Management of morderate-to-severe Marcus-Gunn syndrome by anastomosis of levator and frontal muscles

AIM: To study the effect of clinical management of moderate-to-severe Marcus-Gunn syndrome (MGS) by anastomosis of levator and frontal muscles. METHODS: The medical records of 13 patients with moderate-to-severe MGS who underwent surgeries in our institute between 2000 and 2007 were reviewed retrospectively. They underwent unilateral anastomosis of levator and frontal muscles under local anesthesia. RESULTS: Postoperative follow-up periods ranged from 6 to 36 months, with an average of 12 months. All eyelids (100%) showed complete resolution of jaw-winking, ten eyelids (76.9%) had good correction of ptosis, with equal plapebral apertures and symmetrical contours, three (23.1%) showed residual mild ptosis (<2mm). CONCLUSION: For moderate-to-severe MGS, unilateral ana- stomosis of levator and frontal muscles provides satisfied correction of jaw-winking and ptosis.     

提上睑肌离断额肌瓣悬吊术治疗中重度Marcus-Gunn综合征的临床研究

目的研究提上睑肌离断额肌瓣悬吊术治疗中重度Marcus-Gunn综合征的临床疗效。方法6例中重度Marcus-Gunn综合征患者采用提上睑肌离断额肌瓣悬吊手术治疗。结果随访6个月～5年,6例患者术后颌动瞬目症状消除,双侧睑裂对称,上睑缘无遮盖瞳孔,睑缘弧度自然美观,2例伴有轻度的上下睑闭合困难及上睑迟滞现象。结论提上睑肌离断额肌瓣悬吊术是治疗中重度Marcus-gunn综合征较理想的手术方法。     

Use of the levator muscle as a frontalis sling

PURPOSE: To investigate the use of the distal portion of levator aponeurosis as a flap for frontalis suspension in patients with severe congenital ptosis and poor levator function. METHODS: Eleven procedures were performed on 8 patients with severe congenital ptosis and poor levator muscle function. Three of the 8 patients had Marcus-Gunn jaw-winking phenomenon. Preoperative evaluation included measurements of the degree of ptosis, levator function, superior rectus action, Bell phenomenon, lagophthalmos, and (if present) synkinetic eyelid movement. Surgery involved fashioning a flap from the distal portion of the levator aponeurosis and anchoring it to the frontalis muscle. Postoperatively, patients were followed for at least 6 months to assess the level of the eyelid in the primary position at rest and when the brow is raised, the degree of eyelid margin excursion on brow elevation, persistence of synkinetic eye movement, presence of complications, lagophthalmos, corneal exposure, and symmetry. RESULTS: Synkinetic muscle movements were completely abolished. All cases had good primary eyelid position, no corneal complications, and effective frontalis action on eyelid elevation. CONCLUSIONS: The distal levator muscle flap is an effective frontalis suspension material to correct ptosis with poor levator function and to abolish synkinetic eyelid movement without compromising corneal protection.     

Transconjunctival frontalis suspension: a clinical evaluation.

PURPOSE: To determine the effectiveness of transconjunctival frontalis suspension in patients with blepharoptosis and poor levator function (eyelid excursion less then 5 mm). METHODS: The medical records of 29 patients (50 ptotic eyelids) were reviewed. RESULTS: Four patients (eight eyelids) had blepharophimosis syndrome, ten patients (27 eyelids) had congenital ptosis, seven patients (14 eyelids) had myogenic ptosis, and one patient (one eyelid) had neurogenic ptosis. Surgical results were good and complications were minimal during follow-up intervals ranging from six months to seven years. CONCLUSIONS: Transconjunctival frontalis suspension is technically simpler than traditional external frontalis suspension and yields satisfactory functional and cosmetic results in patients with poor levator function.     

Frontalis muscle flap advancement for jaw-winking ptosis

PURPOSE: To describe the technique and results of frontalis muscle flap advancement for correction of ptosis associated with jaw-winking. METHODS: Four cases of unilateral jaw-winking ptosis were corrected with frontalis muscle flap advancement after excision of levator muscle on the affected side. RESULTS: Three of the 4 patients achieved good results with the correction within 1 mm of the opposite eyelid. The residual asymmetry on downgaze after follow-up of 4 to 18 months ranged from 1 to 5 mm. The main complication was mild forehead hypoesthesia in 2 patients, which resolved over a period of 1 month. CONCLUSIONS: Frontalis muscle flap advancement is an effective procedure when performed unilaterally for jaw-winking ptosis. It is simple, safe, and involves a single surgical field. Eyelid lag on downgaze improves considerably over time, and the remaining asymmetry between the two eyelids is cosmetically acceptable to most patients.     

中重度Marcus-Gunn综合征患者的临床特点及其手术治疗

目的分析中重度Marcus-Gunn综合征患者的临床特点,探讨和评价其手术治疗效果。方法对中山大学中山眼科中心1997年7月至2003年7月收治的33例中重度Marcus-Gunn综合征患者的病历资料进行临床特点分析。患者均行单侧提上睑肌节段切除联合同侧额肌肌瓣悬吊术治疗上睑下垂,评价其术后治疗效果。结果所有患者术前的上睑下垂及颌动瞬目量均大于2mm,无家族史,无明显性别差异（男：女=16：17）,左眼（22例）多于右眼（11例）。术后随访1—6年。随访期末进行的上睑下垂矫治效果评价中26／33例患者满意,3／33例较满意,1／33例不满意,另有3例失访。结论中重度Marcus-Gunn综合征患者伴有的上睑下垂需行手术矫正。单侧提上睑肌节段切除联合同侧额肌肌瓣悬吊术可以满意地矫正中重度Marcus-Gunn综合征患者的上睑下垂。     

Marcus Gunn jaw-winking phenomenon: a new supplemental test in the preoperative evaluation.

PURPOSE: To introduce a new method for the evaluation of Marcus Gunn jaw-winking ptosis that more precisely defines the severity of blepharoptosis. METHODS: A retrospective review of 16 consecutive patients with Marcus Gunn jaw-winking ptosis presenting to our institution between 1993 to 1999 was performed. The position of the affected eyelid was observed after applying a technique of jaw immobilization and disruption of fusion with temporary occlusion of the ipsilateral side. RESULTS: In patients presenting with mild to moderate Marcus Gunn jaw-winking, the majority (62.5%) demonstrated a positive test, uncovering complete or near complete ptosis. Test results were partially positive in 3 patients (18.8%) with increased but not complete ptosis and negative in 3 patients (18.8%) with no change in eyelid position. CONCLUSIONS: Blepharoptosis associated with Marcus Gunn jaw-winking phenomenon is often more severe than found by conventional clinical evaluation. This finding may explain the frequent undercorrection and unpredictable results following levator resection. In patients exhibiting a positive jaw-winking ptosis test, disappointing outcomes with levator resection may be avoided by instead proceeding with a frontalis suspension with levator disinsertion as recommended for ptosis with severe jaw winking.     

提上睑肌离断联合EPTFE额肌悬吊术治疗儿童中重度下颌瞬目综合征的疗效

目的探讨提上睑肌离断联合EPTFE额肌悬吊术治疗中重度儿童下颌瞬目综合征手术疗效。方法回顾性系列病例研究。分析2013-2014年行提上睑肌腱膜节段去除联合EPTFE额肌瓣腱膜悬吊术治疗单眼中、重度下颌瞬目综合征患儿49例的临床资料。其中下颌瞬目中度27例（55%）,重度22例（45%）;上睑下垂中度19例（39%）,重度30例（61%）。年龄18个月～13岁。术后随访10～12个月。计量资料之间的比较采用方差分析,计数资料之间的比较采用卡方检验。结果49例中46例（94%）下颌瞬目治愈,术后未出现下颌联动现象。2例好转,1例无效。上睑下垂矫正后功能与美容疗效满意,其中31例（63%）提高≥6 mm,17例（35%）提高3～5 mm,1例（2%）在术后3个月上睑高度回落,遮盖瞳孔1/2。1例（2%）下颌瞬目联动幅度为3 mm,但功能及美容疗效满意。4例（8%）术后发生暴露性角膜炎,3例（6%）发生结膜撕裂,2例（4%）上睑下垂复发,2例（4%）上睑内翻倒睫,1例（2%）排异,1例（2%）下颌瞬目联动运动矫正失败。结论患眼提上睑肌腱膜节段去除联合EPTFE额肌瓣腱膜悬吊术治疗单眼中、重度下颌-瞬目综合征手术疗效好,术后反应轻,为合并弱视患儿早期治疗提供必要条件。     

Direct Frontalis Flap with and without Levator Pulley for Correction of Severe Ptosis with Poor Levator Function in the Same Patient

Purpose: To compare two techniques of frontalis muscle flap suspension in different eyelids of the same patient for correction of severe ptosis with minimal levator function.

Material and methods: A prospective study of four patients with severe bilateral ptosis and poor levator function, who underwent direct frontalis muscle flap on the right eyelid and frontalis muscle flap with levator pulley on the left eyelid was conducted. Eyelid studies measurements were taken at baseline, 2 months, one year and 5 years after surgery. The presence of complications, flap function and palpebral contour were evaluated.

Results: Despite the surgical technique performed, good results in terms of functionality, contour and aesthetics were observed. In the eyes that underwent frontalis muscle flap (FMF)-direct, there were 2 cases with moderate anteriorization of eyelid margin in extreme upgaze and all patients showed eyelash ptosis that persisted one year after surgery, but improved after 5 years. In the eyes that underwent FMF-pulley, no upgaze anteriorization of eyelid margin was observed and three patients had eyelash ptosis of lesser extent than the fellow eye, improving after 1 year follow-up. FMF-pulley showed more long-term stability in eyelid height, compared with FMF-direct.

Conclusions: Frontalis muscle flap with a pulley in the levator aponeurosis prevents some complications caused by the excessive vertical component of the direct frontalis muscle flap, especially in deep-set eye patients, with better stability of the eyelid height and contour over time.     

Unilateral frontalis sling for the surgical correction of unilateral poor-function ptosis

PURPOSE: To evaluate the functional and cosmetic results after frontalis sling repair for unilateral ptosis associated with either poor levator function or synkinesis. METHODS: Preoperative and postoperative photographs and records of 127 patients who underwent unilateral frontalis sling ptosis repair were retrospectively reviewed. An eyelid crease incision was used in all cases, with suturing of the sling material directly to tarsus. RESULTS: Preoperative diagnosis for all patients was either unilateral poor-function blepharoptosis or ptosis associated with levator synkinesis. Underlying causes included 75 congenital, 13 posttraumatic, 11 congenital "jaw-winking," 10 cranial nerve III palsies, 9 myasthenia gravis, 5 chronic progressive external ophthalmoplegia, and 4 congenital "double-elevator" palsies. There was a mean follow-up of 11.6 months. Twenty-eight eyelids required reoperation: 11 for undercorrection, 6 for overcorrection with keratopathy, 2 for upper eyelid crease revision, 7 for correction of poor contour, 1 for a broken sling, and 1 for removal of an infected exposed polytetraflouroethylene sling. Lagophthalmos of greater than 2 mm was noted in 18 patients, 5 of whom had persistent keratopathy requiring reoperation. No other complications were reported, except for 1 suture granuloma. Good to excellent final postoperative eyelid height was achieved in 121 patients (95%) after all surgeries and with conscious recruitment of the frontalis muscle. A large majority of patients and/or parents expressed satisfaction with the final cosmetic result and were not bothered by any asymmetric lagophthalmos in downgaze or lack of a synchronous blink. However, 19 of 25 amblyopic patients were less satisfied with passive eyelid height as they failed to recruit the ipsilateral frontalis muscle to activate the sling during binocular viewing. In 17 of these 19 patients, good to excellent eyelid height could be achieved with conscious active brow elevation. CONCLUSIONS: Unilateral sling provides good to excellent functional and cosmetic results in unilateral poor-function ptosis. However, patients with amblyopia usually require conscious effort to activate the frontalis muscle to achieve satisfactory eyelid height.     

中重度Marcus-Gunn综合征患者的手术治疗

目的分析中重度Marcus-Gunn综合征患者的临床特点,评价其手术治疗效果。方法收集武汉大学人民医院2006年1月至2010年1月收住院治疗的12例中重度Marcus-Gunn综合征患者的病历资料,对其临床特点进行分析。患者均行患眼提上睑肌离断联合同侧额肌瓣悬吊术治疗颌动瞬目现象,术后随访6个月,依据静态时双眼睑弧度及对称情况、颌动瞬目现象根治情况评价其术后治疗效果。结果所有患者术前下颌运动时上睑至少开大到角膜上缘,男性多于女性(男8例,女4例),均为单眼发病,右眼7例,左眼5例。术前8眼伴随屈光不正,5眼伴有斜视。术后2眼出现角膜点样损害,药物治疗7d后恢复。随访期末进行颌动瞬目矫治效果评价中10眼效果良好,2眼效果满意。结论中重度Marcus-Gunn综合征患者伴有的颌动瞬目现象可通过手术治疗,患侧提上睑肌离断联合同侧额肌肌瓣悬吊术可以满意地矫正Marcus-Gunn综合征患者伴有的中重度颌动瞬目现象。     

Long-term results of frontalis suspension using autogenous fascia lata for congenital ptosis in children under 3 years of age

PURPOSE: To evaluate the outcome of frontalis suspension surgery for congenital ptosis using autogenous fascia lata for children under 3 years of age. DESIGN: Retrospective nonrandomized interventional case series. METHODS: This study included nine children (14 eyelids) with severe congenital ptosis aged less than 3 years. All patients underwent frontalis suspension surgery using autogenous fascia lata. Postoperative lid level results, ptosis recurrence, and cosmetic appearance of leg scars were evaluated after the operation. RESULTS: The mean age of the patients was 15.3 months (range, 6 months to 2.5 years) with a mean follow-up period of 41.6 months (range, 18 to 96 months). All children achieved satisfactory cosmetic and functional results, with no postoperative complications such as wound infection, corneal exposure, eyelid contour abnormalities, or ptosis overcorrection. No recurrence of ptosis was encountered. Harvesting was not difficult, and a sufficient amount of material was achieved. There was one patient (11.1%) with a hypertrophied leg scar. CONCLUSIONS: Frontalis suspension using autogenous fascia is proposed as a possible procedure for correcting congenital ptosis in children younger than 3 years of age. It appears to be an effective and feasible treatment for children with poor levator muscle function in this age group.     

Functional results and complications of Mersilene mesh use for frontalis suspension ptosis surgery

AIMS: To assess the functional results and complications of Mersilene (polyester) mesh frontalis sling suspension to correct poor levator function ptosis. METHODS: Retrospective case series. 32 eyelids of 20 patients (12 children and eight adults). RESULTS: Follow up 1-69 months (mean 32). CHILDREN: eight patients had bilateral and four unilateral surgery (20 eyelids). Good long term functional results were achieved in 73% (8/11 children) and 77% (14/18) eyelids. Two children had early postoperative wound infection requiring removal of mesh in one; the other was lost to follow up following medical treatment. ADULTS: four patients had bilateral and four unilateral surgery (12 eyelids). Good long term functional results were achieved in 75% (6/8 patients, 9/12 eyelids). One postoperative wound infection and one mesh exposure were treated definitively by surgical excision of mesh. CONCLUSION: Mersilene mesh provides good functional results but up to 20% of patients have early soft tissue complications. Other materials such as monofilament suture or autogenous fascia lata should be considered.     

Broad fascia fixation enhances frontalis suspension

PURPOSE: To report the use of a modified frontalis suspension procedure for congenital ptosis patients with minimal to no levator function. METHODS: Retrospective, non-comparative case series of frontalis suspension by 2 surgeons over a 21-year period. RESULTS: Frontalis suspension with broad fascia fixation was performed on 48 eyelids of 25 patients. All patients had visual obscuration due to blepharoptosis with only 0 mm to 2 mm of measurable levator function. Age ranged from 3 to 13 years. Minimum length of follow-up was 6 months (range, 6-174 months). The palpebral fissure was increased in all patients, improving their head position and unmasking their visual axis in primary gaze. Postoperatively, all patients developed transient exposure symptoms. There were no migrations, extrusions, infections, or granulomas. All patients achieved a satisfactory postoperative result based on eyelid position, function, and contour. CONCLUSIONS: This modified frontalis suspension procedure maximizes frontalis muscle recruitment, creating powerful eyelid elevation with stable effect over time. The technique minimizes other procedures' pitfalls and advances in cosmesis are achieved with enhanced eyelid crease formation and adjustable eyelid contour.     

Exposure keratopathy following silicone frontalis suspension in adult neuro‐ and myogenic ptosis

Purpose: To evaluate the incidence of exposure keratopathy following silicone frontalis suspension in adult neuro‐ and myogenic blepharoptosis. Method: Retrospective noncomparative analysis of the charts of 69 cases (101 eyelids) of silicone frontalis suspension. Results: Sixty‐one patients (93 eyelids) had myogenic ptosis, and eight patients (eight eyelids) had neurogenic ptosis. Preoperative diagnoses included chronic progressive external ophthalmoplegia, myotonic dystrophy, oculopharyngeal dystrophy, third cranial nerve palsy because of trauma or other causes. Average age at the time of operation was 54. Mean interval between the intervention and the first and second postoperative control was 8 and 28 months, respectively. Thirty‐one patients (31 eyelids) needed a second follow‐up visit. Postoperative punctate epithelial erosions (PEE) were encountered most frequently in patients with Steinert’s disease (42% of eyes) and congenital ptosis (33% of eyes). Patients with oculopharyngeal dystrophy did not develop PEE. Corneal ulceration developed in three eyes (two patients): one eye was successfully treated with local antibiotic ointments and lubricants, a bilateral corneal ulceration in the second patient was successfully treated with partial conjunctival grafts. Conclusion: This study cohort demonstrated a 26% risk of exposure keratopathy following silicone frontalis suspension. The risk of major corneal complications, such as ulceration, was low (3%).     

Surgical correction of blepharoptosis in patients with myasthenia gravis

PURPOSE: To describe the results of surgical correction of blepharoptosis in a series of patients with myasthenia gravis (MG). METHODS: In this retrospective case series, we reviewed the medical records of all patients with MG who did not respond to medical therapy and underwent surgical correction for blepharoptosis at the Mayo Clinic between 1985 and 1999. The primary outcome measure was change in interpalpebral eyelid fissure height. RESULTS: Sixteen blepharoptosis procedures were performed on 10 patients with MG. Eight of the 10 patients had ocular MG. Two of the 10 patients had systemic MG. Of the 16 procedures performed, 9 were external levator advancements (ELA), six were frontalis slings, and one was a tarsomyectomy. Patients were followed postoperatively for an average of 34 months (range, 14-126 months). The amount of ptosis was quantified pre- and postoperatively for seven of the nine eyelids that underwent ELA. For these seven eyelids (five patients), there was a statistically significant improvement in the mean interpalpebral eyelid fissure height from 3.7 mm preoperatively to 7.8 mm postoperatively, with a mean difference of 4.1 mm (95% confidence interval 1.9 mm to 6.25 mm, p = 0.0038). Postoperative complications included worsened diplopia in one patient with ELA and exposure keratopathy in one patient with frontalis sling. Two of the ELA eyelids developed recurrent ptosis requiring additional surgery more than 2 years after the initial procedure. CONCLUSIONS: Blepharoptosis surgery can achieve eyelid elevation in patients who have failed to respond to medical therapy for MG. Potential complications include worsened diplopia and exposure keratopathy.     

Double rhomboid silicone rod frontalis suspension

The frontalis suspension operation has proven effective for patients with significant ptosis and poor levator function. In patients with poor eye protective mechanisms, silicone rods may be used since the material has enough tensile strength to raise the eyelids yet it is sufficiently elastic to allow closure via action of the orbicularis muscle. This article reports the technique and results of a modified operation that uses two silicone rods per upper eyelid and eliminates fixation sutures to tarsus inferiorly and frontalis muscle superiorly. The results obtained are discussed. This technique provides a better upper eyelid contour and is easily adjustable postoperatively.     

Marcus Gunn张口瞬目综合征手术方式探讨

目的探讨Marcus Gunn张口瞬目综合征不同手术方式和治疗效果。方法Marcus Gunn张口瞬目综合征6例(6眼)采用上睑提肌水化分离及节段切除联合额肌瓣悬吊术。结果6例随访6月～2年,双眼睑裂高度对称,弧度自然,颌动瞬目联合性动作消失。结论采用上睑提肌水化分离及节段切除联合额肌瓣悬吊,是治疗Marcus Gunn张口瞬目综合征的较好术式。     

Temporalis fascia suspension in the treatment of ptosis

Fifty-three eyelids with severe ptosis were treated by frontalis suspension with temporalis fascia. The surgical indications were limited to cases in which there was absent or minimal levator function. The authors used this as a primary modality in patients with congenital ptosis, blepharophimosis syndrome, Marcus Gunn jaw winking syndrome, severe progressive external ophthalmoplegia, severe blepharospasm and post-traumatic ptosis. Temporalis suspension was performed as a secondary procedure after failure of various other procedures, including levator resection, and suspension with PTFE (Goretex), Mersilene, or fascia lata. Results after an average follow-up period of six months (two to 14 months) were good in 51 cases; in two cases release of the suspension and recurrence of ptosis were treated by re-suspension with fascia lata.

Temporalis fascia has been used in the correction of ectropion, lagophthalmos in facial nerve palsy and in reconstruction of post-traumatic eyelid defects. It has three major advantages: (1) it is autogenous and hence better tolerated than synthetic materials such as Goretex or Mersilene; (2) it is easily harvested, requiring only one operative field, under local anesthesia; (3) it yields minimal post-operative morbidity (no interference with ambulation, shorter convalescence). The disadvantage of this procedure is the fragility of temporalis fascia as compared to fascia lata, which limits its use to patients over six years old.     

Early Frontalis Flap Surgery as First Option to Correct Congenital Ptosis with Poor Levator Function

Abstract

In congenital blepharoptosis the upper eyelid cannot be lifted normally because of congenital impairment in the levator function. The descended eyelid margin partially or completely obstructs of the visual axis with the consequent risk of amblyopia. Frontalis suspension is the surgery of choice for ptosis with poor levator function creating a linkage between the frontalis muscle and the tarsus; the frontalis muscle is used to elevate the eyelid. Direct transplantation of frontalis muscle to the upper eyelid has been widely described. We report our experience using frontalis flap in congenital ptosis with poor levator function in children.

Methods: Retrospective study of 30 eyes with severe congenital ptosis and poor levator function treated by means of direct frontalis flap. Mean age 2 years. Eyelid measurements were taken at baseline, 1, 3, 12 months postoperatively and last visit. Mean ptosis degree was 5?mm (3--8?mm) and levator function 2?mm (1--5?mm). The presence of complications, flap function and palpebral contour were evaluated. Mean follow up time was 27 months. At last visit, ptosis degree ranged from 0 to 3?mm.

Discussion: Direct advancement of the frontalis muscle to treat severe eyelid ptosis is effective and stable in the long term avoiding the use of a linking structure, therefore the risk of foreign-body reaction, absorption, granuloma and late exposure, as well as the need for a second visible incision in the forehead. Patients learn how to control the lid height by means of the frontalis muscle achieving more symmetry.