Vascular Anomalies Center
The Vascular Anomalies Center in the Division of Plastic and Maxillofacial Surgery at Childrens Hospital Los Angeles specializes in the treatment of a complex group of conditions caused by abnormally developed blood vessels. These can include arteries, veins, lymphatic channels, and/or capillaries. Vascular anomalies can be difficult to accurately diagnose and treat. For a small percentage of patients, there is no available treatment. To ensure the best possible outcome, children with vascular anomalies require the combined expertise of an interdisciplinary team of specialists whose primary focus is the management of these multifaceted disorders. Aylson Harrison, Manager
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Hemangiomas
What are hemangiomas and what do they look like?
Hemangiomas are the most common vascular anomaly and the most common tumor of childhood and infancy. They are classified as non-cancerous blood vessel tumors that may occur anywhere in the body, whether in the skin or within organs. Abnormally dense collections of dilated small blood vessels (or capillaries) comprise hemangiomas. Although not necessarily present at birth, they do typically appear in the first 2 weeks of life. Deeper hemangiomas may not be apparent until two to three months of age. There may be a faint discoloration or barely visible spot at birth in approximately 30 percent to 40 percent of patients. Most hemangiomas (80 percent) appear as a single lesion on the body. Less commonly (20 percent), they may appear in multiple locations. There are more female patients with hemangiomas than there are males (approximately 3:1).
Hemangiomas often appear on the infant’s head and neck area or other areas that are easily noticed. This can be quite disconcerting to parents as they are frequently asked if their baby was injured or has some tumor. In rare instances, these lesions may occur in locations that interfere with normal function. Depending on the size, hemangiomas may interfere with breathing, feeding, or even urination. For example, hemangiomas involving the eyelid can interfere with development of normal vision and must be treated urgently.
How are hemangiomas diagnosed?
Hemangiomas are most frequently diagnosed by patient history and physical examination. When there is a deep lesion, magnetic resonance imaging (MRI) may be performed to delineate the extent of the problem and any involvement of internal organs. Rarely, hemangiomas are associated with unusual syndromic conditions. A thorough examination and additional studies may be required to detect the presence of these syndromes.
What happens to hemangiomas over time?
During the first six to nine months of infancy, hemangiomas tend to go through a proliferative phase where they grow rapidly. The hemangioma may become more noticeable during this phase. It will appear bright red and take on a slightly raised, or bumpy, configuration. However, if the hemangioma lies deep to the skin, the skin itself may appear bluish and only slightly raised.
The growth of hemangiomas is unpredictable. Similarly, the degree to which these lesions involute (shrink) over time is also difficult to predict. After the initial growth period (proliferative phase), hemangiomas will then enter an involutional phase (regression). This occurs gradually and is sometimes not completed until the child is 10 years of age. During this time, hemangiomas will shrink, and the color will fade to a dull purple or grey. The lesion will feel soft to the touch. Sometimes, the remaining skin overlying hemangiomas may not appear normal and, in fact, may feel and look like crepe paper. If there was an ulceration over the lesion, then there may be an associated scar. If present on the scalp or other hair bearing areas on the body, there may be alopecia (permanent hair loss) in these areas.
There is no reliable way to predict the rate at which a particular hemangioma will shrink. But as a general rule, in approximately 50 percent of patients, regression is complete by five years of age, and by seven years of age in 70 percent of patients. This does not apply, however, to certain types of hemangiomas. Some congenital forms (those present at birth) will shrink more rapidly (rapidly involuting congenital hemagiomas), and others may not shrink at all (non-involuting congenital hemangiomas).
How do you treat hemangiomas?
Most hemangiomas will regress on their own without intervention. For many lesions, patience and time may be the best treatment. However, those lesions interfering with function or for those lesions that may interfere with a normal childhood, we may recommend removal.
In the past, many pediatricians recommended waiting to treat hemangiomas, but we have learned that early treatment provides significant advantages. During the first few months of life, the growing hemangioma acts like a balloon under the skin to expand both the affected and nearby normal skin. In many instances, early intervention allows the surgeon to remove the affected skin and then use the excess normal skin to close with tension; thus, decreasing the size of the scar.
These options may include laser treatment for color changes, surgery to remove redundant skin, or liposuction to remove the fatty tissue under the skin. Makeup is also a good alternative to conceal any minor imperfections.
For serious cases where the hemangioma poses an immediate risk to life or compromises important functions such as eyesight, the use of systemic corticosteroids may be considered. This medication may be given by mouth, through an IV, or injected directly into the hemangioma and surrounding tissues. Corticosteroids can speed the involution phase or prevent further growth of the hemangioma. All interventions have risks and benefits. Using corticosteroids is no different. The benefits of this treatment must be weighed against the potential risks, and a discussion must be initiated with the child’s parents and the treating physicians to determine the best course of action.
In some instances, arterial embolization (blocking the feeding blood vessels) may be possible. This will “choke” the hemangioma and cause it to shrink. However, not all patients are candidates for this therapy, and there is no certainty that the hemangioma will shrink. The procedure is performed by an interventional radiologist.
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What are some of the potential complications of hemangiomas?
The most common complication of hemangiomas is bleeding. This is also the most frightening. Bleeding can be stopped very effectively with direct and continuous hand pressure over the hemangioma for five-15 minutes. If bleeding is uncontrolled with hand pressure, then the child should be seen by a physician in a local emergency department. Bleeding occurs because the skin overlying the hemangioma is much thinner and friable than normal. If this skin dries out and cracks or is scratched, then the area may bleed or become ulcerated (breakdown of skin). Ulcerations can be painful and very uncomfortable. They can also become infected.
Prevention is the preferred treatment. Whether ulcerated or not, we recommend keeping the hemangioma clean with soap and water and moisturized at all times with any fragrance-free, mild moisturizing cream or even simple petroleum jelly. Trimming nails will also lessen the likelihood of inadvertently scratching the lesion. If irritated by clothing or a diaper seam, the hemangioma may be covered with a light gauze dressing to protect it from being rubbed and irritated.
Often, hemangiomas occur on the face or trunk. When present on the face, a hemangioma can cause considerable psychosocial stress for the parents and sometimes the child. Openly discussing these issues with each other and the medical team will alleviate much distress.
Vascular Malformations
Vascular malformations can be classified based on their vessel type (how they are supplied with blood: veins, arteries, and lymphatic vessels) and flow characteristics (how fast the blood flows to the area). For example, slow-flow malformations include capillary, lymphatic, and venous malformations. Fast-flow malformations include arteriovenous malformations.
The most noticeable difference between vascular malformations and hemangiomas is the natural course of the lesions. Hemangiomas grow rapidly, then begin to shrink slowly, whereas vascular malformations grow with the child and do not shrink. Also, vascular malformations can exert growth effects on nearby tissues, causing overgrowth, undergrowth, or disfigurement. Whereas hemangiomas are considered to be non-cancerous tumors because of their ability to proliferate on the cellular level, vascular malformations do not exhibit such proliferative potential. However, they may undergo enlargement during the adolescent growth spurt or in females taking birth control pills. There are five types of vascular malformations.
Capillary Malformations
What are capillary malformations and what do they look like?
Capillary malformations are slow-flow malformations that appear on the skin. As the name suggests, the predominant type of vessel in these lesions is the capillary. The skin will appear deep purple or red and can be patchy and quite extensive. They may be referred to as "port-wine stains," and most often occur on the face.
How do you diagnose capillary malformations?
Most often the diagnosis is made upon obtaining a patient history and physical examination. An MRI may be done to delineate the extent of the lesion and to rule out deeper tissue abnormalities. Capillary malformations may also be associated with rare syndromes. Although not routinely performed, a biopsy of the lesion will reveal a capillary-type lesion.
What happens to capillary malformations over time?
As already mentioned, these vascular lesions do not shrink. In fact, they often become darker and more nodular when the child reaches adulthood. They may also cause skeletal (bone) overgrowth, resulting in changes in the shape of the body parts they occupy, including the arms, legs, trunk, or face.
Capillary malformations may occur in isolation or may be part of a combined vascular anomaly, such as Parkes Weber or Klippel-Trenaunay syndromes.
How do you treat capillary malformations?
Laser therapy may be effective in treating some capillary malformations. Eradication of the red or purple pigmentation is often incomplete with this modality. Surgical excision may be indicated in some instances of skeletal and soft tissue overgrowth, although indications are narrow. This may require multiple staged procedures and must be considered on a case-by-case basis.
What are some of the potential complications of capillary malformations?
Capillary malformations usually do not produce any serious complications. Occasionally, these lesions will bleed or ulcerate. Conservative intervention with direct pressure to stop bleeding and local wound care for ulcerations are usually all that is necessary.
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What are lymphatic malformations and what do they look like?
Lymphatic malformations are composed of malformed, dilated channels filled with lymphatic fluid. The lymphatic system is an extension of the vascular (blood) system. Its function is to remove waste and excess fluid from the soft tissues of the body. These lesions are sometimes referred to as “lymphangiomas.”
Lymphatic malformations can be "microcystic" (small cysts), "macrocystic" (large cysts), or a combination of both. Treatments for each type will vary, therefore, proper diagnosis is essential. Most lymphatic malformations are present at birth, or identified within the first two years of life. However, some may not be evident until adolescence or early adulthood.
(insert picture of LM)
What happens to lymphatic malformations over time?
Lymphatic malformations tend to grow deep beneath the surface, usually between the overlying skin and the underlying musculature. They may cause asymmetry, distortion, and bony hypertrophy (overgrowth) in the areas they occur. A lymphatic malformation of an extremity is frequently associated with lymphedema (swelling of the limb). When they occur closer to the skin, lymphatic malformations may manifest as vesicles (blisters) that are clear or bluish.
Lymphatic malformations do not shrink. In fact, they expand or contract depending on the flow of lymphatic fluid, the presence of inflammation, or bleeding inside the dilated channels. Infection, trauma, or bleeding in these channels can result in rapid growth of the lesion. This sudden increase in the size of the lesion can be alarming for both patient and family.
How do you treat lymphatic malformations?
Compression garments are often the first line of therapy to control filling of dilated lymphatic channels and swelling of extremities. Keeping the area of the lymphatic malformation clean is also extremely important, particularly for lesions involving the feet and legs. Keeping these areas clean and dry helps reduce the risk of skin breakdown and infection.
Sclerotherapy is the process of instilling a caustic material (usually a type of concentrated alcohol) into the dilated channels in an effort to cause scarring and collapse of the vessel walls. This is performed by the interventional radiologist under general anesthesia and is well-described for macrocystic lymphatic malformations. Often, the lesion will shrink in size, but will not completely disappear. Sometimes, multiple sessions are required to effectively treat an area of lymphatic malformation.
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Incisions in tissues overlying lymphatic malformations may be subject to postoperative healing problems, given the compromised vascularity. Infection, hematoma (blood collection), and prolonged fluid drainage (oozing from the wound) are frequently encountered. For lymphatic malformations involving the extremities, periodic monitoring as the child grows will alert the physician to changes in volume or circumference of the extremity in addition to length discrepancies. Once again, compression garments and physical therapy may be helpful to alleviate these symptoms.
If the lymphatic malformation has a superficial component on the surface of the skin (lymphangioma circumscriptum), this may be treated with dermabrasion (scraping) or excision and grafting (surgical removal of the affected skin with coverage using a partial thickness piece of skin from another part of the body).
What are some of the potential complications of lymphatic malformations?
Sudden enlargement of a lymphatic malformation is likely the result of bleeding or an infection (cellulitis). If there is an infection involving an area that contains a lymphatic malformation, antibiotic therapy must be initiated. Cellulitis can be differentiated from bleeding most often by symptoms of redness and heat over the lymphatic malformation. If you suspect cellulitis, you should see your family doctor immediately. Bleeding into a lymphatic malformation can be painful. Administration of pain medication, rest, and time will often treat this problem effectively.
Venous Malformations
What are venous malformations and what do they look like?
Venous malformations are slow-flow vascular lesions composed of dilatations of abnormal veins in the body. They are present at birth, but they are not always evident at birth. They appear as either a faint blue patch or a soft blue vascular mass. Most often, these lesions are located on the face, arms, legs, or trunk. They may frequently be confused with deep hemangiomas, and have often been incorrectly termed “cavernous hemangiomas.”
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Venous malformations are easily compressible (flattened) and generally soft. They tend to increase in size when they are in a dependent position. For example, a venous malformation of an upper extremity may swell when dangled. But when elevated, the blood will drain and the extremity will return to a more normal size.
How do you diagnose venous malformations?
As with all vascular malformations, diagnosis depends on a thorough patient history and physical examination. A history of swelling and flattening of an area containing a malformation may indicate that it is of venous origin. Magnetic resonance imaging (MRI) provides much information as to the specific type and extent of a vascular lesion, including its relation to neighboring vital structures such as arteries and nerves. Treatment plans can be determined accurately based on physical findings and MRI results.
What happens to a venous malformation over time?
Venous malformations grow in proportion with the child, expand slowly, and often enlarge when the child experiences a growth spurt or takes oral contraceptive pills. Women will experience enlargement and worsening symptoms during pregnancy. Unlike lymphatic malformations, venous malformations rarely cause overgrowth when located in the limbs. Some reports have even cited cases of undergrowth in limbs affected by venous malformations.
How do you treat venous malformations?
As with most slow-flow vascular lesions, treatment of venous malformations usually requires application of compression garments as the initial line of treatment when possible. This is true for most venous malformations of the extremities. The child is measured and fitted by a physical or occupational therapist.
Sclerotherapy is the mainstay of invasive treatment for venous malformations. An interventional radiologist will inject a sclerosing agent, such as alcohol, into the abnormally dilated channels of the lesion under general anesthesia. This causes scarring and collapse of the vascular channels, preventing blood from pooling in these locations. Often, multiple sessions are required to address problematic areas of venous malformations. These sessions are spaced several weeks apart and may be required years down the line.
The decision to pursue sclerotherapy is largely dependent on the size and location of the venous malformation. This is determined in our multidisciplinary clinic, with input from all members of the Vascular Anomalies Team. The immediate complications of sclerosing include blistering, full thickness tissue loss, and possible nerve damage.
In select instances, the interventional radiologist may be able to treat a particular venous malformation with an endovenous laser (inside the vein). A probe is inserted into dilated venous channels, and the laser is then activated, causing obliteration of abnormal vessels.
Surgical excision of a venous malformation is performed on symptomatic areas and only when it can be done safely. As with lymphatic malformations, complete eradication of venous malformations is frequently not possible, and previously excised areas may re-expand. Therefore, several excisions may be required for large lesions that develop symptoms.
What are some of the potential complications of venous malformations?
For venous malformations that involve or are located near the skin, varicosities may be quite prominent and unsightly. Also, the bluish discoloration of the skin is sometimes disconcerting. Commonly, the massively dilated veins may cause blood to pool and stagnate, resulting in blood clot formation. These blood clots can become inflamed and painful. When this occurs, it is called “thrombophlebitis.” Treatment centers on application of warm compresses, elevation of the extremity, and administration of anti-inflammatory medication (for example, ibuprofen). In some instances, this can lead to an infection (cellulitis) that will require antibiotics to treat. Repetitive bouts of thrombophlebitis can be distressing and will need to be addressed by more invasive means. Clots may occur frequently and can eventually calcify and harden to form stone-like masses along the varicosities, called “phleboliths.” These masses can be felt when they are superficial. They generally do not cause problems for the patient, although many patients will discontinue use of their compression garments secondary to pain.
Arteriovenous Malformations
What are arteriovenous malformations and what do they look like?
Arteriovenous malformations (AVM) are fast-flow vascular lesions consisting of excess arteries connected directly to enlarged veins. The intervening tiny vascular architecture, or capillary network, is abnormal or absent. Arteriovenous fistulas (abnormal connections between arteries and veins) are frequently present. The result is a pulsatile mass. AVMs are present at birth, but often do not appear until infancy or later. There is no known cause of AVMs. Some occur in a familial disorder called hereditary hemorrhagic telangiectasia (HHT) (Rendu-Osler-Weber syndrome). AVMs are most common in the head and neck, followed in frequency by lesions of the limbs, trunk, and internal organs. Frequently, AVMs occur in the brain, causing headaches or other neurologic signs or with sudden intracranial bleeding.
(insert picture of AVM)
How do you diagnose arteriovenous malformations?
Diagnosis can be made on physical examination. An AVM is warm and not compressible like a venous malformation. It also has a notable pulse, or thrill. Magnetic resonance imaging (MRI) is effective in confirming the diagnosis and developing treatment options. Other studies such as ultrasonography or angiography may detect or delineate the extent of AVMs.
What happens to arteriovenous malformations over time?
The appearance of an AVM is sometimes mistaken for a hemangioma or "port-wine stain.” The "hormonal spurt" of puberty may play a role in the growth of AVMs. Interestingly, trauma to the area also seems to trigger accelerated expansion of these lesions.
In these situations, the skin becomes deep red or purple and warm to the touch. A mass may appear and a pulsation can often be felt. It can also be detected as a “bruit” (whooshing noise) with a stethoscope.
How do you treat arteriovenous malformations?
As with other vascular malformations, surgical excision of an AVM, when possible, gives the best chance for a “cure.” However, this is often difficult and not without risk. For intracranial AVMs, proton-beam irradiation may be used. Generally, treatment is considered when signs of pain, non-healing skin ulcerations, bleeding, or increased cardiac output occur. Frequently, embolization (occlusion of the feeding vessels to the AVM) of the lesion is done to temporarily occlude blood-flow prior to surgical excision. Embolization alone may treat some of the symptoms of AVMs, but it is usually not “curative.” It should be noted that any intervention may trigger expansion of these lesions. The mechanism for this phenomenon is unknown.
What are some of the potential complications of arteriovenous malformations?
Acutely, bleeding, pain, tissue loss, or ulceration may occur. When the lesion is extensive, high output cardiac failure may develop. These symptoms are largely dependent on size and location of the lesion. Intracranial AVMs may cause headaches, seizures, or can suddenly bleed, leading to an emergent situation.
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As the name suggests, combined vascular malformations involve more than one type of vascular channel (ie, capillary, venous, arterial, lymphatic). They are sometimes associated with syndromes and will bear the name(s) of physicians credited with the original descriptions of the disorder. They are often associated with overgrowth of both soft tissues and bone. There are two notable syndromes in this category: Klippel-Trenaunay and Parkes Weber.
Klippel-Trenaunay syndrome
is a combined capillary lymphaticovenous malformation (CLVM). It is a slow-flow anomaly, often associated with limb overgrowth. Less frequently, there may be undergrowth of an affected limb. The capillary malformations are numerous, and a red/purple stain appears over a specific geographic territory of the body. Prominent veins result from abnormal veins with absent valves, and hypertrophy (overgrowth of the limb) can range from minor to extreme. Clots may form in the massively dilated venous channels, which can migrate to the lungs. Any part of the trunk or extremity may be affected. Diagnosis often requires an MRI.Management is often conservative for these lesions. The decision to intervene with sclerotherapy or surgery is dictated by the presence of symptoms and limited only to areas that are problematic.
As with many vascular malformations, elastic compressive stockings are recommended for a limb with venous insufficiency and swelling. These stockings help reduce the pooling of fluid in the involved extremity. Surgery to correct leg length discrepancy is sometimes considered for large differences. Consultation with an orthopedic surgeon is required for this procedure.
Parkes Weber syndrome is a combined capillary arteriovenous malformation (CAVM); it is a fast-flow vascular anomaly. It can affect any extremity, and may result in limb overgrowth. There is commonly a geographic pink, flat, warm stain on the skin and deeper arteriovenous connections which can penetrate muscle. Shunting of blood can be extensive through these anomalous connections, sometimes resulting in high output congestive heart failure. This may require intervention, but management is usually similar to the conservative measures employed in Klippel-Trenaunay syndrome. Surgery and/or embolization/sclerotherapy must be considered carefully. These interventions may trigger expansion of the AVM component of the lesion, as previously described.
Vascular Tumors
Vascular tumors are also seen at the Vascular Anomalies Center. These are rare conditions that can present in a spectrum from benign to malignant (cancerous). Several vascular tumors have been described in the medical literature. Some of the more notable ones are described below. Treatment of these conditions often requires collaboration with an oncologist.
Kaposiform hemangioendothelioma (KHE)
KHE is a rare vascular tumor that can occur prior to or shortly after birth. They typically occur on the trunk or on parts of extremities located near the trunk. The appearance of skin that is involved with KHE is a deep purple or red color with a tense, shiny texture. They may appear bruise-like in certain areas adjacent to the tumor. One of the notable and most serious potential complications is the Kassabach-Meritt phenomenon, which is a bleeding disorder characterized by trapping of platelets within the tumor and depletion of other clotting factors. Treatment of KHE often requires steroid therapy and sometimes a chemotherapeutic agent called vincristine. Certain locations of KHE are related to high mortality rates.
Tufted angioma
Tufted angioma is a rare vascular tumor, usually of early childhood. Small red or purple patches appear on the skin of the upper body and neck. These can be quite extensive in some patients. Kassabach-Meritt phenomenon may occur with large tufted angiomas, which can lead to life-threatening bleeding complications. These tumors, however, grow slowly and are very similar to kaposiform hemangioendothelioma.
Hemangiopericytoma
Hemangiopericytomas are rare vascular tumors that occur mostly in adults and can be benign or malignant. They tend to occur in deep locations within the body, commonly in bones. When present in an infant, they are usually benign and go on to regress on their own. They are often discovered quite late when their large size leads to affects on the surrounding tissues. Malignant varieties of the tumor may metastasize to other parts of the body, usually the lungs. Treatment consists of surgically resecting the mass and surrounding tissues, amputation, radiation therapy, and/or chemotherapy.
Epithelioid hemangioendothelioma
Epithelioid hemangioendotheliomas are rare vascular tumors that may occur anywhere in the body, including bones, liver, and lungs. They are characteristically low-grade malignant tumors that grow slowly and have small potential for invading surrounding tissues or metastasizing to other parts of the body. As with hemangiopericytomas, surgical excision, radiation therapy, and/or chemotherapy are the available treatments.
Further Reading
Mulliken JB, Fishman SJ, Burrows PE. Vascular anomalies. Current Problems in Surgery. 2000; 37: 517-584.
Mulliken JB, Young AE. Vascular birthmarks: hemangiomas and malformations. Philadelphia: Saunders; 1998.
Mulliken JB, Glowacki J. Hemangiomas and vascular malformations in infants and children: a classification based on endothelial characteristics. Plast Reconstr Surg 1982; 69: 412-20.
Enjolras O, Mulliken JB. The current management of vascular birthmarks. Pediatr Dermatol 1993; 10: 311-33.
Appointments
To request a consultation with the Vascular Anomalies Center for your child, please contact us at (323) 361-2154