British Journal of Neurosurgey (1992) 6 , 351-356
ORIGINAL ARTICLE
Intradural spinal cavernomas Br J Neurosurg Downloaded from informahealthcare.com by Chulalongkorn University on 01/07/15 For personal use only.
RAJEEV SHARMA, DAMODAR ROUT & VISHNUPURI V. RADHAKRISHNAN*
Departments of Neurosurgery and *Pathology, Sree Chitra Tirunal Institute for Medical Sciences and Technology, Trivandrum 695 01 1, India
Abstract Intradural cavernomas are rare vascular lesions of the spinal cord. Four cases of histologically verified cavernomas of the cord are reported, of which two were extramedullary and two were intramedullary in location. Progressive neurological deficit was the presenting feature in three cases while one patient had a rapid evolution of neurological deficits and was found at surgery to have had bled from the extramedullary lesion. All the patients were subjected to surgery and total excision of the cavernomas was carried out in each case. While two patients improved after surgery the other two remained static. The available literature on spinal cord cavernomas is reviewed.
Key words: Cauda equina, cavernoma, cavernous angioma, intramedullay , spinal cord.
Introduction
Case reports
Cavernous angiomas or cavernomas are rare Case 1 vascular lesions of the central nervous system A 24-year-old male presented with gradually (CNS). Although more frequent in the cere- progressive quadriparesis for 5 months along bral hemisphere^,'-^ they do afflict the rest of with hesitancy of micturition of 2 weeks the neuraxis as well. In the spine, the vertebrae duration. On examination he was found to have are usually affected4 with occasional direct wasting of the sternomastoid and fasciculation extension into the epidural space.5 However, over the trapezius on the right side. He also pure extradural lesions are also well docu- had a spastic quadriparesis without any sensory m e r ~ t e d . ~Intradural .~ cavernomas are rare and deficit. The deep tendon jerks were exaggera review of the literature revealed only 39 well- ated with bilateral extensor plantor responses. documented c a ~ e s . ~ , *The - ~ ~intradural ex- Plain X-rays of the cervical spine were within tramedullary site is the rarest with only 10 normal limits. Myelography revealed cord cases reported,12J5-17~24-z7~31~33 whereas the re- expansion from C I to C4 suggestive of an maining 29 cases had intramedullary caverno- intrinsic cord lesion. mas. In this report we present four additional A cervical laminectomy was performed. On cases of intradural cavernomas seen over a opening the dura, the cord was widened and a period of 13 years of which two were intrame- brownish discoloration was in evidence at the dullary in location, along with a review of the upper three cervical segments. After myeloliterature. tomy a well-circumscribed lesion could be
351
Br J Neurosurg Downloaded from informahealthcare.com by Chulalongkorn University on 01/07/15 For personal use only.
352
Rajeev Sharma, Damodar Rout & Vishnupuri V. Radhakrishnan
dissected out with moderate difficulty because of adhesions to the surrounding cord tissue. Histopathology was consistent with a cavernous angioma. There was compact arrangement of thin-walled vascular channels of variable calibre, the vast majority of which showed hyaline sclerosis in their walls. There was no cord tissue in between these vascular elements. Post-operatively, after initial worsening in motor power, the patient gradually improved and was ambulatory at discharge 1 month later. On follow-up after 1 year he had no motor weakness but still had hyperreflexia. Case 2 A 63-year-old male, a known diabetic and hypertensive, presented with a complaint of low back pain and weakness of the lower limbs for 2 months along with retention of urine 1 week prior to admission. On examination he was found to have hypotonia in both lower limbs along with wasting of the right thigh and calf muscles. Motor power was grade 0 in the right leg and grade I1 in the left leg. Touch and pain sensation were impaired below L2 on the right while sensation was normal on the left side. The deep tendon reflexes were absent in both lower limbs. Myelography by the cisternal route showed a total block at DI2. At surgery, the dura was tense and bulging. On opening the dura, the cauda equina roots were displaced around a dark bluish large mass which pushed the conus upwards. It was dissected free from the roots and the conus and excised totally. Histological examination revealed dilated thin-walled vascular channels containing recent as well as old thrombi and haemorrhages. Post-operatively he showed no improvement and sphincteric dysfunction persisted. Case 3 A 43-year-old ex-serviceman presented with pain and girdle sensations along the lower part of the chest for 2 weeks. He also experienced ascending numbness from the toes up to nipple level with rapidly progressive weakness of both
lower limbs and urinary retention 3 days prior to admission to hospital. Examination revealed grade 0 power in both lower limbs except for minima1 dorsiflexion at the left ankle. There was loss of all modalities of sensation below D5.The deep tendon jerks were exaggerated with upgoing plantar responses bilaterally. Myelography revealed an intradural extramedullary block at D5. A dorsal laminectomy extending from D3 to D5was carried out and a well-encapsulated extramedullary mass excised in toto. There was evidence of blood in the subarachnoid space as well as within the tumour. The tumour was dissected free from the dorsal roots and the cord after dividing two to three small feeding vessels. Histopathology was consistent with a cavernous haemangioma (Fig. 1). The patient made a good recovery in the post-operative period and was ambulatory with support within 1 month. At follow-up 3 years later he had no motor or sensory deficit except for hypertonia in both lower limbs.
FIG. 1. Photomicrograph showing numerous dilated thinwalled vascular channels. Note fine reticulin fibres bordering these vascular channels. (Gordon and Sweet’s reticdin stain, X85.)
Case 4
A 26-year-old male presented with a history of recurrent attacks of lower limb weakness over the last 10 years. In the first episode, he developed sudden onset of an excruciating low back pain for 3 days followed by paraplegia and retention of urine. From this state he gradually improved in his motor power and
Br J Neurosurg Downloaded from informahealthcare.com by Chulalongkorn University on 01/07/15 For personal use only.
Intradural spinal cavernomas was ambulatory after 6 months. Five years later he was diagnosed as having pulmonary tuberculosis and was treated for this. Later, he noticed slowly progressive spasticity and weakness of both lower limbs along with numbness and hesitancy of micturition. These complaints had been gradually progressive over a period of 5 years. One month prior to admission he developed sudden worsening in power in both lower limbs, on the right more than the left and became bedridden, with retention of urine. General examination was unremarkable except for a diffuse haemangioma on the left side of the chest. He had a grade 11-111 spastic paraparesis, worse on the right. There was dissociated and suspended sensory loss below DI2.A chest X-ray revealed evidence of healed pulmonary tuberculosis. A myelogram showed evidence of focal medullary expansion at D8-9 level. Spinal angiography was non-contributory. The patient was subjected to a dorsal laminectomy from D, to D9. On opening the dura a focal cord expansion was evident and through the thinned-out cord substance a blackish coloured lesion could be made out. After a myelotomy a well-circumscribed, black lesion with marked adhesion to the surrounding xanthochromic discoloured cord substance was excised totally. Histological examination showed features compatible with a cavernous haemangioma. Post-operatively he had some deterioration in his motor power from which he is now showing a gradual recovery. At 1-year follow-up he is still not ambulatory even with support. Discussion
Cavernous angiomas are uncommon vascular malformation affecting the CNS which may present with seizures, haemorrhage and focal They occur most often in the cerebral hemispheres,',2 but any part of the CNS may be involved. In the spine they are estimated to account for 3-16% of all vascular malformation^.^ The majority of spinal cavernous angiomas are confined to the vertebral bodies4 with occasional extension into the
353
extradural space.5 Pure extradural cavernomas, however, are also well documented in the literat~re.~ Spinal . ~ intradural cavernomas are rare lesions and only 39 well-documented cases could be found on a review of the available l i t e r a t ~ r e . ~ Of . ~ - these, ~ ~ intradural extramedullary cavernomas are most rare with only 10 cases reported so far (Table I) while 29 well-documented intermedullary cases have been documented (Table 11). Cavernomas may remain completely asymptomatic during life, being detected as incidental findings at autopsy.15J9,20,31,37 Symptomatic cavernomas of the cord usually present in middle age.2 They are well-circumscribed, dark blue or brown coloured lesions which may be visible through the thinned-out cord substance. They consist of abnormally dilated blood vessels closely clustered together without any intervening normal neural tissue.37 Intramedullary lesions generally present with gradually progressive paraparesis, sensory loss and pain with occasional haemorrhage resulting in a haematomyelia. Extramedullary cavernomas can present as subarachnoid h a e m ~ r r h a g e ~ ~ J ~ , ~ ~with , * ' ,an~ ~abrupt onset of symptoms in addition to focal deficits as a result of gradual cord compression. Cauda equina lesions may present with intermittent radicular symptoms simulating disc prol a p ~ e The . ~ ~symptoms ~ ~ ~ are usually long standing and the diagnosis may be difficult. The CSF may show elevated proteins and rarely xanthochromia.lL Myelography was the usual method of investigation, often in combination with C T before the availability of MRI.2J1933Myelography may be passed as normal, even on several occasions, because there may not be any cord enlargement." CT myelography is valuable, more so after intravenous contrast which may reveal an enhancing lesion at the affected level." Angiographic workup is usually non-contributory because of the absence of any large feeding vessels.2J1,16,32 MRI is now the diagnostic procedure of choice. It shows a well-defined low-intensity signal admixed with high signal intensity areas which represent old clots or haemosiderin deposits in
Sudden onset of low backache, SAH-two episodes Sudden onset of low backache, -two episodes
Low back pain and flaccid paraparesis -two months, urinary retention one week Pain in back and lower limb weakness -two weeks Retention of urine -three days
57, M
20, M
46, M
22, M 24, M
28, M
46, M
63, M
43, M
Floris, 1958’2
Hirsch et d., 1965I’
Pansini & Lore, 196626
Ortner et d., 19732*
Heimberger et d., 198216
Ueda et d., 198713
Pagni ef d., 199Oz5
Sharma et d. (1991) (present series)
Sharma et al. (1991) (present series)
4
5
6
7
8
9
Intermittent back pain, paraplegia, SAH-one episode Intermittent bilateral sciatica and low backache, sphincter dysfunction Sudden flaccid quadriplegia SAH-four episodes
22, F
Roger et d., 195lZ7
3
-
Turner & Kernohan, 1941” Sudden onset of paraplegia, low back pain Progressive paraparesis
Presentation
2
35, F
Age/Sex
Hadlich, 193015
Reference
1
Case no.
TABLE 1. Intradural extramedullary cavernomas Site
Total excision, 1 cm blue mulberry-like mass Total excision, dark blue mass adherent to caudal root Total excision, small bluish black mass adherent to two roots Total excision, large mass compressing the cauda equina roots Total excision, well circumscribed bluish mass, free from cord and roots
Excision
Richly vascularized mass Cauda equina mass. Total excision Small round mass
Incidental at autopsy Autopsy findings Total excision
Operation
Br J Neurosurg Downloaded from informahealthcare.com by Chulalongkorn University on 01/07/15 For personal use only.
Excellent
Unchanged
Excellent
Excellent
Unchanged
Bilateral leg weakness, saddle hypwsthesia
Slow incomplete recovery
-
Worsened 3 years latter due to vertebral angioma
FOUOW-UP
9
P
Ib
w ul
Intradural spinal cavemomas
355
TABLE 11. Intramedullary cavernomas
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Case no. 1 2 3 4 5 6 7 8 9 10 11 12 13 14 15 16 17 18 19 20 21 22 23 24 25 26 27 28 29 30
31
Reference Ohlmacher, 189923 Meyer & Kohler, 1917*l Turner & Kernohan, 194l3I Wyburn-Mason, 194336 Gage1 & Meszaros, 194713 Van Reeth, 195334 Scott, 195630 Odom et al., 195722 Odom et ~ 1 . ~ ~ Odom er ~ 1 . ~ ~ Schroeder & Brunngraber, 196429 Bergstrand er al., 19649 Bergstrand et al., 19649 Goran et al., 196414 Beraud & Meloche, 19658 McCormick & Nofzinger, 196620 Jellinger, 19784 Bicknell et al., 19781° Lindboe & Nordal, 198519 Tyndel et al., 198532 Kitamura er al., 198618 Cosgrove et al., 1988" Cosgrove et aL, 1988" Cosgrove et al., 1988" Cosgrove et al., 1988" Cosgrove et al., 1988l' Vaquero et al., 198835 Vaquero et al., 198835 Sabin et al., 19892s Sharma et al. (1991) (present series) Sharma et al. (1991) (present series)
Age/Sex
Site
48, M 29, M
CI-1 C7D4 Unspecified DS Docsolumbar Cervical D2
-
17, 42, 58, 50, 35, 33, 12,
-
F M M F F F F
50 F 47, F 59, F 13, F
-
62, 32, 28, 27,
F F
M F
34, 51, 48, 41, 33, 25, 27, 31,
M F F F F F F M
c3-4
CS Conus
-
D2 Conus D3-4 Conus Cervical (upper) c3-5
D6-1
Dorsal Cervical D2-3
D2-3
Dll-I2 D2-3 c6-l c6-1
Dl D4
24, M
CI-3
26, M
D8-9
the lesion." However, some authors have with a resultant improvement or stabilization in some while they have continued to worsen." reported high signal intensity areas alone.33 The surgical treatment of intramedullary Cavernous angiomas may enlarge by internal cavernomas has been unrewarding with only a haemorrhage or by proliferation at the outer few cases improving after o p e r a t i ~ n ~edge." ~ ~ ~ 'Some ~ ~ ~of~the cases may not exhibit any (including case 1 of the present series). Purely cord enlargement thereby suggesting that these extramedullary lesions, however, pose no spe- lesions are very slow growing and cause cial technical difficulty and lend themselves to pressure atrophy of the cord." This may partly total excision with rewarding r e s ~ l t s , 2as ~ ,in~ ~ explain the poor prognosis after surgery for case 3 of the present series. In general total intramedullary lesions. extirpation of intramedullary cavernomas is difficult because of marked adhesions with the Acknowledgements surrounding cord tissue due to repeated small haemorrhages. Some cases have been subjected The authors thank Dr M. S. Valiathan, Directo a myelotomy and partial excision or biopsy tor, Sree Chitra Tirunal Institute for Medical
356
Rajeev Sharma, Damodar Rout & Vishnupuri V. Radhakrishnan
Sciences and Technology for his kind permission to publish this paper.
Address for correspondence: Dr Rajeev Sharma, King Faisal Hospital, Taif, Saudi Arabia.
medullary) underlying repeated subarachnoid haemorrhage. J Neurol 1982; 226: 289-93. 17 Hirsch JF, Pradat P, David M. Angiome caverneux de la queue de cheval. Neurochirurgica 1965; 11:323-7 (cited by Pagni ef al. 19W5). 18 Kitamura K, Fukui M, Oka K, et al. Hemangiomas of the central nervous system in Japan: an epidemiological and clinicopathological study with special reference to venous cavernous malformations. Neurosurg Rev 1986; 9221-31. 19 Lindboe CF, Nordal HJ. Multiple neurilemmomas of
Br J Neurosurg Downloaded from informahealthcare.com by Chulalongkorn University on 01/07/15 For personal use only.
References 1 Giombini S, Morello G. Cavernous angiomas of the
20
brain. Account of fourteen personal cases and review of the literature. Acta Neurochir (Wien) 1978; 40~61-82.
2 Simard JM, Gracia-Bengochea F, Ballinger WE Jr, Michle JP, Quisling RG. Cavernous angioma, a review of 126 collected and 12 new clinical cases. Neurosurgery 1986; 18:162-72. 3 Yamasaki T, Handa H, Yamashita J, et al. Intracranial and orbital cavernous angiomas. A review of 30 cases. Neurosurgery 1986; 64:197-208. 4 Jellinger K. Pathology of spinal vascular malformations and vascular tumors. In: Pia HW, Djindjian R, eds. Spinal Angiomas: advances in diagnosis and therapy. New York Springer-Verlag, 1978. 5 Guthkelch AN. Hemangioms involving the spinal epidural space. J Neurol Neurosurg Psychiatry 1948; 11:199-210. 6 Padovani R, Tognetti F, Proietti D, Pozzati E, Servadei F. Extrathecal cavernous hemangioma. Surg Neurol 1982; 18:463-5. 7 Richardson RR, Cerullo LJ. Spinal epidural cavernous hemangioma. Surg Neurol 1979; 12966-8. 8 Beraud R, Meloche BR. A propos de deux cas de malformations vasculaires. Union Med Canada 196% 94:176-88. 9 Bergstrand A, Hook 0, Lidvall H. Vascular malformation of the spinal cord. Acta Neurol Scand 1964; 40:169-83. 10 Bicknell JM, Carlow TJ, Kornfeld M. Familial cavernous angiomas. Arch Neurol 1978; 35:746-9. 11 Cosgrove GR, Bertrand G, Fontaine S, Robitaille Y,
12 13
14
15
16
Melanson D. Cavernous angiomas of the spinal cord. J Neurosurg 1988; 68:31-6. Floris V. Angiomie angiomatosi del sistema nervoso. Lav Neuropsich. 1958; 27:159-379 (cited by Pagni et al. 199OZ5). Gage1 0, Meszaros A. Zur frage der myelopathia necroticans. Arch Psych Neurol 1947; 176:423-9 (cited by Pagni et al. 199OZ5). Goran A, Carlson DJ, Fischer RG. Successful treatment of intramedullary angioma of the cord. J Neurosurg 1964; 21:311-14. Hadlich R. Ein fall von Tumor cavernosus des Ruckenmarks mit besonderer Berucksichtigung der neu en Theorien uber die Genese des Kavernoms. Virchows Arch (A) 1930; 172429-41. Heimberger K, Schnaberth G, Koos W, Pendl G, Auff E. Spinal cavernous haemangioma (intradural extra-
21
22 23 24 25
26
27
28
the cauda equina, cavernous hemangioma of the spinal cord and degenerationof the lateral corticospinaltracts in a man with the clinical diagnosis of multiple sclerosis. Clin Neuropathol 1985; 4260-4. McCormick WF, Nofzinger JD. “Cryptic” vascular malformations of the central nervous system. J Neurosurg 1966; 24865-75. Meyer 0, Kohler B. Uber eine auf Kongenitale Basis entstandene Kavernomahnliche Bildung des Ruckenmarks. Frankf Ztschi Path 1917; 2037-57 (cited by Cosgrove er al. 1988.”). Odom GL, Woodhall B, Margolis G. Spontaneous hematomyelia and angiomas of the spinal cord. J Neurosurg 1957; 14192-202. Ohlmacher AP (1989) As cited in Van Reeth PC.34 Ortner WD, Kubin H, Pilz P. Ein Zervikales Kavernoeses angiom. Fortschr Roengtenstr 1973; 118:475-6. Pagni CA, Canavero S, Forni M. Report of a cavernoma of the cauda equina and review of the literature. Surg Neurol 1990; 33:124-31. Pansini A, LoRe F. Raro caso di angiocavernoma della cauda. Mem Soc Tos Urn Chir 1966; 27679-96 (cited by Pagni el al. 199OZ5). Roger H, Paillas JE, Bonnal J, Vigouroux R. Angiomes de la moelle et des racines. Acta Neurol Psych Belg 1951; 7:491-5 (cited by Pagni et al. 199OZ5). Sabin HI, Daniel S, Wild AM, Symon L. Cavernous angioma of the thoracic spinal cord with intra and extramedullary components. Br J Neurosurg 1989;
3~123-6. 29 Schroeder JM, Brunngraber CV. Ueber ein intra-
30 31
32
33
34 35
medullares cavernoeses angiom. Acta Neurochir (Weim) 1964; 12632-41. Scott M. Lower extremity pain simulating sciatica. JAMA 1956; 160528-34. Turner OA, Kernohan JW. Vascular malformations and vascular tumors involving the spinal cord. Arch Neurol 1941; 46:444-63. Tyndel FJ, Bilbao JM, Hudson AR, Colapinto EV. Hemangioma calcificans of the spinal cord. Can J Neurol Sci 1985; 12321-2. Ueda S, Saito A, Inomori S, et al. Cavernous angioma of the cauda equina producing subarachnoid hemorrhage. J Neurosurg 1987; 66:134-6. Van Reeth PC. Contributien a o’letude de ol’angiomatose medullaire. Acta Neurol Psych Belg 1952; 52249-70 (cited by Pagni et al., 199OZ5). Vaquero J, Martinez R, Martinez P. Cavernomasof the spinal cord report of two cases. Neurosurgery 1988;
2143-4. 36 Wyburn-Mason R. The Vascular Abnormalities and
Tumors of the Spinal Cord and its Membranes. London: Kimpton, 1943.
ORIGINAL ARTICLE
Intradural spinal cavernomas Br J Neurosurg Downloaded from informahealthcare.com by Chulalongkorn University on 01/07/15 For personal use only.
RAJEEV SHARMA, DAMODAR ROUT & VISHNUPURI V. RADHAKRISHNAN*
Departments of Neurosurgery and *Pathology, Sree Chitra Tirunal Institute for Medical Sciences and Technology, Trivandrum 695 01 1, India
Abstract Intradural cavernomas are rare vascular lesions of the spinal cord. Four cases of histologically verified cavernomas of the cord are reported, of which two were extramedullary and two were intramedullary in location. Progressive neurological deficit was the presenting feature in three cases while one patient had a rapid evolution of neurological deficits and was found at surgery to have had bled from the extramedullary lesion. All the patients were subjected to surgery and total excision of the cavernomas was carried out in each case. While two patients improved after surgery the other two remained static. The available literature on spinal cord cavernomas is reviewed.
Key words: Cauda equina, cavernoma, cavernous angioma, intramedullay , spinal cord.
Introduction
Case reports
Cavernous angiomas or cavernomas are rare Case 1 vascular lesions of the central nervous system A 24-year-old male presented with gradually (CNS). Although more frequent in the cere- progressive quadriparesis for 5 months along bral hemisphere^,'-^ they do afflict the rest of with hesitancy of micturition of 2 weeks the neuraxis as well. In the spine, the vertebrae duration. On examination he was found to have are usually affected4 with occasional direct wasting of the sternomastoid and fasciculation extension into the epidural space.5 However, over the trapezius on the right side. He also pure extradural lesions are also well docu- had a spastic quadriparesis without any sensory m e r ~ t e d . ~Intradural .~ cavernomas are rare and deficit. The deep tendon jerks were exaggera review of the literature revealed only 39 well- ated with bilateral extensor plantor responses. documented c a ~ e s . ~ , *The - ~ ~intradural ex- Plain X-rays of the cervical spine were within tramedullary site is the rarest with only 10 normal limits. Myelography revealed cord cases reported,12J5-17~24-z7~31~33 whereas the re- expansion from C I to C4 suggestive of an maining 29 cases had intramedullary caverno- intrinsic cord lesion. mas. In this report we present four additional A cervical laminectomy was performed. On cases of intradural cavernomas seen over a opening the dura, the cord was widened and a period of 13 years of which two were intrame- brownish discoloration was in evidence at the dullary in location, along with a review of the upper three cervical segments. After myeloliterature. tomy a well-circumscribed lesion could be
351
Br J Neurosurg Downloaded from informahealthcare.com by Chulalongkorn University on 01/07/15 For personal use only.
352
Rajeev Sharma, Damodar Rout & Vishnupuri V. Radhakrishnan
dissected out with moderate difficulty because of adhesions to the surrounding cord tissue. Histopathology was consistent with a cavernous angioma. There was compact arrangement of thin-walled vascular channels of variable calibre, the vast majority of which showed hyaline sclerosis in their walls. There was no cord tissue in between these vascular elements. Post-operatively, after initial worsening in motor power, the patient gradually improved and was ambulatory at discharge 1 month later. On follow-up after 1 year he had no motor weakness but still had hyperreflexia. Case 2 A 63-year-old male, a known diabetic and hypertensive, presented with a complaint of low back pain and weakness of the lower limbs for 2 months along with retention of urine 1 week prior to admission. On examination he was found to have hypotonia in both lower limbs along with wasting of the right thigh and calf muscles. Motor power was grade 0 in the right leg and grade I1 in the left leg. Touch and pain sensation were impaired below L2 on the right while sensation was normal on the left side. The deep tendon reflexes were absent in both lower limbs. Myelography by the cisternal route showed a total block at DI2. At surgery, the dura was tense and bulging. On opening the dura, the cauda equina roots were displaced around a dark bluish large mass which pushed the conus upwards. It was dissected free from the roots and the conus and excised totally. Histological examination revealed dilated thin-walled vascular channels containing recent as well as old thrombi and haemorrhages. Post-operatively he showed no improvement and sphincteric dysfunction persisted. Case 3 A 43-year-old ex-serviceman presented with pain and girdle sensations along the lower part of the chest for 2 weeks. He also experienced ascending numbness from the toes up to nipple level with rapidly progressive weakness of both
lower limbs and urinary retention 3 days prior to admission to hospital. Examination revealed grade 0 power in both lower limbs except for minima1 dorsiflexion at the left ankle. There was loss of all modalities of sensation below D5.The deep tendon jerks were exaggerated with upgoing plantar responses bilaterally. Myelography revealed an intradural extramedullary block at D5. A dorsal laminectomy extending from D3 to D5was carried out and a well-encapsulated extramedullary mass excised in toto. There was evidence of blood in the subarachnoid space as well as within the tumour. The tumour was dissected free from the dorsal roots and the cord after dividing two to three small feeding vessels. Histopathology was consistent with a cavernous haemangioma (Fig. 1). The patient made a good recovery in the post-operative period and was ambulatory with support within 1 month. At follow-up 3 years later he had no motor or sensory deficit except for hypertonia in both lower limbs.
FIG. 1. Photomicrograph showing numerous dilated thinwalled vascular channels. Note fine reticulin fibres bordering these vascular channels. (Gordon and Sweet’s reticdin stain, X85.)
Case 4
A 26-year-old male presented with a history of recurrent attacks of lower limb weakness over the last 10 years. In the first episode, he developed sudden onset of an excruciating low back pain for 3 days followed by paraplegia and retention of urine. From this state he gradually improved in his motor power and
Br J Neurosurg Downloaded from informahealthcare.com by Chulalongkorn University on 01/07/15 For personal use only.
Intradural spinal cavernomas was ambulatory after 6 months. Five years later he was diagnosed as having pulmonary tuberculosis and was treated for this. Later, he noticed slowly progressive spasticity and weakness of both lower limbs along with numbness and hesitancy of micturition. These complaints had been gradually progressive over a period of 5 years. One month prior to admission he developed sudden worsening in power in both lower limbs, on the right more than the left and became bedridden, with retention of urine. General examination was unremarkable except for a diffuse haemangioma on the left side of the chest. He had a grade 11-111 spastic paraparesis, worse on the right. There was dissociated and suspended sensory loss below DI2.A chest X-ray revealed evidence of healed pulmonary tuberculosis. A myelogram showed evidence of focal medullary expansion at D8-9 level. Spinal angiography was non-contributory. The patient was subjected to a dorsal laminectomy from D, to D9. On opening the dura a focal cord expansion was evident and through the thinned-out cord substance a blackish coloured lesion could be made out. After a myelotomy a well-circumscribed, black lesion with marked adhesion to the surrounding xanthochromic discoloured cord substance was excised totally. Histological examination showed features compatible with a cavernous haemangioma. Post-operatively he had some deterioration in his motor power from which he is now showing a gradual recovery. At 1-year follow-up he is still not ambulatory even with support. Discussion
Cavernous angiomas are uncommon vascular malformation affecting the CNS which may present with seizures, haemorrhage and focal They occur most often in the cerebral hemispheres,',2 but any part of the CNS may be involved. In the spine they are estimated to account for 3-16% of all vascular malformation^.^ The majority of spinal cavernous angiomas are confined to the vertebral bodies4 with occasional extension into the
353
extradural space.5 Pure extradural cavernomas, however, are also well documented in the literat~re.~ Spinal . ~ intradural cavernomas are rare lesions and only 39 well-documented cases could be found on a review of the available l i t e r a t ~ r e . ~ Of . ~ - these, ~ ~ intradural extramedullary cavernomas are most rare with only 10 cases reported so far (Table I) while 29 well-documented intermedullary cases have been documented (Table 11). Cavernomas may remain completely asymptomatic during life, being detected as incidental findings at autopsy.15J9,20,31,37 Symptomatic cavernomas of the cord usually present in middle age.2 They are well-circumscribed, dark blue or brown coloured lesions which may be visible through the thinned-out cord substance. They consist of abnormally dilated blood vessels closely clustered together without any intervening normal neural tissue.37 Intramedullary lesions generally present with gradually progressive paraparesis, sensory loss and pain with occasional haemorrhage resulting in a haematomyelia. Extramedullary cavernomas can present as subarachnoid h a e m ~ r r h a g e ~ ~ J ~ , ~ ~with , * ' ,an~ ~abrupt onset of symptoms in addition to focal deficits as a result of gradual cord compression. Cauda equina lesions may present with intermittent radicular symptoms simulating disc prol a p ~ e The . ~ ~symptoms ~ ~ ~ are usually long standing and the diagnosis may be difficult. The CSF may show elevated proteins and rarely xanthochromia.lL Myelography was the usual method of investigation, often in combination with C T before the availability of MRI.2J1933Myelography may be passed as normal, even on several occasions, because there may not be any cord enlargement." CT myelography is valuable, more so after intravenous contrast which may reveal an enhancing lesion at the affected level." Angiographic workup is usually non-contributory because of the absence of any large feeding vessels.2J1,16,32 MRI is now the diagnostic procedure of choice. It shows a well-defined low-intensity signal admixed with high signal intensity areas which represent old clots or haemosiderin deposits in
Sudden onset of low backache, SAH-two episodes Sudden onset of low backache, -two episodes
Low back pain and flaccid paraparesis -two months, urinary retention one week Pain in back and lower limb weakness -two weeks Retention of urine -three days
57, M
20, M
46, M
22, M 24, M
28, M
46, M
63, M
43, M
Floris, 1958’2
Hirsch et d., 1965I’
Pansini & Lore, 196626
Ortner et d., 19732*
Heimberger et d., 198216
Ueda et d., 198713
Pagni ef d., 199Oz5
Sharma et d. (1991) (present series)
Sharma et al. (1991) (present series)
4
5
6
7
8
9
Intermittent back pain, paraplegia, SAH-one episode Intermittent bilateral sciatica and low backache, sphincter dysfunction Sudden flaccid quadriplegia SAH-four episodes
22, F
Roger et d., 195lZ7
3
-
Turner & Kernohan, 1941” Sudden onset of paraplegia, low back pain Progressive paraparesis
Presentation
2
35, F
Age/Sex
Hadlich, 193015
Reference
1
Case no.
TABLE 1. Intradural extramedullary cavernomas Site
Total excision, 1 cm blue mulberry-like mass Total excision, dark blue mass adherent to caudal root Total excision, small bluish black mass adherent to two roots Total excision, large mass compressing the cauda equina roots Total excision, well circumscribed bluish mass, free from cord and roots
Excision
Richly vascularized mass Cauda equina mass. Total excision Small round mass
Incidental at autopsy Autopsy findings Total excision
Operation
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Excellent
Unchanged
Excellent
Excellent
Unchanged
Bilateral leg weakness, saddle hypwsthesia
Slow incomplete recovery
-
Worsened 3 years latter due to vertebral angioma
FOUOW-UP
9
P
Ib
w ul
Intradural spinal cavemomas
355
TABLE 11. Intramedullary cavernomas
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Case no. 1 2 3 4 5 6 7 8 9 10 11 12 13 14 15 16 17 18 19 20 21 22 23 24 25 26 27 28 29 30
31
Reference Ohlmacher, 189923 Meyer & Kohler, 1917*l Turner & Kernohan, 194l3I Wyburn-Mason, 194336 Gage1 & Meszaros, 194713 Van Reeth, 195334 Scott, 195630 Odom et al., 195722 Odom et ~ 1 . ~ ~ Odom er ~ 1 . ~ ~ Schroeder & Brunngraber, 196429 Bergstrand er al., 19649 Bergstrand et al., 19649 Goran et al., 196414 Beraud & Meloche, 19658 McCormick & Nofzinger, 196620 Jellinger, 19784 Bicknell et al., 19781° Lindboe & Nordal, 198519 Tyndel et al., 198532 Kitamura er al., 198618 Cosgrove et al., 1988" Cosgrove et aL, 1988" Cosgrove et al., 1988" Cosgrove et al., 1988" Cosgrove et al., 1988l' Vaquero et al., 198835 Vaquero et al., 198835 Sabin et al., 19892s Sharma et al. (1991) (present series) Sharma et al. (1991) (present series)
Age/Sex
Site
48, M 29, M
CI-1 C7D4 Unspecified DS Docsolumbar Cervical D2
-
17, 42, 58, 50, 35, 33, 12,
-
F M M F F F F
50 F 47, F 59, F 13, F
-
62, 32, 28, 27,
F F
M F
34, 51, 48, 41, 33, 25, 27, 31,
M F F F F F F M
c3-4
CS Conus
-
D2 Conus D3-4 Conus Cervical (upper) c3-5
D6-1
Dorsal Cervical D2-3
D2-3
Dll-I2 D2-3 c6-l c6-1
Dl D4
24, M
CI-3
26, M
D8-9
the lesion." However, some authors have with a resultant improvement or stabilization in some while they have continued to worsen." reported high signal intensity areas alone.33 The surgical treatment of intramedullary Cavernous angiomas may enlarge by internal cavernomas has been unrewarding with only a haemorrhage or by proliferation at the outer few cases improving after o p e r a t i ~ n ~edge." ~ ~ ~ 'Some ~ ~ ~of~the cases may not exhibit any (including case 1 of the present series). Purely cord enlargement thereby suggesting that these extramedullary lesions, however, pose no spe- lesions are very slow growing and cause cial technical difficulty and lend themselves to pressure atrophy of the cord." This may partly total excision with rewarding r e s ~ l t s , 2as ~ ,in~ ~ explain the poor prognosis after surgery for case 3 of the present series. In general total intramedullary lesions. extirpation of intramedullary cavernomas is difficult because of marked adhesions with the Acknowledgements surrounding cord tissue due to repeated small haemorrhages. Some cases have been subjected The authors thank Dr M. S. Valiathan, Directo a myelotomy and partial excision or biopsy tor, Sree Chitra Tirunal Institute for Medical
356
Rajeev Sharma, Damodar Rout & Vishnupuri V. Radhakrishnan
Sciences and Technology for his kind permission to publish this paper.
Address for correspondence: Dr Rajeev Sharma, King Faisal Hospital, Taif, Saudi Arabia.
medullary) underlying repeated subarachnoid haemorrhage. J Neurol 1982; 226: 289-93. 17 Hirsch JF, Pradat P, David M. Angiome caverneux de la queue de cheval. Neurochirurgica 1965; 11:323-7 (cited by Pagni ef al. 19W5). 18 Kitamura K, Fukui M, Oka K, et al. Hemangiomas of the central nervous system in Japan: an epidemiological and clinicopathological study with special reference to venous cavernous malformations. Neurosurg Rev 1986; 9221-31. 19 Lindboe CF, Nordal HJ. Multiple neurilemmomas of
Br J Neurosurg Downloaded from informahealthcare.com by Chulalongkorn University on 01/07/15 For personal use only.
References 1 Giombini S, Morello G. Cavernous angiomas of the
20
brain. Account of fourteen personal cases and review of the literature. Acta Neurochir (Wien) 1978; 40~61-82.
2 Simard JM, Gracia-Bengochea F, Ballinger WE Jr, Michle JP, Quisling RG. Cavernous angioma, a review of 126 collected and 12 new clinical cases. Neurosurgery 1986; 18:162-72. 3 Yamasaki T, Handa H, Yamashita J, et al. Intracranial and orbital cavernous angiomas. A review of 30 cases. Neurosurgery 1986; 64:197-208. 4 Jellinger K. Pathology of spinal vascular malformations and vascular tumors. In: Pia HW, Djindjian R, eds. Spinal Angiomas: advances in diagnosis and therapy. New York Springer-Verlag, 1978. 5 Guthkelch AN. Hemangioms involving the spinal epidural space. J Neurol Neurosurg Psychiatry 1948; 11:199-210. 6 Padovani R, Tognetti F, Proietti D, Pozzati E, Servadei F. Extrathecal cavernous hemangioma. Surg Neurol 1982; 18:463-5. 7 Richardson RR, Cerullo LJ. Spinal epidural cavernous hemangioma. Surg Neurol 1979; 12966-8. 8 Beraud R, Meloche BR. A propos de deux cas de malformations vasculaires. Union Med Canada 196% 94:176-88. 9 Bergstrand A, Hook 0, Lidvall H. Vascular malformation of the spinal cord. Acta Neurol Scand 1964; 40:169-83. 10 Bicknell JM, Carlow TJ, Kornfeld M. Familial cavernous angiomas. Arch Neurol 1978; 35:746-9. 11 Cosgrove GR, Bertrand G, Fontaine S, Robitaille Y,
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16
Melanson D. Cavernous angiomas of the spinal cord. J Neurosurg 1988; 68:31-6. Floris V. Angiomie angiomatosi del sistema nervoso. Lav Neuropsich. 1958; 27:159-379 (cited by Pagni et al. 199OZ5). Gage1 0, Meszaros A. Zur frage der myelopathia necroticans. Arch Psych Neurol 1947; 176:423-9 (cited by Pagni et al. 199OZ5). Goran A, Carlson DJ, Fischer RG. Successful treatment of intramedullary angioma of the cord. J Neurosurg 1964; 21:311-14. Hadlich R. Ein fall von Tumor cavernosus des Ruckenmarks mit besonderer Berucksichtigung der neu en Theorien uber die Genese des Kavernoms. Virchows Arch (A) 1930; 172429-41. Heimberger K, Schnaberth G, Koos W, Pendl G, Auff E. Spinal cavernous haemangioma (intradural extra-
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the cauda equina, cavernous hemangioma of the spinal cord and degenerationof the lateral corticospinaltracts in a man with the clinical diagnosis of multiple sclerosis. Clin Neuropathol 1985; 4260-4. McCormick WF, Nofzinger JD. “Cryptic” vascular malformations of the central nervous system. J Neurosurg 1966; 24865-75. Meyer 0, Kohler B. Uber eine auf Kongenitale Basis entstandene Kavernomahnliche Bildung des Ruckenmarks. Frankf Ztschi Path 1917; 2037-57 (cited by Cosgrove er al. 1988.”). Odom GL, Woodhall B, Margolis G. Spontaneous hematomyelia and angiomas of the spinal cord. J Neurosurg 1957; 14192-202. Ohlmacher AP (1989) As cited in Van Reeth PC.34 Ortner WD, Kubin H, Pilz P. Ein Zervikales Kavernoeses angiom. Fortschr Roengtenstr 1973; 118:475-6. Pagni CA, Canavero S, Forni M. Report of a cavernoma of the cauda equina and review of the literature. Surg Neurol 1990; 33:124-31. Pansini A, LoRe F. Raro caso di angiocavernoma della cauda. Mem Soc Tos Urn Chir 1966; 27679-96 (cited by Pagni el al. 199OZ5). Roger H, Paillas JE, Bonnal J, Vigouroux R. Angiomes de la moelle et des racines. Acta Neurol Psych Belg 1951; 7:491-5 (cited by Pagni et al. 199OZ5). Sabin HI, Daniel S, Wild AM, Symon L. Cavernous angioma of the thoracic spinal cord with intra and extramedullary components. Br J Neurosurg 1989;
3~123-6. 29 Schroeder JM, Brunngraber CV. Ueber ein intra-
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34 35
medullares cavernoeses angiom. Acta Neurochir (Weim) 1964; 12632-41. Scott M. Lower extremity pain simulating sciatica. JAMA 1956; 160528-34. Turner OA, Kernohan JW. Vascular malformations and vascular tumors involving the spinal cord. Arch Neurol 1941; 46:444-63. Tyndel FJ, Bilbao JM, Hudson AR, Colapinto EV. Hemangioma calcificans of the spinal cord. Can J Neurol Sci 1985; 12321-2. Ueda S, Saito A, Inomori S, et al. Cavernous angioma of the cauda equina producing subarachnoid hemorrhage. J Neurosurg 1987; 66:134-6. Van Reeth PC. Contributien a o’letude de ol’angiomatose medullaire. Acta Neurol Psych Belg 1952; 52249-70 (cited by Pagni et al., 199OZ5). Vaquero J, Martinez R, Martinez P. Cavernomasof the spinal cord report of two cases. Neurosurgery 1988;
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