Alkharashi M, Dagi AF, Dagi LR. Pericardial patch graft repair of severe localized scleral thinning encountered during strabismus surgery. J AAPOS 2017;21(2):156-156.e1.Abstract

This article presents a surgical technique using a pericardial patch for the permanent repair of severe scleral thinning encountered during strabismus surgery. In the present case scleral thinning resulted from buckle removal. Familiarity with this technique may prove important for the strabismus surgeon treating patients with a history of surface ocular hardware or disease-induced scleral thinning. This video article may be viewed

BJ B, MC W, DG H, EC E. Duane Syndrome [Internet]. GeneReviews® [Internet]. Seattle (WA): University of Washington, Seattle; 1993-2019. 2019; Publisher's Version
Bothun ED, Lynn MJ, Christiansen SP, Kruger SJ, VanderVeen DK, Neely DE, Lambert SR, Lambert SR. Strabismus surgery outcomes in the Infant Aphakia Treatment Study (IATS) at age 5 years. J AAPOS 2016;20(6):501-505.Abstract

PURPOSE: To report strabismus surgery frequency and outcomes after monocular infantile cataract surgery with or without IOL implantation. METHODS: The Infant Aphakia Treatment Study (IATS) is a randomized, multicenter clinical trial comparing treatment of aphakia with a primary IOL or contact lens in 114 infants with a unilateral congenital cataract. This report is a secondary outcome analysis of ocular motor data from IATS patients who underwent strabismus surgery prior to age 5 years. RESULTS: Strabismus surgery was performed in 45 (39%) patients (contact lens group [CL], 37%; IOL group, 42% [P = 0.70]). The indications for strabismus surgery were esotropia (62%), exotropia (33%), and hypertropia (4%). Infants who underwent cataract surgery at a younger age were less likely to undergo strabismus surgery (28-48 days, 12/50 [24%]; 49-210 days, 33/64 [52%]; P = 0.0037). Of the 42 patients who underwent strabismus surgery, 14 (33%) had a postoperative distance alignment within 8(Δ) of orthotropia at age 5 years. The 5-year visual acuity of children with strabismus was the same whether or not strabismus surgery had been performed (1.10 logMAR with surgery vs 1.00 without [P = 0.71]). CONCLUSIONS: In this study cohort, cataract surgery performed in the first 6 weeks of life was associated with a reduced frequency of strabismus surgery. Strabismus surgery outcomes in this population are guarded. Surgical improvement of strabismus does not appear to influence long-term visual acuity.

Bronstad MP, Peli E, Liu R, Doherty A, Fulton AB. High prevalence of strabismic visual field expansion in pediatric homonymous hemianopia. PLoS One 2018;13(12):e0209213.Abstract
If homonymous hemianopia develops in childhood it is frequently accompanied by strabismus. In some of these cases the strabismus increases the size of the binocular visual field. We determined how prevalent visual-field-expanding strabismus is in children who have homonymous hemianopia. Medical records were examined from 103 hemianopic patients with exotropia (XT) or esotropia (ET). For each participant, we determined whether their strabismus was in a direction that resulted in visual field expansion (i.e. left exotropia with left homonymous hemianopia). Ages at which hemianopia and strabismus were first noted were compared to determine which developed first. The prevalence of XT (24%) and ET (9%) with homonymous hemianopia were both much higher than in the general population (1.5% and 5%, respectively). More strabismic eyes pointed to the blind than seeing side (62 vs 41, 60% vs. 40%, p = 0.02). Exotropic eyes were five times more likely to point to the blind side than esotropic eyes (85% vs 15%). Strabismus, especially exotropia, is much more common in pediatric homonymous hemianopia than in the general population. The strabismus is significantly more often in a visual field-expanding direction. These results support an adaptive role for the strabismus. Patients with HH and exotropia or esotropia should be aware that their visual field could be reduced by strabismus surgery.
Cestari DM, Freire MV, Chun BY. Vertical rectus muscle recession versus combined vertical and horizontal rectus muscle recession in patients with thyroid eye disease and hypotropia. J AAPOS 2018;Abstract
PURPOSE: To compare the postoperative vertical drift in patients with thyroid eye disease (TED) with hypotropia who underwent vertical rectus recession alone versus recession combined with horizontal rectus recession. METHODS: The medical records of patients with TED who underwent strabismus surgery for hypotropia between 2006 and 2015 were reviewed retrospectively. Patients were divided into two groups: group 1 underwent vertical rectus recession only; group 2 underwent vertical rectus recession plus horizontal rectus recession. Data collection included pre- and postoperative deviation measurements and amount of surgical recession performed. The amount of postoperative vertical drift between groups was compared. RESULTS: Of 67 patients who underwent surgery during the study period, 18 met inclusion criteria, 9 in each group. Mean postoperative hypotropia was 24.2 in group 1 and 24.5 in group 2 (P = 0.82). Mean vertical deviations were 0.3 and -2.2 (P = 0.134) on postoperative day 1 -0.9 and -8.0 (P = 0.043) at final follow-up for groups 1 and 2. Mean postoperative vertical drift toward hypertropia was 1.2 in group 1 and 6.8 in group 2 (P = 0.048). The surgical success rate for group 1 was superior to that for group 2 (89% vs 67% [P = 0.024]). CONCLUSIONS: There was a significantly larger postoperative vertical drift in TED patients with hypotropia who had combined vertical rectus and horizontal rectus recessions compared with those who underwent vertical rectus recession alone.
Chan KW, Deng L, Weissberg EM. Detection of Strabismus by Non-Health Care Professionals in an Ethnically Diverse Set of Images. JAMA Ophthalmol 2016;134(1):30-6.Abstract

IMPORTANCE: Understanding the criteria for when strabismus becomes detectable by non-health care professionals could influence the goals for determining the success of surgical intervention and how patients with such misalignments are counseled. OBJECTIVE: To examine the magnitude at which strabismus is detectable by lay observers in an ethnically diverse set of images. DESIGN, SETTING, AND PARTICIPANTS: Photographs of 12 ethnically diverse models (black, white, and Asian) were simulated to have strabismus from esotropia of 21 prism diopters (∆) to exotropia of 21∆. From July 1, 2007, to October, 1, 2008, images were presented to 120 non-health care professionals aged 21 years or older from the general community in Boston, Massachusetts, who were asked whether strabismus was present. Analysis was conducted from November 1, 2008, to March 31, 2009. MAIN OUTCOMES AND MEASURES: The threshold angle for detecting strabismus to enable 70% of lay observers to make a positive determination whether strabismus is present. RESULTS: In white and black models, the threshold allowing a 70% positive detection rate was higher for esotropia than for exotropia (P < .001 for both). For white models, the threshold was 23.2∆ (95% CI, 21.0∆ to 26.5∆) for esotropia and 13.5∆ (95% CI, 12.5∆ to 14.6∆) for exotropia. For black models, the threshold was 20.8∆ (95% CI, 19.2∆ to 22.2∆) for esotropia and 16.3∆ (95% CI, 15.5∆ to 17.2∆) for exotropia. Asian models showed an opposite trend, with the threshold allowing a 70% positive detection rate for esotropia (14.3∆; 95% CI, 13.2∆ to 15.7∆) being lower than that for exotropia (20.9∆; 95% CI, 18.0∆ to 24.6∆) (P < .001). CONCLUSIONS AND RELEVANCE: Esotropia was easier for lay observers to detect than exotropia in Asian models, and exotropia was easier to detect than esotropia in white and black models. This information should be considered when managing patients who have concerns about the social significance of their strabismus. Future studies should include diverse individuals and make an effort to account for individual factors that may alter the perception of strabismus.

Chang Y-H, Melvin P, Dagi LR. Goal-determined metrics to assess outcomes ofexotropia surgery. J AAPOS 2015;Abstract

PURPOSE: To present a goal-determined methodology for monitoring outcomes after surgery for exotropia. METHODS: The goal-determined metric required surgeons to rank four possible goals preoperatively: (1) binocular potential, (2) restoration of eye contact, (3) diplopia control; and (4) torticollis management. Potential preoperative risk factors were noted. Goal-specific outcomes criteria were applied to the latest sensory-motor examination, 2-6 months after surgery. The medical records of patients who underwent surgery from 2007 to 2012 were retrospectively reviewed with respect to the goal-directed metric. RESULTS: A total of 852 patients were evaluated in the study period: 411 for restoration of eye contact; 347 for binocular potential; 78 for diplopia resolution; and16 for torticollis management. Excellent (62%) or good (16%) outcomes were achieved in 78%. Procedures to resolve diplopia (OR, 6.56; 95% CI, 3.39-12.68) and to restore eye contact (OR, 3.74; 95% CI, 2.65-5.29) were more likely to result in excellent outcomes than procedures to improve binocular potential. Simultaneous surgery for dissociated vertical deviation (OR, 0.38; 95% CI, 0.16-0.92) and preoperative near deviation ≥50(Δ) (OR, 0.27; 95% CI, 0.17-0.42) limited likelihood of an excellent outcome. Outcomes monitored by simultaneous rather than alternate prism and cover test were more likely graded excellent (OR, 5.16; 95% CI, 3.50-7.62). Applying motor criteria from the binocular potential goal to the entire cohort diminished putative outcomes (P < 0.001). CONCLUSIONS: Goal-determined metric monitoring outcomes of exotropia surgery provides outcomes germane to the reason for intervention, enables analysis of risk factors affecting outcomes, and facilitates reporting on heterogeneous populations.

Cheng W, Lynn MH, Pundlik S, Almeida C, Luo G, Houston K. A smartphone ocular alignment measurement app in school screening for strabismus. BMC Ophthalmol 2021;21(1):150.Abstract
BACKGROUND: Strabismus is the leading risk factor for amblyopia, which should be early detected for minimized visual impairment. However, traditional school screening for strabismus can be challenged due to several factors, most notably training, mobility and cost. The purpose of our study is to evaluate the feasibility of using a smartphone application in school vision screening for detection of strabismus. METHODS: The beta smartphone application, EyeTurn, can measure ocular misalignment by computerized Hirschberg test. The application was used by a school nurse in a routine vision screening for 133 elementary school children. All app measurements were reviewed by an ophthalmologist to assess the rate of successful measurement and were flagged for in-person verification with prism alternating cover test (PACT) using a 2.4Δ threshold (root mean squared error of the app). A receiver operating characteristic (ROC) curve was used to determine the best sensitivity and specificity for an 8Δ threshold (recommended by AAPOS) with the PACT measurement as ground truth. RESULTS: The nurse obtained at least one successful app measurement for 93% of children (125/133). 40 were flagged for PACT, of which 6 were confirmed to have strabismus, including 4 exotropia (10△, 10△, 14△ and 18△), 1 constant esotropia (25△) and 1 accommodative esotropia (14△). Based on the ROC curve, the optimum threshold for the app to detect strabismus was determined to be 3.0△, with the best sensitivity (83.0%), specificity (76.5%). With this threshold the app would have missed one child with accommodative esotriopia, whereas conventional screening missed 3 cases of intermittent extropia. CONCLUSIONS: Results support feasibility of use of the app by personnel without professional training in routine school screenings to improve detection of strabismus.
Dagi LR, MacKinnon S, Zurakowski D, Prabhu SP. Rectus muscle excyclorotation and V-pattern strabismus: a quantitative appraisal of clinical relevance in syndromic craniosynostosis. Br J Ophthalmol 2017;101(11):1560-1565.Abstract
PURPOSE: V-pattern strabismus observed with syndromic craniosynostosis has been attributed to disparate causes. We compared severity of V pattern with degree of excyclorotation of rectus muscles to appraise significance of this proposed aetiology. METHODS: 43 patients with Apert, Crouzon or Pfeiffer syndrome referred to Boston Children's Hospital Department of Ophthalmology were identified. 28 met inclusion criteria for retrospective cohort study, specifically: (1) sensorimotor measurements in minimum of seven cardinal gazes, (2) quantified fundus torsion and (3) orbital CT imaging sufficient to measure rectus muscle cyclorotation in coronal and quasicoronal planes, posteriorly (near orbital apex) and anteriorly (near pulleys). Patients were placed in one of four V-pattern severity groups. The most severe group demonstrated inability to elevate abducted eye above midline with characteristic 'seesaw' misalignment during horizontal saccades. Rectus muscle cyclorotation was measured by paediatric neuroradiologist blinded to group placement. Primary outcome was correlation of severity of V pattern with degree of excyclorotation. Secondary outcome was correlation of severity with craniosynostosis syndrome. RESULTS: Increasing severity of V pattern correlated with greater excyclorotation in anterior coronal (p=0.009), anterior quasicoronal (p=0.021), posterior coronal (p=0.014) and posterior quasicoronal (p=0.040) planes for moderate-to-severe V pattern. Even greater excyclorotation was associated with seesaw V pattern in anterior quasicoronal (p=0.004) and posterior quasicoronal (p=0.001) views. Highly significant association was found between Apert syndrome and severity of V pattern (p=0.004). CONCLUSIONS: Severity of V pattern is associated with magnitude of excyclorotation. More severe V pattern and seesaw strabismus noted with Apert syndrome may relate to distinctive orbital morphology.
Dagi LR, Elhusseiny AM. Adjustable graded augmentation of superior rectus transposition for treatment of abducens nerve palsy and Duane syndrome. J AAPOS 2020;Abstract
PURPOSE: To report the results of adjustable graded augmentation of superior rectus transposition, a novel modification of superior rectus transposition (SRT) designed to reduce postoperative vertical or torsional diplopia. METHODS: The medical records of patients who underwent adjustable graded augmentation of SRT with or without adjustable medial rectus recession (MRc) from February 2017 to December 2019 were reviewed retrospectively. A Mendez ring was used to monitor torsional change after transposition of the superior rectus muscle to the lateral rectus muscle and after sequential placement of 2 or 3 augmentation sutures by superior rectus-lateral rectus loop myopexy. If excessive mechanical intorsion was induced, the responsible augmentation suture was severed intraoperatively. If torsional or vertical diplopia was noted after recovery, the distal-most augmentation suture was cut. Exotropia was managed by severing the distal-most augmentation suture or by medial rectus adjustment. RESULTS: A total of 8 patients who underwent adjustable graded augmentation of SRT were included (6 using the 3-suture technique): 3 for esotropic Duane syndrome, 2 for abducens nerve palsy, 1 for Moebius syndrome, and 2 for combined trochlear and abducens nerve palsies. Of the 8 patients, 4 had prior strabismus surgery, and 1 patient had previously undergone treatment with botulinum toxin. Severing one augmentation suture in 3 cases resolved vertical (n = 2) or torsional (n = 1) diplopia and consecutive exotropia (n = 1), resulting in excellent alignment and reduction of torticollis to <4° in 7 cases. The technique proved insufficient in 1 patient, who had undergone 3 prior strabismus procedures. CONCLUSIONS: In this study cohort, adjustable graded augmentation of SRT effectively managed the risk of postoperative vertical or torsional diplopia.
Dagi LR, Velez FG, Archer SM, Atalay HT, Campolattaro BN, Holmes JM, Kerr NC, Kushner BJ, MacKinnon SE, Paysse EA, Pihlblad MS, Pineles SL, Strominger MB, Stager DR, Stager D, Capo H. Adult Strabismus Preferred Practice Pattern®. Ophthalmology 2020;127(1):P182-P298.
Dorr M, Kwon MY, Lesmes LA, Miller A, Kazlas M, Chan K, Hunter DG, Lu Z-L, Bex PJ. Binocular Summation and Suppression of Contrast Sensitivity in Strabismus, Fusion and Amblyopia. Front Hum Neurosci 2019;13:234.Abstract
: Amblyopia and strabismus affect 2%-5% of the population and cause a broad range of visual deficits. The response to treatment is generally assessed using visual acuity, which is an insensitive measure of visual function and may, therefore, underestimate binocular vision gains in these patients. On the other hand, the contrast sensitivity function (CSF) generally takes longer to assess than visual acuity, but it is better correlated with improvement in a range of visual tasks and, notably, with improvements in binocular vision. The present study aims to assess monocular and binocular CSFs in amblyopia and strabismus patients. : Both monocular CSFs and the binocular CSF were assessed for subjects with amblyopia ( = 11), strabismus without amblyopia ( = 20), and normally sighted controls ( = 24) using a tablet-based implementation of the quick CSF, which can assess a full CSF in <3 min. Binocular summation was evaluated against a baseline model of simple probability summation. : The CSF of amblyopic eyes was impaired at mid-to-high spatial frequencies compared to fellow eyes, strabismic eyes without amblyopia, and control eyes. Binocular contrast summation exceeded probability summation in controls, but not in subjects with amblyopia (with or without strabismus) or strabismus without amblyopia who were able to fuse at the test distance. Binocular summation was less than probability summation in strabismic subjects who were unable to fuse. : We conclude that monocular and binocular contrast sensitivity deficits define important characteristics of amblyopia and strabismus that are not captured by visual acuity alone and can be measured efficiently using the quick CSF.
Elhusseiny AM, Gore C, Sadiq MAA, Dagi LR, Kazlas M, Hunter DG. Self-grading effect of inferior oblique myectomy and recession. J AAPOS 2020;Abstract
PURPOSE: To evaluate the outcomes of inferior oblique (IO) weakening surgery, whether recession or myectomy, and to assess the dose-response relationship and correlation with angle of preoperative hypertropia. METHODS: The medical records of all patients with vertical deviation in primary gaze who underwent unilateral IO-weakening surgery, either recession or myectomy, at Boston Children's Hospital over an 8-year period with a minimum postoperative follow-up of 1 month were reviewed retrospectively. Outcome measures were effect of IO weakening surgery on vertical deviation in primary gaze and its correlation with the preoperative angle of hyperdeviation. Secondary outcomes included resolution of abnormal head posture, reduction of ocular torsion, and postoperative under- and overcorrection RESULTS: A total of 94 patients were identified (mean age at surgery, 29.3 ± 19.8 years; range, 1-69). The mean postoperative follow-up period was 17.2 ± 15 months. IO recession was performed in 30 patients; IO myectomy, in 64. Surgical success in primary position was achieved in 72 patients (77%), with resolution of anomalous preoperative head posture in 93%. The mean effect on alignment in primary position was 11.3 ± 6.8. The response to IO-weakening surgery was strongly correlated with the preoperative hyperdeviation for both recession (R = 0.53) and myectomy (R = 0.87). CONCLUSIONS: As with other types of strabismus surgery, IO weakening has a "self-grading" contribution, in which the surgical effect strongly correlates with the magnitude of preoperative deviation. A large range of vertical misalignment can be corrected with the same surgical approach.
Elhusseiny AM, Huynh EM, Dagi LR. Evaluation and Management of V pattern Strabismus in Craniosynostosis. J Binocul Vis Ocul Motil 2019;:1-6.Abstract
V pattern strabismus is the most common ocular motor disorder reported in patients with craniosynostosis. Strabismus management may prove challenging, and few studies provide perspective on surgical approach. The purpose of this review is to discuss evaluation and surgical options for treating V pattern strabismus in patients with craniosynostosis. We provide a step-by-step approach to facilitate surgical planning.
Elhusseiny AM, Grush A, Dagi LR. Acute, severe dystonia after strabismus surgery in a patient on propofol, ondansetron, and bupropion. J AAPOS 2020;
Escuder AG, Hunter DG. The Role of Botulinum Toxin in the Treatment of Strabismus. Semin Ophthalmol 2019;:1-7.Abstract
: To perform a systematic review of the application of botulinum toxin A (BTA) in the management of strabismus in the adult and pediatric populations. : A systematic literature search was performed using the Medline database. : In 1989, with the FDA approval of botulinum toxin (onabotulinum toxin A, or BTA) for the treatment of strabismus, patients were provided with an alternative to surgical recession. In this review, we discuss the uses of BTA in the treatment of acute onset comitant esotropia or smaller angle esotropia and as an adjunct to surgery for larger angle esotropia or sixth nerve palsy. Its uses are also explored in intermittent exotropia and vertical strabismus, including thyroid-associated orbitopathy, fourth nerve palsies, and other orbital pathology. : Despite its transient kinetics, BTA can have permanent effects on ocular alignment, promoting binocularity and reduction of diplopia, and can serve as a primary treatment or a muscle sparing option in patients at risk of anterior segment ischemia or need for future surgeries.
Graeber CP, Hunter DG. Changes in Lateral Comitance After Asymmetric Horizontal Strabismus Surgery. JAMA Ophthalmol 2015;133(11):1241-6.Abstract

IMPORTANCE: Asymmetric horizontal strabismus surgery is often performed to correct primary gaze alignment without considering the symptoms that may result from misalignment in the patient's side gaze. Surgical choices influence alignment in side gaze and may contribute to functional and social deficits. OBJECTIVE: To identify the surgical procedures associated with changes of alignment in side gaze to help inform surgical planning for patients with horizontal strabismus. DESIGN, SETTING, AND PARTICIPANTS: The medical records of 1081 horizontal strabismus surgical procedures that were performed at Boston Children's Hospital during a 2-year period were retrospectively reviewed. Only records with strabismus measurements recorded in the right and left gaze before and after surgery were included. Data analysis was conducted from September 1, 2012, through June 7, 2015. MAIN OUTCOMES AND MEASURES: Change in comitance (CIC), determined by measuring the horizontal comitance (the difference between right- and left-gaze strabismus measurements) before and after surgery. RESULTS: The review identified 569 patients who met the inclusion criteria. Of the 491 patients with comitant preoperative alignment, 59 developed postoperative incomitance, of whom 53 (89.9%) had asymmetric surgery. Of the 78 patients with incomitant preoperative alignment, 36 patients' (46.2%) deviation had improved to comitance after surgery; 32 (88.9%) of these patients had asymmetric surgery. Asymmetric 2-muscle surgery had a median CIC of 4.0 while symmetric 2-muscle surgery had a median CIC of 1.5 (difference in CIC, 2.5; 95% CI, 2.0-3.0; P < .001). A CIC of 25 prism diopters or more was observed in 6 patients who underwent asymmetric surgery (0 with symmetric surgery). New postoperative incomitance was symptomatic in at least 17 patients (28.8%). CONCLUSIONS AND RELEVANCE: Asymmetric strabismus surgery can treat incomitant deviations, but it can also create symptomatic incomitant deviations in patients who were previously comitant. Surgical planning should include consideration of the potential for CIC, including the potential for unsatisfactory appearance in side gaze. Patients with binocular vision will be sensitive to diplopia in any gaze direction; in such cases, the consequences of asymmetric surgery should be considered with particular care.

Guhan S, Peng S-L, Janbatian H, Saadeh S, Greenstein S, Al Bahrani F, Fadlallah A, Yeh T-C, Melki SA. Surgical adhesives in ophthalmology: history and current trends. Br J Ophthalmol 2018;Abstract
Tissue adhesives are gaining popularity in ophthalmology, as they could potentially reduce the complications associated with current surgical methods. An ideal tissue adhesive should have superior tensile strength, be non-toxic and anti-inflammatory, improve efficiency and be cost-effective. Both synthetic and biological glues are available. The primary synthetic glues include cyanoacrylate and the recently introduced polyethylene glycol (PEG) derivatives, while most biological glues are composed of fibrin. Cyanoacrylate has a high tensile strength, but rapidly polymerises upon contact with any fluid and has been associated with histotoxicity. Fibrin induces less toxic and inflammatory reactions, and its polymerisation time can be controlled. Tensile strength studies have shown that fibrin is not as strong as cyanoacrylate. While more research is needed, PEG variants currently appear to have the most promise. These glues are non-toxic, strong and time-effective. Through MEDLINE and internet searches, this paper presents a systematic review of the current applications of surgical adhesives to corneal, glaucoma, retinal, cataract and strabismus surgeries. Our review suggests that surgical adhesives have promise to reduce problems in current ophthalmic surgical procedures.
Heidary G, MacKinnon S, Elliott A, Barry BJ, Engle EC, Hunter DG. Outcomes of strabismus surgery in genetically confirmed congenital fibrosis of the extraocular muscles. J AAPOS 2019;Abstract
PURPOSE: To detail surgical strategy and strabismus outcomes in a genetically defined cohort of patients with congenital fibrosis of the extraocular muscles (CFEOM). METHODS: A total of 13 patients with genetically confirmed CFEOM (via genetic testing for mutations in KIF21A, PHOX2A, and TUBB3) were retrospectively identified after undergoing strabismus surgery at Boston Children's Hospital and surgical outcomes were compared. RESULTS: Age at first surgery ranged from 11 months to 63 years, with an average of 3 strabismus procedures per patient. Ten patients had CFEOM1, of whom 9 had the KIF21A R954W amino acid (AA) substitution and 1 had the M947T AA substitution. Of the 3 with CFEOM3, 2 had the TUBB3 E410K AA substitution, and 1 had a previously unreported E410V AA substitution. CFEOM1 patients all underwent at least 1 procedure to address chin-up posture. Chin-up posture improved from 24° ± 8° before surgery to 10.0° ± 8° postoperatively (P < 0.001). Three CFEOM1 patients developed exotropia after vertical muscle surgery alone; all had the R954W AA substitution. Postoperatively, 1 CFEOM1 patient developed a corneal ulcer. All CFEOM3 patients appeared to have underlying exposure keratopathy, successfully treated with prosthetic replacement of the ocular surface ecosystem (PROSE) lens in 2 patients. CONCLUSIONS: CFEOM is a complex strabismus disorder for which surgical management is difficult. Despite an aggressive surgical approach, multiple procedures may be necessary to achieve a desirable surgical effect. Knowledge of the underlying genetic diagnosis may help to inform surgical management.
Heidary G, Hunter DG. Outcomes of strabismus surgery in genetically confirmed congenital fibrosis of the extraocular muscles. J AAPOS 2020;