viernes, 30 de marzo de 2012

DX: Pontine cavernoma (deep cavernoma)

This was an easy one..findings are consistent with variable T2-low signal intensities in the pons region clearly suggesting a site of previous bleeding. T1 didn't show hyperintensities so acute hemorraghe is dismissed. It has diffuse surrounding edema that could explain unusual symptoms.

Here are the spot images:


Radiological findings and location makes differentials unnecesary. This is a tipical infratentorial cavernoma knows as deep cavernoma of the pontine region. Deep cavernomas are considered to be those occurring in the brainstem (midbrain, pons or medulla), cerebellar nuclei, thalamus and basal ganglia.

A cavernoma or cavernous malformation is a vascular abnormality of the central nervous system. It consists of a cluster of abnormal, dilated vessels. Pathologically, it is red to purple in colour, appearing as a raspberry. Cavernomas contain blood products at various stages of evolution and are usually less than 3 centimetres in size.

Brainstem cavernomas (or cavernous angiomas)  have recently received a great deal of attention due to enhanced imaging techniques and the realization that even small hemorrhagic events can cause significant neurological deficits. While the majority (approx. 75%) of cavernous angiomas occur in the upper (“supratentorial”) region of the brain, about 1 in 5 are located in the brainstem or in highly sensitive (“eloquent”), lower (“infratentorial “) areas of the brain. The most common symptom for brainstem lesions is focal neurological deficit as opposed to seizure or headache for lesions located in surpratentorial regions.

The nerves that transverse the brainstem control basic, involuntary functions such as respiration, gag reflex, heartbeat regulation, body temperature, pain and heat sensation, and hiccupping as well as other voluntary functions including eye movement, swallowing, facial muscle control, walking, and speech. Both cranial and “long tract” (whole body) nerves can be affected. For the individual, a brainstem cavernous angioma can manifest a disparate range of symptoms making diagnosis difficult.

In a large study reported in the Journal of Neurosurgery in 1997 (Porter et al vol 87, page 190) 37% of patients fully recovered all function following a stroke from a deep cavernoma, 36% had partial recovery and 27% had no recovery of function. This is similar to the experience at the Sydney Aneurysm and AVM Neurosurgical Centre. Whilst it is usual for superficial cavernoma bleeds to fully recover this is not the experience with the deeper cavernomas.

From the time a cavernoma is diagnosed the chances of being unaffected by bleeding over the next 5 years is 57%, over the next 10 years is 33%, over the next 20 years is 11% and over the next 25 years is 6% (Porter et al, J Neurosurg 87:190, 1997). Although some studies have suggested that the risk of bleeding is not influenced by mode of presentation it is likely that if a large number of deep cavernomas could be followed that those with a history of having bled in the past are more likely to bleed in the future.



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