Brief History of Cavernous Malformations

The first description of cavernous malformation was made by pioneer dermatologist Joseph Jakob Plenck in 1776. Although the cavernous malformation was described previously with varying degrees of credibility and probability to actually be the cavernous malformation under the terms: Labii tuberculum atrum cruentum (M.A. Severinus), Telangiectasis (C.F. Graefe), Aneurysm by anastomosin (J. Bell), fungus haematodes (W. Hey), tumeurs erectiles (Dupuytren), substantia cavernosa (Boyer), tumeurs fongueses sanguines, Aftermilzen, placenta-like tissue, etc. [1] it was Carl Freiherr von Rokitansky (Karel Rokytanský) to be the first who presented a detailed pathological description of cavernous malformation (“Cavernose Geschwulst”) in his Handbook of special pathological anatomy (“Handbuch der speciellen patologischen Anatomie”) published in 1844 [2]. Between 1851 and 1854 Robert Ludwig Carl Virchow defined differences between vascular lesions [1] and in 1863 he presented the first complex classification of vascular malformations [3]. In 1854 Hubert von Luschka published a first comprehensive report of brain cavernoma which was found in the brain of a man who committed suicide with such a detailed description that there can be no doubt that it was indeed a cavernoma [4]. The first case report with successful surgical removal of brain cavernoma was introduced by Bremer and Carson in 1890 [5]. The first pathological description of a man with multiple cavernous angioma was reported by Ohlmacher in Gallipolis, Ohio in 1899 [6]. The first overview was presented by Dandy in 1928 in which he described 5 of his own cases and collected 44 previously published cases that described the typical pathological features of the disease. He identified basic clinical signs such as predisposition to bleed causing focal neurological deficits and epilepsy as a major clinical manifestation and also described basic technical aspects of cavernoma removal, concluding that: “the cavernous angiomas… should be treated surgically by complete removal of the solid tumor together with a margin of contiguous brain tissue.” [7]. Also in 1928 Cushing and Bailey published their book Tumors Arising from Blood Vessels of the Brain and classified cavernous malformations as a solid subtype of hemangioblastomas [8].