Congenital Paradoxical Lower Eyelid Retraction With Upgaze due to an Anomalous Extraocular Muscle.

Congenital anomalous orbital structures are rare and associated with strabismus, globe retraction, and dystopia. The authors present a case of congenital paradoxical right lower eyelid retraction with upgaze due to an anomalous extraocular muscle in a 17-year-old, healthy, female patient. Orbital computed tomography showed an intraconal, inferolateral soft-tissue band extending from the orbital apex to the inferior oblique muscle and lower eyelid. There was resolution of lower eyelid retraction and exposure symptoms after resection of the anterior portion of the fibromuscular band from the lower eyelid retractors and eyelid elevation with ear cartilage. To the authors' knowledge, this case is the first to report anomalous orbital structure as a rare cause of congenital paradoxical lower eyelid retraction, which can be improved with resection.

A randomized trial comparing Bangerter filters and patching for the treatment of moderate amblyopia in children.

OBJECTIVE: To determine whether visual acuity improvement with Bangerter filters is similar to improvement with patching as initial therapy for children with moderate amblyopia. DESIGN: Randomized, clinical trial. PARTICIPANTS: We enrolled 186 children, 3 to <10 years old, with moderate amblyopia (20/40-20/80). METHODS: Children were randomly assigned to receive either daily patching or to use a Bangerter filter on the spectacle lens in front of the fellow eye. Study visits were scheduled at 6, 12, 18, and 24 weeks. MAIN OUTCOME MEASURES: Visual acuity in amblyopic eyes at 24 weeks. RESULTS: At 24 weeks, amblyopic eye improvement averaged 1.9 lines in the Bangerter group and 2.3 lines in the patching group (difference in mean visual acuities between groups adjusted for baseline acuity = 0.38 line). The upper limit of a 1-sided 95% confidence interval was 0.76 line, which slightly exceeded a prespecified noninferiority limit of <0.75 line. Similar percentages of subjects in each group improved > or =3 lines (Bangerter group 38% vs patching group 35%; P = 0.61) or had > or =20/25 amblyopic eye acuity (36% vs 31%, respectively; P = 0.86). There was a lower treatment burden in the Bangerter group as measured with the Amblyopia Treatment Index. With Bangerter filters, neither a fixation switch to the amblyopic eye nor induced blurring in the fellow eye to worse than that of the amblyopic eye was required for visual acuity improvement. CONCLUSIONS: Because the average difference in visual acuity improvement between Bangerter filters and patching was less than half a line, and there was lower burden of treatment on the child and family, Bangerter filter treatment is a reasonable option to consider for initial treatment of moderate amblyopia.

Arachnoid cyst causing third cranial nerve palsy manifesting as isolated internal ophthalmoplegia and iris cholinergic supersensitivity.

An 8-month-old boy presented with anisocoria, a sluggishly reactive right pupil, and cholinergic supersensitivity as the only signs of what proved months later to be compressive third cranial nerve palsy due to an arachnoid cyst. Tonic constriction and dilation, segmental iris sphincter palsy, aberrant regeneration phenomena, ductional deficits, and ptosis were absent. The initial diagnosis was postganglionic internal ophthalmoplegia attributed to a viral ciliary ganglionopathy. Nineteen months later, he had developed an incomitant exodeviation and a supraduction deficit. Brain MRI revealed a mass consistent with an arachnoid cyst compressing the third cranial nerve in the right interpeduncular cistern. Resection of the cyst led to a persistent complete third cranial nerve palsy. This is the second reported case of prolonged internal ophthalmoplegia in a young child as a manifestation of a compressive third cranial nerve palsy. Our patient serves as a reminder that isolated internal ophthalmoplegia with cholinergic supersensitivity is compatible with a preganglionic compressive third nerve lesion, particularly in a young child.

Unilateral retinal hemorrhages in shaken baby syndrome.

PURPOSE: To present a detailed series of patients with unilateral retinal hemorrhages in shaken baby syndrome. METHODS: Patients with a diagnosis of Shaken Baby Syndrome evaluated by the Ophthalmology Department at Penn State University between January 1999 and January 2004 were reviewed. Patients were identified and their medical records, photographs, and computed tomographic scans were reviewed. Additional records were obtained from the Children's Hospital of Philadelphia. RESULTS: Of the 12 patients evaluated at Penn State University, the age at presentation ranged from 6 weeks to 15 months, with an average age of 4.3 months. Six (50%) had bilateral intracranial hemorrhage, while 6 (50%) had unilateral intracranial hemorrhage. The six patients with unilateral intracranial hemorrhage all had ipsilateral retinal hemorrhages. The perpetrators were male (100%) and 11 (92%) were the babies' fathers. For the five patients evaluated at Children's Hospital of Philadelphia, the age at presentation ranged from 4 weeks to 1 year, with an average of 4.35 months. Three (60%) had bilateral intracranial hemorrhage and two (40%) had unilateral intracranial hemorrhage. The perpetrators were male in four (80%) cases and three (60%) were the babies' fathers. CONCLUSIONS: These patients demonstrate the varied presentations of shaken baby syndrome. Unilateral retinal hemorrhages do not rule out the diagnosis of shaken baby syndrome.

Reliability of grading retinal hemorrhages in abusive head trauma.

PURPOSE: To study the intra- and inter-observer reliability of pediatric ophthalmologists in grading retinal hemorrhages in abusive head trauma. METHODS: Pediatric ophthalmologists attending the 2009 annual meeting of the American Association for Pediatric Ophthalmology and Strabismus participated in an onsite survey. The participants were required to compare a collection of fundus photographs of patients with abusive head trauma to two standardized photographs, A and B. Participants graded photographs as normal (no retinal hemorrhages), better than A but not normal, same as A, between A and B, same as B, or worse than B. The survey was divided into four parts: part 1 consisted of 20 photographs to be graded; part 2 was a questionnaire about participant's professional experience; part 3 consisted of 20 photographs to be graded (including 15 graded in part 1 but reordered); and part 4 asked about the criteria used to grade the photographs. RESULTS: Of the 95 participants, 92 (97%) were licensed physicians with a mean of 14 years in practice. The intra-rater reliability was found to be high, with overall average of 85% pre- and post-agreement on the common pictures. Kendall's coefficient of concordance was used as the statistical measure of inter-rater reliability and had a high value (0.82). CONCLUSIONS: Pediatric ophthalmologists showed high intra- and inter-observer reliability in grading retinal hemorrhages in abusive head trauma.

Ocular findings of oral sildenafil use in term and near-term neonates.

BACKGROUND: Sildenafil is a phosphodiesterase type 5 inhibitor used as a therapeutic adjunct in critically ill neonates with persistent pulmonary hypertension. Sildenafil is associated with several ocular complications in adults and is suspected to exacerbate retinopathy of prematurity (ROP). The risk of ocular complication in sildenafil-treated newborns, not otherwise at risk for the development of ROP, is unknown. METHODS: Twenty-two neonates with birth gestational age greater than 34 weeks and birth weight over 2,100 g who received oral sildenafil for more than 2 weeks were assessed by a pediatric ophthalmologist for potential ocular complications. RESULTS: Four patients had ocular findings: 2 had bacterial conjunctivitis; 1 had optic nerve hypoplasia, choroidal coloboma, and nystagmus; 1 had previously suffered from a hypotensive episode and had a documented cortical injury accompanied by bilateral optic disk atrophy and nystagmus. All cases seemed unrelated to sildenafil use and improved despite continued use of the drug. CONCLUSIONS: Our results do not support the need for a routine ophthalmologic examination in term and near-term neonates receiving sildenafil.

Recurrent corneal metallic foreign bodies in children with autism spectrum disorders.

Corneal metallic foreign body is a common condition in working-age adults due to occupational exposure, such as grinding, welding, and hammering. Ocular trauma is a leading cause of vision loss in children; however, corneal metallic foreign bodies are rare in the pediatric population. Here we describe 2 children suffering from autism spectrum disorders presenting with recurrent corneal metallic foreign bodies. Meticulous history revealed that both children extensively used a therapeutic home swing with metallic suspensions, suggesting the potential mechanism for the recurrent corneal metallic foreign bodies. Furthermore, the use of protective eyewear during swinging prevented further recurrences. Awareness of this potential danger is particularly important in children with disabilities, who may have difficulty communicating their concerns and cooperating during examination and treatment.