Adult Chest Surgery

Chapter 119. Primary Repair of Chest Wall Defects 

Congenital chest wall abnormalities may be divided into five categories: pectus excavatum, pectus carinatum, Poland's syndrome, sternal defects, and miscellaneous dysplasias or thoracic deformities found with diffuse skeletal disorders. Surgical approaches to the first four categories will be described in this chapter.

PECTUS EXCAVATUM

Pectus excavatum, also known as funnel chest, trichterbrust, or thorax en entonnoir, is the most common anterior chest wall anomaly and results from posterior depression of the sternum and the inferior costal cartilages. The male:female ratio is 3:1, and 90% of cases are appreciated by age 1.The etiology of this deformity is unknown. Theories include intrauterine pressure, rickets, and abnormalities of the diaphragm.2–4 Pectus excavatum has been seen in association with agenesis of the diaphragm and congenital diaphragmatic hernia, especially after surgical repair.There is an association between pectus excavatum and other musculoskeletal anomalies, particularly scoliosis and Marfan's syndrome. A family history has been reported in up to 37% of patients, which may indicate a genetic predisposition.1

Surgical repair of pectus excavatum was first achieved by Meyer in 1911and Sauerbruch in 1913.The method of repair has evolved over the years with increasing experience and as the primary components of the deformity became clear. Early improvements were described by Ochsner and DeBakey in 1939.In 1949, Ravitch described a new technique that involved excision of all the deformed costal cartilages including the perichondrium, division of the xiphoid from the sternum, division of the intercostal bundles from the sternum, and transverse sternal osteotomy, by which the sternum was angled anteriorly and secured initially with Kirschner wires and later in the series with silk sutures.In 1958, Welch reported his technique based on Ravitch's work,10 and this was further modified and improved, culminating in the series reported by Shamberger and Welch in 1988,11 which is the basis for the current open repair.12

Evaluation and Therapeutic Plan

Treatment of pectus excavatum has two parts: the surgical repair and an exercise and posture program. When pectus excavatum patients are evaluated clinically, they are classified as having mild, moderate, or severe deformities. Patients with mild to moderate deformities are placed on an exercise and posture program and then reevaluated at 12-month intervals. Roughly two-thirds of patients may be treated in this fashion nonoperatively. Patients with severe deformities undergo an evaluation to determine whether they are candidates for surgery. The workup includes pulmonary function studies; a cardiology evaluation, including electrocardiogram and echocardiogram; and a CT scan of the chest. The recommended technique for this CT scan is that it be performed during quiet respirations, not during maximal inspiration.

Surgical correction is recommended if the child has two or more of the following criteria: (1) progressive or symptomatic pectus excavatum, (2) restrictive lung disease as measured by pulmonary function studies, (3) a CT scan showing cardiac compression or displacement, pulmonary atelectasis, and a Haller CT index greater than 3.25, (4) cardiac abnormalities including mitral valve prolapse or bundle-branch block, and (5) recurrent pectus excavatum after a failed repair.

Surgical Technique: Open Repair

The Shamberger and Welch technique, based on that of Ravitch, is shown in Figs. 119-1, 119-2, 119-3, 119-4, 119-5, 119-6, 119-7, and 119-8.1,12 A transverse incision is made below and within the nipple lines, preferably at the site of the future inframammary crease (in females) (Fig. 119-1). With the aid of electrocautery, the skin flaps are elevated and mobilized up to the angle of Louis superiorly and down to the xiphoid inferiorly. The pectoralis major muscle is elevated off the sternum, along with some of the pectoralis minor and serratus anterior muscles. This plane can be appreciated by finding the free area just anterior to the costal cartilages at their junction with the sternum. An empty knife handle is helpful for beginning the development of this plane (Fig. 119-2). The lateral extent of the muscle dissection is defined by the costochondral junctions of the third through fifth ribs. To prevent bleeding, careful attention should be paid to avoiding injury of the intercostal bundles. The subperichondrial resection is started by incising the perichondrium anteriorly.

Figure 119-1.

 

Incision and landmarks for open repair of pectus excavatum.

 

Figure 119-2.

 

Skin flaps are elevated, and the muscle attachments to sternum are divided.

 

Figure 119-3.

 

The perichondrium is divided.

 

Figure 119-4.

 

The costal cartilage is removed.

 

Figure 119-5.

 

After the two wedge osteotomies are created, the excess bone is removed.

 

Figure 119-6.

 

Method for fixing the sternum in a forward position without the use of struts.

 

Figure 119-7.

 

Technique for inserting retrosternal or presternal struts.

 

Figure 119-8.

 

A parasternal catheter is placed before the muscles are reattached to the sternum.

The dissection continues in the bloodless plane between the perichondrium and the costal cartilage. Dividing the perichondrium at 90 degrees in each direction at its junction with the sternum permits visualization of the back wall of the costal cartilage (Fig. 119-3). The cartilages are separated from the junction of the sternum with a knife. One can protect the mediastinum by holding a Welch perichondrial elevator posteriorly to the cartilage during this maneuver. The divided cartilage then is held with an Allis clamp and elevated, and the dissection is continued laterally near to the bone and cartilage junction, where it is excised (Fig. 119-4). In this fashion, the bilateral third, fourth, fifth, sixth, and seventh cartilages are removed.

A sternal osteotomy is performed superior to the last deformed cartilage at the posterior angulation of the sternum (Fig. 119-5). This angle is usually located just above the insertion of the third cartilage, but occasionally it lies just above the second. Two transverse sternal osteotomies are created through the anterior cortex with a Hall air drill (Zimmer USA, Inc., Warsaw, IN) 2–4 mm apart. The small segment of anterior cortex between the two osteotomies is removed along with the underlying cancellous bone. The technique diverges at this point depending on whether or not a prosthetic strut is used to reinforce the forward position of the sternum.

The base of the sternum and the rectus muscle flap are elevated with two towel clips, and the posterior aspect of the sternum is fractured (Fig. 119-6A ). The xiphoid is divided from the sternum with electrocautery, which permits entry into the retrosternal space. This step can be avoided with the use of a retrosternal strut (described below). If a strut is not used, the osteotomy is closed with heavy silk sutures while keeping the sternum elevated (Fig. 119-6B , C ).

The techniques for strut placement are depicted in Fig. 119-7. Either retrosternal or presternal (Rehbein) struts may be used. The retrosternal strut is placed behind the sternum and secured to the rib ends laterally to prevent migration (Fig. 119-7B ). Alternatively, Rehbein struts are inserted into the marrow cavity of the third or fourth rib ends and then joined to each other medially to create a metal arch anterior to the sternum (Fig. 119-7C ). The sternum then is sewn to the arch to secure it in a forward position.

After the position of the sternum has been secured by one of the previously described methods, a closed suction drain is brought through the inferior skin flap to the left of the sternum and placed in a right parasternal location at the level of the highest costal cartilage resection (Fig. 119-8). The pectoral muscle flaps are sewn to the midline of the sternum such that the entire sternum is covered. The rectus muscle flap, if it was divided previously, is joined to the pectoral muscle flaps. The remainder of the wound is closed in layers in standard fashion with a subcuticular skin closure.

Surgical Technique: Thoracoscopic Minimally Invasive (Nuss) Repair

The surgical technique of minimally invasive pectus surgery was developed by Nuss.13,14 The procedure begins with an estimation of the length of the pectus bar that will be required for the procedure. This is accomplished by measuring the distance from the right to the left midaxillary line and then subtracting 2 cm from this measurement (Fig. 119-9). The patient is brought to the OR and placed under general endotracheal anesthesia. Placement of a thoracic epidural catheter is also very helpful and may be continued for 3–5 days postoperatively. A Foley catheter is placed, as is a nasogastric tube, and the patient receives IV antibiotic coverage. The patient is positioned with both arms abducted at the shoulder with protective gel pads.

Figure 119-9.

 

Estimating the length of the pectus bar.

 

After the patient is prepped and draped, the Lorenz pectus support bar (Walter Lorenz Surgical, Jacksonville, FL) is shaped into a semicircle, save for the central 2-cm section, which remains flat. The deepest point of the pectus excavatum is marked on the patient with a marking pen. The intercostal spaces that are in the same horizontal plane as the deepest point of the pectus then are identified. These planned entry and exit points are located on either side of the sternum and should be medial to the top of the costochondral ridge. The proposed incision sites on each lateral chest wall then are identified. A thoracoscope is inserted through the right lower lateral chest wall approximately two interspaces inferior to the proposed skin incision (Fig. 119-10). With aid of the thoracoscope, a thorough inspection is made of the right hemithorax and mediastinum. Bilateral thoracic skin incisions are made, and a deep subcutaneous tunnel is raised anteriorly toward the intercostal space previously marked, medial to the top of the pectus ridge. A pocket is also created for the distal end of the pectus bar and stabilizer.

Figure 119-10.

 

Marking the chest for thoracoscopic correction of pectus defect and placement of pectus bar.

 

Under direct thoracoscopic visualization, a Lorenz introducer of appropriate size for the patient is inserted through the right intercostal space at the top of the pectus ridge at the location previously marked. During this maneuver, the electrocardiogram audio signal is monitored carefully for ectopy. The pericardium is gently dissected off the undersurface of the sternum. The introducer is slowly advanced across the mediastinum under thoracoscopic guidance, with the point facing anteriorly and in direct contact with the sternum. When the substernal tunnel has been completed, the introducer is advanced through the contralateral intercostal space at the previously marked exit site and advanced out from the skin incision (Fig. 119-11).

Figure 119-11.

 

Insertion of Lorenz introducer.

 

The introducer then is used to elevate the sternum. The surgeon lifts the introducer on the right side while the assistant lifts on the left side (Fig. 119-12). This lifting maneuver is repeated until the sternum has been elevated out of its depressed position, and the pectus excavatum defect has been corrected. An umbilical tape is attached to the end of the introducer, which is then pulled out, leaving umbilical tape passing through the substernal tunnel. The previously prepared pectus bar then is tied to the umbilical tape and guided through the substernal tunnel by the surgeon using the tape for traction and assisted by thoracoscopic vision (Fig. 119-13). The bar is inserted with its convexity facing posteriorly. Once the bar is in position, it is rotated 180 degrees with the bar flipper. If the bar requires further bending or adjustment, it is turned over and molded where required using the small Lorenz bar bender. If one bar is inadequate, a second bar may be inserted one interspace below or above the first one. Two bars provide a more stable correction, especially in older children. A slight overcorrection is preferable to prevent recurrence after the bar is removed.

Figure 119-12.

 

Lorenz introducer is used to elevate the sternum to the appropriate position.

 

Figure 119-13.

 

The introducer is replaced by the metal bar, and then the bar is rotated by 180 degrees.

 

The bar then must be stabilized. This is done by placing an inserter onto the left end of the bar and wiring it to the bar with no. 3 surgical steel wires (Fig. 119-14). Heavy absorbable 0 or 1 PDS sutures are placed around the bar and the underlying rib using an endoclose laparoscopic needle under thoracoscopic vision on the right side. Once the bar has been stabilized, the incisions are closed in layers, and the pneumothorax is evacuated using the trocar attached to suction or with a chest tube. A chest radiograph is obtained to check for residual pneumothorax.

Figure 119-14.

 

Stabilizing the pectus bars.

PECTUS CARINATUM

Pectus carinatum deformity is encountered less frequently than pectus excavatum and is more common in boys than in girls.15 In nearly 50% of cases, the deformity is not noticed until after the child is 11 years of age. A mild deformity in early childhood frequently becomes worse and as the child grows, especially at puberty. The cause of pectus carinatum has not been established. There is a family history in approximately one-fourth of patients and an association with scoliosis in 15% of patients. The deformity takes several different forms (Fig. 119-15). The most common form of pectus carinatum is symmetric protrusion of the body of the sternum and costal cartilages. The protrusion also may be asymmetric, affecting only one side of the sternum. A mixed deformity may occur with aspects of both protrusion and depression. This entity is seen most frequently in association with Poland's syndrome. The least common form of pectus carinatum, chondromanubrial protrusion, is caused by protrusion of the manubrium and the superior costal cartilages, accompanied by a relative depression of the body of the sternum.

Figure 119-15.

 

Pectus carinatum deformity with protruding sternum.

 

Repair of pectus carinatum is made through a transverse incision in similar fashion to that for open pectus excavatum repair (see Fig. 119-1). The skin and pectoral muscle flaps are mobilized in identical fashion. All the deformed or partially deformed cartilages should be removed. A single or double osteotomy then is performed to facilitate posterior displacement of the sternum (Fig. 119-16). The mixed pectus deformity is best corrected by full and symmetric resection of the third to seventh costocartilages, accompanied by a transverse offset wedge-shaped sternal osteotomy. This technique allows for both anterior displacement and rotation of the sternum. The chondromanubrial deformity is corrected with a broad wedge-shaped sternal osteotomy in the anterior cortex at the obliterated sternal manubrial junction.11 Closure of this osteotomy after fracture of the posterior cortex permits posterior displacement of the superior portion of the sternum. The wound is drained and closed in a fashion comparable to that for pectus excavatum repair.

Figure 119-16.

 

Repair of pectus carinatum by wedge osteotomy.

 

In the mixed deformity, the oblique position of the sternum must be corrected, as well as the position of the depressed and protruding costal cartilages. After subperichondrial resection of the abnormal costal cartilages, a wedge-shaped osteotomy is made in the anterior sternal plate, with the wide portion of the wedge on the depressed side of the sternum. Closure of this osteotomy simultaneously elevates and rotates the sternum into the desired position. It may be secured in this position either with sutures or with a strut.

For the chondromanubrial and mixed deformities, treatment of the sternum requires specialized techniques. In the case of the chondromanubrial variant, in which the upper position of the sternum protrudes and the lower body is angled toward the spine, the osteotomy, once closed, permits posterior depression of the lower portion of the sternum and anterior angulation of the manubrium (Fig. 119-17).

Figure 119-17.

 

Chondromanubrial defect.

Complications of this repair are rare and include infection, pneumothorax, pneumonia, and wound dehiscence. Blood transfusion should be required only rarely. Recurrences are rare.

POLAND'S SYNDROME

Poland's syndrome occurs in 1 in 30,000–32,000 live births and is not often familial.16 The etiology is not well understood. It comprises a constellation of anomalies including absence of the pectoralis major and minor muscles and syndactyly. It also may include absence of ribs, chest wall depression, athelia or amastia, absence of axillary hair, and limited subcutaneous fat. Children with this disorder show remarkable diversity. The predominant defect may vary depending on the degree of involvement of the various components. By definition, all children with Poland's syndrome have aplasia or hypoplasia of the sternocostal portion of the pectoralis major muscle and at least one other associated lesion (Fig. 119-18). The extent of the abnormality of the hand, breast, or chest wall may be quite variable. All patients with absent ribs should be considered candidates for repair. The aplastic ribs usually are some combination of the second to the fifth.

Figure 119-18.

 

Poland's syndrome.

For surgical correction, the initial surgical approach is via a transverse incision, similar to that for pectus excavatum repair.16 For patients requiring surgical repair, there is usually severe depression of the involved side, often with absent ribs, and rotation of the sternum. In patients with aplasia of the ribs, the endothoracic fascia is immediately below the attenuated subcutaneous tissue and pectoral fascia (Fig. 119-18A ). Care must be exercised during this dissection with absent ribs to avoid inadvertent entry into the thoracic cavity. The pectoral muscle flap is elevated on the contralateral side, and the pectoral fascia, if present, is elevated on the involved side. A subperichondrial resection of the costal cartilages then is performed. This is usually done on ribs three through seven. A transverse offset wedge-shaped sternal osteotomy is performed below the second costal cartilage. This defect then is closed either with silk sutures or by strut support, thereby correcting both the posterior displacement and the rotation of the sternum.

In patients with rib aplasia, split-rib grafts are harvested and secured medially with wire sutures into previously created sternal notches and with wire to the hypoplastic ribs laterally (Fig. 119-18B ). The ribs are split along the short axis to maintain maximal mechanical strength. Breast reconstruction is needed in most girls and should be delayed until late puberty to optimize the cosmetic result. Implants are often required in conjunction with a latissimus dorsi flap.

STERNAL DEFECTS

Sternal defects can be grouped into four categories: thoracic ectopia cordis, cervical ectopia cordis, thoracoabdominal ectopia cordis, and cleft or bifid sternum.17 For thoracic ectopia cordis, successful surgical approaches have been based on the concepts of constructing a partial anterior chest cavity around the heart and avoiding attempts to return the heart to an orthotopic position. No successful repairs of cervical ectopia cordis have been reported. In the case of thoracoabdominal ectopia cordis, a combined approach of closing (staged or primary) the abdominal wall defect and surgical correction of the cardiac anomaly has led to some successes.

Cleft or Bifid Sternum

Cleft or bifid sternum is the least severe form of sternal defect. Children in this category have an orthotopic heart, normal skin coverage, an intact pericardium, and a partially or completely cleft sternum. Cardiac defects in this group are rare. These defects are usually asymptomatic. They are repaired to protect the heart. The repair of a bifid sternum is done through a longitudinal incision extending the length of the defect.17 Immediately deep to the subcutaneous tissues lie the sternal bars, with pectoral muscles lateral to the bars (Fig. 119-19A ). The endothoracic fascia and pericardium are just below these structures. By using blunt dissection, the endothoracic fascia is dissected off the sternal bars posteriorly. A wedge of cartilage is excised inferiorly to permit approximation of the sternal bars. Sutures are placed in interrupted fashion around the bars to reapproximate them (Fig. 119-19B ). This repair is best accomplished in the neonatal period when there is greatest flexibility of the chest wall.

Figure 119-19.

 

Bifid sternum.

SUMMARY

The outcomes of operative repair of chest wall defects in children have been very satisfactory.12,14 One prospective multicenter observational study has compared open and Nuss repair of pectus excavatum.18 Although statistical comparisons between the methods were few, owing to small sample size, the authors concluded that surgical repair could be performed safely with successful pain management.

EDITOR'S COMMENT

Excellent results of pectus excavatum repair can be obtained with either the open or the Nuss procedure. Both procedures have their advantages or drawbacks. Long-term follow-up of pulmonary function or functional capacity, as well as recurrence rates may permit more meaningful assessment of these two procedures.

–LZ

REFERENCES

1. Shamberger RC, Welch KJ: Surgical repair of pectus excavatum. J Pediatr Surg 23:615–22, 1988. [PubMed: 3204460]

2. Brodkin HA: Congenital anterior chest wall deformities of diaphragmatic origin. Dis Chest 24:259–77, 1953. [PubMed: 13095240]

3. Brown A: Pectus excavatum (funnel chest): Anatomic basis, surgical treatment of incipient stage in infancy, and correction of the deformity in the fully developed stage. J Thorac Surg 9:164–84, 1939. 

4. Chin E: Surgery of funnel chest and congenital sternal prominence. Br J Surg 44:360–76, 1957. [PubMed: 13510593]

5. Vanamo K, Peltonen J, Rintala R, et al: Chest wall and spinal deformities in adults with congenital diaphragmatic defects. J Pediatr Surg 31:851–4, 1996. [PubMed: 8783122]

6. Meyer L: [Zur chirurgischen behandlung der angeborenen trichterbrust.]. Verh Berl Med Ges 42:364–73, 1911. 

7. Sauerbruch F: Die Chirurgie der Brustorgane. Berlin, Springer, 1920.

8. Ochsner A, DeBakey M: Chone-chondrosternon: Report of a case and review of the literature. J Thorac Surg 8:469–511, 1939. 

9. Ravitch M: The operative treatment of pectus excavatum. Ann Surg 129:429–44, 1949. [PubMed: 17859324]

10. Welch K: Satisfactory surgical correction of pectus excavatum deformity in childhood: A limited opportunity. J Thorac Surg 36:697–713, 1958. [PubMed: 13588724]

11. Shamberger RC, Welch KJ: Surgical correction of chondromanubrial deformity (Currarino Silverman syndrome). J Pediatr Surg 23:319–22, 1988. [PubMed: 3385582]

12. Shamberger RC: Congenital Chest Wall Deformities. St Louis, Mosby Elsevier, 2006.

13. Nuss D, Kelly RE Jr, Croitoru DP, Katz ME: A 10-year review of a minimally invasive technique for the correction of pectus excavatum. J Pediatr Surg 33:545–52, 1998. [PubMed: 9574749]

14. Nuss D, Kelly RE Jr: The Nuss procedure for pectus excavatum. In Shamberger RC (ed): Congenital Chest Wall Deformities. St Louis, Mosby Elsevier, 2006.

15. Shamberger RC, Welch KJ: Surgical correction of pectus carinatum. J Pediatr Surg 22:48–53, 1987. [PubMed: 3819993]

16. Shamberger RC, Welch KJ, Upton J 3rd: Surgical treatment of thoracic deformity in Poland's syndrome. J Pediatr Surg 24:760–5; discussion 766, 1989. 

17. Shamberger R, Welch K: Sternal defects. Pediatr Surg Int 5:156–64, 1990. 

18. Kelly RE Jr, Shamberger RC, Mellins RB, et al: Prospective multicenter study of surgical correction of pectus excavatum: Design, perioperative complications, pain, and baseline pulmonary function facilitated by internet-based data collection. J Am Coll Surg 205:205–16, 2007. [PubMed: 17660066]



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