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-oldwoman. T2-weighted magnetic resonance images showed the cavernoma at the cervical and lower thoracic spine, respectively. The patientwith cervical involvement also had an asymptomatic cavernoma in the cerebellum. In both patients, the outcome was favorable after completesurgical removal of the lesion.

© 2003 Published by Éditions scientifiques et médicales Elsevier SAS.

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

1. Introduction

Cavernous angiomas or cavernomas are uncommon vas-cular malformations of the central nervous system. Theyoccur as sporadic and familial variants and can arise any-where in the central nervous system. Spinal cavernomas havebeen considered to be relatively rare, accounting for only5–12% of all spinal vascular malformations. Zevgardis et al.[8] identified 117 cases of intramedullary cavernomas diag-nosed between 1903 and 1996. The introduction of magneticresonance imaging (MRI) has substantially increased thenumber of diagnosed cases [1–6]. We reviewed two cases ofhistologically documented intramedullary spinal cord caver-nomas seen at our institution.

2. Case-reports

2.1. Case 1

This 24-year-old man was admitted for a 4-month historyof low back pain with gradual weakness in the lower limbsthat had started on the right. He reported constipation anddifficulty passing urine. Neurological findings at admissionconsisted 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 lowerabdominal reflexes. His bladder was empty and his rectaltone diminished. Loss of posterior column sensations wasnoted in both lower limbs. Routine hematological and bio-chemical tests were normal, as were plain radiographs of thethoracolumbar spine. MRI visualized an intramedullarymass at T11-T12, with a mixed signal surrounding by highsignal on T2-weighted images (Fig. 1). After T11-T12 lami-nectomy, myelotomy was performed. A large, grayish-purple, encapsulated, irregular intramedullary mass was ex-cised. The tumor was easily dissectible and was excisedcompletely. Microscopic examination showed large thin-walled vascular spaces lined by flattened endothelium andlacking a true elastic lamina, with intervening fibrous con-nective tissue. Recovery was uneventful and fairly rapid,with complete resolution of the neurological impairmentswithin 8 weeks. One year after the procedure, neurologicalfunction was normal.

2.2. Case 2

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

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

E-mail address: mostarchid@hotmail.com (B. El Mostarchid).

Joint Bone Spine 70 (2003) 538–540

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lower abdominal reflexes. Urinary incontinence and de-creased rectal tone were noted. Plain roentgenograms of thecervical spine were normal. Spinal MRI revealed a cervicallesion generating an isointense signal on T1-weighted imageand a high intensity signal surrounded by a low-intensity rimon T2-weighted images (Figs. 2 and 3). MRI of the braindisclosed a previously unsuspected cavernoma in the leftcerebellar hemisphere (Fig. 4). This lesion was asymptom-atic. After C3-C4 laminectomy and incision of the duramater, a well-circumscribed, typical, intramedullary caver-noma was excised completely under the operating micro-scope. Histological examination confirmed the diagnosis ofcavernoma. Partial neurological recovery occurred immedi-ately. Six months after surgery, the patient was able to walkand had normal sphincter function. The deep tendon reflexesin the limbs remained brisk and the plantar reflexes were inextension. No abnormalities ascribable to the cerebellar cav-ernoma 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 extraordinarilyuncommon [1]. Cavernomas accounted for 5.0% of 280intramedullary lesions in adults and 1.1% of 181 in children[5]. They arise in the vertebral body, from where they canextend to the epidural space, the intradural extramedullaryspace, or the spinal cord [2,7]. Intramedullary cavernomasare extremely rare. Their most common site of developmentis the thoracic cord (54%, with 30% in the upper and 24% inthe lower thoracic cord); 39% of the lesions were found in the

Fig. 1. Sagittal MRI section, T2-weighted sequence showing an intramedul-lary 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 atC3-C4 with isointense signal surrounded by a rim of decreased signalintensity. Cervical intramedullary cavernoma.

Fig. 3. Sagittal MRI section, T2-weighted sequence showing a lesion atC3-C4 as high signal surrounded by a rim of low signal. Cervical intrame-dullary cavernoma.

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cervical, 54% in the thoracic (30% upper, 24% lower) and7% in the lumbar cord [8]. The peak age presentation is in thefourth decade [2,4,8]. According to Zevgardis et al. [8], 117cases have been reported between 1903 and 1996. Caverno-mas are masses of dilated sinusoidal vascular spaces varyingin size from a few millimeters to many centimeters. Compli-cations include bleeding, calcification, and thrombosis. Thevascular walls are often hyalinized and frequently containcalcium. Hemosiderin staining related to multiple small as-ymptomatic hemorrhages is common. The tumor contains avariable amount of clotted and unclotted blood. Arterial feed-ers are small. Cavernomas are well-circumscribed, expandslowly, and may be multiple. The embryogenesis of caverno-mas is controversial. Dysplasia of the angioblastic mesodermhas been suggested by some authors, whereas others considerthat these lesions are hamartomas [2–5]. The primordialvascular plexus with its solitary endothelial lining is indistin-guishable from cavernoma, suggesting that the cause may bean inability of the primitive vascular plexus to differentiate.

The neurological impairment caused by intramedullarycavernomas may be acute or slowly progressive. Acute im-pairment 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 sizeover time. The rate of rebleeding is unknown, but spinalcavernomas seem more aggressive clinically than do cerebralcavernomas, probably because the spinal cord is less tolerantof mass lesions [5,6].

MRI is the investigation of choice because it offers greatersensitivity than do other imaging studies. T2-weighted se-quences are particularly informative. The characteristic pat-tern includes a reticulated core of mixed signal intensitysurrounded by a rim of decreased signal intensity relatedlargely to the presence of hemosiderin. Small lesions may beseen as black dots. MRI may show evidence of bleeding.Evidence on serial MRI of repeated bleeding episodes mayaffect treatment decisions. Surrounding edema or a masseffect is uncommon, except when bleeding has occurredrecently [1,3]. Presence of multiple lesions with a mixed-signal core and low-signal rim in a patient with a positivefamily history is virtually diagnostic. When an intramedul-lary cavernoma is found, MRI of the brain should be per-formed to look for cerebral cavernomas. Computed tomog-raphy may miss intramedullary cavernomas, particularlysmall ones. Because the arterial feeders are small, caverno-mas are rarely visible on angiograms.

Radical resection of the mass is feasible. Surgical morbid-ity is relatively low in patients with limited preoperativedeficits. Given the generally progressive course of the illnessand small but worrisome risk of acute catastrophic myelopa-thy, complete excision of symptomatic cavernomas is recom-mended. Incomplete excision may be followed by symptomrecurrence and continued progressive deterioration related tobleeding 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 thetreatment of cavernoma.

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

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

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