(1) Underlying Diabetic Retinopathy (DR) is the primary cause of poor vision in young adults. There are automatic image reading systems that can aid screening for DR. (2) Methods: Using our automatic reading system we have counted the number of microaneurysms and hemorrhages in the four quadrants of the ETDRS grid and evaluated the differences between them according to the type of DR. The study was carried out using data from two different databases, MESSIDOR and MIRADATASET. (3) Results: The majority of microaneurysms and hemorrhages are found in the temporal and inferior quadrants of the ETDRS grid. Differences are significant with respect to the other two quadrants at p < 0.001. Differences between the type of DR show that severe-DR has a greater number of microaneurysms and hemorrhages in the temporal and inferior quadrant, being significant at p < 0.001. (4) Conclusions: The count of microaneurysms and hemorrhages is higher in the temporal and inferior quadrants in all types of DR, and those differences are more important in the case of severe-DR.

Severe, too many to count retinal hemorrhages (RH) in infants have been associated with abusive head trauma, but can occur in short falls. An 8-month-old male fell backward from a height of 26 cm, landing on his buttocks then hitting the back of his head on a vinyl floor. The fall was videotaped. Acute subdural hemorrhages were found along with extensive, too many to count intra-RH in both eyes. Falls from small heights on to the occiput can lead to extensive RH of the type often associated with abusive head trauma.

Purtscher\'s retinopathy is a rare angiopathy reported in patients with a history of severe trauma and other systemic diseases. The diagnosis is made on clinical grounds, and the severity varies. A 41-year-old gentleman with underlying poorly controlled diabetes mellitus and dyslipidemia was referred to the ophthalmology department for diabetic retinopathy screening. He denied visual complaints. Ocular examination revealed a negative relative afferent pupillary defect with a visual acuity of 6/6 bilaterally. The anterior segment examination was unremarkable. Both eye (oculus uterque, OU) fundus revealed a pink disc with a cup-to-disc ratio of 0.4 and peripapillary flame-shaped hemorrhages. Right eye (oculus dexter, OD) also showed multiple cotton wool spots along the superotemporal arcade involving zones 1 and 2 of the retina, while left eye (oculus sinister, OS) showed a single cotton wool spot along the superotemporal arcade at zone 1 of the retina. Otherwise, there were no visible retinal emboli, dot hemorrhages, or hard exudates, and the macula was normal. The retinal features were not characteristic of diabetic retinopathy. It mimicked hypertensive retinopathy, but the patient was normotensive. The absence of inner retinal thickening and hyperreflectivity on optical coherence tomography of the macula ruled out retinal vein occlusion. This prompted us to elicit further history, and the patient disclosed a recent admission for myocardial infarction in which he received cardiopulmonary resuscitation with chest compressions for seven minutes. Hence, the diagnosis of OU Purtscher\'s retinopathy was made, and the patient was monitored closely in the clinic. Purtscher\'s retinopathy remains a diagnostic dilemma and should not be neglected in complex clinical contexts.

UNASSIGNED: This work reports a case of Evans syndrome first presenting with ophthalmic signs.
UNASSIGNED: A 27-year-old previously healthy man presented with headaches and bilateral blurred vision for 2 weeks. The visual acuity was 20/30-1 and 20/60-2 in the right eye and left eye, respectively. A fundus examination revealed Roth spots, diffuse multilayer retinal hemorrhages in the macula and periphery, and tortuous vessels in both eyes. Optical coherence tomography showed a disrupted foveal contour from intraretinal fluid and hemorrhage in both eyes. Fluorescein angiography demonstrated dilated and tortuous vessels with scattered blocking defects from hemorrhages.
UNASSIGNED: A workup revealed warm hemolytic anemia with severe thrombocytopenia consistent with the diagnosis of Evans syndrome.
UNASSIGNED: Evans syndrome is a rare blood dyscrasia that can first present as subacute vision loss and should be added to the differential of diffuse bilateral retinal hemorrhages spanning a multitude of retinal layers.

OBJECTIVE: Function and anatomy of the visual system were evaluated in children with abusive head trauma (AHT). The relationships between retinal hemorrhages at presentation were examined with outcome measures.
METHODS: Retrospective review of data in children with AHT for 1) visual acuity at last follow-up, 2) visual evoked potentials (VEP) after recovery, 3) diffusion metrics of white matter tracts and grey matter within the occipital lobe on diffusion tensor imaging (DTI), and 4) patterns of retinal hemorrhages at presentation. Visual acuity was converted into logarithm of minimum angle of resolution (logMAR) after correction for age. VEPs were also scored by objective signal-to-noise ratio (SNR).
RESULTS: Of 202 AHT victims reviewed, 45 met inclusion criteria. Median logMAR was reduced to 0.8 (approximately 20/125 Snellen equivalent), with 27% having no measurable vision. Thirty-two percent of subjects had no detectable VEP signal. VEPs were significantly reduced in subjects initially presenting with traumatic retinoschisis or hemorrhages involving the macula (p < 0.01). DTI tract volumes were decreased in AHT subjects compared to controls (p < 0.001). DTI metrics were most affected in AHT victims showing macular abnormalities on follow-up ocular examination. However, DTI metrics were not correlated with visual acuity or VEPS. There was large inter-subject variability within each grouping.
CONCLUSIONS: Mechanisms causing traumatic retinoschisis, or traumatic abnormalities of the macula, are associated with significant long-term visual pathway dysfunction. AHT associated abnormalities of the macula, and visual cortical pathways were more fully captured by VEPs than visual acuity or DTI metrics.

BACKGROUND: In the case of sudden unexpected death in infancy (SUDI), eye examination is systematic to detect retinal hemorrhages (RH) that are a crucial hallmark for abusive head trauma (AHT). The aim of this study is to assess the ability of non-invasive post-mortem fundus photographs (PMFP) to detect RH in case of SUDI.
METHODS: Bicentric retrospective analysis of consecutive cases of SUDI under 2 years of age were managed by two French SUDI referral centers with PMFP by RetCam (Clarity Medical Systems USA). PMFP were reviewed randomly, twice, by three independent ophthalmologists blinded for clinical data.
RESULTS: Thirty cases (60 eyes) were included. Median age was 3.5 months (interquartile [1.6; 6.0]). No child died of AHT. Image quality was sufficient to assert presence or absence of RH in 50 eyes (83%). Sufficient quality rate was significantly higher when the post-mortem interval was inferior to 18 h (91%, 42/46) as opposed to over 18 h (57%, 8/14, p=0.0096). RH were found in six eyes (10%), four children (13%), with excellent inter and intra-raters\' concordance (Cohen\'s Kappa from 0.81 [0.56-1.00] to 1.00 [1.00-1.00]).
CONCLUSIONS: PMFP can detect RH in case of SUDI and is a relevant systematic screening test to be carried out as soon as the deceased child arrives in the hospital. It can decrease the need of eye removal for pathological examination, but further studies are needed to define the best decision algorithm.

Predominantly visual loss, is very rare in Wernicke\'s encephalopathy. A 22 year old lady, in her 28th week of gestation, presented with a confused mental state, bilateral papilloedema with retinal hemorrhages, ophthalmoparesis, and cerebellar signs. Her MRI brain was suggestive of Wernicke\'s encephalopathy and she recovered with intravenous thiamine.

UNASSIGNED: To report family members with familial retinal arteriolar tortuosity (FRAT) identified after sudden visual loss.
UNASSIGNED: A 15-year-old boy had sudden visual loss in his left eye while playing on a horizontal bar. He was referred to Nagoya City University Hospital from an eye clinic. The ophthalmologic examination showed retinal hemorrhage bilaterally. His best-corrected visual acuity (BCVA) was 20/17 in the right eye and 20/67 in the left eye. Bilateral retinal arteriolar tortuosity as well as retinal hemorrhage was seen. Since his mother with 54 years of age also had a history of retinal hemorrhage that improved spontaneously, fundus examination was performed, revealing tortuosity of the retinal arterioles. Consequently, the patient and his mother were diagnosed as FRAT. He was followed without intervention. Retinal hemorrhage gradually decreased and resolved after 3 months. The BCVA of his left eye gradually improved and reached 20/20 after 1 year.
UNASSIGNED: In this case, the family history was very useful for early diagnosis. Immediate and accurate diagnosis allowed the patient to be followed without intervention and achieve subsequent resolution of retinal hemorrhage and improved vision. FRAT should be considered in cases of sub-internal limiting membrane hemorrhages in young patients even in the presence of discrete retinal arteriolar tortuosity.

To evaluate the clinical characteristics of intraocular hemorrhages among babies screened for retinopathy of prematurity (ROP) and thereby their additional risk to the progression of ROP.
A descriptive study was conducted at a tertiary referral hospital, which included 108 eyes of 60 neonates who were discovered to have retinal hemorrhages on retinal screening of 540 babies at risk for ROP. Maternal, obstetric, and neonatal risk factors were assessed in neonates with retinal hemorrhages. Retinal hemorrhages were assessed in terms of type, area, and relation to different retinal zones.
Among 540 neonates who were screened, retinal hemorrhages were found in 11.2% (n = 60 babies). Elderly primigravida mothers and spontaneous vaginal deliveries with prolonged second stage of labor were a common maternal risk factors for retinal hemorrhages. Low birth weight and preterm were fetal risk factors with neonatal retinal hemorrhages. These hemorrhages were more often bilateral (no. of babies = 48, 80%). Flame-shaped hemorrhages were more common than dot and blot ones. The majority of cases (no. of eyes = 74, 65%) resolved within 4 weeks, whereas four babies (8 eyes, 7.4%) progressed to ROP were treated with laser.
Retinal hemorrhages in neonates are commonly associated with prolonged duration of second stage of labor, advanced maternal age, and anemia. Although not all progress to ROP, recognizing preterm and low birth weight babies with junctional hemorrhages is crucial not to delay the treatment. Regular follow-up until the clearance of hemorrhages and monitoring systemic conditions in these babies of amblyogenic age-group are required.

BACKGROUND: Nonaccidental trauma (NAT) is considered when pediatric patients present with intracranial injuries and a negative history of an accidental injury or concomitant medical diagnosis. The evaluation of NAT should include the consideration of possible medical causes including coagulation, hematologic, metabolic and other genetic disorders, as well as witnessed and unwitnessed accidental injuries.
METHODS: We present a 7-month-old male with spells and incidental findings of bilateral subdural hematomas, retinal hemorrhages, and secondary macrocephaly, leading to investigation for NAT. Biochemical analysis showed excretion of a large amount of D-2-hydroxyglutaric in urine consistent with a biochemical diagnosis of D-2-hydroxyglutaric aciduria, a rare neurometabolic disorder characterized by developmental delay, epilepsy, hypotonia, and psychomotor retardation. None of these symptoms were present in our patient at the time of diagnosis. Molecular genetic testing revealed a pathogenic splice site variant (c.685-2A>G) and a variant of uncertain significance (c.1256G>T) with evidence of pathogenicity in the D2HGDH gene, consistent with a molecular diagnosis of D-2-hydroxyglutaric aciduria type I (OMIM #600721).
CONCLUSIONS: Since several metabolic disorders, including D-2-hydroxyglutaric aciduria type I, can present solely with symptoms suggestive of NAT (subdural and retinal hemorrhages), an early metabolic evaluation by urine organic acid analysis should be included in clinical protocols evaluating NAT. A methodical and nonjudgmental approach coordinated between pediatricians and metabolic specialists is also necessary to ensure that rare genetic conditions are not overlooked to prevent devastating social, legal, and financial consequences of suspected child abuse.