Evaluation of 3D tablet-based stereoacuity test ASTEROID in children with normal and abnormal visual acuity.

PURPOSE: To assess the utility of 3D, tablet-based, glasses-free Accurate STEReotest (ASTEROID) in children compared with the Titmus test. METHODS: Children aged 5-13 years were enrolled in a single-center, nonrandomized, observational comparison study and analyzed by age (5-7 vs 8-13 years) and visual acuity (20/25 or better in both eyes vs abnormal). Each participant underwent both the ASTEROID and Titmus stereoacuity tests. Stereoacuity was defined as fine (≤60 arcsec), moderate (61-200 arcsec), coarse (201-1199 arcsec), or very coarse to nil (≥1200 arcsec). Agreement between the tests was assessed using a weighted kappa (κ) statistic based on all four categories. RESULTS: A total of 112 children were included: 28 aged 5-7 with normal visual acuity, 30 aged 5-7 with abnormal visual acuity, 34 aged 8-13 with normal visual acuity, and 20 aged 8-13 with abnormal visual acuity. Mean ASTEROID score was 688 ± 533 arcsec (range, 13-1200 arcsec). Agreement between ASTEROID and Titmus test scores for participants overall was moderate (κ = 0.52). By subgroup, agreement was fair for children 5-7 with abnormal visual acuity (κ = 0.31), moderate for children 5-7 with normal visual acuity (κ = 0.47) and children 8-13 with normal visual acuity (κ = 0.42), and substantial for children 8-13 with abnormal visual acuity (κ = 0.76). Where ASTEROID and Titmus score group varied, ASTEROID score was poorer in 94% (47/50) of cases. CONCLUSIONS: ASTEROID is a digital, tablet-based test that evaluates global stereopsis, does not require glasses, and provides a continuum of scores. Among children, ASTEROID has good agreement with the Titmus test; however, it may be more sensitive at detecting stereovision deficits. Further study is necessary to determine which test is more accurate.

Inferior Rectus Muscle Y-Split for Thyroid-Related Vertical Strabismus.

Incomitant hypotropia in thyroid eye disease can be difficult to manage, especially in the presence of orthotropia with fusion in down gaze and reading position. Recessing the affected ipsilateral inferior rectus muscle may result in an undesirable downgaze diplopia secondary to a hypertropia in downgaze. Various surgical techniques have been described to manage this potential complication including asymmetric recession of both inferior rectus muscles, posterior myoscleropexy operation, and the Scott recess/resect procedure of the contralateral inferior rectus. In 2004, Hoerantner et al. introduced the y-split recession of the medial rectus muscle for near esotropic deviations. The anterior portion of muscle is split and secured in a y-shaped configuration, which reduces the muscle lever arm and helps minimize incomitance and muscle slippage. Unlike the traditional Cüppers Faden, a y-split recession results in torque reduction in all gaze positions. In addition, a y-split recession does not involve scleral passes posteriorly reducing the risk of globe perforation. We report a patient with incomitant strabismus secondary to thyroid eye disease who underwent a combination of traditional recession and y-splitting recession of the contralateral inferior rectus muscle, resulting in good functional alignment in primary gaze and in the reading position.

Teprotumumab for Thyroid Eye Disease-related Strabismus.

PURPOSE: To assess and quantify teprotumumab's effect on thyroid eye disease-related strabismus by change in measured horizontal and vertical deviations and change in extraocular motility. METHODS: We reviewed a series of patients with thyroid eye disease-related strabismus treated with teprotumumab. Exclusion criteria included age under 18 years, strabismus of alternate etiology, or thyroid eye disease-related reconstructive surgery during the treatment course. Primary outcomes were absolute (prism diopters) and relative (%) differences in horizontal and vertical deviations in primary position at distance, as well as change in ductions of the more affected eye. Secondary outcomes included incidence and timing of strabismus surgery postteprotumumab. RESULTS: Thirty-one patients were included, with mean age 63 years and thyroid eye disease duration 10 months. After teprotumumab, there was 6 prism diopters (39%) mean reduction in vertical deviation (p < 0.001), without significant change in mean horizontal deviation (p = 0.75). Supraduction, abduction, adduction, and infraduction significantly improved in the more restricted eye (p < 0.01, p < 0.01, p = 0.04, and p = 0.01, respectively). Thirty-five percent of patients underwent strabismus surgery posttreatment, at an average 10 months after last infusion. CONCLUSIONS: Teprotumumab produced a statistically significant reduction in vertical but not horizontal strabismus angles in primary position at distance. Extraocular motility in all 4 ductions also improved. A substantial minority of patients still required strabismus surgery following teprotumumab.

Outcomes of Strabismus Surgery Following Teprotumumab Therapy.

PURPOSE: To investigate the results of patients undergoing surgical treatment for strabismic diplopia in thyroid eye disease (TED) following teprotumumab. DESIGN: Multicenter, retrospective, case series. METHODS: We report 28 patients who underwent extraocular muscle surgery for strabismic diplopia after treatment with teprotumumab at 7 different academic centers. Elapsed time from last teprotumumab dose to the date of surgery, previous orbital decompression, primary preoperative horizontal and vertical deviation, surgical procedure, and 2-month postoperative results were collected from the patient records. RESULTS: Sixteen (57%) patients were diplopia-free after 1 surgery. Three (11%) chose prism spectacles to correct residual diplopia, 2 (7%) used compensatory head posture to resolve diplopia, and 1 (4%) had intermittent diplopia and was functionally improved (choosing no prisms or further surgery). These were considered treatment successes. Three (11%) patients required reoperation, and all were diplopia-free after their second procedure. CONCLUSIONS: Most patients requiring surgery for strabismic diplopia following teprotumumab achieve good outcomes with success rates comparable to series published before the availability of teprotumumab.

Dry eye disease in the young: A narrative review.

Dry eye disease (DED), a multifactorial ocular disease that significantly impacts quality of life, is most commonly reported in adults. This review describes the prevalence, risk factors, diagnosis and management of DED in children. A literature search, conducted from January 2000-December 2022, identified 54 relevant publications. Using similar diagnostic criteria to those reported in adults, namely standardized questionnaires and evaluation of tear film homeostatic signs, the prevalence of DED in children ranged from 5.5% to 23.1 %. There was limited evidence for the influence of ethnicity in children, however some studies reported an effect of sex in older children. Factors independently associated with DED included digital device use, duration of digital device use, outdoor time and urban living, Rates of DED were higher in children with ocular allergy and underlying systemic diseases. Compared with similar studies in adults, the prevalence of a prior DED diagnosis or a diagnosis based on signs and symptoms was lower in children, but symptoms were commonly reported. Treatment options were similar to those in adults, including lifestyle modifications, blinking, management of lid disease and unpreserved lubricants in mild disease with escalating treatment with severity. Management requires careful exploration of symptoms, medical history and the diagnosis and management of ocular comorbidities such as allergy and anterior blepharitis. Appropriately powered population-based studies are required to understand the prevalence of and risk factors for DED in children. Development of age-appropriate thresholds for signs and symptoms of DED would support better diagnosis of disease and understanding of natural history.

Anterior segment ischemia following strabismus surgery in a young adult using gender-affirming estrogen hormone therapy.

Well-known risk factors for anterior segment ischemia (ASI) following strabismus surgery include ipsilateral surgery on three or more rectus muscles, older age, and vasculopathy. ASI is rarely reported in young patients following uneventful strabismus surgery on two ipsilateral rectus muscles. We report a 30-year-old transgender female on long-term estrogen therapy who underwent strabismus surgery involving recessions of both lateral rectus muscles, the right inferior rectus muscle, and the left superior rectus muscle. The left eye developed severe ASI with hypotony maculopathy that was resistant to topical medications, oral steroids, anterior chamber reformation, and intravitreal steroid injection. Following phacoemulsification with intraocular lens and capsular tension ring insertion 1 year later, intraocular pressure and hypotony maculopathy improved.

Optical Coherence Tomography Findings in Cherry-Red Spot: Implications for Understanding Pathophysiology and Visual Prognosis.

PURPOSE: To report optical coherence tomography (OCT) findings of cherry-red spots from Tay-Sachs and Niemann-Pick disease. METHODS: Consecutive patients with Tay-Sachs and Niemann-Pick disease evaluated by the pediatric transplant and cellular therapy team, for whom a handheld OCT scan was obtained, were included. Demographic information, clinical history, fundus photography, and OCT scans were reviewed. Two masked graders evaluated each of the scans. RESULTS: The study included 3 patients with Tay-Sachs disease (5, 8, and 14 months old) and 1 patient with Niemann-Pick disease (12 months old). All patients had bilateral cherry-red spots on fundus examination. In all patients with Tay-Sachs disease, handheld OCT revealed parafoveal ganglion cell layer (GCL) thickening, increased nerve fiber layer, and GCL reflectivity, and different levels of residual normal signal GCL. The patient with Niemann-Pick disease had similar parafoveal findings, but there was a thicker residual GCL. Sedated visual evoked potentials were unrecordable in all 4 patients despite 3 of them demonstrating normal visual behavior for age. Patients with good vision had relative sparing of the GCL on OCT. CONCLUSIONS: The cherry-red spots in lysosomal storage diseases appear as perifoveal thickening and hyperreflectivity of the GCL on OCT. In this case series, residual GCL with normal signal proved to be a better biomarker for visual function than visual evoked potentials and could be considered for future therapeutic trials. [J Pediatr Ophthalmol Strabismus. 2023;60(6):435-440.].

AAPOS Adult Strabismus Committee's Report on the Status of Adult Strabismus Telemedicine.

PURPOSE: To evaluate the utility of telemedicine in the treatment of adult patients with strabismus. METHODS: A 27-question online survey was sent to ophthalmologists of the American Association for Pediatric Ophthalmology and Strabismus (AAPOS) Adult Strabismus Committee. The questionnaire focused on the frequency of telemedicine utilization, the benefits in the diagnosis, follow-up, and treatment of adult strabismus, and barriers of current forms of remote patient visits. RESULTS: The survey was completed by 16 of 19 members of the committee. Most respondents (93.8%) reported 0 to 2 years of experience with telemedicine. Telemedicine was found to be useful for initial screening and follow-up of established patients with adult strabismus, mainly to reduce wait time for a subspecialist visit (46.7%). A successful telemedicine visit could be completed with a basic laptop (73.3%) or a camera (26.7%) or could be assisted by an orthoptist. Most participants agreed that common forms of adult strabismus (cranial nerve palsies, sagging eye syndrome, myogenic strabismus, and thyroid ophthalmopathy) could be examined via webcam. It was easier to analyze horizontal than vertical strabismus. Among the paralytic forms, sixth nerve palsy was the easiest one to assess. Latent forms of strabismus can be partially diagnosed and evaluated using telemedicine, but half of the respondents underlined the importance of in-person examinations in these cases. Sixty-nine percent believed that telemedicine could be a low-cost and time-efficient health service solution. CONCLUSIONS: Most members of the AAPOS Adult Strabismus Committee consider telemedicine to be a useful supplement to the current adult strabismus practice. [J Pediatr Ophthalmol Strabismus. 2023;60(6):386-389.].

A Potentially Adjustable Modification of the Nishida Procedure.

For patients with a complete, chronic abducens nerve palsy and resulting abduction deficit, a transposition procedure is often the procedure of choice. One such transposition procedure involves transposing the superior rectus (SR) and inferior rectus (IR) laterally without disinserting or splitting either muscle. While effective, this procedure - like many transposition procedures - carries with it the risk of induced torsional or vertical misalignment. Here, we describe an adjustable variation of the above transposition procedure, one which potentially would allow for post-operative correction of induced vertical or torsional deviations.

Nasal Transposition of the Split Lateral Rectus Muscle for Strabismus Associated With Bilateral 3rd-Nerve Palsy.

PURPOSE: To determine the success rate and complications associated with nasal transposition of the split lateral rectus muscle (NTSLR) for treating bilateral 3rd-nerve palsy. DESIGN: Retrospective, interventional case series. METHODS: An international, multicenter registry was used for the study. The study population was all patients with bilateral 3rd-nerve palsy treated with NTSLR. Sensorimotor evaluations were conducted before and 6 months after unilateral or bilateral NTSLR. Outcome measures were postoperative horizontal alignment ≤15 prism diopters (PD), intraoperative technical difficulties, and vision-threatening complications. The association of patient demographics and surgical technique with each outcome was analyzed using multivariable logistic regression. RESULTS: A total of 34 patients were included, with a median age of 46 years (interquartile range [IQR] = 25-54 years) at surgery. The most common etiologies were ischemic (29%), neoplastic (15%), and congenital (12%). NTSLR performed unilaterally with alternative surgery on the opposite eye (65%) resulted in a median postoperative exotropia of 18 PD (IQR = 7-35 PD), and when performed bilaterally (35%) resulted in postoperative exotropia of 14 PD (IQR = 5-35 PD). Success was achieved in 50% of cases, intraoperative technical difficulties were reported in 18%, and vision-threatening complications occurred in 21%. Attachment of the lateral rectus muscle ≥10 mm posterior to the medial rectus insertion was associated with increased vision-threatening complications (odds ratio = 9.0; 95% CI = 1.3-99). CONCLUSIONS: NTSLR can address the large-angle exotropia associated with bilateral 3rd-nerve palsy. Surgeons should be aware that posterior placement of the lateral rectus muscle may increase the risk of vision-threatening complications, particularly serous choroidal effusion.

Association of Strabismus With Mood Disorders, Schizophrenia, and Anxiety Disorders Among Children.

Importance: Children with strabismus have poorer functional vision and decreased quality of life than those without strabismus. Objective: To evaluate the association between strabismus and mental illness among children. Design, Setting, and Participants: This cross-sectional study analyzed claims data from the OptumLabs Data Warehouse, a longitudinal deidentified commercial insurance claims database, from 12 005 189 patients enrolled in the health plan between January 1, 2007, and December 31, 2017. Eligibility criteria included age younger than 19 years at the time of strabismus diagnosis, enrollment in the health plan between 2007 and 2018, and having at least 1 strabismus claim based on International Classification of Diseases, Ninth Revision, Clinical Modification and International Statistical Classification of Diseases and Related Health Problems, Tenth Revision, Clinical Modification codes. Controls were children in the same database with no eye disease codes other than refractive error reported. Demographic characteristics and mental illness claims were compared. Statistical analysis was conducted from December 1, 2018, to July 31, 2021. Main Outcomes and Measures: Presence of mental illness claims. Results: Among the 12 005 189 patients (6 095 523 boys [50.8%]; mean [SD] age, 8.0 [5.9] years) in the study, adjusted odds ratios for the association of mental illnesses with strabismus were 2.01 (95% CI, 1.99-2.04) for anxiety disorder, 1.83 (95% CI, 1.76-1.90) for schizophrenia, 1.64 (95% CI, 1.59-1.70) for bipolar disorder, 1.61 (95% CI, 1.59-1.63) for depressive disorder, and 0.99 (95% CI, 0.97-1.02) for substance use disorder. There was a moderate association between each strabismus type (esotropia, exotropia, and hypertropia) and anxiety disorder, schizophrenia, bipolar disorder, and depressive disorder; odds ratios ranged from 1.23 (95% CI, 1.17-1.29) for the association between esotropia and bipolar disorder to 2.70 (95% CI, 2.66-2.74) for the association between exotropia and anxiety disorder. Conclusions and Relevance: This cross-sectional study suggests that there was a moderate association between strabismus and anxiety disorder, schizophrenia, bipolar disorder, and depressive disorder but not substance use disorder. Recognizing that these associations exist should encourage mental illness screening and treatment for patients with strabismus.

Association of Mood Disorders, Substance Abuse, and Anxiety Disorders in Children and Teens With Serious Structural Eye Diseases.

PURPOSE: We sought to evaluate the association between 5 eye diseases (including glaucoma, cataract, congenital optic nerve disease, congenital retinal disease, and blindness/low vision) and mental illness in a pediatric population. DESIGN: Cross-sectional study. METHODS: A de-identified commercial insurance claims database, OptumLabs Data Warehouse, between January 1, 2007, and December 31, 2018, was used. Children and teens less than 19 years of age at the time of eye diagnosis were included. Demographics and mental illness claims were compared, looking at the association of mental illness and eye disease claims. RESULTS: A total of 11,832,850 children and teens were included in this study with mean age of 8.04 ± 5.94 years at the first claim. Of the patients with at least 1 of the 5 eye diseases (n = 180,297), 30.5% had glaucoma (n = 54,954), 9.5% had cataract (n = 17,214), 21.4% had congenital optic nerve disease (n = 38,555), 26.9% had congenital retinal disease (n = 48,562), and 25.9% had blindness or low vision (n = 46,778). There was a statistically significant association, after adjusting for confounding variables, between at least 1 of the 5 eye diseases and schizophrenia disorder (OR = 1.54, 95% CI = 1.48-1.61, P < .001), anxiety disorder (OR = 1.45, 95% CI = 1.43-1.48, P < .001), depressive disorder (OR = 1.27, 95% CI = 1.25-1.29, P < .001), and bipolar disorder (OR = 1.27, 95% CI = 1.21-1.31, P < .001), but a reversed association with substance use disorder (OR = 0.88, 95% CI = 0.86-0.90, P < .001). CONCLUSIONS: We found associations between eye disease in children and teens and mental illness. Understanding these relationships may improve mental illness screening and treatment in the pediatric population.

OPTICAL COHERENCE TOMOGRAPHY AND OPTICAL COHERENCE TOMOGRAPHY ANGIOGRAPHY FINDINGS AND VISUAL PROGNOSIS IN TWO PATIENTS WITH POSTERIOR MICROPHTHALMOS.

BACKGROUND/PURPOSE: Posterior microphthalmos is a rare ocular condition characterized by a disproportionately small posterior segment and multiple retinal abnormalities. The diagnosis can be challenging, and novel findings on optical coherence tomography (OCT) angiography may help to identify this unusual disorder. The purpose of this study is to report OCT and OCT angiography findings in two siblings with posterior microphthalmos. METHODS: Case series at an academic eye institute including two affected siblings with posterior microphthalmos. RESULTS: The results of OCT testing in two siblings with posterior microphthalmos illustrated bilateral papillomacular retinal fold and loss of the foveal depression. Optical coherence tomography angiography findings included loss of the foveal avascular zone and complete vascularization across the fovea. CONCLUSION: Unique OCT angiography findings in posterior microphthalmos are described herein. These findings may support the diagnosis of posterior microphthalmos and aid in the understanding of the pathogenesis of this disease.

Strabismus Surgery Decreases the Risk of Injuries in Pediatric Patients in the OptumLabs Data Warehouse.

PURPOSE: Previous studies have shown an association between injury risk and strabismus in aged Medicare beneficiaries and children. The injury prevalence in strabismic children was 30% in a study of >10 million patients in the OptumLabs Data Warehouse. The purpose of this study was to determine whether strabismus surgery decreases the risk of injury. DESIGN: Retrospective cohort study. METHODS: The OptumLabs Data Warehouse is a deidentified, longitudinal health database that was queried for strabismic patients aged <19 years. The patients who underwent strabismus surgery were compared with those strabismic patients who did not undergo surgery for injury. Injury risk (fractures, musculoskeletal injuries, and head injuries) during their insurance enrollment was calculated in the nonsurgical patients after their first strabismus claim and compared with the risk in surgical patients postoperatively. RESULTS: There were 344,794 patients with strabismus. Surgery was performed in 26,459 (7.7%). Esotropia was the most common diagnosis (n = 181,195, 52.6%) followed by exotropia (n = 161,712, 46.9%) and hypertropia (n = 43,831, 12.7%). Within these groups, patients with hypertropia were significantly more likely to undergo surgery (24.2%) compared with patients with esotropia and exotropia (10.2% and 9.6%, respectively). Injuries were diagnosed after the first strabismus claim in 94,960 (29.8%) nonsurgical patients vs 5790 (21.9%) postsurgical patients (P < .001) with a mean follow-up of 4.3 ± 3.1 vs 3.8 ± 3.1 years, respectively. The adjusted hazard ratio for injuries was 0.85 (95% confidence interval [CI]: 0.83-0.87) for the risk of any injury after strabismus surgery. The hazard ratio was significantly decreased after surgery for each type of strabismus: esotropia (0.91, 95% CI: 0.88-0.94), exotropia (0.82, 95% CI: 0.80-0.85), and hypertropia (0.89, 95% CI: 0.85-0.93). DISCUSSION: Strabismus surgery was associated with a 15% decrease in the risk of physical injury over approximately 4 years of follow-up. Surgery may be a factor in decreasing injury risk in strabismic patients, particularly in exotropia. Given the large number of children with strabismus in the United States, further assessment of strategies such as strabismus surgery to reduce injuries in children with strabismus is needed.

Prevalence of pediatric eye disease in the optumlabs data warehouse.

PURPOSE: To define the prevalence of medical eye disease diagnoses among children enrolled in commercial insurance plans in the United States and to evaluate differences among groups based on the US census region, race/ethnicity, and familial net worth. METHODS: : Retrospective study of de-identified claims data from the OptumLab® Data Warehouse (OLDW) between 2007 and 2018. All children (<19 years) in the OLDW with coverage were studied and those with a claim for a significant eye disease (strabismus, amblyopia, nystagmus or structural eye disorders) with minimum 6-months follow-up were studied. Baseline characteristics were extracted for the calculation of eye disease prevalence, including age, sex, race/ethnicity, region of residence, and family net worth. The prevalence of each type of eye disease was calculated among all children and by baseline characteristics. RESULTS: : 10,759,066 children met the study criteria. The presence of any significant eye diagnosis was 6.7%. Disease was diagnosed more often in whites (6.9%) than blacks (5.6%) and Hispanics (5.9%). The most common eye disease diagnosed was strabismus (3.2%) followed by amblyopia (1.5%). In the North-East region, there was a 10.6% prevalence of any significant eye disease diagnosis, whereas in the Mid-West, it was 7.4% followed by the South and West (5.9% and 5.3%, respectively) (p < .001). There was an increase in eye disease diagnoses with increasing income (5.5% in<$25,000 and 9.4% in >$500,000 household net worth groups, p > .001). CONCLUSION: : Diagnosis of significant eye diseases is relatively common in American children. The most common medical eye disease diagnosis is strabismus. Prevalence of eye disease diagnosis from claims data varies between geographical regions and different income groups. This may reflect differences in healthcare utilization rather than true disease prevalence.

A 2020 Update on 20/20 X 2 Diplopia after Ocular Surgery: Strabismus Following Retinal Detachment Surgery.

Persistent ocular motility and sensorial abnormalities following retinal detachment surgery are not common. Scleral buckling increases the risk of deviation in secondary gaze positions most commonly horizontal deviations. Suspect superior oblique incarceration in patients with limited downgaze. For patients with persistent strabismus, surgery is an option. Removing the exoplant may be necessary to access the extraocular muscles but the effect on alignment is minimal except in cases were the scleral buckle migrates anteriorly causing restriction. Removing the scleral buckle may increase the risk of retinal re-detachment. Hangback recessions and adjustable sutures can be safely used.

Risk of physical injuries in children and teens with ophthalmic diagnoses in the OptumLabs Data Warehouse.

BACKGROUND: To evaluate how eye diseases affect the risk of injuries (fractures, musculoskeletal, head and eye injuries) in children and teens. METHODS: Claims from the OptumLabs Data Warehouse, a longitudinal deidentified commercial insurance claims database, were analyzed. Eligible subjects were aged <19 years at the time of their eye disease diagnosis, enrolled in the health plan between 2007 and 2018, and had >1 visit for >1 significant eye disease (strabismus, amblyopia, nystagmus, structural diseases), based on ICD9/10 codes. Controls were children in the same database who had no eye disease codes reported. Demographics and injury claims (fractures, musculoskeletal injuries, head injuries and eye injuries) were compared. RESULTS: The overall incidence of any subsequent physical injury (even into adulthood) was 29% in eye disease patients and 23% in controls (P < 0.001). After accounting for covariates, the hazard ratio for injury with any type of eye disease was 1.14 (95% CI, 1.13-1.15), 1.17 (95% CI, 1.16-1.18), 0.97 (95% CI, 0.96-0.98), and 1.63 (95% CI, 1.60-1.66) for musculoskeletal injuries, fractures, head injuries, and eye injuries, respectively. The eye disease with the highest adjusted injury risk was nystagmus (HR = 1.26; 95% CI, 1.23-1.28), followed by optic neuritis and pseudotumor cerebri (HR = 1.25). The eye diseases with the lowest risk included amblyopia, esotropia, and glaucoma (HR ≤ 1.06). CONCLUSIONS: There was an increased long-term risk of physical injury among children and teens with eye disease. The clinical significance of these small differences is unclear.

Acute Onset Variable and Progressive Trochlear Nerve Palsy and Ophthalmoparesis Secondary to Bilateral Carotid Cavernous Fistula.

Purpose: To describe a patient who presented with right unilateral white-eyed posteriorly-draining carotid-cavernous fistulas (CCF) causing an isolated, acute-onset trochlear nerve palsy with atypical progression to a contralateral left red-eyed anteriorly-draining CCF associated with orbital congestion.Observation: A 74-year-old female presented with an acute onset incomitant right hypertropia consistent with the clinical diagnosis of a superior oblique palsy. Initial workup including MRI was normal. During a several-week course, she progressed to a comitant deviation. Six-weeks later she developed an episode of severe headache followed by worsening of her diplopia, reverse left hypertropia and left orbital congestive signs. CTA revealed mild opacification of the left cavernous sinus without a concomitantly present superior ophthalmic vein dilatation. A diagnostic angiogram was obtained which revealed a posterior-draining indirect CCF of the right internal carotid artery (ICA) and an anterior-draining indirect CCF of the left ICA. Transvenous Coil embolization resulted in immediate resolution of diplopia, orbital congestion, and headaches.Conclusion: Although it is atypical for a CCF - or other vascular pathology - to present with an isolated fourth nerve palsy, this case illustrates that vascular imaging should be considered in cases of acquired ocular motor paresis associated with headaches and cases with atypical progression despite normal initial cranial imaging.