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Vascular malformations result from errors in vascular morphogenesis Unlike hemangiomas (discussed later), they are present at birth, grow only in proportion to the child, and not undergo regression They may be of capillary, venous, or arterial origin or combinations of vessel types may exist within the same lesion Clinical manifestations of vascular malformations are determined by their type and anatomic location Port-wine stains are among the more common capillary vascular malformations They have a characteristic deep red to purple hue ( Fig 120.15 ) Children with facial port-wine stains that lie in the distribution of the ophthalmic branch of the trigeminal nerve (which includes the forehead, upper eyelids, and nose) merit careful evaluation for associated anomalies Specifically, Sturge– Weber syndrome is characterized by ipsilateral vascular angiomatosis of the leptomeninges and ocular vessels Clinical manifestations may include seizures, intellectual disability, hemiplegia, and glaucoma These children should be referred to neurology for consideration of neuroimaging and EEG, as well as ophthalmology for a thorough eye examination Serial neuroimaging performed on these children often demonstrates evolution of serpiginous calcifications and progressive atrophy of the cerebral cortex underlying the pial vascular malformations Treatment consists of seizure control, or surgical resection in cases of failed medical management Children with port-wine stains involving an extremity may develop hemihypertrophy of the affected limb as a result of the unusually rich underlying blood supply, known as Klippel–Trenaunay–Weber syndrome All cosmetically significant port-wine lesions should be referred to a dermatologist for consideration of laser therapy (see Chapter 88 Dermatologic Urgencies and Emergencies ) Salmon patches are the most common form of vascular malformation seen in infancy and occur in 30% to 40% of all newborns These flat pink lesions, which become more prominent with crying or exertion, are most commonly located on the nape of the neck (stork bites), on the glabella, or over the eyelids (angel kisses) They consist of distended dermal capillaries and almost always fade or resolve by the end of the first year of life, although nuchal salmon patches may persist into adulthood Lymphatic malformations are errors in lymphatic vessel morphogenesis Presentation is usually before years of age, however can be as late as adulthood They may be isolated or combined with other vessels in the same lesion such as in CLOVES syndrome Similar to other vascular malformations, lymphatic malformations vary in size, grow with the child, and can cause bony overgrowth of the affected side, leading to asymmetry These lesions range from superficial

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