CT: VASCULAR

• benign

  congenital neoplasms of proliferating

  endothelial cell

  Congenital Hemangioma
• abnormal vessel morphogenesis


  Congenital Vascular Malformations:
• Strawberry nevus


  Congenital hemangioma
• Appears around time of birth but may not be apparent until early childhood

  Congenital Hemangioma
• Congenital Hemangioma

  • Capillary

  • Cavernous
• represent arteries and veins

  Vascular channels
• Life threatening: excessive during surgery

  CONGENITAL HEMANGIOMA
• Rapid growth phase → involution

  CONGENITAL HEMANGIOMA
• Persistent lesion that do not

  involute

  • Presence of bruits or thrill upon

  auscultation

  • Bone

  VASCULAR MALFORMATION
• Flat, nodular, vascular


  Hemangioma and Vascular M
• Color of Hemangioma and Vascular M?

  Color: red to blue (Depends on degree of congestion and depth)
• Most common areas of Hemangioma and Vascular M


  lips, tongue, buccal mucosa
• Abundant capillary spaces lined by endothelium without muscular support

  Congenital Hemangioma
• Can be capillary, venous, arteriolar,

  lymphatic channels

  • 1 type or 2 or more vessels

  Congenital Hemangioma:
• Congenital hemangioma, vascular malformation

  (Sturge-Webber Syndrome)
• honeycomb pattern with distinct margin

  congenital hemangioma
• Tx: Involution?

  congenital hemangioma
• Persistent:

  may need definitive treatment
• B-Blocker: 


  Propranolol
• another primary treatment of selected vascular lesions

  Laser therapy
• often ill-defined, total elimination may not be practical or

  possible

  margins (congenital hemangioma)
• Non-inherited neurocutaneous syndrome that include vascular malformations with characteristic distribution

  ENCEPHALOTRIGEMINAL ANGIOMATOSIS (Sturge-Weber Syndrome)
• Sturge-Weber Syndrome other name?

  ENCEPHALOTRIGEMINAL ANGIOMATOSIS
• Venous malformations involve leptomeninges

  of cerebral cortex + vascular malformations of

  face

  ENCEPHALOTRIGEMINAL ANGIOMATOSIS (Sturge-Weber Syndrome)
• skin innervated by one or more branches of CN V


  Port-wine stain/Nevus flammeus
• may extend intraorally involving buccal mucosa and gingiva

  Encephalotrigeminal Angiomatosis
• mental retardation, hemiparesis, seizure disorder

  neuro effects
• meds?


  phenytoin
• vascular malformations, glaucoma


  Ocular lesions
• Caused by mutations in 2 genes (TGF-B signaling pathway for

  vascular development and repair

  Hereditary Hemorrhagic Telangiectasia (Rendu-Osler-Weber Syndrome)
• 2 genes?

  • Endoglin on chromosome 9 (HHT type 1)

  • Activin receptor-like kinase 1 (ALK 1) on chromosome 12 (HHT

  type 2)
• Red macules or papules on face, chest and oral mucosa

  • Appear early in life, persist through adulthood

  • Increase in number with aging

  Hereditary Hemorrhagic Telangiectasia (Rendu-Osler-Weber Syndrome)
• Bleeding from oral lesions is common

  Hereditary Hemorrhagic Telangiectasia (Rendu-Osler-Weber Syndrome)
• Diagnosis: history of spontaneous epistaxis, presence of

  telangiectasias, arteriovenous malformation of internal organs, family history

  Hereditary Hemorrhagic Telangiectasia (Rendu-Osler-Weber Syndrome)
• Management for Hereditary Hemorrhagic Telangiectasia

  follow-up examination, use of antifibrinolytic drugs for

  frequent epistaxis
• Usually appears within 1st 2 decades

  of life

  • Painless, nodular, vesicle-like swelling

  when superficial or as submucosal

  masses when located deeper

  Lymphangioma
• Color of lymphangioma


  red-blue
• Crepitant sound as lymphatic fluid is pushed from one area to another

  Lymphangioma
• Most common intraoral site of lymphangioma

  tongue
• Responsible for macroglossia


  Lymphangioma