Spinal Cavernomas Research Articles

Outcome after microsurgery in 14 patients with spinal cavernomas and review of the literature

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.

Lanzino G, Spetzler RF (Eds), Cavernous malformations of the brain and spinal cord

The first book dedicated to cavernomas has been published in 1993 by Awad and Barrow. The present volume on the same topic gives credit to the fact that a large variety of additional knowledge on that disease has been accomplished within the past 15 years. The publication is edited by Lanzino and Spetzler and comprises the previous knowledge with most of the actual new insights into the interesting biology of cavernomas. It consists of two major well-organized sections addressing (1) “General Aspects”, and (2) “Clinical Presentation, Indication for Surgery, Surgical Techniques, and Outcome”. In the first section, the natural history, pathology, (molecular) biology, genetics, neuroradiology and classification are discussed in eloquent chapters. The potential biological relation of cavernomas to capillary teleangiectasias and developmental venous anomalies is also covered by an article. Within the first section, the most interesting new information is given in the chapters on the molecular biology and genetics of cavernomas. Here, the ultrastructure, role of endothelial proliferation, and neoangiogenesis are as well summarized as the role of the so far reported three genes (CCM1, CCM2, CCM3) that are related to the familial cavernoma syndromes. Further useful information is also given about the potential influence of previous radiotherapy on the de novo development of cavernomas and the possible cutaneous alterations that are sometimes associated mainly with the familial forms of the disease. The second section of the book describes various considerations as for the different types of localization such as supratentorial, superficial, and deep, as well as brainstem and spinal cavernomas, respectively. Indication for surgery, surgical approaches and technique are well illustrated and described. Further chapters deal with atypical giant and pediatric cavernomas. A very nice part discusses the surgical treatment of epilepsy as the main clinical symptom of cortically located supratentorial cavernomas, before the last chapter addresses the potential therapeutic role of radiosurgery, a treatment option that at least in our humble understanding should not be performed. The only objectionable points to discuss are the quality of some of the figures which are generally of a high resolution but lack color printing, particularly for some neuropathological and intraoperative photographs. Although by far not complete, the most recent literature is adequately cited in the majority of the chapters. In summary, the present book belongs in any vascular neurosurgeons bookshelf and might also be of interest for scientists interested in the biology of cavernous malformations because it very well summarizes all necessary information on this pathology.

The microsurgical treatment and prognosis of intramedullary spinal cord cavernoma

Objective To investigate the clinical course, radiolngical characteristics and long term outcome of patients with intramedullary spinal cord cavernoma(ISCC). Method Clinical data on 81 patients who accepted microsurgical treatment in our hospital from 1993 to 2007 were retrospectively analyzed. The function of the spinal cord in pre, post-operation and long term follow-up was assessed by Aminoff & Logue disability scale. Results ISCC constituted 8.7% of all spinal cord vascular disease. The mean age at onset of symptoms was 35.7-year-old. The mean duration of symptom was 22.5 months. The lesions of ISCC located in thoracic, cervical and lumbar segment was 69%、28% and 3% respectively. All patients underwent microsurgical resection of ISCC. The long term follow-up data was obtained in 64 patients. At the end of the follow up, the neurological function improved in 23 cases, unchanged in 35 cases and got worse in 6 cases. Incomplete resection was detected in 4 patients with neurological condition decline due to rebleeding of residual. Conclusions (1) Thoracic segment of spinal cord is the most common site for ISCC and the symptoms usually be onset during the third to fourth decade. (2) MRI is specific for the diagnosis of ISCC and the spinal angiography is necessary for some patients for differential diagnosis. (3) Total resection of lesions is the best way to avoid symptom recurrence due to rebleeding of the residue. Key words: Hemangioma,cavemous,central nervous; Magnetic resonance imaging; Microsurgery

Spinal Cavernoma

Cavernous hemangiomas or cavernomas are uncommon vascular malformations of the central nervous system involving most commonly the cerebral hemispheres where they are detected in young to middle aged adults. We present an unusual case of acute monoparesis caused by an intramedullary cavernoma in a woman of advanced age. A 67-year-old woman presented with walking difficulties with acute onset 2 months previously. On neurologic examination, there was a pure right leg monoparesis with moderate spasticity. Tendon reflexes were brisk and there was a Babinski's sign in her right lower limb. The initial diagnosis was lacunar stroke, but the brain magnetic resonance imaging revealed a right temporal cavernoma-obviously not associated with her monoparesis. The consequent spinal MRI revealed an intramedullary lesion at the T1 level, consistent with a cavernoma. Our patient presented with an acute monoparesis because of a spinal cavernoma, a most unusual occurrence.

Spinal root arteriovenous malformations and same-segment cord cavernous malformation in familial cerebral cavernous malformation

Spinal vascular malformations are uncommon lesions, and controversy persists regarding optimal investigation, classification, and treatment strategies. The authors report on a patient with a spinal root arteriovenous malformation (AVM) associated with a parenchymal cavernous malformation (CM) in the same spinal cord segment and describe a complete familial and molecular investigation. This 35-year-old woman presented with symptoms of progressive clinical spastic paraparesis. Magnetic resonance imaging results were suggestive of a spinal cord cavernoma associated with cerebral CMs. Her family history included 2 sisters treated for epilepsy. At surgery an intraspinal root AVM was found at the same level of the cord CM, and both lesions were completely removed. Cerebral gradient echo MR imaging disclosed multiple cavernomas in her relatives, which prompted the molecular diagnosis. On sequence analysis, a novel mutation on the cerebral CM1 (CCM1) gene (c796insA) was found. The authors report on a unique case of familial cerebral CM in which a spinal root AVM was situated next to a cord CM, and discuss the concomitant occurrence of altered nervous system angiogenesis and vasculogenesis.

Outcome in 53 patients with spinal cord cavernomas

Background 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. Methods Clinical and neuroradiologic findings were retrospectively collected in a multicentric study. Diagnosis was based on pathologic criteria or MR findings. Results 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. Conclusions This study has defined clinical and MR patterns of spinal cavernomas. Surgery lastingly improved more than half of the patients.

Cervical intradural extramedullary cavernoma presenting with isolated intramedullary hemorrhage

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, cavernomas 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.

THE NATURAL HISTORY OF CONSERVATIVELY MANAGED SYMPTOMATIC INTRAMEDULLARY SPINAL CORD CAVERNOMAS

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.

Spinal Vascular Malformations

Spinal vascular malformations are rare diseases with a wide variety of neurological presentations. In this article spinal cavernomas, arteriovenous malformations (both from the fistulous and glomerular type), and spinal dural arteriovenous fistulae are described and an overview about their imaging features on magnetic resonance imaging (MRI) and digital subtraction angiography is given. Clinical differential diagnoses, the neurological symptomatology and the potential therapeutic approaches of these diseases which vary depending on the underlying pathology are given. Although MRI constitutes the diagnostic modality of first choice in suspected spinal vascular malformation, a definite diagnosis of the disease and therefore the choice of suited therapeutic approach rest on selective spinal angiography. Treatment in symptomatic patients offers an improvement in the prognosis. In most spinal vascular malformations except for spinal cavernomas, the endovascular approach is the method of first choice; in selected cases a combined or surgical therapy might be considered.

Combined thoracotomy and laminectomy for spinal cavernomas with intrathoracic growth

Dumbbell shaped epidural cavernomas are uncommon lesions. Of these, the extraforaminal extension is usually limited, and the lesions are removed surgically by an extended laminectomy. We present two cases of dumbbell shaped epidural cavernomas with a considerable intrathoracic growth. They were resected through a combined thoracotomy and laminectomy. Only in the case in which a wide approach to the posterior mediastinum was performed it was possible to completely remove the lesion. Since it is possible to anticipate this histological diagnosis by the magnetic resonance imaging characteristics, we propose that a wide transthoracic approach should be performed associated to a laminectomy if a complete resection of the lesion is the goal.

Extradural spinal cavernous haemangioma: case report and review of the literature

Cavernous haemangiomas (cavernomas) are uncom-mon vascular malformations of the central nervous system (CNS). They occur in both sporadic and familial forms and may involve any site in the CNS. Spinal cavernomas are less common than intracerebral lesion s, and examples in the spinal epidural space are rare. A case of a solitary sporadic spinal extradural cavernoma in a 41 year old male which presented as progressive lower limb numbness and weakness is reported. The literature regarding spinal cavernomas is reviewed and the symptomatology, diagnostic evaluation, pathology, management and prognosis of these lesions are discussed.

Treatment strategies and results in spinal vascular malformations.

We report the treatment strategies and results of 70 patients with spinal vascular malformations. Forty-six had dural arteriovenous fistulas, 12 spinal cavernous angiomas, nine intramedullary angiomas, and three intradural arteriovenous fistulas. The diagnosis was established for cavernomas by magnetic resonance images only and in the other cases by selective spinal angiography in patients whose neurological deficits, myelograms or magnetic resonance images suggested the presence of a spinal vascular malformation. All patients had symptomatic vascular malformations and were treated microsurgically. Intramedullary angiomas were operated when embolization seemed too dangerous or impossible and when they had a contact to the dorsal or lateral surface of the spinal cord. All but one were completely resected. In one angioma a small ventral residual fistula area was left. Complete obliteration of all fistulas was achieved. The cavernomas were primarily resected. Apart from one postoperative permanent deterioration with a paresis of the left arm in a patient with an intramedullary angioma, 16 cases presented only a transitory worsening of their neurological status after surgery. The long-term outcome of all these patients was good. Five patients had to be operated on again: three patients showed difficult localizations of dural fistulas which were still visible in the postoperative angiograms, one patient suffered a spinal epidural hematoma, and another patient showed a cerebrospinal fluid accumulation. We conclude that spinal dural arteriovenous fistulas, small intradural fistulas, spinal cavernomas, and symptomatic spinal angiomas with contact to the lateral or dorsal surface can be treated microsurgically with low perioperative morbidity.

O-26-390 - Microsurgical management of spinal cavernomas

O-26-390 - Microsurgical management of spinal cavernomas