Overhead Mounted Optical Coherence Tomography in Childhood Glaucoma Evaluation.

PRéCIS:: Overhead mounted spectral-domain optical coherence tomography (OCT) enables high-quality imaging of the optic nerve and macula in childhood glaucoma, and is particularly useful when standard tabletop OCT has failed or is not possible. PURPOSE: Tabletop OCT, integral to adult glaucoma management, can be limited in childhood glaucoma patients because of young age, poor cooperation, and/or technical challenges. To address these imaging difficulties, we determined the feasibility and quality of an overhead mounted unit in childhood glaucoma. Secondary aims included evaluation of peripapillary retinal nerve fiber layer (pRNFL), parafoveal total retinal thickness, and parafoveal ganglion cell complex (GCC) thickness. MATERIALS AND METHODS: Children and adults with a diagnosis of childhood glaucoma were imaged with an overhead mounted spectral-domain OCT as part of a prospective cross-sectional study. Participants had poor quality or unobtainable tabletop OCT and were scheduled for an examination under anesthesia and/or surgery as part of standard care. RESULTS: A total of 88 affected eyes in 60 of 65 (92.3%) enrolled patients (mean age, 5.9±5.9 y; range, 0.2 to 24.5) were successfully imaged. The mean image quality for analyzed scans was 22.9±6.0 dB (n=236 images). Mean values for pRNFL (80.5±31.0 µm; n=86), parafoveal total retinal thickness (301.10±39.9 µm; n=79), and parafoveal GCC thickness (96.0±21.6 µm; n=74) were calculated. CONCLUSIONS: Overhead mounted OCT allowed high-quality image acquisition and analysis in childhood glaucoma patients unable to be imaged with the tabletop counterpart, presenting an opportunity for improved clinical management and study of childhood glaucoma-related pathophysiology. pRNFL, parafoveal total retinal thickness, and parafoveal GCC thickness were decreased for affected eyes of children under 6 years of age compared with age-matched controls from a companion normative study.

Longitudinal reproducibility of spectral domain optical coherence tomography in children with physiologic cupping and stable glaucoma.

PURPOSE: To determine whether Spectralis (Heidelberg, Germany) spectral domain optical coherence tomography (SD-OCT) measurements are reproducible over time in children with physiologic cupping and stable glaucoma. METHODS: Subjects were identified from a subset of participants in an earlier retrospective study conducted by our group and included children (<18 years of age) with physiologic cupping and stable primary congenital glaucoma (PCG) having had at least 2 SD-OCTs over a period of more than 1 between April 2010 and September 2015. Thicknesses of average peripapillary retinal nerve fiber layer (pRNFL) and six individual sectors and volumes of three segmented retinal layers and total retina were measured. Spectralis review software was used for segmentation. Intraclass correlation coefficients (ICC) and coefficient of variation (COV) were calculated. RESULTS: A total of 35 eyes of 35 children were included: 15 eyes had physiologic cupping; 20 eyes, PCG. Mean ages at initial SD-OCT were 11.2 ± 3.3 years and 9.7 ± 3.3, respectively; mean intervals between first and last imaging were 2.2 ± 1.1 and 3.0 ± 1.4 years, respectively. ICCs across three visits for both groups for average and sectoral pRNFL thicknesses were 0.887-0.997 and for segmented retinal volumes were 0.806-0.993. ICCs for total retinal volume for physiologic cupping and PCG were 0.993 and 0.954, respectively. COVs for average pRNFL thickness were 0.9% and 1.7%, respectively. For all other measurements, COVs ranged from 0.3% to 5.4%. CONCLUSIONS: Reproducibility of longitudinal SD-OCT measurements for average pRNFL thickness in children with stable glaucoma over about 2 years is comparable to short-term reproducibility (COV) in normal children (1.16%) and normal and glaucoma adults (1.62%-3.4%).

Ultrasound biomicroscopy measurement of Schlemm's canal in pediatric patients with and without glaucoma.

PURPOSE: To compare the diameter of Schlemm's canal in children with and without congenital glaucoma as measured in vivo by means of ultrasound biomicroscopy. METHODS: In this prospective single-center study of pediatric subjects (<18 years of age) the diameter of Schlemm's canal in nonglaucomatous and glaucomatous eyes was compared. An 80 MHz iUltrasound probe (iScience Interventional Inc, Menlo Park, CA) placed near the limbus was used to identify and measure the canal's diameter with special attention to the anterior segment anatomy (especially in subjects with congenital glaucoma). RESULTS: A total of 20 subjects were included; mean age of subjects without glaucoma was 6.6 ± 6.65 years; of those with glaucoma, 9.4 ± 11.80 months. The mean canal diameter in nonglaucomatous eyes was 142 ± 33.2 µm (range, 90-196 µm); in glaucomatous eyes, 64.9 ± 10.90 µm (P = 0.007). Schlemm's canal could not be identified in 50% of patients with congenital glaucoma. There was a trend toward smaller canal diameter in subjects with no glaucoma <2 years old. Mean canal diameter in nonglaucomatous eyes was 103 ± 8 µm (range, 90-115 µm) in subjects <2 years of age and 161 ± 20 µm (range, 110-196 µm) in subjects >2 years of age (P = 0.0012). CONCLUSIONS: In our pediatric study cohort the diameter of Schlemm's canal varied by age and presence of glaucoma.

[Trabeculotomy before the estimated delivery date. Case report of a preterm infant with buphthalamus]. / Trabekulotomie vor dem errechneten Geburtstermin. Falldarstellung eines Frühgeborenen mit Buphthalmus.

Congenital glaucoma is a disease potentially leading to blindness in children. It poses a diagnostic and therapeutic challenge even though new knowledge has been acquired and a sufficient understanding of the pathogenesis has been gained. New discoveries, such as the exact time when Schlemm's canal develops could lead to a prenatal diagnosis and therefore surgical intervention so that other complications including blindness can be avoided. This case report demonstrates that an early prenatal eye screening with ultrasound (after approximately 30 weeks of pregnancy) would be desirable in order to diagnose buphthalmus early and to plan postnatal surgery.

[Buphthalmia secondary to congenital pigmented epithelial iris cyst]. / Buphtalmie secondaire à un kyste congénital de l'épithélium pigmentaire de l'iris.

We report the case of a 2-day-old child with buphthalmia caused by a congenital pigmented epithelial iris cyst. The use of ultrasound biomicroscopy (UBM) and high-resolution echography (20MHz) specified the diagnosis before general anesthesia for complete examination and treatment. Surgery can control intraocular pressure and the opening of the visual axis; there is, however, relative amblyopia. This case confirms the advantage of UBM in examining tumors of the anterior segment. The early diagnosis, facilitated by use of the UBM, provided quick and well-adapted treatment.

The effect of buphthalmos on orbital growth in early childhood: increased orbital soft tissue volume strongly correlates with increased orbital volume.

PURPOSE: Our purpose was to evaluate the effect of increased orbital soft tissue volume on orbital growth. METHOD: Patients with unilateral or significantly asymmetric bilateral buphthalmos as determined by axial computed tomography scan were recruited. Volumetric determinations of the bony orbit with use of axial 1.5 mm sections on computed tomography were undertaken. Statistical analysis of the paired ocular length measurement and bony orbital volume measurements for each patient were performed. RESULTS: Eight patients (mean age 41 months) with a 15% or greater difference in axial length were enrolled. The mean axial length of the buphthalmic globes was 23% greater than that of the contralateral globes. Orbits harboring a buphthalmic globe had an orbital volume 11% greater than on the contralateral side. CONCLUSION: Increased orbital soft tissue volume as evidenced by buphthalmos was significantly associated with enlarged bony orbital volume. This indicates that soft tissue volume is a determinant of orbital volume and suggests that orbital tissue expanders might enhance bony development in patients with anophthalmos or microphthalmos and after early enucleation.