Common visual problems in children with disability

Children with disability are at a substantially higher risk of visual impairment (VI) (10.5% compared with 0.16%) but also of ocular disorders of all types, including refractive errors and strabismus. The aetiology of VI in children with disability reflects that of the general population and includes cerebral VI, optic atrophy, as well as primary visual disorders such as retinal dystrophies and structural eye anomalies. VI and other potentially correctable ocular disorders may not be recognised without careful assessment and are frequently unidentified in children with complex needs. Although assessment may be more challenging than in other children, identifying these potential additional barriers to learning and development may be critical. There is a need to develop clearer guidelines, referral pathways and closer working between all professionals involved in the care of children with disability and visual disorders to improve our focus on the assessment of vision and outcomes for children with disability

A cohort study on acute ocular motility disorders in pediatric emergency department

Background: Acute ocular motility disorders (OMDs) in children admitted to Emergency Department (ED) represents a not so rare condition with a wide spectrum of different etiologies. The emergency physician must be skilled in rapidly identifying patients with potentially life threatening (LT) forms, requiring further diagnostic procedures. The aim of the study was to assess characteristics of children with acute Ocular Motility Disorders (OMDs), and to identify "red flags" for recognition of underlying life-threatening (LT) conditions. Methods: A retrospective cohort study evaluated children (2 months-17 years) admitted to a tertiary Emergency Department in 2009-2014. A subgroup analysis was performed comparing children with and without LT conditions. Results: Of 192 visits for OMDs, the isolated strabismus occurred most frequently (55.6%), followed by pupil disorders (31.8%), ptosis (5.2%) and combined OMDs (11.5%). The majority of acute OMDs involved no underlying LT conditions (n = 136) and most of them were infants or toddlers (50%). In a multivariable analysis, LT conditions included especially children over 6 years of age, increasing the odds ratio by 2% for each months of age (p = 0.009). LT etiologies were 16 times more likely in combined OMDs (p = 0.018), were over 13 times more likely to report associated extra-ocular signs/symptoms (p = 0.017) and over 50 times more likely to report co-morbidity (p = 0.017). Conclusion: OMDs are not an uncommon presentation at ED. Although most of them involve non-LT conditions, the ED physician should consider potential "red flags" for appropriate management of children such as age > 6 years, combined OMDs, extra-ocular symptoms and co-morbidity

Assessing full thickness oral mucosal grafting: complications and postoperative outcomes in a broad collective of patients

Background: Conjunctival defects can be repaired with several mucosal tissues. The simplicity of harvesting oral mucosa and its wide availability makes it the preferred graft tissue for all indications requiring mucosal grafting. Through analysing the postsurgical outcomes and rate of revisions, this study explores the suitability of oral mucosa grafts, depending on the initial diagnosis. Methods: We reviewed all the files of patients with a history of oral mucosal graft surgery, performed at our clinic between 2012 and 2018, focusing on complications and revision rates. Results: In total, we analysed 173 oral mucosa grafts in 131 patients. The most common initial diagnosis was tumour resection, followed by surgical complications, postenucleation socket syndrome, trauma and ocular surface disorders. Complication and revision rates depended highly on the initial diagnosis. Revision rates were highest if the initial diagnosis included ocular surface disorders or chemical trauma. Conclusions: Oral mucosa grafting (OMG) is the most effective treatment for a wide range of ocular conditions involving conjunctival defects. Conjunctival defects that result from trauma or cicatricial surface diseases seem less suitable for OMG and may benefit from alternative graft tissue or treatment options

Rare diseases of the anterior segment of the eye: update on diagnosis and management

This special issue is focused on the current approaches used to identify and manage rare diseases of the anterior segment of the eye, which range from congenital to acquired disorders that are caused by ocular or systemic conditions and often have consequences that extend beyond the anterior segment of the eye

Anterior-segment optical coherence tomography for the detection and therapeutic monitoring of corneal disorders

Background: Over recent years a revolutionary trend happened on imaging technologies to diagnose and monitor treatment of a varied group of ophthalmic pathologies. Recent reports have analyzed the microstructural changes of various ocular surface and corneal disorders, particularly ocular surface squamous neoplasia (OSSN) and keratoconus using anterior-segment optical coherence tomography (AS-OCT). Aim of this short communication is to elaborate on clinical applications AS-OCT for the detection and therapeutic monitoring of corneal disorders. Methods: We performed an English literature search without a time limit and intending to identify articles related to the AS-OCT applications in the detection and therapeutic monitoring of corneal disorders. The most relevant articles were selected.  practical points of selected papers and advantages and disadvantages of AS-OCT were retrieved from them and summarized. Results:.Many records reported the AS-OCT applications for diagnosing many ocular surface disorders, the microstructural changes of different inflammatory, infectious, degenerative, and dystrophic corneal disorders. Its applications in identifying disease activity and therapeutic monitoring of various corneal pathologies, including stromal edema associated with angle-closure glaucoma, Fuchs endothelial dystrophy, infectious keratitis, and bullous keratopathy, are promising. The percentage of diagnostic sensitivity, specificity, and accuracy of artificial intelligence methodologies applied to AS-OCT imaging analysis today has reached 94% to 100%. Moreover, AS-OCT is very useful for analyzing the extension of scar and leukoma depth for surgical planning of partial or total corneal transplantation. Conclusions: There is a clear prospect for expanding application of corneal OCT imaging technology, a rapid, non-invasive, and now a promising lower-cost device, which is becoming an in-office standard-of-care tool for the assessment of different corneal and ocular surface pathologies. KEYWORDS anterior-segment optical coherence tomography, AS-OCT, ocular surface disorders, corneal disorders, ocular surface squamous neoplasia, OSSN, keratoconu

Ophthalmic disorders among students of School for the Deaf

Aim: This study aimed at determining the prevalence and pattern of ophthalmic disorders amongstudents of School for the Deaf, Akure, Ondo State, Nigeria.Methodology: This is a cross sectional descriptive study was conducted in October, 2011 aspart of activities marking the Annual Physicians’ week of Nigerian Medical Association (NMA),Ondo State. Ethical clearance was obtained from the Ethical Review Committee of FederalMedical Centre, Owo prior to commencement of this study. The permission of the SchoolAuthority was also obtained before the commencement of this study. The respondents wereselected by simple random sampling technique. All enrolled participants were interviewed withthe aid of the study instrument (questionnaire) by the authors and interpreters (school teachers).Results: The respondents comprised of 91(56.9%) Males and 69 Females (43.1%). Nearly all therespondents;158(98.8%) were deaf and dumb. Most respondents; 116(72.5%) had ocularexamination in the past. Few respondents; 118(73.75%) had ophthalmic disorder. The commonest ophthalmic disorder was refractive error which was found in 16 respondents(38.1%). Myopia was diagnosed in 9 respondents.CONCLUSION: Most of the respondents were deaf and dumb. Few respondents hadophthalmic disorder. The commonest ophthalmic disorder was refractive error. Myopia was themost predominant refractive error. There is need for periodic ocular screening and treatment atthe School for the Deaf.

A Review of Neuro-ophthalmologic Emergencies

Background: Neuro-ophthalmic emergencies are relatively uncommon, however their outcome cause severe morbidity and even mortality. The ocular manifestations of these disorders are pointers to a more dangerous central nervous system or systemic pathology. The review aims to highlight the major ocular disorders that constitute neuro-ophthalmologic emergencies with a view to increasing the index of suspicion of these visual/life threatening disorders among primary care physicians, neurologists and ophthalmologists.Method: The available literature on neuro-ophthalmologic emergencies was reviewed, using available journals and internet based search engines and resources. Keywords employed were Neuro-ophthalmology Emergency and Ocular Morbidity.Result: The incidence of this group of emergencies is lower than that of other ophthalmic emergencies such as ruptured globe and retinal detachment; however they are associated with higher morbidity and even mortality. These emergencies can be grouped into four major categories for ease of diagnosis. Nigerian literature on neuroophthalmologic emergencies was unavailable.Conclusion: The key to prevention of morbidity and mortality from neuro-ophthalmologic disorders is to have a good knowledge of the  manifestations of these disorders and a high index of suspicion.Keywords: Neuro-ophthalmology; Emergency; Ocular Morbidit

Rare diseases leading to childhood Glaucoma. epidemiology, pathophysiogenesis, and management

Noteworthy heterogeneity exists in the rare diseases associated with childhood glaucoma. Primary congenital glaucoma is mostly sporadic; however, 10% to 40% of cases are familial. CYP1B1 gene mutations seem to account for 87% of familial cases and 27% of sporadic cases. Childhood glaucoma is classified in primary and secondary congenital glaucoma, further divided as glaucoma arising in dysgenesis associated with neural crest anomalies, phakomatoses, metabolic disorders, mitotic diseases, congenital disorders, and acquired conditions. Neural crest alterations lead to the wide spectrum of iridocorneal trabeculodysgenesis. Systemic diseases associated with childhood glaucoma include the heterogenous group of phakomatoses where glaucoma is frequently encountered in the Sturge-Weber syndrome and its variants, in phakomatosis pigmentovascularis associated with oculodermal melanocytosis, and more rarely in neurofibromatosis type 1. Childhood glaucoma is also described in systemic disorders of mitotic and metabolic activity. Acquired secondary glaucoma has been associated with uveitis, trauma, drugs, and neoplastic diseases. A database research revealed reports of childhood glaucoma in rare diseases, which do not include glaucoma in their manifestation. These are otopalatodigital syndrome, complete androgen insensitivity, pseudotrisomy 13, Brachmann-de Lange syndrome, acrofrontofacionasal dysostosis, caudal regression syndrome, and Wolf-Hirschhorn syndrome

Pro-Secretory Activity and Pharmacology in Rabbits of an Aminophenyl-1,3,5-Triazine CFTR Activator for Dry Eye Disorders.

PurposePharmacological activation of ocular surface cystic fibrosis transmembrane conductance regulator (CFTR) chloride channels is a potential pro-secretory approach to treat dry eye disorders. We previously reported the discovery of aminophenyl-1,3,5-triazines, one of which, N-methyl-N-phenyl-6-(2,2,3,3-tetrafluoropropoxy)-1,3,5-triazine-2,4-diamine (herein called CFTRact-K267), fully activated human wildtype CFTR with EC50 ∼ 30 nM and increased tear volume for 8 hours in mice. Here, functional and pharmacological studies of CFTRact-K267 were done in adult New Zealand white rabbits.MethodsCFTR chloride conductance was measured in vivo by ocular surface potential differences and in ex vivo conjunctiva by short-circuit current. Tear volume was measured by the Schirmer tear test II and CFTRact-K267 pharmacokinetics and tissue distribution by liquid chromatography/mass spectrometry. Toxicity profile was studied for 28 days with twice-daily topical administration.ResultsElectrophysiological measurements in vivo and in ex vivo conjunctiva demonstrated CFTR activation by CFTRact-K267. A single topical dose of 3 nmol CFTRact-K267 increased tear production by >5 mm for 9 hours by the Schirmer tear test, with predicted therapeutic concentrations maintained in tear fluid. No tachyphylaxis was seen following 28-day twice-daily administration, and changes were not observed in corneal surface integrity or thickness, intraocular pressure, or ocular histology. At day 28, CFTRact-K267 was concentrated in the cornea and conjunctiva and was not detectable in blood or peripheral organs.ConclusionsThese studies support the development of CFTRact-K267 as a pro-secretory therapy for dry eye disorders