are removed by subperichondrial excision, with careful preservation of the perichondrial sheaths. The

xiphoid is detached from the sternum and the rectus muscles are detached inferiorly. The undersurface

of the sternum is dissected free of the pericardium and pleura. A transverse wedge osteotomy is created

in the sternum at the point where the sternum deflects posteriorly and the posterior surface is gently

fractured but not displaced. A strut is usually placed beneath the sternum to maintain the position of the

sternum. The strut is usually a stainless steel bar and is removed in 6 months to 1 year.2,4 The pectoral

and rectus muscles are reapproximated. Closed suction drains are usually left in place in the

submuscular layer. Patients are discharged when they are able to tolerate a diet and their pain is

controlled by oral analgesics usually on the second to third postoperative day. Multimodal pain

management including regional anesthesia, epidural analgesia, nonsteroidal anti-inflammatory agents,

and narcotics are used. Complications may include pneumothorax not requiring therapy, wound

seromas, and pleural effusions. Recurrence occurs in approximately 7% if repaired early but less than

2% undergo reoperation.2 The incidence of reactive pectus carinatum after excavatum repair is 1%.13

Migration of implanted struts and bars has caused the most serious problems, including cardiac injury.

Figure 102-1. Pectus excavatum. A: Appearance of an adolescent male with a typical depression deformity. Note “stoopshouldered” posture. B: Postoperative appearance at 6 months. Erect posture and subpectoral incision give a good cosmetic result.

The Minimally Invasive Repair/Nuss Procedure

Since the description of a technique not involving resection or osteotomy in 1998, the performance of

the minimally invasive/Nuss procedure has gained widespread popularity. Using small lateral incisions,

a rigid U-shaped bar is inserted transversely across the chest and then flipped and the sternum is pushed

back into a normal position (Fig. 102-2).14 Modifications of the technique including the use of

stabilizers, use of two bars, and routine use of thoracoscopy have been described.2,11,15 Reported

complications have included pneumothorax, cardiac injury, bar displacement, chronic pain, pericarditis,

and extraosseous bone formation.15–17 Postoperative pain management usually involves epidural

analgesia as well as intravenous and oral medications, with a recent prospective study supporting

epidural for early management and patient-controlled analgesia for later management.18 The bar is left

in for up to 3 years during a period of development when most children are active and may engage in

contact sports. Activity restrictions that are necessary while the bar is in place are not well defined. This

technique is an important addition to the surgical treatment of chest wall deformities. Recent studies

have demonstrated that the early-to-late teenage years are the appropriate time for repair.19,20

Other Techniques

Implantation of submuscular silastic molds to alter the chest contour may result in a very acceptable

appearance.21 This technique does not address any of the potential physiologic problems associated with

pectus excavatum and has not been used widely.

Externally applied suction may also be used to treat depression deformities of the chest wall. Finally,

implanted devices that use magnetic forces to pull the sternum forward show promise in correcting the

defect in patients with pectus excavatum.22,23

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Pectus Carinatum

2 Pectus carinatum, from the Latin phrase for “keel,” is a chest wall defect where there is a protrusion

of the sternum and ribs. This type of defect occurs less commonly than pectus excavatum and presents

in approximately 1 in 1,000 to 1 in 1,500 teenagers. Physiologic impairment is absent; however, the

concern for appearance may be even greater than with depression deformities of the chest wall.

Protrusion deformities may be very difficult to hide even when fully clothed. Rapid growth of the costal

cartilages occurring during puberty makes the deformity more pronounced at that time. Compression

bracing has been described and is currently the first line of repair for pectus carinatum with surgery

reserved for failures of therapy.24–29 Bracing is most successful with bracing for 16 to 20 hours per day.

Surgery which involves the transverse sternal osteotomy or use of a minimally invasive technique with

the bar passed anterior to the sternum.22,28

Poland Syndrome

Named after the English surgeon Sir Alfred Poland, this rare congenital syndrome occurs in 1 in 30,000

births and includes a hypoplasia or aplasia of the thoracic wall components. These features include

absence of the sternal portions of the pectoralis major muscle; absence of the pectoralis minor muscle;

absence of portions of the serratus anterior and external oblique muscles; forearm and hand deformities;

ipsilateral hypoplasia of the nipple, breast, and chest wall subcutaneous tissues; absence or deformity of

the second to fifth costal cartilages; and absence of axillary hair on the affected side. Surgical treatment

includes correction of any deformed ribs, formation of the anterior axillary fold using the latissimus

dorsi muscle, and creation of the ipsilateral breast in females.30,31

Figure 102-2. Thoracoscopically assisted Lorenz Bar insertion (Nuss procedure). A: Preoperative appearance with symmetrical

depression deformity. B: Lorenz bar. C: Pectus excavatum bar inserted with thoracoscopic guidance. D: Immediate postoperative

appearance. (Digital images provided by William Hardin, MD, University of Alabama, used with permission.)

Sternal Clefts

Congenital sternal clefts result from failure of midline fusion of the paired sternal bands. The cleft is

usually in the superior portion of the sternum and may extend to but not include the xiphoid. The

defects are rare and are usually repaired in the neonatal period when the chest is more pliable.22,32 The

purpose of the correction is to protect the underlying mediastinal structures. After subcutaneous and

substernal mobilization, the fibrocartilaginous sternal bars are approximated in the midline. If the

cardiac structures are at risk, more elaborate repairs are required. Typically, the cleft is incomplete and

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a V-shaped excision of the sternum at the most inferior aspect of the cleft may be required to avoid

buckling of the sternum in that region.22,32 Cantrell pentology refers to a distal sternal cleft,

omphalocele, diaphragmatic defect, pericardial defect, and intracardiac defect. Thoracic ectopia cordis

(exstrophy of the heart) may occur as a part of this abnormality and this spectrum continues to have a

high mortality.22

Jeune Asphyxiating Thoracic Dystrophy

Jeune asphyxiating thoracic dystrophy is a rare developmental abnormality that results in the failure of

chest wall growth. Traditional surgical procedures have involved sternotomies with placement of graft

material to expand the chest. Most recently, the use of a vertical expandable prosthetic titanium rib

(VEPTR) device allows division of the most posterior aspect of approximately the third to ninth ribs and

division at the costochondral junction of the same ribs. The rib plate is then elevated to the curved

VEPTR strut by wires, thus expanding the chest. This is done for both chests, and the device is serially

expanded every 4 to 6 months to promote chest enlargement.22,33

The “Slipping Rib” Syndrome

Slipping rib syndrome is an unusual cause for recurrent lower chest and upper abdominal pain. The

cause is likely an inadequacy or rupture of interchondral fibrous attachments. The symptoms tend to be

a popping or clicking sensation associated with pain. Pain is usually reproducible with palpation.

Surgical therapy involves excision of the affected rib and cartilage relieving pain on the involved

intercostal nerve.34,35

“Flaring” of the Costal Margin

Patients are sometimes referred for surgical opinions regarding unusual prominence of the costal

margin. The “flaring” is most pronounced when the patient is supine. This finding represents a variation

of the normal development of the chest wall. Surgical correction should be avoided.

Costochondral “Tumor”

Prominence of a single costal cartilage may present as a concern of a tumor of cartilage or bone. This

abnormality is usually found in preadolescents and adolescents. Patients may have undergone a

diagnostic workup that includes chest radiographs, ultrasound, CT, or magnetic resonance imaging

(MRI) prior to surgical consultation. This abnormality most likely represents a minor variation of a

chest wall deformity, such as a pectus carinatum. The area of enlargement is rarely tender so that

costochondritis is an unlikely explanation. Excision of the involved cartilage is rarely helpful. Plain

radiographs or thoracic imaging are usually sufficient to exclude a tumor.36

CONGENITAL LUNG LESIONS

Congenital lung lesions have been characterized as lesions of defective budding, differentiation and

separation of the foregut, or more recently airway maldevelopment with resultant obstruction.37,38

These lesions include pulmonary sequestration (PS), congenital pulmonary airway malformation

(CPAM), formerly CCAM, bronchogenic cyst (BC), and congenital lobar overinflation (CLO). The first

three are in the family of bronchopulmonary foregut malformations. PS is characterized by aberrant

blood supply, CPAM by hamartomatous lung parenchyma, and BC by an isolated cyst. There are

overlaps between the elements so many lesions are found to be hybrid.37,38 CLO is likely a secondary

response to abnormal compression or obstruction of a bronchus.

Pulmonary Sequestration

3 PSs are microscopic cystic masses of nonfunctioning pulmonary tissue without a normal bronchial

communication with the tracheobronchial tree. In addition, these abnormalities receive blood supply

from an aberrant systemic vessel usually from the descending aorta. The lesions are most commonly

found in the lower lobes, more commonly on the left. They are characterized as intralobar or extralobar

based on pleural lining.

Intralobar sequestrations are incorporated into the surrounding lung and are invested by its visceral

pleura. They are most common in the left medial or posterior basal segment of the lower lobe. The

arterial supply is from the descending aorta and the venous return is usually into a pulmonary vein.

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Usually asymptomatic at birth, but aberrant air space connections can result in recurrent pneumonia.

Extralobar sequestrations occur outside of the pleura of the normal lung. They are most common in

the lower left side of the chest but have been reported in a variety of locations including the retro

peritoneum.37–39 In the retroperitoneum, they may be difficult to distinguish from neuroblastoma. These

have occasionally been associated with congenital diaphragmatic hernia (CDH). In extralobar PS, in

addition to systemic arterial supply, the venous return is systemic. In 20% of cases, the blood supply

originates from the infradiaphragmatic aorta. These lesions can present with arteriovenous shunting

which in rare cases can lead to high output heart failure.

Radiographic evaluation of PS involves chest radiographs, ultrasound and CT scans with contrast

enhancement to identify the vascular supply. Intralobar sequestrations are treated with lobectomy.

Extralobar sequestrations may be removed by excision of the sequestration. The lesions may be

removed with thoracotomy or thoracoscopy with attention to safe ligation of the vascular supply (Fig.

102-3).37–42

Congenital Pulmonary Airway Malformations

4 CPAMs result from overgrowth of bronchial structures at the expense of the associated alveoli

associated with an abnormal supplying airway affecting a single lobe in 95% of cases. This group of

anomalies represents 30% to 40% of the developmental lung bud anomalies. On gross appearance, they

can be solid, cystic, or both. The malformations have a normal blood supply and communicate with the

normal tracheobronchial tree. The common classification separates these lesions into three main types.

Type I CPAMs consist of cysts with large, irregular widely dispersed spaces larger than 2 cm or a single

large cyst with multiple small surrounding cysts and represent 50% to 65% of postnatal cases. Type II

CPAM is described as numerous smaller cysts (0.5 to 2 cm) and account for 10% to 40% of cases. Type

III CPAMs are made of small cystic lesions (generally 0.2 cm) or solid lesions appearing similar to

immature lung devoid of cartilage and make up of 5% to 10% of cases. CPAMs generally only affect one

lobe but multilobar and bilateral involvement occurs. Most commonly, the lesions are left sided and

affect the lower lobes. Several genes have been implicated in the development of CPAMs including

HOXB5, Fgf7, and PDGF-B.43

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Figure 102-3. Pulmonary sequestration (PS). A: Sagittal ultrasound view of the aortic branch supplying an extralobar PS. B: Leftsided extralobar PS excised via a muscle-sparing thoracotomy. C: Intraoperative view of extralobar sequestration removed

thoracoscopically. Note the main blood supply with clips.

Prenatal diagnosis is common occurring in 79% of cases. In fact, prenatal ultrasound has led to an

increased diagnosis of lung lesions.44 CPAMs may lead to sufficient pathophysiologic effects including

life-threatening hydrops. Fetal surgery has included open resection and thoracoaminiotic shunting with

survival of some of the affected fetuses.36,42 The natural history of prenatally diagnosed CPAMs is

variable and even large lesions may regress. Growth tends to plateau at 26 to 28 weeks gestation.43,44

The incidence is reported to be between 1:25,000 and 1:35,000 live births which likely

underrepresent the incidence as historically, approximately 14% of cases are stillborn. Although 80%

are asymptomatic at birth, nearly 80% will have respiratory symptoms within the first month of life.38

Less commonly, patients may present with pneumonia later in life (Fig. 102-4).

There is little controversy about symptomatic lesions which require resection of the involved lobe.

Those with severe respiratory compromise may require resection on extracorporeal membrane

oxygenation (ECMO) with excellent results. Other infants may benefit from an ex utero intrapartum

treatment (EXIT) to ECMO approach when gas exchange is predicted to be compromised at delivery.44

Treatment of asymptomatic lesions is controversial as the natural history of the lesion is unknown.

There is concern for future infection and the possibility of malignancy.37,38,45,46 Those who recommend

resection favor timing between 2 and 10 months of age. With antenatal diagnosis, a CT is necessary to

determine resolution of the lesion. There is evidence of inflammation in the resected lesions leading to

recommendation for early resection.47 Lesions can be resected via thoracotomy or thoracoscopy. With

multilobar and bilateral disease, CT scans assist in planning lung-preserving resections. Bilateral and

multilobar lesions are thought to be associated with the development of pleuropulmonary blastoma.46

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Figure 102-4. Congenital cystic adenomatoid malformation (CCAM). A: Newborn chest x-ray of a patient with prenatal diagnosis

of CCAM. Note that immediately after birth, the mass is fluid-filled and appears as a density in the right hemithorax. B: Chest x-ray

of the same child at 5 months of age prior to surgical excision. Note the air-filled cystic appearance of CCAM. C: Transverse

thoracic computerized tomographic view of CCAM.

Bronchogenic Cysts

BCs are uncommon anomalies arising from defective development of the large airways. They can be

mediastinal or intrapulmonary and cause symptoms of cough as well as recurrent and chronic infection.

These cysts appear as discrete masses and develop from the esophageal or tracheal position of the

foregut. They are usually extrapulmonary and do not communicate with the normal tracheobronchial

tree. Resection via thoracotomy or thoracoscopy is recommended due to the risk of bleeding, infection,

or enlargement with compression of adjacent structures (Fig. 102-5).41,48–50

Congenital Lobar Overinflation

CLO (previously labeled congenital lobar emphysema) is caused by abnormal development or

compression of a lobar bronchus resulting in retention of fluid during fetal lung development and

subsequent air trapping and overinflation after birth. The cause ranges from a deficiency of bronchial

cartilage to compression from an extrinsic mass. The presentation is typically with upper lobe of either

lung field and rarely multilobar. Definitive cause is rarely identified. Lobar overinflation may present as

worsening respiratory distress caused by enlargement of the overinflated segment with compression of

the normal surrounding lobe (Fig. 102-6). Symptomatic infants should undergo complete resection of

the lobe. Care must be taken at resection as positive-pressure ventilation may worsen symptoms and

require immediate thoracotomy. With relatively asymptomatic patients, symptoms will improve and

allow for a nonoperative approach.39,51

Diagnosis

Due to the routine use of prenatal ultrasound, many cystic lesions of the lung are now detected and

followed prior to birth.37–39,44,46 Many of the patients with these lesions are asymptomatic in the

perinatal period. Standard chest radiographs remain the standard for diagnosis and follow-up for lesions.

Rapid thoracic CT scanning with contrast provides definitive mapping of the lesions including

demonstration of aberrant vessels. Contrast esophagograms are indicated for children with dysphagia

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and suspicion of connection with the GI tract. In lesions with suspected aberrant arterial supply, MRI

angiography can be helpful if the CT is not definitive.

Treatment

There is controversy about the management of asymptomatic lesions with proponents of resection citing

the rare association with malignant pulmonary neoplasms.37–39 Any cyst enlarging on serial radiographs

should be resected due to the risk of respiratory compromise and the possibility of infection of the cyst

and the surrounding tissue. The reason for long-term follow-up is the demonstration of progressive

resolution.

Cysts of the Mediastinum

5 Benign cysts of the mediastinum are relatively common in the pediatric population. Most of these are

relatively asymptomatic but surgery is recommended due to the possibility of enlargement with

symptoms related to compression, hemorrhage, or infection. The symptoms which occur include cough,

chest pain, stridor, hemoptysis, or dysphagia. The common lesions include thymic cysts, enterogenous

cysts, lymphatic malformations (cystic hygromas), and pericardial cysts.

Figure 102-5. Bronchogenic cyst (BC). A: Transverse thoracic computerized tomographic view of a BC located in the inferior

pulmonary ligament. B: Illustrative representation of thoracoscopic instrumentation for excision of a BC. C: Operative detail.

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Figure 102-6. Congenital lobar overinflation. A: Chest radiograph demonstrating overinflation of the left upper lobe in an infant.

Note the mediastinal shift. B: Operative photograph demonstrating an overexpanded left upper lobe decompressing through the

incision.

Thymic Cysts

Thymic cysts present in the neck and mediastinum and are usually unilocular but may be multilocular.

They are most commonly seen in the anterosuperior mediastinum. They are benign lesions with

presence of cholesterol granulomas. Symptoms may arise from rapid expansion due to infection.

Resection is recommended.52,53

Enteric Duplication Cysts/Enterogenous Cysts

Enteric duplication cysts are usually located in the posterior mediastinum with microscopic evidence of

double-layer smooth muscle and esophageal or gastric mucosa. They are from the same spectrum as

bronchopulmonary cysts. These may communicate with the stomach or esophagus and may transverse

the diaphragm. The cysts may also extend into the spinal canal.52,53 The lesions may have gastric

mucosa leading to bleeding. Other symptoms may include airway compression or dysphagia (Fig. 102-

7). Resection is indicated for all symptomatic lesions and is favored for asymptomatic lesions.

Lymphatic Malformations

Lymphatic malformations (cystic hygromas) are unilocular or multilocular cysts which are thin walled

and derived from failure of adequate drainage of embryonic lymph sacs. These may occur in the head,

neck, and mediastinum. The lesions insinuate around great vessels and nerves in the superior

mediastinum. The incidence is 1 in 6,000 live births. Symptoms if present are related to enlargement

(frequently due to hemorrhage) or infection. Resection while preserving vital structures is

recommended although lesions have been treated with sclerotherapy. The most frequent complications

are recurrence and accumulation of lymph in the resection bed.52–54

Pericardial Cysts

Pericardial cysts are asymptomatic and are found incidentally on chest radiograph. The cysts are found

in the cardiophrenic sulcus as a discrete mass. The cysts are thin walled with a flat mesothelial lining

and contain clear fluid. Resection can be performed with thoracotomy or thoracoscopy.52,54

THORACIC TUMORS IN CHILDREN

6 Thoracic tumors are rare in children and diagnostic evaluation must exclude evaluation for both

primary and metastatic conditions. Tumors may occur in the lung or the mediastinum.54 Primary lung

neoplasms in children are extremely rare.55,56 Metastatic lung lesions are more common in the pediatric

population.57 Mediastinal tumors reflect the nature of the section of the mediastinum in which they

arise. Diagnostic evaluation begins with routine radiographs with CT scans and MRIs for further

evaluation.54

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