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.

Magnetic Resonance Imaging Abnormalities of the Optic Nerve Sheath and Intracranial Internal Carotid Artery in Giant Cell Arteritis.

BACKGROUND: Giant cell arteritis (GCA) is an important diagnostic consideration in elderly patients with vision changes. Superficial temporal artery biopsy (TAB) has long been considered the gold standard diagnostic approach for GCA, but MRI has gained interest as an alternative diagnostic modality. Although most of the literature has focused on imaging abnormalities of branches of the external carotid artery, there have been a few reports of GCA-related inflammatory involvement of the orbit and internal carotid arteries (ICAs) on MRI. METHODS: This was a retrospective cross-sectional study of patients undergoing TAB at a single tertiary referral center over a 5-year period. Patients who had undergone contrast-enhanced MRI of the brain and orbits within 1 month of biopsy were included. Fifty-four TAB-positive and 78 TAB-negative patients were reviewed, with the MRI studies of 7 TAB-positive and 6 TAB-negative patients deemed adequate for interpretation. MRI studies were reviewed by 2 masked neuroradiologists, and the findings were correlated with biopsy results and clinical findings. RESULTS: Intracranial ICA vessel wall enhancement was identified in 6 of 7 TAB-positive patients (sensitivity 86%), compared with 2 of 6 TAB-negative patients (specificity 67%). Optic nerve sheath enhancement was identified in 5 of 7 TAB-positive patients (sensitivity 71%) and in 2 of 6 TAB-negative patients (specificity 67%), bilateral in all such cases. The combination of both abnormal imaging findings was observed in 5 of 7 TAB-positive patients (sensitivity 71%) and in none of the 6 TAB-negative patients (specificity 100%). CONCLUSIONS: Intracranial ICA and optic nerve sheath enhancement were observed in a majority of patients with TAB-proven GCA, and the combination of these findings was highly specific for GCA. Identification of these abnormalities on MRI should raise concern for GCA and prompt a thorough review of systems, laboratory testing, and consideration of TAB in patients with ocular complaints potentially consistent with ischemia.

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.

Optical Coherence Tomography Normative Peripapillary Retinal Nerve Fiber Layer and Macular Data in Children 0-5 Years of Age.

PURPOSE: To determine reference values for the peripapillary retinal nerve fiber layer (pRNFL) and macula in children 0-5 years of age. DESIGN: Prospective cross-sectional study. METHODS: This study was set in a single large academic pediatric ophthalmology practice. Healthy, full-term children 0 to <6 years of age presenting for surgery under general anesthesia were prospectively recruited for participation. Excluded were children with systemic neurologic disease, optic nerve or retinal disease (even if unilateral) or any bilateral ocular disease process, and eyes with amblyopia, ocular disease, or spherical equivalent refractive error outside of -3.00 to +8.00 diopters. Following general anesthesia, OCT scans of the optic nerve and retina were acquired using an HRA+OCT Spectralis with Flex module (Heidelberg Engineering). Automated segmentation of the pRNFL and retinal layers was followed by manual correction. RESULTS: Data were obtained from normal eyes of 57 participants (mean age 2.28 ± 1.50 years). Mean global pRNFL thickness was 107.6 ± 10.3 µm. Mean global pRNFL thickness was not dependent on age but showed a negative relationship with axial length (P = .01). The mean total macular volume was 8.56 ± 0.259 mm3 (n = 38). No relationship was found between total macular volume and age. Ganglion cell layer, ganglion cell complex, and inner nuclear layer volumes showed an inverse relationship with age while the photoreceptor layers showed a logarithmic increase with age. CONCLUSIONS: Global pRNFL thickness measurements remain stable over time. Macular volume and thickness values of segmented retinal layers reflect the development of the macula with age.

Hyperreflective Vitreous Opacities on Optical Coherence Tomography in a Patient With Bilateral Retinoblastoma.

An investigational, portable spectral-domain optical coherence tomography (SD-OCT) unit revealed small hyperreflective opacities in both eyes of a patient with bilateral retinoblastoma. There was no evidence of vitreous seeding on ophthalmoscopy of either eye. Although the opacities may initially raise concern for potential vitreous seeding, this report suggests alternate potential etiologies for such opacities, which were also seen on imaging of a cohort of normal eyes in pediatric patients. [Ophthalmic Surg Lasers Imaging Retina. 2019;50:50-52.].

Emerging Applications of Optical Coherence Tomography in Pediatric Optic Neuropathies.

Limited cooperation and attention span often lead to poorly reliable assessments of visual acuity and visual fields in children, making diagnosis and monitoring of pediatric optic neuropathies challenging. As a noninvasive imaging modality, optical coherence tomography (OCT) could offer particular utility in this patient population. OCT provides high-resolution characterization of the optic nerve head, peripapillary retinal nerve fiber layer, and cellular layers of the macula, all of which can be used to assess the severity of optic nerve disease qualitatively and quantitatively. Application of OCT to pediatric patients has been limited by technical factors and lack of pediatric normative databases, but with recent technological improvements and rapidly expanding research efforts OCT is poised to revolutionize the management of optic neuropathies in children. We review current and emerging applications of OCT to important pediatric optic neuropathies such as glaucoma, papilledema, optic neuritis, optic pathway gliomas, and congenital optic disc anomalies.

Endodermal Cyst of the Third Nerve in a Child.

Endodermal cysts, also known as neurogenic, neuroenteric, foregut, bronchogenic, respiratory, epithelial, teratomatous, or gastrocytoma cysts, can be found in the central nervous system, predominantly in the subarachnoid space of the cervical and thoracic spinal cord. We describe a child with an endodermal cyst of the third nerve and highlight neuroimaging findings, pathological correlation, clinical course, and patient management.

Temporary Lumbar Drain as Treatment for Pediatric Fulminant Idiopathic Intracranial Hypertension.

Fulminant idiopathic intracranial hypertension (FIIH) is a subtype of idiopathic intracranial hypertension (IIH) characterized by rapid, severe, progressive vision loss. Surgical intervention is often performed either as a cerebrospinal fluid (CSF) shunt procedure or an optic nerve sheath fenestration or, at times, both. These surgical procedures carry a significant risk of morbidity and failure. We present 2 patients in whom a temporary lumbar drain was successfully used in the management of medically undertreated pediatric FIIH, and circumvented the need for surgical intervention.

Orbito-Masticatory Syndrome.

We describe 2 unique cases of visual symptoms occurring during mastication in patients with lateral orbital wall defects. A 57-year-old man reported intermittent double vision and oscillopsia after a right fronto-temporal-orbito-zygomatic craniotomy with osteotomy of the lesser wing of the sphenoid for a complex invasive pituitary adenoma. Proptosis of the right globe was present only during mastication. Computed tomography (CT) revealed a bony defect in the right lateral orbital wall. A 48-year-old man presented with transient diplopia and scotoma in the right eye elicited by chewing. CT and magnetic resonance imaging demonstrated a bilobed lesion connecting the temporal fossa to the orbit through a defect in the right lateral orbital wall. The regional neuroanatomy and pathophysiology as pertaining to these cases are discussed.

Microcystic Macular Changes in Primary Open-angle Glaucoma.

PURPOSE: To describe microcystic macular changes in patients with moderate to advanced primary open-angle glaucoma. PATIENTS AND METHODS: Eleven eyes of 6 unrelated patients were retrospectively identified based on a disproportionately preserved macular thickness on optical coherence tomography (OCT) despite severe peripapillary retinal nerve fiber layer thinning. Patient demographic, history, and examination findings were reviewed. RESULTS: All identified patients were African American, relatively young (mean age, 43.8 y) and 5 of the 6 patients were males. Examination of individual macular OCT sections through areas of disproportionately preserved macular thickness invariably demonstrated numerous small cystic cavities within the inner nuclear layer. These microcystic changes were seen in areas of the macula that corresponded with areas of glaucoma-related ganglion cell loss, therefore mimicking the normal appearance of retinal thickness in the macular region. No other retinal pathologies were identified on the macular OCT to account for these changes. CONCLUSIONS: This study describes microcystic macular changes in mostly young, African American males with moderate to advanced primary open-angle glaucoma. Vitreous adherence to the internal limiting membrane preventing retinal collapse is a proposed mechanism. The disproportionately preserved macular volume may confound the diagnosis of glaucoma in these patients.

Retinal Changes in Pediatric Glaucoma and Nonglaucomatous Optic Atrophy.

PURPOSE: To describe the incidence and significance of retinal changes in pediatric eyes with glaucoma and nonglaucomatous optic atrophy by optical coherence tomography (OCT). DESIGN: Retrospective observational case series. METHODS: OCT scans performed on children with either glaucoma or nonglaucomatous optic atrophy were retrospectively reviewed. Excluded were poor-quality scans and diagnoses of ocular hypertension or glaucoma suspects. RESULTS: Included were 227 eyes (227 children), 151 with glaucoma and 76 with nonglaucomatous optic atrophy. Outer retinal changes were present in 20 of 151 glaucoma eyes (13.2%) (12 prior intraocular surgery, 5 uveitis, 3 primary retinopathy) and 6 of 76 nonglaucomatous optic atrophy eyes (7.9%) (1 retinal vasculitis, 3 papilledema, 2 infiltrative optic neuropathy). Excluding eyes with outer retinal changes, isolated inner nuclear layer (INL) cysts were found in 6 of 131 eyes with glaucoma (4.6%) (3 uveitis, 1 prior hydrocephalus, 1 uveitis and pars plana vitrectomy, and 1 juvenile open-angle glaucoma) and 21 of 70 eyes with nonglaucomatous optic atrophy (30%) (5 optic neuritis, 11 anterior visual pathway tumors, 2 papilledema, 3 other) (P < .0001). Compared to eyes without INL cysts, those with INL cysts had thinner average retinal nerve fiber layer (RNFL) (78.2 ± 1.8 µm vs 52.0 ± 4.8 µm, P < .0001) and worse vision (logMAR = 0.33 ± 0.04 vs 0.65 ± 0.09, P < .002). CONCLUSIONS: INL cysts are more frequent in pediatric nonglaucomatous optic atrophy than glaucoma; they are associated with worse vision and thinner RNFL. Outer retinal changes were exclusively seen in pathology that directly affected the retina. In children, INL cysts and/or outer retinal changes without prior history of intraocular surgery or uveitis should prompt further evaluation.

Anatomic and visual function outcomes in paediatric idiopathic intracranial hypertension.

BACKGROUND: There is a paucity of literature describing risk factors for vision loss in paediatric idiopathic intracranial hypertension (IIH). We investigate the final visual function, spectral domain optical coherence tomography (SD-OCT) and enhanced depth imaging (EDI)-OCT findings in children with papilledema caused by IIH. METHODS: Medical records of 31 patients with paediatric IIH (age ≤17 years) were retrospectively reviewed. Optic disc photographs on presentation and automated perimetry, SD-OCT and EDI-OCT imaging on final follow-up visit were statistically analysed to identify patient characteristics and anatomic findings associated with irreversible vision loss. RESULTS: Permanent visual acuity or visual field loss developed in 19% of study eyes. Papilledema of modified Frisén grade ≥3 on presentation was highly predictive of permanent vision loss (p<0.001), while associations between pubertal status and visual function outcome failed to reach statistical significance. SD-OCT revealed optic atrophy in 13% and photoreceptor loss in 19% of eyes, with both findings highly associated with vision loss (p<0.0001). Optic disc drusen was noted in 48% of study eyes by EDI-OCT but was not found to be predictive of visual outcome. CONCLUSIONS: Clinical observation of high papilledema grade on presentation is predictive of poor visual outcomes. Vision loss is associated not only with optic atrophy but also with photoreceptor damage. Interestingly, a high proportion of study eyes had optic disc drusen, which was not associated with vision loss, but can be a diagnostic challenge in distinguishing true papilledema from pseudopapilledema.

Low cerebrospinal fluid protein in prepubertal children with idiopathic intracranial hypertension.

PURPOSE: To evaluate a clinical observation that prepubertal children with idiopathic intracranial hypertension (IIH) have low cerebrospinal fluid (CSF) protein levels compared to healthy children and pubertal patients with IIH. METHODS: The medical records of prepubertal and pubertal IIH patients and controls seen in the pediatric neuro-ophthalmology clinic at Duke between 2003 and 2013 were retrospectively reviewed. The control group consisted of children who had normal intracranial pressure on lumbar puncture performed to evaluate for headaches or anomalous-looking optic nerves. The records were analyzed with attention to demographic characteristics, clinical presentation, course, and lumbar puncture results. RESULTS: A total of 23 prepubertal children with IIH (age range, 0.75-13 years), 16 pubertal patients with IIH (age range, 13-21 years), and 12 controls (age range 3-14 years) were included. CSF analysis revealed that prepubertal children with IIH had significantly lower CSF protein levels (17.3 ± 5.7 mg/dL) compared to pubertal subjects with IIH (23.4 ± 8.4 mg/dL; P = 0.019) or healthy controls (23.5 ± 6.4 mg/dL; P = 0.011). Furthermore, 9 of 23 prepubertal IIH patients (39%) had abnormally low CSF protein level (<15 mg/dL), compared to zero pubertal IIH patients (P = 0.005) and zero controls (P = 0.015). Acetazolamide increased CSF protein level in 100% of patients who underwent repeat lumbar puncture after starting the medication (average increase, 10.3 ± 6.6 mg/dL). CONCLUSIONS: Low CSF protein level may have diagnostic utility as a biomarker for prepubertal IIH. Furthermore, this finding suggests that some cases of prepubertal IIH may be caused by CSF overproduction rather than decreased CSF resorption.

Diagnostic Algorithm for Patients With Suspected Giant Cell Arteritis.

BACKGROUND: To identify clinical and laboratory factors contributing to the diagnosis of giant cell arteritis (GCA) and develop a diagnostic algorithm for the evaluation of GCA. METHODS: Retrospective review of 213 consecutive cases of temporal artery biopsy (TAB) seen at a single academic center over a 10-year period (2000-2009). Pathologic specimens were re-reviewed and agreement between the original and second readings was assessed. A composite clinical suspicion score was created by adding 1 point for each of the following criteria: anterior extracranial circulation ischemia, new onset headache, abnormal laboratory results (erythrocyte sedimentation rate, C-reactive protein (CRP), or platelet count), jaw claudication, abnormal or tender superficial temporal artery, constitutional symptoms, and polymyalgia rheumatica; one point was subtracted if a comorbid condition could explain a criterion. RESULTS: Of the 204 TABs analyzed, pathologic findings were confirmatory in 49 (24.0%) and suggestive in 12 (5.9%). TAB-positive patients were more likely to be older (age 75.2 ± 7.8 vs 69.7 ± 11.0 years, P = 0.0002), complain of jaw claudication (relative-risk = 3.26, P = 0.0014), and have thrombocytosis (relative-risk = 3.3, P = 0.0072) and elevated CRP (relative-risk = 1.8, P = 0.037). None of the patients with a clinical score less than 2 had a positive TAB. Diabetes mellitus and kidney disease were often the explanation for the symptoms and abnormal clinical finding(s) that led to a negative TAB. CONCLUSIONS: We propose a clinical algorithm that is highly predictive for a positive TAB and can be valuable in the evaluation process of suspected cases of GCA.