You are in: eMedicine Specialties > Otolaryngology and Facial Plastic Surgery > HEAD AND NECK ONCOLOGY Skin Tumors, Vascular Lesions, Face and NeckArticle Last Updated: Mar 30, 2006AUTHOR AND EDITOR INFORMATIONSection 1 of 10
  Author: John E McClay, MD, Assistant Professor, Department of Otolaryngology, Division of Pediatric Otolaryngology, Children's Medical Center, University of Texas Southwestern Medical School John E McClay is a member of the following medical societies: American Academy of Otolaryngic Allergy, American Academy of Otolaryngology-Head and Neck Surgery, American College of Surgeons, and American Medical Association Coauthor(s): Jeffrey S Zapalac, MD, Staff Physician, Department of Otolaryngology, University of Texas Southwestern Medical Center Editors: Jaime R Garza, MD, DDS, FACS, Consulting Staff, Private Practice; Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine; Nader Sadeghi, MD, FRCS(C), Associate Professor of Surgery, Director of Head and Neck Surgery, Department of Surgery, Division of Otolaryngology, George Washington University; Christopher L Slack, MD, Otolaryngology-Facial Plastic Surgery, Private Practice, Associated Coastal ENT; Medical Director, Treasure Coast Sleep Disorders; Arlen D Meyers, MD, MBA, Professor, Department of Otolaryngology-Head and Neck Surgery, University of Colorado School of Medicine Author and Editor Disclosure Synonyms and related keywords: vascular anomalies of the head and neck, vascular anomaly, head and neck vascular tumor, cervicofacial vascular anomalies, cervicofacial vascular anomaly, hemangiomas, vascular malformation, vascular tumor, arteriovenous malformation, AVM, arteriovascular malformation, cervicofacial hemangioma, kaposiform hemangioendothelioma, KHE, tufted angioma, TA, capillary malformation, venous malformation, lymphatic malformation, cystic hygroma, lymphangioma, cutaneous vascular lesion, capillary-cavernous hemangioma, Kasabach-Merritt syndrome, port-wine stain, port wine stain, birth mark, birthmark, Sturge-Weber syndrome, cavernous hemangioma, lymphangiohemangioma, macrocystic lymphatic malformation, microcystic lymphatic malformation INTRODUCTIONSection 2 of 10
  Vascular anomalies of the head and neck historically have confused clinicians secondary to inconsistent nomenclature. For many years, this has resulted in difficult study, improper diagnosis, and inappropriate treatment. In 1982, Mulliken and Glowacki introduced a biologic classification of cervicofacial vascular anomalies that correlated clinical presentation, behavior, and histology. As a result, vascular anomalies were divided into 2 main categories: hemangiomas and vascular malformations. Subsequent revision of this classification has divided these lesions into vascular tumors and vascular malformations. Vascular tumors include hemangioma, kaposiform hemangioendothelioma, and tufted angioma. Vascular malformations include capillary, venous, lymphatic, and arteriovenous malformations (AVM). Hemangiomas are the most common tumors of infancy and are characterized by a proliferating and involuting phase. Growth in early infancy, during the proliferative phase, is embodied by rapidly dividing endothelial cells forming syncytial masses; thickened, multilaminated basement membranes; and elevated mast cell concentrations. Proliferating-phase hemangiomas display a 10-fold increase in mast cell concentration over involuting lesions and normal tissue. These mast cells are thought to mediate cell-to-cell communication and angiogenesis. During the involuting phase, endothelial cell activity decreases, and cellular parenchyma is replaced by fibrofatty tissue. Vascular malformations display no proliferative cellular activity. They exhibit flat, quiescent endothelium; normal, single-layer basement membranes; and normal mast cell concentrations. Vascular malformations are considered errors of vascular morphogenesis, displaying abnormal dilatations and channels but no increased cell turnover. Based on the characteristics of the predominant channel type, vascular malformations are subdivided into capillary, venous, arteriovenous, and lymphatic malformations. Despite stability from a cellular standpoint, vascular malformations can be clinically devastating because they grow in proportion to total body growth and never involute. These structural abnormalities often display interval expansion with changes in blood flow, collateral formation, or hormonal modulation. Capillary, venous, and lymphatic malformations are considered low-flow lesions based on their respective pathophysiology, whereas AVMs are considered high-flow lesions. In light of these fundamental distinctions, hemangiomas are best evaluated from a perspective of endothelial proliferation followed inevitably by involution, whereas vascular malformations are best approached by considering rheologic properties in the face of growth over the life of the individual. The vast majority of childhood cutaneous vascular lesions can be accurately classified into the appropriate category of hemangioma versus vascular malformation based on a sound history and physical examination. Angiography and biopsy are not generally necessary to make the diagnosis. DIFFERENTIATION OF HEMANGIOMAS AND VASCULAR MALFORMATIONSSection 3 of 10
Presentation The appearance of hemangiomas is often subtle at birth. Many do not become apparent for several days to months. Hemangiomas are encountered more frequently in females than in males by a ratio of 3:1. Their hallmark is rapid postnatal growth for 8-12 months, followed by slow spontaneous involution over the ensuing 5-8 years. The timing, rate, and full extent of involution are unpredictable, which often confounds issues of therapeutic intervention. Vascular malformations are present at birth. Most become apparent during infancy, except for AVMs, which commonly manifest later in childhood or adolescence. They occur in equal incidence among females and males. The hallmark of vascular malformations is proportionate growth throughout the life of the individual. Expansion may be seen with hormonal changes (eg, puberty, pregnancy), infection, trauma, or surgical intervention. Physicians must distinguish these nonproliferative enlargements from the proliferating phase of hemangiomas. Physical examination Hemangiomas tend to be rubbery, firm, well-circumscribed lesions. When they develop in the superficial dermis, the proliferation of cells causes the skin to become raised and bright red. In contrast, lesions in the deeper dermis or subcutaneous tissues may be less demarcated, and they may have a bluish hue. Historically, these were thought to be different lesions and thus were termed capillary or capillary-cavernous hemangiomas, reflecting supposedly different histology. Histologic evaluation has revealed considerable consistency in endothelial cell proliferation, regardless of tumor depth. Therefore, hue is more indicative of lesion depth than histology. Capillary malformations are well-circumscribed macular lesions that commonly occur on the face in the trigeminal distribution. Venous and lymphatic malformations are not as well circumscribed. They tend to be soft, readily compressible, and nonpulsatile. Expansion occurs in venous malformations with compression of the jugular vein on the affected side, performance of the Valsalva maneuver, or dependent positioning. AVMs manifest as warm, firm masses with thrills, bruits, and pulsatility. Radiographic evaluation Despite the merits of this clinical classification system, radiographic imaging is a useful and often necessary adjunct. Whether differentiating deep hemangiomas from venous or lymphatic malformations or assessing the true extent of soft tissue involvement, imaging modalities often highlight distinguishing characteristics. In capable and experienced hands, ultrasound can help accurately determine the flow characteristics of a vascular lesion. CT scan may be helpful in assessing the extent of soft tissue involvement and is preferable in the setting of bony erosion or remodeling. MRI provides the most information to evaluate cervicofacial vascular anomalies because of its superiority in soft tissue assessment and flow-pattern determination. AVMs and proliferating hemangiomas both show high-flow characteristics on MRI but can be distinguished by the decreased relative parenchyma of AVMs. Venous and lymphatic malformations are low-flow lesions by MRI criteria, consisting predominantly of different-caliber vessels without intervening parenchyma. HEMANGIOMASSection 4 of 10
Presentation Hemangiomas are the most common tumors of infancy. They occur in 2-3% of all neonates, 10-12% of white children, and 22% of preterm babies weighing less than 1000 grams. The most common site of appearance is the skin, usually as a single lesion. However, multiple cutaneous lesions occur 20% of the time. More than 60% of all hemangiomas occur in the head and neck. The natural history is one of proliferation followed by spontaneous involution. Signs of involution include shrinkage, central fading, and softening. Complete resolution occurs in 50% of cases by 5 years. Complete resolution occurs in 70% by 7 years; the remaining 30% resolve to differing degrees over late childhood and early adolescence. The rate and extent of regression is not predictable. Sex, age, site, size, appearance, and extent of proliferative phase do not correlate with extent of regression. However, those children whose lesions begin regression earlier tend to have better results. Complications occur in 10-20% of hemangiomas. Intervention is necessary once complications arise. Local complications Local complications, which tend to occur in the proliferative phase, include bleeding and ulceration. Bleeding occurs when the epithelial basement membrane has been penetrated by the hemangioma. This condition usually responds to local pressure or simple mattress sutures. Ulceration can lead to pain, infection, or recurrent bleeding. Sores can occur anywhere but have a predilection for the lips and perioral region. Ulceration is always an indication for therapy. Therapy for ulceration and superficial infection initially includes improved hygiene and topical antibiotic ointment. Reserve dressings and systemic antibiotics for deeper ulceration and more involved infection. Refractory cases may require local or systemic corticosteroids or surgery. Recently, pulsed dye lasers have been effective for decreasing pain and accelerating healing in an otherwise refractory ulceration. Major complications Major complications include cosmetic disfigurement, functional compromise, Kasabach-Merritt syndrome, and congestive heart failure. Cosmetic disfigurement may result from scarring of large untreated hemangiomas of the lips, nose, and ears. Functional compromise may result from obstruction of the airway, external auditory canal, or visual axis. Airway obstruction may result from hemangiomas occurring at any level from the nose to the trachea. The most common site of airway involvement is the subglottic region. Hemangiomas involving the ear or parotid may enlarge to obstruct the external auditory canal, resulting in refractory external otitis or conductive hearing loss. Periorbital hemangiomas often obstruct the visual axis. Asymmetric refractive errors occur in 60-80% of patients with periorbital lesions. Refractive problems result from astigmatism or myopia and are more common in the setting of upper eyelid lesions. Without intervention, astigmatism may persist, even after the hemangioma resolves. As a result, all patients with periorbital hemangiomas should be evaluated expediently by an ophthalmologist, who can document visual changes and initiate amblyopia therapy. Large visceral or extensive cutaneous hemangiomas may be associated with Kasabach-Merritt syndrome or congestive heart failure. Kasabach-Merritt syndrome refers to consumptive coagulopathy and thrombocytopenia in the setting of a large hemangioma. The coagulopathy is marked by thrombocytopenia, prolonged prothrombin time/activated partial thromboplastin time, and falling fibrinogen levels. Recent research has revealed an association with kaposiform hemangioendotheliomas and tufted angiomas, as opposed to banal hemangiomas. Increased capillary endothelial surface area is thought to play a role in trapping platelets, but the exact mechanism is unknown. The mortality rate in the setting of the Kasabach-Merritt syndrome historically has been 30-40%, despite aggressive therapy. Congestive heart failure is another potentially life-threatening manifestation of hemangiomas, occurring in association with large visceral or cervicofacial lesions. Similar to the subset affected by Kasabach-Merritt syndrome, this complication occurs more commonly in association with kaposiform hemangioendotheliomas and tufted angiomas. The liver is a common site of involvement. Unlike large cutaneous lesions, visceral hemangiomas may go unnoticed. Reliable predictive factors do not exist, aside from physical examination findings, which include abdominal mass, bruit over the liver, or overt signs of heart failure. Once cardiac failure manifests, the mortality rate rises to 50-55%. As a result, evaluate infants presenting with multiple cutaneous hemangiomas using ultrasound to assess for potential internal sites of involvement. Treatment Options for definitive management of hemangiomas include observation, corticosteroid therapy, surgery, and interferon (INF) therapy. Because the vast majority of hemangiomas are small, well circumscribed, and involute with no sequelae and acceptable cosmetic results, observation remains the preferred management option. For hemangiomas that involute but leave excessive skin laxity or fibrofatty residuum in conspicuous locations, surgical correction is best addressed in the postadolescent period, when the patient can take an active role in making decisions. This restraint on the part of the parents and surgeon may be difficult because proliferating hemangiomas may be cosmetically displeasing. Systemic corticosteroids have become the mainstay in treating complicated hemangiomas. They are believed to inhibit angiogenesis and to increase sensitivity to circulating vasoconstrictors. Initial therapy consists of 2-3 mg/kg/d of prednisone, which can be tapered to effect over the ensuing 3-4 months. In responders, steroids tend to decrease hemangioma size and to hasten the rate of involution. Significant shrinkage occurs in a third of patients, stabilization of growth without shrinkage in a third, and no significant response in a third. Concern for permanent growth retardation and infection with long-term steroid usage is legitimate, although the most common reported adverse effects are skin atrophy, behavioral changes, moon facies, and transient growth delay, all of which appear to resolve 3-4 months after cessation of therapy. In the setting of cosmetic disfigurement, functional compromise, or threat to life, initially pursue systemic steroid therapy. In focal lesions, intralesional steroid injection has also been used with moderate success. However, avoid this in persons with periorbital hemangiomas secondary to the risk of blindness from central retinal artery occlusion. Generally, reserve surgical excision for steroid failures. For focal sites, such as the ears, lips, nose, or periorbital region, excision with reconstruction can prevent undue long-term morbidity. Surgery is not usually an option in the setting of life-threatening hemangiomas because of their extensive nature and because of the critically ill nature of the child. INF has been used successfully in the treatment of life-threatening hemangiomas that fail to respond to steroids. INF inhibits angiogenesis by decreasing endothelial cell proliferation and migration. In patients with Kasabach-Merritt syndrome, INF appears to have additional beneficial effects of decreasing platelet adherence, improving flow, and facilitating prostacyclin-mediated protection of the endothelium. The hematologic response seems to be distinct from the antiproliferative response because hematologic profiles (eg, platelets, prothrombin time/activated partial thromboplastin time, fibrinogen levels) often normalize prior to regression of the lesion. Both INF alfa-2a and INF alfa-2b have been used with success, often dosed as 3 million U/m2/d. The duration of therapy usually ranges from weeks to months. Adverse effects include fever, neutropenia, and occasional skin sloughing. Recently, spastic diplegia has been reported in as many as 10% of infants treated with INF. Reserve INF alfa for life-threatening hemangiomas in which corticosteroid therapy has failed. When INF is used, monitoring of neurologic status is mandatory. Supportive therapy is necessary, in addition to steroids (and possibly INF), in the setting of life-threatening hemangiomas. For patients exhibiting the Kasabach-Merritt syndrome, blood products (eg, platelets, cryoprecipitate, fresh frozen plasma, packed red blood cells) must be used. Low-dose heparin has also been used. For patients with congestive heart failure, digitalis and diuretics are often necessary. CAPILLARY MALFORMATIONSSection 5 of 10
Presentation Capillary malformations have been known historically as port-wine stains. Histologically, they consist of abnormally ectatic capillaries and venules in the superficial dermis. They are present at birth as pink or purple macular lesions, often in a unilateral trigeminal distribution. Capillary malformations change in color and texture throughout the life of an individual, from pale pink and flat in childhood to purple-red, raised, and nodular in adulthood. The prevalence rate is 0.5-1% of all newborns. These malformations tend to have more discreet irregular margins than macular hemangiomas and do not involute or lighten with time. Most capillary malformations display soft tissue hypertrophy and underlying skeletal overgrowth, usually involving the alveolar region of the maxilla. Complications Glaucoma, retinal detachment, mental retardation, and seizures occur in 5% of patients, due to involvement of the vasculature of the eye and the CNS. This constellation of findings is known as Sturge-Weber syndrome. Cerebral angiography results from these patients usually reveal capillary malformations, venous malformations, and AVMs of the leptomeninges, which are responsible for the progressive degeneration and atrophy of the cerebral hemispheres. Cutaneous involvement in persons with Sturge-Weber syndrome almost always involves the V1 distribution; therefore, any patient with this presentation must undergo an early neurologic and ophthalmologic assessment. When V2/V3 involvement occurs alone, patients are generally not at risk for leptomeningeal involvement. Treatment Because capillary malformations do not involute, intervention is desirable. Lasers are the preferred intervention modality. The argon laser emits light at 488-514 nm. Selective absorption is facilitated because oxyhemoglobin has absorption maximums at 418, 542, and 577 nm. Several months after treatment with the argon laser, the dermis is replaced by connective tissue containing venules of normal diameter and the color fades to that of normal skin. Because these are low-power, continuous-wave lasers, treatment necessitates exposure times of several hundred milliseconds. These protracted exposure times result in significant nonvascular tissue damage by thermally induced coagulation necrosis. Resultant scarring varies with the population and is often worse in those with lighter lesions (eg, children). Good-to-excellent results are obtained in 70-80% of adult patients. Unacceptable results are observed in 30-40% of children; therefore, the argon laser is not recommended for patients younger than 18 years. Pulsed, tunable dye lasers can emit light at variable wavelengths. The wavelength of 577 nm is chosen to optimize selective absorption of hemoglobin and to minimize absorption by melanin, thereby decreasing epidermal scarring. The decreased pulse length compared to continuous-wave lasers minimizes thermal damage to surrounding tissues. Pulse widths of 300-450 microseconds are chosen to balance the risk of thermal damage to nonvascular structures with that of microvascular hemorrhage. This modality results in minimal scarring and possibly circumvents natural progression to the nodular texture of adulthood lesions. Pulsed dye lasers have become the treatment of choice for capillary malformations. VENOUS MALFORMATIONSSection 6 of 10
Presentation In the past, venous malformations have been erroneously termed cavernous hemangiomas or lymphangiohemangiomas. As errors in morphogenesis rather than proliferative cellular processes, these lesions should not be classified as hemangiomas. Histologically, they manifest ectatic venous channels with normal endothelium. Venous malformations may occur in pure venous form or as a combination of capillary, venous, or lymphatic malformations termed venous-lymphatic malformations. Venous malformations are present at birth but may not become clinically apparent for months. They can manifest as an isolated spongy mass or as a complex infiltrating lesion involving various soft tissue planes. Venous malformations have a predilection for the lips and cheeks and can occur in skeletal muscle, such as the masseter. Their hallmark is a soft, compressible, nonpulsatile mass with an overlying bluish hue that exhibits rapid refilling with release of manual compression. They do not regress spontaneously but tend to enlarge over the life of the individual, with darkening and superficial thickening over time. Complications Complications encountered with venous and venous-lymphatic malformations include thrombophlebitis and infection. Dilated channels with stasis of venous blood predispose patients to thrombus formation, which may manifest as calcified phleboliths radiographically or as recurrent painful nodules clinically. Infection may manifest as pain, swelling, and fever. Often, differentiating thrombophlebitis, cellulitis, or intralesional abscess is difficult. Initial management entails institution of antibiotic therapy, reserving drainage for mature abscesses documented by radiographic imaging. Treatment Many venous malformations do not require specific management apart from reassurance and an explanation of the natural history of the lesion. Intervention depends on the size of the lesion and its functional impact on the patient. Based on the anatomy and pathophysiology of venous malformations, most authorities advocate a trial of sclerotherapy, either alone or preceding attempted surgical therapy. The sclerosants administered are typically 95% ethanol or sodium tetradecyl sulfate (Sotradecol). The former agent yields a slightly decreased incidence of recurrence with a marginally increased risk of systemic toxicity. When a sclerosant is injected into a venous chamber, sludging of erythrocytes and rapid subsequent thrombosis occurs. This is followed by permanent obliteration of vessels by organized thrombi, intimal necrosis, and adventitial fibrosis. For small, well-circumscribed lesions, sclerotherapy alone is often sufficient treatment. For large, infiltrating malformations that are difficult to delineate, combined sclerotherapy with ablative and reconstructive surgery is indicated. In this subset of patients, total surgical extirpation is often difficult and subtotal resection is performed to relieve pain, reduce bulk, or improve function. LYMPHATIC MALFORMATIONSSection 7 of 10
Presentation Historically, lymphatic malformations have been termed either cystic hygromas or lymphangiomas, reflecting both ends of a spectrum of disease. They are thought to arise from sequestered portions of the embryonic lymphatic anlage. As such, they are abnormalities of lymphatic development. Histologically, these lesions consist of multiple dilated lymphatic channels lined by a single layer of flattened endothelium. They show no histologic evidence of endothelial proliferation or increased cell turnover. When combined with capillary or venous elements, they are termed venous-lymphatic malformations. Lymphatic malformations manifest as soft, colorless masses. Sixty percent are apparent at birth, while 80-90% manifest by age 2 years. Overall, they are uncommon congenital lesions, and the head and neck are the most common sites of distribution. Lesions in the neck exhibit few skin changes, while those in the oral cavity have a roughened irregular surface with hemorrhagic or clear bullae. The morphology of the lymphatic malformation relates to its anatomic location. Type I lymphatic malformations generally arise below the mylohyoid and occur in the anterior and posterior triangles of the neck. These lesions consist of large, thick-walled cysts with minimal infiltration of surrounding tissues. Historically, they have been termed cystic hygromas. Type II lymphatic malformations are generally located above the mylohyoid, commonly involving the tongue, floor of the mouth, cheeks, lips, and sometimes the parotid gland. These lesions are highly infiltrating and can produce large deformities. Historically, they have been termed lymphangiomas. Some authors refer to type I lesions as macrocystic lymphatic malformations and to type II lesions as microcystic lymphatic malformations. Complications Management of lymphatic malformations of the head and neck is often difficult secondary to the potential for recurrent infection, airway compromise, feeding difficulties, and concerns regarding cosmesis. Infection may manifest as a precipitous increase in the size of the lesion. Signs and symptoms may reflect localized cellulitis or possibly sepsis. Management consists of intravenous antibiotics. Always consider airway protection when addressing oral cavity and upper aerodigestive tract lesions. Intubation may suffice for temporary enlargements, whereas tracheotomy often becomes necessary in the setting of multilevel involvement. Treatment For definitive management, surgical excision with careful preservation of involved neurovascular structures has been considered the treatment of choice. Incision and drainage, aspiration, radiation therapy, and sclerotherapy have all been attempted in the past, with uniformly poor results. Avoid these interventions because they distort already poorly delineated tissues, thus making ultimate surgical resection exceedingly difficult. Base the surgical approach on the morphologic type of lymphatic malformation. Ideally, type I lymphatic malformations are resected in a single procedure. Repeat excision is complicated by fibrosis and distorted tissue planes. Optimal timing is age 1-2 years, if symptoms permit. Type II lesions present more difficult management issues secondary to their infiltrating nature. Because these lesions are histologically benign, complete excision is often unnecessary and may be harmful. Optimizing and preserving function are the primary objectives. As a result, surgical procedures often are limited in scope and frequently are staged. The carbon dioxide laser has been used with some success in the oral cavity for lesions on the tongue and floor of the mouth. Recent adjuncts to surgical therapy include sclerotherapy with bleomycin and OK-432, a Japanese-developed lyophilized incubation mixture of group A streptococci that induces sclerosis via host-mediated inflammation. Both work favorably in macrocystic lesions. Expense and possible adverse effects of pulmonary fibrosis make bleomycin less attractive. Clinical experience with OK-432 is limited, although early data have been encouraging. OK-432 does not appear to promote widespread cicatricial scarring, thereby rendering subsequent surgical intervention less onerous. ARTERIOVENOUS MALFORMATIONSSection 8 of 10
Presentation AVMs are thought to arise during fetal development when the arteriovenous channels in the primitive retiform plexus fail to regress. Communications persist, although some may not canalize for many years. AVMs are rare in comparison to hemangiomas and venous malformations. They are 20 times more common in the intracranial vasculature than in that served by the external carotid arteries. Cervicofacial involvement is most common in the cheeks, ears, nose, and forehead, in descending order of prevalence. Histologic study of these lesions demonstrates dysmorphic arteries that are thickly walled and irregular in caliber, with fragmented internal elastic lamina and highly disorganized smooth muscle in the media layers. Reactive venous hypertrophy occurs with intimal thickening and sclerosis secondary to increased blood flow. AVMs enlarge by hemodynamic mechanisms rather than cellular proliferation. Under these circumstances, collateral flow is promoted, thereby diverting regional blood from the periphery. The primary pathology occurs at the capillary level, where arteriovenous shunting occurs. Enlarged feeding arteries and veins result from uncontrolled shunting through these channels. As a result, isolated ligation of feeding vessels or embolization does not halt progression of the lesion. These measures ultimately facilitate progression of the AVM and are discouraged except in the context of palliation. Lesions are rarely symptomatic in the neonatal or infancy period. They are most commonly discovered in late childhood, adolescence, or early adulthood. Early lesions may manifest only cutaneous warmth or blush. With expansion, signs and symptoms include pain, ulceration, bleeding, audible bruits, palpable thrills, and pulsatility. During expansion, increased shunting of blood and decreased nutritive blood flow to the skin occurs, resulting in variable degrees of skin necrosis that manifest as ulceration and bleeding. A destructive downward cascade ensues. Staging Stage I lesions (quiescence) exhibit only cutaneous blush or warmth. Stage II lesions (expansion) display bruit, thrill, or other signs of expansion. Stage III lesions (destruction) elicit pain, bleeding, ulceration, or infection. Stage IV lesions (decompensation) manifest cardiac failure. Complications In general, once pain, bleeding, or ulceration has been established, progression of the AVM is inevitable unless the lesion can be resected. Factors associated with expansion include trauma, infection, incomplete resection, and hormonal changes occurring during pregnancy, puberty, or with the use of antiovulants. Treatment Complete surgical resection offers the only hope for cure. Given the highly vascular nature of AVMs, this frequently is difficult. For small lesions, excision alone may be possible. For more extensive lesions, the treatment of choice is combination therapy that consists of preoperative angiography with selective embolization followed by definitive resection within 24-48 hours. Embolization alone promotes neovascularization via collateral formation and recruitment. For unresectable lesions, embolization has been used for palliation, providing temporary relief from pain and bleeding. When used immediately prior to resection, it reduces intraoperative blood loss and facilitates extirpation of the mass. However, embolization does not reduce the extent of resection. The most common cause of treatment failure, even with combined embolization and surgery, is incomplete resection. Some argue that asymptomatic lesions require no treatment. This is controversial because predictive factors for progression of stage I lesions have not been identified. Ideally, limited stage I or II lesions are resected. Painful or rapidly enlarging stage II or III lesions warrant intervention secondary to the risk of progression and hemorrhage. Stage IV lesions are usually treated for palliation. Resection of extensive stage I lesions is controversial because progression is unpredictable. In these cases, resection may be incomplete or may result in prohibitive morbidity. FUTURE AND CONTROVERSIESSection 9 of 10
Progress in the treatment of head and neck vascular anomalies requires attention to the anatomy and pathophysiology of the specific lesion. Meaningful intervention for hemangiomas involves impacting the proliferative phase, which includes endothelial cell proliferation and angiogenesis. Steroids and INF affect this process nonspecifically and by different purported mechanisms. More specific pharmacologic inhibitors of angiogenesis with appropriate delivery systems are needed to curtail proliferation in hemangiomas, thereby preventing functional compromise. Recent research has targeted basic fibroblast growth factor, vascular endothelial growth factor, proliferating cell nuclear antigen, and type IV collagenase, all thought to play pivotal roles in angiogenesis. Interstitial laser therapy, or intralesional fiber photocoagulation, is a relatively new concept. Traditional lasers used in treating vascular anomalies (eg, argon, pulsed dye, carbon dioxide) have limited tissue penetration. The Nd:YAG laser has higher tissue penetration (£1 cm), but it often causes deleterious effects on the skin (eg, permanent scarring). Recently, the Nd:YAG and potassium-titanyl-phosphate lasers have been used, with and without ultrasound guidance, for voluminous hemangiomas and venous malformations, with encouraging results. This is an example of an older modality used with a new delivery system to optimize the therapeutic effect. In the case of hemangiomas, this modality may favorably alter the risk-to-benefit ratio of early intervention, thereby challenging the time-honored role of expectant management. For venous malformations, interstitial laser therapy would prove a welcome addition to the therapeutic armamentarium because pharmacologicintervention plays no rolein these nonproliferating lesions. REFERENCESSection 10 of 10
Skin Tumors, Vascular Lesions, Face and Neck excerpt Article Last Updated: Mar 30, 2006 |
