Pectus Excavatum Treatment & Management

No effective nonoperative management strategies can correct of severe pectus excavatum. Several exercise programs have been suggested. However, no substantial scientific evidence has determined that they are effective in achieving anatomical correction.

External braces have also been used in the nonoperative management of pectus excavatum, but no experience with this approach in the treatment of chest wall deformities in North America has been reported. Pectus carinatum is more likely to improve with the consistent use of an external brace because the exogenous compression of the sternum and anterior ribs by the brace may, over time, result in some degree of correction of the deformity. Good results are unlikely to be achieved with external bracing techniques in the correction of severe pectus excavatum. For more information on this subject, see The Orthopaedic Conservative Method and Information on Pectus Deformities.

Until the mid 1990s, the operative treatment of pectus excavatum had been fairly well standardized and was based on the open operation originally described by Ravitch in 1949.^[6] Few variations to his original description have been added, and the operation became almost universally accepted by pediatric surgeons as the standard for treatment of this condition.

Several published series demonstrated excellent results with low complication rates despite the fairly radical nature of the Ravitch operation, which required anterior chest wall exposure, creation of muscle and skin flaps, extensive cartilage resection, and sternal osteotomy. The images below are operative photographs of the open Ravitch technique for pectus excavatum repair. The surgical exposure of the anterior chest wall, creation of skin and muscle flaps, and resection of the affected cartilages and sternal osteotomy are visible.

The image below is an illustration of another open operative technique used rarely today, the so-called "sternal turn-over operation."

In 1987, during the early stages of laparoscopic and minimally invasive surgery, the creative mind of a pediatric surgeon from Virginia, Donald Nuss, MD, challenged the surgical dogma.^[7] Nuss performed the first minimally invasive operation for the correction of pectus excavatum. Not until May of 1997 was this new and innovative technique introduced to the American Pediatric Surgical Association and, subsequently, published in the Journal of Pediatric Surgery. Because of the excellent results obtained with this new technique and because of the less radical nature of the operation, the popularity of this technique has grown dramatically. The images below illustrate the preoperative and postoperative appearance of the chest in a female and male patient after minimally invasive repair.

Indications for surgical repair

Operative correction should be considered in patients who present with pectus excavatum and cardiopulmonary impairment. The most common goal in operative repair of pectus excavatum is to correct the chest deformity. This is particularly important in teenagers, in whom the appearance of the chest can result in significant problems related to body image and self-esteem. Thus, the desire to improve the appearance of the chest is considered an appropriate medical indication for surgery. The images below illustrate the dramatic appearance of pectus excavatum in young male and female patients.

Other indications include exercise and physical activity limitations, evidence of cardiac or pulmonary dysfunction, chest pain, psychological distress, and potential future need for sternotomy (open-heart surgery). Adult patients with pectus excavatum who undergo open-heart surgery typically have significant displacement and rotation of the heart to the left chest. This can make the operative approach to the heart at the time of open-heart surgery difficult and challenging. With this in mind, elective repair of the pectus deformity prior to open-heart surgery may be indicated in selected cases.

Minimally invasive surgery for repair of pectus excavatum

The clinical observations that provided the rationale for developing a less invasive operation that would allow for the anatomical correction of pectus excavatum deformity are listed below.^[8]

First, children have a very soft and malleable chest. Second, the phenomenon of chest remodeling is well known in adult patients with emphysema who develop a barrel-shaped chest. If the chest wall in older adults can be reconfigured, the same should be possible in children and teenagers because of the increased malleability of their anterior chest wall. Third, the use of braces and internal fixating devices has allowed orthopedic surgeons and orthodontists to correct skeletal anomalies such as scoliosis, club foot, and maxillomandibular malocclusion. The anterior chest wall, which is quite malleable, is ideal for this type of correction.

Such observations resulted in a technique in which a convex stainless-steel bar is placed under the sternum through a small lateral thoracic incision to correct the condition known as funnel chest. Thus, the minimally invasive repair of pectus excavatum (MIRPE), also known as the Nuss technique, was born.^[9]

Prior to surgery, the chest is measured from the midaxillary line to midaxillary line to determine the approximate length of the bar required, and the correct length is typically obtained by subtracting 1 cm from that initial measurement. A mild bowel preparation 1 day before surgery may be helpful in decreasing problems related to postoperative constipation. Administer a first-generation cephalosporin antibiotic (cefazolin) approximately 1 hour prior to surgery and continue for at least 48 hours or until the patient is afebrile. As a rule, patients can be admitted on the same day of surgery.

The combination of general endotracheal anesthesia and a thoracic epidural is considered ideal. The epidural catheter is left in place for up to 3 days following the operation, providing excellent adjuvant therapy to pain management techniques. Patients and families must understand that, although the technique is considered minimally invasive, postoperative pain and discomfort can be significant because of the forceful bending of the sternum and cartilages. An indwelling Foley catheter is placed because urinary retention is common with thoracic epidurals. Orogastric tube decompression of the stomach is recommended only during the operation. Hemodynamic monitoring by the anesthesiologist does not necessitate central venous or arterial lines.

Surgical technique

The patient is placed in the supine position with both arms abducted at the shoulders to allow access to the lateral chest walls.

The patient is prepared for surgery and draped such that the entire anterior chest is exposed, including the lateral chest wall (broad exposure).

The chest is marked with a sterile marking pen in the deepest portion of the pectus (making sure that it is not inferior to the sternum), on the corresponding intercostal spaces on the right and left sides where the bar is to be inserted, and on the points on the pectus ridge that correspond to the horizontal plane from the deepest point of the pectus to the lateral chest wall incisions.

At this time, the measurement of the Lorenz pectus bar is reconfirmed using the marks made on the chest. The length of the Lorenz bar should be measured from the mid-axillary line in one side to the opposite mid-axillary line. The length of the bar is measured in inches. A typical measurement for a teenage patient may range from 13-inches.

The bar is bent from the center out to either end, making small gradual bends with a Zimmer bar bender. The curvature (convexity) of the bar is shaped to fit each individual patient's chest. Occasionally, slightly exaggerating the curvature to allow for the anterior chest wall pressure that may alter the original configuration of the bar may be necessary. The bar must fit snugly over the chest.

A transverse 2-cm skin incision is made on the midaxillary line at the level of the skin marks in line with the deepest point of the depression on the right and left sides.

A skin tunnel is raised anteriorly from both incisions to the top of the pectus ridge at the previously selected intercostal space; the skin pocket is extended posteriorly to allow for the distal end of the pectus bar to hug the chest wall posterior to the midaxillary line.

A thoracoscope is inserted at this point. The authors recommend placement of a 5-mm trocar 1-2 intercostal spaces below the space that has been chosen for the pectus bar on the patient's right side. The image below illustrates the placement of the thoracoscope. A 30° thoracoscope provides excellent visualization of the pleural cavity, lung, and mediastinal structures.

If necessary, the scope can be used bilaterally. Insufflating the pleural cavity with carbon dioxide is rarely necessary; in most cases, controlled ventilation by the anesthesiologist with small tidal volumes results in limited lung expansion and good thoracoscopic visualization of vital structures.

Using a thin but deep retractor, the skin incisions are elevated and the intercostal space previously marked is identified. A long instrument, such as a 15-in Crawford vascular clamp or a Lorenz pectus introducer (S-shaped device), is inserted through the appropriate right intercostal space at the top of the pectus ridge, in line with the point that corresponds to the deepest depression of the sternum (previously marked). The image below illustrates the steps described here.

The clamp or introducer is slowly advanced across the anterior mediastinal space immediately under the sternum with careful videoscopic guidance. Always face the point of the instrument anteriorly (away from the heart) and maintain contact with the sternum to avoid injury to mediastinal structures.

The sternum is forcefully lifted as the instrument is passed to the contralateral side. Monitoring for cardiac ectopy is important to ensure that the instrument is not near the heart or pericardial sac.

Once the instrument is passed behind the sternum, the tip is pushed through the intercostal space at the top of the pectus ridge on the left side (also previously marked) and brought out through the left skin incision. Thoracoscopy on the left side is not usually necessary unless the position of the instrument in the left chest is uncertain.

The Crawford clamp, if used, is advanced such that the tunnel space created is enlarged; if the Lorenz pectus introducer is used, further dilating the space is not necessary. Note that the Lorenz pectus introducer comes in 2 sizes: short, for younger patients aged 4-12 years who have a small chest, and long, for older and larger patients aged 13-20 years.

Using the clamp or the introducer, 2 strands of umbilical tape are pulled through the tunnel; one tape is used as a spare. See the image below.

One of the tapes is used to guide the previously prepared pectus bar through the tunnel and anterior mediastinal space using traction on the tape and concomitant thoracoscopic visualization. The bar is inserted with the convexity facing posteriorly. The image below is an illustration of the bar in place before it is turned over.

Using a Lorenz pectus bar rotational instrument (also known as a "bar flipper"), the bar is turned over so that the concave part now faces posteriorly (to the mediastinum) and the convex part faces anteriorly. The ends of the bars are placed in the subcutaneous tissue, anterior to the muscle fascia (not under it and not within the muscle tissue). Again, the bar must hug the chest so that the ends do not protrude under the skin pocket. The "flipping maneuver" is also performed under careful thoracoscopic visualization.

If, after the bar is flipped, the correction of the pectus excavatum is not ideal (either undercorrected or overcorrected), the bar is flipped back, pulled back out, and bent again to fit the patient's chest in order to achieve the best possible correction of the deformity. If pressure has caused the bar to straighten, it is turned over and, using small hand held benders, the curvature is increased as appropriate. This can be repeated as many times as necessary. Typically, only one bar is necessary to correct the deformity, but, occasionally, a second bar may be necessary. The second bar can be placed above or below the first one. The thoracoscope and trocar are removed at this point.

Once the bar is in place, determining its stability is imperative. Such assessment dictates the need for placement of a stabilizing bar. The stabilizer serves to limit rotation of the pectus bar, and it is sutured around the bar and to the muscle only after being properly fitted. Teenagers usually require one stabilizer bar that can be placed on either side of the pectus bar.

With the bar properly placed and stabilized, figure-of-eight sutures are placed to the lateral chest wall musculature. Number 0 nonabsorbable sutures (Prolene) are placed on one side, and absorbable (Vicryl or PDS) sutures are placed on the opposite side. The image below illustrates the positioning of the bar in relationship to the chest wall and muscles.

Additionally, a third point-of-fixation suture can be placed on the anterior chest to the side of the sternum, around one rib and around the pectus bar, in order to provide another point of fixation for the bar, minimizing the chance of bar displacement. The image below illustrates the technique for placement of the third point of fixation.

At this point, the anesthesiologist places the patient in the Trendelenburg position, and large tidal volumes are used in combination with positive-end expiratory pressure (PEEP) so that any residual pneumothorax is eliminated. A chest tube is rarely needed. The subcutaneous tissue and skin are reapproximated with absorbable sutures. Chest radiography is performed as soon as possible to confirm good lung expansion and to reveal the final positioning of the bar.

The patient is extubated deeply to minimize any movement and/or agitation because this may result in bar displacement as the patient thrashes about.

Pain management typically requires keeping the patient well sedated for the first 1-3 days to prevent bar displacement. Medications and therapies depend on the patient's response to pain and may include an epidural catheter, intravenous narcotics for breakthrough pain, patient-controlled analgesia (PCA), and nonsteroidal anti-inflammatory drugs (NSAIDs). In the first postoperative day, the patient is kept flat, with a straight thoracic spine, and is allowed only a small pillow. Mobilization is permitted on the second postoperative day by flexing the bed at the hip level and keeping the back straight. Do not permit waist bending, twisting, and log rolling. Avoid allowing the patient to sit in bed with the thoracic spine flexed. The patient needs assistance when getting out of bed the first few times.

The epidural catheter is generally removed on the third postoperative day, and the patient should be fully ambulatory after that point. The patient is discharged home when able to walk unassisted. The average length of stay is 4-7 days. Good posture with a straight back is very important, even after discharge. Again, bending at the hip and slouching are not allowed in the first month. Regular activity is permitted as pain reduces and mobility increases. Heavy lifting is not permitted for one month following surgery, and contact sports are to be avoided for at least 3 months. The bar generally remains implanted for 2 years and is removed in an outpatient procedure under anesthesia.

To date, only 2 large series that examine complications and outcomes of this new technique have been reported.^{[10, 11]} In those reports, patient and family satisfaction were found to be very good, with excellent and good results reported at 93% and 96%, respectively. However, the only multi-institutional study, which reviewed 251 cases of MIRPE, demonstrated a significant rate of complications (the overall incidence rate of complications was almost 20%).^[11] By far, the most common complication requiring reoperation was displacement of the retrosternal stainless steel support bar (initially reported to occur in 9.5% of all patients). Such displacement can include a 90° rotation, a 180° rotation, or a lateral migration. Teenaged patients are at higher risk for complications, particularly pectus bar displacement, probably because of the increased pressure on the bar generated by a larger chest and more rigid chest cage.

Since the introduction of thoracoscopy and lateral stabilizers, as well as the third point of fixation technique, bar displacement has become quite unlikely, with an estimated incidence of less than 2.5% (prospective studies are currently underway to analyze outcomes of surgery).

The acceptance and popularity of MIRPE developed quickly since its introduction in 1997. The principal advantages of this new technique are based on the fact that incising the anterior chest wall, raising the pectoralis muscle flaps, resecting the rib cartilages, and performing a sternal osteotomy are not needed. This leads to a much shorter operating time, minimal blood loss, and early return to full activity because the stability and strength of the chest wall is not compromised. The apparent simplicity of the technique, combined with the early good results reported, contributed to the enthusiastic widespread use of this operation by many pediatric surgeons.

Unfortunately, a relatively high rate of complications was reported when many different surgeons performed the operation, probably reflecting the learning curve associated with the introduction of this new technique. Since the first MIRPE was performed, the bar has been modified 4 times and is now strong enough to withstand the pressure of even the most severe deformity. The poor results likely occurred early in the reported series because the bar was too soft, was removed too soon, or was not stabilized adequately. Experience has shown that stabilization of the bar is absolutely essential for success, and the use of a lateral stabilizing bar and the third point of fixation (when appropriate) minimizes the occurrence of bar displacement.

Fortunately, most factors that may lead to complications and poor results were related to early inexperience, and these factors have been corrected. Moreover, the introduction of thoracoscopy when performing MIRPE has significantly enhanced the surgeon's ability to pass the bar precisely behind the sternum, avoiding the risk of cardiac or vascular injury. Reassuringly, one reported case of cardiac perforation occurred prior to the routine use of thoracoscopy.

Another significant advantage of MIRPE over the open surgical procedure is that the dreaded complication of "thoracic constriction" (Jeune syndrome) does not seem to occur with this new technique. Chest wall constriction has been described in a few patients following extensive open pectus excavatum operations. Apparently, the bone growth center can be affected, which results in restriction of chest wall growth with marked limitation of ventilatory function. Such patients are very symptomatic and cannot compete in running games. The forced vital capacity and forced expiratory volume in one second is typically decreased by more than 50% of predicted reference range levels. With the MIRPE, because no resection or incision is made on ribs or cartilages, such a complication does not appear to be a problem. Once the cartilage and bony structures are remodeled, normal or improved pulmonary function is established and the flexibility and malleability of the chest remains unaffected.

Critics of the MIRPE claim that the Nuss procedure is too invasive, risky, and not pain free. Proponents argue that this new approach, compared with the open surgery (modified Ravitch operation), eliminates the need for a large anterior chest wall incision with creation of pectoralis muscle flaps, resection of several ribs and cartilages, and sternal osteotomies. The MIRPE allows for a much shorter operating time, minimal blood loss, and minimal anterior chest wall scar. Moreover, the stability and strength of the chest wall is not compromised as it is with the open repair. For a more detailed review of the pros and cons of both approaches, please refer to the article "To Nuss or Not to Nuss? Two Opposing Views" in the Spring 2009 edition of Seminars of Thoracic and Cardiovascular Surgery.^[12]

The current recommendations support the use of MIRPE in patients aged 5-20 years. The ideal age for undergoing this operation is 8-12 years because the chest wall is still very malleable, stabilization of the bar is easily achieved, thoracic epidural can be safely placed, and the child is mature enough to understand the operation and postoperative instructions, particularly incentive spirometry, which is essential for minimizing pulmonary problems after surgery.

Of note, one should not view operative correction of pectus excavatum as an operation limited exclusively to pediatric patients. Indeed, the open technique has been used in adult patients with excellent results. However, experience with the MIRPE in adult patients has been limited to a few cases, reported in anecdotes. Early results seem to indicate that similar principles apply and that operative correction using MIRPE can be achieved in adult patients. Limiting factors include a larger chest wall and poor malleability of the ribs, cartilage, and sternum. A surgeon experienced in the field of chest wall malformations must carefully evaluate adult patients to determine which operation would best correct the anatomical deformity.

An interesting observation has been that complications, mainly bar displacement, have appeared to be more common in teenaged patients. Initially, the MIRPE was limited to the younger prepubertal patients (aged 3-12 y), which probably accounted for the rare occurrence of bar displacement in the first report by Nuss.^[7] Older patients were offered the procedure because of the success of the procedure in young patients. Only later was the importance of proper stabilization of the bar identified and the lateral stabilizing bar was introduced (in 1998), followed by the addition of the third point of fixation technique (in 2000) to provide additional support and stability for the bar. The results seemed to have improved so much that the operation is now considered in adult patients with pectus excavatum.

Consultation with a cardiologist and pulmonologist are not mandatory and are obtained only if indicated based on the medical history and physical examination findings. However, many physicians and insurance companies believe that such an assessment is important prior to surgery. Experience has shown that, unless medically indicated by the presence of cardiopulmonary symptoms or abnormal physical findings, routine preoperative consultation with such specialists is of little benefit in the clinical evaluation and outcome of patients with pectus excavatum. However, their input can be helpful in cases in which insurance companies deny coverage of the procedure.

Consultation with a physical therapist before or after operative repair of pectus excavatum is considered an important factor in good recovery from surgery. The physical therapist assists and instructs patients on appropriate stretching techniques and exercises that aid the patient in the recovery phase. Continue such therapy for at least one month after surgery, when most patients are free of any significant discomfort with routine activities of daily life. Moreover, the physical therapist can assist patients in establishing proper posture, an important factor for a good functional and cosmetic outcome after surgery.

Dietary restrictions are not indicated. Note that constipation is common after operative repair of pectus excavatum, probably because of the use of narcotics for postoperative pain management. Thus, a high-fiber diet and use of laxatives is recommended early after surgical repair. Normal bowel function should resume after discontinuation of all pain medications.

Good posture with a straight back (ie, the military posture) is very important following surgery. Patients are also instructed to avoid any bending at the hip and to not slouch for the first month. Regular activity is permitted as pain reduces and mobility increases. Heavy lifting is not permitted for one month following surgery (patients are not allowed to carry heavy book bags for at least 4 wk), and contact sports are to be avoided for at least 3 months. After 3 months, most patients return to normal activities, including sports (eg, baseball, basketball, running, swimming, tennis, jumping). Following bar removal (typically performed in the outpatient setting at 2-3 years after the repair of pectus excavatum), patients are not to be restricted from any activities, except for 5-7 days after this minor procedure.