Intramedullary Cavernoma Research Articles

Surgical management of cervical intramedullary cavernoma: case report and systematic review of the literature

BackgroundCavernous malformations can occur throughout the cerebral nervous system, most commonly located in the supratentorial compartment. The intramedullary location is rare and accounts for approximately 2.4% to 5% of all spinal vascular tumors. The cervical cord location and its clinical manifestations are underreported.MethodsThe authors report a case of spinal cord compression at the C5–C6 level by a cervical intramedullary cavernoma with a systematic review of the literature according to the Preferred Reporting Items for Systematic Reviews and meta-analysis guidelines. Relevant studies (1980 to 2023) that reported patients with cervical intramedullary cavernoma were identified from PubMed databases.ResultsA total of 29 studies reporting 423 patients were included in this study with a mean age of 40.5 ± 6.3 years old. The overall male-to-female ratio was 1:2.5 with a median duration of follow-up of 46 months [18.5–63.9]. The type of resection as well as the patient’s outcome was also reported.ConclusionIntramedullary cavernous angiomas are rare. Early surgical total resection of the symptomatic lesions in adequate conditions is crucial for good outcomes.

A Case of Hematomyelia in a Teenager with Behcet’s Disease due to both Warfarin and Intramedullary Cavernous Malformation (P6-5.029)

<h3>Objective:</h3> Highlight the importance of recognizing bleeding of intramedullary cavernous malformation or cavernoma as a cause of spinal cord lesion. <h3>Background:</h3> Intramedullary spinal cord hemorrhage, or hematomyelia, is a rare cause of acute myelopathy. Vascular malformations, anticoagulation therapy, and other bleeding diathesis are the most common causes. Cavernoma is a vascular malformation made of closely clustered, enlarged capillaries with a single layer of endothelium and without the elements of normal vascular structure or normal intervening brain parenchyma. Although they can bleed, cavernomas have a relatively benign prognosis. Hemorrhage of an intramedullary cavernoma during anticoagulation with warfarin was not previously reported. <h3>Design/Methods:</h3> Nineteen-year-old man with Behçet’s disease taking warfarin for intraventricular thrombus and multiple pulmonary emboli complained of acute back pain, unilateral leg weakness, and urinary retention. He had no personal or family history of vascular malformations. Examination was remarkable for paraplegia, a sensory level at T10, lower limb hyporeflexia and Babinski reflex bilaterally. INR was supratherapeutic at 3.6. CT of head, cervical and thoracic spine was unremarkable. <h3>Results:</h3> MRI of spine showed an enhancing intramedullary lesion at T8–T9, 3.8 cm in length, with surrounding edema. The mixed T1 hyperintensity and T2 hypointensity was suggestive of hemorrhage caused by a cavernoma. Warfarin was reversed with prothrombin complex concentrate. He was not a candidate for neurosurgical intervention. At discharge, the patient had not regained any motor or sensory function. <h3>Conclusions:</h3> This case highlights the need to suspect hematomyelia as a cause of acute myelopathy in a patient taking anticoagulation. While anticoagulation is not contraindicated in patients with known cavernoma, there should be a low threshold for considering the presence of a bleeding intramedullary cavernoma in a patient with acute myelopathy. <b>Disclosure:</b> Dr. Sacks has nothing to disclose. Dr. Treidler has nothing to disclose.

Rupture of the recurrent thoracic intramedullary spinal cord cavernoma

The intramedullary spinal cord cavernoma is a rare vascular disease that occur within spinal cord or intramedullary and account for about 5%-12% of all pathology in the spinal vascular disease. We report a clinical progression of disease and evolution of the haemorrhage every years until patient underwent surgical procedure. A 63-years old male with progressive weakness of the lower extremity since 1 month before admission, worse in the left side He also complained loss of sensation at the level of the T4 and below it. Patient suffer three times rebleeding before underwent surgery. MR imaging showing intramedullary spinal cord cavernoma and a histology examination exhibited the characteristics of a cavernoma. Intramedullary spinal cord cavernoma is a rare disease that should be treated before rebleeding occur and enlargement of the lesion. Postoperative neurological function in intramedullary spinal cord cavernoma patient is determined by the preoperative neurological status.

Intramedullary Cavernoma: Case Report and Literature Review

Introduction: Cavernomas are benign vascular anomalies consisting of cavities where the blood circulates at low flow and at low pressure. Intramedullary localization is unusual, represents approximately 5 to 12% of spinal vascular malformations and 3% of intra-dural vascular malformations (5% of medullary vascular lesions). Observation: A patient, aged 59, consulted for the abrupt installation of moderate back pain followed by predominant muscle weakness in the two lower limb of progressive worsening, responsible for gait disorders. The patient reported thermal hypoesthesia and heaviness of the two lower limbs that had been evolving for two years. The examination found a dorsal spinal cord compression syndrome. On the MRI, there were abnormalities of intramedullary signal of the dorsal (D11) spinal cord with bleeding stigmas suggestive of intramedullary cavernomas. Conclusion: The management of the medullary cavernoma is essentially neurosurgical with complete microsurgical resection of the malformation. In the absence of surgical treatment, evolution can be to chronic myelopathy or neurological aggravation.

Cervical Intramedullary Cavernoma in Children: Case report and literature review

Case presentation: A 10-year-old male patient was admitted due to posterior cervicalgia, vomiting and progressive generalized weakness. Physical examination showed difficulty in ambulation and tetraparesis. Magnetic resonance imaging (MRI) of the cervical medulla enhanced by the contrast agent, gadolinium, showed a poorly contrasted mass with a hemorrhagic center. We undertook a posterior cervicotomy (C1-C3). Post-operative evolution was satisfactory with complete regression of the deficits 90 days after surgery. Control MRI at intervals of 1 and 4 years confirmed absence of the tumor. Discussion: Cavernomas are vascular malformations, consisting of coarsely dilated vascular channels and coated by a single layer of endothelial cells, devoid of endothelium and myothelium. They represent only 5-12% of all vascular pathologies of the medulla, with only 10% affecting the pediatric population. There are only thirty cases of pediatric intramedullary spinal cavernomas in the literature, with predominance among males (2.1:1). Thoracic and cervical spinal cavernomas consist in 55% and 45% of the cases, respectively. In six cases (20%) cavernomatous lesions were associated with synchronic intracranial cavernoma. Among the reported cases, only one had poor evolution after surgery, whereas six patients persisted with prior symptoms. Conclusion: Spinal intramedullary cavernomas are rare entities, especially in the pediatric population, and are treated with surgery which improves prior neurologic deficits, besides preventing rebleeding

Intramedullary Cavernoma: Case Report and Literature Review

Introduction: Cavernomas are benign vascular anomalies consisting of cavities where the blood circulates at low flow and at low pressure. Intramedullary localization is unusual, represents approximately 5 to 12% of spinal vascular malformations and 3% of intra-dural vascular malformations (5% of medullary vascular lesions). Observation: A patient, aged 59, consulted for the abrupt installation of moderate back pain followed by predominant muscle weakness in the two lower limb of progressive worsening, responsible for gait disorders. The patient reported thermal hypoesthesia and heaviness of the two lower limbs that had been evolving for two years. The examination found a dorsal spinal cord compression syndrome. On the MRI, there were abnormalities of intramedullary signal of the dorsal (D11) spinal cord with bleeding stigmas suggestive of intramedullary cavernomas. Conclusion: The management of the medullary cavernoma is essentially neurosurgical with complete microsurgical resection of the malformation. In the absence of surgical treatment, evolution can be to chronic myelopathy or neurological aggravation.

Cervical Intramedullary Cavernoma in Children: case report and literature review

Case presentation: A 10-year-old male patient was admitted due to posterior cervicalgia, vomiting and progressive generalizedweakness. Physical examination showed difficulty in ambulation and tetraparesis. Gadolinium-loaded magnetic resonance imaging(MRI) of the cervical medulla, showed a poorly contrasted mass with a hemorrhagic center. We undertook a posterior cervicotomy(C1-C3). Post-operative evolution was satisfactory with complete regression of the deficits 90 days after the surgery. Control MRIat intervals of 1 and 4 years confirmed absence of the tumor. Discussion: Cavernomas are vascular malformations, consisting ofcoarsely dilated vascular channels and coated by a single layer of endothelial cells, devoid of endothelium and myothelium. Theyrepresent only 5-12% of all vascular pathologies of the medulla, with only 10% affecting the pediatric population. There are onlythirty cases of pediatric intramedullary spinal cavernomas in the literature, with predominance among males (2.1:1). Thoracic andcervical spinal cavernomas consist in 55% and 45% of the cases, respectively. In six cases (20%) cavernomatous lesions were associatedwith synchronic intracranial cavernoma. Among the reported cases, only one had poor evolution after surgery, whereas six patientspersisted with prior symptoms. Conclusion: Spinal intramedullary cavernomas are rare entities, especially in the pediatric population,and are treated with surgery which improves prior neurologic deficits, besides preventing rebleeding.

Intramedullary cavernomas – an own experience and a review of the literature

Intramedullary cavernomas – an own experience and a review of the literature

Long-term Postoperative Clinical Outcomes after Intramedullary Cavernoma Resection

Long-term Postoperative Clinical Outcomes after Intramedullary Cavernoma Resection

Intramedullary cavernoma: A surgical resection technique

Intramedullary spinal cavernoma is a rare vascular disease constituting 5–12% of all spinal vascular tumors. The clinical course is usually characterized either by an acute neurological deterioration, recurrent episodes of neurological deficits or by a slowly progressive neurological decline. Microsurgical removal is recommended when the symptoms become clinically relevant and the lesion appears accessible. In this article, we present a surgical technique to completely resect an intramedullary cavernoma with the aid of intraoperative electrophysiological monitoring and intraoperative real-time ultrasound guidance. A brief description of current management of this pathology is also presented.

Surgical management of symptomatic spinal cord and intracerebral cavernomas in a multiple cavernomas case

Abstract Multiple cavernous malformations are associated with familial cases and are present in 10-20% of all cavernoma cases. 5% of cavernomas are located intramedullary and of these only 10% present multiple cavernomas. With the availability of echo gradient MRI the cases of multiple cavernomas are diagnosed earlier and it is not rare that it uncovers multiple cavernomas in cases where only a single lesion can be identified on regular MRI sequences. We present the case of a 55 years old woman presented with a two years history of mild backache, followed by progressive lower legs motor deficit and urinary retention. The spine MRI showed an intramedullary T2/3 lesion and the cerebral MRI established the diagnosis of multiple cavernomas. One year after the intramedullary cavernoma was operated with success, she developed generalized seizures and a new cerebral MRI showed bleeding and volume growth of one right temporal pole cavernoma. The cerebral lesion was resected successfully and the patient was discharged free of seizures. This familial type multiple cavernomas cases should be screened and followed with repeated brain and spine MRI’s every year.

Aim and indications of spinal angiography for spine and spinal cord surgery: Based on a retrospective series of 70 cases

AimFor spinal surgery, computerized tomography (CT scan) and magnetic resonance imaging (MRI) have clear indications and are easily accessible. In contrast, the indications and the use of spinal angiograms (SA) remain unclear, and many centres performing spinal surgery do not have an access to SA. Based on a retrospective study, the role of SA in spinal surgery is assessed and their indications are discussed. Material and methodsA retrospective series of 72 SA in 70 patients is presented. No procedural accident occurred. SA was performed under general anaesthesia in 57 cases (82%). In 61 patients, locating the radiculomedullary arteries (RMA) was obligatory and performed in all cases: for 14 patients (21%), RMA were identified using the forecasted surgical approach (4 patients with degenerative disc disease out of 10 in the entire series were included), and modified. No ischaemic complications were observed in the series. Thirty-nine patients were treated for a tumour that was considered hypervascular (based on a histological hypothesis or the MRI data): 20 of them (51%) were preoperatively embolised and in only 8 cases was the operation considered “haemorrhagic” by the surgeon (among which, 3 intramedullary hemangioblastomas were included). No accident was observed during the embolisations. Thirteen patients presented with a vascular or haemorrhagic lesion (4 arteriovenous malformations, 6 dural arteriovenous fistulas, 3 intramedullary cavernomas): in all these cases, the SA was indispensable for the diagnosis and the decision-making process. Seven patients were treated by embolisation. In the last 8 cases, SA was considered for the diagnosis of a clinical worsening myelopathy with a non-contributive MRI, but it was not useful in providing a positive diagnosis. ConclusionsSAs were performed with different goals: (i) localization of RMA when a surgical approach between T4 and L2 involved the intervertebral foramen, or when an anterior approach was considered in order to avoid severe ischaemic complications (40% of the degenerative disc patients and 33% of the extramedullary tumour patients in this series); (ii) preoperative embolisation in cases of extramedullary tumours probably considered hypervascular (51% of the cases in the series) or in cases of arteriovenous shunt lesions (7 of 13 patients were treated by embolisation); (iii) as a diagnostic tool, SA is indispensable when MRI can reveal vascular abnormalities; it also provides information about the vascularisation as well as the endovascular possibilities in extramedullary tumours. In contrast, SA was not useful for intramedullary tumours because the RMA preoperative localisation is not mandatory (posterior approach), and embolisation seemed ineffective. SA was also not useful for the diagnosis of myelopathy with normal MRI. In the future, angiograms and MR angiography of the spinal cord may be useful in order to avoid general anaesthesia for a diagnostic procedure, but not practical to obtain access for endovascular treatment.

Spinal intramedullary cavernomas. Personal experience reffering to six cases

Abstract Despite cavernous malformations of the CNS are pathologically similar, intramedullary cavernous malformations are very rare lesions, increasingly recognized after introduction of magnetic resonance image, generating gradual neurological decline, with severe deficits or acute loss of spinal function. We report our experience on six patients with intramedullary cavernomas defining the spectrum of presenting symptoms and signs analyzing the role of surgery as a treatment for these lesions. We present our experience with 2 cervical and 4 thoracal spinal intramedullary cavernoma from 2010 to 2014 searching history, onset of clinical manifestation, neurological status, radiological findings, operation, and clinical outcome. Among 6 patients male were 2 cases; female 4 cases; mean age was 42 years (range 25-72 years); mean duration of symptoms were 1,5 years (range 5 days and 2 years) with slowly progressive neurological decline. In two cases there was acute onset of neurological compromise. In all cases diagnosis was made on MRI and lesions were possible to be radically excised and gently extracted from the hemosiderin-stained bed inside of the spinal cord via a laminectomy and midline myelotomy with microsurgical techniques. The surgical outcome on a mean duration of follow up of 12 months were: for 4 cases - the patients neurological conditions remarkably improved 1 month later, for 2 cases no improvement were remarked. No recurrent hemorrhages were recorded. A follow-up MRI examination was made in all cases to confirm complete removal of the cavernous angioma. Spinal intramedullary cavernoma should be early recognized by MRI, can be positioned in a precarious position and generate significant neurologic deficits than cranial cavernomas. For symptomatic intramedullary cavernous malformations extended to the dorsal surface of the spinal cord, total resection with microsurgical techniques can offer good or excellent outcome, restoring neurological status and to stop chronic deterioration and acute rebleeding. To asymptomatic patients with deeper lesions which entail a higher operative risk, but also a surgically manageable cause of myelopathy a closed observation is mandatory.

Clinical features and microsurgical treatment of spinal intramedullary cavernoma

To explore the clinical features and microsurgical treatment of spinal intramedullary cavernomas (SICs). A total of 21 patients with pathologically confirmed SIC undergoing microsurgical resection of cavernomas at Beijing Tiantan Hospital and Taian Central Hospital from June 2005 to December 2012 were reviewed retrospectively. The postoperative neurological status improved (n = 13) and remained unchanged (n = 6) and aggravated (n = 2). The clinical features of SICs are related with their different pathological processes. Magnetic resonance imaging (MRI) is specific for the diagnosis of SICs. Appropriate strategies and refined microsurgical techniques are essential for improving the clinical efficacies of SICs.

18椎体レベルにわたり広がった非外傷性脊髄内出血の1例

Intramedullary spinal cord hemorrhage (hematomyelia) is rare and usually related to trauma. Spinal vascular malformations such as intramedullary cavernomas and intradural arteriovenous malformations are the most common cause of atraumatic hematomyelia. Other considerations include warfarin or heparin anticoagulation, bleeding disorders, spinal cord tumors, and delayed complication of spinal radiation. We report the case of 48-year-old man receiving warfarin and aspirin therapy, who showed upper limb pain and dysesthesia from left axilla to left femur. Paraplegia, sensory disturbance, bladder and rectal disturbance developed gradually over two weeks, accompanied by severe back and neck pain. MRI showed hematomyelia extending from the C1 to T11 segments of the spinal cord. The hemorrhage was located mainly in the left side of the posterior column. Few cases of hematomyelia extending over 18 segments of the spinal cord have been reported. Past literature reports of hematomyelia tend to extend longitudinally above and below the area of initial hemorrhage. We thought that the shape of this hematomyelia extending longitudinally over several segments was formed by a similar pathogenesis to pencil-shaped softening of the spinal cord.

Intractable Neuropathic Pain in Spinal Intramedullary Cavernoma Treated Successfully with a Novel Combination Cream

To the Editor: Herein, we report our successful use of a compounded cream to treat a patient with neuropathic pain due to an old hemorrhage caused by a spinal intramedullary cavernoma, resistant to oral analgesics. In July 2004, a 63-year-old man faced a sudden onset of burning pain in his right armpit, upper back, and neck after strenuous rotational movement of the thorax. Soon tingling in his right arm and numbness and weakness in his legs developed. Diminished sensibility for touch, pinprick, warmth, and cold at the right side, from Th12 downward was found. Magnetic resonance imaging (MRI) showed a cystic process extending from C4 until Th2. Slowed conduction velocities were present in C5. Syringomyelia was diagnosed by two different neurosurgeons. The patient refused surgery because of potential for complications. Six …

Intramedullary cavernoma presenting with hematomyelia: report of two girls

IntroductionLess than 20 children with intramedullary cavernoma (ImC) have been reported in the English literature; however, cases with an unfavorable outcome may be underreported. Whereas these are predominantly boys, we report two girls who presented with hematomyelia (one cervical, one thoracic) and an acute, severe neurological deficit.Case materialA 10-year-old girl complaining about lower thoracic pain for several days suddenly developed lower body dysesthesias and paraparesis. Magnetic resonance (MR) demonstrated hematomyelia (T8–T11), intramedullary edema (T6–L1), and an ImC at T9–T10. Within an hour, she progressed to paraplegia and was therefore operated immediately. She slowly recovered regaining independent ambulation and continence. MR after 2 years shows no recurrence. A 7-year-old girl suddenly developed cervicalgia and paresis of her left arm and leg. MR demonstrated hematomyelia and an ImC at C4–C6. She gradually recovered with minimal residual deficit at 3 months and was subsequently operated uneventfully. Multiple cerebral cavernomas and a familial autosomal cavernous malformation syndrome were diagnosed. The following 1.5 years, she complained of intermittent cervicalgia and left brachial dysesthesias, with MR suggesting active residual cavernoma. Interestingly, her complaints gradually disappeared, and she is currently asymptomatic. MR after 3.5 years shows minimal cord swelling no longer suggesting active residual cavernoma.ConclusionWith adequate surgical treatment either in the acute phase in case of dramatic deterioration or after clinical recuperation, prognosis of symptomatic ImC may be surprisingly good. However, subtotally resected lesions and/or syndromal cases may recur, requiring further treatment. Definitive answers await more cases with longer follow-up.

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.

Intramedullary cavernous angioma of the spinal cord in a pediatric patient, with multiple cavernomas, familial occurrence and partial spontaneous regression: case report and review of the literature

The authors present their experience with the 17th pediatric intramedullary cavernoma reported in English literature. The patient firstly underwent surgery for a left frontal cavernoma when he was 2 years old. Also the child's mother was operated for a C2-C3 intramedullary cavernoma. He grew up normally and the radiological follow-up was negative for other brainstem cavernous malformations. When he was 11 years old he complained a worsening tetraparesis. A cerebral and spinal magnetic resonance (MR) imaging revealed the presence of a C1 intramedullary cavernoma and a pontine cavernoma. He underwent surgery for the cervical lesion, which was completely removed. The postoperative course was regular with a total recovery from the neurological deficit. The boy underwent a radiological follow-up, monitoring the pontine lesion, which spontaneously regressed when he was 19 years old. The rarity of the pediatric intramedullary cavernoma, the familial occurrence, and the spontaneous regression of the pontine cavernoma make this case very peculiar.