Pediatric Ophthalmoplegia and Ptosis in Epidermolysis Bullosa Simplex Associated With Muscular Dystrophy.

Oculomotor dysfunction in epidermolysis bullosa simplex associated with muscular dystrophy has been reported rarely in the ophthalmic literature. In a series of 6 patients with epidermolysis bullosa simplex associated with muscular dystrophy, 3 demonstrated ptosis, ophthalmoplegia, or both. Ptosis and ophthalmoplegia may occur early in epidermolysis bullosa simplex associated with muscular dystrophy and aid in diagnosis. [J Pediatr Ophthalmol Strabismus. 2018;55:e26-e29.].

Animal Model for Retinopathy of Prematurity Laser Surgery Training.

Peripheral retinal laser ablation for high-risk retinopathy of prematurity (ROP) improves visual outcomes. Some pediatric ophthalmology fellows receive little exposure to this procedure. The authors identified and evaluated an animal teaching model that simulates peripheral retinal laser ablation in human infants with ROP. [J Pediatr Ophthalmol Strabismus. 2017;54:e47-e49.].

Validity of ophthalmology surgical competency assessment rubric for strabismus surgery in resident training.

The Accreditation Council for Graduate Medical Education (ACGME) requires US residency programs to assess ophthalmology residents for competency in 6 core areas. Ophthalmic surgical skills are currently part of the ACGME "Patient Care" competency, although some have advocated for a seventh competency, "Surgical Skills." The Ophthalmology Surgical Competency Assessment Rubric for Strabismus Surgery in Resident Training ( OSCAR: Strabismus) tool was designed to aid in the assessment of surgical skills using procedure specific behavioral anchors. The present study evaluated inter-rater agreement of the OSCAR: Strabismus tool in the assessment of resident performance. OSCAR: Strabismus evaluations of resident surgical strabismus cases were performed by a multinational group of faculty strabismus surgeons. Cronbach α statistical analysis of the completed evaluations revealed high inter-rater agreement, indicating the OSCAR: Strabismus is a reliable tool to facilitate assessment of resident strabismus surgical skills.

Optical penalization with contact lenses for children with unilateral aphakia: an alternative to patching.

OBJECTIVES: To evaluate the efficacy of high-plus contact lenses as a form of optical penalization in children with unilateral aphakia. METHODS: An internal database review was used to identify 10 patients with unilateral aphakia after congenital cataract extraction who underwent treatment with a high-plus contact lens in their sound eye after a period of failure with traditional occlusive patching. The details surrounding the switch from patching to optical penalization with contact lens were recorded along with related changes in compliance and any demonstrable effect suggestive of an improvement in visual ability. RESULTS: After an average 15-month delay between the end of occlusive patching and the start of optical penalization with high-plus contact lenses, 6 of 10 patients achieved good compliance with high-plus contact lens therapy and most families were able to successfully administer the lenses according to the prescribed regimen. Three patients showed evidence of a fixation switch to the contact-lens-corrected aphakic eye during optical penalization of the sound eye. No safety issues were reported. CONCLUSION: Using a high-plus contact lens to optically penalize the sound eye of a unilaterally aphakic child seems to have the potential as an effective alternative to occlusive patching for those with compliance issues. A prospective study is warranted to assess long-term safety, and above all, efficacy in visual outcome.

Surgical outcomes following repair of traumatic retinal detachments in cognitively impaired adolescents with self-injurious behavior.

PURPOSE: To assess anatomic outcomes following repair of traumatic retinal detachment (RD) among a cohort of cognitively impaired adolescents with self-injurious behaviors. METHODS: Main outcome measures in this retrospective, consecutive, interventional case series were retinal attachment and postoperative complications. RESULTS: Complete retinal reattachment was initially achieved in all 9 eyes of 9 patients. Six (67%) eyes had chronic RD at initial presentation, and 7 (78%) patients had limited vision from inoperable RD in the fellow eye. Proliferative vitreoretinopathy led to recurrent RD in 4 (44%) eyes at a mean of 2.7 months. Final total or partial retinal attachment was achieved in 6 (67%) and 3 (33%) eyes, respectively, with a mean of 1.7 procedures over a mean follow-up of 22.4 months. Silicone oil emulsification led to progressive glaucoma in 4 (50%) of 8 eyes in which it was used, requiring silicone oil exchange (2 eyes) or removal (2 eyes). Recurrent RD developed in both eyes from which silicone oil was removed despite prior retinal anatomic stability for more than 6 months. CONCLUSION: Cognitively impaired adolescents with self-injurious behaviors typically presented with severe visual impairment from chronic traumatic RD in one or both eyes. Despite initial anatomic success, formation of proliferative vitreoretinopathy and further ocular trauma from continued self-injurious behaviors led to a high rate of recurrent RD. Long-term silicone oil tamponade may prevent recurrent RD in this challenging scenario but is associated with vision loss from progressive glaucoma and corneal decompensation.

Pediatric ophthalmology fellowship training in laser ablation for retinopathy of prematurity.

PURPOSE: To assess the training received by pediatric ophthalmology fellows in retinopathy of prematurity (ROP) laser ablation surgery. METHODS: We surveyed recent graduates of 1-year pediatric ophthalmology fellowships to quantify the ROP laser training they received during their fellowship, the effect of fellowship training on their desire to treat ROP in practice, and interest in additional training modalities. Electronic questionnaires were distributed to all candidates-in-training of the American Association for Pediatric Ophthalmology and Strabismus. RESULTS: The response rate was 64%, and 54 respondents met inclusion criteria. The mean number of ROP laser procedures performed during fellowship was 6.4; the median, 4. Eight respondents (15%) reported having performed no ROP laser procedures during their fellowship. Of the 54, 29 (54%) felt that they were at least adequately trained to perform ROP laser surgery and reported a mean of 10 (± 6.6) cases performed during fellowship. Respondents reporting that fellowship training was less than adequate performed a mean of 1.7 (± 1.5) cases (P < 0.01). A Pearson correlation coefficient of 0.8 revealed a moderately strong correlation between the amount of ROP laser training during fellowship and desire to perform ROP treatment in practice after fellowship. Twenty-six (48%) were very or somewhat interested in further ROP laser training. CONCLUSIONS: The amount of laser experience during fellowship strongly influences the confidence in ROP laser skills of recently graduated pediatric ophthalmologists and the desire to perform laser procedures in their clinical practice. One-half of recently graduated pediatric ophthalmologists believe that their ROP laser training during fellowship was adequate for clinical practice.

The ophthalmology surgical competency assessment rubric for strabismus surgery.

PURPOSE: To produce an internationally valid tool to assess skill in performing strabismus surgery. METHODS: A panel of 7 content experts adapted a previously published tool for assessing phacoemulsification by using a modified Dreyfus scale of skill acquisition and providing behavioral descriptors for each level of skill in each category. The tools were then reviewed by 12 international content experts for their constructive comments. The main outcome measure was a consensus of the experts on the final rubric. RESULTS: Experts' comments were incorporated, establishing face and content validity. CONCLUSIONS: The tool (Ophthalmology Surgical Competency Assessment Rubric for Strabismus Surgery (ICO-OSCAR: strabismus) has face and content validity. It can be used globally to assess strabismus surgical skill. Reliability and predictive validity are yet to be determined.

Outcomes of strabismus surgery for esotropia in children with Down syndrome compared with matched controls.

PURPOSE: Strabismus surgery dosages used in children with various neurodevelopmental disorders have been the subject of controversy. Few data have been reported regarding surgical results in individuals with Down syndrome (DS). METHODS: A retrospective, case-control study was performed in which children with DS and previous bilateral medial rectus recession surgery were matched with similar control patients without DS. Surgical results were compared using a random coefficients model for repeated measurements for each group. RESULTS: Sixteen patients with DS were matched with 16 control patients. Mean preoperative esotropia was 28.4 prism diopters (PD) in the DS group and 27.9 PD in the control group. No significant difference was found in surgical dosages between the two groups (P = .2402). Median surgical dosage was 4.4 mm in the DS group and 4.5 mm in the control group. Preoperative and 4-month and 24-month postoperative mean angles of esotropia were not different between groups (P = .8050). The 4-month postoperative mean angles of esotropia for the DS and control groups were 3.15 and 2.66 PD, respectively. The 24-month mean angles of esotropia for the DS and control groups were 7.09 and 6.60 PD, respectively. CONCLUSION: Standard bilateral medial rectus recession surgical dosages need not be modified for individuals with DS.

Ophthalmic manifestations of mosaic Down syndrome.

PURPOSE: The cognitive and physical stigmata of mosaic Down syndrome (DS) are often considered to be less severe than complete trisomy-21 DS. In contrast to complete trisomy-21 DS, the ophthalmic manifestations in mosaic DS have rarely been reported. The aim of the present study is to report clinically significant ophthalmic abnormalities in a cohort of individuals with mosaic DS. METHODS: A prospective cross-sectional observational case series was designed to evaluate ophthalmic manifestations of mosaic DS. Individuals with mosaic DS were recruited and examined at the biennial meeting of the International Mosaic Down Syndrome Association. A medical, surgical, and ocular history was obtained. Each subject received a complete eye examination on site, including assessment of visual acuity, alignment, motility, sensory function, accommodation, anterior segment, fundus, and cycloplegic refraction. RESULTS: Seventeen individuals with mosaic DS (mean age, 9 years; range, 6 months to 32 years) underwent eye examinations. Clinically significant refractive errors were present in 41% of the subjects, accommodative insufficiency in 59%, strabismus in 35%, nystagmus in 6%, and cataract in 6%. Ten individuals completed optotype visual acuity testing. Mean LogMAR acuity of the better eye of each subject was 0.2 (20/32 equivalent). CONCLUSIONS: Clinically significant ophthalmic disorders are common among children and young adults with mosaic DS. Our findings support regular periodic eye examinations for these individuals.

Pediatric intraoperative floppy iris syndrome associated with persistent pupillary membrane.

Intraoperative floppy iris syndrome (IFIS) occurring during cataract surgery in adults has been widely reported in association with tamsulosin and other α-1(a) adrenergic antagonists; however, only one case of pediatric IFIS has been previously reported and was associated with congenital cataract. We report a case of a 1-month old girl with IFIS associated with bilateral persistent pupillary membranes without cataracts and the use of preoperative topical phenylephrine and pilocarpine for IFIS prophylaxis.

Pediatric Metarrhizium anisopliae keratitis.

Metarrhizium anisopliae (M. anisopliae) is an entomopathogenic fungus used as an agricultural pesticide in many countries, including the United States. M. anisopliae grows optimally at 25°C and is therefore considered safe to humans. We report a case of a 12-year-old girl who is a soft contact lens wearer with M. anisopliae corneal ulcer who was treated effectively with topical natamycin after identification of the organism by corneal culture. Topical amphotericin B was not effective.

Novel mutation in the NHLRC1 gene in a Malian family with a severe phenotype of Lafora disease.

We studied a Malian family with parental consanguinity and two of eight siblings affected with late-childhood-onset progressive myoclonus epilepsy and cognitive decline, consistent with the diagnosis of Lafora disease. Genetic analysis showed a novel homozygous single-nucleotide variant in the NHLRC1 gene, c.560A>C, producing the missense change H187P. The changed amino acid is highly conserved, and the mutation impairs malin's ability to degrade laforin in vitro. Pathological evaluation showed manifestations of Lafora disease in the entire brain, with particularly severe involvement of the pallidum, thalamus, and cerebellum. Our findings document Lafora disease with severe manifestations in the West African population.

Pediatric airbag-associated ocular trauma and endothelial cell loss.

BACKGROUND: Airbag-associated ocular trauma among the adult population has been widely reported, but reports of these injuries in children are sparse. Laboratory experiments suggest that airbag-associated ocular trauma may cause endothelial cell loss, but reports of in vivo human endothelial cell counts are anecdotal. METHODS: A retrospective chart review was performed of all patients with airbag-associated ocular trauma at a pediatric hospital from 1995 to 2001. From 2001 to 2002, endothelial cell counts were obtained from 9 eyes of airbag-associated ocular trauma subjects and 22 eyes of control subjects. RESULTS: Sixteen patients were identified; all had periocular abrasions, edema, and/or ecchymosis. Other ocular injuries included corneal abrasions (n = 9 or 56%), corneal edema (n = 8 or 50%), hyphema (n = 7 or 44%), lens opacities (n = 5 or 31%), and macular scars (n = 2 or 12%). Three eyes of three patients required intraocular surgery. Unilateral visual loss (hand-motions, 20/100) occurred in two patients. A decrease in mean endothelial cell count of 547 cell/mm2 (P =.01) was found in the airbag-associated ocular trauma group eyes when compared with control group eyes. CONCLUSIONS: The present study includes the largest reported case series of pediatric airbag-associated ocular trauma. Airbag-associated ocular trauma may necessitate intraocular surgery, may result in permanent visual loss, and may cause endothelial cell loss in pediatric patients.