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A vascular anomaly is a kind of
congenital and therefore present at birth, others appear within weeks to years after birth and others are aquired by trauma or during pregnancy. Inherited vascular anomalies are also described and often present with a number of lesions that increase with patients’ age. Vascular anomalies can also be a part of a syndrome and, occasionally, they can be acquired by trauma. The estimated prevalence of vascular anomalies is 4.5%. [1] Vascular anomalies can occur throughout the whole body (skin, bone, liver, intestines, i.e.), but in 60% of patients vascular anomalies are localized in the head and neck region. [2]
Vascular anomalies can present in various ways. Vascular anomalies that are situated deep below the skin, appear blue and are often called cavernous. Superfiscial vascular anomalies appear as red-coloured stains and are associated with vascular anomalies affecting the dermis.
Historically, vascular anomalies have been labeled with descriptive terms, according to the food they resembled (port wine, strawberry, cherry, salmon patch). This imprecise terminology has caused diagnostic confusion, blocked communication and even caused incorrect treatment, as it does not differentiate between various vascular anomalies.[3] However, in 1982, Mulliken introduced a classifcation which replaced these descriptive terms and gave direction to the management of various vascular anomalies. This classification, based on clinical features, natural history and cellular characteristics, divides vascular anomalies into two groups: vascular tumors and vascular malformations. [4]
Although the appearance of both vascular tumors and vascular malformations can resemble, there are important differences between both.
Correct term
Incorrect terminology commoly used to describe vascular anomalies
Infantile hemangioma is the most common vascular tumor. It is a benign tumor, which occurs in 4-5% of Caucasian infants, but rarely in dark skinned infants. [5] It occurs in 20% of low weigth premature infants and 2.2 to 4.5 times more frequently in females.[6] IH most commonly presents in the head and neck regio (60%), but also involves the trunk and extremities. One third of these lesions is present at birth as a telangiectatic stain or ecchymotic area. During the first four weeks of life, 70% to 90% appear. Lesions that are situated beneath the skin may not appear until 3 to 4 months of age, when the tumor is large enough. During the first 9 months, IH undergoes rapidly growth, which is faster than the growth of the child. This is called the proliferating phase. After 9 months, the growth of the tumor will decrease and equal the growth of the child for about 3 months. After 12 months, the tumor will start to involute and might even disappear. Involution stops in one-third of patient by the age of 3 years, in 50% by the age of 5 years and in 72% by the age of 7 years. [7] Involution may result in residual telangiestasis, pallor, atrophy, textural changes and sometimes fibrofatty residuum.
Since 90% of IH is small, localized and
corticosteroids
, which accelerate involution: in 95% of patients, growth is stabilized and 75% of tumors decrease in size. Intralesional corticosteroids are most effective, but may require additional injections, as the effect is only temporarily. Systemic corticosteroids may cause lost of side-effects and are only used in problematic IH, which is too large to treat with intralesional injections.
During the proliferating phase, the tumor is highly vascular. Patients who undergo operative treatment during this period, are at risk for blood loss. Moreover, surgery during this phase, often leads to an inferior
laser therapy
.
Congenital Hemangioma
Congenital hemangioma can be distinguished from infantile hemangioma because it is fully developed at birth. It forms during prenatal life and has reached its maximal size at birth. Congenital hemangioma can even be diagnosed in utero by
prenatal ultrasound
. Unlike IH, CH is more common in the extremities, has an equal sex distribution, and is solitary, with an average diameter of 5 cm. It commonly presents in the head and neck and in the lower extremities.
Congenital hemangioma are divided into 2 subgroups: the rapidly involuting congenital hemangiomas (RICHs) and the non-involuting congenital hemangiomas(NICHs).
The
ulceration surrounded by a rim of pallor. It is noted for its involution which typically begins several weeks after birth and is completed no later than 14 months of age.[8] After regression RICH may cause a residual deformity, such as atrophic skin and subcutaneous tissue. It mainly affects the limbs (52%), but also the head and neck region (42%) and the trunk (6%). [5]
The
non-involuting congenital hemangioma, NICH, presents as a solitary, well-circumscribed reddish-pink to purple plaque with central telangiectasia and hypopigmented rim. [8]
In contrast to RICH, NICH does not involute and rarely ulcerates. It persists into late childhood and can even mimic a vascular malformation by growing commensurately with the child. Although NICH can resemble RICH in its external appearance, it can be differentiated from RICH by a greater elevation and coarse telangiectases. It mainly affects the head and neck region (43%), but also the limbs (38%) and the trunk (19%).
chronic infection
.
Although most NICH lesions are non-problematic and do not cause significant deformity, the threshold for resection of NICH is lower, because it neither involutes, nor responds to pharmacotherapy. RICH tumors are observed until involution is completed. Involuted RICH may leave behind atrophic tissue, which can be reconstructed with autologous grafts.[5] It is often best to postpone excision until regression is complete.
There are effective pharmacologic treatments, which include intralesional corticosteroid injection, systemic corticosteroid injection, interferon α-2a or α-2b and angiogenic inhibitors. The use of corticosteroids leads to accelerated regression in 30%, stabilization of growth in 40%, lightening of color and softening of the tumor. However, 30% shows minimal or no response. Another drug treatment is interferon α-2a or α-2b. It is often used for patients who did not respond to corticosteroids. Although the response rate is much slower, it has been successful for 80% of children treated. [9] The most serious side effect of interferon is a spastic diplegia. Other therapeutic options are embolization and pulsed-dye laser, which improves residual telangiectasias in RICH and in NICH.
. Although lesions occur solitary, they often involve large areas of the body, such as the head/neck region (40%), trunk (30%), or extremity (30%).
Usually, it is present at birth as a flat, reddish-purple, tense and
edematous
lesion.
Although half of lesions are congenital, 58% of KHE develop during infancy, 32% between age 1 and 10 years (32%) and 10% after 11 years of age. Moreover, adult onset has been described too with mainly males being affected. Both sexes are affected equally in children.
Lesions are often greater than 5 cm in diameter and can cause visible deformity and pain. During early childhood, KHE may enlarge and after 2 years of age, it may partially regress. Though, it usually persists longterm.
In addition, 50% of patients suffer from
petechiae and bleeding. This is called the Kasabach-Meritt Phenomenon, which is caused by trapping of platelets and other clotting factors within the tumor. Kasabach-Meritt Phenomenon is less likely in patients with lesions less than 8 cm. As two-thirds of adult-onset KHE tumors are less than 2 cm, KHE in adults is rarely associated with Kasabach-Meritt Phenomenon. [5]
Patients with KHE and Kasabach-Meritt Phenomenon present with petechiae and
ecchymosis
.
Most KHE tumors are diffuse involving multiple tissue planes and important structures. Resection of KHE is thus often difficult. Treatment of kaposiform hemangioendothelioma is therefore medical. The primary drug is interferon alfa, which is successful in 50% of children. [9] Another option is vincristine, which has lots of side-effects, but has a respons rate of 90%. Drug therapy is often used in shrinking the tumor and treating the coagulopathy. However, many of these kaposiform hemangioendotheliomas do not completely regress and remain as a much smaller asymptomatic tumor. However, KHE stil has a high mortality rate of 30%.
Although complete surgical removal with a large margin has the best reported outcome, it is usually not done because of the risk of bleeding, extensiveness, and the anatomic site of the lesion. [10]
Operative management may be possible for small or localized lesions. Removal of larger areas also may be indicated for symptomatic patients or for patients who have failed farmacotherapy. Resection is not required for lesions that are not causing functional problems, because KHE is benign and because resection could cause deformity.
lobular capillary hemangioma, is a small benign vascular tumor which primarily involves the skin (88.2%) and mucous membranes.[5] Pyogenic granuloma appears as a red macule that grows rapidly, turns into a papule and eventually becomes pedunculated, being attached to a narrow stalk.[7] The average diameter of these lesions is 6.5 mm.[5] Although these lesions are small, they are often complicated by bleeding, crusting and ulceration. Microscopically, pyogenic granulomas are characterized by vascular proliferation amidst granulation tissue and chronic inflammatory infiltrate. [11]
Pyogenic granulomas are rarely congenital. It commonly develops in infants: 42.1% develops within the first 5 years of life.
capillary malformation
. Of all pyogenic granulomas, 62% is distributed on the head or neck, occuring mainly on the cheek and in the oral cavity. Lesions on the face may cause visible deformity.
Numerous treament methods have been described for pyogenic granuloma. Lesions which involve the
laser therapy
. Furthermore, thorough currettage and cauterization are often used for small lesions and full-thickness excision for larger lesion.
Vascular malformations
Vascular malformation is a collective term for different disorders of the
dysmorphogenesis). However, endothelial turnover is stable in these defects. Congenital vascular malformations are always present at birth, although they are not always visible. In contrast to vascular tumors, vascular malformations do not have a growth phase, nor an involution phase. Vascular malformations tend to grow proportionately with the child. [12]
Vascular malformations never regress, but persist throughout life.
Vacular malformations can be divided in slow-flow vascular malformations, fast-flow vascular malformations and complex-combined vascular malformations. [13]
Venous malformation is a bluish lesion compressible on palpation, the masses enlarge with physical activity or a dependent position. The bluish lesion is caused by dilated venous channels. Venous malformations can be painful in the morning due to stasis and microthrombi within the veins. Venous malformations’ localization is usually in the head and neck. [12] Venous malformations are the most common vascular anomaly, they are 40% of all vascular malformations.[1] Venous malformation can be treated with sclerotherapy and surgical reduction[2]
Lymphatic malformation
Lymphatic malformation is a benign growth of the lymphatic system. [14] They result from a blockage or defect of the lymphatic vessels as they are forming. 28% of all vascular malformations consists of lymphatic malformations. [1] Lymphatic malformations can be treated with sclerotherapy and surgical reduction[2]
Fast flow vascular malformtations
All fast flow malformations are malformations with an arterial type in it. Contain bout 14% of the vascular malformations. [1]
Arterial malformation
Arteriovenous fistula (AVF) : a lesion with a direct transition in fistulas between an artery and a vein. [2]
Arteriovenous malformation: a lesion with a direct connection between an artery and a vein, without an intervening cappillary bed, but with an interposed nidus of dysplastic vascular channels in between. [12]
Combined-complex vascular malformations
a combination of various vascular malformations. They are complex because they’re a combination of two different types of vessels.
CVM: capillary venous malformation
CLM: capillary lymphatic malformation
LVM: lymphatic venous malformation
CLVM: capillary lymphatic venous malformation. CLVM is associated with
