Superior petrosal vein varicosity presenting as an enhancing cerebellopontine angle lesion.
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Biomedical subjects
Publications and source records attributed to R F Spetzler.
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Pretruncal (perimesencephalic) nonaneurysmal hemorrhage is a benign form of subarachnoid hemorrhage (SAH). Angiographic changes of vasospasm are uncommon in patients with this type of hemorrhage, and if vasospasm is present, it is mild and focal. We report two patients with pretruncal nonaneurysmal SAH who developed severe and diffuse vasospasm, expanding the clinical spectrum of this type of SAH. The first patient was a 40-year-old woman who suffered pretruncal nonaneurysmal SAH. Angiography performed on the seventh day post hemorrhage showed diffuse and severe vasospasm affecting both the anterior and the posterior circulation. The patient was treated with hypervolemia, and she remained asymptomatic. Follow-up angiography showed resolution of the vasospasm. The second patient was a 67-year-old woman who suffered pretruncal nonaneurysmal SAH. The results of the initial angiography were normal. Repeat angiography on the ninth day post hemorrhage showed severe vasospasm in the anterior circulation and moderate vasospasm in the posterior circulation. Nine hours later, the patient developed transient dysphasia, and she was treated with hypervolemia. Three days later, a transcranial Doppler examination showed normalization of blood velocities. The presence of diffuse and severe vasospasm does not exclude a diagnosis of pretruncal nonaneurysmal SAH.
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Untreated giant intracranial aneurysms have a dismal natural history as a result of hemorrhage, cerebral compression, and thromboembolism. The poor prognosis of patients with giant aneurysms therefore warrants aggressive treatment. A surgical approach is chosen to maximize the operative exposure of the aneurysm and depends mainly on the aneurysm's location. Once exposed, vascular control of the aneurysm is required not only to manage an intraoperative rupture, but also to collapse the aneurysm, to increase working space, and to improve visualization of the anatomy. Hypothermic circulatory arrest may be indicated in select patients with complex posterior circulation aneurysms. Direct clipping of giant aneurysms, with meticulous preservation of parent and branch arteries, is the preferred method of occlusion. Unclippable aneurysms require alternative techniques (e.g., trapping, parent artery occlusion, excision, and aneurysmorrhaphy) that compromise parent arteries and may require revascularization to restore adequate cerebral blood flow. Giant aneurysms are complex lesions that demand thorough surgical planning, individualized strategies, and a multidisciplinary effort in specialized neurovascular centers.
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OBJECTIVE: To determine the prevalence of multiple neuraxis cavernous malformations in patients who initially presented with intramedullary spinal cord (IMSC) cavernous malformations without knowledge of cavernous malformations elsewhere in the neuraxis. METHODS: Hospital records and radiographic files were analyzed for 17 patients who subsequently underwent surgical resection of an IMSC cavernous malformation (histologically proven) and also underwent brain magnetic resonance imaging studies. These 17 patients represented a subset of 32 patients who underwent surgical resection of an IMSC cavernous malformation during the same period. RESULTS: Of 17 patients, 8 (47%) harbored multiple cavernous malformations. This group was composed of five women and three men (mean age, 35.9 yr). There were four Caucasian and four Hispanic patients. CONCLUSION: The prevalence of multiple cavernous malformations in the neuraxis seems to be increased in patients who harbor IMSC cavernous malformations. This finding has important implications for the evaluation and management of these patients and, in some cases, their family members.
OBJECTIVE: Rhinocerebral mucormycosis is a clinical syndrome resulting from an opportunistic infection caused by a fungus of the order Mucorales. The prognosis of rhinocerebral mucormycosis, once considered uniformly fatal, remains poor. Even with early diagnosis and aggressive surgical and medical therapy, the mortality rate is high. We present a patient with rhinocerebral mucormycosis involving the paranasal sinuses and cranial base who experienced long-term survival after multimodality treatment. Clinical characteristics of the disease are discussed, and the literature is reviewed. CLINICAL PRESENTATION: A 24-year-old diabetic man presented with invasive rhinocerebral mucormycosis involving the paranasal sinuses, right middle fossa, and right cavernous sinus. INTERVENTION: The patient underwent endovascular sacrifice of the involved carotid artery and radical resection of the cranial base, including exenteration of the cavernous sinus. Reconstruction with a local muscle flap was performed. He continued to receive intravenous and intrathecal administration of antibiotics. CONCLUSION: Long-term survival with invasive rhinocerebral mucormycosis is rare, but possible, with aggressive multimodality treatment, including carotid sacrifice for en bloc resection of the pathology, when indicated.
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.
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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OBJECT: This study was undertaken to evaluate the long-term angiographic outcome of surgically treated aneurysms, which is unknown. Specifically, the incidence of recurrent aneurysms, the fate of residual necks, and the de novo formation of aneurysms were evaluated. METHODS: One hundred two patients (80 females and 22 males; mean age 49 years; range 12-78 years) harboring a total of 167 aneurysms underwent late follow-up angiography; 160 aneurysms were surgically treated. Late angiographic follow-up review was obtained at a mean of 4.4 +/- 1.6 years postsurgery (range 2.6-9.7 years). Late follow-up angiography revealed two recurrent aneurysms (1.5%) of 135 clipped aneurysms without residua. Of 12 aneurysms with known residua, there were eight "dog-ear" residua, of which two (25%) enlarged. One hemorrhage was noted, yielding a hemorrhage risk of 1.9% per year. A second subgroup with broad-based residua revealed dramatic regrowth in three of four cases. Eight de novo aneurysms were found in six patients, for an annual risk of 1.8% per year. A history of multiple aneurysms was associated with de novo aneurysm formation (p = 0.049, chi-square analysis). CONCLUSIONS: This study confirms the long-term efficacy of aneurysm clip ligation. In addition, the authors found there is a small but significant risk of de novo aneurysm formation, particularly in patients with multiple aneurysms. Most residual aneurysm rests appear to remain stable, although a subset may enlarge or rupture. These findings support the rationale for late angiographic follow-up review in patients with aneurysms.
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OBJECT: The use of intraoperative angiography of the spine has become available to neurosurgeons as an adjunct in the management of complex spinal vascular malformations. These vascular malformations are rare, and the use of intraoperative angiography of the spine has not been well described. The authors report their recent experience with the use of this diagnostic modality. METHODS: Between 1995 and 1997, nine consecutive patients with type II or Type IV spinal arteriovenous malformations (AVMs) underwent intraoperative spinal angiography. The cervical spine was involved in three patients, the thoracic spine in five, and the thoracolumbar junction in one. In three patients, intraoperative spinal angiography revealed an unexpected finding (residual filling of the AVM). The results obtained using postoperative spinal angiography in all patients showed complete agreement with the intraoperative studies. No complications arose from obtaining the intraoperative spinal angiograms. CONCLUSIONS: Intraoperative spinal angiography is technically feasible, can be performed safely, and has adequate resolution. It detects unexpected residual AVM in one-third of cases.
OBJECT: The authors sought to determine the biomechanics of the occipitoatlantal (occiput [Oc]-C1) and atlantoaxial (C1-2) motion segments after unilateral gradient condylectomy. METHODS: Six human cadaveric specimens (skull with attached upper cervical spine) underwent nondestructive biomechanical testing (physiological loads) during flexion-extension, lateral bending, and axial rotation. Axial translation from tension to compression was also studied across Oc-C2. Each specimen served as its own control and underwent baseline testing in the intact state. The specimens were then tested after progressive unilateral condylectomy (25% resection until completion), which was performed using frameless stereotactic guidance. At Oc-C1 for all motions that were tested, mobility increased significantly compared to baseline after a 50% condylectomy. Flexion-extension, lateral bending, and axial rotation increased 15.3%, 40.8%, and 28.1%, respectively. At C1-2, hypermobility during flexion-extension occurred after a 25% condylectomy, during axial rotation after 75% condylectomy, and during lateral bending after a 100% condylectomy. CONCLUSIONS: Resection of 50% or more of the occipital condyle produces statistically significant hypermobility at Oc-C1. After a 75% resection, the biomechanics of the Oc-C1 and C1-2 motion segments change considerably. Performing fusion of the craniovertebral junction should therefore be considered if half or more of one occipital condyle is resected.