Rebecca S. Braverman

Treatment of intractable posterior uveitis in pediatric patients with the fluocinolone acetonide intravitreal implant (Retisert).

Purpose: To evaluate the efficacy and safety of the fluocinolone acetonide intravitreal implant in pediatric patients with intractable noninfectious posterior uveitis. Methods: A retrospective chart review was performed on all patients aged <18 years on whom a fluocinolone implant was used to treat intractable posterior uveitis at our institution. Conventional treatment with topical and systemic steroids and/or systemic steroid-sparing agents failed in all patients before proceeding with an implant. Each implant contained 0.59 mg of fluocinolone acetonide. Implants were placed in the inferonasal quadrant through a pars plana incision and sutured in place with 8-0 proline suture. Postoperatively, patients were followed for improvement in Snellen visual acuity, control of inflammation, and development of complications such as infection or uncontrolled intraocular pressure in the eyes. Results: The study included six eyes of four patients. Mean age at implant placement was 9.2 years (range, 6–13 years). Four eyes were pseudophakic at the time of surgery; one eye had pars plana lensectomy at the time of surgery. Mean follow-up duration was 698 days (range, 376–1,189 days). Postoperative visual acuity improved by ≥3 lines in 3 eyes. Four eyes had postoperative intraocular spikes ≥30 mmHg, with 2 eyes having ≥40 mmHg. Two of these patients required glaucoma shunting procedures postoperatively for intraocular pressure control. Inflammation was well controlled postoperatively in all six eyes with all eyes successfully weaned off of topical steroids. The single phakic eye developed a visually significant cataract 18 months postoperatively requiring cataract extraction. There were no cases of postoperative infection. There were no postoperative complications of surgical technique including no instances of wound leakage or implant dislocation. Conclusion: The fluocinolone implant can be used effectively for control of posterior inflammation in pediatric patients. As in adults, concerns for development of cataract and secondary glaucoma remain. No other safety concerns were evident in this pilot study.

Diagnosis and treatment of refractive errors in the pediatric population.

Purpose of review The diagnosis and successful treatment of visually significant refractive errors in children are a subject of continued study and debate. Recent findings Treatment of significant refractive errors is widely accepted to reduce lifelong vision loss from amblyopia. Children aged 3–5 years may be screened for unexplained vision loss, refractive errors and amblyogenic factors using traditional eye charts as well as newer modalities such as autorefractors and photoscreeners. The accuracy of various screening methods is variable throughout the literature. Debate remains as to who is best suited to administer vision screening tests. Compliance with follow-up with an eye-care professional once a child is identified with an amblyogenic factor remains suboptimal. Treatment of significant refractive errors in certain populations of pediatric patients with refractive surgery shows promise but requires further study. Summary The timely diagnosis of significant refractive errors in children remains a significant challenge, especially for ages 3–5 years, but treatment may provide significant improvement of visual acuity and quality of life.

Pharmacokinetics and Safety of Intravenous Cidofovir for Life-Threatening Viral Infections in Pediatric Hematopoietic Stem Cell Transplant Recipients

ABSTRACT Children undergoing hematopoietic stem cell transplantation (HSCT) are at risk for life-threatening viral infections. Cidofovir is often used as a first-line agent for adenovirus infections, despite the absence of randomized controlled trials with HSCT patients, and as a second-line agent for resistant herpesvirus infections. The frequency and severity of adverse effects, particularly nephrotoxicity, in pediatric HSCT recipients are unclear, and pharmacokinetics (PK) of cidofovir in children have not previously been reported. This study was an open-label, nonrandomized, single-dose pilot study to determine the safety and PK of cidofovir in pediatric HSCT recipients with symptomatic adenovirus, nucleoside-resistant cytomegalovirus (CMV) or herpes simplex virus (HSV), and/or human papovavirus infections. Subsequent dosing and frequency were determined by clinical response and side effects, as assessed by the treating physician. Blood and urine samples were obtained from patients for PK studies and assessment of toxicity and virologic response. Twelve patients were enrolled (median age, 9 years; 33.5 days posttransplantation). Four of seven patients with adenovirus infection were successfully treated and eventually cleared their infections. Four of twelve patients died of disseminated viral disease and multiorgan failure. Two of twelve patients had evidence of acute kidney injury after the first dose, and one of these patients developed chronic kidney disease; two other patients developed late nephrotoxicity. The mean drug half-life was 9.5 h. There was no correlation between nephrotoxicity and plasma maximum concentration, clearance, or half-life. PK were similar to those reported for adults, although the drug half-life was significantly longer than that for adults. Cidofovir was well tolerated in the majority of patients. However, effective therapeutic strategies are urgently needed to support patients until immune reconstitution is achieved.

Congenital Alacrima in a Patient with Blepharophimosis Syndrome

Purpose: To report a case of congenital alacrima in a patient with Blepharophimosis Syndrome (BPES). Methods: Case report of a 9-month-old female who presented with severe dry eyes. Further investigation revealed bilateral absence of lacrimal glands confirmed by CT. This unique case and its management are discussed. Results: Conservative management with artificial tears and ointment did not treat the ocular surface dryness. A combination of aggressive lubrication with surgical occlusion of the lower lid punctums was required to improve the corneal surface. Conclusion: BPES can be associated with many ophthalmic and facial abnormalities. Review of the pubmed literature, reveals this is the first reported case of alacrima and BPES. Patient with alacrima have severe ocular surface dryness, which requires aggressive and life long lubrication and tear supplementation.

Socioeconomics of Retinopathy of Prematurity In-Hospital Care

OBJECTIVE To determine if there was any uniform experience across the United States relative to retinopathy of prematurity (ROP) services provided, reimbursement, and malpractice insurance coverage. METHODS An online pediatric ophthalmology listserv poll queried pediatric ophthalmologists regarding ROP screening, reimbursement, malpractice insurance, and call and consult coverage. RESULTS Compensation for providing ROP services is quite variable around the United States. The Southern respondents reported the highest contract income while the Northeast reported the lowest. The mean annual contract income was

Pseudohypopyon: Extramedullary relapse of acute myelogenous leukemia with poor prognosis.

63 753 and the median annual contract income was

Socioeconomics of Retinopathy of Prematurity Care in the United States

39 000. There was an even distribution between physicians vs hospitals providing malpractice coverage. There was also a fairly even distribution between physicians who do and do not provide consult and call coverage. CONCLUSIONS Nationwide, there is no standard rate of compensation for ROP in-hospital care, coverage of liability insurance, or providing additional consult or on-call services. Income generation performing ROP screening examinations is roughly half what a pediatric ophthalmologist can generate by seeing patients in the clinic or performing surgery.

Validation of the CHOP model for detecting severe retinopathy of prematurity in a cohort of Colorado infants

An 11‐month‐old female presented to the emergency department with a 2‐week history of fever, increasing fussiness, emesis, and decreased urine output. She was diagnosed with acute myelogenous leukemia. Systemic chemotherapy with intensified intrathecal cytarabine was started, and the patient achieved a clinical remission after the first course of induction. Towards the end of her second course of induction she developed pseudohypopyon in each eye on consecutive days, heralding a central nervous system relapse. Pediatr Blood Cancer 2009;52:885–887.

Ocular Manifestations of Endocrinologic Diseases

Background and Purpose To elucidate the experience of pediatric ophthalmologists across the United States who care for infants with retinopathy of prematurity (ROP). Methods Seven hundred and ten surveys were mailed to U.S. members of the American Association of Pediatric Ophthalmology and Strabismus, and 283 (40%) of 710 surveys were completed. Surveys were reviewed and statistical analysis was completed by the authors. Results There was no uniformity of contract compensation or method for determining the value of ROP care. Almost half of the respondents felt they would generate more income if they did not perform ROP screening. Eighty percent of respondents that were happy with their ROP compensation had a contract for those services. One third of respondents had their malpractice insurance provided by the hospital. Retina specialists performed 40% of inpatient ROP screening and 53% of treatment. Most respondents continued to care for infants with ROP once discharged. Conclusions In the United States, there is no uniform experience regarding compensation for ROP care, or a methodology for determining the value of services and coverage of liability insurance. These findings are consistent with previous studies. Lack of uniform compensation and high liability pose a threat to the future of ROP care.

Pediatric traumatic hyphema: a review of 138 consecutive cases

Editor, A recently published large-scale study (Mimouni et al. 2016) identified hyperopia as the parameter that demonstrated the strongest association with retreatment after excimer laser refractive surgery. We do share this observation as we also note less accurate refractive predictability and, most frequently, final undercorrection after hyperopic corneal refractive surgery. To improve refractive predictability, preceding studies (Zaldivar et al. 2005; Spadea et al. 2006) suggested that preoperative cycloplegic or manifest refraction, or a combination of both (Zadok et al. 2003), could be used in the laser nomogram. Previously, we reported that a manifest-cycloplegic difference (MCD) in spherical equivalent (SE) of 1.00 dioptre (D) or more occurs in about 13% of hyperopic eyes. In these cases, a correction of the manifest SE only did not appear to be adequate (Frings et al. 2016a). In a recent multicentre study, we thus evaluated the efficacy, predictability and safety of LASIK retreatment based on manifest refraction in hyperopic patients with a preoperative difference between cycloplegic and manifest refraction of 1.00 D or less who had LASIK retreatment based on manifest refraction. We analysed the refractive outcome of 113 hyperopic eyes according to standard graphs for reporting the efficacy, predictability and safety of refractive surgery. We found that efficacy (p < 0.001) and safety (p = 0.004) were statistically significant improved by the retreatment without being negatively influenced by preoperative manifest SE, manifest cylinder or keratometry, which we analysed. Still showing a trend towards undercorrection, retreatment resulted in 88 eyes (78.0%) that reached 0.50 D of the attempted correction. The optical zone diameter of the retreatment did not correlate with efficacy, predictability or safety. Treatment predictability, however, was statistically significant worse in eyes with a preoperative SE of more than 2.50 D (p = 0.005, tested with chisquare test). In our opinion, the main objective criterion for a retreatment is a difference between targeted and achieved manifest SE of 0.50 D or more after at least 6 months. We do not retreat eyes earlier after LASIK as keratometric and refractive changes are likely to occur up to 6 months (Frings et al. 2016b). The results of our study indicate that in hyperopic eyes with a preoperative difference between cycloplegic and manifest refraction of 1.00 D or less a LASIK retreatment is efficient, predictable and safe and therefore, finally meets preoperative patients’ expectations. To summarize, the improvement of hyperopic LASIK still is a matter of discussion, hyperopes should not be treated earlier than 6 months and the difference between cycloplegic and manifest refraction should be taken into account during treatment planning. Many hyperopic cases will likely need a retreatment, most of which result in high efficacy, predictability and safety after all. We would highly recommend clarifying this fact to hyperopic LASIK candidates before the treatment.