INTRAVITREAL 0.18-mg FLUOCINOLONE ACETONIDE IMPLANT FOR PEDIATRIC UVEITIS.

PURPOSE: This study reports the outcomes of the 0.18-mg intravitreal fluocinolone acetonide implant in the treatment of pediatric noninfectious uveitis. METHODS: A retrospective cohort study was performed on patients under 18 years old who received fluocinolone acetonide implant between June 1, 2020 and March 1, 2023. Data collected included demographics, uveitis diagnosis, use of anti-inflammatory therapy, visual acuity, intraocular pressure, and grading of uveitis activity. Uveitis recurrence was defined as increased inflammation that required additional anti-inflammatory therapy. RESULTS: Eleven eyes from seven patients were included in this study. One patient (one eye) had a diagnosis of immune recovery uveitis and the remaining six patients (10 eyes) had pars planitis. The rate of remaining recurrence-free was 82% at 6 months, 60% at 12 months, and 60% at 24 months. Two of the six phakic eyes at baseline required cataract extraction during follow-up. Two of the four eyes that did not have intraocular pressure-lowering surgery before implantation required surgery in follow-up. CONCLUSION: The 0.18-mg fluocinolone acetonide implant has a similar efficacy for the treatment of pediatric uveitis, particularly pars planitis, as in the adult population, although with higher rates of ocular hypertension requiring intervention.

Clinical outcomes in paediatric tubulointerstitial nephritis and uveitis syndrome (TINU).

PURPOSE: To evaluate the clinical presentation, course, and outcomes of uveitis in paediatric patients with tubulointerstitial nephritis and uveitis syndrome (TINU). METHODS: Multicentric Retrospective Cohort Study 110 patients ≤21 years of age diagnosed with TINU from 10 sites across the United States and Canada. Clinical diagnosis of TINU required uveitis diagnosed by an ophthalmologist, elevated serum creatinine (SCr) and elevated urine ß2-microglobulin (ß2M) or abnormal urinalysis. Renal biopsy and systemic illness were not mandatory. Univariate and multivariate analysis was performed to analyse risk factors and treatment modalities. RESULTS: Median age was 13 years (Range (5.9-18.4); 52% male); median follow-up, 1.6 years (IQR 0.98-4.02). Uveitis was symptomatic in 90%, with bilateral anterior uveitis in 94%. Ninety-two (84%) patients required immunomodulatory treatment (IMT). Methotrexate (n = 44) and mycophenolate mofetil (n = 39) were the first agents after oral corticosteroids. 45% required addition of biologic agents (Adalimumab [n = 33], Infliximab [n = 8]). Younger age (p = 0.018), male sex (p = 0.011), and higher uveitis grade at presentation (p = 0.031) were associated with greater IMT ( ≥ 2) requirement. 53% had uveitis recurrence compared to 16% with nephritis recurrence. At the most recent visit, nephritis was controlled in 90%, while uveitis in 74%. Four (4%) patients required glaucoma surgery. Nine (8%) patients had renal complications. CONCLUSIONS: Most patients with TINU require steroid-sparing IMT for control of uveitis, with nearly half requiring addition of biologic agents. Urinalysis, urine ß2M and SCr testing should be considered in children presenting with uveitis, especially when the disease is bilateral and anterior.

Ophthalmic manifestations of ruptured arachnoid cysts in children.

BACKGROUND: Intracranial arachnoid cysts are relatively common in the pediatric population. Rarely, they rupture, leading to acute subdural fluid collections, which can cause a sudden increase in intracranial pressure. The purpose of this study was to characterize ophthalmic sequelae in a large cohort of these patients. METHODS: The medical records of all children treated for ruptured arachnoid cysts who presented at a single tertiary pediatric hospital for initial assessment between 2009 and 2021 were reviewed retrospectively. RESULTS: Of 35 children treated for ruptured arachnoid cysts during the study period, 30 received ophthalmological examination. Papilledema was found in 57% of these children, abducens palsy in 20%, and retinal hemorrhages in 10%. Of the 30 children, 22 were seen in outpatient follow-up, of whom 5 had a best-corrected visual acuity of 20/40 or worse in one or both eyes at most recent follow-up. Cranial nerve palsies resolved in all cases without strabismus surgery. CONCLUSIONS: Given high rates of papilledema, cranial nerve palsies, and vision loss, all children with ruptured arachnoid cysts should be evaluated by pediatric ophthalmologists.

Subtenon Triamcinolone Acetonide Injection with Topical Anesthesia in Pediatric Non-Infectious Uveitis.

INTRODUCTION: Subtenon triamcinolone acetonide (Kenalog®; Bristol Myers Squibb) (STA) injections are commonly used in the treatment of adults in an outpatient setting. However, publications on detailing its outpatient use, safety, and efficacy in the pediatric population are scarce. METHODS: We reviewed STA injections performed in children in the outpatient clinics at two tertiary centers from 2014 to 2020. All children were aged ≤ 18 years and had a diagnosis of non-infectious uveitis. STA injections were done using 0.5 cc (20 mg) triamcinolone injected superotemporally with only topical anesthesia. Data on the efficacy and safety of STA in treating inflammation and compiled data on visual acuity improvement and incidence of ocular complications were evaluated. RESULTS: Forty-eight eyes in 30 patients were included. The mean age of patients was 13.1 (range 7-18) years. There were no immediate complications observed in all injections performed. At the 3-month follow-up, inflammation had improved in 85.4% of eyes, macular edema had resolved in 77.8% of eyes, and there was significant vision improvement after STA. At 6 months after STA, the incidence of ocular hypertension was 12.5% and no new cataracts had developed. CONCLUSION: STA injection with topical anesthesia was a well-tolerated, reasonable alternative for short-term treatment of uveitis among this pediatric population.

Clinical Utility of Electroretinograms for Evaluating Vigabatrin Toxicity in Children.

PURPOSE: To determine changes in the clinical treatment of pediatric patients taking vigabatrin for seizure control in response to results of electroretinogram (ERG) performed for retinal toxicity screening. METHODS: The authors retrospectively reviewed the medical records of patients who received ERGs at Children's Hospital of Colorado from 2009 to 2012. Age, indication for ERG, ERG data, and clinical management of vigabatrin were extracted from the records. ERGs were interpreted according to LKC Technologies normative values. A physician trained in ERG analysis interpreted each ERG. RESULTS: One hundred seventy ERGs were performed during the study period, and 147 ERGs were available for analysis. Every patient received general anesthesia for the procedure. Thirty-three ERGs were performed in 29 patients specifically as screening for retinal toxicity due to vigabatrin use, and 30 were available for analysis. Within this cohort, only 2 ERGs were normal (6.6%), and 28 were abnormal (93.3%). In patients who received abnormal results, 1 patient discontinued vigabatrin in response to the screening. CONCLUSIONS: In this study cohort, clinical management generally did not change in response to an abnormal screening result. Given the need for general anesthesia in the pediatric population receiving ERG testing, and minimal change in clinical decision-making in the face of abnormal results, ERG screening for retinal toxicity due to vigabatrin in the pediatric cohort should be reconsidered. [J Pediatr Ophthalmol Strabismus. 2021;58(3):174-179.].

Comparison of Different Surgical Approaches to Inferior Oblique Overaction.

BACKGROUND: The purpose of this study is to compare surgical outcomes of recession, anteriorization, and myotomy for the treatment of inferior oblique overaction (IOOA). METHODS: A retrospective chart review of all patients undergoing IOOA correction from July 2010 to March 2017 at the Children's Hospital of Colorado was performed. Preoperative grading of IOOA (+0.5 to +4.0) was compared to post-operative IOOA (0 to +4.0). The goal was reduction of IOOA to 0, but any decrease in IOOA was measured. RESULTS: There were a total of 260 patients with 357 eyes. Gender and age were similar across surgery types. A 94.6% of eyes had a decrease in IOOA with recession (n = 165) of the inferior oblique while 86.1% decreased to no IOOA. Anteriorization of the inferior oblique (n = 115) decreased overaction in 97.4% of eyes with 81.7% improving to zero degree of IOOA. Myotomy of the inferior oblique (n = 77) was found to decrease overaction in 98.7% of eyes and reduce IOOA to zero in 88.3%. There was no significant difference among type of surgery and outcome. CONCLUSION: All three surgical interventions were found to be equally successful in reducing the amount of IOOA.

Ocular Findings in Pontine Tegmental Cap Dysplasia.

PURPOSE: To describe the ocular complications experienced by patients with pontine tegmental cap dysplasia (PTCD) and the management strategies used to care for these children. METHODS: Subjects with PTCD were recruited through social media advertisement and completed a survey gathering information on potential ocular problems related to the patient's PTCD disease and any current or previous treatments. RESULTS: Twenty-two patients or guardians completed the survey. Neurotrophic cornea was the most common ocular diagnosis (82%), followed by facial palsy (59%), dry eye syndrome (59%), and blepharitis (55%). Other diagnoses included cortical visual impairment (27%), strabismus (27%), amblyopia (18%), and nystagmus (18%). Common treatment modalities included lubricating eye drops (59%) or ointment (50%), contact lenses (14%), punctal plugs (27%), glasses (45%), and patching (18%). The most common surgical interventions were temporary or permanent tarsorrhaphy (64%) and amniotic membrane grafts (23%). In total, 68% of families reported self-injury to eyes and 91% reported the child to be primarily a visual learner. CONCLUSIONS: PTCD is a newly described, very rare disorder with a variety of vision-threatening ocular manifestations. It is essential that the ophthalmologist be aware of the potential for neurotrophic cornea because timely treatment could prevent corneal scarring, perforation, and blindness.

A Cross-Sectional Online Survey Identifies Subspecialty Differences in the Management of Pediatric Cataracts Associated with Uveitis.

INTRODUCTION: To determine if differences exist between pediatric ophthalmologists and uveitis ophthalmologists in the treatment of pediatric uveitic cataracts and placement of intraocular lenses. METHODS: Uveitis ophthalmologists and pediatric ophthalmologists were surveyed via an online poll regarding their therapeutic management of pediatric uveitic cataract and intraocular lens (IOL) placement. RESULTS: Sixty-two responses from uveitis ophthalmologists and 47 responses from pediatric ophthalmologists were recorded. According to 79% of all responses, uveitis was not a contraindication for primary IOL implantation in patients with controlled intraocular inflammation. Pediatric ophthalmologists were more likely to respond that the presence of chronic juvenile idiopathic arthritis-associated iridocyclitis, pars planitis, or recurrent acute anterior uveitis is a contraindication for primary IOL implantation in pediatric cases with full control of intraocular inflammation. There was no consensus within either specialty with regard to the preferred IOL material for lens implantation. Uveitis ophthalmologists were more likely to report the use of intravenous and intravitreal steroids for perioperative treatment. In cataract surgery for a child with recurrent acute anterior uveitis, a higher percentage of uveitis ophthalmologists (71%) than pediatric ophthalmologists (50%) responded that the posterior capsule should be primarily opened. A higher percentage of uveitis ophthalmologists also stated that anterior vitrectomy should be performed at the time of cataract surgery in all three uveitis types. CONCLUSIONS: Pediatric ophthalmologists and uveitis ophthalmologists have similar approaches to the management of pediatric uveitic cataract removal and IOL insertion, but several differences remain between these subspecialties. Continued collaboration between the subspecialties would be helpful to better develop consistent criteria to improve patient care.

Relationship between severe bronchopulmonary dysplasia and severe retinopathy of prematurity in premature newborns.

BACKGROUND: Bronchopulmonary dysplasia (BPD) and retinopathy of prematurity (ROP) are two adverse sequelae of preterm birth associated with abnormal vascular development. The purpose of this study was to characterize the relationship between these two outcomes at a single institution. METHODS: The medical records of infants screened for ROP at the University of Colorado Hospital between January 2012 and December 2017 were reviewed retrospectively. ROP was classified according to Early Treatment Retinopathy of Prematurity (ET-ROP) criteria; BPD, according to the 2010 Criteria from the National Institute for Child Health and Human Development. We examined the relationship between moderate-severe BPD and the development of severe ROP (type 1 or 2) using univariate analysis and multivariable logistic regression with the odds ratio as a measure of association. Covariates included gestational age and birth weight at delivery. RESULTS: A total of 625 cases were reviewed. Of these, 64 infants (10%) developed severe ROP and 176 (28%) infants developed moderate-severe BPD. We found a significant relationship between these two outcomes following adjustments for gestational age, birth weight, and multiparity (OR = 3.2; 95% CI, 1.6-6.5 [P < 0.01]). CONCLUSIONS: In our cohort of preterm infants, we found a significant relationship between moderate-severe BPD with severe ROP. We hypothesize that these two neonatal outcomes have links with a common pathogenesis.

COL4A1 mutations in two infants with congenital cataracts and porencephaly: an ophthalmologic perspective.

COL4A1 mutations present with a spectrum of clinical phenotypes often involving the cerebrovascular and ophthalmic systems. We report 2 cases of COL4A1 mutations that presented with congenital cataracts and porencephaly. Both patients had posterior cortical cataracts and radiographically defined bilateral posterior lenticonus. Considering the long-term clinical implications of these mutations, posterior cortical cataracts, bilateral posterior lenticonus, and porencephaly should raise clinical suspicion for COL4A1 mutations.

Frequency of pediatric traumatic cataract and simultaneous retinal detachment.

PURPOSE: Traumatic cataract in children is a treatable cause of vision loss. In cases of simultaneous retinal detachment, the prognosis for visual recovery is often poor. The purpose of this study was to investigate risk factors for concurrent retinal detachment in patients with traumatic cataract. METHODS: A retrospective review of patients diagnosed with traumatic cataract at Children's Hospital Colorado between 2005 and 2014 was conducted. Demographics, mechanism of injury, and incidence of retinal detachment were recorded. Logistic modeling with generalized estimating equations to account for correlation of eyes within patients was used to analyze associations between potential risk factors and retinal detachment. RESULTS: A total of 62 total eyes with traumatic cataract were included: 52 patients presented with unilateral cataract; 5 presented with bilateral cataracts. Mean patient age was 8.4 ± 4.1 years (range, 0-16 years), and 83% of patients were male. A total of 9 eyes (14.5%) had comorbid retinal detachment. Traumatic cataracts caused by self-injurious hitting were more likely to present with simultaneous retinal detachment than those caused by other mechanisms of injury (OR = 24.0; 95% CI, 3.8-153.3; P = 0.0010). CONCLUSIONS: Patients with traumatic cataract who display self-injurious behavior are at higher risk for concurrent retinal detachment. These patients can often only be examined under sedation. Ophthalmologists should counsel families of high-risk patients and consider involving retinal specialists in surgical planning.

Maternal Serum Eye Drops in the Management of Pediatric Persistent Corneal Epithelial Defects: A Case Series.

PURPOSE: We report our experience with the use of maternally derived serum eye drops as adjunctive treatment in the management of pediatric persistent corneal epithelial defects. METHODS: Five eyes of 4 patients were identified in a retrospective review of pediatric patients with persistent corneal epithelial defects who received maternal serum drops. Diagnoses associated with the defects comprised pontine tegmental cap dysplasia with bilateral cranial nerve V1, V2, V3, and VII palsies; pontine tegmental cap dysplasia with left cranial nerve V1, VII, and VIII palsies; traumatic left cranial nerve II, V1, V2, and VI palsies due to a basilar skull fracture; and Stevens-Johnson syndrome with ocular involvement. We evaluated the feasibility of using maternally derived serum drops; thus, we looked at the ability to prepare and tolerate the drops as well as any complications that could have been associated with treatment. Other data collected included visual acuity, corneal examination, and current and previous treatments. RESULTS: Both the duration of therapy and time of follow-up ranged from 5 to 28 months. All patients experienced improvement or resolution of their corneal epithelial defects within 3 weeks of initiating serum eye drops. Furthermore, there were no adverse effects from the use of allogeneic serum drops. CONCLUSIONS: Maternal serum eye drops are a well-tolerated and potentially beneficial addition to the management of pediatric persistent corneal epithelial defects.

Validation of WINROP for detecting retinopathy of prematurity in a North American cohort of preterm infants.

BACKGROUND: WINROP (weight, insulin-like growth factor 1, neonatal, retinopathy of prematurity) is a web-based retinopathy of prematurity (ROP) risk algorithm that uses postnatal weight gain as a surrogate of insulin-like growth factor-1 (IGF-1) to predict the risk of severe ROP in premature infants. The purpose of this study was to validate the web-based algorithm WINROP in detecting severe (type 1 or type 2) ROP in a North American cohort of infants. METHODS: The records of consecutive infants who underwent ROP examinations between 2008 and 2011 were reviewed retrospectively. Infants were classified into categories of "alarm" (at risk for developing severe ROP) and "no alarm" (minimal risk for severe ROP). RESULTS: A total of 483 were included. Alarm occurred in 241 neonates (50%), with the median time from birth to alarm of 2 weeks. WINROP had a sensitivity of 81.8% (95% CI, 67.3%-91.8%) and specificity of 53.3% (95% CI, 48.5%-58.0%) for identifying infants with severe ROP. Eight of the 44 infants with severe ROP were not detected (5 with type 1 and 3 with type 2). Of these 8 infants, 7 (88%) had birth weight in excess of the 70th pecentile. With additional weight data entry, sensitivity of WINROP rose to 88.6%. CONCLUSIONS: Very preterm infants (gestational age of ≤27 weeks) with relatively high birth weight for gestational age may not be detected by WINROP as high risk for developing severe ROP.