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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?
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?
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?
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?
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?
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?
What is Kassabach-Merritt syndrome?
Profound thrombocytopenia associated with solitary hemangioma or diffuse hemangiomatosis
What percentage of patients with Maffucci syndrome develop chondrosarcoma?
What is Maffucci syndrome?
Multiple enchondromas and cavernous hemangiomas associated with limb foreshortening.

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