Journal of Aapos最新文献

Fluorescein angiography is a fluorescent dye-based imaging procedure, most commonly indicated in the pediatric setting to evaluate peripheral retinal vascular lesions. Fluorescein dye is organic, water soluble, and largely excreted renally, with a reassuring safety profile at therapeutic doses. While toxicity with intrathecal overdose has been reported, the effect of intravenous exposure to supratherapeutic levels has not been previously documented in the literature. We report the case of a 10-month-old girl with incontinentia pigmenti who received more than four times the recommended dosage of fluorescein during scheduled fluorescein angiography and developed marked yellowing of skin, sclera, stool, and urine. She was seen by the toxicology team and underwent monitoring for 8 hours before being discharged, with no adverse consequences detected during follow-up.

Posterior lenticonus is a rare congenital anomaly of the crystalline lens characterized by the conical herniation of the posterior lenticular surface with or without cortex herniation into the anterior vitreous. It is usually unilateral and axial; bilateral cases are usually familial and have syndromic associations. The irregular lenticular surface produces high myopia and irregular astigmatism producing optical distortion and hence deprivation amblyopia. We report a case of a 13-year-old girl with a unilateral pigmented posterior lenticonus associated with a retinochoridal coloboma with deprivation amblyopia.

This study highlights the disparity between the prevalence of adult strabismus in the United States and the limited number of surgeons performing adult strabismus surgery on Medicare beneficiaries. Only 12.2% of pediatric ophthalmologists billed Medicare for adult strabismus procedures during the study period. Additionally, geographic disparities of surgeons performing adult strabismus surgery were evident, with drastically different surgeon-to-patient ratios by state. Overall, the study raises concerns about the adequacy of the surgeon supply to meet the demand for adult strabismus cases.

Straatsma syndrome is an uncommon ocular condition generally comprised of myelinated retinal nerve fiber layer, myopia, and amblyopia. Associated ocular disorders include strabismus, nystagmus, optic nerve hypoplasia, and iris heterochromia. We report the case of a 6-year-old girl with unilateral Straatsma syndrome and an acquired cataract that required surgical extraction.

An 11-month-old boy with nonscarring alopecia was referred for ophthalmic evaluation because of photophobia from the age of 4 months. Whole-exome sequencing identified a heterozygous mutation in the SREBF1 gene, confirming the diagnosis of hereditary mucoepithelial dysplasia. Ocular examination revealed meibomian gland dysfunction and superficial corneal vascularization and opacity. Impression cytology of the sclerocorneal limbus revealed atypical epithelial cells. The patient received treatment for meibomian gland dysfunction, dry eye, and ocular surface inflammation. With appropriate management and close follow-up over 7 years, corneal opacity improved greatly.

In 2021, rapid magnetic resonance imaging (rMRI) became a primary imaging tool for suspected pediatric orbital cellulitis at our institution. We retrospectively reviewed the medical records of patients who underwent rMRI to evaluate the effectiveness of the protocol. A total of 31 patients were included (median age, 5.07 years). Of the 30 diagnostic scans, 11 (37%) showed preseptal cellulitis, and 19 (63%) showed orbital cellulitis. In 5 cases, orbital contrast-enhanced computed tomography (CT) was additionally ordered; rMRI and CT scan findings were similar in all 5 cases. Overall, we observed 93% (28/30) concordance of rMRI with the final clinical diagnosis.

Purpose: To evaluate frequency of surgery and post-treatment outcomes in glaucoma following cataract surgery (GFCS).

Methods: The medical records of patients with GFCS were reviewed retrospectively. Lensectomy and glaucoma surgery details and final examination findings were collected. Inclusion criteria included history of lensectomy at <1 year of age, diagnosis of glaucoma, and at least 1 year of follow-up.

Results: Of 169 eyes of 127 GFCS patients (66 male, 58 bilateral cases), 88 eyes (52%) of 73 (57%) patients underwent glaucoma surgery (median, 3.5 years of age at first glaucoma surgery; median of two glaucoma surgeries). At final follow-up (mean, 13.6 ± 7.0 years), eyes requiring glaucoma surgery had worse visual acuity (P = 0.01) and greater cup:disk ratio (P < 0.01). GFCS patients with history of bilateral congenital cataracts had better visual acuity in affected eyes than those with history of unilateral congenital cataract (P < 0.01). Angle surgery (n = 56), Baerveldt devices (n = 38), Ahmed valves (n = 19), and cycloablation (n = 21) showed 1-year survival rates between 64% and 75%. Baerveldt implants showed the highest 5- and 10-year survival rates, at 65% and 43%, respectively.

Conclusions: More than 50% of eyes with GFCS in our study cohort required at least one glaucoma surgery. Glaucoma surgery and history of unilateral cataract were associated with worse visual acuity outcomes.

A 31-year-old woman presenting with vertical diplopia and history of paresthesia in her hands the previous year was found to have a trochlear nerve palsy. Computed tomography showed no acute intracranial pathology. Magnetic resonance imaging revealed several white matter lesions with a demyelinating pattern. She was diagnosed with relapsing-remitting multiple sclerosis (MS) and started on immunotherapy, with no further exacerbation of MS.

Purpose: To describe the prevalence and risk factors associated with amblyogenic refractive error in children with Coats disease.

Methods: The medical records of children (<18 years of age) with unilateral Coats disease treated at a single tertiary care center were retrospectively reviewed. Data collected included patient demographics, ocular examinations, and treatments. Outcomes included the prevalence and factors associated with amblyogenic refractive error.

Results: A total of 50 children (82% male) were included; of these, 37 (74%) had refractive data to review. The median age at presentation was 5 years (IQR, 2-10). The Coats disease classification was stage 1 in 1 (2%), stage 2 in 29 (58%), and stage 3 or greater in 20 (40%). Most children (76%) had at least one visit with a pediatric specialist; the rest were only seen by a retina specialist. Among patients with refractive data, amblyogenic refractive error was identified in 46%. Glasses were prescribed to 50% of children. Children diagnosed at an earlier age had increased odds of amblyogenic refractive error (OR = 0.72; 95% CI, 0.57-0.91; P = 0.006) than those diagnosed at an older age.

Conclusions: Our results suggest that amblyogenic refractive error is prevalent among children with Coats disease, and refractions are not always performed. There is a need to coordinate care between pediatric and retina specialists caring for children with Coats disease to ensure timely diagnosis of amblyogenic refractive error to optimize visual outcomes in this population.

Brittle cornea syndrome type 2 is associated with corneal thinning, joint hypermobility, dental and skeletal issues, osteal fragility, and deafness. We present a rare association of congenital glaucoma with brittle cornea syndrome type 2 and keratoglobus in a patient with a novel PRDM5 gene mutation. Our case underscores the importance of genetic testing for early clinical diagnosis and tailored surgical approaches.