Hande Taylan Şekeroğlu

Fundus autofluorescence and optical coherence tomography findings in glutathione synthetase deficiency.

We report the autofluorescence pattern and optical coherence tomography (OCT) characteristics in a 6-year-old boy with glutathione synthetase deficiency. The patient underwent complete ophthalmological examination, including full-field electroretinography, visual evoked potentials, fundus autofluorescence, and OCT imaging. Electrophysiological studies showed time-delay and subnormal responses. Fundus autofluorescence imaging revealed increased parafoveal autofluorescence compared to normal, and OCT showed alteration of photoreceptor and retinal pigment epithelium layers in the parafoveal region.

Superior oblique surgery: when and how?

Background The purpose of this paper is to review different types of superior oblique muscle surgeries, to describe the main areas in clinical practice where superior oblique surgery is required or preferred, and to discuss the preferred types of superior oblique surgery with respect to their clinical outcomes. Methods A consecutive nonrandomized retrospective series of patients who had undergone superior oblique muscle surgery as a single procedure were enrolled in the study. The diagnosis, clinical features, preoperative and postoperative vertical deviations in primary position, type of surgery, complications, and clinical outcomes were reviewed. The primary outcome measures were the type of strabismus and the type of superior oblique muscle surgery. The secondary outcome measure was the results of the surgeries. Results The review identified 40 (20 male, 20 female) patients with a median age of 6 (2–45) years. Nineteen patients (47.5%) had Brown syndrome, eleven (27.5%) had fourth nerve palsy, and ten (25.0%) had horizontal deviations with A pattern. The most commonly performed surgery was superior oblique tenotomy in 29 (72.5%) patients followed by superior oblique tuck in eleven (27.5%) patients. The amount of vertical deviation in the fourth nerve palsy and Brown syndrome groups (P = 0.01 for both) and the amount of A pattern in the A pattern group were significantly reduced postoperatively (P = 0.02). Conclusion Surgery for the superior oblique muscle requires experience and appropriate preoperative evaluation in view of its challenging nature. The main indications are Brown syndrome, fourth nerve palsy, and A pattern deviations. Superior oblique surgery may be effective in terms of pattern collapse and correction of vertical deviations in primary position.

Sequential presentation of bilateral Brown syndrome

Brown syndrome, characterized by a limitation of elevation in adduction and positive forced duction testing, is usually unilateral but occurs bilaterally in 10% of all cases. It may present as a congenital condition in one eye and develop in the other eye with no apparent cause. We present a case of bilateral Brown syndrome in which the right eye became involved within 1 year of surgery on the left eye for congenital Brown syndrome.

Congenital Cranial Dysinnervation Disorders

This chapter describes the clinical features, diagnostic evaluation, and management options of patients with congenital fibrosis of the extraocular muscles (CFEOM) as well as those with other types of congenital cranial dysinnervation disorders (CCDD) such as Mobius syndrome, horizontal gaze palsy with progressive scoliosis, and synergistic horizontal deviation. These are rare and challenging strabismus cases to the most experienced pediatric ophthalmologists and strabismologists. The classification and genetics of CCDDs are briefly discussed, and two case presentations are given. Management strategies are discussed and a flowchart for the evaluation and management of CCDD cases is provided.

Effect of Visual Acuity on the Surgical Outcomes of Secondary Sensory Strabismus.

Objectives: To investigate the outcomes of secondary sensory strabismus surgery and to discuss the effect of visual acuity on success. Materials and Methods: The medical records of patients with sensory strabismus who underwent recession-resection on the eye with vision loss were reviewed. Only patients with visual acuity of ≤0.2 in the operated eye were enrolled. Data including age at surgery, visual acuity, etiology of vision loss, preoperative and postoperative deviations, follow-up duration, and surgical outcomes were recorded. Success was defined as a final deviation of ≤10 prism diopters (PD). To evaluate the effect of visual acuity on postoperative success, patients were grouped as follows according to the visual acuity of the operated eye: group 1, visual acuity <0.05; group 2, 0.05-0.1; and group 3, 0.125-0.2. Results: Ten females and 14 males met the inclusion criteria. The mean age at surgery was 21 years (range, 6 to 56 years). The mean preoperative deviation angle was 52.7 PD (range, 20 to 80 PD). Age at surgery, preoperative deviation and follow-up time were similar in patients with esotropia (n=7) and exotropia (n=17) (p>0.05 for all). The success rate was 62.5% at short-term and 42.1% at long-term follow-up. There was no statistically significant difference in short-term success rate among visual acuity subgroups (p=0.331), whereas the difference was statistically significant at long-term follow-up (p=0.002). The long-term success rate was higher in group 3 compared to groups 1 and 2. Conclusion: Better visual acuity seems to be a potential predictor for higher long-term success after strabismus surgery in patients with sensory strabismus.

Bilateral choroidal detachment following cataract surgery in a 40-day-old infant

We report a case of bilateral choroidal detachment that occurred shortly after uneventful bilateral lensectomy and anterior vitrectomy. B-scan ultrasonography showed severe choroidal detachment in the right eye accompanied by limited detachment of the overlying retina and mild choroidal effusion in the left eye. Two bilateral peribulbar injections of corticosteroid 5 days apart resulted in complete resolution of the choroidal effusion in each eye.

Ocular Causes of Abnormal Head Position: Strabismus Clinic Data

Objectives: To determine the most common ocular causes and types of abnormal head position (AHP) and describe their clinical features. Materials and Methods: Patients with AHP who had been followed in the strabismus unit were retrospectively reviewed. Demographic features and orthoptic characteristics were recorded. Results: A total of 163 patients including 61 women (37.4%) and 102 men (62.6%), with a mean age of 19.9±18.3 were recruited. The most common causes of AHP were determined as fourth cranial nerve palsy (33.7%), Duane retraction syndrome (21.5%), sixth cranial nerve palsy (11%), nystagmus blockage syndrome (9.8%) and Brown syndrome (6.7%). Other less frequent causes were A-V pattern strabismus, comitant strabismus, thyroid orbitopathy and third cranial nerve palsy. The most common types of AHP were head tilt (45.4%) and face turn (36.8%). Out of 142 patients whose visual acuity could be evaluated, 28.2% had amblyopia. The frequency of amblyopia varied depending on the diagnosis (p<0.001), while there was no relation between amblyopia and different types of AHP (p=0.497). Stereopsis and fusion could be tested in 128 patients and 43.8% of them had stereopsis and fusion. The presence of stereopsis and fusion was found to be related with the diagnosis (p=0.001), whereas it was not related with the types of AHP (p=0.580). The presence of amblyopia was not significantly associated with fusion (p=1.000) or stereopsis (p=0.602). Conclusion: There are many ocular pathologies that cause AHP. Patients with similar diagnoses may have different types of AHP. Patients may have amblyopia and impaired binocularity despite AHP. Therefore, all patients with AHP should be examined in detail and these points should be considered in the treatment plan.

A Baseline Algorithm for Molecular Diagnosis of Genetic Eye Diseases: Ophthalmologist’s Perspective

Turkish Journal of Ophthalmology, Published by Galenos Publishing House Address for Correspondence: Hande Taylan Şekeroğlu MD, Hacettepe University Faculty of Medicine, Department of Ophthalmology, Ankara, Turkey Phone: +90 312 305 31 33 E-mail: h_taylan@yahoo.com Received: 08.09.2015 Accepted: 23.10.2015 A Baseline Algorithm for Molecular Diagnosis of Genetic Eye Diseases: Ophthalmologist’s Perspective Letter to the Editor

A Severe Aspect of Pediatric Ocular Allergy to Recognize: Vernal Keratoconjunctivitis

Aim: To determine the clinical features of vernal keratoconjunctivitis and to evaluate the safety and the efficacy of the medical treatment on clinical grades. Material and Method: All patients with vernal keratoconjunctivitis who had been treated with mast-cell stabilizers, antihistamines and artificial tear drops previously were enrolled in the study. Topical steroids were added during recurrences, were tapered and discontinued according to the clinical improvement. Topical cyclosporin 0.05% four times daily was used additio- nally in cases of inadequate response to treatment or evident steroid depen- dance. Main outcome measures were the clinical features, change of clini- cal grades, response to treatment, rate of recurrences and side effects of the eyedrops. Results: Twenty patients ( 13 males, 7 females) with vernal kera- toconjunctivitis in different severity scales were included. The median age of the patients was 10 (9-11) years. The median follow-up time was 35 (15-56) months. Ten patients received topical cyclosporine. The rate of recurrences was similar in patients who received topical cyclosporine compared to those who were followed with topical steroids. (p=0.17) No severe adverse reaction to any of the formulations was seen. Discussion: Topical 0.05% cyclosporin is safe and effective for the treatment of vernal keratoconjunctivitis as a stero - id sparing agent. It helps to obtain good clinical response without serious ad- verse effects and provides improvement on the clinical grades.