marți, 7 martie 2023

Pete vasculare

Terminologia e importantă

Malformațiile vasculare 

  • pot fi arteriale, venoase, limfatice, capilare, complexe. Majoritatea sunt malformații venulo-capilare. 
  • Termenii vechi: pată de vin porto (port-wine stain) sau nevus flammeus. 
  • Sunt mereu prezente de la naștere.
  • Nu prezintă tendință spre involuție.
  • Evoluția culorii este adesea de la roz spre roșu, apoi purpuriu închis.

Leziuni vasculare proliferative: 

  • = hemangioame.
  • Apar în primele săptămâni de viață.

Sindromul Klippel Trenaunay 

= triad of port-wine stain, varicose veins, and bony and soft tissue hypertrophy involving an extremity.

In Klippel-Trenaunay-Weber syndrome (KTWS), the capillary hemangioma or port-wine stain usually presents first.

This hemangioma has a distinct, linear border that respects the midline. Hemangioma is often noted on the lateral aspect of the limb.

It is typically of the nevus flammeus type, but cavernous hemangiomas or lymphangiomas may also occur. Nevus flammeus is a salmon pink patch, sometimes with a verrucous quality, which evolves to a deep purple color with time. Unlike strawberry hemangiomas, the port-wine stain hemangioma possesses neither a proliferative nor a regressing phase.

Hemangioma depth is variable. It may be limited to the skin or extend deeper to subcutaneous tissue, including muscle and bone. Visceral organs, such as the pleura, the spleen, the liver, the bladder, and the colon may also be affected. Visceral organ involvement portends greater morbidity secondary to internal hemorrhage that may manifest as hematuria or hematochezia.

If large enough, cutaneous hemangiomas may sequester platelets, leading to possible Kasabach-Merritt syndrome, a type of consumptive coagulopathy. [20] The hemangioma often overlies the vascular malformation.

Sindromul Sturge Webber

= encephalotrigeminal angiomatosis 

neurocutaneous disorder with angiomas that involve the leptomeninges (leptomeningeal angiomas [LAs]) and the skin of the face, typically in the ophthalmic (V1) and maxillary (V2) distributions of the trigeminal nerve. The hallmark of SWS is a facial cutaneous venous dilation, also referred to as a nevus flammeus or port-wine stain (PWS).

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