Intravitreal enzyme replacement therapy slows retinopathy in late infantile ceroid lipofuscinosis type 2.

Ceroid lipofuscinosis type 2 (CLN2) is caused by biallelic pathogenic variants in the TPP1 gene, encoding lysosomal tripeptidyl peptidase 1 (TPP1). The classical late-infantile phenotype has an age of onset between 2 and 4 years and is characterized by psychomotor regression, myoclonus, ataxia, blindness, and shortened life expectancy. Vision loss occurs due to retinal degeneration, usually when severe neurological symptoms are already evident.

Effectiveness of Strabismus Surgery in Intermittent Exotropia and Factors Influencing Outcome.

Intermittent exotropia (IXT) is known to relapse after surgery. No factors to predict or prevent recurrence are known with certainty. This study investigated surgical outcome, potential influencing factors, and reoperation rate in patients with IXT.

[Brown's atavistic superior oblique syndrome: etiology of different types of motility disorders in congenital Brown's syndrome].

Clinical Features: The congenital Brown syndrome is characterized by a mechanical limitation of elevation in adduction, with an orthophoria in down gaze. Brown postulated a shortened superior oblique tendon sheath as the cause of the limitation but this was disproved by Parks et al. in 1975 and the origin of Brown syndrome remains unclear.

[Retinal detachment in children and adolescents. Specific clinical features].

Background: Due to the long life expectancy, retinal detachment is a special threat to visual acuity in children and adolescents. This study presents the clinical features of retinal detachment in childhood and adolescence up to the age of 20 years.

Patients And Methods: A cohort was selected comprising 259 patients who suffered from unilateral or bilateral retinal detachment, were not older than 20 years of age at the first diagnosis of the first or only affected eye and had undergone surgery at least once at the Department of Ophthalmology of the University Medical Center of Munich during a period of 18 years (1980-1998).

Extraocular muscles involved in convergence are innervated by an additional set of palisade endings that may differ in their excitability: A human study.

Palisade endings are located at the myotendinous junction of extraocular muscles in most mammals. Irrespective of their unclarified function as motor or sensory nerve endings, a specialized role in convergence is proposed, based on their high number in the medial rectus muscle (MR). Further support comes from a study in monkey demonstrating that only the MR and inferior rectus muscle (IR) contain an additional population of palisade endings that express the calcium-binding protein calretinin (CR) in addition to choline acetyltransferase (ChAT).

Comparing Alternative Ranibizumab Dosages for Safety and Efficacy in Retinopathy of Prematurity: A Randomized Clinical Trial.

Importance: Anti-vascular endothelial growth factor (VEGF) therapies are a novel treatment option in retinopathy of prematurity (ROP). Data on dosing, efficacy, and safety are insufficient.

Objective: To investigate lower doses of anti-VEGF therapy with ranibizumab, a substance with a significantly shorter systemic half-life than the standard treatment, bevacizumab.

Pediatric idiopathic intracranial hypertension - Is the fixed threshold value of elevated LP opening pressure set too high?

Background: Idiopathic intracranial hypertension (IIH) in children is a rare condition of unknown etiology and various clinical presentations. The primary aim of this study was to evaluate if our pediatric IIH study group fulfilled the revised diagnostic criteria for IIH published in 2013, particularly with regard to clinical presentation and threshold value of an elevated lumbar puncture opening pressure. Additionally we investigated the potential utilization of MR-based and fundoscopic methods of estimating intracranial pressure for improved diagnosis.

[Prism adaptation test before strabismus surgery : Results of a survey and literature review].

Background: Variable preoperative deviations and compensatory mechanisms may cause wrong dosage of strabismus surgery and result in over- or undercorrection. A long-lasting prism adaptation test (PAT) before surgery is supposed to reduce those difficulties and to improve the postoperative results. To date, the use of prism adaptation before surgery has not been systematically examined.

[Conservative and surgical treatment of convergence excess].

Convergence excess is a common finding especially in pediatric strabismus. A detailed diagnostic approach has to start after full correction of any hyperopia measured in cycloplegia. It includes measurements of manifest and latent deviation at near and distance fixation, near deviation after relaxation of accommodation with addition of +3 dpt, assessment of binocular function with and without +3 dpt as well as the accommodation range.

[Possibilities and limitations of amblyopia screening with auto-refractometers].

Amblyopia is a frequent vision disorder with a prevalence of 3-6%, for which early treatment is more effective. More than half of the cases of amblyopia are due to refractive errors so that they are not obvious due to strabismus or other ocular abnormalities; therefore, examinations for early recognition are essential. Because no nationwide ophthalmological examination of infants with cycloplegia has been established in Germany, screening for refractive errors in the first 3 years of life could be very helpful.

Infantile and acquired nystagmus in childhood.

Nystagmus is an involuntary, periodic eye movement caused by a slow drift of fixation which is followed by a fast refixation saccade (jerk nystagmus) or a slow movement back to fixation (pendular nystagmus). In childhood most cases are benign forms of nystagmus: idiopathic infantile, ocular or latent nystagmus. They arise at the age of 3 months, without oscillopsia and show the absence of the physiologic opto-kinetic nystagmus.

[Prescribing spectacles to children].

Refractive errors are the most common visual problem in children apart from squinting. Indications for spectacles include amblyopia prophylaxis and treatment, strabismus, myopia and reading disorders. Objective refraction by retinoscopy is the central part of prescribing spectacles to children.

[Detection of amlyogenic risk factors with the vision screener S 04].

Introduction: The Vision Screener® (Plusoptix) was developed for the recognition of amblyogenic refractive errors without cycloplegia. The aim of this study was to evaluate the sensitivity and specitifity for the detection of amblygenic refractive errors in preschool children. Furthermore it was investigated whether small angle strabismus as another cause for amblyopia could be detected by manual evaluation of corneal reflex images on printouts.

[Retinal detachment in pediatrics : Etiology and risk factors].

Background: Juvenile retinal detachment is uncommon but is a severe threat to visual acuity. This study demonstrates the etiology and risk factors of retinal detachment in patients age 0-20 years.

Patients And Methods: A cohort was selected comprising 259 patients (278 eyes) who were not older than 20 years at the age at onset of retinal detachment in the only or the first affected eye and had undergone surgery at least once at the Department of Ophthalmology of the University Medical Center of Munich between January 1980 and October 1998.

Reversible benign intracranial hypertension in a child with hyperthyroidism.

Endocrine dysfunction is a rare but known cause of benign intracranial hypertensio (BIH) in adults. Here we describe a rare case of BIH in the pediatric age group associated with autoimmune hyperthyroidism. A 12-year-old girl presented with a 3-month history of headaches.

[The latest on amblyopia treatment].

In recent years there has been a lively discussion on the benefits of early detection and treatment of amblyopia, as well as large prospective randomized controlled studies. Furthermore, we now have the opportunity to measure effective occlusion time. This leads to a better scientific base for discussion of amblyopia therapy.

Screening for refractive errors in preschool children with the vision screener.

Background: The Vision Screener is a new, commercial version of the Power Refractor, an off-axis, hand-held video refractor to screen for amblyogenic refractive errors. The aim of our study was to determine the reproducibility of the measurements, compare them to cycloplegic refraction, and evaluate the sensitivity and specificity for the detection of amblyogenic refractive errors.

Patients And Methods: Included in the study were 161 preschool children, age 0.

Foveal contour interaction: detection and discrimination.

Contour interaction, the detrimental effect of flanking features on the discrimination of optotypes, has been studied mainly close to the visual acuity limit. We were interested to know how these results compare with those for the detection of targets. According to the simplest model of contour interaction, comparable detection effects would be expected.

A concept for the surgical treatment of trochlear palsy.

The outstanding clinical symptom of acquired uni- and bilateral trochlear palsy is excyclotropia which increases in down-gaze. Any surgical treatment must aim at reducing this deviation. To achieve this, we have routinely used a modification of the Harada-Ito operation over the last 20 years.

Integration of orientation information in amblyopia.

A recent report suggests that amblyopes are deficient in processing local orientation at supra-threshold contrasts. To determine whether amblyopes are also poor at integrating local orientation signals, we assessed performance for an orientation integration task in which the orientations of static signals are integrated across space. Our results show that amblyopic visual systems can integrate local static oriented signals with the same level of efficiency as normal visual systems.

[The combination of eye muscle surgery with other interventions on or in the eye].

Strabismus and motility disorders of the eye can be linked in many different ways to intraocular diseases and disorders of the lid and orbit. Different functional aspects have to be considered before performing combined eye muscle and intraocular or adnexal surgery. This allows to avoid unnecessary operations, to choose the right dosage of the operation, to limit the risk of diplopia, and finally to achieve a satisfying result for the patient.

[Scanning laser ophthalmoscope multifocal electroretinography and microperimetry in patients with Stargardt's disease].

Purpose: We used a scanning laser ophthalmoscope (SLO) evoked multifocal electroretinography (mf-ERG) to evaluate retinal function in patients with Stargardt's disease. SLO microperimetry could demonstrate the size of central retinal scotoma very well in these patients. The aim of the examination was to correlate the results of SLO mf-ERG and SLO microperimetry.