Figure 80-14. Chest CT of pectus excavatum. CT image at xiphoid with A–B (transverse diameter) and C–D (anterior–posterior

diameter) indicated for the calculation of the pectus severity index A-B/C-D (A). CT image demonstrating angulation of the

sternum to the right in the severe defect (B). (Reproduced with permission from Haller JA Jr, Scherer LR, Turner CS, et al.

Evolving management of pectus excavatum based on a single institutional experience of 664 patients. Ann Surg 1989;209(5):578–

582.)

Figure 80-15. Photograph of a 4.5-year-old girl with a symmetric pectus excavatum deformity. Note that the depression extends to

the sternal notch. (Adapted with permission from Shamberger RC. Chest wall deformities. In: Shields TW, ed. General Thoracic

Surgery. 4th ed. Baltimore, MD: Williams & Wilkins; 1994:529–557.)

2289

The most commonly used open repair was first described by Dr. Ravitch and is based on removing

sections of the deformed cartilage from around the sternum laterally at the ribs, while preserving the

perichondrium. Many advocate using a stabilizing bar for a minimum of 6 months, usually placed in an

intrathoracic position, to minimize the occurrence of flail chest and to allow the sternum to heal in an

anterior position. An essential part of the procedure is a transverse sternal wedge resection through the

anterior table to allow the inferior aspect of sternum to bend forward.13,17 Incisions may be in a vertical

midline over the lower half of the sternum or in an inframammary position (Fig. 80-16). Recurrences

occur in 5% to 10% of patients, with recurrence more common in patients with connective tissue

disorders.

Since the 1990s, the Nuss procedure, which utilizes a minimally invasive approach, has been described

and performed. The Nuss procedure utilizes one or two stabilization bars, secured to the ribs laterally in

multiple points of fixation. It routinely employs thoracoscopy, either unilateral or bilateral. Skin

incisions are limited to small lateral locations at the site of the bar insertion and thoracoscopic portsites. In both the Ravitch and Nuss procedures, the stabilization bars stay in for at least 6 months.18 As

the Nuss procedure has grown in popularity, many modifications have been made, including precise

shaping of the bars and increased bar stabilization with wire fixation around the ribs to minimize bar

displacement.19

Pectus carinatum does not have the same cardiac compression concerns as pectus excavatum, but the

chest is held in an enlarged position, limiting normal respiration mechanics. Patients can have increased

residual lung volumes and their respiratory physiology can mimic emphysema physiology. This can

result in breathing difficulties and limited stamina.17 The Ravitch procedure is most commonly

performed to remove the deformed cartilage. Special attention is placed on the transverse sternal

osteotomy, to depress the sternum and align it with the anterior ribs (Fig. 80-17).1

The most commonly used open repair was first described by Dr. Ravitch and is based on removing

sections of the deformed cartilage from around the sternum laterally at the ribs, while preserving the

perichondrium. Many advocate using a stabilizing bar for a minimum of 6 months, usually placed in an

intrathoracic position, to minimize the occurrence of flail chest and to allow the sternum to heal in an

anterior position. An essential part of the procedure is a transverse sternal wedge resection through the

anterior table to allow the inferior aspect of sternum to bend forward.13,17 Incisions may be in a vertical

midline over the lower half of the sternum or in an inframammary position (Fig. 80-16). Recurrences

occur in 5% to 10% of patients, with recurrence more common in patients with connective tissue

disorders.

Since the 1990s, the Nuss procedure, which utilizes a minimally invasive approach, has been described

and performed. The Nuss procedure utilizes one or two stabilization bars, secured to the ribs laterally in

multiple points of fixation. It routinely employs thoracoscopy, either unilateral or bilateral. Skin

incisions are limited to small lateral locations at the site of the bar insertion and thoracoscopic portsites. In both the Ravitch and Nuss procedures, the stabilization bars stay in for at least 6 months.18 As

the Nuss procedure has grown in popularity, many modifications have been made, including precise

shaping of the bars and increased bar stabilization with wire fixation around the ribs to minimize bar

displacement.19

Pectus carinatum does not have the same cardiac compression concerns as pectus excavatum, but the

chest is held in an enlarged position, limiting normal respiration mechanics. Patients can have increased

residual lung volumes and their respiratory physiology can mimic emphysema physiology. This can

result in breathing difficulties and limited stamina.17 The Ravitch procedure is most commonly

performed to remove the deformed cartilage. Special attention is placed on the transverse sternal

osteotomy, to depress the sternum and align it with the anterior ribs (Fig. 80-17).13

2290

2291

Figure 80-16. A chevron incision is made with flaps elevated. The perichondrium on the deformed cartilage is removed (A). A

transverse wedge osteotomy is made across the anterior table of the sternum. The lower sternum is elevated to the desired level

(B). A metal strut is placed posterior to the sternum and attached to the ribs with fine wire (C).

Sternal Clefts

Sternal clefts are much rarer than either pectus deformity. They result from the failure of the sternum to

fuse in the midline. They can vary from minor defects with the upper half of the sternum separated, to

complete dissociation and thoracic ectopia cordis (the heart being outside the chest wall). Upper sternal

defects have a U- or V-shaped appearance and usually extend to the fourth intercostal space (Fig. 80-18).

Repair of these defects can be easily performed by incision, complete sternotomy, oblique

chondrotomies of the sternal halves, and closure. Complete sternal clefts are associated with diastasis

recti and an opening between the peritoneal and pericardial cavities. Inferior clefts are associated with

the pentalogy of Cantrell, a constellation of defects which include supraumbilical abdominal wall defect,

lower sternal cleft, defect in the anterior diaphragm, defect in the associated pericardium, and

congenital cardiac defects, often tetralogy of Fallot. With the increased use of prenatal ultrasound, most

cases are diagnosed early and are being appropriately referred to specialists prior to birth.20

Figure 80-17. Photograph of (A) symmetric chondrogladiolar pectus carinatum in a 19-year-old man. Postoperative photograph (B)

shows correction of the protruding sternum and costal cartilages. (Adapted with permission from Shamberger RC. Chest wall

deformities. In: Shields TW, ed. General Thoracic Surgery. 4th ed. Baltimore, MD: Williams & Wilkins; 1994:529–557.)

2292

Figure 80-18. Cleft sternum at rest (A) and during forced expiration (B). Superior clefts of the sternum are variously V- or Ushaped. The appearance of the child as he cries explains the term ectopia cordis, although the heart is actually not misplaced. In

the newborn, defects of this kind can be corrected by direct apposition of the sternal halves. In this child, closure of the defect was

made possible by sliding chondrotomies on either side. (Reproduced with permission from Sabiston DC Jr. The surgical

management of congenital bifid sternum with a partial ectopia cordis. J Thorac Surg 1958;35:118.)

Poland Syndrome

Poland syndrome is the clinical absence of a pectoralis muscle and underlying bony support in the chest

wall, and it occurs in 1/30,000 live births. It is usually a right-sided, unilateral defect. Patients

commonly have associated limb abnormalities, and may also present with renal and spinal lesions (Fig.

80-19). Clinical symptoms may be absent, though lung herniation can occur, requiring a more urgent

repair. The surgical approach is usually multidisciplinary and ideally is deferred until after the patient

has stopped growing. Repairs typically utilize mesh placement over the defect, and/or muscle flap

placement.21

Thoracic Outlet Syndrome

2 The thoracic outlet in thoracic outlet syndrome (TOS) refers to the area described at the beginning of

this chapter as the thoracic inlet, the correct anatomic term for the region of the first rib and the middle

and anterior scalene muscles. Through these structures, the brachial plexus, subclavian artery, and

subclavian vein travel to the upper limbs (Fig. 80-20). Because of the close proximity in this area, these

conduits may be subjected to abnormal compression by inflammation around the muscles or abnormal

bony structures such as cervical ribs, causing pain, thrombosis, and other symptoms that are described

as TOS. TOS can be divided into arterial, venous, or neurogenic diseases, or a combination, depending

on the symptoms and clinical findings. Vascular TOS is easier to document with vascular imaging.

Neurogenic TOS is more difficult to diagnose. Unfortunately for neurogenic TOS, there is no gold

standard diagnostic test to confirm the syndrome. As a result, there has been significant controversy

over the diagnosis and the indications for surgical treatment, especially in light of the potential for

significant postoperative complications.22,23

Vascular TOS can be divided into venous disease and arterial disease. Venous disease results in

thrombosis of the subclavian vein and is also called Paget–Schroetter syndrome. This syndrome usually

presents in men who use their upper bodies and dominant arm for work. Symptoms may present as

pain, swelling, and cyanotic discoloration.22 Contrast venography is the best test to make the diagnosis,

and the radiologist should be instructed to perform positional maneuvers with the patient’s arm to

replicate narrowing that occurs with arm movement.23 Arterial TOS accounts for about 5% of all cases

of TOS24 and also may result from repetitive and strenuous use. Patients will present with arm fatigue

and may even have evidence of ischemia in their distal digits. Patient evaluation includes pulse

examination with the arm relaxed and elevated, bruit examination for poststenotic dilatation, and blood

pressure readings in both arms. Duplex scanning and angiography will often confirm the diagnosis.

Decompression of the scalene muscles and any abnormal bony structures will alleviate these

symptoms.22

2293

Neurogenic TOS presents with limb pain, numbness, or weakness. Symptoms may be bilateral, but are

usually worse in the dominant arm. The distribution may not follow a peripheral nerve pattern, thus

making the diagnosis confusing, but also ruling out more distal compression syndromes such as carpal

tunnel in the wrist or cubital compression at the elbow. The physical examination should be focused on

duplicating the patient’s symptoms. The Adson maneuver may detect artery compression and is

performed by palpating the affected radial pulse, and having the patient take a deep inspiration while

turning their head away from the affected arm (Fig. 80-21). The test is positive if the pulse disappears,

but a positive result can also be seen in asymptomatic people without TOS. Another test requires the

patient to elevate their arms in a “surrender” position and open and close their hands repeatedly. Most

people with neurogenic TOS will be fatigued within a minute and be unable to continue (Fig. 80-22).

Plain radiographs may show bony abnormalities, but no other imaging will help diagnose neurogenic

TOS. Physical rehabilitation is the first step to treat patients with neurogenic TOS, and over a third of

patients may get a significant improvement in their symptoms. If there is no significant improvement in

symptoms, then surgical intervention is suggested.23

Figure 80-19. A: Muscular 15-year-old boy with loss of the left axillary fold, orthotopic sternum, and normal cartilages. He

compensates adequately for loss of the pectoralis major and minor muscles. Surgery is not indicated in males with these findings.

B: An 8-year-old boy with Poland syndrome. The pectoralis major and minor muscles and the serratus to the level of the fifth rib

are absent. The boy has sternal obliquity, and the third to fifth ribs are short, ending in points. The corresponding cartilages are

absent. The endothoracic fascia lies beneath a thin layer of subcutaneous tissue. Note the hypoplastic nipple and ectromelia of the

ipsilateral hand. C: A 14-year-old girl with Poland syndrome. Note the high position of the right nipple, amastia, sternal rotation,

and depressed right anterior chest. The second to fourth ribs and cartilages were missing, reconstructed with rib grafts. Breast

2294

augmentation will be required at full growth. (Reproduced with permission from Shamberger RC. Chest wall deformities. In:

Shields TW, ed. General Thoracic Surgery. 4th ed. Baltimore, MD: Williams & Wilkins; 1994:529–557.)

The surgical approach for TOS was traditionally a transaxillary first rib resection (Fig. 80-23). In

recent years, a supraclavicular approach has become increasingly popular as it gives better exposure to

the scalene muscles and any cervical ribs. In either approach, the first rib is exposed and the anterior

and middle scalene muscles are removed from their attachments to the rib. Once this is performed, the

first rib is removed (Fig. 80-24). Special attention must be paid to the phrenic nerve lying along the

anterior scalene and the axillary branches off of the brachial plexus. Small drains may be placed in the

apex of the thorax if the pleural space is entered.23,25 The supraclavicular approach also gives access to

perform a cervical sympathectomy if indicated for symptoms consistent with sympathetic overactivity.

In patients with arterial aneurysms, reconstruction with synthetic grafts or autologous conduits such as

an external iliac artery can be performed. For patients with venous TOS, care should be taken to

perform a circumferential venolysis and to remove the medial aspect of the first rib.25

Postoperative complications include pneumothorax, lymphatic leaks, especially on the left side where

the thoracic duct enters the operative field, phrenic nerve palsy, and traction injury to the brachial

plexus.24 Patients should have good pain control and may need muscle relaxants to minimize muscle

spasm. There should be limited restrictions to the operated extremity to maximize movement and

minimize the risk of developing a frozen shoulder. Rehabilitation should be restarted as soon as possible

after surgery.23,25 Upto 12.5% of patients may not get any symptom relief after the surgery, and longterm physical rehabilitation is recommended.24 Patients with combined symptoms of venous and

neurogenic TOS have worse outcomes when compared to patients with only venous disease. A higher

percentage of patients had persistent pain in the neurogenic group as might be expected.26 The

treatment of this disease process requires a significant investment into the preoperative and

postoperative care of these patients and should only be done in centers with a high volume and

expertise in this syndrome.

Figure 80-20. Important structures in the thoracic outlet. (Reproduced with permission from Scott-Conner CE, Dawson DL, Shirazi

MK, et al. Operative Anatomy. 3rd ed. Philadelphia, PA: Lippincott Williams & Wilkins; 2008.)

2295

Figure 80-21. The Adson maneuver. Patient inspires and turns head away from the affected arm. The test is positive if the pulse

disappears.

Figure 80-22. Surrender position with opening and closing of hand. Early fatigue is seen in neurogenic TOS.

NONNEOPLASTIC LUNG DISEASE

Lung Abscess and Bronchiectasis

3 Complications from pulmonary infections that require surgery are not common relative to the

frequency of pneumonias in both the inpatient and outpatient community. However, in some patients,

treatment of lung abscesses and bronchiectasis may include surgical intervention. Lung abscesses can

result from aspiration or occur after necrotizing pneumonia. Patients may present with chest pain and

fever. Routine use of antibiotics for pneumonias has dramatically reduced the rate of lung abscess

sequelae. In over 80% of cases, a pathogen can be found, and in immunocompromised patients, there

are usually multiple organisms involved including Pseudomonas aeruginosa and Haemophilus species. The

primary treatment for lung abscesses is medical therapy with antibiotics. If a patient is clinically not

improving with medical therapy alone, a guided drainage tube may be placed. A complication of this

approach is the risk of a chronic air leak from a percutaneous tube placed into the lung parenchyma.

Surgery may be indicated in the setting of large abscesses causing hemoptysis or other symptoms

(Algorithm 80-1). Surgical resection may also be of clinical benefit in some patients by removing lung

tissue damaged by necrotizing pneumonia.27

Bronchiectasis is the abnormal dilation of bronchi/bronchioles as the result of multiple episodes of

2296

infection and inflammation. Factors contributing to the development of this disease may be genetic,

anatomic, or even systemic. Genetic causes include cystic fibrosis, which results in diminished mucus

clearance and diffuse bronchiectasis in both lungs.28 Primary ciliary dyskinesia is an autosomal recessive

syndrome resulting in abnormal cilia that cannot clear secretions leading to multiple infections.

Individuals with alpha-1-antitrypsin deficiency can also develop bronchiectasis. Anatomic causes include

postsurgical changes or acquired diseases such as chronic obstructive pulmonary disease (COPD) that

lead to an increased risk of infection, again by the inability to clear secretions. Autoimmune diseases

such as rheumatoid arthritis and inflammatory bowel disease have been associated with increased rates

of bronchiectasis.28,29 Infectious causes include allergic bronchopulmonary aspergillosis and

nontuberculous mycobacterium infections. Reflux disease is also being studied as a potential

contributor. Most often the etiology of the disease is idiopathic.28

Figure 80-23. Position of the patient (A) and skin incision (B) for transaxillary approach. (Reproduced with permission from ScottConner CE, Dawson DL, Shirazi MK, et al. Operative Anatomy. 3rd ed. Philadelphia, PA: Lippincott Williams & Wilkins; 2008.)

There are estimated to be 110,000 patients in the United States being treated for noncystic fibrosis

bronchiectasis each year. The diagnosis is increasingly being made as a result of an increasing number

of chest CT scans being performed for other, nonrelated reasons. Patients can present with respiratory

symptoms like dyspnea, hemoptysis, or pleuritic pain, or alternatively may present only with systemic

changes such as fatigue and weight loss.28 Almost all patients will have a chronic cough with or without

sputum production.29 Pulmonary function tests may demonstrate moderate to severe airflow

2297