Vascular Malformations
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- What findings are associated with Klippel-Trenaunay syndrome?
- Port wine stain of one extremity overlying a venous or lymphatic malformation with limb hemihypertrophy
- What is the inheritance pattern of Rendu-Osler-Weber syndrome?
- Autosomal dominant
- How does Rendu-Osler-Weber syndrome present?
- epistaxis, hematuria, hematemesis and melena
- What is Rendu-Osler-Weber syndrome?
- Multiple ectatic vessels involving skin, mucous membranes and visceral organs?
- What is Parkes-Weber syndrome?
- Port wine stain overlying venous and lymphatic malformations of upper extremity with AV fistulas and skeletal hypertrophy
- What syndrome is associated with vascular malformations, mental retardation and seizures?
- von Hippel-Lindau disease
- What vascular malformations are found in von Hippel-Lindau disease?
- Hemangioms of retina and hemangioblastomas of cerebellum and visceral organs
- What is proteus syndrome?
- Partial gigantism of exremities, hemifacial hemihypertrophy, macrocephaly, macrodactyly, localized exostoses, subcutaneous lipomas and vascular malformations.
- In Sturge-Weber syndrome a large vascular malformation is found in the distribution of what nerve?
- Trigeminal (V1, V2)
- What is the most common vascular malformation found in Sturge-Weber syndrome?
- port wine stain
- What neurological findings are associated with Sturge-Weber syndrome?
- focal motor seizures, hemiparesis, visual field defects, glaucoma and mental retardation
- What is the cause of mental retardation in Sturge-Weber syndrome?
- Leptomeningeal venous malformations and intracranial calcifications
- What vascular malformation contains a central blanching arteriole?
- Spider angioma (arteriolar malformation)
- What is the treatment for spider angiomas?
- Obliteration of the central feeding arteriole with laser or electrocautery.
- What percentage of hemangiomas are noted within the first year of life?
- 80%
- What is the natural history of hemangiomas?
- Present at (or near) birth, proliferate for several months then spontaneously regress
- Hemangiomas contain an increased quantity of what type of cells?
- mast cells
- The mast cells in hemangiomas produce what protien?
- Heparin
- What are the histologic findings of hemangiomas?
- Proliferative primitive endotheilial cell nests in syncytial masses with or without vessel lumens. Hyperplasia
- What is the most common tumor of infancy?
- Hemangioma
- Which vascular malformation is characterized by compressiblility and fills with blood when body position changes?
- Venous malformation
- How can a venous malformation affect the function of an extremity?
- By intertwining with neurovascular structures
- How do venous malformations cause pain?
- thrombosis
- Which vascular malformation is limited to capillaries in the dermis?
- Port wine stain (capillary malformation)
- If left untreated what will happen to a port wine stain?
- Cobblestoning, ectasia and progressive darkening (from deoxyhemoglobin)
- What syndrome is associated with a port wine stain?
- Sturge-Weber syndrome
- What is the treatment for port wine stains?
- 585nm laser
- What are the physical findings associated with Klippel-Feil syndrome?
- short neck and low posterior hairline with fusion of cervical vertebrae
- What syndrome is associated with fusion of cervical vertebrae?
- Klippel-Feil
- What are the physical findings associated with an AV malformation?
- pulsation, temperature changes, bruit, thrill. Does not decompress fully with arm elevation
- How do you make the diagnosis of an AV malformation?
- MRA (MRI with iv gadolinium)
- What is the treatment for an ulcerated or bleeding AV malformation?
- Aggressive resection with immediate flap coverage
- How do superficial lymphatic malformations present?
- Clear vesicles
- Bone overgrowth is associated with which vascular malformations?
- Deep lymphatic malformations
- What is the treatment for lymphatic malformations?
- Erbium laser (2940 nm)
- What vascular malformation occurs secondary to trauma?
- pseudoaneurysm
- What is Kassabach-Merritt syndrome?
- Profound thrombocytopenia associated with solitary hemangioma or diffuse hemangiomatosis
- What percentage of patients with Maffucci syndrome develop chondrosarcoma?
- 20%
- What is Maffucci syndrome?
- Multiple enchondromas and cavernous hemangiomas associated with limb foreshortening.