Severe cranial neuropathies caused by falls from heights in children.

PURPOSE: Falls from heights are the most common traumatic event associated with emergency department visits in children. This study investigated the incidence and clinical course of cranial neuropathies caused by falls from heights in children. METHODS: The computerized records of a tertiary pediatric medical center were searched for all patients admitted to the emergency department in 2004-2014 with a head injury caused by falling from a height. Those with cranial neuropathies involving optic and eye-motility disturbances were identified, and their clinical, imaging, and outcome data were evaluated. RESULTS: Of the estimated 61,968 patients who presented to the emergency department during the study period because of a fall, 18,758 (30.3 %) had head trauma. Only 12 (seven boys, five girls, average age 6.7 years) had a visual disturbance. Eight were diagnosed with traumatic optic neuropathy, one after a 6-month delay, including two with accompanying cranial nerve (CN) III injuries. Five patients had anisocoria or an abnormal pupillary response to light at presentation, one patient had CN VI paralysis and temporary vision loss, and one patient had an isolated CN III injury diagnosed on follow-up. Visual improvement varied among the patients. CONCLUSION: Cranial neuropathies due to falls from heights are rare in children and are associated with high visual morbidity. Vision or ocular motility impairment, especially monocular vision loss, may be missed during acute intake to the emergency department, and a high index of suspicion is needed. Assessment of the pupillary response to light is essential.

Contralateral lateral rectus muscle recession in patients with Duane retraction syndrome type 3.

AIMS: The aim of this study was to evaluate the motor, sensory, functional, and head posture results of recession of the lateral rectus muscle contralateral to the involved eye in patients with exotropic Duane retraction syndrome (DRS) type 3. METHODS: This was a retrospective, longitudinal, observational study of a consecutive clinical case series. Of the 11 patients with DRS type 3 operated on at a tertiary medical center from 1977 to 2012, 8 underwent recession of the lateral rectus muscle contralateral to the involved eye (with combined Y-splitting of ipsilateral lateral rectus muscle in 3 of them). Full ophthalmic, orthoptic, and neurological examination was performed before and after surgery. Main outcome measures included intragroup changes in motor misalignment, abnormal head turn, ocular upshoot, and stereopsis. RESULTS: Mean patient age was 8.75±3.1 years at surgery. Mean exodeviation for distance was -17.3±3.5 prism diopters (PD) preoperatively and -4.0±6.1 PD postoperatively; corresponding values for near were -23.1±7.2 PD and -5.9±8.7 PD. Motor deviation improved by 77% for distance (P=0.017) and 74.5% for near (P=0.01). In 7/8 patients, the postoperative residual exodeviation (distance and near) was <8.0 PD. There was an 80% improvement in head turn, from 15.3±4° before surgery to 3.1±5.0° after (P=0.01). Stereopsis improved significantly in 6/8 patients. Findings remained stable during follow-up (mean duration 35.9±50.8 months, range 5-132 months). CONCLUSIONS: Contralateral lateral rectus muscle recession appears to be a promising technique for the treatment of moderate unilateral DRS type 3, with patients showing significant motor and functional improvement and a decrease in head turn.

Refractive and structural changes in infantile periocular capillary haemangioma treated with propranolol.

PURPOSE: To evaluate the optical and anatomical effects of oral propranolol treatment for infantile periocular capillary haemangioma. METHODS: All children diagnosed with infantile capillary haemangioma in 2008-2010 at a tertiary paediatric medical centre underwent comprehensive evaluation, including imaging, by a multidisciplinary team followed by oral propranolol treatment. Clinical follow-up was performed regularly until the lesions disappeared. Main outcome measures included changes in anatomical extraocular extension, refractive sphere and cylindrical power, and spherical equivalent in the involved eye before and after treatment and between the two eyes. RESULTS: A total of 30 patients (8 male; mean age at diagnosis, 1.6±2.8 months) participated. The lesions affected the left eye in 53.3% and were located preseptally in 83.3%. Four patients (13.3%) received steroids before propranolol. A treatment dosage of 2 mg/kg per day was started at mean age 5.0±4.5 months, 3.3±4.3 months from disease onset. Side effects occurred in 11 patients and warranted a dose reduction (to 1 mg/kg per day) in 3 and treatment termination in 1. Findings were significant for mean reduction in involved extraocular area (P<0.0001), post-treatment reduction in mean cylindrical power in involved eyes (P=0.02), pre- and post-treatment differences in mean cylindrical power between involved and uninvolved eyes (P=0.02 and P=0.01, respectively), and post-treatment change in absolute values of mean spherical power between involved and uninvolved eyes (P=0.025). CONCLUSIONS: Early diagnosis of infantile periocular capillary haemangioma and prompt treatment with propranolol lead to a significant reduction in the involved ocular area, in astigmatism, and prevent ocular/facial disfiguration/deformation, without rebound. Propranolol is recommended as the preferred treatment compared with other accepted therapies.

Lack of association of the WRN C1367T polymorphism with senile cataract in the Israeli population.

PURPOSE: Werner syndrome is an autosomal recessive disease of premature aging caused by a polymorphic C1367T mutation in the Werner (WRN) gene. Although there are differences between the pathobiology of normal aging and the phenotype of Werner syndrome, the clinical age-related changes are similar. The aim of the study was to investigate the incidence of the C1367T (rs1346044) polymorphism in patients with age-related cataract. METHODS: The study group consisted of 81 patients with senile cataract undergoing cataract extraction surgery. Data on age, sex, and medical history of microvascular disease and cancer were obtained from the medical files. Anterior lens capsule material was collected during surgery. DNA was extracted, amplified by polymerase chain reaction, and screened for the C1367T polymorphism in WRN using restriction enzymes followed by sequencing. RESULTS: There were 33 male and 48 female patients of mean age 74.3+/-9 years. Genotypic frequencies were 67% for TT and 33% for TC. None of the patients had the CC genotype. Ten patients had a history of myocardial infarct, 8 cerebrovascular accident, and 8 various tumors. The distribution of these morbidities was similar in the two genotype groups. CONCLUSIONS: The distribution of the C1367T WRN polymorphism in patients with senile cataract is similar to that in the normal population. Cataract formation in the elderly is not linked to a WRN mutation.

Proinflammatory cytokines in a mouse model of central retinal artery occlusion.

PURPOSE: To analyze cytokines in the retina and serum in an experimental model of central retinal artery occlusion (CRAO) in mice. METHODS: CRAO was induced by laser activation of intravenously injected rose bengal, a photosensitive dye, in 60 C57Bl/6 mice. mRNA and protein levels of macrophage inhibitory protein-2 (MIP-2), interleukin-6 (IL-6), and tumor necrosis factor- alpha (TNF-alpha) were analyzed using real-time polymerase chain reaction, and western blot, respectively. Cytokine levels in serum were measured by ELISA. Analysis was performed at various time intervals from CRAO induction. RESULTS: In the retina, MIP-2 and IL-6 mRNA expression decreased 3 h after induction of CRAO and increased thereafter, peaking at 12-24 h. By 7 days, levels were again mostly undetectable. TNF-alpha mRNA expression increased at 3 h and decreased to control levels at 7 days. At the protein level, all cytokines were present at 3 h, following similar patterns to their respective gene expression thereafter. In serum, MIP-2 and TNF-alpha levels peaked early, and decreased to control levels at 12 h, with a second late rise of TNF-alpha. IL-6 levels increased between 3 and 12 h and decreased at 24 h. CONCLUSIONS: Temporal variations in cytokines were observed following the induction of CRAO, both at the retinal mRNA expression and protein levels. These temporal changes, and the variable effects of the cytokines at the different time intervals, should be taken into account during the formulation of therapeutic strategies.

Intralesional and sub-Tenon's infusion of corticosteroids for treatment of refractory periorbital and orbital capillary haemangioma.

PURPOSE: To evaluate the effect of combined intralesional and sub-Tenon's administration of corticosteroids for the treatment of refractory periocular and orbital capillary haemangioma in a retrospective, non-comparative study. METHODS: Seven infants with resistant periorbital and orbital capillary haemangioma who attended our tertiary centre from 2000 to 2005 were treated with an intralesional injection of a mixture of betamethasone 6 mg/cc and triamcinolone 10 mg/cc, by body weight, together with sub-Tenon's infusion of betamethasone 6 mg/cc and triamcinolone 40 mg/cc of a volume of 1 cc, close to the orbital lesion, in the same session. Visual axis, proptosis, pre- and post-treatment changes in four refractive parameters, and parental satisfaction were evaluated. RESULTS: None of the babies had proptosis or visual axis obstruction after treatment. Mean spherical power decreased by 22.7% (P=0.06). Mean spherical equivalent decreased in 34%, but the difference was not statistically significant (P=0.09). No early or late ocular side effects were observed. Mean intraocular pressure results were normal before and immediately after treatment, and during the follow-up period. Parental satisfaction score during follow-up was 9/10. Mean time to improvement was 96.43+/-58.3 days. CONCLUSIONS: Combined local corticosteroid administration by posterior sub-Tenon's infusion and intralesional injection in babies with extensive capillary haemangioma is associated with a satisfactory anatomical and functional outcome and there were no ophthalmic side effects.

C-reactive protein levels do not correlate with retinal artery occlusion but with atherosclerosis.

PURPOSE: To determine C-reactive protein (CRP) levels in acute retinal artery occlusion (RAO) and their association with atherosclerotic risk factors. METHODS: CRP levels in 16 patients with RAO were compared with levels in 16 age-matched controls at risk of atherosclerosis and 16 young volunteers. Repeated CRP testing was performed 6 years later. RESULTS: Elevated CRP levels (>3 mg/l) and risk factors for atherosclerosis were detected in seven patients in the study group (44%) and nine at-risk controls (56%). On follow-up, CRP levels were reduced in all seven retested patients. Six patients died of vascular events within 5 years, of whom four had high CRP levels during RAO. CONCLUSIONS: CRP level correlates with atherosclerosis but it is not significantly elevated in patients with RAO.

Progressive damage along the optic nerve following induction of crush injury or rodent anterior ischemic optic neuropathy in transgenic mice.

PURPOSE: To characterize the histological changes that occur in response to induction of ischemic or mechanical optic nerve damage in transgenic mice. METHODS: Either optic nerve crush injury or rodent anterior ischemic optic neuropathy (rAION) were induced in the right eye of mice transgenic for the Thy1 gene promoter expressing cyan fluorescent protein (CFP; n=40) and mice transgenic for the cyclic nucleotide phosphodiesterase (CNPase) gene promoter expressing green fluorescent protein (GFP; n=40). The left eye served as a control. The mice were euthanized at different times after injury. Eyes were enucleated, and the brain together with the optic nerves was completely dissected. Cryopreserved sections of both optic nerves were analyzed by fluorescence microscopy. In addition, flat-mounted retinas from the Thy1-CFP mice were analyzed for retinal ganglion cell (RGC) loss. RESULTS: Axonal loss was detected in the right eye of the Thy1-CFP mice, and demyelination was detected in the CNPase-GFP mice. Both processes occurred simultaneously in the two models of injury. The damage proceeded retrogradely and, in the crush-injury group, crossed the chiasm within 4 days. At 21 days after injury, RGC loss measured 70% in the crush-injury group and 25% in the rAION group. CONCLUSIONS: Axonal injury and demyelination along the optic nerves occur simultaneously in transgenic mice exposed to ischemic or crush injury. The degree of RGC loss reflects the severity of the injury. Loss of oligodendrocytes and myelin apparently leads to axonal loss. Transgenic mice offer a promising model for exploring the damage caused by optic nerve injury. Use of fluorescence labeling makes it possible to better understand the underlying pathophysiology, which can help researchers formulate neuroprotective agents.

Serum cytokine levels in active uveitis and remission.

Serum levels of interleukin(IL)-8, IL-6, and (TNF)-alpha were measured in 25 patients during active uveitis and uveitis in remission and compared to age-matched controls. Levels of IL-8 and IL-6 were significantly elevated in patients with active disease and were decreased during remission. IL-8 levels were highest in patients with anterior uveitis, with greatest difference between active disease and remission. No consistent pattern was observed for TNF-alpha. In conclusion, serum cytokine levels are elevated in active noninfectious uveitis. The rise in IL-8 may suggest innate immune mechanisms in the acute disease, while IL-6 participates in modulation of inflammation in the chronic disease.

Elevated plasma levels of interleukin 8 in patients with acute anterior ischaemic optic neuropathy.

BACKGROUND/AIM: Alterations of the immune system may have a role in thrombogenesis. Artery sites occluded with thrombi apparently release pro-inflammatory cytokines. Non-arteritic anterior ischaemic optic neuropathy (NAION) results from occlusion of the blood supply to the optic nerve. The aim of this study was to analyse levels of pro-inflammatory cytokines in patients with acute event of NAION. METHODS: Study participants included 10 patients (12 eyes) with NAION and 20 age matched controls with the same risk factors for atherosclerosis disease. Peripheral blood samples were obtained immediately at the acute onset of NAION. Plasma interleukin 8 (IL-8), IL-6, and tumour necrosis factor alpha (TNF-alpha) levels were measured immediately following diagnosis and during the follow up intervals. RESULTS: The plasma levels of IL-8 were significantly higher in NAION patients at the time of diagnosis in comparison to the control group (p = 0.002), and decreased during the follow up period (6-12 months) (p = 0.05). There were no differences in plasma levels of IL-6 and TNF-alpha between NAION patients and controls, either in the acute phase or during the follow up period. CONCLUSION: Plasma levels of IL-8 are elevated during the acute phase of NAION, but not IL-6 and TNF-alpha. These elevated levels are in accordance with other acute vascular thrombosis. The clinical significance of these findings should be further evaluated.

Long term visual and neurological prognosis in patients with treated and untreated cavernous sinus aneurysms.

OBJECTIVE: To determine the long term visual and neurological outcome of patients diagnosed with cavernous sinus aneurysms (CCAs). METHODS: Prospective follow up for at least five years or until death of 31 retrospectively recruited patients (27 women, 4 men) with treated and untreated CCAs. RESULTS: There were 40 aneurysms in all. Mean age at diagnosis was 60.4 years (range 25 to 86; median 64). The most common symptoms were diplopia (61%), headache (53%), and facial or orbital pain (32%). Fifteen patients (48%) were diagnosed after they developed cranial nerve pareses, four (13%) after they developed carotid-cavernous sinus fistulas (CCFs), and 12 (39%) by neuroimaging studies done for unrelated symptoms. Twenty one patients (68%) had treatment to exclude the aneurysm from circulation, 10 shortly after diagnosis and 11 after worsening symptoms. Immediate complications of treatment occurred in six patients and included neurological impairment, acute ophthalmoparesis, and visual loss. Ten patients (32%) were observed without intervention. Over a mean (SD) follow up period of 11.8 (7.7) years, eight had improvement in symptoms, five remained stable, and eight deteriorated. Among the 10 patients followed without intervention, none improved spontaneously, three remained stable, and seven worsened. CONCLUSIONS: Most treated patients in this series improved or remained stable after treatment, but none improved without treatment. The long term prognosis for treated cases is relatively good, with most complications occurring immediately after the procedure. Endovascular surgery has decreased the morbidity and mortality of treatment so should be considered for any patient with a CCA.

Posterior chamber intraocular lens implantation after capsular tear: ultrasound biomicroscopy evaluation.

PURPOSE: To evaluate by ultrasound biomicroscopy (UBM) the exact position of a posterior chamber intraocular lens (PC IOL) implanted above a posterior capsule tear and to correlate the findings with the outcomes and complication rate. SETTING: Department of Ophthalmology, Rabin Medical Center, Petah Tiqva, Israel. METHODS: In a retrospective noncomparative case series, 36 patients (36 eyes) who had extracapsular cataract extraction (ECCE) complicated by capsular tear were evaluated. In all cases, a PC IOL was implanted above the remnant of the capsule without suturing. Ultrasound biomicroscopy was performed to study the exact position of the optic and haptic. The outcomes and complication rate were determined from patient examinations performed during a follow-up of 1 to 6 years. RESULTS: Optic tilt was found in 20 of 36 patients (56%). In 17 patients (47%), both haptics were located in the sulcus. In 15 patients (42%), 1 haptic was observed in the sulcus and 1 in the bag or elsewhere. No correlation was found between the position of the optic and the location of the haptics. Minimal postoperative complications were observed, and only 1 patient required another surgery for a dislocated IOL. CONCLUSION: By UBM, half the patients had optic tilt and more than half the IOLs were not implanted in the sulcus, the intended location.

Role of transesophageal echocardiography in the evaluation of patients with retinal artery occlusion.

OBJECTIVE: To evaluate the role of transesophageal echocardiography (TEE) in detecting cardiac and thoracic aortic sources of retinal emboli. DESIGN: Retrospective observational case series. PARTICIPANTS: The study population consisted of 18 patients who were initially seen with retinal artery occlusion (7 central, 11 branch) and underwent TEE as part of the systemic evaluation. INTERVENTION: All patients underwent TEE, consisting of complete two-dimensional and Doppler color flow examinations. TEE was done immediately after transthoracic echo (TTE) examination. The medical records were reviewed. MAIN OUTCOME MEASURE: Detection of a possible cardiac or thoracic aortic source of retinal embolus. RESULTS: Cardiac or thoracic aortic pathologic conditions, which were a possible source of the retinal emboli, were detected by TEE in 13 of the 18 patients (72%). They included aortic arch atheroma (n = 7), mitral annulus calcification (n = 4), left atrial appendage thrombus (n = 2), valvular abnormalities (n = 5), left atrial smoke (n = 3), and patent foramen ovale (n = 3). In 11 patients (61%), at least one cardiac or aortic source of emboli detected by TEE was missed by TTE. Significant carotid artery disease (>or=40% stenosis) was present in 3 of 16 patients (17%). CONCLUSIONS: TEE is a potentially useful modality for detecting possible sources of retinal artery emboli and may be considered as an adjunct to the routine evaluation of affected patients.

Ophthalmic artery calcification in a patient with renal failure.

We report calcification in cranial arteries, including the ophthalmic arteries, visible on CT in a patient with diabetes mellitus and renal failure.

Ultrasound biomicroscopic detection of anterior ocular segment foreign body after trauma.

PURPOSE: To describe the role of ultrasound biomicroscopy in the detection and localization of foreign bodies in anterior ocular segment foreign body after trauma. METHODS: In a prospective study, ultrasound biomicroscopy was performed in five eyes of five consecutive patients with suspected anterior ocular segment foreign body. RESULTS: In all five eyes, ultrasound biomicroscopy detected and precisely localized small foreign bodies (metallic in two eyes, stone in one eye, plastic in one eye, and ceramic in one eye) in the cornea (one eye), superficial sclera (one eye), and anterior ocular segment (three eyes). Operative procedures to remove the intraocular foreign bodies (three cases) were guided by the ultrasound biomicroscopy information. CONCLUSIONS: Ultrasound biomicroscopy is a noninvasive method for detecting anterior segment intraocular foreign bodies after perforating trauma. It can be used to accurately diagnose foreign bodies and assist in surgical management, particularly when direct visualization is obscured because of the trauma. In eyes with partial-thickness corneoscleral lacerations or sealed full-thickness corneoscleral laceration and suspected anterior ocular segment foreign body, ultrasound biomicroscopy is a safe and effective method for detecting and localizing foreign bodies in the anterior ocular segment.

Long-term follow-up of perfluorocarbon liquid in the anterior chamber.

BACKGROUND: Perfluorocarbon liquids are used as a vitreous substitute in the operative management of several ophthalmologic conditions. We examined the effects of residual perfluorocarbon droplets in the anterior chamber in patients after retinal detachment surgery. METHODS: The study group consisted of seven patients (seven eyes) aged 28-74 years in whom droplets of perfluorocarbon appeared in the anterior chamber subsequent to retinal detachment surgery involving scleral buckling, perfluorodecalin injection, and perfluorocarbon liquid-silicone oil exchange. Mean follow-up was 9.4 months. RESULTS: From 1 -15 droplets of perfluorocarbon liquid were found in the anterior chamber. There were no corneal complications or inflammatory reactions or blood vessel invasion into the corneal stroma. Some of the droplets appeared to be encapsulated in a membrane-like material. Perfluorocarbon liquid and silicone oil had to be removed in three patients at 20, 12, and 4 months postoperatively because of an increase in intraocular pressure due to blockage of the inferior iridectomy by a droplet of perfluorocarbon, emulsification of the silicone oil, or residual perfluorocarbon liquid on the retina (one patient each). Increased intraocular pressure in a fourth patient was successfully treated pharmacologically. CONCLUSION: Based on our experience, residual perfluorocarbon liquid droplets in the anterior chamber are well tolerated and do not induce corneal damage or ocular inflammation. These patients should be closely followed, however, and the liquid removed if complications develop.