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American Journal of Advances in Medical Science www.arnaca.com eISSN: 2347 -2766 pISSN: 2454-9061 Review Article Vascular lesions of the Head and Neck: A Review Upender Malik1*, Shilpa Dutta Malik2, Shally Raina1 Department of Oral Medicine and Radiology, Seema Dental College and Hospital, Rishikesh249201, Uttarakhand, India 2 Department of Oral Pathology, Seema Dental College and Hospital, Rishikesh-249201, Uttarakhand, India 1 Abstract Vascular masses which are capable of causing significant abnormalities and life threatning conditions are heterogeneous group of lesions derived from blood vessels with differing histologies, clinical courses, imaging appearances and treatment options,with vascular malformations always present at birth and growing in proportion to body growth and never regress spontaneously. In the present review article , an attempt has been made to draw attention to the current classification, terminology, clinical features, natural history, diagnosis, various syndromes associated and management of these lesions. Key words: vessel. Hemangiomas, vascular malformations, involuting, non involuting, feeder Cite this article as: Upender Malik1, Shilpa Dutta Malik, Shally Raina. Vascular lesions of the Head and Neck: A Review. American Journal of Advances in Medical Science.2015; 3(4):9-20. Introduction Vascular masses are a heterogeneous group of lesions derived from blood vessels with differing histologies, clinical courses, imaging appearances and treatment options. These lesions are of great importance as they are capable of causing significant abnormalities and life threatning conditions. Before 1982, there was a great confusion regarding the classification of vascular lesions owing to their complex constitution and varied clinical features. Mulliken & Glowacki in 1982 gave a classification for vascular lesions on the basis of histological and clinical characteristics which divided these lesions broadly into Hemangiomas and malformations. [1]. Three types of benign blood and lymph vessel tumor (hemangiomas, lymphangioma and hygroma) American Journal of Advances in Medical Science www.arnaca.com not only have a common origin, but grow in an identical fashion by projecting buds of endothelial tissue. Hemangiomas are the most prevalent benign tumors of infants and children and are generally composed of vascular spaces, they are known as true Hamartomas as they arise from endothelial cells and not by incorporation of nearby vascular channels and hence are haratomas rather than true neoplasms. Vascular malformations on the other hand are thought to result when there is interruption at the particular stage of development of a vessel hence its type is dependent on the stage at which normal morphogenesis is interrupted. As the commonest site of occurrence of these vascular lesions are head and and neck, the massive disfigurement caused by unusually Vol-3: No-4: 2015 9 Conflict of Interest: None declared Page Source of Support: Nil 10 Vascular lesions of the Head and Neck: A Review Page 10 abnormal lesions are of great concern to the patients psychologically and the presence of these lesions at locations such as nose, eyes and viscera’s indicates immediate treatment in order to avoid life threatening complications. In this review article, clinical, radiological, pathological and management aspects of these lesions along with a classification system that can be followed clinically as well as pathologically are discussed. Classification of Vascular lesions Before the 1980s, terminologies such as (portwine stain, strawberry haemangioma, salmon patch) were only based upon clinical appearance and lacks correlation with the biological behavior or natural history of these lesions [2]. Mulliken and Glowacki developed a system of biological classification of congenital vascular lesions & divided these lesions broadly into two distinct entitiesHemangiomas and vascular malformations. Original classification as given by Mulliken and Glowacki in 1982 as follows: Classification of vascular lesions in infants and children Hemangiomas Proliferating phase Involuting phase Malformations Capillary Venous Arterial Lymphatic Fistulae Further, Hemangiomas have been categorized into congenital (which is present at birth) and more common (which appear after birth). Congenital Hemangiomas are further subdivided into rapidly involuting congenital Hemangiomas (RICH), non involuting congenital Hemangiomas (NICH) and congenital non- progressive hemangiomas. On the basis of this further categorization an improvement was made in the original classification [3]. Improved classification of Hemangiomas and vascular malformation Hemangiomas Superficial (capillary hemangioma)* Deep (cavernous hemangioma)* Compound (capillary cavernous hemangioma)* Vascular malformations Simple lesions Low flow lesions Capillary malformation (capillary hemangiomas, port- wine stain)* American Journal of Advances in Medical Science www.arnaca.com Venous malformation (cavernous hemangiomas)* Lymphatic malformation (lymphangioma, cystic hygroma)* High flow lesions Arterial malformations Combined lesions Arteriovenous malformations Lymphovenous malformations Other combinations (*) old terminology within brackets. Hemangiomas Hemangiomas soon appear after birth, although upto 30% may already be apparent at birth. They typically proliferate during the first year of life and then involute during the childhood years. Vast majority of Hemangiomas are found deep in or just deep to the skin, those orginating in papillary dermis stretch the overlying skin as they proliferate and present as a bright red macular or papular mass. Lesions originating within the reticular dermis or subcutaneous tissue sometimes expand the overlying skin and separate it from the epidermis by a layer of collagen, imparting a bluish hue to the epidermis or appear colorless. On occasion both superficial and deep components will be present, and the resultant lesion will have elements of both capillary hemangiomaand a cavernous hemangioma. These lesions are known as ‘capillary cavernous’ hemangiomas. Strawberry and capillary cavernous Hemangiomas are in reality superficial and cavernous Hemangiomas are deep. The term compound hemangiomas is used for both capillary cavernous types. During the proliferative phase, tubules of plump proliferating endothelial cells with frequent mitosis are a dominant histological feature. As the end of proliferation draws near, mast cells become less active, and, as this happens, they progressively flatten until, during involution, hemangiomas are made up of flat, inactive, normal appearing endothelial cells, surrounding large ecstatic vascular channels in a matrix of fibro-fatty tissue. Natural history of Hemangiomas They are the most common tumors in infants with very rare incidence among Asian and black children (0.8% - 1.4%). Familial tendency although not present still 10% of all white infants are affected, (1 out of every 10 children with hemangiomas will have a positive family history) [4]. Although 30% are present at birth, the majority present during the first weeks of life showing an earliest lesion as a blanched macule which increases in contrast when the child cries. Blanching seen in early lesion is due to the over activity of endothelial cells resulting in plumping of Vol-3: No-4: 2015 Vascular lesions of the Head and Neck: A Review 11 Figure-2: Rapidly proliferating superficial (capillary) Hemangiomainvolving the upper lip showing ulcerations in one year old female patient Most hemangiomas are solitary focal lesions, and the head and neck is the most common site (60%) of involovement [5]. In the head and neck, focal lesions may appear anywhere along a line starting in the middle of the cheek and passing lateral to the eyebrow, then over the top of the eyebrow to the glabella. From there the line passes medial to the medial canthus, down to the paranasal sinus area, and then across the alar groove to the nasal tip and columella. The midline of the upper lip and the lateral aspect of the lower lip are also the common and most involved sites (Figure-1). Exact reason for site predominance is not known, but can be contributed to the appearance of lesions American Journal of Advances in Medical Science www.arnaca.com Figure-3: Portwine stains involving the trigeminal dermatome Proliferation History of proliferation generally during the first year of life is present, but lesions fully grown at birth also are seen which involute rapidly within few months of life [6]. Highly variable rate and timing of growth are present usually during neonatal and early infancy and a growth spurt in between 4 and 6 months of age with ultimate size of the lesion depending upon the degree and the duration of proliferation. Features seen in such lesions are characterized by plump proliferating endothelial cells [1]. In the early stages, sheets of proliferating endothelial cells are evident with apparent disorganization and an absence of vascular channels. Mast cells occur in the early and middle of involuting phases [2]. The physical features of the hemangioma depend on its depth with respect to skin, its size, and its stage in involution. A superficial hemangioma will be present as a bright red mass, whereas a deep lesion will be bluish or colorless, the main difference being the depth of the lesion with respect to the collagen layer of the papillary dermis. Since collagen scatters visible light and red light penetrates deeper than the blue light, red blood vessels appear bluish through collagen in the same way a deep nevus made of dark brown pigment, will appear blue through collagen. During involution superficial lesions will turn a more dusky color, and, by late involution, they will have assumed a decidedly purple color. Hemangiomas range in size from a pin head to the size of infants head. Proliferating heamngiomas are firm in Vol-3: No-4: 2015 11 Figure-1: (A) sites of predilection of focal Hemangiomas of the face. (B) An alternative patten with diffuse involvement tends to follow trigeminal (V) dermatomes. corresponding with lines of embryological fusion. Perhaps, totipotential cells at these sites retain their ability to develop into endothelial cells and /or pericytes, and, under the influence of angiogenesis stimulation, they develop into blood vessels. A less common, alternative pattern of diffuse involvement tends to follow the facial dermatome distribution. Page the cells causing temporary block in the internal vascular space, leaving little or no room for red blood cells. Continued proliferation eventually increases the vessel diameter, thereby allowing more room for whole blood and thus eliminates the blanched appearance. As the process continues, an increase in vessel number will give rise to the appearance of discrete telangiectasia, which, by sheer force of vessel number, eventually coalesece to form red macule. 12 Vascular lesions of the Head and Neck: A Review Page 12 consistency and will expand with raised intravascular pressure (Figure- 2). Involution By the end of the first year of life involution begins which is clinically evident as a definite diminution in the rate of growth. In time, hemangiomas will feel less tender to palpation and less tense. Eventually the lesion will soften and begin to shrink from its center to its periphery in a radial pattern. Only 40% of hemangiomas involute with acceptable result. Therefore 60% of the patients require some kind of corrective surgery. Complications of Hemangiomas Ulceration It is one of the most common complications and occurs in up to 5% of cases.. Two possibilities which may explain this are, firstly rapid proliferation may distend the overlying skin, which eventually reaches its elastic limit, splits, and, in doing so, forms an ulcer. Secondly, because of the typical hemangioma derive its blood supply from a circumferential network that in turn gives off feeding branches at right angles to the network, a rapidly proliferating hemangiomamay well outstrip its blood supply and necroses at a point most distant from the blood supply (i.e. the center). Airway Obstruction emangiomas obstructing both nasal passages can cause serious problems in infants. Laryngeal hemangiomas always pose a life threatening problem. Cough, cyanosis, and occasionally hoarseness may be present in about 50% of all the A large cervical parapharyngeal or palatal hemangiomamay also encroach on the upper airway and cause acute or sub-acute obstruction. Auditory obstruction Parotid hemangiomas may obstruct the external auditory canal. Visual Obstruction Periorbital hemangiomas may obstruct the visual axis and this in turn results in stimulus deprivation amblyopia, and may lead to blindness if not aggressively treated [7]. Even in the absence of obstruction, anisometric (astigmatic) amblyopia may occur in association with a hemangiomaof the upper eyelid. Paralytic strabismus and strabismus secondary to ambylopia may be found [8]. Haemorrhage Trauma to the lesion mostly occurs in lesions which are in exposed locations. Usually 10-15 minutes of pressure stops the bleeding. Hemorrhage is of a great concern in lesions associated with bleeding disorders such as Kassabach-Merrit syndrome where it becomes challenging to stop the bleeding. American Journal of Advances in Medical Science www.arnaca.com Congestive Heart Failure High output cardiac failure is a serious complication and carries a significant mortality rate. High cardiac output failure is much more seen in multiple cutaneous and / or visceral and / or hepatic lesions. This complication occurs in rapidly proliferating stage (early infancy), with presenting symptoms of congestive cardiac failure, anemia and hepatomegaly. Skeletal Distortion This is more common due to mass displacement effect, includes anterior displacement of the mandible by massive parotid lesion, outer calvarial changes of the skull, flattening of the nasal pyramid by large glabellar lesion and orbital expansion. Systemic Hemangiomas Multiple cutanoeus Hemangiomas cases should be investigated for the potentiality of systemic involvement. This condition is known as systemic Hemangiomatosis. An abdominal ultrasound or MRI scan is usually diagnostic. Vascular malformations Vascular malformations are true developmental anomalies that were believed to result from sporadic, non familial developmental error in the formation of vascular tissue. Genetic background involves mutation at position R894W resulting in increased activity of tyrosine kinase TIE2 in two families with venous malformations.TIE2 is essential for early vascular development, and an increase in TIE2 activity may lead to abnormal sprouting, branching and modeling of the primary vascular plexus [9]. Abnormality in vascular neural malformation also leads to at least two types of malformations [10]. Relative or absolute deficiency in autonomic innervations of the post capillary venular plexus can be sited as a reason for Venular malformations, and arteriovenous malformations can occur due to the same deficiency but at the level of precapillary sphincters [3]. Venous malformations may seem to be due to uncoupling of proliferating endothelial cells and smooth muscle cells [11]. Involvement of collecting system vessels (i.e. capillaries, venules, veins and lymphatic vessels) and not arteries is common to all vascular malformations. Vascular malformations are always present from birth, even though they may not be obvious and only manifest at a later date [2]. Sexual and racial predilection is not present. Rate of endothelial turn over is normal hence, neither proliferates nor involute. A slow steady increase in size and/or thickness of the lesion will take place due to hyperplasia. With advancing age Vol-3: No-4: 2015 Vascular lesions of the Head and Neck: A Review 13 progressive ectasia (dilatation) of the vascular component will lead to expansion of the lesion and in some instances nodularity [12]. ISSVA Class Trauma, sepsis, hormonal modulation or changes in blood or lymph pressure are factors associated with rapid increase in size. Syndromes associated with vascular lesions [22] Associated Clinical findingsLINICAL FINDINGS conditions Tumor Kaposiform Haemangiond othelioma Tufted angioma Spindle cell Haemangiond othelioma Diffuse neonatal haemangiom as Lumbosacral haemangiom as KasabachMerit Syndrome KasabachMerit Syndrome Maffuci Syndrome Mutifocal cutaneous involvement [24] Hemangiomas with possible visceral Associated with tethered cord imperforate anus renal anomalies and bony abnormalities of the sacrum Thrombocytopenia, Hypofibrinoginemia congulopathy in association with vascular tumor and occasionally in association with lymphatic malformation [25] thrombocytopenia, Hypofibrinoginemia congulopathy in association with vascular tumor and occasionally in association with lymphatic malformation [25] Multiple hemangioendotheliomas endochondromas associated malignancy [26] Venular malformations Midline Venular Malformations are commonly referred to as salmon patch, stork bite or angels kiss (older terminologies). Lesions are often transient and fade within first year of life, often involve midline structures with nape of the neck is the common site followed by anterior midline lesions involving upper eyelids, forehead, glabellum, nasal alae, and philtrum of the upper lip plus the lumbar sacral area. Anterior midline lesions involving forehead and glabellum are typically V shaped and correspond with the distribution of the supra trochlear and supra orbital nerves. Involvement of the nose is typically in the supra alar crease, and the lip involvement is typically in the upper two thirds of the philtrum. These lesions presents as light pink macule, this may be confluent with distinct border or non confluent. Anterior midline lesions are non confluent where as posterior lesions are confluent. Venular malformations (Portwine stains) These are true malformations of post capillary venules within the papillary plexus. Etiology involves sick dermatome theory in which there is an absolute or relative deficiency of vascular autonomic and sensory vascular innervations as the underlying pathology. Lesions with an absolute deficiency will progress more rapidly and early hypertrophy with cobblestoning is likely, whereas a relative deficiency of autonomic innnervation will give rise to slower progression. Males and females are affected equally with lesion presenting as a flat, pink macule mostly on the head and neck. Involvement of sensory dermatome i.e. trigeminal dermatome completely or partially can be seen. V2 dermatome is most commonly involved followed by mandibular V3 and then ophthalmic V1 dermatome (Figure-3) [13]. Mucosal involvement is common, especially if midfacial V2 lesion is present where vermillio-cutaneous border, lip mucosa, and maxillary gingival may be involved. These lesions progress with advancing age and by puberty reaches to a dark deep red lesion which soon progress to dark purple, thick, cobblestoned portwine stain by 30 years of age. The natural history relates to sick dermatome theory according to which early and more rapid progression of the lesion is due to relative or absolute deficiency in the innervations of the venular plexus. Early darkening, thickening, and cobblestone formation can be expected. Patients in American Journal of Advances in Medical Science www.arnaca.com Vol-3: No-4: 2015 13 Haemangioma of infancy Posterior fossa malformations. Haemangioma of the face, arterial Anomalies (especially the cerebral Arteries, cardiac anomalies and coarctation of the aorta. Eye abnormalities (such as microphthalmia.Homer syndrome, cataract increased retinal vascularity). PHACE may also be associated with sternal cleft, supraumbilical raphe and a variety of other brain anomalies. Represents a spectrum of findings. Not all features need be present to diagnose PHACE syndrome marked predominance in females [23]) Page PHACE Syndrome 14 Vascular lesions of the Head and Neck: A Review Page 14 whom there is a relative deficiency will, on the other hand, experience slow progression of their lesion. Skeletal changes are sometimes associated in the form of bony hypertrophy. This is especially true of V2 lesions that extend onto mucosal and gingival surfaces of the maxilla. Localized hypertrophy of the underlying bone, increased spacing between the teeth and a prominence of the upper lip, due in part to underlying hypertrophy of the alveolar ridge is seen. While soft tissue hypertrophy is common, especially in older patients, the role of bony hypertrophy is often unrecognized, and can lead to unnecessary and ineffective surgical debulking of the lip. Struge-Webber syndrome in which dermal vascular plexus involvement along with choroid involvement and ipsilateral meningeal invovlment is associated with portwine stains. Patients with this syndrome are present with glaucoma and sequelae of neural involvement such as seizures, cerebral atrophy, degeneration and subdural hemorrhage, the cutaneous component of strugeweber syndrome is similar to venular malformation accept it involves both sides of face. Hypertrophy of subjacent maxilla and mandible and soft tissue hypertrophy of the skin and appendages are also more common. Combined malformations Capillary Heperkera Venous totic cutaneous Cerebral capillary malformations [Eerola et al, 2000] capillary Venous malformat ions Klipped Capillary, deep and superficial venous and arterial malformations Trenaunay with limb asymmetry [Berry etal. 1998] Complex malformation and CapillaryLym syndrome overgrowth syndrome with vascular and or lymphatic malformation phatic[Biesecker et al 1999] Venous Proteus Similar to Klippel Trenaunay syndrome with AV fistula syndrome Capillary Parkessimilar to Klippel Trenaunay syndrome with AV fistula and possible lymphatic Weber associated high output congestive heart failure Schachnerand and venous syndrome Hansen, 1996]. arterial Venous malformations Venous malformations may be superficial or deep, localized, multicentric or diffuse. The overlying skin or mucosa varies in color depending on the depth and degree of ectasia of the underlying malformation. Because expansion invariably progresses along planes of least resistance, superficial malformations will expand toward the surface or the mucosal surface and impart a deep purple discoloration. Deeper malformations impart varying degrees of bluish hue-the deeper the lesion, the less is the discoloration. Very deep lesions such as buccal fat space lesions appear as flesh color mass. The overlying skin is not discolored and is usually soft and compressible. Expansion of the lesion occurs on crying due to increase in venous pressure. Phlebothrombosis occurs with a greater frequency in ectatic lesions and phleboliths are consider the hallmark of venous malformations. These lesions may be unifocal or multifocal and certain sites such as buccal mucosa, the tongue, the oral commisure, the lower or the upper lip, the buccal pad fat space and neck are more commonly involved in head and neck region. Anatomical borders are not a limitation for venous malformations American Journal of Advances in Medical Science www.arnaca.com and hence, muscle, salivary glands and skin infiltration are common and on occasion, lesions may be entirely intraosseous. Mandible is most commonly involved followed by maxillary nasal, parietal and frontal malformations [14]. Mandibular lesions usually appear as painless, slow growing masses. Increased spacing between teeth and increased mobility of the teeth due to the expansion of the buccal cortical plate and bleeding around the gingival necks of the teeth are all the manifestations [15]. Uncontrolled and massive bleeding from the tooth socket immediately after extraction may be the first sign of an intraosseous venous malformation. Other than this, bony hypertrophy and/distortion along with soft tissue hypertrophy can also be evident. Lymphatic malformations All congenital malformations of the lymphatic system that results in a contiguous mass of dilated lymphatics are known as lymphatic malformations. In these malformations the primary defect is believed to be at the level of the efferent channels. Obstruction at this level, whether relative or absolute, will result in dilation of the proximal channels that form the mass Vol-3: No-4: 2015 Vascular lesions of the Head and Neck: A Review 15 [16]. Although present by birth, some lesions manifest at a later stage. 65% to 75% are diagnosed at birth, and 80 to 90% are Capillary Venous Cutis marmorata telangiectatica congenital Phakomatosis pigmentovascu laris Robert-SCPhocomelia WiedemannBeckwith syndrome Hereditary neurocutaneo us angioma Blue-rubber Bleb Nevus syndrome Glomangiomas Capillary (or mixed malformations) of the skin associated with vascular malformations of the central nervous system. [Zaremba et. al 1979; M1M106070] (33) Cutaneous venous malformations and venous malformations of gastrointestinal tract and other sites. Some cases have autosomal dominant inheritance pattern. Cutaneous venous anomalies with glomus cells [34]. Some cases have autosomal dominant inheritance pattern. Traditionally, these malformations are divided into capillary lymphangioma (smallest lymphatic channel malformation), cavernous (intermediate lymphatic channel malformation) & cystic hygroma (dilated macrocystic lymphatic malformations). Head and neck region is the most common site for lymphatic malformations, and over 90% are found in the neck. The complexity of the cervical lymphatic system has been offered as a possible explanation [17]. Clinical manifestations vary in accordance with the extent and depth of the lesion as well as the degree of fibrous reaction around it. Mucosal and cutaneous involvement usually results in the formation of multiple cutaneous or mucosal fluid filled vesicles. These vesicles may be connected to larger, deeper lymphatic cisterns lying within the subcutaneous or submucosal tissues. The involvement of deeper tissues follows one of the two patterns, either massive generalized oedema with poorly defined borders (the diffuse microcystic variety) or a localized area of multilocular cysts (the macrocystic variety). Cervical American Journal of Advances in Medical Science www.arnaca.com lesions (previously known as cystic hygromas) tend to be localized, macrocystic and thus amenable to complete surgical resection. The rate of growth varies the earlier the diagnosis, the more aggressive the lesion. Low grade lesions present later and are less inclined to result in complications. Sudden or rapid expansion may result from sepsis and spontaneous or traumatic interlesional hemorrhage. Both these complications are frequent, and their abrupt onset may precipitate a life threatening emergency due to airway obstruction. Cervical lesions may displace and eventually compress the the pharynx, and mediastinal extension may compress the trachea [17]. Endolaryngeal cystic mucosal blebs may also embarrass and on occasions, totally obstruct the airway. Soft tissue and skeletal distortion are extremely common. Macroglossia, macrotia and macrochelia are sequelae. Mandibular hypertrophy will give rise to prognathism and malocclusion. Due to the Vol-3: No-4: 2015 15 Sturge-Weber syndrome Simple malformations Capillary or mixed malformation overlying spine(sometimes extending to leg) with associated vascular malformation in the spinal cord within one or two spinal segments [27] Capillary malformation of the face with eye disease (choroidal vascular malformation glaucoma buphthalmos) and neurologic disease (leptomeningeal vascular malformation seizures hemiparesis etc) [28] Reticulated capillary malformation or mixed malformation may be associated with many anomalies especially port wine stain, cutaneous atrophy and or ulceration, macro cephaly, ocular abnormalities neuromotor symptoms limb asymmetry etc. [29] Association of capillary malformation and dermal melanocytosis nevus spilus or the other pigmentary anomalies. May have extracutaneous abnomalities such as psychomotor retardation epilepsy intracranial calification and eye abnormalities [30] Sever tetraphocomelia cleft lip and palate mental retrardation poor survival. Midfacial capillary malformation in many cases. [31] Abdominal wall defects overgrowth facial anomalies with mid facial capillary stain [32] Page Cobb Syndrome diagnosed by the end of the second year of life. The remainder may manifest as late as the first pregnancy or even later. Page 16 16 Vascular lesions of the Head and Neck: A Review establishment of venous lymphatic shunts spontaneous regression has been reported. Arterio-Venus malformations The term arteriovenous malformation refers to a congenital malformation made up of multiple fistulous tracts communicating between arteries and veins resulting from the abnormality at pre capillary sphincters. These lesions are present by birth but clinical presentation usually is delayed with third, fourth, or fifth decade of life may be the time when these are evident. The most common manifestation of an arteriovenous malformation is a mass complex comprised of dilated tortuous arteries and veins, occasionally with an overlying portwine stain. Ulceration of the overlying skin and skeletal hypertrophy are also seen. AV malformations are firmer to palpation than venous and do not empty as readily as venous malformations on compression. Once compressed, they rapidly refill. Pulsation or fluid thrill is uncommonly associated. These malformations are firm vascular lesions surrounded by hypertrophied arteries and dilated veins becoming prominent with advancing age. An increased circulating blood volume will counter the effect of decreased arterial pressure and in turn lead to an increased stroke volume, tachycardia and cardiomegaly. High output cardiac failure is rare, but has been reported in infants with large arteriovenous malformations [18]. Indeed, an otherwise healthy infant can adequately compensate for an increased cardiac output for many years before decompensating [19]. The primary abnormality could be due to an ecstatic capillary bed. An absence of autonomic nerve supply to the sphincters, an absence of actual sphincters, or some deficiency at the neuroreceptors resulting in the free flow across that particular capillary bed could be the cause if ecstatic capillary bed. In time, the vessels in the bed dilate, and eventually the area supplying the arteries hypertrophy and vein dilate. The variation in the age of presentation and speed of progression is due to absolute or relative absence of capillary sphincter control. Ligation of the feeding artery is therefore in effective in both above and below the arteriovenous malformation. A proximal arteriovenous malformation with a significant shunt may cause an increase in cardiac output that in turn may lead to high output cardiac failure. Distal shunting on the other hand has the propensity to significantly reduce the flow rate beyond shunt and so induce peripheral ischaemia (steal syndrome) without adversely affecting cardiac output. Because of the presence of high to low American Journal of Advances in Medical Science www.arnaca.com pressure shunt, an increase flow in the afferent artery may be seen due to the decreased peripheral resistance leading to dilatation and tortuosity of the afferent artery with subsequent thickening of the wall due to hypertrophy in the media. In the face of a large shunt across an arteriovenous malformation, the arterial flow distal to the shunt may reverse direction, thus causing distal ischaemia, the so called steal syndrome. The decrease in the arterial pressure also encourages the development of an extensive collateral circulation. An increased flow through the arteriovenous malformation may also dilate the efferent venous system with consequent thickening of the wall due to hypertrophy of the media. As the lesion matures, the degree of ectasia increases, and the development of venous dilatation and arterial hypertrophy becomes apparent. Oral and maxillofacial hemangiomas and vascular malformations are congenital lesions with various clinical characteristics, manifestations, indications, and possibilities for treatment. Diagnosis is made on the basis of clinical examination and patient’s history. However, the clinical examination underestimates the deep extension of the lesion. The feasibility of treatment and the plan of approach depend on the accurate delineation of the extent, size and location of the lesion, Therefore, complimentary radiographic studies are essential for treatment [20]. The physical examination of the child with a vascular lesion typically reveals a soft, raised, nontender, well circumscribed lesion that varies in color from blue to red or may have no color variation from the overlying skin or mucosa. It may blanch and fill with digital pressure if it is superficial and contains blood. A bruit and pulse may be present in the case of a high-flow arterio-venous malformation, and it should transilluminate if it is a macrocystic lymphatic malformation. A direct flexible laryngoscopy is often very helpful in evaluating for a possible subglottic hemangioma. Because it is a dynamic examination in an awake child, other anatomic and neurogenic problems can be ruled out including laryngomalacia, vocal cord paralysis, reflux laryngitis, and laryngeal web. The subglottic hemangiomas may be visualized as a raised pale to reddish lesion most commonly on the left and posterior, but it can be in any position and bilateral. Because of inherent limitations in flexible laryngoscopy, direct laryngoscopy under general anesthesia may be required, but biopsy should not be needed. If the overlying skin is involved, contrast imaging should be Vol-3: No-4: 2015 American Journal of Advances in Medical Science www.arnaca.com patient sedation. Gray-scale US is good for identifying the extent of the lesion and echogenicity, which relates to cystic versus solid features. When color flow Doppler capabilities and spectral analysis are added, the direction and velocity of flow of blood within the lesion can be assessed, which can differentiate between high and low-flow lesions. Recently it was demonstrated that US can differentiate a Hemangiomafrom a vascular malformation and there are also reports of identification of 48 of 49 Hemangiomas based on their solid appearance on gray-scale US. The nondiagnostic Hemangiomawas isoechoic with surrounding tissue and could not be identified, presumably because it was involuted. Thirty-eight vascular malformations were also studied which showed Characteristics of color Doppler US and spectral analysis to be useful in determining the presence of arteries within the Hemangiomas and in defining the nature of each vascular malformation. Other imaging studies useful in evaluating vascularlesions include plain radiographs, CT scanning, and MRI. Plain films are of value in determining bone or joint involvement, growth disturbance or presence of phleboliths.CT is useful in imaging the detail of underlying bone. MRI is superior in defining soft-tissue differences and vascular anatomy. An MRI protocol suggested by Fordham et al. included T2weighted sequences, T1-weighted images with and without gadolinium, gradient-echo sequences, and MR angiography or MR venography as needed. Also, MR lymphangiography may be used to evaluate lymphatic malformations. In fact, MRI has replaced CT as the imaging study of choice in evaluating these lesions. Dubois and Garel designed a diagnostic algorithm for detecting Hemangiomas and vascular malformations in which lesions are generally categorized as typical or atypical. Typical lesions that do not need to be treated require no investigation, whereas pretreatment investigation should be done for the other typical and all atypical lesions. In this algorithm, US is used as the initial imaging study for all atypical lesions and biopsy is recommended for moderate flow atypical lesions. Tatsurou Tanaka et al, described fusion of 3D phase contrast MRA (3D- PCMRA) images without contrast using heavy T2 weighted images with 3D- FASE sequences in patients having head and neck Hemangiomas and concluded that the fused images allowed for the non invasive assessment of the relationship between the Hemangiomas and the arterial system feeding it [21]. Vol-3: No-4: 2015 Page done to ascertain whether there is contiguity with the subglottic lesion. Investigations Diagnosis in Oral and maxillofacial hemangiomas and vascular malformations is made on the basis of clinical examination and patient’s history. However, the clinical examination underestimates the deep extension of the lesion. The suitability of treatment and the plan of approach depend on the accurate delineation of the extent, size and location of the lesion. Therefore, complimentary radiographic studies are essential for treatment [20]. Clinically the patient with a vascular lesion typically should be examined for characteristic features such as soft, raised, non tender, well circumscribed lesion that varies in color from blue to red or may have no color variation from the overlying skin or mucosa. It may blanch and fill with digital pressure if it is superficial and contains blood. A bruit and pulse may be present in the case of a high-flow arteriovenous malformation, and transillumination is present in macrocystic lymphatic malformation. A direct flexible laryngoscopy is often very helpful in evaluating for a possible subglottic Hemangioma. Because it is a dynamic examination in an awake child, other anatomic and neurogenic problems can be ruled out including laryngomalacia, vocal cord paralysis, reflux laryngitis, and laryngeal web. The subglottic Hemangiomas may be visualized as a raised pale to reddish lesion most commonly on the left and posterior, but it can be in any position and bilateral. Direct laryngoscopy under general anesthesia may be required in cases where there is involvement of overlying skin. Contrast imaging should be done to ascertain the contiguity with the subglottic lesion. Imaging Imaging studies are useful in determining the accurate diagnosis and extent of the lesions in question. Lesions located deep to the subcutaneous tissue appear atypical and are difficult to assess on physical examination. Imaging becomes useful in the diagnosis of such cases also differentiation can be made between Hemangiomas and other high, moderate and low flow vascular malformations and other lesions. Imaging studies also document the size of the lesion pretreatment. Imaging modalities most useful in assessing these lesions are Plain Radiographs, Ultrsonography (US), Magnetic Resonance Imaging (MRI), and Computed Tomography (CT) scans. The advantages of US include its wide availability, low cost, noninvasiveness, ease of use, and no need for 17 Vascular lesions of the Head and Neck: A Review 17 18 Vascular lesions of the Head and Neck: A Review Treatment Oral haemangiomas are quite common and do not necessarily require treatment particularly as they may not be visible from the outside. However, positive therapy is indicative if the lesion is aesthetically unpleasant or haemangiomas producing episodes of bleeding mainly due to trauma or in haemangiomas or lymphangiomas that are associated with pain or discomfort. Different modalities of treatment for the vascular lesions are as follows: a) Cryotherapy: The physical principle behind cryotherapy is based on JouleThompson expansion which enables substances to undergo a drop in temperature when moved from a high pressure area to a lower pressure area. It involves application of a number of agents including liquid nitrogen, nitrogen dioxide, liquefied air, CO2 snow, dry ice, ethyl chloride, acetone- alcohol mixture and Freon [34] b) Sclerotherapy: Itis a treatment of choice for (deep) cavernous haemangiomas which involves injection of a sclerosant, namely 3% sodium tetradecyl sulphate into the lesion with the patient head in the down position(maintained for 5 minutes), this allows sclerosant to work. Head is then brought back into the erect position in order to empty the lesion and pressure dressing is applied. The whole procedure brings intimal damage thereby initiating coagulation and contraction using agents like invert sugar, sodium molybdate, ethanolamine oleate and absolute alcohol have been described. c) Embolization: This method occludes the blood vessels proximal to the lesion and control lethal blood loss. The indications for arterial embolization include the management of acute hemorrhage, the management of tumors, the treatment of arterio-venous malformations etc. embolization as a single treatment modality is rarely successful. In high flow (arteriovenous) malformations, due to occlusion of the proximal vessels resulting only in stimulation of the growth of the collateral blood supply, makes treatment complicated. Thus multiple approaches are usually preferred. Precutaneous embolization using histoacrylic glue is reported in the treatment of giant mandibular arteriovenous malformation of the mandible [36]. d) Lasers CO2 lasers: functioning in the far infrared at 10.6/- Lm may be used to remove small haemangiomas of the cheek and tongue. Its used to carry out excision rather than vaporization in the band of tissue [35]. Nd-YAG lasers: functioning in the near infrared at 1.06/- Lm has maximal tissue penetration combined with minimal absorption by comparison with CO2 laser. It should be used with caution in the region of superficial nerves as it will destroy the integrity of the nerves. Tuneable dye and copper vapour lasers: it is the method of choice for port-wine stains of the facial region and has superseded the earlier use of the argon laser in the visible blue-green. 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