Distinctly less common, yet still well documented, are certain syndromes which include vascular malformations of the brain, spinal cord, or meninges as an integral component. Some of these, such as the Sturge-Weber syndrome and the Louis-Bar syndrome, fall outside the scope of this review. A third, recently described and apparently rare syndrome of disseminated hemangiomatosis13 will be included in this discussion of small vascular hamartomas of the central nervous system. The Rendu-Osler-Weber syndrome, although at times associated with telangiectasis in the central nervous system, will not be considered in detail.17,70,89 The Sturge-Weber syndrome is excluded from this study.37,87 True vascular neoplasms, the hemangioblastomas, are also excluded from detailed consideration, but will be briefly discussed in relation to cerebellar and spinal cord malformations.55,71,72,85,89,94
Margolis et al. first called attention to a group of small vascular malformations when they reported four cases in which this type of lesion caused intracerebral hemorrhage.64 Russell, in reporting her observations in relation to various causes of intracranial hemorrhage, popularized the descriptive term “cryptic” for these small A–V malformations,88 namely those which are clinically silent and which measure less than 2–3 cm. in maximum size. She found 21 such malformations that caused hemorrhage.
There have been many single case reports and small series.1,2,4–6,8–18,21–23,25–28,30,31,34,36,39–42,43,45,48–51,57,59,61,63–66,70,71,73–77,79–80,82,91–93,97,98,101,103,104,107 We have studied 48 “cryptic” vascular malformations. Only four other series of comparable size have been published.19,20,52,54,106 This report of our cases includes a comprehensive review of all the cases in the references cited above.
Reviews of the many varied classifications of vascular malformation of the nervous system have been made by Noran,75 Raynor and Kingman,85 Russell and Rubinstein,89 and by Olivecrona and Ladenheim.78 This aspect will not be discussed in detail here, but has been examined in detail elsewhere.67 The terms telangiectasis, varix, cavernous angioma, venous angioma, and arteriovenous malformations are regarded as comprehensive enough to include the various types of “cryptic” malformations that we, as well as others, have encountered. The term “angiomatous meningioma” used by Noran75 is, in our opinion, a misnomer. This lesion is not a meningioma, but rather a vascular malformation which is confined to the leptomeninges.
CourvilleC. B. Encephalic lesions in hereditary hemorrhagic telangiectasis (Rendu-Osler-Weber disease); Report of case with the disclosure of microscopic telangiectasis in the leptomeninges. Bull. Los Angeles neurol. Soc.195722:28–35.CourvilleBull. Los Angeles neurol. Soc.22:28–35.
CushingH.BaileyP. Tumors arising from the blood-vessels of the brain; Angiomatous malformations and hemangioblastomas. Springfield, Ill.: Charles C Thomas1928219 pp.CushingBaileyTumors arising from the blood-vessels of the brain; Angiomatous malformations and hemangioblastomas.
DavidoffL. M.in discussion of paper by Cowen and Wolf.22Davidoff22
GlobusJ. H.DoshayL. J. Venous dilatations and other intraspinal vessel alterations, including true angiomata, with signs and symptoms of cord compression. A report of four cases with a review of the literature. Surg. Gynec. Obstet.192948:345–366.GlobusDoshaySurg. Gynec. Obstet.48:345–366.
GurdjianE. S.ThomasL. M.HermanL. H.PortnoyH. D. Angiographic findings in 300 cases of aneurysms, arteriovenous malformations, and subarachnoid hemorrhage. In: Intracranial aneurysms and subarachnoid hemorrhage. FieldsW. S.SahsA. L.Springfield, Ill.: Charles C Thomas1965. (see pp. 143–183).GurdjianThomasHermanPortnoyIntracranial aneurysms and subarachnoid hemorrhage.
McCormickW. F. Discussion of paper by Gurdjian et al.42McCormicket al.42
RoizinL.GoldG.BermanH. H.BonafedeV. I. Congenital vascular anomalies and their histopathology in Sturge-Weber-Dimitri Syndrome (naevus flammeus with angiomatosis and encephalosis calcificans). J. Neuropath. exp. Neurol.195918:75–97.RoizinGoldBermanBonafedeJ. Neuropath. exp. Neurol.18:75–97.
Presented in part at meeting of the Tennessee State Pathology Society, Memphis, Tennessee, April 9, 1962, and at meeting of the Iowa Association of Pathologists. Iowa City, Iowa, December 12, 1964.