The long-term response to botulinum toxin injections in patients with blepharospasm undergoing upper eyelid surgery.

BACKGROUND: Upper eyelid surgery (UES) is a therapeutical strategy used for those patients affected by blepharospasm (BSP) who either do not respond or experience a gradual decrease in responsiveness to botulinum toxin (BoNT) injections. Nevertheless, most of them need to restart with BoNT despite the intervention. AIM: To evaluate the long-term post-surgical response to BoNT in patients with BSP and to identify predictive factors associated to treatment outcome. METHODS: We collected data of 60 BS patients, divided into two groups - blepharoplasty YES (8) and NO (52), collecting demographic - age, sex - and clinical data -disease duration, duration of the treatment with BoNT. Respective responses to injections - evaluated through the differences of both Jancovic Rating Scale and the Blepharospasm Disability Index pre and post BoNT (delta JRS and delta BSDI) just before their periodic three-month injection and after 1 month from it - were compared. Finally, clinical and demographics variables were included in multivariate regression and correlation analyses to assess their impact on the long-term response to injections. RESULTS: Patients who underwent UES had significantly lower delta at both scales, showing a poorer outcome after BoNT treatment. No variable was found to be associated with the response. DISCUSSION: Our data seem to suggest that surgery does not improve response to BoNT injections on the long run. As such, UES could be considered as an efficacious treatment in BSP just if evaluated soon after its performing. Long-term BSP management seems still difficult to be performed adequately and new therapeutical approaches are still needed.

Extended frontalis orbicularis oculi muscle flap shortening for treating refractory apraxia of eyelid opening associated with blepharospasm.

BACKGROUND: Refractory apraxia of eyelid opening (AEO) is mostly unresponsive to botulinum toxin (BTx) and inevitably leads to functional blindness. To treat this challenging condition, an innovative surgical technique was proposed. METHODS: The extended frontalis orbicularis oculi muscle (FOOM) flap shortening consisting of frontalis suspension, partial myectomy, and myotomy in situ of eyelid protractors was applied to treat refractory AEO associated with blepharospasm. The postoperative outcomes and patient satisfaction were evaluated. RESULTS: Seven patients (mean ages 64.1 ± 3.9 years) of 14 eyelids in total had an average flap shortening distance of 24.4 ± 1.3 mm. During a mean follow-up of 31.6 ± 11.4 months, the average BTx dosage reduced from 58.6 ± 12. 1 units to 30.0 ± 8.2 units, with a mean injection interval decreasing from 2.3 ± 0.5 months to 4.1 ± 0.9 months (p < 0.05). Palpebral fissure height increased from 1.4 ± 0.5 mm to 7.9 ± 0.7 mm, and the disability scale decreased from 78.8% ± 7.2% to 12.6% ± 7.0% (p < 0.05). The postoperative BTx dosage and frequency were significantly reduced. All patients restored voluntary eyelid opening and reported high postoperative satisfaction (average Likert scale 4.6 ± 0.5). CONCLUSION: Extended FOOM flap shortening is an effective treatment to solve refractory AEO associated with blepharospasm.

[Efficacy of CT-guided partial radiofrequency ablation of bilateral responsible cranial nerves in the treatment of Meige syndrome].

Objective: To evaluate the efficacy of CT-guided partial radiofrequency ablation of bilateral responsible cranial nerves in the treatment of Meige syndrome. Methods: The Clinical data of 56 patients with Meige syndrome in the Department of Pain Medicine, Affiliated Hospital of Jiaxing University from June 2019 to January 2023 were retrospectively analyzed [19 males and 37 females, aged 42-76 (58.6±8.3) years], including 51 cases of blepharospasm, 3 cases of oromandibular dystonia and 2 cases of blepharospasm concomitant with oromandibular dystonia. CT-guided partial radiofrequency ablation of bilateral responsible cranial nerves was performed on different types of Meige syndrome. And the efficacy and complications of the technique were observed. Results: Fifty-one patients with blepharospasm Meige syndrome underwent CT-guided radiofrequency of facial nerve through bilateral stylomastoid foramen punctures, the symptoms of blepharospasm disappeared completely, leaving bilateral mild and moderate facial paralysis symptoms. Three patients with oral-mandibular dystonia underwent CT-guided radiofrequency therapy by bilateral foramen ovale puncture of mandibular branches of trigeminal nerve, masticatory muscle spasm disappeared, the patients had no difficulty opening the mouth, and the skin numbness in bilateral mandibular nerve innervation area was left. Two cases of Meige syndrome with blepharospasm concomitant with oromandibular dystonia were treated by radiofrequency of facial nerve and mandibular branch of trigeminal nerve, and all symptoms disappeared. The patients were followed up for 1-44 months after the operation, and the symptoms of mild and moderate facial paralysis disappeared at (3.2±0.8) months after the operation, but the numbness did not disappear. Three patients with blepharospasm recurred at the 14, 18 and 22 months after the operation, respectively, while the rest cases did not recur. Conclusions: According to different types of Meige syndrome, CT-guided partial radiofrequency ablation of responsible cranial nerves can effectively treat the corresponding type of Meige syndrome. The complications are only mild and moderate facial paralysis which can be recovered, and/or skin numbness in the mandibular region.

Evaluation of Blepharoptosis in Patients With Refractory Blepharospasm by VISA-Video Recordings, Idiosyncratic Expressions, Sensory Tricks, and Ancillary Procedures.

BACKGROUND: How to evaluate blepharoptosis concomitantly presented with refractory and uncontrollable blepharospasm? To date, there is a paucity of publications on the ideal evaluation methods. An innovative method-video recordings, idiosyncratic facial expressions, sensory tricks, and ancillary procedures (VISA)-is developed for preoperative evaluation, and the surgical outcomes are demonstrated. METHODS: A retrospective study using VISA for blepharoptosis evaluation was conducted on 51 patients with refractory blepharospasm. Based on the evaluation, patients underwent blepharoptosis correction simultaneously besides the selective myectomy and myotomy in situ of the eyelid protractors for blepharospasm. Preoperative and postoperative palpebral fissure height, margin reflex distance 1, ptosis severity, and levator function were assessed to identify the effectiveness of VISA. All the procedures were performed by the senior author C.-S.L. RESULTS: There were 42 patients diagnosed with essential blepharospasm and 9 patients with Meige syndrome. Forty-one patients (82/102 eyelids [80.4%]) had concomitant blepharoptosis and blepharospasm. Ptosis severity was mild in 21 eyelids (25.6%), moderate in 12 eyelids (14.6%), and severe in 49 eyelids (59.8%). Preoperative/postoperative (6 months) values of palpebral fissure height, margin reflex distance 1, and levator function were 4.70 ± 2.45 mm/8.35 ± 1.33 mm (P < 0.05), -0.30 ± 3.19 mm/3.73 ± 1.05 mm (P < 0.05), and 13.07 ± 2.56 mm/13.68 ± 2.34 mm (P < 0.05), respectively. Undercorrection and revision rate reported 9.8% and 3.7%, individually. CONCLUSIONS: VISA approach overcomes the difficulty of blepharoptosis assessment in patients with refractory blepharospasm. It provides useful preoperative information required for adequate blepharoptosis correction in blepharospasm surgery and yielded desirable outcomes.

Selective Myectomy and Myotomy In Situ for the Management of Refractory Blepharospasm in Meige Syndrome.

BACKGROUND: Meige syndrome is characterized by involuntary blepharospasm and varied subphenotypes of oromandibular tonic-clonic muscle contraction. Despite botulinum toxin (BTx) being the mainstay of treatment for Meige syndrome, a small subset of patients remain refractory to its effects because the disease is a form of functional blindness. An integrative surgical procedure combining selective myectomy and myotomy in situ of eyelid protractors, blepharoptosis correction, and tightening of the lower eyelid laxity was first applied to treat refractory blepharospasm in patients with Meige syndrome. MATERIALS AND METHODS: This study is a retrospective review conducted on 24 patients with refractory Meige syndrome between 2013 and 2020. Besides selective myectomy and myotomy in situ of eyelid protractors, levator plication and lateral tarsoplasty or canthopexy was performed for blepharoptosis correction and lower eyelid tightening, respectively. Patient demographics, associated diseases, medical treatment history, associated surgical procedures, final aesthetic outcomes, and therapeutic effects as reflected by changes in function disability score and Botox (BTx) treatment were thoroughly recorded and analyzed. RESULTS: The mean age of the patients was 65.2 ± 8.9 years. Twenty-one patients (87.5%) received blepharoptosis correction by levator plication with an average of 11.2 ± 2.9 mm in length. Lateral tarsoplasty was performed in 16 patients (66.7%) by pentagonal tarsal resection with an average of 3.9 ± 0.8 mm in width. Five patients (20.8%) received lateral canthopexy. Among the total of 96 operated eyelids, scar revision with fat graft was performed in 3 eyelids (3.1%). The average amount of BTx treatment decreased from 49.2 ± 12.8 U once every 2.7 ± 0.8 months before surgery to 35.4 ± 7.8 U once every 3.8 ± 0.7 months after surgery. Function disability score improved from 76.7 ± 17.5% preoperatively to 15.6 ± 9.9% postoperatively ( P < 0.001). Only 3 upper eyelids (3.1%) received scar revision and fat grafting due to minor postoperative contour depression. All patients expressed high satisfaction with both functional and aesthetic outcomes (Likert scale 4.5 ± 0.5). CONCLUSIONS: Selective myectomy and myotomy in situ of eyelid protractors combining blepharoplasty correction and lower eyelid tightening can produce long-lasting functional and aesthetic results with minimal complication in patients with refractory Meige syndrome.

Disabling muscle of Riolan: A novel concept of orbicularis oculi myectomy for refractory benign essential blepharospasm.

PURPOSE: To examine the effectiveness of orbicularis oculi myectomy with disabling the muscle of Riolan in patients with benign essential blepharospasm refractory to botulinum toxin-A (BTX-A) injection. METHODS: This retrospective, observational study included 25 patients. After removal of the redundant skin and underlying orbicularis oculi muscle (OOM) with or without extended OOM removal to the area of the superior orbital rim, the tarsal plate and the gray line were vertically severed at 2 points to disable the muscle of Riolan. The surgical effectiveness was evaluated using the visual analogue scale (VAS), functional disability score (FDS), and the presence or absence of necessity or enhanced effectiveness of BTX-A injection after surgery. RESULTS: The symptoms improved in 23 patients (92.0%). The VAS and total FDS were significantly improved from 8.4 ± 1.7 to 4.0 ± 2.4 (reduction rate, 50.7 ± 35.6%) and from 74.6 ± 22.2 to 34.7 ± 25.3 (reduction rate, 53.4 ± 27.4%) after surgery, respectively (both, p < 0.001). Among the 23 patients whose symptoms improved after surgery, BTX-A injection was not required in 11 of them (47.8%). Among the remaining 12 patients (52.2%), the effectiveness of BTX-A was post-operatively enhanced in eight patients (34.8%). There were no serious complications, and none of the patients experienced madarosis. CONCLUSION: Disabling the muscle of Riolan is a valuable option of OOM myectomy in patients with refractory benign essential blepharospasm, without the development of serious complications, including madarosis.

[Tarsal Kink: Pathognomonic Presentation and Therapy of a Rare Congenital Upper Eyelid Malformation]. / Tarsusknickung ("Tarsal Kink"): Pathognomonische Präsentation und Therapie einer seltenen kongenitalen Oberlidfehlbildung.

BACKGROUND: Presentation of a congenital abnormality that is rare, but follows a distinct course and can be diagnosed and cured promptly if the pathognomonic presentation is recognized. A congenital tarsal kink leads to a malposition of the upper eyelid margin that must not be missed, as it will lead to ulcerative keratitis if it is not treated. CASE PRESENTATION: An otherwise healthy newborn was presented after delivery with forceps with marked unilateral purulent secretion and blepharospasm. DIFFERENTIAL DIAGNOSIS: Neonatal dacryocystitis, gonococcal infection, congenital entropion with ulcerative keratitis, tarsal kink. EXAMINATION: It was not possible to fully examine the lid and cornea with the baby awake. Due to total inversion of the lid margin, no lashes could be seen. Under general anesthesia, the tarsal kink, with complete inversion of the lid margin and a corneal ulcer, was confirmed. TREATMENT: The literature offers several methods to correct this rare malposition, all of which aim to strengthen the anterior lamella to correct the kink. After incision of the kink and repositioning of the tarsus and securing the position with fixation sutures, the ulcer healed quickly and completely; lid closure and lid contour were normal and symmetrical. SUMMARY: Complete inversion of the lid margin is the pathognomonic sign of tarsal kink, giving the impression of "missing" lashes, accompanied by blepharospasm, followed by purulent secretion and corneal ulceration. The condition must not be misdiagnosed as only immediate correction can prevent severe damage.

An Aesthetic Orbicularis Oculi Myectomy Technique for Blepharospasm: Use of Topical 5-Fluorouracil and Fibrin Sealant.

PURPOSE: To describe a modified technique of orbicularis oculi myectomy for refractory blepharospasm. This technique includes removal of orbicularis muscle, reformation of the eyelid crease and pretarsal platform using fibrin sealant (Tisseel), and topical 5-fluorouracil to reduce scar formation and improve aesthetic outcome. METHODS: Retrospective chart review of 7 patients who underwent bilateral orbicularis oculi myectomy with our technique from 2013 to 2016. Outcome measures were postoperative botulinum toxin dose, frequency, duration between treatments, the amount of lagophthalmos, severity of dry eye, and patient satisfaction with aesthetic and functional outcome. RESULTS: Patients who underwent the aesthetic myectomy technique had significantly decreased botulinum toxin use with relief of symptoms postoperatively. Only 1 of 7 patients experienced mild dry eye symptoms postoperatively, managed with artificial tears. All patients were satisfied with the aesthetic and functional outcome. CONCLUSIONS: The aesthetic myectomy technique provides effective treatment for blepharospasm with good functional and aesthetic outcome.

Surgical approach to limiting skin contracture following protractor myectomy for essential blepharospasm.

PURPOSE: To report our experience with protractor myectomy in patients with benign essential blepharospasm who did not respond to serial botulinum toxin injection, and to describe intra- and postoperative techniques that limited skin contracture while also providing excellent functional and cosmetic results. METHODS: The medical records of patients with isolated, benign, essential blepharospasm who underwent protractor myectomy from 2005 to 2008 by a single surgeon were reviewed retrospectively. The technique entailed operating on a single eyelid during each procedure, using a complete en bloc resection of all orbicularis tissue, leaving all eyelid skin intact at the time of surgery, and placing the lid under stretch with Frost suture and applying a pressure dressing for 5-7 days. RESULTS: Data from 28 eyelids in 7 patients were included. Average follow-up was 21.5 months (range, 4-76 months). Of the 28 eyelids, 20 (71.4%) showed postoperative resolution of spasm, with no further need for botulinum toxin injections. In the 8 eyelids requiring further injections, the average time to injection after surgery was 194 days (range, 78-323 days), and the average number of injections was 12 (range, 2-23 injections). All but one eyelid had excellent cosmetic results, without signs of contracture; one eyelid developed postoperative skin contracture following premature removal of the Frost suture and pressure dressing because of concerns over increased intraocular pressure. CONCLUSIONS: In our patient cohort, this modified technique resulted in excellent cosmetic and functional results and limited postoperative skin contracture.

Persistent hemifacial spasm after microvascular decompression: a risk assessment model.

OBJECTIVE: Microvascular decompression (MVD) for hemifacial spasm (HFS) provides resolution of disabling symptoms such as eyelid twitching and muscle contractions of the entire hemiface. The primary aim of this study was to evaluate the predictive value of patient demographics and spasm characteristics on long-term outcomes, with or without intraoperative lateral spread response (LSR) as an additional variable in a risk assessment model. METHODS: A retrospective study was undertaken to evaluate the associations of pre-operative patient characteristics, as well as intraoperative LSR and need for a staged procedure on the presence of persistent or recurrent HFS at the time of hospital discharge and at follow-up. A risk assessment model was constructed with the inclusion of six clinically or statistically significant variables from the univariate analyses. A receiving operator characteristic curve was generated, and area under the curve was calculated to determine the strength of the predictive model. RESULTS: A risk assessment model was first created consisting of significant pre-operative variables (Model 1) (age >50, female gender, history of botulinum toxin use, platysma muscle involvement). This model demonstrated borderline predictive value for persistent spasm at discharge (AUC .60; p=.045) and fair predictive value at follow-up (AUC .75; p=.001). Intraoperative variables (e.g. LSR persistence) demonstrated little additive value (Model 2) (AUC .67). Patients with a higher risk score (three or greater) demonstrated greater odds of persistent HFS at the time of discharge (OR 1.5 [95%CI 1.16-1.97]; p=.035), as well as greater odds of persistent or recurrent spasm at the time of follow-up (OR 3.0 [95%CI 1.52-5.95]; p=.002) Conclusions: A risk assessment model consisting of pre-operative clinical characteristics is useful in prognosticating HFS persistence at follow-up.

Frontalis suspension surgery to treat patients with blepharospasm and eyelid opening apraxia: long-term results.

Blepharospasm may be accompanied by eyelid opening apraxia (EOA) reducing the efficacy of botulinum toxin (BT) therapy. The frontalis suspension operation (FSO) is then the only effective treatment option available. We want to report the first long-term results with FSO. We studied 15 patients with blepharospasm and EAO unresponsive to BT therapy (9 females, 6 males, age 61.9 ± 11.5 years). FSO was performed by applying 2 polytetrafluoroethylene threads (PTFE, Gore-Tex®) per eye connecting the frontalis muscle to the upper eye lid. Tension of the two carrés was set to produce a palpebral fissure width of 2-3 mm. Therapy outcome was monitored by a quality-of-life questionnaire (QoL-Q) and a self-assessment calendar reviewing postoperative days 0-9 (T1), 10-89 (T2), 90-179 (T3), 180-365 (T4), and >365 days (T5). Altogether, 40 FSO were performed. Postoperatively, all patients reported improved eyelid opening, 4 (27%) complete remission of symptoms. At T1, this improvement was 74.6 ± 26.4% on the self-assessment scale, after 1 year 68.2 ± 27.5%. Throughout the observation period (T1-T5), the improvement was 71.9 ± 25.6%. All 19 items on the QoL-Q (except for presence of involuntary eye lid closure) showed postoperative improvement. Adverse effects included circumscript upper eyelid haematomas, suture extrusion, suture granuloma, lacrimation, and infections. In all patients, BT therapy had to be continued to treat orbicularis oculi contractions. Our first long-term results demonstrate that FSO is a benign procedure producing robust and stable therapeutic effects on EOA in blepharospasm.

Aesthetic and Non-aesthetic Indications for Orbicularis Oculi Myectomy.

INTRODUCTION: Crows' feet wrinkles are caused by the action of the orbicularis oculi muscle on overlying skin. Treatment options range from botulinum toxin to a multitude of surgical methods. We first described our technique in 2003 and refined it in 2006. We highlight the evolution of our technique and look at our results to assess the effectiveness of our technique. OBJECTIVE: The aim of this article is to show our experience of 13 years in 134 patients with our technique of orbicularis oculi myectomy. METHODS: From September 2000 to July 2013, we operated on 134 patients with an age range of 28-77 years. Of these patients, 104 had myectomies via lifting and 22 via a blepharoplasty approach. Five patients had myectomies for treatment of blepharospasm and a further three patients to restore symmetry in facial palsy. An evaluation of the results was performed by two medical students. RESULTS: Our results showed reduction of the wrinkles in all cases. For the statistical analysis the Wilcoxon test was performed. The p value was less than 0.001 showing a significant reduction of crows' feet wrinkles in both sides when the orbicularis myectomy was performed, via blepharoplasty or lifting. For myectomy performed for blepharospasm or facial palsy, the statistical analysis was not done due to the low numbers. CONCLUSION: In the appropriate patient, orbicularis oculi myectomy is an effective and long-term treatment for crows' feet wrinkles with a low risk of complications and high patient satisfaction. LEVEL OF EVIDENCE IV: This journal requires that authors assign a level of evidence to each article. For a full description of these Evidence-Based Medicine ratings, please refer to the Table of Contents or the online Instructions to Authors www.springer.com/00266 .

Effect of upper eyelid myectomy on subsequent chemodenervation in the management of benign essential blepharospasm.

PURPOSE: Some patients with severe benign essential blepharospasm (BEB) become clinically refractory to chemodenervation with botulinum toxin. In these patients, surgical myectomy is an effective additive treatment for the management of this disabling condition. The purpose of this study is to determine how myectomy for BEB alters subsequent botulinum toxin administration. METHODS: A retrospective chart review of 27 patients undergoing surgical myectomy for the treatment of refractory BEB was conducted. The frequency and dosage of botulinum toxin were compared between preoperative and postoperative treatments. Paired Student t tests were used to determine significance (α = 0.05). RESULTS: The mean interval between botulinum treatments was 10.1 weeks preoperatively and 15.7 weeks postoperatively (p < 0.001). The mean quantity of botulinum toxin administered was 68 units prior to myectomy and 64.9 units postoperatively (p = 0.227). When comparing the quantity of botulinum toxin injected into the upper eyelid region only (site of myectomy), there was a significant decrease in the dose administered preoperatively and postoperatively (46.8 units vs. 39.9 units, p = 0.00310). CONCLUSIONS: Upper eyelid myectomy is an effective treatment modality for BEB in those patients who are refractory to botulinum toxin injections. Myectomy for BEB decreases the morbidity, botulinum toxin treatment frequency, and long-term expense associated with this disabling condition.

Frontalis suspension surgery to treat patients with essential blepharospasm and apraxia of eyelid opening-technique and results.

INTRODUCTION: We describe the results of 15 patients suffering from essential blepharospasm with apraxia of eyelid opening who underwent frontalis suspension surgery. MATERIAL AND METHODS: Patients with apraxia of eyelid opening and unresponsive to botulinum toxin injections were studied. Bilateral frontalis suspension surgery was performed (sling operation) using polytetrafluoroethylene (Gore-Tex®) sutures. The patients reported the degree of improvement using a subjective rating scale to evaluate the benefit of the operation at two times after surgery (0-10 days and 180-360 days). RESULTS: The patients reported a high degree of subjective improvement. In the early postoperative period (0-10 days) the mean degree of subjective improvement was 74.6% (standard deviation (SD) 26.4%). At 180-360 days after surgery the mean improvement was 70.0% (SD 26.7%). Small hematomas of the upper lid occurred postoperatively in all patients. Other complications were suture extrusions (9.1%), suture granulomas (6.1%), lacrimation (5.0%) and local infections (7.5%). Postoperatively, all patients needed additional botulinum toxin injections for optimal outcome. CONCLUSION: Frontalis suspension surgery is a minimally invasive and effective treatment option for apraxia of eyelid opening in patients with essential blepharospasm unresponsive to botulinum toxin injections alone.