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Show ORIGINAL CONTRIBUTION Capillary Hemangioma of the Cauda Equina Presenting with Radiculopathy and Papilledema Nicola G Ghazi, MD, John A. Jane, MD, PhD, M. Beatriz S. Lopes, MD, and Steven A. Newman, MD Abstract: A 42- year- old, non- obese man with a three- month history of headache, pulsatile tinnitus, transient visual obscurations, and scintillations later developed low back pain with right lower extremity radiation. Brain MRI and magnetic resonance venography were normal, but spine MRI revealed a mass in the cauda equina. Neuro- ophthalmologic examination disclosed bilateral optic disc edema with normal visual function. During spine surgery, cerebrospinal fluid, released under high pressure despite prior hyperventilation, contained a glucose level of 51 mg/ dl and a protein level of 1840 mg/ dl. Histologic and immunohistochemical features of the lesion were compatible with a capillary hemangioma. Although spinal cord tumors have been associated with papilledema, this is the first report of a capillary hemangioma of the cauda equina in this context. If papilledema is present, spinal cord imaging should be performed when lumbar puncture discloses unexplained protein elevation and in cases that lack clinical features typical of idiopathic intracranial hypertension. (/ Neuro- Ophthalmol 2006; 26: 98- 102) ncreased intracranial pressure ( ICP) and papilledema have long been associated with various types of spinal cord tumors, especially ependymomas, which are responsible for more than 50% of cases ( 1- 4). Elevated cerebrospinal fluid ( CSF) protein has been proposed as the main cause of increased ICP and papilledema ( 1). A second possible cause of elevated ICP is repeated episodes of subclinical subarachnoid bleeding from spinal cord tumors. A third possible cause is disruption of the spinal sac, particularly its lumbar portion, which is believed to represent Departments of Ophthalmology ( NGG, SAN), Neurosurgery ( JAJ), and Pathology ( MBSL), University of Virginia Health System, Charlottesville, Virginia. Address correspondence to Steven A. Newman, MD, University of Virginia Health System, Department of Ophthalmology, PO Box 800715, Charlottesville, VA 22908; E- mail: raj2f@ virginia. edu a CSF " elastic reservoir" important in maintaining a constant intracranial volume ( 5,6). To our knowledge, increased ICP has not been previously documented in capillary hemangioma of the spinal cord or cauda equina. We describe the clinical, radiologic, and histologic features of a case of capillary hemangioma of the cauda equina that presented with radiculopathy and papilledema. This case underscores the importance of spinal imaging in the workup of patients with unexplained papilledema. CASE REPORT A 42- year- old man had a five- month history of hearing a pulse- synchronous " rushing noise" in both ears and having occasional occipital headaches that sometimes would awaken him at night. He also described transient visual obscurations that lasted " for a couple of minutes at the most," in addition to seeing central orange- and red-colored dots unaccompanied by increased headache, nausea, vomiting, photophobia, or sonophobia. Magnetic resonance imaging and magnetic resonance venography of the brain obtained by the patient's internist were unremarkable. Two months before presentation, he started to have lower back pain that radiated to the right leg without weakness. Neurologic evaluation was intact, and he was diagnosed as having sciatica. The leg pain did not respond to muscle relaxants and prednisone therapy, so an MRI scan of the lumbar spine was performed, which disclosed an L3- L4 lesion of the cauda equina presumed to be a schwannoma ( Fig. 1). The neurosurgeon noted papilledema and referred the patient for neuro- ophthalmologic evaluation. Neuro- ophthalmologic examination disclosed a man whose height was 183 cm and weight was 96 kg. Best-corrected visual acuity was 20/ 20 in both eyes. External evaluation, pupillary reflexes, slit- lamp examination, Goldmann applanation tonometry, color vision testing, stereopsis, and motility assessment of both eyes were unremarkable except for a comitant exophoria noted on Maddox rod testing. Fundus examination disclosed bilateral optic disc elevation with hyperemia and splinter hemorrhages ( Fig. 2). Visual 98 J Neuro- Ophthalmol, Vol. 26, No. 2, 2006 Capillary Hemangioma J Neuro- Ophthalmol, Vol. 26, No. 2, 2006 FIG. 1. Magnetic resonance imaging of the lumbosacral spine. A. Sagittal T2 shows a low- intensity lesion in the cauda equina. B. Post-contrast sagittal T1 shows marked hyperintensity. C. Pre- contrast axial T1 shows an ill- defined lesion. D. Post- contrast axial T1 shows intense homogeneous enhancement. field testing was unremarkable except for mild enlargement of the blind spot in the field of the left eye ( Fig. 3). After L3- L4 laminectomy and dural incision, and despite prior hyperventilation, CSF was released under high pressure. No formal opening pressure was obtained. CSF analysis disclosed a glucose level of 51 mg/ dl and a protein level of 1840 mg/ dl. A cell count was not obtained. A reddish vascular mass was noted at the L3- L4 location underneath the nerve roots. The tumor was bluntly dissected and separated from the surrounding nerve roots except at the L5 root, which was entangled with the tumor and a radicular vessel. The tumor, nerve root, and radicular vessel were excised. After tumor resection, the patient's visual symptoms, headache, tinnitus, and leg symptoms resolved completely. Optic disc swelling ( Fig. 4) and the visual field defects had resolved within eight weeks of tumor removal. Residual numbness in the SI distribution and mild weakness on plantar flexion were present after surgery, attributable to the fact that the nerve root had to be sacrificed during surgery. The surgical specimen consisted of a 2.0 X 1.5 cm, well- circumscribed, red- brown tissue with a 2.5 cm segment of nerve attached. Light microscopic examination disclosed a vascular tumor arranged into large lobules separated by fibrous septae and composed of endothelial cells with no atypical features ( Fig. 5). In some areas, the 99 J Neuro- Ophthalmol, Vol. 26, No. 2, 2006 Ghazi et al FIG. 2. Fundus photographs taken before tumor removal show bilateral acquired optic disc edema. 4 » • :;:•' IIHtr. intir, uuimii • niitv.-. Ill IV. u t tv i: ii! H! si l-. l. :.:::::' :::::::.•• fKJS.- FIG. 3. Humphrey visual fields performed before tumor removal show mild enlargement of the blind spot in the left eye. • 1 • • II • • II • « II tt • • * • t * • « ! • • • • , . • • • - . • • * ' FIG. 4. Fundus photographs taken eight weeks after tumor removal show resolution of optic disc edema. 100 © 2006 Lippincott Williams & Wilkins Capillary Hemangioma J Neuro- Ophthalmol, Vol. 26, No. 2, 2006 tumor contained small capillary channels lined by a single layer of flattened endothelial cells; in other areas, the tumor was largely solid and composed of plump endothelial cells with rare vascular channels. A large artery, probably the feeder radicular vessel, and peripheral nerve tissue traversing the tumor were also identified. No foamy cells, mitotic figures, or hemosiderin were identified. Wilder reticulin stain outlined basement membranes surrounding vascular structures. Immunohis-tochemical staining for neuron- specific enolase and chromogranin was negative. However, the tumor cells stained strongly positively for endothelial markers CD31 and CD34. These findings are compatible with the diagnosis of capillary hemangioma. DISCUSSION To our knowledge, the presentation of a capillary hemangioma of the cauda equina with ophthalmologic symptoms in the setting of increased ICP and papilledema has not been previously reported. Such a presentation has, however, long been recognized in association with other spinal cord lesions ( 1- 4). This benign vascular hamartoma rarely involves the spinal cord, but when it does, it most commonly affects the lower thoracic and lumbar cord, conus meduUaris, spinal nerve roots, and cauda equina ( 7). It usually presents with sensorimotor deficits of the lower extremities ( 7- 9) that frequently resolve with surgical excision of the tumor. In two major reviews, by Abe et al ( 7) and Andaluz et al ( 8), none of the patients presented with increased ICP or papilledema. An important finding in our case was the elevated CSF protein level, which has been suggested to cause increased ICP either by deposition of macromolecules and protein degradation products in the arachnoid villi or by causing aseptic fibroplastic arachnoiditis secondary to the irritative effect of CSF hyperviscosity ( 1). The cause of elevated CSF proteins in spinal cord tumors remains speculative, although multiple factors have been suggested, such as active secretion by the tumor, transudation, tumor breakdown, and meningeal reaction from the tumor ( 10). Although elevated CSF protein has been proposed as the main cause of intracranial hypertension and papilledema associated with spinal cord lesions, two other mechanisms have been recognized. Matzkin et al ( 5) implicated repeated episodes of subclinical subarachnoid bleeding from the tumor causing interference with the passage of CSF through the arachnoid villi. Repeated bleeding is usually associated with the finding of red blood cells in xanthochromic CSF and/ or hemosiderin deposition in the excised tumor. Alternatively, Martins et al ( 6) proposed that the spinal sac serves as the " elastic reservoir" for CSF that is important in maintaining a constant intracranial volume. Because of its greater distensibility and volume, the lumbar portion of the spinal sac is the most critical in this " decompressive mechanism." A tumor in this region might interfere with CSF flow in the spinal canal leading to increased intracranial volume and pressure. More recently, Costello et al ( 11) implicated such a mechanism in a case of sacral schwannoma that caused papilledema even in the absence of markedly elevated CSF proteins. The absence of CSF xanthochromia and hemosiderin deposition in our case, in addition to the markedly elevated CSF protein, makes defective CSF absorption caused by elevated protein a reasonable mechanism for increased ICP and papilledema, although compromise of the CSF " elastic reservoir" by the lumbar tumor cannot be excluded. Previously reported spinal cord tumors that have been associated with intracranial hypertension and papilledema include ependymoma, schwannoma, neurofibroma, and glial tumors ( 1- 4,12,13). The vast majority of the cases are FIG. 5. Tumor histopathology. A. Variably sized capillary- like vessels are lined by a single layer of benign endothelial cells that are flattened in some vessels and plump in others. Asterisk indicates a large artery, probably representing the feeder radicular vessel. There are no foamy cells ( hematoxylin and eosin stain, X100). B. Perivascular rather than pericellular reticulin fiber deposition ( Wilder reticulin stain, X100). C. Stain for endothelial marker CD31 discloses strong positivity of tumor cells, compatible with capillary hemangioma ( X200). 101 J Neuro- Ophthalmol, Vol. 26, No. 2, 2006 Ghazi et al associated with varying degrees of elevated CSF protein level, which, together with the presenting symptoms, usually resolves after surgical excision of the lesion ( 10,13). Although most of these tumors are located in the thoracolumbar or lumbosacral spine, cervical lesions have also been reported ( 10,12). Mechanisms of increased ICP involved in cervical lesions may be similar to those described for lower spinal tumors, but mechanical obstruction of the CSF pathway may be an important factor, especially with extension of the lesion through the foramen magnum. The radiologic, histologic, and immunohistochem-ical findings in our case confirm the diagnosis of capillary hemangioma. MRI findings are helpful in the diagnosis of these lesions ( 14), which usually display isointensity or slight hyperintensity on Tl, hyperintensity on T2- weighted images, with strong homogeneous enhancement after gadolinium administration ( 7- 9,14- 16). The histologic and immunohistochemical characteristics described are also typical of a capillary hemangioma and help differentiate it from other vascular tumors that may affect the cauda equina such as cavernous hemangiomas, capillary telangiectasias, hemangioendotheliomas, and arteriovenous malformations. In addition, tumors with vascular features but poorly understood histogenesis, including capillary hemangioblastomas and hemangiopericytomas, were also ruled out by the absence of characteristic histologic features. 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