Hemangioma is a congenital benign tumor or vascular malformation, mostly seen in infants at birth or shortly after birth, which originates from residual embryonic angiogenic cells, most of which occur in facial skin, subcutaneous tissues, and oral mucous membranes, such as the tongue, lips, and the floor of the mouth, and a few of which occur in the jawbone or the deep tissues. Hemangiomas can generally be classified into capillary hemangioma, cavernous hemangioma and trapezoid hemangioma according to their clinical manifestations and histological features, among which capillary hemangioma and cavernous hemangioma are more common. Classification problems In 2002, at the National Symposium on Treatment and Research of Oral and Maxillofacial Hemangiomas held by the Oral and Maxillofacial Surgery Committee of the Chinese Stomatological Association, the delegates unanimously agreed that the concepts, classifications and nomenclature of hemangiomas and chorioangiomas should be clarified again. Classification of hemangiomas Infantile hemangiomas, congenital hemangiomas, Kaposiform hemangioendotheliomas, tufted hemangiomas, and vascular ectodermal tumors Classification of vascular malformations (1) Micro venous malformations: (Wine discoloration) (Including two types of midline micro venous malformations and micro venous malformations. (2) Venous malformations. (cavernous hemangioma) (3) Arteriovenous malformations. (trapezoidal hemangiomas or grapelike hemangiomas) (4) Lymphatic malformations: they are subdivided into two categories: microcystic and macrocystic. (Lymphangioma) (5) Mixed malformations: containing veno-lymphatic malformations and veno-microvenous malformations of 2 types. (Lymphangioma) Clinical features 1.Micro venous malformation is the common wine stain. It occurs on facial skin, often distributed along the trigeminal nerve, distinguishing from small spots to several centimeters, and large ones can extend to one side of the face or cross the midline to the opposite side. When the lesion is pressed with a finger, the color of the surface recedes; after the pressure is lifted, the blood immediately fills the lesion area again, restoring the original size and color. The so-called midline microvein malformation is mainly located in the midline area, the most common in the neck, followed by the frontal area, between the eyebrows, as well as the upper lip and other parts of the human body. Unlike wine stains, it can subside on its own. 2.Vein malformation (1)Formerly classified as cavernous hemangioma, it is composed of countless blood sinuses lined with endothelial cells. The size and shape of the blood sinuses vary, such as spongy structures. The blood in the sinus cavities coagulates into thrombi and can calcify into vein stones. (2) Venous malformations occur in the cheeks, neck, eyelids, lips, tongue or the floor of the mouth. The location varies in depth, if the location is deeper, the skin or mucous membrane color is normal; superficial lesions appear blue or purple. The borders are less clear, soft to the touch, can be compressed, and sometimes a vein stone can be found. When the head is in a low position, the lesion area is congested and enlarged; after restoring the normal position, the swelling is also reduced and restored to its original state, which is called a positive positional mobility test. (3) Venous malformations are often undetected at birth, and in some cases, the symptoms appear in early childhood or even in adulthood, and then the patient’s attention is drawn to them. (4) Venous malformations are usually asymptomatic when the size of the lesion is small. If it continues to develop and grow, it can cause deformity and dysfunction of face, lips and tongue. If secondary infection occurs, it can cause pain, swelling, surface skin or mucous membrane ulcers, and the risk of bleeding. 3.Arteriovenous malformation (1)It is called trapezoidal hemangioma or grapelike hemangioma in previous classification. It is a kind of tortuous curved, extremely irregular and pulsatile vascular malformation. It is mainly formed by the direct anastomosis of arteries and veins with significant dilatation of the vascular wall, so it is also called congenital arteriovenous malformation. (2) Arteriovenous malformations are common in adults and rare in young children. It often occurs in the temporal region where the superficial temporal artery is located or in the subcutaneous tissue. The lesions are raised in a nymphoid shape and have a higher surface temperature than normal skin. The patient may feel the pulsations themselves; there is a tremor on palpation and a blowing murmur on auscultation. If the blood-supplying arteries are completely compressed, the pulsations and murmurs in the lesion area disappear. The tumor may erode the bone of the basement, or protrude into the skin, making it thin, or even necrotic and hemorrhagic. (3) Arteriovenous malformation can coexist with other vascular malformations. 4.Lymphatic malformation is formed by abnormal development of lymphatic vessels. Common in children and young people. It occurs in the tongue, lips, cheeks and neck. According to its clinical features and organizational structure, it can be divided into two categories: microcystic and macrocystic. (1) Microcystic: Including capillary and spongy lymphangiomas as previously classified. It consists of dilated lymphatic vessels lined with endothelial cells. The lymphatic vessels are extremely dilated and curved, constituting a polycomb cystic cavity, which is rather spongy. The lymphatic vessels are filled with lymphatic fluid. They present as isolated or multiple scattered small garden-shaped cystic nodular or punctate lesions on the skin or mucous membranes, which are colorless, soft, and generally non-compressible, with unclear borders. Lymphatic malformations of the oral mucosa sometimes coexist with microvascular malformations, appearing as small yellow or red blister-like projections called lymphangiomas. Those occurring on the lips, submandibular and buccal areas can sometimes make the affected area significantly hypertrophic deformity. Those occurring on the tongue often present with megalolingualism, causing jaw deformity, openness, inversion, tooth displacement, occlusal disorders and so on. The surface of the tongue mucosa is rough, nodular or foliated, with small yellow blisters protruding. On the basis of chronic inflammation occurring for a long time, the tongue can become hard. (2) Large cystic: Previously classified as cystic type or cystic hydatidiform tumor. It occurs mainly in the supraclavicular region of the neck, but also in the submandibular region and upper neck. They are usually multilocular cystic cavities, spaced apart from each other and containing clear, yellowish, watery fluid. The lesions are of different sizes, with normal surface skin color and filling state, soft and fluctuating sensation on palpation. Unlike deep hemangioma, body movement test is negative, but sometimes transillumination test is positive. 5.Mixed vascular malformation When more than one type of vascular malformation exists, it can be called mixed vascular malformation. Such as the aforementioned micro venous malformations and lymphatic microcystic malformations coexist; arteriovenous malformations accompanied by limited micro venous malformations; naturally, venous malformations can also co-exist with lymphatic vascular cystic malformations. Treatment Since the twenty-first century, with the international in-depth study of vascular diseases, classification and typing of comprehensive treatment has become an international principle, and non-invasive or minimally invasive treatment has become the main development trend. The treatment of hemangioma and vascular malformation is one of our major features. Over the years, the Department of Pediatric Surgery of the Second Hospital of Xi’an Jiaotong University has been exploring the diagnosis and treatment of hemangiomas and vascular malformations, and gradually formed a set of personalized treatment plans for different types of vascular malformations, which has received remarkable therapeutic effects. The degree of severity, pathologic process and impact on patients of these diseases are different. According to different diseases, types, sizes and locations, we choose reasonable treatment plans, and use a combination of drugs (including topical application, oral, local injection, intravenous route), laser, electrochemical, surgical, interventional embolization and other technologies and methods, which significantly improve the treatment effect and greatly reduce the adverse reactions and complications. Treatment Options Non-surgical treatment methods available elastic bandage. Pingyangmycin + urea has better efficacy in the treatment of venous malformation, which can significantly reduce the scope of the lesion and alleviate the symptoms. Our experience is that once a month injection of paxillin with intermittent injections of urea can achieve the therapeutic goal or clinical cure in most patients. Electrochemotherapy can be used in “mass” type cases where the lesion is partially located in a non-critical anatomical location. Intravenous or perivenous injection of polycinnamon. Third, arteriovenous malformations 1, AVMs treatment is very tricky, there is no single treatment method can guarantee the therapeutic effect. The treatment we use is interventional embolization + liquid sclerosing agent or interventional embolization + surgical resection. 2, Arteriovenous fistula using angiography + interventional embolization or angiography + surgical ligation. Fourth, lymphatic malformation 1, large cystic lymphatic malformation For the treatment of large cystic lymphatic malformation, the first choice of drug treatment, commonly used group A streptococcus, pingyangmycin injection, etc. More than 80% of patients with good results. 2, small cystic lymphatic malformation For the treatment of small cystic lymphatic malformation, we currently use mTOR inhibitor treatment, the efficiency can reach more than 70%. When the lesion is limited, surgical treatment is feasible, and the scope of surgery should be thorough, if necessary, the preoperative application of tissue expanders to dilate the skin near the lesion area, to ensure complete resection of the lesion, to avoid postoperative recurrence. V. Mixed vascular malformations Venous malformations can also coexist with lymphatic vascular macrocystic malformations. group A streptococcus plus pingyangmycin intervals mixed local injection therapy. Sixth, mTOR inhibitor treatment of complex vascular disease Following the international frontier, our department was the first to carry out oral mTOR inhibitor treatment of complex vascular malformations in China in 2011, including Kaposiform hemangioendothelioma, tufted hemangioma, capillary lymphatic-venous malformation, venous-lymphatic malformations, microcystic lymphatic malformation, multifocal lymphadenopathy with thrombocytopenia, involving skin/viscera, and other diseases, and also involving the skin/viscera. hemangiomatosis with thrombocytopenia, capillary lymphatic arteriovenous malformations, PTEN overgrowth syndrome with vasculopathy, lympho-capillary dilatation syndrome, and simple venous malformations involving skin/viscera.