British Journal of Oral and Maxillofacial Surgery 44 (2006) 263272

Haemangiomas and vascular malformations of the maxillofacial regionA review

M. Ethunandan, Timothy K. Mellor
Maxillofacial Unit, Queen Alexandra Hospital, Portsmouth PO6 3LY, UK Received 19 January 2005; accepted 29 June 2005 Available online 16 August 2005

Abstract Congenital vascular anomalies have been and remain poorly understood. Since 1982 haemangiomas and vascular malformations have been recognised as distinct entities that exhibit unique characteristics and demand appropriately tailored treatment plans. However, haemangioma still continues to be used as a clinical and pathological description of many different types of vascular anomalies, which complicates both the care of patients and the interpretation of reports in journals. In this review we summarise the current classication, terminology, and the pathological basis of these lesions and discuss their management. The various therapeutic options available and their outcome will be discussed in addition to the recent advances in the psychosocial aspects of care, interventional radiology, laser and pharmacological therapy. 2005 The British Association of Oral and Maxillofacial Surgeons. Published by Elsevier Ltd. All rights reserved.
Keywords: Haemangioma; Vascular malformation; Capillary malformation; Venous malformation; Arteriovenous malformation; Lymphatic malformation; Lymphangioma

Introduction Congenital vascular anomalies are poorly understood. This is in part the result of a lack of a uniformly accepted classication and unclear understanding of the natural history of these lesions.1,2 In addition, clinicians from many specialties are involved in the management of these patients and have looked for solutions within their specialty and applied them to all lesions. It is therefore not surprising that the lack of consensus on the terminology of vascular anomalies has resulted in a wealth of publications that are often imprecise and commonly perpetuate misconceptions and inaccuracies about the diagnosis and management of these lesions. In this review we seek to draw attention to the current classication, terminology, clinical features, natural history, diagnosis, and treatment of congenital vascular anomalies affecting the maxillofacial region.

Classication Before the 1980s, the terminology that was used to describe vascular anomalies was confusing and ambiguous. The descriptive terminology used in the past (port wine stain, strawberry haemangioma, salmon patch) conjure up visual approximation to the lesions but have no correlation with the biological behaviour or natural history of these lesions.1,2 Mulliken and Glowacki introduced a simple classication in 1982 that was based on the clinical, histochemical, and cellular criteria to distinguish between the various vascular anomalies. They described two distinct entitieshaemangiomas and vascular malformations.3 Subsequent modication of the original classication to include the depth of the lesion and characteristics of ow has resulted in a classication that is clinically useful and correlates well with pathological and radiological data (Table 1). Recently, haemangiomas have been categorised into congenital (which is present at birth) and the more common infantile (which appears soon after birth). Congenital haemangiomas are further subdivided into rapidly involuting

Corresponding author. Tel.: +44 2392 286631; fax: +44 2392 286089. E-mail address: tim.mellor@porthosp.nhs.uk (T.K. Mellor).

0266-4356/$ see front matter 2005 The British Association of Oral and Maxillofacial Surgeons. Published by Elsevier Ltd. All rights reserved. doi:10.1016/j.bjoms.2005.06.032

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Table 1 Current classication of haemangioma and vascular malformations A. Haemangiomas Supercial (capillary haemangiomaa ) Deep (cavernous haemangiomaa ) Compound (capillary cavernous haemangiomaa ) B. Vascular malformations Simple lesions Low-ow lesions Capillary malformation (capillary haemangiomaa , port-wine staina ) Venous malformation (cavernous haemangiomaa ) Lymphatic malformation (lymphangiomaa , cystic hygromaa ) High-ow lesions Arterial malformation Combined lesions Arteriovenous malformations Lymphovenous malformations Other combinations
a

Old terminology within brackets, the source of much confusion.

congenital haemangioma (RICH)4 , non-involuting congenital haemangioma (NICH)5 and congenital non-progressive haemangioma.6 Vascular malformations are grouped according to the predominant vessel type and characteristics of ow (Table 1). Diagnosis History and clinical examination Accurate terminology leads to precise identication of the vascular entity. In most cases, an accurate history and physical examination will help establish the diagnosis (Table 2). In cases in which the diagnosis is equivocal a repeat assessment in 34 months will greatly improve the chance of arriving at a diagnosis and special investigations are only occasionally necessary. Investigations Further investigations are considered only if the diagnosis is in doubt or the investigation will inuence the decision and method of treatment. Unfortunately no single investigation is appropriate for all lesions. Imaging is used to conrm the suspected diagnosis, establish the extent of the lesion, and document any associated abnormalities.7 Magnetic resonance imaging is the investigation of choice as it provides accurate information about the extent of the lesion, better contrast between the lesion and surrounding tissues, and has multiplanar capabilities (Fig. 1). It can also help distinguish between the different types of vascular anomalies.810 Contrast-enhanced computed tomography has a role in evaluating intraosseous lesions and the bony margins of extensive lesions that are under consideration for resection.8 Grey-scale ultrasound and Doppler analysis are useful in dening whether the lesion is solid or cystic and to establish the presence or absence of high ow vessels.11 Angiography, particularly digital subtraction angiography (DSA), has a specic but limited role in the diagnosis of vascular lesions, but should not be used as a rst line investigation. It is, however, useful for mapping out the blood supply of the lesion (Fig. 2) and in the assessment of the characteristics of ow of arteriovenous malformations.7,8,12 Angiography is usually reserved for therapeutic endovascular interventions. Direct intralesional injection of contrast medium may have a role in the analysis of venous malformations.7,8,10 Preoperative histological conrmation is seldom necessary, but features are characteristic and help differentiate between the various types of vascular lesion.13
Fig. 1. Magnetic resonance image of an arteriovenous malformation of the left tongue.

Haemangioma This is the most common tumour in white infants (1012%) and the head and neck region is the most commonly involved site (60%).13 Most lesions are solitary (80%) and girls are more affected than boys (3:1). Multiple cutaneous lesions (three or more) are often associated with visceral involvement.13 Facial haemangiomas have a predilection for segmental distribution and for regions of embryological fusion.14 A strong association has been reported between

M. Ethunandan, T.K. Mellor / British Journal of Oral and Maxillofacial Surgery 44 (2006) 263272 Table 2 Simplied diagnostic approach to a congenital vascular lesiona

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H: haemangioma; VM: vascular malformation. a Modied from Waner and Suen1

a beard distribution of facial haemangiomas and symptomatic laryngeal involvement.15 Haemangiomas usually appear soon after birth (though up to 30% may be present at birth), typically proliferate during the rst year of life and then involute during the childhood years (up to 12 years).13 The terms capillary and cavernous haemangioma are out of date and the lesions are more appropriately described according to the depth of the lesion as supercial, deep, and compound haemangioma.1 Supercial haemangiomas originate from the papillary dermis and present as bright red macular or papular masses (previously called capillary or strawberry haemangioma). Deep

Fig. 3. Compound haemangioma of the nasal tip in an infant.

haemangiomas originate from the reticular dermis or subcutaneous tissues and appear as bluish or relatively colourless masses (previously called cavernous haemangioma). Compound haemangiomas have supercial and deep components (Fig. 3) and were previously called capillary cavernous haemangiomas.

Histology
Fig. 2. Digital subtraction carotid angiogram showing an arteriovenous malformation of the tongue involving the lingual artery.

The histological features are dependent on the stage of the lesion.13 In the proliferative phase, the lesion is highly

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cellular and contains plump proliferating endothelial cells and pericytes, with a high mitotic activity and numerous mast cells. Vascular channels are not prominent. In the involutive phase, the endothelial cells are attened, the cell turnover is normal and there are few mast cells. Vascular channels lled with blood cells predominate, and the lesion is eventually replaced by brofatty tissue. Immunohistochemistry can help in further delineating the various stages of a haemangioma.16

Treatment The natural history of haemangiomas should inuence the timing and type of intervention. Benign neglect is often advocated, but can be unacceptable given the natural history of these lesions.17 The widespread belief that haemangiomas will completely disappear in the rst few years of life is misleading, as shown by Finn et al.18 who reported that 80% of lesions that had not involuted by 6 years (and 38% of lesions that had involuted at 6 years) left behind a residual cosmetic deformity. The psychosocial trauma of a visible lesion in a growing child,19,20 and the poor outcome of benign neglect,18,21 taken with the current availability of drugs, lasers, and other techniques to treat these lesions safely, should warrant a change in the philosophy of benign neglect.1 A useful approach to the management of haemangiomas can be based on the stage of the lesion (proliferative or involutive phase), type of lesion (supercial, deep, compound) and the management of residual deformity.1,22 In general, life-threatening and sight-threatening haemangiomas should be dealt with, regardless of the stage of the lesion. Active intervention should be considered in all disguring haemangiomas, but each case should be managed on its merits after careful discussion and counselling, to prevent potential psychosocial trauma and cosmetic deformity.1,23,24 Prednisolone is the rst line drug of choice for the treatment of life- or sight-threatening haemangiomas. The response rate varies from 30 to 90% and depends on the dose, duration, and age at start of treatment.25,26 Steroids are useful only in the proliferative phase. Interferon 2a and have been used, but because of their potentially serious side effects, it is suggested that they are used only in steroid-resistant lifeand sight-threatening lesions.27,28 Proliferative phase Actively proliferating haemangiomas are seen only in infants, as the growth period is restricted to the rst years of life. In general, treatment of obvious haemangiomas should be aggressive in the rst year of life. The aim is to eradicate the lesion completely or at least to stunt its growth so that by the time the proliferation is complete, a smaller, less disguring lesion is left.1,22 Supercial haemangiomas can be treated with pulsed dye laser.2934 Treatment should be started at the earliest sign of

haemangioma and repeated at 46 weekly intervals until the lesion is completely eliminated and in some cases up to 6 treatments may be required.2934 During the later stages of proliferation, haemangiomas usually acquire a thickness that is beyond the depth of penetration of yellow light lasers. Interstitial potassium-titanylphosphate (KTP) and neodymium:yttrium-aluminum-garnet (Nd:YAG) lasers have been used for these lesions with some success.3537 Oral steroids should be considered, depending on the size and location of the lesion, as it will shrink the lesion provided it is given in an appropriate dose for an adequate length of time.25,38 In uncomplicated lesions, which are not cosmetically unacceptable, benign neglect may be chosen, together with parenteral reassurance and careful follow-up up to 34 years of age. By this stage it becomes apparent whether or not involution will be complete before 6 years of age. Involutive phase In the involutive phase, the aim is to improve appearance and function and the treatment should never give a worse result than after natural involution. A wait and watch policy may be pursued until 34 years of age. In lesions that involute completely by 6 years (rapid involuters) no further treatment will be required in 60% of cases whereas in cases of slow involution further treatment will be required in 80% of cases.18 Treatment options are dependent on the depth and location of the lesions. Steroids are not effective against involuting haemangiomas. Supercial lesions with small and intermediate size vessels can be treated successfully with pulsed dye laser.2934 Larger vessels can be treated with a copper bromide and Nd-YAG laser.1,31 Resection remains the mainstay of treatment for deep haemangiomas. In the case of compound lesions, the supercial component should rst be dealt with by laser photocoagulation and the deep component can subsequently be excised.1,31 Intralesional Nd:YAG and argon lasers have been used to treat deep haemangiomas, but can cause substantial pigmentation and scars.3537 Residual deformity This is most likely to be encountered in adolescent and adult patients. The common manifestations are telangiectasia, brofatty masses of tissue, and epidermal atrophy. Telangiectasia These are arborising networks of small, medium, and larger vessels. The small vessels often present as a red blush and are best treated with pulsed dye laser.39 The medium and large vessels can be treated with copper bromide, pulsed KTP laser or Nd:YAG laser.1,39,40 The procedure may have to be repeated a few times before the lesion resolves completely.

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Fibrofatty tissue This is best treated by excision, but can be more vascular than expected. The use of a thermoscalpel and contact laser (Nd:YAG) should be considered.1 The natural history of the lesion and the slow process of involution should always be taken into account particularly when treating children less than 12 years of age. It is preferable to err on the conservative side when excising residual bro-fatty tissue, as overzealous contouring and continued natural involution can result in a considerable defect. Haemangiomas of the lip and nose almost always leave residual tissue and patients benet from its careful removal.1,2,41 Epidermal atrophy Thin atrophic skin often coexists with telangiectasia and residual bro-fatty tissue and is difcult to rectify. Skin resurfacing with carbon dioxide laser and Erbium:YAG (Er:YAG) laser seems to be promising and a recent report on the treatment of atrophic scarring in partially or completely involuted haemangiomas in children, there was considerable improvement in almost all patients.42 Micro pigmentation (tattoo pigmentation) may be useful for areas of hypopigmentation to restore colour to the scarred skin or vermilion.43

Vascular malformations These are errors of morphogenesis that are populated by stable mature vascular endothelium.13 Both sexes are equally affected. They are always present at birth (though some may not be apparent until a later stage) and in contrast to haemangiomas they never proliferate or involute. Instead, they expand slowly and relentlessly throughout life, in pace with the growth of the patient.13 Trauma, puberty, and pregnancy can cause accelerated growth.1,2 These lesions are subclassied according to the predominant type of vessel and characteristics of ow (Table 1). Unlike haemangiomas, vascular malformations are associated with skeletal abnormalities in up to 35% of cases.44 Capillary (venular) malformations (previously called port wine stain, capillary haemangioma) are made up of postcapillary venules within the papillary and supercial reticular dermis.1,2 They present initially as at pink macules but darken and thicken with age, resulting in a cobblestone appearance (Fig. 4). They are thought to result from altered neural modulation of the papillary plexus.45,46 They are graded according to the degree of ectasia of the vessels into grades IIV which correlate well with the clinical features and outcome of treatment.47 Capillary malformations may be associated with SturgeWeber and KlippelTrenaunay syndromes.1,2 Venous malformations are characterised by an abnormal collection of veins, which do not have any demonstrable mitotic activity in endothelial or pericyte cells and often

Fig. 4. Capillary malformation with cobblestone appearance in an adult.

lack a uniform smooth muscle layer.1,2 The degree of ectasia increases with age and the clinical features depend on the depth and size of the lesion. The lesions are usually soft, compressible, and enlarge in size when venous pressure is increased (Fig. 5). Phleboliths may be present in the lesion. Changes in the adjacent skeleton are not uncommon and often take the form of bony hypertrophy or distortion or both.44 Venous malformations can occasionally be completely intraosseous and the mandible is the most common bone involved, although maxillary, nasal, and frontal lesions have also been reported.48,49 It is thought that most lesions that are described as intraosseous haemangiomas are in fact venous malformations.48 Arteriovenous malformations are the least common of the vascular malformations.1,2,12,50 The mid-face, particularly the cheek and ear, are the most common sites.50 These lesions can be associated with a considerable extent of arteriovenous shunting. The shunts are often tiny and numerous and grow with the child by progressive enlargement of the feeding and draining vessels and recruitment of collateral vessels.1,2 In contrast, arteriovenous stulae are generally single and may be congenital or acquired.1,2 The lesions are often deep, but those close to the surface may produce a palpable thrill or pulsation. They are rmer than venous malformations and do not empty readily when they are compressed (Fig. 6). Although arteriovenous malformations are present at birth, clinical

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Fig. 5. Venous malformation of the upper lip in an adult.

presentation is usually delayed and lesions may present in the second, third, or even fth decade.1,2,12,50 The pathogenesis remains speculative, and it is thought that the primary abnormality or nidus is a bed of dilated capillaries and that the hypertrophied arteries and dilated veins are secondary phenomena resulting from increased ow across the nidus.1,2 It is postulated that as the precapillary sphincter regulates the ow of blood through the capillary bed,

abnormalities in control of the sphincter are responsible for arteriovenous malformations and the age of presentation and speed of progression are dependent on the absolute or relative absence of control of the precapillary sphincter.1 Lymphatic malformations constitute a spectrum of disorders that present in various ways, often in childhood and adolescence.1,2,5153 Over 75% of lymphatic malformations are found in the neck and the clinical manifestation varies

Fig. 6. Arteriovenous malformation of the tongue (same patient as in Figs. 1 and 2).

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Treatment Clinicians in many specialties treat vascular lesions in the maxillofacial region in many different ways. The result, combined with the lack of a universally accepted classication, is that there are no properly conducted randomised controlled trials leading to an evidence-based approach to the treatment of these lesions. Therefore, one has to rely on poor evidence when deciding on particular treatment options. The choice of treatment depends on the type of malformation (vascular content), location, depth, and characteristics of ow of the lesion. Options include laser therapy, sclerotherapy, embolisation, resection, and combinations of these. Capillary malformation Capillary malformations (like other vascular malformations) never regress spontaneously. They often darken and thicken with age and the rate of progression is probably related to the degree of absence of the autonomic nerves in addition to other factors such as hormonal inuences and trauma.45,46 Recent studies have emphasised the psychological aspects of the birthmark and have suggested earlier intervention to counteract the undesirable psychosocial effects.23,24 The choice of treatment depends on the degree of ectasia (diameter) of the vessels.1 Pulsed dye laser is now considered the treatment of choice for capillary malformations, using wavelengths of 577 nm and more recently 585 nm.5862 Selective thermolysis is the ability to coagulate a target chromophore specically without damaging the surrounding tissues and is dependent on the use of a laser with an appropriate wavelength, energy uence, and pulse duration. For vascular lesions the target chromophore is oxyhaemoglobin and selection of a laser wavelength that is preferentially absorbed by oxyhaemoglobin is crucial.63 Lesions resistant to pulsed dye laser may respond to KTP, copper vapour, argon, and other new lasers.64,65 However, complications of scarring and hypopigmentation and/or hyperpigmentation are more common with copper and KTP lasers.64 An average of two to six treatments may be necessary and the response rate varies from 4 to 100%.5862,64,65 Factors implicated in the varying response to treatment include depth, colour, and location of the lesion, age at treatment, and number of treatments.1 Orten et al. 62 reported that 40% of lesions recurred within 23 years and the gure was 50% at 45 years. They suggested that the aetiology of the malformation could explain the high recurrence rates, as any abnormally innervated vessels in the supercial papillary plexus that were left behind would lead to recurrence. They suggested that a touching up treatment may be required at intervals, depending on the rate of recurrence. Venous malformations The choice of treatment depends on the depth, extent, and anatomical location of the lesion and the experience and

Fig. 7. Lymphatic malformation of the oor of mouth and submental region with mandibular hypertrophy.

according to the extent and depth of the lesions as well as the extent of brous reaction around them. Mucosal and cutaneous involvement results in the formation of multiple uid-lled vesicles, which may communicate with larger and deeper lymphatic cisterns.54 They are subdivided into microcystic, macrocystic, and combined lesions depending on the size of the vesicles.1,2 Cervical lesions are usually of the macrocystic variety (cystic hygroma) and those involving the oor of the mouth (Fig. 7), cheek, and tongue are more likely to be of the diffuse microcystic variety (lymphangioma). The earlier in life the lesion presents, the more aggressive is it likely to be (high grade) and the more prone to complications. Low grade lesions present later and are less liable to complications. The complications include infection, bleeding, obstruction of the airway, disturbances of speech, and abnormal facial growth. Hypertrophy of both soft tissue and skeleton are common and occur in up to 83% of cases.44,52 The skeletal changes are thought to result from distortion of the function matrix55 or direct involvement of the bone by abnormal lymphatic channels.52,56 The primary defect is believed to be at the level of the efferent channels and obstruction at this level (relative or absolute), results in dilatation of the proximal channels that form the mass.57

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preference of the clinician. The methods available are lasers, excision, sclerotherapy, and combinations. Smaller lesions can be treated successfully by excision or sclerotherapy.1,2,66 Resection must be complete to prevent recurrence and appropriate imaging is necessary to show the full extent of the lesion, particularly if the lesion is large.1,2,7,12,66 Acquired adult venous malformations are commonly seen as small venous lakes in the lower lip and face and can be treated with laser photocoagulation or excision.12,66,67 Cryotherapy can be sometimes benecial in the treatment of small intraoral lesions,68 but its use in cutaneous lesions can result in hypopigmentation and scarring.69 Nd:YAG lasers form the mainstay of treatment for a supercial lesion or the supercial component of a compound lesion, as the vessels are often large.1,70,71 Initial laser treatment will destroy the supercial component of the lesion and provide a relatively avascular plane to allow a ap to be raised to gain access to the deeper lesion and also spares the removal of the cutaneous and mucosal surfaces.1 Derby and Low72 reported that laser treatment is usually palliative and recurrences that need repeated treatments are common. Larger venous malformations are often managed by a combination of sclerotherapy and excision, or sclerotherapy alone. Various sclerosing agents have been used and there are no controlled trials that compare the different agents. The commonly used agents are sodium tetradecyl sulphate, 100% ethanol, and Ethibloc66,7375 and are usually injected by direct puncture under radiographic control. Recurrence after sclerotherapy is not uncommon and is thought to be due to recannalisation.74 Care should be taken when injecting sclerosants, particularly in the upper and mid-face, because of the potential risk of intracranial communication. Resection can be difcult, particularly with more extensive lesions.1,2,12,66 Complete excision of the lesion is necessary to prevent recurrence, but this has to be weighed against the possible complications of a radical operation. Contact lasers and thermoscalpels are useful in minimising blood loss and it is sometimes necessary to stage the resection particularly in more extensive lesions.1 Residual or recurrent lesions can be treated by excision or sclerotherapy. Arteriovenous malformations As with other vascular malformations, slow but relentless growth is the norm and high grade lesions present earlier in life and expand more rapidly, whereas low grade lesions present later and are more likely to expand slowly.1,2 In general, intervention should be planned as soon as the diagnosis is made and timely intervention will not only prevent complications, but also allow the extent of resection to be considerably reduced. The decision to intervene should be based on the age, location, ow characteristics, morbidity of the treatment, and the risks of an untreated lesion. Treatment should aim to eradicate the nidus completely, which is the fundamental abnormality as even the smallest residual nidus

will expand to form a recurrence.1,2,12,50 The options include embolisation, resection, and combinations. Embolisation is useful for larger arteriovenous malformations and can be used as the sole treatment or as an adjunct. As a adjuvant, it is most commonly used before excision.12,50,7678 Once the DSA has mapped out the extent of the nidus, its principal blood supply, and collaterals, embolisation should proceed from distal to proximal. In this way both the nidus and its blood supply will be ablated.1 Because collateral blood supply becomes established quickly, operation should be within 2448 h after embolisation.12,50,7678 Again various agents have been used for embolisation, and there are no published clinical trials comparing the efcacy and side effects of these agents, which include Gelfoam, polyvinyl alcohol, silicone uid, and isobutyl-2 cyanoacrylate (IBCA). Embolisation is used alone as a palliative procedure for unresectable lesions and those that have bled and are likely to do so again.77,78 Recently Persky et al. showed that endovascular techniques used alone were effective in treating mandibular and maxillary arterial malformations with limited soft tissue involvement.49 The aim of surgery is complete removal of the nidus and it may be necessary to sacrice any structure that forms part of the nidus, the most common being muscle. Occasionally, skin, mucosa, and rarely cartilage and bone will have to be sacriced. Early access to the feeding vessels is important for haemostasis and the use of a contact laser and thermoscalpel will help avoid loss of blood.1 If possible proximal ligation of these vessels should be avoided if they do not enter the nidus, as that would preclude further embolisation and encourage development of collaterals from the internal carotid system. Intraoperative doppler examination and the pattern of bleeding of tissues at the margins of resection are useful guides to the completeness of resection.50 Lymphatic malformations These are the most difcult to eradicate. Microcystic lesions in particular are diffuse, do not respect tissues planes, and it is difcult to distinguish involved tissue from normal tissue. Macrocystic lesions on the other hand are more localised and respect tissue planes and are more easily excised. The options for treatment include laser ablation, excision, and sclerotherapy. Carbon dioxide laser should be reserved for supercial mucosal lesions. Because uid-lled vesicles are almost always connected to deeper cisterns, ablation should be continued to a suitable depth to ensure destruction of most or all of these cisterns. Recurrences are not uncommon and can be treated in a similar manner.1 Excision is the preferred method of treatment and localised macrocystic lesions are most easily excised.1,2,12,52,53,79 Diffuse microcystic lesions are more difcult and may require multiple operations.1,52,53,79 Care should be taken to identify and preserve important structures, because tissue planes are often distorted. Postoperative lymphoedema can be a

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considerable problem, particularly after extirpation of lesions in the oor of the mouth, the resultant oedema and protrusion of the tongue taking up to a year to subside.1,79 Skeletal hypertrophy and malocclusion require the use of orthognathic techniques for correction.12,52 Sclerotherapy is a promising, but as yet not fully evaluated option. It can be used either as denitive treatment, or for palliation when the morbidity of operation outweighs the benets. Various agents have been used and recently OK 432 (lyophilised Streptococcus pyogenes treated with benzyl penicillin) has been found to be successful in treating macrocystic lesions, with the advantage of avoiding a scar.8082 Unfortunately multiple treatments are necessary and the results are less impressive with microcystic lesions.8082 In summary, haemangioma continues to be used as a clinical and pathological description for many different types of vascular anomalies. We have summarised the important clinical differences between haemangiomas and vascular malformations and the management of these disguring congenital anomalies. The use of common terminology and better understanding of the natural history will lead to better outcomes for our patients. This depends eventually on the development of multidisciplinary teams to manage these complex problems, similar to the developments in the care of patients with head and neck cancer.

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