Essential blepharospasm and related dystonias

Essential blepharospasm is an idiopathic disorder of progressive involuntary spasms of the orbicularis oculi and upper facial (corrugator, procerus) muscles. Blepharospasm literally means spasm of the eyelids; however, most patients with blepharospasm also have or will develop squeezing in the lower face and neck muscles (Meige's syndrome, orofacial dystonia, or oromandibular dystonia). Some patients develop dystonic, uncontrolled movements in areas outside the facial nerve distribution (segmental cranial dystonia or craniocervical dystonia). Chronic, forceful squeezing by the periocular muscles becomes debilitating for the patient and leads to functional and cosmetic eyelid deformities. Treatment has included a variety of modalities and oral medications that are of limited efficacy. Botulinum-A toxin injections have delivered the best temporary relief from this disorder, while the periorbital myectomy operation has been shown to give the best long-term results.     

A型肉毒杆菌毒素联合眼睑手术治疗面肌痉挛

目的:A型肉毒杆菌毒素(Botox)联合眼睑手术治疗面肌痉挛(HFS)疗效观察。方法:回顾性分析患者的影像和医疗记录,并对注射Botox的患者治疗效果和并发症进行分析。结果:共纳入76例(女性58例)HFS患者至少接受4次Botox注射。平均随访时间为83±50(20~112)mo,平均注射16±10(4~34)次。发病高峰年龄为55~64a,平均发病年龄为66±11(32~85)a。多达23%的面肌痉挛患者存在与面神经关系密切的异常血管结构(右侧8例,左侧7例)(MRI=14,CT=1),其中以椎动脉累及最多(n=6),其次是小脑前下动脉(n=5)。原发性HFS患者的有效期间较继发性HFS患者短(2.5 vs 3.1mo,P<0.05),发病时间较继发性HFS患者长(4.1 vs 3.8d,P=0.739),SSAs较继发性HFS患者低(1.7 vs 1.9,P=0.179)。19例先前存在眼睑疾病的患者中有12例接受了手术矫正,包括上睑成形术(n=12)、肌肉切除(7例)、眉成形术(7例)和提上睑肌腱膜修复术(5例)。5例(41.7%)接受手术矫正和对肉毒杆菌素反应不佳的患者术后6mo症状改善(发病时间:P=0.0256,有效期间:P=0.374,SSAs:P=0.0161)。12例行眼睑手术患者术后并发症发生率低于未行眼睑手术患者(23%vs 42%,P≤0.05)。结论:Botox对于治疗HFS是一种安全有效的方法。继发性HFS患者使用肉毒杆菌毒素的治疗效果优于原发性HFS患者。对于并发眼睑疾病的患者而言,眼睑手术可提高患者满意度,降低并发症发生率,从而提高后续Botox注射的效果。     

Surgical management of essential blepharospasm.

We have reviewed the surgical management of essential blepharospasm over the last 15 years, comparing the results from facial nerve avulsion with those from orbicularis muscle stripping. After facial nerve avulsion 50% of patients remained free of troublesome spasm for 15 months after surgery, but only 25% remained so for more than two years. Following orbicularis oculi myectomy 50% of patients were free of troublesome spasms for 30 months after surgery and 55% of patients had relief from spasm for more than two years. Secondary effects of the two procedures are compared and are found to be fewer after orbicularis myectomy. There were no major complications after either form of surgery. Botulinum toxin is the treatment of first choice for this condition. If this becomes ineffective or inconvenient, surgical treatment is warranted and should not be deferred for fear of severe side effects of treatment, since these are rare. Protractor myectomy gives longer relief from blepharospasm than facial nerve avulsion and has fewer complications. However, it is technically difficult, time consuming, and has greater peroperative morbidity. Facial nerve avulsion may therefore still have a role in selected patients.     

Raising the suborbicularis oculi fat (SOOF): its role in chronic facial palsy

AIMS: To determine the adjuvant role of unilateral suborbicularis oculi fat (SOOF) lift in the periorbital rehabilitation of patients with chronic facial palsy. METHODS: In a non-comparative prospective case series nine adult patients (seven male, two female) aged 34-90 years (mean 60.5) with chronic unrecovered facial palsy (over 1 year), who had not had any previous rehabilitative periorbital surgery, were studied. Lateral tarsal strip and adjuvant transconjunctival approach subperiosteal SOOF lift under local or general anaesthesia were performed; medial canthoplasty was performed where indicated. There was clinical observation of the long term (over 1 year) effect on the ptotic palpebral-malar sulcus and lower eyelid retraction. RESULTS: The patients were followed up for 12-24 months (mean 16). Seven patients (77%) had sustained clinical reduction of palpebral-malar sulcus ptosis. All patients had sustained reduction of lagophthalmos. Early postoperative complications included conjunctival cheimosis in 77%. Three patients with persistent keratitis required further surgical procedures on their upper eyelid to reduce the palpebral aperture. There were no cases of infraorbital nerve anaesthesia or recurrent lower eyelid retraction. CONCLUSIONS: The SOOF lift has an adjuvant role in chronic facial palsy with lower eyelid retraction and ptotic-palpebral malar sulcus. It supports the lower eyelid elevation and tightening achieved with the lateral tarsal strip. The best results were obtained in congenital facial palsy.     

全眶周闭眼肌群切除术治疗特发性眼睑痉挛临床分析

目的 探讨全眶周闭眼肌群切除术治疗特发性眼睑痉挛的有效性及安全性。方法 采用全眶周闭眼肌群广泛切除治疗特发性眼睑痉挛,以特发性眼睑痉挛临床分级(EBCG)评价患者眼睑痉挛的严重程度,并以眼睑痉挛残疾指数 (BSDI)作为患者日常生活和工作情况的自评依据。回顾分析2015年7月~2019年11月在我院行全眶周闭眼肌群切除术的18例特发性眼睑痉挛患者术后眼睑痉挛严重程度和日常生活及工作改善程度,并观察术后并发症。结果 在随访期内,所有术眼(36眼)术后EBCG均较术前有不同程度降低,其中完全缓解11眼(30.56%)、明显缓解15眼(41.67%)、部分缓解10眼(27.78%),总有效率达100%。BSDI评分则从术前的(2.73±2.51)分降至(0.24±0.26)分,较术前明显降低(P<0.05)。术后3个月内,所有病例均有不同程度的前额部麻木,3个月后前额部麻木开始逐步减轻,于术后10个月左右基本消退。所有术眼术后早期均有不同程度的眼睑水肿,并于术后半年开始逐步消退,5眼长期存在不同程度的眼睑水肿。眼睑闭合不全3眼、下睑外翻4眼。未见复发病例。结论 全眶周闭眼肌群切除术可以安全、有效治疗特发性眼睑痉挛。     

Orbicularis oculi muscle graft augmentation after protractor myectomy in blepharospasm

PURPOSE: To evaluate the effectiveness of free orbicularis oculi muscle grafts in correcting volume deficit deformities after protractor myectomy in patients with essential blepharospasm. METHODS: Prospective case series. During the 13-month period from October 2000 through November 2001, all patients with essential blepharospasm undergoing primary eyelid protractor myectomy received an orbicularis oculi muscle graft to replace the volume deficit deformity created by the myectomy. Only patients who had at least 6 months of postoperative follow-up were included in the analysis. RESULTS: Forty-six patients underwent primary eyelid protractor myectomy and had a free orbicularis oculi muscle graft for volume replacement. All patients had significant functional improvement of their eyelid spasms after the myectomy. Of the 38 patients who underwent upper eyelid myectomy, 3 patients were overcorrected and no patients were undercorrected with the orbicularis muscle graft. Two of the overcorrected patients underwent surgical debulking of their muscle grafts. Of the 8 patients who underwent lower eyelid myectomy, no patients were overcorrected and 1 patient was undercorrected. None of the patients were observed to have any spasms, contractions, or other signs of muscular activity or aberrant innervation of the muscle graft.CONCLUSIONS: The orbicularis oculi muscle graft is a useful adjunct to protractor myectomy in improving the aesthetic outcomes for blepharospasm patients. Our study demonstrates the viability of the orbicularis oculi muscle graft and may lead to future applications of the graft in facial aesthetics.     

肉毒杆菌毒素A治疗眼睑及面肌痉挛远期疗效观察

目的 探讨肉毒杆菌毒素Ａ（ｂｏｔｕｌｉｎｕｍ Ａ ｔｏｘｉｎ，ＢＴＸＡ）治疗眼睑及面部肌肉痉挛的远期疗效。方法 对９２例眼睑及面部肌肉痉挛患者用ＢＴＸＡ注射治疗，随诊观察其远期疗效。结果 ＢＴＸＡ注射后１～４ｄ痉挛逐渐缓解，维持１６ｗｋ左右后症状复发。复发多由眼睑痉挛开始，面部痉挛缓解时间长，可达２４ｗｋ。注射后可见表情麻木、上睑下垂、睑裂闭合不全等并发症，但均较轻，且２ｗｉ后多能自行恢复。结论     

Differential section of the seventh nerve as a tertiary procedure for the treatment of benign essential blepharospasm

PURPOSE: To examine the efficacy of differential section of the seventh nerve in treatment of patients with blepharospasm refractory to botulinum toxin and eyelid protractor myectomy. METHODS: A retrospective noncomparative interventional case series consisting of a cohort of 228 patients with benign essential blepharospasm followed from 1987 to 1997 in a university ophthalmic plastic surgery referral practice. Patients were treated with botulinum toxin injections, eyelid protractor myectomy, and differential section of the seventh nerve in stepwise fashion as needed for symptomatic control. RESULTS: Thirty-four patients (15% of total) underwent eyelid protractor myectomy during this period. Eyelid protractor myectomy failed to control blepharospasm in 7 (21%) of these 34 patients, who then underwent differential section of the seventh nerve an average of 2 years after myectomy. Patients were followed up for an average of 36 months, with a success rate of 42% (3 of 7). The remaining 4 patients had repeat differential section of the seventh nerve with a 50% success rate, which brought the overall success rate from differential section of the seventh nerve to 71%. Lower eyelid ectropion requiring surgical repair complicated 27% of differential section of the seventh nerve procedures. CONCLUSIONS: Differential section of the seventh nerve is a reasonable alternative in the treatment of patients who have persistent disability despite treatment with botulinum toxin injections and eyelid protractor myectomy.     

Facial nerve injury and hemifacial spasm

We studied evidence of facial nerve damage in patients with hemifacial spasm. Three types of evidence of nerve damage were analyzed: objectively measured weakness in eyelid protractor strength, clinically evident weakness of muscles innervated by the seventh nerve, and clinically evident aberrant seventh nerve regeneration. Of the 60 patients in the study, 54 (90%) had at least one of these features of seventh nerve damage. Objectively measured eyelid protractor weakness was noted in 27 of 58 patients (47%) who were tested. Clinically apparent weakness of at least one of four facial muscle groups was noted in 42 of 60 patients (70%). Aberrant seventh nerve regeneration was documented in 25 of 60 patients (42%). These findings indicate that facial nerve damage is common in patients with hemifacial spasm.     

Review and update of involuntary facial movement disorders presenting in the ophthalmological setting

We review the existing literature on the involuntary facial movement disorders—benign essential blepharospasm, apraxia of eyelid opening, hemifacial spasm, and aberrant facial nerve regeneration. The etiology of idiopathic blepharospasm, a disorder of the central nervous system, and hemifacial spasm, a condition involving the facial nerve of the peripheral nervous system, is markedly different. We discuss established methods of managing patients and highlight new approaches.     

Treatment of hemifacial spasm with botulinum A toxin. Results and rationale

Hemifacial spasm is characterized by unilateral, periodic, tonic contractions of facial muscles, thought to be caused by mechanical compression at the root-exit zone of the facial nerve. Electrophysiologic abnormalities such as ectopic excitation and synkinesis are typical. Although posterior fossa microsurgical nerve decompression is successful in bringing about relief of the spasm in most cases, it carries a risk to hearing. As an alternative treatment, 15 patients with hemifacial spasm were given a total of 41 sets of injections with botulinum A toxin, with a mean follow-up of 14.3 +/- 1.1 months. Relief of symptoms lasted a mean of 108.3 +/- 4.2 days. Mild transient lagophthalmos and ptosis were the only complications. Although the exact mechanism of its action and beneficial effect is speculative at this time, botulinum A toxin appears to offer an effective, safe alternative to more radical intracranial surgery for patients with hemifacial spasm.     

Hemifacial Spasm Due to Intracranial Tumor: An International Survey of Botulinum Toxin Investigators

Hemifacial spasm (HFS) due to intracranial mass lesions is rare. Most cases are thought to be due to compression of the facial nerve by small vessels near the root of the facial nerve. A survey was undertaken of all botulinum toxin investigators to determine the incidence of imaged mass lesions causing HFS. Responders contributed information on 1676 patients with HFS. Of this group, nine tumors were reported for an incidence of 0.54% of patients. However, of this group only 52.5% underwent computed tomography (CT) or magnetic resonance (MR) scanning so the incidence of tumor causing HFS could be as high as 1.0%. No one tumor type was predominant, and most patients were women older than 50 years of age. The incidence compares with another large series of HFS patients in which one tumor was found in 367 patients. The authors also report as an illustrative case a 26-year-old man with HFS due to a presumed lipoma of the cerebellopontine angle. This diagnosis can be made with increased certainty with MR scanning. If the incidence of unsuspected diagnostically significant mass lesions is 1 in 200 patients with HFS referred for botulinum toxin injection, the cost of detecting one such lesion would be $100,000 at an average imaging cost of $500 per MR imaging or CT examination. Although mass lesions are uncommon, any patient with HFS whose general clinical course could justify intervention should be considered for imaging studies to rule out treatable conditions other than vascular compression.     

Facial Dystonias and Rosacea: Is There an Association?

Abstract

Purpose: Benign essential blepharospasm (BEB) and hemifacial spasm (HFS) belong to a spectrum of focal movement disorders that cause involuntary, spasmodic contractions of the eyelid and facial muscles. In our clinical experience, we have observed an increased prevalence of rosacea in patients who present with BEB and HFS. We investigate our clinical findings with a review of disease pathophysiology and treatment.

Methods: Retrospective study approved by the Ochsner Institutional Review Board and literature review. A total of 140 charts dated from 1990 to 2013 were reviewed, including 87 patients with BEB and 53 patients with HFS. Rosacea, BEB, and HFS were defined by standard diagnostic criteria.

Results: Within our BEB and HFS patient cohort, approximately 15% of patients presented with rosacea, compared to the general American population prevalence rate of 1.34% (p?<?0.001). Of the 140 patients reviewed, a total of 21 patients (13 with BEB and 8 with HFS) exhibited rosacea (p?=?0.995).

Conclusions: Dry eye and tear instability often co-exist in patients with facial dystonias and rosacea, which may provide the initial drive towards tonic eyelid contractions and simultaneously exacerbate rosacea. Studies suggest that neurogenic inflammation and altered vasoregulation jointly contribute to the pathogenesis of rosacea. From our preliminary observations, we suggest the possibility of shared immune-inflammatory pathways involved in both facial dystonias and rosacea. Identification of common inflammatory mediators involved in both disease processes may facilitate a more targeted approach in drug treatment. Further biochemical analysis will likely be necessary to elucidate this potential association.     

Upper Eyelid Retraction After Periorbital Trauma

We report four unusual cases of upper eyelid retraction following periorbital trauma. Four previously healthy patients were evaluated for unilateral upper eyelid retraction following periorbital trauma. A 31-year-old man (Case 1) and a 24-year-old man (Case 2) presented with left upper eyelid retraction which developed after blow-out fractures, a 44-year-old woman (Case 3) presented with left upper eyelid retraction secondary to a periorbital contusion that occurred one week prior, and a 56-year-old man (Case 4) presented with left upper eyelid retraction that developed 1 month after a lower canalicular laceration was sustained during a traffic accident. The authors performed a thyroid function test and orbital computed tomography (CT) in all cases. Thyroid function was normal in all patients, CT showed an adhesion of the superior rectus muscle and superior oblique muscle in the first case and diffuse thickening of the superior rectus muscle and levator complex in the third case. CT showed no specific findings in the second or fourth cases. Upper eyelid retraction due to superior complex adhesion can be considered one of the complications of periorbital trauma.     

Graded full-thickness anterior blepharotomy for correction of upper eyelid retraction not associated with thyroid eye disease

PURPOSE: To evaluate the efficacy of graded full-thickness anterior blepharotomy for upper eyelid retraction of various causes not associated with Graves eye disease. METHODS: Twenty-one eyelids of 18 patients with upper eyelid retraction not caused by Graves eye disease were treated with graded full-thickness anterior blepharotomy. Preoperative and postoperative symptoms, midpupil to upper eyelid distance, lagophthalmos, and superficial punctuate keratopathy were evaluated. RESULTS: Upper eyelid retraction was due to facial nerve palsy in 4 patients (22%), overcorrected ptosis in 5 patients (28%), and cicatrix after trauma in 6 patients (33%). One patient each (6% each) had retraction from graft-versus-host disease, after blepharoplasty, and after orbicularis oculi myectomy for blepharospasm. At a mean of 10 months follow-up, presenting symptoms resolved or improved in 17 patients (94%) and remained unchanged in 1 patient (6%). Midpupil to upper eyelid distance, lagophthalmos, and superficial punctuate keratopathy all improved significantly (all p < 0.001). No surgical complications occurred. CONCLUSIONS: Graded full-thickness anterior blepharotomy is a safe, effective, and rapid technique for patients with symptomatic upper eyelid retraction due to etiologies other than Graves eye disease. This technique improves symptoms and signs of ocular exposure while addressing relative upper eyelid height symmetry and contour.     

Asymmetry of blinking

PURPOSE: Too investigate asymmetry in eyelid movements with blinking, the stability of the asymmetry, and its modifiability in normal humans. METHODS: Differences in the start time and amplitude between the two eyelids were assessed for voluntary blinks and reflex blinks evoked by supraorbital trigeminal nerve stimulation. These variables were also measured before and up to 18 months after 2 hours of unilateral upper lid restraint. RESULTS: With voluntary blinks, one eyelid consistently began to close earlier and made a larger eyelid movement than the other eyelid. Stimulation of the supraorbital branch of the trigeminal nerve evoked relatively larger amplitude blinks in one eyelid that correlated with the asymmetries of voluntary blinks. There was a continuum of eyelid asymmetry across all subjects that was stable and independent of other biological asymmetries, such as handedness. Briefly reducing eyelid mobility created a long-lasting change in eyelid asymmetry with blinking. CONCLUSIONS: Eyelid asymmetry results from differences in the excitability of motoneurons in the left and right facial motor nuclei and does not appear to involve asymmetries in cortical inputs to the brain stem. Because adaptive processes modify the motoneuron excitability that creates eyelid asymmetry, these processes may underlie changes in blinking associated with facial palsy and may play a role in the development of disorders that affect one side of the face, such as hemifacial spasm.     

Supportive care of facial nerve palsy with temporary external eyelid weights.

BACKGROUND: Patients with facial nerve palsy can have many ocular complications. Lagophthalmos, or poor eyelid closure, and loss of blink secondary to lack of nerve supply to the orbicularis oculi, can lead to exposure keratopathy, corneal breakdown, ulcers, and even perforation. Management of patients should be directed toward the severity of ocular findings and ranges from supportive care to surgical reanimation and soft tissue repositioning. Patients with facial nerve palsy who present at earlier stages can benefit from conservative treatment. Use of temporary external eyelid weights can help restore a functional blink mechanism and prevent corneal decompensation. CASE REPORT: A 29-year-old patient with lagophthalmos secondary to left facial nerve palsy after surgical excision of an acoustic neuroma was treated with a temporary external eyelid weight. CONCLUSION: Temporary external eyelid weights are part of the armamentarium in the supportive care of patients with lagophthalmos and exposure keratopathy secondary to facial nerve palsy. They are most useful in patients who have temporary paralysis or as a bridge until further surgery can be performed.     

Eyelid reanimation for the treatment of paralytic lagophthalmos: historical perspectives and current applications of the gold weight implant.

Gold weight implants have been used for over 30 years in the setting of eyelid rehabilitation following facial nerve paralysis; however, there has been a renewed interest by ophthalmologists in this reanimation technique in recent years. This article reviews the history of gold weight eyelid implantation and presents the results of gold weight eyelid implantation over a 15-month period in 23 patients. A 92% success rate was obtained (average follow-up, 12 months). Surgical technique and indications are discussed along with postoperative complications.     

重度特发性睑痉挛的手术治疗

目的 探讨重度特发性睑痉挛患者施行不同手术方法的临床效果和安全性.方法 选取 重度特发性睑痉挛20例40只眼,均为双眼发病,根据患者不同的临床表现,分别采取三种术式之一:次全肌切除术、全肌切除术、Anderson手术法.术后随访1年至1年半.结果 20例患者,均明显减轻睑痉挛的程度,所有病例眉下垂、上睑下垂、睑裂横径缩小、睑皮松弛等现象均减轻或消失.双眼睑外观基本对称,睑裂开闭自如,上睑缘位于上角巩缘与上瞳孔缘之间,功能性视力障碍消失.结论 手术治疗重度特发性睑痉挛是安全有效的方法.     

重度特发性睑痉挛的手术治疗

目的 探讨重度特发性睑痉挛患者施行不同手术方法的临床效果和安全性.方法 选取 重度特发性睑痉挛20例40只眼,均为双眼发病,根据患者不同的临床表现,分别采取三种术式之一:次全肌切除术、全肌切除术、Anderson手术法.术后随访1年至1年半.结果 20例患者,均明显减轻睑痉挛的程度,所有病例眉下垂、上睑下垂、睑裂横径缩小、睑皮松弛等现象均减轻或消失.双眼睑外观基本对称,睑裂开闭自如,上睑缘位于上角巩缘与上瞳孔缘之间,功能性视力障碍消失.结论 手术治疗重度特发性睑痉挛是安全有效的方法.