Subarachnoid hemorrhage: the first 24 hours. A surgeon's perspective.

BACKGROUND: The first 24 h in the management of aneurysmal subarachnoid hemorrhage (SAH) represent a critical time period for medical intervention. METHODS: We review the current literature and discuss our current clinical practices related to management of acute SAH. RESULTS: A brief objective review of the current evidence along with a subjective overview of the authors practices in the management of aneurysmal subarachnoid hemorrhage in the first 24 h is outlined. CONCLUSIONS: The first 24 h following aneurysmal subarachnoid hemorrhage is a critical time period in which medical and interventional management paradigms continue to evolve.

A community-based cohort of 201 consecutive patients with primary Sjögren's syndrome in Israel: Ashkenazi patients compared with those of Sephardic descent.

OBJECTIVE: To determine the spectrum and prevalence of the varied manifestations, associated conditions and laboratory abnormalities of patients with primary Sjögren's syndrome in Israel and compare them between individuals of Sephardic and Ashkenazi descent and with data from the literature. METHODS: A retrospective study of a cohort of 201 consecutive patients diagnosed and followed at a single academic medical center. All cases were diagnosed using stringent criteria according to the American European Concensus Group including a labial minor salivary gland biopsy in all cases. RESULTS: Patients' mean age was 57 years and 84% were women. Overall, more than 98% of patients had sicca symptoms of dry eyes and mouth. About 35% of the cohort had hematological manifestations--primarily immune cytopenias, protein immunoelectrophoresis abnormalities and lymphoma. About 20% had associated neurological conditions (not only peripheral but often central nervous system) and 15% had pulmonary involvement. In addition, thyroid disease, liver disease, vascular or cutaneous manifestations, synovitis, ocular and renal disease could be found. In fact, the presenting manifestation was extraglandular or an abnormal test result in 39% of the patients. CONCLUSION: No significant differences were found in glandular or extraglandular manifestations or laboratory test results between Ashkenazi and Sephardic patients, despite their genetic differences. A negative history of sicca symptoms effectively rules out primary Sjögren's syndrome in this cohort. These symptoms may not be volunteered by patients and the large variety of extraglandular involvement patterns and associated conditions observed may dominate the patient's presentation, and mandate physicians' awareness and a high index of suspicion for a timely diagnosis.

Animal models of spinal cord injury for evaluation of tissue engineering treatment strategies.

Tissue engineering approaches to spinal cord injury (SCI) treatment are attractive because they allow for manipulation of native regeneration processes involved in restoration of the integrity and function of damaged tissue. A clinically relevant spinal cord regeneration animal model requires that the model mimics specific pathologic processes that occur in human SCI. This manuscript discusses issues related to preclinical testing of tissue engineering spinal cord regeneration strategies from a number of perspectives. This discussion includes diverse causes, pathology and functional consequences of human SCI, general and species related considerations, technical and animal care considerations, and data analysis methods.

A simple technique for minor salivary gland biopsy appropriate for use by rheumatologists in an outpatient setting.

Minor salivary gland biopsy is a potentially simple procedure with high diagnostic value used primarily in the diagnosis of Sjögren's syndrome. We summarise our experience performing a simplified biopsy procedure, which is modified to allow its use by rheumatologists and other non-surgical physicians in an outpatient setting.

Small cerebral aneurysms presenting with symptoms other than rupture.

BACKGROUND: Recent natural history studies have suggested that unruptured intracranial aneurysms smaller than 1 cm have a low risk of rupture. Symptomatic aneurysms may be underrepresented in natural history studies because they are preferentially treated. The authors compared the number of patients with symptoms caused by unruptured intracranial aneurysms smaller than 1 cm treated surgically at their institution with similar patients enrolled in the International Study of Unruptured Intracranial Aneurysms (ISUIA) from their institution over the same time period. METHODS: The records of all unruptured aneurysms treated surgically at the Mayo Clinic from 1980 through 1991 were reviewed. There were 97 patients with 117 unruptured aneurysms smaller than 1 cm by angiography. Aneurysms with a history of rupture or larger than 1 cm on cross-sectional imaging were excluded from analysis. The presence and characteristics of symptoms directly attributable to the aneurysm were recorded. Comparison was made with patients from the Mayo Clinic enrolled in the ISUIA retrospective natural history cohort over the same time period. RESULTS: Of the 97 patients studied, 15 presented with symptoms other than rupture (15.5%). The symptoms were third nerve deficit (seven patients), cerebral ischemia owing to emboli originating from within the aneurysm (five patients), and visual acuity loss (three patients). Eleven other aneurysms had possibly but not definitively caused symptoms; these were considered asymptomatic. No patient from the Mayo Clinic enrolled in the retrospective cohort of the ISUIA had a symptomatic aneurysm smaller than 1 cm on both angiography and cross-sectional imaging. CONCLUSIONS: Unruptured intracranial aneurysms smaller than 1 cm occasionally present with neurologic symptoms. These symptoms are typically owing to mass effect on the second and third cranial nerves or cerebral ischemia as a result of emboli originating from within the aneurysm. Patients with symptomatic unruptured aneurysms less than 1 cm at the Mayo Clinic were preferentially treated. Although existing natural history data may be applied to most unruptured aneurysms, small symptomatic aneurysms may be underrepresented in natural history studies.

Remote hemorrhage.

The authors report a case of infra- and supratentorial intracerebral hemorrhage complicating the postoperative course of a patient who had undergone surgical removal of a cervical schwannoma with an hourglass configuration. To their knowledge, this is the first case in which this neurosurgical procedure was followed by such a complication. Possible mechanisms are discussed; however, pathological events leading to this complication are unclear. The development of new neurological deficits not attributable to the surgical procedure should suggest this possibility.

Post-traumatic cerebrospinal fluid leakage.

Posttraumatic cerebrospinal fluid (CSF) leakage frequently complicates skull base fractures. While most CSF leaks will cease without treatment, patients with persistent CSF leaks may be at increased risk for meningitis, and many will require surgical intervention. We reviewed the medical records of 51 patients treated between 1984 and 1998, with CSF leaks that persisted for 24 hours or longer after head trauma. Twenty-eight patients (53%) had spontaneous resolution of the leakage at an average of 5 days. Twenty-three patients (47%) required surgery. Eight patients (16%) had occult leaks presenting with recurrent meningitis at an average of 6.5 years posttrauma. Forty-three (84%) patients with CSF leaks had an associated skull fracture, most commonly involving the frontal sinus, while only 18 patients (35%) had parenchymal brain injury or extra-axial hematoma. Eight patients (16%) had delayed leaks at an average of 13 days posttrauma. Among patients with clinically evident CSF leakage the frequency of meningitis was 10% with antibiotic prophylaxis, and 21% without antibiotic prophylaxis. Thus, prophylactic antibiotic administration halved risk of meningitis. A variety of surgical approaches was used, with minimal morbidity. Three of 23 surgically treated patients (13%) required additional surgery for continued leakage. Patients with CSF leaks that persist greater than 24 hours are at risk for meningitis, and many will require surgical intervention. Prophylactic antibiotics may be effective and should be considered in this group of patients. Patients with skull fractures involving the skull base or frontal sinus should be followed for delayed leakage. Surgical outcome is excellent.

Awake craniotomy for aggressive resection of primary gliomas located in eloquent brain.

OBJECTIVE: To determine with intraoperative neurologic and language examinations the maximal tumor resection achievable with acceptable postoperative neurologic dysfunction in patients undergoing awake stereotactic glial tumor resection in eloquent regions of the brain. PATIENTS AND METHODS: Between October 1995 and December 2000, 65 patients underwent frameless stereotactic resection of glial tumors located in functioning tissue. During the resection, continuous examinations by a neurologist and speech pathologist were performed. The goal of surgery was to resect the maximum neurologically permissible tumor volume defined on preoperative T2 imaging. Tumor resection was stopped at the onset of neurologic dysfunction. Novel segmentation software was used to measure tumor cytoreduction based on pre- and postoperative magnetic resonance imaging. All patients underwent 3-month postoperative neurologic examinations to determine functional outcomes. RESULTS: The cortical and subcortical white matter tracts at risk for injury were the left frontal operculum in 15 patients, the central lobule in 38, the insula in 11, and the left angular gyrus in 1. Thirty-four (52%) had a greater than 90% reduction in T2 signal postoperatively. In 26 patients thought to have low-grade tumors based on preoperative imaging, 12 proved to have grade 3 gliomas. Forty-eight patients (74%) developed intraoperative deficits; 34 (71%) recovered to a modified Rankin grade of 0 or 1 at 3 months postoperatively, 11 (23%) achieved a modified Rankin grade of 2, and 3 patients (6%) achieved a modified Rankin grade of 3 or 4 at 3-month follow-up. There was no operative mortality; 17 patients (26%) died from tumor progression during the follow-up period. CONCLUSIONS: Combining frameless computer-guided stereotaxis with cortical stimulation and repetitive neurologic and language assessments facilitates tumor resection in functioning brain regions. Resecting tumor until the onset of neurologic deficits allows for a good functional recovery. Imaging software can objectively and accurately measure preoperative and postoperative tumor volumes.

Results of combined stereotactic radiosurgery and transarterial embolization for dural arteriovenous fistulas of the transverse and sigmoid sinuses.

OBJECT: Most dural arteriovenous fistulas (DAVFs) of the transverse and sigmoid sinuses do not have angiographically demonstrated features associated with intracranial hemorrhage and, therefore, may be treated nonsurgically. The authors report their experience using a staged combination of radiosurgery and transarterial embolization for treating DAVFs involving the transverse and sigmoid sinuses. METHODS: Between 1991 and 1998, 25 patients with DAVFs of the transverse and/or sigmoid sinuses were treated using stereotactic radiosurgery; 22 of these patients also underwent transarterial embolization. Two patients were lost to follow-up review. Clinical data, angiographic findings, and follow-up records for the remaining 23 patients were collected prospectively. The mean duration of clinical follow up after radiosurgery was 50 months (range 20-99 months). The 18 women and five men included in this series had a mean age of 57 years (range 33-79 years). Twenty-two (96%) of 23 patients presented with pulsatile tinnitus as the primary symptom; two patients had experienced an earlier intracerebral hemorrhage (ICH). Cognard classifications of the DAVFs included the following: I in 12 patients (52%), IIa in seven patients (30%), and III in four patients (17%). After treatment, symptoms resolved (20 patients) or improved significantly (two patients) in 96% of patients. One patient was clinically unchanged. No patient sustained an ICH or irradiation-related complication during the follow-up period. Seventeen patients underwent follow-up angiographic studies at a mean of 21 months after radiosurgery (range 11-38 months). Total or near-total obliteration (> 90%) was seen in 11 patients (65%), and more than a 50% reduction in six patients (35%). Two patients experienced recurrent tinnitus and underwent repeated radiosurgery and embolization at 21 and 38 months, respectively, after the first procedure. CONCLUSIONS: A staged combination of radiosurgery and transarterial embolization provides excellent symptom relief and a good angiographically verified cure rate for patients harboring low-risk DAVFs of the transverse and sigmoid sinuses. This combined approach is a safe and effective treatment strategy for patients without angiographically determined risk factors for hemorrhage and for elderly patients with significant comorbidities.

Fatal progression of posttraumatic dural arteriovenous fistulas refractory to multimodal therapy. Case report.

The authors report the case of a man who suffered from progressive, disseminated posttraumatic dural arteriovenous fistulas (DAVFs) resulting in death, despite aggressive endovascular, surgical, and radiosurgical treatment. This 31-year-old man was struck on the head while playing basketball. Two weeks later a soft, pulsatile mass developed at his vertex, and the man began to experience pulsatile tinnitus and progressive headaches. Magnetic resonance imaging and subsequent angiography revealed multiple AVFs in the scalp, calvaria, and dura, with drainage into the superior sagittal sinus. The patient was treated initially with transarterial embolization in five stages, followed by vertex craniotomy and surgical resection of the AVFs. However, multiple additional DAVFs developed over the bilateral convexities, the falx, and the tentorium. Subsequent treatment entailed 15 stages of transarterial embolization; seven stages of transvenous embolization, including complete occlusion of the sagittal sinus and partial occlusion of the straight sinus; three stages of stereotactic radiosurgery; and a second craniotomy with aggressive disconnection of the DAVFs. Unfortunately, the fistulas continued to progress, resulting in diffuse venous hypertension, multiple intracerebral hemorrhages in both hemispheres, and, ultimately, death nearly 5 years after the initial trauma. Endovascular, surgical, and radiosurgical treatments are successful in curing most patients with DAVFs. The failure of multimodal therapy and the fulminant progression and disseminated nature of this patient's disease are unique.

Cerebrovascular manifestations in 321 cases of hereditary hemorrhagic telangiectasia.

BACKGROUND AND PURPOSE: Patients with hereditary hemorrhagic telangiectasia (HHT) are at risk for developing cerebral vascular malformations and pulmonary arteriovenous fistulae. We assessed the risk of neurological dysfunction from these malformations and fistulae. METHODS: Three hundred twenty-one consecutive patients with HHT seen at a single institution over a 20-year period were studied. Any evidence of prior neurological symptoms or presence of an intracranial vascular malformation was recorded. All cases of possible cerebral arteriovenous malformation were confirmed by conventional arteriography. RESULTS: Twelve patients (3.7%) had a history of cerebral vascular malformations. Ten patients had arteriovenous malformations, 1 had a dural arteriovenous fistula, and 1 had a cavernous malformation. Seven patients (2.1%) presented with intracranial hemorrhage, 2 presented with seizures alone, and 3 were discovered incidentally. The average age at the time of symptomatic intracranial hemorrhage was 25.4 years. All patients with a history of intracranial hemorrhage were classified as Rankin grade I or II at a mean follow-up interval of 6.0 years. A history of cerebral infarction or transient ischemic attack was found in 29.6% of patients with HHT and a pulmonary arteriovenous fistula. CONCLUSIONS: The risk of intracranial hemorrhage is low among people with HHT. Furthermore, a majority of these patients have a good functional outcome after hemorrhage. The data do not suggest a compelling indication for routine screening of patients with HHT for asymptomatic cerebral vascular malformations. By comparison, pulmonary arteriovenous fistulae are a much more frequent cause of neurological symptoms in this population.

Ischemic complications of surgery for anterior choroidal artery aneurysms.

OBJECT: Anterior choroidal artery (AChA) aneurysms account for 4% of all intracranial aneurysms. The surgical approach is similar to that for other supraclinoid carotid artery lesions, but surgery may involve a higher risk of debilitating ischemic complications because of the critical territory supplied by the AChA. METHODS: Between 1968 and 1999, 51 AChA aneurysms in 50 patients were treated using craniotomy and clipping at the Mayo Clinic. There were 22 men (44%) and 28 women (56%) whose average age was 53 years (range 27-79 years). Twenty-four AChA aneurysms (47%) had hemorrhaged; nine patients (18%) had subarachnoid hemorrhage from another aneurysm. Three AChA aneurysms (6%) were associated with symptoms other than rupture. Forty-one patients (82%) achieved a Glasgow Outcome Scale (GOS) score of 4 or 5 at long-term follow up. The surgical mortality rate was 4%, and major surgical morbidity (GOS < or = 3) was 10%. Eight patients (16%) had clinically and computerized tomography-demonstrated AChA territory infarcts. Five of these strokes manifested in a delayed fashion 6 to 36 hours after the operation, and progressed from mild to complete deficit over hours. In 41 patients the aneurysm arose from the internal carotid artery adjacent to the AChA, and in nine patients the aneurysm arose directly from the origin of the AChA itself; four of these nine patients had postoperative infarction. CONCLUSIONS: Surgical treatment of AChA aneurysms involves a significant risk of debilitating ischemic complications. Most postoperative strokes occur in a delayed fashion, offering a potential therapeutic window. Patients with aneurysms arising from the AChA itself have an extremely high risk for postoperative stroke.

Surgical repair of clinoidal segment carotid artery aneurysms unsuitable for endovascular treatment.

OBJECTIVE: Clinoidal segment carotid artery aneurysms are surgically challenging lesions. The aneurysm neck originates proximal to the distal dural ring, and the aneurysms typically are larger. Therefore, endovascular techniques are often considered to be the primary treatment option. Treatment techniques and results for 40 clinoidal segment carotid artery aneurysms that were considered unsuitable for contemporary endovascular intervention are analyzed in this report. METHODS: Forty aneurysms in 33 female and 3 male patients were treated surgically. Fifteen patients had bilateral aneurysms; of these patients, four underwent bilateral craniotomies. Twenty-seven aneurysms were 10 to 14 mm in size, eight were 15 to 24 mm, and five were more than 25 mm. The most common presentation was visual loss, which occurred in 13 patients. Seven patients presented with subarachnoid hemorrhage. RESULTS: Thirty-seven aneurysms were directly repaired with clipping, two were trapped with bypass, and one was trapped without bypass. The complication rate was 10%, with one major stroke, two minor strokes, and one successfully treated brain abscess. CONCLUSION: Surgical treatment of clinoidal segment carotid artery aneurysms can produce acceptable outcomes. Specific preoperative and intraoperative techniques facilitate improved surgical results for aneurysms that are not treatable with contemporary endovascular techniques.

Management of malignant pineal germ cell tumors with residual mature teratoma.

OBJECTIVE: The treatment of intracranial mixed germ cell tumors presents a unique challenge, since eradication of malignant tumor by radiation and/or chemotherapy may spare the benign tumor component. We reviewed our surgical experience with residual malignant pineal germ cell tumors after neoadjuvant therapy. METHODS: Between 1987 and 1997, 16 patients with malignant intracranial germ cell tumors were treated at the Mayo Clinic with a protocol of neoadjuvant chemotherapy and radiation therapy. After the diagnosis was confirmed by histopathological examination, all patients were treated with four cycles of etoposide and cisplatin as well as external beam radiation therapy (range, 3030-5940 cGy). Six patients had an incomplete response to therapy, as demonstrated by observation of residual tumor on magnetic resonance imaging scans. Initial pathology in these six patients was germinoma in four and combinations of yolk sac tumor, embryonal carcinoma, malignant teratoma, and germinoma in two. Two patients had synchronous pineal and suprasellar tumors, with leptomeningeal dissemination. Tumor markers were elevated in four of the six patients at presentation. RESULTS: All patients with residual pineal tumors underwent surgical resection via an infratentorial, supracerebellar approach. Pathological examination revealed mature teratoma in five patients and amorphous debris in one patient. No patient had recurrent malignancy. Significant neurological morbidity occurred in one patient, with no mortality. At a mean follow-up of 23 months, no recurrence on magnetic resonance imaging has been documented. CONCLUSION: Residual pineal tumor occurring after treatment of malignant intracranial germ cell tumor with neoadjuvant therapy is likely to be mature teratoma. Operative resection of these benign recurrences is safe and effective.

Remote cerebellar hemorrhage after supratentorial surgery.

OBJECTIVE: Remote cerebellar hemorrhage (RCH) is an infrequent and poorly understood complication of supratentorial neurosurgical procedures. We retrospectively compared 42 patients who experienced RCH with a case-matched control cohort, to delineate risk factors associated with the occurrence of this complication. METHODS: Between 1988 and 2000, 42 patients experienced RCH after supratentorial neurosurgical procedures at our institution. Diagnoses were made on the basis of postoperative computed tomographic or magnetic resonance imaging findings in all cases. The medical records for these patients were reviewed and compared with those for a control cohort of 43 patients, matched for age, sex, surgical lesion, and type of craniotomy, who were treated during the same period. RESULTS: RCH most commonly occurred after frontotemporal craniotomies for unruptured aneurysm repair or temporal lobectomy and was frequently an incidental finding on postoperative computed tomographic scans. However, some cases of RCH were associated with significant morbidity, and two patients died. Preoperative aspirin use and elevated intraoperative systolic blood pressure were significantly associated with RCH (P = 0.026 and P = 0.036, respectively). Pathological findings for two cases demonstrated hemorrhagic infarctions in both. CONCLUSION: RCH most commonly follows supratentorial neurosurgical procedures, performed with the patient in the supine position, that involve opening of cerebrospinal fluid cisterns or the ventricular system (such as unruptured aneurysm repair or temporal lobectomy). Preoperative aspirin use and moderately elevated intraoperative systolic blood pressure are potentially modifiable risk factors associated with the development of RCH. Although RCH can cause death or major morbidity, most cases are asymptomatic or exhibit a benign course. Cerebellar "sag" as a result of cerebrospinal fluid hypovolemia, causing transient occlusion of superior bridging veins within the posterior fossa and consequent hemorrhagic venous infarction, is the most likely pathophysiological cause of RCH.

Development of a cerebral arteriovenous malformation documented in an adult by serial angiography. Case report.

This 61-year-old man with a right-sided tentorial dural arteriovenous fistula (DAVF) was initially treated with staged stereotactic radiosurgery and transarterial embolization. Results of follow-up cerebral angiography performed 4 years later demonstrated complete obliteration of the dAVF and development of a previously undetected cerebellar arteriovenous malformation (AVM). The newly diagnosed AVM was treated with repeated stereotactic radiosurgery. This represents the first reported case of the development of a cerebral AVM documented in an adult by serial angiography.