Intralesional hemorrhage and thrombosis without rupture in a pure spinal epidural cavernous angioma: a rare cause of acute lumbal radiculopathy

Abstract

Pure spinal epidural cavernous angiomas are extremely rare lesions, and their normal shape is that of a fusiform mass in the dorsal aspects of the spinal canal. We report a case of a lumbo-sacral epidural cavernous vascular malformation presenting with acute onset of right-sided S1 radiculopathy. Clinical aspects, imaging, intraoperative findings, and histology are demonstrated. The patient, a 27-year-old man presented with acute onset of pain, paraesthesia, and numbness within the right leg corresponding to the S1 segment. An acute lumbosacral disc herniation was suspected, but MRI revealed a cystic lesion with the shape of a balloon, a fluid level and a thickened contrast-enhancing wall. Intraoperatively, a purple-blue tumor with fibrous adhesions was located between the right S1 and S2 nerve roots. Macroscopically, no signs of epidural bleedings could be denoted. After coagulation of a reticular venous feeder network and dissection of the adhesions the rubber ball-like lesion was resected in total. Histology revealed a prominent venous vessel with a pathologically thickened, amuscular wall surrounded by smaller, hyalinized, venous vessels arranged in a back-to-back position typical for the diagnosis of a cavernous angioma. Lumina were partially occluded by thrombi. The surrounding fibrotic tissue showed signs of recurrent bleedings. There was no obvious mass hemorrhage into the surrounding tissue. In this unique case, the pathologic mechanism was not the usual rupture of the cavernous angioma with subsequent intraspinal hemorrhage, but acute mass effect by intralesional bleedings and thrombosis with subsequent increase of volume leading to nerve root compression. Thus, even without a sudden intraspinal hemorrhage a spinal cavernous malformation can cause acute symptoms identical to the clinical features of a soft disc herniation.

Fig. 1

Pure Cavernous vascular malformation, MR-imaging features: sagittal and axial T2 (a1, a2), sagittal T1 (b) and Gd enhanced sagittal and axial T1w MRI (c1, c2) demonstrates a cystic lesion with a fluid level (arrowa1, b) located posterior to the upper sacrum. The content predominantly consists of a fluid with deposition of the corpuscular blood particles at the bottom of the lesion. The lesion occupies the right half of the spinal canal and leads to compression of the right S1 nerve root (arrowa2, c2) laterally at the recess. Note the lesion’s circumscribed, relatively thick external wall that shows a ring enhancement (arrowc1)

Fig. 2

Cavernous vascular malformation, histopathological features: prominent venous vessel with an excessively thickened, amuscular wall (a, H&E). Hyalinized vessels (b, H&E) are arranged in a back-to-back fashion (c, Elastica van Gieson stain) as typical for cavernous angioma. Signs of recurrent hemorrhage with fresh bleedings (b) and residues of older bleedings in form of hemosiderin deposition (d, Berlin blue stain). Magnification of images a and c, ×100; magnification of images b and d, ×200; V vessel lumina