Intramedullary cavernous angioma. Two case-reports

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Case report

Intramedullary cavernous angioma. Two case-reports

Brahim El Mostarchid *, Jaoud Lrhezzioui, Ali Akhddar, Bouchaib Kadiri, Miloudi Gazzaz, Mohamed Boucetta

Neurosurgery Department, Mohammed V Military Teaching Hospital, Rabat, Ryad, Morocco Received 3 June 2002; accepted 17 September 2002

Abstract

Intramedullary cavernous angiomas are extremely rare. We retrospectively reviewed two cases in a 24-year-old man and 40-year-old woman. T2-weighted magnetic resonance images showed the cavernoma at the cervical and lower thoracic spine, respectively. The patient with cervical involvement also had an asymptomatic cavernoma in the cerebellum. In both patients, the outcome was favorable after complete surgical removal of the lesion.

Keywords:Cavernous angioma; Cavernoma; Cervical spinal cord; Conus medullaris; Intramedullary; T2-magnetic resonance imaging

1. Introduction

Cavernous angiomas or cavernomas are uncommon vascular malformations of the central nervous system. They occur as sporadic and familial variants and can arise any- where in the central nervous system. Spinal cavernomas have been considered to be relatively rare, accounting for only 5–12% of all spinal vascular malformations. Zevgardis et al.

[8] identified 117 cases of intramedullary cavernomas diagnosed between 1903 and 1996. The introduction of magnetic resonance imaging (MRI) has substantially increased the number of diagnosed cases [1–6]. We reviewed two cases of histologically documented intramedullary spinal cord cavernomas seen at our institution.

2. Case-reports 2.1. Case 1

This 24-year-old man was admitted for a 4-month history of low back pain with gradual weakness in the lower limbs that had started on the right. He reported constipation and difficulty passing urine. Neurological findings at admission consisted of spastic paraparesis with a sensory level at T11-

T12, exaggerated deep tendon reflexes in both lower limbs, bilateral extensor plantar reflexes, and absence of the lower abdominal reflexes. His bladder was empty and his rectal tone diminished. Loss of posterior column sensations was noted in both lower limbs. Routine hematological and bio- chemical tests were normal, as were plain radiographs of the thoracolumbar spine. MRI visualized an intramedullary mass at T11-T12, with a mixed signal surrounding by high signal on T2-weighted images (Fig. 1). After T11-T12 laminectomy, myelotomy was performed. A large, grayish- purple, encapsulated, irregular intramedullary mass was excised. The tumor was easily dissectible and was excised completely. Microscopic examination showed large thin- walled vascular spaces lined by flattened endothelium and lacking a true elastic lamina, with intervening fibrous con- nective tissue. Recovery was uneventful and fairly rapid, with complete resolution of the neurological impairments within 8 weeks. One year after the procedure, neurological function was normal.

2.2. Case 2

A 40-year-old woman with a family history of documented intracranial cavernomas, was admitted for a 4-month history of slow progressive neurological impairments involv- ing all four limbs. Neurological examination showed spastic tetraparesis with exaggerated deep tendon reflexes in all four limbs, bilateral extensor plantar reflexes, and absence of

* Corresponding author. 33, Jbal Ayachi, Appt. 7, Agdal, Rabat, Morocco.

E-mail address:[email protected] (B. El Mostarchid).

Joint Bone Spine 70 (2003) 538–540

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doi:10.1016/S1297-319X(03)00072-1

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lower abdominal reflexes. Urinary incontinence and decreased rectal tone were noted. Plain roentgenograms of the cervical spine were normal. Spinal MRI revealed a cervical lesion generating an isointense signal on T1-weighted image and a high intensity signal surrounded by a low-intensity rim on T2-weighted images (Figs. 2 and 3). MRI of the brain disclosed a previously unsuspected cavernoma in the left cerebellar hemisphere (Fig. 4). This lesion was asymptomatic. After C3-C4 laminectomy and incision of the dura mater, a well-circumscribed, typical, intramedullary cavernoma was excised completely under the operating micro- scope. Histological examination confirmed the diagnosis of cavernoma. Partial neurological recovery occurred immedi- ately. Six months after surgery, the patient was able to walk and had normal sphincter function. The deep tendon reflexes in the limbs remained brisk and the plantar reflexes were in extension. No abnormalities ascribable to the cerebellar cavernoma were noted.

3. Discussion

Cavernous angiomas rarely involve the spine and contrib- ute only 5–12% of all spinal vascular abnormalities. Involve-

ment of both the brain and spinal cord is extraordinarily uncommon [1]. Cavernomas accounted for 5.0% of 280 intramedullary lesions in adults and 1.1% of 181 in children [5]. They arise in the vertebral body, from where they can extend to the epidural space, the intradural extramedullary space, or the spinal cord [2,7]. Intramedullary cavernomas are extremely rare. Their most common site of development is the thoracic cord (54%, with 30% in the upper and 24% in the lower thoracic cord); 39% of the lesions were found in the

Fig. 1.Sagittal MRI section, T2-weighted sequence showing an intramedullary mass with mixed signal surrounded by a high-signal rim at T11-T12.

Intramedullary cavernoma.

Fig. 2.Sagittal MRI section, T1-weighted sequence showing a lesion at C3-C4 with isointense signal surrounded by a rim of decreased signal intensity. Cervical intramedullary cavernoma.

Fig. 3.Sagittal MRI section, T2-weighted sequence showing a lesion at C3-C4 as high signal surrounded by a rim of low signal. Cervical intramedullary cavernoma.

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cervical, 54% in the thoracic (30% upper, 24% lower) and 7% in the lumbar cord [8]. The peak age presentation is in the fourth decade [2,4,8]. According to Zevgardis et al. [8], 117 cases have been reported between 1903 and 1996. Caverno- mas are masses of dilated sinusoidal vascular spaces varying in size from a few millimeters to many centimeters. Compli- cations include bleeding, calcification, and thrombosis. The vascular walls are often hyalinized and frequently contain calcium. Hemosiderin staining related to multiple small asymptomatic hemorrhages is common. The tumor contains a variable amount of clotted and unclotted blood. Arterial feeders are small. Cavernomas are well-circumscribed, expand slowly, and may be multiple. The embryogenesis of cavernomas is controversial. Dysplasia of the angioblastic mesoderm has been suggested by some authors, whereas others consider that these lesions are hamartomas [2–5]. The primordial vascular plexus with its solitary endothelial lining is indistin- guishable from cavernoma, suggesting that the cause may be an inability of the primitive vascular plexus to differentiate.

The neurological impairment caused by intramedullary cavernomas may be acute or slowly progressive. Acute impairment is related to bleeding within the spinal cord.

Chronic progressive myelopathy is a result of multiple mi- crohemorrhages with a gliotic reaction to the blood compo- nents. There is no evidence that cavernomas increase in size over time. The rate of rebleeding is unknown, but spinal cavernomas seem more aggressive clinically than do cerebral cavernomas, probably because the spinal cord is less tolerant of mass lesions [5,6].

MRI is the investigation of choice because it offers greater sensitivity than do other imaging studies. T2-weighted se- quences are particularly informative. The characteristic pat- tern includes a reticulated core of mixed signal intensity surrounded by a rim of decreased signal intensity related largely to the presence of hemosiderin. Small lesions may be seen as black dots. MRI may show evidence of bleeding.

Evidence on serial MRI of repeated bleeding episodes may affect treatment decisions. Surrounding edema or a mass effect is uncommon, except when bleeding has occurred recently [1,3]. Presence of multiple lesions with a mixed- signal core and low-signal rim in a patient with a positive family history is virtually diagnostic. When an intramedullary cavernoma is found, MRI of the brain should be performed to look for cerebral cavernomas. Computed tomog- raphy may miss intramedullary cavernomas, particularly small ones. Because the arterial feeders are small, cavernomas are rarely visible on angiograms.

Radical resection of the mass is feasible. Surgical morbid- ity is relatively low in patients with limited preoperative deficits. Given the generally progressive course of the illness and small but worrisome risk of acute catastrophic myelopathy, complete excision of symptomatic cavernomas is recom- mended. Incomplete excision may be followed by symptom recurrence and continued progressive deterioration related to bleeding from the residual malformation. Among the surgi- cally treated patients studied by Zevgardis et al. [8], 66%

improved, 28% experienced no change, and 6% deteriorated [8]. Radiation therapy is not effective and has no place in the treatment of cavernoma.

First-degree relatives of patients with more than one af- fected family member should undergo intracranial contrast CT or, preferably, craniospinal MRI screening, and should receive genetic counseling.

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Fig. 4.Cranial axial MRI in case 2: T2-weighted sequence showing a small lesion with low signal in the left cerebellar hemisphere. Asymptomatic cavernoma in a patient with a cervical cavernoma.

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