Coloboma Accompanying Microphthalmos With Orbital Cyst in a Mother and Child.

Microphtalmos with orbital cyst is a rare congenital abnormality of the eye and orbit that is caused by incomplete closure of the embryonic fissure. The cysts project through in a coloboma of the affected eye. It may be sporadic or genetic. Herein, the authors present a 32-year-old mother with unilateral and her 4-month-old daughter with bilateral microphtalmos and accompanying orbital cyst.

Retinal detachment in a child with Frank-ter Haar syndrome.

BACKGROUND: To present a rare case of ocular involvement in a child with Frank-ter Haar syndrome (FTHS) presenting retinal detachment. MATERIALS AND METHODS: Detailed ophthalmological evaluation including examination under general anesthesia, ocular ultrasound, and visual evoked potential testing was completed. Photographic documentation of the physical findings was obtained. RESULTS: A 3-year-old female patient with FTHS was referred to evaluate for possible ophthalmic involvement. The patient presented with the classical dysmorphic abnormalities of the syndrome. Ophthalmologic evaluation revealed a high, against-the-rule corneal astigmatism in the right eye. In the left eye, the red reflex was absent with a suspicious membrane behind the lens, and a sensory exotropia was present. Ultrasonography confirmed retinal detachment with no history of previous trauma. Due to poor visual evoked potentials, no surgery was planned. Astigmatic refractive error was corrected with routine follow-up. CONCLUSIONS: FTHS is associated with multiple ocular involvement such as megalocornea, congenital glaucoma, or colobomas. This case report is the first to describe a high, against-the-rule astigmatism and retinal detachment in a female child with FTHS and demonstrates that an early and detailed ophthalmological examination is essential for these patients.

Evaluation of Choroidal Vascular Index in Amblyopic Patients.

Objectives: To compare subfoveal choroidal thickness (SFCT) and choroidal vascular index (CVI) in patients with hyperopic refractive and strabismic amblyopia and healthy subjects. Materials and Methods: The study included 17 patients with strabismic amblyopia (Group 1), 29 patients with hyperopic refractive amblyopia (Group 2), and 16 eyes of 16 healthy volunteers (Group 3). Best corrected visual acuity was noted in all patients and volunteers. In addition to detailed anterior and posterior segment examinations, macular images were obtained by enhanced-depth imaging mode of optical coherence tomography (OCT). SFCT measurements were made from these images and CVI was calculated using the Image J program. Results: No significant difference was found between the groups in terms of age, gender, and intraocular pressure (p=0.27, 0.64, and 0.85, respectively). Mean BCVAs in Group 1 were 0.57±0.16 (0.3-0.8) in the amblyopic eyes, 0.94±0.08 (0.8-1.0) in the fellow eyes, and in Group 2 were 0.61±0.17 (0.2-0.8) in amblyopic eyes, 0.92±0.1 (0.8-1.0) in fellow eyes. BCVA in Group 3 was 1.0±0 (1.0-1.0). Mean SFCT of the amblyopic eyes in Groups 1 and 2 was 341.50±60.4 (277-481) and 370.06±65.3 (247-462), respectively, and in the healthy eyes of Groups 1 and 2 and Group 3 was 321.92±68.26 (251-440), 330.35±74.00 (194-502), and 327.62±40.79 (238-385), respectively. SFCT was significantly greater in the amblyopic eyes of Group 2 compared to Group 3 (p=0.01). Mean CVI was 0.681±0.032 (0.642-0.736) in the amblyopic eyes and 0.685±0.054 (0.587-0.788) in the fellow eyes of Group 1 patients; 0.664±0.033 (0.592-0.719) in the amblyopic eye and 0.707±0.039 (0.625-0.779) in the fellow eye in Group 2 patients; and 0.689±0.031 (0.612-0.748) in Group 3 patients. CVI was significantly lower in the amblyopic eyes of Group 2 compared with fellow eyes (p=0.02) and Group 3 (p=0.025). Conclusion: Morphological changes may be seen in the choroid in amblyopic eyes. We observed that the choroidal stromal component is increased in hyperopic amblyopic patients especially.

Sheath-Preserving Complete Optic Nerve Avulsion Following Closed-Globe Injury: A Case Report.

A 29-year-old man presented with a sudden loss of vision after a closed-globe injury. At presentation, he had no light perception in the right eye and the right pupil was dilated and nonreactive to light. On ophthalmological examination, the area of the optic nerve head was excavated, suggesting optic nerve avulsion. Magnetic resonance imaging scan showed optic nerve avulsion without rupture of the optic nerve sheath. Four months after the injury, the patient's visual acuity remained unchanged. Gliosis developed at the avulsion site. Closed-globe injuries may cause severe posterior injury even if there is no anterior damage in the eye. To prevent unnecessary treatment, trauma patients should be examined carefully appropriate imaging to confirm the diagnosis.

Stereoacuity, Fusional Vergence Amplitudes, and Refractive Errors Prior to Treatment in Patients with Attention-Deficit Hyperactivity Disorder

Objectives: To evaluate stereoacuity, fusional vergence amplitudes, and refractive errors in patients with attention-deficit hyperactivity disorder (ADHD). Materials and Methods: Twenty-three patients who were newly diagnosed as having ADHD and had not started medication, and 48 children without ADHD were included. Retrospective data analysis of comprehensive eye examination, stereoacuity, and fusional vergence amplitudes of the patients were performed. Results: The mean age at ADHD diagnosis was 10.68±2.34 (7-16) years in the ADHD group (14 male, 9 female) and 12.23±2.16 (7-15) years in the control group (25 male, 23 female) patients (p=0.605). The mean stereoacuity was 142.14±152.65 (15-480) sec/arc in patients with ADHD and 46.3±44.11 (15-240) sec/arc in the control group (p<0.001). For ADHD patients, the mean convergence and divergence amplitudes at distance were 19.87±8.40 (6 to 38) prism diopter (PD) and -9.09±-4.34 (-4 to -25) PD, and 37.30±12.81 (14 to 70) PD and -13.13±-3.45 (-4 to -20) PD at near, respectively. The mean cycloplegic spherical equivalent was 1.06±1.13 (-1 to 4.63) diopter in ADHD patients, with 6 patients having significant refractive errors (hyperopia in 4 patients, astigmatism in 2 patients). There were no significant differences between groups in terms of spherical equivalents (p=0.358) or convergence and divergence amplitudes at distance (p=0.289 and p=0.492, respectively) or near (p=0.452 and p=0.127, respectively). Conclusion: Fusional vergence amplitudes did not present significant difference, while the mean value of stereoacuity was significantly lower in newly diagnosed ADHD patients prior to treatment.

Functional and morphological assessment of ocular structures and follow-up of patients with early-stage Parkinson's disease.

PURPOSE: To evaluate and follow-up of functional and morphological changes of the optic nerve and ocular structures prospectively in patients with early-stage Parkinson's disease. MATERIALS AND METHODS: Nineteen patients with a diagnosis of early-stage Parkinson's disease and 19 age-matched healthy controls were included in the study. All participants were examined minimum three times at the intervals of at least 6 month following initial examination. Pattern visually evoked potentials (VEP), contrast sensitivity assessments at photopic conditions, color vision tests with Ishihara cards and full-field visual field tests were performed in addition to measurement of retinal nerve fiber layer (RNFL) thickness of four quadrants (top, bottom, nasal, temporal), central and mean macular thickness and macular volumes. RESULTS: Best corrected visual acuity was observed significantly lower in study group within all three examinations. Contrast sensitivity values of the patient group were significantly lower in all spatial frequencies. P100 wave latency of VEP was significantly longer, and amplitude was lower in patient group; however, significant deterioration was not observed during the follow-up. Although average peripapillary RNFL thickness was not significant between groups, RNFL thickness in the upper quadrant was thinner in the patient group. While there was no difference in terms of mean macular thickness and total macular volume values between the groups initially, a significant decrease occurred in the patient group during the follow-up. During the initial and follow-up process, a significant deterioration in visual field was observed in the patient group. CONCLUSION: Structural and functional disorders shown as electro-physiologically and morphologically exist in different parts of visual pathways in early-stage Parkinson's disease.

Long-term Results in Pediatric Developmental Cataract Surgery with Primary Intraocular Lens Implantation.

OBJECTIVES: The aim of this study was to evaluate the outcomes of pediatric developmental cataract surgery with primary intraocular lens (IOL) implantation. MATERIALS AND METHODS: Patients between 2 and 16 years old who underwent cataract surgery with primary IOL implantation were retrospectively evaluated. Age at time of surgery, pre- and postoperative best corrected visual acuities, postoperative ocular complications, and any accompanying ocular pathologies were obtained from the patients' charts. Mean refractive changes and degree of myopic shift were analyzed according to the age groups. Operated eyes were also compared with the fellow eyes in unilateral cases. RESULTS: A total of 101 eyes of 65 patients were included. The average age at time of surgery was 76±40 months and the average follow-up period was 44±30 months. Among the 78 eyes that could be assessed for visual acuity improvement, 66 (84.6%) of them showed ≥2 lines of improvement. The difference in the mean refractive change between the 2-5 years old and 8-16 years old age groups was found to be statistically significant. However, the mean refractive change per year was not found to be significant between the same age groups. In unilateral cases, the operated eyes showed a greater myopic change than the fellow eyes, with no statistically significant difference. The most common postoperative complication was visual axis opacity. CONCLUSION: Good visual outcomes can be achieved following pediatric cataract surgery with primary IOL implantation. Optic axis opacities were the most common postoperative complications. Overall, refractive changes following surgery are inevitable, and more prominent in younger age groups.

Evaluation of the Radiation Dose-Volume Effects of Optic Nerves and Chiasm by Psychophysical, Electrophysiologic Tests, and Optical Coherence Tomography in Nasopharyngeal Carcinoma.

PURPOSE: To evaluate the radiation dose-volume effects of optic nerves and chiasm by visual psychophysical, electrophysiologic tests, and optical coherence tomography in patients with locally advanced nasopharyngeal carcinoma. MATERIALS AND METHODS: A series of visual tests including visual acuity, visual field, contrast sensitivity, visual evoked potential, and optical coherence tomography were administered to 20 patients with locally advanced (T3-T4) nasopharyngeal carcinoma who were treated with definitive chemoradiotherapy. Volume that received 55 Gy (V55), mean dose (Dmean), highest dose to 5% of the volume (D5), and maximum dose (Dmax) for optic nerves and chiasm were evaluated for each patient. Cutoff values were identified as V55: 50%, Dmean: 50 Gy, D5: 55 Gy, and Dmax: 60 Gy. The effects of radiation dose-volume on ophthalmologic tests were evaluated. RESULTS: Ophthalmological evaluation revealed optic neuropathy with simultaneous retinopathy in 6 eyes of 4 patients and radiation retinopathy alone in both eyes of 1 patient. Regarding radiation dose-volume effects of the optic nerve, significant detrimental effect of all parameters was observed on visual acuity. Visual field and contrast sensitivity were affected significantly with V55 ≥ 50% and Dmean ≥ 50 Gy. Visual evoked potential latency was affected significantly with Dmean ≥ 50 Gy, D5 ≥ 55 Gy, and Dmax ≥ 60 Gy. For the chiasm, significant detrimental effect of all parameters was observed on visual acuity as well. Retinal nerve fiber layer thickness and visual evoked potential amplitude were not affected by any of the dose-volume parameters neither optic nerves nor chiasm. CONCLUSION: The volume receiving the threshold dose, mean dose, and 5% of the volume receiving the maximum dose are important parameters besides maximum dose to optic nerves and chiasm. A comprehensive ophthalmological evaluation including visual field, contrast sensitivity, visual evoked potential latency, and amplitude should be performed for these patients. Visual evoked potential latency is an objective predictor of vision loss before the onset of clinical signs.

The effect of convergence and divergence stress on near stereoacuity.

The aim of this study was to evaluate the effect of convergence and divergence stress on near stereoacuity in healthy individuals. In this experimental study, 38 subjects with fine stereoacuity (≤30 arcsec in TNO test, 17th edition) were enrolled. Near fusional convergence and divergence amplitudes were measured. Near stereoacuity was evaluated at different levels of convergence and divergence stress induced with base-in and base-out prisms. The amount of base-in and base-out prisms that cause a decreased stereoacuity level of >240 and >60 arcsec were noted for each subject. There were 21 female and 17 male visually normal adults (aged 25-32 years) in the study. The mean near convergence amplitude of the subjects was 37.7 ± 6.9 (25-54) prism dioptres (PD), and the mean near divergence amplitude was 16.5 ± 2.8 (10-25) PD. The mean convergence stress that decreased the stereoacuity level to >240 and >60 arcsec were 20.1 ± 7.9 (4-35) PD and 13.9 ± 5.4 (4-30) PD, respectively. The mean divergence stress that decreased the stereoacuity levels to >240 arcsec was 11.5 ± 2.9 (4-18) PD, and to >60 arcsec was 8.7 ± 2.9 (4-18) PD. Decreased stereoacuity is an important criterion for judging deterioration for patients with intermittent deviations. However, it is not clear what might be occurring in these deteriorating patients. We specifically determined that short periods of prism-induced convergence and divergence are accompanied by a decrease in fine near stereoacuity in visually normal adults. Controlling intermittent deviations by using vergence system could be the simple cause of decreased stereoacuity in these patients.

Massive iatrogenic orbital cysts following glaucoma drainage implant and strabismus surgery.

We describe 2 paediatric patients who developed large orbital conjunctival epithelium-lined inclusion cysts postsurgery. Case 1 underwent Ahmed glaucoma valve implant surgery 8 months ago and case 2 underwent strabismus surgery 7 years ago. Both cases had either symptomatic strabismus or a mass causing some degree of proptosis or incomitance due to lesions in the intraorbital space. Following total excision of the cysts all associated symptoms were resolved. Conjunctival cysts following ocular surgery can develop anywhere in the anterior segment; however, large orbital cysts following anterior segment surgery are quite rare. We think that implantation cysts should be considered in the differential diagnosis of patients that have signs of orbital mass with a history of previous anterior segment surgery. Complete excision of the cysts results in resolution of all associated signs and symptoms.

Strabismus in infants following congenital cataract surgery.

PURPOSE: This study aimed to determine the incidence and characteristics of strabismus following congenital cataract surgery in infants. MATERIALS AND METHODS: Patients aged <12 months who underwent surgery for congenital cataract and were followed-up for ≥1 years were included. Patients that had strabismus prior to surgery were excluded. Data regarding gender, cataract laterality, morphology, and density, age at the time of cataract surgery, ocular motility post surgery, and the presence of nystagmus were retrospectively obtained from the patients' records. RESULTS: The study included 79 patients (48 male and 31 female). Unilateral surgery was performed in 14 of the patients, versus bilateral surgery in 65. Strabismus did not occur post surgery in 32 (40.5 %) of the patients (group 1), whereas 47 (59.5 %) (group 2) developed strabismus following surgery. The patients in group 1 were followed-up for a mean 50.7 ± 38.5 months, versus 50.3 ± 39 months in group 2. Mean age at the time of cataract surgery in groups 1 and 2 was 3.6 ± 1.9 years and 4.6 ± 3.2 months respectively. Mean duration of time between cataract surgery and the development of strabismus was 13.3 ± 13 months (range: 1-60 months). Unilateral cases were more prone to develop strabismus, which was statistically significant (p = 0.028). Age at the time of cataract surgery, gender, cataract density, the occurrence of aphakic glaucoma, the presence of nystagmus, and additional ocular surgery were not significantly associated with the development of strabismus. CONCLUSION: Strabismus develops more frequently in children undergoing cataract surgery. In the present study strabismus occurred in more of the patients that underwent unilateral surgery. Based on the present findings, we think that long-term careful follow-up to monitor the development of strabismus is required in all infants undergoing cataract surgery, especially unilateral cases.

In Vivo Confocal Microscopy and Anterior Segment Optic Coherence Tomography Findings in Ocular Ochronosis.

Purpose. To report clinical and in vivo confocal microscopy (IVCM) findings of two patients with ocular ochronosis secondary due to alkaptonuria. Materials and Methods. Complete ophthalmologic examinations, including IVCM (HRT II/Rostock Cornea Module, Heidelberg, Germany), anterior segment optical coherence tomography (AS-OCT) (Topcon 3D spectral-domain OCT 2000, Topcon Medical Systems, Paramus, NJ, USA), corneal topography (Pentacam, OCULUS Optikgeräte GmbH, Wetzlar, Germany), and anterior segment photography, were performed. Results. Biomicroscopic examination showed bilateral darkly pigmented lesions of the nasal and temporal conjunctiva and episclera in both patients. In vivo confocal microscopy of the lesions revealed prominent degenerative changes, including vacuoles and fragmentation of collagen fibers in the affected conjunctival lamina propria and episclera. Hyperreflective pigment granules in different shapes were demonstrated in the substantia propria beneath the basement membrane. AS-OCT of Case 1 demonstrated hyporeflective areas. Fundus examination was within normal limits in both patients, except tilted optic discs with peripapillary atrophy in one of the patients. Corneal topography, thickness, and macular OCT were normal bilaterally in both cases. Conclusion. The degenerative and anatomic changes due to ochronotic pigment deposition in alkaptonuria can be demonstrated in detail with IVCM and AS-OCT. Confocal microscopic analysis in ocular ochronosis may serve as a useful adjunct in diagnosis and monitoring of the disease progression.

The effect of optical correction on refractive development in children with accommodative esotropia.

PURPOSE: To compare the effects of partial or full hyperopic optical correction on refractive development in children with accommodative esotropia. METHODS: Children with accommodative esotropia and hyperopia >3 D were enrolled in this prospective, nonrandomized study. All children underwent an ophthalmologic examination, including refraction, keratometry, and axial length. Subjects were divided into either full- or partial-correction groups according to their tolerance of the full hyperopic correction. Routine follow-up examinations were performed for at least 3 years. The main outcome measure was cycloplegic spherical equivalent at the end of the study period. RESULTS: A total of 120 children were enrolled. The mean cycloplegic spherical equivalent, corneal radius, and axial length were significantly (p < 0.05) different between the first and last visits in both groups. However, when all the measurements were adjusted for the age difference between groups, only the difference in axial length between the first and last visits was statistically significant (p < 0.05). Partial or full optical correction in age-adjusted cohorts with accommodative esotropia did not result in a significant change in refraction, keratometry, or axial length between the first and last visits. CONCLUSIONS: Partial or full optical correction of hyperopia had similar effects on refractive development of the eye in children with accommodative esotropia. Treatment of accommodative esotropia in children older than age 5 did not appear to impair refractive development.