Hemangiomas are formed by the proliferation of vascular networks during the embryonic period. In the early embryonic period, the primitive vessels are tubular masses composed solely of endothelial cells that form a dense network in the mesenchyme. Later, as organs and tissues develop, the primitive vascular network gradually differentiates into a number of vascular tufts that are connected to each other. Depending on the flow and pressure within the primitive vessels, supply vessels (arteries) or drainage vessels (veins) are formed. The vascular network of the body surface and subcutaneous tissues is prone to irregular localized hyperplasia, which later becomes a hemangioma. In 1982, Mulliken proposed two types of hemangiomas based on their physiological characteristics: (1) hemangioma: the tumor is an embryonic benign vascular tumor with biological characteristics of growth by vascular endothelial cell proliferation. (2) Vascular malformations: abnormal expansion and traffic of capillaries or arteries or veins with normal intracutaneous cellular structure and biological characteristics. Generally, hemangiomas have the possibility of natural regression. 70%~80% of strawberry capillary hemangiomas and 5%~50% of capillary spongy mixed tumors will regress on their own within l~3 years after birth. The process first stops increasing in size and then shows a lightening of the central part of the tumor, followed by the appearance of a longitudinal and horizontal white texture, which gradually expands to the entire lesion, and finally the hemangioma completely atrophies and disappears without leaving a trace. The histologic regression of pediatric hemangiomas is proportional to age. strawberry hemangiomas regress at an earlier age than spongy and mixed types, which sometimes fail to regress. vascular malformations of the Mulliken typing, i.e., orange-red spots, wine-colored spots, and other intradermal capillary hemangiomas, do not regress for life. Section I. Capillary hemangioma Neonatal nevus (neonatal staining), also known as epidermal capillary hemangioma, is a light red or light blue epidermal discoloration, usually located in the posterior midline of the head, neck or sacral area, also seen in the skin between the eyebrows or chest and abdomen, is present at birth and usually disappears gradually within a few months on its own, usually without treatment. There are three clinical variants of intradermal capillary hemangiomas, which are often present at birth. All are characterized by the presence of mature endothelial cell tissue-type capillaries in the dermis. The color of hemangiomas ranges from pale red to pale purple basket and are mostly located within areas of sensory nerve distribution, such as the trigeminal nerve in the face. I. Orange-red spots or salmon patches (salmon patch) are characterized by an orange-red to rust-colored patch that is flat and not higher than the skin surface. Most of them are on the forehead, upper eyelids, around the nostrils, occipital area or back of the neck. It is present at birth, varies in size, and may fade temporarily with finger pressure. It differs from neonatal nevus in that it is within the dermis and does not disappear spontaneously. No matter surface drying method, freezing method and friction method or x-ray irradiation, all of them are ineffective. The need for treatment should depend on the location and size of the lesion, but generally no treatment is needed. If necessary, cosmetics can be used to disguise it, and occasionally excision, wound suture or skin implant can be performed. Wine stain is darker than salmon stain and is dark purple in color. The capillary lesions are located in the dermis, but also involve the superficial subepidermal layer, and therefore can produce bleeding papules. They are present at birth and rarely extend, but often appear as jagged punctate hyperkeratotic lesions on the skin surface, while eczema sometimes occurs. Wine stains, especially on the face, cause psychological troubles to patients because of their aesthetic impact. It must be noted that facial wine stains, may be accompanied by intracranial hemangioma, known as Sturge-weber syndrome. In terms of treatment, rubbing method was used in the past, but the effect was not good. Medical tattoo method was widely used by Brown, Cannon, etc., which has a masking effect on the wine discoloration on the extremities and chest and back. In recent years, foreign plastic surgery has applied surgical excision treatment to wine spots on the face quite often, using extensive subcutaneous submarine relaxation and normal skin grafting. In most cases, staged surgery is required. When wine spots occupy a large area of the face, skin grafting should be performed on the area with “blush” reaction, such as the skin behind the neck, so that it can be integrated with the facial skin, but of course, it should be minimized and the trauma edge should be in a more concealed area. According to Edgerton’s report, the results are extremely satisfactory. He believes that children should be operated after reaching the age of 5 to avoid psychological effects. Spider naevus, or stellate angioma, is characterized by many radially expanded intracutaneous capillaries from a small subcutaneous central artery, which resembles a spider. The central point of the lesion is slightly elevated, usually as small as the eye of a needle, with a maximum diameter of only 2-3 mm, while the length of the surrounding radial vessels can be 0.5~lcm. The color of the nevus is mostly bright red, most often seen on the face, arms, hands and upper part of the trunk, and rarely occurs below the umbilicus. The number of spider nevi can be large, and they often appear in children aged 3 to 4 years. The clinical importance of this disease is that it can be complicated by bleeding, which is uncommon in childhood and increases significantly in adulthood. In terms of etiology, congenital spider nevi in children should be distinguished from spider nevi in cirrhosis and hepatitis disease, which may be associated with estrogenic changes. If the spider nevus does not fade naturally, good results can be obtained by cauterizing the central part of the nevus with a red needle under magnification to embolize the nutrient vessels. Strawberry hemangioma, also known as juvenile capillary hemangioma, differs from intra-dermal capillary hemangioma in that it has the characteristic of disappearing completely or partially on its own. The disease is very common, with an incidence of l/100 in newborns, and is usually found within the first few days of life, or as a very small erythematous spot within a few weeks of birth, and then gradually increases in size, often rising above the skin surface, bright red, and showing many lobules, hence the name strawberry. This type of hemangioma is usually found on the face, scalp and neck, but also on the trunk and extremities. They vary in size from a few millimeters to 2-4 cm in diameter and do not change significantly in color or size when examined with finger pressure. Occasionally, the surface of the hemangioma may ulcerate and bleed. Strawberry hemangiomas usually fade gradually between the ages of 1 and 4 years, and the process of fading can continue after the age of 4 years, but progresses very slowly and rarely disappears completely beyond this age. Therefore, every effort should be made to persuade parents to be patient and allow the tumor to resolve naturally at an early age. In cases of repeated injury, ulceration, secondary infection, and possible permanent scarring, surgical excision should be performed. For resection of facial hemangioma, special care should be taken to ensure that the incision scar is not significant and does not affect the eyes, face and corners of the mouth. CO2 snow or liquid nitrogen cryotherapy can also be used for strawberry hemangioma. Liquid nitrogen cryotherapy machine is equipped with various shapes and sizes of freezing heads, and the low temperature is -170~190℃, which only needs to contact with the hemangioma for l~2min. for wide range, the treatment can be performed in stages and pieces. For those with irregular shape, liquid nitrogen spray method can be used. Although cryotherapy can cure capillary hemangioma, the disadvantage is that it often leaves significant scars. Sclerotherapy, mostly using 5% sodium cod liver oil solution, requires multiple injections at different points, which is protracted and has the same risk of ulceration or scar formation as cryotherapy, and has been abandoned. Low-voltage, short-distance radiation therapy, although there is a – certain effect, but in children should be particularly cautious, because it can cause a series of complications, such as ulcers and scars. Sometimes the side effects of X-rays appear years later, or even tens of afternoons later, when local deformities occur and skin cancer can grow. Hormonal therapy can be indicated for giant crawling hemangiomas and can stop the continued growth of the hemangioma, which will be described later along with mixed hemangiomas. Section II. Capillary cavernous hemangioma and cavernous hemangioma Capillary cavernous hemangioma (capillary cavernous hemangioma), also known as mixed hemangioma, is usually present at birth and initially resembles strawberry hemangioma, but soon expands beyond the range of cutaneous hemangioma and invades deep dermal and subcutaneous tissues. Capillary cavernous hemangioma mainly occurs in the face and neck, but can also be seen in other parts of the body. The tumor can reach a large size. The growth process is similar to that of strawberry capillary hemangioma, with rapid growth and great invasiveness in the first 6 months. The tumor is often irregular in shape, bluish-red in color and prone to ulceration, bleeding, infection, necrosis and scar formation. In addition, this tumor can cause a series of secondary problems such as blocked nostrils, partially obscured eyes and ears, swollen lips, etc., and thus dysfunction in breathing, eating, seeing, hearing, etc. Although many capillary cavernous hemangiomas have rapid development at the initial stage, most of them can partially recede naturally and will not atrophy completely, and its degeneration process is far slower than that of strawberry hemangioma, so active treatment measures should be taken. The surgical excision of capillary cavernous hemangioma is often hesitant because it also brings destruction and disfigurement. (A) Hormone therapy In 1967, zarem and Edgerton first reported the treatment of pediatric giant mixed hemangioma with a large number of corticosteroids, and achieved remarkable results in a short period of time. 1. Shock phase Oral prednisone 40mg every other day (6~7kg weight children) for 7 times, then 20mg every 2 days for 2 weeks or 7 times, followed by 10mg every 2 days for 2 weeks or 7 times, and 5mg every 2 days for 2 weeks or 7 times. 2.Maintenance phase Prednisone 2.5mg every 2 days orally for 60-90 days. In about 3()% of hemangiomas, when the dose is reduced to 10 or 5mg every 2 days, the phenomenon of hemangioma enlargement can appear again, then the dose should be increased to 15-20mg every 2 days for 2 weeks, and then enter into the dose of maintenance phase. The whole course of treatment is 3 months. 3.Local hormone therapy Adopt hormone (dexamethasone or other regular-acting reserve class in alcohol) 5~10mg, add 1% procaine 2-4ml and pure ethanol 0.1~0.5m1, can be increased to 1m1, inject into tumor by point partition, generally once a week, 5 times for a course of treatment. Most of the tumors become mechanized and shrink, or even disappear completely. It has been clinically proven that long-term application of hormones, such as in renal disease, leukemia, and after organ transplantation, does not cause severe hypertension, sodium and water retention, or other complications. Treatment with oral hormones. Hemangioma does not cause significant physiological disorders. Inhibition of growth and development had been one of the biggest concerns. Because high doses of corticosteroids can lead to weight loss and negative nitrogen balance, this is not a sign reflecting permanent inhibition of tissue growth. Many studies in pediatric and immature animals have shown that following cessation of exogenous corticosteroid therapy, growth can be accelerated beyond that of the control group that received the hormone at the end. This “catch-up” growth phenomenon is accompanied by a corresponding acceleration of cell proliferation. The use of alternate day oral dosing is preferable to daily oral dosing to minimize the effects of hyperadrenocorticism. In conclusion, there have been no cases of permanent inhibition of growth in children treated with hormonal therapy, and Edgerton et al. have followed a group of children treated with high doses of hemangiomas for more than 10 years and have seen no clinically significant abnormalities. The treatment of hemangiomas with hormones has proven to be an effective therapy in the last 20 years, but the mechanism of the therapy has not been fully understood to date, as observed by Zweifech et al. in experimental animal studies: rats receiving corticosteroids had increased vascular sensitivity to vasoactive amines compared to control rats. It has been shown that intramuscular injection of cortisone acetate leads to constriction of the small arteries of the anterior capillary sphincter, which may be an important mechanism for the inhibition of rapidly proliferating vascular tissue in hemangiomas. Even so, no direct effects of corticosteroids on intratumoral vascular structures have been reported to date. Another mechanism by which corticosteroids may be effective is their effect on cellular metabolism; Munck demonstrated in animal studies that corticosteroids inhibit glucose uptake by skin and fat, which not only leads to changes in basal metabolism but also affects protein synthesis; Loeb demonstrated that protein synthesis in the liver was significantly inhibited in young animals treated with cortisone. In the actual clinical treatment of a rapidly growing mixed or cavernous hemangioma, the family’s apprehension is understandable. The application of hormones to stop the growth and reduce the size constantly fills both the child, the family and the medical staff with confidence, and then the treatment plan is developed according to the specific circumstances of each case. Antibiotics are applied for those with infections, and facial scars from ulcers usually require plastic surgery. Hemangiomas elsewhere on the body surface that have shrunk to a resectable extent are treated with excisional wood and, if necessary, skin grafting. (ii) Cryotherapy and other therapies Cryotherapy and sclerotherapy are not suitable for huge capillary cavernous hemangiomas on the face. Radiation (X-rays, radium, radionuclides, etc.) has been successfully reported in the treatment of hemangioma, but this therapy has been almost completely abandoned in recent years, especially dangerous for children, because it can cause early ossification of epiphysis and stop growth of limb, destroy eye risk and crystal, and most serious is carcinogenic effect. In recent years, some people investigated in 4000 cases with low dose [3~6Gy) radiation treatment of hemangioma, there are 3 cases died of malignant tumor, and this is only found in 10 years of follow-up, there are potential dangers later. Cavernous hemangioma is formed by a large number of blood-filled cavities or sinuses, the walls of which are lined with a fine running layer of endothelium. The cavities are separated by a fibrous connective tissue barrier. Spongiform hemangioma differs from capillary spongiform mixed hemangioma in that it has no or very little capillary tissue on the surface, and the tumor grows mostly in the subcutaneous tissue and often penetrates deep into the muscle. Spongiform hemangioma also has the tendency to grow and can reach a large volume, severely damaging the surrounding tissues, deforming the limbs and destroying the appearance. However, some tumors are fixed in size, have an intact skin envelope, and are easily separated from the surrounding tissues. However, some tumors are fixed in size and have complete skin envelope, so they can be easily separated from surrounding tissues. Almost any part of the body can have cavernous hemangioma, especially the extremities and trunk, and also the neck and face. Most of the hemangiomas on the bones belong to this category, and sometimes spongiform hemangiomas also grow in the liver, spleen, gastrointestinal tract and other internal organs. Spongiform hemangiomas can be normal or dark blue in color depending on the depth of the hemangioma and the color of the skin on the hemangioma. On palpation, it is a soft mass that, as the name implies, feels like a sponge or dough. The shape can be more regular and flat or a bumpy mass (Figure 19-4). Application of pressure may cause the mass to temporarily sink. The mass may cause heaviness and soreness in the affected area, especially after movement of the affected area and, in the case of the lower extremity, after prolonged standing. Spongiform hemangiomas, if limited, may shrink slowly on their own, but will not disappear completely. Diffusely large cavernous hemangioma cannot subside automatically. Regarding the treatment of cavernous hemangioma, although sclerotherapy, radiotherapy and electrocoagulation have been used in the past, they cannot completely treat the tumor and there is a risk of complications, so they are rarely used. At present, the effective treatment for cavernous hemangioma is mainly surgery and hormone therapy. In addition, according to the report of Xi’an Medical College in China, local injection with urea has good efficacy. Hormonal therapy has been described in detail in the treatment of capillary cavernous hemangioma, and surgical therapy and urea injection therapy are discussed below. (Surgical excision of limited hemangioma is the most commonly used method. It is generally safe and effective in about 3/4 of cases. The risk of surgical resection is massive bleeding, and adequate blood must be prepared for intraoperative input, and various methods of hemostasis must also be planned. Various tourniquets can be considered for hemangiomas of the extremities. Adequate visualization around the hemangioma is one of the major steps in preventing bleeding so that the major vessels entering the hemangioma can be more easily located and ligated. Buried sutures around the tumor are one of the reliable methods of hemostasis, and sometimes this method must be used to stop bleeding. For hemangiomas that penetrate deep into the muscle tissue, surgery is often difficult and the affected muscle must be removed separately, but the possibility of motor dysfunction after surgery must also be considered. In some hemangiomas, the skin cannot be peeled off from the overlying skin, and if too much skin is removed, a skin grafting procedure should be performed at the same time. The residual hemangioma tissue is prone to disorders of the coagulation mechanism, which may cause diffuse intravascular coagulation (DIC). If hemangioma imaging can be done before surgery to identify the nutritional branches of the hemangioma, the supply vessels can be ligated at the proximal end of the hemangioma to reduce intraoperative bleeding and facilitate total resection. (B) Urea injection method To treat large cavernous hemangioma with urea, refined medical urea (98% or more) is diluted to 30%~40% concentration and injected into the tumor. In case of hemangioma of the extremities, an intravenous tourniquet can be placed first, and 5m1 urea can be injected into the ends or edges of the tumor by adding 2% procaine to 1m1, and then pressure bandage can be applied to prevent the liquid from spilling out of the needle hole. The injection was given once a day for 10 to 20 times. The mechanism of efficacy may be due to the fact that parotoxin is a hypertonic fluid, which is injected into the hemangioma and dehydrates the cells in the hemangioma, followed by metabolic disorders, destroying the endothelial cells of the blood vessels and causing aseptic necrosis, followed by proliferation of fibrous connective tissue and fibrosis of the hemangioma, resulting in atrophy. The treatment of giant cavernous hemangioma can also be treated by selective arterial cannulation with urea injection, which is reported by Xi’an Medical University with good effect. In 1940, Kasabach and Merritt described a giant cavernous hemangioma of the calf in a 2-month-old infant, and after biopsy surgery, bruising and persistent bleeding, due to the development of DIC and thrombocytopenia. Winston reported 2 cases out of 300 cases. Giant hemangiomas can be spontaneous with mild DIC, but are more often promoted by surgery and may be head and neck, extremity, or visceral hemangiomas (e.g., liver, spleen, etc.). (I) The mechanism of K-M syndrome is due to slow blood flow in hemangioma, large amount of blood is retained in the tumor, injury, surgery further damages the intima in hemangioma, coagulation area increases and coagulation mass in circulating blood is released, so there is thrombus formation in the whole microcirculatory system, large amount of platelets and fibrinogen and coagulation factors II, V, VII and Ⅻ are consumed, and the result is massive bleeding occurs. (B) Clinical manifestations of K-M syndrome Clinical manifestations of K-M syndrome are common in infants under 1 year of age, especially at 6 months of age. Before the onset of the disease, the tumor suddenly and rapidly increases in size and spreads to the surrounding area, with a purple surface, hard and painful to the touch, resembling a soft tissue infection, with localized bleeding spots and/or bruises, which may be accompanied by generalized bruises until the manifestation of DIC. Blood tests show thrombocytopenia, often around 60×10∧9/L, low hemoglobin, and severe anemia. (C) Diagnosis of K-M syndrome The diagnosis can be highly suspected based on its clinical manifestations, and further laboratory tests are promptly performed to determine the diagnosis. Platelet count, prothrombin time and fibrinogen assay are used as screening items for DIC. If all three are abnormal, DIC can be identified, and thrombocytopenia is particularly important. When thrombocytopenia occurs in occult hemangiomas, such as retroperitoneal, hepatic, splenic and skeletal hemangiomas, because the mass is difficult to detect, every effort should be made to exclude or confirm the presence of the hemangioma, in addition to considering primary thrombocytopenic violet epilepsy. (D) Treatment of K-M syndrome 1. Systemic hormone therapy has considerable efficacy in cavernous and mixed hemangiomas, and is the treatment of choice when K-M syndrome occurs, using shock prescriptions, most of which can stop this complication. Intravenous administration of hydrocortisone 4-5 mg/kg/day or dexamethasone 1-2 mg/kg/day in 2-3 doses for 7-14 days has also been advocated. If the condition is stable, oral prednisone 2-3mg/kg daily for 2-3 months will not only control the K-M syndrome, but also shrink the hemangioma to the extent that it can be surgically removed. A comprehensive coagulation mechanism examination should be done before surgery to prevent uncontrollable bleeding. Surgical resection is a complete treatment, but must be performed in the absence of systemic DIC manifestations. After surgery, platelets rapidly rebound and coagulation abnormalities disappear. 2.Radiotherapy When the effect of hormone treatment is not significant and surgical removal of hemangioma is not possible, small-dose deep x-ray local irradiation can be used. Use 140~180kV, 0.25~0.5mm copper plus 1.0omm aluminum filter, irradiation field 1~1.5Gy each time, usually 6~10 times as a course of treatment. The irradiation should be stopped when the tumor starts to shrink and harden and platelets rise. When DIC occurs, heparin must be used, the usage is 0.5~1mg/kg each time, once every 6h or 8h, for 3~5 days. If the clotting time exceeds 25 min, heparin should be temporarily discontinued to avoid overdose of heparin. In anticoagulant therapy, dextran 40 is applied, regardless of the presence of DIC, at a dose of 10 ml/kg per dose, every 6h or 8h. Pansentine at 0.4mg/kg every 8h can also be used. K-M syndrome is a serious complication of hemangioma, with a mortality rate of 30% in the past. The mortality rate can be reduced to a minimum with regular and aggressive treatment. In summary, before surgery for giant hemangioma, thorough preparations must be made, with preoperative hematological examinations and postoperative control examinations, and, if necessary, the assistance of a hematologist. In this way, surgical removal of all hemangiomas reduces the likelihood of postoperative changes in coagulation mechanisms. Cirsoid angioma (Racemose) is much less common than capillary angioma and cavernous angioma, accounting for only 1.5% of cases. It is a hemangioma that contains small arterial and venous anastomoses, but differs from the extensive multiple aneurysms of the extremities. More common are scalp or limb trapezius hemangiomas. The skin is often flushed and the pulsation and peristalsis of tortuous blood vessels are faintly visible under the skin, resembling an aggregated mass of roundworms. On auscultation, a murmur may be heard, and the hand may feel pulsation and feel the soft, enlarged blood vessels in the form of cords, with an increased local temperature. In trapezius hemangioma confined to the ends of the extremities, several irregularly associated cystic masses may be seen on the fingers or toes, palms or soles of the feet, visible through the skin as a purplish-blue color, soft in texture and temporarily shrinking when compressed. These manifestations are easily misdiagnosed as cavernous hemangioma, but like subscalp trabecular hemangioma, they can be felt as mild pulsations and tremors, whereas cavernous hemangioma has no such symptoms. Cranial hemangiomas can be more painful because the subcutaneous nerves can intertwine with the vessels, pulling on the nerves when the vessels pulsate and causing tinnitus. In infants, subcutaneous trapezius hemangiomas tend to expand rapidly and can destroy the outer plate of the skull and invade the plate vein and connect to the intracranial venous sinus. These hemangiomas do not disappear automatically, so they should be treated early. Treatment of confined trapezius hemangioma is difficult with surgical excision. In the case of hand and foot, staged surgery can be performed if necessary. It is advisable to dissect the area around the hemangioma and ligate the blood vessels that enter the tumor. In the case of head hemangioma, sometimes a small artery penetrates through the skull plate and enters the tumor, which makes the surgery much more difficult. Depending on the circumstances, if the scalp cannot be preserved, a transfer flap may be required to cover the wound.