Impaired vision in children prenatally exposed to methadone: an observational cohort study.

BACKGROUND/OBJECTIVES: To examine prevalence of failed visual assessment at 8-10 years in children born to methadone-maintained opioid dependent (MMOD) mothers and relate this to known in utero substance exposure. SUBJECTS/METHODS: Follow up of observational cohort study of methadone-exposed and comparison children matched for birthweight, gestation and postcode of residence at birth. Participants were 144 children (98 exposed, 46 comparison). Prenatal drug exposure was previously established via comprehensive maternal and neonatal toxicology. Children were invited to attend for visual assessment and casenotes were reviewed. Presence of acuity poorer than 0.2 logMAR, strabismus, nystagmus and/or impaired stereovision constituted a 'fail'. Fail rates were compared between methadone-exposed and comparison children after adjusting for known confounding variables. RESULTS: 33 children attended in person: data were also derived from casenote review for all children. After controlling for maternal reported tobacco use, methadone-exposed children were more likely to have a visual 'fail' outcome, adjusted odds ratio 2.6, 95% CI 1.1-6.2; adjusted relative risk 1.8 (95% CI 1.1-3.4). Visual 'fail' outcome rates did not differ between methadone-exposed children who had (n = 47) or had not (n = 51) received pharmacological treatment for neonatal abstinence/opioid withdrawal syndrome (NAS/NOWS); fail rate 62% vs 53% (95% CI of difference-11-27%). CONCLUSIONS: Children born to MMOD mothers are almost twice as likely as unexposed peers to have significant visual abnormalities at primary school age. Prenatal methadone exposure should be considered in the differential diagnosis of nystagmus. Findings support visual assessment prior to school entry for children with any history of prenatal opioid exposure. TRIAL REGISTRATION: The study was prospectively registered on ClinicalTrials.gov (NCT03603301), https://clinicaltrials.gov/ct2/show/NCT03603301 .

A population-based study of Coats disease in the United Kingdom II: investigation, treatment, and outcomes.

AIMS: coats disease is an uncommon form of retinal telangiectasia. We conducted a prospective population-based study of Coats disease in the United Kingdom to provide a more balanced picture. This paper reports the investigations and treatments used for Coats disease and their anatomic and visual outcomes. METHODS: the study was conducted through the British Ophthalmic Surveillance Unit. All ophthalmologists notifying cases of Coats disease were sent a baseline questionnaire and a follow-up questionnaire after 6 months. RESULTS: in total, 55 baseline and 42 follow-up questionnaires were returned. All cases were unilateral. Ultrasound was performed in 26% of cases, fluorescein angiography in 35%, and examination under anaesthesia in 42% of the cases. Laser photocoagulation was by far the primary treatment modality, used in 92%, with cryotherapy used mainly as a second-line or adjunctive treatment. In more advanced cases with significant retinal detachment, laser photocoagulation combined with pars plana vitrectomy and drainage of sub-retinal fluid can produce anatomic stability and prevent progress to end-stage disease. Intravitreal injections of steroids or VEGF inhibitors are currently of unproven efficacy in Coats disease. Overall, treatment resulted in stabilisation of visual acuity. Anatomic stabilisation or improvement was achieved in virtually all eyes. CONCLUSION: laser photocoagulation to telangiectatic retinal vessels is the most commonly used treatment for Coats disease. Other treatments are either adjunctive or of unproven efficacy. A realistic treatment goal is to achieve anatomic stability and avoidance of enucleation for painful end-stage disease. Visual improvement is however unlikely.

A population-based study of Coats disease in the United Kingdom I: epidemiology and clinical features at diagnosis.

AIMS: coats disease is an uncommon form of retinal telangiectasis. Published case series mostly originate from tertiary referrals centres and may provide a skewed view of disease severity. We conducted a prospective population-based study of Coats disease in the United Kingdom to ascertain the incidence and provide a more representative picture. METHODS: the study was conducted through the British Ophthalmological Surveillance Unit. This first paper reports the features at presentation; gender, mode of presentation, visual acuity, anterior and posterior segment findings, amount of retinal exudation, and disease staging. RESULTS: a total of 55 eligible cases of Coats disease were identified giving an estimated population incidence of 0.09 per 100.000 of the population. All cases were unilateral and 85% were male. Mean age at presentation was 146 months (median 96 months). The mean age of diagnosis was markedly different with differing mechanisms of presentation. Cases presenting with leucocoria or strabismus presented early whereas subjective visual loss presented much later. A large proportion of eyes (44%) were blind at diagnosis. The great majority of eyes (71%) had 6 or fewer clock hours of retinal exudation. More severe forms/stages of Coats disease were more common in the youngest patients. CONCLUSIONS: compared with published studies of Coats disease, we have found milder disease severity at presentation. This is most likely because of the population-based nature of our study reflecting the full disease spectrum. A large proportion of eyes with Coats have poor visual acuity and disease severity is worse in younger patients.

Ocular teratogens: old acquaintances and new dangers.

Recent research into animal studies has contributed significantly to understanding the pathophysiology of some well-known teratogens, such as alcohol. Techniques, such as positron emission tomography (PET) and retinal synaptogenesis studies, have helped determine the specific areas in the developing brain and ocular structures, which are targeted by various teratogens. In this article, we also highlight a few newer agents, such as benzodiazepines, with potential for ocular malformation and morbidity in the developing foetus.

Classification, assessment, and management of childhood ptosis.

Ptosis in childhood may both impair normal visual development and be cosmetically disfiguring. Ptosis may sometimes be a component part of a more extensive disorder involving the extraocular muscles, facial structures, or nervous system. Each patient must receive a thorough ocular examination, as well as careful assessment of the ptosis itself. Optimum outcome following surgical repair depends on the correct choice of operation for the specific type of ptosis and degree of levator muscle function.

Ocular findings among patients with late-diagnosed or poorly controlled homocystinuria compared with a screened, well-controlled population.

INTRODUCTION: Untreated homocystinuria (HCU) leads to systemic and ocular complications preventable by early treatment. METHODS: This study describes the ocular features in HCU patients who had late diagnosis or were noncompliant with treatment compared with a control group of early-diagnosed and well-controlled subjects. RESULTS: Fourteen late-diagnosed HCU patients with a median age at diagnosis of 4 years (range, 1.25-28 years) were studied. Five patients were born outside of Ireland or before screening began. All 14 patients had lens subluxation or dislocation at diagnosis. Only 28.6% of eyes had 20/40 vision or better. Three patients were tested for HCU following the diagnosis in a sibling. Four patients attended ophthalmology departments for a median of 12.8 years (range, 4-23 years) prior to diagnosis of HCU; all had steadily progressive myopic astigmatism and lens subluxation. Six patients who became poorly controlled in their teens or early twenties showed significant progression of their myopia, and 3 had phacodonesis or lens subluxation develop. All eyes in this group had 20/40 vision or better. Fifteen patients who were detected in the newborn period and remained well controlled had no evidence of lens subluxation. All of the control group patients had 20/20 vision bilaterally. The difference in visual acuity between late-diagnosed patients and the control group was highly significant (P =.0002). The differences in refractive errors between the groups were also highly significant (P =.0001). CONCLUSIONS: Lens subluxation is a principal feature of untreated HCU, yet we found a median lag period of 5.5 years in 4 cases before diagnosis. Young persons with marked and progressive myopia or idiopathic lens subluxation should be screened for HCU.

Classification of the spectrum of Coats' disease as subtypes of idiopathic retinal telangiectasis with exudation.

PURPOSE: An increasing variety of eponymous terms have been used to describe atypical cases of Coats' disease. A group of typical cases of Coats' disease and other cases of differing severity were classified as one of four subtypes of idiopathic retinal telangiectasis with exudation and compared with regard to clinical outcome. METHODS: In a retrospective clinical review patients with typical and atypical Coats' disease were classified as severe, focal, juxtafoveal or associated (with another disease) forms of idiopathic retinal telangiectasis with exudation. RESULTS: 53 eyes in 50 patients were examined of which 62% (n=31) were male. 12 eyes were classified as severe (group 1), 22 focal (group 2), 12 juxtafoveal (group 3) and 7 associated (group 4). The mean age at diagnosis was lowest in group 1 eyes (6.8 years). The best visual acuity at presentation was 6/60 in group 1 whereas high proportions of eyes in the other groups had initial visual acuities of 6/24 or better. In group 1 only one eye was treated, the majority of eyes were blind or had been enucleated whereas 34 (79%) of eyes in the other groups were suitable for treatment and 29 eyes (67%) retained pre-treatment visual acuity or better at last follow-up. CONCLUSIONS: Idiopathic retinal telangiectasis with exudation is a spectrum of disease, which is synonymous with Coats' disease. In this retrospective study eyes with severe idiopathic retinal telangiectasis with exudation corresponding to typical Coats' disease, have poorer vision at presentation, are less suitable for treatment and have worse outcomes than eyes with other subtypes. The spectrum of disease severity seen in idiopathic retinal telangiectasis with exudation may be due to second somatic mutations in genes with an existing germline mutation (the two hit theory) and a mosaic phenotype.

Disinsertion of the inferior oblique muscle for treatment of superior oblique paresis.

PURPOSE: To examine the safety and efficacy of disinsertion of the inferior oblique muscle in the treatment of long-standing ipsilateral superior oblique paresis. METHODS: This retrospective study included 52 patients who underwent disinsertion of the inferior oblique muscle over a 5-year period. Mean age at presentation was 30.8 years (range: 1-70 years). All patients had long-standing unilateral superior oblique paresis and overaction of the ipsilateral inferior oblique muscle. Information recorded included pre- and postoperative vertical deviation, pre- and postoperative Hess chart error scores, reoperation rate, and symptom relief. RESULTS: Following surgery, mean vertical deviation was reduced from 12.9 to 4 prism diopters, while mean Hess chart error score decreased from 596 to 258. This procedure alone resulted in satisfactory symptom relief in 84.6% of patients. The only complication was significant undercorrection requiring further surgery in 13.6% of patients and prisms in 1.8%. When further surgery was needed, the procedure of choice was tucking of the affected superior oblique. Recession of the contralateral inferior rectus was carried out when superior oblique tucking was believed to be unsuitable. CONCLUSIONS: Disinsertion of the inferior oblique muscle is a safe and effective treatment for the majority of long-standing idiopathic unilateral superior oblique pareses.

The pattern of perforating eye injuries in Ireland.

BACKGROUND: Almost all perforating eye injuries present to dedicated eye units. Data from eye units are therefore reliable indicators of the incidence of perforating injuries. AIMS: To establish the aetiology and visual outcome of perforating eye injuries presenting to one unit over a twelve-month period. METHODS: Patients with eye injury were identified from the operating room register. Case-notes were reviewed and eye examinations were performed to determine current level of vision. RESULTS: A total of 37 perforating eye injuries were operated on in the 12-month period from January to December 1995. Thirty one patients (83.8%) were male. The mean age was 33.9 years. The chief causes were work related or do-it-yourself (DIY) in 32.4%, miscellaneous accidents in 27.0%, assault in 16.2%, road traffic accidents in 10.8%. Twenty eyes (54.1%) were blind with acuity < 6/60 at their most recent review. CONCLUSION: Work-related and DIY injuries are the most common causes of eye perforation. Many of these injuries are easily preventable with appropriate eye protection. The proportion of injuries resulting from road traffic accidents was low. Injuries due to assault are becoming more prevalent and carry a poor visual prognosis.

Outcome in refractive accommodative esotropia.

AIM: To examine outcome among children with refractive accommodative esotropia. METHODS: Children with accommodative esotropia associated with hyperopia were included in the study. The features studied were ocular alignment, amblyopia, and the response to treatment, binocular single vision, requirement for surgery, and the change in refraction with age. RESULTS: 103 children with refractive accommodative esotropia were identified. Mean follow up was 4.5 years (range 2-9.5 years). 41 children (39.8%) were fully accommodative (no manifest deviation with full hyperopic correction). The remaining 62 children (60.2%) were partially accommodative. At presentation 61.2% of children were amblyopic in one eye decreasing to 15.5% at the most recent examination. Stereopsis was demonstrated in 89.3% of children at the most recent examination. Mean cycloplegic refraction (dioptres, spherical equivalent) remained stable throughout the follow up period. The mean change in refraction per year was 0.005 dioptres (D) in right eyes (95% CL -0. 0098 to 0.02) and 0.001 D in left eyes (95% CL -0.018 to 0.021). No patients were able to discard their glasses and maintain alignment. CONCLUSIONS: Most children with refractive accommodative esotropia have an excellent outcome in terms of visual acuity and binocular single vision. Current management strategies for this condition result in a marked reduction in the prevalence of amblyopia compared with the prevalence at presentation. The degree of hyperopia, however, remains unchanged with poor prospects for discontinuing glasses wear. The possibility that long term full time glasses wear impedes emmetropisation must be considered. It is also conceivable, however, that these children may behave differently with normal and be predestined to remain hyperopic.

Visual outcome and complications of bilateral intraocular lens implantation in children.

PURPOSE: To determine the safety and efficacy of bilateral intraocular lens (IOL) implantation in children. SETTING: Tertiary referral pediatric ophthalmology department. METHODS: This retrospective study comprised 13 children (26 eyes) who had bilateral cataract surgery with IOL implantation. Patients were divided into 2 groups: congenital cases, diagnosed during the first year of life, and developmental cases, diagnosed after 1 year of age. All patients had small incision phacoemulsification with primary implantation of a poly(methyl methacrylate) or a foldable acrylic IOL. Primary posterior capsulotomy was performed in 16 eyes (61.5%). RESULTS: Age at surgery ranged from 1 week to just under 8 years. Seven patients (53.8%) had a systemic diagnosis: Down's syndrome (n = 4); developmental delay or cerebral atrophy (n = 3). Five children with systemic problems could not cooperate with formal vision testing; 2 could fix and follow bilaterally, and 3 had central, steady, and maintained vision bilaterally. In the congenital group, 37.5% of eyes attained a visual acuity of 20/20 and 87.5%, 20/120 or better. In the developmental group, formal vision testing was possible in 4 children. Five eyes (83.3%) attained a visual acuity of 20/40 or better. Thirteen eyes (50.0%) required posterior capsulotomy. Primary posterior capsulotomy reduced the incidence of posterior capsule opacification (PCO), but there was no correlation between PCO and IOL material. One patient developed glaucoma bilaterally. CONCLUSION: Bilateral IOL implantation was safe and produced good visual results in children of all ages with bilateral cataract.

Uniocular childhood blindness: a prospective study.

PURPOSE: This prospective study examines uniocular blindness among children younger than 16 attending a large pediatric ophthalmology department. The aim was to identify the causes of uniocular blindness and determine how much is preventable. We defined blindness according to the World Health Organization definition of vision worse than or equal to 3/60. METHODS: All children who attended the department and were blind in one eye during the period of the study were included. A history was taken, visual acuity was assessed by an age-appropriate method, and an ocular examination was carried out. When necessary, the child also was seen by an orthoptist or pediatrician. RESULTS: A total of 71 patients were identified. At the time of diagnosis, the patients varied in age from a few weeks to 15 years. The causes were classified into one of the following diagnostic categories: chromosomal/genetic (8.5%), prenatal (47.9%), perinatal (7.0%), or childhood (36.6%). Many cases are prenatal in origin and are not preventable. Other causes that are difficult to prevent include retinoblastoma, toxocariasis, and trauma. One cause, amblyopia, may be preventable or treatable in most instances if detected early. CONCLUSIONS: Most cases of uniocular blindness are not preventable; however, protective glasses should be encouraged to prevent injury to the good eye.

Pneumatic retinopexy versus scleral buckling: a randomised controlled trial.

Pneumatic retinopexy (PR) is a technique for repairing certain retinal detachments which is easier to perform than conventional sceral buckling (SB) surgery but has comparable results. We performed a prospective, randomised, controlled trial to determine for ourselves whether PR is a safe and acceptable procedure. Twenty patients presenting consecutively with retinal detachments which fulfilled the selection criteria were randomised to have their detachments repaired by either PR or SB, ten patients in each group. The suitable patients had a single retinal break or small group of breaks of not greater than one clock hour in size, situated within the superior eight clock hours of retina. Patients with significant proliferative vitreoretinopathy or other fundus disorders were excluded. All patients in the PR group had local anaesthesia while all those in the SB group had general anaesthesia. Successful reattachment of the retina was achieved with one or more procedures in 90 percent of the PR group and in 100 percent of the SB group. We feel that narrowing the selection criteria for PR may further improve the success rate.

Association of HLA type with pseudoexfoliation of the lens capsule.

AIMS/BACKGROUND: To identify an HLA association with pseudoexfoliation of the lens capsule to support the hypothesis that there is a genetic predisposition to pseudoexfoliation. A randomised trial would be ideal for establishing a relation but is more costly and time consuming to conduct. Case-control studies provide an alternative method of establishing a relation. METHODS: The study group comprised 128 subjects who presented to a major eye centre with pseudoexfoliation. Data from blood donors representative of the population of Ireland were used to form a control group. RESULTS: An HLA association with pseudoexfoliation is identified for 14 antigens. Eleven antigens (HLA A1, A33, B8, B47, B51, B53, B57, B62, DR3, DR12, and DR13) are significantly more common in the pseudoexfoliation group while three antigens (HLA B12, B17, and DR2) are significantly less common. Four HLA antigens are strongly associated, with odds ratios of over 7.5. CONCLUSION: The strength of this HLA association is supportive evidence for a genetic component to the development of pseudoexfoliation of the lens capsule.