1.What are hemangioma and vascular malformation? This has always been a vague concept, and most doctors refer to hemangioma and vascular malformation collectively as hemangioma. In fact, hemangioma and vascular malformation are two completely different diseases, and their pathogenesis, clinical manifestations, prognosis and treatment methods are completely different. Hemangioma is a tumor, while vascular malformation is a congenital malformation. Hemangiomas include: Hemangioma of infancy, Pyogenic granuloma, Lobular capillary hemangioma, Rapidly involuting congenital hemangioma (RICH). hemangioma (RICH), Non-involuting congenital hemangioma (NICH), Tufted angioma (Angioblastoma of Nakagawa), Kaposiform hemangioendothelioma (Kaposiform hemangioendothelioma, Congenital eccrine angiomatous hamartoma, Spindle-cell hemangioendothelioma The vascular malformation includes capillary malformation, venous malformation, lymphatic malformation, arteriovenous malformation and mixed vascular malformation, etc. 2.Can infant hemangioma fade away on its own? When will it recede? Can it return to normal skin after receding? Infantile hemangiomas often appear in the neonatal period, but subcutaneous or visceral hemangiomas are usually detected at 2-3 months of age, while some develop fully at birth. The natural progression of infantile hemangiomas is divided into a proliferative phase, a receding phase, and a late receding phase. The proliferative phase begins a few weeks after birth and lasts for 4-10 months. The tumor tissue is composed of hyperplastic vascular endothelial cells, which grow rapidly and are bright red or purplish in color. The tumor cells begin to decrease in activity, flatten in shape, and darken in color for 5 to 6 years; in the late receding stage, the skin of most children returns to normal, but in 20% to 40% of cases, skin laxity, pigmentation, capillary dilation, fiber and fat deposits, yellow spots, and scar tissue remain. 50% of infants and children with hemangioma regress by the age of 5 and 90% by the age of 9. 3.Where do hemangiomas in infants and children usually occur? Are there any complications afterwards? Although infantile hemangiomas can occur anywhere in the body, about 60% occur on the head and neck. The trunk (25%) and extremities (15%) are less commonly affected, and the internal organs are rarely involved. Occurrence in the face is also non-random in distribution and appears to be related to fusion lines of embryonic development and subunits of facial development. The anatomic location of infantile hemangiomas is related to their occurrence of comorbidities to determine whether they require special attention for observation. Site and morphology of infantile hemangiomas and corresponding possible comorbidities Site and morphology Comorbidities Larger facial stage infantile hemangiomas PHACE syndrome Nasal tip, ear, and large infantile hemangiomas (especially protruding epidermis) Permanent scarring and disfigurement Periorbital and retrobulbar ocular axial obstruction, amblyopia, glaucoma, and lacrimal duct obstruction Periorbital, lip Ulceration, deformity Lumbosacral Spinal cord Embolism and genitourinary malformations Perianal, axillary and cervical ulcers Multiple visceral involvement (especially liver and gastrointestinal tract) 4. What is the natural course of hemangioma? What are the consequences? Superficial infantile hemangiomas grow very rapidly in the first 6 months of life, especially in the first 3 to 4 months, and from June to October, they still grow but at a significantly slower rate, and generally reach their peak growth in September to December. However, it is still difficult to predict the trend of an individual child because some infantile hemangiomas continue to develop between the ages of 1 and 2 years, especially some “mixed” infantile hemangiomas. Deep infantile hemangiomas are generally found later and have longer growth cycles than superficial infantile hemangiomas. The typical superficial infantile hemangioma begins to fade around the age of 1 year, when the central part of the tumor changes from bright red to dark red, gradually expanding to the periphery and eventually turning grayish white. Sometimes, although the central part of the superficial area begins to recede, the deeper part of the tumor or the edges are still in a proliferative state. When the tumor recedes, it is replaced by some fibrous tissue. When superficial infantile hemangiomas recede, the local skin becomes lax. When deep infantile hemangiomas recede, the local color becomes lighter, the temperature decreases, and the tissue becomes flabby. They recede about 10% per year, about 50% by age 5, about 70% by age 7, and about 90% by age 9. The regression of infantile hemangiomas does not mean that the skin is completely normalized, and about 20% to 50% of infantile hemangiomas have residual skin changes after regression. The characteristic changes are localized capillary dilation, skin wrinkling, slight pigmentation, and minor structural changes; in severe cases, skin laxity, scar formation, and fibrofatty deformation result in significant local structural changes. Most small infantile hemangiomas do not cause cosmetic changes, but some specific areas, such as between the eyebrows, the tip of the nose, and the ears, may have cosmetic changes. Larger areas of infantile hemangioma have a potential risk of scar formation after regression, especially superficial infantile hemangiomas. Ulcer formation can lead to scar formation of varying degrees, the severity of which is determined by factors such as the size and depth of the tumor and the thickness of the skin invaded by the tumor itself. 5. What are the most common complications of hemangioma? Most complications occur within the first 6 months of life, i.e. during the proliferation period. Common complications include ulceration, bleeding, infection and other organ damage, and occasionally, congestive heart failure. 6.What is the treatment method for infant hemangioma? The most common treatment methods include clinical observation, oral medication, surgery, hormone, topical medication, laser, freezing, sclerosing agent, etc. The treatment methods for infant hemangioma are very diverse and should be chosen according to the specific conditions. 7.What kind of cases of infantile hemangioma are suitable for clinical observation and no treatment is needed? Observe, wait and follow up regularly, explain the natural course of hemangioma to family members, preferably show them photos of other patients to further explain the process of hemangioma regression, and make it clear to them that natural regression is superior to any other active treatment. The main indications are: infantile hemangiomas in the regressive or late regressive phase; small, proliferative infantile hemangiomas in areas less prone to complications and with a slow growth rate; rapidly regressing congenital hemangioma (RICH), which was first described by Boone et al. Boon et al. first described RICH. The typical presentation is a purplish-blue, elevated tumor with distended veins; or a raised gray tumor with surface capillary dilatation and a white ring of surrounding vasoconstriction; or a flatter, infiltrative growth with purplish-blue surface skin.RICH varies in size and in most cases is only a few centimeters in diameter. The surface skin temperature is slightly elevated, and murmurs and palpable tremors can occasionally be heard. Unlike infantile hemangiomas, RICH does not have a rapid growth phase after birth, but rather regresses rapidly, completely resolving within 12-18 months. There is another type of so-called benign neonatal hemangiomatosis that also presents with multiple hemangiomas throughout the body, but not in combination with visceral hemangiomas. Most neonatal hemangiomas can be regressed before the age of 2 years; parents have certain knowledge of infant hemangioma and have a very high possibility of regressing infant hemangioma with very high treatment effect. 8.What kind of cases should be treated surgically? The possibility of hemangioma regression and the child’s psychological ability to tolerate the surgical scar should be fully considered before surgery. The main indications are: tumors located on the trunk and extremities, the scar is hidden after surgery, and the family has the psychological ability to tolerate the scar after surgery; infantile hemangiomas that are likely to produce or have produced complications hemangiomas that are limited, outward-growing, tipped, and may have skin changes after regression; eyelid hemangiomas that have ulcer formation, bleeding, and are ineffective to other non-surgical treatments and to medication. Non-involuting congenital hemangioma (NICH), which is fully formed at birth, is more common in boys than girls and typically presents with a round or oval shape, slightly protruding from the skin, a pale central or peripheral color, and a dilated capillary surface. The diameter varies from a few to a dozen centimeters, with an average size of 5M. The skin temperature was slightly elevated, and ultrasound Doppler was able to detect rapid arterial blood flow. The pathology is characterized by a lobular distribution of cells with stellate vessels surrounding the central vessel, with predominantly abnormally developed veins between the lobules, and negative expression of GLUT-1 immunohistochemistry. nich never regresses. For large hemangiomas, staged surgery is possible. 9. What are the indications for hormone therapy? What is the dosage of the drug? Are there any toxic side effects afterwards? Since 1960, systemic application of hormones has been the main treatment for hemangioma, and the mechanism of treatment is not yet understood. The usual treatment is a single daily dose of prednisolone or prednisone (2-4 mg/kg), which is usually effective for 2-4 weeks. The duration of treatment varies from several weeks to several months, depending on the age of the child, the indications for treatment, and the characteristics of the growth. Hormone therapy is most effective within the first 6 months of life, during the rapid growth period of hemangioma, but in a few patients, it is also effective in infancy. If the drug is stopped midway, it will inevitably lead to a rebound of the hemangioma. Despite some side effects, most children grow well and can catch up with children of the same age after discontinuation of the drug. The main indications are: periorbital hemangioma; airway hemangioma is usually located under the voice box and may be combined with cutaneous hemangioma. 60% of large stage hemangiomas involving the face and neck are complicated by airway hemangioma; neonatal multiple hemangioma is rare and is characterized by multiple cutaneous hemangiomas with visceral hemangiomas. The clinical manifestations are pinpoint to soybean-sized, bright red, multiple hemangiomas that may have formed at birth or in the weeks after birth; hepatic hemangiomas may present with increased arteriovenous flow in the liver (by ultrasonography), cardiac hypertrophy, and tachycardia before causing congestive heart failure. 10.What are the indications for Pingyangmycin treatment? What is the dosage of the drug? Does it produce toxic side effects? The clinical dosage of the drug depends on the site, type and size of the tumor, but usually the dosage of the drug is 0.5~1mg/cm3 of Pingyangmycin, and the injected dose is usually not more than 8mg each time, and not more than 16mg for large lesions. Toxic side effects are very rare. The main clinical indications include: facial hemangiomas that affect the patient’s appearance and are not suitable for surgical excision or will seriously affect the appearance after surgical excision; hemangiomas with characteristics of infantile embryonic vascular endothelium; Deep hemangiomas that invade the deep dermis and subcutaneous tissues, but the epidermis maintains normal thickness, the surface of the tumor is light blue or normal skin color, and capillaries are visible on the surface The surface of the tumor is light blue or normal in color, and the surface of the tumor is covered with capillaries (Telangiectases) and the surrounding reflux veins. Hemangioma that is not treated by other methods. 11.What are the indications for imiquimod treatment? Does it cause toxic side effects? Imiquimod is an immunomodulatory agent. In recent years, there are scattered reports in the literature on the efficacy of topical imiquimod in the treatment of infantile hemangiomas, mainly for superficial hemangiomas (so-called strawberry hemangiomas) that only invade the dermis and appear as lobulated, bright red erythema. Toxic side effects are very rare. 12.What are the indications for laser treatment? Are there any toxic side effects? Several types of lasers have been used for the treatment of hemangiomas. Since 1990, pulsed fuel laser (PDL) has been used in clinical practice. Most of the literature reports that PDL is suitable for the treatment of superficial hemangiomas and residual erythema after the hemangioma has faded. Treatment is given once every 2-3 weeks until the hemangioma is controlled. PDL is not suitable for the treatment of deep hemangiomas because of its limited penetration ability and inability to reach deeper areas. Nd-YAG laser is also effective for hemangioma, but it may cause ulceration and scarring. 13.What is septic granuloma and how to treat it? Pyogenic granuloma (Lobular capillary hemangioma) is also a common pediatric vascular tumor, second only to infantile hemangioma in terms of incidence. There is no age limit for the onset of the disease, but it is most often seen in children and is less common before the age of 1 year and can be easily misdiagnosed as infantile hemangioma. Pustular granulomas initially present as bright red papules several millimeters to 2M in size with intact epithelium, indicating that ulcers often form and may have a tip. It can occur anywhere with skin mucosa, and is more common on the head and neck. More infantile hemangiomas are rich in blood flow and bleed easily. A small number of patients have a history of local trauma. Purulent granulomas can be de novo or secondary to other vascular malformation lesions. It is not an infectious disease, nor is it granulation tissue, but rather a proliferative vascular tumor, which has been described as lobular capillary hemangioma. The treatment should be local injection of Pingyangmycin, surgery, electrocoagulation, freezing or laser depending on the location and size of the lesion. Larger purulent granulomas are prone to recurrence after treatment. Another rare postoperative complication is satellite-like multiple lesions. 14. What is a plexiform hemangioma and how is it treated? Tufted angioma (Angioblastoma of Nakagawa) is a rare vascular tumor that occurs in infancy or childhood in most cases and rarely develops congenitally. They are erythematous nodules with localized capillary hyperplasia in the dermis, with nodular, discontinuous, peripheral elevation, and glomeruloid changes. The typical pathology of plexiform hemangioma is characterized by tightly packed capillary plexuses that are spherically distributed in the dermis. The lesion is surrounded by fissured vascular spaces. Individual cases may develop Kasabach-Merritt syndrome. The natural course of plexiform hemangioma is unpredictable. Some patients can regress on their own, leaving only small skin lesions, while others cannot regress and may even progress further. Treatment: Surgical excision 15. What is Kasabach-Merritt syndrome and how is it treated? Kasabach-Merritt syndrome was first described in 1940 and was thought to be a complication of infantile hemangioma, but is actually a complication of another type of vascular tumor, especially KHE (Kaposiform hemangioendothelioma, KHE), which is prone to Kasabach-Merritt syndrome. Kasabach-Merritt syndrome can be present at birth, but in most cases it develops several months after birth and presents as a fast-growing soft tissue tumor with significant tenderness. The predominant clinical feature is a wasting coagulopathy with reduced platelets and prothrombin. Other hematologic abnormalities are D-dimer formation, abnormalities of prothrombin time, partial thromboplastin time, and hemolytic anemia. Kasabach-Merritt syndrome is easily confused with a wasting coagulopathy due to a large venous or mixed vascular malformation. Treatment of Kasabach-Merritt syndrome must be multidisciplinary, including pediatrics, hematology, dermatology, surgery, and interventional radiology. Dermatology, surgery and interventional radiology. Sclerotherapy is effective but hardly curative; hormone therapy alone is rarely effective in Kasabach-Merritt syndrome, but is often used as a first-line drug. Surgical treatment is an effective approach, but has a low success rate. Alpha-interferon is effective in some patients, but cannot be used as routine treatment. Vincristine 1-1.5 mg/m2 or 0.05-0.65 mg/kg has recently been reported to be a very effective approach, and treatment needs to be repeated after relapse. Although supportive therapies such as blood transfusion, fibrinogen transfusion or fresh plasma have some efficacy, they should be avoided as much as possible except when fatal bleeding occurs preoperatively, because the newly transfused platelets are quickly consumed by the tumor, further promoting its enlargement and worsening the disease instead.