Pediatric Thoracic Surgery (eBook)

Mr Dakshesh H Parikh is Consultant Paediatric Surgeon and Lead Clinician in the Trauma Service, Paediatric Thoracic Service and The Minimal Access Surgery – Laparoscopy/ Thoracoscopy Service at The Birmingham Children’s Hospital NHS Trust, UK.

Pediatric Thoracic Surgery 2
Title Page 3
Copyright Page 4
Foreword 5
Preface 6
Contents 7
Contributors 11
Part I: Fundamental Considerations 15
Chapter 1 16
Applied Anatomy 16
Introduction 16
Historical perspective 16
Thoracic skeleton 16
Sternum 16
Ribs 16
Surgical anatomy 16
Cervical ribs 17
Clavicle 17
Thoracic vertebrae 17
Thoracic wall 17
Intercostal space 17
Muscles and fasciae of the chest wall 19
Suprapleural membrane (Sibson’s fascia) 19
Endothoracic fascia 19
The diaphragm 19
The openings of the diaphragm 20
Surgical incisions in the diaphragm 20
The mediastinum 21
Superior mediastinum 21
Trachea 21
Surgical anatomy 21
Phrenic nerve 22
Vagus nerve 22
Thymus glan 22
Middle mediastinum 23
Left pulmonary hilum (Figs. 1.3, 1.5, 1.6) 23
Right pulmonary hilum (Figs. 1.4 – 1.6) 23
Pericardium 25
Posterior mediastinum 25
Descending aorta 25
Esophagus 25
Lymphatics of the thorax 26
Thoracic duct 26
Azygos veins 27
Thoracic sympathetic chain 27
Pleura 28
The lungs27 28
Right upper lobe 28
Right middle lobe 29
Right lower lobe 29
Left upper lobe 29
Left Lower Lobe 30
Further Reading 30
References 30
Chapter 2 31
Developmental Biology and Embryology of the Thorax 31
Introduction 31
Developmental Biology 31
Evolution 32
Overlapping Hox Genes Specify the Different Thoracic Vertebrae and Ribs 32
The Foregut 32
The Origins of the Foregut Endoderm 32
Products of the Foregut Endoderm 33
The Airways and Blood Vessels 33
Foregut Septation 33
The Laryngotracheal Groove 33
After Normal Separation 34
Septation Failure as a Cause of EA/TEF 34
Lung Development 34
Histological Phases of Lung Development 34
Genes Involved in Foregut and Lung Development 34
The Diaphragm 35
Animal Models of CDH 35
Genetic Associations 35
Left-Right Asymmetry in the Chest 36
Surfactant Deficiency 36
Cystic Fibrosis 36
References 37
Further Reading 37
Chapter 3 38
Applied Physiology and Pulmonary Function Testing 38
Introduction 38
Measurements of Respiratory Function 38
Measurements of Spirometry 38
Equipment 38
Procedure 39
Indices Derived from Spirometry 39
Interpretation 39
Application 41
Measurements of Lung Volumes 41
Equipment and Procedure for Plethysmographic Measurement of Lung Volume 41
Equipment and Procedure for Lung Volume Measurement by Gas Dilution 42
Indices 43
Interpretation 43
Application 44
Measurements of Ventilatory Efficiency 44
Equipment 44
Procedure 44
Indices 44
Interpretation and Application 44
Maximum Respiratory Pressures 44
Equipment and Procedure for Measuring Maximal Respiratory Pressures at the Mouth 45
Equipment and Procedure for Measuring Sniff Pressure 46
Indices 46
Interpretation 46
Application 46
Contribution of Respiratory Function Tests 46
Conclusions 48
References 48
Chapter 4 51
Radiology of the Chest 51
Introduction 51
Requesting Imaging 51
Care of the Patient 51
Risks Associated with Ionizing Radiation 51
Use of Contrast Media 51
Imaging Modalities 52
Radiography 52
Fluoroscopy 52
Ultrasound Imaging 52
Computed Tomography 53
Magnetic Resonance Imaging 54
Radionuclide Imaging 55
Interpretation of the Pediatric Chest X-Ray 55
Preliminary Steps 55
Pattern Recognition 56
Trachea, Main Bronchi, and Hilar Regions 56
The Superior Mediastinum 57
The Heart and Great Vessels 58
Lungs/Pleural Cavities 58
Pleural Fluid 59
Tumor 60
Increased Translucency 60
Air Leaks 61
Pulmonary Masses and Nodules 62
Ring Shadows 62
Diaphragms 63
Thoracic Skeleton and Soft Tissues 63
References 65
Chapter 5 67
Anesthesia for Thoracic Surgery 67
Introduction 67
Historical Perspective 67
Anatomical and Physiological Considerations in the Neonate 68
Physiology of Single-Lung Ventilation 69
Lateral decubitus Position: Adults 69
Lateral decubitus position: Children 70
Investigations 70
Chest Roentgenogram 70
Computed Tomography 70
Magnetic Resonance Imaging 71
Thoracic Sonography 71
Bronchoscopy 71
Bronchography 71
Echocardiography 71
Angiography 71
Pulmonary Function Tests 71
Management of Anesthesia 72
Monitoring 72
Single-Lung Ventilation 72
Postoperative Analgesia 74
Specific Procedures 75
Rigid Bronchoscopy 75
Flexible Fiber-Optic Bronchoscopy 76
Conditions in Neonates and Infants 76
Tracheoesophageal Fistula 76
Congenital Diaphragmatic Hernia 77
Subglottic Stenosis 77
Pulmonary Sequestration 77
Congenital Cystic Lung Lesions 77
Congenital Lobar Emphysema 77
Infection 77
Anterior Mediastinal Mass 78
Anesthetic Considerations 78
Thoracic Trauma 79
Anesthetic Considerations 79
Thymectomy 80
Anesthetic Considerations 80
Nuss Procedure 81
References 81
Chapter 6 85
Thoracoscopic Surgery 85
Introduction 85
Indications 85
Preoperative Workup 85
Anesthetic Considerations 86
Positioning 87
Trocar Placement 88
Instrumentation 88
Postoperative Care 89
Conclusions 89
References 89
Chapter 7 91
Thoracic Incisions and Operative Approaches 91
Introduction 91
Historical Perspective 91
General Considerations 91
Thoracic Incisions 92
Posterolateral Thoracotomy 92
Limited (Mini) Thoracotomy: Muscle-Sparing Incisions 93
Axillary Thoracotomy 94
Median Sternotomy 94
Thoracoabdominal Incisions 95
Intercostal Chest Tube Insertion and Management 95
Chest Drain: Ward Management 97
General Techniques for Pulmonary Resection 97
Dissection and Control of Major Arteries and Veins 98
Dissection and Control of Major Bronchi 98
Raw Surface of the Lung 98
Lobectomy 98
Specific Technical Considerations 99
Segmental Resection 101
Pneumonectomy 101
Complications 101
Intraoperative Complications and Their Management 101
Postoperative Complications 102
References 102
Part II: Infection 103
Chapter 8 104
Pneumonia 104
Introduction 104
Historical Perspective 104
Basic Science and Pathogenesis 104
Etiology 104
Epidemiology 104
Clinical Features 104
Neonatal Period 104
Older Children 105
Pneumonia Presenting with Acute Abdominal Pain 105
Immunocompromised Children 106
Children with Sickle Cell Disease 107
Postoperative Children 107
Unusual Organisms: Actinomycoses, PVL producing MRSA 108
Children with Neurodisability 108
Children with Tracheostomies 108
Diagnosis 109
Pulse Oximetry 109
Acute Phase Reactants 109
Blood Cultures 109
Sputum Culture 109
Nasopharyngeal Aspirates 109
Serology 109
Other 109
Radiology 109
In a Child less than 5 Years with Pyrexia of Unknown Origin 109
In Children Presenting with Clinical Signs Suggestive of Parapneumonic Pleural Effusion/Empyema 109
In Children Admitted for Treatment of Pneumonia Where There Is No Clinical Improvement After 48 h antibiotic Therapy 110
As Follow-Up in Children Who Have Had Lobar Collapse 110
In Children with Any History Suggestive of an Inhaled Foreign Body 110
Differential Diagnosis 110
Bronchiolitis 110
Inhaled Foreign Body 110
Empyema 110
Management 110
Criteria for Admission 110
Monitoring 111
Oxygen 111
Fluid Management 111
Antipyretics 111
Physiotherapy 111
Antibiotic Therapy 111
Antibiotic Resistance 111
Route of Administration 111
Duration of Antibiotic Treatment 111
Criteria for Transfer to Intensive Care 112
Complications 112
Bronchiectasis 112
Pneumatoceles 112
Siadh 112
Pericarditis 112
Bronchiolitis Obliterans 112
Extrapulmonary Inflammatory Complications Following Mycoplasma Pneumonia 112
Rare and Usual Cases 113
When to Suspect Congenital Abnormality 113
When to Consider Immunodeficiency, Cystic Fibrosis, or Primary Ciliary Dyskinesia 113
Controversies 114
When is Treatment Indicated? 114
Aspiration as an Etiological Factor 114
Duration of Treatment 114
Investigations 114
Prognosis and Long-Term Outcome 114
Conclusions and Future Perspective 114
References 115
Chapter 9 118
Empyema Thoracis 118
Introduction 118
Historical Perspective 118
Pathogenesis and Basic Science 118
Etiology 118
Pathogenesis 119
Stage I: Exudative Phase 119
Stage II: Fibropurulent Phase 120
Stage III: Organization Phase 120
Pneumatocele and Necrotizing Pneumonia 120
Extrapulmonary Complications 120
Microbiology 120
Clinical Features 121
Clinical History 121
Physical Examination 121
Diagnosis 122
Investigations 122
Acute Phase Reactants 122
Biochemistry, Hematology, and Coagulation Studies 122
Microbiology 122
Diagnostic Pleural Aspiration 122
Radiology 122
Bronchoscopy 124
Differential Diagnosis 124
Intrathoracic Tumors Presenting as Empyema 124
Management 125
Medical Management 125
Antibiotics 125
Pleural Aspiration 125
Surgical Management 126
Intercostal Tube Drainage and Fibrinolytics 126
Technique: Intercostal Chest Tube Drainage 126
Intrapleural Fibrinolytic Agents 127
Early Surgery 128
Minithoracotomy and Debridement 128
Technique of Minithoracotomy and Debridement 129
Video-Assisted Thoracic Surgery (VATS) Debridement 129
Technique of VATS Debridement 129
Thoracotomy and Decortication 130
Technique of Decortication 130
Thoracoplasty 130
Management of Complex Empyemas 130
Empyema with Lung Abscess and Necrotizing Pneumonia 130
Bronchopleural Fistula and Pyopneumothorax 131
Serratus Anterior Myoplastic Flap Technique 131
Bilateral Empyema 132
Secondary Empyema 132
Outpatient Follow-Up After Empyema 132
Conclusion and Future Perspective 132
References 132
Chapter 10 137
Pulmonary Tuberculosis 137
Introduction 137
Epidemiology 137
Historical Perspective 137
Basic science 138
Etiology 138
Epidemiology 138
Spread 138
The pyramid of childhood tuberculosis 138
Microbiology 139
Pathogenesis 139
Clinical features 141
Diagnosis 141
Investigations 141
Tuberculin sensitivity testing 141
Hematological investigations 142
Microbiological Investigations 142
Newer Diagnostic Techniques 142
Radiology 142
Differential Diagnosis 143
Management 143
Medical Management: 143
Anti-tuberculosis Drugs 144
General principles of antituberculous therapy 144
Directly Observed Treatment, Short Course 144
Corticosteroids 145
Evaluation during treatment 145
Evaluation at the end of treatment 145
Medical management of special cases 145
Surgical Management 146
Technical aspects of TB surgery 147
Prevention of tuberculosis 147
BCG Vaccine 147
Tuberculosis in children with HIV 147
Multi drug resistant TB 148
Mycobacteria other than tuberculosis 148
Clinical features 148
MOTT pulmonary disease 148
Management 148
Future Perspective 149
References 150
Chapter 11 152
Lung Abscess 152
Introduction 152
Historical Perspective 152
Pathogenesis 153
Etiology 153
Pathophysiology 154
Microbiology 155
Clinical Features 155
Diagnosis 156
Investigations 156
Radiology 156
Microbiology 157
Other Investigations 157
Differential Diagnosis 157
Management 157
Medical Management 157
Surgical Management 158
Indications for Surgical Intervention 158
Anesthetic Considerations 158
Surgical Considerations 159
Nosocomial Pneumonia and Lung Abscess 160
Lung Abscess in the HIV-Infected Patient 162
Morbidity and Mortality 163
Conclusion and Future Perspective 163
References 164
Chapter 12 167
Pulmonary Hydatid Disease 167
Introduction 167
Historical Perspective 167
Incidence 167
Pathogenesis 167
Control Programs 168
Clinical Features 168
Investigations 168
Management 170
Medical Treatment 170
Conservative Management 170
Surgical Management 170
Intact Cysts 170
Multiple Pulmonary Cysts 171
Rupture into the bronchial tree 172
Rupture into the Pleura 172
Summary 172
References 172
Chapter 13 174
Bronchiectasis 174
Introduction 174
Historical Perspective 174
Pathogenesis 175
Etiology 175
Pathophysiology 176
Clinical Features 178
Diagnosis 178
Investigations 178
Management 180
Medical Management 180
Prevention 180
Management of the Cause 180
Management of the Bronchiectasis 180
Other Agents 181
Surgical Management 181
Principles 181
Preoperative Preparations 181
Anesthetic Considerations 181
Surgical Technical Considerations 182
Morbidity and Mortality 182
Future Perspective 182
References 183
Chapter 14 184
Chronic Pleuro-Pulmonary and Chest Wall Infections 184
Introduction 184
Historical Perspective 184
Chronic Pleuropulmonary Infections 185
Fungal Infections 185
Histoplasmosis 185
Coccidioidomycosis 187
Blastomycosis 187
Aspergillosis 187
Aspergilloma 187
Invasive Pulmonary Aspergillosis 188
Allergic Bronchopulmonary Aspergillosis 189
Chronic Necrotizing Aspergillosis 189
Pulmonary Candidiasis 189
Actinomycosis 189
Nocardiosis 190
Parasitic Infections 190
Pleuropulmonary Amebiasis 190
Management 190
Ascariasis 190
Management 191
Paragonimiasis 191
Eosinophilic Pneumonia 191
Management 191
Chronic Pulmonary Infections with Underlying Pathology 191
Late presenting inhaled foreign body 191
Management 192
Late Infective Complications of Congenital Lung Malformations 192
Lung Resection in the Presence of Chronic Infection 193
Bony Chest Wall Infections 193
References 193
Part III: Trauma 196
Chapter 15 197
Thoracic Trauma. General Considerations 197
Introduction 197
Historical Perspective 197
Physiology 197
The Pediatric Thorax 198
Incidence 198
Mechanisms 199
Injury Types 199
Associated Injuries 200
Mortality 200
Initial Management 200
Prehospital 200
Emergency Department 200
Conclusion 201
References 201
Chapter 16 203
Blunt Thoracic Trauma 203
Introduction 203
Chest Wall Injury 204
Rib and Sternal Fractures 204
Diagnosis 204
Clinical Examination 204
Investigations 205
Management 205
Complications 205
Pulmonary Contusion 205
Diagnosis 205
Clinical Examination 205
Investigations 205
Management 206
Complications 206
Pulmonary Laceration 206
Diagnosis 207
Clinical Examination 207
Investigations 207
Management 207
Complications 207
Pulmonary Hematoma 207
Diagnosis 207
Clinical Examination 207
Investigations 207
Complications 207
Simple Pneumothorax 207
Diagnosis 208
Clinical Examination 208
Investigations 208
Management 208
Complications 208
Tension Pneumothorax 208
Diagnosis 209
Clinical Examination 209
Investigations 209
Management 209
Complications 209
Hemothorax 209
Clinical Examination 209
Investigations 210
Management 210
Complications 210
Traumatic Asphyxiation 210
Diagnosis 211
Clinical Examination 211
Investigations 211
Management 211
Complications 211
Cardiac Injury 211
Pericardial Tamponade 211
Diagnosis 211
Clinical Examination 211
Investigations 211
Management 211
Complications 212
Myocardial Contusion 212
Diagnosis 212
Clinical Examination 212
Investigations 212
Management 212
Great Vessel Injury 212
Diagnosis 212
Clinical Examination 212
Investigations 212
Management 213
Diaphragmatic Rupture 213
Diagnosis 213
Clinical Examination 213
Investigations 213
Management 213
Complications 213
Esophageal Injury 214
Diagnosis 214
Clinical Examination 214
Investigations 214
Management 214
Complications 214
References 214
Chapter 17 217
Penetrating Thoracic Trauma 217
Introduction 217
Open Pneumothorax 218
Diagnosis 219
Clinical Examination 219
Investigations 219
Management 219
Complications 219
Hemothorax 219
Diagnosis 220
Clinical Examination 220
Investigations 220
Management 220
Complications 220
Tracheobronchial Injuries 220
Diagnosis 221
Clinical Examination 221
Investigations 221
Management 221
Complications 222
Esophageal Injury 222
Diagnosis 222
Clinical Examination 222
Investigations 222
Management 222
Complications 222
Pericardial Tamponade 222
Diagnosis 223
Clinical Examination 223
Investigations 223
Management 223
Complications 223
Cardiac Laceration 223
Diagnosis 223
Clinical Examination 223
Investigations 223
Management 223
Complications 224
Great Vessel Injuries 224
Diagnosis 224
Clinical Examination 224
Investigations 224
Management 224
Complications 224
Thoracic Duct Injury 224
Diagnosis 225
Clinical Examination 225
Investigations 225
Management 225
Complications 225
Conclusion 225
References 225
Part IV: Tumours 226
Chapter 18 227
Anterior Mediastinal Tumors 227
Embryologic and Anatomic Features of the Anterior Mediastinum 227
Classification 227
Clinical Features 227
Diagnostic Tests 227
Principles of Management 228
Lymphoid Tissue Tumors 228
Non-Hodgkin Lymphoma (NHL) 228
Hodgkin Lymphoma (HL) 229
Germ Cell Tumors 229
Teratomas 229
Non-Teratomatous Germ Cell Tumors 231
Tumors and Cysts of the Thymus 231
Thymic Hyperplasia 231
Thymic Cysts 231
Malignant Thymic Tumors 231
Vascular Tumors and Anomalies 231
Vascular Tumors 231
Vascular Malformations 231
Other rare Anterior Mediastinal Tumors 232
References 233
Chapter 19 236
Posterior Mediastinal Tumors 236
Introduction 236
Neuroblastoma 236
Introduction 236
Historical Perspective 236
Incidence & Epidemiology
Basic Science 237
Embryology 237
Pathology 237
Tumour Biology, Cytogenetics & Molecular Pathology
Tumour Markers 238
Diagnosis 238
Clinical Features 238
Laboratory Investigations 239
Imaging 239
Radiographs 239
Computerized Tomography 239
Magnetic Resonance Imaging 239
Investigations for staging 240
Other 240
Disease Staging 241
Tumour stage 242
Tumour site 242
Antenatal diagnosis 242
Differential diagnosis 242
Management options 242
An Overview of Treatment Strategies 242
Management protocols 243
Low Risk Group 243
Intermediate Risk Group 243
High Risk Group 243
Special cases 243
Surgical Management 243
Controversies in surgical resection 243
Biopsy 244
Tumor Resection 245
The Dartevelle Approach 245
Minimally invasive resection 246
Complications of surgery 246
Survival 246
Future perspective 246
Other primary posterior mediastinal tumours 247
Neurilemmoma (Schwannoma) 247
Neurofibroma 247
Neurofibrosarcoma 248
Extra-adrenal pheocromocytoma (paraganglioma /chemodectoma) 248
Other primary non-neurogenic tumors 248
References 249
Chapter 20 252
Lung Tumors 252
Introduction 252
Historical Perspective 252
Pathology 252
Primary Lung Tumors3 252
Clinical Presentation 253
Diagnosis 253
Radiology 253
Benign Tracheo-Bronchial Tumors 254
Malignant Tracheo-Bronchial Tumors 254
Carcinoid Tumors 254
Fibrosarcoma of the Lung 255
Bronchogenic Carcinoma 255
Benign Parenchymal Lung Tumors 255
Inflammatory Myofibroblastic Tumor 255
Hamartoma 255
Haemangiopericytoma 256
Sclerosing Haemangioma 256
Malignant Parenchymal Tumors 256
Pleuropulmonary Blastoma 256
Rhabdomyosarcoma of the Lung 257
Other Malignant Parenchymal Tumors 257
Metastatic Lung Tumors 257
Conclusions and Future Perspectives 259
References 259
Chapter 21 261
Chest Wall Tumors 261
Introduction 261
Historic Perspective 261
Basic Science/Pathogenesis 261
Clinical Features 262
Diagnosis 262
Investigations 262
Radiology 262
Biopsy 262
Benign Chest Wall Tumors 263
Benign Rib Tumors 263
Osteochondroma 263
Chondroma 263
Fibrous Dysplasia 263
Eosinophilic Granuloma/Histocytosis X 263
Mesenchymal Hamartoma 264
Chondromatous Hamartoma 264
Osteoblastoma 264
Osteoid Osteoma 264
Benign Soft Tissue Tumors 264
Fibroblastic-Myofibroblastic Tumors 264
Lipoblastomatosis/Liposarcoma 265
Giant Cell Fibroblastoma 266
Malignant Chest Wall Tumors 267
Malignant Bony Tumors 267
Chondrosarcoma 267
Malignant Small Round Cell Tumors (MSRCT) 267
Premitive Neuroectodermal Tumor (PNET) and Mesenchymal Sarcoma 267
Ewings sarcoma and Askin’s Tumor 267
Osteosarcoma 269
Rhabdomyosarcoma 269
Congenital-Infantile Fibrosarcoma 270
Neurofibrosarcoma 270
Lymphoma 271
Differential Diagnosis 271
Infection 271
Chest Wall Deformity 271
Management 271
Surgical Management 271
Chest Wall Reconstruction 271
Skeletal Reconstruction 272
Soft Tissue Reconstruction 272
Long-Term Complications 272
Scoliosis 272
Restrictive Pulmonary Function 273
Secondary Tumors 273
Sternal Tumors 273
Pediatric Sternal Tumors 273
Enchondroma 273
Osteoblastoma 273
Clinical Features 273
Diagnosis 274
Surgical Management 274
Technical Considerations 274
Conclusions and Future Perspective 274
References 274
Part V: Esophagus 278
Chapter 22 279
Congenital Malformations 279
Introduction 279
Historical Perspective 279
Basic Science/Pathogenesis 279
Embryogenesis of Esophageal Atresia 279
Tracheoesophageal Separation 279
The Abnormal Notochord in Esophageal Atresia 280
The Upper Esophageal Pouch 281
Role of Sonic Hedgehog and Other Genes 281
Anatomical Considerations 282
Upper Esophageal Segment 282
Lower Esophageal Segment 282
Innervation of the Esophagus 282
Blood Supply to the Esophagus 283
The Trachea 283
Epidemiology 283
Antenatal Diagnosis 284
Postnatal Diagnosis 284
Clinical Features 284
Confirmation of Esophageal Atresia 284
Determination of the Type of Esophageal Atresia 285
Associated Abnormalities 286
Congenital Heart Disease 286
Coexistence of Atresias of the Esophagus, Duodenum, and Anorectum 286
Urinary Tract Abnormalities 287
Management 287
Antenatal Management 287
Preoperative Care 287
Repair by Open Thoracotomy 287
Position 287
Surgical Approach 287
Division of the Distal Tracheoesophageal Fistula 287
Dissection of the Upper Esophagus 288
Anastomosis to Gain Esophageal Continuity 288
Closure 288
Thoracoscopic Repair 288
Position and Port Placement 289
Closure of the Tracheoesophageal Fistula 290
Mobilization of the Upper Pouch 290
Esophageal Anastomosis 290
Postoperative Management 290
Role of Postoperative Ventilation and Neck Flexion 290
Surgical Techniques for Long-Gap Esophageal Atresia 291
Initial Management of Esophageal Atresia and a Gasless Abdomen 291
Surgical Techniques Used for Long-Gap Atresia 293
Modified Scharli Technique 293
Gastric Transposition 293
Gastric Tube Procedures 293
Other Methods 293
Right-Sided Aortic Arch 295
Coexisting Duodenal Atresia and Imperforate Anus 295
Extreme Prematurity 296
Tracheomalacia 297
Indications for Surgery 297
Aortopexy 297
Complications 297
Anastomotic Leak 297
Esophageal Stricture 298
Recurrent Tracheoesophageal Fistula 298
Gastroesophageal Reflux and Esophageal Dysmotility 299
Rare and Unusual Variants 299
The Congenital “H” Fistula 299
Diagnosis 299
Investigation 299
Operative Management 300
Complications 300
Other Rare Variants 300
Congenital Lower Esophageal Stenosis 300
Prognosis and Long-Term Outcomes 301
Predictors of Outcome 301
Esophageal Function 301
Growth 301
Risk of Esophageal Malignancy After Repair of Esophageal Atresia 301
Quality of Life 302
Parent Support Groups 302
References 302
Chapter 23 308
Esophageal Stricture 308
Introduction 308
Historical Perspective 308
Etiology 308
Congenital Lesions 309
Clinical Features and Diagnosis 309
Management 309
Acquired Lesions 310
Peptic Stricture 310
Pathogenesis 310
Clinical Features and Diagnosis 310
Management 310
Schatzki’s Rings 311
Eosinophilic esophagitis 311
Management 311
Post-anastomotic strictures 311
Management 311
Clinical Features and Diagnosis 311
Management 312
Corrosive strictures 313
Pathogenesis 313
Clinical features and Diagnosis 313
Management 313
Esophageal Dilation 315
Bouginage 315
Summary 315
References 316
Chapter 24 318
Esophageal Replacement 318
Introduction 318
Historic Perspective 319
Stomach: Gastric Tube 319
Stomach: Gastric Pull-Up 319
Jejunum 320
Colon 320
Management Options 320
Gastric Tube 321
Results and Discussion on Gastric Pull-Ups 323
Gastric Pull-Up 324
Results and Discussion on Gastric Pull-Ups 325
Jejunal Transposition 325
Colonic Transposition 326
Results and Discussion on Colonic Transposition 326
Complications 327
References 327
Chapter 25 331
Achalasia 331
Introduction 331
Historical Perspective 331
Pathogenesis 331
Clinical Features and Diagnosis 332
Radiology and Manometric Studies 332
Management 333
Medical 333
Surgical 333
Dilatation 333
Injection of Botulinum Toxin 333
Modified Heller’s Cardiomyotomy 334
Prognosis and Long-term Outcome 335
Conclusion 335
References 335
Chapter 26 337
Gastro-esophageal Reflux 337
Introduction 337
Historic Perspective 337
Basic Science/Pathogenesis 338
Mechanisms Preventing Reflux 338
Etiology of GERD 338
Associations/Predisposing Factors 339
Clinical Features 339
Presentation 339
Diagnosis 340
Management 342
Laparoscopic Nissen Fundoplication 343
Other Antireflux Procedures 345
Redo Fundoplication 345
Esophagogastric Dissociation 345
Complications of Antireflux Surgery 345
Special Cases 346
Reflux in Children with Neurological Impairment 346
Reflux in Children and Adults with Previous Esophageal Atresia Repair 347
Controversies 347
Barrett’s Esophagus in Children with GERD 347
Conclusion 348
References 348
Chapter 27 352
Esophageal Foreign Bodies 352
Introduction 352
Historic Perspective 352
Basic Science/Pathogenesis 352
Clinical Features 353
Diagnosis 353
Management 354
Technique of Rigid Esophagoscopy 354
Rare Case: Tracheoesophageal Fistula Caused by Electrolysis of a Button Battery Impacted in The Esophagus 355
Aortoesophageal Fistula 356
References 356
Part VI: Trachea 357
Chapter 28 358
Foreign Bodies in the Airway 358
Introduction 358
Historical Perspective 358
Basic Science and Epidemiology 358
Diagnosis 359
Signs and Symptoms 359
Investigations 360
Management 361
Immediate Management of Inhaled Foreign Bodies 361
Definitive Management 361
Complications 363
References 363
Chapter 29 365
Vascular Rings 365
Introduction 365
Historical Perspective 365
Incidence 365
Pathogenesis and Embryology 365
Classification 365
Clinical Features 368
Diagnosis and Investigations 369
Differential Diagnosis 370
Management 370
OutcomesSurgery is safe 372
Complications 372
Conclusions and Future Perspectives 372
References 372
Chapter 30 374
Foregut Duplication Cysts 374
Introduction 374
Historic Perspective 374
Basic Science 374
Clinical Features 374
Diagnosis 375
Management 376
Postoperative Care 377
Complications 377
Rare and Unusual Cases 378
Controversies 378
Prognosis and Long-Term Outcome 378
Conclusion 378
References 378
Part VII: Lung Disease 380
Chapter 31 381
Cystic Adenomatoid Malformation 381
Introduction 381
Historical Perspective 381
Pathogenesis 381
Clinical Features 382
Presentation 382
Diagnosis 383
Treatment 384
Controversies 387
Conclusion 387
References 387
Chapter 32 389
Pulmonary Sequestration 389
Introduction 389
Historic Perspective 389
Pathogenesis 389
Clinical Features 390
Diagnosis 390
Treatment 392
Surgical Resection 392
Postoperative Care 393
Unusual Cases 394
Controversies 394
Conclusion 394
References 394
Chapter 33 396
Congenital Lobar Emphysema 396
Introduction 396
Historical Perspective 396
Pathogenesis 396
Clinical Features 397
Diagnosis 397
Treatment 398
Conclusion 398
References 399
Chapter 34 400
Respiratory Disorders of the Newborn 400
Introduction 400
Historical Perspective 400
Acute Respiratory Diseases 400
Respiratory Distress Syndrome 400
Pathogenesis and Basic Science 401
Clinical Features 401
Diagnosis 401
Radiological Diagnosis 401
Differential Diagnosis 401
Management 402
Prenatal Steroids 402
Surfactant Replacement 402
Neonatal Intensive Care 402
Respiratory Support 402
Pulmonary Air Leak Syndromes 402
Pathogenesis and Basic Science 403
Pulmonary Interstitial Emphysema 403
Pathogenesis and Basic Science 403
Clinical Features 403
Diagnosis 403
Management 403
Prognosis 404
Pneumothorax 405
Pathogenesis and Basic Science 405
Clinical Features 405
Diagnosis 406
Management 406
Pneumomediastinum 407
Pneumopericardium 407
Pneumoperitoneum 407
Pleural Effusions 407
Pathogenesis and Basic Science 407
Clinical Features 407
Diagnosis 407
Management 407
Prognosis 407
Chylothorax 408
Hemothorax 408
Pulmonary Hemorrhage 408
Management 408
Meconium Aspiration Syndrome 408
Pathogenesis and Basic Science 408
Clinical Features 409
Diagnosis 409
Management 409
Prognosis 409
Persistent Pulmonary Hypertension of the Newborn 409
Pathogenesis and Basic Science 409
Clinical Features and Diagnosis 409
Management 409
Chronic Lung Disease 410
Bronchopulmonary Dysplasia 410
Pathogenesis and Basic Science 410
Clinical features 410
Diagnosis 410
Management 410
Prognosis 411
Tracheobronchomalacia 411
Pathogenesis and Basic Science 411
Clinical features 411
Diagnosis 411
Management 411
References 412
Chapter 35 415
Cystic Fibrosis 415
Introduction 415
Background and History 415
Pathogenesis 415
Diagnosis 416
Immunoreactive Trypsin 416
Sweat Test 416
DNA Analysis for Genotype 417
Fecal Elastase 417
Neonatal Screening 417
Clinical Presentations 417
Meconium Ileus 417
Malabsorption and Failure to Thrive 418
Recurrent Respiratory Infections 418
Pseudo-Bartter’s Syndrome 419
Rectal Prolapse 419
Rarer Neonatal Presentations 419
Clinical Features and Their Management 419
Respiratory Disease 419
Infection and Respiratory Exacerbations 419
Respiratory Viruses 420
Staphylococcus aureus 420
Hemophilus influenzae 420
Pseudomonas aeruginosa 420
Stenotrophomonas maltophilia 420
Burkholderia Cepacia Complex 420
Methicillin-Resistant Staphylococcus aureus (MRSA) 421
Aspergillus Species 421
Non-Tuberculous Mycobacteria 421
Chest Physiotherapy 421
Wheeze and Small Airways Disease 421
Bronchodilators 422
Inhaled Corticosteroids 422
Long-Acting beta2 Agonists 422
Other Respiratory Therapies 422
Mucolytics 422
Long-Term Azithromycin 422
Allergic Bronchopulmonary Aspergillosis 422
Pneumothorax 422
Hemoptysis 423
Pulmonary Resection 423
Gastrointestinal Disease 424
Pancreatic Insufficiency and Nutrition 424
Distal Intestinal Obstruction Syndrome 424
Gastro-Esophageal Reflux 424
Fibrosing Colonopathy 425
Pancreatitis 426
Liver Disease 426
CF-Related Diabetes 426
Otorhinolaryngology 426
Nasal Polyps 426
Sinusitis 427
Bone Disease 427
Joint Disease 427
Puberty and Fertility 427
Stress Incontinence 428
Other Surgical Issues 428
Totally Implantable Venous Access Devices 428
Peri-Operative Management of the Patient with CF 428
Lung Transplantation 428
Absolute Contraindications 429
Relative Contraindications 429
Conclusions 429
References 429
Chapter 36 432
Pulmonary Hemorrhage and Hemoptysis 432
Introduction 432
History 432
Pathophysiology 432
Clinical Presentation and Physical Examination 432
Specific Causes of Pulmonary Hemorrhage 433
Cystic Fibrosis (CF) 433
Pulmonary Renal Syndromes 434
Idiopathic Pulmonary Hemosiderosis 434
Heiner’s Syndrome 435
The Cleveland “Epidemic” 435
Pulmonary Hemorrhage Secondary to Cardiovascular Abnormalities 435
Infection 435
Foreign Body Aspiration 435
Pulmonary Hemorrhage in NewbornInfants 435
Trauma 436
Congenital Lung Abnormalities 436
Neoplasm 436
Vascular Malformations 436
Diagnosis 436
Laboratory Investigations 436
Pulmonary Function Tests 437
Bronchoscopy 437
Radiological Diagnosis 437
X-Ray 437
Computed Tomography (CT) 437
MRI 437
Radionulide Imaging 437
Lung Biopsy 437
Management 437
Conservative Management 438
Medical 438
Bronchial Artery Embolisation (BAE) 438
Lobectomy 438
Complications 438
Conclusions 439
References 439
Chapter 37 441
Interstitial Diseases of the Lung 441
Introduction 441
Historical Perspective 441
Epidemiology 441
Pathogenesis and Basic Science 442
Etiology 442
Clinical Features 442
Diagnosis 444
Laboratory Studies 444
Immunological Tests 444
Radiology 444
Chest Radiograph 444
Computed Tomography (CT Scan) 444
Other Radiological Investigations 444
Lung Function Tests 444
Other Noninvasive Investigations 445
Bronchoalveolar Lavage (BAL) 445
Lung Biopsy 445
Acute Interstitial Disease 445
Chronic Interstitial Diseases 446
Management 446
Supportive Care 446
Antiinflammatory Treatment 446
Specific Conditions 446
Disorders Specific to Infancy and Childhood 446
Developmental Abnormalities 447
Surfactant Protein Disorders 447
Other Rare Disorders of Infancy 447
NEHI (Neuroendocrine Hyperplasia of Infancy) 447
PIG (Pulmonary Interstitial Glycogenosis) 447
Desquamative Interstitial Pneumonitis (DIP) 447
Idiopathic Pulmonary Hemosiderosis (IPH) 447
Pulmonary Alveolar Proteinosis 447
Extrinsic Allergic Alveolitis (EAA) 448
Primary Pulmonary Lymphangiectasia 448
Systemic Diseases 448
Conclusion 448
References 448
Chapter 38 450
Pneumothorax 450
Introduction 450
Historic Perspective 450
Basic Science 451
Resorption of Gas from the Pleural Space 451
Etiology 451
Pathogenesis 452
Malignancy 453
Cystic Fibrosis 453
Clinical Features 454
Diagnosis 454
Radiological Diagnosis 454
Other Investigations 456
Differential Diagnosis 456
Management 456
Initial Management 456
Removal of Air vs. Observation 456
Aspiration vs. Drainage 457
Recurrence Prevention 457
Chemical Pleurodesis 457
Surgical Management 457
Thoracotomy 458
Limited Thoracotomy 458
Trans-Axillary Minithoracotomy 458
VATS 458
Complications 459
Complications of Pleurodesis 459
Complications of Surgery 460
Video-Assisted Thoracoscopic Surgery (VATS) 460
Thoracotomy 460
Special Cases 460
Acquired Immune Deficiency Syndrome (AIDS) 460
Cystic Fibrosis 461
Malignancy 461
Alveolar-Pleural Fistula 461
Tension Pneumothorax 462
Prognosis/Long-Term Outcome 462
Advice on Air Travel 462
Conclusion/Future Perspective 462
References 462
Part VIII: Diaphragm 466
Chapter 39 467
Congenital Diaphragmatic Hernia 467
Definition and History 467
Embryology 467
Lung Development 467
Pulmonary Vascular Development 468
Prevalence of Congenital Diaphragmatic Hernia 469
Pathophysiology 469
Pulmonary Hypertension in CDH 470
Etiology and Genetics 470
Experimental Models 471
Teratogen-Induced CDH Models 471
Vitamin A-Deficient Rodents 471
Genetic Models 471
Lamb CDH Model 472
Associated Anomalies/Malformations 472
Diagnosis and Management 472
Antenatal Diagnosis 472
Antenatal Surgery 473
Antenatal Steroid Therapy 474
Delivery 474
Postnatal Diagnosis 475
Postnatal Management 475
Gentle Ventilation and Permissive Hypercapnia 475
High-Frequency Oscillating Ventilation 475
Inhaled Nitric Oxide 476
Extracorporeal Membrane Oxygenation (ECMO) 476
Liquid Ventilation 476
Surgery 476
Postnatal Prognosis 477
Overall Survival 477
Long-Term Sequelae 478
Future Directions 478
References 478
Chapter 40 484
Diaphragmatic Eventration and Phrenic Palsy 484
Introduction 484
Historical Perspective 484
Basic Science, Embryology, and Pathogenesis 484
Diagnosis 486
Management 488
Complications 489
Prognosis and Long-Term Outcome 489
Conclusions 489
References 489
Chapter 41 492
Extra-corporeal Membrane Oxygenation 492
Introduction 492
History of ECLS 492
Physiology and Components of the ECLS Circuit 492
Types of ECLS 494
Venoarterial ECLS 494
Methods of Cannulation for VA ECLS 495
Venovenous ECLS 496
Cannulation Techniques for VV ECLS 497
Indications and Exclusions for ECLS 498
Management of the Patient on ECLS 498
Respiratory Support During ECLS 498
Cardiac Support During ECLS 498
Anticoagulation 498
Sedation/Paralysis 499
Nutritional Support During ECLS 499
Weaning and Decannulation 499
Complications 499
Oxygenator Failure 500
Tubing or Raceway Rupture 500
Air Embolism 500
Bleeding 500
Infection 500
Renal Failure 500
Common Conditions Requiring ECLS and Outcome 500
Neonatal Respiratory Failure 500
Pediatric Respiratory Failure 501
Adult Respiratory Failure 501
ECLS for Cardiac Failure 501
Innovations Using ECLS 502
Summary 502
References 502
Chapter 42 506
Diaphragmatic Pacing in Children 506
Introduction 506
Historical Background: Ondine’s Curse 506
Pathophysiology 506
Physiologic Abnormalities in Ventilatory Control 507
Genetics of CCAHS 507
Associated Anomalies 508
Clinical Features of CCAHS 508
Diagnosis of CCAHS 508
Respiratory Failure Associated with Spinal Cord Trauma 509
Management 509
Pharmacologic Management 509
Mechanical Ventilation 509
Diaphragmatic Pacing 509
Complications 512
Summary 513
References 513
Part IX: Chest Wall Abnormalities 516
Chapter 43 517
Pectus Excavatum 517
Introduction 517
Historical Perspective 517
Pathogenesis and Basic Science 518
Clinical Features 519
Diagnosis 519
Investigations 519
Cardiology 520
Management 521
Antenatal/Perinatal Management 521
Medical 521
Surgical 521
Conventional Operative Techniques: Description of Modified Ravitch Operation 521
Minimally Invasive Operative Techniques: Description of Nuss Operation 521
Complications 522
Description and Incidence 522
Recurrence 524
Infection 524
Allergy 525
Controversies 525
Prognosis and Long-Term Outcome 525
Conclusions and Future Perspectives 525
References 526
Chapter 44 528
Thoracic Outlet Syndrome 528
Introduction 528
Anatomical Considerations 528
Causation and Pathological Anatomy 529
Presentation and Management 529
Neurogenic TOCS 529
Arterial TOCS 530
Venous TOCS 530
Conservative Management in TOCS 533
Surgical Techniques for Thoracic Outlet Decompression 533
The Supraclavicular Approach 533
The Transaxillary Approach 534
Outcomes After TOCS Surgery 535
Conclusion 535
References 535
Chapter 45 538
Thoracopagus Conjoined Twins 538
Introduction 538
Incidence 538
Historical Perspective 539
Clinical Features 540
Diagnosis 540
Prenatal Diagnosis 540
Obstetric Implications 541
Postnatal Diagnosis and Management 542
Ethical and Moral Considerations 542
Anesthetic Considerations 542
Anesthetic Challenges 543
Preoperative Work-Up 543
Equipment Preparation and/or Adaptation 543
Anesthesia for Procedures Prior to Separation 543
Surgical Management 544
Preoperative Preparation 544
Intraoperative Management 544
General Principles of Surgery 544
Skin Closure 544
Hepatobiliary and Gastro-Intestinal Tract 545
Musculoskeletal System 545
Cardiorespiratory System and Chest Wall 545
Postoperative Managemen 547
Results 547
Surgical Outcome 548
Conclusions 549
References 549
Part X: Miscellaneous 551
Chapter 46 552
Chylothorax 552
Introduction 552
Embryology 552
Anatomy 552
Chyle Physiology 552
Pathophysiology of Chylothorax 552
Congenital 553
Acquired 553
Antenatal Primary Fetal Hydrothorax 553
Associated Systemic Abnormalities 553
Diagnosis 553
Medical Therapy 554
Dietary 554
Adjunctive Medications 554
Surgical Approaches 554
Drainage 554
Thoracic Duct Ligation 554
Pleuroperitoneal Shunt 555
Treatment Recommendations 555
References 556
Chapter 47 557
The Thymus and Myasthenia Gravis 557
Introduction 557
Historical Perspective 557
Anatomy and Embryology 557
Consequences of Thymectomy 559
Thymic Hyperplasia 559
DiGeorge Syndrome 559
Thymoma 559
Diagnosis 559
Paraneoplastic Conditions 559
Pathology 560
Diagnosis and Imaging 560
Staging 560
Management 560
Myasthenia Gravis 560
Basic Science 561
Role of the Thymus in Myasthenia Gravis 561
Clinical Features 561
Clinical Course 562
Diagnosis 562
Management 562
Medical Management 562
Surgical Management 562
Surgical Exposure of the Thymus 563
Median Sternotomy 563
Trans-Cervical Approach 563
VATS Thymectomy 563
Results After Thymectomy 563
Other Thymic Tumors 564
Thymolipoma 564
Mediastinal Lymphangioma 564
Thymic Cysts 564
Conclusion and Future Perspective 564
References 564
Chapter 48 567
Hyperhidrosis and Thoracic Sympathectomy 567
Introduction 567
Historic Perspective 567
Basic Science 567
Etiology 568
Clinical Features 569
Diagnosis 569
Management 569
Conservative Management 569
Medical Therapy 569
Ionotophorosis 570
Surgical Management 570
Thoracic Sympathectomy 570
Operative Technique 573
Results 575
Side Effects of Sympathectomy 575
Conclusions and Future Perspective 576
References 576
Index 579