Characterizing Patients with Age-Related Divergence Insufficiency Esotropia M. X. Repka, E. Downing Johns Hopkins University, Baltimore, Maryland, USA ABSTRACT: Introduction/Purpose: To describe the clinical characteristics of a group of patients older than 40 years of age with divergence insufficiency esotropia and the results of surgery performed on a subgroup. Methods: A retrospective review of 86 adult patients diagnosed with divergence insufficiency. Clinical features evaluated include age at diagnosis, visual acuity, primary position deviations at distance and near, treatment offered, post- surgical intervention deviations, and presence of neurological disease. Results: We analysed 86 patients, 33 men and 49 women. The average age of diagnosis was 73.2 years, the average visual acuity was 20/22 in the better eye and 20/24 in the worst eye. The average initial deviation at near was 12 prism diopters at distance and 5.2 prism diopters at near. Following surgery the average deviations were less than 1 prism diopter at near and about 2 prism diopters at near. The percentage of patients with neurological disease was 8%. Conclusions: In this study bilateral medial rectus recession surgery was effective at reducing esotropia at distance with a slight tendency of overcorrection at near. Few (8.5%) patients had accompanying neurological disease. 1 INTRODUCTION Divergence insufficiency has been described in the literature for many decades. In some studies there was concern about the use of this term and differentiating the condition from bilateral mild abducens nerve pareses.1 In recent years, Mittelman has called attention to an increasingly common form of esotropia in adults he has termed, “Adult Onset Age-Related Distance Esotropia”.2 The strabismus was an acquired esotropia in adults with no prior history of strabismus or history of neurological problems. More recently he found that medial rectus recession surgery was an effective treatment. He reported surgical success in in a group of 10 patients using bilateral medial rectus recession.3 In 2009 Rutar and Demer described the neuroimaging findings of an adult with acquire esotropia without high myopia which they called “Divergence Paralysis Esotropia”.4 They noted connective tissue degeneration with downward displacement of the lateral rectus muscle along with thinning of the LR-SR band. Recently, Chaudhuri and Demer reported in a group of 13 patients that bilateral medial rectus recessions are an effective treatment of this condition.5 However, a substantial increase in surgical dose was required to achieve success. Stager et al have also reviewed their experience with surgical treatment, preferring a unilateral lateral rectus resection, finding excellent success.6 In our clinic there is no standardized management protocol; bilateral medial rectus recessions were most often performed, but unilateral recess-resect surgery was performed on a limited basis. Our purpose in this report is to describe a larger cohort of patients and evaluate surgical outcomes in the subgroup that underwent strabismus surgery. 2 METHODS A retrospective chart review of adults older than 40 years of age at onset of an esotropia classified as divergence insufficiency in the Wilmer electronic database of patients seen between 1977 and 2011. Chart review was conducted confirming no prior history of strabismus, comitancy of the esotropia in lateral gazes and a distance esotropia 5 or more prism diopters greater than the near angel. Demographic data, age at onset, treatment with prisms, treatment with surgery, ocular deviations at distance and near at diagnosis and following surgery if applicable. Neurological assessment was by history and follow-up monitoring. Imaging was not standard care for evaluation of these patients and performed on occasion. 3 RESULTS There were 86 patients identified; 67% were female. The mean age was 74.0 years (range 43-95 years). A neurological diagnosis was reported in 7. Four of these were previously diagnosed with chronic CN VI, but no pathology was identified on imaging. No patient was subsequently diagnosed with neurological impairment. Mean refractive error was -1.49 D (+3.83 to 11.69 D). Mean angles of esotropia were 12 at distance (range = ET 30 to orthotropia) and 5.2 at near (range = ET 20 to XT6). Therapeutic prism was used by 60 of 86 (69%) with varying success. The frequency of the diagnosis age-related divergence insufficiency esotropia has risen dramatically in our clinic with only 15 cases recorded between 1980 and 2005, but 40 cases between 2005 and 2011. Surgery was performed with no specific protocol on 45 patients. All surgeries were performed with adjustable sutures. Thirty-eight cases (84%) were bilateral MR recessions with a mean = 4.4 mm of recession per eye. There were 7 cases (16%) of unilateral recess-resect procedures. The outcomes for the two surgical techniques are in the Tables. Each surgical approach collapsed the difference. However, there was a tendency for overcorrection at near with medial rectus surgery and overcorrection at distance with recess-resect surgery Table 1 – Outcome from Bilateral Medial Rectus Recessions N=38 Preoperative () Postoperative () Mean Distance Angle Mean Near Angle 13.6 0.7 5.4 -3.2 Distance-Near Difference 8.2 3.9 Table 2 – Outcome for Unilateral Recess Medial Rectus – Resect Lateral Rectus Distance-Near N=7 Mean Distance Angle Mean Near Angle Difference 19.1 9.4 9.7 Preoperative () -1.7 0.3 -2.0 Postoperative () For both surgical approaches we found the surgical effect to be substantially diminished compared with conventional surgical tables. There was a small 0.6 prism diopter change per mm of medial rectus recession surgery shown in the Figure below. 4 DISCUSSION Adult onset age-related distance esotropia is an increasingly common condition found in a adult strabismus practice. In our practice the diagnosis was uncommon prior to 2005, but has increased dramatically in the last few years. While many ophthalmologists have worried in the past about the possibility that this condition represents occult abducens nerve palsy or other neurological disease, which has not been our experience. While our study is non-randomized, we found, as did Chaudhuri and Demer,5 strabismus surgery could correct the esotropia in most cases, but the amount of medial rectus recession surgery required was substantially more than used for other forms of comitant strabismus. For this reason we would recommend using adjustable sutures. We believe that use of the term divergence insufficiency esotropia as a specific diagnostic category for these adult patients should be abandoned. We agree with the more specific term suggested by Mittelman, adult onset age-related distance esotropia, is preferable. This terminology could reasonably be shortened without losing specificity to “age-related distance esotropia” to be more manageable in clinical use. Limitations of this study include the retrospective chart review design. Additionally, neurological diagnoses were made by history and follow up monitoring with no development of neurological disease. Neuro-imaging was not required per any existing clinical protocol, but was performed in many cases at clinician discretion. In no case was there a positive finding. Comparison of surgical techniques in this report or the earlier reports from Mittelman2 and Chaudhuri and Demer5 is not possible because of the lack of randomization and the small number of unilateral recess-resect operations that were performed. REFERENCES 1. Mittelman D. Divergence insufficiency esotropia is a misnomer. JAMA Ophthalmol 2013;131:547. 2. Mittelman D. Age-related distance esotropia. J AAPOS 2006;10:212-3. 3. Mittelman D. Surgical management of adult onset age-related distance esotropia. J Pediatr Ophthalmol Strabismus 2011;48:214-6 4. Rutar T, Demer J. “Heavy Eye” syndrome in the absence of high myopia: A connective tissue degeneration in elderly strabismic patients. J AAPOS 2009;13:36-44. 5. Chaudhuri Z, Demer JL. Medial rectus recession is as effective as lateral rectus resection in divergence paralysis esotropia. Am J Ophthalmol 2012;130:1280-4. 6. Stager Sr DR, Black T, Felius J. Unilateral lateral rectus resection for horizontal diplopia in adults with divergence insufficiency. Graefes Arch Clin Exp Ophthalmol 2013;251:1641-4.