The classification of the various types of vascular malformations was described above. These lesions are delivered to the attention from the plastic surgeon for many reasons. The mainstay of treatment, except for small ones in the extremities, is really a combined approach with radiologic-guided superselective embolization, and surgical debulking when necessary. Properly done, risks are minimized, although these can include ulceration, infection and occasionally catastrophic complications such as limb loss.
Unfortunately, due to their extensive intercalation and incorporation within tissues, dissection planes are extremely difficult to reliably discern. These are usually space-occupying lesions, and recidivism is common. Like endothelial cells elsewhere in the body, the endothelial cells in these lesions are quiescent until stimulated to proliferate by injury, distressingly often with a surgical insult. This makes up about their recalcitrance following monotherapy for example surgery.
Therefore, the treatment goals need to be clearly explained to the individual. They rarely include cure and instead are mainly palliative to control the complications of ulceration, bleeding, as well as for improved hygiene. Reoperation is unfortunately the rule, rather than the exception. Vascular malformations can be categorized as arteriovenous (AVM), venous, capillary, or lymphatic malformations. Unlike hemangiomas of infancy, these lesions don't display a proclivity for either sex.
A brief history and physical can advice the diagnosis. Capillary malformations are typically noted at birth; they range from the well-known port wine stain. Eighty percent of lymphatic lesions will become apparent either at birth or throughout the first year of life. Venous malformations will be noted at any time from birth to adulthood, a well known fact which is important when evaluating the young adult with prominent leg veins who presents for sclerotherapy. They're typically darker lesions seen on the skin or mucosal surfaces.
Arteriovenous malformations can become evident in times of hormonal fluctuations, for example puberty or pregnancy. Lymphatic malformations are composed of dilated lymphatic channels and vesicles; these lesions will typically swell by having an infectious episode. They might be either macrocystic or microcystic. Plain radiographs are only utilized when there exists a potential for bone or joint involvement. MRI and ultrasound could play a complementary role in the treatment of these lesions.
Ultrasound could be a confirmatory screening test to show the vascular nature of the soft tissue mass, and MRI will show the lesion's relationship to adjacent structures as well as feeding and draining vessels. Due to these benefits, MRI is clearly the first-line diagnostic tool. However, angiography plays a larger role in the diagnosis and treatment of vascular malformations than in hemangiomas. The differential diagnosis of an AVM includes malignant vascular tumors such as angiosarcoma and rhabdomyosarcoma. Once the benign nature of these lesions cannot be definitively ascertained through an imaging approach, a biopsy from the lesion is warranted.
The optimal treatment should be tailored towards the type of lesion and its location. Due to the wide variety of presenting symptoms and large number of lesions, this can be a discipline that is continuing to evolve. There are several lesions that require immediate treatment. Although the plastic surgeon may not be directly involved in the treatment phase of those lesions, he should be conscious of what constitutes a life-threatening lesion so that the individual could be promptly referred if necessary.
Venous malformations are treated mainly by sclerotherapy (occasionally by an interventional radiologist), typically when the lesion becomes symptomatic. Debulking surgery, when indicated, is performed 6-8 weeks following sclerotherapy. Occasionally, lesions in or near vital structures (such as the hand/digits) are treated by surgical excision alone.
Lymphatic malformations are notoriously hard to treat. Most patients present for treatment of cellulitis and can benefit initially from elevation of the affected body part and intravenous antibiotics. Macrocystic lymphatic malformations are best given sclerotherapy. Most lesions will need surgical resections, a process which is tedious, bloody and fraught with recidivism. Due to the often bloody procedures, they're best staged.
Capillary malformations is often treated with laser therapy. Up to 75% of patients will benefit from this therapy. As these lesions can be accompanied by underlying soft tissue and bony hypertrophy, surgical resections from the involved tissues may be indicated.
AVMs would be the most treacherous of vascular malformations to deal with. They're treated when they become symptomatic; unfortunately, the herald symptoms can include life-threatening bleeding or heart failure. There are some guidelines to treatment from the AVM which all members of a multidisciplinary vascular anomalies team should be conscious of. In relation to embolization of these lesions, proximal feeder vessels should not be embolized, since the lesion will only become more vascular via reactive neovascularization.
As these lesions may require multiple interventions, a poorly planned embolization will make it hard or impossible to do future embolizations. These lesions will need wide, deep, extensive resections, often necessitating a microvascular free flap to attain wound closure.
The most crucial point in treating vascular anomalies is establishing the right diagnosis. Incorrect treatment for any lesion can have dire, even life-threatening, consequences. Radiologic imaging with a specialist radiologist is important to preventing the above mentioned. Furthermore, there exist a set of vascular malignancies which demand treatment and not a "watchful waiting" approach.
Even within the more benign subset of patients with hemangiomas, a sizable proportion will need some surgical treatment whether or not the lesions involute, particularly on the face. This really is generally not appreciated by the referring physicians. In addition, these lesions often take years to involute, and also the parents need to be so informed.
The use of novel markers, with GLUT-1 being a paradigm with this, will in the future enable more precise identification of the type and life-stage of these lesions. Novel approaches using anti-angiogenic factors may ease the effects of steroids or another anti-neoplastic regimens currently accustomed to treat these lesions, and could be particularly beneficial for patients with vascular malformations, a lot of which turn into medical and surgical orphans.
It may be possible to bar vascular stem cell incorporation within these lesions, or conversely, exploit the proclivity of those lesions to draw in vascular progenitors by genetically modifying vascular stem cells to impair the development of hemangiomas or accelerate their involution.
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Note: This article was sent to us by: Patricia Phillips at 02102011
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