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Pediatric Surgery E-Book

Pediatric Surgery E-Book

Arnold G. Coran | Anthony Caldamone | N. Scott Adzick | Thomas M. Krummel | Jean-Martin Laberge | Robert Shamberger

(2012)

Additional Information

Book Details

Abstract

Pediatric Surgery, 7th Edition - edited by Arnold G. Coran, Anthony Caldamone, N. Scott Adzick, Thomas M. Krummel, Jean-Martin Laberge, and Robert Shamberger - features comprehensive, up-to-date guidance on all aspects of childhood surgery, including congenital malformations, tumors, trauma, and urologic problems. Apply the latest developments in fetal surgery, adolescent bariatric surgery, minimally invasive surgery in children, and tissue engineering for the repair of congenital anomalies, such as the separation of conjoined twins.

  • Get comprehensive coverage of cutting-edge technology in pediatric surgical diseases, including imaging concepts, minimally invasive techniques, robotics, diagnostic and therapeutic advances, and molecular biology and genetics.
  • Find information quickly and easily with an intuitive organization by body region and organs.
  • Apply the guidance of world-renowned experts in pediatric surgery.
  • Stay current on recent developments in fetal surgery, adolescent bariatric surgery, minimally invasive surgery in children, and tissue engineering for the repair of congenital anomalies, such as the separation of conjoined twins.
  • Master the latest surgeries available for fetal and neonatal patients and provide life-saving options at birth.
  • Tap into the expertise of new editors who bring fresh perspectives to cutting-edge techniques.

Table of Contents

Section Title Page Action Price
e9780323072557v1.pdf 1
Front Cover 1
Pediatric Surgery 3
Copyright 5
About the Editors 6
Contributors 8
Preface 24
Contents 26
Part I: General 30
Chapter 1: History of Pediatric Surgery: A Brief Overview 32
Early Years 32
20th Century: The Formative Years 33
United States 33
Education, Organizational Changes, and Related Activities 35
Research 36
Clinical Advances Related to Research 37
Canada 38
United Kingdom and Ireland 39
Ireland 40
Europe 41
Australia and New Zealand 44
Asia 44
Developing countries 46
Chapter 2: Molecular Clinical Genetics and Gene Therapy 48
Molecular Clinical Genetics 48
Human molecular genetics and pediatric surgical disease 48
Disease-specific examples of changing concepts in molecular genetics 49
Monogenic Disorders 49
Oligogenic Disorders 49
Polygenic or Complex Disorders 50
Utility of molecular genetics in clinical pediatric surgery 51
Genetic Counseling and Prenatal Diagnosis 51
Postnatal Treatment 51
Gene Therapy 52
Viral vectors for gene transfer 52
Clinically relevant challenges in gene transfer 54
Overview of the current status of gene transfer 55
Chapter 3: Impact of Tissue Engineering in Pediatric Surgery 56
Interdisciplinary Approach 56
Cartilage and Bone Tissue Engineering 58
Cardiac Tissue Engineering 59
Vascular Tissue Engineering 60
Gastrointestinal Tissue Engineering 61
Liver Replacement and Tissue Engineering 62
Future Directions: Vascular Networks 62
Chapter 4: Advanced and Emerging Surgical Technologies and the Process of Innovation 66
Current and Future Diagnostic Technologies 67
Ultrasonography 67
Three-Dimensional Ultrasonography 67
Ultrasound Contrast Imaging and Ultrasound Harmonic Imaging 69
Ultrasonography and Fetal Surgery 69
Computed tomography 69
Multidetector Computed Tomography 70
Three-Dimensional Computed Tomography 71
Electron Beam Computed Tomography 71
Magnetic resonance imaging 72
Higher Field Strength MRI Systems 72
Ultrafast MRI 73
Motion Artifact Reduction Techniques 73
Functional Magnetic Resonance Imaging 73
Fetal Magnetic Resonance Imaging 74
Positron emission tomography imaging 74
Molecular imaging 75
DNA microarrays 77
Innovative Therapeutics: Technologies and Techniques 77
Hemostatic and tissue ablative instruments 77
Electrocautery 78
Argon Beam Coagulator 78
Surgical Lasers 78
Photodynamic Therapy 78
Ultrasonography 78
Harmonic Scalpel 78
Cavitation Devices 79
Radiofrequency Energy 79
Microwave Energy 79
Cryotherapy 79
Image-guided therapy 79
Significance 79
General Requirements 79
Patient-Specific Models 79
High Image Quality 79
Real-Time Feedback 79
High Accuracy and Precision 79
Repeatability and Robustness 79
Correlation of Intraoperative Information with Preoperative Images 80
Intuitive Machine and User Interfaces 80
Ultrasound Image-Guided Therapy 80
Computed Tomography and Magnetic Resonance Image-Guided Therapy 80
Radiotherapy and Fractionation 80
Stereotactic Radiosurgery 80
Stereotactic Radiosurgical Platforms 81
Linear Accelerator Radiosurgery 81
Frameless Image-Guided Radiosurgery 81
Clinical application of stereotactic radiosurgery in children 82
Radioimmunoguided Surgery 83
Next-generation minimal access surgery 83
Subcutaneous Endoscopy 83
Single Incision Laparoscopy 84
Natural orifice translumenal endosurgery 85
Endolumenal Therapies 86
Surgical robotics 86
Robotic Technology in Surgery 87
Classification of Robotic Surgical Systems 87
Current Status of Robotic Technology Used in Pediatric Surgery 87
The da Vinci Surgical System 87
Current Advantages and Limitations of Robotic Pediatric Surgery 88
Applications of Robotic Technology to Pediatric Surgery 89
Microtechnologies and Nanotechnologies-Size Matters 89
Microelectromechanical Systems 89
Sensors 90
Data Knife and H-Probe Surgical Instruments 90
Arterial Blood Gas Analyzer 90
Blood Pressure Sensor 90
Other MEMS Sensors in Medicine 90
Actuators 90
Drug Delivery Systems 90
Next Steps for MEMS 91
Nanoelectromechanical systems 91
Coating and Surface Manipulation 91
Self Assembly 91
NEMS in Oncology 91
Surgical Innovator 92
Innovative devices 92
Innovative procedures 92
Pediatric device development 92
Innovative Surgical Training 94
Surgical simulation 94
Visual Display Systems in Simulation 94
Human/Simulator Interface and Tactile Feedback 95
Image Generation 95
Simulation in Education, Training, and Practice 95
Looking Forward 96
Virtual reality 96
Components of Virtual Reality 97
Patient-Specific Virtual Reality 97
Surface Rendering 97
Volume Rendering 98
Finite Elements 98
Visual Displays 99
Input Devices 99
Force and Tactile Feedback 100
Tracking in Virtual Reality 100
Challenges of Virtual Reality 101
Virtual Reality Preoperative Planning 101
Virtual Reality-Based Three-Dimensional Surgical Simulators 102
Simulation in surgical education 102
Training the minimal access therapist 103
Training the surgical innovator 103
Conclusion 104
Chapter 5: Prenatal Diagnosis and Fetal Therapy 106
Fetal Diagnosis 106
Biochemical Screening 106
Fetal Sampling 106
Fetal Imaging 107
Ultrasonography 107
Echocardiography 107
Magnetic Resonance Imaging 107
Fetal Access 108
Management of Mother and Fetus 109
Risks of Maternal-Fetal Surgery 111
Prenatal Diagnosis Dictates Perinatal Management 111
Fetal Anomalies 111
Urinary Tract Obstruction 111
Airway Obstruction 112
Congenital Pulmonary Airway Malformation 112
Congenital Diaphragmatic Hernia 114
Myelomeningocele 115
Sacrococcygeal Teratoma 115
Gastroschisis 116
Intestinal Abnormalities 116
Anomalies of Monochorionic Twins 116
Twin-Twin Transfusion Syndrome 116
Twin Reversed Arterial Perfusion Sequence 117
Inherited Defects Correctable by Fetal Stem Cell Transplantation 117
Past and Future of Fetal Intervention 117
Chapter 6: Neonatal Physiology and Metabolic Considerations 118
Premature, Small for Gestational Age, and Neonates with Intrauterine Growth Retardation 118
Predicting Neonatal Mortality 119
Fluid and Electrolyte Balance 120
Body water composition 120
Neonatal fluid balance 121
Renal function 122
Common fluid and electrolyte disturbances and their treatment 122
Sodium 122
Hyponatremia 122
Hypernatremia 122
Potassium 122
Hypokalemia 122
Hyperkalemia 122
Calcium 122
Hypocalcemia 123
Hypercalcemia 123
Magnesium 123
Acid-Base Balance 123
Intravenous Fluid Administration 124
Fluid Maintenance 124
Energy Metabolism 124
Energy intake 124
Energy storage 125
Energy of growth and tissue synthesis 126
Energy losses 126
Energy used in activity 126
Basal metabolic rate and resting energy expenditure 126
Age 126
Body Composition 126
Size of Vital Organs 126
Dietary Intake 126
Thermoregulation 127
Response to cold 127
Carbohydrate, Fat, and Protein Metabolism of the Neonate 128
Neonatal glucose metabolism 128
Glucagon/Insulin Axis in the Perinatal Period 129
Glycogen and Glycogenolysis in the Perinatal Period 129
Gluconeogenesis in the Neonate 129
Neonatal Hypoglycemia 129
Neonatal Hyperglycemia 130
Neonatal lipid and fat metabolism 131
Fatty Acid Oxidation and Ketogenesis in Neonates 131
Ketone Body Use 131
Neonatal Protein and Amino Acid Metabolism 131
Metabolic Response to Stress 132
Operative trauma 132
Cytokines 133
Endocrine Response 134
Effect of Surgery on Glucose Metabolism in Neonates 134
Effect of Surgery on Fat Metabolism in Neonates 135
Effect of Surgery on Protein and Amino Acid Metabolism in Neonates 136
Chapter 7: Respiratory Physiology and Care 138
Lung Development 138
Embryonic phase 138
Pseudoglandular phase 138
Canalicular phase 138
Terminal saccular phase 140
Alveolar phase 140
Arterial growth 140
Mediators of fetal lung development 141
Pulmonary Physiology 141
Lung volumes 141
Closing capacity 142
Pulmonary compliance 142
Airway resistance 143
Time constants 143
Pulmonary circulation 143
Pulmonary gas exchange 143
Diffusion 143
Dead Space 143
Ventilation-Perfusion Matching 144
Oxygen Transport 144
Carbon Dioxide Equilibrium and Acid-Base Regulation 144
Monitoring 144
Noninvasive monitoring 145
Pulse Oximetry 145
Capnometry 145
Transcutaneous Measurement of Gas Tension 145
Invasive monitoring 145
Mixed Venous Oxygen Monitoring 145
Arterial Catheterization 145
Pulmonary Artery Catheterization 146
Mechanical Ventilators 146
Cycling mechanisms 146
Mechanical Breath Phases 146
Ventilator types 146
Pressure-Controlled Ventilation 146
Volume-Controlled Ventilation 147
Modes of ventilation 147
Control Mode 147
Assist-Control Mode 147
Intermittent Mandatory Ventilation 147
Synchronized Intermittent Mandatory Ventilation 147
Pressure Support Ventilation 147
Continuous Positive Airway Pressure and Positive End-Expiratory Pressure 147
Inverse Ratio Ventilation 147
High-Frequency Ventilation 148
Extreme Modes of Gas Exchange 148
Extracorporeal Life Support 148
Intravascular Oxygenation 148
Extracorporeal Carbon Dioxide Removal 148
Liquid Ventilation 148
Investigational Adjuncts to Mechanical Ventilation 149
Prone positioning 149
Inhaled nitric oxide 149
Pharmacologic Adjuncts in Acute Respiratory Distress Syndrome 149
Management of Respiratory Failure 149
Manipulating the ventilator settings 150
Adjusting the partial pressure of carbon dioxide 150
Adjusting the partial pressure of oxygen 150
Weaning 150
Weaning failure 150
Complications of Mechanical Ventilation 151
Chapter 8: Extracorporeal Life Support for Cardiopulmonary Failure 152
Background 152
Indications 153
Methods of Extracorporeal Support 154
Extracorporeal Life Support Circuit 154
Patient Management 155
Cannulation for Cardiac Support 156
Complications 158
Results and Follow-Up 159
Future of Extracorporeal Life Support 161
Chapter 9: Neonatal Cardiovascular Physiology and Care 162
Cardiovascular Physiology 162
Heart rate 162
Preload 162
Afterload 163
Contractility 163
Fetal Circulation 163
Neonatal Management of Common Cardiovascular Problems 164
Congestive heart failure 164
Abnormalities in cardiac rhythm 167
Heart Block 167
Tachyarrhythmias 167
Management of selective types of congenital heart disease 168
Chapter 10: Sepsis and Related Considerations 170
Sepsis Terminology and Epidemiology 170
Terminology 170
Epidemiology 171
Pathogenesis 173
Host defense mechanisms 174
Barriers to Infection 174
Cell-Mediated Immunity 175
Neutrophils 175
Monocytes-Macrophages 175
Lymphocytes 176
Humoral Factors 177
Immunoglobulins 177
Complement System 177
Cytokines 177
Bacterial Virulence 178
Neonatal Host Defense 179
Diagnosis 181
Goldstein criteria 181
Age Group-Specific Definitions for Abnormal Vital Signs and Leukocyte Count 181
Definitions of the Pediatric Sepsis Continuum 181
Pediatric Organ Dysfunction Criteria 181
Diagnosis of neonatal sepsis 182
Biochemical markers 182
PIRO system 183
Management 183
Prevention 183
Early goal-directed therapy 183
Surviving Sepsis Campaign 183
American College of Critical Care Medicine/Pediatric Advanced Life Support Guidelines 183
Surviving sepsis campaign and american college of critical care medicine/pediatric advanced life support recommendations and ma 184
Initial Resuscitation 184
Airway, Breathing, and Circulation 184
Crystalloid Versus Colloid 184
Blood Products 187
Antibiotics 187
Source Control 188
Resuscitation Goals 188
Fluid-Refractory Shock 188
Stabilization and Continued Resuscitation 189
Catecholamine-Resistant Shock 189
Steroids 189
Cold Versus Warm Shock 190
Persistent Catecholamine-Resistant Shock 190
Refractory Shock 190
Other considerations 191
Intravenous Immunoglobulin 191
Recombinant Human Activated Protein C 191
Neonatal septic shock 191
Chapter 11: Surgical Implications of Hematologic Disease 194
Anemia 194
Nonhemolytic anemias 194
Marrow Failure 194
Blood Loss 196
Nutritional Anemias 196
Hemolytic anemias 197
Sickle Cell Diseases 197
-Thalassemia 197
Hereditary Spherocytosis and Erythrocyte Enzyme Deficiencies 198
Thrombocytopenia and Disorders of Platelet Function 198
Genetic thrombocytopenia 198
Acquired thrombocytopenia 198
Disorders of platelet function 199
Disorders of Coagulation 200
Coagulation factor deficiencies 200
Other factor deficiencies 201
Acquired defects of coagulation 202
Thrombotic disorders 203
Transfusion Therapy 204
Transfusion in patients with cancer or immunodeficiencies 205
Choice of red blood cell product 205
Transfusion reactions, toxicity, and other complications 205
Platelet Transfusion 206
Chapter 12: Nutritional Support in the Pediatric Surgical Patient 208
Normal Pediatric Growth 208
Nutritional Assessment 208
Biochemical measurements of nutritional status 209
Direct measurement of body composition 209
Nutritional Requirements 209
Energy requirements 209
Water 210
Protein 210
Carbohydrates 211
Fat 211
Minerals, trace elements, and vitamins 212
Fat-Soluble Vitamins 212
Vitamin A 212
Vitamin D 212
Vitamin E 212
Vitamin K 212
Water-Soluble Vitamins 212
Trace Elements 213
Zinc 213
Copper 213
Selenium 213
Manganese, Chromium, and Molybdenum 213
Enteral Nutrition 213
Indications 213
Delivery modalities 215
Enteral formulas 215
Administration of Enteral Nutrition 216
Human milk 216
Complications of enteral feeding 217
Parenteral Nutrition 217
Indications for parenteral nutrition 217
Venous access 217
Parenteral nutrition: composition and requirements 218
Amino Acids 218
Dextrose 218
Lipid Emulsions 218
Multivitamins 218
Trace Elements 219
Fluids and Electrolytes 219
Sodium 219
Potassium 219
Chloride and Acetate 219
Calcium and Phosphate 220
Additives to parenteral nutrition 220
Heparin 220
Histamine-2 Receptor Antagonists 220
Regular Insulin 220
Iron Dextran 220
Carnitine 220
Complications of parenteral nutrition 221
Metabolic Complications 221
Hyperglycemia 221
Hypoglycemia 221
Hypertriglyceridemia 221
Metabolic Acidosis 221
Electrolyte Disturbances 221
Metabolic Bone Disease 222
Hepatobiliary Complications 222
Infectious Complications 222
Complications from Overfeeding 223
Technical Complications 223
Administration of parenteral nutrition 223
Monitoring of Laboratory Values 225
Special Problems in the Nutritional Support of the Pediatric Surgical Patient 225
Nutrition in the pediatric surgical patient 225
Indications for preoperative nutrition 225
Indications for postoperative nutrition 225
Nutritional support in the critically ill surgical patient 226
Biliary atresia 226
Short-bowel syndrome 227
Obesity 227
Failure to thrive 227
Children with Special Care Needs 228
Chapter 13: Pediatric Anesthesia 230
Physiologic Considerations 230
Anesthetic Risk and Common Complications 230
Laryngospasm 232
Postoperative apnea 232
Obstructive sleep apnea syndrome 232
Preanesthesia Evaluation and Preparation 233
Preoperative fasting 233
Preanesthesia medications 233
Fluid Requirements 234
Maintenance fluid requirements 234
Premature or Critically Ill Infants 234
Intraoperative fluid replacement 234
Estimated Fluid Deficit 234
Role of Glucose 234
Choice of Intraoperative Fluid 235
Blood replacement 235
Estimating Allowable Blood Loss 235
Blood Products 235
Inhalation Anesthetic Agents 236
Minimum alveolar concentration 237
Nitrous oxide 237
Halothane 237
Isoflurane 237
Desflurane 237
Sevoflurane 237
Emergence Delirium 238
Neuromuscular Blocking Agents 238
Complicating conditions of depolarizing blocking agents 238
Malignant Hyperthermia 239
Intravenous Anesthetic Agents 240
Propofol 240
Thiopental 241
Ketamine 241
Etomidate 241
Monitoring 241
Noninvasive monitoring 241
Temperature Monitoring 242
Pulse Oximetry 242
Capnography 242
Monitoring Neuromuscular Function 242
Invasive monitoring 243
Pain Management 243
Perioperative planning and general approach 243
Development and physiology 243
Hypersensitization and preemptive analgesia 244
Pain assessment 244
Nonopioid analgesics 244
Acetaminophen 245
Nonsteroidal Antiinflammatory Drugs 245
Ketamine 246
Opioid analgesics 246
Oral Opioids 247
Intravenous Opioids 247
Patient-Controlled Analgesia 248
Regional Anesthesia 249
Topical anesthesia 250
Infiltration anesthesia 250
Peripheral nerve and plexus blocks 250
Rectus sheath block 251
Ilioinguinal-iliohypogastric block 251
Fascia iliaca block 252
Penile block 252
Neuraxial block 253
Caudal block 253
Continuous techniques 254
Comments 255
Chapter 14: Clinical Outcomes Evaluation and Quality Improvement 256
Study Design and Sources of Evidence 256
Case reports 256
Case series 257
Cross-sectional studies 257
Case-control study 257
Retrospective cohort 258
Prospective cohort 259
Prospective randomized controlled trial 259
Summaries of evidence 261
Review Articles 261
Meta-analysis 261
Systematic Reviews 261
Application of Clinical Evidence 262
Hypothesis testing 262
Subjective versus objective data and risk 262
Error 262
Identifying bias and determining validity 262
Guidelines and pathways 263
Outcomes and Measuring Quality of Care 263
Quality of care assessment 263
Specific Measurements 263
Improving Performance 264
Databases and Networks 264
Conclusions 265
Chapter 15: Ethical Considerations 266
Resolution of Ethical Dilemmas 266
Informed Consent and Assent 267
Prenatal Surgical Consultation 268
End of Life 269
Ethical Issues in Pediatric Bariatric Surgery 270
Surgeons and Industry 271
Multiculturalism 272
Surgical Error 273
Innovation and Research 274
Conclusion 275
Suggested readings 275
Chapter 16: Patient- and Family-Centered Pediatric Surgical Care 276
Definition 276
Background 276
Core Concepts 277
Respect and dignity 277
Communication 277
Participation 279
Preoperative Preparation 279
Intraoperative Period 279
Postoperative Care 280
Collaboration 280
Putting It All Together: PFCC in Action 281
Acknowledgments 281
Part II: Trauma 282
Chapter 17: Injury Prevention 284
Prevention Priorities 284
Injury prevention design strategies 286
Selected injury prevention initiatives 287
Child Passenger Safety 287
Fire Safety 287
Firearm Storage 288
Helmet Use 288
Pedestrian Injury 288
Poisoning 288
Measuring Success (Programmatic Evaluation) 289
Chapter 18: Infants and Children as Accident Victims and Their Emergency Management 290
Epidemiology of Childhood Injury 290
Resuscitation and Impact on Outcome 291
Resuscitation Principles 292
Prehospital care 292
Primary survey and treatment of life-threatening injuries 292
Airway and Cervical Spine Control 292
Breathing 294
Circulation and Vascular Access 295
Disability 297
Exposure 297
Resuscitation phase 297
Neuroresuscitation 297
Coagulopathy 298
Damage Control 299
Pain Management 299
Conclusion 299
Chapter 19: Thoracic Injuries 300
Epidemiology and Prevention 300
Clinical Presentation 301
Diagnosis and Initial Resuscitation 301
Treatment 303
Blunt injuries 304
Chest Wall 304
Soft Tissue 304
Rib Fractures 304
Flail Chest 304
Sternal Fractures 304
Lung and Airway 304
Pneumothorax 304
Simple Pneumothorax 304
Open Pneumothorax 304
Tension Pneumothorax 305
Hemothorax 305
Lung 306
Hematoma and Contusion 306
Laceration 306
Trachea and Bronchi 306
Esophagus 308
Diaphragm 308
Heart and Pericardium 309
Myocardial Contusion 310
Myocardial Rupture 310
Valve Injury 310
Pericardial Tamponade 311
Pericardial Laceration 311
Aorta and Great Vessels 311
Chylothorax 315
Traumatic Asphyxia 315
Penetrating injuries 315
Thoracoabdominal injuries 315
Transmediastinal injuries 316
Complications 316
Outcome 316
Summary 316
Chapter 20: Abdominal Trauma 318
Diagnostic Modalities 318
Computed tomography 318
Focused abdominal sonography for trauma 319
Diagnostic peritoneal lavage and laparoscopy 320
Solid Organ Injuries 320
Spleen and liver 320
Summary 323
Associated Abdominal Injuries 323
Complications of nonoperative treatment 323
Sequelae of damage-control strategies 323
Abdominal compartment syndrome 327
Bile duct injury 328
Injuries to the Duodenum and Pancreas 328
Duodenum 328
Pancreas 331
Injuries of the Stomach, Small Intestine, and Colon 334
Seat-belt sign 336
Imaging for gastrointestinal injury 336
Injuries to the Perineum, Anus, and Genitalia 337
Diaphragmatic Injuries 337
Chapter 21: Genitourinary Tract Trauma 340
Epidemiology 340
Mechanisms of Injury 340
Anatomic Considerations 340
Clinical Features 341
Diagnostic Evaluation 341
Injury Grading and Scoring Systems for Genitourinary Injuries 343
Management of Specific Injuries 344
Kidney 344
Blunt Injuries 344
Penetrating Injuries 344
Renovascular Injuries 345
Complications 346
Follow-Up and Outcomes 347
Operative Management of Renal Trauma 348
Ureter 348
Bladder 349
Anatomy 349
Causes 349
Classification and definitions 349
Diagnosis 350
Management 350
Bladder Contusions 350
Intraperitoneal Rupture 350
Extraperitoneal Rupture 350
Penetrating Injuries 351
Urethra 351
External Genitalia 353
Girls 353
Boys 353
Chapter 22: Musculoskeletal Trauma 356
Musculoskeletal Systems of Children and Adults 356
Anatomy 356
Biomechanics 356
Physiology 357
Evaluation of Musculoskeletal Injuries 358
Clinical assessment 358
Radiographic assessment 360
Management of Musculoskeletal Injuries 361
Immediate treatment 361
Definitive fracture management 361
High-Priority Musculoskeletal Injuries 361
Open fractures and traumatic arthrotomies 363
Compartment syndrome 363
Femoral neck fracture 363
Mangled extremities 364
Spine trauma 364
Child abuse 365
Chapter 23: Hand, Soft Tissue, and Envenomation Injuries 366
Hand and Soft Tissue Injuries 366
Evaluation 366
Vascularity 366
Peripheral Nerves 366
Skeleton, Tendons, and Ligaments 367
Soft Tissue 368
Early treatment 368
Vascular Structures 368
Peripheral Nerves 368
Skeleton, Tendons, and Ligaments 368
Soft Tissue 369
Amputations 369
Envenomation Injuries 369
Snakebites 369
Other bite injuries 370
Chapter 24: Central Nervous System Injuries 372
Basic Strategy of the Therapy of Central Nervous System Injury 372
Immediate Issues: Resuscitation and Transport of Injured Children 373
Traumatic Brain Injury 373
Epidemiology 373
Spectrum of traumatic brain injury 374
Focal or Diffuse Brain Injury? 374
Focal Brain Injury 374
Diffuse Brain Injuries 376
Gunshot Wounds 377
Crush Injuries 377
Inflicted Injuries 378
Initial assessment of brain-injured children 378
Early management of severe brain injury 379
Management of Minor Brain Injuries 380
Early complications of head injury 381
Complications of Skull Fractures 381
Basilar Skull Fractures 381
Direct Cerebrovascular Injuries 382
Post-traumatic Seizures 382
Postconcussion Syndromes 382
Outcomes from traumatic brain injury 382
Postconcussion Syndromes 383
Spine and Spinal Cord Injury 383
Epidemiology 383
Anatomy 383
Evaluation of spine and spinal cord injury 383
History 384
Physical examination 384
clinical spectrum of spine and spinal cord injury 384
Spinal Cord Injury Without Radiographic Abnormality 386
Initial management of spine and spinal cord injury 386
Spine Stabilization 386
Imaging 387
Plain Radiographs 387
Computed Tomography 388
Magnetic Resonance Imaging 388
Early management of spinal cord injury 388
Complications 388
Outcomes 389
Acknowledgments 389
Chapter 25: Vascular Injury 390
Epidemiology 390
Evaluation 391
Traumatic Injuries 392
Torso injuries 392
Extremity injuries 393
Mangled Extremity 394
Iatrogenic Injury 394
Vasospasm 395
Digital Ischemia Syndrome 396
Conclusion 396
Chapter 26: Burns 398
Pathophysiology 398
Acute Management 400
Initial evaluation 400
Fluid resuscitation 403
Inhalation injury 404
Burn Wound Care 405
Topical antimicrobials 405
Burn wound dressings 406
Excision and grafting 408
Hypermetabolic Response 409
Nutrition 410
Pharmacotherapy 411
Analgesia 411
Sedatives and anxiolytics 411
Intravenous antibiotics 412
Nonthermal Injuries 412
Chemical burns 412
Electrical burns 412
Outpatient Therapy 413
Rehabilitation 413
Chapter 27: Child Abuse and Birth Injuries 414
Child Abuse 414
Epidemiology 414
Caregiver or Parent 414
The Child 415
Family Dynamics 415
Presentation 415
Traumatic Brain Injury 416
Fractures 417
Burns 418
Thoracoabdominal Injury 419
Birth Injuries 420
Soft tissue injury 420
Fractures 420
Neurologic injury 421
Thoracoabdominal injury 421
Part III: Major Tumors of Childhood 424
Chapter 28: Principles of Pediatric Oncology, Genetics of Cancer, and Radiation Therapy 426
Epidemiology and Survival Statistics 426
Molecular Biology of Cancer 427
Normal cell physiology 427
Cell Cycle 427
Signal Transduction 427
Programmed Cell Death 428
Malignant Transformation 428
Abnormal DNA Content 429
Chromosomal Translocations 429
Proto-oncogene Activation 430
Inactivation of Tumor Suppressor Genes 430
Epigenetic Alterations 431
DNA Methylation 431
Histone Modification 431
MicroRNA 431
Metastasis 431
Angiogenesis 432
Molecular Diagnostics 432
Childhood Cancer and Heredity 433
Genetic Screening 434
General Principles of Chemotherapy 434
Risk stratification 435
Targeted therapy 435
Inhibition of Angiogenesis 439
Immunotherapy 440
General Principles of Radiation Therapy 440
Clinical considerations 440
Definitive Irradiation 440
Preoperative Irradiation 440
Postoperative Irradiation 441
Interactions of Chemotherapy and Radiation 441
Chemotherapeutic Enhancement of Local Irradiation 441
Irradiation Combined with Agents Having Limited or No Sensitizing Effect 441
Agents That Increase Radiation Toxicity 441
Fractionation of radiation therapy 441
Radiation therapy treatment techniques 441
Traditional Radiation Therapy 441
Focal Radiation Therapy 442
Conformal Radiation Therapy 442
Intensity-Modulated Radiation Therapy 442
Proton Beam Radiation Therapy 442
Brachytherapy 442
Palliative radiation therapy 442
Acute and late toxicities of radiation therapy 443
General Principles of Stem Cell Transplantation 444
Clinical Trials 444
Conclusion 445
Chapter 29: Biopsy Techniques for Children with Cancer 446
Handling of Specimens 446
Percutaneous Needle Biopsy 447
Fine-Needle Aspiration Biopsy 447
Core Needle Biopsy 447
Minimal Access Surgery 449
Laparoscopy 449
Thoracoscopy 449
Open Incisional Biopsy 451
Conclusion 451
Chapter 30: Wilms' Tumor 452
Wilms' Tumor 452
History 452
Epidemiology 453
Molecular Biology and Genetics 454
Loss of heterozygosity and DNA ploidy 454
TP53 Gene 455
Clinical Presentation 455
Diagnosis 456
Screening 456
Pathology 456
Pretreated tumors and pathology 457
Nephrogenic rests and nephroblastomatosis 458
Staging 458
Treatment 459
Prognostic factors 459
Operative therapy 459
Technical Concerns: Unilateral Tumors 459
Spill 460
Unresectable Tumors 460
Special considerations 460
Management of Tumor Extension in the Renal Vein, Inferior Vena Cava, and Atrium 460
Management of Tumor Extension in the Ureter 460
Horseshoe Kidney, Single Kidney, and Nonfunctioning Kidney 461
Patients with Wilms' Tumor Treated Only with Surgery 461
Neonatal Tumors 461
Acquired von Willebrand Disease in Children with Wilms' Tumor 461
Bilateral wilms' tumor 462
Chemotherapy 463
Recurrent Tumor 464
Radiotherapy 464
Lung Radiotherapy 465
Late effects 465
Pregnancy 465
Secondary Malignancies 465
Congestive Heart Failure 466
Thoracic 466
Other Renal Tumors 466
Clear cell sarcoma of kidney 466
Rhabdoid tumor of the kidney 466
Renal cell carcinoma 467
Congenital mesoblastic nephroma 467
Solitary Multilocular Cyst and Cystic Partially Differentiated Nephroblastoma 468
Cystic nephroma 468
Cystic partially differentiated nephroblastoma 469
Chapter 31: Neuroblastoma 470
Mass Screening 471
Clinical Presentation 471
Diagnosis 473
Staging 474
Pathology and Histology 474
Biologic and Genetic Alterations 477
Neuroblastoma in Infancy 478
Stage IV-S 479
Cystic Neuroblastoma 480
Multifocal and Bilateral Neuroblastoma 481
Risk Stratification and Risk-Based Management 481
Operative Management 482
Chemotherapy 485
Radiotherapy 485
Myeloablative Therapy 486
Immunotherapy 486
Additional Therapies 487
Summary and Future Directions 487
Chapter 32: Nonmalignant Tumors of the Liver 488
Clinical Presentation 488
Diagnosis 488
Laboratory tests 488
Imaging techniques 488
Hepatic Hemangioma 489
Focal liver hemangioma 489
Multifocal liver hemangioma 489
Diffuse lesions 489
Arteriovenous malformation 489
Mesenchymal hamartoma 490
Hepatocellular adenoma 490
Focal nodular hyperplasia 490
Teratoma 491
Inflammatory pseudotumor 491
Nonparasitic cysts 491
Chapter 33: Malignant Liver Tumors 492
Historical Context 492
Diagnosis 492
Clinical presentation 492
Differential diagnosis 493
Laboratory evaluation 493
Radiology 494
Malignant Liver Tumors 495
Hepatoblastoma 495
Epidemiology, Biology, and Genetics 495
Pathology 496
Pretext, staging, and risk group stratification 498
Treatment Strategy, Chemotherapy, and Surgery 499
Liver Transplantation for Hepatoblastoma 501
Transplantation Outcomes for Hepatoblastoma 502
Indications and Contraindications for Transplantation in Hepatoblastoma 502
Transplantation for Multifocal Hepatoblastoma 502
Major Venous Involvement: Transplantation versus Extreme Resection 503
Transplantation for Hepatoblastoma with Pulmonary Metastasis at Diagnosis 503
Rescue Transplantation for Local Relapse Hepatoblastoma 504
Type of Allograft and Immunosuppression 504
Pediatric Liver Unresectable Tumor Observatory (PLUTO) 504
New Agents and Treatment Modalities 504
Hepatic Arterial Chemoembolization (HACE), Transarterial Chemoembolization (TACE) 504
Ototoxicity 504
Hepatoblastoma Risk Stratification and International Collaboration 504
New Agents, Tumor Relapse 505
Hepatocellular carcinoma 505
Epidemiology, Biology, and Genetics 505
Pathology 505
Fibrolamellar Hepatocellular Carcinoma (FL-HCC) 506
Transitional Liver Cell Tumor (TLCT) 506
PRETEXT and Staging 506
Treatment Strategies 506
Liver Transplantation for Hepatocellular Carcinoma in Children 507
Outcomes, Indications, and Contraindications 507
Response to Chemotherapy 507
Milan Criteria 507
Metastatic Disease 508
Post-transplantation Chemotherapy 508
New Agents and Treatment Modalities 508
Antiangiogenesis, Sorafenib 508
Chemoembolization and Theraspheres 508
Portal Venous Embolization 509
Percutaneous Ablative Therapies 509
Hepatic Sarcomas 509
Biliary Rhabdomyosarcoma 509
Rhabdoid Tumor 509
Undifferentiated Sarcomas 509
Angiosarcoma 509
Aggressive Hemangiomatous Tumors 509
Locally Aggressive Infantile Hepatic Hemangioma 509
Metastatic and Other Liver Tumors 510
Metastatic Liver Tumors 510
Liver Tumors as Secondary Malignancies 511
Hemophagocytic Lymphohistiocytosis 511
Langerhans' Cell Histiocytosis 511
Megakaryoblastic Leukemia 511
Chapter 34: Pediatric Gastrointestinal Tumors 512
Esophageal Smooth Muscle Tumors 512
Esophageal and Gastric Adenocarcinoma 512
Gastrointestinal Stromal Tumors 513
Epidemiology 513
Clinical presentation 513
Pathology 513
Associated conditions 513
Treatment 513
Survival 514
Intestinal Tumors 514
Myofibromatosis 514
Lymphoma 514
Carcinoid Tumors 514
Epidemiology 514
Diagnosis 515
Treatment 515
Survival 515
Colorectal Adenocarcinoma 515
Polypoid Disease of the Gastrointestinal Tract 515
Hereditary Associations 516
Other Associations 518
Diagnosis 518
Sporadic Colorectal Carcinoma 518
Treatment 519
Summary 519
Chapter 35: Diagnosis and Treatment of Rhabdomyosarcoma 520
Rhabdomyosarcoma Patient Demographics 520
Rhabdomyosarcoma Tumor Biology 520
Presentation of Rhabdomyosarcoma 521
Preoperative Workup 521
Pretreatment Clinical Staging 522
Surgical Principles 522
Biopsy 522
Resection of the mass 523
Lymph node sampling/dissection 523
Clinical group 523
Second-look operations and aggressive resection for recurrence 524
Chemotherapy 524
Radiation Therapy 525
Assessment of Response to Treatment 525
Specific anatomic sites 525
Head and Neck (Superficial Nonparameningeal) 525
Parameningeal Sites 525
Trunk 525
Abdominal Wall 526
Chest Wall 526
Biliary Tract 526
Paraspinal Sites 526
Retroperitoneum/Pelvis 526
Perineal/Perianal Sites 526
Extremities 526
Genitourinary Sites: Bladder/Prostate 527
Genitourinary Sites: Vulva/Vagina/Uterus 527
Paratesticular Sites 527
Metastatic Disease 527
Prognosis 527
Chapter 36: Other Soft Tissue Tumors 530
Nonrhabdomyosarcoma Soft Tissue Sarcoma in Children: Background and Overview 530
Infantile fibrosarcoma 530
Synovial sarcoma 531
Malignant peripheral nerve sheath tumor 531
Surgical Approach and Presentation of Nonrhabdomyosarcoma Soft Tissue Sarcoma 531
Desmoplastic Small Round Cell Tumor 532
Desmoid Tumors 532
Dermatofibrosarcoma Protuberans 534
Chapter 37: Teratomas and Other Germ Cell Tumors 536
Embryology and Classification 536
Classification 536
Genetics and Risk Factors 537
Risk-Based Therapy 537
Testes 538
Clinical presentation and initial evaluation 538
Operative management 538
Postsurgical treatment 539
Ovary 539
Clinical presentation and evaluation 539
Sacrococcygeal Tumors 540
Clinical presentation and initial evaluation 540
Classification and association with malignancy 541
Surgical management 541
Postoperative management 542
Mediastinal Germ Cell Tumors 543
Abdominal and Retroperitoneal Germ Cell Tumors 545
Genital (Vaginal) Germ Cell Tumors 545
Cervicofacial Teratomas 545
Gastric Teratomas 545
Chapter 38: Hodgkin Lymphoma and Non-Hodgkin Lymphoma 546
Hodgkin Lymphoma 546
Incidence and epidemiology 546
Clinical presentation 547
Diagnosis 547
Histopathology 548
Staging 548
Treatment 549
Risk Classification 549
Surgery 549
Chemotherapy and Radiation Therapy 549
Therapy for Low-Risk Disease 550
Therapy for Intermediate-Risk Disease 550
Therapy for High-Risk Disease 550
Therapy for Lymphocyte-Predominant Hodgkin Disease 550
Novel Therapy 550
Treatment Toxicities 551
Growth Problems 551
Cardiopulmonary Dysfunction 551
Thyroid 551
Infertility 551
Second Cancers (SC) 551
Non-Hodgkin Lymphoma 551
Incidence epidemiology and classification 551
Clinical presentation and staging 552
Staging 552
NHL subtypes in children and adolescents 553
Mature B-cell NHL: Burkitt Lymphoma, Burkitt-Like Lymphoma, and Diffuse Large B-Cell Lymphoma 553
Burkitt Lymphoma and Burkitt-Like Lymphoma 553
Diffuse Large B-Cell Lymphoma 554
T-Cell Tumors 554
Lymphoblastic Lymphoma 554
Anaplastic Large T-Cell Lymphoma 554
Post-Transplant Lymphoproliferative Disorders 554
Treatment and outcomes 554
Chemotherapy for Non-Hodgkin Lymphoma 554
Surgery 555
Radiation Therapy 555
Burkitt Lymphoma and Burkitt-Like Lymphoma and Diffuse Large B-Cell Lymphoma 555
Lymphoblastic Lymphoma 555
Anaplastic Large Cell Lymphoma 555
Toxicities 556
Chapter 39: Ovarian Tumors 558
Ovarian Tumors Incidence 558
Epidemiology 558
Clinical Presentation 559
Diagnosis 559
Laboratory tests 559
Tumor Markers 559
Alpha Fetoprotein 559
Beta-Human Chorionic Gonadotropin 559
Serum Lactate Dehydrogenase 560
CA 125 560
Value of frozen section for intraoperative diagnosis 560
Immunohistochemistry 560
Cancer genetics 560
Role of Tumor Markers in the Incidentally Identified Ovarian Mass 561
Imaging techniques 561
Disease Classification and Staging 562
Treatment 564
Nonneoplastic ovarian tumors 564
Follicular Cysts 565
Corpus Luteum Cysts 565
Parovarian Cysts 566
Endometriosis 566
Neoplastic ovarian tumors 566
Surface Epithelial-Stromal Tumors 566
Tumors of Low Malignant Potential 567
Sex Cord-Stromal Tumors 568
Granulosa-Theca Cell Tumors 568
Fibromas and Thecomas 569
Sclerosing Stromal Tumors 569
Sertoli-Stromal Cell Tumors 569
Sex Cord Tumors with Annular Tubules 570
Steroid Cell Tumors 570
Germ Cell Tumors 570
Germinoma 570
Endodermal Sinus Tumors 572
Embryonal Carcinomas 572
Choriocarcinomas 572
Teratomas 572
Mature Teratomas 572
Immature Teratomas 573
Monodermal Teratomas 573
Gonadoblastomas 574
Mixed Germ Cell Tumors 575
Surgical Guidelines for Ovarian Germ Cell Tumors 575
Chemotherapy for Ovarian Germ Cell Tumors 575
Miscellaneous Tumors 576
Secondary Tumors 576
Unclassified Benign Tumors 577
Summary 577
Chapter 40: Testicular Tumors 578
Risk Factors for Testicular Cancer 578
Clinical Presentation 579
Diagnosis 579
Tumor Markers 579
Classification and Stage 579
Primary testicular tumors 579
Epithelial-Based Tumors 579
Epidermoid Cysts 579
Stromal Tumors 580
Sex Cord-Stromal Tumors 580
Germ Cell Tumors 580
Teratoma 580
Yolk Sac Tumor 580
Embryonal Carcinoma 581
Gonadoblastoma 581
Choriocarcinoma 581
Rhabdomyosarcoma 581
Secondary testicular tumors 581
Surgical Management 582
Testis-sparing surgery 582
Radical inguinal orchiectomy and retroperitoneal lymph node dissection 583
Chemotherapeutic strategies and survival in children with malignant germ cell tumors 585
Chapter 41: Adrenal Tumors 586
Anatomy 586
Embryology 586
Physiology 587
Adrenal medullary function 587
Adrenal cortical function 587
Lesions of the Adrenal Medulla 587
Pheochromocytoma 587
Symptoms 588
Diagnosis 588
Treatment 589
Associated Disorders 589
Lesions of the Adrenal Cortex 590
Cushing syndrome 590
Sex hormone-producing tumors 592
Treatment of adrenocortical tumors 592
Hyperaldosteronism 592
Addison Disease 593
Incidental Adrenal Mass 593
Adrenalectomy 593
Chapter 42: Tumors of the Lung and Chest Wall 596
Benign Tumors of the Lung 596
Plasma cell granuloma (inflammatory pseudotumor) 596
Hamartoma 596
Malignant Tumors of the Lung 596
Bronchial adenoma 596
Bronchogenic carcinoma 597
Pulmonary blastoma 598
Rhabdomyosarcoma 599
Comments 599
Treatment of Metastatic Disease 600
Osteosarcoma 600
Soft tissue sarcoma 600
Wilms' tumor 601
Comments 601
Tumors of the Chest Wall 601
Epidemiology 601
Presentation 601
Diagnostic adjuncts 602
Diagnosis 602
Therapeutic principles 602
Tumor types 602
Benign Chest Wall Tumors 602
Aneurysmal Bone Cyst 602
Chondroma 602
Desmoid 602
Fibrous Dysplasia 603
Mesenchymal Hamartoma 603
Osteochondroma 603
Malignant Chest Wall Tumors 604
Chondrosarcoma 604
Ewing Sarcoma Family/Primitive Neuroectodermal Tumors 604
Fibrosarcoma 604
Osteosarcoma 605
Rhabdomyosarcoma 605
Chapter 43: Bone Tumors 606
General Considerations 606
Pathophysiology 606
Benign Lesions 606
Size of the Tumor 607
Fracture Through a Benign Lesion 607
Location in Relation to the Physis 608
Multiplicity of Bone Tumors 608
Site of Involvement 608
Metastatic Potential 609
Malignant lesions 609
Epidemiology 609
Genetics 609
Diagnosis and staging 610
Clinical Evaluation 610
Radiology 610
Laboratory Evaluation 610
Preoperative Planning 610
Staging 611
Biopsy 611
Presurgical Considerations 611
Surgical Considerations 611
Postsurgical Considerations 611
Adjuvant therapy 612
Surgery 612
Minimally Invasive Options 613
Radiofrequency Ablation 613
Injection 613
Resection 613
Benign Lesions 614
Malignant Lesions 614
Reconstruction 616
Benign Lesions 616
Malignant Lesions 616
Chapter 44: Brain Tumors 620
Clinical Features 620
Radiographic Evaluation 621
Surgical Intervention 622
Tumor Types 623
Cerebellar astrocytomas 623
Primitive neuroectodermal tumor and medulloblastoma 623
Ependymomas 625
Brainstem gliomas 626
Hypothalamic/chiasmatic astrocytomas 626
Craniopharyngioma 627
Low-grade supratentorial astrocytomas 628
Pineal region tumors 629
Atypical teratoid/rhabdoid tumors 629
Malignant supratentorial astrocytomas 629
Choroid plexus tumors 629
Meningiomas 630
Metastases and dural-based masses 630
Tumor Genetics 630
Part IV: Transplantation 632
Chapter 45: Principles of Transplantation 634
Enigma of Acquired Tolerance 634
Phase 1: 1953 to 1968 634
``Pseudotolerant´´ Organ Recipients 634
False Premises of Phase 1 635
Phase 2: 1969 to 1979 636
Phase 3: 1980 to 1991 637
Phase 4: 1992 to Present 638
Historical Dogma 638
Discovery of Microchimerism 639
Immune Regulation by Antigen Migration and Localization 639
Therapeutic Implications 640
Organ Preservation 642
Procurement 642
Extended preservation 642
Continuous Vascular Perfusion 642
Static Preservation 643
Tissue Typing 643
Antigen matching 643
Crossmatching 644
Future Prospects 644
Chapter 46: Renal Transplantation 646
End-Stage Renal Disease in Children 646
Recipient Evaluation 646
Urologic Issues 648
Dialysis Access 648
Donor Selection 648
Evaluation of the potential living donor 649
Evaluation of the deceased donor 649
Transplantation 650
Preoperative preparation 650
Anesthesia 650
Operative procedure 650
Operative techniques 650
Small Children (<20.0kg) 650
Larger Children (20.0kg) 651
Ureteral Reconstruction in Patients with Previous Urologic Procedures 651
Postoperative Care 652
Evaluation of Early Allograft Dysfunction 652
Immunosuppression 653
Antibody preparations 653
Antilymphocyte Antibodies 653
Anti-interleukin-2 Receptor Monoclonal Antibodies 653
Calcineurin inhibitors 653
Mycophenolate 653
Prednisone 654
Proliferation signal inhibitors 654
Treatment of rejection 654
Outcomes 654
Graft and patient survival 654
Post-transplantation outcomes and risk factors associated with graft loss 655
Delayed graft function 655
Vascular thrombosis 656
Acute rejection 656
Chronic allograft nephropathy 656
Nonadherence 656
Recurrent disease 656
Medical complications 657
Infection 657
Cytomegalovirus 657
Varicella-Zoster Virus 657
BK Virus 657
Malignancy 657
Other Medical Issues 658
Cognitive and Psychosocial Development 658
Chapter 47: Pancreas and Islet Cell Transplantation 660
Pancreas Transplantation 660
History 660
Details of surgical techniques 661
General information, pancreas transplantation categories, and immunosuppression 663
Pancreas transplantation outcomes 664
Islet Transplantation 666
Islet autotransplantations after pancreatectomy for benign disease 667
Islet allotransplantations 667
Acknowledgments 670
Chapter 48: Liver Transplantation 672
Indications and Pretransplant Care 672
Indications for liver transplantation 672
Cholestatic liver disorders 673
Noncholestatic cirrhosis 673
Acute liver failure 674
Metabolic liver disease 674
Tumors 674
Miscellaneous conditions 674
Organ Allocation and Pretransplant Care 674
Donor Procurement and Hepatobiliary Anatomy 675
Hepatobiliary anatomy 675
Donor Operation 675
Segmental Liver Transplantation: Living Donor, Reduced Size, and Split 676
Liver Transplant Operation 676
Post-transplant Care 677
Early postoperative care 677
Technical Complications 678
Vascular complications 678
Biliary complications 678
Immunosuppressive Therapy and Rejection 678
Infectious Complications 679
Outcome and the Future 680
Assessment and Preparation for Intestinal Transplantation 682
Transplantation Surgery 682
Postoperative Care 683
Results of Intestinal Transplantation 684
Conclusion 686
Acknowledgments 686
Chapter 50: Heart Transplantation 687
Historical Notes 687
Indications 687
Preoperative Evaluation 689
Donor Evaluation and Organ Procurement 691
Recipient Preparation and Techniques of Implantation 692
Postoperative Management 693
Transplant Immunosuppression 693
Early Complications 695
Late Complications 696
Results 696
Conclusion 698
Chapter 51: Pediatric Lung Transplantation 699
Organ Allocation 699
Indications 699
Cystic fibrosis 699
Pulmonary vascular disease 700
Pulmonary fibrosis 701
Bronchiolitis obliterans and retransplantation 701
Miscellaneous 702
Contraindications 702
Special circumstances 703
Donor Evaluation and Organ Procurement 703
Technique of Transplantation 704
Immunosuppression 704
Post-transplantation Surveillance 705
Post-transplantation Complications 705
Airway anastomotic complications 705
Vascular anastomotic complications 705
Bleeding 706
Phrenic nerve injury 706
Hoarseness 706
Gastrointestinal complications 706
Atrial flutter 706
Graft complications 706
Infection 708
Other complications 708
Survival 708
Pulmonary Function and Growth 709
Future Considerations 709
Chapter 52: Surgical Implications Associated with Pediatric Bone Marrow Transplantation 711
Stem Cell Harvest and Vascular Access 711
Complications of Immune System Ablation and Immunosuppression 712
Intestinal complications 712
Hepatobiliary complications 712
Hemorrhagic cystitis 713
Pulmonary complications 713
Soft tissue infections 714
Post-transplantation Malignancies 714
Conclusion 714
Part V: Head and Neck 717
Chapter 53: Craniofacial Anomalies 719
The Craniosynostoses 719
Etiology and pathologic anatomy 719
Common Patterns of Single Suture Craniosynostosis 720
Syndromic Craniosynostosis 720
Diagnosis 721
Treatment 722
Orthognathic Surgery 722
Craniofacial Clefts 723
Cleft number 7 724
Treacher Collins Syndrome 725
Acknowledgments 726
Selected readings 726
Chapter 54: Understanding and Caring for Children with Cleft Lip and Palate 727
Epidemiology 727
Etiology 727
Embryology 727
Anatomy 727
Unilateral cleft lip 727
Bilateral cleft lip 728
Cleft palate 729
Treatment Protocols 729
Cleft lip surgical repair 730
Unilateral 730
Bilateral Cleft Lip Repair 731
Cleft palate surgical repair 731
Multidisciplinary Care 732
Secondary Cleft Management 733
Conclusions 734
Chapter 55: Otolaryngologic Disorders 735
Ear 735
Anatomy 735
Embryology 736
Examination 736
Otitis media with effusion and inflammatory disorders 737
Acute otitis media 737
Otitis media with effusion/chronic otitis media/chronic suppurative otitis media 738
Trauma 739
Tumors 740
Nose 740
Anatomy 740
Embryology 740
Inflammatory conditions 741
Bacterial rhinosinusitis 741
Complications of sinusitis 741
Fungal sinusitis 741
Congenital malformations 741
Pyriform Aperture Stenosis 741
Choanal Atresia 741
Nasal Dermoid 742
Nasal Glioma and Encephalocele 743
Trauma 743
Anosmia 743
Nasal Fracture 743
Nasal Foreign Bodies 743
Nasal Lacerations 743
Nasal/Nasopharyngeal tumors 743
Oral Cavity/Pharynx 744
Anatomy 744
Acute pharyngotonsillitis 744
Recurrent pharyngotonsillitis 745
Chronic pharyngotonsillitis 745
Oral trauma 745
Peritonsillar cellulitis/Abscess 745
Retropharyngeal/Parapharyngeal space infections 746
Sleep-Disordered breathing 746
Ankyloglossia 748
Macroglossia 748
Benign lesions 748
Malignant lesions 749
Larynx 750
Anatomy 750
Upper airway assessment 750
Congenital laryngeal anomalies 751
Inflammatory disease of the upper airway 753
Chronic airway obstruction 754
Benign laryngeal neoplasms 754
Neck 754
Anatomy 754
Clinical evaluation 754
Congenital tracts and cysts 755
Inflammatory and infectious masses 755
Malignant neoplasms 756
Chapter 56: Salivary Glands 757
Classification 757
Embryology 757
Anatomy and Physiology 757
Pathology 757
Diagnosis 757
History 757
Physical examination 757
Diagnostic imaging 758
Computed Tomography 758
Magnetic Resonance Imaging 758
Biopsy 758
Sialendoscopy 758
Inflammatory Disease 759
Viral sialadenitis 759
Bacterial suppurative sialadenitis 759
Chronic sialadenitis 759
Cystic Disease 759
Ranula 760
Neoplasms 760
Benign neoplasms and malformations 760
Hemangiomas 760
Lymphatic Malformations 760
Pleomorphic Adenoma 760
Monomorphic Adenomas 761
Papillary Cystadenoma Lymphomatosum (Warthin Tumor) 761
Malignant neoplasms 761
Mucoepidermoid Carcinoma 761
Acinic Cell Carcinoma 761
Adenoid Cystic Carcinoma 761
Rhabdomyosarcoma 761
Surgical Considerations 762
Parotid gland 762
Submandibular gland 763
Conclusion 763
Chapter 57: Lymph Node Disorders 765
Anatomy 765
Differential Diagnosis 765
Malignancy 765
Evaluation 765
Investigation 767
Laboratory Studies 767
Radiologic Evaluation 767
Diagnostic Procedures 767
Fine-Needle Aspiration 767
Excisional Biopsy 768
Management of Adenopathy 768
Surgical Management 768
Acute Lymphadenitis 768
Persistent Lymphadenitis 769
Atypical Mycobacterial Adenitis 769
Mycobacterial Adenitis 770
Cat-Scratch Disease 770
Miscellaneous Lesions 771
Infectious Lymphadenopathy 771
Inflammatory Disorders 771
Malignant disorders 771
Summary 771
Chapter 58: Childhood Diseases of the Thyroid and Parathyroid Glands 773
Thyroid Embryology and Physiology 773
Evaluation of the Thyroid Gland 773
Non-neoplastic Thyroid Conditions 774
Goiter 774
Thyroiditis 775
Hypothyroidism 775
Hyperthyroidism 775
Neoplastic Thyroid Conditions 776
Management of thyroid nodules 776
Well-differentiated thyroid carcinoma 776
Medullary thyroid cancer 778
Parathyroid Embryology and Physiology 778
Disorders of the Parathyroid Glands 779
Digeorge syndrome 779
Hyperparathyroidism 779
Parathyroid Carcinoma 780
Chapter 59: Neck Cysts and Sinuses 781
Embryology 781
Thyroglossal Duct Cysts 783
Surgical management 784
Branchial Anomalies 785
Second branchial anomalies 785
First branchial anomalies 786
Third and fourth branchial anomalies 786
Dermoid Cysts 788
Congenital Midline Cervical Clefts 788
Cervical Thymic Cysts 788
Chapter 60: Torticollis 791
History 791
Etiology 791
Pathology 791
Clinical Features 791
Sternomastoid torticollis 791
Differential diagnosis 792
Secondary Effects of Torticollis 793
Plagiocephaly 794
Hemifacial hypoplasia 794
Postural compensation 794
Conservative Management 794
Operative Treatment 795
Indications for surgery 795
Operative technique 795
Complications 795
Follow-up 795
Index 796
Expertconsult 844
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