Congenital Cystic Lung Disease : Comprehensive Understanding of Its Diagnosis and Treatment From Fetus to Childhood

This book offers readers a comprehensive understanding of congenital cystic lung disease based on a novel classification system recently proposed by leading researchers in the field. Presented in detail here, it includes various aspects, from fetal diagnosis and treatment to postnatal diagnosis and treatment, while also taking into account endoscopic surgical treatment and pathological diagnosis. Given the depth and breadth of its coverage, __Congenital Cystic Lung Disease__ will be of interest not only to pediatric surgeons but also to pediatric respiratory physicians, neonatologists, and obstetricians engaged in fetal diagnosis and therapy, as well as pediatric radiologists and pathologists. Preface 5 Contents 7 1: Novel Classification of Congenital Cystic Lung Disease 9 1.1 Introduction 9 1.2 Novel Classification Based on the Embryology 10 1.3 Pathology Secondary to Lung Maldevelopment 13 1.4 Precise Definition of Each Entity of CCLD and Exclusion of the Unfit Lesions 13 1.5 Future Aspect of the Novel Classification 15 References 16 2: Epidemiology of Congenital Cystic Lung Disease: From Japan Nationwide Survey 17 2.1 Perinatal Clinical Features 18 2.2 Later Onset of Clinical Symptoms in Asymptomatic Neonates 21 2.3 Surgery, Complications, and Clinical Outcome 21 2.4 Respiratory Function Measured During Late Postoperative Period 22 2.5 Lung Lesions and Pathological Diagnosis 22 2.6 Discussion 23 References 24 3: Fetal Diagnosis and Therapy for Congenital Cystic Lung Disease 26 3.1 Introduction 26 3.2 Prenatal Diagnosis of CLCD 27 3.2.1 Origin of the Blood Supply 27 3.2.2 Appearance of the Lung Cyst 28 3.2.3 Lung Cystic Lesion Size 28 3.2.4 Presence of Hydrops and Changes in the Appearance 29 3.3 Fetal Therapy 30 3.3.1 TAS 30 3.3.2 Fetal Surgical Resection 30 3.3.3 Maternal Steroid Therapy 31 3.3.4 Other Fetal Therapies 31 References 31 4: Fetal Diagnostic Imaging of Congenital Cystic Lung Disease 33 4.1 Introduction 33 4.2 Safety, Magnetic Fields, and Gestational Age in Fetal MRI 34 4.3 Fetal MR Image Acquisition Technique and Normal Lung Anatomy 34 4.4 Image Findings 35 4.4.1 Congenital Pulmonary Airway Malformation (CPAM) 35 4.5 Bronchopulmonary Sequestration (BPS) 39 4.6 Bronchial Atresia (BA) 40 4.7 Congenital Lobar Emphysema (CLE) 42 4.8 Congenital Bronchogenic Cyst 42 4.9 Summary 42 References 43 5: Perinatal Care for Severe Congenital Pulmonary Airway Malformation 44 5.1 Introduction 44 5.2 Prenatal Management and Clinical Course 45 5.2.1 Classification Based on Pathological Findings and Fetal Sonographic Appearance 45 5.2.2 Evaluation of Cystic Lesion Size Using Fetal Ultrasonography 46 5.2.3 Treatment of Fetuses with CPAM 46 5.2.4 Response to Fetal Treatment and Condition Just Before Birth 46 5.3 Management of Neonates with CPAM at Birth and Infancy 47 5.3.1 Emergency Surgery Strategy 48 5.3.2 Points and Pitfalls of Each Department’s Role in Emergency Surgery Strategy for Neonates with Severe CPAM 48 5.3.2.1 Department of Obstetrics for Fetal Medicine 48 5.3.2.2 Department of Neonatology 48 5.3.2.3 Department of Anesthesia 49 5.3.2.4 Department of Surgery 49 5.4 Management of Patients with CPAM in Infancy and Childhood 49 5.5 Discussion and Future Perspective 50 References 51 6: Postnatal Imaging Diagnosis of Congenital Cystic Lung Disease 53 6.1 Introduction 53 6.2 Diagnostic Modalities 53 6.2.1 Chest Radiography 53 6.2.2 Computed Tomography: CT 54 6.2.3 Magnetic Resonance Imaging: MRI 54 6.2.4 Ultrasound: US 54 6.3 Bronchial Obstruction 54 6.3.1 Congenital Bronchial Atresia 54 6.3.2 Infantile Lobar Emphysema/Congenital Lobar Emphysema 55 6.4 Congenital Pulmonary Airway Malformation: CPAM 55 6.5 Bronchopulmonary Sequestration: BPS 57 6.6 Foregut Duplication Cysts 58 References 60 7: Perinatal Natural History and Treatment of Congenital Lung Malformations in Prenatally Diagnosed Neonates 61 7.1 Introduction 61 7.2 Diversity in Congenital Lung Malformations 62 7.3 Bronchial Atresia as a Distinct Entity 62 7.4 Postnatal Natural History, Treatment, and Prognosis 63 References 66 8: Surgical Treatment for and Prognosis of Congenital Pulmonary Airway Malformation 67 8.1 Introduction 67 8.2 Definition 68 8.3 Classification 68 8.4 Prevalence 68 8.5 Differences Between CPAM and Other CCLDs 69 8.5.1 Anatomical Differences 69 8.5.2 Pathophysiological Differences 69 8.6 Symptoms and Pathophysiology 70 8.7 Neonatal Onset Cases 70 8.7.1 Perioperative Management for Neonatal Onset Cases 70 8.7.2 Surgical Treatment for Neonatal Onset Cases 71 8.8 Asymptomatic or Postnatal Onset Cases 71 8.8.1 Surgical Indications for Asymptomatic Cases 71 8.8.2 Timing of Surgery for Asymptomatic Cases 71 8.8.3 Surgery for Postnatal Onset Cases 72 8.9 Selection of Surgical Procedures 72 8.10 Surgical Approach and Technique 73 8.11 Outcome 73 8.12 Complications 73 References 74 9: Surgical Treatment for Bronchial Obstruction 76 9.1 Classifications 77 9.2 Clinical Presentation 77 9.3 Diagnosis 78 9.4 Treatment (Surgical and Medical) 79 9.5 Pathology 80 9.6 Prognosis 80 References 80 10: Intralobar Pulmonary Sequestration 82 10.1 Introduction 82 10.2 Our Cases 83 10.2.1 The Experience of Treatment of Pediatric ILS Cases in Our Center from 2002 to 2019 83 10.3 Previous Case Series 83 10.4 Clinical Aspects 84 10.5 Treatment 86 10.6 Prognosis 88 References 88 11: Surgical Treatment and Its Prognosis for Extra-lobar Pulmonary Sequestration Without Foregut Communication 90 11.1 Introduction 90 11.2 Location and Features of ELS 91 11.2.1 Torsion 91 11.2.2 Intrapericardial ELS 91 11.2.3 Intraabdominal ELS 92 11.2.4 Intradiaphragmatic ELS 92 11.3 Treatment 92 11.3.1 Indications 92 11.3.1.1 Symptomatic ELS 92 11.3.1.2 Asymptomatic ELS 92 11.3.2 Surgery 93 11.3.2.1 Thoracotomy 94 11.3.2.2 Endoscopic Surgery 94 VATS/Thoracoscopic Surgery 94 Laparoscopic Surgery 95 11.3.3 TAE 95 11.3.4 Conservative Management 95 11.4 Prognosis 95 11.5 Conclusion 96 References 96 12: Surgical Treatment and Its Prognosis for Extralobar Pulmonary Sequestration with Foregut Communication: Communicating Bronchopulmonary Foregut Malformation (CBPFM) 98 12.1 Introduction 98 12.2 Definition 99 12.3 Epidemiology 99 12.3.1 Classification and Affected Side [7] 100 12.3.2 Gestation and Family History [7] 100 12.4 Pathogenesis 101 12.5 Anatomical Characteristics 101 12.6 Clinical Findings and Symptoms 101 12.6.1 Evaluation and Diagnosis 101 12.7 Treatment 102 12.8 Outcome 103 12.9 Conclusion 103 References 103 13: Endoscopic Surgery for Pediatric Congenital Cystic Lung Disease 105 13.1 Introduction 105 13.2 Indications for Surgery 105 13.3 Preoperative Imaging Studies 105 13.3.1 Computerized Tomography 105 13.3.2 Chest X-Ray Radiography 106 13.4 Factors that Will Affect Surgery 107 13.4.1 Status of Fissures 107 13.4.2 Past History of Chest Infections 107 13.4.3 Other Factors 107 13.5 Tips for Facilitating TPL 108 13.5.1 Patient Preparation and Positioning 108 13.5.2 Trocar Insertion 108 13.5.3 Trocar Positions and Switching Instruments 108 13.6 Treating an Incomplete Fissure 110 13.6.1 Right Lung 110 13.6.2 Left Lung 112 13.6.3 Delivering the Excised Lung 112 13.6.4 Stapling the Bronchus 112 13.7 Surgical Techniques for TPL 113 13.7.1 Left Lower Lobectomy 113 13.7.2 Right Lower Lobectomy 113 13.7.3 Left Upper Lobectomy 114 13.7.4 Right Upper Lobectomy 115 13.7.5 Right Middle Lobe Lobectomy 116 13.8 Postoperative Care 116 13.9 The Authors’ Experience 117 13.10 In Closing 118 References 118 14: Pathology of Congenital Cystic Lung Diseases 119 14.1 Bronchogenic Cyst 119 14.2 Bronchial Atresia/Infantile Lobar Emphysema 120 14.2.1 Bronchial Atresia 120 14.2.2 Infantile (Congenital) Lobar Emphysema (Overinflation) 121 14.3 Congenital Pulmonary Airway Malformation 122 14.3.1 CPAM Type 0 123 14.3.2 CPAM Type 1 123 14.3.3 CPAM Type 2 124 14.3.4 CPAM Type 3 125 14.3.5 CPAM Type 4 126 14.4 Sequestration 127 14.4.1 Intralobar PS 128 14.4.2 Extralobar PS Without Foregut Communication 129 14.4.3 Extralobar PS with Foregut Communication 130 14.5 Pulmonary Interstitial Emphysema 131 14.6 Other Rare Diseases 131 14.6.1 Alveolar Capillary Dysplasia 131 14.6.2 Fetal Lung Interstitial Tumor 132 References 134 15: The Long-Term Outcome of Congenital Cystic Lung Disease 135 15.1 Thorax Deformation and Scoliosis 136 15.2 Pectus Excavatum 137 15.3 The Respiratory Function 137 15.4 Developmental Assessment 138 15.5 Pneumonia and Other Complications 138 15.6 Pneumothorax 139 15.7 Malignancy 139 References 140 Front Matter ....Pages i-viii Novel Classification of Congenital Cystic Lung Disease (Tatsuo Kuroda)....Pages 1-8 Epidemiology of Congenital Cystic Lung Disease: From Japan Nationwide Survey (Yasushi Fuchimoto)....Pages 9-17 Fetal Diagnosis and Therapy for Congenital Cystic Lung Disease (Haruhiko Sago)....Pages 19-25 Fetal Diagnostic Imaging of Congenital Cystic Lung Disease (Osamu Miyazaki)....Pages 27-37 Perinatal Care for Severe Congenital Pulmonary Airway Malformation (Yushi Ito)....Pages 39-47 Postnatal Imaging Diagnosis of Congenital Cystic Lung Disease (Kumiko Nozawa)....Pages 49-56 Perinatal Natural History and Treatment of Congenital Lung Malformations in Prenatally Diagnosed Neonates (Toshihiko Watanabe)....Pages 57-62 Surgical Treatment for and Prognosis of Congenital Pulmonary Airway Malformation (Noriaki Usui)....Pages 63-71 Surgical Treatment for Bronchial Obstruction (Hiroomi Okuyama)....Pages 73-78 Intralobar Pulmonary Sequestration (Yutaka Kanamori)....Pages 79-86 Surgical Treatment and Its Prognosis for Extra-lobar Pulmonary Sequestration Without Foregut Communication (Kyoko Mochizuki, Masato Shinkai, Norihiko Kitagawa, Hidehito Usui)....Pages 87-94 Surgical Treatment and Its Prognosis for Extralobar Pulmonary Sequestration with Foregut Communication: Communicating Bronchopulmonary Foregut Malformation (CBPFM) (Kosaku Maeda)....Pages 95-101 Endoscopic Surgery for Pediatric Congenital Cystic Lung Disease (Hiroyuki Koga, Atsuyuki Yamataka)....Pages 103-116 Pathology of Congenital Cystic Lung Diseases (Mio Tanaka, Emi Takakuwa)....Pages 117-132 The Long-Term Outcome of Congenital Cystic Lung Disease (Yuko Tazuke)....Pages 133-140