ASSESSMENT OF MACULAR VASCULATURE OF CHILDREN WITH SICKLE CELL DISEASE COMPARED TO THAT OF HEALTHY CONTROLS USING OPTICAL COHERENCE TOMOGRAPHY ANGIOGRAPHY.

PURPOSE: This study aims at describing the macular vascular features of the eyes of pediatric patients with the homozygous form of sickle cell disease (SCD) using optical coherence tomography angiography. METHODS: This is a retrospective case series of eyes of pediatric patients with SCD, race- and age-matched to those of healthy individuals. Using optical coherence tomography angiography images of superficial capillary plexus and deep capillary plexus, the nonflow area, vascular density, and foveal avascular zone were measured. The remodeled microvascular foveal architectures obtained on optical coherence tomography angiography and fluorescein angiography were compared. RESULTS: A total of 19 eyes of patients with SCD and 18 eyes of race-matched healthy controls were examined. The best-corrected visual acuity ranged from 75 to 85 letters in all cases. In the SCD series, 8 eyes displayed signs of retinopathy on funduscopy. The foveal avascular zone was significantly enlarged, the foveal vascular density was 8% lower, and there were more perifoveal vascular alterations in the eyes of patients with SCD compared to those of controls. CONCLUSION: The foveal vascular density was significantly reduced and area of the foveal avascular zone was significantly increased in the eyes of patients with SCD. Consistent architectural changes in the perifoveal capillaries were also observed in the pediatric patients with SCD.

Idiopathic Intracranial Hypertension-A Comparison of Clinical Characteristics Between 4 Medical Centers in Different Geographic Regions of the World.

BACKGROUND: Idiopathic intracranial hypertension (IIH) is a well-characterized syndrome, most commonly affecting obese women of childbearing age. Differences in its prevalence have been reported in various populations. The aim of this article was to determine whether differences in clinical presentation and management exist for patients with IIH between different regions the world. METHODS: Retrospective database analysis of adult patients with IIH from 4 different neuro-ophthalmology clinics. The data collected included gender, age of onset, body mass index (BMI), lumbar puncture opening pressure, initial visual acuity (VA), initial visual field (VF) mean deviation (MD), pharmacological or surgical treatment, length of follow-up, final VA, and final VF MD. RESULTS: The study population consisted of 244 patients, with significant regional variations of female to male ratio. Overall, there was no significant difference regarding the age of diagnosis or the BMI. Acetazolamide was the first line of treatment in all groups but there was a difference between countries regarding second-line treatment, including the use of surgical interventions. Mean initial VA differed between groups but the final change in VA was the same among all the study groups. CONCLUSIONS: There are differences in IIH presentation, treatment, and response to therapy among different countries. International prospective studies involving multiple centers are needed to determine the potential influence of environmental and genetic factors on the development of IIH and to improve the management of this potentially blinding disorder.

Transglabellar approach for resection of anterior midline skull base meningiomas: technical note.

BACKGROUND: We describe our experience of minimally invasive approach of the anterior skull base through the transglabellar approach. METHOD: The technical details of the transglabellar approach are described in this article as we have been using it for the past 3 years. After an inter-eyebrow skin incision, the scalp is elevated allowing the creation of a 3-cm bone flap in the frontal sinus, which gives direct access to the anterior midline skull base. Removal of the tumour is carried out without brain retraction. The closure requires obstruction of nasofrontal ducts and sinus mucosa removal. RESULTS: We operated on 24 patients using this approach. The tumour was completely removed in every case. We reported one case of meningitis and three cerebrospinal fluid leaks. Every patient was satisfied with the aesthetic results. CONCLUSIONS: The transglabellar approach is a reasonably easy and efficient way to resect anterior midline skull base meningiomas.

Sixth Nerve Palsy from Cholesterol Granuloma of the Petrous Apex.

Herein, we report a patient who had an isolated sixth nerve palsy due to a petrous apex cholesterol granuloma. The sixth nerve palsy appeared acutely and then spontaneously resolved over several months, initially suggesting a microvascular origin of the palsy. Subsequent recurrences of the palsy indicated a different pathophysiologic etiology and MRI revealed the lesion at the petrous apex. Surgical resection improved the compressive effect of the lesion at Dorello's canal and clinical improvement was observed. A relapsing-remitting sixth nerve palsy is an unusual presentation of this rare lesion.

Melanopsin-Mediated Acute Light Responses Measured in Winter and in Summer: Seasonal Variations in Adults with and without Cataracts.

Seasonal adaptation is a ubiquitous behavior seen in many species on both global hemispheres and is conveyed by changing photoperiods. In humans this seasonal adaptation is less apparent, in part because changes in daylength are masked by the use of electrical lighting at night. On the other hand, cataracts which reduce light transmission, may compound seasonal changes related to the reduced daylength of winter. To better understand the effects of different photoperiod lengths in healthy adults without and with cataracts, we tested their melanopsin-mediated light responses in summer vs. winter. Fifty-two participants (mean age 67.4 years; 30 with bilateral cataracts and 22 age-matched controls with clear lenses; pseudophakes) were tested twice, once in summer and once in winter. At each test session we assessed the electroretinogram and pupil responses during daytime and we determined melatonin suppression, subjective sleepiness and mood in response to light exposure in the evening. Circadian rest-activity cycles and sleep from activity recordings were also analyzed for both seasons. Both groups had similar visual function. There were no seasonal differences in the electroretinogram. For the pupil responses to bright blue light, the post-illumination pupil response (PIPR) was greater in winter than summer in pseudophakes, but not in cataract participants, whereas melatonin suppression to acute light exposure showed no differences between both groups and seasons. Overall, intra-daily variability of rest-activity was worse in winter but participants felt sleepier and reported worse mood at the laboratory in evening time in the summer. Those with cataracts had poorer sleep quality with lower sleep efficiency, and higher activity during sleep in winter than summer. In this study, the PIPR showed a seasonal variation in which a larger response was found during winter. This variation was only detected in participants with a clear intraocular lens. In the cataract group, visual function was not impaired yet these participants showed a lack of seasonal changes in the pupil response to blue light and poorer sleep in winter. These findings raise the question for tailored lighting conditions for cataract patients in order to counter potentially deleterious effects of living with chronically lower light exposure.