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To describe a rare case of unilateral, endogenous endophthalmitis caused by Aggregatibacter aphrophilus (HACEK group) confirmed in vitreous and blood cultures, in a patient with dentophobia.Case report.75-year-old male patient with type 2 diabetes, previous myocardial infarction, and pacemaker implantation.Patient was seen with sudden loss of vision at the Dept of Ophthalmology, Uppsala University. Initial diagnosis was posterior vitreous detachment and anterior uveitis, but progression of disease led to vitrectomy, which actually demonstrated endophthalmitis and growth of Aggregatibacter aphrophilus of the HACEK group. Aggregatibacter bacteremia and pacemaker endocarditis were also identified and dental examination confirmed growth of Aggregatibacter in the oral cavity. Intravitreal treatment with ceftazidime and vancomycin according to EVS (Endophthalmitis Vitrectomy Study) protocol was administered with quick resolution of endophthalmitis.Aggregatibacter endophthalmitis is a rare but devastating cause of vision loss where immediate diagnosis may be delayed. Prompt diagnosis may be facilitated by a thorough medical history and early vitreous biopsy. Systemic investigation by an infectious disease specialist and multidisciplinary assessment are mandatory. Ophthalmological treatment is effective with intravitreal injections of ceftazidime and vancomycin.

Achmed Pircher,1,2 Albert Neutzner,3 Margherita Montali,4 Andreas Huber,5 Hendrik PN Scholl,2,6 Jatta Berberat,7 Luca Remonda,7 Hanspeter E Killer3 1Department of Neuroscience/Ophthalmology, Uppsala University, Uppsala, Sweden; 2Department of Ophthalmology, University Hospital Basel & University Basel, Basel, Switzerland; 3Department of Biomedicine, University Hospital Basel & University Basel, Basel, Switzerland; 4Department of Ophthalmology, San Bassiano Hospital, Bassano del Grappa, Italy; 5Department of Medicine, Private University of the Principality of Lie Triesen, Triesen, Liechtenstein; 6Institute of Molecular and Clinical Ophthalmology Basel, Basel, Switzerland; 7Department of Neuroradiology, Cantonal Hospital Aarau, Aarau, SwitzerlandCorrespondence: Achmed PircherDepartment of Neuroscience/Ophthalmology, Uppsala University, Uppsala, SwedenTel +46 76 496 37 51Email achmed.pircher@gmail.comObjective: To report on the lipocalin-type prostaglandin D synthase (L-PGDS) concentrations in the cerebrospinal fluid (CSF) of the perioptic and lumbar subarachnoid space (SAS) in patients with radiologically proven optic nerve (ON) sheath compartmentation presenting as normal-tension glaucoma (NTG).Methods: Retrospective biochemical analysis of CSF in thirteen patients with ON sheath compartmentation presenting as NTG (four females, mean age 70± 8 years). CSF was sampled from the SAS of the ON during ON sheath fenestration for ON sheath compartmentation and from the lumbar SAS at the time of lumbar puncture. Nephelometry was used for the quantification of L-PGDS and albumin concentration. Albumin was measured in order to assess the amount of contamination with serum in the CSF samples taken from the ON SAS. Main outcome measures were L-PGDS concentrations in the CSF of the perioptic and lumbar SAS.Results: Mean L-PGDS concentration was 24± 8 mg/L in the lumbar SAS compared to 33± 27 mg/L without correction of serum contamination and 45± 39 mg/L after correction of serum contamination in the perioptic SAS. The difference between the lumbar and the perioptic SAS was statistically significant (P=0.0047 without correction of serum contamination, P=0.0002 with correction of serum contamination; Mann–Witney U-test).Conclusion: This study demonstrates a concentration gradient of L-PGDS levels within the CSF with a statistically significant higher concentration in the compartmentalized perioptic SAS compared to that in the lumbar SAS. Biochemical changes in the perioptic SAS might be involved in the pathophysiology in NTG patients with ON sheath compartmentation.Keywords: lipocalin-type prostaglandin D synthase, cerebrospinal fluid, optic nerve sheath compartment syndrome, normal-tension glaucoma, optic nerve

Hanspeter E Killer,1 Achmed Pircher2,3 1Department of Biomedicine, University Hospital Basel & University Basel, Basel, Switzerland; 2Department of Neuroscience/Ophthalmology, Uppsala University, Uppsala, Sweden; 3Department of Ophthalmology, University Hospital Basel & University Basel, Basel, SwitzerlandCorrespondence: Achmed PircherDepartment of Neuroscience/Ophthalmology, Uppsala University, Uppsala, SwedenTel +46 0 76 496 37 51Email achmed.pircher@gmail.com View the original paper by Dr Pircher and colleagues This is in response to the Letter to the Editor

IMPORTANCE This study offers a new approach for the quantification of CSF dynamics. BACKGROUND Non-invasive method to quantify the CSF dynamics in the subarachnoid space of the optic nerve is highly desirable. The aim of the study was to measure slow-flow CSF velocities in healthy controls and normal tension glaucoma patients between the intracranial cavity and the subarachnoid space of the optic nerve. DESIGN Prospective observational study. PARTICIPANTS Eleven age-matched healthy volunteers and 15 normal tension glaucoma patients. METHODS Using phase contrast images, the phase shift in MRI diffusion images can be used to determine the flow velocity. Flow-range ratio between the intracranial cavity and the subarachnoid space of the optic nerve was calculated. MAIN OUTCOME MEASURE Flow-range ratio between the intracranial cavity and the subarachnoid space of the optic nerve was calculated. RESULTS First, phantom measurements were provided to validate the slow-flow velocity calculations. Second, flow-range ratio was validated for the healthy controls (0.63 ± 0.05), with the range being similar for the right and left optic nerve (P = 0.1). Statistically significant results were obtained (P < 0.05) when comparing the flow-range ratio in the optic nerve of healthy controls (n = 22 eyes, 0.63 ± 0.05) with the flow-range ratio in pathological optic nerves (n = 23, 0.55 ± 0.08) of normal tension glaucoma patients. MANOVA revealed no dependency between flow-range ratio and patient dependent variables. CONCLUSION AND RELEVANCE Diffusion-weighted imaging provides a method to evaluate CSF flow within the subarachnoid space of the optic nerve in a non-invasive manner. Compared to healthy controls, patients with normal tension glaucoma measure a significantly lower flow-range ratio. This finding suggests a possible role of impaired CSF dynamics in the pathophysiology in normal tension glaucoma.

PurposeTo investigate on the relationship between the optic nerve sheath diameter (ONSD) and the lumbar cerebrospinal fluid pressure (CSF-p) in Caucasian patients with normal tension glaucoma (NTG).Patients and methodsRetrospective analysis of medical records of patients with open-angle glaucoma in the period from 2005 to 2015 from the Ophthalmology Department, Cantonal Hospital Aarau, Switzerland was performed. A total of 38 patients (mean age 68.6±11.3 years, 21 females and 17 males) fulfilled the diagnostic criteria of NTG and underwent computed tomography (CT) of the orbit and lumbar puncture (LP). In total, 38 age- and gender-matched Caucasian subjects (mean age: 68.9±10.9 years) without known ON diseases served as controls for ONSD measurements. ONSDs were measured at a distance of 3 mm from the posterior globe and lumbar CSF-p was related to the measurements. Statistical analysis was performed by using the independent two-tailed t-test and the non-parametric Spearman's correlation test.ResultsThe mean ONSD in NTGs measured 6.4±0.9 mm and in controls 5.4±0.6 mm. The difference between NTGs and controls showed statistical significance (t-test: P0.000). The mean CSF-p in NTG was 11.6±3.7 mm Hg. There was no statistical significant correlation between ONSD and CSF-p (Spearman's correlation coefficient ρ=0.06, P=0.72).ConclusionsThis study demonstrates enlarged ONSDs and normal lumbar CSF-p in 38 Caucasian NTG patients. As enlarged ONSDs generally are associated with increased intracranial CSF-p, these results can be explained by a disturbed communication of CSF-p between the intracranial and intraorbital subarachnoid spaces.

Importance Optic nerve (ON) dysfunction is a common feature of different diseases. The pathophysiology is not yet fully understood. Background This study describes five patients with ON sheath (ONS) compartment syndrome (ONSCS) and contributes to the hypothesis that impaired cerebrospinal fluid (CSF) flow can play a role in the development of ON dysfunction. Design Retrospective case series. Participants Five patients with ONSCS were included in the study. Methods Elaboration of medical histories and clinical and diagnostic findings over a long time period was carried out by analysing medical records and by a detailed medical consultation. Main outcome measures The main outcome measures include clinical history; visual acuity; field, intraocular and CSF pressures; and contrast-loaded computed tomographic (CT) cisternography. Results Compartmentation of the ONS demonstrated by contrast-loaded CT cisternography was the consistent finding in all five patients who demonstrated findings of ON dysfunction. The aetiologies varied and included meningitis, papilloedema, sphenoid wing meningioma, disc herniation and normal-tension glaucoma. Conclusion and relevance Compartmentation of the ONS with consecutively impaired CSF dynamics within the ON subarachnoid space can lead to ON dysfunction. Different aetiologies can cause the development of ONSCS.

Introduction: Ophthalmological complications such as orbital compartment syndrome (OCS) and ischemic optic neuropathy are rare complications in patients with burns and have been described in patients where aggressive fluid resuscitation was performed. While OCS requires urgent surgical intervention, no current treatment is established to treat, or prevent, ischemic optic neuropathy in patients with burns. Methods: The authors report a case of a 38-year-old woman with flame burns including the periorbital regions who developed OCS on the left side and anterior ischemic optic neuropathy (AION) on the right side despite non-aggressive fluid resuscitation. Immediate lateral canthotomy combined with inferior cantholysis was performed on the left side. Discussion and Conclusion: OCS and AION need to be considered as potential complications even in critically ill patients with facial burns who do not receive aggressive fluid resuscitation. Whether an early surgical intervention will lower the risk of AION development is, however, speculative. Lay Summary Ophthalmological complications such as orbital compartment syndrome and ischemic optic neuropathy are rare complications in patients with burns and have been described in patients where aggressive fluid resuscitation was performed. We present a case of a critically ill patient with severe facial burns who developed orbital compartment syndrome on the left side and anterior ischemic optic neuropathy on the right side even though our patient did not receive aggressive fluid resuscitation. Our case is particular because both of these rare complications are seen in a single patient and neither received aggressive fluid resuscitation. The fact that the patient did not develop ischemic optic neuropathy on the side where the lateral canthotomy was performed (only on the side where the patient had orbital compartment syndrome), this case might raise the discussion of whether an early surgical intervention might lower the risk of ischemic optic neuropathy development in patients with facial burns.

Pressure and velocity in intraocular and subarachnoid space fluid chambers : an inseparable couple

Response to : 'Comment on: Pressure and velocity in intraocular and subarachnoid space fluid chambers: an inseparable couple'

This study offers a new approach for the quantification of CSF dynamics.Non-invasive method to quantify the CSF dynamics in the subarachnoid space of the optic nerve is highly desirable. The aim of the study was to measure slow-flow CSF velocities in healthy controls and normal tension glaucoma patients between the intracranial cavity and the subarachnoid space of the optic nerve.Prospective observational study.Eleven age-matched healthy volunteers and 15 normal tension glaucoma patients.Using phase contrast images, the phase shift in MRI diffusion images can be used to determine the flow velocity. Flow-range ratio between the intracranial cavity and the subarachnoid space of the optic nerve was calculated.Flow-range ratio between the intracranial cavity and the subarachnoid space of the optic nerve was calculated.First, phantom measurements were provided to validate the slow-flow velocity calculations. Second, flow-range ratio was validated for the healthy controls (0.63 ± 0.05), with the range being similar for the right and left optic nerve (P = 0.1). Statistically significant results were obtained (P0.05) when comparing the flow-range ratio in the optic nerve of healthy controls (n = 22 eyes, 0.63 ± 0.05) with the flow-range ratio in pathological optic nerves (n = 23, 0.55 ± 0.08) of normal tension glaucoma patients. MANOVA revealed no dependency between flow-range ratio and patient dependent variables.Diffusion-weighted imaging provides a method to evaluate CSF flow within the subarachnoid space of the optic nerve in a non-invasive manner. Compared to healthy controls, patients with normal tension glaucoma measure a significantly lower flow-range ratio. This finding suggests a possible role of impaired CSF dynamics in the pathophysiology in normal tension glaucoma.

Strabismus influences not only the individual with nonparallel eyes but also the observer. It has previously been demonstrated by fMRI that adults viewing images of strabismic adults have a negative reaction to the images as demonstrated by limbic activation, especially activation of the left amygdala. The aim of this study was to see if mothers would have a similar reaction to viewing strabismic children and whether or not that reaction would be different in mothers of strabismic children. Healthy mothers of children with strabismus (n = 10, Group I) and without strabismus (n = 15, Group II) voluntarily underwent fMRI at 3T. Blood oxygen level dependent signal responses to viewing images of strabismic and non-strabismic children were analyzed. Group II, while viewing images of strabismic children, showed significantly increased activation of the limbic network (p < 0.05) and bilateral amygdala activation. Group I showed considerably less limbic activation, compared to the group II, and had no amygdala activation. Both groups revealed statically significant activation in the FEF (frontal eye field) when they were viewing images of strabismic children as compared to when they were viewing children with parallel eyes. The activated FEF area for Group II was much larger than for group I. Mothers of non-strabismic children showed similar negative emotional fMRI patterns as adults did while viewing strabismic adults. Strabismus is an interpersonal organic issue for the observer, which also impacts the youngest members of our society.

PURPOSE To investigate the cerebrospinal fluid (CSF) dynamics along the entire optic nerve (ON) in patients with normal-tension glaucoma (NTG). METHODS Retrospective analysis of computed tomographic (CT) cisternographies in Caucasian patients with NTG. Fifty-six patients (99 of 112 eyes) fulfilled the diagnostic criteria of NTG and underwent CT-cisternography. Twelve subjects without NTG (24 eyes) served as controls. Contrast-loaded cerebrospinal fluid (CLCSF) density measurements in Hounsfield units (HU) were performed at four defined regions along the ON and in the basal cistern. RESULTS In NTG patients, the mean density CLCSF in the bulbar segment measured 76 ± 49 HU right and 88 ± 74 HU left, in the mid-orbital segment 117 ± 92 HU right and 119 ± 73 HU left, in the intracanalicular ON portion 209 ± 88 HU right and 216 ± 101 HU left, in the intracranial ON portion 290 ± 106 HU right and 286 ± 118 HU left and in the basal cistern 517 ± 213 HU. The distribution of CLCSF along the ON showed a statistically significant reduction in the intraorbital ON segments in NTG patients compared to controls without NTG with the far largest difference within the retrobulbar segment (-150 HU right and -117 HU left; right: p

Purpose To report on the optic canal cross-sectional area (OCA) in Caucasian patients with normal-tension glaucoma (NTG) compared with Caucasian control subjects without known optic nerve (ON) diseases. Methods Retrospective analysis of computed tomographic images of the cranium and orbits in 56 NTG patients (30 females and 26 males; 99 of 112 eyes; mean age 67.7 ± 11.1 years). Fifty-six age- and gender-matched subjects (mean age: 68.0 ± 11.2 years) without known ON diseases served as controls. The OCA at the orbital opening was measured in square millimeters by using the tool “freehand.” Statistical analysis was performed by using the independent two-tailed t-test. Results The mean orbital opening OCA in NTGs measured 14.5 ± 3.5 mm2 (right OCA: 14.4 ± 3.6 mm2, left OCA: 14.5 ± 3.4 mm2) and in controls measured 18.3 ± 2.6 mm2 (right OCA: 18.5 ± 2.7 mm2, left OCA: 18.1 ± 2.5 mm2). The difference between NTG and controls was statistically significant (p

Purpose The aim of this study was to examine the translaminar pressure difference (TLP) in Caucasian patients with normal tension glaucoma (NTG) and its possible impact onto the pathogenesis of NTG. Methods Retrospective analysis of medical records of patients with open-angle glaucoma (OAG) in the period from 2005 to 2015 from the Ophthalmology Department, Cantonal Hospital Aarau, Switzerland. A total of 67 eyes of 38 patients (mean age 68.6 ± 11.3 years, 21 women and 17 men) fulfilled the diagnostic criteria of progressive NTG and underwent lumbar puncture (LP) during computer-assisted cisternography (CT – cisternography). The intraocular pressure (IOP) and lumbar cerebrospinal fluid pressure (CSF-p) were analysed and the TLP calculated. The TLP was compared with the mean defect (MD) of visual fields. Statistical analysis was performed with the one and two-tailed paired and unpaired t-test and the non-parametric Spearman correlation test. Results The mean lumbar opening CSF-p measured 11.6 ± 3.7 mmHg. The mean IOP in the right eye measured 14.7 ± 2.4 mmHg, in the left eye 14.7 ± 2.5 mmHg. The calculated mean TLP was 3.0 ± 4.2 mmHg in the right and 3.3 ± 4.3 mmHg in the left eye. There was no significant correlation between TLP and the MD of visual fields in both eyes. Conclusions This study did not confirm either a lower lumbar CSF-p or increased TLP compared to previous retrospective and prospective studies. As cerebrospinal fluid (CSF) flow is not homogenous throughout all CSF spaces and CSF-p and IOP fluctuate, the current view on TLP needs modifications to improve its validity.