Vascular lesions are relatively common abnormalities of the skin and underlying tissues, more commonly known as birthmarks. There are three major categories of vascular lesions: Hemangiomas, Vascular Malformations, and Pyogenic Granulomas. While these birthmarks can look similar at times, they each vary in terms of origin and necessary treatment.
Hemangiomas are the most common type of vascular lesion in children They are benign, or noncancerous, tumors of the cells that line blood vessels. They usually appear at or shortly after birth as faint areas of pinkish-red discoloration of the skin and then quickly undergo a period of rapid growt
During this time, the lesions enlarge in size and thickness, darken in color and can have problems with ulceration (breakdown of the overlying skin), bleeding and/or infection. The appearance of a hemangioma will vary based on the depth of involvement of the lesion. Superficial hemangiomas appear a bright red color and frequently grow outward on top of the skin surface whereas deeper lesions look more like a bruise on the skin with a soft tissue mass beneath it.
The growth phase lasts from 6-12 months, after which, the hemangioma goes through a period of stabilization and then slow involution (shrinkage) that usually takes many years before it’s complete. After involution, the lesions are smaller and faded in color, but may have some residual skin or fatty tissue remaining. In addition, previous ulceration can leave permanent scarring of the skin.
Hemangiomas involving the eyelid can cause permanent loss of eyesight if they grow to block an infant’s vision. Similarly, lesions that obstruct the ear canal can interfere with hearing and speech development. Because of this, lesions in these critical areas will be treated more aggressively.
Ulcerated lesions (often around the mouth, perianal or genital areas) may benefit from early laser treatment to stabilize the surface, promote healing, and help minimize bleeding. Finally, patients with multiple hemangiomas or extensive lesions around the neck and face may have associated internal lesions of the airway or vital organs that put them at risk for life threatening complications, and should be referred to an experienced practitioner for further diagnosis and care.
Many hemangiomas can be monitored by your the pediatrician or dermatologist as they grow naturally without the need for treatment. However, extensive lesions or lesions with ulceration, bleeding or super-infection should be seen by a specialist. Those occurring in cosmetically or functionally sensitive areas (such as the nose tip or lip) should also be seen by a doctor experienced in the management of hemangiomas, as well as a pediatric plastic surgeon.
Treatment of Hemangiomas
The treatment of hemangiomas depends on their characteristic life-cycle and location and can be categorized into medical and surgical therapies. Your child’s plan will be customized depending on their specific lesion and its behavior.
Medication: Patients may be placed on medications such as oral corticosteroids like prednisone or an oral blood pressure medication called propranolol, in order to help slow the rapid growth phase and promote involution. These medications can be effective, but have some side effects that make careful patient selection and monitoring important. Bulky hemangiomas may sometimes benefit from an injection of a corticosteroid into the lesion, which is usually done under a brief anesthetic. Other medications (such as interferon given by serial injection) can have more serious potential side effects, and are reserved for patients with extensive lesions that do not respond to other therapies.
Surgery: The surgical treatment of hemangiomas must carefully balance the need for early treatment with the scarring that will be created by the procedure. Because most lesions undergo at least a fair amount of involution on their own, it is important to delay most surgery until this occurs to allow tissue to stabilize before reconstruction. This will maximize healing and minimize the length of scars. An exception to waiting may occur if surgery provides the quickest option to relieve obstruction or allows better staging of reconstruction the lesion(s). These situations are best determined by an experienced pediatric plastic surgeon.
Laser Therapy: Finally, laser therapy can be used to treat ulcerated lesions, as well as any residual blood vessels or discoloration that may remain after involution. Extensive airway lesions may also require CO2 laser therapy for management, which is carried out by an otolaryngologist (ENT surgeon) under anesthesia.
Hemangiomas are fairly common infantile vascular birthmarks whose noticeable appearance and growth cycle can cause significant distress for the family of affected children. Our pediatric plastic surgeons will customize a treatment plan for your child’s distinct lesion, working with other experienced practitioners to achieve the best long-term aesthetic outcome and a happy, healthy child.
Vascular malformations represent a group of congenital errors in the formation of vessels. They are classified based on the type of vessel(s) that comprise the lesion and the speed of blood flow occurring within them. There are four types of Vascular Malformations:
- Capillary Malformations
- Venous Malformations
- Lymphatic Malformations
- Arteriovenous (or arterial) Malformations
Each type has its own distinct characteristics and prognosis, which affects the treatment plan required.
Also known as port wine stains, capillary malformations (CM) are comprised of irregular capillaries and small veins running through the deeper layers of the skin. These low flow lesions appear at birth as flat pink areas of skin discoloration and usually darken over time to become a deep purple color. They can eventually develop changes in skin texture with a cobble-stone effect and can also be associated with overgrowth of the underlying bone and soft tissues.
When found in the distribution of the first division of the trigeminal nerve, capillary malformations can indicate the presence of Sturge-Weber syndrome, a rare autosomal dominant condition. Patients with Sturge-Weber syndrome can have associated vascular malformations of the central nervous system including CM, venous, and arteriovenous malformations which can lead to seizure disorder, learning and behavioral disorders. In addition, the eyes must be followed regularly by an ophthalmologist to watch for the early development of glaucoma.
Treatment of Capillary Malformations
CMs present a significant cosmetic deformity to the patient. They are best treated with serial laser treatments using a pulse-dye laser, which targets the hemoglobin molecules found in the lesions. This helps shut down the irregular vessels and fade the lesions. Laser therapy is most effective in treating lesions when they are lighter in color. Multiple laser treatments under anesthesia are usually initiated in childhood. Early treatment can also help to prevent some of the skin texture changes by slowing the progression of the lesion. Although somewhat uncommon, bony and soft tissue asymmetries can develop in these patients due to tissue overgrowth. These conditions require a combination of removal and suspension techniques to help achieve better balance and symmetry.
Venous malformations (VM) are the most common type of vascular malformation found in 1-4% of the population. They are low flow lesions made up of irregular collections of abnormal venous channels within the skin, soft tissue or even bone and vital organs. VMs are present at birth, but may not be apparent until they grow. They appear as soft bluish compressible masses without any pulsations that swell in the dependent position or with straining. These lesions can range in severity from barely noticeable to extensive lesions that cause significant cosmetic and functional problems due to the mass itself and associated overgrowth of the bones and soft tissue. Episodes of pain can occur when a blood clot called a phlebolith develops within the VM and causes blocked blood flow and inflammation. These areas can also become infected requiring antibiotics for treatment.
VMs can be associated with Maffucci syndrome where patients have VMs of the skin and soft tissue and multiple benign bony tumors. These bony tumors develop during childhood causing abnormal bone growth and contour, and must be followed, as they can turn malignant over time.
Your doctor may order tests such as an MRI to help diagnose your child’s lesion and distinguish it from some of the other vascular malformations in order to plan the best course of treatment. Elevation of the affected area or special compression garments or wrappings may be recommended to help manage the discomfort associated with swelling of the lesions. He or she may also prescribe aspirin therapy for your child. This can decrease the chances of a phlebolith developing, and help treat any episodes of pain associated with these by decreasing inflammation. If there is a sudden change in size or swelling of the VM associated with pain or fever, it is important to see your doctor to check for infection which needs antibiotic therapy.
Treatment of Venous Malformations
The treatment of VM falls into two major categories: sclerotherapy and excision.
Sclerotherapy: Sclerotherapy involves injection of a chemical sclerosant into the venous channels of the lesion to cause inflammation and eventual shutdown of the vessels with shrinkage of the VM. This is usually performed by an interventional radiologist under anesthesia using guidance from ultrasound, X-ray, or MRI. The most common agents used today are ethanol or sodium tetradecyl sulfate (STS). If possible, occlusion of the VM using tourniquets or applied pressure during treatment may help the agent to remain within the lesions longer and be more effective. Sclerotherapy alone can be effective for some lesions, but concerns arise when the lesion lies close to vital structures or beneath the skin. Complications such as nerve dysfunction, kidney damage, cardiac arrest, and severe allergic reaction have also been reported.
Excision: Excision of VMs is often required either alone or in combination with sclerotherapy. It allows direct de-bulking of the area while preserving vital structures with tissue rearrangement and suspension to help improve form and function. Major complications include bleeding, scarring, and injury to surrounding tissues.
Lymphatic malformations (LM) are another relatively common vascular malformation made up of collections of abnormal lymphatic channels found usually within the skin and soft tissue and rarely in vital organs or bones. They are divided into subtypes, microcystic or macrocystic, which have different presentations and treatment strategies. Lesions can also present in a mixed form with both elements present.
Microcystic LMs appear as groups of small clear vesicles (blister like clusters). When found at the skin or mucosal level (often in the mouth) they can cause problems with weeping and pain. Microcystic LM lesions can also infiltrate the deeper soft tissues causing firm masses.
Macrocystic LMs are composed of larger cyst-like lymphatic channels and appear as deeper-seated, softer compressible masses often in the neck or axilla with overlying bluish skin discoloration. Large lesions can sometimes be detected on prenatal ultrasound.
LMs of any type are at risk for infection, which is noted by rapid and sometimes massive swelling of the lesion, warmth or redness, and pain. This requires quick treatment with antibiotic therapy. Other complications include possible airway compromise due to obstruction by a large neck or oral lesion either at birth or after rapid swelling associated with infection. In addition, disfigurement or functional impairment from the lesion itself or associated soft tissue or bony overgrowth can be significant.
Treatment of Lymphatic Malformations
Often, the diagnosis of LM and its subtype can be made clinically. However, further definition of the extent of a lesion and its relationship to vital structures can be seen well on MRI and is often necessary when planning treatment. Treatment of LM depends on the subtype.
Symptomatic superficial microcystic lesions of the skin and mucosa can often be effectively treated with laser therapy to help remove and seal off oozing vesicles. Deeper areas of infiltrating microcystic LM require surgical excision for de-bulking, and are often only partially removed as they can be intimately associated with vital structures and hard to distinguish from the surrounding normal tissue. Because the cysts are small and do not freely communicate, sclerotherapy is usually ineffective for these lesions.
Macrocystic LMs can be well treated by either sclerotherapy or surgical excision. Sclerotherapy is a good option especially in poor surgical candidates or when lesions lie in locations that are hard to access surgically or are intimately associated with vital structures. As with VMs, sclerotherapy is usually performed by interventional radiologists with radiologic guidance under anesthesia. Common side effects include fever, local pain, and redness. Major complications such as swelling and airway compromise are possible and may require hospitalization for observation or airway management after the procedure. Although most patients have a significant reduction in the size of their LM after one treatment, multiple injections may be necessary for the best result.
Surgical excision is another good treatment option for macrocystic lesions, especially prior to any other treatments, as they can be more easily separated out from normal tissue. It can also be used in combination with sclerotherapy for final de-bulking and re-suspension of tissues if needed. Drawbacks include the introduction of scarring and possible complications of bleeding and tissue injury.
Arteriovenous malformations (AVM) are the rarest of the vascular lesions made up of abnormal vascular connections between arteries and veins. These are high flow lesions as they allow fast travel of blood from the higher-pressure arterial system directly into the veins without first travelling through the capillary system to supply the tissues.
Although the cause is unknown, they are thought to occur because of abnormal persistence of vascular connections between arteries and veins that exist during embryologic development. AVMs occur most commonly in the head and neck regions; the brain is the most common location. AVMs are present at birth, but are usually asymptomatic and often innocent appearing pinkish-red blushes on the overlying skin.
Although these lesions do not technically multiply, they do appear to grow over time as collateral channels for blood flow are recruited, increasing the blood flow through the area. This often occurs in late childhood or around puberty, and is thought to be associated with hormonal changes or trauma. Eventually they become warm, pulsating, subcutaneous masses with purple to red discoloration of the overlying skin. AVMs within the head are usually silent and go undiagnosed until complications such as headaches, stroke, or hemorrhage arise.
Because the increased blood flow through an AVM does not effectively travel through the tissues to supply them with adequate nutrients and oxygen, tissues and the surrounding skin and underlying bone may die. This can cause pain and lead to episodes of bleeding that can be life-threatening. Because of the high-speed flow through the lesion, large AVMs also put extra strain on the heart and can lead to exaggerated heart growth and/or heart failure. AVMs in the brain and lining of the brain can be associated with stroke and seizures. Finally, continued expansion of these lesions can cause significant disfigurement and functional problems.
AVMs are usually diagnosed clinically as they begin to expand, but can be confused early in life for a hemangioma or other vascular malformation. Ultrasound can be used early on to detect increased flow in the area and distinguish it from the other lesions. When planning possible treatment strategies MRI/MRA may be ordered to provide the most detailed information about the extent of the AVM and its relationship to the surrounding tissues. Angiography gives the most information about blood flow patterns through the lesion. Dye is injected and the blood flow is examined as it passes through the vessels.
Treatment of Arteriovenous Malformations
The treatment of AVM is complicated and potentially dangerous due to the large amount of blood flow through the lesion. Treatment, therefore, requires an experienced surgeon and team familiar with the management of AVM.
Small lesions not presenting any symptoms are usually best left alone. For larger lesions causing significant bleeding complications, severe disfigurement, or functional problems, a combination of embolization and surgical removal usually offer the best chance of a cure.
One to two days before surgery, an interventional radiologist or vascular surgeon will perform embolization to shut down blood flow through the AVM. This decreases some of the blood loss during removal. In order to truly cure these lesions, they must be removed completely. Otherwise, the residual lesion can recruit blood flow from other sources leading to recurrence.
These removals often result in substantial tissue defects that require considerable reconstructive efforts. Complete excision is often not practical when a lesion involves crucial structures and underlying bones. Significant complications including loss of function, massive blood loss, and even death can occur.
As an alternative, palliation by partial embolization or removals may be beneficial. In the case of AVMs within the head, treatment by radiation, embolization, or surgical removal can be used depending on the lesion.
The treatment of AVMs is complex. The best results are achieved with careful analysis and planning by an experienced team. Together, a customized plan of treatment can be developed to help safely manage this difficult and rare lesion.
Pyogenic granulomas are benign, acquired vascular lesions of the skin that can form in response to local tissue injury. They appear as rapidly-growing, red, and often ulcerating bump or polyp-like masses which bleed easily when disturbed. They are commonly found in children or pregnant women especially on the mouth or fingertips.
Treatment of Pyogenic Granulomas
Although these lesions can go away on their own, they usually require treatment. Surgical strategies include removal by incision with a margin of normal skin. As well as, primary repair or shaving with cauterization of the base; this can result in more scarring.