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.

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.

Strabismus surgical time-out: an illustrated whiteboard modification.

BACKGROUND: Errors in strabismus surgery-including wrong eye, wrong muscle, and wrong procedure-can occur when there is confusion about the surgical plan among members of the surgical team, including surgeons, anesthesia staff, nurses, and technicians. The purpose of this study was to assess whether implementation of a strabismus-specific whiteboard combined with oral statement of the surgical plan using nonophthalmological terminology could improve communication among the team before commencement of surgery. METHODS: A strabismus-specific whiteboard with labeled diagram of the eyes and extraocular muscles was designed. Patient identifiers, diagnosis, deviation, and procedure name were included. This whiteboard was completed preoperatively and referenced during time-out. The surgeons and operating room staff were trained in its use, and surveys were completed before and 6 months after implementation of the whiteboard time-out. RESULTS: The pre-implementation survey was completed by 19 operating room staff members, of whom 15 completed the post-implementation survey. The strabismus specific whiteboard increased staff member understanding of procedure laterality (74% to 93%), muscle(s) to be operated (37% to 93%), and specific procedure(s) planned (37% to 87%). Surgeon surveys also showed increased confidence in staff member understanding of each parameter with whiteboard use. CONCLUSIONS: A standardized time-out combined with an illustrated strabismus surgery whiteboard improves communication between team members and has the potential to reduce surgical errors.

Band keratopathy in children previously treated with diode laser for type 1 retinopathy of prematurity.

Band keratopathy is a corneal degeneration caused by chronic inflammation, systemic abnormalities, or, rarely, a primary biallelic SLC4A4 mutation leading to calcium hydroxyapatite deposition in Bowman's layer. We report a series of 16 eyes of 10 children with a remote history of diode laser treated retinopathy of prematurity who developed late-onset band keratopathy without evidence of other prior risk factors. The majority of patients developed band keratopathy bilaterally. Five eyes had visually significant central band keratopathy that required treatment with disodium ethylenediaminetetracetic acid (EDTA) chelation or phototherapeutic keratectomy. Band keratopathy may be an underreported late ophthalmic complication of diode-laser treated retinopathy of prematurity.

Corneal indices following photorefractive keratectomy in children at least 5 years after surgery.

PURPOSE: To evaluate long-term corneal outcomes in pediatric patients who underwent photorefractive keratotomy (PRK) for the treatment of refractive amblyopia. METHODS: In this prospective interventional case series, children with refractive amblyopia underwent PRK between January 1, 2007, and December 31, 2011, at Texas Children's Hospital's Department of Ophthalmology, a single tertiary eye center, and were followed for at least 5 years after surgery. Main outcome measures were 5+ years postoperative indices of corneal thickness, keratometry, degree of corneal haze, and presence or absence of keratectasia. RESULTS: Twelve eyes of 8 subjects aged 3-9 years who underwent PRK and were followed for at least 5 years were included. The mean PRK treatment dose was 8.46 D for the myopic cohort and 4.49 D for the hyperopic cohort, which removed an average of 72 µm of corneal stromal tissue in addition to the 50 µm of corneal epithelium that was removed prior to laser ablation. The mean corneal thickness was 563 µm preoperatively, which decreased to 441 µm immediately following the PRK. The mean corneal thickness 5+ years after PRK was stable, at 498 µm, because of epithelial regrowth. None of the subjects developed visually significant corneal haze or topographic evidence of keratectasia. CONCLUSIONS: In this study cohort, there were no topographic signs of keratectasia or corneal haze in children treated with PRK for high refractive error 5 years or more after surgery.

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.].

Neural Plasticity Following Surgical Correction of Strabismus in Monkeys.

Purpose: Although widely practiced, surgical treatment of strabismus has varying levels of success and permanence. In this study we investigated adaptive responses within the brain and the extraocular muscles (EOM) that occur following surgery and therefore determine long-term success of the treatment. Methods: Single cell responses were collected from cells in the oculomotor and abducens nuclei before and after two monkeys (M1, M2) with exotropia (divergent strabismus) underwent a strabismus correction surgery that involved weakening of the lateral rectus (LR) and strengthening of the medial rectus (MR) muscle of one eye. Eye movement and neuronal data were collected for up to 10 months after surgery during a monocular viewing smooth-pursuit task. These data were fit with a first-order equation and resulting coefficients were used to estimate the population neuronal drive (ND) to each EOM of both eyes. Results: Surgery resulted in a ∼70% reduction in strabismus angle in both animals that reverted toward presurgical misalignment by approximately 6 months after treatment. In the first month after surgery, the ND to the treated MR reduced in one animal and ND to the LR increased in the other animal, both indicating active neural plasticity that reduced the effectiveness of the treatment. Adaptive changes in ND to the untreated eye were also identified. Conclusions: Active neural and muscle plasticity corresponding to both the treated and the untreated eye determines longitudinal success following surgical correction of strabismus. Outcome of surgical treatment could be improved by identifying ways to enhance "positive" adaptation and limit "negative" adaptation.

Heart rate variability changes and its association with the development of severe retinopathy of prematurity.

PURPOSE: To evaluate differences in autonomic nervous system (ANS) activity associated with the development of retinopathy of prematurity. METHODS: Heart rate variability (HRV) as an indicator of ANS activity was calculated in two groups of premature infants: (1) a treatment group of infants who developed type 1 ROP and underwent treatment and (2) a control group of infants who did not develop ROP more severe than stage 1 and who were matched to the treatment group in terms of age, weight, and similar risk factors, including similar frequency of intraventricular hemorrhage, bronchopulmonary dysplasia, and sepsis. HRV was analyzed during the first 5 days of life, within 5 days of initial ROP examination, and within 5 days of ROP treatment for the treatment group or, for controls, on the day of last electrocardiogram data prior to discharge. Calculations were performed for the high frequency, low frequency, and low frequency-high frequency values of the HRV components for all infants. RESULTS: Between the initial ophthalmologic evaluation and the final evaluation, there was a tendency for reduction in both the low- and high-frequency components of the HRV indices in the treatment group, whereas there was a tendency for an increase in both components of the HRV indices in the control group. The difference in the rate of change of the high frequency between groups was statistically significant (P = 0.021). CONCLUSIONS: Disruption in ANS activity may play an important role in the development and severity of ROP.

Long-term Visual and Ocular Sequelae in Patients With Congenital Cytomegalovirus Infection.

BACKGROUND: Cytomegalovirus (CMV) is the most common congenital viral infection in the United States. Visual and ocular sequelae in adolescents and adults who are congenitally infected with CMV have not been well studied. Better understanding of the long-term visual and ocular sequelae can help with early detection, intervention and appropriate educational accommodations. METHODS: This study evaluated 237 patients (77 symptomatic, 109 asymptomatic and 51 control) who underwent a series of age-appropriate ophthalmologic, audiologic and neurodevelopmental examinations from 1982 to 2013. The frequency and etiology of visual impairment and other nonophthalmologic findings were recorded for each patient. Ophthalmologic findings were tabulated, and risk factors for abnormalities were analyzed. RESULTS: Fourteen of the 77 (18.2%) symptomatic and none of the asymptomatic and control subjects had severe visual impairments (P ≤ 0.006). Moderate visual impairment did not differ between symptomatic and asymptomatic subjects. Three asymptomatic subjects had retinal scars. The most common visual or ocular sequelae in the symptomatic group were strabismus (23.4%), chorioretinal scars (19.5%), cortical visual impairment (14.3%), nystagmus (14.3%) and optic nerve atrophy (11.7%). Three symptomatic patients had delayed visual deterioration because of later occurring retinal disorders: peripheral retinal scar, rhegmatogenous retinal detachment and Coats' disease. CONCLUSION: Symptomatic CMV patients experienced more ophthalmologic sequelae and significantly worse visual outcomes than asymptomatic CMV and control patients. Later occurring retinal disorders were found in symptomatic patients, and there is no clear evidence that CMV can reactivate in the retinas of children who were congenitally infected. Major risk factors for severe visual impairment included symptomatic status, optic nerve atrophy, chorioretinitis, cortical visual impairment and sensorineural hearing loss.

Longitudinal Evaluation of Eye Misalignment and Eye Movements Following Surgical Correction of Strabismus in Monkeys.

PURPOSE: Strabismus correction surgery is well documented in both the literature and practice with varying levels of success and permanence. Our goal was to characterize longitudinal changes in eye alignment and eye movements following strabismus correction surgery in a monkey model for developmental strabismus. METHODS: We studied two juvenile rhesus monkeys with exotropia previously induced via an optical prism-rearing paradigm in infancy. Eye misalignment was corrected via a resection-recession surgery of the horizontal rectus muscles of one eye. Binocular search coils were used to collect eye movement data during smooth-pursuit, saccades, and fixation tasks before surgical treatment, immediately after surgery, and through 6 months after treatment. RESULTS: Both animals showed an immediate ∼70% reduction in misalignment as a consequence of surgery that regressed to a 20%-40% improvement by 6 months after treatment. Significant changes were observed in saccade and smooth-pursuit gain of the nonviewing eye after surgery, which also reverted to presurgical values by 6 months. A temporary improvement in fixation stability of the nonviewing eye was observed after surgery; naso-temporal (N/T) asymmetry of monocular smooth-pursuit remained unchanged. CONCLUSIONS: Surgical realignment is followed by plastic changes that often lead to reversal of surgery effects. Immediate improvement in misalignment and changes in eye movement gains are likely a result of contractility changes at the level of the extraocular muscle, whereas longer-term effects are likely a combination of neural and muscle adaptation.

Complications of Strabismus Surgery.

All surgeries carry risks of complications, and there is no way to avoid ever having a complication. Strabismus surgery is no different in this regard. There are methods to reduce the risk of a complication during or after surgery, and these steps should always be taken. When a complication occurs, it is important to first recognize it and then manage it appropriately to allow for the best outcome possible. This article will discuss some of the more common and/or most devastating complications that can occur during or after strabismus surgery as well as thoughts on how to avoid them and manage them should they happen.

Pharmacokinetics of bevacizumab and its effects on serum VEGF and IGF-1 in infants with retinopathy of prematurity.

PURPOSE: To measure serum levels of bevacizumab and to compare serum levels of free vascular endothelial growth factor (VEGF) and insulin-like growth factor-1 (IGF-1) in infants who were treated with either intravitreal injection of bevacizumab (IVB) or laser for type 1 retinopathy of prematurity (ROP). METHODS: Twenty-four infants with type 1 ROP were randomized into three treatment groups: IVB at 0.625 mg per eye per dose, IVB at 0.25 mg per eye per dose, and laser. Blood samples were collected prior to treatment and on posttreatment days 2, 14, 42, and 60. Weekly body weights were documented from birth until 60 days post treatment. Serum levels of bevacizumab, free VEGF, and IGF-1 were measured with enzyme-linked immunosorbent assay (ELISA). RESULTS: Serum bevacizumab was detected 2 days after the injection, peaked at 14 days, and persisted for up to 60 days with half-life of 21 days. Area under the curve (AUC) analysis showed that systemic exposure to bevacizumab was variable among the subjects and was dose dependent. Serum free VEGF levels decreased in all three subgroups 2 days post treatment, with more significant reductions found in both IVB-treated groups, P = 0.0001. Serum IGF-1 levels were lower in both IVB-treated groups. CONCLUSIONS: Clearance of bevacizumab from the bloodstream in premature infants takes at least 2 months. Although serum free VEGF levels decreased following either laser or bevacizumab treatment, the reductions were more significant in the IVB-treated groups. Potential long-term effects of systemic exposure to bevacizumab in infants need to be studied further.

Evaluating the association of autonomic drug use to the development and severity of retinopathy of prematurity.

PURPOSE: To explore the association of autonomic agents with the development and severity of retinopathy of prematurity (ROP). METHODS: The medical records of all preterm infants screened for ROP were retrospective reviewed. The association between development and severity of ROP and the use and dose(s) of autonomic agents was analyzed, after adjustment for the covariates gestational age, weight, development of septicemia, intraventricular hemorrhage, and respiratory distress syndrome. RESULTS: A total of 350 infants were screened. Caffeine was used in 338 infants; dopamine in 98 infants. There was a significant association between the use of dopamine and development of ROP (P < 0.001; relative risk [RR] = 1.6 [95% CI, 1.23-2.06]) and the need for ROP treatment (P = 0.001; RR = 4.63 [95% CI, 1.82-11.79]). The number of dopamine doses was significantly associated with the development of any ROP (P < 0.001; RR = 1.07 [95% CI, 1.03-1.1]), the severity of ROP (P < 0.001; RR = 1.09 [95% CI, 1.05-1.14]), and the need for treatment (P < 0.001; RR = 1.09 [95% CI, 1.05-1.14]). The total dose of caffeine was significantly associated with the development of any ROP (P = 0.003; RR = 1.03 [95% CI, 1.01-1.05]) and the need for treatment (P = 0.006, RR = 1.073 [95% CI; 1.021-1.13]). CONCLUSIONS: Although a causal relationship was not identified, the use of the autonomic agents caffeine and dopamine was associated with the development and severity of retinopathy of prematurity in this cohort.

Periosteal Flap fixation of the Globe for Surgical Treatment of Severe Restrictive Strabismus: A Report of Eight Cases with Outcomes.

PURPOSE: Severe paretic and restrictive strabismus presents a challenging surgical problem. Despite aggressive, and often multiple, surgical attempts, patients can have recurrence of large angle binocular misalignments. In this paper, we present a series of patients who underwent apically-based orbital bone periosteal flap fixation of the globe in cases of restrictive strabismus due to isolated third-nerve and sixth-nerve palsies, multiple cranial nerve palsies, and severe ocular fibrosis syndrome. METHODS: We performed a retrospective study at our institution of patients who underwent a periosteal flap fixation. In all cases presented, the creation of the periosteal flap was performed by an orbital surgeon, and the strabismus surgery and follow-up data points were performed and collected by a strabologist. RESULTS: A total of 8 patients underwent a periosteal flap fixation of the globe. The mean age was 48 years old. Three patients had a third cranial nerve palsy, one patient had congenital fibrosis, one patient had sixth cranial nerve palsy, and three patients had multiple cranial nerve palsies. Five patients had a medial periosteal flap constructed, and 3 patients had a temporal periosteal flap. Seven of the 8 patients had stable postoperative strabometry (binocular misalignment) measurements. A single patient required an additional procedure secondary to postoperative drift (a mild recurrence of binocular misalignment). CONCLUSIONS: The surgical correction of severe paretic and restrictive strabismus is complex and can present a formidable challenge. The use of an orbital bone-based periosteal fixation flap, at our institution, has shown satisfactory outcomes not only with regard to improved postoperative deviation, but also, in that most patients required only this single procedure, usually after several prior unsuccessful interventions by standard strabismus surgery procedures.

Treatment of vertical deviations secondary to other causes.

INTRODUCTION AND PURPOSE: Surgical treatment of vertical deviations secondary to other causes can be difficult and treatment approaches may vary. The purpose of this discussion is to present surgical treatment options for the management of vertical deviations secondary to other causes including dissociated vertical deviation, primary inferior oblique overaction, superior oblique overaction, and nonspecific eccentric gaze hypertropia with diplopia. RESULTS: Surgical treatment of vertical deviations secondary to other causes is beneficial to patients. Treatment approaches may vary depending upon patient circumstances and surgeon preference. Side effects and complications from surgery to manage these conditions can occur but are usually of minor significance and not vision threatening. CONCLUSIONS: Treatment of vertical deviations secondary to other causes is effective and results usually satisfactory.

An evidence-based approach to physician etiquette in pediatric ophthalmology.

PURPOSE: Little objective evidence exists to guide physician etiquette in pediatric ophthalmology. This article describes the preferences of families visiting a pediatric ophthalmology clinic for the first time. METHODS: Review of 149 questionnaires completed by the families of patients visiting a pediatric ophthalmology clinic in a tertiary care center. The Fisher exact and chi-square tests were used to compare subpopulations. RESULTS: Most respondents preferred that their physician wear a white coat. Men preferred a handshake to a verbal greeting (P = .0264) and professional to business casual attire for both male and female physicians (P = .01, both). African-American parents were more likely to prefer being addressed by surname than other races (P = .008). No statistically significant differences were found comparing the preferences of parents with an advanced education (bachelor and graduate degrees) to those without. CONCLUSION: Pediatric ophthalmologists may wish to consider wearing white coats and business casual attire in clinic and addressing parents informally as "mom" or "dad" or by their first name, although etiquette should ultimately be determined on an individual patient basis.