Intramedullary spinal cord cavernous malformations

Abstract

<jats:p>Although originally the subject of rare case reports, intramedullary spinal cord cavernous malformations (CMs) have recently surfaced in an increasing number of case series and natural history reports in the literature. The authors reviewed 27 publications with 352 patients to consolidate modern epidemiological, natural history, and clinical and surgical data to facilitate decision making when managing these challenging vascular malformations. The mean age at presentation was 42 years without a sex predilection. Thirty-eight percent of the cases were cervical, 57% thoracic, 4% lumbar, and 1% unspecified location. Nine percent of the patients had a family history of CNS CMs. Twenty-seven percent of the patients had an associated cranial CM. On presentation 63% of the patients had motor deficits, 65% had sensory deficits, 27% had pain, and 11% had bowel or bladder dysfunction. Presentation was acute in 30%, recurrent in 16%, and progressive in 54% of cases. An overall annual hemorrhage rate was calculated as 2.5% for 92 patients followed up for a total of 2571 patient-years. Across 24 reviewed surgical series, a 91% complete resection rate was found. Transient morbidity was seen in 36% of cases. Sixty-one percent of patients improved, 27% were unchanged, and 12% were worse at the long-term follow-up. Using this information, the authors review surgical nuances in treating these lesions and propose a management algorithm.</jats:p>

Surgical outcomes and natural history of intramedullary spinal cord cavernous malformations: a single-center series and meta-analysis of individual patient data

“…10,16,25,33,52,66 By contrast, spinal cord cavernous malformations are very rare entities, accounting for 5%-12% of intraspinal vascular malformations. 14,24,29 In comparison with cavernous malformations within the cerebral hemispheres, those involving the spinal cord are thought to result in poorer outcomes. 10,33 Historically, cases of intramedullary spinal cord cavernous malformations (ISCCMs) have been infrequently reported and considered exceptionally rare.…”

Clinical presentation and surgical outcomes of an intramedullary C2 spinal cord cavernoma: a case report and review of the relevant literature

“…Secondly, the patient presented with features of both a slow-progressive and acute neurological decline-a presenting pattern that is not commonly noted. In fact, of the 205 patients reviewed by Gross et al, variation in the rapidity of neurological decline within a single patient was not reported (6). Compared to the median duration of symptoms prior to admission (12 months) reported by Kivelev et al, our patient had a relatively shorter period of slow progression (6 months) followed by an acute neurological decline in the days prior to surgery (7).…”

“…This case is atypical for a number of reasons. Firstly, at 56 years of age, the patient was 14 years above the average age of presentation (42 years) based on the 27 series and 305 patients reviewed by Gross et al (6). Secondly, the patient presented with features of both a slow-progressive and acute neurological decline-a presenting pattern that is not commonly noted.…”

“…Given the implications of incontinence in both clinical and social contexts, this symptom often causes patients significant distress in the longer term. According to Gross et al, only 11% of cases had bladder or bowel instability (6). Furthermore, there is a lack of reports noting recovery of bladder function postoperatively (7).…”

“…Unlike their intracranial counterpart, SCICs are more likely to give rise to significant neurological findings and are associated with mass effect, myelopathy, hemorrhage and significant morbidity (1,(3)(4)(5). The clinical presentation varies depending on site, with the thoracic (58%) and cervical (38%) spinal cord being the most frequent locations (3,6). Most patients present with progressive neurological decline, with sensory deficits and motor weakness being the © OSS Press Ltd. All rights reserved.…”