Vascular malformation

1. Vascular malformation

2. Etiology • Birth defect affecting arteries, capillaries and veins • once referred to as port-wine stains • prevalence at birth of capillary malformations is reported at 0.3% without predisposition to either sex. • True neoplastic growth, structural malformations, and hamartomatous transformations

3. Classification Hemangiomas -rapidly progresses -embryobic or arises from de novo in origin -disordered angiogenesis as the precipitating cause -Hormonal influence is suggested by the 3:1 female-to-male ratio. -The mechanism by which angiogenesis is stimulated and then turned off is not known. Vascular malformations -abnormal embryonic and fetal morphogenesis during the retiform stage of development of endothelial channels -non neoplastic/ non proliferative, they grow commensurate with the child -best characterized by their principal cell type, the degree of blood or lymph flow and the structures involved -Capillary vascular malformations possibly occur because of a lack of proper sympathetic vasomotor control. -Venous malformations are due to errors in development of the venous system and may result in hypoplasia, hyperplasia, and aplasia of the deep and superficial systems. -represented by ectatic capillaries and medium-sized venules with thin walls and flat endothelial cells -lack of sympathetic innervation regulating blood flow in vessels with capillary malformation is believed to produce progressive ectasia

4. Hemangiomas • small red dot appearing during the first few weeks of life • lesion then grows disproportionately fast compared with the child. • exhibit spontaneous involution, by 5 yrs, 50% resolve, by 7yrs, 70% resolve • head and neck area is involved in 60% of cases, followed by the trunk and then the extremities • Multiple lesion can occur

5. Hemangiomas • disproportionate growth compared to the child. • Superficial hemangiomas are characterized by a bright strawberry-red pigmentation. • Deeper hemangiomas appear as purple- or blue-pigmented lesions. • Rapid growth occurs over the next 3-6 months. • Plateau and cessation of growth usually occur when patients are aged 6-18 months. • Ungergoes involution through the years

6. Vascular malformations • always are present at birth. They may be subtle at first, but the color does not fade and often darkens. • growth of vascular malformations is proportionate to the growth of the child and never extends beyond its initial boundaries. • Capillary vascular malformations (port-wine stain) roughly follow sensory nerve distribution

7. Vascular malformations • Initially pale with normal overlying skin texture but may darken as the patient ages. • Nodularity and a darker purple pigmentation also may occur in adulthood because of increasing dilation of the dermal vessels. • Venous malformations become more evident as the child grows. • gradually dilate and may cause distortion of facial features

8. Treatment • determine which lesions require early intervention to minimize long-term complications or disfigurement. • Steroids both systemic and intralesional, have are used to treat rapidly growing hemangiomas • interferon alfa-2a : treatment of pulmonary hemangiomatosis, life-threatening hemangiomas, and diffuse neonatal hemangiomatosis • Chemotherapy is reserved for life-threatening hemangiomas.se neonatal hemangiomatosis • Surgical care is most commonly used when involution is incomplete or leaves excess fibrofatty tissue or redundant skin • Excisional treatment is warranted for venous malformations because spontaneous involution is not anticipated • Laser photocoagulation is the treatment of choice for superficial capillary vascular malformations. • Venous stripping may be required to treat varicose veins associated with certain vascular malformations • Complete excision of the AV malformation is required because subtotal resections result in recurrence