Intraoperative Mounted Optical Coherence Tomography Findings Following Reversal of Optic Nerve Head Cupping in Childhood Glaucoma.

PURPOSE: To examine the structural changes occurring in the optic nerve head (ONH) and macula in infants with childhood glaucoma and clinically observed ONH cupping reversal following intraocular pressure (IOP)-reducing glaucoma surgery, as captured by intra-operative spectral-domain optical coherence tomography (SD-OCT). DESIGN: Retrospective observational case series from an ongoing prospective cohort study. METHODS: Included were 18 eyes of 14 patients with childhood glaucoma. All eyes had SD-OCT imaging pre- and post-glaucoma intervention and clinically identified ONH cupping reversal. Patients with poor quality images or persistent optic nerve swelling following IOP reduction were excluded. Outcome measurements included IOP, cup-to-disc ratio, axial length and SD-OCT measurements of the peripapillary retinal nerve fiber layer (pRNFL), transverse horizontal diameter of Bruch membrane opening (BMO-D), cup depth, and macula. RESULTS: Mean age at surgery was 1.14±0.93 years and mean interval between pre- and post-operative imaging was 127 days (range 35-595). Following intervention, mean IOP reduction was 45%, accompanied by significant reductions in the cup-to-disc ratio (0.30±0.12, p<0.001), axial length (0.43±0.28mm, p<0.001) and cup depth (46%, p<0.001). Mean global pRNFL thickness pre- vs. post-treatment was 93.1±14.7µm vs. 93.1±17.1µm, respectively, p=1.0. There was no significant difference in pre- and post-treatment global or sectoral pRNFL, 3mm macular total and segmented retinal layer volumes, or the BMO-D. CONCLUSIONS: Clinical ONH cupping reversal after IOP-lowering surgery was associated with axial length reduction and decrease in cup depth, but no significant change in the pRNFL or macular volume measures. ONH cupping reversal likely marks stabilization but any pre-intervention ONH damage persists.

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%).

Nonglaucomatous Cupping: Fundus Photography and Spectral Domain Optical Coherence Tomography Imaging Features.

Nonglaucomatous cupping is commonly encountered in neuro-ophthalmic practice. However, the progression of clinical and imaging findings over time has not been well described. We present serial fundus photographs and spectral domain optical coherence tomography from a pediatric patient with neuromyelitis optic spectrum disorder, which demonstrated progression of both cupping and optic atrophy in the setting of normal intraocular pressure.

The macula in pediatric glaucoma: quantifying the inner and outer layers via optical coherence tomography automatic segmentation.

BACKGROUND: Recent Spectralis (Heidelberg, Germany) spectral domain optical coherence tomography (SD-OCT) research software can automatically quantify the thickness of each individual retinal layer. The macular ganglion cell layer (GCL) and ganglion cell complex may be more sensitive for detecting glaucoma than the peripapillary retinal nerve fiber layer (pRNFL). The aim of this study was to characterize and compare the volume of each macular layer in the eyes of children with glaucoma versus those of normal controls. METHODS: The medical records of children with primary glaucoma and physiologic cupping who had undergone Spectralis SD-OCT imaging of the macula and pRNFL were reviewed retrospectively. Controls were recruited from a separate prospective study. Children with refractive error of <±5 or retinal or neurologic abnormalities were excluded. The average volume of each of the 8 retinal layers in the macula (central 6 mm) and pRNFL were compared among diagnostic groups. RESULTS: A total of 80 eyes of 80 children were included: 37 glaucoma eyes (25 with primary congenital and 12 with juvenile open-angle glaucoma) and 43 nonglaucoma eyes (28 with physiologic cupping). Eyes with glaucoma had significantly thinner mean macular nerve fiber layers, ganglion cell layers, inner plexiform layers, and pRNFLs than nonglaucomatous eyes: 0.82 ± 0.24 µm versus 1.00 ± 0.12 µm; 0.93 ± 0.22 µm versus 1.13 ± 0.10 µm; 0.80 ± 0.14 µm versus 0.91 ± 0.07 µm; 81.6 ± 26.5 µm versus 102.7 ± 10.0 µm, respectively (P < 0.00556 for all). Eyes without cupping and those with physiologic cupping were equivalent for all variables tested. CONCLUSIONS: Children with glaucoma have thinning of the three innermost retinal macular layers.

Cupping reversal in pediatric glaucoma--evaluation of the retinal nerve fiber layer and visual field.

PURPOSE: To identify optic nerve head (ONH) cupping reversal and associated optical coherence tomography (OCT) and Humphrey visual field changes in pediatric glaucoma. DESIGN: Retrospective observational case series. METHODS: Sequential surgical cases of juvenile open-angle glaucoma (OAG) or primary congenital glaucoma (PCG) with sustained postoperative intraocular pressure (IOP) reduction. Group 1 had preoperative and postoperative ONH photographs and OCT; Group 2 had preoperative clinical ONH assessment and postoperative imaging. Cupping evaluation was confirmed by masked glaucoma and neuro-ophthalmology specialists. RESULTS: Of 80 cases, 9 eyes (9 children) met criteria for Group 1; 24 eyes (19 children) met criteria for Group 2. Group 1: Five of 9 eyes (56%) demonstrated cupping reversal, with preoperative vs postoperative mean IOP 34.2 ± 6.6 mm Hg vs 10.6 ± 4.1 mm Hg (P < .00001) and mean average retinal nerve fiber layer (RNFL) 71.0 ± 30 µm vs 62.8 ± 24 µm (P = .4), respectively. RNFL was stable in 4 of 5 eyes (all juvenile OAG), but thinned (Δ = -41 µm) in 1 eye with PCG. Humphrey visual fields (reliable in 2 of 3 eyes) showed no significant change. Group 2: Fourteen of 24 PCG eyes (58%) demonstrated cupping reversal, with preoperative vs postoperative mean IOP 36.1 ± 8.9 mm Hg vs 13.3 ± 2.1 mm Hg (P < .00001). Two eyes had thin RNFL postoperatively despite healthy-appearing ONH. Postoperative RNFL showed statistically significant linear correlation with preoperative (but not postoperative) cup-to-disc ratio. Limitations include small numbers, few reliable Humphrey visual fields, and absent preoperative imaging (Group 2). CONCLUSION: Some eyes with IOP reduction and ONH cupping reversal show continued RNFL thinning postoperatively. The preoperative ONH cup-to-disc ratio predicted the postoperative RNFL better than the postoperative "reversed and smaller" cup-to-disc ratio. Cupping reversal in pediatric glaucoma may not predict improved ONH health and deserves further study.

Optical coherence tomography in paediatric glaucoma: time domain versus spectral domain.

BACKGROUND/AIMS: Spectral-domain (SD)- Optical Coherence Tomography (OCT) can track eye movements, has faster acquisition time and higher resolution than time-domain(TD)-OCT. The aim of the study was to assess the utility of SD-OCT in paediatric glaucoma and determine its agreement with TD-OCT. METHODS: Children who had SD-OCT(Spectralis, Heidelberg-Engineering,Germany) were retrospectively and prospectively identified from Duke paediatric glaucoma clinic. The peripapillary retinal nerve fibre layer (RNFL) and macular thickness and volume (MV) were compared amongst four groups: normal eyes, eyes with physiologic cupping (C:D >0.5 and <0.8, IOP <21), mild glaucomatous eyes (C:D <0.5, intra-ocular pressure (IOP) >21) and severe glaucoma (C:D>0.5, IOP>21). SD-OCT values were compared to TD-OCT(OCT-3, Carl-Zeiss-Meditec, Dublin, CA) values in a subset of subjects who had same day scans using both instruments. Children with neurologic disorders, refractive error >±5D, pseudophakia and prematurity were excluded. RESULTS: Included were 83 eyes of 83 children, mean age 11.9 ± 4.2 years. SD-OCT measurements of average RNFL thickness and MV differed among normals(n=24), physiologically cupped (n=31), mild (n=15) versus severe glaucoma (n=13): (RNFL:104 ± 9, 99 ± 6, 98 ± 9 vs 62 ± 18 µm, respectively, p<0.05; MV: 8.7 ± 0.3, 8.6 ± 0.3, 8.8 ± 0.4 vs 8.0 ± 0.6 mm³;, respectively, p<0.05). Same-day SD-OCT and TD-OCT measures in 53 eyes correlated linearly (RNFL r(2)=0.88; MVr(2)=0.58). SD-OCT measured lower RNFL and higher macular thickness than TD-OCT. Among eyes with severe glaucoma, 4 of 13 (30%) had unreliable TD-OCT but reliable SD-OCT. CONCLUSIONS: SD-OCT was easier to obtain than TD-OCT in children. SD-OCT and TD-OCT measurements correlated, but values were not interchangeable.

Longitudinal reproducibility of optical coherence tomography measurements in children.

PURPOSE: To evaluate the longitudinal reproducibility of optical coherence tomography (OCT) measurements in normal and glaucomatous eyes of children. METHODS: In this 2-setting prospective study, OCT-3 was used to obtain fast retinal nerve fiber layer (RNFL) and macular thickness scans. In the first study setting, the normal eyes of healthy children were scanned on presentation, at 2 weeks, and 3 years, with axial length measured at the first and last examinations. In the second setting, OCT scans of patients in the pediatric glaucoma clinic were performed over 4 years as clinically indicated. Eyes were classified as "normal" (normal eyes and those with physiologic cupping but normal intraocular pressure [IOP]); "mild glaucoma" (increased IOP and a normal optic nerve appearance); or "advanced glaucoma" (severe cupping or progressive glaucoma). Intraclass correlation coefficients were used to evaluate the reproducibility of measurements on the same day and over time. RESULTS: In the first setting, 8 normal eyes were included. Axial length increased 0.11 ± 0.04 mm/year over an average of 3.3 years (P = 0.03); there was no statistically significant change in RNFL thickness (P = 0.30). In our second setting, 27 normal eyes and 37 eyes with glaucoma were included. Intraclass correlation coefficients across the 3 visits for total macular volume were 0.80-0.91 and for average RNFL were 0.73-0.95. CONCLUSIONS: Global OCT measurements in children were reproducible over years and were not affected by normal increase in axial length. OCT shows promise as an objective tool for longitudinal assessment of children.

Differentiating glaucomatous from non-glaucomatous optic nerve cupping by optical coherence tomography.

BACKGROUND: In clinical practice, the differentiation of glaucomatous from non-glaucomatous cupping can be difficult, even for experienced observers. The purpose of this study was to evaluate the role of optical coherence tomography (OCT) in differentiating glaucomatous from non-glaucomatous optic nerve cupping in a cross-sectional pilot study. METHODS: Eleven consecutive patients presenting to the Duke Eye Center from September 2007 to July 2008 with non-glaucomatous optic nerve cupping and 12 patients with glaucomatous optic nerve cupping were identified. All patients underwent Stratus® OCT imaging: fast macular map, fast retinal nerve fiber layer (RNFL) 3.4 thickness, and fast optic disc protocols. Automated visual field perimetry was performed on the date of OCT scan in non-glaucomatous cupping patients, and from 0-9 months of scan date in glaucoma patients. Eyes were matched by optic nerve cup-to-disc area ratio; average and mean deviation were calculated for each variable. RESULTS: For a similar average RNFL, patients with non-glaucomatous optic nerve cupping had lower nasal and temporal RNFL thickness, as well as lower macular thickness and volume compared to patients with glaucomatous optic nerve cupping. CONCLUSION: OCT appears to be a useful technology in differentiating glaucomatous from non-glaucomatous optic nerve cupping. The pattern of RNFL loss appears more diffuse in non-glaucomatous optic nerve cupping compared to glaucomatous optic nerve cupping. Future studies with larger sample size and specific neuro-ophthalmic causes of optic nerve cupping may further elucidate the role of OCT in this clinical setting.

Inferior oblique overaction/contracture following retrobulbar anesthesia for cataract extraction with a positive Bielschowsky Head Tilt test to the contralateral shoulder. A report of one case.

BACKGROUND AND PURPOSE: This is a report of a patient with diplopia, hypertropia, extorsion and a positive Bielschowsky Head Tilt Test following retrobulbar anesthesia, due to an inferior oblique muscle overaction-contracture. CASE REPORT: Oculomotor and sensorimotor examinations and ocular motility recordings and Bielschowsky Head Tilt Test demonstrated overaction characteristics overpowering the contracture characteristics of the involved inferior oblique muscle. RESULTS: Weakening of the overacting inferior oblique eliminated the vertical and torsional deviations and the forced head tilt difference upon tilting the head to either shoulder. It also normalized ocular motility and resulted in a symptom-free patient. CONCLUSION: Oblique muscle dysfunction with vertical and torsional deviations and a positive Bielschowsky Head Tilt Test can result from retrobulbar anesthesia.