- Department of Neurousurgery, Japanese Red Cross Fukui Hospital, Fukui, Japan
- Department of Neurosurgery, Kyoto University Graduate School of Medicine, Kyoto, Japan
- Department of Neurology, Japanese Red Cross Fukui Hospital, Fukui, Japan.
- Department of Pathology, Japanese Red Cross Fukui Hospital, Fukui, Japan.
Correspondence Address:
Noritaka Sano, Department of Neurosurgery, Kyoto University Graduate School of Medicine, Kyoto, Japan.
DOI:10.25259/SNI_531_2022
Copyright: © 2022 Surgical Neurology International This is an open-access article distributed under the terms of the Creative Commons Attribution-Non Commercial-Share Alike 4.0 License, which allows others to remix, transform, and build upon the work non-commercially, as long as the author is credited and the new creations are licensed under the identical terms.How to cite this article: Noritaka Sano1,2, Takeshi Kawauchi1, Narufumi Yanagida3, Sadaharu Torikoshi1, Hiroyuki Ikeda1, Tadakazu Okoshi4, Makoto Hayase1, Masaki Nishimura1, Hiroki Toda1. Diagnosis of spinal dural defect using three-dimensional fast steady-state MR in patient with superficial siderosis: A case report. 08-Jul-2022;13:296
How to cite this URL: Noritaka Sano1,2, Takeshi Kawauchi1, Narufumi Yanagida3, Sadaharu Torikoshi1, Hiroyuki Ikeda1, Tadakazu Okoshi4, Makoto Hayase1, Masaki Nishimura1, Hiroki Toda1. Diagnosis of spinal dural defect using three-dimensional fast steady-state MR in patient with superficial siderosis: A case report. 08-Jul-2022;13:296. Available from: https://surgicalneurologyint.com/surgicalint-articles/11702/
Abstract
Background: Spinal dural defects can result in superficial siderosis (SS) of the central nervous system. Closure of the defect can stop or slow the progression of the disease. Here, we evaluated, whether preoperative three-dimensional fast steady-state acquisition MR could adequately detect these defects and, thus, facilitate their closure and resolution.
Case Description: A 65-year-old right-handed male presented with a 33-year history of the left C8 root avulsion and a 3-year history of slowly progressive gait difficulties and hearing loss. The T2*-weighted imaging revealed symmetrical hemosiderin deposition throughout his central nervous system. A left C6-C7 dural defect involving only inner layer was identified using a three-dimensional MR (3D-FIESTA). It was treated through a left C6-7 hemilaminectomy and successfully sealed with adipose tissue and fibrin glue. Subsequently, the progression of cerebellar ataxia was halted, nevertheless the sensorineural hearing loss worsened even over the next 2 years.
Conclusion: 3D-FIESTA reconstruction was approved to be useful tool for identifying the tiny hole of the inner dural layer responsible for SS.
Keywords: Detection, Dural defect, Magnetic resonance imaging, Superficial siderosis
INTRODUCTION
Superficial siderosis (SS) of the central nervous system is a progressive disease caused by hemosiderin deposition due to repeated hemorrhage into the subarachnoid space.[
CASE DESCRIPTION
A 65-year-old man presented with a history of the left C8 root avulsion 33 years before, 3-year history of slowly progressive gait difficulties and hearing loss, and a 2-week history of a headache. A neurological examination revealed sensorineural deafness, mild truncal ataxia, and findings consistent with the long-standing left C8 root avulsion. The brain CT on arrival demonstrated a left chronic subdural hematoma, and following evacuation, the headache was promptly resolved [
Figure 1:
Key features and imaging findings before the dural defect closure operation. (a) Brain CT: chronic thin left subdural hematoma. (b) Brain T2*-MRI: thick symmetrical deposition of hemosiderin on surface of cerebellum/brainstem. (c) Contrast Brain MRI: marked dural thickening/ enhancement, consistent with CSF hypovolemia. (d) Intraoperative view during aneurysmal clipping: diffuse hemosiderin deposition, and engorged pial vessels throughout the surface of the cerebrum (arrowheads). (e) Hematoxylin-eosin stain of arachnoid: hemosiderin-laden macrophages (arrowheads) is prominent on the surface (original magnification ×200, black bar = 100 µm). (f) Sagittal reverse FIESTA-MRI: longitudinal ventral/dorsal fluid collection interdurally from C6 to Th4. (g and h) Axial reverse FIESTA-MRI: fluid collection (asterisk) adjacent to the dural sac, and dural defect at the left C6/7 level (arrowhead). Cross-sections as indicated by the lines in Figure 1f.
MRI results and laboratory studies
T2*WI after the evacuation surgery revealed hemosiderin deposition throughout both cerebral hemispheres and cerebellar hemispheres as well as spinal cord [
Figure 2:
(a) A 3D-reconstruction of the FIESTA-MRI: preoperatively estimated location of the dural defect left side at C6/7. (b) A dorsal view of 3D-FIESTA-MRI showing the characteristic surface structure. (c) After opening the outer layer of the dura, a blue-colored spinal cord is shown through the opening. (d) Intraoperative photograph showing the identical duplicated dural folds around C6/7 (broken line: incision in outer dura). (e) Another intraoperative photograph showing the inner dural defect. All red arrows/red dots indicate the same dural defect, with black arrowheads in (b) and (d) indicate the same characteristic folds of the outer dural layer.
A lumbar puncture before the craniotomy revealed opening pressure of 7 cmH2O, xanthochromia (4200 red blood cells/µL), and an elevated protein level (146 mg/dL) [
Surgery and postoperative course
After completing a left C6-7 hemilaminectomy, no extradural fluid collection was identified, and a nearly identical dural structure to the preoperative 3D-FIESTA-MRI was confirmed [
Postoperatively, the reverse FIESTA-MRI revealed slight decrease of CSF in the interdural space [
DISCUSSION
Etiology of SS
Here, we reported a case of SS related to duplicated dura matter caused by a defect of the inner dural layer, which is detected utilizing 3D-FIESTA-MRI and minimized the operative invasiveness. Kumar et al. first reported cases of spinal dural defect treated by surgical repair.[
Repair of spinal dural defects to treat SS
Various imaging modalities have been utilized to identify the dural defects responsible for SS. Kumar et al. reported the usefulness of dynamic CT myelography,[
CONCLUSION
The 3D-FIESTA-MRI was an excellent tool for visualizing the dural defect responsible for SS of the central nervous system and was critical for preoperative planning to occlude the fistula with a minimal invasiveness.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent.
Financial support and sponsorship
Nil.
Conflicts of interest
There are no conflicts of interest.
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