✓Among cavernomas of the central nervous system, spinal ones are rare. The true incidence of spinal cavernomas is unclear, but with widespread use of magnetic resonance imaging the number of cases is increasing. Furthermore, cav-ernomas represent only 5–12% of all vascular anomalies of the spinal cord, with a mere 3% reported to be intradural and intramedullary in location. Cervical spine intradural extramedullary cavernomas are very seldom seen, and only 4 cases have been reported in world literature previously. In this report, a unique case of an intradural extramedullary spinal cavernoma was surgically treated in a patient who presented only with an intramedullary hemorrhage.
Juri Kivelev, Christian N. Ramsey, Reza Dashti, Matti Porras, Olli Tyyninen and Juha Hernesniemi
Juri Kivelev, Mika Niemelä, Riku Kivisaari and Juha Hernesniemi
Intraventricular cavernomas (IVCs) occur in only 2–10% of patients with cerebral cavernomas. Reports concerning IVC are scarce and are limited mostly to sporadic case reports. In this paper, the authors present a series of 12 patients with IVCs that were treated at a single neurosurgical department. In addition, the authors reviewed the literature.
All clinical data were analyzed retrospectively. Follow-up questionnaires were sent to all patients. Outcome was assessed using the Glasgow Outcome Scale. The authors also conducted a PubMed search and found 77 cases of IVC.
The patients' median age was 47 years, and the male/female ratio was 2:1. A cavernoma occurred in the lateral ventricle in 6 patients, in another 5 it was in the fourth ventricle, and 1 had a lesion in the third ventricle. Almost all patients presented with acute headache on admission and in more than half, the symptoms were related to cavernoma bleeding. In total, 8 rebleedings occurred in 5 patients during a median of 0.4 years. Three patients with a cavernoma of the fourth ventricle presented with a cranial nerve deficit. In 8 cases, a cavernoma was surgically treated an average of 1.3 years after the diagnosis. Only 1 patient underwent surgery in the acute phase after a major intraventricular/intracerebral hemorrhage. The median follow-up time was 2 years. No patient was lost to follow-up, and no patient died. In total, on follow-up 9 patients improved and 3 had a persistent neurological deficit, of which 2 existed before surgery.
In the present series, the IVCs had a high tendency for rehemorrhage. Surgery is advocated when hemorrhages are frequent, and the mass effect causes progressive neurological deficits. Microsurgical removal of the IVC is safe, but in the fourth ventricle it can carry increased risk for cranial nerve deficits.
Juri Kivelev, Mika Niemelä and Juha Hernesniemi
Spinal cavernomas are rare, but can cause significant neurological deficits due to mass effect and extralesional hemorrhage. The authors present their results of microsurgical treatment of 14 consecutive patients with spinal cavernoma, and review the literature.
Of the 376 patients with cavernomas of the CNS treated at Helsinki University Central Hospital (a catchment area close to 2 million inhabitants) between January 1980 and June 2009, 14 (4%) had a spinal cavernoma. The authors reexamined and analyzed the patient files and images retrospectively. Median patient age at presentation was 45 years (range 20–57 years). The female/male ratio was equal. Median duration of symptoms before admission to the department was 12 months (range 0.1–168 months). Patients suffered from sensorimotor paresis, radicular pain, or neurogenic micturition disorders in different combinations or separately. Hemorrhage had occurred in 7 patients (50%) before surgery. In 9 patients (64%) the cavernoma was intramedullary, in 4 (29%) extradural, and in 1 intradural extramedullary. On MR imaging, 6 patients (43%) had a cavernoma in the cervical region, 7 (50%) in the thoracic region, and 1 (7%) in the lumbar region.
Postoperatively, patients were followed up for a median of 3 years (range 1–10 years). At follow-up, 13 patients (93%) experienced significant improvement in motor ability after surgery, and all patients were able to walk with or without aid. Ten of the 11 patients with pain syndrome (91%) showed significant pain relief without recurrence. Micturition disorder was noted in 6 patients (43%) at follow-up, but in 5 the condition had existed before surgery. No patient improved in bladder function after surgery, and 1 patient developed micturition dysfunction postoperatively.
Microsurgical removal of spinal cavernomas alleviates sensorimotor deficits and pain caused by mass effect and hemorrhage. However, bladder dysfunction remains unchanged after surgery.
Juri Kivelev, Elina Koskela, Kirsi Setälä, Mika Niemelä and Juha Hernesniemi
Cavernomas in the occipital lobe are relatively rare. Because of the proximity to the visual cortex and incoming subcortical tracts, microsurgical removal of occipital cavernomas may be associated with a risk of visual field defects. The goal of the study was to analyze long-term outcome after operative treatment of occipital cavernomas with special emphasis on visual outcome.
Of the 390 consecutive patients with cavernomas who were treated at Helsinki University Central Hospital between 1980 and 2011, 19 (5%) had occipital cavernomas. Sixteen patients (4%) were surgically treated and are included in this study. The median age was 39 years (range 3–59 years). Seven patients (56%) suffered from hemorrhage preoperatively, 5 (31%) presented with visual field deficits, 11 (69%) suffered from seizures, and 4 (25%) had multiple cavernomas. Surgery was indicated for progressive neurological deterioration. The median follow-up after surgery was 5.25 years (range 0.5–14 years).
All patients underwent thorough neuroophthalmological assessment to determine visual outcome after surgery. Visual fields were classified as normal, mild homonymous visual field loss (not disturbing the patient, driving allowed), moderate homonymous visual field loss (disturbing the patient, driving prohibited), and severe visual field loss (total homonymous hemianopia or total homonymous quadrantanopia). At the last follow-up, 4 patients (25%) had normal visual fields, 6 (38%) had a mild visual field deficit, 1 (6%) complained of moderate visual field impairment, and 5 (31%) had severe homonymous visual field loss. Cavernomas seated deeper than 2 cm from the pial surface carried a 4.4-fold risk of postoperative visual field deficit relative to superficial ones (p = 0.034). Six (55%) of the 11 patients presenting with seizures were seizure-free postoperatively. Eleven (69%) of 16 patients had no disability during the long-term follow-up.
Surgical removal of occipital cavernomas may carry a significant risk of postoperative visual field deficit, and the risk is even higher for deeper lesions. Seizure outcome after removal of these cavernomas appeared to be worse than that after removal in other supratentorial locations. This should be taken into account during preoperative planning.