12 The Surgical Management of Vascular Malformations
The treatment of vascular malformations is a multidisciplinary endeavor. Vascular malformations are rare and clinically may be quite extensive and disfiguring. Their biologic nature, ability to distort normal anatomy, and vascular nature make them a challenge to treat. Because various modalities may be used, a multidisciplinary team approach is necessary. In consideration of the surgical management, and in keeping with our belief that a multidisciplinary approach is best, in this chapter, we discuss the surgical and laser management in this context.
12.2 Port-Wine Stains (Capillary Malformations)
12.2.1 Laser Treatment
The treatment of choice for port-wine stain (PWS) is laser. Because of their proven efficacy and safety, pulsed dye lasers (PDLs) have become the standard device, and their use is widespread. 1 , 2 Despite progress in the design of PDLs, treatment results are only marginally improved. 3 , 4 At best, only a small percentage of PWSs are completely cleared with treatment. The vast majority will lighten significantly, and a small percentage (30%) will improve only marginally. 5 , 6 These PWSs are referred to as resistant lesions. A multitude of devices and treatment strategies have been advocated for the so-called resistant lesions, but to date, no single device has been consistently shown to be superior. 7 , 8 , 9
The best age to start treatment is also in question. Although early treatment is advocated by some authorities, others found no advantage. 10 , 11 Recurrence after treatment is now widely accepted. 5 , 12 Orten et al were the first to describe this, 5 and although their findings were unpopular at first, others have corroborated their findings. Although reported incidences of recurrence vary, it stands to reason that, given a sufficient passage of time, all PWSs will eventually recur. Nonetheless, laser treatment is appropriate. One should treat until maximal lightening has been achieved. After this, a maintenance treatment, given periodically, will maintain this result ( Fig. 12.1 ).
12.2.2 Surgical Management
A proportion of patients with PWS experience soft tissue hypertrophy in its distribution. 13 , 14 This hypertrophy appears to involve the entire dermatome, although some areas, such as the upper or lower lip, seem to be more commonly involved ( Fig. 12.2 ). As a result, mesodermal and ectodermal elements are involved. The upper lip and maxilla are commonly involved with skeletal, muscle, and subcutaneous fat hypertrophy ( Fig. 12.3 ).
A frequent finding is cobblestone formation, which appears to be made up of hypertrophic vascular tissue ( Fig. 12.4 , Fig. 12.5 ). In early cobblestones, the lesion is clearly vascular and empties on compression. An established cobblestone is more fibrous and less compressible. It is therefore obvious that correction of soft tissue hypertrophy and established cobblestones should be surgical.
The surgical approach should be a zonal problem-oriented approach. Some of these problems have been illustrated ( Fig. 12.6 , Fig. 12.7 ). The most commonly encountered problems are upper and lower lip hypertrophy. The affected lip is usually too long and too thick. The length is usually addressed through a wedge resection and the thickness through a wet-dry margin or a vermiliocutaneous junction debulking procedure. It is often necessary to remove a wedge of muscle together with submucosal or subcutaneous tissues. Cheek debulking is usually carried out through a nasolabial incision, and the forehead can be approached through a bicoronal flap or a suprabrow incision. In these instances, subcutaneous fat is usually reduced, leaving intact muscle and nerves. The frontal or maxillary bone can also be recontoured.
Early cobblestones are vascular and can be treated with pulsed dye laser or a neodymium:yttrium aluminum garner (Nd:YAG) laser. A more established lesion should be excised. Relaxed skin tension lines should be respected during these procedures.
Staged procedures are common because correction in more than one vector is needed. It must be remembered that if the patient is still growing, a repeat procedure should be anticipated. Also, hormonal surges (puberty, pregnancy) will also lead to accelerated growth. In some cases, tissue expanders are used to redrape larger subunits of the face. Skin grafts are not ideal: PWS will ultimately grow through the graft, and skin color is often not well matched.
Treatment is aimed at improving the patient’s quality of life. Despite the fact that the possibility of a repeat procedure exists, this possibility should not discourage the surgeon from performing surgery early. Removal of disfiguring tissue will improve the patient’s self-esteem and, therefore, his or her quality of life.
12.3 Venous Malformations
Venous malformations (VMs) are slow-flow congenital lesions that occur in one to two live births per 10 thousand. 15 They are mostly simple solitary lesions but may be mixed (i.e., capillary venous) or syndromic (blue rubber bleb nevus syndrome or Maffucci syndrome). 16 Whereas the vast majority are considered to be inborn errors of development, inherited forms have been described and are more often multifocal. VMs are soft, compressible masses that expand when in a dependent position. The overlying skin usually shows bluish discoloration, the intensity of which depends on the depth of the lesion.
Treatment of VMs is almost always a multidisciplinary endeavor. In most cases, the lesion is sclerosed as a primary treatment or sclerosed preoperatively. 17 , 18 , 19 Surgical resection of an unsclerosed VM can be extremely difficult. Profuse intraoperative hemorrhage is not only hazardous, but it will often make preservation of tissue planes and important structures extremely difficult. The addition of preoperative sclerotherapy some 24 hours before surgery will dramatically reduce intraoperative hemorrhage, and the attendant edema will enhance the ease and safety of the procedure.
The decision of when and how to treat should be mindful of the natural history of the disease. VMs naturally increase in size over the life of the patient. The rate of expansion will vary. “High-grade” or “active” lesions will expand rapidly, whereas other lesions expand in a more benign fashion. Trauma, hemorrhage, sepsis, hormonal fluctuations (puberty and pregnancy), and thrombosis will result in more rapid expansion. Advancing age results in a thinning of the supportive connective tissues, resulting in more rapid expansion. Three modalities have a role in the management of VMs:
Laser treatment (we typically use an Nd:YAG laser)
Sclerotherapy (percutaneous or transmucosal)
The choice of modality will depend on the depth of the lesion and its anatomical location. In general, superficial lesions and the superficial component of a compound lesion are treated with am Nd:YAG laser ( Fig. 12.8 ). 20 , 21
Deep lesions will be treated with sclerotherapy as a primary modality or with combined sclerotherapy followed by surgical removal 24 hours later ( Fig. 12.9 ). 17 , 22 In anatomical locations where surgery will add significant morbidity, sclerotherapy is advocated. It is important to realize that although sclerotherapy may seem innocuous, it carries significant risk of morbidity and mortality. 17 , 22 , 23 The risks and benefits should be carefully weighed before selecting a treatment plan.
12.3.1 Laser Treatment
We typically prefer to use an Nd:YAG laser (1,064 nm), although it is possible to use a similar wavelength from a different laser source. At this wavelength, light will penetrate to a depth of approximately 1 cm. 20 It is therefore effective only for superficial lesions ( Fig. 12.8 ). When it is used transcutaneously, we prefer a larger spot size with surface cooling. We have become accustomed to using dynamic cooling with cryogen. The coolant will cool the skin’s surface and thereby prevent thermal necrosis. The laser pulse follows the coolant. The end point should be vasoconstriction or darkening of the lesion. Darkening represents intravascular coagulation. No blanching of the overlying skin should be allowed because this will lead to necrosis and the risk of scarring. When treating the middle third and posterior third of the tongue or the larynx, using a hand piece with cooling is not possible. In these cases, a bare fiber is preferred. The fiber should be held about 1 mm from the treated surface, and a grid pattern (“snowstorm pattern”) of treatment should be delivered ( Fig. 12.10 ). 24
This form of treatment may be a primary treatment for superficial lesions or an adjunct for compound lesions. In compound disease, laser treatment is used to treat the superficial component of a compound lesion. By treating the skin or mucosa, a layer of subcutaneous or submucosal scarring is achieved, which will enable the surgeon, during surgery, to raise a mucosal or skin flap that is avascular and will not be at risk for necrosis. Laser treatment should be performed about 6 weeks before surgery to be effective.
When treating laryngeal disease, a 600-μm quartz fiber, taped to a zero-degree telescope can be used to deliver energy to the larynx. We usually place the airway in suspension on a mayo stand and deliver the laser pulses with the fiber and telescope apparatus ( Fig. 12.11 , Fig. 12.12 ). It is important to not deliver too many pulses during one treatment session because treatment invariably produces edema, which will reach a maximum over 18 to 24 hours. The patient should be admitted postoperatively for observation, and corticosteroids should be administered intraoperatively as well as postoperatively for 4 days.
12.3.2 Surgical Resection
Lesions of the head and neck should be considered according to their anatomical site. We have divided these lesions into facial and cervical. Facial lesions can be focal or diffuse. Focal lesions should be approached according to their anatomical location and their depth. Diffuse lesions are generally treated in stages, and during each stage, the area being treated is approached in accordance with its depth and anatomical location. Superficial lesions and the superficial component of a complex lesion are treated with a Nd:YAG laser as described previously.
Facial lesions may involve one or more of the following:
The parotid space
The buccal fat space
The premaxilla or premandibular space.
The approach to each of these spaces should be considered separately as each space has distinct anatomical considerations. 25
The Parotid Space
Small focal lesions of the parotid may be sclerosed or surgically removed. We have no firm guidelines for this decision and believe that the preference of the multidisciplinary team gives consensus. Diffuse lesions within the parotid can be extremely difficult to resect without causing intraoperative hemorrhage and facial nerve damage. We may treat these primarily with sclerotherapy or, if a decision is made to proceed with surgery, the lesion will be sclerosed 24 to 48 hours before the surgery. The surgical approach is via a parotidectomy incision ( Fig. 12.13 ). The main trunk of the facial nerve can be found at the stylomastoid foramen. If too much bleeding is encountered during this dissection, then the individual branches can be found in front of the parotid gland, over the fascia on the masseter, and then traced retrograde toward the foramen. We prefer this approach because it may avoid a bloody dissection and safe dissection of the facial nerve branches. The parotid gland is then removed in the same fashion as it is in tumor dissection.