Optic nerve infiltration in systemic non Hodgkin lymphoma.

Optic nerve infiltration secondary to systemic non-Hodgkin lymphoma (NHL) is a rare phenomenon. We present a 55-year-old man with low-grade systemic NHL who initially presented with an isolated optic neuropathy and non-specific neurological symptoms. We further present a literature review of systemic NHL with radiological evidence of optic nerve infiltration. On magnetic resonance imaging, the characteristic features include optic nerve enhancement and enlargement, while leptomeningeal enhancement is uncommon. Cerebrospinal fluid analysis and optic nerve sheath biopsy can return false negative results, and when such investigations are inconclusive, biopsy of the optic nerve substance has a high diagnostic yield. Although rare, lymphomatous optic nerve infiltration must be considered in the differential diagnosis of a pale swollen optic disc.

Clinico-radiological Diagnosis of Optic Nerve Choristomas.

Optic nerve choristomas are very rare lesions. They are characterised by the presence of fat cells, mesodermal collection of fibrous tissue and smooth muscle, and atrophic optic nerve tissue. Although the condition can be diagnosed on histology, it is not always possible to carry out surgical exploration for tissue diagnosis in view of the apical location of the lesion and the inherent risk to vision from surgery. Detailed neuro-imaging becomes vital in these cases for diagnosis. We report a case of a patient with an optic nerve choristoma diagnosed on neuroimaging, review the current literature related to this condition, and discuss the key clinico-radiological features of optic nerve choristomas.

Natural history of primary paediatric optic nerve sheath meningioma: case series and review.

PURPOSE: To study the natural history, clinical and radiological characteristics of primary paediatric optic nerve sheath meningioma (PPONSM). METHODS: Retrospective study of eight paediatric patients who were treated between 1994 and 2016 at the University Hospital Zurich, Switzerland and the Royal Adelaide Hospital, Australia. Clinical records and imaging studies were reviewed. RESULTS: The mean age at presentation was 11 years (range: 6-17 years). There were six female patients and two male patients. 2/8 patients had associated neurofibromatosis type 2. Patients were followed up for 71-297 months (mean 156±70 months). 6/8 patients were observed through the course of their disease and 2/8 patients were treated with radiotherapy. 2/8 patients who were observed had minimal change in vision and did not experience tumour growth after long-term follow-up. CONCLUSIONS: This is the largest PPONSM case series with long-term data on patients treated conservatively. We highlight that a small subset of these tumours are indolent and can be managed using observation alone.

Parinaud syndrome: a 25-year (1991-2016) review of 40 consecutive adult cases.

PURPOSE: To characterize the clinical features, aetiology and management of ophthalmic symptoms in adult patients with Parinaud syndrome. METHODS: This is a retrospective, non-comparative observational case series. We reviewed 40 consecutive charts of adult patients with the clinical diagnosis of Parinaud syndrome at the Royal Adelaide Hospital Department of Ophthalmology in Adelaide, South Australia, between 1991 and 2016. Charts were reviewed for the following: (1) demographic information, (2) clinical presentation, (3) neuro-ophthalmology signs, (4) aetiology of Parinaud syndrome, and (5) management. Examination findings were collected at initial evaluation, throughout the course of follow-up, and at last follow-up. RESULTS: All the cases were assessed by one of the authors (JLC). The commonest presenting symptoms were diplopia (67.5%) and blurred vision (25%) followed by visual field defect (12.5%), ataxia (7.5%) and manifest squint (7.5%). The commonest presenting signs were vertical gaze palsy (100%), convergence-retraction nystagmus (87.5%) and light-near dissociation (65.0%). Only 65.0% patients had the classical triad of vertical gaze palsy, convergence-retraction nystagmus and light-near dissociation. Midbrain pathologies including haemorrhage (30.0%), infarction (20.0%) and tumour (15.0%) were the commonest aetiology. Pineal region tumours accounted for 30.0% of presentations. Symptoms were managed conservatively in 45% of cases with temporary occlusion, prisms or refractive correction, and observation in 42.5% of cases. Surgical intervention for refractory diplopia was required in 12.5% of cases, of which 80% reported symptom resolution following surgery. CONCLUSION: Our series highlights the variable clinical presentation of Parinaud syndrome. The classic triad of conjugate upgaze paralysis, convergence-retraction nystagmus and light-near dissociation was only present in 65% of cases. Pineal neoplasms remain an important aetiological consideration; however, primary midbrain pathology including infarction and haemorrhage constituted the majority of our cases and should be considered in all patients. Conservative management approaches for ocular symptoms are sufficient in most cases although surgical treatment of upgaze palsy can be a useful option in refractory cases.

Teaching Neuro-Ophthalmology in the Asia-Pacific Region and China: A Personal Perspective.

Over the last 30 years, I have been involved in a number of projects helping to educate and train local ophthalmologists in many parts of Asia and the Pacific Islands, which lack adequate training and service in neuro-ophthalmology. In this article, I offer an overview of a number of different teaching initiatives and offer practical suggestions to anyone who might wish to become involved.

Visual Acuity, Quality of Life and Visual Function Outcomes after Cataract Surgery in Bali.

PURPOSE: To assess post-cataract surgery intermediate-term (>6 months) data of visual acuity (VA), surgical complications, refractive outcomes, quality of life (QOL) and visual function (VF) from a non-governmental organization program in Indonesia. METHODS: Retrospective cohort study design. Participants were a selection of patients who underwent cataract surgery by the John Fawcett Foundation between 2006 and 2011, with at least 6 months follow-up. Patients underwent comprehensive ophthalmic examinations. QOL and VF questionnaires were administered. RESULTS: From a total of 1557 invited to attend, 547 patients participated; 99.8% of eyes had presenting VA <6/60. At day 1 postoperatively, 52.1% of patients had a good outcome by World Health Organization criteria (unaided VA ≥ 6/18). Six months or later postoperatively, 85% of eyes had best-corrected VA ≥ 6/18 and 5.6% of eyes had best-corrected VA < 3/60. At final follow-up, mean postoperative spherical equivalent refraction was -2.35 diopters (standard deviation 1.75, n = 542). On a scale of 1-100, median converted values for QOL were 25.0 preoperatively and 74.0 postoperatively, a statistically significant improvement (p < 0.001). Of eyes with VA better than 3/60 on postoperative day 1, 5.7% (95% confidence interval 3.6-7.9%) became blind (VA < 3/60) at the 6-month or later time point. CONCLUSION: Cataract surgery in this population markedly improved QOL, despite a trend towards myopic refractive outcomes. These findings raise questions about biometric methodology, intraocular lens power calculations and refractive targets. Postoperative day 1 VA data may be a reasonable proxy of the intermediate-term rate of non-blind eyes.

Nerve Fiber Layer Infarcts in Thiamine Deficiency.

Thiamine deficiency classically manifests as the triad of Wernicke encephalopathy: acute confusional state, ataxic gait, and ocular motor dysfunction. However, most patients do no present with this classic triad. Optic neuropathy in thiamine deficiency is a rare manifestation and is usually associated with fundus appearances of optic disc swelling or optic disc pallor. We present 2 unique cases of thiamine deficiency where the fundus demonstrated peripapillary retinal nerve fiber layer thickening without florid disc swelling or pallor.

Neuro-ophthalmology of invasive fungal sinusitis: 14 consecutive patients and a review of the literature.

BACKGROUND: Invasive fungal sinusitis is a rare condition that usually occurs in immunocompromised patients and often presents as an orbital apex syndrome. It is frequently misdiagnosed on presentation and is almost always lethal without early treatment. DESIGN: Retrospective case series of 14 consecutive patients with biopsy-proven invasive fungal sinusitis from four tertiary hospitals. PARTICIPANTS: Fourteen patients (10 men and 4 women; age range 46-82 years). METHODS: Retrospective chart review of all patients presenting with invasive fungal sinusitis between 1994 and 2010 at each hospital, with a close analysis of the tempo of the disease to identify any potential window of opportunity for treatment. MAIN OUTCOME MEASURES: Demographic data, background medical history (including predisposing factors), symptoms, signs, radiological findings, histopathological findings, treatment approach and subsequent clinical course were recorded and analysed. RESULTS: Only one patient was correctly diagnosed at presentation. Only two patients were not diabetic or immunocompromised. The tempo was acute in two patients, subacute in nine patients and chronic in three patients. In the subacute and chronic cases, there was about 1 week of opportunity for treatment, from the time there was a complete orbital apex syndrome, and still a chance for saving the patient, to the time there was central nervous system invasion, which was invariably fatal. Only two patients survived - both had orbital exenteration, as well as antifungal drug treatment. CONCLUSIONS: Invasive fungal sinusitis can, rarely, occur in healthy individuals and should be suspected as a possible cause of a progressive orbital apex syndrome.

Case series of cat-scratch-inflicted full-thickness corneal lacerations and a review of the literature.

BACKGROUND: To describe the clinical features and management of cat-scratch-inflicted corneal lacerations. DESIGN: Retrospective, observational case series. PARTICIPANTS: Three patients (aged 3, 7 and 35 years) with cat-scratch-inflicted full-thickness corneal lacerations. METHODS: Retrospective medical chart review and review of the published literature. MAIN OUTCOME MEASURES: Details of clinical presentation, surgical management, antibiotic treatment and clinical outcomes on longitudinal follow-up. RESULTS: Cat-scratch-inflicted corneal lacerations are rare. Only five other cases were found in the literature. Wide spectrum of clinical presentation and severity of injuries exists. Two of the cases here required emergency surgical repair of the laceration; however, one case had spontaneously healed and was only diagnosed 5 years after the initial injury. One case required secondary cataract extraction and subsequent excision of a vascularized posterior lens capsule. There were no cases of secondary microbial keratitis or endophthalmitis. All cases had a favourable ocular outcome after at least 6 months of follow-up. CONCLUSIONS: Cat-scratch-inflicted corneal injuries are rare but do occur in Australia, in particular among younger children. If the principles of prompt surgical repair and antibiotic prophylaxis are adhered to, excellent visual outcomes are possible.

Horner Syndrome: A Practical Approach to Investigation and Management.

Horner syndrome is typically described by the classic triad of blepharoptosis, miosis, and anhydrosis resulting from disruption along the oculosympathetic pathway. Because of the complex and extensive course of this pathway, there are a large number of causes of Horner syndrome ranging from benign to life-threatening diseases. This review article aims to provide a practical approach to investigation and management, including evaluation of the more recent use of apraclonidine for pharmacological testing.

Neuro-ophthalmic manifestations and outcomes of pituitary apoplexy--a life and sight-threatening emergency.

OBJECTIVE: To report the neuro-ophthalmic manifestations and outcomes in patients with pituitary apoplexy. METHOD: Retrospective chart review. RESULTS: 23 patients were identified (17 men, mean age 54.1 years (range 23-86 years). The onset was abrupt in 22 patients; one patient had a subclinical presentation. Headache was the commonest presenting symptom (82.6%, 19/23). Neuro-ophthalmic manifestations were present in more than three-quarters of the patients (82.6%, 19/23). At presentation, 55 % (11/20), 47.6 % (10/21) and 60.9 % (14/23) of the patients had reduced visual acuity, field defects and cranial nerve palsies respectively. Management was conservative in 4 patients and surgical in 18 patients; one patient died shortly after presentation. The median follow up period was 10.5 months (22 patients, range 0.2-168 months). At final follow up, improvement was present in 100% of the patients with reduced acuity (8/8) and ocular palsy (13/13) and 81.8% of patients with field deficits (9/11). Age, sex, presence of precipitating factors and timing of surgery did not have an impact on neuro-ophthalmic recovery. CONCLUSION: Pituitary apoplexy should be considered in any patient with abrupt onset of neuro-ophthalmic deficits. Prompt medical and surgical management is lifesaving and can lead to significant improvement in visual and cranial nerve deficits.

Clinicopathological correlation in pituitary gland metastasis presenting as anterior visual pathway compression.

Pituitary gland metastases, albeit rare, remain an important differential in sellar and suprasellar tumours. Clinical and radiological features of pituitary metastases may be indistinguishable from benign suprasellar lesions such as a pituitary adenoma. Histopathology with immunohistochemical assay remains the key to the diagnosis of pituitary metastasis. We describe four patients with sellar lesions presenting with anterior visual pathway compression initially diagnosed as pituitary adenomas who on immunohistochemistry were found to have metastases to the pituitary. Classification of the cell histology determined the primary site of origin in some patients. This series demonstrates the importance of combining histopathology and immunohistochemistry in the diagnosis of suprasellar lesions.

Sudden loss of vision--history and examination.

BACKGROUND: Sudden loss of vision requires careful history and examination to identify the underlying cause. OBJECTIVE: This article discusses the various causes of sudden loss of vision and provides the general practitioner with a guide to examination. DISCUSSION: Rapidity of onset, duration and associated symptoms provide vital clues to the nature of the disease process. Simple examination techniques such as visual acuity measurement, confrontational visual field testing, pupil assessment and fundoscopy are integral to the appropriate assessment, treatment and referral of patients presenting with sudden loss of vision.

Sudden loss of vision--investigation and management.

BACKGROUND: Sudden vision loss usually requires urgent ophthalmic assessment. Diagnosis and management requires the judicious use of a wide range of serological and imaging investigations to guide appropriate treatment and referral. OBJECTIVE: This article follows on from the previous discussion of the role of history and examination to discuss the appropriate investigation and management of common causes of sudden visual loss. DISCUSSION: The key historical and examination findings have now been extracted and synthesised and these inform the next step. The general practitioner must now decide upon the most appropriate and timely investigation pathway or the need for, and urgency of, referral.