- Email: [email protected]
Intraneural cavernous malformation of the cauda equina
984
Case reports / Journal of Clinical Neuroscience 14 (2007) 984–986
Intraneural cavernous malformation of the cauda equina Paolo Cipriano Cecchi a
a,*
, Paolo Rizzo b, Franco Faccioli b, Lamberto Bontempini c, Andreas Schwarz a, Albino Bricolo b
Operative Unit of Neurosurgery, Regional General Hospital, 39100, Bolzano, Italy b Department of Neurosurgery, University Hospital, Verona, Italy c Service of Pathology, University Hospital, Verona, Italy Received 6 April 2006; accepted 4 June 2006
Abstract Spinal cavernous angiomas are rare vascular malformations occurring mainly in the vertebral body with or without an extradural extension. Only 3–5% of these lesions are entirely located in the spinal canal where they can occupy an extradural, intradural–extramedullary or intramedullary position. We present a 75-year-old woman with signs and symptoms of multiple lumbar radiculopathy. The lumbosacral MRI showed an intradural cauda equina lesion with heterogeneous contrast enhancement that was subsequently radically removed through an L3–L4 laminectomy. The microscopic appearance was suggestive of cavernous angioma with intraneural growth. Clinical, radiological, and surgical features of this unusual lesion (to date, only 12 cases are reported) are discussed. 2006 Elsevier Ltd. All rights reserved. Keywords: Cavernous angioma; Cavernoma; Cavernous malformation; Cauda equina; Cauda equina syndrome; Lumbar radiculopathy
1. Introduction Cavernous angiomas (or cavernomas) are vascular malformations that can occur throughout the central nervous system. The spinal variants are most frequently located in the vertebral bone with or without an extradural extension,1 but only in rare cases are they exclusively extradural, intradural–extramedullary or intramedullary.2–5 The following case depicts an unusual example of an intraneural cavernous angioma of the cauda equina, followed by a brief review of the pertinent literature. 2. Case report A 75-year-old woman with no relevant medical history, was admitted to our hospital with complaints of painful paresthesias in both legs without a typical radicular distribution. Her symptoms had been present for 2 months. The neurological examination was unremarkable for her lower extremities, with normal sphincter function and deep tendon reflexes; straight leg-raising was positive bilaterally at 45; an abnormal gait was present due to burning paresthesias in both legs. A lumbo-sacral spine MRI revealed an oval-shaped intradural lesion at L3–L4 measuring 1.5 cm in its major axis, pressing on the cauda equina roots, irregularly iso-hypointense both in the T1 and T2-weighted images, with a significant but heterogeneous contrast enhancement after gadolinium administration (Figs. 1,2). She underwent an L3–L4 laminec*
Corresponding author. Tel.: +39 0471 909716; fax: +39 0471 908451. E-mail address: [email protected] (P.C. Cecchi).
tomy followed by opening of the dura and complete microsurgical removal of a vascular lesion together with a completely encased nerve root. Microscopically the specimen appeared as an angiodisplastic lesion with features of a cavernous malformation, intra- and perineurally located, with signs of thrombosis and recanalization (Fig. 3). The post-operative course was uneventful with significant improvement of leg paresthesias and no additional neurological deficit. 3. Discussion Cavernous angiomas are vascular malformations composed of abnormal, dilated and packed vascular sinusoidal channels without interposed neural tissue.6 They can be found in all locations within the central nervous system, with an incidence of 0.02–0.9% (according to clinical, radiological or autopsy studies),7 but seem quite rare in the spine where they account for 5–12% of all vascular lesions.8 In this setting, they are mainly located in the vertebral bone (mostly the vertebral body) with occasional extradural involvement,1 but purely extradural, intradural–extramedullary or intramedullary forms are also rarely described. Only 3– 5% of these lesions are found inside the spinal canal.2–5 There are very few descriptions (to date, to our knowledge, only 12 cases are documented in the literature) of intradural cavernous angioma of the cauda equina.6,9–18 They can appear as space-occupying or hemorrhagic lesions, independently from their location. These vascular malformations can increase their volume in time, either as a consequence of small repeated intralesional haemorrhage
Case reports / Journal of Clinical Neuroscience 14 (2007) 984–986
985
Fig. 3. Hematoxylin-eosin stain ·100. The microscopic histological appearance of the cavernous malformation from the surgical specimen.
Fig. 1. T1-weighted sagittal lumbo-sacral MRI showing an intradural L3– L4 mass with heterogeneous contrast enhancement.
Fig. 2. T1-weighted axial lumbo-sacral MRI showing the intradural posteriorly located lesion with contrast enhancement after gadolinium administration.
with subsequent thrombosis, hyalinization, fibrosis and relative enlargement of the cavernoma, or as a result of an intrinsic capacity of the cavernomatous tissue to grow independent from bleeding due to endothelial proliferation and/ or neoangiogenesis.2,19 Nevertheless, when the cavernous angioma reaches a ‘critical volume’ (this varies from patient to patient), it compresses the cauda equina roots leading to a clinical behaviour typical of any mass at the same level (low back pain with or without a mono- or pluri-radicular irradiation, sphincter disturbances, gait abnormalities, generally with a subacute-chronic course). On the other hand, in cases of significant extralesional haemorrhage, these malformations can manifest acutely or subacutely, with a complete or incomplete cauda equina syndrome, as well as in the form of a spinal subarachnoid haemorrhage.6,9–18 Small repeated hemorrhages in the subarachnoidal space may also lead to the development of a communicating hydrocephalus, as reported by Ramos and Makino.14,18 Magnetic resonance imaging is the gold standard for the diagnosis of these lesions given the characteristic appearance of cavernous angiomas (a heterogeneous iso-hypointense core on the T1 and T2-weighted images with a hypointense ring of hemosiderin in the T2-weighted
986
Case reports / Journal of Clinical Neuroscience 14 (2007) 986–989
sequences with no or little contrast enhancement);3,5,20 an ‘atypical’ radiological presentation with contrast enhancement after gadolinium administration is also possible,16 with difficulty obtaining a correct pre-operative diagnosis, as in our case. In the differential diagnosis of cauda equina lesions, tumors (meningiomas, neurinomas, ependymomas, haemangioblastomas, paragangliomas), vascular pathologies (arteriovenous malformations, capillary hemangiomas), infections and large herniated disc fragments9,15 must be taken into account. Given the benign nature of cavernous angiomas and the good neurological outcome reported in the literature, in our opinion their management should be a complete microsurgical removal not only to obtain a neurological improvement in symptomatic cases, but also to achieve a definitive diagnosis of a lesion not always easy to interpret radiologically preoperatively, especially in cases of asymptomatic patients. References 1. Padovani R, Tognetti F, Proietti D, et al. Extrathecal cavernous hemangioma. Surg Neurol 1982;18:463–5. 2. McCormick PC, Stein B. Spinal cavernous malformations. In: Awad DL, Barrow DL, editors. Cavernous Malformations. Park Ridge, IL: AANS Publications Committee; 1993. p. 145–50. 3. Ogilvy CS, Louis DN, Ojemann RG. Intremedullary cavernous angiomas of the spinal cord: clinical presentation, pathological features, and surgical management. Neurosurgery 1992;31: 219–30. 4. Talacchi A, Spinnato S, Alessandrini F, et al. Radiological and surgical aspects of pure spinal epidural cavernous angiomas. Report on 5 cases and review of the literature. Surg Neurol 1999;52:198–203. 5. Zevgaridis D, Medele RJ, Hamburger C, et al. Cavernous hemangiomas of the spinal cord. A review of 117 cases. Acta Neurochir (Wien) 1999;141:237–45. 6. Duke BJ, Levy S, Lillehei KO. Cavernous angiomas of the cauda equina: case report and review of the literature. Surg Neurol 1998;50:442–5.
7. Brown RD. Epidemiology and natural history of vascular malformations of the central nervous system. In: Jafar JJ, Awad IA, Rosenwasser RH, editors. Vascular Malformations of the Central Nervous System. Philadelphia: Lippincott Williams & Wilkins; 1999. p. 129–48. 8. Jellinger K. Pathology of spinal vascular malformations and vascular tumors. In: Pie HW, Djindjian R, editors. Spinal Angiomas: Advances in Diagnosis and Therapy. New York: Springer-Verlag; 1978. 9. Bruni P, Massari A, Greco R, et al. Subarachnoid hemorrhage from cavernous angioma of the cauda equina: case report. Surg Neurol 1994;41:226–9. 10. Cervoni L, Celli P, Gagliardi FM. Cavernous angioma of the cauda equina: report of two cases and review of the literature. Neurosurg Rev 1995;18:281–3. 11. 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. 12. Hirsch JF, Pradat P, David M. Angiomes caverneux de la queue de cheval. Neurochirurgie 1965;11:323–7. 13. Pansini A, Lo Re F. Raro caso di angiocavernoma della cauda. Boll Mem Soc Tosco Umbra Chir 1966;27:679–96. 14. Ramos Jr F, De Toffol B, Aesch B, et al. Hydrocephalus and cavernoma of the cauda equina. Neurosurgery 1990;27: 139–42. 15. Ueda S, Saito A, Inomori S, et al. Cavernous angioma of the cauda equina producing subarachnoid hemorrhage. J Neurosurg 1987;66:134–6. 16. Falavigna A, Neto OR, Nassr dos Santos JA, et al. Cavernous angioma of the cauda equina. Case report. Arq Neuropsiquiatr 2004;62:531–4. 17. Moreno R, Romero J, Serrano V, et al. Cavernoma intradural extramedular de la cola de caballo. Rev Neurol 1995;23:1228–30. 18. Makino K, Takamura H, Gotoh S, et al. Cauda equina cavernous hemangioma associated with hydrocephalus: case report. No To Shinkei 1995;47:783–7. 19. Sure U, Freman S, Bozinov O, et al. Biological activity of adult cavernous malformations: a study of 56 patients. J Neurosurg 2005;102:342–7. 20. Fontaine S, Melanson D, Cosgrove R, et al. Cavernous hemangiomas of the spinal cord: MR imaging. Radiology 1988;166:839–41.
doi:10.1016/j.jocn.2006.06.015
Brain abscess following intracerebral haemorrhage A. Siatouni *, T. Mpouras, E.J. Boviatsis, S. Gatzonis, M. Stefanatou, D. Sakas Neurosurgery Department, Athens Medical School, ‘Evangelismos’ Hospital, 45-47 Ipsilandou str, Athens, Greece Received 9 April 2006; accepted 25 June 2006
*
Corresponding author. Tel.: +30 2107201704. E-mail address: [email protected] (A. Siatouni).
Case reports / Journal of Clinical Neuroscience 14 (2007) 984–986
Intraneural cavernous malformation of the cauda equina Paolo Cipriano Cecchi a
a,*
, Paolo Rizzo b, Franco Faccioli b, Lamberto Bontempini c, Andreas Schwarz a, Albino Bricolo b
Operative Unit of Neurosurgery, Regional General Hospital, 39100, Bolzano, Italy b Department of Neurosurgery, University Hospital, Verona, Italy c Service of Pathology, University Hospital, Verona, Italy Received 6 April 2006; accepted 4 June 2006
Abstract Spinal cavernous angiomas are rare vascular malformations occurring mainly in the vertebral body with or without an extradural extension. Only 3–5% of these lesions are entirely located in the spinal canal where they can occupy an extradural, intradural–extramedullary or intramedullary position. We present a 75-year-old woman with signs and symptoms of multiple lumbar radiculopathy. The lumbosacral MRI showed an intradural cauda equina lesion with heterogeneous contrast enhancement that was subsequently radically removed through an L3–L4 laminectomy. The microscopic appearance was suggestive of cavernous angioma with intraneural growth. Clinical, radiological, and surgical features of this unusual lesion (to date, only 12 cases are reported) are discussed. 2006 Elsevier Ltd. All rights reserved. Keywords: Cavernous angioma; Cavernoma; Cavernous malformation; Cauda equina; Cauda equina syndrome; Lumbar radiculopathy
1. Introduction Cavernous angiomas (or cavernomas) are vascular malformations that can occur throughout the central nervous system. The spinal variants are most frequently located in the vertebral bone with or without an extradural extension,1 but only in rare cases are they exclusively extradural, intradural–extramedullary or intramedullary.2–5 The following case depicts an unusual example of an intraneural cavernous angioma of the cauda equina, followed by a brief review of the pertinent literature. 2. Case report A 75-year-old woman with no relevant medical history, was admitted to our hospital with complaints of painful paresthesias in both legs without a typical radicular distribution. Her symptoms had been present for 2 months. The neurological examination was unremarkable for her lower extremities, with normal sphincter function and deep tendon reflexes; straight leg-raising was positive bilaterally at 45; an abnormal gait was present due to burning paresthesias in both legs. A lumbo-sacral spine MRI revealed an oval-shaped intradural lesion at L3–L4 measuring 1.5 cm in its major axis, pressing on the cauda equina roots, irregularly iso-hypointense both in the T1 and T2-weighted images, with a significant but heterogeneous contrast enhancement after gadolinium administration (Figs. 1,2). She underwent an L3–L4 laminec*
Corresponding author. Tel.: +39 0471 909716; fax: +39 0471 908451. E-mail address: [email protected] (P.C. Cecchi).
tomy followed by opening of the dura and complete microsurgical removal of a vascular lesion together with a completely encased nerve root. Microscopically the specimen appeared as an angiodisplastic lesion with features of a cavernous malformation, intra- and perineurally located, with signs of thrombosis and recanalization (Fig. 3). The post-operative course was uneventful with significant improvement of leg paresthesias and no additional neurological deficit. 3. Discussion Cavernous angiomas are vascular malformations composed of abnormal, dilated and packed vascular sinusoidal channels without interposed neural tissue.6 They can be found in all locations within the central nervous system, with an incidence of 0.02–0.9% (according to clinical, radiological or autopsy studies),7 but seem quite rare in the spine where they account for 5–12% of all vascular lesions.8 In this setting, they are mainly located in the vertebral bone (mostly the vertebral body) with occasional extradural involvement,1 but purely extradural, intradural–extramedullary or intramedullary forms are also rarely described. Only 3– 5% of these lesions are found inside the spinal canal.2–5 There are very few descriptions (to date, to our knowledge, only 12 cases are documented in the literature) of intradural cavernous angioma of the cauda equina.6,9–18 They can appear as space-occupying or hemorrhagic lesions, independently from their location. These vascular malformations can increase their volume in time, either as a consequence of small repeated intralesional haemorrhage
Case reports / Journal of Clinical Neuroscience 14 (2007) 984–986
985
Fig. 3. Hematoxylin-eosin stain ·100. The microscopic histological appearance of the cavernous malformation from the surgical specimen.
Fig. 1. T1-weighted sagittal lumbo-sacral MRI showing an intradural L3– L4 mass with heterogeneous contrast enhancement.
Fig. 2. T1-weighted axial lumbo-sacral MRI showing the intradural posteriorly located lesion with contrast enhancement after gadolinium administration.
with subsequent thrombosis, hyalinization, fibrosis and relative enlargement of the cavernoma, or as a result of an intrinsic capacity of the cavernomatous tissue to grow independent from bleeding due to endothelial proliferation and/ or neoangiogenesis.2,19 Nevertheless, when the cavernous angioma reaches a ‘critical volume’ (this varies from patient to patient), it compresses the cauda equina roots leading to a clinical behaviour typical of any mass at the same level (low back pain with or without a mono- or pluri-radicular irradiation, sphincter disturbances, gait abnormalities, generally with a subacute-chronic course). On the other hand, in cases of significant extralesional haemorrhage, these malformations can manifest acutely or subacutely, with a complete or incomplete cauda equina syndrome, as well as in the form of a spinal subarachnoid haemorrhage.6,9–18 Small repeated hemorrhages in the subarachnoidal space may also lead to the development of a communicating hydrocephalus, as reported by Ramos and Makino.14,18 Magnetic resonance imaging is the gold standard for the diagnosis of these lesions given the characteristic appearance of cavernous angiomas (a heterogeneous iso-hypointense core on the T1 and T2-weighted images with a hypointense ring of hemosiderin in the T2-weighted
986
Case reports / Journal of Clinical Neuroscience 14 (2007) 986–989
sequences with no or little contrast enhancement);3,5,20 an ‘atypical’ radiological presentation with contrast enhancement after gadolinium administration is also possible,16 with difficulty obtaining a correct pre-operative diagnosis, as in our case. In the differential diagnosis of cauda equina lesions, tumors (meningiomas, neurinomas, ependymomas, haemangioblastomas, paragangliomas), vascular pathologies (arteriovenous malformations, capillary hemangiomas), infections and large herniated disc fragments9,15 must be taken into account. Given the benign nature of cavernous angiomas and the good neurological outcome reported in the literature, in our opinion their management should be a complete microsurgical removal not only to obtain a neurological improvement in symptomatic cases, but also to achieve a definitive diagnosis of a lesion not always easy to interpret radiologically preoperatively, especially in cases of asymptomatic patients. References 1. Padovani R, Tognetti F, Proietti D, et al. Extrathecal cavernous hemangioma. Surg Neurol 1982;18:463–5. 2. McCormick PC, Stein B. Spinal cavernous malformations. In: Awad DL, Barrow DL, editors. Cavernous Malformations. Park Ridge, IL: AANS Publications Committee; 1993. p. 145–50. 3. Ogilvy CS, Louis DN, Ojemann RG. Intremedullary cavernous angiomas of the spinal cord: clinical presentation, pathological features, and surgical management. Neurosurgery 1992;31: 219–30. 4. Talacchi A, Spinnato S, Alessandrini F, et al. Radiological and surgical aspects of pure spinal epidural cavernous angiomas. Report on 5 cases and review of the literature. Surg Neurol 1999;52:198–203. 5. Zevgaridis D, Medele RJ, Hamburger C, et al. Cavernous hemangiomas of the spinal cord. A review of 117 cases. Acta Neurochir (Wien) 1999;141:237–45. 6. Duke BJ, Levy S, Lillehei KO. Cavernous angiomas of the cauda equina: case report and review of the literature. Surg Neurol 1998;50:442–5.
7. Brown RD. Epidemiology and natural history of vascular malformations of the central nervous system. In: Jafar JJ, Awad IA, Rosenwasser RH, editors. Vascular Malformations of the Central Nervous System. Philadelphia: Lippincott Williams & Wilkins; 1999. p. 129–48. 8. Jellinger K. Pathology of spinal vascular malformations and vascular tumors. In: Pie HW, Djindjian R, editors. Spinal Angiomas: Advances in Diagnosis and Therapy. New York: Springer-Verlag; 1978. 9. Bruni P, Massari A, Greco R, et al. Subarachnoid hemorrhage from cavernous angioma of the cauda equina: case report. Surg Neurol 1994;41:226–9. 10. Cervoni L, Celli P, Gagliardi FM. Cavernous angioma of the cauda equina: report of two cases and review of the literature. Neurosurg Rev 1995;18:281–3. 11. 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. 12. Hirsch JF, Pradat P, David M. Angiomes caverneux de la queue de cheval. Neurochirurgie 1965;11:323–7. 13. Pansini A, Lo Re F. Raro caso di angiocavernoma della cauda. Boll Mem Soc Tosco Umbra Chir 1966;27:679–96. 14. Ramos Jr F, De Toffol B, Aesch B, et al. Hydrocephalus and cavernoma of the cauda equina. Neurosurgery 1990;27: 139–42. 15. Ueda S, Saito A, Inomori S, et al. Cavernous angioma of the cauda equina producing subarachnoid hemorrhage. J Neurosurg 1987;66:134–6. 16. Falavigna A, Neto OR, Nassr dos Santos JA, et al. Cavernous angioma of the cauda equina. Case report. Arq Neuropsiquiatr 2004;62:531–4. 17. Moreno R, Romero J, Serrano V, et al. Cavernoma intradural extramedular de la cola de caballo. Rev Neurol 1995;23:1228–30. 18. Makino K, Takamura H, Gotoh S, et al. Cauda equina cavernous hemangioma associated with hydrocephalus: case report. No To Shinkei 1995;47:783–7. 19. Sure U, Freman S, Bozinov O, et al. Biological activity of adult cavernous malformations: a study of 56 patients. J Neurosurg 2005;102:342–7. 20. Fontaine S, Melanson D, Cosgrove R, et al. Cavernous hemangiomas of the spinal cord: MR imaging. Radiology 1988;166:839–41.
doi:10.1016/j.jocn.2006.06.015
Brain abscess following intracerebral haemorrhage A. Siatouni *, T. Mpouras, E.J. Boviatsis, S. Gatzonis, M. Stefanatou, D. Sakas Neurosurgery Department, Athens Medical School, ‘Evangelismos’ Hospital, 45-47 Ipsilandou str, Athens, Greece Received 9 April 2006; accepted 25 June 2006
*
Corresponding author. Tel.: +30 2107201704. E-mail address: [email protected] (A. Siatouni).