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Venous capillay malformations
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SeminarDermatology society
1/11/1394Isfahan University of
medical sciences Gita Faghihi
Professor of dermatology
Venous capillay malformations
Venous malformations Venous malformations :either
superficial or deep veins that are abnormally formed and dilated.
usually present at birth.Both sexes equal
manifest clinically in infancy, childhood. no regression
Depending on their location, they may become symptomatic
Venous malformations
The following cellular characteristics are important to remember:• Flat endothelium, slow turnover• Normal mast cell count• Dysplastic walls• Thin basement membranes• No expression of vascular endothelial growth factor (VEGF) or basic
fibroblast growth factor (bFGF)• Low urinary bFGF
Clinically :• The closer these vessels are to the
surface, the deeper the color. A superficial lesion thus tends to be maroon-red, whereas a deep lesion may show a bluish hue. A very deep lesion may have no color, but just reveal itself as a protruding mass• lesions can be emptied by compression
or in the upright positionsymptoms related to :• mass effect / or• stasis
Natural history • slow, steady enlargement. • events such as surgery,
trauma, infection, or hormonal changes associated with puberty, pregnancy or menopause may cause rapid expansion. • These lesions may be
present in the skin, mucous membrane or in any organ (brain, bowel, liver, or spleen…)
Diagnosis confirmed by :
Ultrasonography
Evaluating the extent of a lesion may be done by: magnetic resonance imaging (MRI).
They are usually solitary isolated
events
but may occur in multiple areas.
• If they are present in multiple areas, • take a family history, because
autosomal dominant
Complications: • Thromboses : ( associated with large venous
malformations).
• Large or multiple venous malformations can be associated :with coagulopathy abnormalities that increase the risk of bleeding
• Recurrence is a common complication of therapy.
Patients with Turner syndrome may have venous malformations of the
intestine and feet.• Another rare dominant form is represented by the familial cutaneous-
mucosal venous malformation. • Cerebral cavernous venous malformations could also be familial.
Vascular ectasia in the ascending colon in a patient with Turner syndrome
Maffucci syndrome • is a rare developmental disorder
characterized by:• multiple enchondromas
associated with • subcutaneous
hemangioendothelioma of the distal extremities.
Maffucci's syndrome• coexistence of venous malformations and multiple enchondromas • The child appears normal at birth, but before puberty, a hard nodule
(small, localized outgrowth) appears most commonly on a finger or toe.
• This is soon followed by other nodules that involve the extremities and limbs.
• Dilated veins and soft bluish tumors occur in the affected limbs and elsewhere, and fracture of one or more bones often follows a minor injury.
• Healing is slow and unsatisfactory, resulting in other deformities that increase throughout childhood.
• Malignant (cancerous) degeneration of masses of tumor growth can occur.
• Diagnosis is made by clinical examination and X-rays. • Multiple cosmetic and reconstructive operations are necessary. • Because of the risk of malignancy, ongoing radiographic (X-ray)
assessment as well as biopsy of lesions that enlarge rapidly or cause symptoms are necessary.
As well as appearing on the skin, VMs can occur
• in the mucous membranes, such as the • eyelids or• inside the mouth, in• muscles or • internal organs.
Blue rubber bleb nevus syndrome
• This is characterized by : multiple rubbery blue lesions over the skin (trunk, limbs).• Lesions also can be internal and
are seen mostly in the GI tract (stomach and intestines) ,can cause severe GI bleeding and abdominal pain. • These lesions have a lymphatic
component.
Blue Rubber Bleb Nevus Syndrome
Klippel-Trenaunay Syndrome.
• association of a capillary malformation with a venous and a lymphatic malformation.• The birthmark tends to be small or patchy • The lymphatic malformation can contain large
cysts (macrocystic) or clusters of smaller ones (microcystic). • The affected limb is bulkier than the other side,
and sometimes longer. • The syndrome can be complicated by
(thrombophlebitis),, infections (cellulitis), ulceration and bleeding.
Klippel-Trenaunay syndrome
Note unilateral enlargement of one leg and capillary malformation with lymphatic vesicles and venous malformations .
Klippel-Trenaunay syndromeCombined capillary, lymphatic, and venous malformation. Involvement of the right leg with extremely increased girth, axial overgrowth, and macrodactyly. The cutaneous capillary malformation is studded with lymphatic vesicles.
localized intravascular coagulopathy • The likelihood of occurring such in venous
malformations is greater in patients in whom:• such lesions, as seen on magnetic resonance
imaging (MRI), are larger,• have visualized phleboliths,• are located on the trunk rather than the extremities,
and • have a spongiform morphology. Such characteristics suggest that coagulopathy is related to larger capacitance, slower flow, and reduced physiologic compression in these malformations.
elevated D-dimer level as the first biomarker of venous malformations within vascular anomalies
Duplex ultrasound is the best examination to confirm slow-flow, to identify the anatomy of feeding vessels and to offer a graphic visual demonstration of vascularity.
Diagnostics:
MRI imaging with spin-echo T1 and T2-weighted sequences is the gold standard for pre therapeutic evaluation of VMs
Outcome and Prognosis• related to the size and location of the venous malformation. The likelihood of significant perioperative morbidity and recurrence increases :• with more diffuse malformations
• malformations intimately involving vital neurovascular structures.
Capillary malformation
(portwine stain)
Capillary malformation(portwine stain)
• Capillary malformation is diagnosed at birth as a red stain that grows with the child. • Facial port wine stain can, in
some instances, be responsible for epilepsy and glaucoma (Sturge-Weber syndrome).
Sturge-Weber syndrome • encephalotrigeminal angiomatosis. • It is the association of capillary
malformation affecting the skin supplied by one branch of the trigeminal nerve of the face with defects in the underlying tissues.• These may result in a shrunken brain,
calcification inside the skull, seizures, meningeal angioma and eye abnormalities (glaucoma, optic atrophy).
Diagnosis :• Capillary malformations are usually diagnosed clinically and no
investigations are necessary for the majority of flat lesions. • However, when there is uncertainty about the diagnosis or
whether underlying tissues are affected, an ultrasound is often performed. • Characteristically, a vascular malformation shows blood vessels
set in normal background tissue.
• In more complicated cases it may be necessary to perform Magnetic Resonance Imaging (MRI) or angiography to help plan treatment.
vessels with a single layer of endothelial cells containing erythrocytes.
Histology of capillary malformation
Sturge-Weber syndrome
Unilateral involvement
Congenital glaucoma in sturge weber syn
Sturge-Weber syndrome
Bilateral involvement. Note extension onto the neck and chest.
Sturge-Weber syndromeCapillary malformation of the face,
chest, back, buttocks, arms, and legs. This patient has only Sturge-Weber
syndrome.
She does not have Sturge-Weber syndrome with Klippel-Trenaunay syndrome.
There are capillary malformations only.
For Klippel-Trenaunay syndrome, additional features include lymphatic malformations, lymphatic vesicles, venous malformations of the lower limb, venous flares, and enlargement of a lower limb often involving the toes.
The end