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Ning-Ning Dou, MD, Jun Zhong, MD, PhD, Ming-Xing Liu, MD, Lei Xia, MD, Hui Sun,
MD, PhD, Bin Li, MD, PhD, Shi-Ting Li, MD, PhD
PII: S1878-8750(15)01509-0
DOI: 10.1016/j.wneu.2015.10.091
Reference: WNEU 3386
Please cite this article as: Dou N-N, Zhong J, Liu M-X, Xia L, Sun H, Li B, Li S-T, Management of
bilateral hemifacial spasm with microvascular decompression, World Neurosurgery (2015), doi: 10.1016/
j.wneu.2015.10.091.
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Nerve Disease Center of Shanghai), Shanghai JiaoTong University School of
Medicine,
Shanghai 200092, China.
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Ming-Xing Liu, MD, Department of Neurosurgery, XinHua Hospital (The Cranial
Nerve Disease Center of Shanghai), Shanghai JiaoTong University School of
Medicine,
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Shanghai 200092, China.
Lei Xia, MD, Department of Neurosurgery, XinHua Hospital (The Cranial
Nerve Disease Center of Shanghai), Shanghai JiaoTong University School of
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Medicine,
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Shanghai 200092, China.
Hui Sun, MD, PhD. Department of Neurosurgery, XinHua Hospital (The Cranial
Nerve Disease Center of Shanghai), Shanghai JiaoTong University School of
Medicine,
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Medicine,
Shanghai 200092, China.
*Corresponding author Jun Zhong(ZhongMDPhD@sjtu.edu.cn)
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P.R.China
Telephone +8621-25078021
This study was supported by fundings from the Science & Technology Commission of
Shanghai Government (key project, #124119a0800) and National Nature Science
Foundation of China(#81471317).
ACCEPTED MANUSCRIPT
Management of bilateral hemifacial spasm with microvascular decompression
Ning-Ning Dou, Jun Zhong*, Ming-Xing Liu, Lei Xia, Hui Sun, Bin Li and
Shi-Ting Li
Abstract
Background Bilateral hemifacial spasm(BHFS) is very rare. Only about 3 dozen
clinical reports have been highlighted in the literature. Although microvascular
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decompression (MVD) is widely accepted as the effective therapy for hemifacial
spasm (HFS), the etiology and surgical treatment of BHFS is seldom addressed.
Hereby, we reported our experience with MVD for those BHFS patients.
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Methods Ten cases who suffered this disease were included. All patients underwent
one or two times MVD and were followed up for 5 to 92 months. The clinical data
were respectively analyzed. The etiology as well as treatment strategies were
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discussed.
Results All the 10 patients stopped spasm on the operative sides completely. The
symptom of the other side also stopped in three patients, improved in one case, did
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not get any improvement in 6 patients, five of them had another MVD on the
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contralateral side whinthin 1 year and became relieved, one patient refused the
surgery. The neuro-vascular confliction was found in all the operations. During
the follow-up period, neither complications of hearing loss, facial palsy nor the
recurrence was observed.
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Conclusion Vascular compression was also the reason of BHFS, and MVD could also
relieve the symptom, as a result, we recommend MVD for BHFS patients. Crowed
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cerebellopontine angle (CPA) and easy attrition of the neurovascular interfaces may
play important roles in the occurrence of BHFS patients. For some patients, one
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etiology;
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Introduction
Hemifacial spasm (HFS) is defined as unilateral, irregular clonic or tonic movement
of muscles innervated by the ipsilateral seventh cranial nerve (1, 6, 24, 30). Since
Dandy first proposed that cranial nerves compressed by ectatic vessels could cause
clinical syndromes in 1934(6), an increasing volume of evidences have been presented
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to support the neurovascular confliction theory (3, 10, 44). In 1970s, Jannetta (14-17)
published a series of papers regarding successful treatment of HFS with
microvascular decompression (MVD) surgery, i.e., transposition of the offending
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artery from the facial nerve root. MVD has become a highly effective and safe
treatment for HFS from then on (4, 11, 23, 25, 35, 39). Despite HFS and MVD have
been well known today, merely 3 dozen bilateral hemifacial spasm(BHFS) cases have
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been highlighted in the literature (table 1)(8, 9, 12, 13, 21, 22, 32, 38, 40, 41, 43).
BHFS often presents a diagnostic challenge for doctors and it may be erroneously
attributed to facial tic, oromandibular dystonia, and other facial dyskinesias.
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neurovascular confliction(9), multiple sclerosis(43), and Pagets disease(12) have all
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been suggested. As the etiology of BHFS remains controversial, most of the BHFS
patients have been treated with prescription drugs or botulinum toxin injection, which
may relive the symptoms in the early stage, but they could not cure the disease. It
is obviously different in the clinical characteristic, diagnosis and treatment between
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BHFS and HFS. We also find sometimes unilateral MVD could resolve bilateral
symptoms. In this paper, we will report our experience with MVD for those BHFS
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Methods
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Between April 2007 and December 2014, a total of 10 patients with BHFS were
treated with MVD in our department by the senior author (Dr. Zhong) (table 2).
These patients contained eight females and two males with mean age at onset of 49
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years old (ranging 965). The initial symptom began on the left side in 6 patients,
while right side in 4. About 3.8 years (ranging 19) later, the symptom occurred on
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the contralateral side. Typical symptoms of HFS on both sides were presented in 7
patients, while 3 patients presented with only eyelid twitches on the contralatral side.
All patients had been medicated before but in vain.
Surgery
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The operation was performed with a routine retrosigmoid approach. The dissection
started from the caudal cranial nerves. While the arachnoid membrane around the
nerves was being opened, the cerebellum was gradually raised until the
pontomedullary sulcus was visualized. The vascular relationship was carefully
studied to identify the vessel in contact with cranial nerve VII. After the offending
vessel was moved away from the nerve, small pieces of shredded Teflon sponge were
gently placed between the vessel and the nerve. Finally, the durra mater was sutured,
and a cranioplasty with titanium wire mesh was completed.
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Outcome evaluation and follow-up
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All the 10 patients were followed-up by telephone interview. The evaluation of
postoperative result was divided into three categories as follows: (1) excellent:
complete spasm relief without medication; (2) good: substantial relief but with
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minimal persistent twitch without medication or complete relief with well-tolerated
medication; (3) poor: little or no relief or severe twitch despite medication.
Follow-up and assessment of the results were carried out by Dr. Liu.
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Results
Preoperatively, 3D-TOF MRI scans delineate a crowded CPA space in all the
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posterior fossa. We could see that the volume of the posterior cranial fossa (VPCF)
is very small in all the patients (Figure 1). We find bilateral offending artery among
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Postoperatively, all the 10 patients stopped spasm on the operative sides completely.
The symptom of the other side also stopped in 3, improved in 1 immdeiately after the
surgery. Among the remaining 6 patients without improvement of the contralateral
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symptom, 5 underwent another MVD on the contralateral side within 1 year(Figure 2).
After the second operation, all the patients achieved a satisfactory result except the
one who denied the contralateral MVD (Figure 3) (table 2). During 5-92 months
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Discussion
Regardless of 0.6-5% prevalence of BHFS in HFS patients were reported (2, 26, 40),
only 3 dozen cases have been highlighted in the literature (table 1) (8, 9, 12, 13, 21,
22, 32, 38, 40, 41, 43). We noticed that non-synchronus is the main character of
BHFS which is manifested by the onset and symptom. Usually, BHFS begins in one
side and then extend to the other side.
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Although considerable evidences have demonstrated that the HFS is caused by
vascular compression of the facial nerve at its REZ (root exit zone)(31), the etiology
of BHFS is still controversial. As facial nerve demyelination in HFS had been
reported (20, 27-29, 33, 36, 37, 42), it is suspected BHFS might be caused by
demyelinated diseases which could cause bilateral facial nerve demyelination.
However, only one BHFS case associated with multiple sclerosis was reported in the
literature(43). Through our research, BHFS seems to be also caused by
neurovascular confliction (NVC). Then what interested us was what caused bilateral
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NVC? HFS is primarily a problem of middle age and older(18), in which the
offending vessels tend to be elongated, redundant. However, such age-related
changes of the arterial vasculature could not explain BHFS well for that it is absent in
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children and young patients like case 23and case 5. Although we have only 10
cases in the study, it could still give us some valuable clues. We believe two factors
may play important roles in the occurrence of BHFS patients. Crowed
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cerebellopontine angle (CPA) is one of them. It has been reported (5, 19, 34) the
CPA cistern of HFS patients is narrower compared with non-HFS patients. A
crowed CPA may increase the chance of NVC. From the preoperative MRI scans, a
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smaller gap between cerebellar and petrosal bone and crowed CPA could be seen
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among these BHFS patients. During the operation, the vascular decompression is
difficult in such a small operation space because of exposure of facial nerve and
offending artery. Easy attrition of the neurovascular interfaces somehow caused is
the other factor. Our recent study regarding the mechanism of HFS demonstrated
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that the sympathetic ends in the adventitia of the offending vessel may trigger an
ectopic action potential in these injured nerve fibers. We believe that the
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sympathetic nerves in the offending artery wall and the demyelinated nerve root in
close contact with each other. This theory could explain the phenomenon that the
attack is inclined to occur when the patient is emotional or nervous (7, 48). Here we
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think the BHFS has the same pathogenesis. But we still do not know what caused
the easy attrition of BHFS patients.
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Our study demonstrated that MVD is an effective treatment of BHFS, but why
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one-side MVD could solve the bilateral symptoms? We assumed that the
contralateral offending artery might be also transposed while the ipsilateral offending
artery was removed. As the cerebellar arteries were communicated by the basilar
artery, a little transposition of basilar artery might lead to a separation of
neurovascular contact. According to our theory, even a bit transposition may
separate the artery from the nerve, which could stop the neurotransmitter spreading
onto the nerve. It has also been reported that the abnormal muscle response(AMR)
could disappeared before the neurovascular conflict was discovered, because those
procedures, e.g. suction of cerebrospinal fluid or retraction of cerebellum, etc., may
cause the offending artery detached from contact with the facial nerve root(45, 47).
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For the patients with typical BHFS patients, the selection of first operation side
depends on the onset and severity of symptoms as well as MRI findings. We
recommend a second operation within one year if the contralateral symptom
unimproved(46).
Conclusion
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BHFS also seems to be caused by vascular compression. Two factors including
crowed CPA and easy attrition of the neurovascular interfaces may play an important
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role in the occurrence of BHFS. MVD is an effective remedy for the disease.
Sometimes, unilateral MVD could resolve bilateral symptom.
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Conflicts of interest: none.
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Acknowledgements:
This study was supported by fundings from the Science & Technology Commission of
Shanghai Government (key project, #124119a0800) and National Nature Science
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Legend
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Figure 1. Magnetic resonance imaging findings
Three-dimensional time-of-flight magnetic resonance imaging delineated a crowded
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cerebellopontine angle space in the posterior fossa.
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Figure 2. Intraporative findings ( Case 2 Right side)
a. On the way of the routine approach, with microscopy, The CPA was visualized
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small and crowded, and we started to dissect from the caudal cranial nerves (XI-X-IX),
then facial nerve was visualized compressed by AICA and many small vessels at the
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REZ; b.The AICA was moved; c. Small pieces of shredded Teflon (T) were
placed between the AICA and the nerve. VII: facial and vestibulocochlear nerve; IX:
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After the surgery, the ipisilateral symptom was relieved, but the contralateral spasm
did not improve, so we gave her another MVD on the right side. g.The dissection
was also started from the caudal cranial nerves (XI-X-IX), then facial nerve was
visualized compressed by AICA at the REZ. h. Small pieces of shredded Teflon (T)
were placed between the AICA and the nerve. After the surgery, the symptom on
the right side relieved. VII: facial nerve;: vestibulocochlear nerve; IX:
glossopharyngeus nerve; IX: glossopharyngeus nerve; AICA: anterior inferior
cerebellar artery; PICA: posterior inferior cerebellar artery
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Table 1 Summary of reported bilateral hemifacial spasm cases in the literature
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1990 Jordan[12] 1 / / / / /
1992 Vande[37] 1 L 1 Multiple M relieved
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sclerosis
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1994 Rosso[28] 1 L 6 L:AICA+PICA MVD relieved
; R:AICA
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1998 Schulze[32] 1 L / Vascular MVD Ipsilateral
compression relieved
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1999 Tan[35] 5 5L 100(mean) Vascular M+BTX, relieved
compression
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Findings ipsilateral Second-time
side
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Surgery
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R: E 8ysF 7ys; L: (-);
Carbamazepine
good
2 F /35y L: E 5ys; L: (+); R/AICA
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R:E10ysF 7ys, R: (+);
/
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MVD/1y/AICA,
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excellent
4 F/57y L: E 7ysF 5ys; L: (+); L/AICA excellent poor No
R:E 3ysF 2ys; R: (+);
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MVD/3m/AICA,
excellent
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MVD/1y/AICA
excellent
9 F/39y L:E 3ysF 2ys; L: (+); L/PICA excellent poor No
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R:E 1ys F R: (-);
MVD/9m/AICA
0.5ys;
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excellent
10 F/54y L:E4ys, F 3ys; L: (+); L/AICA excellent poor No
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R:E,0.25ys,F R: (+); MVD/5m/AICA
2ys; excellent
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R: E 8yF 7y: right Eyelid twitches for 8 years, facial muscles twitches for 7 years; (+) positive; (-) negative;
VAD: Vertebrobasilar Ateries Deviation; AICA: anterior inferior cerebellar artery; PICA: posterior inferior
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Highlights
1. Bilateral hemifacial spasm(BHFS) is very rare.
2. As the etiology of BHFS remains controversial, most of the BHFS patients have
been treated with prescription drugs or botulinum toxin injection.
3. Vascular compression was also the reason of BHFS, and MVD could also relieve
the symptom
4. Crowed cerebellopontine angle (CPA) and easy attrition of the neurovascular
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interfaces may play important roles in the occurrence of BHFS patients
5. For some patients, one MVD could solve bilateral symptoms.
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Abbreviations
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HFS -- hemifacial spasm
3D-TOF MRI -- three-dimensional time-of-flight
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magnetic resonance imaging scan
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NVC-- neurovascular confliction
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CPA -- cerebellopontine angle
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AMR -- abnormal muscle response
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