Treatments for hemangiomas consist of medical management (non-surgical treatment), laser therapy, and surgical excision. More to the point and most debatable is when to treat the patient as in many children the hemangioma will involute spontaneously with treatment then directed at removal of residual mass, hypertrophic, or atrophic scarring after involution has occurred. The situation calls for wide and lengthy clinical experience guiding those engaged in clinical decisions.
Medical management of hemangiomas is limited to three drugs: steroids, vincristine, and propranolol. All three have advantages and disadvantages with all three having some therapeutic effect while not being completely curative.
Steroids or prednisone may be given by mouth or injected (intra-lesional treatment) into the growth itself. The mechanism of action of steroids on hemangiomas remains unknown. The primary disadvantage is that steroids should not be given to children for long periods of time due to serious systemic side effects on the immune and endocrine systems.
Vincristine is a chemotherapeutic drug that has long been used for the treatment of cancer in children. It requires the placement of a catheter in a large vein in the neck or other location with infusions of the agent performed once a week. It is usually reserved for patients who have hemangiomas in the liver or those who have lesions that are not responsive to steroids.
Propranolol, a drug originally developed to treat high blood pressure in adults, was recently found to be effective in treating both segmental and focal hemangiomas both during the growth phase and the involutional phase. Its only side effect is a possible drop in blood sugar if the child happens to miss a meal or feeding. The results to date are very encouraging.
Laser Treatment is reserved for the treatment of superficial (confined to the outer layer of the skin, the epidermis) hemagiomas only. The pulsed dye laser is the only laser that delivers a broad wavelength of lased light with a low enough energy so there is no risk of scarring. Treatments are usually begun at two to three months of age on a monthly basis and continued until the lesion is no longer apparent. No anesthesia is required. Prior to the invention of the pulsed dye laser other types of lasers were used with variable amounts of scarring complicating their use.
Surgical Treatment is necessary in patients who have eyelid involvement due to the risk of blindness, ulceration especially with large hematomas, and in cases of tracheal hemangiomas with threatened airway obstruction. Actual surgical techniques are not standardized as they are as variable as the lesions themselves and are complex. The major risk is excessive blood loss during the operation due to the immense vascularity of the growth.
Treatments for Venular Malformations (Port Wine Stains)
The pulsed dye laser is the gold standard for treating venular malformations. Ideally, treatment should begin as soon as possible. Laser treatments are performed every six to eight weeks initially. After the first few treatments time between treatments is increased to twelve to eighteen weeks. The treatment regimen usually is a total of eight to ten treatments. Very few lesions disappear entirely but most lighten significantly with treatment. With time most will darken somewhat and require repeated treatments over the years. Fortunately, since only topical cream or local anesthesia is required the procedure may be done in the office or outpatient center.
Surgical resection of facial, head and neck venular malformations is usually reserved for massive lesions in children that are life-threatening due to ulceration and hemorrhage. This is a rare event. More commonly in middle-aged adults, resection is performed because of the formation of unsightly cobblestones, a blood blister appearing dilation of the vessels on the surface of the lesion or thickening of the lesion, both of which are unsightly. Occasionally, resection may need to be repeated due to continued cobblestone formation and dermal thickening with the passage of time.
Treatments for Venous Malformations
Sclerotherapy is the injection of irritating agents into the arteries supplying the venous malformation. The result is scarring of the inner lining of the blood vessel (intima). A clot then forms in the lumen (inside) of the vessel depriving the lesion of its blood supply. This result is death (necrosis) of the areas of the malformation supplied by the vessel treated. With necrosis of areas of the lesion the areas will initially blacken and then be replaced by scar tissue and lighten.
Sclerotherapy injected into the arteries is done by an interventional radiologist using a dye that can be seen on a real time x-ray so expertise in the procedure is required. Often sclerotherapy is done prior to surgical removal to lessen blood loss during the operative procedure or as noted above it may be done as a stand-alone procedure. No anesthesia is required unless the patient is a child. Sclerotherapy may also be used in combination with laser treatments to enhance results as it treats the deeper layers of the lesion that laser treatment cannot reach.
Sclerosing agents may be injected into the skin (percutaneous sclerotherapy) in random sites. This will also result in areas of necrosis. The procedure may be repeated several times. Noteworthy is the development of significant post-treatment swelling that may take time to resolve.
The Neodinium YAG (yttrium aluminum garnet) laser is the laser of choice for treating venous malformations since it is highly effective. However, it only has effect on superficial lesions or the superficial layer of deep lesions. It is critical that the treating surgeon have experience with the laser as the power used to treat the lesion is highly variable between individual patients. The use of an incorrect power setting for an individual patient will result in scarring. The choice of a power setting is based on clinical instinct rather than a “cookbook” guide. Sclerotherapy and or surgery may be combined with Nd:YAG laser treatment. Treatment may be performed with a topical anesthetic cream in an office setting or as an outpatient.
Complete removal of the lesion is the goal of surgical therapy. This ideal was not often achieved prior to the use of angiographically-guided sclerotherapy in order to lessen blood loss during the procedure. Nowadays, even large lesions may be removed either in stages or during a single operation with this dual technique with an acceptable number of post-operative recurrences. The sclerosing agent must be injected within a short time prior to the procedure as vessels will regrow into the lesion rather rapidly defeating the whole purpose of sclerosis treatment in the first place.
Treatments for Arterio-Venous Malformations
Angiography is performed in the radiology department by an interventional radiologist who specializes in injecting arteries through a small catheter introduced into the artery with a special dye that may be seen on x-rays in real time. Once the artery or arteries supplying the malformation are identified plastic beads, glue or foam; substances that form a plug much like stop-leak for a radiator on a car, are injected into the artery or arteries (embolization) thus depriving the malformation of its blood supply. Embolization may be used as a stand-alone treatment or as part of staged therapy prior to surgical resection to lessen blood loss during the operation.
Due to the fact that arterio-venous malformations lie deep under the skin laser treatment has very limited if any application in the treatment of these lesions. When used it may be of some help with the bluish skin discoloration overlying the lesion. When employed the pulsed dye laser is the type that is utilized.
Essentially, the decision today is surgical and embolization each as the sole therapy, or both in combination. A degree of clinical judgment is demanded on the course to take and the decision should be made by a multidisciplinary team in conjunction with the patient or those responsible if the patient is a child considering all of the risk factors as well as the expected results. Operative therapy combined with surgery in larger lesions in the final analysis yields the best outcome with the fewest risks for larger lesions. Unquestionably, one the other or both are the gold standard today in treatment of arterio-venous malformations.
Treatments for Lymphatic Malformations
A sclerosing agent injected through the skin (percutaneous sclerotherapy) is the only practical method of sclerotherapy as lymph vessels due not have a pulse to drive angiographic emboli into the supplying lymph vessels. Two types of lesions are recognized based on the predominant structural anatomy of the lesion: microcystic (small cysts) and macrocystic (large cysts). In general, macrocystic lesions are more responsive to percutaneous sclerotherapy. Three agents are currently in use in the United States for percutaneous sclerotherapy. One, a chemotherapeutic agent (anti-cancer drug) OK-432 (Picibinil), is currently in the approval process as an orphan drug with the Food and Drug Administration (FDA). The other two are doxycycline, an antibiotic related to tetracycline developed many years ago and bleomycin also a chemotherapeutic agent, one of the first chemotherapeutic agents still in use today in both children and adults. Pure alcohol (ethanol), the type consumed, is one of the first sclerosing agents used but it is of limited efficacy. Bleomycin alone has effect on both micro- and macrocystic lesions as well as superior effectiveness with regard to shrinkage of the lesion which is defined as decrease in size by over 50%. Regardless of the agent used, the agent must contact the wall of the cysts making up the lesion in order to irritate the lining and induce scarring for closure to occur. Usually, several treatments are required with general anesthesia necessary in for each encounter.
Surgical excision of macrocystic lymphatic malformations in one operation often results in a cure. Unfortunately, with the microcystic type the rule is multiple resections for recurrence after recurrence. The operations are further complicated by the location of these lesions in the cheek or the neck areas. Coursing over, under and through the lesion are multiple small nerves of tremendous importance as they supply vital functions of such diverse structures as the facial muscles, tongue muscles, sensation in the face and neck, swallowing and speech, and finally the upper limb muscles. This fact puts the nerves at substantial risk of injury even in experienced hands, particularly when scarring from previous excisions further complicates the operative field. However, surgery with or without sclerotherapy still represents the best opportunity for cure of lymphatic malformations.
Patients who have lymphatic malformations of the tongue will frequently develop blebs on the surface of the lesion that will often rupture with the extrusion of blood and lymph fluid. The blebs are also a source of pain especially with the consumption of spicy foods, carbonated beverages, or warmer foods. The carbon dioxide laser is the laser used. Treatments are effective but must be frequently repeated. The procedures are done under a general anesthetic and there is post-operative pain that requires medication.