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Table of Contents
Year : 2009  |  Volume : 11  |  Issue : 1  |  Page : 1-11

Hemangiomas and Vascular Malformations of the Head and Neck

1 Department of Otolaryngology, Head & Neck Surgery, Philipps University of Marburg, Germany
2 Department of Otolaryngology, Faculty of Medicine, Cairo University, Cairo, Egypt
3 Department of Otolaryngology, Head & Neck Surgery, Karl-Hansen-Klinik, 33175 Bad Lippspringe, Germany

Date of Web Publication7-Jan-2020

Correspondence Address:
MD Jochen A Werner
Dept. of Otolaryngology, Head and Neck Surgery, Philipps University of Marburg, Deutschhausstrasse 3 35037 Marburg
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/1319-8491.275318

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The successful treatment of vascular anomalies is closely related to a sound knowledge of the biologic behaviour of vascular lesions and their correct classification. Based on the clinical progress, Mullicken and Glowacki developed a biological classification that was accepted as the official classification system of the International Society for the Study of Vascular Anomalies. (ISSVA) As a result of an extended literature research an overview will be given on the different internationally accepted treatment concepts. Even if a wait-and-see strategy can often be recommended in cases of uncomplicated hemangiomas of infants the rapid proliferative growth of such lesions requires an adequate therapy. In comparison to that vascular malformations that generally persist for lifetime require treatment, in particular when clinical symptoms occur. Based on individual parameters such as diameter, location, or growth behaviour different therapeutic measures might be successfully performed such as for example cryotherapy, corticosteroid application, laser therapy, sclerotherapy, operative interventions, and/or embolisation. However, none of the mentioned strategies represents the only treatment of choice.

Keywords: Hemangiomas, vascular malformations, classification, diagnosis, therapy

How to cite this article:
Werner JA, Eivazi B, Wiegand S, Negm H, Murthum T, Mandic R, Folz BJ. Hemangiomas and Vascular Malformations of the Head and Neck. Saudi J Otorhinolaryngol Head Neck Surg 2009;11:1-11

How to cite this URL:
Werner JA, Eivazi B, Wiegand S, Negm H, Murthum T, Mandic R, Folz BJ. Hemangiomas and Vascular Malformations of the Head and Neck. Saudi J Otorhinolaryngol Head Neck Surg [serial online] 2009 [cited 2022 Dec 4];11:1-11. Available from: https://www.sjohns.org/text.asp?2009/11/1/1/275318

  Introduction Top

Vascular anomalies belong to the most frequently occurring congenital and neonatal soft tissue malformations [1],[2],[3]. Many different classification systems have been elaborated using descriptive terms or anatomic labels [Table 1]. Mullicken and Glowacki [4] developed a biologic classification framework of vascular anomalies encompassing physical findings, clinical behaviour and cellular kinetics. Their classification stands out from numerous other classification systems not only because of its simple clinical practicality, but also because of its accuracy for the forecast of the clinical course of a lesion. So it has become widely accepted as an official classification by the International Society for the Study of Vascular Anomalies (IS SVA) [5]. However, the limitations of the mentioned system must also be noted. In certain cases the criteria may fail to correctly classify particular vascular lesions. Generally, the ISSVA differentiates between hemangiomas in infancy and vascular malformations, according to the classification system of Mulliken and Glowacki [4].
Table 1

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  1. Hemangiomas Top

Generally, hemangiomas appear only few days or weeks after birth. Typically they grow more rapidly than the body volume of the infant [6]. This proliferation is then followed by a spontaneous slow involution [7],[8]. As a rule of the thumb for the clinical practice it was observed that the involution phase is finished at the age of 5 years in 50% of such lesions [9]. By the age of 7 , the percentage increases to about 70% and at the age of 9 to nearly 90%. Waner and Suen [7] observed that 40-50% of the children affected show residues of their hemangiomas, like fibrous-fatty tissue degeneration, telangiectatic cutaneous vessels, or scar formations. Furthermore, the residual skin may appear pale and minor atrophy or wrinkling can be observed [10]. Hemangiomas occur more frequently in females than in males at a ratio of 3:1. They manifest mainly (60%) in the head and neck region [Figure 1]. Twenty-five percent are located on the trunk and 15% affect the extremities [11]. The preferred location of hemangiomas in the face of the patients is of highest relevance for the parents who often fear social stigmatisation because of lacking knowledge, uncertainty, and worry. They fear that their child may not be socially accepted because of cosmetic reasons. With this background it becomes even more important to discuss intensively the clinical appearance, the possible treatment options and especially the indication for therapy as it is well known that the first medical consultation often decides about the further process. With consideration of their singular clinical features and the application of non-invasive imaging procedures, hemangiomas can be reliably diagnosed without radiological examination in most of the cases [12]. In infants with multiple (>3) cutaneous hemangiomas, Waner and Suen [7] recommend ultrasound and/or magnetic resonance imaging (MRI) scans to exclude visceral and cerebral angiomas that can be found frequently in those cases. It is also recommended that such infants are seen at frequent intervals and examined thoroughly, especially during the first three months of life. The child’s growth and development should be monitored since failure to thrive is sometimes associated with large hemangiomas or multiple small lesions, in particular in cases of visceral and/or cerebral involvement. One special form that should be mentioned here is the benign neonatal hemangiomatosis. However, the term “benign” must not belie the fact that those children have a very poor prognosis resulting from the recurrent bleedings of involved visceral hemangiomas.
Figure 1: Hemangioma of the left cheek in an infant

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During the last years cellular markers have been detected that possibly contribute to differentiate prolif-erative from involuting hemangiomas. Proliferative hemangiomas show a high level of bFGF, (basic fibroblast growth factor), VEGF, urokinase, and type IV collagenase have been identified as markers. In comparison to that, involuting hemangiomas exhibit endostatin, glucocorticoids, platelet factor IV, interferon, interleukin-12, increased mast cells, angiostatin, tissue inhibitor of metalloproteinases (TIMP), and throbospondin and metalloproteinase inhibitors [6],[13],[14],[15]. With regard to distinguishing between hemangiomas and vascular malformations, the urinary bFGF level appears to be a focus of increased interest [14]. While only low or even zero urinary bFGF levels have been found in healthy children with vascular malformations children suffering from proliferative hemangiomas revealed elevated bFGF levels [16].

  2. Vascular malformations Top

In comparison to hemangiomas, vascular malformations appear already at birth with a male to female ratio of 1:1 . Clinically they are sometimes not initially evident. In these cases they may appear at different stages of life which seems to depend on the circulatory type of the different vascular malformations. With this background, the different types of malformations are classified according to the involved vessels. Vascular malformations may present as low-flow lesions; those are capillary, lymphatic, and venous malformations. The other types are arterial and arterio-venous malforma-tions as well as arterio-venous fistulas that are classified as high-flow lesions [17]. They may occur uniquely or as part of a syndrome. Typically vascular malformations grow proportionately to the child growth. In contrast to hemangiomas, vascular malformations never involute spontaneously [1].

Arterio-venous malformations occur less frequently in the head and neck compared to low-flow lesions. The clinical presentation, however, is mostly more dramatic as such malformations cause significant bleedings. The risk of hemorrhage is life-long and increses with age. Non-hemorrhagic presentations often include functional disorders, facial deformity, or pains. Generally, soft-tissue fullness underlies an area of increased temperature and discoloured skin. Further, dilated veins may be visible. Superficial lesions and arterio-venous fistulas may reveal palpable thrill and audible sounds [7],[18],[19]. Port-wine stains (nevus flammeus) must not be confounded up with the term of nevus simplex. Nevus simplex is extremely frequent in newborns. It appears on the nape of the neck, eyelids or glabella. Such lesions often fade or even disappear with time. Probably, the nevus simplex does not belong to the family of vascular malformations but represents focal areas of physiologic vascular dysfunction [7]. This hypothesis is supported by histological examinations showing dilated capillary vessels with otherwise normal morphology [20]. In contrast to that, nevus flammeus occur in about 0.3% of all newborns [21]. Generally, it occurs on the skin of the head and neck, often involving the branching of the trigeminal nerve. Typically, nevus flammeus becomes more prominent with age and shows darker discoloration [Figure 2]. Sometimes nevi flammei is located directly above deeper malformations or it is associated with syndromes such as the Klippel-Trénaunay syndrome, Parkes- Weber syndrome More Details, or the more common  Sturge-Weber syndrome More Details [22].
Figure 2: High flow vascular malformation of the upper lip

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The most common location of lymphatic malformations is the head and neck region. About 40% of the lymphangiomas can be identified at the time of birth; about 50% of the lesions appear by the end of the first year of life, 75% by the end of the second year. Occuring in the neck area, they frequently involve the posterior triangle [Figure 3] although an extension into or the primary involvement of the parotid or submandibular region or the floor of the mouth, and the supraclavicular region can be observed [23]. Generally lymphatic malformations are painless, non-pulsatile soft-tissue masses; the overlying skin is normal [24]. Typically a sudden enlargement can be observed in cases of infections of the upper aerodigestive tract or less frequently in cases of internal bleedings. While lymphangiomas commonly displace adjacent organs, the infiltration of the parenchyma of structures like the parotid gland, the tongue muscle, or the larynx may be observed. Ultrasound is appropriate for superficial examination. In order to completely assess the lesion, MRI or CT scans are required [7].
Figure 3: Lymphangioma of the neck

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The mainly historic classification of lymphangiomas into capillary, cavernous, and cystic lesions is meanwhile out of use. Marler and Mulliken limited their description of the morphology to “microcystic”, “macrocystic”, and “combined” lesions [25]. De Serres and co-workers [26] introduced a staging that classifies lymphangiomas of the head and neck according to anatomic aspects into five stages [Table 2]. In this context the authors were able to show that the complication rate after surgical therapy and the number of the necessary surgical interventions correlate and increase with the stage number. Hamoir and co-workers confirmed this observation [27]. The clinical relevance, however, should be verified in the context of prospective, multicenter studies. In this context, the team of Wittekindt [28] developed a morbidity correlated score system (Cologne Disease Score) with regard to the respiratory, swallowing, and speaking functions as well as the cosmetic aspects. They pursued the objective to quickly and completely assess lymphatic malformations as well as to compare therapeutic studies.
Table 2: Staging system of cervico-facial lymphangiomas according to de Serres and co-workers [26]

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  3. Treatment concepts Top

3.1 Hemangiomas

Despite multiple criticisms, the wait-and-see strategy is justified in small, superficial and non-proliferative hemangiomas, as published by Batta et al. [29] in 2002. The prospective evaluation of 121 children assessed the effectiveness and complication rate of a controlled wait- and-see policy and yellow light laser treatment with a 12 month follow-up. The results of this evaluation confirm the necessity to find individual therapy indications for every case. The number of children showing a lesion that completely disappeared or who had only minimal residues was not significantly different in both groups. Children who had undergone laser therapy showed more frequently atrophic skin and hypopigmentation. Those observations confirm that uncomplicated hemangiomas may justify clinical observation as sole treatment. Further the findings justify the question of general therapeutic intervention in all infantile hemangiomas in order to avoid or at least minimize residues.

The above-mentioned critical explanations are confirmed by the current publication of Witman and co-workers [30]. The authors report on an increased rate of hypopigmentation and atrophic scars after yellow light laser therapy for treatment of superficial hemangiomas in infants while their results correspond to the results of Batta and co-workers.

The decisive factor for indication of each therapy is the growth behaviour of the lesions. Wait-and-see strategy is nearly always justified in hemangiomas without proliferation and without cosmetic or functional problems. Sudden proliferation, however, should always be reacted on with a targeted therapy by applying all available treatment options. This is especially true for hemangiomas located in the face of the infant that may cause severe complication (disfiguring of the cartilaginous skeleton of the nose, blindness) which may indicate early treatment. Regarding the psychosocial stigmatisation, early treatment of obvious lesions offers the possibility of complete removal or size reduction of the hemangioma so that the result is considerably smaller and less disfiguring. This is especially true for the problem of residual disease in the sense of fibrous fatty tissue or atrophic skin and telangiectases as well as scars.

Possible treatment concepts may be the cryotherapy, the application of corticosteroids, laser therapy, and/or conventional surgery [13],[31],[32],[33],[34]. The choice of the most appropriate strategy is made according to the size and location of the lesion as well as its growth behaviour.

3.1.1 Cryotherapy

The main application of cryotherapy is the treatment of very early, superficial and limited hemangiomas in newborns [35],[36]. Early cryotherapy may transfer hemangiomas from proliferative to regressive phases, thus inducing cessation of growth and involution. Good to excellent results have been described for contact cryotherapy at a constant application temperature of - 32°C without adverse effects such as scarring or hypopigmentation [37].

3.1.2 Corticosteroids

Oral corticosteroids (methylprednisolone or prednisone) are sucessfully applied in the conservative treatment of proliferative hemangiomas in early childhood [31],[38]. The recommended dosage is 2-5 mg/kg body weight per day of methylprednisolone or prednisone for a period of 3-4 weeks. This dosage should then be reduced gradually over the following 8 weeks. Higher dosage does not lead to a better overall result, however, an increased initial response rate could be observed. Sometimes such a procedure is required in cases of much extended lesions. Therefore such regimens may be useful in the treatment of hemangiomas that do not respond to low dosage, especially in centers where other treatment options are not available [39].

Most reports describe only few possible adverse effects [39],[40],[41]. Changes in behaviour including irritability, frequent crying, and increased appetite disappear after treatment cessation [39],[41]. Despite the rapid success that may be achieved under certain conditions, corticosteroids cannot be seen as the treatment of choice for all proliferative hemangiomas. If progressive growth of a lesion is observed within the first 8-10 days of systemic corticosteroid therapy, continuation of the application should be abandoned and surgery should be considered. Further, corticosteroids are no longer effective when the proliferative phase of the hemangioma has passed [7].

3.1.3 Interferon-a-2a

In recent years, interferon-a-2a has gained some importance for the treatment of life-threatening hemangiomas in infants [42],[43],[44]. The attending physician, however, must carefully consider the rate of sometimes severe complications against the response rate of about 50% reported in literature [43]. Interferon-a-2a should be reserved to selected cases of extremely proliferative hemangiomas that are life-threatening or cosmetically deforming the head and neck region.

3.1.4 Laser therapy

Despite the critical annotations with regard to general application of laser therapy, it must be mentioned at this point that laser therapy plays also an important role in the treatment of proliferative hemangiomas in infancy [34],[45],[46],[47],[48],[49]. Successful treatments are described for different laser systems such as yellow light laser, KTP laser, argon laser, and Nd:YAG laser. Each laser system is characterised by a specific laser light spectrum with unique penetration depth of the laser radiation.

Superficial hemangiomas in infancy can be treated successfully with excellent result by yellow light laser systems that have a limited penetration depth of 0.5 mm [50]. Photocoagulation must be performed very carefully in order to avoid postoperative hypopigmentation or even scarring. The treatment may be repeated at intervals of 4-6 weeks. It is the attending physician’s responsibility to inform the parents about the gradual approach to treatment of such lesions because otherwise a cosmetically unfavourable result may be achieved. Based on this background, it has proved beneficial to treat such lesions in up to 6 sessions to achieve the complete removal of the lesion without scarring [33].

Poetke and co-workers [51] evaluated the effectiveness of flashlamp pumped dye laser therapy in the treatment of superficial and deep hemangiomas in 165 children aged between two and seven years. The authors could show that the flashlamp pumped dye laser is only effective in superficial cutaneous hemangiomas at sites of possible functional impairment or on the face. Hemangiomas with deep infiltration cannot be treated with flashlamp pumped dye laser because the effectiveness of the laser beam is limited due to the low penetration depth. The authors recommend applying flashlamp pumped dye laser therapy only in the first days or weeks of life when the hemangioma is still flat in order to avoid proliferation, promote involution, or even eliminate the vascular lesion completely. The indication to wait-and-see strategy versus immediate laser therapy must be thoroughly considered with regard to the publications of Batta et al. [29] and Witman et al. [30]. Without any doubt, the experience of the treating surgeon plays a major role in this context.

The physical properties of Nd:YAG laser make this laser type particularly suitable for the deeper parts of hemangiomas [52]. A selective destruction of larger vessels that may vary from 400μm to several millimetres in diameter, with little or no damage of the overlying skin can be achieved when laser energy is kept at a low level. Raulin and Greve [53] reported about a retrospective clinical evaluation of hemangioma therapy in infancy by flashlamp pumped dye laser (585 nm) compared to Nd:YAG laser (532 nm). The authors revealed that the flashlamp pumped dye laser was more effective than Nd:YAG laser and should be the initial therapy of choice for superficial hemangiomas in infants. Nd:YAG laser, however, seems to be more appropriate in the continued treatment of hemangiomas in the regressive phase in order to optimize the cosmetic result.

Interstitial Nd:YAG laser therapy can be applied successfully to treat voluminous subcutaneous hemangiomas in infancy [46]. Such a therapy is especially appropriate for the treatment of hemangiomas with a minimal diameter of 1-1.5 cm [54]. In interstitial Nd:YAG laser therapy, a bare fiber is coupled to an Nd:YAG laser and inserted into the hemangioma through an intravenous cannula [34]. Ultrasound imaging reveals changes in tissue texture at the tip of the fiber. Using a low laser light intensity of 1-2W over an application time of 500-1000 s, the laser light of the bare fiber is absorbed by tissue pigments and converted to heat, resulting in controlled thermal coagulation and tissue necrosis. The application of 5-8 W over 20-60 s also proved to be suitable. The extent of necrosis may be influenced by the use of different fiber tips. So the use of a Nd:YAG laser with a 600 μm blunt fiber tip induced necrotic tissue of 16 mm width [55]. The laser fiber should be passed through the bulk of the hemangioma several times to coagulate as much tissue as possible. Hemangiomas with superficial and deep parts should receive combined treatment [34].

3.1.5 Conventional surgery

Special anatomic locations or hemangiomas with rapid proliferative growth require solitary surgical treatment, surgery combined with embolisation, and/or laser therapy in order to avoid skin defects that may otherwise result from extensive skin necrosis [32].

Conventional surgery is also indicated in cases where interstitial laser therapy may lead to severe edema with obstruction of functionally relevant structures. One example for such a case is a hemangioma of the eye-lid where obstruction of the visual axis may cause amblyopia. Especially in early childhood this risk is very high which may even lead to blindness [56]. Further, timely surgical intervention may be required in the management of proliferative hemangiomas of the lip because such lesions are prone to bleeding and cause difficulties in nutrition.

Surgical resection is performed to improve the function and/or outer appearance of a child. The result of surgery must not be worse than residues after natural involution of a hemangioma.

Finally the cosmetically impairing fibrous fatty tissue should be removed surgically with possibly minimal scarring. In such cases the surgeon must be aware that, although most vascular channels diminish during involution, residual ecstatic vessels may persist. Thus, vascularization, even of degenerated hemangiomas, may be stronger than expected [54].

3.2 Vascular malformations

Since vascular malformations do not involute, the indication of therapy is not controversially discussed. Generally, sclerotherapy, laser therapy, and/or embolisa- tion followed by surgical removal have proved to be suitable. The choice of the therapeutic concept depends on the anatomic location, the depth, and the extent of the lesion.

3.2.1 Sclerotherapy

Sclerotherapy causes destruction and scarring of the normal adjacent tissue and shows an inconsistent effect on low-flow vascular malformations [8]. However, despite such reports there are indications of sclerotherapy in low-flow vascular lesions. Jain and co-workers [57] emphasize that the ultrasound-guided percutaneous injection of polidocanol 1% for sclerosis of peripheral vascular lesions is a simple and effective procedure with a good safety profile for the treatment of soft-tissue venous malformations and lymphangiomas. Puig and co-workers [58] developed a classification system for venous malformations including four types (Table III). The authors showed that the decision for sclerotherapy should be made only in exceptional cases in types III and IV while patients with type I or II malformations show excellent results after sclerotherapy. Usually 1-3 injections are sufficient to achieve sclerosis with satisfactory cosmetic results [59],[60].

The sometimes euphoric reports on the effectiveness of sclerotherapy in low-flow malformations must not belie the fact that sclerotherapy is still an unsafe procedure for treating lesions located in the middle and upper third of the face because the facial veins have no valves and thus the venous drainage might pass through the cavernous sinus. Because of inflammations and scarring, previous sclerotherapy in unresponsive cases may impede the use of other surgical measures.

The above-mentioned aspects apply first to most angiomas located in the area of the parotid gland. Inflammatory processes and extensive scarring may impede safe and thorough dissection of the facial nerves over a longer distance which leads to an increased risk of accidental damage to the facial nerve.

An intensively discussed substance for the treatment of lymphangiomas is picipanil (OK 432) [61]. Picipanil is a lyophilized incubation mixture of group A  Streptococcus pyogenes Scientific Name Search an origin. The induced leukocytosis increases the endothelial permeability of the lymphatic vessels which leads to an increased lymphatic drainage and thus an involution of the lymphangioma [62]. Luzzatto and co-workers [63] reported about the treatment with picibanil that they achieved successful treatment with 50% volume reduction in 8 children and a persisting volume in 7 of a total of 15 treated children. A review published in 2004 [64] by the authors evaluated the treatment of OK-432 as alternative to surgical therapy in cases of lymphan-giomas with large cystic parts [Figure 4]. Lymphangiomas, however, consisting of small cystic parts, show no or only very low response to this therapy.
Figure 4: Cervical lymphangioma with large cystic parts, suitable for OK-432 therapy

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3.2.2 Laser therapy

The yellow light laser is successfully applied in the treatment of superficial capillary vascular malformations. The selective destruction with little or no damage to the overlying skin can be achieved by keeping the laser energy on a low level [54]. The treatment of very small superficial ecstatic vessels (400μm) should be performed with a pulse rate of 1ms with 1W laser intensity [65]. The treatment of so-called spider veins and superficial ecstatic vascular malformations proved to be successful with a pulse rate of 40-60 ms and an intensity depending on the vascular diameter (6mm/80- 200W; 3mm 200-300W; 1.5mm/280-580W) [66].

Results of other publications [52] show that wave lengths longer than 585 nm may increase the efficacy of the treatment of some kinds of port-wine stains. Due to the reduced light absorption by haemoglobin at longer wave lengths, and thus increased depth of vascular injury, larger vessels can be treated more adequately. The authors concluded that lasers (1.5 ms) at 585 nm seem to be slightly superior to other laser treatments in the therapy of port-wine stains. The availability of yellow light laser and interstitial Nd:YAG laser may increase the therapeutic options in the treatment of port-wine stains.

Applying laser therapy requires generally more than one session in order to achieve complete involution of the vascular lesion. The attending physicians should be aware of the postoperative risk of local swellings. However, this may be reduced by administering corticosteroids peri-operatively which is essential in anatomic locations such as the orbit, the oral cavity, and the upper airways. In cutaneous vascular malformations, our postoperative management includes the application of a thin layer of antibacterial corticosteroid powder for the first two weeks. This application should be performed as often as necessary in order to dry up any wound secretion.

In order to reach deeper layers of vascular malformations, interstitial Nd:YAG laser therapy may be applied, in particular when vessels with a diameter of 400 μm to several millimetres must be treated. Using a low intensi- ty up to 4 W, the laser light of the fiber is absorbed and converted into heat so that a thermal coagulation and tissue necrosis is produced.

Even CO2 lasers can be employed successfully for the treatment of vascular malformations. This is especially true for lymphangiomas of the tongue [Figure 5] that may then be reduced in the sense of ablation.
Figure 5: Lymphangioma of the tongue, recurrent bleedings as a sign for involving also blood vessels

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3.2.3 Conventional surgery

In contrast to involuting hemangiomas, the complete resection of vascular malformations is recommended to avoid recurrences. Although the complete resection should be tried it may not be possible to achieve such an optimal result (for example in cases of diffuse lymphatic or extended venous malformations) without compromising functional impairment and/or significant cosmetic features. Under those circumstances, partial resection should be considered. Sometimes it is possible to perform surgery of the same location or to treat by an interventional radiologic intervention if this is required at a later stage [7]. Considering the enormous difficulties that lymphangiomas present to children, their parents, and the physicians, the ultimate results of all types of conventional surgery may be appropriate. Excessive or radical surgery will not necessarily guarantee complete elimination of the disease and may even worsen the case [23].

Beside the preferred complete elimination of the lesion, establishing normal facial appearance, preserving functionally important structures, and thorough hemostasis are important challenges that the surgeon have to face when removing vascular and lymphatic malformations. The buccal fat compartment is a common location for such lesions. In those cases, special attention must be paid to preserving the facial nerve.

Operative resection must be performed with nerve monitoring and careful hemostasis [55].

3.2.4 Embolisation and optional surgery

In general the treatment of most of the hemangiomas requires neither preoperative angiography nor embolisation. Even low-flow malformations may be treated success fully without preoperative embolisation, while for many arterio-venous and arterial vascular malformations embolisation with or without surgery is recommended. This statement applies in particular for arterio-venous fistulas. In this context the therapeutic aim is the occlusion of the nidus that may be achieved most reliably if embolisation is performed in a distal to proximal direction.

Catheter embolisation can be considered as the most appropriate method to treat extended arterio-venous fistulas if total or subtotal occlusion of the fistula can be achieved. However, if partial or complete collateralisation can be expected in persistent arterio-venous fistulas, combined therapy should probably be applied. In addition to occlusion of hemodynamically effective arterio-venous fistulas, conventionally surgical resection of the angioma and persisting smaller arterio-venous fistulas as well as arterialized dysplastic veins and secondary varicose veins should be performed as soon as the post-embolisation edema has resolved [67]. Establishing a collateral circulation depends on different previous manipulations and on hemodynamic requirements of the vascular lesion. An assessment of the exact blood supply of a lesion is essential [7]. Therefore, in many cases at least once an initial angiography is required for exact identification of the vascular malformation.

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  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5]

  [Table 1], [Table 2], [Table 3]


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