141: Surgical Repair of Complex (Recurrent) Pectus Excavatum in Adults

Since the first pectus repair was reported by Meyer in 1911, several different techniques have been described.^1,2 The Ravitch procedure, first described in 1949,³ became the mainstay of repair until Nuss described a minimally invasive repair in the early 1990s.⁴ The techniques for primary repair of congenital chest wall deformities, including pectus excavatum, are described in Chapter 140. None of these techniques is perfect, however, and recurrences do occur. Although the incidence of recurrent pectus excavatum in the adult population is most rare, it is usually a consequence of technical failure. The rate of recurrence, although significantly reduced in the hands of a more experienced surgeon, ranges from 2% to 10%.

The open repair, described by Ravitch and modified by Haller,⁵ involves the excision of all deformed costal cartilages from the sternum to the costochondral junctions. The overlying perichondrium is left intact. This procedure is combined with a transverse sternal osteotomy at the point of maximal declination, elevation of the inferior sternal fragment, and placement of a transverse metal bar or rod to maintain the sternum in this elevated position. The ends of the bar are supported on either side by the bony ribs of the lateral chest wall.^4,6 The bar is left in place until the costal cartridges have regenerated and the chest wall has become firm and rigid. This process usually takes 6 to 9 months in adults.

The rates of recurrence from a series of experienced centers are depicted in Table 141-1. The most common reasons for recurrence may be divided into two categories: technical and disease-related. As with any operation associated with remodeling in which there are several sequential steps to which one needs to adhere, there is a learning curve. Failure to tackle the full extent of the deformity aggressively, inadequate stabilization of the bar resulting in early displacement, premature removal of the bar before adequate healing has taken place, failure to resect the xiphoid process and mobilize the retrosternal space, significant injury to the perichondrial sheaths, and failure to pay sufficient attention to the asymmetry of the defect, all can result in a technical failure of the primary repair.

Patients with connective tissue disorders such as Marfan syndrome are at increased risk for recurrence. In these instances, the repair should be delayed until skeletal maturity has been reached. Also, children who undergo a rapid adolescent growth phase may overcome the benefits of an early repair, resulting in a suboptimal cosmetic outcome. The appropriate timing for surgical correction of a pectus deformity is the subject of controversy and continues to be debated.

Since introduction of the minimally invasive Nuss technique, an increasing number of children are being treated for pectus deformities according to this method. The benefits of the Nuss technique include the avoidance of extensive dissection and cartilage resection and smaller incisions. The complications of this procedure include severe postoperative discomfort or pain, longer periods of bar retention before removal, catastrophic injuries to the heart and pulmonary outflow tract, and inadequate cosmetic results with asymmetric defects. Miller and colleagues report a series of children (mean age 11.4 years) who underwent a redo of the minimally invasive repair after a failed initial procedure. The average time to repeat procedure was approximately 9 years.⁷ In adults with recurrent pectus deformity, the role of minimally invasive repair is unproved and may not permit a satisfactory or safe repair.

The selection of patients for repair of recurrent pectus deformities should be undertaken very carefully. Unfortunately, not every patient can undergo intervention. In a series of 19 patients who had an original Ravitch procedure and presented with recurrence, three patients had such severe adhesions between the sternum and pericardium that reconstruction was too dangerous to attempt.⁸ The most important questions to be answered before undertaking a reoperation include the underlying physiologic status of the patient, the reasons for failure of the primary repair, and the patient's expectations of a satisfactory cosmetic result.

A chest CT scan is a useful tool to assess the degree of deformity and asymmetry, to determine the extent of substernal and pleural adhesions, and to assess the presence of cardiac displacement or compression. This information is helpful in planning and carrying out a successful reoperation. Patients who are considered for reoperation should undergo preoperative physiologic testing, including a battery of pulmonary function tests and an echocardiogram. Should underlying cardiac disease be identified, this needs to be addressed before embarking on an elaborate repair. Patients must be adequately counseled on their reasons for seeking a reoperation and warned of the potential surgical risks, including an unsatisfactory cosmetic result. A multidisciplinary approach with involvement of the plastic surgery service also should be considered.

Anesthesia

The use of an epidural catheter is an important adjunct in patients undergoing reoperation because it will alleviate postoperative pain and reduce morbidity. General anesthesia is necessary because the operation is usually protracted, and there is significant dissection. Hemodynamic monitoring may be necessary depending on the patient's underlying physiologic status. Patients usually are extubated at completion of the repair.

Surgical Management

Reoperative Dissection and Sternal Mobilization

The task of repairing a recurrent pectus deformity may be formidable depending on the nature of the recurrent deformity and the amount of scar tissue encountered. Few centers have had a vast degree of experience with recurrent operations in the adult population.

The incision for the open technique is usually made through the old scar. If the old scar is hypertrophied, it should be excised. The skin and muscle flaps are raised superiorly, inferiorly, and laterally to encompass the full extent of the defect by using the electrocautery unit. Scar tissue may render identification of tissue planes more difficult than in a primary operation, but the dissection needs to be carried down to the chest wall to fully expose the sternum and regenerated cartilaginous matrix.

The subsequent dissection will vary depending on the previous operation. Extensive cartilage resection or injury to the overlying perichondrial sheaths may have resulted in a chaotic array of costosternal connections with no identifiable tissue planes or points of demarcation between one costal cartilage and another. Whereas with a virgin operation, a delicate dissection of the subperichondrial space in a bloodless plane is feasible, in a reoperation, a stone hard outline of the cartilage will be observed, and typically, there is no obvious tissue plane of dissection identified. The goal of the dissection is to excise a sufficient amount of cartilage to achieve adequate anterior mobility of the sternum. (Ideally, a lateral dissection beneath the pectoralis muscles where the territory is less disrupted and the ribs are intact may be pursued.) This frequently also requires detachment of the cartilaginous connections with the sternum and a sternal osteotomy. Both a rongeur and bone cutters can be used to remove the hardened tissue of the fibrocartilaginous plate. Ideally, a layer of scar should be left behind on the chest wall, lateral to the sternum and medial to the osseous portion of the rib, to provide a matrix for hardening and regeneration postoperatively. Careful preservation of the perichondrium is a key maneuver during this stage of the operation.

Osteotomy and Strut Support

As with a primary repair, an osteotomy is performed at the angle of maximal declination. In rare circumstances, creation of a second osteotomy may be required to achieve the best apposition. If the cartilaginous–sternal connections have been severed, they should be reapproximated using an absorbable suture. Then a bar/strut is placed beneath both the sternum and the excised fibrocartilaginous plate to maintain the repair in its desired position (Fig. 141-1). It is critical to choose the correct length, position, and contour of the bar. The bar is passed through the fibrocartilaginous matrix to rest on the bony chest wall above the serratus musculature and should extend from midaxillary line to midaxillary line. To accommodate the desired contour of the chest wall, the bar must be positioned beneath the sternum midway between the point of osteotomy and the sternal tip. The bar should not be bent in too much at its ends because it may erode into the intercostal muscles and penetrate the surface of the lung. Neither should the bar be too straight at its ends such that it rubs on the over-lying skin and is uncomfortable for the patient. The bar is held in position by suturing it to the underlying chest wall and sternum on either side with heavy absorbable sutures. Once the bar is deemed to be in the correct position, a closed drainage system is placed beneath and above the sternum. Secure attachment of the xiphoid to support the rectus muscles and cover the lower chest is critical. The pectoralis major and rectus abdominis muscles are then reapproximated in the midline, and the skin is closed (Fig. 141-2).

Postoperative Management

The patient is extubated at completion of the operation, and postoperative pain is controlled with the use of a continuous thoracic epidural catheter. Early ambulation is encouraged, and a transition toward oral narcotics is made within 48 hours of the operation. The patient can be discharged with the closed drainage system in place 3 to 5 days after operation. The drains are left in place until the drainage is less than 30 mL per day to prevent seroma formation.

The metal bar should be left in place for 6 to 9 months after the operation to prevent recurrence of the deformity.⁹ When the time comes to remove the bar, caution is required because scar tissue overgrowth may render removal hazardous. Continuous, slow traction on one end usually results in ready removal of the bar.

Complications related to recurrent pectus repair are similar to those experienced during routine pectus surgery and include pneumothorax, pneumonia, wound infection, infection of the prosthetic bar, seroma, and bar migration. These have been discussed previously. Recurrence after a redo repair is rare if the preceding principles are maintained.

For the patient with a pectus excavatum, the condition often represents a significant source of physiologic and psychological stress. Fortunately, this is more widely recognized by all health care practitioners caring for children, and patients are now referred for surgery at an earlier age. As these children progress to adulthood, it can be expected that a small proportion will present with recurrent pectus excavatum deformities that produce symptoms or serious cosmetic concerns. Whether these recurrences are due to imperfect initial operations or other factors, they require operative revision.

The repair of a recurrent pectus excavatum defect can be a complicated ordeal and poses a significant challenge for the surgeon. The decision to undertake a recurrent pectus repair should not be considered lightly and requires a frank discussion with the patient and a realization that the end result may not be cosmetically perfect. The surgical technique used will depend on a number of factors, including the previous operative technique, individual patient factors, and the extent of the underlying fiber-cartilaginous scar tissue. Strict adherence to sound surgical principles, as well as a certain degree of surgical creativity, as our index case illustrates, can lead to a very successful reoperation in most instances.

A 34-year-old man was referred for recurrent pectus excavatum. He had undergone a primary repair of his pectus excavatum deformity as a 3-year-old child. Over the last 10 years, he had been experiencing progressively worsening shortness of breath, resulting in severe limitation of his physical activity. His past medical history was significant for mitral valve prolapse. The patient was otherwise healthy and had no known history of connective tissue disorder or Marfan syndrome.

On physical examination, the patient was tall and slender with a moderate pectus excavatum deformity. The old midline incision was identified over the lower half of the sternum and was well healed. Cardiac examination revealed a middle to late systolic murmur and a midsystolic click consistent with mitral valve prolapse. The lungs were clear to auscultation. Pulmonary function tests demonstrated a forced vital capacity of 3.11 (58%) and a forced expiratory volume in 1 second of 2.82 (63%). A posteroanterior and lateral chest x-ray is shown in Figure 141-3.

A chest CT scan was ordered to better evaluate the extent of the patient's deformity. It is most notable for compression of the right ventricle and displacement of the heart to the left chest, as well as asymmetry of the lung fields (Fig. 141-4).

An echocardiogram was consistent with mild global decreased function, mild mitral regurgitation, and an estimated pulmonary artery systolic pressure of 21 mm Hg plus right atrial pressure. Although the echocardiographic findings were not entirely consistent with his symptoms, the patient was subjectively feeling more tired and had a definite decrease in his exercise tolerance. He wished to have the deformity corrected.

Operation

A midline incision was made, and the old scar was excised. Musculocutaneous flaps were developed bilaterally from the midline to the costochondral junctions and from the point of maximal declination superiorly to the sternoxiphoid junction inferiorly. On inspection, the costal cartilages were noted to be turned inward. The involved cartilages on either side of the sternum were removed, leaving the posterior perichondrium intact. A sternal osteotomy was made just below the manubrium through the anterior and posterior tables, allowing the caudal portion of the sternum to be deflected anteriorly. A second transverse osteotomy was performed about 12 cm inferior to the first to prevent protrusion of the xiphosternal junction. The sternum was then reapproximated using sternal wires, and a bar was used to support the repair.

An omental flap was harvested by extending the incision inferiorly and opening the peritoneal cavity. The omentum was delivered into the wound to fill the large existing space created by correction of the deformity. The pectoral muscles then were reapproximated in the center using absorbable sutures. Closed suction drains were placed, and the remainder of the wound was closed in layers.

The patient was discharged home after 4 days, and the closed suction drains were removed during the first postoperative visit. The patient had marked improvement of his cardiopulmonary function over the ensuing months, and the support bar was removed 1 year later through a small left chest incision as an outpatient procedure.

The authors have highlighted the key surgical principles important in the surgical repair of a recurrent pectus excavatum, carefully outlining when – and when not – to operate, and reviewing the common technical reasons for recurrence and the pitfalls to avoid.