Microneurosurgery, Volume IIIA. Mahmut Gazi Yasargil
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Classification of Vascular Malformation
“The classification of the vascular malformations of the brain has been the subject of considerable discussion and the extensive literature on this topic reflects a varying and, at times, confusing nomenclature.” (Bebin and Smith 1982, p. 13). The confusion continues and applies not only to vascular malformations of the brain but also to those of all other organs. We agree with Mulliken (1983) “the words to describe the common vascular birthmarks reflect our ignorance of their pathogenesis”. There are majors problems with both nomenclature and classification.
Nomenclature
A. Both Greek and Latin roots are used: Vascular malformation (Latin roots), Angiodysplasia (Greek roots).
B. The suffix oma (= neoplasm) is commonly used not only for true vascular tumors such as hemangioblastoma, but also for vascular malformations. The use of the suffix osis (e. g. “angiomatosis”) has sometimes been inappropriate. The term should be reserved for diffuse or multiple lesions only.
At the present time the English version of “malformation” has found general acceptance and there is little point in entering further into sophisticated linguistic struggles.
Classification
As ever more sophisticated means of studying vascular malformations have developed, systems of classification have diversified from the early descriptive terms based purely on gross morphological observation. In some instances, old terminology has been retained, in others changed and in yet further (often simultaneous) publications regrouped under different headings.
Noran presented and discussed all the proposed classifications in the literature up to 1945 and concluded: “a comprehensive evaluation of the literature is warranted in order that one may arrive at some correlation between these various nomenclature and classification.”
Within the last 40 years further new concepts have been proposed. Table 3.6a contains some of the more notable historic and modern classifications, and shows the development of thinking regarding the malformations.
Virchow (1863) conducted his own thorough studies and described 4 main types of malformation and stated, as early as 1851: “one type of angioma can transform into another by changes in flow and pressure or by cellular proliferation.”
The venous anomalies, and plexiform angioma of Dandy’s classification (1928) would nowadays be called AVM, and the cyst with angioma in the wall a hemangioblastoma. We assume that he did not describe any “venous angiomas” as now recognized by Huang et al. (1984) and McCormick (1985).
Table 3.6a
Virchow (1863)
1. Angioma simplexTelangiectasia (can change to cavernoma)
2. Cavernous angioma
3. Racemous angioma
a. Arterial (aneurysma anastomoseon)
b. Venous angioma
c. Arteriovenous aneurysm
4. Lymphangioma
Dandy (1928)
1. Angioma
a. Cyst with angioma in the wall (actually angioblastoma)
b. Cavernous angioma
c. Plexiform angioma (nowadays a form of AVM)
2. Arteriovenous aneurysm (nowadays a form of AVM)
3. Venous abnormalities (nowadays also AVM)
Cushing – Bailey (1928)
1. Hemangioblastoma (true neoplasm)
a. Cystic
b. Solid
α capillary
β cellular
γ cavernous (nowadays = cavernous angioma)
2. Angiomatous malformation
a. Telangiectasias
b. Venous angiomas
c. Arterial or arteriovenous angiomas (AVM)
Bergstrand – Olivecrona – Tönnis (1936)
1. Angioma cavernosum
2. Angioma racemosum
a. Telangiectasias
b. Angioma capillare et venosum calcificans (Sturge-Weber disease)
c. Angioma racemosum arteriale
d. Angioma racemosum venosum
e. Aneurysma arteriovenosum
3. Angioblastoma, angioreticuloma or Lindau tumors
4. Angioglioma (!)
Turner – Kernohan (1941) (spinal cord)
1. Vascular malformations
a. Telangiectasia
b. Angioma or hamartoma
α angioma venosum
β angioma arteriovenosum or
γ angioma arteriale
2. Vascular neoplasms
a. Capillary
α capillary hemangioma
β hemangioendothelioma
γ capillary hemangioblastoma
b. Cavernous
α cavernous hemangioma
β cavernous hemangioblastoma
c. Hemangiosarcoma
Wyburn-Mason – Holmes (1943) (spinal)
1. True tumors
a. Hemangioblastoma
α angioreticuloma
β extradural hemangioblastoma
2. Malformations
a. Telangiectasia
b. Venous malformation
α secondary venous anomalies
β venous angioma
c. Arteriovenous angioma
d. Arterial anomalies
Manuelidis (1950)
1. Telangiectasia
a. Primary
b. Secondary
2. Cavernous hemangioma
3. Venous hemangioma
4. Arteriovenous hemangioma
Zülch (1951)
1. Angioreticuloma
2. Malformation
a. Cavernous angioma
b. Racemous capillary angioma (telangiectasia)
c. Capillar et venous angioma (Sturge-Weber)
d. Venous angioma
e. Arteriovenous aneurysmatic angioma
Asenjo (1953)
I. Congenital lesions
A. Expansive malformation
a. Arteriovenous aneurysm
b. Arterial racemous aneurysm
c. Venous racemous aneurysm
B. Angiosis
d. Congenital arterial aneurysm
e. Meningeal varix
f. Sinus pericranii
II. Acquired lesions
A. Aneurysms
a. Arteriosclerotic
b. Mycotic
c. Syphilitic
B. Carotid-cavernous fistula
C. Traumatic aneurysms
III. Tumors
A. Hemangioblastoma
a. Benign
b. Malignant
B. von Hippel-Lindau disease
C. Angiomatous meningioma
Pluvinage (1954)
I. Angioreticuloma
II. Angioma
1. a. Cavernous angiomab. Telangiectasia
2. Sturge-Weber
3. Venous angioma
a. Cerebral varix
b. Racemous venous angioma
c. Peleton de veines (!)
4. Arterial angioma
a. Racemous arterial angioma
b. Arteriovenous aneurysm
Olivecrona – Ladenheim (1957)
Etiology
1. Acquired
2. Congenital
a. Anomalous arteriovenous
b. Angiomatous arteriovenous
Pathology
1. Cavernous
2. Racemous
a. Telangiectasia
b. Sturge-Weber
c. Venous racemous
d. Arterial racemous
e. Angiomatous arteriovenous
Russel – Rubinstein (1963)
1. Hemangioblastoma
2. Vascular malformation
a. Capillary telangiectasia
b. Cavernous angiomas
c. Venous and arteriovenous malformation
McCormick (1985) (in Fein and Flamm)
I. Angioblastoma
II. Angiomas
1. Venous angiomas 112 cases
2. Capillary angiomas (telangiectasias) 41 cases
3. AVM 11 cases
4. Cavernous angiomas 5 cases
5. Transitional 4 cases
Classification of Plastic Surgeons Kaplan (1983)
A. Stage 1 (undifferentiated capillary network)
1. Capillary
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