Video 1 Nuss bar procedure: past, present and future.
Anesthesia using an ether mask was a landmark development in 1846; however, it did not provide a safe airway for thoracic surgery. For this reason, thoracic surgery remained in its infancy until endotracheal anesthesia was introduced in the 1920’s (1,2). It is therefore not surprising that initial attempts to correct pectus excavatum were designed to avoid opening the chest and to correct the deformity via an incision on the anterior chest wall. In 1911, Meyer removed two rib cartilages on the right side but concluded that the result was unsatisfactory (3). In 1913, Sauerbruch resected the depressed section of the anterior chest wall which relieved the patient’s cardiac compression, palpitations, and blackouts, allowing him to return to work, but left him with acquired Poland’s syndrome. Sauerbruch similarly considered this result to be unsatisfactory (4). Therefore, in the 1920’s, when confronted by a young girl with a severe pectus excavatum he developed the technique of only partial cartilage resection and sternal osteotomy which is now frequently referred to as a “modified Ravitch technique” (5). In order to prevent the sternum from sinking back into the chest, Sauerbruch used external traction by passing a steel wire through the sternum and attaching it to an orthopedic traction device for several weeks.
In 1949, Ravitch popularized an approach which avoided the need for external traction and that included radical resection of all the costal cartilages, complete disconnection of the sternum from the chest wall and a wedge osteotomy of the sternum (6). He advocated doing the procedure in infants and pre-school age children, which resulted in a very rigid and corrugated chest wall, and sometimes, damage to the growth centers, causing the development of acquired asphyxiating chondrodystrophy in later years. As pediatricians provided most of the longitudinal care of these patients, they were the first to recognize this problem and referrals for pectus excavatum repair significantly decreased during this period (7).
In 1956, Wallgren and Sulamaa developed a novel strategy of internal support to prevent sternal regression by placing a subcutaneous bar across the chest that went through the body of the sternum (8). In 1961, Adkins and Blades went one step further and placed the bar behind the sternum (9). These surgeons were essentially responsible for the standard rib resection, sternal osteotomy, and substernal support method of pectus excavatum repair during the subsequent 40 years.
Thoracic surgeons and trauma surgeons had noted the flexibility and malleability of the chest wall for a considerable period of time, as noted by Samuel Kelly in 1929: “dislocations of the sternum and of the ribs are very unusual in children, owing to the softness and elasticity of the chest walls” (10). This principle was reasserted by Haller in 1986 when he stated: “the flexible chest of a child makes disruption of the chest wall unlikely” and by Wesson in 1998: “Chest wall injuries in children require substantial impact to a pliable chest wall” (11,12).
By the mid 1980’s Nuss had become disillusioned with the results of the wide resection technique, not only in patients seen in his own practice, but also in patients operated on at other centers who came for a “second opinion” seeking help for a poor outcome. He therefore realized that another option needed to be found.
He was well aware of the “soft, elastic, flexible and pliable chest of young children” as well as the admonition in The Textbook of Advanced Pediatric Life Support that “only two fingers should be used for cardiac compression” to avoid crushing the heart (13). He therefore could see no reason for excising these soft and malleable structures. In addition to this knowledge, he knew that there was an avascular sub-sternal plane from the experience gained as a result of performing sub-sternal colon interpositions, median sternotomy, and placement of Adkins struts.
When, in 1987, a 4-year-old male patient presented with a severe pectus excavatum, he discussed with the parents the option of not removing the chest wall structures. They allowed him to proceed with his proposed surgical solution which included placing a bar under the sternum without cartilage resection or sternal osteotomy. The procedure was uneventful and gave an excellent correction.
Several key innovations were developed in the following two decades. The first one was to strengthen the bar as the original one was not strong enough to provide a durable correction. The company that made the original bar agreed to make it stronger, but after two years stated that they could no longer accept the risk involved and stopped making it. Fortunately, another company agreed to partner with Nuss on the project and not only was the bar completely redesigned, but new instruments were designed and developed specifically for the minimally invasive repair of pectus excavatum (MIRPE) (14,15). The incision was moved from the anterior chest wall to bilateral lateral chest wall incisions.
Other important additions included the use of thoracoscopy in 1998, which made the substernal dissection safer and helped optimize bar placement. Bar displacement proved to be a major problem and therefore a stabilizer was developed in 1998 to improve bar fixation (15). However the addition of pericostal sutures in 2002 reduced bar displacement to less than 1% when combined with the stabilizer (16). Park has also developed several new devices such as a “claw fixator” and “hinge plate” to help prevent bar displacement (17).
Improvements in the substernal dissection have been crucial to prevent cardiac injury during this part of the procedure. Initially, a more cephalad tunnel was created superior to the deepest point of the defect with one tunneler, before passing a second tunneler under the deepest point. Later, sternal elevation prior to substernal dissection was developed and found to be facilitated by using a variety of retractors, towel clamps, Klobe’s suction cup, or Park’s “Crane Technique” (16,17).
Initially there were no criteria to determine which patients had a deformity severe enough to warrant surgical correction. A treatment algorithm was developed which took into account the following: symptoms, severity and phenotype of the deformity, deformity progression, objective study results (computed tomography (CT) scan index, electrocardiogram (EKG), echocardiogram), and Pulmonary Function Tests (18) (Figure 1).
Figure 1 Evaluation and treatment algorithm.
This algorithm resulted in 33% to 50% of the patients being selected for surgical correction, while the other 50 to 66% were started on an exercise and posture program and re-evaluated at yearly intervals. More recently these patients have been offered treatment with the Vacuum Bell (19,20)
The advent of the MIRPE resulted in a dramatic increase in the number of patients presenting for repair (15,21). This large increase in the numbers of patients undergoing repair has allowed many centers to develop technical refinements, thereby making the procedure even safer and has allowed numerous pre-and post-operative studies to be performed, showing that pectus excavatum causes significant cardiac and pulmonary compression and dysfunction which is markedly improved after repair (22-24).
Patients are evaluated to see if they are candidates for surgical correction based on the abovementioned criteria (Figure 1).
Patients are deemed suitable candidates for MIRPE if they manifest two or more of the following criteria:
- The medical history reveals that the patient is symptomatic. The most common complaints are shortness of breath with exercise, lack of endurance, and chest pain. Frequently there is also a history of progression of the deformity, especially during puberty;
- Physical exam shows that there is a moderate to severe pectus excavatum deformity which may be symmetric or asymmetric;
- The chest wall imaging [CT or magnetic resonance imaging (MRI)] shows a severe pectus excavatum deformity defined as a Haller CT index higher than 3.2 or correction index greater than 10% (25), cardiac and/or pulmonary compression or displacement;
- Pulmonary Function Studies demonstrate a restrictive or obstructive pattern;
- Cardiology evaluation elucidates cardiac compression or displacement, rhythm disturbance, and/or mitral valve prolapse;
- Poor body image and psycho-social maladjustment.
If the patient has a mild deformity and does not fulfill the abovementioned criteria for surgical correction, then he/she will be started on a deep breathing with breath holding and posture exercise program, and may be offered non-operative treatment with the Vacuum Bell (19,20). These patients are typically re-evaluated every 3 to 6 months to ensure that they are complying with the conservative treatment and to check whether the deformity is improving or progressing.
The patients who fulfill the criteria for MIRPE are also started on the deep breathing and posture exercise program. They are checked for metal allergy and their chest is measured between bilateral mid-axillary lines to determine the length of the bar to be inserted. The bar should be about 1 inch (2.5 centimeters) shorter than the measurement from the bilateral mid-axillary lines.
After induction of general endotracheal anesthesia and insertion of an indwelling bladder catheter, the patient is positioned supine with both arms abducted approximately 70-degrees at the shoulder. The chest is carefully palpated and the deepest point of the depression is marked with a circle using a marking pen. Next the intercostal spaces that are in the same horizontal plane as the deepest point of the excavatum are marked with an “X” just inside or medial to the top ridge of the depression. That is just medial to where the chest starts to cave in on each side. Finally, the incision sites are marked laterally between the anterior axillary line and the mid axillary line in the same horizontal plane as the deepest point of the depression (Figure 2).
Figure 2 Shows how the chest is marked before starting surgery. The deepest point of the depression is marked with a circle, the bilateral incision sites are marked with straight lines and the thoracic entry and exit sites are marked with an "X", all of which are in the same horizontal plane.
The chest is measured from right mid axillary line to left mid axillary line and a pectus bar is selected based on this measurement, minus 2.5 cm (1 inch). Pilegaard has advocated the use of a shorter and eccentrically placed bar with the stabilizer in a more medial position, but this can only be used in patients who have completed their growth spurt (26). The bar is bent into a smooth convex shape such that it fits comfortably against the skin leaving a 2–4 cm flat section in the middle to support the sternum (Figure 3).
Figure 3 The length of the bar is determined by measuring from right to left mid-axillary line and subtracting 2.5 cm (1 inch) from that measurement.
An intravenous first-generation cephalosporin is given as prophylaxis and maintained for up to 48 hours postoperatively. The chest is prepared and draped in a sterile manner, taking care to prep the skin down to the posterior axillary line.
Thoracoscopy, using CO2 up to a pressure of 5 mmHg, is then carried out by inserting a 5 mm trocar at the mid-axillary line, approximately two interspaces below the proposed right lateral skin incision. It is important to direct the trocar in a superior direction to avoid perforating the diaphragm and penetrating the liver. It is inserted at the mid-axillary line level so that one will be able to advance the thoracoscopy across the mediastinum. A 5-mm scope with a flexible tip is preferable, and if that is not available then a 30-degree scope is helpful. While looking through the thoracoscopy, it is important to check whether the internal anatomy corresponds well with the external markings. In addition, the mediastinum should be evaluated to see whether it will be necessary to elevate the sternum prior to starting the trans-mediastinal dissection. In complex cases, bilateral thoracoscopy may be helpful (Figure 4).
Figure 4 The thoracoscope is inserted in the right mid-axillary line approximately 2 intercostal spaces inferior to the incision site and directed in a superior direction to avoid injuring the diaphragm.
Some centers now use sternal elevation in all cases as it makes the mediastinal dissection easier and safer by markedly decreasing the risk of pericardial or cardiac perforation. There are several options available for sternal elevation before starting the mediastinal dissection. One option is to create an additional tunnel higher up where the depression is not so deep, and leaving that introducer in place to keep the sternum elevated while dissecting under the deepest point of the depression. A second option, in younger patients with a flexible chest, is to elevate the sternum by using the Vacuum Bell. A third option is to elevate the sternum by using a retractor inserted either through an additional sub-xiphoid incision or laterally” (27,28). A fourth option is to elevate the sternum by using Park’s Crane Technique (17,29) (Figure 5).
Figure 5 (A) Shows the sub-sternal, trans-thoracic introducer in position. However, in order to minimize the risk of cardiac and pulmonary laceration during dissection, sternal elevation should be accomplished prior to creating the substernal tunnel by means of one of several external elevation techniques; (B) Shows sternal elevation using the vacuum bell to facilitate the mediastinal dissection in young patients with a malleable chest wall; (C) Shows sternal elevation with a hook inserted through a small sub-xiphoid incision; (D) Shows sternal elevation using the Crane technique where a wire is passed through the anterior table of the sternum and attached to a Thompson or Rultract retractor to elevate the sternum.
Bilateral skin incisions are made at the previously marked sites between the anterior and mid axillary lines. The incisions are carried down to the rib cage and the subcutaneous tunnels are created following the rib cage up to the previously marked “X” just medial to the top of the ridge. If the planned subcutaneous tunnel is superior to the origin of the pectoralis muscles, then the tunnel should proceed under this muscle.
A retractor is inserted into the tunnel and the introducer is slowly advanced up the tunnel with the tip facing posteriorly. At the previously marked “X”, the introducer is gently pushed through the intercostal muscles under direct vision via the thoracoscopy. The introducer is turned over so that the tip faces anteriorly and very gently advanced towards the mediastinum. Keeping the tip in constant view, the introducer is then used to gently dissect the pleura and mediastinal tissues off the undersurface of the sternum by using an anterior to posterior or “pawing” motion. When the tunnel extends for about 1 centimeter then the introducer may be moved in a side-to-side motion in order to enlarge the tunnel. Forward progress should always proceed by using the anterior to posterior motion since this decreases the risk of perforating the pericardium. The introducer should never be simply pushed forward and the tunnel should be wide enough to ensure good visibility. It cannot be stressed enough that the tip of the introducer should be kept in view at all times. When the left pleural cavity has been entered, the introducer is advanced up to the “X” on the left side of the chest and gently pushed through the intercostal space and then slowly advanced out of the subcutaneous tunnel. In order to prevent stripping the intercostal muscles during advancement, the assistant should insert a hook through the tip of the introducer when it first appears at the “X” on the left side. The assistant then pulls upward on the hook in an anterior direction while the surgeon pushes on his end in a transverse direction (Figure 6).
Figure 6 (A) Shows insertion of the introducer through the sub-cutaneous tunnel, under the pectoralis major muscle before entering the thoracic cavity at the previously marked "X"; (B) Shows the introducer passing immediately under the sternum with the tip always in view and using a "pawing" motion for dissection; (C) Shows the introducer having emerged from the thorax through the left intercostal spaced marked with an "X" and having passed under the left pectoralis muscle and out through the left subcutaneous tunnel.
Once the introducer is in place it is lifted on both sides in an anterior direction to elevate the sternum out of its depressed position, while simultaneously pressing down on the lower chest wall and on any uneven protrusions. This molding process is repeated several times in an attempt to remodel the sternum by causing it to bow anteriorly. The lifting and molding maneuver decreases the corrective force which consequently decreases post-operative pain, decreases the risk of bar displacement, and need for re-shaping the bar after rotation (Figure 7).
Figure 7 Shows sternal elevation using the introducer in order to correct the deformity, loosen up the anterior chest wall and stretch the ligaments. This greatly facilitates bar rotation, decreases pressure on the bar and minimizes bar displacement. This maneuver should be repeated several times.
Umbilical tape is attached to the hole in the tip of the introducer which is then slowly pulled back out of the chest under direct thoracoscopic visualization. The tape is cut off the introducer and attached to the previously selected and appropriately bent pectus bar. The bar is gently guided through the substernal tunnel with the convexity facing posteriorly by applying gentle traction on the umbilical tape. The bar insertion must also be performed under direct thoracoscopic visualization to avoid injuring the heart or pericardium (Figure 8).
Figure 8 After the chest wall elevation procedure, umbilical tape is attached to the introducer which is then slowly withdrawn from the chest cavity under thoracoscopic guidance. When the introducer is out of the chest, the tape is cut off and attached to the pectus bar which is then slowly pulled through the media stinal tunnel with the convexity facing posteriorly under thoracoscopic control.
Once the bar is in place with an equal amount of the bar protruding on each side, it is rotated 180 degrees using a bar flipping tool. The bar may be turned clockwise or counter-clockwise depending on whether there is more depression superiorly or inferiorly. After the bar is rotated into its correct position, it should be evaluated for proper fit against the chest wall and adjusted as needed with a bar bending tool to achieve a slightly loose fit. If the bar is too loose it will protrude and may be less stable, and if it is too tight it will cause persistent pain, rib erosion and calcification around the bar (Figure 9).
Figure 9 Shows how the pectus bar is rotated 180 degrees using the instrument specially designed for this purpose. The "bar flipper" should be applied close to the tip of the bar so that it can be easily removed once the bar has been rotated. A flipper may be applied to each end to facilitate rotation.
Fixation of the bar to the thorax is essential to prevent bar displacement. Typically a stabilizer is attached to the left side of the bar and secured to the bar with non-absorbable suture by engaging the grooves on the end of the bar to prevent slippage. Several absorbable sutures are also placed between the fascia of the lateral chest wall and the holes in the bar and stabilizer. If two bars are placed, the stabilizers are placed opposite each other. Stabilizers are important for long-term bar stability.
Through the right incision, several pericostal absorbable monofilament (polydioxanone) sutures are placed around the bar and the underlying rib where they intersect. Fixation to at least two separate ribs is crucial to improve stability of the bar and resist rotational forces postoperatively. All pericostal sutures should be placed under direct thoracoscopic visualization. The pericostal sutures are especially important for short-term bar stability (Figure 10).
Figure 10 Bar fixation is essential to prevent displacement and is accomplished by applying a stabilizer on the left and multiple pericostal sutures on the right. The stabilizer is attached to the bar with wire or fiberwire suture. Sutures are also placed between the holes in the stabilizer and the pre-muscular fascia. Multiple pericostal sutures consisting of zero PDS are placed around the bar and ribs on the right side at the point where the bar crosses each rib.
Before closing the skin incisions, a final inspection of the mediastinum, heart, lungs, bar, and pericostal sutures is prudent to ensure hemostasis. The CO2 insufflation is discontinued and the anesthesiologist re-inflates the lungs. A three layered closure with absorbable suture is recommended to provide maximum coverage over the bar and stabilizer. While the incisions are being closed, the insufflation tubing attached to the thoracic trocar is divided and the proximal end is placed under water. Additionally, the patient is placed in the Durant position and placed on positive end-expiratory pressure (5 mmHg) to maximize evacuation of air from the thoracic cavity. Prior to removing the trocar, the surgeon must ensure that there is no air remaining in the chest cavity (Figure 11).
Figure 11 After the incisions are closed the pneumothorax is evacuated by cutting the insufflation tubing and placing the proximal end under water. The operating table is placed in Durant position and the anesthesiologist applies positive pressure ventilation until no more air bubbles escape from the tubing. The trocar is then pulled while the anesthesiologist applies positive pressure.
The anesthesiologist should be keenly aware of signs of pain during emergence from anesthesia. Every effort to achieve a smooth emergence from anesthesia is necessary to block the pain cascade and prevent the patient from excessive motion postoperatively. In the absence of bleeding concerns, a dose of ketorolac is given intravenously before the end of the procedure. Local anesthesia may be administered via liberal infiltration around the incisions, intercostal nerve blocks, or On-Q catheters. A patient controlled analgesia (PCA) pump using morphine or fentanyl is started in the operating room and transitioned to oral pain medication over the next 2–3 days. Other strategies to control postoperative pain include oral non-steroidal anti-inflammatory drugs (NSAIDs), muscle relaxants, and sedatives. Laxatives and stool softeners are given to prevent constipation and IV fluids and histamine-receptor 2 blockers are given to decrease the side-effects of the NSAID on the gastro-intestinal tract and kidneys. Recently one dose of methadone has been found to be effective when given at the end of surgery by the group from Phoenix (30).
Vigorous pulmonary toilet using an incentive spirometer (ICS) is started hours after surgery and continued for several weeks to prevent atelectasis and pneumonia. Respiratory therapists are helpful to provide directions for proper use of the ICS. Using the ICS also helps to expand the chest and loosen the ligaments which decreases the pressure and thus the pain. Ambulation is started on the first postoperative day with the help of the physical therapists.
The patients are instructed to lie on their back and avoid any pressure on the sides of their chest for the first 6 weeks.
A portable chest X-ray is obtained on the first postoperative day to check for pneumothorax, pleural effusion, and bar position. On the third postoperative day a posterior-anterior and lateral chest X-ray is obtained in the X-ray department to again check for bar position, pneumothorax and pleural effusion.
When the patients are able to take all of their medications orally and ambulate unassisted they are discharged home, usually on postoperative day 4 or 5.
At home, the patients are encouraged to walk as much as possible and do deep breathing exercises using the ICS multiple times a day. The oral narcotics, muscle relaxants, and sedatives are typically weaned off in the first one to two weeks. Oral anti-inflammatories may be required for another 1 to 2 weeks or more. Patients may return to school after 3 weeks but are not permitted to play sports or do heavy lifting for 6 weeks. At that time, if all is well, they may slowly resume normal activities and by 3 months they may participate in competitive sports.
Early complications occurring before discharge from hospital in 1,463 primary repairs performed between 1987 and 2012 are listed in (Table 1).
Table 1 Early postoperative complications
These complications have decreased in frequency since the early learning period. Pneumothorax is usually due to incomplete evacuation of the CO2, since a leak in the lung parenchyma should only occur if adhesions to the lung need to be lysed. Drug reactions can be minimized by careful history taking. Suture site infections can be minimized by careful antiseptic technique and prophylactic antibiotics. Pneumonia can be prevented by careful pre-operative examination to ensure that the patient is healthy and postoperatively by encouraging hourly use of ICS. Pericarditis should be a rare event due to routine pre-op metallic allergy testing and the use of titanium bars when nickel or other metallic allergy is suspected. Hemothorax is minimized by avoiding injury to the intercostal and internal mammary arteries and checking that there is no bleeding before pulling the trocar at the end of the procedure. Temporary paralysis was due to the epidural in two cases and this method of pain control was subsequently abandoned.
Late post-operative complications are listed in (Table 2).
Table 2 Late post-operative complications
Bar displacements presented an initial challenge and occurred in 15% of the patients in the first few years when the only method for bar stabilization was to create a soft tissue bed for the bar with mattress sutures. When stabilizers were developed, the rate of displacement dropped to 5%, and with the addition of pericostal sutures, the displacement rate fell to 1%.
Over-correction tends to occur in patients with a very deep and asymmetric deformity and a narrow chest. It is very important that these patients do deep breathing with breath holding exercises twice every day in order to open up the chest and make room for the cartilages. A carinatum brace may be applied if the over-correction does not improve after one year.
Bar allergy complications have been minimized by the use of routine pre-operative metal allergy testing and using titanium bars when an allergy to any of the components of stainless steel is proven or suspected. If the patient develops allergic complications after bar insertion, prednisone given until the ESR and CRP return to normal is usually successful, but if symptoms recur then removal of the stainless steel bar may be required.
Recurrence is inversely related to the length of time the bar remains in place (16). The shorter the time the bar is in place, the higher the recurrence rate. Since the duration of bar placement was increased to 3 years before removal there have been no recurrences. Patients are advised to do deep breathing exercises every morning and evening, in order to open up the entire chest and loosen up the chest wall ligaments. Patients are also encouraged to participate in exercises such as running and swimming starting 6 weeks after repair.
A review of our prospective database shows a good to excellent outcome in 98% of patients, where an excellent result implies a normal chest and a good result shows minimal residual depression. Most of the failures occurred during the learning curve in the first few years when the bar was too soft and it was removed too soon (Table 3).
The results of cardiac and pulmonary function studies show that there is an immediate beneficial effect from the release of right heart compression, giving rise to an increase in stroke volume and cardiac output, and a more gradual improvement of pulmonary function which is especially notable after bar removal (22,31,32).
There has been a huge shift in the age at which patients are scheduled for pectus excavatum repair. From 1949 until 2000, the surgical literature recommended that children have the open pectus procedure before going to school (6,33-35). However, after an article by Haller and colleagues drew attention to the effects of too early and extensive open procedures (36), the median age shifted to 14 years. This shift was further accentuated by the introduction of MIRPE procedure since it could be done at any age (37). In addition, there have been reports in the literature of patients in their late 40’s and 50’s developing cardiopulmonary failure with no pathology other than pectus excavatum to account for their incapacitation (38). As a result, there are now numerous reports of successful repair via the Nuss procedure in older adult patients (24,29,38,39).
Bar removal should be scheduled between 2 to 4 years and done under general endotracheal anesthesia with PEEP. The EKG monitor should be audible. The bar should be mobilized at both ends, the stabilizer is removed, and the bar slowly straightened out with the bar flippers. When it is straight enough to slide out, it should first be slightly rotated to see the effect on the EKG. If the EKG remains stable then it should be slowly withdrawn from the chest. Local anesthetic is injected and the incisions closed in layers and pressure dressings applied. A chest X-ray is taken in the recovery room to check for pneumothorax.
Since the introduction of the Minimally Invasive Pectus Repair, there has been a dramatic increase in the number of patients presenting for repair. There has also been a dramatic increase in the number of papers published in the medical literature—300 papers in the decade before the Nuss bar procedure and over 600 in the decade after. The increase in the number of pectus excavatum patients presenting for repair has allowed for a large increase in the number of studies of cardio-pulmonary function, showing that these patients do suffer from right heart compression and pulmonary restriction, which is significantly improved after repair (22,24,29,31,32,37-39). It should be noted that a 10% reduction in pulmonary function studies is significant as it represents a full standard deviation below normal. Although these patients may be asymptomatic at rest, they are not able to compete with their peers. In addition, there are now more reports of patients in their 4th and 5th decade becoming incapacitated due to their cardiopulmonary compression (39).
In the decade since the MIRPE procedure was first published, many modifications have been made to make the procedure safer and more efficient including: addition of thoracoscopy, stabilizers, pericostal sutures, sternal elevation before tunneling, instruments designed specifically for the procedure, prophylactic antibiotics, and optimization of pain control (37). It is anticipated that this trend will continue and that the procedure and instruments will constantly be improved (17,18).
Even the open procedure has benefitted as it has been modified from the original radical resection Ravitch procedure to a much less aggressive minimal cartilage resection open procedure (40,41).
Centers of excellence will develop in which there will be a team of well-trained surgeons, anesthesiologists, nurses, pain specialists, physical therapists and respiratory therapists who will provide expert care with even fewer complications and outstanding results.
The instruments will be constantly improved both as a result of new ideas but also because of the discovery of new products which are more suitable for the procedure such as new alloys and bio-absorbable materials.
New approaches to the problem will also appear, as exemplified by Klobe’s Vacuum Bell, which is now finding a place as the treatment of choice in younger patients with a soft and malleable chest who have a mild to moderate deformity (19,20). At one time there was only one procedure available for patients with pectus excavatum and that was the Ravitch procedure. Now, there is a spectrum of techniques extending from the non-operative Vacuum Bell treatment for mild and moderate cases, to the MIRPE procedure for more severe cases, to the modified open procedure for the very severe and recurrent cases, as well as combined open and closed procedures (42,43).
Other new ideas are presently at the experimental stage, including Bardaji’s Pectus Up procedure, in which a screw is used to pull the sternum up, and Harrison’s Magnetic Mini-Mover Procedure, in which magnets are used to pull the sternum up over a 2 year time frame (44).
In the future, the treatment of pectus excavatum will be more proactive as primary care doctors and parents begin to fully appreciate the range of treatment methods available for treating chest wall malformations. The patients will be referred at an earlier age, which will allow the conservative treatment options to be more successful. This may cause a slight decline in the number of patients requiring surgical repair, but in those patients requiring surgery, the timing will be optimized.
Thanks to Trisha Arnel, Executive Secretary.
Conflicts of Interest: Dr. Nuss has consultation and royalty with Zimmer Biomet. Dr. Kelly and Dr. Obermeyer have consultation agreements with Zimmer Biomet.
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Rehabilitation may be helpful in the prevention of complications associated with the treatment of patients with pectus excavatum who are subjected to surgery using the Ravitch and Nuss methods. This paper presents the case of a patient who underwent successful physical rehabilitation after 8 weeks from the surgery using the combined method. As part of the Nuss procedure, two plates were implanted to form a scaffolding for the patient's chest, which had previously been corrected with the Ravitch method. The plates were to be removed after 24 months of treatment. After the procedure, in spite of the favorable cosmetic effect of the repair, there was a significant decrease in the spirometric values and physical fitness of the patient. He underwent an individual physiotherapy program, which lasted four weeks. The streamlining of the respiratory system has significantly improved the spirometric values and raised the overall performance of the patient's organism.
Keywords: rehabilitation, Ravitch method, Nuss method, spirometry
Rehabilitacja może być pomocna w zapobieganiu powikłaniom związanym z wadą i leczeniem pacjentów z klatką piersiową szewską (pectus excavatum), u których wykonano zabieg operacyjny metodą Ravitcha i Nussa. W pracy przedstawiono przypadek pacjenta, u którego z powodzeniem zastosowano rehabilitację ruchową po 8 tygodniach po zabiegu torakochirurgicznym metodą złożoną. Polegała ona na wprowadzeniu w uprzednio skorygowaną klatkę piersiową metodą Ravitcha dwóch płytek tworzących rodzaj rusztowania według metody Nussa. Implanty były niezbędne do korekcji nawrotu deformacji klatki piersiowej. Po zabiegu – mimo korzystnego efektu wizualnego – stwierdzono znaczne obniżenie wartości spirometrycznych i wydolności fizycznej pacjenta. Operowany pacjent został poddany indywidualnemu programowi fizjoterapii, który trwał 4 tygodnie.
In normal anatomy of the chest, the sternum and the whole anterior chest wall form the most anterior part of the body. Pectus excavatum is a condition in which the lower part of the sternum and the adjoining rib segments are sunken into the chest [1–3]. The displacement of the sternum and ribs in the direction of the vertebral column reduces the anteroposterior dimension of the chest; as a result, the vital capacity of the lungs is reduced, which contributes to the development of disorders of the circulatory and respiratory systems. These changes are a frequent cause of recurrent bronchitis and pneumonia, dyspnea, and fatigability. The etiology of this deformation is not fully understood. The condition may be acquired as a result of inflammatory processes and rachitis; its congenital form (5%) may be associated with impairments in the development of the diaphragm or disproportionate growth of the costal cartilages. There is no evidence indicating that rehabilitation can slow the development of the deformation or reverse it; the only effective method of treatment is surgery.
The aim of the study is to present the case in which pectus excavatum recurred and was treated with two methods of surgical repair; good results of respiratory rehabilitation were achieved, which significantly contributed to the improvement of the patient's general and respiratory fitness.
In 2000, at the age of 7, the patient underwent repair of the chest deformity using the Ravitch method at the Lower Silesian Center for Lung Diseases in Wrocław. The cosmetic and functional effect of the procedure was good. However, during the period of the boy's quickened growth between the ages of 15 and 17, the deformation began to recur, and the chest's anatomy deteriorated systematically. The patient experienced dyspnea and chest pain during intense cough. After he turned 20, examinations showed that the lower part of his sternum, sunken inwards, was displaced dangerously close to the spine (Fig. 1). On 15 August 2014, the patient underwent a redo procedure in order to repair the recurrent chest deformity, which had first been repaired using the Ravitch method (Fig. 2).
Section through the patient's thorax: pectus excavatum
The patient prepared for the surgery
The surgery was performed using a modified Nuss procedure. The whole scar left after the previous procedure was excised. As the operators dissected the hard fibrous tissue, the anterior surface of the mesosternum was uncovered. Next, the medial segments of the 3rd, 4th, and 5th intercostal spaces were uncovered bilaterally. Two canals were formed under the sternum using both dull, and sharp dissection. The dissection was performed under visual control using a thoracoscopic camera introduced into the intercostal spaces at the axillary line. Subsequently, the sternum was raised on two thick sutures (Fig. 3). Skin on both sides of the chest was incised between the anterior and middle axillary lines, at the level of the 4th intercostal spaces. Bilaterally, two tunnels were formed under the muscles of the chest wall, at the level of the 3rd and 5th intercostal spaces (Fig. 4).
The sternum was raised on two thick sutures
Two implants were embedded under the sternum
During the dissection, the right internal thoracic artery was injured. The bleeding was stopped with sutures, and the bleeding site was monitored with a thoracoscope. Two implants were modelled (32 and 36 cm in length) and placed under the sternum. Concurrently, both implants were twisted under the mesosternum. At the level of the 3rd intercostal space, a lateral fissure was observed in the mesosternum. Therefore, the forces had to be distributed onto two implants. After the implants were screwed into position, the cosmetic effect of the chest repair was very good. The implants were bilaterally sutured to the chest wall. Air was suctioned out of the pleural cavity. A Redon drain was placed on the floor of the sternal wound; the wound was closed with a layered suture and covered with sterile dressing. The postoperative course was uneventful. Control X-ray examinations demonstrated normal lung expansion (Fig. 5). The patient was discharged from the Lower Silesian Center for Lung Diseases in Wrocław in good general condition; he was advised to report to his local surgery clinic for a postoperative control examination and suture removal. The patient was to report back to the Center after 2-3 years in order to plan the second stage of the procedure – the removal of the implants.
Thoracic X-ray of the 21-year-old patient after Ravitch and Nuss procedures
After the thoracic procedure, the patient received postoperative advice. During the first 3 postoperative months, he was to avoid bending or twisting the torso. He was also prohibited from carrying weights and discouraged from participating in contact sports. The patient adhered to the postoperative recommendations; over the next 8 weeks, he limited physical activity to the minimum and tried to make all upper extremity movements in symmetry. During the same year, on 16 October, the 21-year-old patient was admitted to the physiotherapy unit of the Subcarpathian Center for Pulmonary Diseases in Rzeszów. Ultrasonographic examination of the patient's chest revealed no postoperative complications; however, when spirometry was performed, the results were unsatisfactory, which was in line with the information obtained from the patient during an interview. He reported fatigability even during little exertion and significantly reduced strength.
According to the results of the spirometric examination performed before the start of the rehabilitation process, his forced vital capacity (FVC) was limited to 48%, forced expiratory volume in 1 second (FEV1) was reduced to 55%, and the FEV1/FVC ratio amounted to 116%. The patient's peak expiratory flow (PEF) was 65%. Additionally, a Voldyne 5000 device was used to measure his inspiratory volume. According to the patient characteristics (age, height, sex), the result should fall within the range of 3600 ml/min on the nomogram. However, the measured inspiratory volume was 2300 ml/min.
Further physiotherapeutic examination demonstrated that the patient's head jutted forward, his shoulders slouched and jutted forward, and the shoulder blades were protruding. Moreover, the anteroposterior dimension of the flattened chest was reduced, and the shoulder girdle was contractured. Additionally, the stretching of the dorsal muscles (the trapezius, rhomboid, latissimus dorsi) contributed to the development of thoracic hyperkyphosis, a defect also known as round back. The chest muscles were excessively tense, while the abdominal muscles demonstrated excessive flaccidity, which led to the impairment of breathing (Fig. 6).
Patient's position assumed in order to protect the surgical site
The patient was offered a personalized rehabilitation program aimed to improve the fitness of his circulatory and respiratory systems and to correct his posture.
Three fundamental aspects were addressed by the therapeutic proceedings:
morphological: to eliminate muscle dystonia and strengthen the muscular corset,
physiological: to teach the patient to adopt the correct posture and form the habit of maintaining it,
environmental: to ensure that the patient's life conditions are conducive to eliminating the defect.
The occurrence of muscle contractures limited the mobility of the patient's joints and precluded him from assuming the proper posture. The first step to eliminate the dystonia was to stretch the muscles and restore full joint mobility. When stretching the muscles, the following rules were observed:
during the initial stage of the stretching, the exercise consisted mostly in passive stretching,
the direction of stretching movements addressed the functional structure of the muscles,
when a muscle was stretched, moving one of its attachments further away, the other attachment site was stabilized.
Reducing the contractures of the thoracic muscles and the serratus anterior enabled the patient to assume the proper posture. Improving the posture began with partial/local corrections (adjusting the position of the head, moving back the shoulder girdle, reducing thoracic kyphosis, expanding the chest), which were later combined to achieve holistic/global correction. Initially, the exercises were limited to positions that freed the vertebral column from axial compression (with the patient lying down or kneeling on his hands and knees); gradually, sitting and standing exercises were introduced as well. This allowed the patient to control his posture first by adhering to a stable surface (floor, walls), then by visual control using a mirror, and finally using proprioception.
To strengthen the weakened muscles, exercises in the trained corrected position were employed. The corrected position was maintained throughout the duration of the exercise, and the use of resistance (Thera-Band resistance bands) did not result in the loss of correction (Fig. 7). The program included formative, strengthening, and stretching exercises. The next stage of postoperative rehabilitation consisted in respiratory kinesitherapy (Fig. 8). The aim of these exercises was for the patient to learn how to breathe by increasing the vital capacity of the lungs (Fig. 9), to strengthen his respiratory muscles, and to increase the mobility of the chest during thoracic and diaphragmatic breathing, especially during inspiration (raising the ribs up and forward). Systematic exercise and ergometer training allowed the patient to develop the habit of assuming the proper posture during exercises. In terms of morphology, the mobility of the shoulder joints was increased, as was the strength of the dorsal, abdominal, and other postural muscles.
Kinesitherapy with Thera-band resistance bands
Breathing exercises aimed at increasing thoracic mobility during thoracic and diaphragmatic breathing
Breathing and strengthening exercises in a closed system
After the conclusion of the rehabilitation program, conducted at the Subcarpathian Center for Pulmonary Diseases in Rzeszów and lasting 4 weeks, spirometry was repeated. FVC amounted to 75%, indicating an increase of 27%. FEV1 was 77% (an increase of 22%), while the FEV1/FVC ratio amounted to 103% (a decrease of 13%). The patient's PEF after the rehabilitation increased by 14% (Fig. 10). Additionally, a Voldyne 5000 device was used to measure his inspiratory volume. The result, 3150 ml/min, indicated an improvement of 850 ml/min.
Selected spirometric values obtained before and after the rehabilitation process
Surgical treatment for pectus excavatum is a complex process requiring the cooperation of a medical team. The introduction of a new complex surgical method increased interest in the treatment, primarily due to cosmetic considerations and the visual effect after the procedure . According to scientific reports, 81.4% of patients report for the procedure due to cosmetic reasons and low self-acceptance . Despite the pain associated with the presence of the implants, persisting for up to several months, the patients view the impact of the procedure on their quality of life as favorable, as demonstrated in a study by Krasopoulos et al. . Concurrently, the literature includes reports in which treatment with the combined methods was followed by reduced spirometric parameters and negative electrocardiography changes. A study by Dzielicki et al. demonstrated the occurrence of electrocardiographic changes in 60% of cases and spirometric changes in 38% of patients . In his report, Adamczyk observed reduced VC and FEV1 . Pectus excavatum is characterized by a reduction in the sagittal dimension of the chest, often accompanied by a displacement of the heart to the left, resulting in the occurrence of exercise tachycardia . A study on the postoperative functioning of the lungs and the circulatory system after the surgical repair of pectus excavatum by Malek et al. demonstrated a statistically significant influence of the procedure on selected circulatory parameters and no such influence on spirometric values . The literature lacks results concerning the effects of postoperative rehabilitation in patients undergoing combined Nuss and Ravitch procedures. The rehabilitation process as such is often mentioned as conservative treatment before the surgery. There is no unequivocal evidence that rehabilitation has an impact on the reduction of the chest defect.
After the conclusion of the rehabilitation program, performed by a 21-year-old patient who had undergone surgery using the Ravitch and Nuss methods, the patient's spirometric parameters and general fitness improved significantly. Surgical treatment for pectus excavatum is a safe procedure that is successfully employed in young adults . This patient group is characterized by stronger will to engage in everyday physical activity. However, after the surgery, these patients are faced with safety procedures limiting their ability to engage in physical work or participate in sports. Proceeding in accordance with the postoperative guidelines leads to a reduction in their fitness . In turn, failing to adhere to the postoperative guidelines increases the risk of complications, of which the most frequent include: implant displacement, pneumothorax, and wound inflammation. The presented method of rehabilitation should find a wider application in comprehensive treatment provided for pectus excavatum. Apart from surgical repair, it should include pre- and postoperative rehabilitation and analgesic pharmacotherapy. The rehabilitation process should address the individual condition and fitness of the patient, obliging the physiotherapist to conduct a personalized program based on spirometric results .
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