What are hemangiomas and vascular malformations?

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 (KLE) (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 consists of vascular endothelial cells that divide and proliferate vigorously and grows rapidly, with a bright red or purplish color. In the late stage of regression, most of the children’s skin completely returns to normal, but in 20% to 40% of cases, skin laxity, pigmentation, capillary dilatation, fiber and fat deposits, yellow spots, and scar tissue are left behind. 50% of infants and children’s hemangiomas 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. The distribution of occurrence on the face is also non-random and seems to be related to the fusion line of embryonic development and the 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 3-4 months, and from June to October, although they are still growing, the growth rate slows down significantly and generally reaches its peak 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, mild 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 impairment of other organ functions, and occasionally congestive heart failure. 6.What is the treatment method for infant hemangioma? The most common treatment methods include clinical observation, surgery, hormones, topical drugs, laser, freezing, sclerotherapy, etc. The treatment methods for infant hemangioma are very diverse and should be chosen according to the specific conditions. 7.When is it appropriate to observe infant hemangioma clinically 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 explain to them that the effect of natural regression is better than the result of any other active treatment. Main indications: ? Infantile hemangiomas in the regressive or late regressive phase; ? Infantile hemangiomas in the proliferative phase that are small, in areas less prone to complications, and with a slow growth rate; ? Rapidly involuting congenital hemangioma (RICH), which was first described by Boone et al. RICH typically presents as a purplish-blue, bulging tumor with distended veins; or as a bulging gray tumor with surface capillary dilatation and a white ring of surrounding vasoconstriction; or as a relatively flat, 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. ; ? Another type of so-called benign neonatal hemangiomatosis (BNEH) also presents with multiple hemangiomas throughout the body, but not in combination with visceral hemangiomas. Neonatal hemangiomatosis mostly regresses by 2 years of age; ? Infantile hemangioma whose parents have some knowledge of infantile hemangioma and who have a very high likelihood of regression with very high treatment effect. 8.What kind of cases should be treated surgically? The possibility that the hemangioma can regress and the child’s psychological ability to tolerate the surgical scar should be fully considered before surgery. Main indications: ? The tumor is located on the trunk or extremities, and the post-operative scar is relatively hidden, and the family has the psychological ability to tolerate the post-operative scar; ? Infantile hemangiomas that are likely to produce or have produced complications ? Hemangiomas that are limited, outward growing, with a tip, and may have skin changes after receding. Eyelid hemangioma with ulcer formation, bleeding, ineffective to other non-surgical treatments and ineffective to medication. ? Non-involuting congenital hemangioma (NICH), which is fully formed at birth and is more common in boys than girls, typically has a round or oval shape, slightly protrudes from the skin, is pale centrally or peripherally, and has dilated capillaries on the 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 area hemangioma can be staged surgery 9.What are the indications for hormone therapy? What is the dosage of the drug? Does it produce toxic side effects afterwards? Since 1960, systemic application of hormones has been the main method of 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 stopping the drug. Main indications: ? periorbital hemangioma; ? Airway hemangiomas are usually located under the voice box and may be combined with cutaneous hemangiomas. 60% of large stage hemangiomas involving the face and neck are complicated by airway hemangiomas; ? Neonatal multiple hemangiomas are rare clinically and are characterized by multiple cutaneous hemangiomas associated with visceral hemangiomas. The clinical presentation is a pinpoint to soybean-sized, bright red, multiple hemangioma that may have formed at birth or in the weeks following birth; ? Hepatic hemangiomas may present with increased arteriovenous flow to the liver (by ultrasound), 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 afterwards? The clinical dosage of the drug depends on the location, 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 hemangioma affects the patient’s appearance and is not suitable for surgical excision and will seriously affect the appearance after surgical excision; ? Hemangiomas with endothelial characteristics of infantile embryonic blood vessels; ? Deep hemangiomas invade the deep dermis and subcutaneous tissues, the epidermis remains normal thickness, the surface of the tumor is light blue or normal skin color, the surface of capillary dilatation (Telangiectases) is visible, and the surrounding reflux veins are visible. ? Hemangioma that other methods of treatment are not effective. 11.What are the indications for imiquimod treatment? Are there any toxic side effects after treatment? 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. The Nd-YAG laser is also effective in treating hemangiomas, but there is a risk of ulceration and scarring.