BACKGROUND: Cortical visual impairment (CVI) is a severe loss of visual function caused by damage to the visual cortex or its afferents, often as a consequence of hypoxic insults during birth. It is one of the leading causes of vision loss in children, and it is most often permanent.
OBJECTIVE: Several studies have demonstrated limited vision restoration in adults who trained on well-controlled psychophysical tasks, after acquiring CVI late in life. Other studies have shown improvements in children who underwent vision training. However, little is known about the prospects for the large number of patients who acquired CVI at birth but received no formal therapy as children.
METHODS: We, therefore, conducted a proof-of-principle study in one CVI patient long after the onset of cortical damage (age 18), to test the training speed, efficacy and generalizability of vision rehabilitation using protocols that had previously proven successful in adults. The patient trained at home and in the laboratory, on a psychophysical task that required discrimination of complex motion stimuli presented in the blind field. Visual function was assessed before and after training, using perimetric measures, as well as a battery of psychophysical tests.
RESULTS: The patient showed remarkably rapid improvements on the training task, with performance going from chance to 80% correct over the span of 11 sessions. With further training, improved vision was found for untrained stimuli and for perimetric measures of visual sensitivity. Some, but not all, of these performance gains were retained upon retesting after one year.
CONCLUSIONS: These results suggest that existing vision rehabilitation programs can be highly effective in adult patients who acquired CVI at a young age. Validation with a large sample size is critical, and future work should also focus on improving the usability and accessibility of these programs for younger patients.

BACKGROUND: Deprivation amblyopia is a great concern in hyperplastic persistent pupillary membranes (PPM) which blocked visual axis. Other ocular abnormality may accompany and further hinder the visual development of the infants. We evaluate the long-term visual prognosis and complications in patients with dense PPM and other associated abnormalities treated with early surgical intervention and timely visual rehabilitation.
METHODS: Medical records of patients with surgical removal of PPM from 2000 to 2020 and also receiving visual rehabilitation were retrospectively reviewed. Besides visual axis blocked PPM, patients combined with other amblyopic risk factors or ocular abnormalities were included. Due to preparation for subsequent lens extraction if an underlying cataract was present, the surgical settings including the instruments and wound direction were similar to cataract surgery. All patients were enrolled in a visual rehabilitation program as soon as possible. The results including sex, age, timing of operation, initial and final visual acuity, refractive errors, and complications were recorded.
RESULTS: Seven cases of five patients were included in this case series. Mean age at surgery was 42.3 ± 21.1 months (range, 5 to 66 months) and the post-operative follow-up period was 4.9 years (range, 1.2 to 8.2 years). The patient age at time of surgery ranged from 2.5 months to 2.5 years (mean, 14 months). Mean postoperative follow-up was 5.3 years (range, 2.5-8 years). There were no intra-operative and post-operative complications. Final BCVA varied with a mean value of 0.29 logMAR (range, 0 to 1 logMAR). An associated ocular abnormality of ametropia and strabismus led to the best visual prognosis.
CONCLUSIONS: In patients with PPM, there were no significant complications in any patient using our technique. The surgical settings are easier to handle and more familiar with pediatric surgeons. Besides deprivation with patching, early PPM intervention and timely visual rehabilitation achieve the best visual prognosis in patients associated with risk of ametropic and strabismic amblyopia.
BACKGROUND: This retrospective, interventional case series study was conducted at China Medical University Hospital between April 1, 2000 and April 31, 2020. (IRB number: CMUH109-REC2-069 ).

OBJECTIVE: To report the anatomical and functional outcomes of Argus II retinal prosthesis implantation in Korean patients.
METHODS: We included 5 consecutive patients with end-stage retinitis pigmentosa (RP) who underwent Argus II retinal prosthesis implantation and were followed for at least 12 months. The transcorneal electrical evoked response was utilized for patient selection. We used intraoperative optical coherence tomography (OCT) for optimal placement of the array and provided specialized vision rehabilitation training. A morphological evaluation using SD-OCT and a functional evaluation using computer-based visual function tests, a letter-reading ability test, and the Functional Low-Vision Observer Rated Assessment (FLORA) were conducted.
RESULTS: Postoperatively, the array was completely apposed to the retinal surface in all eyes, except for one eye which had a preexisting macular concavity. Fibrosis-like tissues of ≥50-μm thickness developed at the interface in 2 eyes. All of the patients showed improvement in computer-based visual function tests and could read ETDRS letters at a distance of 50 cm. Three patients could read Korean words. FLORA was improved in all patients, mainly in tasks of visual mobility, daily activities, and social interactions.
CONCLUSIONS: Along with good anatomical outcomes and specialized rehabilitation practices, recipients of the Argus II implant showed profound improvements in functional vision and mobility.

There is evidence that channelopathies are the cause of many different neurological diseases. The epileptic perinatal encephalopathy due to mutation in the KCNQ2 gene is a rare disease involving severe tetraparesis and cerebral visual impairment. Diseases of this kind are associated with severe disability that involves multiple systems and requires accurate genetic diagnosis and early multidisciplinary care once clinical stability is reached.
We describe a case of a baby girl with KCNQ2 encephalopathy who came to our observation for rehabilitation at age 2 years and 6 months.
We stress the importance of a correct clinical, pharmacological and visual diagnosis. Correct diagnosis made it possible to involve the baby girl and her care-giver in an early process of visual rehabilitation lasting 6 months, the effects of which proved to persist at follow-up after more than a year, making it possible to start a useful inter-professional rehabilitation plan.