eMedicine Specialties > Pediatrics: Surgery > Vascular Surgery
Arterial Vascular Malformations Including Hemangiomas and Lymphangiomas: Treatment
Updated: Aug 8, 2008
Treatment
Medical Therapy
Vascular 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:
- Systemic glucocorticoids are the first-line therapy for lesions that are life threatening or cause severe deformity.22 Efficacy rates have been reported at 84% with prednisone equivalent doses of 2.9 mg/kg if administered during the proliferative phase.
- 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.23 It is subcutaneously administered at a dose of 1-3 million U/m2 of body surface area.
- Flashlamp-pumped PDL is indicated for superficial residual lesions that remain after involution; this is not effective on deep hemangiomas. Recent studies show no advantage to early laser intervention versus conservative treatment.
- Local wound care is shown to alleviate pain and infection in ulcerated lesions.24 Wet compresses can be used to debride the ulcer in conjunction with topical mupirocin, bacitracin, or metronidazole.
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 Therapy
Vascular 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-up
Vascular 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:
- 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 of which were permanent, have also been reported in infants following 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.
Complications
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:
- Jaundice, which may be a sign of liver hemangiomas
- Bloody stools, which may be a sign of GI hemangiomas
- Stridor or dyspnea, which may be a sign of airway hemangiomas
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.
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Further Reading
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
