Evaluation of the utility of hospital databases to provide data in assessing the quality of strabismus surgery.

AIMS AND BACKGROUND: Rates of re-operation, which may be related to an unsatisfactory surgical outcome, can provide a long-term index of the quality of strabismus surgery. This study aims to evaluate the utility of the Scottish Morbidity Records (SMR1) in determining nature and rates of re-operation for strabismus at the Royal Hospital for Sick Children (RHSC), Glasgow. METHODS: SMR1 data on strabismus surgery performed on children aged between 0 and 17 years at the RHSC, Glasgow, between January 2000 and March 2009 were analysed. RESULTS: In total, 1376 strabismus procedures were carried out on 1274 individuals. The median time between first and subsequent procedures was 19 months; the commonest reasons being under-correction or recurrence. The Kaplan-Meier rate of undergoing re-operation was 7.4% after 9 years with a 95% confidence interval of 5.4-9.9%. CONCLUSIONS: The SMR1 is a useful source of hospital-based and population data. With supplementation from parallel databases, routine administrative databases like the SMR1 can provide better quality data to inform practice.

The inter-relationships between cerebral visual impairment, autism and intellectual disability.

From birth, vision guides our movement, facilitates social interaction and accords recognition and understanding of the environment. In children, vision underpins development of these skills, and is crucial for typical development. Deficits in visual processing may lead to impairment of cognitive, motor, and social development, placing children at risk of developing features of autism. Severe early onset visual dysfunction accords the greatest risk. Cerebral Visual Impairment (CVI) can lead to disorders of cognitive and social development that resemble Autism Spectrum Disorder (ASD). Similarly, children who appear primarily affected by cognitive and social developmental disorders, can manifest a range of visual and perceptual deficits that may be contributory to their disorder. This dual perspective highlights the need for links between impaired vision and neurodevelopmental disorders to be identified and acted upon by means of applying appropriate social and educational strategies. There is good evidence to show that targeted systematic screening for visual and perceptual impairments, and implementation of long-term management approaches, is now required for all at risk children.

Congenital corneal anesthesia.

Congenital corneal anesthesia is a rare clinical entity that poses a diagnostic dilemma, particularly in the pediatric age group. The sensory deficit may be confined to the cornea, or extend to other divisions of the trigeminal nerve. The sensory deficit may occur as an isolated abnormality, as part of a complex neurological syndrome, or it may occur in association with multiple somatic abnormalities and congenital insensitivity to pain. This condition usually presents between the ages of 8 to 12 months. Poor vision, photophobia, conjunctival injection, and corneal ulceration in the absence of pain and distress in a child should alert the clinician to the possibility of anesthetic cornea. In the early stages of presentation, punctuate keratopathy is the main feature, which may progress to non-healing persistent corneal epithelial defects. This stage may progress to acute corneal lysis and perforation. In most patients, conservative approaches such as copious lubrication, prevention of self-harm and cautious use of bandage contact lenses are effective in preventing progressive corneal damage. Tarsorrhapy is effective in promoting epithelial healing and permanent lateral tarsorraphy may prevent further development of epithelial defects. Amniotic membrane graft may be considered in order to improve epithelial healing. Corneal grafts carry a poor prognosis. Accurate initial diagnosis, evaluation, and proper management are paramount to prevent visual loss due to long-term complications of corneal anesthesia. This review of the literature outlines the problems and approaches in diagnosis, evaluation, and management of this rare condition.

Evolving concepts on the pathogenic mechanisms of aniridia related keratopathy.

Heterozygosity for PAX6 deficiency (PAX6+/-) results in aniridia. Corneal changes in aniridia-related keratopathy (ARK) include corneal vascular pannus formation, conjunctival invasion of the corneal surface, corneal epithelial erosions and epithelial abnormalities, which eventually result in corneal opacity and contribute to visual loss. Corneal changes in aniridia have been attributed to congenital deficiency of corneal limbal stem cells. The aim of this paper is to review the potential mechanisms that may underlie the pathogenesis of aniridia related keratopathy. Current evidence, based on clinical observations and an animal model of aniridia suggest that the proliferative potential of the corneal limbal stem cells may not primarily be impaired. The corneal changes in aniridia may be related to an abnormality within the limbal stem cell niche. The mechanisms underlying progressive corneal pathology in aniridia appear multi-factorial and include: (1) abnormal corneal healing responses secondary to anomalous extracellular matrix metabolism; (2) abnormal corneal epithelial differentiation leading to fragility of epithelial cells; (3) reduction in cell adhesion molecules in the PAX6 heterozygous state, rendering the cells susceptible to natural shearing forces; and (4) conjunctival and corneal changes leading to the presence of cells derived from conjunctiva on the corneal surface.

Short-pulsed neodymium-YAG laser trabeculotomy. An in vivo morphological study in the human eye.

The in vivo response to short-pulsed Nd-YAG laser damage to the trabecular meshwork has not been studied in the human eye. The nature of the response will determine the potential efficacy of this treatment for glaucoma. We have investigated short-pulsed laser trabeculotomy lesions created in the trabecular meshwork of four human eyes within 18 hr prior to enucleation for intraocular melanoma. Scanning electron micrographs showed irregular craters (150-300 micron diameter) in the trabecular meshwork surrounded by trabecular beams which were splayed towards the anterior chamber. The adjacent damage to trabecular and corneal tissues was characterized by denudation of endothelial cells and deposition of debris. Light and transmission electron micrographs of the edge of the trabeculotomy lesions revealed fragmentation of the endothelial cells and splitting of the trabecular beams. Preservation of normal morphology was noted in the deeper tissues within 50 micron of the edge of the crater. Neutrophils were present within 20 min of laser treatment whilst macrophages characterised the inflammatory response at later stages. Perforation of the canal of Schlemm was only obtained with lesions in the middle of the trabecular meshwork but not with lesions placed more anteriorly.

Color Doppler ultrasound of orbital and optic nerve blood flow: effects of posture and timolol 0.5%.

Color Doppler ultrasound allows simultaneous imaging with real-time ultrasound and superimposed color-coded vascular flow, allowing visualization of vessels previously beyond the resolution of conventional imaging, such as those in the orbit. With this technique, 20 healthy volunteers were studied. Three regional vessels named 1, 2, and 3 were identified. No significant difference in maximum or minimum blood velocity or resistive index was detected between vessels 1 and 2, although significant differences were noted between both these vessels and vessel 3 (P less than 0.01 and P less than 0.001, respectively). These regional variations are unaffected by small but significant rises in pulse (P less than 0.05) and diastolic blood pressure (P less than 0.01) induced by postural change. No significant change within each vessel was recorded in response to posture, reflecting autoregulation within these vessels. Using a similar technique, 10 healthy volunteers were studied at baseline and at 2 hr and 3 d following the unilateral instillation of 0.5% timolol eye drops. A fall in resistive index was recorded in vessel 3 for both eyes (P less than 0.05, timolol administered eye; P less than 0.01 timolol-free eye). This effect was independent of any simultaneous fall in intraocular pressure that occurred only in the eye receiving timolol drops (P less than 0.01). These results support the presence of B receptors in the vessels at the optic nerve head (vessel 3). A fall in resistive index should not compromise the blood supply in this region, and may even increase it.(ABSTRACT TRUNCATED AT 250 WORDS)

Spatial localization in esotropia: does extraretinal eye position information change?

PURPOSE: To investigate the accuracy of spatial localization in children with a specific type of convergent strabismus, fully accommodative esotropia. METHODS: Two groups of children, with right and left fully accommodative esotropia, respectively, pointed at targets located centrally and eccentrically on a computer touchscreen without being able to see their hands. The size and the direction of the horizontal pointing responses were recorded under two conditions: when their eyes were aligned (wearing spectacles) and when they were squinting (not wearing spectacles). A group of children without strabismus but with hypermetropia were assessed as controls. RESULTS: For both fully accommodative groups, the pointing responses to the central target shifted in the direction of the nonsquinting eye when deviations were manifest. No difference was found for the eccentric targets. No difference was found for the hypermetropia group with any target. CONCLUSIONS: These results are consistent with an alteration in the extraretinal eye position information (efference copy, extraocular muscle proprioception, or both) that is used in spatial localization.

The simultaneous interocular brightness sense test. A test of optic nerve function.

OBJECTIVE: To describe and establish control data from controls for a simultaneous interocular brightness sense test in which the apparent brightness of two independently viewed light patches was compared. DESIGN: Dichoptic viewing was achieved by dissociation of right and left eyes using a septum and cross-polarization. Brightness matches were obtained by using adjustments to the illumination of the left retina while the illumination of the right retina was kept constant. OBSERVERS: Ninety-one control observers (age range, 20 to 91 years) participated in the study to provide normal brightness-matching data. RESULTS: Effects from ocular dominance, age, and long- and short-term adaptation were negligible, but the test was sensitive to anisocoria. Just noticeable differences in dichoptic brightness matching were proportional to retinal illuminance; this finding was consistent with the Weber-Fechner law. CONCLUSION: The test is quick and simple to complete and appears to offer considerable scope for the assessment of visual function when differences in the functional integrity of the optic nerves of the right and left eyes are suspected.

Simultaneous interocular brightness sense testing in ocular hypertension and glaucoma.

OBJECTIVE: To investigate the performance of the simultaneous interocular brightness sense test in patients with ocular hypertension and primary open angle glaucoma (POAG). DESIGN: Brightness matches were obtained for an age-matched control sample and for patients with ocular hypertension and POAG. In addition, for the patients with POAG, visual field defects were quantified and a risk factor count was established for those with ocular hypertension. PATIENTS: Nineteen patients with ocular hypertension, 20 patients with POAG, and 61 age-matched controls participated in the study. RESULTS: Seven patients with ocular hypertension exhibited brightness perception asymmetry outside normal limits for age-matched controls. Of these, six were at high risk of having glaucoma develop and only one of the remaining 12 was found to be at similar risk. All 20 patients with established POAG had results outside the normal limits for a test sensitivity of 100%. Three control observers had abnormal results, giving a test specificity of 95%. CONCLUSION: The simultaneous interocular brightness sense test is quick and simple to perform and is suitable for a wide age range of patients. It warrants further assessment as a means of screening for ocular hypertension and glaucoma.

Penetration of topically applied dexamethasone alcohol into human aqueous humor.

Gas chromatography combined with mass spectrometry was used to determine the absorption of topically applied dexamethasone alcohol into the aqueous humor of human subjects undergoing routine intraocular surgery. The dexamethasone concentration in aqueous humor was greatest in the interval between 91 and 120 minutes following instillation (mean concentration, 31 ng/mL). Dexamethasone was still detectable in the aqueous 12 hours after instillation.

Periventricular leukomalacia: an important cause of visual and ocular motility dysfunction in children.

The immature visual system in infants born preterm is vulnerable to adverse events during the perinatal period. Periventricular leukomalacia affecting the optic radiation has now become the principal cause of visual impairment and dysfunction in children born prematurely. Visual dysfunction is characterized by delayed visual maturation, subnormal visual acuity, crowding, visual field defects, and visual perceptual-cognitive problems. Magnetic resonance imaging is the method of choice for diagnosing this brain lesion, which is associated with optic disk abnormalities, strabismus, nystagmus, and deficient visually guided eye movements. Children with periventricular leukomalacia may present to the ophthalmologist within a clinical spectrum from severe cerebral visual impairment in combination with cerebral palsy and mental retardation to only early-onset esotropia, normal intellectual level, and no cerebral palsy. Optimal educational and habilitational strategies need to be developed to meet the needs of this group of visually impaired children.

Current concepts in giant cell (temporal) arteritis.

Giant cell (temporal) arteritis continues to be a sight-threatening, systemic vasculitis with a poorly understood pathogenesis. The characteristic granulomatous inflammation of the vessel wall commonly leads to local ischemia. Recent advances in immunological investigations have characterized the cellular components of the disease process, but the etiology has so far remained unresolved. A reappraisal of the clinical features of giant cell (temporal) arteritis demonstrates the heterogeneity of the manifestations of the disease, including ischemic optic neuropathy. A range of new laboratory investigations and blood flow studies with color Doppler imaging have demonstrated promising roles, with respect to diagnosis and long-term follow-up. Prompt diagnosis and expeditious treatment require a high index of clinical suspicion, particularly for atypical cases. Corticosteroids remain the treatment of choice, other immuno-suppressive agents being used as second line steroid-sparing agents. Giant cell (temporal) arteritis leads to increased vascular and visual morbidity and, if untreated, may prove fatal. To maintain high standards of management of this enigmatic disorder, ophthalmologists need to be aware of the clinical spectrum of giant cell (temporal) arteritis and currently available diagnostic tests and treatment strategies.

Congenital toxoplasmic retinochoroiditis in the mouse--the use of the peroxidase anti-peroxidase method to demonstrate Toxoplasma antigen.

The peroxidase anti-peroxidase immunocytochemical staining method has been used to demonstrate Toxoplasma antigen within paraffin-embedded sections of the eyes of mice congenitally infected with Toxoplasma. Intact Toxoplasma tissue cysts were demonstrated within the retina but in no other ocular structure. No endozoites and no extra-cystic antigens were detected by this technique within any of the eyes examined. The possible implications of these findings in relation to the pathogenesis of toxoplasmic retinochoroiditis are discussed.

Photocoagulation of raised new vessels by long-duration low-energy argon laser photocoagulation--a preliminary study.

Direct laser treatment of retinal neovascularisation is indicated when regression has not been brought about by retinal photocoagulation. Current treatment regimens involve multiple laser applications to the neovascular complex. Long-duration low-energy burns have the advantage of slowly heating the tissue without engendering disruption. We report the use of this treatment as a means of occluding the feeder vessels to a neovascular network. The application of argon blue-green laser treatment at 0.1 watt for 60 seconds at two adjacent points on a feeder vessel was found to give rise to permanent vascular occlusion without causing complications.

The Pulfrich phenomenon and its alleviation with a neutral density filter.

A case is described in which a presumed vascular accident resulted in long-standing visual difficulties in both reading and the analysis of vectors of moving objects. Clinical examination revealed minimal right optic atrophy with a relative superior altitudinal visual field defect associated with a positive Pulfrich effect. A partial head turn to the right in association with paresis of saccades and pursuit eye movements to the right was also evident. Spectacles for distance which incorporated a neutral density filter before the left eye were prescribed. These practically eliminated the Pulfrich effect and alleviated the problems of vector analysis. A near correction was provided which incorporated prisms with bases to the right. This eliminated the reading difficulties. The visual problems experienced by patients suffering from a positive Pulfrich effect are described and discussed.

Optic nerve hypoplasia in children.

Optic nerve hypoplasia (ONH) is characterised by a diminished number of optic nerve fibres in the optic nerve(s) and until recently was thought to be rare. It may be associated with a wide range of other congenital abnormalities. Its pathology, clinical features, and the conditions associated with it are reviewed. Neuroendocrine disorders should be actively sought in any infant or child with bilateral ONH. Early recognition of the disorder may in some cases be life saving.

Optic foraminal radiography--a redundant investigation?

A retrospective study of all plain radiographs taken of optic foramina during the six years 1984-9 inclusive, comprising a total of 318 examinations, was carried out in order to determine the role of this investigation in patients with ophthalmic disorders. All but one of the radiographs were reported as showing no abnormality. However, for this exception, review of the radiographs showed that the optic foramina were asymmetrical but fell within the normal range. This study indicates that plain radiographs of the optic foramina have little or no useful function. Abandoning the use of these views as a routine will result both in a reduction in radiation hazard and in considerable financial saving.

Pulsed neodymium-YAG laser trabeculotomy: energy requirements and replicability.

Short pulsed laser trabeculotomy has been shown to reduce intraocular pressure in patients with primary open angle glaucoma. This study seeks to determine the energy levels required to produce a fistula into the canal of Schlemm for four different Q-switched neodymium-YAG lasers. The laser was fired at fixed human trabecular meshwork specimens at a range of energy settings for each laser and the characteristics and replicability of the lesions produced were analysed. Energy levels between 3 and 5 mJ were sufficient to produce fistulae into the canal of Schlemm with an approximately 50% success rate for each instrument.

Reappraisal of the ratio of disc to macula/disc diameter in optic nerve hypoplasia.

The ratio of disc to macula/disc diameter is characteristically increased in eyes with optic nerve hypoplasia. We present the largest reported series of patients with a definitive diagnosis of optic nerve hypoplasia for whom this ratio has been determined. All measurements were made by an independent masked observer. Our results are in accordance with previous reports. A ratio of 2.94 provides a one-tailed upper population limit of 95%. An attempt has been made to correlate optic disc size and visual acuity. In 75% of bilateral cases the eye with the relatively smaller optic disc was found to have a better Snellen visual acuity than the fellow eye. This suggests that additional pathogenic mechanism(s) may have determined the eventual visual outcome in such eyes. Such mechanisms include macular hypoplasia, high refractive error, refractive amblyopia, central scotoma, and optic atrophy.

Measurement of optic nerve sheath diameter by ultrasound: a means of detecting acute raised intracranial pressure in hydrocephalus.

AIM: To evaluate the utility of measuring the optic nerve sheath diameter in children with shunted hydrocephalus, suspected of having raised intracranial pressure. METHODS: 23 children with shunted hydrocephalus were examined, six had well controlled ICP, 17 however manifested symptoms suggestive of intracranial hypertension. A clinical history was taken from all patients and their parents or carers. The shunt valve was examined clinically, and signs of raised intracranial pressure were sought. Ultrasound examination was performed in both eyes to measure the optic nerve sheath diameters 3 mm behind the globe. These measurements were compared with control data obtained from 102 children who attended the radiology department for unrelated renal ultrasound examination. RESULTS: Control data suggested that the upper limit of normal for optic nerve sheath diameter is 4.5 mm (measured 3 mm behind the globe) in patients over 1 year of age, and 4.0 mm in children less than 1 year of age. Those patients with functioning ventriculoperitoneal shunts had a mean optic nerve sheath diameter of 2.9 (SD 0.5) mm; those with raised intracranial pressure had a mean optic nerve sheath diameter of 5.6 (0.6) mm (p<0.0001). These results confirm that optic nerve sheath diameters in excess of the control data are strongly suggestive of raised intracranial pressure. CONCLUSION: The evaluation of the optic nerve sheath diameter is a simple non-invasive procedure, which is a potentially useful tool in the assessment and monitoring of children with hydrocephalus suspected of having raised intracranial pressure.