Successful endoscopic identification of the bleeding source in the ventral dura of the cervical spine in a case of superficial siderosis

Abstract

The authors report on the case of a 65-year-old man suffering progressive gait disturbance and hearing impairment due to superficial siderosis (SS). According to the literature, repeated hemorrhage into the subarachnoid space causes SS; however, the bleeding source remains unknown in half of SS patients. In the presented case, preoperative MRI revealed a fluid-filled intraspinal cavity extending from C2 to T8 with a dural defect at the ventral C7 level. During surgery, the dural defect was seen to connect to the intraspinal cavity filled with xanthochromic fluid. Importantly, endoscopic observation verified that the rupture of fragile bridging veins in the cavity was the definite bleeding source. Postoperative MRI confirmed disappearance of the intraspinal cavity, and the patient’s symptoms gradually improved. The use of endoscopy helped to establish the diagnosis and led to definite treatment. Fragile bridging veins in the fluid-filled interdural layers were novelly verified as a bleeding source in SS. Recognizing this phenomenon is important since it can establish closure of the dural defect as a definite treatment in SS with an intraspinal cavity.

Superficial Siderosis: A Clinical Review

“…Limited pathologic data in patients with SS and an intraspinal fluid collection suggests that, although the intraspinal fluid collection appears epidural, it may, at least in some cases, be intradural and result from dissection of CSF through a partial ventral dural tear into an intradural plane 27,28 . Similar observations have been made during peri‐operative endoscopy wherein fragile and bleeding bridging veins with trabeculae have been noted to run between the dural layers 29 . Exudation of blood from these engorged, friable, or damaged epidural or intradural vessels has been speculated to be a possible source of chronic bleeding that results in SS 29–31 .…”

“…27,28 Similar observations have been made during peri-operative endoscopy wherein fragile and bleeding bridging veins with trabeculae have been noted to run between the dural layers. 29 Exudation of blood from these engorged, friable, or damaged epidural or intradural vessels has been speculated to be a possible source of chronic bleeding that results in SS. [29][30][31] Reports of subarachnoid hemorrhage in patients with spontaneous intracranial hypotension due to dural tears provide further support for the association between SS and CSF leak due to dural tears.…”

“…29 Exudation of blood from these engorged, friable, or damaged epidural or intradural vessels has been speculated to be a possible source of chronic bleeding that results in SS. [29][30][31] Reports of subarachnoid hemorrhage in patients with spontaneous intracranial hypotension due to dural tears provide further support for the association between SS and CSF leak due to dural tears. 32,33 Red blood cells can be seen in patients with intracranial hypotension due to dural tears and engorgement of the epidural venous plexus has been suspected to be a contributory factor for the CSF in some cases being xanthochromic or even blood tinged.…”

“…The meninges in SS adjacent to the defect in SS may be fibrotic, thickened, and discolored. Spinal endoscopy with CT myelography 69 or during surgical exploration 29 and intra‐operative ultrasonography 70 are innovative approaches that have also been used to aid in detection and repair of small dural defects in SS. Symptoms associated with craniospinal hypovolemia, if present, often resolve, and the clinical progression attributable to SS may be halted, but given the slowly progressive nature of the disorder, long‐term follow‐up is necessary to be confident about this.…”

A superficial siderosis‐related transient focal neurological episode in a patient with Marfan syndrome

“…12 In some observations with perioperative endoscopy, fragile and bleeding bridging veins have been noted to run between the dural layers. 13 Exudation of blood from these damaged and engorged epidural or intradural vessels has been speculated to be a possible source of chronic bleeding that results in SS. 13 We hypothesize that the co-occurrence of dural ectasia in the background of Marfan syndrome in our patient could be the potential cause of chronic microhemorrhages leading to the development of cortical SS.…”