You are in: eMedicine Specialties > Pediatrics: Surgery > Vascular Surgery Arterial Vascular Malformations Including Hemangiomas and LymphangiomasArticle Last Updated: Aug 8, 2008AUTHOR AND EDITOR INFORMATIONSection 1 of 13
  Author: Robert A Schwartz, MD, MPH, Professor and Head of Dermatology, Professor of Medicine, Professor of Pediatrics, Professor of Pathology, Professor of Preventive Medicine and Community Health, UMDNJ-New Jersey Medical School Robert A Schwartz is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American College of Physicians, and Sigma Xi Coauthor(s): Richie L Lin, MD, Staff Physician, Department of Dermatology, University of Medicine and Dentistry of New Jersey-New Jersey Medical School; Tzongjer J Wei, MD, MPH, Assistant Professor of Pediatrics, UMDNJ-New Jersey Medical School; Chief and Director, Neonatal Intensive Care Unit, Department of Pediatrics, University Hospital; Herbert Dardik, MD, FACS, Clinical Professor of Surgery, Mount Sinai School of Medicine; Chief, Department of Surgery, Chief, Vascular Surgical Service, Englewood Hospital and Medical Center Editors: Jeff L Myers, MD, PhD Chief, Pediatric and Congenital Cardiac Surgery, Department of Surgery, Massachusetts General Hospital; Associate Professor of Surgery, Harvard Medical School; Mary L Windle, PharmD, Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy, Pharmacy Editor, eMedicine.com, Inc; Andre Hebra, MD, Chief, Division of Pediatric Surgery, Medical University of South Carolina; Professor of Surgery and Pediatrics, Medical University of South Carolina; Daniel Rauch, MD, FAAP, Director, Pediatric Hospitalist Program, Associate Professor, Department of Pediatrics, New York University School of Medicine; Mary C Mancini, MD, PhD, Professor, Department of Surgery, Louisiana State University Health Sciences Center Author and Editor Disclosure Synonyms and related keywords: vascular malformation, vascular tumor, lymphangioma, angioma lymphaticum, cystic hygroma, cystic hydroma, hemangioma, birthmark, birth mark, nevus, port-wine stain, port-wine mark, fading macular stain, salmon patch, nevus flammeus, flame nevus, stork bite, angel's kiss, Klippel-Trenaunay-Weber syndrome, Sturge-Weber syndrome, high-flow vascular malformation, arterial malformation, arteriovenous malformation, slow-flow vascular malformation, venous malformation, capillary malformation, lymphatic malformation, internal hemangioma, hemangiomatosis INTRODUCTIONSection 2 of 13
  Vascular malformations result from abnormal-sized vascular structures or an abnormal number of vascular structures. These malformations usually manifest as cutaneous birthmarks and have had a number of classifications, producing an array of confusing terms. The evaluation and treatment of common vascular malformations, particularly hemangiomas and lymphangiomas, are discussed in this article. The widely accepted Mulliken and Glowacki classification system is used, and attempts are made to sort out the nomenclature that has been used over the years.1 The indications and value of the various interventions are also discussed. When evaluating a patient with these types of malformations, one must also consider syndromes associated with vascular malformations, such as Klippel-Trenaunay-Weber syndrome and Sturge-Weber syndrome (see Klippel-Trenaunay-Weber Syndrome and Sturge-Weber Syndrome).2, 3Other uncommon forms of vascular malformations have been reported. For example, PHACE syndrome (OMIM 606519) is a neurocutaneous syndrome that associates large, plaquelike, segmental hemangiomas of the face, with one or more of the following: posterior fossa brain malformations, arterial cerebrovascular anomalies, cardiovascular anomalies, eye anomalies, and ventral developmental defects, specifically sternal defects, and/or supraumbilical raphe. In a prospective, cohort study of 1,096 children with hemangiomas, 25 children met the criteria for PHACE, representing 20% of infants with segmental facial hemangiomas.4 History of the ProcedureVascular tumors and malformations are rather unsightly lesions that often occur in children. Parents of these children often (understandably) pressure physicians to deliver definitive medical or surgical treatment. However, the acknowledgment of the natural involution of vascular tumors (hemangiomas) in the mid-twentieth century has changed the way these lesions are managed. In this article, the medical and surgical treatments of vascular tumors are discussed, and the way in which this involutional phase fits into the treatment plan is explained. However, less consensus surrounds the management of vascular malformations (lymphangiomas), especially in patients with diffuse disease. In patients with vascular tumors, watchful waiting is now the accepted first step in the management of nonproblematic hemangiomas because 75% of these lesions involute, leaving a minimal residual scar. Medical treatment is the first-line approach to treating potentially destructive lesions and is discussed in detail below (see Treatment). The technique and timing for surgical resection of hemangiomas has been a topic of contentious debate. Because, in most cases, these lesions involute without intervention, the surgeon must identify situations in which an excision procedure would produce a more cosmetically acceptable result than conservative treatment. In addition, the surgeon must choose a technique that leads to the best possible outcome. See Treatment for the recognized indications for surgery and a brief discussion on the various techniques. Note that, depending on the location of the lesion, surgical resection may be the initial treatment of choice. In the past, capillary malformations were managed using various treatment modalities, including electrocautery and cryosurgery. However, these methods have fallen out of favor because of their poor efficacy and the degree of scarring that they cause. Today, most of these lesions are treated with an argon laser or a flashlamp-pumped pulsed dye laser (PDL). Most surgeons agree that surgical excision of lymphangiomas is the treatment of choice in patients with localized disease. Studies have been performed to correlate the depth of the excision with the rate of recurrence (see Treatment). In addition, new medical treatments are being developed with encouraging results. Management of diffuse lymphatic disease has historically been surgical, and, although techniques have improved over the years, treatment still involves multiple procedures with substantial morbidity. ProblemVascular tumors and malformations have often been described using a classification system based on morphologic characteristics. This practice has given rise to a countless number of names that are interchangeably used, often befuddling medical professionals. In 1982, Mulliken and Glowacki proposed a biologic classification of vascular tumors and malformations that has since gained wide acceptance.1 This system correlates histologic features with historical and physical findings to provide a simplified classification of vascular lesions. The system has been modified slightly over the years to incorporate new information.
Capillary and lymphatic malformations are the focus of this article and are used as models for vascular malformations. The fading macular stain (ie, stork bite, salmon patch, nevus flammeus) is a common lesion that is often grouped with capillary malformations because of their similar appearance. The terms used to describe fading macular stains have often been used interchangeably with port-wine stain. This usage is incorrect because they are not permanent anomalies and, hence, not true vascular malformations. Fading macular stains are discussed only briefly because they should be included in the differential diagnosis of port-wine stains. FrequencyVascular tumors (ie, hemangiomas) are the most common tumors in infants. They are apparent in 1-2.6% of neonates at birth across all races, according to one series.5 Approximately 30% of hemangiomas are recognized in the newborn nursery. Prevalence is increased in preterm infants that weigh less than 1000 g and in white children younger than 1 year. The male-to-female ratio is 3:1.6 Vascular malformations include capillary and lymphatic malformations. Capillary malformations (ie, port-wine stains) occur in 3 per 1000 neonates.7 They are the most common type of vascular malformations. The lesions are present in neonates and darken during adolescence and middle age. The sex distribution is equal. The incidence rate of lymphatic malformations is 1.2-2.8 per 1000 live births.8 Approximately 50% of lymphatic malformations are apparent at birth; 90% appear before age 2 years. Most reports indicate an equal male-to-female distribution. EtiologyVascular tumors (hemangiomas) are believed to result from developmental errors that occur at 4-10 weeks’ gestation. Most cases are sporadic; however, they are occasionally inherited in an autosomal dominant fashion with moderate-to-high rates of penetrance.9 See Pathophysiology for more details on the nature of these lesions. Capillary malformations are generally considered to be sporadic lesions; however, pedigrees of autosomal dominant inheritance have been reported. Port-wine stains are also associated with Klippel-Trenaunay-Weber and Sturge-Weber syndromes. Mutations in the RASA1 gene may underlie the capillary malformation–arteriovenous malformation syndrome. A novel mutation in RASA1 has been reported to cause capillary malformation and limb enlargement.10 An extensive degree of phenotypic heterogeneity may be associated with deleterious mutations in RASA1. The exact cause of lymphangioma formation is unknown, but most cases are believed to be sporadic. The formation of lymphangiomas possibly reflects a failure of lymph ducts to connect with the venous system during embryogenesis, abnormal sequestration of lymphatic structures, or both. Ongoing research has elucidated some of the vascular growth factors that may be involved in formation of lymphatic malformations such as VEGF-C and flt-4. Cases secondary to trauma and infection have also been reported. PathophysiologyHemangiomas are the result of abnormal changes in angiogenesis that allow the overproliferation of vascular entities. Several authors have elucidated the complex interplay of angiogenic and angiostatic forces involved in normal and pathologic processes.11 However, fetal vascular development remains poorly understood. Many of the angiogenic markers (ie, FGF, VEGF, E-selectin, type IV collagenase) are increased during the proliferative phase.12 During the involutional phase of hemangiomas, a subsequent decrease in angiogenic factors occurs, with a 5-fold increase in endothelial cell apoptosis.13 These alterations in angiogenic factors may account for the increased vascular proliferation that occurs in hemangiomas. Microscopic hemangioma tissue reveals proliferating endothelial cells. During involution, endothelial cells flatten, the vessel lumens dilate, and fibrous tissue is deposited. Recent studies have also discovered hemangioma-specific antigens not found in normal skin. These include GLUT1, merosin, and Lewis Y antigen. Capillary malformations (port-wine stains) are groups of tortuous blood vessels located in the upper layers of the dermis. One study revealed that these lesions have decreased innervation in perivascular regions, which generates the hypothesis that the lesions are secondary to impaired vascular tone.14 Lymphangiomas are collections of lymph vessels filled with serous fluid. Their histology ranges from capillary-sized vessels to macroscopic fluid–filled vessels. Lymphatic malformations can be associated with gross anatomic deformities with severe involvement of the surrounding structures on the face or extremities. Research has begun to reveal some of the cell signals that may be involved in the formation of lymphangiomas. For example, VEGF-C has been found to be adequate in causing lymphatic hyperplasia. ClinicalHemangiomas are most commonly located on the head and neck (59%), followed by the trunk (24%), lower extremities (10%), and upper extremities (7%).15 Most are less than 2 cm in diameter but, in some instances, can cover large portions of the body. Typical presenting symptoms occur superficially, and the appearance can vary from a hypopigmented macule to a bruiselike macule.16 The course of these lesions includes a proliferative postnatal growth phase that lasts for 3-9 months, with a gradual involution that occurs over 2-6 years. Involution is usually complete by age 7-10 years. Only 50% of patients have completely normal-appearing skin at this time. Recognizing atypical presentations and subclassifications of hemangiomas is critical for prognostic and treatment purposes. A congenital hemangioma is a lesion that is fully developed at birth. Deep hemangiomas have a blue discoloration because of their proliferation in the dermis and subcutaneous tissues. Multiple hemangiomas, often referred to as hemangiomatosis, appear as multiple smaller lesions. Pseudoclubbing may be the first sign of an underlying subungual hemangioma.17 When evaluating hemangiomas, a family history should be elicited. Although most are sporadic, infantile hemangiomas may be the result of an autosomal dominant trait. One study suggests that the formation of hemangiomas may be associated with mutational events that result in a loss of heterozygosity at a specific locus on chromosome 5.18 Mutations in genes responsible for regulation of vascular development, such as FGF-4, PDGF-b, and a tyrosine-kinase gene, have been linked to familial forms of hemangioma.19 Most lymphangiomas are clinically apparent at birth, and almost all are apparent by age 2 years. Most appear as soft doughy masses that are located in the head and neck region, and most have no associated symptoms. Clinical manifestations are dependent on the flow of lymph within the channels of the lesion. Lymphangiomas may manifest as lymphedema, and larger lesions can involve the skeletal system and cause gross disfigurement. Large malformations in the neck or mediastinum can compromise the airway, leading to stridor, dysphonia, or dyspnea. Lymphangiomas have also been found in patients with Turner syndrome, Klinefelter syndrome, and Noonan syndrome. INDICATIONSSection 3 of 13
Vascular tumors Surgical excision of vascular tumors (hemangiomas) is controversial because most lesions involute with little intervention (75%). Surgery is indicated if the procedure is anticipated to leave a scar that is more cosmetically acceptable than a scar due to medical therapy or following involution. Furthermore, in the following few specific situations, surgical intervention is considered inevitable: (1) if an abnormal scar or excess tissue is present after natural involution; (2) if lesions are ulcerated and bleed excessively or are associated with pain; and (3) if lesions interfere with the development or activities necessary for life, such as lesions of the eye, ear, or larynx. Capillary malformations Surgical management of port-wine stains has largely fallen out of favor with the advent of the laser treatment. Surgical methods have included dermabrasion, electrocautery, and cryosurgery. Lymphatic malformations For the purposes of treatment, lymphangiomas are often divided into local and diffuse disease. In localized disease, a conservative period of watchful waiting is appropriate if the lesion causes no immediate compromise to life functions. However, fewer than 15% of lesions spontaneously regress. Some surgeons believe that if the lesion has not spontaneously regressed by age 5 years, surgical intervention is warranted. Other authors believe that excision should be performed sooner to avoid complications of lymphangioma, such as infection.20 Infection can be a severe sequela of lymphangiomas. Most surgeons currently agree that complete excision is the preferred treatment for localized lymphangiomas, depending on the accessibility of the location. However, the close quarters of the head and neck provide for a tedious dissection that often results in sacrificing vital structures and significant impairment following surgery. In these cases, surgery may have to be avoided, and an alternate therapy should be pursued. Partial excision is frequently possible and is an acceptable alternative to complete excision, particularly if surgical resection would require removal of vital structures. Management of diffuse lymphatic disease is a complex multistaged treatment associated with high complication rates. Patients and parents must be informed that management may be a lifelong endeavor and that significant morbidity may occur. Some studies report the use of nonsurgical therapy with special drugs that may promote regression of complex lymphangiomas. However, this treatment is not yet approved by the US Food and Drug Administration (FDA). RELEVANT ANATOMYSection 4 of 13
Vascular tumors Hemangiomas located on the trunk or face may be psychologically damaging when children approach school age. This must be considered when discussing early intervention in these lesions. Lesions of the eye, ear, or larynx warrant early intervention because they may affect proper development of these organs or compromise the airway. Lesions involving the orbit should be properly evaluated by an ophthalmologist. In cases involving the larynx, the first priority is ensuring a secure airway. Capillary malformations Capillary malformations are frequently located on the face, where they often present a psychological problem. Lymphatic malformations In most series, the head and neck are the most common sites for lymphatic malformations, followed by the trunk, axilla, and extremities. In the head, the oral cavity and face area are the most common sites. In the neck, the posterior triangle is reportedly the most common site. However, many series have reported no difference between the anterior and posterior triangles. Right-sided lesions seem to predominate. Laryngeal involvement is not uncommon. Cases of diffuse disease may occur, encompassing large portions of the body. CONTRAINDICATIONSSection 5 of 13
Vascular tumors No absolute contraindications to surgical resection of hemangiomas have been recognized. However, in cases of nontroubling vascular tumors, the surgeon should thoroughly discuss the alternatives before proceeding. Capillary malformations No absolute contraindications to laser surgery have been identified in patients with capillary malformations. The physician should assess the risk of complications. For example, the 585-nm PDL can damage choroidal and retinal vasculature if the patient is not wearing the appropriate protective eyewear. In addition, the possibility of damage to surrounding structures and tissues must be evaluated. Lymphatic malformations No absolute contraindications to surgery have been identified in the treatment of lymphangiomas. Surgical treatment of diffuse lymphatic disease is a complex multistaged treatment that has high complication rates. Patients and parents must be informed that management may be a lifelong endeavor and that significant morbidity may occur. WORKUPSection 6 of 13
Lab Studies
Imaging Studies
Diagnostic Procedures
TREATMENTSection 7 of 13
Medical therapyVascular tumors Approximately 75% of hemangiomas involute without intervention and are in anatomically benign locations, making them a cosmetic issue. Therefore, parents must be educated about their child's condition, and the typical course of these lesions must be explained to them. In this manner, the physician can avoid hastily instituting medical therapy that may lead to a less desirable outcome. Regular follow-up visits should be scheduled to monitor the course of the lesion and to provide continuous reassurance to the parents. Medical intervention is indicated if hemangiomas are located in areas that hinder normal development, interfere with important life processes, or cause severe pain. Such locations include the eye, nose, and airways. Current practices include the following:
Capillary malformations The first-line treatment of capillary malformations involves the flashlamp-pumped PDL or the argon laser. The PDL is the treatment of choice in infants and children. Treatment involves a brief pulse that is relatively specific for the lesion, compared with that from the argon laser. This results in a lesser degree of damage to the surrounding tissues, minimizing the hypopigmentation often associated with treatment. In one series, 15.3% of patients treated with the PDL experienced more than 90% lesional resolution, and 65.3% of patients had lightening from 50-90%. Poor or no response was observed in 17.8% and 1.2% of patients, respectively.25 The 577-nm, 0.36-ms PDL has proven to be efficacious in light-colored stains and is more effective in younger compared with older children. The 585-nm PDL was developed and became popular in the mid 1990s because one study demonstrated an improved response in deeper lesions. With the use of the 585-nm, 0.45-ms PDL, some authors report the highest degree of blanching in pink lesions, while others find a lower degree of lightening in pink compared with red lesions. Some authors report better results with fewer treatments in infants and young children, whereas others do not find significantly better results in the youngest age groups. Most studies report a more favorable response in lesions on the face or neck and on the torso, but one study did not find any significant difference in the required number of treatments for different body locations. The argon laser is currently used in adults with darker or raised port-wine stains. This is because of the hypopigmentation and textural changes that are often the result of treatment. Lymphatic malformations For localized lymphatic malformations, various pharmacologic agents have been used around the world to treat lymphangioma. Some of the agents used in sclerotic therapy include boiling water, tetracycline, bleomycin, and cyclophosphamide. None of the treatments has been completely effective. Sclerotherapy may also be of value in venous malformations, acquired digital arteriovenous malformations, and hemangiomas.26 Special considerations should be taken with lymphatic malformations on the tongue or glottis. Malformations on the tongue (formerly known as lymphangioma circumscriptum) should be managed with laser resurfacing. If these lesions are large enough to interfere with respiration, tongue reduction surgery should be performed. Malformations on the glottis should be treated with carbon dioxide laser and debulking therapy with aggressive airway management. Aspiration of lymphangiomas has been used in the past but has largely fallen out of favor because of the high rate of recurrence. However, it can still be useful to manage life-threatening lymphangiomas that are in need of immediate reduction. Management of diffuse disease is usually surgical (see below), although cyclophosphamide may be a good choice for life-threatening diffuse neonatal hemangiomatosis that is unresponsive to corticosteroids.27 However, an essential component of care in children with diffuse disease should involve a child psychiatrist because management of diffuse disease often entails lifelong morbidity. Surgical therapyVascular tumors When resecting hemangiomas, the surgeon must decide whether the result from surgical intervention would be more cosmetically acceptable than that from medical treatment or watchful waiting. In addition, the timing of resection should be considered because facial deformities can have a psychologic impact on children, especially when they enter the school years. Some surgeons, however, advocate postponing excision until after the involution phase of hemangiomas. In April 2002, Mulliken studied the outcome of surgical resection of hemangiomas involving a circular excision with purse-string closure compared with that involving a standard lenticular incision.28 A line of excision was drawn to include the area affected by hemangiomatous infiltration and any fibrofatty residuum. In involuted lesions, this included all areas that were atrophic, scarred, pigmented, or otherwise deemed irreversibly scarred. The marked area was then excised to the depths of the subcutaneous tissue, and the circular incision was drawn together using a single 4-0 or 5-0 purse-string suture. When the suture was tightened, ridges of skin were created at the newly formed wound margin. If a small opening remained at the center of the purse-string, a gauze wick was placed inside, and the wound was dressed. The ridges leveled off over the course of several weeks, and after several months of scar remodeling, the physician and parents decided whether to accept the result or have the patient undergo a second procedure. The study concluded that after a circular incision with purse-string closure, the mean scar area was 15% of the original area. The study also concluded that the mean scar length with circular excision would be 72% shorter than with lenticular excision. The results did not vary significantly among the phases in which the procedure was performed or between the techniques used in a second procedure (circular or lenticular). To date, no other excision and closure technique produces a smaller scar. Lymphatic malformations As stated above, surgical excision is the treatment of choice for localized lymphangioma if anatomically possible. Of the various surgical techniques that have been explored over the years, total removal of the tumor, leaving behind no cystic epithelium, has been the most reliable procedure. In one study, total resection led to no recurrences following the procedure. This is compared with recurrences in 6 of 7 patients if excision of only protruding cystic epithelium was performed.29 The surgical management of diffuse disease is often a complex and lifelong endeavor with substantial rates of morbidity. Patients and parents must be aware of this before surgery is undertaken so that the high likelihood of complications can be factored into the initial decisions in management. The first step in managing diffuse cervicofacial disease is to allow for an adequate airway and adequate feeding. This often requires a tracheotomy and possibly a gastrostomy. The next task is to divide the patient into anatomic zones and then attempt to manage those zones as individual areas of localized disease until a given zone is completely free of disease. Failure to completely excise a diseased area predisposes the patient to a high rate of recurrence in that region. Approaching the divided components of the total malformation from the top down is also advisable, if possible, to avoid lymphatic swelling at more proximal locations. Children with diffuse cervicofacial disease also frequently require maxillomandibular reconstruction because of overgrowth of the facial bones. Depending on the severity of the disease and infiltration into local structures, these additional procedures can attenuate an already lengthy treatment process. Follow-upVascular tumors As stated above, regular follow-up care is an integral part of the management of hemangiomas to monitor the natural history of the lesion and to provide ongoing comfort to the patient's parents. The physician should also monitor for potential adverse effects of treatment, including the following:
If the patient has undergone surgical excision, the physician and the parents should decide whether the results of the excision are acceptable or whether a second excision and closure should be performed. Capillary malformations Patients who received laser treatment for capillary malformations should be monitored for recurrence and for the adverse effects of laser therapy. The argon laser can result in hypopigmented lesions and textural changes in the skin. Although both of these outcomes are factored into the decision to use the argon laser, the physician and the patient should come to a consensus on an acceptable result after treatment. Recurrence can occur up to several years after treatment. Lymphatic malformations Patients who undergo excision of a localized lymphangioma should simply be monitored for recurrence. Individuals treated for diffuse disease are often lifelong patients and should receive regular follow-up. The support of a child therapist may be useful to help manage the psychosocial impact of the disease and treatment. COMPLICATIONSSection 8 of 13
Vascular tumors In some cases, hemangiomas can be life threatening or severely problematic, interfering with eating, breathing, seeing, hearing, and speaking. These cases require immediate and aggressive intervention because delayed treatment can lead to improper development of these organ systems. The most common sequela of untreated problematic vascular tumors is amblyopia secondary to lesions of the upper eyelid. Such cases should be immediately evaluated by an ophthalmologist. Hemangiomas located on internal organs can be dangerous because they are difficult to detect. Furthermore, by the time they are detected, the infant often requires aggressive treatment. Despite intervention, internal hemangiomas are still associated with high mortality rates. Infants who have hemangiomatosis (ie, multiple smaller hemangiomas) may also have internal lesions. Visceral hemangiomas occur in the liver, intestines, airway, and brain. If an infant has more than 3 clinically apparent hemangiomas, ultrasonography of the entire body should be performed to rule out internal lesions. Other suspicious findings include the following:
Capillary malformations Patients with port-wine stains should be evaluated and monitored for a larger syndromic entity. Hemangiomas that are part of the Klippel-Trenaunay-Weber syndrome can be located on the lungs, spleen, liver, bladder, or colon. Visceral involvement can often lead to substantial morbidity in the form of internal hemorrhage. The recognized components of Sturge-Weber syndrome can appear at various points in the patient's life. Neurologic findings are evident within the first 2 years of life, often manifesting as seizures. In addition, patients can exhibit hemiparesis, visual field defects, or lower intelligence quotient (IQ) scores. Ocular defects, such as glaucoma or ocular hemangiomas, can lead to visual loss or retinal detachment that requires prompt evaluation and treatment. Lymphatic malformations Complications of lymphangiomas depend on the location and extent of disease. Diffuse cervicofacial disease can result in mandibulomaxillary hypertrophy because of direct invasion of the bone and growth of the malformation within the bone. A secure airway is also essential in these patients; a tracheostomy may be required to avoid acute respiratory problems. Lymphangiomas often swell with the onset of general viral infection or remote bacterial infection. This typically resolves with the resolution of the infection. Occasionally, lymphangiomas can become infected, and intravenous (IV) antibiotic treatment is required. OUTCOME AND PROGNOSISSection 9 of 13
Vascular tumors As mentioned earlier, approximately 75% of hemangiomas of infancy involute without intervention. Involution is usually complete by age 7-10 years. Ulceration secondary to infection or trauma increases the risk of residual scarring. A large facial lesion is also more likely to leave an unacceptable scar. Patients with significant residual scarring may choose to undergo a surgical procedure. If systemic glucocorticoids are used in the manner stated above, efficacy rates of 84% have been reported. Side effects during and after treatment have also been previously mentioned. All treatments listed above are used primarily for troublesome situations or cases where scarring after involution is considered unacceptable. Studies to date have been unable to prove a significant change in outcome with earlier intervention versus watchful waiting. Capillary malformations The PDL has vastly enhanced the treatment efficacy of cutaneous capillary malformations. Laser wavelengths that are specific for the lesion results in a lesser degree of damage to the surrounding tissues, minimizing the risk of hypopigmentation. In one series, 15.3% of patients treated with the PDL experienced more than 90% lesional resolution, and 65.3% of patients had lightening from 50-90%.25 Poor or no response was observed in 17.8% and 1.2% of patients, respectively. Lymphatic malformations Complete surgical resection of localized disease has proven to be extremely effective. Recurrence rates are low if complete removal of cystic epithelium is achieved. Patients and parents undergoing surgical resection of diffuse disease should be aware that despite intervention, diffuse lymphatic disease entails lifelong morbidity. An essential component of care in these children should involve a child psychiatrist to cope with this stressful situation. FUTURE AND CONTROVERSIESSection 10 of 13
Vascular tumors To develop more effective medical treatments, studies are needed to clarify the pathogenic mechanism behind the formation of sporadic hemangiomas. Cases of autosomal dominant inheritance suggest a genetic predisposition to some hemangiomas. Identification of these genetic alterations may shed light on the pathogenesis behind sporadic cases. Vascular malformations Because some lesions spontaneously regress, evidence suggests that conservative treatment or a period of watchful waiting may be beneficial in cases in which disease is not life threatening. The efficacy of OK-432 (Picibanil) has been well documented for the treatment of localized lymphangioma in ACKNOWLEDGMENTSSection 11 of 13
The authors and editors of eMedicine gratefully acknowledge the contributions of previous author Neal Ammar, MD, to the development and writing of this article. MULTIMEDIASection 12 of 13
REFERENCESSection 13 of 13
Arterial Vascular Malformations Including Hemangiomas and Lymphangiomas excerpt Article Last Updated: Aug 8, 2008 | ||||||||||||||||||||||||||
