Presenting Features of Giant Cell Arteritis with Active Versus Healed Arteritis on Biopsy.

Giant cell arteritis (GCA) is often categorised as "active" or "healed" on temporal artery biopsy (TAB). The purpose of this study was to compare the initial clinical presentation of patients with GCA according to active versus healed arteritis on TAB. A retrospective chart review was performed for patients with biopsy-proven GCA (BP-GCA) at a single academic medical institution from a previously reported cohort. The arteritis on TAB was categorised as "active" or "healed" based on the pathological reports. Demographic information, clinical presentation, past medical history, and test results were collected from the date of TAB. These baseline characteristics were entered into the GCA Risk Calculator. Of 85 patients with BP-GCA, 80% had active and 20% had healed disease according to histopathology. A higher percentage of those with active arteritis had ischaemic optic neuropathy (ION) (36% versus 6%, p = .03), elevated erythrocyte sedimentation rates (92% versus 63%, p = .01), elevated C-reactive protein levels (79% versus 46%, p = .049), GCA risk score > 7.5% (99% sensitivity, 100% versus 71%, p < .001), higher mean GCA risk calculator scores (neural network p = .001; logistic regression p = .002). Patients with healed arteritis were less likely to have visual manifestations than the active arteritis group (38% versus 71%, p = .04). Patients with active vasculitis on biopsy had higher rates of ION and elevated inflammatory markers, as well as higher predictive scores from the GCA risk calculator. Further research is needed regarding correlation of biopsy findings and risk of complications or relapses.

Baseline Characteristics and Clinical Presentation of Biopsy-Proven Giant Cell Arteritis in White Compared with Black Patients.

BACKGROUND: Giant cell arteritis (GCA) is the most prevalent systemic vasculitis in the elderly and can lead to permanent vision loss if left untreated. Most earlier studies have evaluated GCA in primarily white populations, and GCA was traditionally thought to occur at nearly negligible frequency in black populations. Our previous study showed that GCA may occur at similar rates in white and black patients, but little is known about the presentation of GCA in black patients. The purpose of this study is to examine baseline presentation of biopsy-proven GCA (BP-GCA) in a tertiary care center-based population with a sizeable proportion of black patients. METHODS: Retrospective study from a single academic institution of a previously described cohort of BP-GCA. Presenting symptoms, laboratory findings, and GCA Calculator Risk score were compared in black and white patients with BP-GCA. RESULTS: Among 85 patients with biopsy-proven GCA, 71 (84%) were white and 12 (14%) were black. White patients had higher rates of elevated platelet count (34% vs 0%, P = 0.04), whereas black patients had higher rates of diabetes mellitus (67% vs 12%, P < 0.001). There were no statistically significant differences in age, gender, biopsy classification (active vs healed arteritis), cranial symptoms, visual symptoms/ophthalmic findings, rates of abnormal erythrocyte sedimentation rate or C-reactive protein, unintentional weight loss, polymyalgia rheumatica, or GCA risk calculator score. CONCLUSIONS: Presenting features of GCA were similar between white and black patients in our cohort, except for rates of abnormal platelet level and diabetes. Physicians should feel comfortable relying on the usual clinical features for the diagnosis of GCA independent of race.

Effect of Temporal Artery Biopsy Length and Laterality on Diagnostic Yield.

BACKGROUND: Giant cell arteritis (GCA) is the most common vasculitis in adults and is associated with significant morbidity and mortality. Temporal artery biopsy (TAB) remains the gold standard for diagnosis in the United States; however, practices vary in the length of artery obtained and whether bilateral simultaneous biopsies are obtained. METHODS: Retrospective chart review of all TABs performed at the Johns Hopkins Wilmer Eye Institute between July 1, 2007, and September 30, 2017. RESULTS: Five hundred eighty-six patients underwent TAB to evaluate for GCA. Of 404 unilateral biopsies, 68 (16.8%) were positive. Of 182 patients with bilateral biopsies, 25 (13.7%) had biopsies that were positive and 5 patients (2.7%) had biopsies that were discordant, meaning only 1 side was positive. There was no significant difference in the average postfixation length of positive and negative TAB specimens (positive mean length 1.38 ± 0.61 cm, negative mean length 1.39 ± 0.62 cm, P = 0.9). CONCLUSIONS: There is no significant association between greater length of biopsy and a positive TAB result in our data. Although the rate of positive results was not higher in the bilateral group compared with the unilateral group, 2.7% of bilateral biopsies were discordant, similar to previously published rates. Overall, this suggests that initial bilateral biopsy may increase diagnostic yield, albeit by a small amount.

Terson syndrome in reverse: intraventricular haemorrhage following primary intraocular haemorrhage.

Terson syndrome (TS) describes the presence of intraocular haemorrhage in patients with intracranial haemorrhage or traumatic brain injury. The aetiology of TS is controversial as an anatomical conduit between the vitreous humour and subarachnoid space remains contested. We herewith present a case of primary vitreous haemorrhage with secondary intracranial extension into the ventricles. Cranial CT demonstrates blood within the left optic nerve and chiasm but not within the subarachnoid space. This unusual phenomenon, which has not been reported before, may be described as 'Terson syndrome in reverse'. We explore mechanisms by which blood within the globe may track into the ventricular system, contextualising recent advances in the understanding of ocular-intracranial fluid transport.

An explanation for Terson syndrome at last: the glymphatic reflux theory.

Terson Syndrome (TS) describes the presence of intraocular hemorrhage in patients with intracranial hemorrhage, typically subarachnoid hemorrhage. Despite TS being a well-defined and frequently occurring phenomenon, its pathophysiology remains controversial. This review will present the current understanding of TS, with view to describing a contemporary and more plausible pathomechanism of TS, given recent advances in ophthalmic science and neurobiology. Previously proposed theories include a sudden rise in intracranial pressure (ICP) transmitted to the optic nerve sheath leading to rupture of retinal vessels; or intracranial blood extending to the orbit via the optic nerve sheath. The origin of blood in TS is uncertain, but retinal vessels appear to be an unlikely source. In addition, an anatomical pathway for blood to enter the eye from the intracranial space remains poorly defined. An ocular glymphatic system has recently been described, drainage of which from the globe into intracranial glymphatics is reliant on the pressure gradient between intraocular pressure and intracranial pressure. The glymphatic pathway is the only extravascular anatomical conduit between the subarachnoid space and the retina. We propose that subarachnoid blood in skull base cisterns near the optic nerve is the substrate of blood in TS. Raised ICP causes it to be refluxed through glymphatic channels into the globe, resulting in intraocular hemorrhage. We herewith present glymphatic reflux as an alternative theory to explain the phenomenon of Terson Syndrome.

Association of Giant Cell Arteritis With Race.

IMPORTANCE: Giant cell arteritis (GCA) is the most common vasculitis in adults and is associated with significant morbidity and mortality. Its incidence has been carefully studied in white populations, yet its relevance among other racial and ethnic groups is less well known. OBJECTIVE: To examine the incidence of biopsy-proven GCA (BP-GCA) in a tertiary care center-based population with a sizeable proportion of black patients. DESIGN, SETTING, AND PARTICIPANTS: This retrospective cohort study identified all patients who underwent temporal artery biopsy (TAB) from July 1, 2007, through September 30, 2017, using the electronic medical record system at the Johns Hopkins Wilmer Eye Institute. Associations between self-reported race, sex, and age were explored and compared with all other patients attending the hospital over the same period. Data were analyzed from November 1, 2017, through July 31, 2018. MAIN OUTCOMES AND MEASURES: Estimated incidence rates of BP-GCA in black and white patients. RESULTS: Among 586 patients who underwent TAB (mean [SD] age, 70.5 [11.1] years; age range, 32-103 years; 423 [72.2%] women), 167 (28.5%) were black, 382 (65.2%) were white, and 37 (6.3%) were other or unknown. Of 573 individuals 50 years and older, 92 (16.1%) had BP-GCA; 14 were black (8.4% of all black patients undergoing testing) and 75 were white (19.6% of all white patients undergoing testing). Crude annual incidence rates for BP-GCA were 2.9 (95% CI, 1.3-5.5) per 100 000 for black and 4.2 (95% CI, 3.0-5.6) per 100 000 for white patients within the study population. Population-adjusted age- and sex-standardized incidence rates were 3.1 (95% CI, 1.0-5.2) and 3.6 (95% CI, 2.5-4.7) per 100 000 for black and white patients, respectively (difference, 0.5; 95% CI, -1.7 to 2.7; P = .70). The incidence rate ratio was 1.9 in women compared with men (95% CI, 1.1-3.4; P = .03) but was not significant in white compared with black patients (1.2; 95% CI, 0.6-2.4; P = .66). CONCLUSIONS AND RELEVANCE: In our cohort, BP-GCA occurred more commonly in women, but rates were similar between races. These findings do not appear to support the conclusion that GCA occurs more frequently in white compared with black patients.

Temperature changes associated with bone drilling in an orbital model: comparison of ultrasonic bone curette and conventional high-speed rotational drill.

Purpose: Thermal injury to the optic nerve is a potential complication of bony decompression of the orbital apex. An animal model was used to compare and contrast temperature change while removing orbital bone with ultrasonic and conventional drills. Methods: Two devices, Sonopet ultrasonic bone curette and TPS CORE Micro drill, were used to remove bone from six unpreserved exenterated porcine orbits at fixed distances from the optic canal while temperature was recorded. Increasing irrigation flow rate and decreasing saline temperature with the ultrasonic bone curette were also investigated. Results: The mean change in temperature at the optic canal using the ultrasonic bone curette with 18 ml/min 24°C irrigation was +7.2 ± 3.3°C (range 3.3-12.7°C) at 60 s, and using the rotational drill was +1.7 ± 1.3°C (range 0.1-3.3°C), representing a statistically significant increase above baseline for both drills (p < 0.01 for each). The difference in temperature change between drills was statistically significant (p < 0.01). When irrigation fluid was changed from room temperature saline (24°C), rate 18 ml/min to chilled (10°C) irrigation saline, rate 40 ml/min, the magnitude of the mean temperature increase was reduced by 3.1°C, p < 0.05. Conclusions: In this orbital decompression model, both ultrasonic and rotational drills induced a rise in temperature. This increase was significantly greater with the ultrasonic bone curette, measuring up to 13.7°C. Clinicians should be aware of the potential for temperature spikes when decompressing the orbital apex. Increasing irrigation flow rate and using chilled saline may mitigate increases in temperature.

Macular and Retinal Nerve Fibre Layer Thinning in Xeroderma Pigmentosum: A Cross-sectional Study.

The purpose of this study was to evaluate retinal thickness in different Xeroderma Pigmentosum (XP) complementation groups using spectral-domain optical coherence tomography (SD-OCT). This was a cross-sectional pilot study of 40 patients with XP. All patients had healthy-looking retinae and optic nerves on slit lamp biomicroscopy, and subtle or no neurological deficits. Patients were divided into two groups based on the known tendency for neurodegeneration associated with certain XP complementation groups. A third control group was obtained from a normative database. Using SD-OCT, we compared peripapillary retinal nerve fibre layer (pRNFL) and macular thickness between the groups. XP patients with a known tendency for neurodegeneration were found to have a statistically significant reduction in both pRNFL (p < 0.01) and macular thickness (p < 0.001) compared with healthy controls. In contrast, there was no statistically significant difference between pRNFL and macular thickness in XP patients not expected to develop neurodegeneration compared to the same control group. When both XP groups were compared, a statistically significant reduction in total pRNFL (p = 0.02) and macular thickness (p = 0.002) was found in XP patients predisposed to neurodegeneration. Our results suggest that pRNFL and macular thickness are reduced in XP patients with a known tendency for neurodegeneration, even before any marked neurological deficits become manifest. These findings demonstrate the potential role of retinal thickness as an anatomic biomarker and prognostic indicator for XP neurodegeneration.

Nonsurgical Management of Retained Needlefish Jaw.

While scuba diving, the left medial canthus of a 53-year-old man was pierced by a needlefish. He immediately lost vision in his left eye. An orbital computed tomographic scan showed the needlefish jaw in the left optic canal. The left medial orbit was explored surgically but no foreign object was removed. One month later, MRI confirmed the presence of the retained needlefish jaw. A conservative approach was taken and the patient remained stable over 3 months of follow-up.

Isolated endogenous Fusarium endophthalmitis in an immunocompetent adult after a thorn prick to the hand.

PURPOSE: To report the case of an immunocompetent adult presenting with endogenous Fusarium endophthalmitis. OBSERVATIONS: A woman in her thirties presented with symptoms and signs of a unilateral anterior uveitis. After initial improvement with topical corticosteroids, she continued to develop a panuveitis with an associated drop in vision to counting fingers. A vitreous biopsy confirmed Fusarium solani by 18S rRNA fungal gene detection and PCR sequencing. Despite treatment with pars plana vitrectomy, intravitreal amphotericin B and systemic voriconazole her visual outcome was poor. Detailed review of her antecedent history revealed the route of acquisition to be a thorn prick to the hand two weeks prior to presentation. CONCLUSIONS AND IMPORTANCE: This patient's endophthalmitis most likely resulted from cutaneous inoculation of Fusarium solani with subsequent hematogenous spread. Endogenous Fusarium endophthalmitis is well recognized in the immunocompromised but is very rarely seen in the immunocompetent. This case highlights the importance of thorough history-taking and consideration of fungal endophthalmitis in the differential diagnosis of a treatment-refractory uveitis.

Relative Frequencies of Arteritic and Nonarteritic Anterior Ischemic Optic Neuropathy in an Arab Population.

BACKGROUND: To evaluate the relative frequencies of arteritic and nonarteritic anterior ischemic optic neuropathy (AION) in an Arab population and to compare and contrast these findings with known epidemiological data from Caucasian populations. METHODS: A retrospective review of the medical records of all patients diagnosed with AION at the King Khaled Eye Specialist Hospital (KKESH) in Riyadh, Saudi Arabia, between 1997 and 2012. RESULTS: Of 171 patients with AION, 4 had biopsy-proven giant-cell arteritis (GCA). The relative frequencies of arteritic anterior ischemic optic neuropathy (AAION) and nonarteritic anterior ischemic optic neuropathy (NAION) in this Arab cohort were 2.3% and 97.7%, respectively. CONCLUSIONS: The relative frequencies of arteritic anterior ischemic optic neuropathy and nonarteritic anterior ischemic optic neuropathy differ between Arab and North American clinic-based populations, with giant-cell arteritis-related ischemia being much less frequent in Saudi Arabia.

Orbital Disease in Neuro-Ophthalmology.

Many abnormalities of the orbit present with neuro-ophthalmic findings, such as impaired ocular motility or alignment, and sensory changes, including optic neuropathy. Comprehensive coverage of all orbital diseases is beyond the scope of this article. This review focuses on diagnosis and management of the most common and the most vision- or life-threatening orbital conditions as well as more recently discovered entities and points of active controversy. These conditions include orbital trauma, vascular disease, inflammatory and infectious diseases, and neoplasms. Common presenting symptoms and associated neuro-orbital diseases also are summarized.

Aspergillus fumigatus Endophthalmitis with Necrotizing Scleritis following Pars Plana Vitrectomy.

We present a case of Aspergillus fumigatus endophthalmitis complicated by necrotizing scleritis in a 68-year-old man with diet-controlled diabetes, after retinal detachment repair. He was initially treated with systemic steroids for surgically induced necrotizing scleritis following routine pars plana vitrectomy. An additional diagnosis of endophthalmitis was made when the patient developed a hypopyon. Repeat vitreous culture isolated Aspergillus fumigatus. Symptoms improved following antifungal treatment leaving the patient with scleromalacia and an advanced postoperative cataract. Fungal scleritis and endophthalmitis are rare complications of intraocular surgery with sight-threatening consequences, and, as this case demonstrates, may even occur concomitantly. The overlapping features of both conditions can make differentiating one from the other difficult. A fungal aetiology should be considered in cases of postoperative scleritis and endophthalmitis that are protracted and refractory to standard therapy. Even in cases of early diagnosis and treatment, visual outcomes in Aspergillus endophthalmitis and scleritis are variable and often disappointing, not infrequently necessitating enucleation of a painful blind eye.

Uveitis secondary to leishmaniasis immune reconstitution syndrome in a HIV-positive patient.

We describe the case of a HIV-positive patient treated for visceral leishmaniasis who developed uveitis as part of a leishmaniasis immune reconstitution syndrome. Visceral leishmaniasis is increasingly found in HIV-positive adults. Its ophthalmic manifestations can range from relatively minor to complicated anterior uveitis, leading to secondary glaucoma and loss of vision. Clinicians caring for people living with HIV should be alert to the complications of leishmaniasis that can occur before and during treatment.