Chapter 96

Thoracic Surgery: 

Surgical Considerations 

96 

J. Ted Gerstle CHAPTER 

INTRODUCTION 

The topic of pediatric thoracic surgery is large and constantly 

changing. This change is being driven in part by issues including: 

(1) the increasing use of antenatal ultrasounds with the subsequent 

identification of lung lesions, which previously may have remained 

quiescent, asymptomatic, and thus, undiagnosed; (2) higher-quality 

diagnostic imaging studies, which have the ability to identify 

smaller and smaller abnormalities in the lung; and (3) the 

increasing use of minimal access surgical techniques in the chest, 

both diagnostically and therapeutically. The latter has been gaining 

great momentum as surgical skills have matured and operative 

equipment has improved, including the adaptation of this 

equipment to the smaller body habitus of a child. This chapter on 

pediatric thoracic surgery will emphasize the role of thoracoscopy 

and highlight some of the new indications for its use. Where 

possible, data will be presented in an evidence-based fashion. The 

format of individual thoracic diseases and abnormalities will 

include (where it is indicated and it gives clarity to the discussion 

of the topic): (1) clinical presentation, (2) diagnostic moda - 

lities, (3) preoperative considerations, (4) intraoperative consi - 

derations, and (5) postoperative considerations. Purposefully, 

certain topics will be excluded from discussion in this chapter, 

because they will be reviewed in other chapters of this textbook, 

including esophageal atresia, tracheoesophageal fistula, congenital 

diaphragmatic hernia (neonatal), tracheomalacia and tracheal 

stenosis. 

NEOPLASMS 

The use of thoracoscopic procedures in children has been 

broadened to include its use in the diagnosis and treatment 

of neoplasms. The size of solid malignancies and benign tumors 

in children limits the use of therapeutic thoracoscopy. Therefore, 

minimally invasive diagnostic procedures are more common 

in children than therapeutic procedures for malignancy. 

Current diagnostic and therapeutic thoracoscopic procedures 

discussed in this section include: (1) diagnostic thoracoscopy, 

(2) thoracoscopic lung biopsies, (3) thoracoscopic mediastinal 

biopsies, and (4) thoracoscopic tumor resections of the media - 

stinum. 

When considering the use of thoracoscopy for pediatric 

malignancies and tumors, the potential short-term and long-term 

benefits must be reviewed. The short-term benefits are similar to 

many procedures using thoracoscopy (e.g., less pain, shorter 

recovery period, etc.). With malignancies, however, it is the longterm 

benefits that are more important, specifically recurrence rates 

and survival. A significant amount of basic science has been 

carried out to investigate the role of minimal access surgery (MAS) 

and its potential to either positively or negatively alter the outcome 

of children with malignancies. 1–3 

Thoracic Lung Biopsy 

Waldhausen et al. published a study that reviewed their experience 

with MAS and clinical decision-making with pediatric 

cancer patients. 4 Video 1 demonstrates a right-sided computed 

tomography (CT)-guided lung tattooing in which two lesions in 

the superior lower lobe have been marked before a thoracoscopic 

lung biopsy. The investigators thought that the benefits of the 

thoracoscopic procedures were (1) alleviating the need for open 

thoracotomy and thus, avoiding the associated morbidity of 

this procedure, (2) hastening of the recovery time, and 

(3) improved visualization of the pleural surfaces. Limitations 

where thoracoscopy was not useful included (1) medial-located 

pulmonary lesions (where CT-guided needle localization could 

not be used), (2) children who had uncorrectable coagulopathies, 

and (3) children in whom an ipsilateral pneumothorax could 

not be created safely. Overall, thoracoscopy was felt to be accurate 

and safe. 

On the basis of this study and other studies, a few conclusions 

can be made 5–7 ; (1) thoracoscopic biopsies are accurate, have a low 

complication rate and appear to have a faster rate of recovery; 

(2) the initial approach to a pulmonary lesion may be with a 

percutaneous approach where the biopsy is for diagnostic 

purposes and the lesion is larger than 5 mm in diameter; and 

(3) in the case of a percutaneous biopsy, there is a relatively high 

chance that a second biopsy may have to be performed to obtain 

a definitive diagnosis. Although thoracoscopy is effective for 

diagnostic purposes, its role in the therapeutic realm remains 

untested. Presently, there is no role for thoracoscopic surgery in 

the treatment of children requiring pulmonary resections for 

multiple metastatic lesions, including osteogenic sarcoma and 

peripheral nerve sheath tumors. In these tumors, it is imperative 

that all palpable lesions be removed. There is no means to locate 

these lesions such that thoracoscopy can be effective; they are 

often missed on CT scan imaging 8 and only noted on direct 

palpation. As the precise excision of these metastatic lesions will 

directly influence the patient’s survival, they should always be 

addressed with a thoracotomy.

1642 PART 5 ■ Anesthetic, Surgical, and Interventional Procedures: Considerations 

Thoracoscopic Mediastinal Biopsies 

In the pediatric patients of various studies, thoracoscopic biopsy 

of mediastinal masses has been demonstrated to be safe and 

effective. 4,9 Thoracoscopic mediastinal biopsy is contraindicated, 

however, in children in whom carinal and subcarinal compression 

from the anterior mediastinal mass can be demonstrated. For 

these situations, percutaneous biopsy with local anesthesia only 

or 24 hours of intravenous steroids are recommended. 10–13 

Alternatively, a Chamberlain procedure in a semiupright position 

under local anesthesia, with spontaneous ventilation, can be 

helpful in patients in whom a needle diagnosis of Hodgkin disease 

(HD) is unable to be obtained. 12 A single case has been reported 

in which radiation was administered to a limited area of the tumor 

before the initial biopsy was performed. A diagnosis was 

established based on tissue from the shielded area after the main 

mass had decreased in size. 14 To minimize morbidity and mortality 

in those children undergoing a biopsy of a mediastinal mass the 

following have been recommended: a tracheal diameter of at least 

50% of normal and a peak expiratory flow rates of at least 50% of 

predicted value. 12 Deviation from these standards may result in 

loss of the airway or hemodynamic collapse while undergoing 

induction for a general anesthetic with subsequent mortality. 

Thoracoscopic Tumor Resections 

of the Mediastinum 

Thoracoscopic resection of mediastinal masses in children has 

been limited. There have been reports of resection of benign 

neurogenic tumors of the posterior mediastinum in adults and a 

few children. 15–16 In a more recent study by Lacreuse et al., the 

authors examined the role of thoracoscopy in the resection of 

posterior mediastinal tumors that were neurogenic in origin: 

ganglioneuroma, ganglioneuroblastoma, and neuroblastoma. 17 

The procedures had minimal complications, including two 

chylothoraces that improved with conservative management and 

one conversion to an open thoracotomy. The authors felt 

thoracoscopic resection is a feasible, safe, and efficient procedure, 

as it allows for improved visualization of the tumor and its 

anatomic connections and resection can be as complete as an open 

procedure without having to complicate the operative technique in 

the same operating time. 

Mediastinal Neoplasms 

In general, these malignancies are separated into groups on the 

basis of location within the mediastinum, including the anterior, 

middle, and posterior regions of the mediastinum; for purposes 

of this discussion, the superior region of the mediastinum is 

grouped with the anterior region (Table 96–1). The relative 

incidence of the common mediastinal neoplasms is given in Table 

96–2. Although lymphangiomas are not mediastinal neoplasms 

per se, they have important clinical issues as masses in the 

mediastinum and will be discussed in this section. 

Hodgkin Disease 

Lymphoid tumors which include HD and non-Hodgkin 

lymphoma (NHL) account for the highest percentage of 

mediastinal masses in children. 18 The majority of patients with 

Hodgkin disease will be older than 17 years; 11% are 11 to 16 years 

and 4% are younger than 10 years. Children who are diagnosed at 

TABLE 96-1. Different Types of Mediastinal Neoplasms, 

by Region Within the Mediastinum 

Anterior a Middle Posterior 

Thymoma Non-Hodgkin Ganglioneuroma 

lymphoma 

Thymic carcinoma Hodgkin Ganglioneuroblastoma 

lymphoma 

Non-Hodgkin Pericardial Neuroblastoma 

lymphoma 

teratoma 

Hodgkin lymphoma Cardiac rhab- Pheochromocytoma 

domyoma 

Teratoma Lipoblastoma PNET 

Germ cell tumor 

Lipoblastoma 

Lipoblastoma 

PNET = primitive neuroectodermal tumor. 

a 

Anterior includes the anterior and superior regions. 

an age of less than 16 years have the best prognosis. Clinically, 

cervical, mediastinal, axillary, and inguinal lymphadenopathy are 

noted in 80%, 50%, 33% and 5% of the patients, respectively; some 

children will also have symptoms of weight loss, fever, and night 

sweats, which portend a worse prognosis. The treatment of this 

disease is nonsurgical, consisting of chemotherapy and radiotherapy. 

The challenge posed to the surgical and anesthesia teams 

is how to safely obtain pathologic tissue to confirm the diagnosis 

and determine the histologic subtype of HD. As a high percentage 

of patients will have a concurrent mediastinal mass, the risk of a 

major intraoperative complication of cardiorespiratory collapse is 

potentially very high. To avert this risk, a series of preoperative 

tests can be performed: echocardiography, chest CT scan, and 

pulmonary function tests. 

Shamberger et al. used the cross-sectional area of the trachea on 

CT scan scanning to predict anesthetic complications and used it 

to determine who would be a candidate for local anesthesia versus 

general anesthesia. 19 Hack et al. found that a tracheal crosssectional 

area of

CHAPTER 96 ■ Thoracic Surgery: Surgical Considerations 1643 

Figure 96-1. Computed tomography scan (axial) of the chest of 

a patient with an anterior mediastinal mass related to Hodgkin 

disease. The mass is projecting in the left hemithorax and is 

shifting the mediastinal structures into the contralateral chest. 

In addition, there is extension of the mass into the soft tissues 

of the anterior chest and sternum. 

The most significant predictor of anesthetic complications appears 

to be a clinical finding of stridor and/or positional dyspnea; in 

these patients, general anesthesia should be avoided and a 

peripheral lymph node which can be biopsied under local anesthesia 

should be sought. 20–21 

Non-Hodgkin Lymphoma 

NHL is the third most common malignancy in childhood. There 

are three types of NHL that occur in children, making up >90% of 

diagnoses: lymphoblastic lymphoma, small–noncleaved-cell 

(Burkitt and non-Burkitt) lymphoma and large-cell lymphoma. 

Various immunodeficiency states are associated with NHL: 

human immunodeficiency virus, Wiskott-Aldrich syndrome, 

Bloom syndrome, ataxia telangiectasia, severe combined immunodeficiency 

disease, X-linked lymphoproliferative syndrome, 

and organ transplantation. Clinically, NHL presents abruptly and 

can progresses rapidly, having wide spread lymph node and 

extralymphoid involvement. Mediastinal involvement is predicted 

by the type of NHL with lymphoblastic lymphoma presenting 

most commonly in the anterior mediastinum in 50 to 75% of 

cases. In addition, some types of NHL, such as small–noncleavedcell 

(Burkitt’s and non-Burkitt’s) lymphoma, can have a tumor 

doubling time of 24 hours, making rapid diagnosis of paramount 

importance to avoid the complication of tumor lysis syndrome 

(hyperuricemia related to rapid cell death). The approach to the 

management of an anterior mediastinal mass in NHL is similar to 

HD; however, there are often other options to safely obtain a tissue 

diagnosis with NHL using local anesthesia which should be 

considered. These include bone marrow biopsy, aspiration of 

thoracic effusions, and aspiration of ascites. 

Neurogenic Tumors 

Neurogenic tumors, which include ganglioneuroma, ganglioneuroblastoma, 

and neuroblastoma, are the second most common 

mediastinal neoplasm in children; the vast majority of these 

are located in the posterior mediastinum. From a thoracic 

Figure 96-2. Thoracoscopic view of a neuroblastoma arising 

from a stellate ganglion in the apex of the right hemithorax in a 

patient who presented with a Horner syndrome. 

presentation perspective, these children may present with respiratory 

distress or a Horner syndrome (ipsilateral ptosis, myosis, 

anhydrosis, and heterochromia). Figure 96–2 demonstrates a 

thoracoscopic view of a neuroblastoma arising from a stellate 

ganglion in the right chest in a patient who presented with Horner 

syndrome; figure 96–3 illustrates this same right-sided thoracic 

tumor on a coronal view of a magnetic resonance image. These 

patients may also present with neurologic symptoms related to a 

dumbbell-shaped tumor involving the chest and spinal canal 

simultaneously; the latter may have cauda equina syndrome or 

paraplegia. 23 

Figure 96-3. Magnetic resonance image (coronal) of a patient 

with clinical features of a Horner syndrome and a right-sided 

thoracic tumor in the apex of the chest, likely arising from the 

ipsilateral stellate ganglion.


A 

Figure 96-4. Magnetic resonance image (axial) of a large rightthoracic 

neuroblastoma with extension into the spinal canal and 

compression of the cord. This patient presented with bilateral 

lower-extremity weakness. 

The diagnosis and treatment of these tumors is complex and is 

actively altered per international, multicenter clinical trials 

conducted by the Children’s Oncology Group. Prognosis is also 

complex and is based on the stage of the disease, the age of the 

patient (age

intrapericardial GCT and cardiac GCT. Intrapericardial GCT 

occurs most commonly in infants who are less than 12 months old 

and present with findings of cardiac tamponade and congestive 

heart failure; as most are benign, they are treated with primary 

resection. They can have a very complicated intraoperative course 

because they often compress the adjacent atria and ascending aorta. 

Cardiac GCTs, which are malignant in 25% of the cases, present 

with congestive heart failure and/or arrhythmias (as intraventricular 

block) and are most commonly located in the right side 

of the heart. They are associated with congenital heart disease 

(atrial and ventricular septal defects). Although primary surgical 

resection is indicated to avert complete cardiac outflow 

obstruction, there are times when the GCT is unresectable, making 

cardiac transplantation an option. 24 


Lymphangioma 

Although these lesions are also referred to as cystic hygromas, the 

more current term is lymphangioma. They have an incidence of 

1 in 12,000 births and approximately 50 to 65% of them are present 

at birth; the vast majority becomes clinically evident before 2 years 

of life. 25 They develop as a result of the failure of primitive 

lymphatic buds to form communications with adjacent developing 

veins; these buds dilate into lymphatic cysts which subsequently 

become lymphangiomas. Anatomically, they are most commonly 

found in the neck (75%) and less commonly in the mediastinum 

(


A 

B 

C 

Figure 96-8. A and B: Intraoperative series demonstrates a posterior approach to resection of a superior, left-sided Ewing sarcoma 

of the chest wall. The muscles on the medial side of the scapula are divided and it is reflected laterally and superiorly. After the ribs 

are divided, that portion of the lung that is adherent to the chest wall tumor is divided with a stapler to deliver the chest wall, tumor, 

and lung en bloc. The remaining chest wall defect will be closed with Marlex mesh. 

D 

Ewing sarcoma; the muscles on the medially side of the scapula 

are divided and it is reflected laterally. 

Intraoperative considerations include one-lung ventilation and 

epidural catheter placement. The former allows confirmation of 

which ribs must be removed and facilitates the resection of 

contiguous structures as the adjacent lung which may or may not 

be adherent to the tumor; the latter provides good postoperative 

analgesia. With this careful planning and approach, large areas of 

chest wall can be safely resected in sections up to six ribs (and 

seven intercostal spaces) in height. 27 Figure 96–9 shows a large 

Ewing sarcoma on the left chest wall after resection. 

Pulmonary Neoplasms 

Primary neoplasms of the lung in children, including bronchogenic 

carcinoma, bronchial carcinoid tumor, and pleuropulmonary 

blastoma, are extremely rare. 28 Metastatic disease to 

the lung is more common, and this entity will be briefly reviewed 

in this section. Many neoplasms may develop metastatic disease, 

including those listed in Table 96–3. Osteogenic sarcoma, alveolar 

soft part sarcoma, and adrenocortical carcinoma are tumors which 

can result in pulmonary metastases and are associated with a more 

favorable clinical outcome with pulmonary metastasectomy; the 

other six tumors listed have unclear or no impact on clinical 

outcome. 29 When preoperatively assessing candidates for 

pulmonary metastasectomy, it is important to consider the 

number of lesions, the size of the lesions, whether the lesions are 

unilateral or bilateral, the proximity of the lesions to major bronchi 

and main pulmonary vessels, and the anticipated amount of lung 

that will need to be resected to obtain clear surgical margins (and 

the corollary: how much lung will remain). In addition, it is 

important to recognize and plan for resecting more pulmonary 

lesions than are identified on CT scan; this is particularly relevant 

with metastatic disease from osteogenic sarcoma, where one study 

has reported that more than half of the lesions that are ≤5 mm 

will be missed on CT scan. 8 Figure 96–10 demonstrates a very 

small right-sided lung lesion that is typical of metastatic 

osteogenic sarcoma.


Figure 96-9. A large Ewing sarcoma on the left chest wall after 

resection. The ribs are oriented anterior to the mass as though 

the tumor were still in vivo. 

The surgical team must consider the surgical approach: (1) 

bilateral posteriolateral thoracotomies give optimal access to the 

lung in each hemithorax, but such approaches may be limited 

if a relatively large amount of lung parenchyma will need to be 

resected (in addition, such approaches make postoperative pain 

difficult); (2) median sternotomy gives access to both hemithoraces 

simultaneously and are associated with less postoperative 

pain, but it is difficult to assess and resect pulmonary lesions 

in the posterior portions of the lung with this approach; and (3) 

staged unilateral posteriolateral thoracotomies allow for optimal 

access to the lung in each hemithorax, are better if it is anticipated 

that a large amount of lung will need to be resected, but do 

require a second anesthetic. Usually, a 2-week hiatus between 

procedures allows the ipsilateral lung to recover from postoperative 

hemorrhage and edema before resection of parts of 

the contralateral lung. It is this author’s preference to optimize 

surgical exposure and resectability of these pulmonary lesions 

by using the latter approach for bilateral lung lesions. Preoperative 

pulmonary function tests (PFT) are required to assess changes 

in respiratory function between thoracotomies and may predict 

the need for postoperative critical care bed needs; in general, PFTs 

are not effective in predicting whether a patient will tolerate 

one-lung ventilation or not. Intraoperative considerations 

mandate that one-lung ventilation be established to allow optimal 

palpation of the entire lung and resect all metastatic lesions; 

TABLE 96-3. Types of Neoplasms Linked to Pulmonary 

Metastatic Disease, by Indication for Surgical Metastasectomy 

Indicated Controversial Generally Not Indicated 

(increases (may increase (likely has no impact 

survival) survival) on survival) 

Osteogenic Wilm Tumor Neuroblastoma 

sarcoma 

Alveolar soft Ewing sarcoma Rhabdomyosarcoma 

part sarcoma 

Adrenocortical Hepatoblastoma Differentiated 

carcinoma 

thyroid cancer 

Figure 96-10. Computed tomography scan (axial) of a very 

small right-sided lung lesion (arrow) typical of metastatic 

osteogenic sarcoma. 

classically, the lesions associated with osteogenic sarcoma can be 

very small (


can accommodate the repair, which includes closure of the 

sternal defect, repair of the diaphragmatic hernia, and closure of 

the omphalocele. 30 

Pectus Excavatum 

Pectus excavatum accounts for approximately 90% of chest wall 

defects, with an incidence of 1 in 300 live births. The etiology is 

possibly related to the overgrowth of cartilage in the adjacent ribs, 

displacing the sternum posteriorly. Clinically, it presents more 

commonly in boys at a ratio of 3:1, becoming more progressive in 

posterior displacement as the child enters puberty. It is associated 

with Marfan’s syndrome (2% of cases), diaphragmatic abnormalities 

(2% of cases) and congenital heart disease (1.5%). 31 

Patients may present asymptomatically or may have complaints of 

chest pain, palpitations, and asthma-like symptoms. There 

continues to be considerable debate about the clinical impact of 

this defect upon cardiovascular and pulmonary function. 

Intuitively, the compression of the heart and the lungs by the 

sternum should result in dysfunction. Lower lung volumes and 

decreased cardiac output have been recorded in patients with 

severe defects during exercise; 32–33 improvements in function have 

been demonstrated in patients postoperatively. Indications for 

surgical repair are more commonly psychological, related to issues 

around poor self-image, and less commonly related to issues of 

physiologic compromise of clinically relevant cardiopulmonary 

dysfunction; the latter may be important for a patient with a severe 

defect who also happens to be a competitive athlete. 

The occasional child with a concurrent diagnosis of Marfan’s 

syndrome or congenital heart disease should be considered for 

repair of the chest wall defect before open heart surgery to avert 

the issues of compression of the cardiac structures. The surgical 

options are essentially two: the Ravitch procedure (an open 

surgical approach) and the Nuss procedure (a minimally invasive 

approach). The former technique has been used for more than 50 

years and has undergone minor revisions over time. Figure 

96–11AB shows a patient with pectus excavatum before and after 

a Ravitch repair. Intraoperative and postoperative considerations 

include pneumothorax, hemorrhage, and pain management. In 

long-term follow-up, patients will experience minor recurrences of 

the defect in 10 to 15% of cases and major recurrences in 10 to 

15% of cases for an overall recurrence rate of 20 to 30%. 34–35 

Because of an infrequent but devastating long-term complication 

of thoracic dystrophy, children should be at least 6 years of age 

before undergoing surgical correction of this defect; some authors 

now recommend delaying repair until 12 to 16 years of age to 

reduce the chance of this complication. 36 

The Nuss procedure has become the de facto procedure for the 

correction of pectus excavatum in the United States. The attraction 

of this technique is its cosmetically more acceptable outcome, 

because it eliminates the transverse chest incision of the Ravitch 

procedure and replaces it with two small lateral chest wall 

incisions; it functions on the premise that the chest wall can be 

remodeled over a nondeformable metal bar that forcibly pushes 

the sternum and ribs from the spine, increasing the anteriorposterior 

distance; the bar is usually left in place for 2 to 3 years to 

reduce the chance of recurrence of the defect. The intraoperative 

and postoperative considerations include pain management, 

pneumothorax, and metal bar displacement. More serious 

complications have included cardiac perforation, empyema, 

pericarditis, and the development of thoracic outlet syndrome. 

The overall complication rate is 20 to 28%, which is higher than for 

the Ravitch procedure (


A 

Figure 96-12. A and B: This series demonstrates a patient with pectus carinatum before and after a Ravitch repair (looking from the 

side of the patient). 

B 

defects may result in changes in pulmonary function, which 

should be assessed with a complete set of PFTs. 

The classical surgical treatment is similar to the approach for 

pectus excavatum with a Ravitch repair. This treatment, which 

usually yields a very good cosmetic result, is associated with a 

transverse scar across the low3er portion of the chest; figure 96– 

12AB shows a patient with pectus carinatum before and after a 

Ravitch repair. As such, some authors have advocated the use of 

orthotic compression bracing of the chest wall to induce 

remodeling of the underlying ribs and sternum. 42–43 One of these 

studies found that the bracing led to positive outcomes, provided 

that the patients were compliant with wearing their compression 

vests. The difficulty with compliance was the requirement to wear 

the compression vest for 14 to 16 hours a day for 24 months. 44 The 

role of surgical intervention in this chest wall abnormality will 

likely decrease over time as the nonoperative options of bracing 

becomes more accepted and will only be reserved for children and 

adolescents who are noncompliant with the bracing regimen. 

DIAPHRAGMATIC ANOMALIES 

Among diaphragmatic anomalies, the most common are 

Bochdalek (or posteriolateral) congenital diaphragmatic hernias, 

at an incidence of 1 in 4000-5000 live births/year. This specific 

topic will be reviewed and discussed in Chapter 85. Other 

anomalies of the diaphragm, including Morgagni diaphragmatic 

hernias and diaphragmatic eventrations, will be discussed below. 

All of these hernias develop as a result of an abnormality of 

embryological development. The diaphragm, whereby the 

pleuroperitoneal cavity is separated into two distinct cavities, 

usually forms between 4 and 8 weeks of gestation. The diaphragm 

is normally composed of a central tendon and a peripheral 

muscular section; the former arises from the transverse septum 

and the latter arises from the posterolateral pleuroperitoneal folds. 

Some diaphragmatic anomalies form as a result of failure of fusion 

of the septum and folds; others arise from defects in formation of 

the diaphragmatic muscle itself. Morgagni diaphragmatic hernias 

develop as a failure of fusion in which the anterior diaphragm 

muscle attaches to the sternum. 

Morgagni Diaphragmatic Hernia 

Morgagni diaphragmatic hernias account for approximately 2% 

of all congenital diaphragmatic hernias. They occur in the anterior 

midline directly posterior to the sternum or in the parasternal 

location through the foramen of Morgagni. Unlike the classical 

Bochdalek congenital diaphragmatic hernia, these hernias usually 

present with features of intestinal obstruction instead of 

respiratory compromise. Usually, children are older when they 

become symptomatic, or defects are discovered incidentally on 

chest imaging. The hernia sac often contains a portion of the liver 

and transverse colon; occasionally it can also contain the small 

bowel and stomach, depending on size. These diaphragmatic 

defects can be surgically treated with open and laparoscopic 

techniques, including either primary closure of the defect or 

placement of a synthetic patch. The MAS approach has become 

the operation of choice for patients, who are not in any respiratory 

distress and can tolerate peritoneal CO 2 

insufflation. Surgical 

repair involves a laparoscopic approach with either primary 

closure of the defect or placement of a synthetic patch. Video 

2 demonstrates a laparoscopic view of a moderately-sized 

Morgagni hernia. Various authors have successfully applied this 

technique 45–46 ; in addition to primary closure of these defects, 

some of the authors were able to repair them with prosthetic 

patches with only one recurrence in long-term follow-up. 

Diaphragmatic Eventration 

Diaphragmatic eventration, which usually presents with unilateral 

elevation of a hemidiaphragm, can present as congenital or 

acquired disease. The former is a result of a defect in adequate 

muscularization of the diaphragm; in this case, the diaphragm is 

abnormally thin, often lacking muscle and being membranous in 

quality. 47 The latter is usually a result of iatrogenic injury of the 

phrenic nerve from surgery or from direct invasion of the phrenic 

nerve by a neoplasm; in this case, the diaphragmatic muscle is 

normal in quality. A portion of or all of the hemidiaphragm may 

be involved. The diagnosis can be made on history, especially in 

the case of acquired disease. Although most children with


congenitally-acquired disease may be asymptomatic, patients with 

acquired disease may be symptomatic from a respiratory perspective, 

owning to the acute change in diaphragmatic function. 

The diagnosis, regardless of etiology, can be assessed with 

diagnostic imaging tests, including ultrasound and fluoroscopy of 

the diaphragm, looking for paradoxical motion of the diaphragm. 

In the event that the diagnosis continues to be unclear, an upper 

gastrointestinal series can be obtained. Once a diagnosis is 

secured, the option of surgical repair is generally considered for all 

patients who are symptomatic and those patients who are 

asymptomatic but also have a markedly elevated hemidiaphragm 

that is compressing the ipsilateral lung. The surgical procedure 

involves plication of involved diaphragm; infrequently, a 

redundant portion of the diaphragm will have to be excised. The 

approach can be via abdominal or thoracic approach and can be 

via an open or thoracoscopic/laparoscopic technique. The latter 

technique has been slowly gaining favor among surgeons. A 

number of case reports and small case series have been published 

that advocate for the use of the laparoscopic/thoracoscopic 

techniques. 48 A study by Sato et al. noted that all of the cases were 

successfully completed with the thoracoscopic technique and there 

were no recurrences in long term follow-up. 

ANOMALIES OF THE 

LUNG AND AIRWAY 

Anomalies of the lung and airway are an area of pediatric surgery 

that is undergoing changes with respect to optimal treatment and 

management. Some authors have suggested that not all of these 

lesions need to be surgically excised and some can be safely 

monitored. 49 Aziz et al. have suggested that asymptomatic 

congenital cystic adenomatoid malformations can be observed. 

Other authors, such as Azizkhan et al., have suggested that all of 

these asymptomatic lesions be surgically excised. 50 This remains a 

very controversial area and there remains considerable disagreement 

on this topic. This section will focus on the current 

treatment options for these largely congenital anomalies and will 

mention areas of controversy where it alters clinical management; 

included in this section is a discussion of spontaneous pneumothorax, 

which may be congenital or acquired in origin. 

Congenital Pulmonary 

Airway Malformation 

These lesions are referred to as congenital cystic adenomatoid 

malformations (CCAMs) or more recently as congenital 

pulmonary airway malformations (CPAMs). They can be 

macrocystic, macrocytic, or solid masses which may involve all or 

a portion of the lobe of the lung; they communicate with the 

airway and usually do not have anomalous blood vessels. There 

are a number of histologic subtypes, which embryologically 

contain no cartilage and arise from the excessive proliferation of 

bronchial structures without alveoli. 51 These lesions are now 

diagnosed in three clinical settings: (1) on antenatal ultrasound 

examination, (2) when the child presents with symptoms related 

to respiratory distress or pulmonary infection (pneumonia and/ 

or intrapulmonary abscess), or (3) when the lung anomaly is 

discovered incidentally on diagnostic chest imaging. The 

former examinations have demonstrated that these lesions can 

have dramatically distinct clinical courses, including complete 

resolution of the lesion before birth, little change in the size of the 

lesion, progressive enlargement of the lesion, and in utero demise 

related to compression of the heart with impairment of central 

venous return, ultimately leading to hydrops. 

The primary preoperative indications for surgical resection 

of these lesions are: (1) respiratory distress from direct compression 

of adjacent intrathoracic structures, (2) prevention of 

pulmonary infection (or recurrent pulmonary infection), and (3) 

prevention of possible pulmonary malignancy. The latter 

indication is one of the most controversial, because it is unclear 

whether these malignancies have arisen from a benign congenital 

lung lesion or whether the diagnosis of the benign congenital lung 

lesion has been in error and the lesion is, in fact, a malignancy. 

These lesions are optimally assessed with CT scan imaging, 

although ultrasound can be used for lesions that are fluid-filled or 

solid and are adjacent to the chest wall. Figure 96–13 illustrates a 

CPAM in the right chest that is primarily cystic with a small solid 

component. The surgical approach usually involves complete 

resection of the congenital lesion including the surrounding lobe 

of the lung via an open or thoracoscopic technique. The latter 

technique has been advanced with devices that effectively seal 

large vessels and can also go through a 5-mm port; the stapling 

devices that are now available are too long for most hemithoraces 

in small children and require a 12-mm port. In addition, it 

has become common for pediatric surgeons to use low-pressure 

intrathoracic insufflation (3–7 mmHg) to safely compress 

the ipsilateral lung, instead of one-lung ventilation. which is 

prone to technical difficulty given the small airways of children. 

Various authors have reported low conversion rates to open 

procedures and overall good success rates with the approach. 

Albanese et al. have noted maximal conversion rates of 2%, few 

other intraoperative complications, and lower mean hospital 

lengths of stay. 52 

Figure 96-13. Computed tomography scan (axial) of a congenital 

pulmonary airway malformation in the right chest, which is 

primarily cystic with a small solid component.


Pulmonary Sequestration 

These lesions are also referred to as bronchopulmonary 

sequestrations (BPSs). They classically have anomalous arterial 

and pulmonary or systemic venous blood supply and do not 

formally communicate with the normal tracheobronchial tree. The 

former arterial vessels which arise from the thoracic (85–90%) or 

abdominal aorta (10–15%) can be very large, resulting in a large 

arteriovenous shunt and possible high-output cardiac failure; 

moreover, these vessels, if not properly ligated and divided at the 

time of surgical resection, can result in major intraoperative 

hemorrhage and can occasionally retract into the subdiaphragmatic 

space (if they originate from the abdominal aorta). 

There are two types of BPS, intralobar BPS and extralobar BPS. 

They differ based on their location within the lung parenchyma; 

intralobar BPS, which is more common (making up 90% of BPS), 

lies within the parenchyma of the lobe and indirectly communicates 

with the adjacent normal lung parenchyma via the 

pores of Kohn; these pores create an opportunity for infection of 

the BPS. Extralobar BPS is separate from the adjacent lung 

parenchyma and is invested with its own visceral pleura but does 

similarly have systemic arterial and pulmonary or systemic blood 

supplies. As such, it rarely becomes infected but is at risk for 

creating an arteriovenous shunt, causing high-output cardiac 

failure. Unlike intralobar BPS, extralobar BPS is associated with 

congenital diaphragmatic hernias and can be anatomically located 

in the thorax (more commonly on the left side) or the subdiaphragmatic 

space. 

BPS lesions can be imaged with transthoracic ultrasound, chest 

CT scan, or MRI. These lesions tend to occur more in the lower 

portion of the chest; they are differentiated from other congenital 

lesions by identification of a lung mass with an adjacent large 

arterial vessel which can be followed to the aorta. Preoperative 

indications for surgical resection of intralobar and extralobar BPS 

include recurrent pulmonary infection, congestive heart failure, 

or resection for diagnosis of a paraspinal mass (to differentiate if 

from a neoplasm as a neurogenic tumor). As with CCAM/CPAM, 

they can be resected via open or thoracoscopic techniques and 

authors have noted equal success with resection of these lesions 

with minimal access techniques. 52 Intraoperative considerations 

include the rare risk of major hemorrhage if the arterial blood 

vessel if not properly ligated; moreover, approximately 15% of BAS 

lesions will have more than one arterial blood vessel which can be 

inadvertently missed leading to unanticipated blood loss. 51 

Congenital Lobar Emphysema 

Congenital lobar emphysema (CLE) has multiple etiologies that 

lead to overdistention (air trapping) of the lobe of lung, resulting 

in compression of adjacent normal lung parenchyma. Some have 

implicated the embryologic absence of cartilage in the involved 

bronchus, leading to a ball-valve effect of progressive over inflation 

of the lung tissue, and others have implicated the polyalveolar lobe 

syndrome, which involves the development of abnormally high 

numbers of alveoli, leading to gigantic alveolar units. 51 Clinically, 

these children present with respiratory symptoms such as 

tachypnea; some of these symptoms will resolve with time averting 

the need for surgery. Optimal diagnostic imaging is achieved with 

a chest CT scan; the upper and middle lobes are most commonly 

involved. Figure 96–14 illustrates a child with left-sided CLE on 

CT scan. Bronchoscopy can also be used to identify abnormal areas 

Figure 96-14. Computed tomography scan (axial) of a child 

with left-sided, posterior congenital lobar emphysema with 

compression of adjacent normal lung parenchyma. 

of absent cartilage in the tracheobronchial tree. Intraoperative 

complications can occur at the time of induction of anesthesia, 

because positive pressure ventilation can cause acute over 

distention of the lobe involved with CLE. Strong consideration 

should be given to an anesthetic based on spontaneous ventilation 

and the surgeon should be in the operating room at the time of 

induction in case an emergent thoracotomy is required to relieve 

compression of adjacent intrathoracic structures. 

Bronchogenic Cysts 

Bronchogenic cysts are noncommunicating cystic structures 

adjacent to the tracheobronchial tree which usually exist at the 

level of the carina or main stem bronchi. They lack normal 

alveolar structures but do contain a ciliated columnar epithelial 

lining that produces mucus; as the cyst collects mucus, it 

extrinsically compressed the adjacent normal bronchial structures. 

Although this compression can cause air trapping, resulting in a 

clinical picture of CLE, many of these cysts are asymptomatic at 

diagnosis and are only noted incidentally on diagnostic chest 

imaging. Preoperative indications include surgical excision of the 

cysts to address respiratory compromise, to prevent infection of 

the cyst, to prevent the unusual occurrence of acute hemorrhage 

into the cyst causing acute extrinsic compression of the trachea, 

and to prevent the rare case of adenocarcinoma. These patients 

are often ideal candidates for a thoracoscopic approach for 

resection of the cyst, provided that the ipsilateral lung is not 

over inflated. Many authors have demonstrated the benefit of 

thoracoscopic resection over open thoracotomy. 53–55 The 

complication of injuring the adjacent airway at the time of 

resection is now lessened as it is recognized that the entire cyst 

wall need not be resected to achieve a low recurrence rate, if the 

remaining mucosal lining is cauterized.


Spontaneous Pneumothorax 

Spontaneous pneumothoraces arise from the rupture of apical 

pulmonary blebs in lung parenchyma without underlying disease. 

The incidence of this disease is higher in boys with an incidence 

of 7 to 18/100,000 in boys and 1 to 6/100,000 in girls. 56 Classically, 

the patient is a thin, tall teenage boy who presents with unilateral 

chest pain and cough. Although some patients may be more 

symptomatic related to significant pulmonary collapse and/or 

tension pneumothorax, most are clinically very stable. The 

diagnosis is confirmed with a chest radiograph. Although small, 

nonincreasing pneumothoraces many resolve with the application 

of increase O 2 

via nasal prongs or face mask, larger or increasing 

pneumothoraces will require placement of a chest tube. As part of 

the assessment, a chest CT scan should be obtained to determine 

the quality and quantity of apical blebs. 

Indications for surgical intervention are: (1) persistent air leak 

after 5 to 7 days of chest tube suction, (2) recurrence of the 

pneumothorax, and (3) the presence of bilateral or large blebs. 

The recurrence rate for spontaneous pneumothorax after the 

first spontaneous pneumothorax is highest for teenagers and 

approaches 60%. 56 There are three approaches for surgical 

treatment: (1) open thoracotomy, (2) transaxillary minithoracotomy, 

and (3) thoracoscopy. All involve resection of the 

apical lung tissue containing the blebs and pleurodesis of the apex 

of the parietal pleura by means of mechanical abrasion, application 

of a chemical sclerosant, or apical parietal pleurectomy. The trend 

in surgical management has been towards thoracoscopy and away 

from transaxillary minithoracotomy. 57,58 One retrospective study 

by Butterworth et al. demonstrated a lower postoperative narcotic 

requirement, shorter length of hospital stay, and no difference in 

recurrence rates for thoracoscopy compared to transaxillary 

minithoracotomy. 59 In a large series by Rogers et al., the thoracoscopic 

technique had no complications and a low recurrence 

rate of 7.4%. 60 

EMPYEMA 

This disease refers to the accumulation of infected, purulent fluid 

in the pleural space. It is often secondary to a bacterial pneumonia, 

may occur after thoracic surgery, may be a complication of 

trauma, or may be related to other underlying pulmonary diseases. 

Empyemas go through three distinct stages; they include (in 

order): exudative, fibropurulent, and organizing stages. 61 

Management of this disease has undergone multiple iterations; 

indications for surgical intervention, as opposed to conservative 

management with chest tube drainage, antibiotics, and instillation 

of fibrinolytics, have changed over time and have been assessed in 

various studies. In the past, the primary surgical approach 

involved a classical posteriolateral thoracotomy followed by 

removal of the peel of the organized parapneumonic collection; 

this procedure was associated with significant intraoperative and 

postoperative hemorrhage. When this approach was further 

modified to a limited, muscle-sparing thoracotomy, it was better 

tolerated by patients. It was advocated that it should be used at an 

earlier time in the treatment of the empyema and this approach 

was found to decrease the overall morbidity when compared to 

conservative management strategies. 62 

Subsequently, a thoracoscopic debridement approach was 

suggested as a less invasive means of achieving a better clinical 

outcome with less morbidity. It has become a first-line therapy for 

the management of empyema and is especially efficacious in the 

exudative-fibropurulent stages of the disease. 63 The evidence 

behind this change includes a study which demonstrated that 

children who received thoracoscopic debridement early in the 

management of their empyema had no failures, as defined by a 

need for open thoracotomy, and had a shorter hospital length of 

stay; historical controls who were treated with chest tube drainage 

alone had a longer hospital length of stay; some required a delayed 

thoracoscopic debridement and a few ultimately required an open 

thoracotomy. 64 Other authors have advocated primary use of 

thoracoscopic debridement, because it was associated with lower 

overall hospital costs, when it was compared to patients who 

received conservative chest tube drainage, some of whom would 

fail, requiring a delayed thoracoscopic debridement procedure; 

the costs of the primary operations were off set by the longer 

hospital length of stays in the conservatively managed patients. 65 

Despite a relatively large amount of data supporting early 

thoracoscopic debridement, the application of this clinical 

approach remains very controversial and has not been well 

accepted by all clinicians; practices very greatly between and 

within institutions. 

TRAUMATIC THORACIC INJURIES 

Thoracic injuries are the second most common cause of mortality 

in children, with a rate of 15 to 22% among hospitalized patients. 66 

When combined with other concurrent injuries, the mortality rate 

increases to as high as 40%, 43%, and 50% for cardiac, esophageal, 

and vascular injuries, respectively (U.S. National Trauma Registry 

data, 1985–1991). Blunt traumatic injuries outnumber penetrating 

injuries by a factor of greater than 4:1. Approximately 75% of blunt 

injuries are a result of motor vehicular collision, with the child as 

an occupant of the vehicle or as a pedestrian who is struck by a 

vehicle. Specific differences in the anatomy of the child allow for 

unique physiologic responses to injuries of the thorax compared 

to adults. These differences include a smaller airway that is more 

susceptible to obstruction, a larynx that is tilted in a cephalad 

direction, making inadvertent esophageal intubation more 

common, and a short trachea, making right bronchial intubations 

more possible. In addition, the chest wall is more compliant, 

allowing for less protection of the intrathoracic organs, and the 

mediastinum is more mobile, allowing for a higher chance of a 

tension pneumothorax developing with the right clinical 

TABLE 96-4. Injuries to the Thorax, by Likelihood of 

Resulting Death 

Life-Threatening Potentially Life- Serious 

Injuries Threatening Injuries Injuries 

Airway injuries Major pulmonary Pneumothorax 

contusion 

Tension Tracheobronchial Traumatic 

pneumothorax injuries asphyxia 

Massive hemothorax Myocardial contusion Hemothorax 

Cardiac tamponade Diaphragmatic rupture 

Flail chest 

Esophageal rupture 

Open pneumothorax Aortic rupture


TABLE 96-5. Various Thoracic Injuries, by Indication 

for Surgical Intervention 

Emergent or Urgent 

Thoracotomy 

Massive, persistent pneumothorax 

(related to tracheobronchial 

injury) 

Massive hemothorax 

Cardiac tamponade 

Open pneumothorax 

Esophageal rupture 

Vascular injury 

Rupture of the diaphragm a 

Delayed Thoracotomy 

Chronic rupture of the 

diaphragm 

Chylothorax 

Chronic hemothorax 

Removal of foreign bodies 

a 

In the setting of a rupture of the diaphragm, it is important to recognize that the 

risk of a concurrent intra-abdominal injury is high enough to warrant repair of 

the diaphragm via a laparotomy so that other intra-abdominal injuries can be 

simultaneously assessed. 

conditions. It should be noted that a child with rib fractures 

signifies that a major amount of force has been applied to the 

chest, and there needs to be a high index of suspicion of 

concurrent injury to the lung, heart, and other mediastinal 

structures. 

Thoracic injuries can be categorized by the degree to which 

they threaten the child’s life (Table 96–4). Indications for surgical 

intervention with these injuries can be divided into those that 

require emergent or urgent surgery and those which can be 

performed in a more delayed fashion (Table 96–5). 

Clinical findings, as indicated by the setting in which the 

thoracic injuries have occurred and the observations in the 

trauma room and/or critical care unit, dictate the need for 

surgery. Survival with cardiac tamponade is highest in children 

who have vital signs before arrival in the emergency department 

and have single penetrating wounds (stab wounds and lowvelocity 

gunshot wounds) and lowest in children who have absent 

vital signs in the prehospital setting and have sustained highvelocity 

gun shot wounds or blunt trauma. The latter patients have 

an overall survival that approached zero. 66 Other clinical findings 

for surgery can be determined by the rate of blood loss as 

measured via a chest tube. The current indication for a thora - 

cotomy in adults is approximately 1500 mL/24 hours acutely. 

Similarly, children whose blood loss approaches 25 to 30% of their 

blood volume (20–25 mL/kg/24 h) should undergo urgent 

thoracotomy to avoid the high mortality associated with ongoing 

massive blood loss and required transfusion. Usually, most 

children requiring chest surgery would have it via an open 

thoracotomy. The role of thoracoscopic surgery has been 

recognized as an important adjunct in the management of these 

injuries and it is slowly finding a formal position under specific 

clinical conditions. 67 In the setting of a patient with stable vital 

signs, some authors have reported success rates as high as 

89% with the evacuation of residual empyema and hemothoraces 

after thoracic injury. 68 Other authors have found thoracoscopy 

to be effective in the diagnosis of diaphragmatic injuries (accurate 

to 98%) and the management and control of chest tube 

hemorrhage (in 82% of the patients where it was used). The use 

of the thoracoscopy averted a need for thoracotomy in 62% of 

the cases. 69 

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Chapter 96

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