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Natural course of untreated spinal cord cavernous malformations: a follow-up study within the initial 5 years after diagnosis

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Abstract

OBJECTIVE Cavernous spinal cord malformations (SCMs) are believed to have a high rate of bleeding. The risk of intramedullary hemorrhage (IMH) or recurrent IMH and the neurological impact of bleeding events are important for clinical decision-making and could impact current treatment strategies. METHODS The authors screened their institutional database for patients with cavernous SCM treated between 2003 and 2020. Patients with complete MRI data sets and clinical baseline characteristics were included. Surgically treated patients were censored after cavernous SCM removal. Neurological functional status was obtained using the modified McCormick (MMcC) scale at diagnosis, first IMH, and second IMH. Kaplan-Meier and Cox regression analyses were performed to determine the cumulative 5-year risk for hemorrhage or rehemorrhage. RESULTS Seventy-one patients with cavernous SCM were analyzed. Cox regression analysis identified previous IMH (hazard ratio 7.86, 95% confidence interval 1.01–61.47, p = 0.049) as an independent predictor for rehemorrhage during the 5-year follow-up. The cumulative 5-year risk of bleeding or rebleeding was 41.3% for cavernous SCM. The MMcC score significantly deteriorated in 75% of patients after recurrent hemorrhage (p = 0.012). CONCLUSIONS During untreated 5-year follow-up, a considerably increased risk for hemorrhage or rehemorrhage was found in cavernous malformations of the spinal cord compared to cerebral cavernous malformations. Neurological function significantly deteriorates after the second bleeding. The probability of recurrent IMH increased significantly after initial presentation with hemorrhage.

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The authors conducted a study to assess the clinical pattern, radiological features, therapeutic strategies, and long-term outcomes in patients with intramedullary spinal cord cavernomas (ISCCs) based on a large case series. This retrospective study identified 96 patients (60 males, 36 females) surgically (81 cases) or conservatively (15 cases) treated for ISCCs between May 1993 and November 2007. Each diagnosis was based on MR imaging and spinal angiography evidence. For all surgically treated patients, the diagnosis was verified pathologically. The neurological outcomes pre- and postoperatively, as well as long-term follow-up, were assessed using the Aminoff-Logue Disability Scale. The mean age at the onset of symptoms was 34.5 years (range 9-80 years). Of the lesions, 68 (71%) were located in the thoracic spine, 25 (26%) in the cervical spine, and only 3 (3%) in the lumbar spine. The median symptom duration was 19.7 months. The clinical behavior of the lesion was a slow progression in 73 cases and an acute decline in 23 cases. Long-term follow-up data (mean 45.8 months, range 10-183 months) were available for 75 patients (64 surgical cases and 11 conservative cases). In the surgical group, a complete resection was achieved in 60 patients, and incomplete resection was detected in 4 patients after operation. At the end of the follow-up period in the operative group, 23 patients (36%) improved, 35 (55%) remained unchanged, and 6 (9%) worsened. In the nonoperative group, 5 patients improved, 6 patients remained unchanged, and none worsened. For differential diagnosis, spinal angiography was necessary in some cases. For most symptomatic lesions, complete microsurgical resection of the symptomatic ISCC is safe and prevents rebleeding and further neurological deterioration. However, in patients whose lesions were small and located ventrally in the spinal cord, one can also opt for a rigorous follow-up, considering the high surgical risk.
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The purpose of this study was to define the clinical features and the surgical technique of unilateral hemilaminectomy for treating intramedullary cavernous malformations. Retrospective chart was performed in 16 patients with histologically diagnosed intramedullary cavernous malformations. All patients were treated with unilateral hemilaminectomy and microsurgical resection of the malformations. The pre- and postoperative neurological state was evaluated using Frankel scale. There were nine females and seven males (mean age 38 years) harbouring symptomatic intramedullary cavernous malformations. The annual retrospective haemorrhage rate was 3.1% per patient/year. All cavernous malformations were completely resected. Twelve of 16 patients experienced the improvement of the neurological state and in four patients, clinical features remained unchanged during the follow-up period. Static and dynamic plain radiograph film showed none of them had spinal deformity or spinal instability. According to the defined bleeding risk, symptomatic and MRI-morphologically growing intramedullary cavernous malformations should be totally surgically removed, to avoid the recurrence and rebleeding of the residue. A least traumatic myelotomy, as well as a meticulous microsurgical technique and the intraoperative somatosensory evoked potentials monitoring, together with selection of a minimally invasive microsurgical approach (hemilaminectomy), leads to a favourable outcome and prevents additional morbidity.
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Cavernous malformations of the brain (CMs) cause intracranial hemorrhage, but its reported frequency varies, partly attributable to study design. To improve the validity of future research, we aimed to develop a robust definition of CM hemorrhage. We systematically reviewed the published literature (Ovid Medline and Embase to June 1, 2007) for definitions of CM hemorrhage used in studies of the untreated clinical course of >or=20 participants with CM(s), to inform the development of a consensus statement on the clinical and imaging features of CM hemorrhage at a scientific workshop of the Angioma Alliance. A systematic review of 1426 publications about CMs in humans, revealed 15 studies meeting our inclusion criteria. Although 14 (93%) studies provided a definition of CM hemorrhage, data were less complete on the confirmatory type(s) of imaging (87%), whether CM hemorrhage should be clinically symptomatic (73%), and whether hemorrhage had to extend outside the CM or not (47%). We define a CM hemorrhage as requiring acute or subacute onset symptoms (any of: headache, epileptic seizure, impaired consciousness, or new/worsened focal neurological deficit referable to the anatomic location of the CM) accompanied by radiological, pathological, surgical, or rarely only cerebrospinal fluid evidence of recent extra- or intralesional hemorrhage. The definition includes neither an increase in CM diameter without other evidence of recent hemorrhage, nor the existence of a hemosiderin halo. A consistent approach to clinical and brain imaging classification of CM hemorrhage will improve the external validity of future CM research.
Article
Cavernous angiomas of the spinal cord are rare lesions that can cause severe neurological symptoms. We add a series of 6 patients with intramedullary cavernous angiomas of the spinal cord to 30 patients with 31 histologically verified lesions already described in the literature. Four types of clinical presentation were seen in the 36 patients: 1) discrete episodes of neurological deterioration with varying degrees of recovery between episodes (13 patients); 2) slow progression of neurological decline (12 patients); 3) acute onset of symptoms with rapid decline (8 patients); and 4) acute onset of mild symptoms with subsequent gradual decline lasting weeks to months (3 patients). Of the 36 patients, 25 (69%) were women and 11 (31%) were men. The peak age of presentation was in the fourth decade. The thoracic spinal cord was affected in more than half the patients, with the cervical cord being the next most common location. Histological examination demonstrated the closely apposed vascular channels characteristic of cavernous angiomas. While most vascular channels were thickened and hyalinized, three angiomas had foci of small vessels resembling a capillary angioma. The available data suggest that surgical management of intramedullary angiomas should attempt complete extirpation. The lesions are often well circumscribed with a glial plane between the lesion and normal cord. However, spinal cord angiomas, unlike cranial lesions, have little room for enlargement before devastating symptoms occur. We achieved complete excision in all of our patients, who are all ambulatory and have improved symptoms. Little, if any, increase in neurological deficit was caused by myelotomy and lesion excision. These spinal lesions should be considered for surgery early, before repeated hemorrhage or enlargement can occur.
Article
Six patients with intramedullary cavernous malformations of the spinal cord are presented. Four men and two women presented with acute, subacute, or episodic signs and symptoms of spinal cord dysfunction, ranging in duration from 3 days to 25 years. All patients underwent operative resection of the malformation. Complete removal was achieved in five patients. Neurological function either stabilized or improved postoperatively in all patients; follow-up ranged from 4 to 84 months. The increasing awareness of the propensity for recurrent hemorrhage, clinical features, and resectability of these malformations are discussed.
Article
The aim of this study was to review the natural history of symptomatic intramedullary spinal cord (IMSC) cavernous malformations in order to analyze the underlying mechanisms leading to symptoms and determine the potential risk of lesional hemorrhage. Between January 1990 and June 2001, ten consecutive patients with IMSC cavernous malformations were treated surgically in our institution. Age ranged from 17 to 73 years (mean 34.5). All patients became symptomatic due to one or more hemorrhages leading to neurological deficits of different severity, with a more aggressive course for upper cervical lesions. Pre- and postoperative patient condition was classified according to the Frankel scale. Four patients experienced one hemorrhage, four patients two, one patient three, and another one five repeated hemorrhages. The annual retrospective hemorrhage rate for symptomatic IMSC cavernous malformations was 4.5% per patient/year, with a prospective rehemorrhage risk of 66% per patient/year. The postoperative condition was improved in four patients and unchanged in six, and none grew worse. Detailed analysis of history and clinical course in all patients revealed an acute onset of symptoms with subsequent neurological deterioration after each bleeding episode. Based on the significant risk of rehemorrhage and the gratifying functional results, surgery is indicated for symptomatic IMSC cavernous malformations.
Article
Once believed to be extremely uncommon, due to magnetic resonance imaging cavernous hemangiomas of the spinal cord are detected with increasing frequency. Management of both symptomatic and asymptomatic intramedullary cavernous hamangiomas is therefore of growing importance. However, experience with treatment and follow-up is very limited. In particular, patients with multiple central nervous system cavernous hemangiomas represent a therapeutical dilemma. We present a patient with a ruptured intramedullary and multiple cerebral cavernous hemangiomas and a survey of current knowledge of epidemiology, pathophysiology and treatment options. We conclude that the benefit of operative treatment possibly decreases with the number of clinically silent vascular malformations.
Article
The presentation and natural history of untreated, symptomatic intramedullary spinal cavernomas at our institution were analyzed. The objective is to provide additional information regarding the natural history of conservatively managed, symptomatic, intramedullary spinal cord cavernous malformations. The medical records of patients treated in our institution between 1989 and 2002 were reviewed to identify those with intramedullary cavernomas. The medical, radiological, surgical, and pathological records from these patients were retrospectively reviewed and analyzed. Fourteen patients were included in the study. The mean age at presentation was 42 years. Four lesions (29%) were located in the cervical region and 10 lesions (71%) were present in the thoracolumbar spinal cord. All patients were symptomatic at the time of presentation. In this cohort of 14 patients, 10 patients (71%) were conservatively managed. For these patients, the mean duration of symptoms before presentation was 10 months. The mean duration of follow-up from the time of presentation was 80 months. The median McCormick grade for conservatively treated patients at presentation was II. During this period, none of the conservatively managed patients had an acute intramedullary bleed. In nine patients, the McCormick grade at the last follow-up evaluation was the same as or better than their score at presentation. Four patients (29%) were treated surgically. The mean duration of symptoms before presentation was 33 months. The mean duration of follow-up from the time of presentation was 42 months. In two surgical patients, the McCormick grade at the last follow-up evaluation remained unchanged compared with their score at presentation, whereas the McCormick grade improved in one patient and deteriorated in another patient. This cohort of conservatively managed patients with symptomatic, intramedullary spinal cord cavernomas was clinically stable throughout the follow-up period. In this series, patients harboring symptomatic spinal cord cavernous malformation did not have significant, permanent neurological decline during the follow-up period when treated with the conservative approach of observation. This data provides additional information for determining the appropriate treatment strategy for patients with intramedullary spinal cavernomas.
Article
Prevalence of cerebral cavernomas in the general population is close to 0.5%. In contrast, SCCs are rare. The aim of this study was to determine the outcome of SCC in a large sample of patients. Clinical and neuroradiologic findings were retrospectively collected in a multicentric study. Diagnosis was based on pathologic criteria or MR findings. Fifty-three patients were included (26 males, 27 females). Mean age at onset of symptoms was 40.2 years (11-80 years). Initial symptoms were progressive (32) or acute myelopathy (20). One case was asymptomatic. Triggering factors were found in 14 of the patients (26%). Clinical symptoms were related to spinal cord compression (27) and hemorrhage (22). Spinal cord cavernoma was thoracic in 41 cases and cervical in 12. Mean size of the lesions was 16.3 mm (3-54 mm). In the 40 surgical patients, long-term follow-up was available in 37 cases for a mean time of 7.3 years (0.4-50 years). During the follow-up period, 20 patients improved, 6 remained on their preoperative baseline, and 11 got worse. Surgical improvement was more often found in posterior rather than anterior location. Using McCormick classification, 22 patients were autonomous (grades 1-2), 12 handicapped (grade 3), and 3 bedridden (grade 4) at the end of the follow-up. This study has defined clinical and MR patterns of spinal cavernomas. Surgery lastingly improved more than half of the patients.