Other rare vascular anomalies of the cord In the Klippel-Trenaunay-Weber syndrome, a sometimes extensive vascular malformation of the spinal cord is associated with a cutaneous vascular nevus overlying the AVM or in a limb supplied by the affected cord level; when the malformation lies in the low cervical region, there may be enlargement of finger, hand, or arm (the hemangiectatic hypertrophy of Parkes Weber; neurofibromatosis is another cause of limb enlargement). Spinal segmental and tract lesions may occur at any age, but the patients we have observed were young adults. Vascular occlusion or hemorrhage was responsible for the myelopathy. Some of these vascular lesions have been treated by defining and ligating their feeding vessels. In a few reported cases it has been possible to extirpate the entire lesion, especially if it occupied the surface of the cord.
Other rare vascular anomalies of the spinal cord include aneurysm of a spinal artery with coarctation of the aorta and telangiectasia of the cord, which may or may not be associated with the hereditary hemorrhagic type of Osler-Rendu-Weber. Over the years, the authors have had under their care patients with the latter disease who developed acute hemorrhagic lesions of the spinal cord. We have also observed several cavernous hemangiomas of the spinal cord. In two of our patients, an angiographically negative solitary cavernous angioma was the source of an acute partial transverse myelopathy. The lesions were clearly demonstrable only in the T2-weighted MR images. McCormick and associates have reported similar cases. Characteristically, the angiomas cause partial syndromes and are followed by considerable recovery of function just as when they occur in the brain. There may or may not be blood in the CSF. Rarely, the same disease is responsible for one or more hemorrhagic lesions of the brain. The association of cavernous angiomas with arteriovenous fistulas of the lung is a rare finding, and the latter may be a source of brain abscess. In coarctation of the aorta, the circulation to the lower part of the spinal cord may be deficient, with resultant paresis of the legs, sensory loss, and sphincteric impairment. Or there may be intracranial subarachnoid hemorrhage from a ruptured saccular aneurysm, an associated condition in a small number of cases.
Treatment The rate of progression of the myelopathy from these various lesions varies greatly. In some cases, as already noted, it may become a matter of some urgency to reverse the venous congestion and avoid infarction of the cord. Other lesions require a more measured approach. By occluding the feeding artery of a spinal AVM or fistula, which is often single, and thereby eliminating the excess pressure in veins, the course of disease can be arrested and any pain reduced (Symon et al). In most of our patients, there has been postoperative improvement in the neurologic deficit over a few weeks or months. In cases of the larger racemose AVMs, stripping the enlarged veins along the dorsal cord is no longer considered necessary and may be dangerous. Increasingly, one resorts to obliteration of a fistula or a reduction of the AVM by the use of endovascular techniques and various types of embolic particles. The procedure is long and painstaking, for the operator must identify and embolize all the feeding vessels of the malformation; general anesthesia is required in most cases.