A Case of Osteopetrosis with Orbital Inflammation Secondary to Maxillary Osteomyelitis.

Introduction: Osteopetrosis is a rare heritable disorder characterized by increased bone density resulting from osteoclast dysfunction. Major complications include bone fracture, osteomyelitis, anemia, and cranial nerve compression. Optic atrophy can occur due to compression of the optic nerve. Although osteomyelitis of the jaw is a common complication, it rarely occurs in the maxilla. Here, we report a case of a 74-year-old female with osteopetrosis who developed maxillary osteomyelitis, leading to orbital inflammation. Case Presentation: She was referred to our clinic for 2 months of ptosis and swelling of the left eyelid and temporal region. Previous imaging revealed a left intraorbital occupying lesion, but a biopsy of the temporal subcutaneous tissue did not provide a definitive diagnosis. After 7 months, she presented with severe temporal swelling and purulent discharge. Upon examination, maxillary osteomyelitis resulting from caries of the upper jaw was observed. Treatment with oral antibiotics, drainage of the temporal skin fistula, and regular cleaning of the maxillary drainage improved her symptoms. Conclusion: This is a rare case of maxillary osteomyelitis associated with osteopetrosis, causing orbital inflammation.

FIVE-YEAR VISUAL OUTCOME OF TREATMENT FOR RETINOPATHY OF PREMATURITY IN INFANTS WEIGHING <500 G AT BIRTH: A Multicenter Cohort Study From J-CREST.

PURPOSE: To investigate the 5-year treatment outcomes of retinopathy of prematurity in infants <500 g birth weight and compare laser and anti-vascular endothelial growth factor therapies. METHODS: A multicenter retrospective study comprised 24 eyes of 13 patients treated for Type 1 retinopathy of prematurity, followed for 5 years. Initial treatment was laser and anti-vascular endothelial growth factor in 13 and 11 eyes, respectively. Data collected included sex, birth characteristics, retinopathy of prematurity characteristics at the time of treatment, best-corrected visual acuity (BCVA), spherical equivalent, and astigmatism at 5 years posttreatment. RESULTS: Median BCVA was 0.15 logarithm of the minimum angle of resolution (interquartile range, 0.0-0.5). Snellen BCVA was ≥20/40 in 73% and ≥20/20 in 27% of eyes. Median spherical equivalent was -2.37 (interquartile range, -6.1 to -0.1); 75% had myopia (≤-0.5 D), and 25% had high myopia (≤-6.0 D). Median astigmatism was 1.25 (interquartile range, 0.9-3.0); 46% had ≥1.5 D. Anti-vascular endothelial growth factor-treated eyes showed less myopia ( P < 0.009), with no BCVA or astigmatism difference ( P = 0.997, P = 0.271) compared with laser-treated eyes. CONCLUSION: One-quarter of the eyes exhibited good visual acuity (Snellen BCVA of ≥20/20) 5 years after retinopathy of prematurity treatment. Refractive errors were common. Anti-vascular endothelial growth factor therapy may be superior to laser therapy in myopic refractive error.

Comparison of long-term treatment outcomes of laser and anti-VEGF therapy in retinopathy of prematurity: a multicentre study from J-CREST group.

OBJECTIVES: To compare real-world, long-term outcomes of laser and anti-vascular endothelial growth factor (VEGF) therapies in patients with retinopathy of prematurity (ROP). METHODS: This was a multicentre retrospective study. We included 264 eyes of 139 patients treated for type 1 ROP or aggressive ROP (AROP) who were followed for at least 4 years. Laser treatment was initially performed in 187 eyes (the laser group), and anti-VEGF therapy was initially performed in 77 eyes (the anti-VEGF group). We collected data on sex, birth characteristics, zone, stage, and the presence of plus disease at the time of treatment and best-corrected visual acuity (BCVA), spherical equivalent (SE), and ocular complications (amblyopia and strabismus) in patients aged 4-6 years. We investigated the associations between treatment outcomes (BCVA, SE and the presence of amblyopia and strabismus) and influencing factors, including treatment procedure (anti-VEGF or laser therapy), sex, birth characteristics, zone, stage, and the presence of plus disease, using multivariable analysis and logistic regression analyses. RESULTS: The initial treatment procedure was not associated with any specific treatment outcome. Subgroup analysis of patients with zone I ROP revealed that the anti-VEGF-treated eyes had significantly better BCVA and higher SE than laser-treated eyes (p = 0.004, p = 0.009, respectively). Female patients presented significantly better BCVA, less amblyopia and less strabismus than male patients (p < 0.001, p = 0.029, p = 0.008, respectively). CONCLUSIONS: In zone I ROP, anti-VEGF therapy led to better visual acuity and less myopic refractive error than laser treatment.

Effect of foveal morphology on visual acuity in 4-6-year-old children with retinopathy of prematurity: a J-CREST study.

Laser therapy is the most effective treatment considered for retinopathy of prematurity (ROP). We compared the foveal morphology of the retina in eyes with a history of ROP to that of full-term children. This cross-sectional comparative study included 74 patients with a history of ROP, aged 4-6 years. Among them, 41 underwent laser treatment for ROP. The clinical findings and retinal morphology in these patients were compared to that of 33 patients who had spontaneous ROP regression and 30 age-matched full-term controls. All the patients with ROP had 20/40 or better best-corrected visual acuity (BCVA). The foveal thickness was significantly thicker in laser-treated ROP eyes than in regressed ROP eyes and controls. The outer nuclear layer was significantly thicker, and the inner segment (IS) of the photoreceptors and the inner retinal layer were significantly thicker in the laser-treated ROP eyes than that in the control eyes. In the patients with ROP and controls, better BCVA was associated positively with deeper foveal depression, which was associated with a later gestational age. Our results suggest that prematurity and laser treatment affect the foveal morphology and BCVA.

Depth Perception with a Newly Developed Microscope Stereotest.

PURPOSE: We aimed to compare the depth perception under a microscope between binocular and monocular conditions using a newly developed microscopic stereotest (M-stereotest) and examine the effect of anisometropia on depth perception under a microscope. SUBJECTS AND METHODS: Thirty young and healthy subjects were examined. A manipulator attached with a stainless wire was placed under the objective lens of a stereomicroscope. The wire was moved up manually, and the subjects were instructed to stop the knob movement once the wire reached the same height as the fixed two wires. The deviation from the height of the fixed wires was measured under a best optically corrected binocular, a best optically corrected monocular, or an anisometropic binocular [±3 diopters (D) in the dominant or nondominant eye] condition. RESULTS: The deviation was significantly smaller in the binocular condition than in the monocular dominant eye condition (0.38 ± 0.26 mm vs 1.89 ± 1.15 mm, p < 0.001) and was also significantly smaller in the best optically corrected binocular than in the anisometropic binocular condition [1.07 ± 1.00 mm (-3.0 D), p = 0.003; 0.85 ± 0.67 mm (+3.0 D), p < 0.001]. CONCLUSION: Depth perception under the microscope is more accurate under the binocular than in the monocular condition, indicating the potential role of stereopsis. Anisometropia deteriorates depth perception, suggesting that refractive error should be corrected during microscopic work, such as during ophthalmic surgery.

Effect of optical correction on choroidal structure in children with anisohypermetropic amblyopia.

The aim of this study was to assess the effect of wearing optical correction on the choroidal structure in eyes of children with anisohypermetropic amblyopia. This study was conducted at the Nara Medical University Hospital and at the Tokushima University Hospital. Twenty-nine anisohypermetropic amblyopic eyes and their fellow eyes of 29 amblyopic patients (mean age, 5.7 ± 1.7 years, range 3- to 8-years) and twenty eyes of 20 age-similar control children (4.9 ± 0.8 years, range 4- to 6-years) were studied. All patients wore optical correction and 15 patients had both optical correction and patching. The values at the baseline were compared to that at one year later. The binarization method was used to determine the total, luminal, and stromal areas of the choroid in the enhanced depth imaging optical coherence tomographic images. The best-corrected visual acuity (BCVA) of the amblyopic eyes was significantly improved after the one-year period. A large luminal area was characteristic of the amblyopic eye at the baseline, and it was significantly reduced after the optical treatment. The stromal area widened significantly in the amblyopic and fellow eyes after one year whereas there were no significant changes in the choroid of the control eyes after one year. After one-year of optical correction, the luminal/stromal ratios in the amblyopic and fellow eyes were decreased and were then not significantly different from that of the normal control eyes. There was a significant and positive correlation between the improvement of the BCVA and the stromal area at the baseline (r = 0.64, P = 0.001). Wearing corrective lenses on the amblyopic eyes improves the BCVA, and the choroidal structure of the amblyopic eye becomes closer to that of the control eyes. The narrowed luminal area is a specific response of the amblyopic eye associated with the correction of the refractive error. The larger stromal area in the amblyopic eyes at the baseline is a predictive factor for improvements of the BCVA.

Changes in choroidal thickness in healthy pediatric individuals: a longitudinal study.

AIM: To investigate the changes in the choroidal thickness in healthy pediatric children in a longitudinal study, and to determine the ocular and systemic parameters that were significantly correlated with the changes in the choroidal thickness. METHODS: This study included 64 eyes of 34 healthy Japanese children with a mean age (±SD) of 4.4 (±0.4)y (range, 3.6-5.8y) at baseline. Swept-source optical coherence tomography (SS-OCT) was used to record images of the retina and choroid at the baseline and after a mean follow-up period of about 1.5y. The 3D raster scan protocol was used to construct the choroidal thickness map. Mean choroidal thickness was calculated for each of the nine sectors of the Early Treatment Diabetic Retinopathy Study grid. Best-corrected visual acuity, axial length, body height, and weight were also measured. Changes in measurements were defined as the baseline values subtracted from the values at the final visit. A generalized estimating equation was used to eliminate the effect of within-subject intereye correlations. RESULTS: The mean central choroidal thickness was significantly reduced during the follow-up period (baseline, 301.8±8.6 µm; final visit, 286.6±8.0 µm, P<0.001). The decrease in the choroidal thickness was greatest in the central sector, followed by the sectors of the inner and outer rings. The inner and outer rings had diameters of 1 to 3 mm and 3 to 6 mm, respectively. The changes in the choroidal thickness in the central, inner ring, and outer ring sectors were significantly and negatively correlated with the age, baseline body height, baseline body weight, and elongation of the axial length. CONCLUSION: These results indicate that the choroidal thickness among preschool-aged Japanese children decreased significantly during the follow-up period. The choroidal thinning is significantly associated with the elongation of axial length. These characteristics should be considered in the evaluation of choroidal thickness in younger children with retinochoroidal disorders.

Observation of Ciliary Body Changes during Accommodation Using Anterior OCT.

AIM: To evaluate the anatomical changes in the ciliary body (CB) during naturally stimulated accommodation in children using anterior segment optical coherence tomography (OCT). METHODS: This study was a prospective, observational, noncomparative case series included 18 eyes of 9 children (8 males and 1 female) aged from 4 to 10 years. Ciliary body thickness (CBT) was measured temporally by Anterior OCT at 1, 2 and 3 mm posterior to the scleral spur. Measurements were performed in the accommodated state and the unaccommodated state (with cycloplegia). RESULTS: The mean CBT significantly increased by accommodation at 1 mm posterior to the scleral spur from 751 42 to 818 40 µm (P 0.001) and significantly decreased at 2 and 3 mm from 506 66 to 445 59 µm and from 290 54 to 240 50 µm respectively (P 0.001). CONCLUSION: During accommodation, the anterior portion of the ciliary body thickens, while the posterior portion decreases in thickness. These findings imply that the circular ciliary muscles are mainly involved in accommodation and not the longitudinal muscles. J. Med. Invest. 65:60-63, February, 2018.

Effect of optical correction on subfoveal choroidal thickness in children with anisohypermetropic amblyopia.

The purpose of this study was to determine the effect of optical correction on the best-corrected visual acuity (BCVA) and subfoveal choroidal thickness (CT) in the eyes of children with anisohypermetropic amblyopia. Twenty-four anisohypermetropic amblyopic eyes and their fellow eyes of 24 patients and twenty-three eyes of 23 age-matched control children were studied. After one year of optical correction, the BCVA in the anisohypermetropic amblyopic eyes was significantly improved. Before the treatment, the mean subfoveal CT in the amblyopic eyes was 351.9 ± 59.4 µm which was significantly thicker than that of control eyes at 302.4 ± 63.2 µm. After the treatment, the amount of change in the subfoveal CT in the amblyopic and fellow eyes was greater than that in the control eyes. The amblyopic and fellow eyes with thicker choroids had a greater thinning of the choroid whereas eyes with thinner choroids had a greater thickening of the choroid. We conclude that wearing corrective lenses improves the visual acuity, and induces changes of the subfoveal CT in eyes with anisohypermetropic amblyopia.

Diurnal variations in luminal and stromal areas of choroid in normal eyes.

AIMS: To determine the diurnal variations of the luminal and stromal areas of the choroid in normal eyes. METHODS: This was a prospective observational study of 38 eyes of 38 normal subjects. The blood pressure, heart rate, intraocular pressure and enhanced depth imaging optical coherence tomographic (EDI-OCT) images were recorded every 3 hours between 6:00 and 21:00 hours. The horizontal EDI-OCT images of the subfoveal choroid were converted to binary images. The central choroidal thickness (CCT), total cross-sectional choroidal area, the luminal areas, stromal areas and the ratio of luminal area to total choroidal area (L/C ratio) were determined. RESULTS: There were significant diurnal variations in the CCT, total choroidal area, luminal area and L/C ratio with the maximum values at 6:00 hours and the minimum values at 15:00 hours (p<0.001 for the CCT, p=0.011 for the total choroidal area, p<0.001 for the luminal area and p=0.014 for the L/C ratio). There was no significant variation in the stromal area (p=0.216). The range of fluctuation in the CCT was significantly correlated with that in the luminal area and the total choroidal area (p<0.001). However, there was no significant correlation between the fluctuation range in the CCT and that in the stromal area (p=0.095). There was no statistical relationship between the systemic parameters and the choroidal parameters. CONCLUSIONS: The changes in the luminal area are most likely responsible for the diurnal change in the CCT and subfoveal choroidal area. TRIAL REGISTRATION NUMBER: UMIN000019060, Pre-results.

Correction: Choroidal Structure in Children with Anisohypermetropic Amblyopia Determined by Binarization of Optical Coherence Tomographic Images.

[This corrects the article DOI: 10.1371/journal.pone.0164672.].

Choroidal Structure in Children with Anisohypermetropic Amblyopia Determined by Binarization of Optical Coherence Tomographic Images.

PURPOSE: To compare the choroidal structure of the subfoveal area in the eyes of children with anisohypermetropic amblyopia to that of the fellow eyes and to age-matched controls using a binarization method of the images obtained by enhanced depth imaging optical coherence tomography (EDI-OCT). METHODS: This study was performed at Nara Medical University Hospital, Tokushima University Hospital, and Kagoshima University Hospital, Japan. Forty amblyopic eyes with anisohypermetropic amblyopia and their fellow eyes (5.9 ± 2.1 years, mean ± standard deviation), and 103 age-matched controls (6.7 ± 2.4 years) were studied. The control eyes were divided into myopic, emmetropic, and hyperopic eyes. The total choroidal area, luminal area and stromal area of the subfoveal choroid were measured by the binarization method. The luminal/stromal ratio and the axial length of the amblyopic eyes were compared to that of the control eyes. RESULTS: The total choroidal area in the amblyopic eyes was significantly larger than that of the fellow eyes (P = 0.005). The luminal/stromal ratio was significantly larger in the amblyopic eyes than that of the fellow eyes (P<0.001) and the control hyperopic eyes (P<0.001). There was a significant negative correlation between the luminal/stromal ratio and the axial length in the control eyes (r = -0.30, P = 0.001), but no significant correlation was found in the amblyopic eyes. CONCLUSIONS: The choroidal structure of the amblyopic eyes was different from that of the fellow and the control hyperopic eyes. The choroidal changes are related to amblyopia.

Optical Coherence Tomography Reveals New Insights into the Accommodation Mechanism.

Purpose. To evaluate the movement of the anterior and posterior lens poles during naturally stimulated accommodation in children using anterior segment optical coherence tomography (OCT). Methods. This is a prospective, observational, noncomparative case series including 18 eyes of nine children. Analysis of the anterior segment in the accommodated and unaccommodated state (with cycloplegia) was done using anterior segment OCT. The main outcome measures were the position of the anterior and posterior lens poles (in relation to the cornea) and lens thickness (LT). Results. A Statistically significant forward movement of the anterior lens pole and backward movement of the posterior lens pole with an increase in LT were found during accommodation (P < 0.001). There was no significant difference between the degree of movement of the anterior lens pole and the posterior lens pole during accommodation (P = 0.944). Conclusions. Anterior segment OCT provides a rapid noncontact method for studying accommodation in children. The backward movement of the posterior lens pole during accommodation nearly equals the forward movement of its anterior pole. These data minimize the theoretical hydraulic effect of the vitreous during accommodation, adding more support to the capsular theory of Helmholtz.

Macular choroidal thickness and volume in healthy pediatric individuals measured by swept-source optical coherence tomography.

PURPOSE: We evaluated the choroidal thickness and volume in healthy pediatric individuals by swept-source optical coherence tomography (SS-OCT) and compared the findings to those of adults. METHODS: We examined 100 eyes of 100 healthy pediatric volunteers (3-15 years) and 83 eyes of 83 healthy adult volunteers (24-87 years) by SS-OCT with a tunable long wavelength laser source. The three-dimensional raster scan protocol was used to construct a choroidal thickness map. When the built-in software delineated an erroneous chorioscleral border in the B-scan images, manual segmentation was used. RESULTS: The central choroidal thickness and volume within a 1.0-mm circle were significantly larger in the children (260.4 ± 57.2 µm, 0.205 ± 0.045 mm(3)) than in the adults (206.1 ± 72.5 µm, 0.160 ± 0.056 mm(3), both P < 0.0001). In the children, the mean choroidal thickness of the nasal area was significantly thinner than that of all other areas (P < 0.005). Pediatric choroidal thinning with increasing age in the central area was faster than that in the outer areas. Stepwise regression analysis showed that the axial length and body mass index had the highest correlation with the choroidal thickness (R(2) = 0.313, P < 0.0001). CONCLUSIONS: The macular choroidal thickness and volume in the pediatric individuals were significantly larger than those in the adults. The pediatric choroidal thinning with increasing age is more rapid in the central area. Pediatric choroidal thickness is associated with several systemic or ocular parameters, especially the axial length and body mass index. These differences should be remembered when the choroidal thickness is evaluated in pediatric patients with retinochoroidal diseases.

Analyses of the characteristics of potential and cross-talk at each electrode in electro-oculogram.

We placed negative electrodes on the body and positive electrodes at the medial and lateral canthi, measured their potentials separately, evaluated their characteristics, and analyzed cross-talk. We recorded EOG in 6 normal subjects and found the following. The potential at the medial was lower than that at the lateral canthus in the bilateral eyes. The polarity of waves at the lateral was reverse to that at the medial canthus in the bilateral eyes. We recorded EOG in 6 patients with unilateral anophthalmia and found the following. On the anophthalmia side, the potential at the lateral was considerably lower than that at the medial canthus. The polarity of the potential was the same between the medial and lateral canthi. The mean cross-talk to the medial canthus on the anophthalmia side was 8.7%-54.0% of the potential at the medial canthus on the normal side. The mean cross-talk to the lateral canthus on the anophthalmia side was 4.4%-16.9%. The influence of cross-talk of the other eye was marked at the medial but slight at the lateral canthus. In EOG recording, results with minimum errors due to cross-talk can be obtained by paying attention to the potential at the lateral canthus.

Renal failure caused by eyedrops containing phenylephrine in a case of retinopathy of prematurity.

The patient was a low birth weight infant with a history of renal failure. She was referred to our department 29 days after birth to undergo fundus examination. She experienced renal failure after undergoing a mydriatic test and needed medical treatment. Eyedrops containing phenylephrine were instilled several times and additional drops were also instilled during the fundal examination using an eyelid retractor, therefore the blood concentration of the drug was elevated sufficiently to contract the renal vessels, ultimately inducing renal failure. The present case suggests that since the use of mydriatic eyedrops in low birth weight infants could induce renal failure, the following points should be considered: 1) Mydriatic eyedrops should be used with caution by monitoring mydriasis and avoiding excessive instillation; 2) After instillation, the lacrimal region should be compressed to prevent the flow of mydriatic drops to the nasolacrimal canal; and 3) Vital signs should be monitored to check the onset of any adverse reactions for 12 hours after fundal examination.