DefinitionThis section has been translated automatically.
Frequent, syndromal or non-syndromal, benign, mono- or polytopic vascular malformations as a result of abnormal embryonic tissue development. The clinical pictures summarized under the term "vascular malformation" can include vascular malformations in different tissues (skin, muscle, bone) or in different organs (e.g. skin and CNS).
In contrast to the infant's hemangioma, vascular malformations are always present in an established form at the time of birth, even though they may initially manifest themselves only discreetly over a period of years or remain completely unnoticed clinically.
Vascular malformations initially show a growth in size proportional to the growth of the body, do not regress and may show an autonomous proliferation in the course of life (see also Naevus flammeus - Port-wine stain).
ClassificationThis section has been translated automatically.
The sometimes confusing morphological diversity of vascular tumours and malformations (which vascular area is affected: capillary, vein, artery, mixed?), their localisation in different parts of the skin-subcutaneous-muscle compartment, their often difficult etiological classification (malformation or true neoplasia?) makes a universally satisfactory classification difficult but unavoidable. In the difficult systematic classification of vascular malformations, one can proceed according to the following aspects:
- true neoplasia
- capillary (capillary malformation, KM - Port-wine stain - Naevus flammeus)
- venous (venous malformation or mixed, VM - or KM/VM - e.g. angiokeratoma circumscriptum)
- arterial (AM or mixed KM/VM/AM/AVM)
- lymphatic (LM or mixed KM/VM/AM/LM)
Haemodynamic/ functional assignment:
Classification of vascular malformations according to pathological-anatomical aspects:
- Arterial malformations (rarely isolated; mostly truncal malformations with hypo- and aplasia of peripheral arteries)
- Arterio-venous malformations (usually within the framework of complex malformations):
- Venous malformations (dysplasia of deep vein complexes - often affecting the vessels of the extremities; venous aneurysms):
- Dysplasias of deep veins of the extremities (clinical: picture of chronic venous insufficiency)
- Dysplasia of the venous valves (avalvulie, hypovalvulie)
- Phlebectasia (deep/extrafascial vein systems or combination = Bockenheimer's syndrome).
- Capillary and post-capillary malformations (KM):
- Non-syndromal capillary malformation (naevus flammeus/port wine stain):
- Syndromal capillary malformations (complex mixed images - capillary/venous/capillary/post-capillary/arterial)
- Teleangiectatic malformations (see below teleangiectasia)
- Suction. Angiokeratomas (etiopathogenetically not coherent)
- Lymphangiomas, superficial and deep:
Classification according to haemodynamic criteria (low-flow and high-flow lesions):
- high-flow: AV fistulas, arterial and arteriovenous malformations.
- Low-flow: Capillary (e.g. nevus flammeus) and lymphatic malformations(lymphangiomas) show little or no flow velocity.
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EtiopathogenesisThis section has been translated automatically.
In sporadic nonfamilial patients with venous malformations, somatic mutations in the TEK gene associated with the presence of activating PIK3ca (phosphatidylinositol 3-kinase) mutations are detected in about half of the cases (Castillo P et al. 2016).
Note(s)This section has been translated automatically.
The clinical subclassification of vascular malformations can be based on the patient's medical history, clinical course and clinical examination.
Venous and lymphatic malformations are soft and compressible; "high-flow" lesions appear solid. While lymphangiomas show a sudden growth in size during infection, venous malformations increase in volume when pressure is increased (pressing, bending forward). "High-flow lesions can be accompanied by an increase in temperature above the affected area and a palpable pulsation.
In the case of capillary/post-capillary malformations, the group of so-called angiokeratomas is listed. The diseases listed under 1-5 are based on metabolic diseases which appear dermatologically under the clinical picture of "Angiokeratoma corporis diffusum".
LiteratureThis section has been translated automatically.
- Baumann NM et al (1997) Treatment of massive or life-threatening hemangiomas with recombinant alfa-2a interferon. Otolaryngol Head Neck Surg 117: 99-110
- Castel P et al(2016) Somatic PIK3CA mutations as a driver of sporadic venous malformations. Sci Transl Med 8(332):332ra42.
- Mulliken JB, Glowacki J (1982) Hemangiomas and vascular malformations of infants and children: A classification based on endothelial characteristics. Plast reconstruction surgery 69: 412-422
- Mulliken JB (1988) Diagnosis and natural history of hemangiomas. In: Vascular birthmarks: Hemangiomas and vascular malformations. Saunders, Philadelphia
- Seyberth HW et al (2002) Advanced extracranial hemangiomas and vascular malformations. Dtsch Ärztebl 99: A-188 / B-153 / C-149
Incoming links (15)Angiodysplasia; Angiodysplasia syndromes; Clove syndrome; Fibrolipomatous hamartoma of the nerves; Focal nodular hyperplasia; hamartoma of the skin; Hemangiomas; Lymphangioma; Lymphangioma; Lymphangioma circumscriptum; ... Show all
Outgoing links (30)Angiokeratoma circumscriptum; Angiokeratome, solitary; Angioma serpiginosum; Asymmetrical nevus flammeus (overview); Ataxia teleangiectatica; Blue rubber bleb nevus syndrome; Chronic venous insufficiency (overview); Cutis marmorata teleangiectatica congenita; Fabry's disease; Fucosidosis; ... Show all
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