a challenge. Our own pain management is the use of an anesthetic–narcotic mix (bupivacaine–hydromorphone; bupivacaine–fentanyl) delivered by epidural via a patient-controlled epidural pump and regular administration of nonsteroidal anti-inflammatories. As many of our patients have anorexia in the early postoperative period, intravenous ketorolac is typically given in the first 48 hours. We continue regular dosing of naproxen or ibuprofen once the patient’s appetite improves. As the patient tolerates diet, we transition to oral pain medications including oxycodone or hydromorphone and continue the oral nonsteroidal anti-inflammatories. Once the pain is controlled with oral medication, the patient is ready for discharge, typically on the fourth to sixth hospital day.
Before discharge, standard posteroanterior and lateral chest x-rays are obtained. In particular, the lateral chest x-ray helps to determine the baseline position of the bar. This baseline position is an important comparison point to determine if bar rotation has occurred, particularly if there are complaints of chest pain or “bar movement” at any point in the postoperative recovery.
As many patients are discharged home with oral opioids for continued pain management, the bowel management program begins in the hospital with the initiation of diet, using polyethylene glycol and docusate sodium. These medications continue until the patient is evacuating regularly and without discomfort. If the patient has not been able to evacuate before discharge, we strongly encourage them to accept an enema as severe constipation at home has been an issue for some patients.
There is no restriction in sleeping position although most patients will sleep in the supine position in the early recovery. Many patients report that they are more comfortable with a slight head up position, and a reclining cushioned chair is often reported as the most comfortable sleeping platform.
The patients ambulate with assistance on the first postoperative day and are encouraged to sit for short periods of time (15 to 30 minutes) in their room. As the postoperative pain abates, further activity is encouraged. At home, a walking program is initiated and recommended to continue at discharge. The program should gradually advance the distance that is covered over the first 7 to 14 days at home. Weightlifting of moderate to heavy weights is not allowed in the first month after the surgery. As the patient becomes more comfortable, aerobic activity such as running or swimming can resume, even in the first month. Activity that is associated with torso rotational motion is not allowed for the first 2 postoperative months. This includes most sports, particularly tennis, golf, baseball batting, hockey, and soccer. After 2 months, we allow our patients to resume sports, including contact sports.
At the first postoperative visit, it is our practice to give the families a letter for airplane travel, explaining that metal hardware has been surgically placed, and we encourage our patients to obtain a medical alert bracelet to identify the pectus bar in the chest in the event of an emergency.
Bar Removal
Although the bars may remain in place without the need for removal, most patients report vague chronic pain and discomfort at the chest wall site and removal of the bars would be indicated. For the best long-term result and to minimize the risk of recurrence, the bars need to remain in place for at least 2 years. In our own experience with older patients, we typically recommend leaving the bar in place for a longer period of time, typically 3 to 4 years. In these late teenage patients and young adults, we may see chest wall remodeling into the third and fourth year. An examination is performed, and we do perform CT imaging of the chest to check surgical anatomy, location of the bar, and to determine if there is heterotopic calcification around the bar before removal.
Unlike bar placement, the bar removal is typically performed as a day surgery operation. As it has been our practice to place stabilizing bars, if possible, on both ends of the pectus bar, the surgery begins by opening through the previous incisions and dissecting down to the fibrous capsule that surrounds the pectus bar and the stabilizing bars. These capsules are embedded in the serratus anterior muscle. The stabilizing bars are removed after removing the wires used for fixation. The pectus bar is straightened using bar benders and then withdrawn through either the left or right chest. Thoracoscopy is not required for bar removal.
With dissection into the muscles, there is discomfort although this postoperative pain is typically managed easily by oral analgesics. The procedure is performed as an outpatient surgery, and the patient is discharged home after awakening in the recovery room. A postoperative chest x-ray is obtained to exclude a pneumothorax.
Six months following bar removal, exercise pulmonary tests are performed as a baseline to determine if exercise tolerance has remained stable, worsened, or improved. In our experience, we almost invariably find that the values are stable or slightly improved.
COMPLICATIONS
Early Complications
Early complications are problems commonly associated with chest surgery. In our own experience, we have not had to place a chest tube for a pneumothorax, pleural effusion, or hemothorax in the typical preteen or teenager who presents for an initial repair. Patients undergoing repair of recurrent pectus excavatum defect do have a higher incidence of pleural adhesions and are at greater risk for postoperative air leaks. In those cases, if an air leak is found when the chest tube is placed to evacuate the carbon dioxide following the Nuss procedure, the chest tube is left in place until the air leak ceases, typically within 48 to 72 hours.
Our practice is to continue antibiotics for 48 hours following the hardware implantation. Infection is unusual but has occurred once. Suture reactions will occur in rare patients and reports with a larger population suggest that this problem is present in approximately 1% of their cases.
With postoperative chest wall pain, atelectasis is common on follow-up x-rays although clinical symptoms associated with atelectasis (cough and fever) are infrequent as our practice is to encourage early mobilization and incentive spirometry is started on the afternoon of the operative day. On occasion, pleural effusions complicating the procedure are identified although the need to drain a pleural effusion has not occurred in our practice, and a large pleural effusion requiring drainage would be considered a rare complication in larger series (less than 1%).
Late Complications
Identification of technical problems that occurred in the initial Nuss repair experience led to compositional changes in the pectus bar as well as development of hardware and techniques to stabilize the pectus bar. During that early experience, bar displacement or rotation was considered a common problem, occurring in up to 15% of cases. More recent experience would suggest that the incidence of bar rotation or displacement is low but may occur in 2% to 5% of cases following an initial repair. This rate of displacement is higher in an adult patient and in patients undergoing repair of a recurrent pectus excavatum defect.
In the current era, the risk of retrusion (recurrent sternal depression) after bar removal appears to be in the range of 1% to 2%, a risk that appears to be lower than the risk of recurrence that has been previously reported with the open repair of the pectus excavatum defect.
Even after screening for a nickel allergy, a macular, erythematous rash, typically limited to the distribution of the bar may appear early or late in the postoperative course. This rash may be along the entire path of the pectus bar or limited to one or both surgical sites. Other skin and soft tissue problems, including the sudden appearance of granulation tissue and swelling at the lateral chest wall incisions, may be the first manifestation of a nickel or metal allergy. Interestingly, some children, who develop the subcutaneous and soft tissue signs after pectus bar implantation, are not positive for a nickel allergy on subsequent testing. Persistent pleural effusions have also been associated with the placement of the pectus bar in a child with a known metal allergy. At many centers performing the Nuss procedure, testing for a metal allergy has become part of the routine preoperative screening and should be considered postoperatively in patients who have patchy erythema around the location of the pectus bar. If a nickel allergy is discovered after bar placement, a short course of systemic steroids may avoid the need for bar removal. In those rare patients who recently have had corrective surgery but had serious cutaneous manifestations including skin breakdown, replacement of the bar with a titanium pectus bar has been shown to resolve the allergic response while preserving the pectus correction.
Rare patients have chronic severe pain that is poorly explained by the operative repair and appears to be neuropathic. In our own experience, one patient had the bar removed at 7 months because of the severity of the chest wall discomfort. However, an additional 1% to 2% of patients in our experience have had additional consultation by the pain service to try to address this chronic neuropathic pain. In some instances, additional psychiatric consultation may help design a program of behavioral modification, meditation, or guided visualization, and occasionally prescribe medication that can help with anxiety that may be associated with chronic pain.
Figure 13.10 A: CT imaging of a patient with a Haller index of 11.2 before surgical correction. B: Haller index of 2.6 in the preoperative evaluation before bar removal, 4 years following the Nuss repair.
Unfortunately, catastrophic cardiac injury has occurred with placement and removal of the bar and needs to be considered as a potential risk that is seen with the Nuss repair and unlikely to occur with the more classic Ravitch repair. In some centers, a sternotomy operating room surgical setup is immediately available as part of their practice with bar placement and/or removal.
RESULTS
The reports of improvement in the physiologic parameters following the Nuss pectus excavatum repair are mixed. Studies vary with some showing an improvement in static pulmonary function although other reports show stable but unimproved pulmonary function in postoperative testing. Most authors agree that with successful correction of the pectus defect using the Nuss repair, exercise stress test does show improvement in cardiac function with improved cardiac filling and an increased stroke volume with exercise, possibly related to relief of right-sided cardiac compression (Fig. 13.10).
Studies evaluating both quality of life and overall patient satisfaction show that the patients and their families perceive a significant improvement in the appearance of the chest wall (Fig. 13.11). Most report that the surgical correction is good to excellent in greater than 90% of cases. This perceived improvement often is associated with improved psychosocial scores on standardized testing.
Figure 13.11 Preoperative and immediate postoperative appearance of the chest wall following Nuss repair of a moderate pectus excavatum defect.
CONCLUSIONS
Modifications and improvement of hardware and technique have led many pediatric surgeons and some thoracic surgeons to use the Nuss repair as their preferred technique for pectus excavatum repair. As experience has been gained, the length of time the hardware needs to be in place has been elucidated, the importance and technique of stabilization has minimized the early risk of bar rotation, and modifications of the composition of the hardware has resulted in less malleable bars that are able to withstand posteriorly directed forces of the pectus defect. In our own practice, placing a chest tube through the Thoracoport site at the end of the procedure has essentially eliminated postoperative pneumothoraces.
Overall, our experience with the Nuss repair of pectus excavatum defects has been rewarding. Like many groups, our initial experience was with the pediatric patient. We gradually extended the use of this minimally invasive surgical correction to adults for a primary repair, as well as pediatric and adult patients with recurrence of the pectus excavatum defect following open or previous Nuss repairs. The patient satisfaction following repair is generally excellent, with improved appearance of the chest, the subjective feeling that exercise tolerance is better, and the psychosocial benefits of an improved body image.
Recommended References and Readings
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Kelly RE, Goretsky MJ, Obermeyer R, et al. Twenty-one years of experience with minimally invasive repair of pectus excavatum by the Nuss procedure in 1215 patients. Ann Surg. 2010;252:1072–1081.
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