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Robert F. Spetzler, Paul W. Detwiler, Howard A. Riina and Randall W. Porter

The literature on spinal vascular malformations contains a great deal of confusing terminology. Some of the nomenclature is inconsistent with the lesions described. Based on the experience of the senior author (R.F.S.) in the treatment of more than 130 spinal cord vascular lesions and based on a thorough review of the relevant literature, the authors propose a modified classification system for spinal cord vascular lesions.

Lesions are divided into three primary or broad categories: neoplasms, aneurysms, and arteriovenous lesions. Neoplastic vascular lesions include hemangioblastomas and cavernous malformations, both of which occur sporadically and familially. The second category consists of spinal aneurysms, which are rare. The third category, spinal cord arteriovenous lesions, is divided into arteriovenous fistulas and arteriovenous malformations (AVMs). Arteriovenous fistulas are subdivided into those that are extradural and those that are intradural, with intradural lesions categorized as either dorsal or ventral. Arteriovenous malformations are subdivided into extradural-intradural and intradural malformations. Intradural lesions are further divided into intramedullary, intramedullary-extramedullary, and conus medullaris, a new category of AVM.

This modified classification system for vascular lesions of the spinal cord, based on pathophysiology, neuroimaging features, intraoperative observations, and neuroanatomy, offers several advantages. First, it includes all surgical vascular lesions that affect the spinal cord. Second, it guides treatment by classifying lesions based on location and pathophysiology. Finally, it eliminates the confusion produced by the multitude of unrelated nomenclatural terms found in the literature.

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Daniel L. Barrow

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Paul W. Detwiler, Randall W. Porter, Stephen W. Coons, Robert F. Spetzler, Carlos A. Carrion and Harold L. Rekate

✓ Infantile myofibromatosis involving the skull is a benign disease if there is a solitary lesion. However, the multifocal form with skull involvement may portend a lethal course in the 1st year of life if there is involvement of the heart, lungs, or gastrointestinal tract. The authors report the case of a 3-year-old boy with an enlarging left parietal skull lesion that had been present since infancy. Increasing pain and the need to obtain tissue for diagnosis led to resection of the lesion by means of a small craniectomy. Further evaluation revealed no other lesions. A distinctly rare disease is presented, and the need for staging in children younger than 2 years of age is suggested to rule out cardiac, pulmonary, or gastrointestinal involvement.

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Patrick P. Han, Nicholas Theodore, Randall W. Porter, Paul W. Detwiler, MichaeL T. Lawton and Robert F. Spetzler

✓ The authors report a patient in whom a subdural hematoma developed from a Type I spinal arteriovenous malformation (AVM). The patient became symptomatic with back pain, and magnetic resonance imaging revealed a spinal subdural hematoma. Selective spinal angiography, however, failed to demonstrate a pathological process. The patient underwent exploratory laminoplasty that revealed a subdural extraarachnoid hematoma with an underlying Type I spinal AVM, which was surgically obliterated. The patient recovered completely. Subdural hematomas that affect the spine are rare. Although a negative result was obtained using selective spinal angiography, exploratory surgery should be considered for the evacuation of a subdural hematoma and possibly for the definitive treatment of a spinal AVM.

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Randall W. Porter, Paul W. Detwiler, Robert F. Spetzler, Michael T. Lawton, Jonathan J. Baskin, Patrick T. Derksen and Joseph M. Zabramski

Object. In this study the authors review surgical experience with cavernous malformations of the brainstem (CMBs) in an attempt to define more clearly the natural history, indications, and risks of surgical management of these lesions.

Methods. The authors retrospectively reviewed the cases of 100 patients (38 males and 62 females; mean age 37 years) harboring 103 lesions at treated a single institution between 1984 and 1997. Clinical histories, radiographs, pathology records, and operative reports were evaluated. The brainstem lesions were distributed as follows: pons in 39 patients, medulla in 16, midbrain in 16, pontomesencephalic junction in 15, pontomedullary junction in 10, midbrain—hypothalamus/thalamus region in two patients, and more than two brainstem levels in five. The retrospective annual hemorrhage rate was most conservatively estimated at 5% per lesion per year. Standard skull base approaches were used to resect lesions in 86 of the 100 patients. Intraoperatively, all 86 patients were found to have a venous anomaly in association with the CMB. Follow up was available in 98% (84 of 86) of the surgical patients. Of these, 73 (87%) were the same or better after surgical intervention, eight (10%) were worse, and three (4%) died. Two surgical patients were lost to follow-up review. Incidences of permanent or severe morbidity occurred in 10 (12%) of the surgically treated patients. The average postoperative Glasgow Outcome Scale score for surgically treated patients was 4.5, with a mean follow-up period of 35 months.

Conclusions. The natural history of CMBs is worse than that of cavernous malformations in other locations. These CMBs can be resected using skull base approaches, which should be considered in patients with symptomatic hemorrhage who harbor lesions that approach the pial surface. Venous anomalies are always associated with CMBs and must be preserved.

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Paul W. Detwiler, Randall W. Porter, Timothy R. Harrington, Volker K. H. Sonntag and Robert F. Spetzler

✓ Vertebral artery tortuosity and loop formation are rare causes of cervical radiculopathy. The authors present the case of a 70-year-old man with 9 years of progressive right-sided cervical and scapular pain but no history of trauma. Computerized tomography myelography and magnetic resonance imaging revealed an ovoid mass in the right C3–4 intervertebral foramen. The patient underwent a right C-3 and C-4 hemilaminectomy and a complete C3–4 facetectomy. A pulsatile vascular structure was found compressing the right C-4 nerve root. The bone overlying the vascular structure was removed, producing decompression of the nerve root. Immediate postoperative angiography showed that this lesion was a focal vertebral artery loop. The patient's symptoms resolved after surgery, supporting the use of vascular decompression of a cervical nerve root compressed by a vertebral artery loop for the relief of radicular symptoms.