Bilateral Corneal Opacities with Galactokinase Deficiency and Spinal Muscular Atrophy

PURPOSE@#To report a case of bilateral corneal opacities and rare spinal muscular atrophy type III (Kugelberg-Welander disease) in a galactokinase-deficiency patient.CASE SUMMARY: A 6-year-old female presented with bilateral ground glass-like superficial stromal corneal opacities in the corneal center. The patient exhibited developmental motor delay. On muscle biopsy, skeletal muscle neurogenic atrophy consistent with spinal muscular atrophy type III, i.e., Kugelberg-Welander disease, was diagnosed, as well as galactokinase deficiency. On genetic examination, mutation analysis showed an A198V mutation of galactokinase in chromosome 17q24 (“Osaka” variant).@*CONCLUSIONS@#Bilateral corneal opacities and spinal muscular atrophy type III was found in a galactokinase-deficiency patient.

Comparison of Long-term Surgical Outcomes of Two-muscle Surgery in Basic-type Intermittent Exotropia: Bilateral versus Unilateral

PURPOSE: To compare long-term surgical outcomes after bilateral lateral rectus recession (BLR) and unilateral lateral rectus recession-medial rectus resection (RR) for the treatment of basic-type intermittent exotropia. METHODS: Consecutive patients who underwent BLR or RR for treatment of intermittent exotropia between 1999 and 2010 and underwent ≥5 years of follow-up were recruited for this study. Surgical outcomes were grouped according to postoperative angle of deviation: overcorrection (esophoria/tropia >8 prism diopters [Δ]), success (esophoria/tropia ≤8Δ to exophoria/tropia ≤8Δ), and undercorrection/recurrence (exophoria/tropia >8Δ). Outcomes were compared between the BLR group and the RR group at postoperative week 1, months 1 and 6, and years 1, 2, 3, 4, and 5. RESULTS: Of 99 patients, 37 underwent BLR and 62 underwent RR. At postoperative month 6 (97.3% vs. 82.3%, p = 0.045) and year 1 (91.9% vs. 74.2%, p = 0.040), the surgical success rates in the BLR group were significantly higher than in the RR group. Recurrence of exophoria/tropia most commonly occurred between 2 and 3 years after surgery in the BLR group, but continuous recurrences were found in the RR group. At postoperative year 5, the surgical success rate was 54.1% in the BLR group and 41.9% in the RR group (p = 0.403). The reoperation rate was 24.3% in the BLR group and 33.9% in the RR group (p = 0.317). CONCLUSIONS: Surgical outcomes 5 years after surgery for intermittent exotropia were comparable between the BLR and RR groups. The surgical success rate and the reoperation rate were not significantly different between the BLR and RR groups.

Prevention of myopia progression

Myopia is a common disorder affecting 38.7%-73.1% of the total population in East Asian countries. A high prevalence of myopia is associated with an increased risk of sight-threatening problems, such as retinal detachment, retinal tears, glaucoma, and choroidal neovascularization. Slowing progression of myopia could benefit many children in East Asia. However, only a few methods used for myopia control have proven to be effective. The most effective methods are topical pharmaceutical agents such as atropine, orthokeratology lens (Ortho-K lens), and outdoor activity. To minimize its side effects, a lower dose of atropine instillation is recommended. An Ortho-K lens can be a good option in mild myopia. Increasing outdoor activity and light exposure prevent myopia onset and development, as well as axial growth. Conventional methods such as pin-hole glasses, ocular movement exercise, and bifocal or multifocal glasses have all been proven to be ineffective. This lecture provides an overview of the prevention of myopia progression available in the literature, so that we can learn the methods that might lower the risk of sight-threatening complications in myopic children.

Comparison of Surgical Outcomes with Unilateral Recession and Resection According to Angle of Deviation in Basic Intermittent Exotropia

PURPOSE: The purpose of this study is to compare the surgical outcomes and near stereoacuities after unilateral medial rectus (MR) muscle resection and lateral rectus (LR) recession according to deviation angle in basic intermittent exotropia, X(T). METHODS: Ninety patients with basic type X(T) were included in this study. They underwent unilateral recession of the LR and resection of the MR and were followed postoperatively for at least 12 months. Patients were divided into three groups according to their preoperative deviation angle: group 1 or =40 PD. Surgical outcomes and near stereoacuities one year after surgery were evaluated. Surgical success was defined as having a deviation angle range within +/-10 PD for both near and distance fixation. RESULTS: Among 90 patients, groups 1, 2, and 3 included 30 patients each. The mean age in groups 1, 2, and 3 was 9.4 years, 9.4 years, and 11.0 years, respectively. The surgical success rates one year after surgery for groups 1, 2, and 3 were 80.0%, 73.3%, and 73.3% (chi-square test, p = 0.769), respectively. The undercorrection rates for groups 1, 2, and 3 were 16.7%, 23.3%, and 26.7%, and the overcorrection rates were 3.3%, 3.3%, and 0%, respectively. The mean preoperative near stereoacuities for groups 1, 2, and 3 were 224.3 arcsec, 302.0 arcsec, and 1,107.3 arcsec, and the mean postoperative near stereoacuities were 218.3 arcsec, 214.7 arcsec, and 743.0 arcsec (paired t-test; p = 0.858, p = 0.379, p = 0.083), respectively. CONCLUSIONS: In basic X(T) patients, the amount of angle deviation has no influence on surgical outcomes in unilateral LR recession and MR resection. The near stereoacuities by one year after LR recession and MR resection for intermittent X(T) were not different among patient groups separated by preoperative deviation angle.

The Correlation of Differences in the Ocular Component Values with the Degree of Myopic Anisometropia

PURPOSE: To determine the relationship between the differences in the ocular component values with the degree of anisomyopia. METHODS: Refraction, corneal power (CP), and biometry were examined in 50 myopic adults with refractive differences (RD) over 1.50 diopters (D). Ocular components were measured by ultrasound biometry and keratometry. The correlation between the differences in the ocular component values with the degree of anisomyopia was analyzed by linear regression analysis. RESULTS: Among 50 adults with anisomyopia, 5 had RD from 1.50 to 2.99 D, 11 had RD from 3.00 to 3.99 D, 9 had RD from 4.00 to 5.99 D, 12 had RD from 6.00 to 7.99 D, 7 had RD from 8.00 to 11.99 D, and 6 had > or =12.00 D. There was no significant correlation between the ocular components (CP, crystalline lens thickness [LT], and anterior chamber depth [ACD], and the length from the cornea to the posterior surface of the lens [ACD + LT]) and the RD (p > 0.05). The RD showed a significantly positive correlation with vitreous chamber depth (VCD), and axial length (r = 0.963, p < 0.0001). CONCLUSIONS: The severity of anisomyopia was not correlated with the between-eye differences in the anterior chamber values of the eye (CP, ACD, LT, ACD + LT). The severity of anisomyopia was significantly correlated with the between-eye differences in VCD.

Clinical Evaluation of Excyclotorsion in Patients with Primary Inferior Oblique Overaction

PURPOSE: To evaluate the amount of excyclotorsion according to degree of inferior oblique overaction (IOOA) in patients with primary IOOA. METHODS: Fifty-nine primary IOOA patients who underwent inferior oblique muscle surgery were evaluated. Visual acuity, ocular movement test, prism cover test, Bielschowsky head tilt test, fundus photograph and photographic examination for excyclotorsion were performed. The correlation analysis was performed for the excyclotorsion according to the amount of IOOA (Control group; IOOA < 1, Group 1; 1 < or = IOOA < 2, Group 2; 2 < or = IOOA < 3, Group 3; 3 < or = IOOA < 4, Group 4; IOOA = 4). RESULTS: Excyclotorsion was 7.37 +/- 2.36degrees in the Control group, 9.29 +/- 3.79degrees in Group 1, 10.04 +/- 4.39degrees in Group 2, 17.98 +/- 4.62degrees in Group 3, and 24.70 +/- 4.61degrees in Group 4. The amount of IOOA and excyclotorsion showed a positive correlation (Pearson's correlation coefficient r = 0.675). Asymmetric IOOA was observed in 35 patients (59.3%) and symmetric IOOA was observed in 24 patients (40.7%). Asymmetric IOOA combined with hypertropia was presented in 9 patients (37.5%) and symmetric IOOA combined with hypertropia was presented in 3 patients (8.6%). Therefore, patients with asymmetric IOOA have a higher incidence of vertical diplopia. The amounts of IOOA and excyclotorsion were greater in primary IOOA with esotropia than in subjects with exotropia (p = 0.001). CONCLUSIONS: The excyclotorsion was proportional to the amount of IOOA in Group 3 and Group 4. The aspect of excyclotorsion was widely distributed in Group 1 and Group 2 compared to that in the Control group.