Spinal cord cavernous malformation cavernous malformation case series

Seventeen patients with spinal cavernomas underwent surgery between 02/2004 and 06/2020. The detailed neurological and clinical outcomes as well as IONM data including motor-evoked potential (MEP) and somatosensory-evoked potential (SSEP) monitoring were retrospectively analyzed. Intraoperative IONM changes were compared to outcomes at 3-month and 1-year follow-ups in order to identify surrogate parameters for an impending neurological deficit.

Compared to the preoperative state, McCormick's score at 1-year follow-up remained unchanged in 12 and improved in five patients, none worsened, while detailed neurological examination revealed a new or worsened sensorimotor deficit in 4 patients. The permanent 80% amplitude reduction of MEP and 50% amplitude reduction of SSEP showed the best diagnostic accuracy with a sensitivity of 100% and 67% respectively and a specificity of 73% and 93% respectively. The relative risk for a new neurological deficit at 1-year follow-up, when reversible IONM-deterioration was registered compared to irreversible IONM deterioration, was 0.56 (0.23-1.37) for MEP deterioration and 0.4 (0.18-0.89) for SSEP deterioration.

Reversible IONM changes were associated with a better neurological outcome at follow-up compared to irreversible IONM deterioration during SCCM surgery. Our study favors the permanent 80% amplitude reduction criterion for MEP and 50% amplitude reduction criterion for SSEP for further prospective evaluation of IONM significance and the effectiveness of corrective maneuvers during SCCM surgeries ¹⁾.

All patients with a pediatric diagnosis of familial cerebral cavernous malformation (FCCM) evaluated at three tertiary pediatric hospitals between January 2010 and August 2021 with 1 whole spine magnetic resonance imaging available were included. Brain magnetic resonance imaging and spine magnetic resonance imaging studies were retrospectively evaluated, and clinical and genetic data were collected. Comparisons between spinal cord cavernous malformations (SCCM) + and SCCM- groups were performed using Student's t-test/Mann–Whitney U test or Fisher's exact test, as appropriate.

Thirty-one children (55% boys) were included. Baseline spine MR was performed (mean age = 9.7 years) following clinical manifestations in one subject (3%) and as a screening strategy in the remainder. Six SCCM were detected in five patients (16%), in the cervical-medullary junction (n = 1), cervical (n = 3), and high thoracic (n = 2) regions, with one appearing during follow-up. A tendency towards an older age at first spine MR (P = 0.14) and [Formula: see text] 1 posterior fossa lesion (P = 0.13) was observed in SCCM + patients, lacking statistical significance. No subject demonstrated ISVM.

Although rarely symptomatic, spinal cord cavernous malformations can be detected in up to 16% of pediatric familial cerebral cavernous malformation patients using diverse spine magnetic resonance imaging protocols and may appear de novo. intraosseous spinal vascular malformations (ISVM) were instead absent in this cohort. Given the relative commonality of asymptomatic spinal cord cavernous malformations, serial screening spine MR should be considered in familial cerebral cavernous malformation starting in childhood ²⁾.

Jeon et al., retrospectively evaluated MR images of spinal cord tumors collected at the hospital from 2007 to 2015. From this cohort of images, 11 pathologically confirmed cavernous angiomas and 14 pathologically confirmed hemorrhagic ependymomas were compared with respect to the size of the tumor, longitudinal location, axial location, enhancement pattern, syrinx, edema, tumor margin, signal intensity of T2 weighted image, signal intensity of T1WI, and longitudinal spreading of the hemorrhage. Serial MR images of seven spinal cavernous angiomas were reviewed. Small size, eccentric axial location, minimal enhancement, and absence of edema were more frequently observed on images of cavernous angioma compared to those of hemorrhagic ependymoma (p < 0.01). Serial MRI changes in cases of cavernous angioma included increased longitudinal spreading of the hemorrhage (6/7, 86 %) and emergence of high signal intensity on T1WI (1/7, 14 %). Small size, eccentric axial location, minimal enhancement, and absence of edema are significant MRI findings that may be used to distinguish Type I and Type II spinal cavernous angiomas from hemorrhagic ependymomas. Furthermore, longitudinal spreading of the hemorrhage may be observed on follow-up MRIs of cavernous angiomas ³⁾.

Of 85 patients; 51 (60.0%) were male. Mean age of patients was 40.5 years. Fifty-eight patients underwent microsurgical removal, and 27 patients underwent conservative management. All patients except 1 harbored a single symptomatic intramedullary CM. Mean follow-up time was 42.8 months. For the surgical group (n = 58), 51 CMs were completely resected. During the follow-up period, 40 patients (69.0%) within the surgical group had improvement in neurological state, 16 patients (27.6%) remained unchanged, and 2 patients (3.4%) experienced deteriorated functional status. In the conservative group, 4 patients (14.8%) had improvement of their symptoms, 19 patients (70.4%) remained in baseline, and 4 patients (14.8%) deteriorated. No significant statistical difference was observed in follow-up Karnofsky Performance Status assessment (odds ratio = 0.89; 95% confidence interval = 0.73-1.08; P = .15) or Modified McCormick Scale assessment (odds ratio = 0.90; 95% confidence interval = 0.74-1.10; P = .30) after adjustment for preoperative lesion size and location. Annual hemorrhagic risk was 3.9% in conservatively managed patients. In contrast, no patients experienced subsequent hemorrhages after surgical resection.

Surgical resection of intramedullary CMs eliminates the risk of subsequent hemorrhagic and may achieve satisfactory outcome when patients are carefully selected. Although conservative management is recommended in patients at high surgical risk, they should be closely monitored because of persistent hemorrhagic risk ⁴⁾.

96 patients (60 males, 36 females) surgically (81 cases) or conservatively (15 cases) treated 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 and 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 ⁵⁾.

In 14 patients, 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 ⁶⁾.