Rapid arteriovenous shunting in a spinal cord ependymoma Case report
ERIC T. YUHL, M.D., AND JOHN R. BENTSON, M.D.
Divisions of Neurosurgery and Neuroradiology, University of California of Los Angeles School of Medicine, Los Angeles, California v' A case of ependymoma of the conus medullaris and cauda equina is described in which spinal angiography demonstrated rapid arteriovenous shunting, an angiographic sign which is typical of arteriovenous malformations and which has not been previously reported to occur with ependymomas. KEY WORDS ependymoma spinal cord tumor ~
9 arteriovenous shunting
9
NGIOGRAPHY of the spinal cord has been found to be particularly useful in the diagnosis of arteriovenous malformations (AVM's) of the spinal cord. It is also helpful in the study of certain cord tumors, particularly hemangioblastomas. The importance of distinguishing AVM's from cord tumors is apparent, since the surgical approach may differ and since certain AVM's are amenable to embolization or other techniques. 1'5's We are reporting a case in which the angiographic findings of an ependymoma mimicked those of an AVM. Case Report
This 12-year-old girl was first examined in September, 1972. She complained of back pain which had been present since a minor injury suffered 4 months prior to examination. Three months after the injury, she noted increasing attacks of right paravertebral pain which frequently radiated down the posterior aspect of the right thigh and calf and into the heel. Back pain was increased by lying down
744
FIG. 1. Spine film with Pantopaque injected into the cervical canal revealing a block of intramedullary type at T-12. A tortuous defect is visible above the block. There is mild scoliosis but no canal widening.
J. Neurosurg. / Volume 44 / June, 1976
AV shuntin~ in spinal vpeudym~m~a
Fro. 2. Spine film (left) and subtraction (right). A branch of the right first lumbar artery, probably the artery of Adamkiewicz, fills tortuous ascending and descending veins. and severely exaggerated by coughing, sneezing, or straining. There was no numbness, tingling, or sphincter disturbance. One week prior to consultation, fleeting episodes of radicular left leg pain had developed. Examination showed complete loss of the lumbar lordotic curve. Flexion and extension of the lumbar spine caused severe pain in the back and right buttock. The right Achilles tendon reflex was absent and the left, diminished. Both knee jerks were diminished. There was weakness of plantar flexion of the right foot. No atrophy or fasciculations were observed. Sphincter tone was normal. All sensory modalities were intact. Straight leg raising at 30 ~ on the right produced severe, radicular right leg pain increased by Las~gue's maneuver. Routine laboratory data including sedimentation rate were normal. Chest x-rays were normal. Spine films revealed a mild J. Neurosurg. / Volume 44 / June, 1976
scoliosis but no expansion of the spinal canal. Lumbar puncture was attempted at several levels, using fluoroscopic control, but these were "dry taps." A lateral C1-2 approach revealed clear spinal fluid. Approximately 4 cc of Pantopaque were instilled into the subarachnoid space. With the patient erect, a complete block was observed at T-12, apparently due to widening of the cord (Fig. 1). A tortuous defect extending upward from the block indicated the presence of abnormally dilated vessels. Selective spinal angiography was then performed (Figs. 2 and 3). On injection of the right first lumbar artery, a slightly enlarged branch was seen to enter the spinal canal, ascend to the lower border of T-11, then immediately fill dilated tortuous veins which ran both cepbalad and caudad. No diffuse tumor stain was visible. No other abnormal vessels were encountered on injecting other lumbar and thoracic arteries. Because 745
E. T. Yuhl and J. R. Bentson and will be briefly summarized here? ,4,6 The arterial supply may be single or multiple. There is generally immediate filling of large tortuous veins, the flow rate of which may vary depending upon the size of the shunt and number of feeding vessels. There is no diffuse stain at the point of communication which may occur within or outside the cord. There is usually no complete block to the flow of Pantopaque or similar positive contrast material, although blockage due to enlarged vessels in the spinal canal or within the cord may occasionally be present? The angiographic appearance of spinal cord hemangioblastoma has also been well described2 ,*'6 The typical appearance is an area of dense staining, within which no vessels are clearly discernible. This blush lingers for several seconds and the large draining veins fill late. Other spinal cord tumors have not been found to be so angiographically characteristic. Pathological vessels were found in one cervical cord astrocytoma by Goldenberg. 7 Djindjian, et al., ~ studied two dorsolumbar ependymomas, noting displacement of enlarged posterolateral spinal arteries indicating cord enlargement. Enlarged veins were also observed but no arteriovenous shunting or pathological vessels were described. DiChiro and Wener3 have recently FIG. 3. Subtraction print of a nearly lateral projection. Note anterior position of the arterio- described angiographic findings in four cases venous communication and lack of tumor stain. of spinal cord ependymomas. Although they The anterior (open arrow) and posterior (black did not find a pathognomonic appearance, arrow) borders of the spinal cord as seen on the un- they did find enlargement and displacement subtracted film are shown. of arteries, large tortuous veins, and enlarged anastomotic loop(s) of the conus in some of these cases. One case had pathological vessels postoperatively. No rapid arteriovenous of this rapid arteriovenous shunting, AVM of shunting was found. the conus was suspected, although it was Our case illustrates some of the difficulties recognized that the apparent cord widening in the differential diagnosis of spinal cord was atypical. Laminectomy revealed an exlesions. Although the myelographic appeartensive tumor, firmly adherent to the conus ance of the block in this case suggested cord medullaris. The tumor adhered to all of the tumor, the defects caused by large draining roots of the cauda equina and extended veins raised the possibility of AVM or caudally to the attachment of the ilium terhemangioblastoma. The angiographic findminale at S-2. The subarachnoid space was ings of rapid arteriovenous shunting and completely obliterated by the neoplasm. absence of tumor staining appeared to rule Multiple biopsies revealed a low-grade epenout hemangioblastoma and were typical of dymoma. AVM. This experience is in keeping with the earlier observations that the angiographic Discussion appearances of spinal cord tumors are The angiographic findings of AVM's of the variable and not pathognomonic. It points spinal cord have been extensively described out the necessity of obtaining histological 746
J. Neurosurg. / Volume 44 / June, 1976
AV s h u n t i n g in spinal epet~dyn/{~ma proof of the nature of any lesion, when not all radiographic and clinical signs are typical of an AVM, before a course of therapy is instituted. References I. Bentson JR, Crandall PH: Use of the Fogarty catheter in arteriovenous malformations of the spinal cord. Radiology 105:65-68, 1972 2. DiChiro G, Doppman JL: Differential angiographic features of hemangioblastomas and arteriovenous malformations of the spinal cord. Radiology 93:25-30, 1969 3. DiChiro G, Wener L: Angiography of ependymomas of the spinal cord and filum terminale. Am J Roentgenoi Radium Ther Nucl Med (In press) 4. Djindjian R, Hurth M, Houdart R, et al: Angiography of the Spinal Cord. Baltimore: University Park Press, 1970
J. Neurosurg. / Volume 44 / June, 1976
5. Doppman JL, DiChiro G, Ommaya AK: Obliteration of spinal-cord arteriovenous malformation by percutaneous embolisation. Letter to the editor. Lancet 1:477, 1968 6. Doppman JL, DiChiro G, Ommaya AK: Selective Arteriography of the Spinal Cord. St Louis: WH Green, 1969 7. Goldenberg M: Glioma of spinal cord demonstrated by angiography. J Can Assoc Radiol 21:113-115, 1970 8. Newton TH, Adams JE: Angiographic demonstration and nonsurgical embolization of spinal cord angioma. Radiology 91:873-876, 1968
Address reprint requests to." John R. Bentson, M.D., Neuroradiology Section, UCLA Center for the Health Sciences, Los Angeles, California 90024.
747
ERIC T. YUHL, M.D., AND JOHN R. BENTSON, M.D.
Divisions of Neurosurgery and Neuroradiology, University of California of Los Angeles School of Medicine, Los Angeles, California v' A case of ependymoma of the conus medullaris and cauda equina is described in which spinal angiography demonstrated rapid arteriovenous shunting, an angiographic sign which is typical of arteriovenous malformations and which has not been previously reported to occur with ependymomas. KEY WORDS ependymoma spinal cord tumor ~
9 arteriovenous shunting
9
NGIOGRAPHY of the spinal cord has been found to be particularly useful in the diagnosis of arteriovenous malformations (AVM's) of the spinal cord. It is also helpful in the study of certain cord tumors, particularly hemangioblastomas. The importance of distinguishing AVM's from cord tumors is apparent, since the surgical approach may differ and since certain AVM's are amenable to embolization or other techniques. 1'5's We are reporting a case in which the angiographic findings of an ependymoma mimicked those of an AVM. Case Report
This 12-year-old girl was first examined in September, 1972. She complained of back pain which had been present since a minor injury suffered 4 months prior to examination. Three months after the injury, she noted increasing attacks of right paravertebral pain which frequently radiated down the posterior aspect of the right thigh and calf and into the heel. Back pain was increased by lying down
744
FIG. 1. Spine film with Pantopaque injected into the cervical canal revealing a block of intramedullary type at T-12. A tortuous defect is visible above the block. There is mild scoliosis but no canal widening.
J. Neurosurg. / Volume 44 / June, 1976
AV shuntin~ in spinal vpeudym~m~a
Fro. 2. Spine film (left) and subtraction (right). A branch of the right first lumbar artery, probably the artery of Adamkiewicz, fills tortuous ascending and descending veins. and severely exaggerated by coughing, sneezing, or straining. There was no numbness, tingling, or sphincter disturbance. One week prior to consultation, fleeting episodes of radicular left leg pain had developed. Examination showed complete loss of the lumbar lordotic curve. Flexion and extension of the lumbar spine caused severe pain in the back and right buttock. The right Achilles tendon reflex was absent and the left, diminished. Both knee jerks were diminished. There was weakness of plantar flexion of the right foot. No atrophy or fasciculations were observed. Sphincter tone was normal. All sensory modalities were intact. Straight leg raising at 30 ~ on the right produced severe, radicular right leg pain increased by Las~gue's maneuver. Routine laboratory data including sedimentation rate were normal. Chest x-rays were normal. Spine films revealed a mild J. Neurosurg. / Volume 44 / June, 1976
scoliosis but no expansion of the spinal canal. Lumbar puncture was attempted at several levels, using fluoroscopic control, but these were "dry taps." A lateral C1-2 approach revealed clear spinal fluid. Approximately 4 cc of Pantopaque were instilled into the subarachnoid space. With the patient erect, a complete block was observed at T-12, apparently due to widening of the cord (Fig. 1). A tortuous defect extending upward from the block indicated the presence of abnormally dilated vessels. Selective spinal angiography was then performed (Figs. 2 and 3). On injection of the right first lumbar artery, a slightly enlarged branch was seen to enter the spinal canal, ascend to the lower border of T-11, then immediately fill dilated tortuous veins which ran both cepbalad and caudad. No diffuse tumor stain was visible. No other abnormal vessels were encountered on injecting other lumbar and thoracic arteries. Because 745
E. T. Yuhl and J. R. Bentson and will be briefly summarized here? ,4,6 The arterial supply may be single or multiple. There is generally immediate filling of large tortuous veins, the flow rate of which may vary depending upon the size of the shunt and number of feeding vessels. There is no diffuse stain at the point of communication which may occur within or outside the cord. There is usually no complete block to the flow of Pantopaque or similar positive contrast material, although blockage due to enlarged vessels in the spinal canal or within the cord may occasionally be present? The angiographic appearance of spinal cord hemangioblastoma has also been well described2 ,*'6 The typical appearance is an area of dense staining, within which no vessels are clearly discernible. This blush lingers for several seconds and the large draining veins fill late. Other spinal cord tumors have not been found to be so angiographically characteristic. Pathological vessels were found in one cervical cord astrocytoma by Goldenberg. 7 Djindjian, et al., ~ studied two dorsolumbar ependymomas, noting displacement of enlarged posterolateral spinal arteries indicating cord enlargement. Enlarged veins were also observed but no arteriovenous shunting or pathological vessels were described. DiChiro and Wener3 have recently FIG. 3. Subtraction print of a nearly lateral projection. Note anterior position of the arterio- described angiographic findings in four cases venous communication and lack of tumor stain. of spinal cord ependymomas. Although they The anterior (open arrow) and posterior (black did not find a pathognomonic appearance, arrow) borders of the spinal cord as seen on the un- they did find enlargement and displacement subtracted film are shown. of arteries, large tortuous veins, and enlarged anastomotic loop(s) of the conus in some of these cases. One case had pathological vessels postoperatively. No rapid arteriovenous of this rapid arteriovenous shunting, AVM of shunting was found. the conus was suspected, although it was Our case illustrates some of the difficulties recognized that the apparent cord widening in the differential diagnosis of spinal cord was atypical. Laminectomy revealed an exlesions. Although the myelographic appeartensive tumor, firmly adherent to the conus ance of the block in this case suggested cord medullaris. The tumor adhered to all of the tumor, the defects caused by large draining roots of the cauda equina and extended veins raised the possibility of AVM or caudally to the attachment of the ilium terhemangioblastoma. The angiographic findminale at S-2. The subarachnoid space was ings of rapid arteriovenous shunting and completely obliterated by the neoplasm. absence of tumor staining appeared to rule Multiple biopsies revealed a low-grade epenout hemangioblastoma and were typical of dymoma. AVM. This experience is in keeping with the earlier observations that the angiographic Discussion appearances of spinal cord tumors are The angiographic findings of AVM's of the variable and not pathognomonic. It points spinal cord have been extensively described out the necessity of obtaining histological 746
J. Neurosurg. / Volume 44 / June, 1976
AV s h u n t i n g in spinal epet~dyn/{~ma proof of the nature of any lesion, when not all radiographic and clinical signs are typical of an AVM, before a course of therapy is instituted. References I. Bentson JR, Crandall PH: Use of the Fogarty catheter in arteriovenous malformations of the spinal cord. Radiology 105:65-68, 1972 2. DiChiro G, Doppman JL: Differential angiographic features of hemangioblastomas and arteriovenous malformations of the spinal cord. Radiology 93:25-30, 1969 3. DiChiro G, Wener L: Angiography of ependymomas of the spinal cord and filum terminale. Am J Roentgenoi Radium Ther Nucl Med (In press) 4. Djindjian R, Hurth M, Houdart R, et al: Angiography of the Spinal Cord. Baltimore: University Park Press, 1970
J. Neurosurg. / Volume 44 / June, 1976
5. Doppman JL, DiChiro G, Ommaya AK: Obliteration of spinal-cord arteriovenous malformation by percutaneous embolisation. Letter to the editor. Lancet 1:477, 1968 6. Doppman JL, DiChiro G, Ommaya AK: Selective Arteriography of the Spinal Cord. St Louis: WH Green, 1969 7. Goldenberg M: Glioma of spinal cord demonstrated by angiography. J Can Assoc Radiol 21:113-115, 1970 8. Newton TH, Adams JE: Angiographic demonstration and nonsurgical embolization of spinal cord angioma. Radiology 91:873-876, 1968
Address reprint requests to." John R. Bentson, M.D., Neuroradiology Section, UCLA Center for the Health Sciences, Los Angeles, California 90024.
747
