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Print version ISSN 0004-282X
Arq. Neuro-Psiquiatr. vol.68 no.5 São Paulo Oct. 2010
Neuralgia do trigémio causada por angioma venoso
Marcus André AciolyI,II; Elington L. SimõesI; Maud PariseI; Carlos TellesI; Flávio NigriI
IDepartment of Surgical Specialties, Division of Neurosurgery, Pedro Ernesto University Hospital, State University of Rio de Janeiro, Rio de Janeiro RJ, Brazil
IIDepartment of Neurology, University of São Paulo, São Paulo SP, Brazil
Trigeminal neuralgia (TN) is a syndrome characterized by paroxysms of facial pain affecting mainly middle and old-aged patients1,2. Although TN's pathogenesis is not fully understood, there is increasing evidence that TN is caused by demyelination of trigeminal sensory fibres1 mostly owing to an arterial neurovascular compression on the root entry zone (REZ) of the trigeminal nerve1-5. Veins, on the other hand, can be regarded as the only offending vessel in up to 13% of the patients1,4,6.
Other unusual causes of TN include intrinsic brainstem lesions or even compression of the trigeminal nerve root by tumors, in which the compression can occur by the tumor itself, by an interposed blood vessel or by a distortion of the posterior fossa contents displacing the nerve root towards blood vessels or the skull base1. Nonetheless, in a small number of patients, the pathogenesis remains to be determined1.
Although vein compression is usually accepted in TN's pathogenesis, either alone or in combination to arterial compression, developmental venous anomaly (DVA) causing TN is seldom reported with 17 cases described to date2,3,5,7-15. A patient with a classical longstanding right frontal division TN caused by a DVA is reported emphasizing the clinical features and management of such condition.
A 57-year-old man sought treatment for a longstanding classical right frontal division TN. The symptoms were first presented for seven years having benign evolution. The patient showed years of remittent symptoms while developing pain for just a few days. The neurological examination was unremarkable. Magnetic resonance imaging (MRI) of the brain revealed a large transparenchymal draining vein together with caput medusae involving the right brachium pontis thereby contacting the trigeminal REZ (Fig 1). Digital subtraction angiography (DSA) showed the typical angiographic features of DVA, namely the collection of dilated medullary veins draining into a single large draining vein. This vein was located in the cerebellopontine angle ultimately draining into the superior petrosal sinus and right jugular bulb (Fig 2). The symptoms were well controlled with a low dose of carbamazepine schema staying without medication during the remission period. The patient has been handled conservatively ever since. The Ethics Committee from the Hospital Universitário Pedro Ernesto approved this publication.
DVA, also known as venous angioma, is formally the most common type of intracranial vascular malformation15. The term DVA was initially postulated by Lasjaunias et al.16 following a careful analysis of DVA's morphological and clinical details. Lasjaunias et al.16 proposed that DVAs are anatomical variants because they consistently drain normal cerebral tissue within a normal arterial territory, are typically associated with absence of normal venous drainage pathways, demonstrate opacification in the same time as the normal veins at DSA and their surgical removal or ligature may lead to serious ischemic complications.
DVAs are usually incidental but have been controversially associated with cerebellar symptoms, headache, epilepsy, hydrocephalus, haemorrhage, and spontaneous thrombosis3,7,8,11,13,15,16. The true incidence of clinical symptoms remains unknown and it does not seem to be associated with a specific clinical presentation14.
Apart from the clinical controversies8,14, DVAs should be considered symptomatic when their location match with the patient's clinical presentation15. In this way, TN is possibly the only symptom directly attributable to DVA owing to contact or compression of the trigeminal REZ by the dilated vessel or even to irritation of the spinal trigeminal nucleus2,3,5,7,8,11-14. To the best of our knowledge, 17 cases of DVA causing TN have been reported to date2,3,5,7-15. Three cases were excluded from our analysis due to lack of information provided by the original authors11,15 (Table).
When associated with a DVA, TN occurs in a younger population than usual (median age 44 years)2,3,5. It is worth mentioning that in all patients affected of TN from DVA compression a deep venous drainage was encountered, either predominantly to the superior petrosal sinus or incidentally to the vein of Galen, the jugular bulb or even to the occipital sinus. Like in ordinary TN1,6, these patients commonly complain about pain for years (up to 47 years) before being considered for surgery (Table).
DVA management remains controversial3,12. Generally, conservative treatment for DVA has been advocated by many authors due to the unacceptable high morbidity and mortality rates related to its resection15. In patients affected of TN caused by DVA, conservative treatment has been performed rendering partial2,12,14 or complete pain relief, as the presented case.
For patients with progressive symptoms or in life-threatening situations, the surgical treatment should be considered for posterior fossa DVA16. Surgery was performed in 10 of 15 patients reviewed for DVA causing TN2,3,5,7-10,13 (Table). Microvascular decompression (MVD) was performed in all patients but one, in which a glycerol rhizotomy resulted in complete pain relief. DVA ligature was performed in 5 patients rendering complete pain relief in 4 patients2,8,10 and death in one patient due to cerebellar infarction13. Besides to vein ligature, MVD with DVA reposition was accomplished in four patients3,5,7,9, all of them reaching complete pain relief.
Currently, MVD is the surgical treatment of choice for TN owing to good long-term results together with low mortality rates (up to 1%)1,2,6,17. Considering that TN is not a lethal condition per se17 and the unacceptable higher mortality rate (20%) following DVA ligature in comparison to reposition, we believe that DVA ligature for TN treatment should be no longer recommended. Alternatively to MVD, percutaneous procedures, namely radiofrequency thermorhizotomy and percutaneous balloon compression may also play an important role in TN management1 providing good long-term relief of pain, while glycerol rhizotomy should be limited to selected cases due to higher pain recurrence rates17.
In conclusion, DVA is a rare cause of TN occurring in a younger population than usual. Preoperative diagnosis of DVA may contribute for the decision making concerning TN management. Conservative treatment is a reasonable option rendering satisfactory pain control due to benign evolution of the disease while maintaining the functional status. When indicated, the surgical treatment in these given patients should consider MVD of the trigeminal nerve with vein repositioning or percutaneous procedures rather than vein coagulation in order to avoid ischemic complications.
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Marcus André Acioly
Avenida 28 de Setembro 77 / 4º Andar
20551-030 Rio de Janeiro RJ - Brasil
Received 17 May 2009
Received in final form 28 August 2009
Accepted 9 October 2009