Management of Head and Neck Vascular Lesions. A Guide for Surgeons

Patient’s Testimony
Acknowledgments
Contents
List of Contributors
1: History, Terminology, and Classifications of Vascular Anomalies
	1.1	 Introduction
	1.2	 History
	1.3	 Incidence
	1.4	 Terminology and Classifications
		1.4.1	 Historical Terminology on Appearances
		1.4.2	 Histopathologic Terms Used Historically
	1.5	 Mulliken and Glowacki’s Biological Classification (1982)
	1.6	 Vascular Malformations (Figs. 1.5 and 1.6)
		1.6.1	 Classifications
	1.7	 CNS and Non-CNS List of Vascular Lesions
	1.8	 Schobinger Classification
		1.8.1	 Jackson’s Classification
		1.8.2	 Surgical Management Based Classification for Head and Neck Lesions
	1.9	 Conclusion
	References
2: Pathogenesis, Genetics, and Molecular Developments in Vascular Lesion Therapy and Diagnosis
	2.1	 Introduction
	2.2	 Pathogenesis
	2.3	 Genetic Basis of Vascular Lesions with Mutation and Inheritance
		2.3.1	 Mutations (Table 2.1, See Also Fig. 2.6 Consolidated Review)
		2.3.2	 Cancer and Vascular Lesions
	2.4	 VEGF and PDGF, Growth factors of Raf, Ras in Tyrosine class receptors
	2.5	 G Protein-related Spectrum
	2.6	 PIK3CA-related Overgrowth Spectrum (PROS) [31–36]
	2.7	 Mutations and Vascular Lesions: The Essentials
	2.8	 Genetic Testing and Advances in the Diagnosis of Vascular Anomalies [3, 4, 26, 30, 31]
		2.8.1	 Test Type and Reasons
	2.9	 Biopsy and Immunohistochemistry
		2.9.1	 GLUT-1 and the Importance of the Vascular Lesion Identification
		2.9.2	 GLUT 1 Receptor (Fig. 2.5)
	2.10	 VEGF -3, D2 -40, PROX 1, LYVE -1
	2.11	 CD31 and CD 34 Antibodies
		2.11.1	 Factor VIII Related Antigen
	2.12	 Next-generation Sequencing and Its Promise for Vascular Lesions
		2.12.1	 DNA Sequencing, What his Next-generation Sequencing?
		2.12.2	 Sanger Method
	2.13	 Next-Generation Sequencing (NGS)
		2.13.1	 #1 Copy Number Variations (CNV)
		2.13.2	 #2 Single Nucleotide Variation (SNV)
	2.14	 Cell-free DNA (cfDNA) Testing in Prenatal Conditions for Vascular Anomalies
	2.15	 Therapeutic Hypotheses for the Treatment of Vascular Diseases [39–41, 46–48, 51] (Fig. 2.7)
		2.15.1	 Targeting the PI3K/AKT/mTOR Pathway
		2.15.2	 Sirolimus (Rapamycin)
		2.15.3	 mTOR (Mammalian Target for Rapamycin) [30, 31, 39–45, 50, 52]
	2.16	 Conclusion
	References
3: Syndromes Associated with Vascular Anomalies
	3.1	 Syndromes Associated with Vascular Tumours
		3.1.1	 PHACE Syndrome (Fig. 3.1)
		3.1.2	 Kasabach–Merritt Syndrome/Phenomenon
	3.2	 Syndromes Associated with Capillary Malformations
		3.2.1	 Sturge–Weber Syndrome (Fig. 3.2a, b)
		3.2.2	 Klippel–Trenaunay Syndrome
		3.2.3	 Rubinstein–Taybi Syndrome (Fig. 3.3a, b)
		3.2.4	 Beckwith–Wiedemann Syndrome (Fig. 3.4a, b, c)
	3.3	 Syndromes Associated with Low-flow Venous Malformations, Lymphatic Malformations or Mixed Malformations
		3.3.1	 Blue Rubber Bleb Nevus Syndrome (Fig. 3.5)
		3.3.2	 Parkes Weber Syndrome
		3.3.3	 Proteus Syndrome (Figs. 3.6, 3.7)
		3.3.4	 Maffucci Syndrome
		3.3.5	 Gorham–Stout Syndrome (Fig. 3.8a, b)
	3.4	 Syndromes Associated with High-flow Vascular Malformations
		3.4.1	 Rendu–Osler–Weber Syndrome (Hereditary Haemorrhagic Telangiectasia)
		3.4.2	 Bonnet–Dechume–Blanc Syndrome or Wyburn–Mason Syndrome
	3.5	 Summary
	References
4: Radiological Diagnosis of Head and Neck Vascular Anomalies
	4.1	 Introduction
	4.2	 Variations in the Radiological Presentations of Vascular Lesions of the Head and Neck Region
	4.3	 Imaging Modalities
		4.3.1	 Ultrasound
		4.3.2	 Magnetic Resonance Imaging
	4.4	 Major Imaging Finding of Vascular Tumors
		4.4.1	 Infantile Hemangioma
		4.4.2	 Congenital Hemangioma
		4.4.3	 Kaposiform Hemangioendothelioma
	4.5	 Low-flow Vascular Malformations
		4.5.1	 Venous Malformation
		4.5.2	 Lymphatic Malformation
		4.5.3	 Capillary Malformation
	4.6	 High-flow Vascular Malformations
		4.6.1	 Arteriovenous Malformation (AVM)
	References
5: Medical Management of Vascular Lesions: Current and the Future
	5.1	 General Approach for Treatment
	5.2	 Goals of Treatment [1]
	5.3	 Uncomplicated Hemangiomas
		5.3.1	 Orbital, Mucosal and Maxillofacial Vascular Tumors/Infantile Hemangiomas
			5.3.1.1	 Beta-blockers
			5.3.1.2	 Contraindications
	5.4	 Special Situations
		5.4.1	 Ulcerated Hemangiomas (Fig. 5.3a, b)
		5.4.2	 Sclerosing Agents (Table 5.1)
		5.4.3	 Bleomycin
		5.4.4	 Protein Chain in a Vascular Cell that Transmits Signal from a Cell Surface Receptor to the DNA in the Cell Nucleus
		5.4.5	 Sirolimus/Rapamycin
	References
6: The Role of Interventional Radiology
	6.1	 Outline
		6.1.1	 Section 1: Diagnostic Imaging of the Head and Neck
		6.1.2	 Section 2: General Interventional Radiology [1–18]
			6.1.2.1	 Vascular Access
			6.1.2.2	 Embolization [9–18, 20–35]
		6.1.3	 Section 3: Nonvascular Procedures of the Head and Neck
		6.1.4	 Section 4: Vascular Procedures of the Head and Neck
			6.1.4.1	 Management of Acute Hemorrhage
		6.1.5	 Case: Epistaxis from Traumatic Nasotracheal Intubation Requiring Embolization (Fig. 6.2)
			6.1.5.1	 Case: Refractory Epistaxis of Unknown Etiology
			6.1.5.2	 Case: Large Left Glomus Vagale Paraganglioma Treated with Embolization (Fig. 6.4)
		6.1.6	 Section 5: Complications and Post-procedure Management
			6.1.6.1	 Additional Case of Direct Stick embolization
	References
7: General, Surgical, and Functional Anatomy for Vascular Lesions of Head and Neck
	7.1	 Layers of Scalp, Face, and Neck (Superficial to Deep)
	7.2	 Important Nerves of Head and Neck
	7.3	 Arterial Supply of Head and Neck (Figs. 7.9, 7.10, and 7.11)
		7.3.1	 Vessel and Luminal Dimensions
		7.3.2	 Maxillary Artery Branches
		7.3.3	 Arterial Supply of the Neck
	7.4	 Venous Drainage of Head and Neck (Fig. 7.12)
	7.5	 Lymphatic Drainage of Head and Neck (Fig. 7.13)
		7.5.1	 Approaches to the Face for Vascular Tumors and Malformations
	7.6	 Approaches to Upper Face and Temporal Region
		7.6.1	 Approaches to the Temporal Region (Fig. 7.18)
		7.6.2	 Approaches to Parotid
		7.6.3	 Supra Orbital Approach
		7.6.4	 Upper Eyelid Approach
	7.7	 Approaches to the Midface
		7.7.1	 Subciliary Incision
		7.7.2	 Lateral Rhinotomy Incision
		7.7.3	 Nasolabial with Subciliary Extension
		7.7.4	 Weber–Fergusson Approach
		7.7.5	 Intraoral Approach to Maxilla
		7.7.6	 Midface Degloving Approach
	7.8	 Approaches to the Lower Face
		7.8.1	 Mandibular Vestibular Approach
		7.8.2	 Visor Incision
		7.8.3	 Submandibular Approach
	7.9	 Mandibular Access Osteotomy [35]
	References
		Section A
		Section B
8: Surgical Management
	8.1	 Introduction
	8.2	 Decision-Making
	8.3	 Case Series
	8.4	 Technique of External Carotid Artery (ECA) Control [1]
	8.5	 Combination of ECA Control with Intralesional Embolisation
	8.6	 Corset Suturing Technique [2]
	8.7	 Complications
	8.8	 Conclusion
	References
9: Reconstruction
	9.1	 Introduction
	9.2	 Indications for Reconstruction and Tissue Requirements
	9.3	 Methods of Reconstruction
		9.3.1	 Type of Lesion
		9.3.2	 Type of the Defects
			9.3.2.1	 Bony Defects
			9.3.2.2	 Soft Tissue Defects
		9.3.3	 Timing of Reconstruction
		9.3.4	 Options of Reconstruction
	9.4	 Use of Tissue Expanders
	9.5	 Free Tissue Transfer in Reconstruction of These Defects
		9.5.1	 Pedicle Selection in Free Tissue Transfer
		9.5.2	 Prevention of Flap Failures
		9.5.3	 Advantages of Free Tissue Transfer apart from its Reconstructive Benefits
	9.6	 Computersied Planning, Surgical Guides and Patient-Specific Implants
	9.7	 Points to remember while undertaking reconstruction of defects after resection of major vascular malformations
10: Lasers and Nonsurgical Modalities
	10.1	 Introduction
	10.2	 History of Laser and Current Modalities
		10.2.1	 Flash Lamp Pumped Pulsed Dye Laser (585-nm, 595-nm)
		10.2.2	 Frequency-Doubled Q-Switched Nd:YAG/KTP Laser (532 nm)
		10.2.3	 Long-Pulsed Nd:YAG Laser (1064-nm)
		10.2.4	 CO2 Laser (10,600-nm)
		10.2.5	 Diode Laser (532-, 800-, 810-, 940-, and 980-nm)
	10.3	 Laser Application in Vascular Anomalies
		10.3.1	 Capillary Malformations
		10.3.2	 Infantile Hemangiomas (IHs)
		10.3.3	 Telangiectasia
		10.3.4	 Venous Malformations
	10.4	 Conclusion
	References