Vascular anomalies in maxillofacial region—Review M.E. Shama,∗, Nishat Sultanab. Journal of Oral and Maxillofacial Surgery, Medicine, and Pathology

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Journal of Oral and Maxillofacial Surgery, Medicine, and Pathology xxx (2012) xxx–xxx

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eview article

ascular anomalies in maxillofacial region—Review

.E. Shama,∗, Nishat Sultanab,1

Department of Oral and Maxillofacial Surgery, Vydehi Institute of Dental Science and Research Center, EPIP Area, Bangalore, IndiaDepartment of Oral Medicine and Radiology, Vydehi Institute of Dental Science and Research Center, EPIP Area, Bangalore, India

r t i c l e i n f o

rticle history:eceived 6 July 2011eceived in revised form 10 February 2012ccepted 30 March 2012vailable online xxx

a b s t r a c t

Vascular tumors in the maxillofacial region are more frequently encountered in both youngest and old-est ages. For centuries, a myriad of cutaneous or visceral masses, pigmentations, or spaces that resultedin a predominantly disturbed vascular morphologic pattern were named and categorized according toappearance, location, fluid content, and often inconsistent or unpredictable clinical behavior. These vascu-

eywords:emangiomalood vessel abnormalityrteriovenous-malformations

lar lesions localized especially on exposed sites affect the physiognomic aspect and may cause significantpsychological distress. Advancements in the knowledge of pathogenesis of such vascular malformationsare continuously changing their treatment protocols. In this review we seek to draw attention to thecurrent classification, terminology, development, clinical features, diagnosis and treatment of congenitalvascular anomalies affecting the maxillofacial region.

Crown Copyright © 2012 Published by Elsevier Ltd. on behalf of Asian Association of Oral andMaxillofacial Surgeons. All rights reserved.

ontents

1. Introduction . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 002. Classification . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 003. Diagnosis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 00

3.1. History and clinical examination . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 003.1.1. Investigation . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 003.1.2. Selection of imaging modalities . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 00

3.2. Conventional radiography . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 003.2.1. Doppler US . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 003.2.2. Computed tomography . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 003.2.3. MR imaging . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 003.2.4. Direct percutaneous phlebography . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 003.2.5. Diagnostic angiography . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 003.2.6. Nuclear medicine . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 003.2.7. Pathogenesis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 00

3.3. Clinical presentation . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 003.3.1. Hemangioma . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 003.3.2. Treatment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 003.3.3. Vascular malformation . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 003.3.4. Capillary (venular) malformation . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 003.3.5. Venous malformation . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 00

3.4. Lymphatic malformations . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 003.4.1. Arteriovenous malformation . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 00

Please cite this article in press as: Sham ME, Sultana N. Vascular anomPathol (2012), http://dx.doi.org/10.1016/j.ajoms.2012.03.009

3.4.2. Treatment of vascular malformation . . . . . . . . . . . . . . . . . . . . .3.4.3. Venular malformation. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .3.4.4. Venous malformation . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .

∗ Corresponding author at: No-37, 1st Cross, Silver Oak Street, JP Nagar 7th Phase, BangE-mail addresses: [email protected] (M.E. Sham), nishat [email protected]

1 Mobile: +91 8088264916.

212-5558/$ – see front matter. Crown Copyright © 2012 Published by Elsevier Ltd. on bettp://dx.doi.org/10.1016/j.ajoms.2012.03.009

alies in maxillofacial region—Review. J Oral Maxillofac Surg Med

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alore 78, India. Mobile: +91 9886232626.(N. Sultana).

half of Asian Association of Oral and Maxillofacial Surgeons. All rights reserved.

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3.4.5. Lymphatic malformation . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 003.4.6. Arteriovenous malformation . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 00

4. Summary. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 00. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 00

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Table 1bUpdated ISSVA classification of vascular anomalies. (International Society for theStudy of Vascular Anomalies) [8].

Vascular tumor Vascular malformation• Infantile hemangiomas• Congenital hemangiomas (RICHand NICH)• Tufted angioma (with or withoutKasabach-Merritt syndrome)• Kaposiformhemangioendothelioma (with orwithout Kasabach-Merrittsyndrome)• Spindle cellhemangioendothelioma• Other, rarehemangioendotheliomas(epithelioid, composite, retiform,polymorphous, Dabska tumor,lymphangioendotheliomatosis,etc.)• Dermatologic acquired vasculartumors (pyogenic granuloma,targetoid hemangioma,glomeruloid hemangioma,microvenular hemangioma, etc.)

Slow-flow vascular malformations:• Capillary malformation (CM)Port-wine stainTelangiectasiaAngiokeratoma• Venous malformation (VM)Common sporadic VMBean syndromeFamilial cutaneous and mucosalvenous malformation (VMCM)Glomuvenousmalformation(GVM) (glomangioma)Maffucci syndrome• Lymphatic malformation (LM)Fast-flow vascular malformations:• Arterial malformation (AM)• Arteriovenous fistula (AVF)• Arteriovenous malformation (AVM)Complex-combined vascularmalformations:• CVM, CLM, LVM, CLVM, AVM-LM,CM-AVM

Table 2Classification of vascular anomalies [9].

Classification of surface vascular lesions• Traditional classification• Capillary hemangioma• Strawberry hemangioma• Strawberry nevus• Port wine stain• Flame nevus• Cavernous hemangioma• Venous angioma• Lymphangioma• Arteriovenous malformationClassification of Jackson et al. [6]• Hemangioma• Vascular malformationLow-flow lesionHigh-flow lesion

References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .

. Introduction

Vascular anomalies are poorly understood, though are com-only encountered, with an estimated incidence of 1–2 in 10,000

irths [1], and a prevalence of 1% [2,3]. Clinicians from many spe-ialties are involved in the management of these patients and haveooked for solutions within their specialty and applied them toll lesions. Furthermore, the power of a name has greatly influ-nced understanding and progress within the realm of vascularnomalies. For centuries, a myriad of cutaneous or visceral masses,igmentations, or spaces that resulted in a predominantly dis-urbed vascular morphologic pattern were named and categorizedccording to appearance, location, fluid content, and often incon-istent or unpredictable clinical behavior [4].

In this review we seek to draw attention to the current classifi-ation, terminology, development, clinical features, diagnosis andreatment of vascular anomalies affecting the maxillofacial region.

. Classification

The current classification system for vascular anomalies can bettributed to Mulliken and Glowacki who based their concept uponhe clinical presentation, biologic behavior, and histology of theseesions [5]. In 1993, Jackson et al. [6] proposed another system forlassifying hemangiomas, vascular malformations, and lymphaticalformations on the basis of vascular dynamics. A classification

ystem for vascular anomalies based on cellular features, flow char-cteristics, and clinical behavior was updated during the meetingf the International Society for the Study of Vascular AnomaliesTables 1a and 1b) [7,8a] now recognized worldwide as the offi-ial system for classification of congenital disorders of vascularevelopment. Prior to the establishment of this system, vascu-

ar anomalies were often misdiagnosed, mismanaged, and poorlynderstood. In 1996, Kawanabe et al. [8b] reported a system forractical classification of vascular lesions in which the treatmentrocedure is selected according to the characteristic flow withinhe lesion (Table 2) [9]. Subsequent modification of the originallassification to include the depth of the lesion and characteris-

Please cite this article in press as: Sham ME, Sultana N. Vascular anomalies in maxillofacial region—Review. J Oral Maxillofac Surg MedPathol (2012), http://dx.doi.org/10.1016/j.ajoms.2012.03.009

ic flow has resulted in a classification that is clinically useful andorrelates well with pathological and radiological data (Table 3)10]. Recently, hemangiomas have been categorized into congenital

able 1aSSVA classification of vascular anomalies (International Society for the Study ofascular Anomalies) [7].

Vascular tumor Vascular malformation

Simple Combined

Hemangioma Capillary malformation Arteriovenous fistula,arteriovenousmalformation,capillary-venousmalformation, capillary-lymphatic-venousmalformation.

Other Lymphatic malformation Lymphatic-venousmalformation, capillary-lymphatic-arteriovenousmalformation

Venous malformation

• Lymphatic malformationFlow-related classification and recommended treatment• Slow-flow lesion: sclerotherapy• Intermediate-flow lesion: sclerotherapy (plus embolization)• High-flow lesion: embolization (plus sclerotherapy)

Table 3Classification of hemangiomas and vascular malformation [10].

A: HemangiomasSuperficial (capillary hemangiomas)Deep (cavernous hemangiomas)Compound (capillary cavernous hemangiomas)

B: Vascular malformationsSimple lesions

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

High flow lesionsArterial malformation

Combined lesionsArteriovenous malformationLymphovenous malformationOther combinations

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which is present at birth) and the more common infantile (whichppears soon after birth). Congenital hemangiomas are further sub-ivided into rapidly involuting congenital hemangioma (RICH) [11],on-involuting congenital hemangioma (NICH) [12] and congenitalon-progressive hemangioma [13].

. Diagnosis

.1. History and clinical examination

Accurate terminology leads to precise identification of vascularntity. In most cases, an accurate history and physical examinationill help establish the diagnosis.

Simplified diagnostic approach to a congenital vascular lesion10]. Yes= VM Yes=H Yes=H

resent at birth Rapid proliferation Involution

No= H No= VM No= VM

= Hemangioma ; VM= Vascular malformation.

.1.1. InvestigationFurther investigations are considered only if the diagnosis is in

oubt or the investigation will influence the decision and methodf treatment. Unfortunately no single investigation is appropriateor all lesions.

.1.2. Selection of imaging modalitiesMultiple imaging modalities should be used to evaluate char-

cteristics of the lesion, such as size, flow velocity, flow direction,elation to the surrounding structures (vessels, muscle, nerve, bone,kin), and lesion contents.

.2. Conventional radiography

Conventional radiography plays only a small part in the diag-osis and classification of vascular lesions, but it provides useful

nformation about bone and joint involvement. Bone erosion, scle-otic change, periosteal reaction, and pathologic fracture eachuggest bone involvement. Phleboliths are specific to venous mal-ormation [14].

.2.1. Doppler USUltrasound is usually the first modality used in the imag-

ng of a suspected vascular malformation because it is widelyvailable, low cost, non invasive and does not use ionizingadiation [15].

Doppler US is essential in differentiating venous malformationsrom other vascular anomalies. US should be performed with aigh-frequency linear array transducer (5–10 MHz). Explorationegins with a gray-scale examination to delineate the margins ofhe malformation. Venous malformations appear as hypoechoic oreterogeneous lesions in 80% of cases [15,16]. Anechoic channelsan be visualized in less than 50% of cases. Sometimes, isoechoichickening of the subcutaneous tissues without a solid mass oriscernible channels is the only feature [15]. The pathognomic phle-


olith, as expected appears as a hyperechoic focus with acoustichadowing; however, unfortunately this is only detected in 16% ofases [16]. Color and pulsed Doppler analysis of the VMs revealow in 84% of lesions, with monophasic and biphasic flow seen

PRESSrgery, Medicine, and Pathology xxx (2012) xxx–xxx 3

dual Hemangioma

ent in adulthood

ascular malformation

in 78% and 6%, respectively. Only 16% reveal no discernible flow,which has been proposed may indicate lesion thrombosis or flowbelow detectible limits [16]. The focus or goal of the ultrasoundexamination is to differentiate between a high flow (AVM) andlow flow lesion, and if the latter, then to decide if the lesion is aVM, or a microcytic or macrocytic LM, in case of the atypical pre-sentation where the clinical probability of malformation is muchlower, one must take into account that there is significant over-lap of imaging features between vascular malformation and otherbenign, aggressive, or malignant entities. The same can be statedfor any imaging modality at any point in the work up of a suspectedvascular anomaly [17].

3.2.2. Computed tomographyCT is of limited use in the work up of most focal VMs because of

several factors. CT, even with contrast enhancement, usually pro-vides poor lesion conspicuity relative to adjacent potentially criticalstructures and does not usually provide assessment of internalmalformation vascular architecture, two variables that have sig-nificant impact on therapeutic decisions [18]. CT scan can identifydystrophic calcifications and phleboliths when present, and canbe extremely helpful in providing detailed anatomic informationregarding adjacent bony pathology if required [4].

With the introduction of multidetector CT technology, the abil-ity rapidly to perform thinly collimated contrast-enhanced scansover large body segments with subsequent multiplanar reconstruc-tions has provided the provided the opportunity to evaluate verylarge vascular malformation [19]. The high temporal resolution ofCT and the ease with which findings can be interpreted are advanta-geous in evaluating vascular lesions. However, because CT involvesconsiderable exposure to ionizing radiation and provides less infor-mation about blood flow, magnetic resonance (MR) imaging hasreplaced CT in the evaluation of vascular malformations [14].

3.2.3. MR imagingThe introduction of MR imaging has allowed a giant leap forward

in the noninvasive assessment of vascular anomalies by providingsuperior lesion and soft tissue discrimination to CT, semiquanti-tative flow assessment and three-dimensional reconstruction, allwithout subjecting to the patient to ionizing radiation [4,14]. As aresult, MR imaging has become the imaging modality of choice forthese lesions [20,21] not only can MR imaging influence therapeuticdecision making by defining the internal architecture of a malfor-mation and its relationship to adjacent critical structures, but it canalso serve an objective method quantitatively to assess therapeuticoutcomes through serial MR imaging monitoring of treated lesionsize and segmental characteristics [4,14,18,21]. Recent advancesnow even use MR image guidance during percutaneous therapy of


vascular malformations [22–24]. The disadvantages of MR imag-ing are not unique to malformations, because imaging requires acooperative, nonclaustrophobic patient and sometimes long scanduration for larger lesions [17].

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The usual basic MR imaging protocol used in the evaluationf a suspected VM should ideally start with an spin echo or fastpin echo T1-weighted evaluation of the lesion morphology allow-ng maximal definition of tissue planes and relationship to criticalsseous or neurovascular structures. This should be followed byat-saturated T2-weighted and T2-weighted short tau inversionecovery sequences, which allow one to define maximal extent ofhe lesion. T1- and T2 weighted sequences should be performed int least two planes. The identification of hemosiderin, dystrophicalcification, or phleboliths can be achieved through the use ofradient echo T2-weighted sequences that can also aid in evalu-tion of high venous flow. The study should be completed withre-gadolinium and post-gadolinium-enhanced fat-saturated T1-eighted imaging [4,18,20].

.2.4. Direct percutaneous phlebographyAs the name implies, involves direct fine-needle puncture of the

esion and contrast injection under fluoroscope and is currentlysed in several distinct scenario [17]. It provides the diagnosticold standard in situations requiring confirmation of a VM thatay be equivocal on previous imaging modalities, or for treat-ent planning, or to exclude the possibility of neoplasm, in caseshere biopsy is being contemplated. It is more commonly per-

ormed as the initial diagnostic evaluation of venous (or lymphatic)alformation morphology and flow characteristics within the scle-

otherapy procedures [4,18].

.2.5. Diagnostic angiographyIn current practice, there is no role for diagnostic angiography in

he diagnostic work-up or management of purely low-flow vascularalformations if one follows a prescribed algorithm [20].Arteriography may have a role in the specialized work-up of

ixed lesions that may have a high flow component. Arteriogra-hy of VMs reveals either no findings, or delayed mass-associatedenous or capillary blush with variables stasis, pooling [4,18,25].

.2.6. Nuclear medicineTc-99m-tagged red blood cell whole-body blood pool scintig-

aphy is a rarely described technique that uses scintigraphyuantification of systemically administered radiotracer activityithin venous or AVMs to evaluate baseline lesion size and changeith therapy. Whole body blood pool scintigraphy can also allowifferentiation of venous from LMs [26,27].

.2.7. PathogenesisBoth infantile and congenital hemangiomas are the result of

ndothelial cell hyperplasia. The cause of the aberrant and focalroliferation of endothelial cells in hemangiomas remains unclear.he origin of hemangiomas, although still under debate, has beenarrowed to embolic placental angioblasts or intrinsic endothelialrogenitor cells with the ability to clonally duplicate in a preciseilieu of cytokines and estrogen [28,29]. The placental theory of

emangioma growth discovered that the histology and molecu-ar markers unique to placental tissue, namely GLUT1, Lewis Yntigen, Merosin, and Receptor II were also present in infantileemangiomas [30]. Staining for GLUT1 is now standard prac-ice to pathologically delineate hemangiomas from other vascularnomalies when the diagnosis is in question [31]. The presencef circulating progenitor and stem cells, identified by their spe-ific cell-surface proteins CD133+/CD34+, within hemangiomasnd the blood circulation of these patients brought to the forehe theory that these tumors arise from embryonic endothelial


recursors [32]. The source is suspected to be a single somaticutation leading to clonal duplication of primitive endothelial cells

33]. Although controversial, it is possible that both the theoriesegarding the origin of hemangiomas are correct. Hemangiomas

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likely arise from progenitor cells with directional preponderanceto become placental-like tissue in specific organs such as skin andliver. However, the appropriate milieu of local tissue factors andcytokine signals must be present to foster their development [34].

Venous malformations are caused by an error in developmentwithin the venous system. The vast majority of the lesions representsporadic cases, and the etiology of these malformations remains anarea of speculation and continuing research [35]. There are alsofamilial cases of venous malformations which are inherited in anautosomal dominant fashion and are linked to a locus on chro-mosome 9P [36]. Somatic mutations in the angiopoietin receptors(TEK) have been found in a significant percentage of tested solitaryand multiple sporadic venous malformations. These mutations leadto loss of function of the TIE2 receptor [37]. In addition to this loca-tion, later studies have found another Y897S mutation within theTIE-2 gene [38]. Ongoing research in these areas points to errors invasculogenesis of which the TIE2 receptor is an important compo-nent. Other vascular growth factors such as tissue growth factor[39] beta (TGFbeta) and beta fibroblast growth factor (betaFGF)also appear to be upregulated. This upregulation may representevidence toward a proliferative component in these lesions asopposed to the accepted hypertrophy growth theory althoughongoing research is needed to delineate the exact interactions ofthese findings. The role of neural components in the developmentof vascular anomalies has been debated. The presence of increasedneural cells has been found in both slow-flow and high-flow malfor-mations. A study found a significant increase in nerve componentsin venous malformations and more so in AVMs, while lymphaticmalformation tissue showed almost a complete absence of neuraltissue [40]. The matter by which malformations present at birthcontinue to grow is unanswered. It has long been accepted thatthese lesions grew by slow expansion (hypertrophy) rather thanby proliferation (hyperplasia). Clinically, it can be difficult to pre-dict how much growth will occur or where, as some lesions appearto expand well beyond their initial clinical boundaries and thepatient may even develop multifocal lesions later in life suggestingthat proliferation and actual invasion of surrounding tissue may beoccurring [34]. Recent findings of increased metalloproteinase-9in intramuscular venous malformations suggest that invasion andvasculogenesis may indeed be a mechanism of growth beyond thatof hypertrophy with increased hydrostatic pressure [41]. Proges-terone receptors were also found on venous malformations, whichmay explain their propensity for rapid growth during hormonalchanges [42].

3.3. Clinical presentation

3.3.1. HemangiomaThis is the most common tumor in white infants (10–12%) and

the head and neck region is the most commonly involved site(60%) [43,44]. Most lesions are solitary (80%) and girls are moreaffected than boys (3:1) [10]. Facial hemangiomas have a predilec-tion for segmental distribution and for regions of embryologicalfusion [45]. A strong association has been reported between abeard distribution of facial hemangiomas and symptomatic laryn-geal involvement [46].

Infantile hemangiomas become evident shortly after birth aswell demarcated, red, vertically expansive lesions. They can beoverlooked during the first month of life as they are difficultto differentiate from other common skin blushes seen in thenewborn. During this period, they are flat, pink, and sometimesmore pale or bluish than their surrounding tissue. In this light,


and unlike vascular malformations, infantile hemangiomas areconsidered characteristically absent at birth. In time, however,progressive endothelial cell proliferation leads to skin and sub-cutaneous changes consistent with a growing tumor. The skin

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requently becomes bright red, cobblestoned, and elevated duringhe proliferative phase of growth [34]. Rapid expansion can leado adjacent skin and soft tissue ischemia, necrosis, and ulceration.lceration and subsequent bleeding is common in watershed areas,

uch as the lip and ear, and will appear within the first few monthsf life during the rapid growth phase [47]. Massive blood loss isncommon and bleeding can typically be stopped by applied pres-ure to the site. Despite the appearance of superficial hemangiomasnd the risk of ulceration in some areas, it is uncommon for heman-iomas to be more susceptible to injury or disrupt when exposedo minor trauma [34]. Infantile hemangiomas are heterogeneousn their appearance and have further been classified according toheir depth, number, distribution, and, sometimes, location. Theapidly involuting congenital hemangioma and its cousin, the non-nvoluting congenital hemangioma (NICH), were thought to beongenital forms of IH until North and colleagues demonstratedn 2000 that GLUT-1 (Glucose Transporter-1 Protein) is expressedn IH but not in these congenital subtypes. The rapidly involutingongenital hemangioma seems to be uncommon but not rare. It isikely often under-recognized and/or misdiagnosed. By definition,ICH is present at birth, and most commonly occurs on the limb,ead, or neck [48]. In some cases it has been associated with heart

ailure from cardiac overload [49]. The rapidly involuting congeni-al hemangioma has also been associated with decreased platelets,hought to be due to localized intravascular coagulation [49]. In

ost cases, RICH involutes completely by age 1-year, although gen-rally with some degree of atrophic or redundant skin. Most cases ofICH can be diagnosed by history and physical examination. How-ver, it is important to keep in mind the possibility of a sarcoma orther malignant tumor in the differential diagnosis. Therefore, if theumor enlarges or develops a firm texture, a biopsy is warranted.he noninvoluting congenital hemangioma (NICH) is a rare vascu-ar tumor that is fully formed at birth, grows proportionally withhe patient or expands slightly over time, and does not regress [50].his growth pattern distinguishes NICH from the infantile heman-ioma (IH). Clinically, NICH presents as a round or oval mass thats pink-to-blue-violet in color, with central coarse telangiectasesnd peripheral pallor. The lesions are warmer than is the surround-ng skin, and large draining veins sometimes can be observed athe periphery. The lesions of NICH are found most often on theead and neck and commonly overly the mandible or occur on thextremities near a joint [48,50]. Histopathologic features includehe presence of dilated veins and sometimes arteries between theascular lobules, dermal arteriovenous microfistulae, a large stel-ate vessel in the center of the lobules, hobnailing of endothelialell nuclei, and thin basement membranes [49,50]. Sonographi-ally visible vessels (veins or arteries) represented the only featurehat was appreciably more common in NICH (72%, 13/18 lesions)han it was in IH (15%, 4/26 lesions). Evidence of heterogeneitynd calcifications with ultrasound analysis also were suggestivef a diagnosis of NICH. It is important to distinguish NICH from IHecause of the differences in their management. Congenital heman-iomas should be observed for 18 months to monitor for regression.f the lesion appears to be noninvoluting, it can be examined byltrasound to confirm the presence of fast flow; GLUT1 stainingf a biopsy specimen also can be considered. Treatment of NICHs surgical excision. Some patients may require arterial emboliza-ion to control bleeding, but most lesions can be excised withoutomplications or recurrence [51].

Superficial hemangiomas involve only the skin and will remainelatively flat throughout their growth phases. Compound heman-iomas involve both the skin and subcutaneous tissue while deep


emangiomas involve the subcutaneous surface and not the over-ying skin [34]. Hemangiomas are also described as either focal oregmental. Focal hemangiomas are classically reported as uniloc-lar masses that undergo clear phases of growth and regression.


Hemangiomas involving multiple contiguous cervical facial sub-units with indistinct borders are termed as segmental hemangioma.Segmental hemangiomas are as compared with their focal counter-parts. These entities, to a variable extent, involve the head and neckin the distribution of the trigeminal nerve. Extensive deeper com-ponents are typically present especially within the parotid glandand deep facial planes of the head and neck. Segmental disease isbest illustrated by the characteristic ‘beard’ distribution heman-gioma that involves lower lip, chin, cheeks and pre-auricular areas[34].

3.3.2. TreatmentThe widespread belief that hemangiomas will completely dis-

appear in the first few years of life is misleading; studies reportedthat 80% of lesions that had not involved by 6 years and 38% oflesions that had involuted at 6 years left behind a residual cos-metic deformity [52,47]. The useful approach to the managementof hemangiomas can be based on the stage of the lesions (pro-liferative or involutive phase), type of lesion (superficial, deep,compound) and the management of residual deformity [53]. Sys-temic corticosteroid treatment has been established as the mostefficient medical therapy for common cutaneous infantile heman-giomas [54]. It is appropriate to administer the steroid therapyduring the proliferative phase, and it will have only a negligibleeffect on involuting hemangiomas [55]. There is a wide variation inresponse rates, from less than 40% to greater than 90%, dependingon dose, duration of treatment, and age at which corticosteroidsare initiated [55]. Interferon � and 2a and beta are used only insteroid-resistance life and sight threatening lesions [56]. Intrale-sional steroid therapy, often requiring multiple injections, is aneffective and safe first-line option for nasal tip and deep parotidlesions in the proliferative phase to control accelerated growthand terrible esthetic consequences [34,57]. Massive hemangiomas,liver disease with enzyme dysfunction, airway lesions, segmentaldisease, and periorbital involvement often require systemic ther-apy to control progression and devastating functional outcomes.Both systemic steroids and vincristine have been the workhorsefor these conditions [34]. Recent studies reported that Propra-nolol regress hemangiomas in newborns rapidly when treated with(2–3 mg/kg), a known non-selective beta-blocker used in treatinginfants with cardiac and pulmonary conditions. Side effects of thisdrug are rare and benign, making it a promising new therapeutictarget [58,59]. The mechanism of propranolol on reducing or curinghemangiomas remains unclear although the most likely mecha-nism is the pharmacologic stimulation of programmed endothelialcell death (apoptosis) [60].

Superficial hemangiomas can be treated with pulsed dye laser.Treatment should be started at the earliest sign of heman-giomas and repeated at 4–6 weekly intervals until the lesionis completely eliminated [61,59,62]. During later stages of pro-liferation, hemangiomas usually acquire a thickness that isbeyond the depth of penetration of yellow light laser. Intersti-tial potassium-titanyl-phosphate (KTP) and neodymium:yttrium-aluminum-garnet (Nd:YAG) lasers have been used for these lesionswith some success [57,61].

In involutive phase, the aim of the treatment should be toimprove appearance and function. A wait and watch policy may bepursued until 3–4 years of age [34]. In lesions that involute com-pletely by 6 years no further treatment will be required in 60% ofcases whereas in cases of slow involution further treatment willrequired in 80% of cases [48].

Superficial lesions with small and intermediate size vessels are


treated successfully with pulsed dye laser [61,59,62]. Larger ves-sels can be treated with a copper bromide and Nd–YAG laser [59].Residual telangiectasia and rubor following hemangioma involu-tion is amenable to flash lamp dye laser (FPDL) therapy with low

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isks and superb outcomes. FPDL works by photothermal ablationf its chromophore hemoglobin that contributes to the superficialed discoloration in hemangiomas [34]. Intraoral lesions respondimilar to their skin counterparts although these entities arencommon. Plasma knife (PK) surgery is a new treatment modalityor excision of these lesions. PK technology provides an electri-ally conducting site by means of obtaining the use of controlledadiofrequency energy, and by using the intra and extracellularuid. 90% coagulation and 10% cut mode choice for the PK excisionf the hemangioma of the tongue and shown to be capable to con-rol the bleeding [62]. Uncomplicated ulcerated hemangiomas cane managed conservatively, while ulcerated hemangiomas com-licated by repetitive trauma such as located on lips are oftenefractory to conservative management and can be considered forulse dye laser treatment. The availability of the pulsed dye laserrovides a recognized alternative to conservative management63].

.3.3. Vascular malformationVascular malformation (VM) is considered an abnormality of

he embryonic development, a structural anomaly. VM may beomposed by arteries, veins and/or capillaries. Clinically, they areimilar to hemangiomas; however, they are always present at birthnd grow as the patient physically develops. It does not sponta-eously regress, remaining stable throughout life. Frequently, bone

nvolvement is present as a radiolucent, multilocular and well-ircumscribed image [64,65]. Trauma, puberty and pregnancy canause accelerated growth [44]. Unlike hemangiomas, vascular mal-ormations are associated with skeletal abnormalities in up to 35%f cases [66].

.3.4. Capillary (venular) malformationPreviously called port wine stain, capillary hemangiomas. They

re thought to result from altered neural modulation of the papil-ary plexus. They present initially as flat pink macules but darkennd thicken with age, resulting in cobblestone appearance [67,68].hey are graded according to the degree of ectasia of the vesselsnto grade I–IV which correlate well with the clinical features andutcome of treatment [69].

Capillary malformations may be associated with Sturge-Webernd Klippel Trenaunay syndromes [10].

Venular malformations can be divided into midline malfor-ations and traditional venular malformations known as portine stains, telangiectatic nevus, or nevus flammeus [21]. Midline

esions are pink macules, may or may not be confluent, are alwaysresent from birth, appear on the midline of the head, and are com-only known as “salmon stains,” “stork bites,” or “angel kisses.”

hey occur in 40% of white newborns and 30% of black ones. Theyre usually transient and tend to disappear during the first yearf life in 65% of boys and 54% of girls [36], particularly in the casef lesions on anterior sites [70]. Capillary/venular malformationsre reddish-pink macules that darken over time. Although they arelways congenital, they do not become visible until several daysfter birth. They occur in 0.4% of newborns and equally in boysnd girls. In 83% of cases, they appear on the head and neck [71].hey affect the right side of the face more often than the left side21]. Port wine stains are located on one or more facial dermatomesefined by branches of the trigeminal nerve. The V2 dermatome ishe one most often implicated (57%), followed by the mandibularne (V3), and the ophthalmic one (V1) [71]. They may sometimes bessociated with small skeletal changes in the form of bone hyper-rophy, particularly when the V2 lesion extends to the gingival


nd maxillary mucosa. This localized hypertrophy may lead to gapsetween the teeth and a striking increased volume of the affected

ip that requires surgical correction [21]. Several syndromes cane associated with venous malformations including blue rubber


bleb nevus syndrome, glomuvenous malformation (associated withglomulin mutation), and multiple cutaneomucosal venous malfor-mation (mutations in the TIE2 receptor) [34].

3.3.5. Venous malformationVenous malformations are part of a spectrum of vascular mal-

formations commonly found in adults. A venous malformation isdefined as a simple malformation with slow flow and an abnormalvenous network [18]. They are characterized by abnormal collec-tion of veins, which do not have any demonstrable mitotic activityin endothelial or pericyte cells and often lack a uniform smoothmuscle layer [10].

3.4. Lymphatic malformations

Lymphatic malformations are congenital collections of ectaticlymph vessels that form endothelial lined cystic spaces [34]. Likeother vascular malformations, lymphatic malformations are alwayscongenital, although only 65–75% are diagnosed at birth; by the endof the second year of life, 80–90% have been diagnosed [21]. Themost common site is the head and particularly the neck (90%) [21].The main divisions in classifying these lesions are whether theycontain macrocysts (≥2 cm), microcysts (<2 cm), or both. Macrocys-tic lesions are more easily treated and carry a better prognosis thanits microcystic counterpart [34]. The embryologic development ofthe lymphatic system remains an area of active investigation. Asthe development is not completely understood, it is difficult tofully understand the causal factors involved in lymphatic mal-formations [72]. Several studies have been published regardingpossible lymphangiogenic growth factor involvement in the eti-ology of lymphatic malformations These factors include VEGF-C,vascular endothelial growth factor receptor 3 (VEGFR-3), and tran-scription factor Prox-1 [73].

Lymphatic malformations can present in a wide variety of waysin the head and neck. Lesions can be focal, multifocal, diffuse,macrocystic, or microcystic. The oral cavity and airway are com-monly involved in more diffuse lesions. Lymphatic malformationsare frequently diagnosed on prenatal ultrasound and may requirespecial preparations for delivery if the perinatal airway compro-mise is suspected. Diagnosis is usually made in childhood, andif not obvious from birth, it may become apparent with infec-tions such as upper respiratory infection or otitis media, whichcan cause swelling of the malformation caused by increased lymphflow. The lesion will typically grow slowly but as mentioned mayrapidly swell with infections or with hormonal changes such aspuberty. Patients may have complaints of deformity, pain, airwayobstruction, odynophagia, dysphagia, speech difficulty, and possi-bly infection of the malformation itself [72].

On physical examination, the lesions usually feel fluid filledand are non-compressible, which can help in distinguishing themfrom venous malformations. Mucosal and skin surfaces can beaffected with vesicle formation, which represents small externalfluid-filled cysts which resembles “frog spawn” [74]. Vesicle forma-tion causes problems with bleeding, weeping of lymph fluid, andpain [34].

3.4.1. Arteriovenous malformationHigh-flow malformations can be separated into arteriovenous

fistulas (AVFs) and AVMs. AVFs are the result of trauma and initiallyconsist of one or several shunts between arteries and veins. AVFsare usually treated with surgery or with intravascular embolization.AVMs are generally felt to be congenital malformations consist-


ing of a ‘nidus’ of abnormal capillary beds shunting blood fromthe arterial system directly into the venous system resulting ina high-flow vascular abnormality [34]. Arteriovenous malforma-tions of the head and neck (extra-cranial) are high-flow lesions

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nd among the most serious of the vascular malformations becausehey are difficult to diagnose, treat, and cure. They grow through-ut life with frequent, dramatic, and aggressive growth spurts as aesult of various environmental influences. AVMs are very destruc-ive, infiltrative, and often life-threatening secondary to massiveleeding.

Clinically, extracranial AVMs behave differently from AVMsound in the brain. Most common areas of occurrence are the cheek,ips, neck, scalp, neck, ear, tongue, and mandible [75–77]. Arteriove-ous malformations vary in their histopathology and presentation,uggesting a spectrum of disease and not just one disease process78]. Most AVMs presenting before the age of 20 will have a his-ory that includes the presence of a vascular blush in the overlyingkin as a child that then began to expand and bleed. The historyhows that the vascular lesion grew more rapidly as the patientntered puberty or had other hormonal changes. Patients over thege of 40 will often give a history of recent onset of the lesionsot having been noticed previously. They may also give a historyf trauma to the involved area prior to noticing it. Bleeding, pain,nd tissue destruction are often subsequent signs in AVM growth,nd patients suffer significantly when the malformation reacheshis stage. On physical examination, early AVM lesions may haven overlying vascular blush in the skin similar to an early port-winetain. The underlying tissue is usually thickened and is not fluctuantr compressible but can be pulsatile. If mucosa is involved, it is usu-lly thickened and vascular. The more advanced lesions may havebviously enlarged vessels in the skin and underneath and pulsa-ion usually present. AVMs can invade the skin where ulcerationsnd bleeding are common [34].

.4.2. Treatment of vascular malformationThe choice of treatment depends on the type of malformation

vascular content), location, depth, and characteristics of flow ofhe lesion [10].

.4.3. Venular malformationLike other vascular malformation, capillary malformation never

egresses spontaneously. The choice of the treatment depends uponctasia (diameter) of the vessels [43]. Pulse dye laser is now con-idered the treatment of choice for capillary malformations, usingavelength of 577 nm and more recently 585 nm [79].

For vascular lesions the target chromophore is oxyhemoglobinnd selection of a laser wavelength that is preferentially absorbedy oxyhemoglobin is crucial. Lesions resistant to pulsed dye laseray respond to KTP, copper vapor, argon and other new lasers

80–82].Lin et al. [83] reported a study where capillary mal-

ormation treated with photodynamic therapy also called ashotochemotherapy results in endothelial cell injury and death ofbnormal capillaries under overlying epidermis. Tremaine et al.84] showed the enhance port wine stain treatment efficacy byombination of photothermolysis and 5%imiquimod cream whichas immunomodulator with antiangiogenic effect. Other promis-

ng modalities include topical and systemic photodynamic therapy,lectrical optical synergy technology, pulse stacking of similar oriffering wavelengths, use of optical clearing agents in conjunc-ion with laser, and erbium laser epidermal stripping before laserreatment. Orten et al. suggested that the etiology of the malfor-

ation could explain the high recurrence rates, as any abnormallynnervated vessels in the superficial papillary plexus that were leftehind would lead to recurrence [80].


.4.4. Venous malformationMultiple treatment options exist for venous malformations,

ncluding conservative measures such as head of bed elevationnd compression, laser therapy, sclerotherapy, and surgery [34].


Conservative management of venous malformations is usuallyreserved for smaller isolated asymptomatic lesions and is alsoimportant in controlling the growth and symptoms of larger lesionsbeing treated with other treatment modalities. Low doses of aspirinminimize phlebothromboses. Preoperative control of intravascularcoagulopathy with heparin is considered before the resection oflarge venous malformations [85]. Acquired adult venous malfor-mations are commonly seen as small venous lakes in the lower lipand face and can be treated with laser photocoagulation or excision[86,87]. Laser therapy is a mainstay of management of mucosal andskin malformations [88]. Nd:YAG laser form the mainstay of treat-ment for superficial lesion, although the use of KTP laser has alsobeen described [10,34]. According to the literature, sclerotherapywith various agents is the treatment of choice. In large lesions, amultidisciplinary approach may be necessary. It remains a goodoption for the treatment of venous malformations in the head andneck. Sclerotherapy involves percutaneous injection of a substanceto induce inflammation and thrombosis of the lesion which thenwill lead to more long-term fibrosis and hopefully decrease or elim-inate the expansion of the lesion. It is important during injectionthat the sclerosing substance remain within the lesion and notwashout to the main vascular stream to prevent embolic injuryor cardiovascular collapse [34]. To minimize washout, sclerosantshave been developed to slow outflow from the malformation, suchas sotradecol foam or ethibloc (glue), or the sclerosant is mixedwith fibrin glue or ethyl cellulose [89]. Other commonly used scle-rosant are sodium tetradecyl sulfate, 100% alcohol, ethanolamineoleate, a mixture of zein (a corn protein) [10,14,18]. Bleomycin(pingyangmycin) and OK-432 have recently been used as scle-rosants in Asia with promising result [90]. Potential complicationsof sclerotherapy include skin and mucosal injury, airway compro-mise, infection, nerve injury, and cardiovascular collapse. Whilethe more serious of these complications such as cardiovascularcollapse and death are rare, problems with skin injury and scar-ring are more common [91]. Analgesics and anti-inflammatoryagents (nonsteroidal anti-inflammatory agents or corticoids) mustbe given to minimize the symptoms. There should be a time delayof 1–3 months between each sclerotherapy session [18]. Venousanomalies have a propensity for recanalization and recurrence[34]. Surgery is generally contemplated after sclerotherapy whentreatment is incomplete or when an esthetic prejudice requires cor-rection [18]. Surgical therapy is a part of total care management ofVM on the basis of multidisciplinary approach, and it has been fullyintegrated to the embolo/sclerotherapy. Active incorporation of thepreoperative and/or postoperative embolo/sclerotherapy providessubstantial improvement on the overall results of the therapy andexpands the role of surgical therapy.

3.4.5. Lymphatic malformationMany treatment methods have been documented in the litera-

ture, including surgery, various sclerotherapy and laser therapy.Localized microcystic lesions can be resected completely, butimpossible for extensive and diffuse ones, the reasons being: (1)most of them involve the lip, cheek and tongue, complete exci-sion may result in severe tissue defects, leading to cosmetic andfunctional complications; (2) the lesions are poorly demarcated;(3) the walls of lymphatic vessels of LMs are thin and friable; (4)diffuse LMs often involve important structures such as the cranialnerves or vital blood vessels, making complete resection more dif-ficult; (5) complete resection of the lesions is especially challengingbecause of the potential complications, such as facial nerve dam-


age, Horner’s syndrome, postoperative lymphatic leakage, seromaand poor wound healing [92]. These are the most difficult to treat.Macrocystic lesions are more localized and are more easily excised[93].

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Table 4Hudart classification of high-flow arteriovenous malformations according to num-ber and type of arteriovenous connection [104].

Type 1 Arteriovenous No more than 3 separatearteries.

Type 2 Arteriolovenous Multiple arteries shunt to asingle vein.

Type 3 Arteriolovenulous Multiple shunts between



Emery et al. [94] reported that the incidence of facial palsyfter surgical extirpation was 5.9–33%; Hancock et al. [95] demon-trated seromas in 9.8% of wounds in which local drains weresed and 3.6% of the wounds in which drains were not used. Car-ondioxide laser is most commonly used for superficial lesions,ince these vesicles are connected deeply to cisterns, laser abla-ion should be done to depth to insure destruction of most orll of these cistern [10]. Sclerotherapy was advocated by Watsonnd McCarthy early in 1964 [96]. Although a multitude of scleros-ng agents have been tried including hypertonic glucose solution,thanol, quinine, doxycycline, sodium morrhuate, bleomycin andK-432, corticosteroids, etc., bleomycin and OK-432 remain theainstay therapeutic agents for LMs. Yura et al. [97] and Ogita

t al. [98] first reported intralesional injection of bleomycin andK-432 for lymphatic malformations with excellent results. Cura-

ive effect was also achieved with Pingyangmycin, bleomycin A5roduced by Streptomyces pingyangensisn in China [99]. Concernstill remain regarding complications with each sclerosing agents.oxycycline can cause neural damage, OK-432 may be associatedith sepsis, shock, myalgia, and bleomycin still carries a warn-

ng of pulmonary fibrosis [95]. Ethanol is an effective sclerosantut has the highest complication rate. The injection of ethanol isainful and may injure nerves [93]. Bleomycin has good effect forMs located in the tongue, lip, cheek, floor of mouth, palate, etc.,hich could cure small to medium-sized microcystic LMs through

nfiltration injection. Eight milligrams of Pingyangmycin powder isissolved in 5 mL normal saline with addition of 2 mL 2% lidocaineydrochloride and 1 mL dexamethasone (5 mg) [97].

The dosage per injection is 1 mL/cm2 of the lesion as deter-ined by clinical measurement, the maximal dose for one injection

s 8 mg, and the total dose should not exceed 40 mg in an adultatient. Injection is performed in the way of infiltration anesthe-ia, until the surface of the lesion becomes expanded and pale.ultiple injections are needed at different sites for larger or more

xtensive lesions. After injection, the lesion is compressed for 5 mino prevent bleeding and effusion of the sclerosant. If repeatedreatment is required, the appropriate interval is 3–4 weeks. If noesponse is noted after two injections, other treatment optionshould be considered rather than repeated injections. A treat-ent cycle often consists of 3–5 sessions. The dosage for children

hould be reduced accordingly. Laser therapy has been used toreat superficial lymphatic malformations, especially for patientsith localized infection. The advantage of laser therapy is the ease

f use, less bleeding, minimal pain, reliable effect and repeatablereatment. Atropine can be used preoperatively to reduce salivaryecretion. The wavelength of CO2 laser is 10,600 nm (far-infraredight) with 0.2 mm spot size and 0–6 W continuous output power.he laser probe should be kept at a distance of 0.5–1 cm from theurface of the lesions during operation. The laser beam should beligned toward the lesion and gradually cauterize the malformedtructures from superficial surface to deep layer. Besides CO2 laser,d:YAG laser (wavelength 1064 nm, near infrared light), pulsedye laser and diode laser can also be used [100]. Sclerotherapyith Pingyangmycin or OK-432 is the mainstay of treatment foracrocystic lymphatic malformations, and surgery is usually used

s complementary therapy [101]. Surgical resection should be asadical as possible in the given anatomic regions. Single macrocys-ic LMs of the neck have a high cure rate through surgery, whileecurrence is common in neonatal patients. The result of surgicalxtirpation is poor for macrocystic LMs involving the tongue, floorf the mouth, cheek, pharynx or multiple anatomic sites, often withigher complication rates, including facial nerve injury. Surgery


hould be performed early in some circumstances, for example, LMsn the orbital cavity and surrounding regions, which often causeisfigurement and diminished vision and early surgical excisionusually subtotal) may alleviate these problems [96]. If complete

arteries and venules.Multiple nidi.

resection is impossible, it is advisable to manage different anatomicareas as individual problems, and a “top down” approach is appliedto prevent superior swelling of the untreated zone [95].

3.4.6. Arteriovenous malformationThere is no agreement on the single best method to access

and treat AVMs. A number of access routes is available; transve-nous (TV), direct stick (DS), transarterial (TA) or a combination.The best approach is to treat each AVM on a case-by-case basis,depending on AVM site and classification (Table 4) [102]. Inter-vention should be planned as soon as the diagnosis is made andtimely intervention will not only prevent complications, but alsoallow the extent of resection to be considerably reduced [10]. Theoptions include embolization, resection and combination. Many ofthe studies reported using arteriogram and embolization with vary-ing substances, such as Gelfoam, polyvinyl alcohol, silicone fluidand isobutyl-2-cyanoacrylate (IBCA) [103].

Surgical resection after embolization is another common treat-ment modality [78]. This combination is indicated when the AVM issmall and appears to be focal and completely resectable. Surgery isalso indicated in a very large AVM, which is life-threatening becauseof bleeding and invasion [10]. Electrochemical treatment is gainingin popularity as a high technology method to treat tumors. Its firstapplication in China was to treat malignant tumors by Xin et al.The fundamental principle is the severe chemical change in lesionsinduced by electricity through platinum needles, which changesthe ion concentrations among cells. It results in electrolyte distur-bances; acid–base imbalances; electroosmosis and electrophoresisas a result of the effects of electrolysis; and changes in the electro-chemistry and electrophysiology of the local lesions. On the positivecolumn the pH went down to 1–2 and showed a strong acid reactionthat would result in dehydration of tissues and extensive formationof microthrombi. On the cathode column the pH rose to 11–13 andshowed a strong alkaline reaction that would result in edema ofthe tissues and compression of capillaries. All these changes lead tocoagulation of protein, and denaturation and necrosis of the lesions.These in turn destroy the structure of the tumor and activate therepair mechanism of the organism. Xue et al. used this procedurein high flow venous malformation with good results [104].

4. Summary

Vascular anomalies represent a wide variety of vessel abnor-malities. In this review, we summarized the important clinicaldifferences between hemangiomas and vascular malformationsand the management of these disfiguring congenital anomalies. Theuse of proper terminology and better understanding of the naturalhistory will lead to better outcomes for these patients.

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Vascular anomalies in maxillofacial region—Review M.E. Shama,∗, Nishat Sultanab. Journal of Oral and Maxillofacial Surgery, Medicine, and Pathology