Acute Acquired Concomitant Esotropia

Acute acquired concomitant esotropia (AACE) is a rare, distinct subtype of esotropia. The purpose of this retrospective study was to describe the clinical characteristics and discuss the classification and etiology of AACE. Charts from 47 patients with AACE referred to our institute between October 2010 and November 2014 were reviewed. All participants underwent a complete medical history, ophthalmologic and orthoptic examinations, and brain and orbital imaging. Mean age at onset was 26.6 12.2 years. Of the 18 cases with deviations 20 PD, 16 presented with diplopia at distance and fusion at near vision at the onset of deviation; differences between distance and near deviations were< 8 PD; all cases except one were treated with prism and diplopia resolved. Of the 29 cases with deviations> 20 PD, 5 were mild hypermetropic with age at onset between 5 and 19 years, 16 were myopic, and 8 were emmetropic with age at onset> 12 years; 24 were surgically treated and 5 cases remained under observation; all 24 cases achieved normal retinal correspondence or fusion or stereopsis on postoperative day 1 in synoptophore; in 23 cases diplopia or visual confusion resolved postoperatively. Of the 47 cases, brain and orbital imaging in 2 cases revealed a tumor in the cerebellopontine angle and 1 case involved spinocerebellar ataxia as revealed by genetic testing. AACE in this study was characterized by a sudden onset of concomitant nonaccommodative esotropia with diplopia or visual confusion at 5 years of age or older and the potential for normal binocular vision. We suggest that AACE can be divided into 2 subYuan Sun, MD, P MD, PhD, Qiwen Chen, MB, and Xuelian Ye, MB (Medicine 94(51):e2273) Abbreviations: AACE = acute acquired concomitant esotropia, D = diopter, LE = left eye, PD = prism diopter, RE = right eye. INTRODUCTION A cute acquired concomitant esotropia (AACE) represents a relatively rare, distinct subtype of esotropia, which occurs in older children, adults, and even the elderly. It was reported that AACE made up 0.3% of childhood strabismus. AACE is characterized by a sudden onset of concomitant esotropia with diplopia. In 1958, Burian and Miller reported 5 cases of AACE and described the 3 categorical features of AACE, which have been accepted as the fundamental symptoms and the pivotal work in this area. Since then, findings from a number of reports have added to this work. However, cases series in most of these reports were quite limited, and, there remain disputes over its etiology, clinical features, and classification. In this report, we present 47 cases with AACE, cuss its classification as well as its relationship with neurologic diseases. PATIENTS AND METHODS Charts of patients diagnosed with AACE were reviewed. These records were derived from cases seen from October 2010 to November 2014 at the Pediatric Ophthalmology and Strabismus Services of the Zhongshan Ophthalmic Center of Sun Yat-Sen University. This study adhered to the tenets of the Declaration of Helsinki and was approved by the ethics committee of the Zhongshan Ophthalmic Center of Sun Yat-Sen University (Guangzhou, China). Patients who met the following criteria were included in this study: (1) concomitant esotropia with acute onset (deviation in all directions of gaze differing by 2 prism diopters when primary deviations were 20 prism diopters and by 5 prism diopters when primary deviations were >20 prism diopters), (2) patients experiencing diplopia or visual confusion, and (3) best corrected visual acuity of not <0.5 in each eye. Patients with a history of eye surgery or those with a reduction of 10 PD in their esotropia with a full hyperopic spectacle correction for 1 month were excluded. Comprehensive medical histories, ophthalmologic, and orthoptic examinations were performed on all patients. The age at onset was determined by the ophthalmologist according to the medical history and patients’ photographs. Deviation size was assessed using prism and alternate cover tests in the with refractive correction. Synoptophore he 3 grades of binocular single vision. was performed after administration of www.md-journal.com | 1 1% atropine ointment once daily for 3 days in patients under 7 years old and 1 % cyclopentolate eye drops every 5 min for 3 times for those >7 years old. A full cycloplegic refraction was prescribed for those with refractive errors 1.0 diopter (D) (spherical equivalent). Additionally, all patients received brain and orbital computed tomography scans, and, in some cases, magnetic resonance imaging. Neurological examinations were Chen et al performed and comprehensive medical histories were taken for cases with intermittent esotropia or variable deviations to rule out cyclic esotropia and myasthenia gravis. RESULTS A total of 47 cases were included in this study, with 18 cases showing deviations 20 PD (Figure 1) and 29 with deviations> 20 PD (Figure 2). There were 25 men and 22 women. Age at onset was 26.6 12.2 years (range: 5.0–59.0 years). The follow-up period was 1.8 0.9 years (range: 0.5– 4.0 years). Slitlamp and funduscopy examinations revealed no abnormality. Mild hypermetropia was observed in 5 cases, 9 cases were emmetropic, and 33 had myopia. Cerebellopontine angle tumors were present in 2 cases and 1 patient experienced spinocerebellar ataxia. All cases showed normal retinal correspondence or fusion or stereopsis in the synoptophore before therapy. Diplopia or visual confusion resolved in 40 cases following prism or surgical treatment. Clinical features of cases with deviations 20 PD are summarized in Table 1. Of these 18 cases, none had a family history of strabismus; 1 case resulted following 24 h of driving; 1 case who presented with esodeviation accompanied by nystagmus and poor coordination of gait and was subsequently diagnosed as spinocerebellar ataxia as revealed from genetic testing; 1 case had a history of chemotherapy and radiotherapy FIGURE 1. Composite 9-gaze photograph of an AACE patient with a re years, presented 20 PD of concomitant esotropia, normal ocular version 2.25 D. His diplopia resolved following treatment with bilateral base o 2 | www.md-journal.com for nasopharyngeal cancer 8 years prior; 14 cases had diplopia at distance but single vision at near vision; 2 cases initially had distance diplopia and later developed near diplopia; 2 cases had both distance and near diplopia at onset; 17 cases were myopic (–0.75 to –9.75D) and 1 case was emmetropic; 15 cases with myopia were using refractive corrections at presentation; in the synoptophore, all 18 cases showed preoperative normal retinal correspondence and stereopsis; brain and orbital imaging were not remarkable; the duration between onset and treatment ranged from 2 weeks to 10 years; diplopia resolved after being corrected with bilateral base out prisms of 2 to 7 PD in all but the one case of spinocerebellar ataxia. Clinical features of cases with deviations> 20 PD are summarized in Table 2. Of these 29 cases, none had a family history of strabismus; brain and orbital imaging in 2 cases revealed masses in the cerebellopontine angle which consisted a 15 11 mm lipoma or a 40 23 mm cholesteatoma; 5 cases involved mild hypermetropic (þ1.0 toþ 1.75D) with age at onset between 5 and 19 years, 16 cases were myopic (–0.75 to – 11.75D) with age at onset between 15 and 59 years, and 8 cases were emmetropic with age at onset between 12 and 38 years; 3 cases experienced visual confusion, 1 case had diplopia at distance whereas both distance and near diplopia were present in the others; 4 cases showed an occasional orthophoria; 1 case whose brain imaging revealed cholesteatoma showed bilateral nystagmus; in the synoptophore, 29 cases showed preoperative normal retinal correspondence or fusion or stereopsis; 4 cases had a minimal (< 1) bilateral lateral rectus muscle underactions; 16 cases were subjected to unilateral medial rectus recession and lateral rectus resection, 7 cases received bilateral Medicine Volume 94, Number 51, December 2015 or unilateral medial rectus recession, 1 case involved a unilateral lateral rectus resection, 5 cases remained under observation; age at surgery was 26.0 2.7 years (range: 5.5–64.0 latively small angle. A 17-year-old boy with distance diplopia for 2 and duction, normal stereopsis in synoptophore, and a myopia of ut prisms of 7 PD. AACE1⁄4 acute acquired concomitant esotropia. Copyright # 2015 Wolters Kluwer Health, Inc. All rights reserved. h a d d tera Medicine Volume 94, Number 51, December 2015 Acute Acquired Concomitant Esotropia years); the duration between onset and surgery was 2.4 0.3 years (range: 0.5–5.0 years); the interval between diagnosis and surgery was 0.4 0.1 years (range: 0.1–1.0 years); in 23 cases diplopia or visual confusion resolved at 1 day to 2 months postoperatively and diplopia remained in 1 case for residual deviation; all 24 cases achieved normal retinal correspondence or fusion or stereopsis on postoperative day one in synoptophore; with the exception of 1 case, all 24 cases were aligned within 10 PD or less of orthotropia; no recurrences were observed at postoperative follow-up as performed at 0.5 to 3 years; no improvements were observed for the patients under observation. DISCUSSION FIGURE 2. Composite 9-gaze photograph of an AACE patient wit presented 40 PD of concomitant esotropia, normal ocular version an Her diplopia resolved following left medial rectus recession and la Acute acquired concomitant esotropia (AACE), or acute late-onset concomitant esotropia, are generally agreed to be characterized by a sudden onset of concomitant esotropia with TABLE 1. Clinical Features of Cases With Deviations 20 PD (n Age Onset (Year) Refraction (Spherical Equivalent)