Pediatric Vitreoretinal Surgery 2023

Foreword Preface Acknowledgements List of Videos Contents Contributors General Guidelines for Pediatric Vitreoretinal Surgery 1 Embryology of Developmental Surgical Disorders of the Posterior Segment Abstract Introduction Development of the Retina Development of the Vitreous Development of the Optic Nerve Retinopathy of Prematurity Persistent Fetal Vasculature Congenital Cavitary Optic Disc Anomalies Optic Nerve Coloboma Optic Disc Pit Morning Glory Optic Disc Peripapillary Intrachoroidal Cavitation Retinochoroidal Coloboma Microphthalmos Nanophthalmos Review Questions SN Flashcard References 2 Surgical Anatomy of Pediatric Eyes: Differences from Adults Abstract Introduction Anterior Segment Differences Pars Plana Development Axial Eye Length Pediatric Lens Intraoperative Pressure Control During Pediatric Vitrectomy Pediatric Sclera: Implications for Sclerotomies and Scleral Buckling in Children Pediatric Hyaloid and Vitreous Pediatric Retinal Vasculature Retina and Retinal Pigment Epithelium Adherence in the Pediatric Eye Pediatric Anatomical Anomalies Retinopathy of Prematurity Persistent Fetal Vasculature Vitreoretinopathies Clinical Case Review Questions References 3 Preoperative Visual Assessment in Neonates and Preverbal Children Abstract Introduction Visual Acuity Assessment Fixation The Induced Tropia Test (ITT) Optokinetic Nystagmus (OKN) Preferential Looking Test (PLT) Visual Evoked Potential (VEP) Conclusion Review Questions References 4 Surgical Decision Making in Pediatric Retinal Diseases Abstract Introduction General Considerations Scleral Buckling Vitreous Surgery Vitreous Infusion Vitreous Tamponades and Replacements Pharmacologic Adjuncts Conclusion References 5 Preparation for the Surgery: Preoperative Measures Abstract Introduction Informed Consent in Pediatric Patients Types of Interventions in an Operating Room Planning the Follow-Up Clinical case scenario Conclusion Review Question References 6 Vitreoretinal Surgical Equipment and Instruments Why and How Are They Different in Children? Abstract Introduction Anatomical and Physiological Differences in Children Preoperative Considerations Draping Speculum Buckle Elements Sclerotomies Visualization System MIVS (Microincision Vitrectomy Systems) Modifications Suture Materials Eye Shield Conclusion Review Questions References 7 Philosophical Considerations in Pediatric Vitreoretinal Surgery Abstract Introduction Training Expectations and Rapport—the Physician: Parent + Patient Interaction Surgical Considerations Skill Acquisition and Transfer Aftercare Considerations Conclusion References Conservative Interventions for Pediatric Retinal Diseases 8 Examination Under General Anesthesia in Children Abstract Introduction General Anesthetic Drugs Components of Anterior Segment Examination Intraocular Pressure Measurement Corneal Diameter Measurement Corneal Thickness Anterior Segment Examination Gonioscopy Refraction Posterior Segment Examination Checklist for EUA Commonly Used Agents in EUA Advantages of EUA Case Scenario Which Needs Frequent EUA Review Questions References 9 Imaging in Pediatric Eyes Abstract Introduction Types of Imaging Modalities Fundus Photography Autofluorescence Fluorescein Angiography Indocyanine Green Angiography Optical Coherence Tomography Optical Coherence Tomography Angiography Ultrasonography Ultrasound Biomicroscopy Review Question References 10 Laser Photocoagulation of the Retina in Babies and Children Abstract Introduction Our Experience Instruments for Laser Coagulation Laser Treatment Step-By-Step: (Figs. 10.9, 10.10, 10.11, 10.12, 10.13, 10.14, 10.15, 10.16, 10.17, 10.18 and 10.19) The Surgery in Detail In Conclusion Angiography Assisted Laser Photocoagulation for Newborn and Children The Technique Step-by-Step (Fig. 10.20): Review Questions References 11 Intravitreal Injections for Pediatric Retinal Diseases Abstract Introduction Indications for Anti-VEGF in ROP Indications for Anti-VEGF in FEVR Indications for Anti-VEGF in Coats’ Disease Considerations in Anti-VEGF Injections in ROP Technique for Anti-VEGF Injection in ROP Follow up for Anti-VEGF Injection in ROP Conclusion Case Discussion Review Questions References 12 Cryotherapy for Pediatric Vitreoretinal Diseases Abstract Introduction Background Principle Mechanism of Action Indications Cryotherapy for Surface Eye Pathology Benign Pathology Pre-Malignancy and Malignancy Cryotherapy for Intraocular Pathology Contraindications Technical Consideration Equipment Cryotherapy Probes Patient Preparation Anaesthesia Positioning Application of Cryoprobe Complications Indications of Cryotherapy in Retinopathy of Prematurity Outcome Indications of Cryotherapy in Coats Disease Indications of Cryotherapy in Retinoblastoma Outcome Indications of Cryotherapy in Toxoplasmosis Outcome Indications of Cryotherapy in Rhegmatogenous Retinal Detachment Review Questions References 13 Prophylactic Treatment of High Risk Fellow Eyes in Pediatric Vitreoretinopathies Abstract Introduction Stickler Syndrome Indication of Prophylaxis Methods of Prophylaxis Outcomes After Prophylaxis Laser Cryotherapy Scleral Buckling Prophylaxis Failure Follow-Up Case Presentation Traumatic Retinal Detachment Associated with Self-Injurious Behavior Giant Retinal Tears Adult Retinopathy of Prematurity and other Pediatric Vitreoretinopathies Conclusion Review Questions References Retinopathy of Prematurity (ROP) 14 Classification of Retinopathy of Prematurity Abstract Classification of Retinopathy of Prematurity Location of Vascularization The Plus Disease Spectrum Stage of Disease Acute Phase Disease Stage 1–Demarcation Line Stage 2–Ridge Stage 3–Extraretinal Neovascular Proliferation Aggressive ROP (A-ROP) Retinal Detachment (Stages 4–5) Stage 4: Partial Retinal Detachment Stage 5: Total Retinal Detachment Extent of Disease Regression, Reactivation, and Long-term Sequelae Regression Reactivation Long-Term Sequelae Conclusion Review Questions References 15 Major Clinical Trials in ROP Abstract Introduction Interventional Clinical Trials on Treatment of ROP Interventional Clinical Trials on Prevention of ROP Oxygen Light Nutrition Pharmacological Prevention of ROP Non-interventional Clinical Trials on ROP Conclusion Review Questions References 16 Types of ROP: Threshold, Prethreshold, Type 1, 2, Aggressive-ROP Abstract Introduction Epidemiology Pathophysiology of ROP Hypoxia-Inducible Factor (HIF) Maternal Factors, Nutrition and Extrauterine Growth Restriction VEGF and Receptors Terminology Review Questions References 17 Type 1 ROP Management: Laser Versus Anti-VEGF Injection Abstract Advantages of Laser Treatment Disadvantages of Laser Treatment Anti-VEGF Treatment Advantages of Anti-VEGF Drugs Disadvantages Clinical Case Scenario (Fig. 17.5) Conclusion Review Questions References 18 ROP Treatment in Complex Presentation Abstract Introduction: General Principles of Approaching Complexity Systemic Medical Barriers to Diagnosis and Treatment Local: Ophthalmic Complex Presentation of ROP Socioeconomic Complexity Adjustment in Clinical Practice for the Complex Presentation Review Questions: (May Have More Than 1 Correct Response) References 19 Vitreoretinal Surgery for Stage 4 ROP Abstract Case Presentation Introduction Goals of Surgery in Stage 4 ROP Pre-operative Considerations Timing Laser Status Laterality Surgical Setup Considerations Intraoperative Considerations Operative Strategy (See the Video) Surgical Setup and Entry Operative Considerations Maneuvering in the Crowded Operative Field Closure Postoperative Considerations Surgical Outcome and Prognosis Conclusions Return to Case Presentation Review Questions References 20 Vitreoretinal Surgery for Stage 5 ROP Abstract Introduction Surgical Technique Scleral Buckling Surgical Principles of Vitrectomy Adjuncts to Vitreoretinal Surgery Bilateral Sequential Surgery Complications of Vitreoretinal Surgery Surgical Outcomes Case Presentation Conclusions Review Questions References 21 Vitreoretinal Surgery for Cicatricial Retinopathy of Prematurity Abstract Introduction The International Classification for ROP (ICROP) Staging of Cicatricial ROP Poor Prognostic Factors Related to Retinal Detachment Pre-Operative Evaluation Surgery for Cicatricial ROP Scleral Buckling Lens-Sparing Vitrectomy (LSV) Macular Folds Surgical Technique for Management of Macular Folds Lensectomy—Vitrectomy Surgical Technique Results of LSV and Non-lens Sparing Vitrectomy for Cicatricial ROP Case Scenario Supplemental digital contents (SDC) Review Questions References 22 Complications of Vitreoretinal Surgery for ROP Abstract Introduction Taking the Decision for Surgery Vitrectomy for Stage 4 ROP Improving Outcomes in Stage 4 ROP Surgery Improving Outcome in Stage 5 ROP Case Scenario Review Questions References 23 Genetics of Retinopathy of Prematurity from the Point of a Surgical Approach Abstract Introduction Identifying a Genetic Predisposition Wnt Signaling Wnt Signaling and Placental Insufficiency Retinopathy of Prematurity or Familial Exudative Vitreoretinopathy Trophic Factors in Retinal Development Future Directions—Genome Sequencing for ROP Clinical Considerations Conclusions Review Questions References 24 Late Visual Prognosis in ROP Survivors Abstract Late Visual Prognosis in ROP Survivors Visual Acuity Reactivation Retinal Detachment and Other Vitreoretinal Sequelae Myopia Amblyopia Cataract Glaucoma Cortical Visual Impairment and Other Neurologic Comorbidities Screening and Prevention Techniques Conclusion Review Questions References Surgical Management of Pediatric Retinal Vascular Disorders 25 Familial Exudative Vitreo-Retinopathy Abstract Introduction Review Questions Funding/Support References 26 Coats’ Disease Abstract Introduction Epidemiology Clinical Characteristics and Stages Differential Diagnosis and Investigations Treatment Options Prognosis and Visual Outcomes Review Questions References 27 Incontinentia Pigmenti Abstract Introduction Background Epidemiology Pathophysiology Clinical Features Medical Management Laser Photocoagulation Therapy Cryotherapy Intravitreal Anti-vascular Endothelial Growth Factor Surgical Management Tractional RD Repair (see the video, by courtesy of Şengül Özdek) Rhegmatogenous RD Repair Conclusion Review Questions References 28 Norrie Disease Abstract Introduction Diagnosis Clinical Features Genetic Testing Prenatal Testing Differential Diagnosis Management Medical management Surgical management Prognosis Clinical Scenario Case 1 Case 2 Conclusion Revision Questions References 29 Sickle Cell Retinopathy Abstract Introduction Background Epidemiology Pathophysiology Clinical Features Medical Management Surgical Management Peri-Operative Considerations Surgical Considerations Conclusion Review Questions References Surgical Management of Developmental Disorders 30 Persistent Fetal Vasculature Syndrome Abstract Introduction Normal Embryology Clinical Presentation Diagnosis Differential Diagnosis Natural Course of the Disease Treatment PFV Surgery: General Considerations Clinical Scenarios and Surgical Strategies Case Presentation 1. Mild Anterior PFV Case Presentation 2. Severe Anterior PFV Case Presentation 3. Anterior PFV Presenting with a Pseudo-Hyaloidal Stalk [20] Case Presentation 4. Posterior PFV with Tent-Shaped TRD Case Presentation 5. Severe Mixed PFV with Closed Funnel-Shaped TRD Conclusions Review Questions References 31 Retinochoroidal Coloboma Abstract Introduction Background Epidemiology Typical/Atypical Histopathology of Retinochoroidal Coloboma Clinical Features Vitreoretinal Complications Retinal Breaks Surgery for Retinal Detachment Case Presentation (see the Video) Conclusion Review Questions References 32 Optic Nerve Head-Related Maculopathy Abstract Introduction Optic Disc Pits Clinical Case Scenario Optic Disc Coloboma Morning Glory Disc Anomaly Clinical Scenario Take Home Messages Review Questions References Pediatric Rhegmatogenous Retinal Detachment 33 Genetic Diseases Causing RRD: Marfan, Stickler and Wagner Syndrome Abstract Marfan Syndrome Epidemiology Pathophysiology/Genetics Clinical Features Management Prognosis Stickler Syndrome Epidemiology Pathophysiology Clinical Features Management Prognosis Wagner Syndrome Epidemiology Pathophysiology Clinical Features Management Review Questions References 34 Pediatric Traumatic Retinal Detachments Abstract Introduction Diagnosis Clinical Profile Assessment Management Surgical Tips Timing to Surgery Treatment Choice Intraoperative Considerations Incision Management of the Lens Management of the Posterior Hyaloid Proliferative Vitreoretinopathy Tamponade Choice Self-inflicted RD Outcomes Complications Predictors of Visual and Anatomic Success Conclusion Review Questions References 35 Retinopathy of Prematurity Related Late Rhegmatogenous Retinal Detachment Abstract Introduction Background Clinical Features Retinal Detachment Pathophysiology Management Conclusion Review Questions References 36 Congenital X-Linked Retinoschisis and Retinal Detachment Abstract Background Epidemiology Pathophysiology Clinical Findings Classification Imaging and Electrophysiology Genetics Differential Diagnosis Management Case Presentation: (See Video 2) Gene Therapy Conclusions Review Questions References 37 Congenital Glaucoma Related Retinal Detachment Abstract Introduction Pathophysiology Clinical Characteristics Investigations Examination Under General Anesthesia and Treatment Planning Surgical Indications and Surgical Challenge Special Instruments in Congenital Glaucoma Related Retinal Detachment Surgery Postoperative Follow-Up Surgical Outcomes Long-Term Follow-Up Surgical Case Example Conclusion Review Questions References 38 Non-syndromic Pathologic Myopia Associated Rhegmatogenous Retinal Detachment in Pediatric Eyes Abstract Introduction Clinical Features Management Review Questions References 39 Giant Retinal Tear Related Retinal Detachment Abstract Introduction Epidemiology Pathophysiology Surgical Treatment Outcomes and Potential Complications Case Presentation Conclusions Review Questions References 40 Scleral Buckling Surgery for Pediatric RRD Abstract Introduction The Surgical Technique Case Presentations Conclusion Review Questions References 41 Combined Buckling-Vitrectomy Surgery for Pediatric Rhegmatogenous Retinal Detachment Abstract Introduction General Considerations for Surgery Intraoperative Considerations for Vitrectomy Posterior Hyaloid Separation and Membranes Tamponade Considerations Post-Operative Considerations Surgical Outcomes Conclusions Surgical Video Courtesy of Prof. Dr. Şengül Özdek Review Questions References 42 Pneumatic Retinopexy: Is There a Role in Pediatric Rhegmatogenous Retinal Detachment (RRD)? Abstract Introduction Role of Pneumatic Retinopexy Selection Criteria Potential Complications Pneumatic Retinopexy Follow-up Summary Review Questions References Pediatric Ocular Trauma 43 Closed Globe Injuries Abstract Introduction Background Epidemiology Pathophysiology Clinical Features Special Cases for Pediatric Closed Globe Injury Review Questions References 44 Open Globe Injuries Abstract Introduction Terminology Epidemiology Evaluation Special Recommendations History General Implications External Inspection Slit Lamp and Ophthalmoscopy Imaging Studies Counseling Management and Decision-Making Timing The Extent of the Primary Surgery Surgical Reconstruction Conjunctiva Cornea Sclera Anterior Chamber Iris and Pupil Lens Ciliary Body Vitreous and Retina Endophthalmitis Case Report Review Questions References 45 Posttraumatic Endophthalmitis Management at Pediatric Age Abstract Introduction Epidemiology and General Considerations on Pediatric Post-traumatic Endophthalmitis Diagnosing PTE Management of PTE Treatment Prognosis Conclusions Review Questions Appendix 1: Tap and Inject Technique Appendix 2: How to Prepare Intravitreal Antibiotics References 46 Pediatric Intraocular Foreign Body Management Abstract Introduction Assessment Management Conclusion Review Questions References 47 Management of Proliferative Vitreoretinopathy in Pediatric Trauma Related Vitreoretinal Surgery Abstract Introduction What is Different in Pediatric Eyes Compared to Adults? Management of PVR Case Presentation Review Questions References 48 Pediatric Macular Holes Abstract Introduction Etiology and Pathophysiology Clinical Features and Diagnosis Management Case Scenario 1: Traumatic Macular Hole Case Scenario 2: Macular Hole Secondary to Pars Planitis (Fig. 48.3) Case Scenario 3 Conclusion Review Questions References 49 Shaken Baby Syndrome Abstract Introduction Epidemiology Pathophysiology Clinical Features Differential Diagnoses Medical Management Workup Treatment Clinical Case Surgical Management Prognosis Conclusion Review Questions References 50 Management of Vitreous Hemorrhage at Pediatric Age Abstract Introduction Case 1 Case 2 Conclusions Review Questions References Inflammatory Disorders in Children 51 Ocular Toxoplasmosis Abstract Introduction Epidemiology Life Cycle and Mode of Transmission Clinical Presentation and Phenotypes Congenital Ocular Toxoplasmosis Acquired Toxoplasma Infection Characteristics of Pediatric Cases that Differ from Adults Diagnosis Management Indications for Medical Treatment Conclusion Clinical Case (With Courtesy of Prof. Dr. Şengül Özdek [43]) Review Questions References 52 Ocular Toxocariasis Abstract Background Epidemiology Pathophysiology Clinical Manifestations Diagnosis Medical Treatment Surgical Treatment Conclusion Review Questions References 53 Pars Planitis in Children Abstract Introduction Epidemiology Signs and Symptoms—Clinical Presentation Diagnosis Management Medical Surgical Complications Cataract Macular Edema Epiretinal Membrane Peripheral Neovascularization Vitreous Hemorrhage and Visually Obscuring Vitreous Opacities Retinoschisis Retinal Detachment Prognosis Review Questions References 54 Other Inflammatory Disorders in Children Abstract Pediatric Uveitis: Posterior and Panuveitis Management of Complications from Uveitis Epiretinal Membrane Hypotony Posterior Capsule Opacification Clinical Case Scenario Review Questions References Approach to Cataract and the Crystalline Lens in Pediatric Retinal Surgery 55 To Preserve or Not to Preserve the Crystalline Lens Abstract Introduction Retinopathy of Prematurity Persistent Fetal Vasculature Syndrome Pediatric Rhegmatogenous Retinal Detachment Case Scenario Conclusion Review Questions References 56 General Guidelines for Lens Surgery at Pediatric Age Abstract Introduction Critical Periods Amblyopia Surgery Planning When to Do Surgery? What Type of Surgery? How to Do Surgery? IOL Power Selection Parent Counseling Immediate Postoperative Care Long Term Care Case Scenario Review Questions References 57 Complications of Lens Surgery at Pediatric Age Abstract Introduction Epidemiology Intraoperative Complications Iris Prolapse and Damage Bleeding Posterior Capsule Rupture Retained Cortex and Lens Fragment in the Vitreous Postoperative Complications Ocular Hypertension and Glaucoma Persistent Corneal Edema Secondary Opacification of Visual Axis: Capsular Opacity, Lens re-Proliferation and Pupillary Membranes Pupil Abnormalities Dislocated Lens Retinal Detachment Endophthalmitis Phthisis Bulbi Video Case Conclusions Review Questions References 58 Secondary IOL Implantation at Pediatric Age Abstract Introduction Contraindications IOL Power Calculation in Children Surgical Techniques When Capsular Support is Adequate When Capsular Support is Insufficient 2 Point Fixation Versus 4 Point Fixation Sutureless Intrascleral Fixation (SL-SFIOL) Complications of Secondary IOLs Review Questions References 59 Management of Aphakia at Pediatric Age Abstract Introduction Aphakic Glasses Contact Lenses Intraocular Lenses (IOL) Intraocular Lens Power Calculation Case Scenarios Conclusions Review Questions References Retinoblastoma 60 Genetic Counseling Abstract Introduction Retinoblastoma Genetics Hereditary Versus Non-hereditary RB MYCN as a Genetic Cause of Non-hereditary Retinoblastoma Secondary Chromosomal Alterations that Cause Tumorigenesis Genetic Testing for Retinoblastoma: Current and Future Applications The Impact of Hereditary Retinoblastoma: Tumor Predisposition Syndrome and Increased Risk for Secondary Cancers Unilateral Hereditary Clinical Case Bilateral Hereditary Clinical Case Counselling for Families with Retinoblastoma Conclusion Review Questions References 61 Differential Diagnosis of Retinoblastoma Abstract Introduction Coats’ Disease Persistent Fetal Vasculature (PFV) Familial Exudative Vitreoretinopathy Astrocytic Hamartoma Medulloepithelioma Ocular Toxocariasis Review Question References 62 Treatment Modalities and Enucleation Abstract Introduction Treatment Modalities Focal Treatment Modalities Transpupillary Thermotherapy Cryotherapy Chemotherapy Intravenous Chemotherapy (IVC) Intra-Arterial Chemotherapy (IAC) Intravitreal Chemotherapy (IVitC) Intracameral Chemotherapy Radiotherapy Plaque Brachytherapy External Beam Radiotherapy Enucleation Conclusion Clinical Case Scenario Review Questions References Miscellaneous 63 Pediatric Choroidal Neovascularization Abstract Introduction Epidemiology Etiology CNV Characteristics Management Conclusion Example Case Review Questions References 64 Nanophthalmos in Children Abstract Introduction Ultrasound Biomicroscopy Review Questions References 65 Management of Deformed Blind Eye Abstract Introduction Examination of Microphthalmic Eye: The Oculoplastic Surgery Perspective Management of Microphthalmic Blind Eye Anophthalmia, Microphtlamia, and Orbital Growth Intraocular Tumor Endophthalmitis Sympathetic Ophthalmia Implant Selection Conclusions Review Questions References 66 Management of Combined Hamartoma of the Retina and Retinal Pigment Epithelium in Children Abstract Introduction Non-surgical Management Surgical Management Conclusion Review Questions References 67 Telemedicine for Pediatric Retinal Disorders: Retinopathy of Prematurity Screening Programs Abstract Introduction Background Approach to Screening Case Presentation Future Applications Conclusion Review Questions References 68 Artificial Intelligence for Pediatric Retinal Diseases Abstract Introduction AI in Pediatric Retina Beginnings of AI for ROP AI for ROP Today Future of AI: Retinal Surgery Barriers to Implementation Conclusion Review Question References Visual Rehabilitation 69 Refractive Rehabilitation Abstract Introduction How to Check Refractive Error Options for Correction of Refractive Error Refractive Challenges in Children with Retinal Pathologies and/or Prior Vitreoretinal Surgery Case Scenario Conclusion Review Questions References 70 Amblyopia Management Abstract Introduction General Remarks Classification Special Remarks on Pediatric Vitreoretinal Problems Diagnosis Management Conclusions Review Questions References 71 Low Vision Aids Abstract Introduction Interventions Non-optical Aids Visual Habilitation Case Conclusion Review Questions References 72 Guidance for Social/School Life and Future Directions Abstract Introduction Background Low Vision Incidence and Comorbidities Epidemiology Psychosocial Impact of Visual Impairment on Quality of Life Impact on Academics Clinical Case Physical Activities and Restrictions in Children with Low Vision Vision Rehabilitation on Pediatric Low Vision: Devices and Specialists Resources for Pediatric Low Vision Practical Guidance and Future Directions Conclusion Review Questions Acknowledgements References