Editor: Robert M.
Friedlander, MD, MA |
Winter 2003 |
What Would You Do?
Case contributed by Arun
Amar, MD, and
Murat Gunel, MD
To contribute a case to the next issue, click here.
The patient is a 40-year-old, right-handed male who presents with a several month history of progressive numbness and weakness in the right arm and leg. The onset of symptoms was gradual. There were no episodes of incontinence. In July 2003 he underwent magnetic resonance imaging of the spine, which revealed a solid, intramedullary lesion at the C3-4 level (Figure 1). The center of the mass was to the right of midline and appeared to occupy about half the dimensions of the normal cord (Figure 2). It demonstrated uniform enhancement with gadolinium. There was mild surrounding edema, as well as a small syrinx extending from the lower brainstem to the upper thoracic cord. However, there was no evidence of associated hemorrhage.
Click image to view larger picture.
| Fig. 1 | Fig. 2 | Fig. 3 |
The patient reported smoking 15 packs of cigarettes per year; otherwise, his medical history and family history were negative for risk factors associated with tumors.
Physical examination was significant for mild atrophy and weakness (4+/5) of the right upper extremity as well as mild weakness (5-/5) of the right lower extremity. His gait was mildly spastic. Hyperreflexia and clonus were present in both legs. Proprioception was nearly absent in the right arm.
The patient was admitted to a community-based hospital and underwent attempted resection via a C3-4 laminectomy. Numerous engorged, tortuous vessels consistent with arterialized veins were draped over the dorsal surface of the cord. A biopsy was taken, and the diagnosis of hemangioblastoma was made. However, due to the excessive vascularity, no excision was performed. Postoperatively, the patient remained at his neurological baseline. He subsequently underwent a limited catheter angiogram, which demonstrated the presence of a hypervascular mass in the cervical cord. Arteriovenous shunting was present, but there was no definite nidus of a vascular malformation. The feeding arteries appeared to arise from branches of the right and left vertebral arteries. However, their association with the anterior and posterior spinal arteries was not clear (Figure 3).
The patient was then referred to an academic medical center for further management. At the time, the possibility of Von Hippel-Lindau disease was considered. Several therapeutic options were contemplated, including repeat surgery, repeat angiography with possible embolization, and stereotactic radiosurgery.
What would you do?
Please take a few moments to submit your response to this edition of What Would You Do? This case closes on Feb. 15.
Copyright© 1998-2004; American Association of Neurological Surgeons /
Congress of Neurological Surgeons