Optical coherence tomography of the macular ganglion cell layer in children with neurofibromatosis type 1 is a useful tool in the assessment for optic pathway gliomas.

BACKGROUND: Optic pathway glioma (OPG) is a feared complication to neurofibromatosis type 1 (NF1) since it can cause visual impairment in young children. The main goal of screening is to detect symptomatic OPGs that require treatment. Optical coherence tomography (OCT) has been suggested as a tool for detection of neuro-retinal damage. AIMS: To investigate whether the ganglion cell layer assessed by OCT is a reliable measure to identify and detect relapses of symptomatic OPGs in children with NF1. METHODS: Children (3-6 years) with NF1, with and without known OPG and children with sporadic OPG (S-OPG) resident in the Stockholm area, were invited and followed in a prospective study during a three-year period. Brain magnetic resonance tomography (MRI) had been performed in children with symptoms of OPG. Outcome measures were VA in logMAR, visual field index (VFI), average thicknesses of the ganglion cell-inner plexiform layer (GC-IPL), and peripapillary retinal nerve fiber layer (pRNFL). RESULTS: There were 25 children with MRI-verified OPG and 52 with NF1 without symptomatic OPG. Eyes from NF1 patients without symptoms of OPG showed significantly better results in all four analyzed parameters compared to eyes with NF1-associated OPG. Mean GC-IPL measurements seemed stable and reliable, significantly correlated to pRNFL (correlation coefficient (r) = 0.662, confidence interval (CI) = .507 to .773 p<0.001), VA (r = -0.661, CI = -7.45 to -.551, p<0.001) and VFI (r = 0.644, CI = .452 to .774, p<0.001). GC-IPL measurements were easy to obtain and acquired at considerably younger age than pRNFL (5.6±1.5 vs 6.8±1.3; p<0.001). CONCLUSIONS: The mean GC-IPL thickness could distinguish well between eyes with OPG and eyes without symptomatic OPG in children with NF1. As thinning of GC-IPL assessed with OCT could indicate underlying OPG, it should be included in the screening protocol of children with questionable VA measurements and in particular in children with NF1.

Glucocorticoids in the treatment of non-infectious superior ophthalmic vein thrombosis - Three cases and a review of the literature.

Purpose: Superior ophthalmic vein thrombosis (SOVT) is a rare clinical entity, which can have a septic and an aseptic cause. Aseptic SOVT is typically treated with anticoagulation. Glucocorticoids are reserved for cases with concurrent orbital inflammation.We present three cases of SOVT due to carotid cavernous fistula not responding to standard treatment, subsequently successfully treated with glucocorticoids. Observations: Three patients with various degrees of proptosis, ophthalmoplegia, orbital stasis and reduced vision are presented. One patient was confirmed to have isolated SOVT, while the other two had associated cavernous sinus thrombosis. All patients had underlying carotid-cavernous fistula without signs of infection. All patients were initially treated with parenteral anticoagulation. Two patients were treated with intraocular pressure-reducing medication. One of whom underwent canthotomy-cantholysis. Two patients experienced a gradual worsening of symptoms during treatment with anticoagulation, while one patient improved before deteriorating. All patients received additional treatment with glucocorticoids consisting of a three-day treatment with intravenous methylprednisolone 500 mg, followed by oral glucocorticoids resulting in total regression of symptoms. Two patients regained 20/20 vision, with some vision field defects, while the third patient regained 20/25 vision. Conclusion and importance: The addition of glucocorticoids in the treatment of aseptic SOVT can lead to improvement of symptoms and a potentially better prognosis. However, the risk of complications of glucocorticoid treatment must be carefully considered on a case-by-case basis.

Optical Coherence Tomography Identifies Visual Pathway Involvement Earlier than Visual Function Tests in Children with MRI-Verified Optic Pathway Gliomas.

This study investigates whether optical coherence tomography (OCT) could add useful information in the examination of children with optic pathway glioma (OPG) at high risk of developing vision loss. For this purpose, the relationship between ganglion cell-inner plexiform layer (GC-IPL) thickness and visual function, evaluated with tests of visual acuity (VA) and visual field (VF), as well as tumor site according to magnetic resonance imaging (MRI), were examined in a geographically defined group of children with OPG. METHODS: Children aged <18 years with OPG underwent ophthalmic examination including VA, VF (Zeiss HFA perimetry) and OCT imaging (Zeiss Cirrus HD-OCT). RESULTS: Out of 51 patients included, 45 provided 77 eyes with MRI-verified OPG, and 19 patients provided 25 eyes without OPG. Significant correlations were found between GC-IPL, VF and VA (p < 0.001). The GC-IPL pattern loss corresponded in 95% to VF defects and in 92% to MRI findings. CONCLUSIONS: Our study indicates that GC-IPL measures could serve as an early marker of vision-threatening changes related to OPG and as a valuable link between MRI and visual function tests. Thinning of GC-IPL and differences in topography between eyes are strong indicators of and predictive of vision loss related to OPG.

Profile of macular ganglion cell-inner plexiform layer thickness in healthy 6.5 year- old Swedish children.

BACKGROUND: The purpose was to study the macular ganglion cell- inner plexiform layer (GC-IPL) thickness in healthy 6.5 year- old Swedish children using Optical Coherence Tomography (OCT) and to study topography symmetry within eyes and between eye pairs. METHODS: A total of 181 eyes of 92 healthy children (39 girls, 53 boys) aged 6.5 and serving as a term-born control group in the Extremely Preterm Infants in Sweden Study (EXPRESS), were examined with Cirrus HD-OCT. Main outcome measures were average and minimum values of GC-IPL thickness of the device's predefined macular sectors. Single sectors, combined sectors defined as superior and inferior hemispheres and temporal and nasal sectors were evaluated. Intra-individual GC-IPL thickness between eye pairs was analyzed. Visual acuity, refraction and general cognition were assessed and correlated to GC-IPL outcome. RESULTS: Eighty-five children completed the OCT examination and 155 out of 181 scans (86%) were analyzed. The mean average GC-IPL thickness was 85.9 µm (± 5.3; 5th and 95th percentiles were 76.0 and 94.6 µm). The mean minimum GC-IPL thickness was 83.6 µm (± 4.9; 5th and 95th percentiles were 75.4 and 92.3 µm). The difference in thickness between nasal and temporal sectors and between superior and inferior hemisphere sectors were less than 2 µm. The difference between average GC-IPL thickness and minimum GC-IPL thickness was 2.3 µm (± 1.9; 5th and 95th percentiles were 0.0 and 6.0 µm). The difference between the thickest and thinnest sector within eye was 6.4 µm (± 2.2; 5th and 95th percentiles were 3.0 and 10.0 µm). There was a moderate correlation in the difference between the nasal combined and the temporal combined sectors within eye pairs (p < 0.0001, Spearman's ρ 0.58). The average GC-IPL thickness was weakly positively correlated with SE (spherical equivalent; combined sphere and ½ cylinder) (p = 0.031, Spearman's ρ 0.23). CONCLUSIONS: This study provides normative GC-IPL thickness values for healthy 6.5 year- old Swedish children. The GC-IPL thickness variations within eyes and within eye pairs are generally small. It could therefore be assumed that larger variations are sensitive markers of focal GC-IPL thinning due to damage to the primary visual pathways in children.