IMI-Management and Investigation of High Myopia in Infants and Young Children.

Purpose: The purpose of this study was to evaluate the epidemiology, etiology, clinical assessment, investigation, management, and visual consequences of high myopia (≤-6 diopters [D]) in infants and young children. Findings: High myopia is rare in pre-school children with a prevalence less than 1%. The etiology of myopia in such children is different than in older children, with a high rate of secondary myopia associated with prematurity or genetic causes. The priority following the diagnosis of high myopia in childhood is to determine whether there is an associated medical diagnosis that may be of greater overall importance to the health of the child through a clinical evaluation that targets the commonest features associated with syndromic forms of myopia. Biometric evaluation (including axial length and corneal curvature) is important to distinguishing axial myopia from refractive myopia associated with abnormal development of the anterior segment. Additional investigation includes ocular imaging, electrophysiological tests, genetic testing, and involvement of pediatricians and clinical geneticists is often warranted. Following investigation, optical correction is essential, but this may be more challenging and complex than in older children. Application of myopia control interventions in this group of children requires a case-by-case approach due to the lack of evidence of efficacy and clinical heterogeneity of high myopia in young children. Conclusions: High myopia in infants and young children is a rare condition with a different pattern of etiology to that seen in older children. The clinical management of such children, in terms of investigation, optical correction, and use of myopia control treatments, is a complex and often multidisciplinary process.

Strabismus Surgery in Hemophilia: A Novel Surgical Technique and Guideline Recommendations.

Hemophilia is a serious X-linked inheritance coagulation factor deficiency. Clinically, prolonged bleeding or delayed clotting in any area of vascular disturbance is the main manifestation of all hemophilia. We presented a 23-year-old male with a history of left sensory esotropia since the age of three. The patient had not undergone any previous eye surgery and refused to wear glasses. Hematologic studies confirmed a diagnosis of hemophilia A. Upon ophthalmologic examination, the patient's visual acuity was 20/20 in the right eye and 20/120 in the left eye, with deep amblyopia. The patient exhibited left inferior oblique overaction and a V pattern. The ophthalmologic examination otherwise revealed no abnormalities. Preoperative correction of factor VIII was deemed necessary, and the recommended dose was administered to raise the factor VIII level to 52%. The patient underwent bilateral medial rectus recession, left lateral rectus plication, and left inferior oblique myectomy. A new technique utilizing viscodissection with subconjunctival injection of a viscoelastic solution was employed to minimize intraoperative bleeding, resulting in reduced bleeding compared to standard strabismus surgery. No unusual bleeding occurred during the procedure. No postoperative bleeding was observed. The patient was discharged on the fourth postoperative day, having achieved satisfactory cosmetic alignment in the primary position with no complications related to hemophilia. In conclusion, strabismus surgery can be performed safely in strabismic patients with hemophilia. Viscodissection is a helpful novel surgical technique to decrease the risk of bleeding during surgery, and we recommend using this technique in patients using anticoagulants. A multidisciplinary team approach and strict post-operative monitoring are essential in order to achieve optimal results.

Developmental Improvement in Children With Intellectual Disability After Photorefractive Keratectomy for Severe Isoametropia.

PURPOSE: To assess the impact of refractive error correction from photorefractive keratectomy on development in children with severe isoametropia, subnormal visual acuity, and intellectual disability unable to use refraction correction. DESIGN: Prospective noncomparative interventional case series. METHODS: Before and after photorefractive keratometry (PRK), subjects who had plateaued developmentally for 18 or more months were assessed using a battery of developmental tests. The primary outcome measure was the change in the developmental quotient (DQ) 6 months after PRK. Secondary outcomes were the change in the DQ, uncorrected visual acuity, cycloplegic refraction, and corneal status 12, 24, and 36 months after PRK. RESULTS: Sixteen subjects aged 2 to 8 years were included. Twelve were highly myopic (mean, -9.69 ± 3.82 diopters [D]), 3 highly hyperopic (mean, +5.75 ± 0.59 D) and 1 highly astigmatic (mean, +3.50 D). Six months after PRK, the DQ significantly improved for expressive communication (mean, 4.51 ± 2.27 months; P = .04), interpersonal relationships (mean, 9.45 ± 4.18 months; P = .02) and coping (mean, 6.44 ± 2.10 months; P = .05). Twelve months after PRK, the DQ significantly improved for receptive communication (8.04 ± 1.80 months; P < .001), expressive communication (6.99 ± 2.27 months; P < .05), written communication (9.28 ± 3.72 months; P < .04), domestic skills (6.50 ± 2.43 months; P < .03), interpersonal relationships (10.57 ± 4.17 months; P < .02), and coping (8.41 ± 3.25 months; P < .5). CONCLUSIONS: PRK significantly improves developmental abilities of children with intellectual disability, severe isoametropia, and previously plateaued development, in addition to improving visual acuity and refractive error.

Severe congenital chorioretinitis caused by congenital lymphocytic choriomeningitis virus infection.

PURPOSE: To describe a case of congenital lymphocytic choriomeningitis virus (LCMV), a potentially severe and under-diagnosed etiology of congenital chorioretinitis. OBSERVATIONS: A 5-month old boy presented with esotropia. Examination revealed light perception vision in the right eye and normal fixation and following behavior in the left eye, and a 50PD esotropia with full versions. The external, anterior segment, and pupil exams were normal. Fundus examination demonstrated slightly pale optic nerves, numerous geographic atrophic and hyperpigmented lesions along the vascular arcades in both eyes that extended into the fovea of the right eye. Head computed tomography (CT) imaging demonstrated bilateral cerebral volume loss with consequential ex vacuo dilation of the lateral ventricles and scattered intracranial calcifications. Serum IgG and IgM titers for toxoplasmosis, rubella, cytomegalovirus (CMV), herpes simplex virus (HSV), syphilis, and zika were all negative. Upon communication of negative TORCHS titers, the mother recalled a severe rat infestation of their home during the pregnancy. A LCMV antibody titer was then ordered and which resulted positive for IgG antibodies. CONCLUSIONS AND IMPORTANCE: Congenital LCMV infection is an under-recognized cause of congenital chorioretinitis.

Persistent epithelial defect after photorefractive keratectomy in a patient with autism.

Refractive surgery has been performed under general anesthesia on pediatric and neurobehaviorally challenged adults without reported loss of vision or serious complications. Persistent epithelial defect (PED) is a rare complication of photorefractive keratectomy (PRK) in the general refractive surgery population. We report a case of PED following PRK under general anesthesia for high myopia in a man with autism and ocular history of juvenile open-angle glaucoma and dry eye syndrome.

Novel finding of atrophic extraocular muscles in Loeys-Dietz syndrome: a case report and review of the literature.

A 19-year-old man with Loeys-Dietz syndrome and right exotropic Duane syndrome after bilateral lateral rectus recessions at age 22 months presented with recurrent progressive exotropia 17 years after his initial surgery. Surgical correction was aborted intraoperatively when extreme atrophy of the right medial rectus, lateral rectus, and superior rectus muscles was observed, later corroborated by orbital magnetic resonance imaging.

Three-year observation of children 12 to 35 months old with untreated intermittent exotropia.

PURPOSE: To describe the clinical course of untreated intermittent exotropia (IXT) in children 12-35 months of age followed for 3 years. METHODS: We enrolled 97 children 12-35 months of age with previously untreated IXT who had been randomly assigned to the observation arm of a randomised trial of short-term occlusion versus observation. Participants were observed unless deterioration criteria were met at a follow-up visit occurring at 3 months, 6 months, and 6-month intervals thereafter for 3 years. The primary outcome was deterioration of the IXT by 3 years, defined as (1) a constant exotropia ≥10 prism dioptres (∆) at distance and near (i.e., motor deterioration) or (2) treatment prescribed despite not having met motor deterioration. The primary analysis used the Kaplan-Meier method to determine the cumulative proportion of participants meeting deterioration by three years and 95% confidence interval (CI). RESULTS: The cumulative probability of deterioration by 3 years was 28% (95% CI = 20%-39%). Of the 24 participants meeting the primary outcome of deterioration, seven met motor deterioration and 17 were prescribed treatment without meeting motor deterioration. The cumulative probability of motor deterioration by 3 years was 10% (95% CI = 5%-19%). CONCLUSIONS: Given the modest rate of motor deterioration over three years, watchful waiting may be a reasonable management approach in 12- to 35-month-old children with IXT. To confirm this recommendation would require a long-term randomised trial of immediate treatment versus observation followed by deferred treatment if needed.

Cortical Visual Impairment in Congenital Cytomegalovirus Infection.

PURPOSE: To describe the presentation, evolution, and long-term outcome of cortical visual impairment (CVI) in patients with symptomatic congenital cytomegalovirus (CMV) infection, and to identify risk factors for the development of CVI in patients with symptomatic congenital CMV. METHODS: Retrospective subanalysis of a long-term prospective cohort study with data gathered from 1982 to 2013. RESULTS: Eleven of 77 (14.3%) patients with symptomatic CMV, 0 of 109 with asymptomatic CMV, and 51 control patients had CVI. Overall, patients with symptomatic CMV had worse vision than patients with asymptomatic CMV, who in turn had worse vision than control patients. Microcephaly, intracranial calcification, dilatation of ventricles, encephalomalacia, seizure at birth, optic atrophy, chorioretinitis/retinal scars, strabismus, and neonatal onset of sensorineural hearing loss were risk factors associated with CVI. CONCLUSIONS: CVI may result from symptomatic congenital CMV infection. The relationship of CVI and its risk factors in patients with CMV suggests the potential to predict the development of CVI through predictive modeling in future research. Early screening of CVI in children born with symptomatic congenital CMV can facilitate educational, social, and developmental interventions. [J Pediatr Ophthalmol Strabismus. 2019;56(3):194-202.].