Spinal Cavernomas Research Articles

Cauda Equina Cavernous Angioma

AbstractCavernous angiomas are rare vascular lesions, most occurring supratentorially, with spinal cavernomas being even rarer. They have a varied magnetic resonance imaging (MRI) appearance which is usually not diagnostic. A 42-year-old man presented with progressive low backache for the past 5 years and was found to have a mild right ankle weakness. MRI showed an intradural extramedullary lesion at the L1 vertebral body level. Intraoperatively, a dark reddish-blue mulberry-like lesion was found attached to a nerve root that had to be sacrificed during the excision. Histopathology confirmed that it was a cavernous angioma and the patient had no added deficits postoperatively. Cauda equina cavernomas are extremely rare and hence not thought of as a differential preoperatively. They are relatively simple to remove but will mostly need the sacrifice of the adherent nerve root. Most cases do well postoperatively with stable deficits and some improving.

Cobb Syndrome Associated with Spinal Cavernoma – Case Report

AbstractCobb syndrome, or cutaneomeningospinal angiomatosis, is a rare condition that affects young adults, and its etiology has not been completely elucidated. It is characterized by a cutaneous sign, or stigma, associated with spinal or intracranial malformations. The symptoms are quite diverse, but, in most cases, the disease presents motor deficit and pain. The present study reports the case of a 48-year-old female patient, who initially sought dermatological medical care for a single skin lesion in the posterior cervical region. During the excision, it was noticed that the lesion had contiguous behavior to the deep anatomical planes, thus requiring the evaluation of the neurosurgical team. The purpose of this report is to describe this rare disease, covering more details about diagnosis and therapy.

Acute Flaccid Paralysis as Initial Presentation of Spinal Cavernoma (P12-5.010)

<h3>Objective:</h3> Cavernous angioma can occur in pediatrics throughout the central nervous system. However, the intramedullary spinal cord accounts for just 1% of all intramedullary pediatric spinal cord lesions <h3>Background:</h3> Cavernous angiomas consist of enlarged, packed vessels without interposition of the normal brain parenchyma. In addition, surrounding hemosiderin deposits and gliosis, and sometimes calcifications can be noted. The clinical presentation in pediatrics is usually characterized by acute neurologic decline that occurs because of acute hemorrhage with extension into the spinal parenchyma, rather than gradual onset of myelopathy as we see in adults. The most widely used diagnostic tool for detecting cavernous angiomas is MRI. Unfortunately, MRI can be negative before the occurrence of bleeding. <h3>Design/Methods:</h3> We report a 13-year-old female presented with 3-day history of worsening back pain associated with bilateral lower extremities, weakness, excruciating pain, tenderness and paresthesia. In addition, she also reported urinary retention. On admission, her motor power grade was 2/5 (Medical Research Council Scale for Muscle Strength) in both lower limbs with absent deep tendon reflexes and sever asymmetrical sensory loss below the T7 dermatome bilaterally. Initial MRI neuroaxis showed enhancing 12 mm focal nodular lesion at T10 level with perilesional edema. Twelve hours later, the patient had acute worsening in her strength, on examination of her motor strength was found to be 0/5 (MRC). Repeat imaging showed expansion of hemorrhage. She underwent T9-T11 laminectomy for resection of the spinal cord lesion. On the gross examination benign lesion with abnormal vascular anatomy was identified. Histology was consistent with cavernous angioma with reactive changes. <h3>Results:</h3> NA <h3>Conclusions:</h3> Cavernous angiomas are considered as rare cause for intramedullary pediatric spinal cord lesions. It still should be considered as one of the differentials for acute flaccid paralysis. Early surgical intervention and resection should be considered early whenever feasible. <b>Disclosure:</b> Dr. Naz has nothing to disclose. Dr. Gharaibeh has nothing to disclose. Dr. Saleem has nothing to disclose. Dr. Mahfooz has nothing to disclose.

Spinal Epidural Cavernoma of the Thoracic Spine: a case report

BACKGROUND AND OBJECTIVE Cavernomas are well-circumscribed and multilobulated vascular malformations that can occur nearly anywhere in the body. Cavernomas are found 80% supratentorially, 15% in posterior fossa and only 5% in the spinal cord. Spinal cavernomas are most commonly intramedullar. Purely epidural spinal cavernoma constitute of less than 4% of all spinal epidural lesions. We report a case of histopathologically confirmed epidural cavernoma of the thoracic spine. CASE PRESENTATION A 24-year-old woman presented with a 6-month history of progressive chronic upper back pain. Physical examination revealed no neurological deficit. Her VAS pain scale was 6/10. Conservative treatments did not relieve her symptoms. Thoracal MRI without contrast shown a lesion that hyperintense on T2-weighted images and isointense on T1-weighted images. The lesion sized 14x13x7 mm at T5 vertebral level located at posterolateral spinal canal. Thoracal MRI with contrast showed extradural mass with homogenous contrast enhancement. The mass was located at left posterolateral spinal canal suspected extradural meningioma. The surgery was done via posterior approach. Left hemilaminectomy was done at T4-T5 vertebral level. Reddish soft tissue mass was observed at the epidural space. The mass was well defined and there were no signs of bony invasion. Gross tumor removal was done under the microscope. Histopathological examination of the mass showed large dilated vessel filled with erythrocyte and lined by endothelium which support the diagnosis of cavernoma. After the surgery, her pain symptom was improved. CONCLUSION Spinal epidural cavernomas are rare, but it should be considered as one of the differential diagnosis for spinal epidural lesions. Exact preoperative diagnosis is hard because there are no pathognomonic MRI findings of spinal epidural cavernoma. Total surgical resection of the lesions was the treatment of choice in symptomatic patients to prevent progressive neurological deficit and prospective hemorrhage.

Intramedullary spinal cord cavernous malformations—association between intraoperative neurophysiological monitoring changes and neurological outcome

BackgroundMicrosurgical resection of spinal cord cavernous malformations can be assisted by intraoperative neurophysiological monitoring (IONM). While the clinical outcome after surgical resection has been discussed in several case series, the association of intraoperative IONM changes and detailed neurological outcome, however, has not been analyzed so far.MethodsSeventeen patients with spinal cavernomas underwent surgery between 02/2004 and 06/2020. Detailed neurological and clinical outcome 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 outcome at 3-month and 1-year follow-up in order to identify surrogate parameters for an impending neurological deficit.ResultsCompared to the preoperative state, McCormick 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.ConclusionsReversible 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.

Cerebral and spinal cavernomas

Cavernous malformations or cavernomas belong to the angiodysplasias. They may be sporadic or familial and cause symptoms (epilepsy) despite the absence of aleft-to-right shunt. In addition to intracranial locations, spinal cavernomas are also found. Magnetic resonance imaging (MRI) and computed tomography (CT) are used for diagnosis PERFORMANCE: MRI, except for acutely or subacutely hemorrhaged cavernomas, is superior to CT for lesion detection. CT is reserved for acute diagnosis. MRI, especially susceptibility-sensitive gradient echo sequences, can also detect cavernomas without (sub-)acute hemorrhage or calcifications. MRI is also useful for differentiating the familial form vs. sporadic form. Digital subtraction angiography (DSA) is used for differential diagnosis in rare cases, as cavernomas show no correlate here.

P.171 Intradural-extramedullary spinal cavernoma with nerve root association: a case report and review of the literature

Background: Although 5% of cavernomas occur in the spine, intradural-extramedullary cavernomas are exceptionally rare. We present one such case of cavernoma associated exclusively with a nerve root, and review the literature for similar lesions. Methods: Case Report. A 45-year old male patient presented with a six-month history of numbness and paresthesia affecting his lower extremities bilaterally. MRI demonstrated a 16mm intradural-extramedullary lesion at T3-4, compressing the cord. The lesion was surgically removed en-bloc, requiring root sacrifice. The patient’s symptoms completely resolved post-operatively, and he remained asymptomatic at 3-month follow up. Results: Including the present case, 71 cases of intradural-extramedullary spinal cavernoma have been reported, including 50 with confirmed nerve root involvement. Patients most frequently presented between the ages of 40-59 (41%) with lesions at the lumbar level (54%). Confirmed subarachnoid hemorrhage was present in 14% at presentation, although 23% presented acutely. 49% presented with longstanding/progressive symptoms, and 11% with mixed acute-on-chronic presentation. 37% presented primarily with radiculopathy, 21% with myelopathy, and 11% with pain. Good postoperative recovery was documented in 80% of cases. Conclusions: Intradural-extramedullary spinal cavernomas may demonstrate a propensity for radiculopathy at presentation and exhibit substantial subarachnoid hemorrhage risk. Literature review supports the role of surgical resection.

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.

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

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.

Spinal Cavernomas: Outcome of Surgically Treated 10 Patients.

We report the preoperative and postoperative findings and also neurological follow-up results from 10 spinal cavernoma patients treated in our clinic. Several representative cases are presented in terms of clinical features, imaging results, and surgical outcomes. The data were retrospectively collected from patients' files in the hospital records and sorted with regards to clinical presentation, radiologic features, and operative findings. Patients received spinal MRI scans for the diagnosis of spinal cavernomas (SC) and postsurgical evaluation. Clinical presentation was evaluated via Ogilvy classification and symptoms were checked preoperatively and postoperatively at third month and first year using McCormick scale. Primary treatment was microsurgical operation aiming a gross total lesion resection. 10 spinal cavernoma patients between the ages 30 and 63 were treated. Six (60%) of the patients were diagnosed with cervical and four (40%) others were diagnosed with thoracic SC. Among the patient group, mean preoperative Ogilvy classification score was 2.3 ± 0.7.8 and McCormick score was 1.9 ± 0.7. There was no residual mass or relapse after surgery. One patient developed surgery-related left hemiparesis, which was normalized at 1 year follow-up. Patients must be diagnosed with MRI since it is nowadays a gold standard. Preoperative and postoperative scores are important in evaluating the patients' condition and improvement. The results from our patient series also reinforce the notion that immediate surgery should be the preferred treatment method for cavernomas. Intraoperative neurophysiologic monitarization should assist the surgery in order to prevent complications. In conclusion, microsurgery is a gold standard method that we recommend for cases of cavernomas, which will not recur if gross total resection is achieved.

Surgical outcome of microsurgical excision of brain and spinal cord cavernomas: Our 13 year single center experience

Surgical outcome of microsurgical excision of brain and spinal cord cavernomas: Our 13 year single center experience

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.

A retrospective and consecutive analysis of the epidemiology and management of spinal cavernomas over the last 20 years in a single center.

Spinal cavernous malformations (SCM) are rare lesions often presenting with acute onset of symptoms and progressive neurological deterioration due to hemorrhage into the spinal cord. With the aid of modern techniques, their surgical removal became much safer. The present study was undertaken to analyze the outcome of our series of surgically and conservatively treated patients with SCM. Over a period of 20 years, 20 surgically treated and 5 conservatively managed patients with spinal cavernous malformations were identified and enrolled into this analysis. Demographic data, clinical symptoms, localization and extension of the cavernoma, as well as pre- and postoperative neurological status were obtained. The clinical status was assessed using the Frankel score. Patients were followed up clinically and by MRI. Before surgery, 90% (18/20) of our surgical patients were classified as Frankel D (93.8%), whereas two patients (10%) were graded C. None of the patients had a worse Frankel score at the time of discharge. Eighty percent of them (16 cases) remained unchanged, and 20% (4 patients) improved during the first follow-up (mean 6.3 months, range 2-17 months). All improved patients had a superficially located SCM and were operated early (≤3 months). No worsening was observed during extended follow-up (range 9-134 months, mean 44.7 months). Five nonsurgically treated patients showed no significant clinical deterioration over a period of 6.7 years (mean, range 2.9-8 years). SCM localization and number of involved segments had no influence on outcome. Our data show that SCM can be resected with favorable neurological outcome by using intraoperative neuromonitoring. Within the follow-up period, patients treated conservatively remained in a stable neurological condition.

Intramedullary spinal cavernoma: clinical presentation, microsurgical approach, and long-term outcome in a cohort of 48 patients.

OBJECT Intramedullary spinal cavernoma (ISC) is a rare entity and accounts for approximately 5%-12% of all spinal vascular pathologies. The purpose of the present study was to examine the influence of clinical presentation, localization, and different surgical approaches on long-term outcome in patients treated for ISC. METHODS The authors performed a retrospective single-center study of 48 cases of ISC treated microsurgically over the past 28 years. Analyzed factors included preoperative clinical history, microsurgical strategies, neurological outcome (American Spinal Injury Association [ASIA] grade, Epstein and Cooper grade), and the occurrence of postoperative spinal ataxia. Univariate analysis was performed to identify factors influencing long-term outcome. RESULTS Preoperatively, 18.8% of all patients experienced a slow, progressive decline in neurological function and 33.3% suffered repetitive episodes of acute neurological deterioration over a time frame of months to years. Moreover, 16.7% noted the sudden onset of a severe neurological deficit, whereas 25% experienced the sudden onset of symptoms with a subsequent gradually progressive decline in neurological function. On long-term follow-up after treatment (mean ± SD, 79.3 ± 35.2 months), 70.8% of patients showed no change in neurological function, 6.3% suffered from a decline, and 22.9% improved neurologically. Thoracolumbar localization (p = 0.043), low preoperative Epstein and Cooper grade for the lower extremities (p < 0.001), and a low preoperative ASIA grade (p < 0.001) were identified as factors associated with an unfavorable outcome (ASIA Grade A-C). The rate of spinal ataxia related to surgical approach was 16.7%. CONCLUSION Postoperative neurological function in ISC patients is determined by the preoperative neurological status. On long-term follow-up after microsurgical treatment, 93.7% of patients presented with a stable or improved condition (ASIA grade); thus, definite microsurgical treatment should be considered as long as patients present with only mild symptoms after the diagnosis of symptomatic ISC.

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.

Cerebral cavernous malformation: Clinical report of two families with variable phenotype associated with KRIT1 mutation

We report two families with a variable presentation in association with a KRIT1 mutation. The index patient in Family 1 was a 9-year old girl who presented with left hemi-dystonia and a cerebral cavernous malformation was identified in the right lentiform nucleus. The maternal grandmother presented with a spinal cavernoma, which was operated at 35-years of age. The mother presented with intractable temporal lobe epilepsy in childhood and underwent temporal lobe resection at 27-years of age. The second family has also presented variably with the youngest member of this family presenting with generalised tonic-clonic seizures at 18-months of age.We report both these families with variable presentation of an autosomal dominant condition and describe the phenotypic presentation in both these families in further detail and review the published literature on this condition.

Spinale Gefäßmalformationen

Spinal vascular malformations are a group of rare diseases with different clinical presentations ranging from incidental asymptomatic findings to progressive tetraplegia. This article provides an overview about imaging features as well as clinical and therapeutic aspects of spinal arteriovenous malformations, cavernomas and capillary telangiectasia.

Spinal Cord Cavernoma—Operative Strategy and Results in 30 Patients

Many case reports and small series addressing the surgical management of spinal cord cavernoma have been published. However, only few larger series that would allow identifying operative strategies exist. After having treated 30 patients, we feel encouraged to report our experiences. A total of 30 patients (13 men and 17 women) were mainly admitted to our institution because of sensory deficits (83.3%), paresis (33.3%), and bladder dysfunction (26.6%). Magnetic resonance imaging revealed a spinal cavernoma mostly in the thoracic region (63.3%). All patients underwent surgery. The relation between pial surface and cavernoma guided the choice of approach and the myelotomy site. After laminectomy/laminoplasty, median myelotomy was done in 16.7% to reach a medially located cavernoma. In 60.0%, myelotomy was located at the dorsal root entry zone, for which a (partial) hemilaminectomy was sufficient. The laterality of the location guided the bony approach in the remaining 23.3% with exophytic cavernoma. Immediately after surgery, neurological worsening was seen in 56.7%, an improvement in 10.0% and an unchanged neurological status in 33.3%. During the follow-up, the rate of neurological worsening dropped to 10.0%, the improvement rate increased to 50.0%. In 40.0% of the patients, the symptoms remained unchanged or returned to preoperative status. The exact localization in relation to the pial surface guides the approach and area of myelotomy. In the majority of patients limited approaches are sufficient for successful cavernoma removal. In our series anterior approaches had not been necessary.

A Proposed Grading System of Brain and Spinal Cavernomas

Most cavernomas in the central nervous system are characterized by a benign natural course. Progressive symptoms warrant surgical removal. In the literature, the factors affecting long-term postoperative outcome are not statistically well confirmed. To perform a multifactorial analysis of risk factors on a large patient series and to use the results to propose a simple grading scale to predict outcome. We studied 303 consecutive patients with cavernomas treated surgically at our department from 1980 to 2009. Follow-up assessment was performed on average 5.7 years postoperatively (range, 0.2-36 years). The main outcome measure was the patients' condition at the last follow-up on Glasgow Outcome Scale. For statistical analysis, the outcome measure was dichotomized to favorable (Glasgow Outcome Scale 5) and unfavorable (Glasgow Outcome Scale 1-4). Binary logistic regression analysis was used to estimate the effect of age, sex, seizures, preexisting neurological deficits, hemorrhage, and size and location of cavernoma on long-term outcome. Infratentorial, basal ganglia, or spinal location and preexisting neurological deficit were the only independent risk factors for unfavorable outcome, with relative risks of 2.7 (P = .008) and 3.2 (P = .002), respectively. We formulated a grading system based on a score of 1 to 3. When applied to our series, the proposed grading system strongly correlated with outcome (P < .001, Pearson χ test). The risk for long-term unfavorable outcome was 13%, 22%, and 55% for grades 1 through 3, respectively. The proposed grading system showed a convincing correlation with postoperative outcome in surgically treated cavernoma patients.