1 vascular anomalies

05/02/2023vascular lesions of head and neck , v.lakshm

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VASCULAR LESIONS

Presented by Y Vijaya lakshmiOMFS

05/02/2023vascular lesions of head and neck , v.lakshmi

2INTRODUCTION

Before the 1980s, the terminology that was used to describe vascular anomalies was confusing and ambiguous.

terminology used in the past port wine stain strawberry haemangioma salmon patch conjure up visual approximation to the lesions but have no correlation with the biological

behavior or natural history of these lesions


3Mulliken and Glowacki (1982)

Based on the clinical,histochemical,cellular criteria to distinguish between the various vascular anomalies.

1. HAEMANGIOMAS 2. VASCULARMALFORMATIONS.3


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Modification of the original classification to include Predomonant vessel type The depth of the lesion Characteristics of flowClinically useful correlates with pathological & Radiological data


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HAEMANGIOMASSuperficial (capillary haemangiomaa)Deep (cavernous haemangiomaa)Compound (capillary cavernous haemangiomaa)

VASCULAR MALFORMATIONS

Simple lesions

Low-flow lesionsCapillary malformation (capillary haemangioma, port-wine stain)Venous malformation (cavernous haemangioma)Lymphatic malformation (lymphangioma, cystic hygroma)

High-flow lesionsArterial malformation

Combined lesionsArteriovenous malformationsLymphovenous malformationsOther combinations


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INFANTILE CONGENITAL RICH NICH


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Based on anatomical presentation : Type I – Mucosal/cutaneous Type II – Submucosal/subcutaneous

Surgical management of vascular lesions of the head and neck: a review of 115 casesInt. J. Oral Maxillofac. Surg. 2011; 40: 577–583

http://dx.doi.org/10.1016/j.ijom.2011.02.005


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Type III – Glandular ( LVM/VM + SG Type IV – Intraosseous Type V – Deep visceral ( para pharyngeal , infratemporal)


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Type I – Mucosal/cutaneous lesion Excision with overlying skin or mucosa Primary closure or regional flap

Type II – Submucosal or subcutaneous Surgical access to lesion with total excision and primary closure

Type III – Lesions involving glands ex-parotid/ 5 submandibular Surgical access to glandular lesions with excision along with the involved gland and primary closure (Fig. 4A–D)

Type IV – Skeletal – involving the facial skeleton ex-maxilla/mandible/zygoma

Excision of involved skeletal structure with reconstruction

Type V – Deep visceral ex-parapharyngeal/ infratemporal Mandibulotomy to access the lesion followed by total excision

Surgical management of vascular lesions of the head and neck: a review of 115 casesInt. J. Oral Maxillofac. Surg. 2011; 40: 577–583

http://dx.doi.org/10.1016/j.ijom.2011.02.005


11HEMANGIOMA vs MALFORMATIONS

INFANTILE HEMANGIOMA Benign tumor Endothelial cell over proliferation Not always present at birth Rapid growth phase as the child

Involution during childhood

VASCULAR MALFORMATION Congenital dysplastic vessel Normal endothelial cell function Always present at birth but not

evident Grow with child , possible rapid

growth phase No involution


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During fetal life, capillary plexuses morphologically differentiate into arteries, veins, and lymph channels while unneeded vessels are destroyed through apoptosis

PATHOGENESIS


13IMMUNOHISTOCHEMISTRY MARKER

GLUT 1 VEGF CYCLIN EGFR


14EPIDERMOLOGY

CAUCESIANS LOW BIRTH WEIGHT FEMALE PRE MATURITY MULTIPLE GESTATIONS


15CLINICAL PRESENTATION

Present as blanching of skin Telengectiasis Appearance depend on depth of lesion Papillary dermis – violaceous / crimson red Reticular dermis – raised smooth lesion with bluish hue / normal color FIRM RUBBERY NON COMPRESSIBLE


16History and clinical examination


17Grey-scale ultrasound and Doppler analysis

defining whether the lesion is solid or cystic to establish the presence or absence of high flow vessels


18MAGNETIC RESONANCE IMAGING

extent of the lesion Better contrast between the lesion and surrounding tissues has multiplanar capabilities distinguish between the different types of vascular anomalies.


19Contrast-enhanced computed tomography

evaluating intraosseous lesions bony margins of extensive lesions that are under consideration for resection.


20Digital subtraction angiography (DSA)

mapping out the blood supply of the lesion assessment of the characteristics of flow of arteriovenous malformations therapeutic endovascular interventions

submandibular hi-flow AVM


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the left mandibular AVM with feeder vessels from both the facial (A) and lingual (B) arteries.


22Haemangioma

Most common “ tumour” in white infants (10–12%) head and neck region – common (60%) solitary (80%) girls - boys (3:1) Multiple cutaneous lesions (three or more) are often associated with visceral involvement Facial haemangiomas have a predilection for segmental distribution and for regions of embryological fusion

Haemangiomas and vascular malformations of the maxillofacial region—A review British Journal of Oral and Maxillofacial Surgery 44 (2006) 263–272


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Haemangiomas usually appear soon after birth (though up to 30% may be present at birth proliferate during the first year of life involute during the childhood years (up to 12 years)according to the depth of the lesion as superficial deep compound haemangioma


24Superficial papillary dermisbright red macular or papular masses

Capillary/ strawberry haemangioma

DEEP reticular dermis or subcutaneous tissues bluish or relatively colourless masses cavernous haemangioma

COMPOUNDsuperficial and deep components capillary cavernous haemangiomas.


25Clinical presentation

Present at birth or within the first year of life Color depends on vessel type Bright red – purple Soft and compressible May present with bleeding, infection, lymphatic leakage Trauma may result in sudden enlargement Spontaneous hemorrhage Pain occurs in 50% of patients Sudden pain may occur from thrombosis


26NATURAL HISTORY

LIFE CYCLE - 3 PHASES

PROLIFERATIVE PHASE ( rapid growth 2wks – 1 yr ) INVOLUTING PHASE ( slow regression 1- 7 yrs ) INVOLUTED PHASE ( complete regression after 8 yrs)

Hall mark is PROLIFERATIVE phase followed by involution phase Involution begins at 6- 10 months complete by 10 yrs 50 % cases resolve by 5 yrs 70% cases by 7 yrs


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28Histology• proliferating endothelial

cells• highly cellular pericytes• high mitotic activity • numerous mast cells• Vascular channels are

not prominent

proliferative phase

• endothelial cells are flattened

• cell turnover is normal • few mast cells• Vascular channels filled

with blood cells predominate

involutive phase


29Treatment

useful approach to the management of haemangiomas can be based on stage of the lesion (proliferative or involutive phase), type of lesion (superficial, deep, compound) management of residual deformity

Active intervention - disfiguring haemangiomas


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Prednisolone is the first line drug of choice Steroids are useful only in the proliferative phase Interferon 2a – potentially serious side effects


31Proliferative phase

Only in infants Superficial haemangiomas - pulsed dye laser repeated at 4–6 weekly intervals later stages of proliferation acquire a thickness beyond the depth of penetration of yellow light lasers Interstitial potassium-titanylphosphate (KTP) (Nd:YAG)


32Involutive phase

wait and watch policy until 3–4 years of age. involute completely by 6 years (rapid involuters) - no further treatment 60% of cases slow involution – need treatment in 80% of cases Steroids are not effective against involuting haemangiomas Superficial lesions - small and intermediate size vessels - pulsed dye laser Larger vessels - copper bromide, Nd-YAG laser deep haemangiomas – Resection compound lesions - superficial component - laser photocoagulation deep - excised


33Residual deformity

adolescent and adult patients

common manifestations are telangiectasia, fibrofatty masses of tissue, epidermal atrophy.


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TELANGIECTASIASMALL VESSELS

red blush pulsed dye laser

medium large vessels copper bromide, pulsed KTP laser

Nd:YAG laser


35Fibrofatty tissue

Excision can be more vascular than expected thermoscalpel and contact laser (Nd:YAG) Haemangiomas of the lip and nose


36Epidermal atrophy

Skin resurfacing with carbon dioxide laser and Erbium:YAG (Er:YAG) laser atrophic scarring in partially or completely involuted haemangiomas in children

Micro pigmentation (tattoo pigmentation) may be useful for areas of hypopigmentation to restore colour to the scarred skin or vermilion


37DRUG THERAPY

mixed hemangiomas, proliferative hemangiomas, hemangiomas that affect vital organs are lifethreatening.

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IMIQUIMOD novel immunomodifier, used for small and intermediate-sized hemangiomas located in inconspicuous sites with alternate day topical application, for a cycle of 3 to 5 monthsADVANTAGES ease of use, controllability, safety, lack of local irritation or systemic effectsDISADVANTAGE cause hyperpigmentation; thus care has to be taken for application on the face for aesthetic reasons

vascular lesions of head and neck , v.lakshmi


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ORAL PREDNISOLONE (3.0–5.0 mg/kg) every other morning for 6 to 8 weeks. tapered after that for 2 or 3 weeks 2 or 3 cycles interval of 4 to 6 weeks. localized hemangiomas orbital or parotid lesions, intralesional steroids can be very effective.

TRIAMCINOLONE 1 to 2 mg/kg of body weight (maximum of 60 mg) at monthly intervals, depending on the age of the patient and size of the lesion.

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40PINGYANGMYCIN (bleomycin A5)

intralesionally for hemangiomas based specifically on a high sclerosing effect on vascular endothelium,

> 90% success rate12 and 49% complete resolution

IND : easy, safe, effective therapeutic modality cutaneous hemangiomas ,proliferative hemangiomas which respond poorly to steroids and/or

laser therapy.



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The injection begins from 1 point of the lesion toward the center, infiltrates evenly within the lesion via change of the injection direction until the surface of the lesion appears pale

Compression is applied for 15 to 30 minutes after injection to prevent effusion of the solution repeated for 2-3 wks

Pingyangmycin hydrochloride (8 mg/syringe2% lidocaine normal saline dexamethasone (5 mg/1 mL).


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each dosage is not more than 8 mg, and reduced accordingly for infants (1/4–2/3). cutaneous superficial or mucosal hemangiomas - 1.0 mg/mL

subcutaneous or deep hemangiomas - 1.5 to 2.0 mg/mL

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43VINCRISTINE

0.5 to 1.0 mg/ kg/ week IV over 6 weeks Infants with Kasabach–Merritt syndrome, which is caused by Kaposiform hemangioendothelioma, .



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ALPHA-INTERFERON serious side effect of spastic diplegia, which is permanent and disablingBBLOCKERS propranolol for problematic hemangiomas with dramatic shrinkage and control of the

hemangiomas. There was no rebound noted, and toxicity was minimal


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LASER THERAPY superficial proliferating hemangiomas. may accelerate the regression and reduce the size of the lesion, creating favorable situations

for subsequent treatments. If the lesion continues to enlarge during laser therapy, supplementary pharmacotherapy

should be considered.ADVANTAGE simplicity of use, which can be repeated at an interval of 2 to 4 weeks. choice of laser should be based on the location, size, and depth of the lesion.


46FLASH LAMP PUMPED PULSED DYE LASER

585 nm or 595 nm, selective destruction of the blood vessels, and is the only laser that delivers

photocoagulation of the targeted vessels while keeping the overlying skin intact.

INDIC : superficial hemangiomas those at the involution stage little effect on subcutaneous and deep-seated hemangiomas because of the limited

penetration depth


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NEODYMIUM: YTTRIUM-ALUMINUM-GARNET (ND:YAG) 1064 nm penetration depth of up to 5.0 mm larger and up to 2 cm deep hemangiomas


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PERCUTANEOUS LASER THERAPY deep hemangiomas cooling devices should be used to lower the temperature to protect the

epidermis from thermal damage effectiveness of laser therapy is 77% to 100% smaller the lesion, the better the result SIDE EFFECTS tissue necrosis and scarring .


49SURGERY

INDIC : located in the nose and lip that do not respond well to other treatments in the eyelids that impair sight and aesthetics, occurring on the forehead and scalp, repeated bleeding from the hemangiomas.

AIM : to remove or re-contour the residual deformity, scar hypertrophied abnormality, hyperpigmentation fibrofatty tissues to improve cosmetics and function


50COMPLICATIONS

Ulceration <5% of hemangiomas Usually with rapidly growing lesions that become secondarily infected Almost never occurs in vascular malformations Topical antibiotics +/- systemic abx Bleeding Spontaneous hemorrhage is rare May result from trauma


51Vascular Malformations

Normal rate of endothelial turnover “grow with the child” Subclassification based on predominant vessel: Lymphatic Capillary Venous Arterial


52Capillary (venular) malformations

port wine stain, capillary haemangioma made up of postcapillary venules within the papillary and superficial reticular dermis flat pink macules but darken and thicken with age, resulting in a cobblestone appearance result from altered neural modulation of the papillary plexus associated with Sturge–Weber and Klippel–Trenaunay syndromes


53 VENOUS MALFORMATIONS

present from birth and, unlike hemangiomas, they do not have a cycle of growth and subsequent spontaneous regression

They grow proportionately during infancy and childhoodCLINICAL FEATURES bluish, compressible, nonpulsating masses Extensive venous malformations can cause a localized intravascular

coagulopathy sclerosing agents used to destroy the vascular endothelium


54Factors implicated in varying response to treatment

Depth, Colour, Location of the lesion, Age at treatment Number of treatments


55Sclerosing agents

chemical agents (iodine or alcohol), osmotic agents (salicylates or hypertonic saline), DETERGENTS morrhuate sodium, sodium tetradecyl sulfate, polidocanol diatrizoate sodium anti-cancer drugs which change the surface tension of the cell, producing tissue maceration


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VENOGRAM

High draining venous malformations<5 min in veins sodium morrhuate 5%, ethanol ( toxic liver <5ml)-

Low drainage venous malformation> 5min in veinsIntralesional pingamycin 1.5- 2 mg/dlMax dose – 8 mg


57ETHANOL:

Most effective Effective rate 100% Digital subtraction angiography (DSA)-guided percutaneous ethanol for grade 2 and 3 (>5 cm)

facial and cervical venous malformations

MRI classification of Goyal et al grade 2A, well defined, >5 cm grade 2B, ill defined, < 5 cm in diameter grade 3, ill defined, >5 cm in diameter

DISADV :

Pain Soft tissue odema Base of tongue ,

pharynx,larynx – prophylactic tracheostomy


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acute inflammatory reactionformation of blood clots

Thrombosisfibrosis

occlusion of the malformed veinsresolution of the lesions

Care should be taken not to damage the facial nerves during treatment of parotid venous malformations

21-gauge butterfly needle 2/3 to 3/4 of the contrast agent BP,HR Monitered Dexamethsone tid before, after proced BP , kidney function test moniter vth IV RL, NAHCO3 ( hemoglobinuria) antibiotics


59VM

before immediately After 1 hr


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VM - Sclerotherapy – 8 hrs, 12 hrs


61Nd:YAG laser therapy

superficial venous malformations deeper lesions in parotid, masseteric, and deep facial areas -severe damage of the overlying skin

will result in unwanted scarring 30 to 70 W/cm2 + cooling photocoagulates - avoiding damage to the facial nerves. larger and thicker lesions- pharynx and larynx- low-power laser at several times allows shrinkage


62Glomovenous malformation (GVM)

an autosomal condition characterized by multiple venous malformations in the skin. differ from standard venous malformations by being multiple, slightly raised, or blue or

bluish-purple in appearance. Histopathologically, presence of numerous glomus cells (abnormally formed smooth muscle

cells). located on chromosome 1p, which is called glomulin


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surgical excision Sclerotherapy - sodium tetradecyl sulfate, polidocanol, and hypertonic saline Ablative therapy with argon and CO2 lasers is of potential benefit for small, superficial lesions.


64ARTERIOVENOUS MALFORMATIONS

AVMs of the head & neck region

Soft tissue plexiform hemangioma

arteriovenous fistulaintraosseous

central hemangioma


65EMBOLOTHERAPY

PURPOSE to control the growth of AVMs and frequent bleeding. key is to use sufficient liquid embolizing agents to eradicate the nidus. currently used liquid agents are ethanol N-butyl-2-cyanoacrylate (NBCA).


66Said to be complete :

For females with AVMs who are planning to become pregnant, it is best to do the embolization before pregnancy, because the hormonal changes during pregnancy may accelerate the progress of AVMs.

active bleeding has stopped localized pulsation - disappeared lesion - lighter in color expanded veins - normal new bone - cystic zones.


67Soft tissue AVMs

Infiltrativemixture of ethanol and

contrast at a ratio of 1:1Nidus

require absolute alcohol Fistularequire absolute alcohol .

EMBOLISATION

affect important anatomic structures with severe disfigurements, the most effective treatment is preoperative embolization and radical resection


68TREATMENT OF LYMPHATIC MALFORMATIONS

Lymphatic malformations can be divided into 2 types: macrocystic microcystic.The treatment options include surgery, sclerotherapy laser therapy


69Choice of Treatment Methods

Superficial oral mucosal

microcystic• Intralesional injection

of pinyangmycin (1.0 mg/ml)38

• Laser therapy

Deep-seated microcystic

• Preoperative intralesional injection of pinyangmycin

Macrocystic lymphatic

malformations • Ethanol • Doxycycline• Sodium tetradecyl

sulfate (STS),• Ok-432


70MACROCYSTIC LYMPHATIC MALFORMATIONS

• Pinyangmycin (1.5– 2.0 mg/mL)

• OK-432Intralesional

injection

• doxycycline • safe and effective methodPercutaneous

sclerotherapy

Diffuse- inflammatory reaction

Activate WBC

Release cytokines

Increase permeability of endothelium

FibrosisShrinkage of cystic

spaces


71Percutaneous sclerotherapy with Ethibloc (alcoholic solution of zein)

acts on the proteins destroying their

structure

triggering their clotting

obstruct vessels reduce the volume of

the malformation

direct effect on the internal surface on the

vesselsdamaging the cells that

form the internal layer of the vessels

occlusion

safe and effective procedure in the treatment of macrocystic and mixed lymphatic malformations.


72Macrocyst access

• ultrasound guidance and placement of a coaxial 5F pigtail catheter system• Massive lesions may accept larger catheters such as 8 to 10F size

• multilocular lesions, each macrocyst is treated with separate catheter • Following complete drainage of the macrocysts, contrast cystogram with fluoroscopy is performed to

document the native cyst volume,

• Following contrast aspiration, the macrocysts are treated with dual-drug chemoablation• 50% original volume sequential intracystic injections of STS 3% (2 minute dwell time) followed by aspiration

and injection of ethanol 98% solution (dwell time 15 minutes).


73STURGE WEBER SYNDROME

Neurocutaneous disorder Facial vascular malformation Port wine stain Post capillary venules V1/V2 distributions Leptomeninges Neurodevelopmental disorders Seizures Ocular symptoms Glaucoma, conjunctival/choroidal hemangiomas

ref : takeoka m. sturge-weber syndrome. medscape. may 2011


74Klippel-Trenaunay syndrome

Geographic capillary malformation


75Cutis marmorata telangiectatica congenita

noted at birth and may have a localized orgeneralized distribution. When localized, it often has a sharply segmental pattern

involving upper extremity


76. Blue rubber bleb nevus syndrome

a venous malformation located on the palm.

characteristic skin and gastrointestinal features.


77Proteus syndrome

hemihypertrophy and deformity of the foot, in addition to capillary malformation on abdomen

rare sporadic disorder that is characterized by soft tissue and bony hypertrophy of the hands and feet, hemihypertrophy, exostosis, cranial hyperostosis, visceral hamartomas including lipomas, vascular anomalies, and epidermal nevi.


78. Maffucci syndrome

Venous malformations and enchondromas

a rare sporadic genetic disorder typically comprising enchondromas and vascular anomalies


79Osler-Rendu-Weber syndrome

Hereditary hemorrhagic telangiectasia , characterized by mucocutaneous and visceral telangiectases and AVMs


80KASABACH MERRITT SYNDROME

Result of platelet trapping within the angioma and spleen Due to short platelet survival time, internal bleeding Infection can produce DIC and consumptive coagulopathy Occurs during proliferative phase Found in patients whose lesion > 5 cm


81Case : 1

Coil placed contrast medium was injected the varix was verified.

Coil placement (arrow) and no residual arteriovenous shunting were showed on the control angiography performed via the indewelling femoral arterial catheter.

13 months after


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Post embolisisation angiogram


83Case 2

Axial CT scan with contrast, soft tissue (A), and bony (B) windows showing an intrabony nonhomogeneous mass


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stage I AVM involving the left posterior mandible


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patient 6 months after initial presentation now with stage II AVM involving the left posterior mandible


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left posterior mandible stage II AVM after embolization with Onyx


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AVM left posterior mandible 2 years after treatment with selective embolization and excision after removal of hardware


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Patient 6 years after diagnosis and treatment of left posterior mandible AVM, with new-onset facial swelling from recurrence of AVM with involvement of left parotid.


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left face AVM recurrence with no bone involvement but with extensive involvement of the left parotid gland. (A) Anteroposterior plane skull film. (B) Representative view of the AVM during angiography


90Case 3

Panoramic radiograph of 12year-old girl with stage II AVM involving the right posterior mandible.


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Axial CT scan with contrast. Soft tissue (A) and bony (B) windows show an intrabony nonhomogeneous mass of the right posterior mandible


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Right mandible AVM progressive fill


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Selective embolisation Surgical excision

vijaya lakshmi


94references

Treatment guidelines for treatment of vascular malformations Haemangiomas and vascular malformations of the maxillofacial region—A review British

Journal of Oral and Maxillofacial Surgery 44 (2006) 263–272 Direct-Puncture Embolization of Intraosseous Arteriovenous Malformation of Jaws JOMS 2002 Surgical management of vascular lesions of the head and neck: a review of 115 cases Int. J.

Oral Maxillofac. Surg. 2011; 40: 577–583 Vascular Malformations and Their Treatment in the Growing Patient , clinics 2015 Vascular malformations ,Part II: Associated syndromes (Maria C. Garzon, MD,a Jennifer T. Huang,

MD,b Odile Enjolras, MD,c and Ilona J. Frieden, MDd New York, .

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1 vascular anomalies

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