12.09.19 Vascular anomalies

Terminology

Classification

• • Vascular tumors

    • Infantile Hemangioma

    • Congenital Hemangioma

    • Kaposiform Hemangioendothelioma

    • Pyogenic granuloma

  • Vascular malformations

    • Slow-flow vascular malformations

    • Fast flow vascular malformtations

    • Combined-complex vascular malformations

Vascular tumors

• • Infantile Hemangioma

    • Appears in first 4 weeks of life

    • Grows over 9-12 months

    • Then often involutes

  • Congenital Hemangioma

    • Fully formed at birth

    • Types:

      • Rapidly involuting congenital hemangioma (RICH)

      • Non-involuting congenital hemangioma (NICH)

  • Kaposiform Hemangioendothelioma

    • Locally aggressive but not metastatic

    • => Thrombocytopenia due to sequestration of platelets

    • Hard to treat surgically

    • Use interferon alpha

  • Pyogenic granuloma

    • Small, benign

    • May be complicated by bleeding, crusting and ulceration

Vascular malformations

• • Slow-flow vascular malformations

    • Capillary malformation

      • Flat, reddish lesions that typically affect the skin, mostly around the head and the neck

      • Darken with age

    • Venous malformation

      • Bluish lesion

      • Compressible on palpation

      • Enlarge with physical activity or a dependent position

    • Lymphatic malformation

  • Fast-flow vascular malformations

    • Arterial malformation

    • Arteriovenous fistula (AVF)

    • Arteriovenous malformation

  • Combined-complex vascular malformations

    • CVM: capillary venous malformation

    • CLM: capillary lymphatic malformation

    • LVM: lymphatic venous malformation

    • CLVM: capillary lymphatic venous malformation

    • AVM-LM: Arteriovenous malformation- lymphatic malformation

    • CM-AVM: capillary malformation- arteriovenous malformation

Phakomatoses

• • Disorders of central nervous system that additionally result in lesions on the skin and the eye

    • These tissues have a common ectodermal origin

  • Examples:

  • Neurofibromatosis

  • Tuberous sclerosis

  • Ataxia telangiectasia

  • Sturge-Weber syndrome

  • von Hippel-Lindau disease

  • Incontinentia pigmenti

  • Nevoid basal cell carcinoma syndrome