1. What are the characteristics of vascular malformations? Vascular malformations consist of malformed blood vessels and/or lymphatic vessels lined with quiescent endothelial cells. Although vascular malformations are considered to be congenital, a few cases do develop in childhood. Based on hemodynamic characteristics, vascular malformations can be categorized as hypovolemic and hypervolemic malformations. Low-flow malformations include capillary malformations, venous malformations, lymphatic malformations, and mixed malformations that include these malformations. Aneurysms and arteriovenous fistulas are high flow malformations. Vascular malformations are lifelong and develop in proportion to the child’s body; some patients experience exacerbation or expansion. Vascular malformations do not resolve spontaneously. Trauma and estrogen (during puberty and pregnancy) can stimulate the rapid growth of vascular malformations. 2.What is wine stain? What are its characteristics and how is it treated? Wine stain is the most common capillary malformation, the incidence is about 0.3% of newborns, and there is no difference between male and female. Wine stains are present at birth, but in a few patients, they develop later and do not subside on their own, developing in proportion to the child’s physical growth and development. Wine stains are usually unilateral and phasic in distribution and can appear on any part of the body.Tallman et al. retrospectively analyzed 310 cases of wine stains, 85% were unilateral and 15% were bilateral.68% of the patients had more than one skin involvement. In this cohort, ocular and CNS lesions occurred in 8% of cases if area V1 was involved; however, ocular and CNS lesions occurred in 25% of cases if area V1 was involved bilaterally or in all three areas. Wine discoloration is pink in infancy, and the color of the lesions decreases to some extent between 1 and 6 months of age, which may be due to a somewhat significant decrease in the hemoglobin concentration in the circulation during this period. With age, the color of wine stains gradually becomes dark red or purplish, the skin surface becomes irregular, the epidermis is thickened, and nodules appear. These changes are most often seen in facial wine stains, and wine stains on the trunk and extremities are rare. Wine stains, especially in the V2 region, are complicated by hypertrophy of soft tissues and bones. Wine stains have progressive hypertrophy with thickening of the surface lesions and nodular changes. In addition, wine stains can develop secondary pyogenic granulomas. The pathology of wine stains is characterized by a normal number of mature capillaries located in the superficial dermis, with no signs of hyperplasia; with further progression, the lesion infiltrates into the deeper layers of the dermis and subcutaneous tissues, with a marked dilatation of the capillaries, which are surrounded by loose collagen fibers. Wine discoloration can develop alone or in conjunction with other malformations. Wine stains are a typical clinical manifestation of SturgeCWeber (SWS) syndrome, KlippelCTrenaunay (KT) syndrome and Proteus syndrome. Treatment:Wine stains, especially on the face, can cause great psychological stress to children, so the treatment of wine stains emphasizes early treatment, preferably before school age.PDL is the most important treatment for wine stains, with a wavelength of 585-595nm and a pulse duration of 450-1500ms.Over the past 10 years, PDL has been proven to have the advantage of complication in the treatment of wine stains. In the past 10 years, PDL has been proven to have the advantages of fewer complications and higher cure rate in the treatment of wine pigmentation. Most of the literature reports that the lesions can be significantly reduced, but not completely cured. 3.What is SturgeCWeber (SWS) syndrome and how is it treated? SWS, also known as cerebral trigeminal angiomatosis and Sturge-CWeber syndrome, is a triad of wine-colored spots in the V1 area of the face, ipsilateral molluscum contagiosum vascular malformations, and vascular malformations of the choroid of the eyelids, which can lead to ipsilateral glaucoma and atrial hydrocephalus. There is no direct evidence of a genetic predisposition and the etiology is unknown. All three parts involved are derivatives of the orthodromic exophthalmos.Wine discoloration in SWS is distributed in zone V1, the area innervated by the first branch of the trigeminal nerve, but wine discoloration located on the upper face and eyelids has never been complicated by intracranial vascular malformations. About 10% of patients with wine stains in area V1 have SWS, even though area V1 is involved bilaterally, and often wine stains are also present on the face and other parts of the body, most often with intracranial and eyelid involvement on one side as well. About 15% of patients have intracranial lesions bilaterally, and such patients often have signs of neurologic damage.Patients with SWS do not necessarily present with all the manifestations of SWS, and some present with only wine-colored spots and ocular lesions, or only wine-colored spots and CNS lesions, or only ocular and CNS lesions.Common disorders of CNS include seizures with contralateral seizures, hemiparesis, headaches, and mental retardation. Seizures tend to be confined petit mal seizures or generalized seizures, often with onset before the age of 2 years. Persistent seizures predispose the child to severe mental retardation. Ocular lesions, including choroidal vascular malformations, do not have to be present to diagnose SWS, and the incidence of glaucoma is about 50% if the wine stain is located near the eyelids. Increased intraocular pressure is the result of multiple factors; ciliary body congestion, anterior chamber angle malformations, and arteriovenous malformations of the scleral vascular plexus may all contribute to glaucoma. In these patients, regular ophthalmologic examinations should be performed at 6- to 12-month intervals. Long-term follow-up is important because glaucoma often progresses further, and early detection of increased intraocular pressure can stop the progressive progression of the disease. Sixty percent of patients with SWS and glaucoma present with symptoms in infancy, with lengthening of the eye, enlarged eyeballs, bull’s-eye and myopia due to increased intraocular pressure. Another 40% of patients present with symptoms in childhood, which typically do not manifest as eye enlargement. Because most children with SWS have a normal initial presentation, imaging is helpful in determining the disease. In children over 2 years of age, the characteristic calcified dots are found in 2/3 of these cases, but the incidence of such dots is very low in infancy. The calcified dots are distributed in parallel lines along the cortical sulci, similar to a “railroad track”, and are mostly found in the soft meningeal vascular malformations near the occipital bone. after the age of 1 year, the diagnosis is most often made by CT, and MR is the most effective means of detecting early SWS, not only by clearly identifying malformations, but also by demonstrating concomitant cortical hypertrophy. Even if MR does not detect vascular malformations, enlarged choroid plexus can be detected. Functional imaging of brain tissue, such as positron emission scanning, or single photon emission computerized scanning, can detect abnormal metabolic activity or increased cerebral blood flow in infants and children. Brain damage with hypometabolism and hemispheric hypoperfusion occurs after seizures. Treatment Anticonvulsant therapy is required for seizures.