Ultrawide Field Imaging of Progressive Retinal Arteriovenous Malformation in a Pediatric Patient with Wyburn-Mason Syndrome.

Wyburn-Mason syndrome (WMS) is a rare congenital disease that presents with unilateral arteriovenous malformation (AVM) in the visual pathway, midbrain, and/or skin. We report a case of a 5-year-old girl with a history of cerebral and orbital AVM who presented with left exotropia and was found to have group 3 retinal AVM consistent with WMS. Here, we use ultrawide field imaging to show the progression of retinal AVM and peripheral nonperfusion areas for a period of 1 year in a pediatric patient with WMS. [Ophthalmic Surg Lasers Imaging Retina. 2022;53:46-48.].

Real-World Visual Outcomes of Laser and Anti-VEGF Treatments for Retinopathy of Prematurity.

PURPOSE: To characterize visual outcomes in children screened for retinopathy of prematurity (ROP). DESIGN: Retrospective, interventional case series. METHODS: Patients who received ROP screening examinations at UCLA Medical Centers and were followed with outpatient eye examinations at Stein Eye Institute and/or Doheny Eye Institute (Los Angeles, California) were included. Data were collected on birth characteristics, worst type of ROP, and ROP treatment. Adverse visual outcomes included myopia, strabismus, amblyopia, macular dragging, and optic atrophy. Snellen visual acuity was reported for children 4 years and older. RESULTS: A total of 175 infants (350 eyes) were included for analysis (mean gestational age = 28.2 weeks and birth weight = 1059 g) from a screening population of 539 infants (1078 eyes, 32.4% follow-up) over a 9-year period. Fifteen eyes received primary anti-vascular endothelial growth factor (anti-VEGF) therapy, whereas 59 eyes received primary laser therapy. Primary anti-VEGF therapy, as compared with primary laser treatment, was associated with a decreased incidence of amblyopia (adjusted odds ratio [aOR] = 0.6-0.86, P < .0001) after controlling for gestational age and birth weight. The rates of optic atrophy (P = .79), strabismus (P = .98), and myopia (P = .93) were not different between anti-VEGF and laser treatment groups. Infants receiving anti-VEGF therapy had more posterior disease than laser-treated infants (P = .041). Infants receiving laser therapy were more likely to have severe myopia (aOR = 1.02-1.3, P = .023), amblyopia (aOR = 1.12-1.61, P = .002), and optic atrophy (aOR = 1.01-1.32, P = .045) than infants not treated. CONCLUSION: These findings add to the advantages of anti-VEGF treatment compared with primary laser treatment, particularly in posterior ROP.

Prevalence of Sagging Eye Syndrome in Adults with Binocular Diplopia.

PURPOSE: Sagging eye syndrome (SES), horizontal and/or vertical strabismus caused by orbital connective tissue degeneration, was first defined 10 years ago. This study investigated SES and other causes of acquired binocular diplopia in adults presenting to a single institution since the description of SES. DESIGN: Retrospective observational case series. METHODS: Medical records were reviewed of all new patients over the age of 40 who presented to the Stein Eye Institute with binocular diplopia between January 2015 and December 2018. Clinical causes of diplopia were tabulated in patients grouped by age and sex. In patients with SES, we tabulated binocular alignment, types of treatment, and surgical outcomes. RESULTS: There were 945 patients of mean age 66.5 years, of whom 514 (54.4%) were female. The most common cause of diplopia was SES (31.4%). The 297 patients with SES were older at 71.2 years (P < 0.0001) and more predominantly female at 59.9% than other patients (52.0%; P = 0.023). The relative proportion of SES patients among all diplopic patients increased with age from 4.7% under age 50 years to 60.9% over the age of 90. Age-related distance esotropia was present in 35% and cyclovertical strabismus in 65% of cases of SES. Strabismus surgery was performed in 50% of cases of SES. Mean esotropia at distance decreased from 6.9 ± 0.7Δ preoperatively to 0.3 ± 0.3Δ postoperatively. Preoperative hypertropia decreased from 3.0 ± 0.3Δ to 0.7 ± 0.2Δ postoperatively. Surgery resolved diplopia in all cases. CONCLUSIONS: It is important to recognize that SES is a very common cause of adult binocular diplopia.

Comparative study of lateral rectus recession versus recession-resection in unilateral surgery for intermittent exotropia.

PURPOSE: To compare the outcomes of unilateral lateral rectus recession to unilateral recession-resection in the treatment of patients with intermittent exotropia. METHODS: The medical records of patients with intermittent exotropia with exodeviation of 20(Δ) to 25(Δ) who underwent unilateral lateral rectus recession or recession-resection at a single center from 2002 to 2010 were retrospectively reviewed, and surgical outcomes between groups were compared. RESULTS: Of 70 patients, 37 underwent lateral rectus recession; 33, recession-resection. The mean preoperative exodeviation was 22.2(Δ) ± 2.1(Δ) at distance and 22.3(Δ) ± 3.3(Δ) at near in the lateral rectus group and 24.5(Δ) ± 1.4(Δ) at distance and 26.4(Δ) ± 3.6(Δ) at near in the recession-resection group. Successful surgical outcome was defined as esodeviation of ≤5(Δ) to exodeviation of ≤10(Δ) at distance in primary position. The mean follow-up period was 37.1 months in the lateral rectus group and 44.6 months in the recession-resection group (P = 0.078). The surgical success did not differ significantly between groups at the final follow-up (45.9% in the lateral rectus group and 39.4% in the recession-resection group; P = 0.215). However, posteroperative overcorrection was less common in the lateral rectus group through 12 months' follow-up. CONCLUSIONS: Surgical outcomes at a mean of 3.4 years did not differ significantly between groups. In our study cohort, unilateral lateral rectus recession showed a low risk of overcorrection in the treatment of mild to moderate angle exotropia.

Inferior oblique weakening surgery on ocular torsion in congenital superior oblique palsy.

AIM: To investigate changes in fundus excyclotorsion after inferior oblique myectomy or myotomy. METHODS: The records of 21 patients undergoing strabismus surgery by a single surgeon between 2009 and 2012 were examined. Only patients who had undergone an inferior oblique myectomy or myotomy, with or without horizontal rectus muscle surgery, were evaluated. Digital fundus photographs were obtained, and the angle formed by a horizontal line passing through the optic disc center and a reference line connecting the foveola and optic disc center was measured. Associated clinical factors examined include age at the time of surgery, presence or absence of a head tilt, degree of preoperative vertical deviation, torsional angle, inferior oblique muscle overaction/superior oblique muscle underaction, and surgery laterality. Whether the procedure was performed alone or in combination with a horizontal rectus muscle surgery was also examined. RESULTS: Mean preoperative torsional angle was 12.0±6.4°, which decreased to 6.9±5.7° after surgery (P<0.001, paired t-test). Torsional angle also decreased from 15.1±7.0° to 6.2±4.3° in the myectomy group (P<0.001, paired t-test) but there were no significant changes in the myotomy group (P=0.093, Wilcoxon signed rank test). Multivariable linear regression analysis showed that preoperative torsional angle, degree of inferior oblique overaction, and age at surgery independently and significantly affected postoperative torsional angle. CONCLUSION: Mean torsional angle decreased after inferior oblique myectomy. Degree of preoperative torsional angle, inferior oblique overaction, and age at surgery influence postoperative torsional angle.

Squinting and photophobia in intermittent exotropia.

PURPOSE: To report factors associated with preoperative squinting, defined as transient eye closure in bright light, and photophobia and the factors affecting improvement of these symptoms postoperatively in intermittent exotropia. METHODS: In this retrospective study, patients (N = 99) were divided into groups according to the presence (n = 54) or absence (n = 45) of preoperative squinting and the presence (n = 64) or absence (n = 35) of photophobia. Clinical characteristics, including overaction or underaction of the oblique muscle and fundus intorsion and extorsion, were compared between the two groups. The squinting and photophobia groups were further categorized into two subgroups each according to postoperative improvement. The extended list of characteristics, including the duration from onset to surgery, postoperative angle of deviation, and fusion, was compared between the two subgroups. RESULTS: Preoperatively, 54 (54.5%) and 64 (64.6%) patients had squinting and photophobia, respectively. The coincidence of squinting and photophobia was marginally significant (p = 0.05). Postoperatively, squinting and photophobia disappeared in 64.8 and 59.4% of the patients, respectively. The photophobia group had a younger onset age of strabismus than the nonphotophobia group (39.3 vs. 56.4 months; p = 0.03). Good fusional status at the near range was more common in the nonsquinting group than in the squinting group (74.3 vs. 47.6%; p = 0.02). Superior oblique overaction was significantly more common in the squinting group than in the nonsquinting group (11.1 vs. 0%; p = 0.03). Early surgical correction and successful outcomes were associated with squinting improvement (p = 0.001 and p = 0.02, respectively). CONCLUSIONS: More than 50% of patients with intermittent exotropia had squinting or photophobia, and approximately 60% of symptomatic patients experienced improvement postoperatively. The onset of strabismus, near fusion, superior oblique overaction, and fundus intorsion were related to these symptoms. Early surgery and successful eye position realignment were beneficial for improving squinting postoperatively.

Surgical dose-effect relationship in single muscle advancement in the treatment of consecutive strabismus.

PURPOSE: To evaluate the dose-effect relationship for single muscle advancement in consecutive esotropia and consecutive exotropia. METHODS: Medical records from 22 patients with consecutive esotropia (n = 11) or exotropia (n = 11) were retrospectively reviewed. All patients had undergone either single lateral rectus or medial rectus advancement surgery. The alterations in muscle position and the angle deviation were measured in millimeters and prism diopters (PD) and the change in deviation was determined by subtracting the postoperative angle of deviation at 1 week from the preoperative angle. To quantify the clinical effect of muscle advancement, the ratio of the change in muscle position to the change in visual angle deviation was calculated (ie, the surgical dose-effect relationship). RESULTS: The mean deviation was 25.5 ± 10.4 PD preoperatively and 0 ± 6.9 PD at 1 week postoperatively. The success rate was 82% in the consecutive esotropia group and 91% in the consecutive exotropia group. The average correction ratio was 4.31 ± 0.96 PD/mm. In multiple regression analysis of total patients with consecutive strabismus and the consecutive esotropia group, the amount of muscle advancement and preoperative angle deviation were positively correlated with the correction ratio. In the consecutive exotropia group, there was no significant relationship between variables. CONCLUSIONS: Single muscle advancement generally provides enough correction for most consecutive strabismus cases. Surgical dose-effect relationship increases with preoperative angle deviation and amount of muscle advancement. Surgeons should consider reducing the amount of muscle advancement in patients with larger angle deviations, especially patients with consecutive esotropia.

IIH with normal CSF pressures?

Idiopathic intracranial hypertension (IIH) is a condition of raised intracranial pressure (ICP) in the absence of space occupying lesions. ICP is usually measured by lumbar puncture and a cerebrospinal fluid (CSF) pressure above 250 mm H2O is one of the diagnostic criteria of IIH. Recently, we have encountered two patients who complained of headaches and exhibited disc swelling without an increased ICP. We prescribed acetazolamide and followed both patients frequently; because of the definite disc swelling with IIH related symptoms. Symptoms and signs resolved in both patients after they started taking acetazolamide. It is generally known that an elevated ICP, as measured by lumbar puncture, is the most important diagnostic sign of IIH. However, these cases caution even when CSF pressure is within the normal range, that suspicion should be raised when a patient has papilledema with related symptoms, since untreated papilledema may cause progressive and irreversible visual loss.

Systemic steroid-induced cataracts in children: long-term changes in morphology and visual acuity.

PURPOSE: To report the long-term visual prognosis and surgical rates of steroid-induced cataracts in children. METHODS: The medical records of children diagnosed with steroid-induced cataracts who were followed up for more than 5 years were retrospectively reviewed. Children over 3 years of age who cooperated with the initial visual acuity test (Snellen chart) were included. The following data were evaluated: best-corrected visual acuity at the time of diagnosis and at final follow-up, morphologic changes in the cataract, and surgical outcomes and associated systemic diseases that required steroid treatment. RESULTS: A total of 59 eyes of 30 patients with posterior subcapsular cataracts were included. The mean follow-up period after diagnosis was 7.6 ± 2.9 years (range, 5-14.5 years). Cataracts were graded on the basis of the size of lens opacity. Cataract progression was defined as widening of the lens opacity. Cataracts progressed in 20 eyes (34%) and remained unchanged in 39 eyes (66%). Three patients underwent bilateral cataract surgery because of cataract progression and significant loss of visual acuity. Of the 53 eyes of 27 children (90%) who did not undergo surgery, best-corrected visual acuity did not change significantly during the follow-up period. CONCLUSIONS: In this study cohort, cataract progressed in 34% of the eyes with steroid-induced cataract; however, surgery was required in only 10% of the children, and in these patients the postoperative visual outcome was favorable.