Cavernomas

Intramedullary cavernomas are rare pathologies and represented 3% of our intramedullary pathologies [69]. On even rarer occasions they may be associated with familial multiple cavernomas of the central nervous system (Fig. 3.85) [69,187]. Despite being so rare, this entity has attracted a considerable number of publications [1, 32, 34, 61, 69, 70, 109, 216, 219, 244, 246, 293, 308, 315, 337, 354]. The majority of patients are women [32, 61, 244].

The preoperative clinical course tends to be characterized by a stepwise neurological progression due to repeated hemorrhages [34, 61, 69, 109, 219, 244, 288, 354]. Years maygo bybetween such phases of deterioration [246]. Other patients present a slowly deteriorating course due to smaller hemorrhages and increasing gliosis [34, 61, 69, 244, 246] or an acute onset of symptoms associated with major bleedings [32, 69, 70, 244, 246]. Pediatric patients tend to present acutely more often than adult patients [70]. Pain and sensory problems usually predominate the clinical picture [32, 109], but acute paraplegias due to massive hemorrhages are described [123]. Patients have also been described with subarachnoid hemorrhages [205] and large intramedullary bleedings causing hemato-myelia [235].

A controversial discussion centers around the question of what to recommend for incidentally discovered intramedullary cavernomas. Cantore et al. [34] and Cristante and Hermann [61] recommend observation of such patients. Ojemann et al. [246] suggest removal of incidentally found intramedullary cavernomas if they are located posteriorly (i.e., in a favorable position for resection). The risk of hemorrhage is thought to be about 1.6% per year [32]. San-dalcioglu et al. [288] calculated a higher bleeding rate of 4.5% per patient year and a rebleeding rate of 66% per patient year in those with a previous hemorrhage,

Fig. 3.85. These T2-weighted MRI scans of the thoracic cord neurological symptoms. Given the pathology of his son, he had

(a), cerebrum (b), and cerebellum (c) demonstrate multiple asked for this MRI scan. No recommendation for surgery has cavernomas in a father of a boy with a cavernoma of the third been given so far because of the small sizes of the lesions ventricle. The father complained of minor headaches but no

Fig. 3.85. These T2-weighted MRI scans of the thoracic cord neurological symptoms. Given the pathology of his son, he had

(a), cerebrum (b), and cerebellum (c) demonstrate multiple asked for this MRI scan. No recommendation for surgery has cavernomas in a father of a boy with a cavernoma of the third been given so far because of the small sizes of the lesions ventricle. The father complained of minor headaches but no

Table 3.29. Overview of patients with intramedullary cavernomas

Sex

Age (years)

Level

History (months)

Symptoms

Surgery

Outcome

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