Neurology India Medknow Publications on behalf of the Neurological Society of India ISSN: 0028-3886 EISSN: 1998-4022 Vol. 52, Num. 4, 2004, pp. 439-442

Neurology India, Vol. 52, No. 4, October-December, 2004, pp. 439-442

Original Article

Intracranial cavernomas: Analysis of 37 cases and literature review

Kayali Hakan, Sait Sirin, Serdar Kahraman, Kaan Oysul, Ilker Solmaz, Erdener Timurkaynak

Departments of Neurosurgery, Oncology of Gulhane Military Medical Academy, Ankara
Correspondence Address:Department of Neurosurgery, Gulhane Military Medical Academy, 06018, Etlik-Ankara, hakankayali@hotmail.com

Code Number: ni04150

ABSTRACT

Key words:

INTRODUCTION

Cavernoma is a benign vascular hamartoma. Approximately 50% of the cases are multiple.[1] They rarely occur in the spinal cord.[2],[3] The onset of symptoms is usually in the third or fourth decade of life, although some cavernomas have presented in childhood.[4] Retrospective surgical series show good results after surgery, but the outcome in such patients if they had not undergone surgery is unknown.[5] Recently, radiosurgery has been proposed for the treatment of cavernomas located in an eloquent region of the brain.[6],[7],[8]

We review our experience with 37 cases of cavernomas.

MATERIALS AND METHODS

The data of 37 cavernoma cases (30 males, 7 females) admitted to our department between 1995 and 2003 were reviewed retrospectively. The median age was 26 years (range, 9-57 years). Clinical features are summarized in [Table - 1] and [Table - 2]. In all the patients, computed tomography (CT) and magnetic resonance imaging (MRI) were performed. Locations of the lesions were 57% supratentorial. Electroencephalogram (EEG) was performed in 19 patients presenting with seizures. Except for two cases, the EEG was abnormal and concurrent with the site of the lesion. Digital substraction angiography (DSA) was performed in 5 patients who had hemorrhage. The angiogram did not reveal any vascular abnormality. Four cases were treated surgically [Figure - 1], 12 cases were treated by SRS and the rest of the patients were managed conservatively. The criteria for the conservative management of cavernoma were when the seizures were controlled with antiepileptics, there was no progressive neurological deficit and when there were less than two hemorrhages. The patients′ preference was taken into consideration. Two patients with frontal cavernoma were treated by SRS as they did not accept the surgical treatment. In radiosurgical treatment, Linear Accelerator (Philips SL-25, UK), Isocentric subsystem (Philips K-X 200, UK), Brown-Roberts-Wells Head Frame (Radionics Co., USA) and X knife3 planning system (Radiosonics Software Applications Inc., USA) were used. 6 MeV X-ray was produced by Linear Accelerator. In all cases single isocenter, median 310 degree total arch angle (300-320) and median 6 arch (5-7) were used. Median 15 Gy (14-20) was applied to the peripheral zone of the lesion (80%) [Figure - 2].

RESULTS

A 40-year-old female who also had multiple sclerosis and was treated surgically for brainstem cavernoma died on the 11th postoperative day. Of the 13 patients treated by SRS, one patient with frontal cavernoma (9-year-old patient whose surgical treatment was not accepted by his parents) had an increase in seizure frequency refractory to medical treatment and underwent surgery in the 12th month after radiosurgery. The median follow-up period was 2.5 years (1-8 years). None of the patients in the series had any neurological or radiological deterioration.

DISCUSSION

The etiology of cavernomas is unknown. Cranial radiation, coexistent vascular malformation, genetic, and hormonal factors all have been implicated for the cavernomas. The proportion of patients developing clinical symptoms is higher in the hereditary form than in the sporadic form of the cavernoma.[9],[10],[11],[12],[13],[14],[15],[16] De novo formation of a cavernoma during immunosuppressive treatment has also been reported.[17] There are some radiation-induced cavernomas occurring in childhood and adolescence.[18]

Cavernoma was reported in 0.39% of 8131 patients evaluated with craniospinal MRI.[19] Our incidence was 3%, with 37 patients with cavernous angioma out of 1228 patients with intracranial mass lesion treated during the period. The majority of the cavernomas are supratentorial in location.[19] In our series 43% of the lesions were located infratentorially. Approximately 60% patients present with seizures, 50% patients have progressive neurological deficit and 20% patients have hemorrhage in intracranial cavernomas.[20],[21] Despite the preponderance of cavernomas above the tentorium cerebelli, the general belief is that hemorrhage is more likely to occur from infratentorial lesions.[19],[21] The location of the lesion is an important factor in predicting hemorrhage or the neurological course. In another series all hemorrhages occurred in the cavernomas located supratentorially.[22] In our series hemorrhage was observed in 5 patients (13.5%). Three of the hemorrhages (60%) were supratentorial.

MR features of cavernomas are characteristic and diagnostic of these lesions obviating the need for angiography.[23] Evidence of a recent or old hemorrhage is commonly present in cases of cavernomas, including hemosiderin-laden macrophages, cholesterol crystals and deposits of hemosiderin in the surrounding parenchyma.[24] We observed calcified cavernomas in two patients. Calcified cavernomas are called "hemangioma calcificans" or "brain stone".[25],[26],[27] Cavernomas have been located in a variety of cranial sites.[28],[29],[30],[31],[32],[33],[34],[35],[36],[37],[38] Pediatric cavernomas are still diagnostically and therapeutically challenging lesions. There is a higher risk of hemorrhage in children when compared to adults.[39]

Although some authors have stated that there was no difference in the hemorrhage rate before and after radiosurgery,[6] other authors recommend it especially for the brainstem, deep and eloquent located cavernomas, and a significant reduction has been observed in the annual hemorrhage rate after radiosurgery.[40],[41],[42],[43],[44],[45],[46]

X-knife radiosurgery was performed for 13 patients in our series and the results were uneventful except for one pediatric case. Hemorrhage, edema around the lesions and increase in the size of the lesions were not observed in our 13 patients treated radiosurgically up to date [Figure - 2].

Although the dominant role of surgery in cavernomas is recently undergoing re-evaluation, an accessible causative cavernoma is an indication for surgical resection.[19]

CONCLUSION

Conservative treatment for cavernoma should be the choice for the patients without new or progressive neurological deficits and two or more documented hemorrhages and with seizure responsive to the medical treatment. For the others, the treatment is primarily surgical for the cases with non-eloquent locations and the results have been best where complete excision is achieved. Radiosurgery may be an alternative for treatment of deep and eloquent area located cavernomas and for the patients who do not accept surgical treatment.

REFERENCES

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