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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 rapidly involuting congenital hemangioma, RICH, presents at birth as a solitary raised tumor with a central depression, scar, or 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. 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%).
The non-involuting congenital hemangioma, NICH, presents as a solitary, well-circumscribed reddish-pink to purple plaque with central telangiectasia and hypopigmented rim. 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%).
Surgical resection for congenital hemangiomas is rarely needed, because RICH undergoes postnatal regression and NICH is benign and often asymptomatic. Resection may be indicated to improve the appearance of the affected area, as long as the surgical scar is less noticeable than the lesion. Other indications are problematic ulcers with persistent bleeding or 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. 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. 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.
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. It occurs in 20% of low weight premature infants and 2.2 to 4.5 times more frequently in females. IH most commonly presents in the head and neck region (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 rapid 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 occurs 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. Involution may result in residual telangiestasis, pallor, atrophy, textural changes and sometimes fibrofatty residuum.
Since 90% of IH is small, localized and asymptomatic, treatment mainly consists of observation and awaiting until involution is complete. IH can be treated with 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 a number 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 aesthetic outcome. However, patients may require intervention during childhood, because 50% of IH leave residual fibrofatty tissue, redundant skin, or damaged structures after involution. Waiting until involution is completed, ensures that the least amount of fibro fatty residuum and excess skin is resected, giving the smallest possible scar. Another option for treatment in the pulsed-dye laser. After involution residual telangiectasias can be treated with laser therapy.