George K. Escaravage

Age-related changes in the pediatric human orbit on CT.

Purpose: The purpose of this study was to characterize the relationships among orbital dimensions, globe diameter, and subject age. Methods: A cross-sectional, retrospective analysis of 124 CT scans of subjects with no appreciable orbital or globe disease was performed by 1 observer (G.K.E.). Seventeen length measurements and 5 angle measurements of various aspects of the orbit were obtained. Subjects of similar age were grouped and analysis was performed to define the changes in these parameters in association with age. Results: One hundred twenty-four CT scans of 124 subjects without identifiable globe or orbital disease were included in this study. Twenty-one subjects ≥17 years of age were considered mature adults and grouped together, while the remaining 106 subjects were grouped according to age. Intraobserver variability between orbital measurements was excellent, r > 0.95 (p ⩽ 0.01) for most measurements. There was no difference between right and left orbital measurements, as each was highly correlated to its contralateral counterpart. Globe diameter and all length measurements except globe protrusion increased most rapidly over the first 12 to 24 months and reached 86% to 96% of their respective adult means by age 8 years. Globe diameter and linear orbital measurements were highly correlated. Globe protrusion and measurements of orbital angles exhibited a different pattern. Conclusions: The pattern of human orbital growth is strongly correlated with the pattern of ipsilateral globe growth. This change is most rapid during the first 12 to 24 months of life and maintains a significant pace until reaching 86% to 96% of adult values for most parameters at age 8 years. After this age, the rate slows considerably until maturity. With this attempt to define normal age-related orbital change, the authors report a pattern of growth which may be clinically applicable in the management of pediatric anophthalmos.

Infiltrative subcutaneous juvenile xanthogranuloma of the eyelid in a neonate.

An 18-day-old child presented with rapid enlargement of a firm subcutaneous eyelid mass present since birth. Imaging revealed extension into the anterior orbit. Incisional biopsy revealed juvenile xanthogranuloma. Multidisciplinary systemic evaluation revealed no other lesions, although thrombocytopenia was discovered, prompting a bone marrow biopsy to rule out malignancy. Once none were uncovered, the thrombocytopenia was felt to be consistent with idiopathic thrombocytopenia. The patient was treated with intralesional steroid injections and concurrent oral prednisolone to normalize platelet count. The lesion softened, and reinjection 4 weeks later induced further regression, allowing the eyelid to clear the pupil. Although ocular involvement is a commonly reported extracutaneous manifestation of juvenile xanthogranuloma, a congenital subcutaneous eyelid lesion with orbital involvement has not been previously reported. Juvenile xanthogranuloma is typically benign and self-limiting; however, it can be associated with systemic disease, and treatment may be necessary to prevent amblyopia if the eyelid is involved. Early multidisciplinary evaluation for systemic lesions and associated malignant conditions should be considered.

Quantification of macular and optic disc hyperfluorescence after phacoemulsification in diabetes mellitus

PURPOSE: To quantify changes in areas of hyperfluorescence of the macula, perifovea, and optic disc after phacoemulsification in patients with diabetes mellitus and evaluate relationships between hyperfluorescence, macular thickness, diabetic retinopathy (DR), and logMAR best corrected visual acuity (BCVA). SETTING: Ophthalmology Clinic and Operating Room, Ambulatory Care Center, University of North Carolina at Chapel Hill, School of Medicine, and the University of North Carolina Hospitals, Chapel Hill, North Carolina, USA. METHODS: This prospective case‐control study comprised 30 eyes of 24 subjects with cataracts and diabetes mellitus with different levels of DR. Preoperatively and 2 months postoperatively, BCVA was recorded, digital retinal photography and fluorescein angiography were performed, macular thickness was measured (optical coherence tomography), and DR and hyperfluorescence of the optic disc, macula, and perifovea were quantified. RESULTS: Postoperatively, BCVA improved in surgical eyes but was unchanged in nonsurgical eyes. Preoperatively, between eyes, there was no difference in hyperfluorescence. Postoperatively, hyperfluorescence of the optic disc, macula, and perifovea increased in surgical eyes and was unchanged in nonsurgical eyes. Macular thickness increased in surgical eyes, although DR was unchanged in surgical and nonsurgical eyes. Although there was no correlation between hyperfluorescence and macular thickness in surgical eyes, increased hyperfluorescence of the perifovea was associated with less improvement in BCVA. CONCLUSIONS: After phacoemulsification, hyperfluorescence and macular thickness increased in the eyes of diabetic subjects. These short‐term changes are more likely an effect of the surgery than a worsening of DR.

Lipoblastomatosis: Case report and review of the literature

Purpose: To describe an unusual presentation of lipoblastomatosis and review the existing literature on lipoblastomatosis and lipoblastoma. Methods: Case report and literature review. Results: A 9-year-old girl was originally diagnosed as having preseptal cellulitis. After failure of systemic therapy, a CT scan showed a mass in the orbit. Biopsy of the lesion confirmed the presence of lipoblastomatosis, consisting of immature lipoblasts admixed with more-mature adipocytes. Conclusions: Lipoblastomatosis is a rare benign diffuse lipomatous tumor of embryonal fat that is uncommonly found in the head and face of children.

Monozygotic twins with incompletely concordant simple congenital ptosis in a 4-generation pedigree.

This case reports 4-year-old monozygotic twins incompletely concordant for simple congenital ptosis. Pedigree analysis demonstrates 4 generations of autosomal dominant transmission. The case and pedigree are reviewed in light of a number of recent advances in the understanding of genetic influences on simple congenital ptosis.

Pediatric Orbital Tumors

Though pediatric orbital tumors are rarely encountered outside referral centers, ophthalmologists must maintain a strong familiarity with these entities as efficient diagnosis and treatment are often paramount for optimal outcome. Most pediatric orbital tumors are biologically benign, but depending on their growth rate and anatomic location, benign tumors in the orbit can lead to vision loss, disfigurement, and even death. In this chapter, we review the literature on and discuss the most common benign and malignant orbital tumors in the pediatric population. We discuss tumor presentation, imaging characteristics, histopathologic appearance, treatment, and prognosis. Retinoblastoma and rhabdomyosarcoma are extensively discussed in other chapters in this book.