Arterial Vascular Malformations Including Hemangiomas and Lymphangiomas Treatment & Management

Updated: Feb 24, 2017
  • Author: Robert A Schwartz, MD, MPH; Chief Editor: Mary C Mancini, MD, PhD, MMM  more...
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Treatment

Approach Considerations

Vascular 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-20th century changed the way in which these lesions are managed.

Medical and surgical treatments of vascular tumors are discussed below, and the way in which the involutional phase fits into the treatment plan is explained. However, there is less of a consensus regarding 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.

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 excision would produce a more cosmetically acceptable result than conservative treatment would. The surgeon must also choose a technique that leads to the best possible outcome (see Surgical Therapy). Depending on the location of the lesion, surgical resection may be the initial treatment of choice.

In the past, capillary malformations were managed by using various treatment modalities, including electrocauterization 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. In addition, new medical treatments are being developed, with encouraging results.

Management of diffuse lymphatic disease has historically been surgical. Although techniques have improved over the years, treatment still involves multiple procedures with substantial morbidity.

Future and controversies

To develop more effective medical treatments for vascular tumors, 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.

Because some vascular malformations 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 has been well documented for the treatment of localized lymphangioma in Japan; studies are under way in the United States. [38] A better understanding of the pathogenesis behind lymphangioma is needed to develop newer and more efficacious medical treatments.

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Medical Therapy

Vascular tumors

Approximately 75% of hemangiomas involute without intervention and are in anatomically benign locations; thus, they are 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.

The synthesis of informational advances in functional and phenotypic effects may enable the use of animal models to test less-invasive, targeted, perhaps locally administered, biologic therapies. [39]

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:

  • Systemic glucocorticoids are the first-line therapy for lesions that are life threatening or cause severe deformity [40] ; efficacy rates have been reported at 84% with prednisone equivalent doses of 2.9 mg/kg if administered during the proliferative phase
  • The beta blocker propranolol is a promising an oral agent for first-line systemic therapy of infantile hemangiomas [41] ; it can produce a dramatic and rapid response, though serious consideration of the risks and benefits of its use must be considered [42, 43] ; the topical beta-blocker timolol lotion may also be strikingly effective, [44]  but premature and low-birthweight infants must be watched for signs of bradycardia, hypotension, apnea, and hypothermia [45]
  • Triamcinolone is indicated for smaller hemangiomas; intralesional injections administered every 4-6 weeks have been shown to result in faster rates of involution in 30% of patients
  • Interferon alfa-2a is used for life-threatening or deforming lesions that do not respond to glucocorticoid therapy [46] ; it is subcutaneously administered at a dose of 1-3 million U/m 2 of body surface area
  • Flashlamp-pumped PDL is indicated for superficial residual lesions that remain after involution, though it is not effective on deep hemangiomas; some studies have shown no advantage to early laser intervention versus conservative treatment, but one study suggests possible benefit [47]
  • Local wound care is shown to alleviate pain and infection in ulcerated lesions [48] ; wet compresses can be used to debride the ulcer in conjunction with topical mupirocin, bacitracin, or metronidazole
  • Ethanol sclerotherapy for venous malformation has been utilized [49]

Capillary malformations

First-line treatment of capillary malformations involves use of 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% to 90%. Poor response was observed in 17.8% of patients and no response in 1.2%. [22]

The 577-nm 0.36-ms PDL has proven to be efficacious in light-colored stains and is more effective in younger than in 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, whereas others find a lower degree of lightening in pink lesions than in red ones.

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. [50] None of the treatments has been completely effective. Sclerotherapy may also be of value in venous malformations, acquired digital arteriovenous malformations, and hemangiomas. [51]

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), though cyclophosphamide may be a good choice for life-threatening diffuse neonatal hemangiomatosis that is unresponsive to corticosteroids. [52] 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.

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Surgical Therapy

Indications

Vascular tumors

Surgical excision of vascular tumors (hemangiomas) is controversial because most lesions involute with little intervention (75%). Generally, surgery is indicated if the procedure is expected 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:

  • If an abnormal scar or excess tissue is present after natural involution
  • If lesions are ulcerated and bleed excessively or are associated with pain
  • If lesions interfere with the development or activities necessary for life (eg, 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 laser treatment. Surgical methods have included dermabrasion, electrocauterization, and cryosurgery.

Lymphatic malformations

For therapeutic purposes, lymphangiomas are often divided into localized and diffuse disease. In localized disease, a period of watchful waiting is appropriate if the lesion is not immediately compromising life functions. However, fewer than 15% of lesions regress spontaneously. Some surgeons believe that if the lesion has not spontaneously regressed by age 5 years, surgical intervention is warranted; others believe that excision should be performed sooner to avoid complications of lymphangioma (eg, infection). [53] Infection can be a severe sequela of lymphangiomas.

At present, most surgeons agree that complete excision is the preferred treatment for localized lymphangiomas, depending on the accessibility of the lesion. However, the close quarters of the head and neck give rise to a tedious dissection that often results in sacrifice of vital structures and significant postoperative impairment. In these cases, it may be best to avoid surgery and pursue an alternative therapy. 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).

Contraindications

Vascular tumors

No absolute contraindications for 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 for 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 for 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.

Vascular tumors

When resecting hemangiomas, the surgeon must decide whether the result of surgical intervention would be more cosmetically acceptable than that of medical treatment or watchful waiting. In addition, the timing of resection should be considered because facial deformities can have a psychological impact on children, especially when they enter the school years. Some surgeons, however, advocate postponing excision until after the involution phase of hemangiomas.

Mulliken et al studied the outcome of surgical resection of hemangiomas involving a circular excision with purse-string closure compared with that involving a standard lenticular incision. [54] 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 with a single 4-0 or 5-0 purse-string suture. [54] 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 skin 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. [54]

The study concluded that after a circular incision with purse-string closure, the mean scar area was 15% of the original area. [54] 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 six of seven patients if excision of only protruding cystic epithelium was performed. [55] Another option for lymphangioma circumscriptum may be treatment with fractional carbon dioxide laser ablation. [56]

The surgical management of diffuse disease is often a complex and lifelong endeavor that is accompanied by substantial 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 ensure an adequate airway and adequate feeding; this often requires tracheotomy and possibly gastrostomy.

The next task is to divide the patient into anatomic zones and then try to manage those zones as individual areas of localized disease until a given zone is completely disease-free. Failure to excise a diseased area completely predisposes to a high recurrence rate of in that area. 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 the extent of infiltration into local structures, these additional procedures can draw out an already lengthy treatment process.

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Long-Term Monitoring

Vascular tumors

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:

  • With local glucocorticoid treatment, a case of retrobulbar hematoma with subsequent blindness has been reported
  • With systemic glucocorticoid treatment, adrenal suppression and other common adverse effects of systemic steroid therapy may occur
  • With interferon alfa-2a administration, adverse effects include transient fever, neutropenia, and elevated liver function test results; several cases of spastic diplegia (some permanent) have also been reported in infants after treatment
  • Laser therapy may produce hypopigmented lesions

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.

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