Intramedullary Angioma Research Articles

Thoracic Spinal Cord Angiomatous Malformation Mimicking Intramedullary Tumor, Associated With Neurofibromatosis I (NF-1) And Advanced Scoliosis

Spinal angiomas are very rare vascular malformations that are clinically presented by slowly progressive myelopathy or sudden neurological deterioration when they bleed. Early diagnosis and surgical removal is mandatory before a rebleeding or a devastating lesion of the cord can occur. A 35-year old man with severe thoracic scoliosis since lateteenage and neurofibromatosis type I (NF-1) was gradually deteriorating, manifesting a spastic tetraparesis that was misinterpreted. Magnetic resonance images showed a space occupying lesion at D2-D5 level resembling an ependymoma or astrocytoma. The patient underwent surgical treatment and histology revealed an intramedullary angioma of venous type.

Clinical Syndromes, Natural History, and Pathophysiology of Vascular Lesions of the Spinal Cord

With superselective angiography and careful clinical evaluation, dural fistulas were found to be the most important and most frequent origin of spinal arteriovenous malformations. Diagnosis may not be made for months or years, however, because dural fistulas never bleed and clinical deterioration is slow. Therapy with interventional techniques or microsurgery is relatively safe. Perimedullary fistulas and intramedullary angiomas mostly deteriorate quickly because of hemodynamic or hemorrhagic problems. Close teamwork of interventionalists and neurosurgeons is mandatory.

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.

Intramedullary cavernous angiomas of the spinal cord: clinical presentation, pathological features, and surgical management.

Cavernous angiomas of the spinal cord are rare lesions that can cause severe neurological symptoms. We add a series of 6 patients with intramedullary cavernous angiomas of the spinal cord to 30 patients with 31 histologically verified lesions already described in the literature. Four types of clinical presentation were seen in the 36 patients: 1) discrete episodes of neurological deterioration with varying degrees of recovery between episodes (13 patients); 2) slow progression of neurological decline (12 patients); 3) acute onset of symptoms with rapid decline (8 patients); and 4) acute onset of mild symptoms with subsequent gradual decline lasting weeks to months (3 patients). Of the 36 patients, 25 (69%) were women and 11 (31%) were men. The peak age of presentation was in the fourth decade. The thoracic spinal cord was affected in more than half the patients, with the cervical cord being the next most common location. Histological examination demonstrated the closely apposed vascular channels characteristic of cavernous angiomas. While most vascular channels were thickened and hyalinized, three angiomas had foci of small vessels resembling a capillary angioma. The available data suggest that surgical management of intramedullary angiomas should attempt complete extirpation. The lesions are often well circumscribed with a glial plane between the lesion and normal cord. However, spinal cord angiomas, unlike cranial lesions, have little room for enlargement before devastating symptoms occur. We achieved complete excision in all of our patients, who are all ambulatory and have improved symptoms. Little, if any, increase in neurological deficit was caused by myelotomy and lesion excision. These spinal lesions should be considered for surgery early, before repeated hemorrhage or enlargement can occur.

腫よう内骨化病変を伴なった胸髄部血管腫の１例

腫よう内骨化病変を伴なった胸髄部血管腫の１例

Vascular malformations of the central nervous system: a morphological overview.

Vascular malformations of the central nervous system (C.N.S.) are classified by size, location, and morphologic type, distinguishing capillary telangiectasias, cavernous malformations, venous angiomas, arteriovenous malformations (AVMs) including varix of the great vein of Galen, and other vascular malformations (e.g. Sturge-Weber syndrome). The morphology and predominant location pattern of the different types of vascular malformations in the brain and spinal cord, and their embryology are reviewed. In the brain and its coverings, all types mainly AVMs and venous angiomas do occur, representing 5-9% of all intracranial space-occupying lesions and 20-40% of the sources of surgically treated intracranial hemorrhages. 50-80% of the angiomas are located in the cerebral hemispheres, 10-18% in central brain areas (basal ganglia, internal capsule, choroid plexus), and 10-30% in the posterior fossa. The major types of cerebral vascular malformations are described with reference to their anatomical features, location, chief arterial and venous supply, and prominent complications. Spinal vascular malformations, accounting for 3 to 12% of spinal space-occupying lesions, include vertebral, extradural, dural, subpial and intramedullary angiomas which occur as isolated or complex vascular anomalies and may involve various covering layers at the same level. The preferential occurrence of angiomas on the dorsal surface of the cord and in the caudal regions is related to the embryologic development of spinal vasculature. Frequent association of spinal angiomas (20-25%) with other vascular anomalies and dysplasias emphasizes their hamartomatous nature and developmental origin. Spinal angiomas include capillary telangiectasias with extra- or intradural and, rarely, intramedullary location, cavernomas, mainly arising in vertebral bodies, venous angiomas, mainly located in vertebral bodies and in the extradural space, and AVMs constituting the commonest type, that may affect both the pial and radicular vessels and can penetrate into the cord. They present as simple AV fistulas, cirsoid angiomas with localized vascular plexuses and large complex convolutions ("juvenile" type). The complications of spinal angiomas include subarachnoid hemorrhage, rare epidural hematoma, hematomyelia, compression lesions of the cord and roots, and ischemic changes causing chronic progressive radiculomyelopathy, previously referred to as Foix-Alajouanine syndrome. Chronic damage to the cord and spinal roots results from pressure effects, thrombosis of the abnormal vessels, disorders of venous drainage, and "steal" phenomena related to the vascular anomalies.

SUCCESSFUL TREATMENT OF INTRAMEDULLARY ANGIOMA OF THE CORD.

SUCCESSFUL TREATMENT OF INTRAMEDULLARY ANGIOMA OF THE CORD.

Spontaneous Hæmatomyelia: A Report of Two Cases, One in Association with an Intramedullary Angioma, and the other in Association with Syphilis

Spontaneous Hæmatomyelia: A Report of Two Cases, One in Association with an Intramedullary Angioma, and the other in Association with Syphilis