Ophthalmic Abnormalities Research Articles

Diagnosis and Surgical Removal of a Corneal Dermoid in a Red-eyed Tree Frog, Agalychnis callidryas (Anura, Hylidae), From the Tenorio Volcano, Costa Rica

Abstract A male, red-eyed frog (Agalychnis callidryas) was collected from the Tenorio Volcano region in Costa Rica due to the presence of a unilateral ocular abnormality affecting its left eye. Approximately 70% of the cornea was replaced by a well demarcated, 5.0 mm in diameter, opaque, bright green and roughened tissue compatible with skin. The affected eye was enucleated under established ophthalmic surgical procedures. A dermoid was confirmed by histopathology based on the finding of amphibian cutaneous (epidermal and dermal) tissue replacing the cornea and characterized by the presence of keratinized epidermis, spongy dermis, and integumentary glands. After a successful post-operative recovery period the frog was released back to the wild, demonstrating the viability of medical intervention and surgery in wild amphibians. Dermoids are congenital lesions that result from a failure in the differentiation of the superficial ectoderm to generate normal corneal epithelium and the subsequent development of epidermis and dermal tissues in the area. Corneal dermoids have been reported in horses, cattle, sheep, wildebeest, pigs, rats, dogs, cats, birds, and humans, this being the first case reported in amphibians. In the locality where the frog was found, several other ophthalmic abnormalities have been documented in other amphibians. Active monitoring of wild amphibians is ongoing, and possible causes of ophthalmic abnormalities in the area are being investigated.

Keratin 8/18a.1 Expression Influences Embryonic Neural Crest Cell Dynamics and Contributes to Postnatal Corneal Regeneration in Zebrafish.

A complete understanding of neural crest cell mechanodynamics during ocular development will provide insight into postnatal neural crest cell contributions to ophthalmic abnormalities in adult tissues and inform regenerative strategies toward injury repair. Herein, single-cell RNA sequencing in zebrafish during early eye development revealed keratin intermediate filament genes krt8 and krt18a.1 as additional factors expressed during anterior segment development. In situ hybridization and immunofluorescence microscopy confirmed krt8 and krt18a.1 expression in the early neural plate border and migrating cranial neural crest cells. Morpholino oligonucleotide (MO)-mediated knockdown of K8 and K18a.1 markedly disrupted the migration of neural crest cell subpopulations and decreased neural crest cell marker gene expression in the craniofacial region and eye at 48 h postfertilization (hpf), resulting in severe phenotypic defects reminiscent of neurocristopathies. Interestingly, the expression of K18a.1, but not K8, is regulated by retinoic acid (RA) during early-stage development. Further, both keratin proteins were detected during postnatal corneal regeneration in adult zebrafish. Altogether, we demonstrated that both K8 and K18a.1 contribute to the early development and postnatal repair of neural crest cell-derived ocular tissues.

Care of children with congenital rubella syndrome (CRS) in Indonesia.

Congenital rubella syndrome (CRS) is associated with severe birth defects, that lead to disability in later life. Hence, early detection and intervention are needed to prevent permanent disability and mortality in children with CRS. We evaluated the time to diagnosis or correction intervention related to survival rate using survival analysis. A retrospective cohort study was conducted to evaluate the follow-up of CRS-confirmed cases from 2011-2018 at a national referral hospital in Jakarta, Indonesia. Parents of eligible children who registered in the national CRS registry as laboratory-confirmed CRS cases were contacted through phone calls or home visits and interviewed about the current situation of their child's health. We also obtained clinical data from the medical records. Fifty children, age 4 to 14 years, identified with laboratory-confirmed CRS were included in this study. Half (54%) of these children were female. All were born from mothers with no previous rubella vaccination history. Ophthalmic abnormalities such as congenital cataracts (88%) were the most common birth defect. Multiple congenital abnormalities including congenital heart disease, ocular abnormalities, and auditory defects were identified in 52% of the children. Based on Kaplan-Meier analysis, 50% of children were diagnosed at four months. Ophthalmic corrections such as cataract surgery were performed earlier than heart or auditory correction, with 50% of children undergoing eye correction one month after the diagnosis. There is a vital need to implement CRS surveillance in Indonesia to know the burden of CRS and reinforce the preventive actions, including vaccination against rubella.

Dyskeratosis congenita with squamous cell carcinoma of the tongue: A rare case report.

Dyskeratosis congenita is a rare inherited bone marrow failure disorder characterized by a classic triad: nail dystrophy, reticulate skin pigmentation, and oral leukoplakia. Patients with dyskeratosis congenita have also been described as having gastrointestinal, genitourinary, neurological, ophthalmic, pulmonary, and skeletal abnormalities. Also, 80% of patients are affected with oral leukoplakia and these areas are more prone to develop into oral squamous cell carcinoma. We hereby report a rare case of oral squamous cell carcinoma of the tongue in a young male patient with dyskeratosis congenita.

Automated classification of multiple ophthalmic diseases using ultrasound images by deep learning

BackgroundUltrasound imaging is suitable for detecting and diagnosing ophthalmic abnormalities. However, a shortage of experienced sonographers and ophthalmologists remains a problem. This study aims to develop a multibranch transformer network...

Preliminary study on intraocular abnormalities found in senior dogs by ultrasonography in a veterinary hospital in Guayaquil, Ecuador

Research on ophthalmology in veterinary medicine in Ecuador is scarce, and ocular health in older dogs should be studied. This study aimed to determine the incidence of ophthalmic abnormalities in senior dogs attending a hospital at Guayaquil and relate them to the type of lesion, breed, sex, age, and type of dog head shape. One hundred healthy senior dogs (> six years old) who underwent consultation during the study were subjected to bilateral eye ultrasonography (USG 10-MHz transducer) and examined for ocular alterations. Breed, sex, age, and head shape were also recorded. Thirty-eight animals presented with ocular abnormalities: presumed cataracts (19%), crystalline luxation (6%), presumed senile retinal degeneration (6%), retinal detachment (4%), nuclear sclerosis (4%), masses and vitreous degeneration (3%), presumed glaucoma (2%), and cysts in the anterior and posterior chambers (1%), mainly as bilateral lesions (24%). Cataracts were present in a more significant proportion in dolichocephalic dogs (8/18) than in brachycephalic (3/22) and mesocephalic dogs (8/60) (p =0.01). In addition, older animals were more affected, both unilaterally and bilaterally, and in both locations (lens or retina)(p <0.05), whereas crystalline luxation and senile retinal degeneration were significantly associated with age(p <0.05), whereas trends were observed for cataracts (p =0.065). Although ultrasound alone does not allow a definitive diagnosis, these preliminary results provide insights into the ocular abnormalities in apparently healthy senior dogs from Guayaquil (Ecuador). Unsuspected intraocular lesions might be relatively high in these patients; however, more extensive studies with complete ocular examination are recommended to confirm these results. Keywords: Aging dog; Cataracts; Retinal lesion; Ultrasonography

Preliminary study on intraocular abnormalities found in senior dogs by ultrasonography in a veterinary hospital in Guayaquil, Ecuador

Research on ophthalmology in veterinary medicine in Ecuador is scarce, and ocular health in older dogs should be studied. This study aimed to determine the incidence of ophthalmic abnormalities in senior dogs attending a hospital at Guayaquil and relate them to the type of lesion, breed, sex, age, and type of dog head shape. One hundred healthy senior dogs (> six years old) who underwent consultation during the study were subjected to bilateral eye ultrasonography (USG 10-MHz transducer) and examined for ocular alterations. Breed, sex, age, and head shape were also recorded. Thirty-eight animals presented with ocular abnormalities: presumed cataracts (19%), crystalline luxation (6%), presumed senile retinal degeneration (6%), retinal detachment (4%), nuclear sclerosis (4%), masses and vitreous degeneration (3%), presumed glaucoma (2%), and cysts in the anterior and posterior chambers (1%), mainly as bilateral lesions (24%). Cataracts were present in a more significant proportion in dolichocephalic dogs (8/18) than in brachycephalic (3/22) and mesocephalic dogs (8/60) (p =0.01). In addition, older animals were more affected, both unilaterally and bilaterally, and in both locations (lens or retina)(p <0.05), whereas crystalline luxation and senile retinal degeneration were significantly associated with age(p <0.05), whereas trends were observed for cataracts (p =0.065). Although ultrasound alone does not allow a definitive diagnosis, these preliminary results provide insights into the ocular abnormalities in apparently healthy senior dogs from Guayaquil (Ecuador). Unsuspected intraocular lesions might be relatively high in these patients; however, more extensive studies with complete ocular examination are recommended to confirm these results. Keywords: Aging dog; Cataracts; Retinal lesion; Ultrasonography

The Shih Tzu eye: Ophthalmic findings of 1000 eyes.

To evaluate the frequency of ophthalmic disorders in Shih Tzus. Five hundred client-owned Shih Tzu dogs (1000 eyes). Medical records of 500 Shih Tzu dogs were reviewed and ophthalmic examination data were collected and analyzed. In total, out of 1000 eyes, 964 (96.4%; OD: 480 eyes, OS: 484 eyes) had at least one ophthalmic abnormality, and only 36 eyes (3.6%; OD: 20 eyes, OS: 16 eyes) were diagnosed as normal. A total of 1375 individual ophthalmic abnormalities were observed in 964 diseased eyes. Fifty-nine different unique diagnoses were made in the study population, and only three conditions [medial canthal entropion (10.4% of eyes; bilateral in 10.2% of dogs), caruncular trichiasis (21.1% of eyes; bilateral in 20.2% of dogs), and reduced tear film breakup time (17.6% of eyes; bilateral in 17.4% of dogs)] affected 49.1% of the eyes. Findings suggested that the most prevalent disorders for the Shih Tzus were hairy caruncle, reduced TBUT, medial canthal entropion, keratoconjunctivitis sicca, pigmentary keratitis, lagophthalmos, and mature cataract.

TREX1 Mutations in Aicardi–Goutières Syndrome with Congenital Glaucoma

TREX1 Mutations in Aicardi–Goutières Syndrome with Congenital Glaucoma

RETINAL AGING IN COMPANION DOGS

Abstract Companion dogs cohabit with humans in approximately 45% of US households. They experience similar environmental exposures and lifestyles to their human companions, and have shown sensory, cognitive, and neurological decline with aging. The relationship between age and retinal neurologic function in dogs has not been described. The aim of this study was to determine associations between age and retinal full-field electroretinographic measures in companion dogs, an important translational model species for human neurologic aging. Healthy adult companion dogs with no ophthalmic abnormalities were included (n=54, 18 male, 36 female, median weight 25.7kg, median age 89 months). Unilateral full-field light-adapted and dark-adapted electroretinography (ERG) was performed using a handheld device, with mydriasis and topical anesthesia. Standard least squares effect leverage models were used with log transformed data to determine the cross-sectional effect of age, sex, and bodyweight on ERG amplitudes and peak times. Age and sex were significantly associated with ERG outcome variables, more consistently with ERG amplitude than peak time; age had the largest and most significant effect leverage on outcomes (significantly affecting 3/9 peak time and 8/10 amplitude outcome variables). Younger and female dogs had higher ERG amplitudes. Body weight had no significant effect on any outcome. Our finding that aged companion dogs have reduced ERG amplitudes and prolonged peak times mirror ERG findings in aging humans. Because dogs have a shorter lifespan compared with humans, they may act as a sentinel species to monitor for emerging risk factors for age-related neurologic decline.

Perinatal characteristics and longer-term outcomes in Brazilian children with confirmed or suspected congenital Zika infection: ZIKAction Paediatric Registry.

Despite growing scientific knowledge of Zika virus (ZIKV) infection, questions remain regarding ZIKV infection in pregnancy and congenital ZIKV syndrome (CZS). The ZIKAction Paediatric Registry is an international registry of children with documented ZIKV exposure in utero and/or with confirmed or suspected CZS. Its aim is to characterize these children (i.e., clinical, radiological, neurodevelopmental features) and describe outcomes, longer-term sequelae and management through retrospective case note review. This analysis described the maternal and perinatal characteristics of children in the Registry's Bahia arm, assessed their neuroimaging, ophthalmic, hearing and electroencephalography abnormalities by microcephaly classification and reported on hospitalisations. Children born in 2015-2018 and enrolled 2020-2021 in three public health facilities in Salvador were included. Of 129 (57% female) children, 15 (11·6%) had laboratory-confirmed congenital ZIKV infection and 114 (88·4%) suspected CZS. At delivery, 15 (11·6%) were normocephalic, 30 (23·3%) moderately microcephalic, and 84 (65·1%) severely microcephalic. Median birth head circumference z-score was -3·51 [IQR, -4·69,-2·73]. During follow-up, all children had abnormal neuroimaging, 80·3% (94/117) abnormal electroencephalogram, 62·2% (77/120) ophthalmic abnormalities, and 27·4% (34/124) hearing impairment. Microcephaly classification was significantly associated with gestational age, and ophthalmological and electroencephalography abnormalities. Of 125 children with hospitalisation data, 52 (41·6%) had been hospitalised by most recent follow-up, at median age of 15·8 [4·0, 34·4] months; infections were the leading cause. Congenital ZIKV infection is an emerging disease with a varied and incompletely understood spectrum. Continued long-term follow-up is essential to understand longer-term prognosis and to inform future health and educational needs.

Ocular manifestation of an adult Niemann-Pick disease type B

Niemann-Pick disease is a rare, autosomal recessive inherited lysosomal storage disorder. The pathophysiological background for this condition is the deficiency or reduced function of the enzyme sphingomyelinase, as well as a deficiency in the intracellular cholesterol transporter protein. Due to the breakdown defect, sphingomyelin and cholesterol accumulate in the lysosomes of cells. The disease is divided into 5 subtypes (A, A/B, B, C, D). The authors present the case of a 24-year-old young man diagnosed with Niemann-Pick disease type B as a child, focusing on the ophthalmic manifestation of the disease. During the examination of the patient, fundus photographs and fundus autofluorescence imaging were taken, and optical coherence tomography (OCT), optical coherence tomography angiography (OCTA), and visual field (perimetry) examinations were performed. The characteristic macular halo and the cherry-red spot in the fovea were clearly visible during ophthalmoscopy and on the fundus photographs. The OCT images showed focal thickening with high reflectivity in the ganglion cell layer corresponding to the macular halo, and the area of the foveola was spared. With visual field examination, an intact field of vision was found on both eyes. Similar to the presented patient, symptoms in patients with the B subtype are milder, and besides the visceral symptoms, there are no neurological symptoms, and the specific ophthalmic abnormalities do not cause visual impairment. Currently, Niemann-Pick disease is considered a rare disease, and the diagnosis of the patients is often delayed or even missed due to non-specific or mild symptoms. Through consultation between medical specialties, ophthalmological examination can also contribute to the correct diagnosis in cases with mild general symptoms. Timely diagnosis can potentially lead to mitigation of symptoms thanks to the ever-expanding therapeutic options, stabilization of the disease progression, and increase of the patients' life expectancy. Orv Hetil. 2023; 164(46): 1838-1844.

Diminished pupillary light reflexes, elevated third eyelids, and decreased tear production are commonly associated with canine dysautonomia.

To retrospectively describe ocular abnormalities reported in dogs with presumed dysautonomia. 79 dogs with dysautonomia. Medical records from the Kansas State University Veterinary Health Center from 2004 to 2021 were reviewed for dogs with a clinical or histopathologic diagnosis of canine dysautonomia (CD). Ophthalmic exam abnormalities, nonocular clinical signs, and outcomes were recorded. Most dogs (73/79 [92.4%]) with CD exhibited at least 1 ocular abnormality. The most common ocular abnormalities were diminished pupillary light reflexes (PLRs) in 55 of 79 (69.6%) dogs and elevation of the third eyelids in 51 of 79 (64.6%) dogs. Schirmer tear test values were bilaterally decreased in 32 of 56 (57.1%) dogs. Other ocular abnormalities included resting mydriasis, ocular discharge, photophobia, blepharospasm, corneal ulceration, and conjunctival vessel pallor. The most common nonocular clinical signs were vomiting or regurgitation in 69 of 79 (87.3%) and diarrhea in 34 of 79 (43.0%) dogs. Pharmacologic testing with dilute 0.01%, 0.05%, or 0.1% pilocarpine yielded pupillary constriction in 42 of 51 (82.4%) dogs. Thirty-two of 79 (40.5%) dogs survived to discharge. Resolution of ocular abnormalities was variable. Ophthalmic abnormalities such as diminished PLRs, elevation of the third eyelids, and decreased tear production are commonly associated with CD and provide support for its antemortem clinical diagnosis, though dogs with normal PLRs can be diagnosed with the disease. Pharmacologic testing with dilute topical pilocarpine in dogs with clinical signs suggestive of dysautonomia supports a diagnosis of CD. Ophthalmic abnormalities may improve or resolve over time.

SAT335 Bilateral Pheochromocytomas In Multiple Endocrine Neoplasia Type 2B: A Frozen Abdomen, A Slow Growing Mass, And A Backdoor Solution

Abstract Disclosure: O.A. Shariq: None. T.J. McKenzie: None. W.F. Young: None. Background: Multiple endocrine neoplasia type 2B (MEN2B) is most commonly caused by a germline methionine-to-threonine substitution at codon 918 (M918T) of the RET proto-oncogene. The syndrome is characterized by medullary thyroid carcinoma, pheochromocytoma, and extra-endocrine manifestations (intestinal ganglioneuromas, mucosal neuromas, and skeletal and ophthalmic abnormalities). We describe a patient with MEN2B and bilateral pheochromocytomas, in whom the slow-growing nature of the lesions, and previous history of an extra-endocrine manifestation, precluded the typical approach to surgical resection. Clinical Case: A 58-year-old-male with MEN2B, diagnosed at age 15, presented with a 6-month history of episodic palpitations and labile blood pressures. His past surgical history included a total abdominal colectomy and ileorectal anastomosis at age 7 years for megacolon, secondary to intestinal ganglioneuromatosis. He subsequently required multiple laparotomies and adhesiolysis procedures for recurrent small bowel obstructions. During his most recent laparotomy at age 44, he was noted to have a ‘frozen’ abdomen with dense adhesions involving the small bowel. CT abdomen revealed bilateral adrenal masses, measuring 2.9 cm and 1.5 cm on the right and left, respectively. Review of previous imaging revealed that the right adrenal mass had been present 16 years prior, measuring 1.0 cm at that time, while the left-sided mass was not seen. Biochemical workup was negative previously, but now showed elevated plasma metanephrine (4.1 nmol/L [N<0.5 nmol/L ]) and normetanephrine 2.3 nmol/L [N<0.9 nmol/L ]) levels. In the setting of extensive intra-abdominal adhesions and the slow-growing nature of both masses, the decision was made to perform a unilateral right adrenalectomy through a retroperitoneoscopic, rather than a transabdominal, approach. Pathology confirmed a 4.4 cm pheochromocytoma. The patient was discharged on postoperative day 1 and was symptom free at 1 year follow-up. His most recent plasma metanephrine and normetanephine levels were 0.7 nmol/L and 0.5 nmol/L, respectively. Clinical Lessons: Patients with MEN2B have a 50% lifetime risk of developing pheochromocytoma. Unlike medullary thyroid cancer, which exhibits an aggressive phenotype in this syndrome, MEN2B-associated pheochromocytomas may be slow growing, thereby avoiding the need for an initial bilateral adrenalectomy. Many patients with MEN2B develop gastrointestinal manifestations, including megacolon during infancy, for which multiple surgical interventions may be required. These procedures carry a risk of adhesion formation, which presents a challenge if further abdominal surgery is required later in life for pheochromocytoma. Retroperitoneoscopic adrenalectomy provides a safe approach in such patients, as the need to perform risky adhesiolysis and mobilize adjacent organs is obviated. Presentation: Saturday, June 17, 2023

Prevalence of Retinal Venous Malformations in Patients With Cerebral Cavernous or Arteriovenous Malformations.

Cerebral vascular malformations (CVMs) may result in hemorrhage, seizure, neurologic dysfunction, and death. CVMs include capillary telangiectasias, venous malformations, cavernous malformations, and arteriovenous malformations. Cavernous and arteriovenous malformations carry the highest risk of complications. Retinal venous malformations (RVMs) have been proposed as an associated finding. Our objective was to determine the prevalence of RVMs in patients with high-risk CVMs. We retrospectively reviewed patients diagnosed with cerebral cavernous or arteriovenous malformations (high-risk CVMs) who were evaluated by the ophthalmology service at Northwestern University between 2017 and 2020. Patients were stratified into 3 cohorts based on level of certainty: dilated funduscopic examination (DFE), DFE with any form of ocular imaging, and DFE with complete imaging of the macula. We recorded ophthalmic examination abnormalities, ocular imaging findings, and major CVM complications. We evaluated 156 patients with high-risk CVMs who had undergone DFE. Ocular imaging of any type was performed in 56 patients, of whom 46 had complete imaging of the macula. Zero RVMs were identified in any cohort (95% confidence interval: 0%-1.9% for the entire cohort, 0%-5.4% for any ocular imaging cohort, and 0%-6.5% for the complete macular imaging cohort). Cerebral hemorrhage or seizure occurred in 15%-33% of patients. Associated visual field defects or cranial nerve palsies were found in 14%-20% of patients. Zero RVMs were identified in patients with high-risk CVMs. However, neuro-ophthalmic findings were common. Therefore, we recommend neuroimaging for patients with RVMs and neuro-ophthalmic signs or symptoms. In asymptomatic patients with RVMs, a potential algorithm for neuroimaging is proposed.

High-fat diet reveals the impact of Sar1b defects on lipid and lipoprotein profile and cholesterol metabolism

Biallelic pathogenic variants of the Sar1b gene cause chylomicron retention disease (CRD) whose central phenotype is the inability to secrete chylomicrons. Patients with CRD experience numerous clinical symptoms such as gastrointestinal, hepatic, neuromuscular, ophthalmic, and cardiological abnormalities. Recently, the production of mice expressing either a targeted deletion or mutation of Sar1b recapitulated biochemical and gastrointestinal defects associated with CRD. The present study was conducted to better understand little-known aspects of Sar1b mutations, including mouse embryonic development, lipid profile, and lipoprotein composition in response to high-fat diet, gut and liver cholesterol metabolism, sex-specific effects, and genotype-phenotype differences. Sar1b deletion and mutation produce a lethal phenotype in homozygous mice, which display intestinal lipid accumulation without any gross morphological abnormalities. On high-fat diet, mutant mice exhibit more marked abnormalities in body composition, adipose tissue and liver weight, plasma cholesterol, non-HDL cholesterol and polyunsaturated fatty acids than those on the regular Chow diet. Divergences were also noted in lipoprotein lipid composition, lipid ratios (serving as indices of particle size) and lipoprotein-apolipoprotein distribution. Sar1b defects significantly reduce gut cholesterol accumulation while altering key players in cholesterol metabolism. Noteworthy, variations were observed between males and females, and between Sar1b deletion and mutation phenotypes. Overall, mutant animal findings reveal the importance of Sar1b in several biochemical, metabolic and developmental processes.

Age-associated changes in electroretinography measures in companion dogs.

To determine the association between age and retinal full-field electroretinographic (ERG) measures in companion (pet) dogs, an important translational model species for human neurologic aging. Healthy adult dogs with no significant ophthalmic abnormalities were included. Unilateral full-field light- and dark-adapted electroretinography was performed using a handheld device, with mydriasis and topical anaesthesia. Partial least squares effect screening analysis was performed to determine the effect of age, sex, body weight and use of anxiolytic medication on log-transformed ERG peak times and amplitudes; age and anxiolytic usage had significant effects on multiple ERG outcomes. Mixed model analysis was performed on data from dogs not receiving anxiolytic medications. In dogs not receiving anxiolytics, median age was 118months (interquartile range 72-140months, n = 77, 44 purebred, 33 mixed breed dogs). Age was significantly associated with prolonged peak times of a-waves (dark-adapted 3 and 10cds/m2 flash p < 0.0001) and b-waves (cone flicker p = 0.03, dark-adapted 0.01cds/m2 flash p = 0.001). Age was also significantly associated with reduced amplitudes of a-waves (dark-adapted 3cds/m2 flash p < 0.0001, 10cds/m2 flash p = 0.005) and b-waves (light-adapted 3cds/m2 flash p < 0.0001, dark-adapted 0.01cds/m2 flash p = 0.0004, 3cds/m2 flash p < 0.0001, 10cds/m2 flash p = 0.007) and flicker (light-adapted 30Hz 3cds/m2 p = 0.0004). Within the Golden Retriever breed, these trends were matched in a cross-sectional analysis of 6 individuals that received no anxiolytic medication. Aged companion dogs have slower and reduced amplitude responses in both rod- and cone-mediated ERG. Consideration of anxiolytic medication use should be made when conducting ERG studies in dogs.

Benign Episodic Mydriasis as a Cause of Isolated Anisocoria.

A 22-year-old female presented to the emergency department with a dilated right pupil and mild blurry vision. Physical examination revealed a dilated, sluggishly reactive right pupil, without other ophthalmic or neurologic abnormalities. Neuroimaging was normal. The patient was diagnosed with unilateral benign episodic mydriasis (BEM). BEM is a rare cause of acute anisocoria with an underlying pathophysiology that is not well understood. The condition has a female predominance and is associated with a personal or family history of migraine headaches. It is a harmless entity that resolves without intervention and results in no known permanent damage to the eye or visual system. Benign episodic mydriasis is a diagnosis of exclusion that may be considered only after the life- and eyesight-threatening causes of anisocoria.

Ocular manifestations of children with atopic dermatitis in Saudi Arabia.

To examine the incidence of ocular abnormalities in children with atopic dermatitis (AD) in Saudi Arabia and its association with the severity of AD. This is a cross-sectional study on 50 children with AD who were between 5 and 16 years of age. The severity of AD was evaluated using the SCORing Atopic Dermatitis (SCORAD) index. All the children underwent slit lamp exams, visual acuity assessment, intraocular pressure measurement, and corneal topography. The children were considered to have an ophthalmic abnormality if one or more of the following signs were present: glaucoma, keratoconus suspicion, in addition to lid, conjunctival, corneal, lenticular, or retinal abnormalities. Based on the SCORAD severity index, 14% of children had mild AD (7/50), 38% had moderate AD (19/50), and nearly half had severe AD. More than half the children exhibited facial involvement, and half had peri-orbital signs. The mean SCORAD index was 35.75. The mean age was 10.48±3.6y, and the cohort showed a slight male predominance (54% males). Both eyes of the 50 children in the cohort were studied. Based on the ocular examinations, 92% of the patients showed ocular abnormalities: lid abnormalities (27/50) followed by keratitis (22/50). Four patients had moderate risk for keratoconus in one eye and eight patients were suspected to have keratoconus. However, SCORAD severity index was not associated with age, sex, or the number or presence of ophthalmic abnormalities. This is the first study in Saudi Arabia to evaluate the prevalence of ocular manifestations in children with AD. The results indicate that the majority of children with AD have ocular abnormalities that mainly include lid abnormalities. Based on these findings, larger scale studies are needed to affirm whether regular screening for ophthalmic abnormalities would be beneficial for children with AD in terms of early intervention and prevention of sight-threatening complications.

Evaluation of auditory and ophthalmic abnormalities in adult vitiligo patients: a case-control study

Background: The association of vitiligo with autoimmune disorders and affliction of auditory and ocular melanocytes in vitiligo patients suggests that a systemic autoimmune process directed against pigment cells might play a role in the development of the disease. Aim: The aim of our study was to find out associated ocular and auditory abnormalities in patients with vitiligo and correlate the same with the extent and duration of vitiligo. Methods:An analytical, case-control study was undertaken in 100 newly diagnosed cases of vitiligo (age &gt;18 years) and an equal number of age- and sex-matched non-vitiligo controls. A detailed cutaneous examination was carried out taking note of the type, duration, and extent (vitiligo extent tensity index) of vitiligo. All relevant baseline laboratory investigations, complete ophthalmic examination, and a thorough auditory evaluation using pure tone audiometry were carried out in all cases and controls. Statistical analysis: Statistical analysis was done using the chi-square test and Fisher exact probability test and a P &lt; 0.05 was considered statistically significant. Results:Fifteen cases (15%) and five controls (5%) had ocular abnormalities (P = 0.018). No statistically significant correlation between ocular abnormalities and the duration, extent, or anatomic location of the disease in vitiligo cases was observed (P &gt; 0.05). Sensorineural hypoacusis was found in 22% of vitiligo cases and 2% of non-vitiligo controls (P &lt; 0.001). A statistically significant association was found between sensorineural hearing loss and the duration of the disease in vitiligo cases (P = 0.019). No association between age of onset of vitiligo and auditory alterations was observed. Hypothyroidism was seen in 16% cases versus 3% in controls (Pp = 0.004). Diabetes mellitus was present in 14% versus 3% in controls (P = 0.012). Conclusion: The results of our study add more evidence to the auditory and visual involvement and the presence of systemic associations in patients suffering from vitiligo.