Hironobu Tampo

Applying artificial intelligence to disease staging: Deep learning for improved staging of diabetic retinopathy

Purpose Disease staging involves the assessment of disease severity or progression and is used for treatment selection. In diabetic retinopathy, disease staging using a wide area is more desirable than that using a limited area. We investigated if deep learning artificial intelligence (AI) could be used to grade diabetic retinopathy and determine treatment and prognosis. Methods The retrospective study analyzed 9,939 posterior pole photographs of 2,740 patients with diabetes. Nonmydriatic 45° field color fundus photographs were taken of four fields in each eye annually at Jichi Medical University between May 2011 and June 2015. A modified fully randomly initialized GoogLeNet deep learning neural network was trained on 95% of the photographs using manual modified Davis grading of three additional adjacent photographs. We graded 4,709 of the 9,939 posterior pole fundus photographs using real prognoses. In addition, 95% of the photographs were learned by the modified GoogLeNet. Main outcome measures were prevalence and bias-adjusted Fleiss’ kappa (PABAK) of AI staging of the remaining 5% of the photographs. Results The PABAK to modified Davis grading was 0.64 (accuracy, 81%; correct answer in 402 of 496 photographs). The PABAK to real prognosis grading was 0.37 (accuracy, 96%). Conclusions We propose a novel AI disease-staging system for grading diabetic retinopathy that involves a retinal area not typically visualized on fundoscopy and another AI that directly suggests treatments and determines prognoses.

Correlations between choroidal abnormalities, Lisch nodules, and age in patients with neurofibromatosis type 1.

To evaluate correlations between choroidal abnormalities, Lisch nodules, and age in patients with neurofibromatosis type 1 (NF1), we examined ten cases with NF1 using near-infrared reflectance imaging. Patients ranged in age from 4 to 39 years. The angle used for near-infrared reflectance imaging was 55°. We counted the total number of choroidal abnormalities in an area within a 55° angle centered on the fovea and the total number of Lisch nodules on the iris by slit-lamp examination. No positive correlation was found between the number of Lisch nodules and patient age (Spearman’s rank correlation coefficient ρ=0.117, P=0.7414). Choroidal abnormalities tended to increase with age (ρ=0.6150), but this difference was not statistically significant (P=0.0650). A positive correlation was found between the number of choroidal abnormalities and Lisch nodules (ρ=0.783, P=0.0267). In conclusion, choroidal abnormalities tend to increase with patient age and are correlated with the number of Lisch nodules.

Optical coherence tomography and fundus autofluorescence imaging study of chorioretinal atrophy involving the macula in Alagille syndrome

We report the first case in the literature of chorioretinal atrophy involving the macula in an 11-year-old girl with Alagille syndrome, as examined by optical coherence tomography, and fundus autofluorescence imaging. Funduscopy revealed diffuse choroidal hypopigmentation with increased visibility of the choroidal vessels and symmetric, well circumscribed macular discoloration. Anomalous oblique configuration of the optic disc and peripapillary tortuous vessels were also detected. Optical coherence tomography demonstrated decreased retinal thickness, especially the outer retinal layer, and the photoreceptor inner segment/outer segment junctions were irregular and discontinued, corresponding to macular discoloration. Fundus autofluorescence imaging clearly defined hypofluorescent areas in the peripapillary regions that extended along the macula and had a sleep mask appearance. We suggest that transient hypovitaminosis due to Alagille syndrome early in life might contribute to the retinal degeneration seen in this case.

Ocular findings in two siblings with Joubert syndrome

We describe two brothers with Joubert syndrome (JS). JS diagnosis was made on the basis of neurological findings and the presence of the characteristic “molar tooth sign”, which was subsequently confirmed by magnetic resonance imaging. Both brothers demonstrated ptosis, hypotropia, exotropia, and horizontal pendular nystagmus. The younger brother had mild chorioretinal discoloration at the peripapillary region in both eyes, and a small coloboma at the inferior region of his right optic disc. The elder brother had coloboma in his right eye and a colobomatous optic disc in his left eye. Optical coherence tomography showed that the foveal architecture was preserved in both patients. We discuss the ocular findings, including those from optical coherence tomography, in JS, which has recently been recognized as ciliopathy.

Retinal Microvascular Abnormalities in Neurofibromatosis Type 1 Associated with Congenital Retinal Macrovessels

Here, we report a case of retinal microvascular abnormalities in a patient with neurofibromatosis type 1 (NF1) associated with congenital retinal macrovessels. An abnormal retinal macrovessel, crossing the macula horizontally, was detected in the right eye. Additionally, retinal microvascular abnormalities were detected. Eight years after the initial visit, the retinal microvascular abnormalities were noted to have changed substantially. We speculate that retinal microvascular abnormalities in NF1 may change dynamically over the years.

Changes in Optical Coherence Tomography Findings in Acute Zonal Occult Outer Retinopathy

Purpose: To report a case of acute zonal occult outer retinopathy (AZOOR) in a 39-year-old woman. Methods: Images were obtained with fundus photography and optical coherence tomography (OCT). A multifocal electroretinogram (mfERG) was also obtained to evaluate retinal function. Results: The patients right visual acuity was 0.8. Fundus photographs showed no specific abnormal findings. OCT showed attenuation of the photoreceptor inner segment/outer segment (IS/OS) line and the cone outer segment tip (COST) line in the right eye. The mfERG showed corresponding amplitude reductions. One month after the initial visit, her right visual acuity improved to 1.2. There was partial recovery of the IS/OS line; however, the COST line was still absent, and there was no apparent improvement in the mfERG responses. Conclusions: Our findings showed a discrepancy between the microstructural findings and visual function during recovery of AZOOR.

An Optical Coherence Tomography and Fundus Autofluorescence Imaging Study of Peripapillary Acute Zonal Occult Outer Retinopathy

Purpose: To evaluate peripapillary acute zonal occult outer retinopathy in a 67-year-old man. Methods: Images were obtained using fundus photography, fluorescein angiography (FA) and indocyanine green angiography (ICGA), fundus autofluorescence (FAF) imaging, and optical coherence tomography (OCT). Visual field testing and multifocal electroretinogram (mfERG) were also performed to evaluate retinal function. Results: Fundus photographs showed subtle pigmentary changes in the peripapillary region, while FAF imaging showed clearly defined hypofluorescent areas in the peripapillary region. Intense hyperfluorescent lesions were also seen underneath hypofluorescent areas. A transmission defect with a granular hyperfluorescence was visible on FA, and ICGA showed hypofluorescence within the lesion. The outer border of the peripapillary zone appeared to block the underlying choroidal fluorescence. The photoreceptor inner segment/outer segment line was absent on OCT images from this area in both eyes, and hyperreflective punctate drusen-like materials were present at the outer border of the peripapillary zone. Visual field testing by Goldmann perimetry showed blind-spot enlargement, and mfERG showed corresponding amplitude reductions. Conclusions: We speculate that the intense hyperautofluorescent material at the outer border of the peripapillary zone might be accumulated drusenoid lipofuscin.

Acute Macular Neuroretinopathy in a 15-Year-Old Boy: Optical Coherence Tomography and Visual Acuity Findings

Purpose: To report a case of acute macular neuroretinopathy (AMN) in a 15-year-old boy. Methods: Images were obtained with fundus photography and optical coherence tomography (OCT). Results: The patient complained of blurred vision and a small central scotoma in the left eye. Left visual acuity was 0.3. Fundus photographs revealed a small dark area in the fovea of the left eye. OCT showed attenuation of the photoreceptor inner segment (IS)/outer segment (OS) line and the OS/retinal pigment epithelium (RPE) line in the left eye. One week after the initial visit, left visual acuity had improved to 0.6, and there was partial recovery of the IS/OS line with focal thinning of the OS/RPE line. One month later, left visual acuity had improved to 0.8, and OCT showed complete restoration of the IS/OS line and the OS/RPE line. Conclusions: To our knowledge, this is the youngest patient reported with a diagnosis of AMN. Changes in microstructural findings and visual acuity were analogous during recovery of AMN in our patient.

Optical Coherence Tomography Imaging of Choroidal Abnormalities in Neurofibromatosis Type 1

We report a case of neurofibromatosis type 1 (NF1) examined by infrared fundus autofluorescence (IR-FAF) and optical coherence tomography (OCT) to characterize the associated choroidal abnormalities. The conventional ophthalmoscopic findings were unremarkable. However, IR-FAF revealed multiple bright patchy lesions in the choroid of the posterior pole, in both eyes. OCT demonstrated irregular hyperreflectivity at the sites of these lesions. Patients with NF1 may have typical choroidal lesions that are visible on IR-FAF, which can be confirmed through OCT.

Double ring sign at the macula in a patient with Valsalva retinopathy.

A 52-year-old woman with hypertension presented with a sudden, painless and profound loss of vision in the left eye while doing farm work. Visual acuity was 0.08 only in the affected eye. Ophthalmoscopic examination revealed a large dome-shaped sub-internal limiting membrane (sub-ILM) haemorrhage and a subhyaloid haemorrhage located anterior to the sub-ILM haemorrhage. The latter also showed a niveau formation. This finding …