Angiodysplasia
| Classification according to ICD-10 | |
|---|---|
| D18 | Hemangioma and lymphangioma, any location |
| D18.0- | Hemangioma, any location |
| D18.1- | Lymphangioma, any location |
| I78.0- | Hereditary hemorrhagic telangiectasia (HHT), also known as Osler-Weber-Rendu disease |
| K31.81 | Angiodysplasia of the stomach and duodenum with no indication of bleeding |
| K31.82 | Angiodysplasia of the stomach and duodenum with bleeding |
| K55.2 | Angiodysplasia of the colon (Heyde syndrome) |
| D23- | Other benign neoplasms of the skin |
| ICD-10 online (WHO version 2019) | |
As angiodysplasia ( altgriech. Ἀγγεῖον ( angeion ) vessel δυσ- ( dys ) 'mis-, un-', πλάσσειν ( plassein ) form of πλάσσω, shapes) is called vascular malformations of arteries , veins or lymphatic vessels , which in enormous complexity and diversity can occur. As a synonym, they are also known as vascular dysplasias .
Hamburg classification
In the past one could only describe these clinical pictures and their pathomorphology could not be recorded. Depending on the vessel, the malformations were given numerous syndrome names , sometimes for the same malformation. The assignment of a clinical picture to one of these syndromes is not always successful and overlaps are possible. The Hamburg classification developed in 1988 avoids the syndrome terms and tries to describe angiodysplasia according to its anatomical and functional changes. On the one hand, a distinction is made between two forms, truncular and extratruncular, in which the disorders in the embryonic development phases are taken into account.
| Art | Truncular form | Extra-truncular shape |
|---|---|---|
| Mainly arterial defects | Aplasia (lack of attachment), obstruction (narrowing), dilation (widening) | Infiltrating or circumscribed |
| Mainly venous defects | Aplasia, obstruction, dilation | Infiltrating or circumscribed |
| Mainly lymphatic defects | Aplasia, obstruction, dilation | Infiltrating or circumscribed |
| Mainly arteriovenous defects | Deep and superficial AV fistulas | Infiltrating or circumscribed |
| Combined errors | Arterial and venous and lymphatic with and without AV fistula | Infiltrating or circumscribed hemolymphatic |
Syndromes
Developmental Venous Anomaly
The Developmental Venous Anomaly (DVA) is a benign, venous malformation in the brain . The anomaly consists of a bundle of dilated, medullary veins that converge on a large-caliber central drainage vein.
Naevus flammeus
The fire mark or nevus flammeus is a benign skin lesion. The fire mark is based on a congenital malformation. The fine blood vessels that run beneath the epidermis are abnormally enlarged and tend to grow . The stain of fire can in some cases be an indication of another disease. Up to 30 syndromes have so far been linked to the occurrence of a fire mark:
Sturge Weber Syndrome
Sturge-Weber syndrome is a congenital progressive disease belonging to the group of neurocutaneous phacomatoses . It is characterized by hollow benign vascular tumors ( angiomas ) in the facial area, in the area of the meninges , in the area of the ipsilateral soft meninges ( leptomeninx ) and the choroid of the eye ( choroid ). Affected children can suffer from epileptic seizures , glaucoma and mental retardation .
Klippel-Trénaunay-Weber syndrome
The Klippel-Trénaunay-Weber syndrome is a malformation syndrome of the vessels, which is associated with a large nevus flammeus , lymphangioma , which is sometimes accompanied by gigantic growth of the trunk or extremities . In addition, causal malformations of the deep blood and lymphatic system can occur.
Maffucci syndrome
The syndrome is characterized by enchondromatosis (multiple benign cartilage tumors ) and deep cavernous hemangiomas and lymphangiomas of the skin and internal organs.
Hemangioma
A hemangioma (blood sponge ) is an embryonic tumor with endothelial proliferation and secondary formation of vascular lumen. A distinction is made between capillary hemangioma, cavernous hemangioma, grape or berry-shaped hemangioma, sclerosing hemangioma, hemangioma planotuberosum, hemangioma of the eye socket and hemangioma of the liver .
Kasabach-Merritt syndrome
In Kasabach-Merritt syndrome, benign, cavernous and large to huge hemangiomas develop. It is more likely to occur in benign tufted angioma (synonym: Angioblastoma of Nakagawa) and in Kaposi-like malignant epithelioid hemangioendothelioma .
Pyogenic granuloma
Pyogenic granuloma is a benign vascular skin tumor belonging to the group of hemangiomas. It is an exophytic vascular proliferation .
Vascular malformation
A vascular malformation (vascular malformation) is a malformation of one or more blood or lymph vessels. The vascular malformations include the arteriovenous malformations (AV malformations), the cavernomas (venous malformations), and lymphatic malformations . A special form of a lymphangioma is the hygroma colli . Also the Gorham's disease is associated with lymphangioma in conjunction. Malformations are mostly congenital, but show an increase in size over the course of life. Osler's disease is one of the capillary malformations in the digestive tract .
Osler's disease
Osler's disease is an autosomal dominant inherited disease in which there is pathological dilatation of blood vessels. These so-called telangiectasias can occur anywhere, but are particularly found in the nose (leading symptom nosebleed ), mouth , face and the mucous membranes of the gastrointestinal tract, but they can also occur in the lungs , liver and brain, which - especially from the age of 40 - internal bleeding can occur, which can even have life-threatening effects.
Heyde syndrome
Heyde syndrome consists of the combination of acquired aortic valve stenosis and bleeding from malformed blood vessels in the ascending part of the colon . The anemia caused by the bleeding is also considered part of the syndrome.
Kommerell's diverticulum
Kommerell's diverticulum is a sac-like enlargement or protuberance of the aortic arch or the descending main aorta (aorta descending) in the case of a right subclavian artery ( arteria subclavia dextra) emerging from the aorta .
Hippel-Lindau disease
Hippel-Lindau's disease is a rare, hereditary tumor disease from the so-called phakomatoses . The patients develop benign, tumor-like tissue changes ( angiomas ) primarily in the area of the retina of the eye and the cerebellum . In the central nervous system , the brain stem and spinal cord , and rarely the cerebrum, can also be affected. It is characteristic that tumors develop consisting of blood vessels. Many patients also have tissue changes in the kidney ( renal cell carcinoma ), adrenal gland ( pheochromocytoma ), and pancreas . In men, the epididymis can be affected.
See also
literature
- W. Hepp, H. Kogel: vascular surgery. 2nd Edition. Urban & Fischer, 2013, ISBN 978-3-437-21841-5 .
Web links
- Federal Association of Congenital Vascular Malformations V.
- Loose: Hamburg classification (PDF; 71 kB)
Individual evidence
- ↑ St. Belov, DA Loose, J. Weber: Vascular Malformations. In: Periodica Angiologica. 16, Einhorn Presse Verlag, Reinbek 1989, pp. 25-30.
