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Subarachnoid hemorrhage from cavernous angioma of the cauda equina: Case report
226
~ ELSEVIER
Subarachnoid Hemorrhage from Cavernous Angioma of the Cauda Equina: Case Report Patrizio Bruni, M.D., Alberto Massari, M.D., Romano Greco, M.D.,
Regulo Hernandez, M.D., Giuseppina Oddi, M.D., and Francesco Chiappetta, M.D. Division of Neurosurgery, San Camillo Hospital and Department of Pathology, C. Forlanini Hospital, Rome, Italy
Bruni P., Massari A, Greco R, Hernandez R, Oddi G, Chiappetta, F. Subarachnoid hemorrhage from cavernous angioma of the cauda equina: case report. Surg Neurol 1994;41:226-9.
A case of spinal subarachnoid hemorrhage from a cavernous angioma of the cauda equina is reported. The patient was a 28-year-old m a n w h o complained of a sudden lowback pain and neck stiffness. Laminectomy at L1-L2 was performed with total removal of the tumor. KEYWORDS:
Subarachnoid hemorrhage; Cavernous Angioma;
Cauda equina
Subarachnoid hemorrhage of spinal origin is rare. According to Walton [21], cavernous angioma represents 1.5% of all cases of subarachnoid hemorrhage (SAH). The most frequent causes are trauma and vascular malformations and, regarding the latter, cavernous angiomas localized in the spine are extremely rare. According to Jellinger [8], they represent 5 to 12% of all spinal vascular anomalies. Cavernomas, which are vascular malformations [18] created by anomalous vessels that are dilated and packed with no interposition of neural tissue [13], rarely occur in the cauda. The literature reports five cases and only three, including ours, presented with subarachnoid hemorrhage (Table 1).
The objective examination performed on arrival indicated a bilaterally positive Lasegue maneuver at 50 degrees, nuchal rigidity, an increase in lower limb osteotendinous reflexes, and an increase in muscular tone. The patient was conscious. Cranial computed tomography (CT) scan and angiography disclosed nothing abnormal. Suspecting a spinal hemorrhage, a magnetic resonance imaging (MRI) of the spine (Figure 1A) was performed and this showed, at L2, a hyperintense intradural lesion compressing the cauda roots and a presumed osseous L1 vertebral cavernoma (Figure 1B). Operation After laminectomy at L1 and L2, the dura and the arachnoid were opened. A small 1-cm diameter reddish mass surrounded by a recent bleeding appeared. After separation from the arachnoidal membranes, the small tumor still adhered to a thin root spraying over the tumor with three crowfoot extensions. After having been successfully detached from the root, the neoplasm was removed (Figure 2). The postoperative course was normal, and the patient was discharged after 7 days. Histologic Findings
Case R e p o r t The 28-year-old patient, a worker, 3 days before admission, woke up suddenly during the night with an acute frontal headache with pain spreading through the whole spine down to the sacral region. This symptomatology lasted about 3 days. On the third day, fever, nausea, and vomiting appeared. Hospitalized with suspected meningitis, the lumbar puncture showed hemorrhagic cerebrospinal fluid (CSF). From this hospital he was then transferred to ours. Address reprint requests to: Patrizio Bruni, M.D., Via L. Greppi 134,
00149 Rome, Italy. Received December 18, 1992; accepted April 14, 1993. © 1994 by Elsevier Science Inc.
The samples taken were included in paraffin, and the sections obtained were stained with hematoxylin-eosin. At microscopic examination, the neoplasm appeared made up of connective tissue rich in hematic pigments that indicated previous hemorrhagic focuses. The vessels were thin, and their dilated lumen was totally or partly obstructed by thrombi at different organization stages (Figure 3). The diagnosis was cavernoma. Discussion Cavernomas, which appear as isolated, well-defined, and blueish formations, are relatively rare in the central nervous system (CNS) where they represent 5 to 12% of 0090-3019/94/$7.00
Hemorrhagic Cavernous Angioma of the Cauda Equina
Surg Neurol
227
1994;41:226-9
A
B
Figure 1. (A) Tl-weighted images of M R l showing at L2 the intradural cavernous angioma as a small area of hyperintensity. (B) Preoperative T1weighted M R ! of the spine also showing an osseous angioma of the L1 vertebral body.
cerebral vascular anomalies [17] and 5 to 16% of spinal vascular anomalies preferably located at the vertebral bodies [9]. In their recent and exhaustive review of literature concerning spinal cavernomas, Pagni et al [11] could
Figure 2. Operation: (A) After retraction of the roots of the cauda (c_), a little berry-like tumor (t) with a clot (arrow) is shown; (B) the tumor is separating from the roots by a blunt dissection; and (C) is almost removed while only a rootlet remains adherent (arrow). ~*) caudad.
228
Surg Neurol 1994;41:226-9
Bruni et al
Table 1. Reported Cases of Cavernous Angioma of the Cauda Equina Case
Author/yr
M/F
Age
SAH
Symptoms
syndrome of the cauda; headache; nuchal rigidity syndrome of the cauda; bilateral sciatica and low back pain; lumbar tenderness; neck stiffness; low back and sciatic pain; sphynct, loss; difficulty in walk; headache; vomiting; neck stiffness; lumbar tenderness
1.
Hirsch et al 1965 [7]
M
20
YES
2.
Pansini and Lo Re 1966 [12]
M
46
No
3.
Ueda et al 1987 [20] Pagni et al 1990 [11] Ramos et al 1990 [13] Bruni et al (present case, 1993)
M
28
YES
M
46
No
F
67
?
M
28
YES
4. 5. 6.
Level
L2-L3 L2 L1-L2 D12-L1 L3 L2
Abbreviation:SAH, subarachnoidhemorrhage.
identify 28 intramedullary and ten extramedullary cases. Concerning localization at the cauda equina level, we could identify only five cases and, out o f these, only two [7,20] resulted in subarachnoid hemorrhage. In the case o f Ramos [ 13], in which the patient developed a secondary hydrocephalus, it is not certain that a subarachnoid h e m o r r h a g e had b e e n present. From a clinical point o f view, the spinal cavernous angioma usually behaves as an expansive process but, if the presentation is acute, the S A H signs could mask the clinical picture and m a k e the diagnosis difficult. The clinical s y n d r o m e o f spinal subarachnoid hemorrhage
Figure 3. Photomicrograph: connective tissue rich in hemosiderinepigment and dilated vessels (H & H, x 10).
includes sudden headache, acute sciatic pain, and hemorrhagic xanthochromic CSF, and it is important that awareness is preserved [5]. As in the case of other spinal tumors causing subarachnoid h e m o r r h a g e [2,15], for cavernous angiomas also, apart from their vascular structure [4], a dynamic mechanism may be superimposed due to the extreme mobility o f the lumbosacral spine. In fact, considering that all cavernous angiomas o f the cauda described in literature were m o r e or less strongly adherent to one or m o r e spinal roots and that in one case a root had to be sacrificed to allow removal [11], it is possible to relate the acute
Hemorrhagic Cavernous Angioma of the Cauda Equina
hemorrhagic outcome with the stretching of the roots and the angioma or the squashing of the tumor against the walls of the vertebral canal [2]. On the other hand, chronic symptomatology can be caused by repeated small bleedings. These can explain both the emergence of complications (hydrocephalous like in the Ramos case) [13] and the increase of the lesion itself due to organization, fibrosis, and calcification [18]. In fact, small hemorrhages can obstruct and thrombose the vascular canals which, in turn, can determine necrosis of the thin vasal walls and cause further hemorrhages. This growth mechanism and the possible compression of one or more spinal roots, can obviously mimic symptomatologies typical of ruptured disc or cauda equina tumor. From a histogenetic point of view, although cavernous angiomas are considered entities of their own, the hypothesis was made in the past that they might represent a stage in the evolution of a telangiectatic malformation, supported also by the lack of smooth and elastic tissue in the walls of both vascular anomalies [14,17]. According to Enhi and Love [3] this could represent a differentiation of the primitive pluri-potent mesenchymal cells under traumatic or other stimuli. Willis [22], on the other hand, underscores the congenital origin of the lesion and considers it as an actual "hamartoma." Magnetic resonance imaging is useful to rule out occult sources of subarachnoid hemorrhage when they occur from the spine [4,10,16].
References 1. Bailey P, Bucy PC. Cavernous hemangioma of the vertebrae. JAMA 1929;92:1748-51. 2. Bruni P, Esposito S, Oddi G, Hernandez R, Martines F, Patricolo A. Subarachnoid hemorrhage from multiple neurofibromas of the cauda equina: case report. Neurosurgery 1991;28:910-3. 3. Enhi G, Love JC. Intraspinal lipomas. Report cases: review of literature and clinical and pathological study. Arch Neurol Psychiatry 1945;53:1-27. 4. Fontaines S, Melanson D, Cosgrover R, Bertrand G. Cavernous hemangiomas of the spinal cord: MR imaging. Radiology 1988;166:839-41.
Surg Neurol 1994;41:226-9
229
5. Fincher EF. Spontaneous subarachnoid hemorrhage in intradural tumors of the lumbar sac. J Neurosurg 1951;8:576-84. 6. Gray F, Gherardi R, Benhaiem-Sigaux N. Les hemangiomes vertebraux. Definition, limites, aspect anatomo-pathologiques. Neurochirurgie 1988;35:267-9. 7. Hirsch JF, Pradat P, David M. Angiomes caverneux de la queue de cheval. Neurochirurgie 1965;11:323-7. 8. JeUinger K. The morphology of centrally situated angiomas. In Pia HW, GleaveJRW, Grote E, ZierskiJ, eds. Cerebral angiomas. Advances in diagnosis and therapy. New York: Springer-Verlag, 1975:9-20. 9. Newton Th M, Potts DG, eds. Computed tomography of the spine and spinal cord. Chapter 21: Vascular malformations. San Anselmo, CA: Clavadel Press, 1983: 398. 10. Ogilvy CS, Louis DM, Ojemann RG. Intramedullary cavernous angiomas of the spinal cord: clinical presentation, pathological features, and surgical management. Neurosurgery 1992;31: 219-30. 11. Pagni A, Canavero S, Forni M. Report of a cavernoma of the cauda equina and review of the literature. Surg Neurol 1990;33:12431. 12. Pansini A, Lo Re F. Raro caso di angiocavernoma della cauda Mem Soc Tos Uni Chir 1966;27:679-96. 13. Ramos F, Jr, De Toffol B, Aesch B, Jan M. Hydrocephalus and cavernoma of the cauda equina. Neurosurgery 1991 ;27:139-42. 14. Russell DL, Rubinstein LJ: Pathology of tumors of the nervous system. Fourth edition. Baltimore: William and Wilkins, 1977. 15. Scotti G, Filizzolo F, Scialfa G, Tampieri D. Repeated subarachnoid hemorrhage from a cervical meningioma. J Neurosurg 1987;66:779-81. 16. Rigamonti D, Drayer BP, Johnson PC, Hadley MN, Zabramski J, Spetzler RF. The MRI appearance of cavernous malformations (angiomas). J Neurosurg 1987;67:518-24. 17. Rigamonti D, Johnson PC, Spetzler RF, Hadley MN, Drayer BP. Cavernous malformations and capillary telangiectasia: spectrum within a single pathological entity. Neurosurgery 1991;28:60-4. 18. Simard JM, GarciaoBengochea F, Ballinger WE, MickleJP, Quisling RG. Cavernous angioma: a review of 126 collected and 12 new cases. Neurosurgery 1986;18:162-72. 19. Vaquero J, Martinez R, Martinez P. Cavernomas of the spinal cord: report of two cases. Neurosurgery 1990;22:143-4. 20. Ueda S, Saito A, Inomori S. Cavernous angioma of the cauda equina producing subarachnoid hemorrhage. J Neurosurg 1987;66:134-6. 21. WaltonJN: Subarachnoid hemorrhage of unusual aetiology. Neurology (Minneapolis) 1953;3:517-43. 22. Willis RA, ed. Pathology of tumors. London: Butterworth, 1948:658.
~ ELSEVIER
Subarachnoid Hemorrhage from Cavernous Angioma of the Cauda Equina: Case Report Patrizio Bruni, M.D., Alberto Massari, M.D., Romano Greco, M.D.,
Regulo Hernandez, M.D., Giuseppina Oddi, M.D., and Francesco Chiappetta, M.D. Division of Neurosurgery, San Camillo Hospital and Department of Pathology, C. Forlanini Hospital, Rome, Italy
Bruni P., Massari A, Greco R, Hernandez R, Oddi G, Chiappetta, F. Subarachnoid hemorrhage from cavernous angioma of the cauda equina: case report. Surg Neurol 1994;41:226-9.
A case of spinal subarachnoid hemorrhage from a cavernous angioma of the cauda equina is reported. The patient was a 28-year-old m a n w h o complained of a sudden lowback pain and neck stiffness. Laminectomy at L1-L2 was performed with total removal of the tumor. KEYWORDS:
Subarachnoid hemorrhage; Cavernous Angioma;
Cauda equina
Subarachnoid hemorrhage of spinal origin is rare. According to Walton [21], cavernous angioma represents 1.5% of all cases of subarachnoid hemorrhage (SAH). The most frequent causes are trauma and vascular malformations and, regarding the latter, cavernous angiomas localized in the spine are extremely rare. According to Jellinger [8], they represent 5 to 12% of all spinal vascular anomalies. Cavernomas, which are vascular malformations [18] created by anomalous vessels that are dilated and packed with no interposition of neural tissue [13], rarely occur in the cauda. The literature reports five cases and only three, including ours, presented with subarachnoid hemorrhage (Table 1).
The objective examination performed on arrival indicated a bilaterally positive Lasegue maneuver at 50 degrees, nuchal rigidity, an increase in lower limb osteotendinous reflexes, and an increase in muscular tone. The patient was conscious. Cranial computed tomography (CT) scan and angiography disclosed nothing abnormal. Suspecting a spinal hemorrhage, a magnetic resonance imaging (MRI) of the spine (Figure 1A) was performed and this showed, at L2, a hyperintense intradural lesion compressing the cauda roots and a presumed osseous L1 vertebral cavernoma (Figure 1B). Operation After laminectomy at L1 and L2, the dura and the arachnoid were opened. A small 1-cm diameter reddish mass surrounded by a recent bleeding appeared. After separation from the arachnoidal membranes, the small tumor still adhered to a thin root spraying over the tumor with three crowfoot extensions. After having been successfully detached from the root, the neoplasm was removed (Figure 2). The postoperative course was normal, and the patient was discharged after 7 days. Histologic Findings
Case R e p o r t The 28-year-old patient, a worker, 3 days before admission, woke up suddenly during the night with an acute frontal headache with pain spreading through the whole spine down to the sacral region. This symptomatology lasted about 3 days. On the third day, fever, nausea, and vomiting appeared. Hospitalized with suspected meningitis, the lumbar puncture showed hemorrhagic cerebrospinal fluid (CSF). From this hospital he was then transferred to ours. Address reprint requests to: Patrizio Bruni, M.D., Via L. Greppi 134,
00149 Rome, Italy. Received December 18, 1992; accepted April 14, 1993. © 1994 by Elsevier Science Inc.
The samples taken were included in paraffin, and the sections obtained were stained with hematoxylin-eosin. At microscopic examination, the neoplasm appeared made up of connective tissue rich in hematic pigments that indicated previous hemorrhagic focuses. The vessels were thin, and their dilated lumen was totally or partly obstructed by thrombi at different organization stages (Figure 3). The diagnosis was cavernoma. Discussion Cavernomas, which appear as isolated, well-defined, and blueish formations, are relatively rare in the central nervous system (CNS) where they represent 5 to 12% of 0090-3019/94/$7.00
Hemorrhagic Cavernous Angioma of the Cauda Equina
Surg Neurol
227
1994;41:226-9
A
B
Figure 1. (A) Tl-weighted images of M R l showing at L2 the intradural cavernous angioma as a small area of hyperintensity. (B) Preoperative T1weighted M R ! of the spine also showing an osseous angioma of the L1 vertebral body.
cerebral vascular anomalies [17] and 5 to 16% of spinal vascular anomalies preferably located at the vertebral bodies [9]. In their recent and exhaustive review of literature concerning spinal cavernomas, Pagni et al [11] could
Figure 2. Operation: (A) After retraction of the roots of the cauda (c_), a little berry-like tumor (t) with a clot (arrow) is shown; (B) the tumor is separating from the roots by a blunt dissection; and (C) is almost removed while only a rootlet remains adherent (arrow). ~*) caudad.
228
Surg Neurol 1994;41:226-9
Bruni et al
Table 1. Reported Cases of Cavernous Angioma of the Cauda Equina Case
Author/yr
M/F
Age
SAH
Symptoms
syndrome of the cauda; headache; nuchal rigidity syndrome of the cauda; bilateral sciatica and low back pain; lumbar tenderness; neck stiffness; low back and sciatic pain; sphynct, loss; difficulty in walk; headache; vomiting; neck stiffness; lumbar tenderness
1.
Hirsch et al 1965 [7]
M
20
YES
2.
Pansini and Lo Re 1966 [12]
M
46
No
3.
Ueda et al 1987 [20] Pagni et al 1990 [11] Ramos et al 1990 [13] Bruni et al (present case, 1993)
M
28
YES
M
46
No
F
67
?
M
28
YES
4. 5. 6.
Level
L2-L3 L2 L1-L2 D12-L1 L3 L2
Abbreviation:SAH, subarachnoidhemorrhage.
identify 28 intramedullary and ten extramedullary cases. Concerning localization at the cauda equina level, we could identify only five cases and, out o f these, only two [7,20] resulted in subarachnoid hemorrhage. In the case o f Ramos [ 13], in which the patient developed a secondary hydrocephalus, it is not certain that a subarachnoid h e m o r r h a g e had b e e n present. From a clinical point o f view, the spinal cavernous angioma usually behaves as an expansive process but, if the presentation is acute, the S A H signs could mask the clinical picture and m a k e the diagnosis difficult. The clinical s y n d r o m e o f spinal subarachnoid hemorrhage
Figure 3. Photomicrograph: connective tissue rich in hemosiderinepigment and dilated vessels (H & H, x 10).
includes sudden headache, acute sciatic pain, and hemorrhagic xanthochromic CSF, and it is important that awareness is preserved [5]. As in the case of other spinal tumors causing subarachnoid h e m o r r h a g e [2,15], for cavernous angiomas also, apart from their vascular structure [4], a dynamic mechanism may be superimposed due to the extreme mobility o f the lumbosacral spine. In fact, considering that all cavernous angiomas o f the cauda described in literature were m o r e or less strongly adherent to one or m o r e spinal roots and that in one case a root had to be sacrificed to allow removal [11], it is possible to relate the acute
Hemorrhagic Cavernous Angioma of the Cauda Equina
hemorrhagic outcome with the stretching of the roots and the angioma or the squashing of the tumor against the walls of the vertebral canal [2]. On the other hand, chronic symptomatology can be caused by repeated small bleedings. These can explain both the emergence of complications (hydrocephalous like in the Ramos case) [13] and the increase of the lesion itself due to organization, fibrosis, and calcification [18]. In fact, small hemorrhages can obstruct and thrombose the vascular canals which, in turn, can determine necrosis of the thin vasal walls and cause further hemorrhages. This growth mechanism and the possible compression of one or more spinal roots, can obviously mimic symptomatologies typical of ruptured disc or cauda equina tumor. From a histogenetic point of view, although cavernous angiomas are considered entities of their own, the hypothesis was made in the past that they might represent a stage in the evolution of a telangiectatic malformation, supported also by the lack of smooth and elastic tissue in the walls of both vascular anomalies [14,17]. According to Enhi and Love [3] this could represent a differentiation of the primitive pluri-potent mesenchymal cells under traumatic or other stimuli. Willis [22], on the other hand, underscores the congenital origin of the lesion and considers it as an actual "hamartoma." Magnetic resonance imaging is useful to rule out occult sources of subarachnoid hemorrhage when they occur from the spine [4,10,16].
References 1. Bailey P, Bucy PC. Cavernous hemangioma of the vertebrae. JAMA 1929;92:1748-51. 2. Bruni P, Esposito S, Oddi G, Hernandez R, Martines F, Patricolo A. Subarachnoid hemorrhage from multiple neurofibromas of the cauda equina: case report. Neurosurgery 1991;28:910-3. 3. Enhi G, Love JC. Intraspinal lipomas. Report cases: review of literature and clinical and pathological study. Arch Neurol Psychiatry 1945;53:1-27. 4. Fontaines S, Melanson D, Cosgrover R, Bertrand G. Cavernous hemangiomas of the spinal cord: MR imaging. Radiology 1988;166:839-41.
Surg Neurol 1994;41:226-9
229
5. Fincher EF. Spontaneous subarachnoid hemorrhage in intradural tumors of the lumbar sac. J Neurosurg 1951;8:576-84. 6. Gray F, Gherardi R, Benhaiem-Sigaux N. Les hemangiomes vertebraux. Definition, limites, aspect anatomo-pathologiques. Neurochirurgie 1988;35:267-9. 7. Hirsch JF, Pradat P, David M. Angiomes caverneux de la queue de cheval. Neurochirurgie 1965;11:323-7. 8. JeUinger K. The morphology of centrally situated angiomas. In Pia HW, GleaveJRW, Grote E, ZierskiJ, eds. Cerebral angiomas. Advances in diagnosis and therapy. New York: Springer-Verlag, 1975:9-20. 9. Newton Th M, Potts DG, eds. Computed tomography of the spine and spinal cord. Chapter 21: Vascular malformations. San Anselmo, CA: Clavadel Press, 1983: 398. 10. Ogilvy CS, Louis DM, Ojemann RG. Intramedullary cavernous angiomas of the spinal cord: clinical presentation, pathological features, and surgical management. Neurosurgery 1992;31: 219-30. 11. Pagni A, Canavero S, Forni M. Report of a cavernoma of the cauda equina and review of the literature. Surg Neurol 1990;33:12431. 12. Pansini A, Lo Re F. Raro caso di angiocavernoma della cauda Mem Soc Tos Uni Chir 1966;27:679-96. 13. Ramos F, Jr, De Toffol B, Aesch B, Jan M. Hydrocephalus and cavernoma of the cauda equina. Neurosurgery 1991 ;27:139-42. 14. Russell DL, Rubinstein LJ: Pathology of tumors of the nervous system. Fourth edition. Baltimore: William and Wilkins, 1977. 15. Scotti G, Filizzolo F, Scialfa G, Tampieri D. Repeated subarachnoid hemorrhage from a cervical meningioma. J Neurosurg 1987;66:779-81. 16. Rigamonti D, Drayer BP, Johnson PC, Hadley MN, Zabramski J, Spetzler RF. The MRI appearance of cavernous malformations (angiomas). J Neurosurg 1987;67:518-24. 17. Rigamonti D, Johnson PC, Spetzler RF, Hadley MN, Drayer BP. Cavernous malformations and capillary telangiectasia: spectrum within a single pathological entity. Neurosurgery 1991;28:60-4. 18. Simard JM, GarciaoBengochea F, Ballinger WE, MickleJP, Quisling RG. Cavernous angioma: a review of 126 collected and 12 new cases. Neurosurgery 1986;18:162-72. 19. Vaquero J, Martinez R, Martinez P. Cavernomas of the spinal cord: report of two cases. Neurosurgery 1990;22:143-4. 20. Ueda S, Saito A, Inomori S. Cavernous angioma of the cauda equina producing subarachnoid hemorrhage. J Neurosurg 1987;66:134-6. 21. WaltonJN: Subarachnoid hemorrhage of unusual aetiology. Neurology (Minneapolis) 1953;3:517-43. 22. Willis RA, ed. Pathology of tumors. London: Butterworth, 1948:658.