Do infants with isolated congenital sixth nerve palsy require comprehensive work-up? A retrospective cohort and review of the literature.

PURPOSE: The purpose of this study is to describe a case series of infants with isolated congenital sixth nerve palsy (ICSNP) and suggest a management algorithm based on our experience and a review of the literature. METHODS: A retrospective cohort design was used. The clinical database of a single tertiary medical center was reviewed to identify all patients diagnosed with ICSNP from January 2020 to November 2022. Data were collected as follows: demographic parameters, age at initial presentation, presenting symptoms and signs, findings on ophthalmic and neurologic examinations, findings on follow-up, and outcome. RESULTS: Six patients were included. All were born at term. The average gestational weight was 3675.7 ± 262.7 g. Three mothers had gestational diabetes. Five deliveries necessitated labor induction either by oxytocin (n = 4) or by membrane stripping followed by oxytocin (n = 1). One had also gone a forceps assisted delivery. Symptoms were noticed in all newborns by their parents within the first week of life. Ophthalmological and neurological examinations were otherwise unremarkable apart of one patient with a head turn to the side of the involved eye. Four patients underwent brain imaging that were unremarkable. All abduction deficits resolved by 1 to 3 months of age. Follow up examinations were unremarkable (mean follow up 14.3 ± 5.0 months, range 4-23). CONCLUSIONS: This case series, together with previous reports, support ICSNP's benign nature. We suggest an initial basic work-up that solely includes ophthalmological and neurological examinations which will be elaborated in case of any additional pathologic findings or if ICSNP does not fully resolve by 3 months.

Elevated intraocular pressure in children with acute lymphoblastic leukaemia: A prospective study.

Most childhood acute lymphoblastic leukaemia (ALL) protocols include high-dose steroid therapy. However, the known potential of high-dose steroids to significantly elevate intraocular pressure (IOP) and lead to glaucomatous optic neuropathy has not been intensively investigated in children with ALL. Moreover, as children with ALL do not routinely undergo IOP measurements, the need for IOP monitoring and therapy is unknown. We prospectively measured IOP in 90 children with newly diagnosed ALL attending a tertiary paediatric haematology/oncology centre, at diagnosis and at the middle and end of induction therapy. Ocular hypertension (IOP > 21 mm Hg) at any time point was documented in 64 children (71%), and the prevalence increased during induction. Thirty-six children (40%) had elevated IOP at ALL diagnosis before therapy initiation, and stratification to non-standard ALL was a risk factor. IOP reduction therapy was administered to 13 children (14%); none required surgery. Values normalised in all cases. On multivariate logistic regression analysis, dexamethasone therapy was a significant risk factor for ocular hypertension. High body mass index was an additional risk factor in children with elevated IOP at ALL diagnosis. Routine evaluation of IOP during steroid therapy is very important in children with ALL to ensure early intervention which may prevent permanent ocular damage.

A modified pediatric ocular trauma score for predicting visual outcome post open globe injury.

PURPOSE: To construct a new pediatric ocular trauma score for predicting visual outcome after open globe injuries (OGI) and to compare it to the ocular trauma score (OTS) and pediatric ocular trauma score (POTS). METHODS: This is a retrospective chart review study. For each case, the following data were collected: demographics, mechanism of the injury, initial ophthalmologic findings, presented and last follow-up visual acuity (VA), ocular treatments, and final ocular findings. We then analyzed the risk factors for the poor visual outcome (VA ≤ 20/200), and a modified pediatric ocular trauma score (MPOTS) was constructed accordingly and compared to the OTS and POTS for predicting poor outcome. Finally, a different cohort of pediatric OGIs was used for score validation. RESULTS: Forty-five cases were included, significant predicting factors for poor visual outcome were initial VA ≤ 20/200, zone 2-3 locations of injury, presence of retinal detachment, vitreous hemorrhage, hyphema, and iris prolapse at initial presentation. The calculated Spearman correlation coefficients between each system score and poor visual outcome were OTS 0.56, POTS 0.57, and MPOTS 0.64 (P < 0.001 for all). A total of 71 new cases were used as validation cohort, and calculated Spearman correlation coefficients between each system score and poor visual outcome were: OTS 0.50, POTS 0.51, and MPOTS 0.53 (P < 0.001 for all). CONCLUSIONS: We suggest a new scoring system for predicting poor final visual outcomes after OGI's in children, which is simpler and more clinically suitable for this study population. It was found to be a better predictor of visual outcome in this scenario compared with existing scoring systems.

Trametinib for orbital plexiform neurofibromas in young children with neurofibromatosis type 1.

INTRODUCTION: Plexiform neurofibromas (PNF) in neurofibromatosis type 1 (NF1) are usually diagnosed in childhood and can grow rapidly during this period. In 10% of patients, PNF involve the orbital-periorbital area and may cause visual problems including glaucoma, visual loss from amblyopia (deprivational, strabismic, or refractive), optic nerve compression, or keratopathy. Ptosis, proptosis, and facial disfigurement lead to social problems and decreased self-esteem. Complete surgical removal involves significant risks and mutilation, and regrowth after debulking is not uncommon. Inhibitors of the RAS/MAPK pathway have recently been investigated for their activity in PNF. We administered the oral MEK inhibitor trametinib to five young children with NF1 and PNF of the orbital area, with visual compromise and progressive tumor growth; and followed them clinically and by volumetric MRI. METHODS: Treatment was initiated at a mean age of 26.8 months (SD ± 12.8) and continued for a median 28 months (range 16-51). Doses were 0.025 mg/kg/day for children aged > 6 years and 0.032 mg/kg/day for those aged < 6 years. RESULTS: Volumetric MRI measurements showed a reduction of 2.9-33% at 1 year after treatment initiation, with maximal reductions of 44% and 49% in two patients, at 44 and 36 months, respectively. No change in visual function was recorded during treatment. One child reported decreased orbital pain after 2 weeks; and another, with involvement of the masseters, had increased ability to chew food. Toxicities were mostly to skin and nails, grades 1-2. CONCLUSIONS: Trametinib can decrease tumor size in some young children with orbital PNF and may prevent progressive disfigurement.

Ocular Trauma Caused by Confetti Cannons.

BACKGROUND: Eye trauma is an unfortunate and often preventable cause of vision loss. Confetti cannons are common causes of injury. Awareness of ocular hazards of confetti cannons remains low because of limited reports describing ophthalmic injuries following their use. OBJECTIVES: To describe outcomes of ocular trauma caused by confetti cannons and to increase recognition of their ocular risks. METHODS: A retrospective analysis was conducted of eye injuries caused by confetti cannons presenting to a single medical center between 2016 and 2020. Data collected included age, gender, eye injured, ocular damage, visual outcome, and details of surgeries performed. RESULTS: Overall, six consecutive patients (2 males, mean age 19.5 ± 9.74 years) were identified and studied. In all patients only one eye was injured (3 right eyes) during a private celebration, most commonly (n=5) to a bystander while in the vicinity of a cannon operated by someone else. Most common eye injuries included corneal erosion (n=4), traumatic hyphema (n=4), and retinal edema (n=3). Mean initial logMAR visual acuity in the injured eye was 0.73 ± 0.18, improving to 0.25 ± 0.16 at the final visit (P = 0.125). Two patients underwent eye surgery due to their trauma: one to repair globe penetration and another to undergo intravitreal injection of tissue plasminogen activator and C3F8 for submacular hemorrhage, followed 8 months later by intravitreal bevacizumab injection for choroidal neovascularization. CONCLUSIONS: Confetti cannons pose hazards that can cause severe ocular trauma resulting in permanent vision loss. Increasing awareness of device hazards is necessary to prevent eye injuries.

Incidence of non-glaucomatous ocular disease in patients with asymmetric optic disc cupping.

PURPOSE: To evaluate the incidence of non-glaucomatous ocular disease in patients with asymmetric optic disc cupping. METHODS: A retrospective case series, including consecutive patients with cup-to-disc ratio (CDR) asymmetry greater than 0.2. All patients underwent a complete neuro-ophthalmological examination, automated perimetry with the Humphrey 24-2 visual fields program. Retinal nerve fibre layer thickness was measured by optical coherence tomography (OCT). The results of neuroimaging, macular OCT and blood tests were recorded as well. Patients were assigned a diagnosis of glaucomatous optic neuropathy (GON) or non-glaucomatous disease (NGD). The main outcome measure was the rate of non-glaucomatous ocular disease. RESULTS: A total of 120 (67 males) patients with a mean age of 71.1 ± 12.5 years met the inclusion criteria and were included in this study. The mean asymmetry in CDR between the eyes was 0.3 ± 0.13 (range, 0.2-0.9). Twenty patients (16.6%) had a visual field defect not typical for glaucoma and positive relative afferent pupillary defect was found in 24 patients (20%). Six patients were found to have newly diagnosed non-glaucomatous ocular disease: maculopathy in three patients, retinopathy in one patient and traumatic optic neuropathy in two patients. Patients with NGD were significantly younger than the patients with GON (59.8 ± 23.3 vs. 71.3 ± 11.5 years, P = 0.001). Optic disc pallor was found in 4/93 patients with glaucoma compared to 3/6 with newly diagnosed non-glaucomatous disease (4.7% vs. 50.0%, P = 0.03). CONCLUSIONS: Asymmetric optic disc cupping can be associated with non-glaucomatous disease and may warrant neuro-ophthalmological evaluation, especially in younger patients or those with optic disc pallor.

Comparison of keratometry data using handheld and table-mounted instruments in healthy adults.

PURPOSE: To compare keratometry data between the handheld Retinomax K-plus 3 and the table-mounted IOLMaster 700. METHODS: Healthy adult volunteers were prospectively recruited to the study. All participants underwent 3 consecutive keratometry measurements using the Retinomax K-plus 3 and a single biometry assessment using the IOLMaster 700. Differences between the Retinomax K-plus 3 and the IOLMaster 700 were assessed using Wilcoxon test for paired samples, Spearman correlation, Bland-Altman and mountain plots. RESULT: Twenty-eight healthy subjects with a median age of 37 years (interquartile range (IQR) 28-44 years) were included in the study. The median mean keratometry (mean K) reading was higher using the Retinomax K-plus 3 (44.04D; IQR 42.96-45.61D) compared to the IOLMaster 700 (43.78D; IQR 43.22-44.90D, p < 0.01), with a mean difference of 0.18D (95% confidence interval (CI) 0.11-0.23D). Mean K readings were highly correlated between the 2 devices (r = 0.995, p < 0.01). Bland-Altman plots showed 95% limits of agreement between -0.14D and 0.49D. Frequency histogram of mean K reading differences between the Retinomax K-plus 3 and the IOLMaster 700 showed that 56% of cases were between ± 0.2D, 93% of cases were between ± 0.4D and all cases were between ± 0.5D. Mean corneal astigmatism measurement was higher using the Retinomax K-plus 3 (1.01 ± 0.40D) compared to the IOLMaster 700 (0.77 ± 0.36D), with a mean difference of 0.23 ± 0.37D (p < 0.01) between the devices. CONCLUSIONS: A good agreement exists between the Retinomax K-plus 3 and the IOLMaster 700 regarding keratometry readings. This enables cataract surgeons to safely use the Retinomax K-plus 3 device when indicated.

The great mimicker: Phenotypic overlap between constitutional mismatch repair deficiency and Tuberous Sclerosis complex.

Constitutional mismatch repair deficiency is a rare cancer predisposition syndrome caused by biallelic mutations in one of the four mismatch repair genes. Patients are predisposed to various tumors including hematological malignancies, brain tumors and colorectal carcinomas. Phenotypic overlap with Neurofibromatosis-1 is well known, with most patients presenting with café-au-lait macules. Other common features include axillary and/or inguinal freckling and intracranial MRI foci of high T2W/FLAIR signal intensity similar to the typical FASI seen in Neurofibromatosis-1. In this cohort of eight patients with constitutional mismatch repair deficiency we describe overlapping phenotypical features with Tuberous Sclerosis complex. In addition to "ash-leaf like" hypomelanotic macules (five patients), we detected intracranial tuber-like lesions (three patients), renal cysts (three patients) and renal angiomyolipomas (two patients). All our patients also had Neurofibromatosis-1 like features, mainly café-au-lait macules. This study suggests that features of Tuberous sclerosis especially when overlapping with those of Neurofibromatosis 1 or malignancies atypical for these syndromes should raise the possibility of constitutional mismatch repair deficiency. Correct diagnosis is essential for appropriate genetic counseling and pre-emptive cancer surveillance.

Demographic, clinical features, and outcomes of pediatric non-penetrating ocular foreign bodies.

PURPOSE: Data regarding ocular foreign body (FB) in the pediatric population is sparse. The purpose of this study is to describe the demographic features and the outcomes of pediatric non-penetrating ocular FB. METHODS: The charts of all children with non-penetrating ocular FB who presented at a tertiary medical center between 2011 and 2018 were retrospectively reviewed. Data analyzed included demographics, ocular FB site, the need for general anesthesia, or sedation for FB removal and clinical outcomes. RESULTS: Three hundred and fifty-two children (58.8% boys) with a mean age of 7.7 ± 3.7 years were included. Two hundred and fifty-one (71.3%) children presented on the same day of injury. Patients with developmental delay presented more often with restlessness than patients without developmental delay (p < 0.0001). One hundred and forty-six (41.5%) of FBs were found on the conjunctiva, 128 (36.4%) under the eyelid, and 62 (17.6%) on the cornea. In 19 (4.5%) cases, general anesthesia or sedation was required for FB removal. A multivariate analysis identified young age (OR 0.976, 95% CI 0.961-0.992, p = 0.003), corneal FB (OR 50.84, 95% CI 10.08-256.37, p < 0.0001), and developmental delay (OR 18.56, 95% CI 1.22-283.45, p = 0.036), as significant predictors for the need of general anesthesia or sedation. Among patients with corneal FB, in two (3.2%) cases, the corneal FB was complicated by infectious keratitis, resulting in mild corneal scar. CONCLUSION: The rate of general anesthesia for non-penetrating ocular FB removal in children is low. Children presenting with non-penetrating ocular FB have good prognosis without long-term complications.

Vitamin A levels are comparable between children with newly diagnosed coeliac disease and non-coeliac controls.

AIM: Nutritional deficiencies associated with coeliac disease include iron, folic acid and fat-soluble vitamins. This study compared the prevalence and degree of vitamin A deficiency among newly diagnosed coeliac disease patients to controls in a developed country. METHODS: This prospective cohort study included all children evaluated by gastroscopy at Dana-Dwek Children's Hospital, Israel, between September 2014 and February 2017. Vitamin A, haemoglobin, C-reactive protein (CRP), ferritin, tissue transglutaminase, immunoglobulin A and vitamin D levels were measured. RESULTS: Of the 113 children (69% females), 47 were diagnosed with coeliac disease whereas the others were the controls (mean age of 8.2 ± 3.8 years and 12.4 ± 3.5 years, respectively). There was no group difference in vitamin A, vitamin D or CRP levels. Among coeliac children, two had true vitamin A deficiency compared with three controls, while 18 coeliac children had subclinical vitamin A deficiency compared with 25 controls (p > 0.05). CONCLUSION: Paediatric coeliac disease was not associated with increased prevalence of vitamin A deficiency. Children evaluated for gastrointestinal complaints in a developed country were found to have an unexpectedly high prevalence of subclinical vitamin A deficiency. Further prevalence and causality assessments of vitamin A deficiency in developed countries are needed.

Familial Occurrence of Idiopathic Intracranial Hypertension.

BACKGROUND: Idiopathic intracranial hypertension (IIH) is a disorder of unknown etiology. Its occurrence in the general population is 1/100,000, and 20/100,000 among overweight women of childbearing age. Familial occurrence is reportedly uncommon and not well-characterized. OBJECTIVES: To describe a familial association with IIH. METHODS: We conducted a retrospective chart review of all familial cases of IIH examined in the neuro-ophthalmology clinic of our medical center between January 2006 and June 2013. RESULTS: Of a total of 520 patients with IIH, 15 had other family members with IIH (from seven different families). The family relation was a mother and daughter in two families, a brother and sister in four families, and an aunt and two first-degree cousins in the seventh family. Symptoms, course of disease, and risk factors were similar among the relatives of all seven families, except for the age at diagnosis, which was different in one family. All of the adult patients of six families were obese (body mass index 25-35 kg/m2), and all of the members of the other family were morbidly obese. There was no association between other systemic risk factors and IIH. CONCLUSIONS: IIH occurrence within a family is more common than previously believed, and its incidence in families is more common than in the general population. The clinical course appears to be similar in family members. Our findings suggest a genetic predisposition. Further investigation of familial cases may yield useful information on the pathogenesis and genetic nature of this condition.

Reduced Suprathreshold Odor Identification in Patients with Pseudotumor Cerebri: A Non-Randomized Prospective Study.

BACKGROUND: Recent evidence suggests that olfaction is impaired in patients with pseudotumor cerebri (PTC). OBJECTIVES: To measure suprathreshold olfactory function by using the University of Pennsylvania Smell Identification Test (UPSIT), assessing its usefulness for routine clinical use. METHODS: Forty PTC patients underwent USPIT olfactory testing. RESULTS: Twenty-nine out of 40 (73%) PTC patients (36 women, 4 men; mean age 34 years) had reduced suprathreshold smell sensation according to UPSIT scores: 19 (47%) had mild microsmia, 9 (23%) had moderate microsmia, and one (3%) was classified as having severe microsmia. The mean UPSIT score of all patients was 32.4 (95% confidence interval 31.4-33.4). Multivariate regression analysis found that UPSIT scores were not related to disease activity, disease duration, initial intracranial pressure (ICP), or visual function. CONCLUSIONS: Many PTC patients have reduced suprathreshold olfactory dysfunction that can be discovered by UPSIT, a rapidly administered smell test, which is suitable for clinical office use.

Visual outcome following bilateral non-arteritic anterior ischemic optic neuropathy: a systematic review and meta-analysis.

BACKGROUND: Many patients who suffer unilateral non-arteritic anterior ischemic optic neuropathy (NAION) will eventually develop the same condition in their other eye, worrying them about losing vision in both eyes. The purpose of this meta-analysis is to determine whether it is possible to predict the visual outcome of the consecutive NAION event based on initial presentation and to compare mean visual loss of firstly versus secondly affected eyes. METHODS: A systematic review and meta-analysis of studies published between January 1st 1966 and May 31st 2016 reporting on visual acuity and/or visual field loss of both affected eyes, measured either at presentation or follow-up following bilateral NAION. RESULTS: Ten studies were included in the meta- analysis of visual acuity, including 9 retrospective reports and one randomized clinical trial, and five retrospective studies were included in visual field meta-analysis. A significant correlation exists for visual acuity (R = 0.387, P < 0.001) in both eyes of the same patient following bilateral NAION, and also for visual field loss (R = 0.445, P < 0.001) in the two eyes. The calculated coefficient of determination (R2) of 0.149 for visual acuity, and 0.198 for visual field loss indicates that for any given individual suffering from unilateral NAION only 15% of visual acuity and 20% of visual field loss in the secondly affected eye can be explained by these outcomes in the first eye. In addition, there was no difference in mean visual outcome of the first versus second NAION events (standardized mean differences of visual acuity 0.008, P = 0.890; and visual field loss, -0.019, P = 0.819). CONCLUSION: Even though a weak connection exists between visual outcome in both eyes following bilateral NAION it is still impossible to predict with certainty the visual outcome of a sequential contralateral NAION event based on the severity of visual loss in the first affected eye. Measures often taken after the first event are ineffective in improving the visual outcome of a second event should it occur.

Comparison of Ishihara Booklet with Color Vision Smartphone Applications.

PURPOSE: To compare the standard Ishihara booklet with color-vision-testing smartphone applications. METHODS: A prospective observational diagnostic study on 42 normal trichromats and 38 color-deficient subjects. Patients were presented with three color vision tests in random order: an Ishihara test booklet and two color-vision-testing smartphone applications: Eye2Phone and the Color Vision Test application (CVT app). Sensitivity and specificity of the electronic tests was compared with Ishihara results, and in each one of these applications every plate was individually analyzed for success/failure rate. RESULTS: Average age was 42.7 ± 12.9 years. There were 57 males (71.2%). Sensitivity and specificity of each test was 100% (38/38) and 95.2% (40/42) for the Eye2Phone, and 100% (38/38) and 54.8% (23/42) for the CVT app. There was no significant difference between the Ishihara booklet and the Eye2Phone (p = 0.500), with a high kappa measure of agreement (0.950, p < 0.001). The CVT app was significantly different than both other tests (p < 0.001) with a low kappa measure of agreement (0.535 with the Ishihara and 0.575 with the Eye2Phone). Of the 21 tested plates, color-deficient subjects failed 11.8 ± 3.1 plates in the Ishihara booklet and 14.1 ± 2.1 plates in the Eye2Phone (p < 0.001). Significant plate-specific differences for the color-deficient group were found in plate numbers 3, 6, 7, 8, 9, 15, and 16. Both tests were poorly able to give an indication of the specific dichromatism type. The Ishihara booklet was rated more comfortable and clearer than the Eye2Phone in color-deficient subjects. The CVT app was rated lowest for comfort and clarity in both groups. CONCLUSIONS: Smartphone applications testing for color vision deficiency are readily available; however, users of these apps should be aware that some may have different sensitivity for detection of color vision deficiency compared to Ishihara booklet, limiting their usefulness for clinical use. Therefore, further validation of these applications is required.

Should patients with ocular genetic disorders have genetic testing?

PURPOSE OF REVIEW: To discuss the risks, benefits and value of genetic testing for ocular genetic disease. RECENT FINDINGS: Testing for ocular genetics diseases is becoming more available and successful gene therapy is being reported. Clinicians must prepare for this trend by considering diagnostic genetic testing for their patients. SUMMARY: As advances continually occur in genetic testing for ocular genetic disorders, clinicians must develop an understanding of the potential risks and benefits for their patients.

Uncorrected amteropia among children hospitalized for headache evaluation: a clinical descriptive study.

BACKGROUND: Headache is a common complaint in children occasionally requiring hospital admission. The purposes of the present study were to analyze the prevalence of uncorrected ametropia in children with headache admitted to the hospital, and evaluate the importance of refraction assessment as part of their evaluation. METHODS: A retrospective review of children admitted to the Tel Aviv Medical Center for headache evaluation from December 2008 to March 2013, in whom the only abnormality found was an uncorrected refractive error. RESULTS: During the study period 917 children with headache were hospitalized for evaluation and 16 (1.7%) of them (9 boys, mean age 12 years, range 8-18 years) were found to have an uncorrected ametropia. Average headache duration was 4 months (range, 1 week to 1 year) and mean follow-up was 15 months (range, 1 month to 3 years). Twelve (75%) children had brain imaging and 4 children (25%) had a lumbar puncture before their refractive abnormality was identified. Anisometropia and myopia were the most common refractive errors encountered (n = 10 each), followed by hyperopia (n = 6) and astigmatism (n = 3). Despite having uncorrected refractive errors most children (n = 10) did not complain of any visual difficulty. All children were given proper refractive correction and 14 of them reported complete headache resolution on re-examination one month later. CONCLUSIONS: Uncorrected ametropia is a possible cause of headache among hospitalized children. Therefore, complete ophthalmic evaluation, which includes proper refraction assessment, is important as it can identify a treatable headache etiology. Children without visual difficulty should be equally evaluated, as many children with headache and uncorrected amteropia do not have vision complaints.

The obesity pattern of idiopathic intracranial hypertension in men.

BACKGROUND: Idiopathic intracranial hypertension (IIH), also known as pseudotumor cerebri, is a disorder of unknown etiology, predominantly affecting obese women of childbearing age. IIH is uncommon in men, with a reported female-to-male ratio of 8:1. The pathogenesis of IIH is poorly understood. Several mechanisms have been suggested, but no one mechanism has been able to account for all manifestations of the disease. This research aims to characterize the obesity phenotype(s) of men with IIH in order to find potential inducers for this disease. METHODS: This is a cross-sectional study based on subjects' medical records. It compared anthropometric parameters between 22 men with IIH, 60 healthy men, and 44 females with IIH. One-way analysis with age and body mass index included as covariates was applied for the assessment of the difference in fat distribution among the three groups. RESULTS: No significant differences were observed between the male IIH cohort and healthy males for age, BMI, and waist measurements, whereas hip circumference was significantly larger in the IIH cohort (114 ± 13 vs. 104 ± 16 cm; respectively, p < 0.001). Consequently, waist-to-hip ratio (WHR) was significantly lower in the male IIH cohort (0.88 ± 0.08 vs. 0.95 ± 0.12; p < 0.001). While no significant differences were observed for age and hip measurements between male IIH and female IIH cohorts, waist circumference and waist-to-hip ratio (WHR) were significantly larger in the male cohort (102 ± 19 cm vs. 95 ± 13 cm, p < 0.001; 0.88 ± 0.08 vs. 0.78 ± 0.06, p < 0.001, respectively). All these results maintained after adjustment for age and BMI. CONCLUSIONS: This is the first report of body fat distribution patterns in men with IIH. Whereas male IIH have larger central fat deposition than female IIH patients, abdominal fatness is less accentuated in IIH men compared to normal obese men. The later observation is in agreement with similar results regarding female IIH patients. We believe that these findings justify further investigation into the involvement of various fat depots in the pathogenesis of IIH in men and women alike.

The role of neuroimaging in the evaluation process of children with isolated sixth nerve palsy.

PURPOSE: The purpose of this study was to assess the role of neuroimaging in identifying the etiology of pediatric isolated sixth nerve palsy (SNP). METHODS: A retrospective cohort study was conducted of all patients younger than 18 years of age with an isolated SNP seen at our medical center between 2003 and 2011. RESULTS: Sixteen children (nine girls; mean age, 4.5 years) with isolated SNP were identified during an 8-year period. Only cases with normal optic disk appearance and otherwise normal neurological examination were included into this study. Thus, 12 other children with SNP were excluded: ten children with papilledema, one child who developed a SNP following a resection of a brain tumor, and one with hydrocephalus and a shunt malfunction. All cases of isolated SNP were unilateral (ten left eyes). The most common cause for the SNP in these children was a tumor that was found in five patients. Other etiologies encountered in decreasing frequencies were: benign recurrent SNP (in four children), postviral or vaccination (in three children), and one case each of post trauma, Chiari malformation, congenital, and undetermined. Children who were found to have a tumor (9.9 ± 5.5 years) were significantly older (P = 0.019) than children who did not have a tumor (2.1 ± 1.8 years). CONCLUSIONS: Isolated SNP can be the presentation of a brain tumor in children, and therefore, early neuroimaging of the brain is recommended, especially in older children.

Spontaneous cerebrospinal fluid otorrhea and rhinorrhea in idiopathic intracranial hypertension patients.

BACKGROUND: Spontaneous cerebrospinal fluid (CSF) leakage may occur in patients with normal or increased intracranial pressure (ICP). We describe herein spontaneous CSF leakage as a result of chronic increased ICP in 4 patients with idiopathic intracranial hypertension (IIH). Although rhinorrhea previously has been described in IIH patients, to our knowledge this is the first report of otorrhea in these patients. METHODS: Four patients with spontaneous CSF leakage were examined between 2001 and 2011; 3 presented with rhinorrhea and 1 with otorrhea. Clinical settings and manifestations were analyzed. RESULTS: All patients were found to have IIH. Three had been diagnosed with IIH several years earlier and had been noncompliant with their medical treatment, whereas in 1 patient, CSF rhinorrhea was the presenting symptom of IIH. CONCLUSION: CSF leak is a rare complication in IIH patients. We have shown that rhinorrhea can be the presenting sign in these patients and that rhinorrhea and otorrhea can be a late sign of the disease.

[Non-arteritic anterior ischemic optic neuropathy associated with erectile dysfunction medications].

Erectile dysfunction medications such as sildenafil citrate (Viagra) or tadalafil (Cialis) are commonly prescribed worldwide. They are selective phosphodiesterase-5 inhibitor and partial phosphodiesterase-6 inhibitors causing smooth muscle relaxation in the corpus cavernosum, allowing penile vasodilatation and erection in response to sexual stimuli. Over the years, there have been an increasing number of case reports concerning patients who developed ischemic optic neuropathy soon after the ingestion of these drugs. Although a cause and effect relationship between usage of the drugs and the development of ischemic optic neuropathy is difficult to prove, it is common nowadays to advise patients, especially those suffering from diabetes, hypertension, and ischemic heart disease, regarding the potential risk of visual loss due to ischemic optic neuropathy and treatment with erectile dysfunction drugs. Patients who were diagnosed with ischemic optic neuropathy soon after the ingestion of these erectile dysfunction drugs should be warned about a similar event in their fellow eye and should be advised regarding drug discontinuation.