spinal cavernous malformations

Spinal cord cavernous malformations, also known as spinal cavernomas, are vascular malformations that occur within the spinal cord.

This article specifically relates to spinal cord cavernomas. For a discussion of cerebral cavernomas and a general discussion of the pathology refer to the main article cerebral cavernous malformation.

Epidemiology

Spinal cord cavernomas are rare, representing ~5% of intramedullary lesions in adults and 1% of intramedullary lesions in children .

Peak presentation is during the fourth decade, which is similar to the peak incidence of cerebral cavernomas . Females are more commonly affected than males.

Clinical presentation

Common presenting symptoms include pain, weakness and paresthesias. Four patterns of clinical presentation have been described :

• discrete episodes of neurological deterioration with varying degrees of recovery between episodes
• slow progression of neurological decline
• acute onset of symptoms with rapid decline
• acute onset of mild symptoms with subsequent gradual decline lasting weeks to months

Episodes of hemorrhage have been proposed as the mechanism underlying acute episodes of neurological deterioration. Progressive myelopathy may be caused by micro hemorrhages and gliosis.

Pathology

Like intracranial cavernomas, spinal cavernomas consist of blood-filled endothelial-lined spaces lined by thickened, hyalinised walls that lack elastic fibers and smooth muscle .

Site

More than half of spinal cavernomas are located in the thoracic cord. The second most common location is the cervical cord .

Radiographic features

CT

• often occult

Angiography

• often occult

MRI

Minimal cord expansion or edema unless there has been recent hemorrhage.

• rounded regions of heterogeneous signal intensity on T1 and T2 weighted images due to blood products of varying ages (“popcorn appearance”)
• low signal intensity rim on T2 weighted images (hemosiderin)
• gradient echo (GE): there can be hypointense “blooming” on gradient echo sequences (hemosiderin)
• T1 C+ (Gd): may demonstrate minimal enhancement on post-contrast images

Treatment and prognosis

Early surgical resection should be considered for all symptomatic patients before repeated hemorrhage or enlargement occur .

Siehe auch:

• zerebrale Kavernome

und weiter:

• Kavernom
• spinales Hämangioblastom
• spinal myxopapillary ependymoma
• spinales Ependymom
• spinales Ependymom des Filum terminale
• intracranial cavernous angioma
• cavernoma of cervical cord