J Neurosurg 77:148-150, 1992
Isolated intramedullary spinal artery aneurysm presenting with quadriplegia Case report TAKASHI HANDA, M.D., YOSHIO SUZUKI, M.D., KIYOSHI SAITO, M.D., KENICmRO SUGn'A, M.D., ANO SUNXL J. PATEL, M.D.
Department of Neurosurgery, Nagoya UniversitySchool of Medicine, Nagoya, Japan L,, The case is presented of an isolated intramedullary posterior spinal artery aneurysm at the C-2 level in a 3-year-old girl. Quadriplegia related to probable intramedullary hemorrhage was the presenting symptom. Magnetic resonance imaging revealed findings consistent with an intramedullary vascular lesion, and vertebral angiography confirmed this to be an aneurysm of the posterior spinal artery. No associated vascular abnormalities were noted, and the aneurysm was successfully resected. Previous reports of isolated spinal aneurysms are reviewed. KEY WORDS
"
aneurysm
9 posterior spinal artery
PINAL artery aneurysms are rare and are usually associated with arteriovenous malformations (AVM s). 2,6,~2 Symptoms of subarachnoid hemorrhage (SAH) and/or spinal cord involvement guide the physician to the correct diagnosis, although this is often difficult. Isolated spinal artery aneurysms are infrequently reported, ~-5'9-'L~3.~4.~7-t9and little is known about their clinicopathology. The unusual presentation of such a case prompted this contribution.
S
9 spinal cord compression
a presumed diagnosis of transverse myelitis, steroids and glycerol were administered. Over a period of 2 months, she gradually improved. She was extubated and was able to walk. Examination at the time revealed a residual quadriparesis with a distinct cervical sensory level. Magnetic resonance images and CT scans of the cervical spine showed an intramedullary mass at the C-2 level. A dark ring noted around the hyperintense center of the mass was thought to be hemosiderin encompassing a vascular lesion (Fig. 1).
Case Report This 3-year-old girl initially developed quadriparesis 8 months prior to transfer to our institution. Her symptoms began with a transient episode (lasting 24 hours) of difficulty with walking, followed by complaints of headache and abdominal pain. A few days later, she developed weakness in her right arm which progressed to a hemiparesis. There was no history of trauma. On admission to the referring hospital, the patient was lethargic and profoundly quadriparetic (the fight paresis more marked than the left) with a facial palsy. She had hyporeflexia and difficulty in voiding. Sensory examination was not easy due to her age and mental status. A computerized tomography (CT) scan of the head showed no abnormalities. Lumbar puncture revealed a normal opening pressure and clear, colorless cerebrospinal fluid with a normal protein content. The patient's condition deteriorated further to complete quadriplegia, requiting artificial ventilation. With 148
FIG. 1. Left:Computerized tomography scan of the cervical spine showing a round homogeneously enhancing mass (aneurysm) with an apparent neck. Right: Magnetic resonance T2-weighted image, sagittal projection, demonstrating a well-demarcated intramedullary lesion with surrounding low signal intensity, probably indicating aneurysmal wall with hemosidefin.
J. Neurosurg. / Volume 77 ~July, 1992
I s o l a t e d i n t r a m e d u l l a r y spinal a r t e r y a n e u r y s m neck was identified and cauterized, and the lesion was excised. Hemosiderin deposition was noted around the aneurysm. Further inspection revealed that the neck of the aneurysm arose from a large aberrant arterial loop branching off the posterior spinal artery. No other vascular abnormalities were seen. Postoperative Course. The patient's postoperative course was uneventful. Pathological studies revealed the wall of the aneurysm to be mostly collagenous with very little internal elastic lamina. Discussion
FtG. 2. Left vertebral angiograms, anteroposterior (left) and lateral (righO views, revealing an aneurysm fed by a large tortuous posterior spinal artery.
Examination. At this point, the patient was transferred to our institution. Vertebral angiography confirmed the intramedullary mass to be an aneurysm fed by a large tortuous posterior spinal artery (Fig. 2). Operation. Aneurysm resection was performed via the posterior approach. When the dura was opened, a large serpiginous posterior spinal artery was seen on the left dorsolateral aspect of the spinal cord. A slightly bluish tint of the cord medial to this vessel indicated the location of the aneurysm. After temporary occlusion of the feeding artery, which was identified using microvascular Doppler ultrasonography, the aneurysm was exposed via a midline myelotomy. The aneurysm
Spinal artery aneurysms are rare and are usually reported in association with vascular malformations. 2,6-8, ~z,~5The reported incidence of spinal artery aneurysms associated with spinal AVM's is 7.7% to 8.0%, which is similar to that of intracranial aneurysms with AVM's. Patients with either of these lesions seem to have a higher incidence of SAH, 6'~z the source of which remains questionable. In a few cases, successful obliteration or excision of the AVM has resulted in spontaneous regression of the associated aneurysm) TM A review of the literature has disclosed only I0 previous cases of isolated spinal artery aneurysms (Table 1). 3-5'9-11'13"14'17-19A few cases were excluded due to either confusion in terminology or uncertainty over the existence of an associated vascular malformation. Spinal artery aneurysms have also been described in patients with aortic coarctation and syphilis) Henson and Croft 5 were the first to report an isolated aneurysm, seen at autopsy in a patient with SAH. The first report of an isolated intramedullary aneurysm and also the first successful excision of the aneurysm was by Kinal and Sejanovich. 9 Of the 11 reported cases (including ours), seven presented with symptoms typical of spinal SAH. The other four had cord compression syndromes, two with intramedullary aneurysms and hematomyelia
TABLE 1
Published cases of isolated spinal artery aneurysms* Case No.
Authors & Year
Age (yrs), Sex
1 2
5l, M 41, F
3 4 5 6
Henson & Croft, 1956 Kinal & Sejanovich, 1957 Leech, et al., 1976 Garcia, et al., 1979 Thomson, 1980 Vincent, 1981
7 8
Kito, et al., 1983 Smith, et al., 1986
37, F 29, M
9
10
Saunders, et aL, 1987 Goto, el al., 1988
44, F 53, M
SAH SAH
T-I C-2
11
Handa, et aL, 1992
3, F
quadriparesis
C-2
25, 34, 66, 30,
F F F F
Clinical Symptom
Site
Vascular Supply
PSA unidentified intramedullary artery ASA paraparesis T-7 SAH T-6 artery of Adamkiewicz ASA quadriparesis C1-2 SAH C-I ASA (anomalous branches) ASA SAH T-10 SAH T-12 ASA (serpiginous) SAH paraparesis
C-I C-7
Relation to Cord
Operation
Outcome
extramedullary no died intramedullary aneurysm resection improved extramedullary aneurysmresection improved extramedullary no died extramedullary aneurysm dipping improved extramedullary aneurysm dipping improved extramedullary no extramedullary clipping of feeding vessels extramedullary aneurysm resection extramedullary aneurysm resection
improved improved
branch of ASA improved PSA (posterolateral arimproved tery variant) aberrant branch of PSA intramedullary aneurysm resection improved (serpiginous)
* Abbreviations:SAH = subarachnoidhemorrhage;PSA = posteriorspinal artery;ASA = anteriorspinal artery. J. Neurosurg. / Volume 77~July, 1992
149
T. Handa, et al. and two with extramedullary aneurysms. In total, nine of the 11 cases had aneul3'sm rupture. The anterior spinal artery was the predominant site of these aneurysms (six cases). Unusual arterial anatomy was noted in four patients (Cases 6, 8, 10, and 11). Although there appeared to be an equal distribution between aneurysms located in the cervical and thoracic regions, in five cases the aneurysms were found at the C-1 or C-2 level. A female preponderance was noted (eight females and three males). Our patient was the only child in the group. It is of interest that of the 11 patients, eight had their aneurysm obliterated or excised and improved postoperatively. The initial presentation in our patient was probably due to acute hematomyelia from rupture of a smaller lesion, thus leaving a collagenous aneurysmal wall with no elastic tissue, as was noted. The patient described by Kinal and Sejanovich 9 also had a collagenous aneurysreal wall with an intraluminal subacute clot. It should be noted that pathological confirmation of a true saccular aneurysm with internal elastic lamina was made in only four patients (Cases 4, 8, 9, and 10). Two of the patients reported had vasculopathies, one with pseudoxanthoma elasticum (Case 7) and the other with fibromuscular hyperplasia (Case 9). In one patient (Case 4), the fusiform aneurysm noted at autopsy had inflammatory changes in its wall.
Conclusions We present the case of a child with an isolated intramedullary spinal artery aneurysm that was successfully resected. The diagnosis of spinal artery aneurysms is usually difficult without clear evidence of SAH or spinal cord involvement; however, once the diagnosis is made, the surgical indications are clear.
References I. Blackwood W: Vascular disease of the central nervous system, Jn Blackwood W, McMenemy WH, Meyer A (eds): Greenfield's Neuropathology, ed 4. London: Edward Arnold, 1971, pp 71-137 2. Caroscio JT, Brannan T, Budabin M, et al: Subarachnoid hemorrhage secondary to spinal arteriovenous malformation and aneurysm. Report of a case and review of the literature. Arch Neurol 37:101-103, 1980
150
3. Garcia CA, Dulcey S, Dulcey J: Ruptured aneurysm of the spinal artery of Adamkiewicz during pregnancy. Neurology 29:394-398, 1979 4. Goto S, Kamijyo Y, Yonekawa Y, et al: Ruptured aneurysm of the posterior spinal artery of the upper cervical spinal cord: case report. Neurosurgery 22:558-560, 1988 5. Henson RA, Croft PB: Spontaneous spinal subarachnoid haemorrhage. Q J Med 25:53-66, 1988 6. Herdt JR, Di Chiro G, Doppman JL: Combined arterial and arteriovenous aneurysms of the spinal cord. Radiology 99:589-593, 1971 7. H66k O, Lidvall H: Arteriovenous aneurysms of the spinal cord. A report of two cases investigated by vertebral angiography. J Neurosurg 15:84-91, 1958 8. Hopkins CA, Wilkie FL, Voris DC: Extramedullary aneurysm of the spinal cord. Case report. J Neurosurg 24: 1021-1023, 1966 9. Kinal ME, Sejanovich C: Spinal cord compression by an intramedullary aneurysm. Case report and review of the literature. J Nenrosurg 14:561-565, 1957 10. Kito K, Kobayashi N, Mori N, et al: Ruptured aneurysm of the anterior spinal artery associated with pseudoxanthoma elasticum. Case report. J Neurosurg 58:126-128, 1983 11. Leech PJ, Stokes BAR, ApSimon T, et al: Unruptured aneurysm of the anterior spinal artery presenting as paraparesis. Case report. J Neurosurg 45:331-333, 1976 12. Miyamoto S, Kikuchi H, Karasawa J, et al: Spinal cord arteriovenous malformations associated with spinal aneurysms. Neurosurgery 13:577-580, 1983 13. Moore DW, Hunt WE, Zimmerman JE: Ruptured anterior spinal artery aneurysm: repair via a posterior ap-, proach. Neurosurgery 10:626-630, 1982 14. Saunders FW, Birchard D, Willmer J: Spinal artery aneurysm. Surg Neurol 27:269-272, 1987 15. Scoville WB: lntramedullary arteriovenous aneurysm of the spinal cord. Case report with operative removal from the conus medullaris. J Neurosurg 5:307-312, 1948 16. Shenkin HA, Jenkins F, Kim K: Arteriovenous anomaly of the brain associated with cerebral aneurysm. Case report. J Neurosurg 34:225-228, 1971 17. Smith BS, Penka CF, Erickson LS, et al: Case report: subarachnoid hemorrhage due to anterior spinal artery aneurysm. Nenrosurgery 18:217-219, 1986 18. Thomson RL: Aneurysm in the cervical spinal canal. Med J Aust 1:220-222, 1980 19. Vincent FM: Anterior spinal artery aneurysm presenting as a subarachnoid hemorrhage. Stroke 12:230-232, 1981 Manuscript received October 17, 1991. Address reprint requests to: Takashi Handa, M.D., Department of Neurosurgery, Nagoya University School of Medicine, 65 Tsuruma-cho, Showa-ku, Nagoya 466, Japan.
J. Neurosurg. / Volume 77 / July, 1992
Isolated intramedullary spinal artery aneurysm presenting with quadriplegia Case report TAKASHI HANDA, M.D., YOSHIO SUZUKI, M.D., KIYOSHI SAITO, M.D., KENICmRO SUGn'A, M.D., ANO SUNXL J. PATEL, M.D.
Department of Neurosurgery, Nagoya UniversitySchool of Medicine, Nagoya, Japan L,, The case is presented of an isolated intramedullary posterior spinal artery aneurysm at the C-2 level in a 3-year-old girl. Quadriplegia related to probable intramedullary hemorrhage was the presenting symptom. Magnetic resonance imaging revealed findings consistent with an intramedullary vascular lesion, and vertebral angiography confirmed this to be an aneurysm of the posterior spinal artery. No associated vascular abnormalities were noted, and the aneurysm was successfully resected. Previous reports of isolated spinal aneurysms are reviewed. KEY WORDS
"
aneurysm
9 posterior spinal artery
PINAL artery aneurysms are rare and are usually associated with arteriovenous malformations (AVM s). 2,6,~2 Symptoms of subarachnoid hemorrhage (SAH) and/or spinal cord involvement guide the physician to the correct diagnosis, although this is often difficult. Isolated spinal artery aneurysms are infrequently reported, ~-5'9-'L~3.~4.~7-t9and little is known about their clinicopathology. The unusual presentation of such a case prompted this contribution.
S
9 spinal cord compression
a presumed diagnosis of transverse myelitis, steroids and glycerol were administered. Over a period of 2 months, she gradually improved. She was extubated and was able to walk. Examination at the time revealed a residual quadriparesis with a distinct cervical sensory level. Magnetic resonance images and CT scans of the cervical spine showed an intramedullary mass at the C-2 level. A dark ring noted around the hyperintense center of the mass was thought to be hemosiderin encompassing a vascular lesion (Fig. 1).
Case Report This 3-year-old girl initially developed quadriparesis 8 months prior to transfer to our institution. Her symptoms began with a transient episode (lasting 24 hours) of difficulty with walking, followed by complaints of headache and abdominal pain. A few days later, she developed weakness in her right arm which progressed to a hemiparesis. There was no history of trauma. On admission to the referring hospital, the patient was lethargic and profoundly quadriparetic (the fight paresis more marked than the left) with a facial palsy. She had hyporeflexia and difficulty in voiding. Sensory examination was not easy due to her age and mental status. A computerized tomography (CT) scan of the head showed no abnormalities. Lumbar puncture revealed a normal opening pressure and clear, colorless cerebrospinal fluid with a normal protein content. The patient's condition deteriorated further to complete quadriplegia, requiting artificial ventilation. With 148
FIG. 1. Left:Computerized tomography scan of the cervical spine showing a round homogeneously enhancing mass (aneurysm) with an apparent neck. Right: Magnetic resonance T2-weighted image, sagittal projection, demonstrating a well-demarcated intramedullary lesion with surrounding low signal intensity, probably indicating aneurysmal wall with hemosidefin.
J. Neurosurg. / Volume 77 ~July, 1992
I s o l a t e d i n t r a m e d u l l a r y spinal a r t e r y a n e u r y s m neck was identified and cauterized, and the lesion was excised. Hemosiderin deposition was noted around the aneurysm. Further inspection revealed that the neck of the aneurysm arose from a large aberrant arterial loop branching off the posterior spinal artery. No other vascular abnormalities were seen. Postoperative Course. The patient's postoperative course was uneventful. Pathological studies revealed the wall of the aneurysm to be mostly collagenous with very little internal elastic lamina. Discussion
FtG. 2. Left vertebral angiograms, anteroposterior (left) and lateral (righO views, revealing an aneurysm fed by a large tortuous posterior spinal artery.
Examination. At this point, the patient was transferred to our institution. Vertebral angiography confirmed the intramedullary mass to be an aneurysm fed by a large tortuous posterior spinal artery (Fig. 2). Operation. Aneurysm resection was performed via the posterior approach. When the dura was opened, a large serpiginous posterior spinal artery was seen on the left dorsolateral aspect of the spinal cord. A slightly bluish tint of the cord medial to this vessel indicated the location of the aneurysm. After temporary occlusion of the feeding artery, which was identified using microvascular Doppler ultrasonography, the aneurysm was exposed via a midline myelotomy. The aneurysm
Spinal artery aneurysms are rare and are usually reported in association with vascular malformations. 2,6-8, ~z,~5The reported incidence of spinal artery aneurysms associated with spinal AVM's is 7.7% to 8.0%, which is similar to that of intracranial aneurysms with AVM's. Patients with either of these lesions seem to have a higher incidence of SAH, 6'~z the source of which remains questionable. In a few cases, successful obliteration or excision of the AVM has resulted in spontaneous regression of the associated aneurysm) TM A review of the literature has disclosed only I0 previous cases of isolated spinal artery aneurysms (Table 1). 3-5'9-11'13"14'17-19A few cases were excluded due to either confusion in terminology or uncertainty over the existence of an associated vascular malformation. Spinal artery aneurysms have also been described in patients with aortic coarctation and syphilis) Henson and Croft 5 were the first to report an isolated aneurysm, seen at autopsy in a patient with SAH. The first report of an isolated intramedullary aneurysm and also the first successful excision of the aneurysm was by Kinal and Sejanovich. 9 Of the 11 reported cases (including ours), seven presented with symptoms typical of spinal SAH. The other four had cord compression syndromes, two with intramedullary aneurysms and hematomyelia
TABLE 1
Published cases of isolated spinal artery aneurysms* Case No.
Authors & Year
Age (yrs), Sex
1 2
5l, M 41, F
3 4 5 6
Henson & Croft, 1956 Kinal & Sejanovich, 1957 Leech, et al., 1976 Garcia, et al., 1979 Thomson, 1980 Vincent, 1981
7 8
Kito, et al., 1983 Smith, et al., 1986
37, F 29, M
9
10
Saunders, et aL, 1987 Goto, el al., 1988
44, F 53, M
SAH SAH
T-I C-2
11
Handa, et aL, 1992
3, F
quadriparesis
C-2
25, 34, 66, 30,
F F F F
Clinical Symptom
Site
Vascular Supply
PSA unidentified intramedullary artery ASA paraparesis T-7 SAH T-6 artery of Adamkiewicz ASA quadriparesis C1-2 SAH C-I ASA (anomalous branches) ASA SAH T-10 SAH T-12 ASA (serpiginous) SAH paraparesis
C-I C-7
Relation to Cord
Operation
Outcome
extramedullary no died intramedullary aneurysm resection improved extramedullary aneurysmresection improved extramedullary no died extramedullary aneurysm dipping improved extramedullary aneurysm dipping improved extramedullary no extramedullary clipping of feeding vessels extramedullary aneurysm resection extramedullary aneurysm resection
improved improved
branch of ASA improved PSA (posterolateral arimproved tery variant) aberrant branch of PSA intramedullary aneurysm resection improved (serpiginous)
* Abbreviations:SAH = subarachnoidhemorrhage;PSA = posteriorspinal artery;ASA = anteriorspinal artery. J. Neurosurg. / Volume 77~July, 1992
149
T. Handa, et al. and two with extramedullary aneurysms. In total, nine of the 11 cases had aneul3'sm rupture. The anterior spinal artery was the predominant site of these aneurysms (six cases). Unusual arterial anatomy was noted in four patients (Cases 6, 8, 10, and 11). Although there appeared to be an equal distribution between aneurysms located in the cervical and thoracic regions, in five cases the aneurysms were found at the C-1 or C-2 level. A female preponderance was noted (eight females and three males). Our patient was the only child in the group. It is of interest that of the 11 patients, eight had their aneurysm obliterated or excised and improved postoperatively. The initial presentation in our patient was probably due to acute hematomyelia from rupture of a smaller lesion, thus leaving a collagenous aneurysmal wall with no elastic tissue, as was noted. The patient described by Kinal and Sejanovich 9 also had a collagenous aneurysreal wall with an intraluminal subacute clot. It should be noted that pathological confirmation of a true saccular aneurysm with internal elastic lamina was made in only four patients (Cases 4, 8, 9, and 10). Two of the patients reported had vasculopathies, one with pseudoxanthoma elasticum (Case 7) and the other with fibromuscular hyperplasia (Case 9). In one patient (Case 4), the fusiform aneurysm noted at autopsy had inflammatory changes in its wall.
Conclusions We present the case of a child with an isolated intramedullary spinal artery aneurysm that was successfully resected. The diagnosis of spinal artery aneurysms is usually difficult without clear evidence of SAH or spinal cord involvement; however, once the diagnosis is made, the surgical indications are clear.
References I. Blackwood W: Vascular disease of the central nervous system, Jn Blackwood W, McMenemy WH, Meyer A (eds): Greenfield's Neuropathology, ed 4. London: Edward Arnold, 1971, pp 71-137 2. Caroscio JT, Brannan T, Budabin M, et al: Subarachnoid hemorrhage secondary to spinal arteriovenous malformation and aneurysm. Report of a case and review of the literature. Arch Neurol 37:101-103, 1980
150
3. Garcia CA, Dulcey S, Dulcey J: Ruptured aneurysm of the spinal artery of Adamkiewicz during pregnancy. Neurology 29:394-398, 1979 4. Goto S, Kamijyo Y, Yonekawa Y, et al: Ruptured aneurysm of the posterior spinal artery of the upper cervical spinal cord: case report. Neurosurgery 22:558-560, 1988 5. Henson RA, Croft PB: Spontaneous spinal subarachnoid haemorrhage. Q J Med 25:53-66, 1988 6. Herdt JR, Di Chiro G, Doppman JL: Combined arterial and arteriovenous aneurysms of the spinal cord. Radiology 99:589-593, 1971 7. H66k O, Lidvall H: Arteriovenous aneurysms of the spinal cord. A report of two cases investigated by vertebral angiography. J Neurosurg 15:84-91, 1958 8. Hopkins CA, Wilkie FL, Voris DC: Extramedullary aneurysm of the spinal cord. Case report. J Neurosurg 24: 1021-1023, 1966 9. Kinal ME, Sejanovich C: Spinal cord compression by an intramedullary aneurysm. Case report and review of the literature. J Nenrosurg 14:561-565, 1957 10. Kito K, Kobayashi N, Mori N, et al: Ruptured aneurysm of the anterior spinal artery associated with pseudoxanthoma elasticum. Case report. J Neurosurg 58:126-128, 1983 11. Leech PJ, Stokes BAR, ApSimon T, et al: Unruptured aneurysm of the anterior spinal artery presenting as paraparesis. Case report. J Neurosurg 45:331-333, 1976 12. Miyamoto S, Kikuchi H, Karasawa J, et al: Spinal cord arteriovenous malformations associated with spinal aneurysms. Neurosurgery 13:577-580, 1983 13. Moore DW, Hunt WE, Zimmerman JE: Ruptured anterior spinal artery aneurysm: repair via a posterior ap-, proach. Neurosurgery 10:626-630, 1982 14. Saunders FW, Birchard D, Willmer J: Spinal artery aneurysm. Surg Neurol 27:269-272, 1987 15. Scoville WB: lntramedullary arteriovenous aneurysm of the spinal cord. Case report with operative removal from the conus medullaris. J Neurosurg 5:307-312, 1948 16. Shenkin HA, Jenkins F, Kim K: Arteriovenous anomaly of the brain associated with cerebral aneurysm. Case report. J Neurosurg 34:225-228, 1971 17. Smith BS, Penka CF, Erickson LS, et al: Case report: subarachnoid hemorrhage due to anterior spinal artery aneurysm. Nenrosurgery 18:217-219, 1986 18. Thomson RL: Aneurysm in the cervical spinal canal. Med J Aust 1:220-222, 1980 19. Vincent FM: Anterior spinal artery aneurysm presenting as a subarachnoid hemorrhage. Stroke 12:230-232, 1981 Manuscript received October 17, 1991. Address reprint requests to: Takashi Handa, M.D., Department of Neurosurgery, Nagoya University School of Medicine, 65 Tsuruma-cho, Showa-ku, Nagoya 466, Japan.
J. Neurosurg. / Volume 77 / July, 1992
