Cavernomas

In the presence of an intraspinal hemorrhage spinal vascular malformation, cavernoma, coagulopathy and tumor have to be differentiated. 

Pontine hemorrhages are fatal in around 50% of patients (Wijdicks and St. Louis 1997). Those caused by cavernomas or arteriovenous malformations have a better outcome (Rabinstein et at 2004). The management of patients with hypertensive pontine hemorrhage is usually conservative, but some case reports have documented successful stereotactic aspiration. However, there is likely publication bias and the natural history of the condition is difficult to predict since patients with small hemorrhages do well with conservative management. 

A brainstem cavernoma demonstrating a dramatic spontaneous decrease in size during follow-up case report and review of the literature. Surgical Neurology 63 170-173... 

Fig. 20.12a,b. Portal vein pathologies, a MIP image reconstruction from a CT dataset shows complete thrombosis of the portal vein and of its right branch, and partial thrombosis of the superior mesenteric vein, b MIP image reconstruction from a MR dataset shows a portal cavernoma... 

The coincidence of DVAs and cavernomas, however, is evidence-based and therefore has to be taken into consideration whenever facing a cavernoma or a DVA. Up to one third of DVAs are associated with cavernomas (Figs. 1.5-1.9). 

A major problem of most studies reporting hemorrhages due to a DVA is how they ruled out an associated cavernoma. It is clearly not enough just to obtain T2-weighted images in patients with DVAs. All... 

However, having in mind the association of cavernoma and DVA, all these findings have to be critically reviewed. In 1998, McLaughlin and colleagues published their series on 80 patients with DVAs fo-... 

Fig. 1.8. a,b Patient with two interconnected developmental venous anomalies (DVAs) periinsular and frontal on the left side (contrast-enhanced Tl-images). c T2-image reveals additional intracerebral cavernomas... 

Fig. 1.9a-c. Patient with two developmental venous anomalies, one below (a) and one above (b) a cerebellar cavernoma (c)... 

Another reason for patient s referral to a MRI unit is seizures (Gumus et al. 2007). There might be an association between DVA and epilepsy, even if the EEG focus is not congruent with the location of the DVA. If this is true, one should take a careful look for an associated cavernoma, cavernomas being known to cause different types of epilepsy, mainly due to their content of hemosiderin. 

Fig. I.IOa-g.Non enhanced CT showed a cerebellar hematoma in a 30-year-old male (a), after contrast injection (b) vascular structures were seen adjacent to the bleeding. Contrast enhanced Tl weighted images (c,d,e) showed clearly a DVA, on T2 (f) an associated cavernoma could be suggested which was proved after extirpation, DSA (g) showed a large DVA in the cerebellar hemisphere and revealed no other vascular pathology...

Most of the DVAs are solitary, although multiple lesions might occur in the blue rubber bleb naevus syndrome, characterized by bluish discolored skin and mucocutaneous lesions. However, there are multiple DVAs in patients that do not have any syndromes or any genetic defect (Figs. 1.8 and 1.9), but the likelihood of a coincident cavernoma seems to be increased in these patients. 

In conclusion, patients with head and neck venous malformations obviously have an increased probability of having a DVA and an increased chance of having multiple DVAs. There are no data on whether the risk of having cavernomas is also increased in this patient subgroup. 

However, multiple DVAs are associated with at least one, sometimes even more than one, cavernoma (Figs. 1.8 and 1.9). 

However, if the DVA is associated with a cavernoma, the therapeutic implications are related to the cavernoma. The difference is the surgical approach in a pure cavernoma, the goal is to remove the whole cavernoma including the hemosiderin rim. If associated with a DVA, the draining vein has to be preserved and, therefore, sometimes the cavernoma cannot be removed totally. 

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