Surgical management of severe cocontraction, globe retraction, and pseudo-ptosis in Duane syndrome.

BACKGROUND: Correction of severe cocontraction and pseudo-ptosis present unique surgical challenges in patients with Duane syndrome. METHODS: We report four Duane syndrome patients with esotropia in primary position, poor abduction, and severe cocontraction causing limitation to adduction, globe retraction, and pseudo-ptosis. All were treated with partial tendon transposition of the vertical rectus muscles augmented with Foster fixation sutures and surgical weakening of the ipsilateral lateral rectus muscle. One patient had a large recession of the lateral rectus muscle, and in three patients, the lateral rectus muscle was inactivated by removing from the globe and attaching its insertion to the lateral orbital wall. RESULTS: Postoperatively, all patients were aligned within eight prisms diopters of orthotropia, had no face turn, and improved adduction and abduction. The two patients who had restriction to abduction on intraoperative forced ductions also had residual esotropia in primary position and underwent recession of the ipsilateral medial rectus muscle as a second procedure. Postoperative binocular single visual field was enlarged by 56 to 500% in the three patients who were tested preoperatively and postoperatively. Globe retraction and cocontraction were markedly relieved. Palpebral fissure widened 1.0 and 6.0 mm in two patients who had preoperative and postoperative measurements. CONCLUSION: In Duane syndrome patients, severe cocontraction, globe retraction, and limitation to adduction may improve if the lateral rectus muscle is maximally recessed or its insertion is inactivated from the globe. Partial transposition of the vertical rectus muscles augmented with Foster sutures improved the angle of esotropia in primary position and abduction. Medial rectus muscle recession is indicated when the passive forced duction test reveals moderate-to-severe restriction to abduction.     

The Binocular Visual System in Amblyopia

We treated a 27-year-old man who suffered from a congenital eye movement abnormality. The patient could not adduct the left eye, and showed exotropia, hypertropia and excyclotropia of the left eye. The range of abduction was also limited. The left eye showed marked upshoot on right gaze and eye globe retraction on right down gaze under conditions of right-eye viewing.The superior rectus muscle of the left eye was recessed and transposed nasally.The insertion of the superior oblique muscle was found to be defective. The upshoot and face turn improved after surgery. The globe retraction on right down gaze was no longer evident.We believe that the superior rectus muscle was innervated by an anomalous branch of the oculomotor nerve, which was originally destined to innervate the medial rectus, and that simultaneous contraction of the superior and inferior rectus muscles caused the globe retraction on right down gaze.This case also suggests the possibility that some part of the upshoot in adduction in Duane's syndrome is due to the co-contraction of the superior and medial rectus muscles due to this innervation anomaly.     

A Case of Acquired Brown Syndrome after Surgical Repair of a Medial Orbital Wall Fracture

A case of acquired Brown syndrome caused by surgical repair of medial orbital wall fracture is reported in the present paper. A 23-year-old man presented at the hospital with right periorbital trauma. Although the patient did not complain of any diplopia, the imaging study revealed a blow-out fracture of the medial orbital wall. Surgical repair with a calvarial bone autograft was performed at the department of plastic surgery. The patient was referred to the ophthalmologic department due to diplopia that newly developed after surgery. The prism cover test at distant fixation showed hypotropia of the right eye, which was 4 prism diopters (PD) in primary gaze, 20 PD in left gaze, while orthophoric in right gaze. Eye movement of the right eye was markedly limited on elevation in adduction with normal elevation in abduction with intorsion in the right eye present. Forced duction test of the right eye showed restricted elevation in adduction. Computerized tomography scan of the orbits showed the right superior oblique muscle was entrapped between the autografted bone fragment and posterior margin of the fracture. When repairing medial orbital wall fracture that causes Brown syndrome, surgeons should always be careful of entrapment of the superior oblique muscle if the implant is inserted without identifying the superior and posterior margin of the orbital fracture site.     

Unilateral recession and resection in Duane syndrome.

PURPOSE: To assess the efficacy of lateral rectus resection with medial rectus recession in the affected eye of patients with Duane retraction syndrome (DRS) with esotropia and limited abduction, compared with bilateral medial rectus recessions. METHODS: The charts of 9 patients with DRS who underwent a recession-resection procedure and 10 patients with DRS who underwent bilateral medial rectus recessions were reviewed. Ocular ductions (graded from 0 = full duction to -4 = total deficit), severity of retraction, alignment, head position, and binocular single vision field (for study group only) were recorded before and after surgery. RESULTS: Before surgery, the study and control groups did not differ in mean primary position esotropia (16.9 and 18.8 PD, respectively), face turn (16.5 degrees and 15.0 degrees, respectively), average limitation of abduction (-3.9 and -3.7, respectively), or adduction (-0.1 and -0.3, respectively). After surgery, both groups had similar mean face turns (3.9 degrees and 1.0 degrees ), esotropia (3.3 PD and 1.0 PD), and abduction limitation in the affected eye (-2.4 and -2.6). However, mean adduction was significantly worse in the control group than in the study group (-1.5 vs -0.6, P = .02). Globe retraction improved in all control subjects. It worsened in 5 study subjects and did not improve in the other 4. In the study group, 1 patient required reoperation for undercorrection and another was overcorrected. CONCLUSION: Seven of 9 patients with DRS, selected on the basis of esotropia, limited abduction, and mild retraction, benefited from a recession-resection procedure. Abduction improved to the same degree as seen after bilateral medial rectus recessions, with less tendency to limit adduction.     

Duane眼球后退综合征13例手术治疗临床分析

目的:探讨Duane眼球后退综合征(DRS)手术治疗的方法和效果。方法:对13例因DRS行手术治疗患者的临床表现、手术治疗方式和效果进行回顾性总结和分析。结果:单眼受累12例,双眼受累1例。Ⅰ型内斜视9例(双眼1例);Ⅱ型外斜视3例;Ⅲ型1例。其中2例外斜视患者合并内转眼急速上射和(或)下射现象。行单眼或双眼水平肌后徙术后代偿头位、眼球后退及睑裂变小体征均得到改善,术后斜视度数≤10△者11例(85%)。行患眼外直肌后徙术,可使内转眼上射和下射现象得到明显改善。结论:水平肌大量后徙可以使原在位的斜视得到矫正,代偿头位消失或得到明显改善,患眼内转的时候,眼球后退、睑裂变小明显减轻,上射和下射的现象减轻或消失。术前牵拉试验及术中彻底消除牵制因素是手术成功的关键。     

改良Yokoyama术治疗高度近视固定性内下斜视

目的探讨改良Yokoyama术治疗高度近视限制性内下斜视的效果。方法回顾性病例研究。分析2011-2014年江苏省中医院6例（8眼）高度近视固定性内下斜视的手术效果。术前CT检查眼外肌位置及组织变化。术前、术后9个诊断眼位检查眼运动功能。术前、术中行被动牵拉试验以评估肌肉的限制程度和功能。以改良Yokoyama+内直肌后徙术对患眼的眼位进行矫正。结果CT显示患眼眼外肌出现移位。术前被动牵拉患眼内直肌出现挛缩,患眼外上转严重受限。术后观察半年,患眼第一眼位正位,外、上转明显改善,无一例出现眼前节缺血并发症。结论改良Yokoyama术术后患者眼位及眼球运动均恢复良好,是矫正高度近视固定性内下斜视较好的一种手术方法。     

A case of congenital oblique retraction syndrome with upshoot in adduction

We treated a 27-year-old man who suffered from a congenital eye movement abnormality. The patient could not adduct the left eye, and showed exotropia, hypertropia and excyclotropia of the left eye. The range of abduction was also limited. The left eye showed marked upshoot on right gaze and eye globe retraction on right down gaze under conditions of right-eye viewing. The superior rectus muscle of the left eye was recessed and transposed nasally. The insertion of the superior oblique muscle was found to be defective. The upshoot and face turn improved after surgery. The globe retraction on right down gaze was no longer evident. We believe that the superior rectus muscle was innervated by an anomalous branch of the oculomotor nerve, which was originally destined to innervate the medial rectus, and that simultaneous contraction of the superior and inferior rectus muscles caused the globe retraction on right down gaze. This case also suggests the possibility that some part of the upshoot in adduction in Duane's syndrome is due to the co-contraction of the superior and medial rectus muscles due to this innervation anomaly.     

Surgical correction of Hallermann-Streiff syndrome: a case report of esotropia, entropion, and blepharoptosis

We report a case of surgical treatment for Hallermann-Streiff syndrome in a patient with ocular manifestations of esotropia, entropion, and blepharoptosis. A 54-year-old man visited Yeouido St. Mary's Hospital complaining of ocular discomfort due to cilia touching the corneas of both eyes for several years. He had a bird-like face, pinched nose, hypotrichosis of the scalp, mandibular hypoplasia with forward displacement of the temporomandibular joints, a small mouth, and proportional short stature. His ophthalmic features included sparse eyelashes and eyebrows, microphthalmia, nystagmus, lower lid entropion in the right eye, and upper lid entropion with blepharoptosis in both eyes. There was esodeviation of the eyeball of more than 100 prism diopters at near and distance, and there were limitations in ocular movement on lateral gaze. The capsulopalpebral fascia was repaired to treat the right lower lid entropion, but an additional Quickert suture was required to prevent recurrence. Blepharoplasty and levator palpebrae repair were performed for blepharoptosis and dermatochalasis. Three months after lid surgery, the right medial rectus muscle was recessed 7.5 mm, the left medial rectus was recessed 7.25 mm, and the left lateral rectus muscle was resected 8.0 mm.     

Presence of thyroid-associated ophthalmopathy in Hashimoto’s thyroiditis

AIM:To determine the prevalence of ophthalmopathy in Hashimoto’s patients and to make a comparison in subgroups of patientsMETHODS:The study involved 110 Hashimoto’s thyroiditis patients and 50 control subjects attending to the endocrinology department of the hospital. Subgroup classification of patientswas made as euthyroid, subclinic and clinic in Hashimato’s thyroiditis. All patients were evaluated by a single experienced ophthalmologist for the prevalence and characteristics of eye signs.RESULTS: The overall prevalences of eye changes were 22.7% (25 patients) in patients and 4% (2 persons) in control subjects respectively (P=0.002). In patients the most common symptom was retrobulbar eye pain with or without any eye movement. Thirteen patients had significant upper eyelid retraction (11.8%). Six patients had eye muscle dysfunction as reduced eye movements in up gaze. In control patients one person had proptosis and another had lid retraction. The clinical activity score and classification of the ophthalmopathy did not show any significant differences among subgroups.CONCLUSION:The eye signs were mostly mild (22.7%) and the most common eye sign was the presence of upper eyelid retraction (11.8%). Additionally six patients had eye muscle dysfunction as reduced eye movements in up gaze. Therefore we recommend to make a routine ophthalmic examination in Hashimoto’s thyroiditis patients in order not to omit the associated ophthalmopathy.     

甲状腺相关眼病眼眶减压术后继发内斜视伴复视的手术疗效观察

目的探讨甲状腺相关眼病(TAO)眼眶减压术后继发内斜视伴复视的斜视矫正手术治疗效果。方法回顾性系列病例研究。选取2016年3月至2018年10月在天津市眼科医院因TAO行眼眶减压术后继发内斜视伴复视行斜视矫正手术治疗的11例患者资料。斜视矫正手术前、后检查患者斜视度数、眼球运动,观察复视情况。手术均在监护下麻醉联合局部麻醉下进行,术中采用被动牵拉试验结合调整缝线方法,调整至第一眼位复视消失。术后定期随访。结果11例患者中男性1例,女性10例;年龄26~42岁;均为单纯内斜视伴复视;内斜视度数10~98三棱镜度;眼眶CT提示患者内直肌不同程度增厚,外直肌增厚程度较内直肌轻。2例患者行单眼内直肌后徙术,2例行双眼内直肌后徙术,2例行单眼内直肌后徙联合外直肌缩短术,另外5例行双眼内直肌后徙联合单眼外直肌缩短术。11例患者术中内直肌后徙量为3.5~7.5 mm,7例联合外直肌缩短术患者外直肌缩短量为2.0~6.0 mm。全部患者术后复视消除,第一眼位正位,均达到治愈标准。眼球运动术前外转受限分级为(-1.91±1.04)级,术后外转受限分级为(-0.64±0.81)级。11例患者均对手术结果满意,术后随访6~24个月,效果稳定,未发现远期过矫患者。结论应用术中调整缝线技术,斜视矫正手术可以有效治疗TAO眼眶减压术后继发的内斜视伴复视。     

眶内异常结构与垂直后退综合征

目的 探讨6例临床诊断为垂直后退综合征患儿的临床与MRI特征.方法 6例单眼垂直后退综合征患儿,年龄1岁3个月至8岁,平均(5.01±1.27)岁.斜视检查包括:屈光度、三棱镜、眼球运动、双眼视觉和眼底照相检查.眼球运动神经脑池段采用头线圈,3 D-FIESTA序列,层厚0.8 mm;眼眶段采用表面线圈,横断面+冠状面+矢状面,FSET1与T2加权扫描,层厚2.0mm,层间距0.3mm.结果 4例患儿表现为上斜视,下转受限,下转时伴轻度眼球后,且皆伴有患眼上睑迟落.MRI检查发现4例均有眼眶内异常结构位于肌锥内间隙上象限内直肌及上直肌群之间或上直肌水平.2例患儿表现为原在位间歇性外斜视,眼球上转受限伴轻度眼球后退睑裂缩小,侧转位时明显.MRI检查发现肌锥内间隙外下象限异常等信号影,1例可疑下直肌分出,1例靠近外直肌.结论 眼眶内异常结构是导致垂直后退综合征的主要原因之一.眼球运动检查结合MRI特征性表现是最重要诊断依据,出生早期发现上睑迟落提示眼眶内异常结构.     

Vertical rectus muscle transposition and botulinum toxin for complete sixth nerve palsy

BACKGROUND: Effective surgical treatment of complete unrecovered sixth nerve palsy must include the transfer of abducting power to the temporal aspect of the globe with release of medial rectus contracture nasally. We describe our experience in the treatment of five such patients who underwent full vertical rectus transposition combined with botulinum toxin chemodenervation of the ipsilateral medial rectus muscle. METHODS: The five patients all had primarily unilateral complete unrecovered sixth nerve palsy. They all underwent a complete preoperative and postoperative eye examination and an orthoptic assessment. Excursion into abduction was graded from -8 (globe immobilized in extreme adduction) to -4 (abduction as far as primary position) to 0 (full abduction). Abduction saccades and a forced muscle generation test confirmed the presence of complete unrecovered sixth nerve palsy, and forced duction testing measured the degree of medial rectus contracture. All patients received ipsilateral medial rectus injection of botulinum toxin in the preoperative (8 to 2 months before surgery) and perioperative periods, and underwent complete superior rectus-inferior rectus transposition temporally. RESULTS: The average length of follow-up was 21 (range 6 to 48) months. The average preoperative distance alignment was 52 (range 25 to 80) prism dioptres (PD). Vertical rectus transposition combined with botulinum toxin injection resulted in an average distance alignment change of 66 PD (range 50 PD to 82 PD) of exoshift. The average final deviation was 1 PD of esotropia (range 4 PD of esotropia to 6 PD of exotropia). Average abduction improved from -6 (range -3 to -8) preoperatively to -1.7 (range -1 to -2) postoperatively. Saccades averaged -4 preoperatively and improved to -2 postoperatively. Normal vertical eye movements were preserved in all patients. A total field of single binocular vision was created in all patients, which averaged 55 degrees (range 30 degrees to 75 degrees) in the horizontal meridian. The field of single binocular vision from primary position into abduction averaged 23 degrees (range 18 degrees to 28 degrees). INTERPRETATION: Temporal transposition of the vertical rectus muscles combined with perioperative botulinum toxin injection of the ipsilateral medial rectus muscle is a reliable and effective way of restoring functional binocular vision in patients with complete unrecovered sixth nerve palsy.     

An Unexpected Outcome of Blunt Ocular Trauma: Rupture of Three Muscles

Introduction: Traumatic strabismus due to isolated extraocular muscle rupture is uncommon. Treatment usually depends on the severity of both the subjective and objective findings. Methods: We report a male patient with restricted abduction and supraduction in the right eye follow ing a blunt ocular trauma. The exploration revealed the rupture of superior rectus, superior oblique, and lateral rectus muscles. Only lateral rectus muscle could be sutured to the proximal segment. Superior rectus and superior oblique muscles were severed brutally, so that repairing was not possible.

Results: On the day after exploration and primary suturation, there was 25 prism diopters (PD) hypotropia and 15 PD esotropia in his right eye with severe limited supraduction and abduction. His major complaint was a large vertical diplopia which resolved partially with the prismatic glasses prescribed. After 6 months follow-up, medial rectus and inferior rectus recession was performed in the right eye. The patient had a limited but improved abduction after the operation. He was orthotropic and had a single binocular vision in the primary position.

Discussion: In suspected extraocular muscle ruptures, orbital imaging methods and surgical exploration should be considered promptly. MRI may be mandatory to demonstrate the severed muscles in cases with persistent diplopia and normal CT. Prognosis is usually better in patients having partial extraocular muscle damage and treatment options should be evaluated on patient basis.     

Congenital isolated absence of the inferior oblique muscle and compensatory aberrant insertion of the inferior and lateral recti

Absence of various extra-ocular muscles has previously been described. However isolated absence of the inferior oblique has not yet been described. Our patient was found to have a small right esotropia and limitation of right eye abduction with an abnormal head posture. Ocular movements showed a marked adduction overshoot in elevation. Exploration of the musculature revealed an absent inferior oblique with abnormally inserted and tight inferior and lateral rectus muscles. Imaging, forced duction testing and surgical exploration is recomended in cases where the signs do not fit into a clear syndrome.     

Duane眼球后退综合征的诊治要点

Duane眼球后退综合征（Duane retraction syndrome,DRS）的共同临床特征为眼球水平运动障碍,内转时睑裂缩小、伴眼球后退,外转时睑裂开大,部分患者眼球内转时伴有急速的上射或下射;其可分为Ⅰ、 Ⅱ、 Ⅲ型。DRS的发病机制既往认为是解剖结构异常所致,但目前多认为神经肌肉支配异常是其根本原因。因DRS患者临床表现各异,需对其特征性表现仔细观察方可做出正确诊断。同时,在治疗上,也需遵循一定的原则针对每例患者的特点设计出个性化的治疗方案。     

Duane's retraction syndrome and spastic paraparesis

Duane's retraction syndrome (DRS) is a neurogenic eye movement abnormality frequently associated with other congenital defects involving ocular, skeletal and neural structures. The authors report on a patient who had DRS associated with a severe spastic paraparesis, which progressed from childhood until young adulthood with subsequent stabilization. The patient had severe limitation of abduction of the left eye, narrowing of the palpebral fissure, and globe retraction in adduction. Neurological examination revealed a severe spastic paraparesis. Extensive clinical neurophysio-logical evaluation, including electromyography, nerve conduction studies, somatosensory evoked responses and transcranial magnetic stimulation, was consistent with a severe myelopathy. Magnetic resonance imaging of the spinal cord demonstrated significant thinning of the cervical and thoracic spinal cord. This previously unreported association is unlikely coincidental, given the rarity of both findings, and may be explained by the abnormal development of neural structures during a critical period of gestation.     

Pseudo-Monocular Nystagmus Associated with Duane’s Syndrome: Report of Two Cases

Abstract

Purpose: To present clinical findings and eye movement recordings of two children who had clinically apparent monocular nystagmus.

Methods: Full orthoptic and ophthalmological examination and eye movement recordings.

Results: An 8-year-old girl (patient 1) and a 13-month-old girl (patient 2) presented with right monocular nystagmus and right esotropia. A magnetic resonance imaging (MRI) scan of the brain obtained previously had been unremarkable for patient 2. Patient 1 had right amblyopia with visual acuity (VA) reduced to 20/400. Both patients had left abduction deficit and left palpebral fissure narrowing on adduction indicative of Duane’s retraction syndrome. Patient 2 also had mild enophthalmos. Both patients had constant horizontal nystagmus in the right eye and very fine nystagmus in the left eye, which could only be detected on video and eye movement recordings.

Conclusion: The existence of Duane’s syndrome in both patients was masking the presence of nystagmus in the left eye, highlighting that detailed examination in this case can eliminate the need for neuroimaging. Interestingly, the dominant eye of both patients was on the side which was affected by Duane’s syndrome, as there was less nystagmus in this eye.     

Unilateral lateral rectus muscle recession and medial rectus muscle resection with or without advancement for postoperative consecutive exotropia.

PURPOSE: To evaluate the effectiveness of unilateral lateral rectus muscle recession and medial rectus muscle resection with or without advancement in treating postoperative consecutive exotropia. METHODS: We performed a retrospective review on 31 patients with consecutive exotropia who were treated with unilateral lateral rectus muscle recession and medial rectus muscle resection (17 patients) or unilateral lateral rectus muscle recession and medial rectus muscle partial resection combined with advancement (14 patients). All patients had exotropia with a less than 10 prism diopters (PD) distance near-disparity. The characteristics studied before surgery included type of esotropia surgery, detection of amblyopia, presence of an "A" or "V" pattern, dissociated vertical deviation, limitation of adduction, deviation angle measurement, and forced duction testing. Ocular alignment and status of adduction postoperatively at the last follow-up were recorded. RESULTS: Nineteen patients (61.3%) had amblyopia, 17 patients (54.8%) had limitation of adduction, 8 patients (25.8%) had dissociated vertical deviation, and 5 patients (16.1%) had an "A" or "V" pattern. The mean preoperative exodeviation was 47.3 PD. Overall 21 (67.7%) of 31 patients achieved a successful postoperative result (alignment within 10 PD of orthophoria). There was no significant difference in successful alignment in patients treated with unilateral medial rectus muscle resection compared with those treated with unilateral medial rectus muscle partial resection combined with advancement. There was no influence of amblyopia on the result. Twelve (70.6%) of the 17 patients with limited adduction preoperatively showed normalization of adduction postoperatively. CONCLUSIONS: Unilateral lateral rectus muscle recession and medial rectus muscle resection with or without advancement is an effective alternative for treating postoperative consecutive exotropia.     

松眼综合征研究进展

眼眶结缔组织在眼球运动中发挥重要作用,其随年龄增长发生退行性改变,这种变化能够引起眼球控制眼外直肌运动的Pulley结构的位置发生改变,从而引起某些特殊类型的斜视发生,即“松眼综合征”,包括分开功能不足性内斜视及小度数的垂直斜视.眼眶磁共振检查发现其产生与眼外直肌Pulley位置的退行性改变有关.对于松眼综合征产生的不同类型的斜视,其治疗方法及手术方式不同,对于分开功能不足性内斜视,临床上既有内直肌后退手术,也可采用外直肌缩短手术,而对于小度数的垂直斜视,则可采用不同程度的垂直肌部分切除术.     

Kimura's Disease Involving the Ipsilateral Face and Extraocular Muscles

Kimura''s disease (KD) is a rare, chronic inflammatory disorder, which is characterized by tumor-like masses mainly located in the head and neck region. Extraocular muscle involvement in KD is uncommon. We report a case of KD that involved both the extraocular muscles and buccal area. A 13-year-old male presented to our clinic with a two-year history of exophthalmos of the left eye and facial swelling. Facial CT and MRI showed a 1.5 × 1.5 cm² soft tissue mass located at the left masticator and buccal area, exophthalmos of the left eye, and diffuse thickening of the left extraocular muscles. We performed a lateral rectus muscle incisional biopsy of the left eye. Oral methylprednisolone therapy was initiated and tapered following the incisional biopsy.Histopathologic findings of the lateral rectus muscle incisional biopsy showed abnormal vascular proliferation with marked eosinophilic infiltration in hypertrophied collagenous tissue. Post-operative histopathologic findings of the facial mass confirmed the diagnosis of KD.Although KD with extraocular muscle involvement is uncommon, an ophthalmologist can diagnose KD by the clinical presentation of exophthalmos, eyelid swelling, and an orbital massas well as by histological examination of a biopsy of the orbital mass.