Case Report Stereotact Funct Neurosurg 2015;93:110–113 DOI: 10.1159/000369562

Received: August 24, 2014 Accepted after revision: November 4, 2014 Published online: February 18, 2015

Gamma Knife Stereotactic Radiosurgery for Trigeminal Neuralgia Caused by a Developmental Venous Anomaly Gillian Harrison a L. Dade Lunsford b Edward A. Monaco III b   

 

b

 

Department of Neurosurgery, New York University Langone Medical Center, New York, N.Y., and Department of Neurological Surgery, University of Pittsburgh Medical Center, Pittsburgh, Pa., USA

 

Key Words Trigeminal neuralgia · Developmental venous anomaly · Gamma knife · Stereotactic radiosurgery

Abstract Background: Trigeminal neuralgia (TN) is mostly caused by vascular compression of the nerve’s root entry zone due to an ectatic artery. Rarer causes include compression from tumors, vascular malformations or multiple sclerosis plaques. Developmental venous anomalies (DVAs) are benign, aberrantly appearing venous structures that drain normal cerebral tissue. DVAs are a rare etiology of TN. The management of TN caused by a DVA is controversial as disruption of the DVA can be catastrophic. Methods: We report a case of a young man with severe medically refractory TN related to a brachium pontis DVA who was successfully treated by gamma knife stereotactic radiosurgery (GKSR) to the trigeminal nerve. Results: Within 2 weeks of GKSR, the patient reported experiencing 60% pain relief; 5 years postoperatively, he remains completely pain free with some mild sensory loss in the V2 and V3 areas. Conclusions: GKSR has an established role in the management of TN. This is the first reported case of using GKSR to treat TN caused by a DVA. In the setting of a DVA, GKSR should be an initial consideration for TN therapy after medical failure because of the high surgical risk related to disrupting the DVA. © 2015 S. Karger AG, Basel

© 2015 S. Karger AG, Basel 1011–6125/15/0932–0110$39.50/0 E-Mail [email protected] www.karger.com/sfn

Introduction

The first reported case of trigeminal neuralgia (TN) caused by a developmental venous anomaly (DVA) was by Pelz et al. [1] in 1983. To our knowledge, there appear to be only 20 total reported cases [1–16]. Although microvascular decompression (MVD) is the favored surgical treatment of refractory TN, surgical disruption of a DVA can lead to severe neurological deficits or even death due to venous infarction [16]. Gamma knife stereotactic radiosurgery (GKSR) is an established minimally invasive treatment option for TN and is especially suited for patients in whom the surgical risks are prohibitive. Herein, we describe the first reported case of a DVA-related TN successfully treated by GKSR to the trigeminal nerve.

Case Report A 24-year-old man with a 4-year history of left V2 and V3 distribution TN presented with several weeks of acute worsening of pain, which was medically refractory to treatment with carbamazepine, prednisone, diazepam, baclofen, and pregabalin. The patient described a component of constant pain, but the most bothersome feature was the bursts of pain caused by typical triggers such as face-washing, chewing, cold temperature, and wind. Neurological examination was otherwise normal. The patient

Edward A. Monaco III, MD, PhD Department of Neurological Surgery, Suite B-400 UPMC Presbyterian 200 Lothrop Street Pittsburgh, PA 15213 (USA) E-Mail monacoea2 @ upmc.edu

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a

 

Fig. 1. Axial contrast-enhanced T1-weighted MRI revealed a large DVA with characteristic caput medusae (a, arrowhead) coursing

through the left brachium pontis and a large draining vein exiting the brainstem adjacent to the root entry zone of the trigeminal

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c

nerve (b, arrow). Axial image from the gamma knife radiosurgery treatment plan showing the placement of the single 4-mm isocenter, with a prescription dose of 42.5 Gy at the 50% isodose line (c, inner circle).

TN, defined as sudden, usually unilateral, severe, brief, stabbing, recurrent episodes of pain in the distribution of one or more branches of the trigeminal nerve is a relatively uncommon pain syndrome with an incidence of 27 per 100,000 [18]. Modern treatment options begin with medical management in the form of carbamazepine or other anticonvulsants. Often, the patient’s pain becomes refractory to medication or there can be intolerable medication side effects. With the failure of medical management, surgical interventions such as MVD, glycerol rhizotomy and GKSR are often considered. MVD remains the gold standard surgical treatment of TN, with nearly two thirds of patients achieving and maintaining complete pain relief without the need for medication 10 years after the procedure [19]. However, MVD may not be appropriate for all patients, including those with multiple medical comorbidities precluding surgery, those on anticoagulation, patients with multiple sclerosis, and the elderly.

DVAs, often called venous angiomas, are relatively common vascular malformations. It was first proposed by Lasjaunias et al. [20] that DVAs are simply anatomical variants that drain normal cerebral tissue in the absence of the usual venous drainage systems. The clinical significance of DVAs is often uncertain but they have been cited as the etiology of a number of conditions, including headaches, ataxia, epilepsy, and hydrocephalus [4, 21, 22]. Despite such reports, the vast majority of DVAs are incidentally discovered, benign lesions that are not associated with a clinical condition [16]. Because DVAs serve as the venous outflow for a certain territory of brain tissue, disruption can be catastrophic, whether by spontaneous thrombosis or iatrogenically [16, 23]. Of the cases of TN caused by a DVA with sufficient treatment descriptions in the literature, 5 were treated conservatively, 1 underwent glycerol rhizotomy and 10 underwent MVD [2, 15]. Table 1 summarizes these cases. Of the 11 MVDs performed in the literature, 1 resulted in a death, with the patient dying from a massive cerebellar venous infarct due to disruption of the DVA [9]. In the cases where the DVA was ligated, the mortality rate was 1 in 4. This is compared to a 0.2% mortality rate for a large series of patients with a typical etiology [19]. Indeed, in the rhizotomy case, the operating surgeon avoided MVD for fear of disrupting the large brachium pontis DVA causing the patients symptoms and achieved complete pain relief in doing so [11]. GKSR is a minimally invasive, safe and effective treatment for TN and is particularly relevant for treating high-risk surgical patients such as those with TN due to a DVA. In a large case series of over 500 patients with idiopathic TN, 80% of patients had adequate pain relief following GKSR after 1 year [17]. Nearly 50% main-

Radiosurgery for Tic due to Venous Angioma

Stereotact Funct Neurosurg 2015;93:110–113 DOI: 10.1159/000369562

was admitted for immediate management with intravenous phenytoin, hydromorphone and fluid resuscitation. MRI revealed a large, left-sided DVA in the brachium pontis and cerebellar hemisphere exiting adjacent to the trigeminal nerve root entry zone (fig. 1a, b). He elected to undergo GKSR. He was treated per our usual technique [17]. The patient was administered a prescription dose of 42.5 Gy at the 50% isodose line, or a maximum dose of 85 Gy, via a single 4-mm isocenter to the left trigeminal nerve, with a treatment volume of 126.9 mm3 (fig. 1c). At 2 weeks postoperatively, the patient reported experiencing a 60% reduction in pain intensity and frequency and was able to taper off all opiates, diazepam, prednisone, and pregabalin. Over 5 years postoperatively, the patient has reported some mild sensory loss in the V2 and V3 regions but remains completely pain free on a reduced dose of carbamazepine.

Discussion

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a

Table 1. Treatment summary of available cases of TN caused by DVAs

Treatment

DVA location

Outcome

Authors

Conservative

Cerebellar hemisphere, posterior brachium pontis Cerebellar hemisphere, pons Brachium pontis Brachium pontis, posterior pons Brachium pontis

Partial pain relief Not reported Partial pain relief Partial pain relief Complete pain relief

Raveau et al. [12] Küker et al. [7] Peterson et al. [11] Peterson et al. [11] Acioly et al. [2]

Glycerol rhizotomy Brachium pontis

Complete pain relief

Peterson et al. [11] 1

Microvascular decompression

Brachium pontis Brachium pontis Not reported Brachium pontis Cerebellar hemisphere Brachium pontis Brachium pontis Brachium pontis Brachium pontis, posterior pons Pons, cerebellar hemisphere

Complete pain relief Complete pain relief1 Complete pain relief1 Complete pain relief, V3 hypoesthesia2 Dead2 Complete pain relief1 Complete pain relief1 Complete pain relief1 Complete pain relief1 Complete pain relief1

Pelz et al. [1] Martin et al. [8] Isu et al. [5] Trost et al. [14] Mori et al. [9] Nagata et al. [10] Korinth et al. [6] Peterson et al. [11] Peterson et al. [11] Yamamoto et al. [15]

Gamma knife

Brachium pontis, cerebellar hemisphere

Complete pain relief

Present report

repositioning. 2 Vein ligature.

tained this after 5 years. Importantly, there were no mortalities associated with GKSR, and the only meaningful morbidity was related to postprocedural sensory dysfunction. Interestingly, some degree of sensory dysfunction correlated to improved pain control. It is also noteworthy that GKSR can be repeated with a similar safety and efficacy profile in the setting of recurrent pain [24]. Specifically, patients for whom the initial GKSR was particularly effective are more likely to have a successful outcome after a repeat procedure. Similar results have been achieved in other large series from experienced centers [25, 26]. It is important to delineate that the target of GKSR in our case was the trigeminal nerve itself, as is done for idiopathic TN [17]. Thus, the dual goals of treatment are to lesion the nerve and preserve the DVA. Targeting of the DVA can lead to venous infarction and a high rate of radiation-related side effects (30%) and often does not completely obliterate the lesion [27]. The case presented herein is strikingly similar to the cases previously described in the literature. Patients with TN due to a DVA tend to be younger than those who suffer from idiopathic TN. Imaging shows the stereotypical caput medusae appearance of a DVA. All DVAs were located in the brachium pontis or nearby, with veins exiting adjacent to the trigeminal nerve. These cases reinforce the 112

Stereotact Funct Neurosurg 2015;93:110–113 DOI: 10.1159/000369562

importance of diagnostic imaging in order to identify rarer causes of TN like a DVA. The previously reported cases in the literature would have been excellent candidates for GKSR.

Conclusions

We propose that GKSR should be considered as an upfront treatment for patients who have failed medical therapy and suffer from TN related to a DVA. Although the efficacy of GKSR may decline over time, it can be repeated if a patient develops recurrent facial pain in this setting. It is safer and more effective than percutaneous procedures like glycerol rhizotomy, although it does not provide the same immediate relief [28]. After less invasive approaches have failed, MVD is an effective but relatively high-risk option in the setting of a DVA. When performing an MVD for TN due to a DVA, every effort should be made to preserve the DVA.

Disclosure Statement Dr. Lunsford is a consultant and shareholder of Elekta AB.

Harrison/Lunsford/Monaco III

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1 Vein

References

Radiosurgery for Tic due to Venous Angioma

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Gamma Knife Stereotactic Radiosurgery for Trigeminal Neuralgia Caused by a Developmental Venous Anomaly.

Background: Trigeminal neuralgia (TN) is mostly caused by vascular compression of the nerve's root entry zone due to an ectatic artery. Rarer causes i...
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