The endeavours of general surgeons in dealing with the dangerous and disfiguring extracranial vascular malformations (scalp, external ear, eyelids, orbits, cheeks, lips, tongue, palate and neck) are most informative for the interested neurosurgeon (Beck, Berger, Billroth, Brodie, von Bruns, Bryant, Busch, Caradec, Clairmont, Dalrymple, Dupuytren, Emanuel, Enderlen, German, Goldmann, Heineke, Krause, Lefort, Lieblein, Nélaton, Pilz, Roth, Russell, Schwalbe, Schwartz). Their methods of treatment have, in the past, included:
1) Injection of the lesion with: ferrous chloride, glycerin, tannin, chlorzin, carbonic acid, alcohol,
2) Electrocauterization,
3) Ligation,
4) Extirpation.
The variety of modern therapy of external vascular malformations (Williams 1983) shows that therapeutic difficulties still remain in the treatment of these easily approachable lesions:
1) Corticotherapy (new born children),
2) Radiotherapy,
3) Electrocoagulation,
4) Cryotherapy,
5) Surgery,
6) Use of laser beam,
7) Embolization.
As in neurosurgery, the advice of most plastic surgeons is that simple ligation of feeding vessels is inadequate and inadvisable.
Intracranial Angiomas
The Contributions of Virchow and his Contemporaries
After exhaustive research work on cavernomas of the liver, Rokitansky (1842–46) came to the conclusion that these were either benign or malignant tumors independent of the surrounding vascular system. Volume 6 of Virchow’s Archive (1854) contains 3 remarkable papers: Esmarch (pp. 34–57): “Über cavernöse Blutgeschwülste”, Luschka (pp. 458–470): “Cavernöse Blutgeschwülste des Gehirns” and Virchow (pp. 526–554): Über cavernöse (erectile) Geschwülste und Teleangiektasien.” Esmarch und Luschka fully supported the neoplasia hypothesis of Rokitansky.
Luschka provided one of the earliest descriptions of an intracranial arteriovenous anomaly in a patient with a frontal cavernoma.
Luschka recognized two types of “Blut-Geschwülste”:
1) Telangiectases (non neoplastic) arising due to a metamorphosis of capillary systems.
2) Cavernous tumors (neoplastic) containing large blood-filled compartments.
The young Virchow, who was involved with research into infection of blood vessels, also published (1851) a remarkable paper concerning “the dilatation of small vessels”. In this paper he described and discussed thoroughly his own observations and thoughts and clearly refuted the hypothesis of Rokitansky. In 1863 Virchow published a comprehensive study which may be called the first real milestone in the history of the AVM. In the 3rd volume of his monograph, 200 pages (pp. 306–496) are devoted to the phenomenon of the physiological and pathological changes of blood vessels in all organs. His descriptions profoundly contradicted contemporary opinion. He described telangiectases, venous, arterial, arteriovenous and cystic angiomas (nowadays angioblastomas), and their transitional types, and discussed in detail the pathogenesis of these malformations. He reflected on the atlas of Cruveilhier and the pioneering work of John Bell (The Principle of Surgery, London 1826, Volume 3, pp. 326–383, First Edition, London 1796). Bell described cavernoma, AVM and angioblastoma but gave all of them the nomenclature of “Aneurysm per Anastomosis”. Virchow said: “This description is still perfectly valid, (p. 328): Aneurysm per anastomosis is an entire change of structure; it is a dilatation of veins, in which they are forced and enlarged by the diseased action of their corresponding arteries. Those happen in consequence of original malformation, a violent action of arteries, and a mutual enlargement of arteries and veins, while the intermediate substance of the part is slowly distended into large intermediate cells, which are dilated to formidable reservoirs of blood. – The blood is poured into the cells of such a tumor by innumerable arteries: from these the blood is continually following into veins, which receive it with such patent orifices etc. The veins form a conspicious part of such a tumor, but the intermediate cells are an appreciable part of the structure… (p. 397). All this proves that it is a tissue of small arteries and veins; it fills not like a varix slowly; its filling is by distinct thrombs; it is filled by its small and numerous arteries, and its swelling is (like the erection of the penis) produced by the pulsation of the arteries, stroke after stroke, pouring out their blood into cells.”
“The tumor is a congeries of active vessels and the cellular substance through which these vessels are expanded, resembles the cellular parts of the penis, the gills of a turkey cock or the substance of the placenta, spleen worms.”
It is interesting to speculate as to whether Bell was describing a cavernoma or an AVM. It certainly sounds like the modern description of an AVM and its nidus.
Virchow (1854), cited Gerdy (1852), who differentiated eight types of “erectile tumors” and noted the great number of publications concerning the “erectile tumors” and the difficulties with their classification. He preferred the term of “angioma” which was introduced by J. Hughes Bennet (1854) instead of the term of “angionoma” which was advocated by Follin (1861). He credited to Plenck (1776) the term “cavernoma”, a nomenclature well recognized in the German literature (Meckel 1818).
Cushing and Bailey (1928) concluded, quite wrongly, that Virchow believed in the neoplastic nature of vascular malformations as proposed by Rokitansky. This error was most likely due to difficulties with translation of the original papers.
Virchow (1863) divided angiomas into cavernous, simple telangiectatic, racemose, and lymphatic types. Racemose angiomas were divided further into arterial and venous types.
Page 474 of Virchow’s 3rd Volume (1863) relates to a case of a large extracranial parietooccipital AVM in a man from Florence and described by Vidus Vidius in 1665. Virchow commented that this type of malformation originates through accommodation between artery and vein with consequent dilatation of them (arteriectasie and phlebectasie, p. 471). They are of congenital origin (p. 475). They may grow or spontaneously regress (p. 482). The following nomenclature has been used: aneurysma per anastomosis (Bell) or aneurysma anastomoseon (v. Walther), aneurysma per transfusionem (Dupuytren 1834) and other authors used the term of aneurysma arteriovenosum, or aneurysma varicosum.
Virchow argued (p. 472) that aneurysms would not arise as a result of arteriovenous communication in traumatic cases, therefore the best term would be aneurysma spurium arteriovenosum.
Virchow’s main concern was not so much nomenclature as the pathophysiology of the lesions. The founder of cellular pathology had a profound interest in pathophysiology. He performed injection studies on the pregnant uterus and placenta and was fascinated by the temporary but enormous increase in capacity of vessels during gestation. In 1851 he spoke of “The physiologic paradigm in the corpora cavernosa of sex organs and the paradigm of pathology in cavernoma and telangiectasis”, and further questioned as to whether one type of angioma can transform into another by changes in flow and pressure or by cellular proliferation.
Early Clinical Observations on Intracranial AVMs
Pfannenstiel (1887) and Kaufmann (1897) observed young (22 and 23 years) primipara patients, who died with acute cerebral symptoms. Autopsy study showed a ruptured varicose anomaly of left thalamus opticus and the vena Galeni in one case, and a ruptured varicose anterior callosal anomaly in another.
D’Arcy Power (1888) found, a large AVM in the left sylvian fissure at autopsy on a 20-year-old man who had suffered a hemiplegic stroke and died.
Steinheil (1894) described the history and pathologicoanatomical findings in a patient (59 years) with a large right frontal AVM which drained partially to the vein of Galen. He may thus be credited as being among the earliest to describe the symptomatology of the disease.
Rizzoli 1873 observed a right occipital pulsating swelling in a 9-year-old