Intramedullary Cavernous Angioma Research Articles

Multiple spinal intramedullary cavernous angiomas with bleeding episode mimicking an intramedullary tumor

Intramedullary cavernous angioma is a rare vascular malformation compared to cerebral cavernous malformation. The incidence of cavernous angioma is about 3 - 5% of all central nervous system lesions, 5 - 12% of all spinal vascular lesions, and 1% of all intramedullary lesions in pediatric patients. Although intramedullary cavernous angioma has the same histological picture as cerebral cavernous angioma, the natural history, and surgical approach are different from cerebral cavernous angioma. Due to its location in the eloquent area of the spinal medulla, a slight change in the size of the lesion can affect the neurological function of the patient. We describe a case of an intramedullary cavernous angioma with hemosiderin post bleeding, located in the cervical cord which was initially misdiagnosed as hemorrhagic ependymoma. On whole spine MR imaging we also found an intramedullary cavernous angioma in the lower thoracal cord at the T12 level with mild hemorrhage. Abdominal MRI showed cavernous angiomas in both kidneys. The patient underwent surgical treatment with removal of the lesion in the cervical cord and T12 level, with histopathologic findings consistent with cavernous angioma. No malignancy was detected. Post-surgery, the symptoms gradually improved. Symptomatic intramedullary cavernous angioma tends to bleed repeatedly and being unstable. Early diagnosis and surgical treatment can prevent rebleeding and more severe symptoms.

Symptomatic Intramedullary Cavernous Angioma During Pregnancy. Case report

The cavernous angioma is a vascular malformation that can affect various organs, and its location in the spinal cord is unusual. We report a case of a pregnant patient who presented with pain in the lower limbs and urinary retention, diagnosed with cavernous angioma of the spinal cord and treated with surgical excision. Our case highlights this rare pathology and its initial presentation during pregnancy.

Multiple Intramedullary Cavernous Angioma of the Cervicothoracic Spine: Case Report and Review of the Literature

Intramedullary cavernous angioma (ImCA), which was originally thought to be an uncommon vascular lesion, has shown an increased incidence since the advent of magnetic resonance imaging. We present a case of multiple ImCAs in the cervicothoracic region. The patient presented with slow, progressive motor weakness in the upper and lower limbs. Surgical intervention was performed because of misdiagnosis as a spinal cord tumor. The patient underwent decompression surgery because no neoplasm was noted in the course of the operation. The angioma regressed on follow-up magnetic resonance images 3 months later.

Surgical management of intramedullary cavernous angiomas and analysis pain relief.

The objective of this study was to conduct a retrospective analysis of the clinical characteristics of 20 individuals with intramedullary cavernous angiomas (ICA) presented with serious pain complaints. This study was to investigate the efficacy of short- and long-term pain relief following surgical resection. Between 2006 and 2012, 55 patients with ICA were surgically managed in our institution. Of these 20 (36.4%) patients presented with serious pain as a unique clinical feature. Numerical pain scores (NPS: 0-10) were used to assess the patients' pain levels preoperatively, as well as at 1 month and 1 year postoperatively. All the patients had magnetic resonance imaging (MRI) preoperatively and during follow-up. IBM SPSS Statistics 19.0 was used to analysis the outcomes. Of the 20 patients with ICA, 9 (45%) required cervical and 11 (55%) thoracic surgery. Seven (35%) patients presented with radicular pain and 13 (65%) presented with central pain. Pain improved from a total mean preoperative score of 8.60 to total mean score of 2.95 (P < 0.01) at one month and 3.35 (P < 0.01) at one year post-surgery. However, the pain symptoms completely disappeared in the long-term follow-up only in three (15%) patients. Five (25%) patients reported new pain symptoms with no lesion reoccurrence postoperatively. Pain is the common complaint in patients with ICA. Surgery is effective in providing short- and long-term pain relief. However, long-term follow-up measures on postoperative pain levels show that the pain does recur in the months following surgery.

Coexistence of multiple cavernous angiomas in the spinal cord and skin: a unique case of Cobb syndrome

Cobb syndrome is a rare, noninherited, neurocutaneous disease characterized by vascular abnormality of the spinal cord and is associated with vascular lesions in the skin at the same metamere. The majority of spinal vascular lesions are arteriovenous malformations, and skin lesions are mostly port-wine angiomas. The authors report the first case of multiple intramedullary cavernous angiomas (CAs) accompanied by skin CAs within the same metamere. A 42-year-old man presented with an acute onset of gait disturbance, low-back pain, and urinary incontinence. Magnetic resonance imaging of the thoracolumbar spine showed homogeneously enhanced lesions on a contrast-enhanced T1-weighted image and a hypointense area on a T2*-weighted image surrounding this enhanced lesion, between the T-12 and S-1 levels. Purple protruding skin lesions were detected on the left side of his gluteal region. The patient received a laminectomy followed by evacuation of a hematoma and partial removal of the tumor, which completely resolved his neurological symptoms. Pathological examinations showed that the spinal and skin lesions were CAs, suggesting that these vascular lesions developed congenitally. Cavernous angiomas associated with Cobb syndrome present with multiple lesions spanning more than 3 vertebral levels, making it difficult to completely resect these tumors. Although Cobb syndrome is an uncommon disease entity, it should be considered if a patient manifesting with neurological deficits has skin vascular lesions, including CAs.

Spinal cavernous angioma complicated by hemorrhage: susceptibility-weighted imaging findings

A 16-year-old boy presented with posterior cervical pain radiating to the right upper limb. Cervical magnetic resonance imaging (MRI) revealed an intramedullary cavernous angioma (CA) in the level of C2. Susceptibility-weighted imaging (SWI) showed longitudinal hypointensity from the bulb to the level of T1, which was suggestive of a longitudinal fiber-dissecting hemorrhage ( ). Intramedullary CAs are associated with an increased risk of hemorrhage. SWI is optimized for detection of magnetic susceptibility effects of blood products . SWI is more [...]

Intramedullary Cavernous Angiomas of the Spinal Cord: Clinical Characteristics of 13 Lesions

Magnetic resonance imaging has increased the incidence of the diagnosis of intramedullary cavernous angioma. Surgical therapy tends not to be recommended for asymptomatic lesions, but symptomatic lesions that bleed recurrently should be treated. The natural course of intramedullary cavernous angioma remains unknown and arguments have been raised against the surgical treatment of symptomatic lesions. We reviewed the clinical features of 13 intramedullary cavernous angiomas in 12 patients surgically treated between 1988 and 2009. The 7 men and 5 women were aged from 14 to 60 years, the preoperative interval ranged from 0 to 161 months, and the mean number of hemorrhages in the 13 lesions was 2.5. Sixteen operations were performed to treat the 13 lesions. The surgical approach depended on the lesion location. The outcome of patients with mild to moderate preoperative symptoms (McCormick grades I-III) was significantly better than that of patients with severe symptoms (McCormick grade IV) (p < 0.05). Symptomatic intramedullary cavernous angioma tends to bleed repeatedly. The lesion should be surgically removed to avoid further deterioration due to recurrent hemorrhages. The shortest path approach should be selected based on preoperative images and complete removal should be attempted. Residual lesion may be masked by surrounding gliosis, so careful postoperative follow up is necessary.

Hematomas espinales: la apoplejía espinal

IntroductionSpinal haematomas (SH) are a rare pathology. They can produce a rapid and irreversible neurological deterioration. MethodIn this retrospective study, we review 8 cases of SH treated in our centre over the last five years. Data collected were: age, sex, predisposing factors, clinical status, radiological features, treatment and outcome. ResultsFive patients were female and three male. Age ranged between 13 and 81 years. Five patients were hypertensive. Four had a coagulation disorder. In three patients SH occurred after physical effort. One case appeared after a lumbar puncture and another was secondary to intramedullary cavernous angioma. All patients presented with pain followed by neurological symptoms. Four cases were epidural, two subdural and two were intramedullary. All epidural SH and one subdural SH, were located dorsal to medulla. Five patients were operated on due to progressive neurological deterioration. Only one of them showed neurological improvement. Patients who were not operated on, had a better neurological status and they improved spontaneously. Two of them were discharged without neurological symptoms. ConclusionsThere is controversy over the physiopathology of SH. The triggering mechanisms are unknown. The vessel (artery or vein) and the anatomical compartment in which SH arise are also unknown. Prompt diagnosis and urgent surgical treatment are needed when the patient has neurological symptoms. In these cases, the most important prognostic factor is the preoperative neurological status.

Spinal haematomas: The spinal aplopexy

Introduction Spinal haematomas (SH) are a rare pathology. They can produce a rapid and irreversible neurological deterioration. Patients and method In this retrospective study, we review 8 of SH treated in our centre over the last five years. Data collected were: age, sex, predisposing factors, clinical status, radiological features, treatment and outcome. Results Five patients were female and three male. Age ranged between 13 and 81 years. Five patients were hypertensive. Four had a coagulation disorder. In three patients SH occurred after physical effort. One case appeared after a lumbar puncture and another was secondary to intramedullary cavernous angioma. All patients presented with pain followed by neurological symptoms. Four cases were epidural, two subdural and two were intramedullary. All epidural SH and one subdural SH were located dorsal to medulla. Five patients were operated on due to progressive neurological deterioration. Only one of them showed neurological improvement. Patients who were not operated on had a better neurological status and they improved spontaneously. Two of them were discharged without neurological symptoms. Conclusions There is controversy over physiopathology of SH. The triggering mechanisms are unknown. The vessel (artery or vein) and the anatomical compartment in which SH arise are also unknown. Prompt diagnosis and urgent surgical treatment are needed when the patient has neurological symptoms. In these cases, the most important prognostic factor is the preoperative neurological status.

Infantile Cervical Intramedullary Cavernous Angioma Manifesting as Hematomyelia -Case Report-

A 2-month-old infant presented with a cervical intramedullary spinal cord cavernous angioma manifesting as left hemiparesis caused by hematomyelia. Osteoplastic laminotomy of the cervical spine was carried out with subsequent microsurgical excision of the intramedullary spinal cord cavernous angioma. Magnetic resonance imaging revealed no residual tumor. At 25 months after the surgery, she presented no neurological deficit without recurrence and cervical deformity. This case of infantile intramedullary spinal cord cavernous angioma presenting with cervical hematomyelia shows osteoplastic laminotomy of the cervical spine might be helpful to prevent consequent cervical deformity in pediatric cases.

Ventrally Located Cervical Intramedullary Cavernous Angioma: Selection of Posterior and Anterior Approaches -Case Report-

A 41-year-old woman presented with a rare cervical intramedullary cavernous angioma manifesting as acute paralysis in her left upper extremity. Magnetic resonance imaging revealed hematomyelia. Laminectomy (C2-C7) was performed, followed by posterior midline myelotomy and removal of the intramedullary hematoma and the tumor. She showed marked improvement after the surgery. However, 3 years and 10 months later the patient showed slight intramedullary bleeding located ventrally at the same level. Anterior approach with corpectomy was performed. Complete removal of the tumor was attained and salvage surgery was performed. Postoperative stabilization was achieved with excellent clinical outcome. The anterior approach is useful method as a salvage surgery for ventrally located cavernous angioma.

Clinical characteristic of spinal vascular malformation in pediatric patients

Seventy-two pediatric spinal vascular malformation cases were reviewed and the characteristics of their clinical symptoms, diagnoses, and therapies were analyzed. A thorough overview was compiled examining patient sex, age, location, history, development, treatment, clinical, and anatomical results. Spinal cord arteriovenous malformation was the most common (44.4%) subtype to be seen in these pediatric patients, while subdural perimedullary arteriovenous fistula (23.6%) was the second, followed by Cobb's syndrome (13.9%) and intramedullary cavernous angioma (5.6%). No spinal dual arteriovenous fistulae were found in infants. The highest incidence was seen during the infant and adolescent periods. Sixty-nine cases were treated by surgeries, embolizations, or a combination of both, and 71.5% of them had improved. Early diagnosis and treatment are required. Surgery and embolization, or a combination of the two, are the current candidates for treatment.

The clinical outcomes after complete surgical resection of intramedullary cavernous angiomas: changes in motor and sensory symptoms

Evaluation of the surgical effects of patients with intramedullary spinal cord cavernous angiomas (CAs). To assess the sensory and motor deficits in the clinical symptoms of patients with intramedullary CAs after complete surgical resection. Seoul National University Hospital, Korea. The authors reviewed 14 patients with intramedullary spinal cord CAs who underwent surgical resection between 1995 and 2006. The average follow-up duration was 55.0+/-45.1 months. To analyze the changes in the patients' symptoms after surgery, the ASIA (American Spinal Injury Association) and JOA (Japanese Orthopedic Association) scoring systems were used at different time points: preoperative, postoperative and during the final follow-up period. The patients' mean ASIA score at follow-up was increased in comparison to the preoperative score (97.2+/-5.3 vs 95.2+/-7.6, P=0.201), but this difference was not significant. The mean JOA motor score at follow-up was slightly increased in comparison to the preoperative score (7.2+/-1.3 vs 6.8+/-1.3, P=0.107). There was a significant difference between the patients' preoperative and follow-up JOA sensory score (4.3+/-1.0 vs 4.9+/-0.9, P=0.007). The rates of complete resolution of motor and sensory deficits were 45.5% (5/11 patients) and 7.1% (1/14 patients), respectively. Although complete surgical resection could improve the clinical symptoms of intramedullary CAs, the sensory deficits usually remain in the long-term period after complete resection.

The diagnosis and surgical management for intramedullary spinal cord cavernous angioma

The clinical diagnosis and surgical management of intramedullary spinal cord cavernous angioma were discussed. Total 19 patients with intramedullary cavernous angioma were analyzed retrospectively on the clinical manifestation, radiographic feature, diagnosis and differentiation, surgical technique and caution. Of all the 19 patients, averaging 38.7 years old, 14 were male and 5 were female. Nine patients were followed. All the 19 patients pathologically diagnosed with spinal cord cavernous angioma got good surgical results, besides one patient showed loss of proprioception. Nine patients were followed up and all demonstrated improvement on neurological function. The clinical symptom of most of the patients with spinal cord cavernous angioma presented mildly at onset, but deteriorated gradually because of repeated prehemorrhage. Since the lesion showed some characteristic in MRI, MRI examination was regarded as an important diagnostic tool. Dissection should be done between the tumor and the gliosis during the surgical procedure, special attention should be paid to avoiding tumor residual. It was not necessary to aggressively evacuate the hematoma derived from tumor hemorrhage, which extended along the central canal up and down, except obvious occupied syndrome exited.

Intramedullary spinal cord cavernous angioma in children: case report and literature review

Intramedullary cavernous angiomas (ImCA) of the spinal cord are very uncommon malformations especially in the pediatric population where only ten cases have yet been reported within the available literature. In this paper, the authors report the case of a 12-year-old girl presenting with a T11 level hematomyelia because of a cavernous angioma. Microsurgical excision was performed with good clinical outcome and no magnetic resonance imaging evidence of residual cavernoma 30 months later. Despite the small number of cases reported in children, this one contributes to the literature identifying special features of presentation and management of ImCA for pediatric patients. Because a higher risk of recurrent bleeding has been demonstrated for ImCA, with dramatic clinical consequences, microsurgical removal remains the only definitive treatment.

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.

Klippel-Trenaunay-Weber syndrome and intramedullary cervical cavernoma: a very rare association. Case report

Background Klippel-Trenaunay-Weber syndrome is a rare mesodermal phakomatosis characterized by cutaneous hemangiomata, venous varicosities, and osseous–soft tissue hypertrophy of the affected limb. As the pathologic aspect of KTWS arises from the site in which malformations occur, the clinical picture varies widely from patients who complain for cosmetic reasons to patients with life-threatening lesions. Case Description We describe a very rare case in which KTWS was associated with a cervical intramedullary cavernous angioma surgically treated. Conclusion This report confirms the wide range of expression of vascular abnormalities in neurocutaneous developmental diseases and the need of a careful multisystemic evaluation of these patients.

The Anterolateral Partial Vertebrectomy Approach for Ventrally Located Cervical Intramedullary Cavernous Angiomas

We report three cases with ventral cervical intramedullary cavernous angiomas. An anterolateral partial vertebrectomy was performed to surgically approach and successfully resect these lesions. All three patients presented with numbness in the upper extremities and investigation revealed a cavernous angioma on the ventral aspect of the spinal cord in each case. The lesion was exposed by an anterolateral partial vertebrectomy in all cases. Postoperative stabilization was achieved by using autografted iliac bone in all patients. In two patients, locking screws and plates were also used. Complete resection of the cavernous angioma was performed in all patients. There was symptomatic relief in all cases, and there was no postoperative morbidity. Anterolateral partial vertebrectomy provides direct exposure and is probably an ideal approach for selected cases with ventrally located intramedullary cavernous angiomas.

Intramedullary cavernous angioma. Two case-reports

Intramedullary cavernous angiomas are extremely rare. We retrospectively reviewed two cases in a 24-year-old man and 40-year-old woman. T2-weighted magnetic resonance images showed the cavernoma at the cervical and lower thoracic spine, respectively. The patient with cervical involvement also had an asymptomatic cavernoma in the cerebellum. In both patients, the outcome was favorable after complete surgical removal of the lesion.

Angiome caverneux intramédullaire. À propos de deux cas

Intramedullary cavernous angiomas are extremely rare. We retrospectively reviewed two cases in a 24-years-old man and 40-years-old woman. T2-weighted magnetic resonance images showed the cavernoma at the cervical and lower thoracic spine, respectively. The patient with cervical involvement also had an asymptomatic cavernoma in the cerebellum. In both patients, the outcome was favorable after complete surgical removal of the lesion.