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.

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.

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.

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.

Ocular colobomata, polydactyly, cleft palate and panhypopituitarism: a new syndrome.

We describe a male patient with ocular colobomata, cleft palate, polydactyly, panhypopituitarism and possible craniosynostosis, whom we have followed for 30 years. Although there are some similarities to other documented syndromes, this previously unreported combination of features appears to constitute a new syndrome.

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.

Practical clinical approaches to functional visual loss.

Functional visual loss (FVL) refers to subnormal vision or altered visual fields where no underlying pathology of the visual system can be found. It may be seen in a continuum from frank malingering to hysteria. FVL may first present to the general practitioner or physician and the financial burden of evaluation and potential disability-related claims may be substantial. Diagnosis relies on a high index of suspicion and demonstration with a few simple tests that the patient has better vision than alleged. The aim of this review is to provide a practical approach to examination of patients with suspected functional visual loss. An accurate and early diagnosis of FVL starts with a high index of suspicion. Only a few of the tests need to be learned well, performed smoothly and confidently. These clinical tests obviate the need to perform expensive imaging such as magnetic resonance imaging and if used in the correct setting have the potential to reduce further the cost of diagnosis. Management requires an understanding approach and confrontation is seldom helpful. It is important to stress to the patient that FVL has a good prognosis, thereby providing "a way out" and giving the patient the opportunity to recover.

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.

Recurrent multiple cavernous hemangiomas of the orbit in association with systemic tumors.

PURPOSE: To report the clinical findings and management of multiple, recurrent cavernous hemangiomas of the orbit in a patient with concurrent liver involvement. DESIGN: Interventional case report. METHODS: An analysis of clinical findings, radiology and treatment was conducted with a literature review. RESULTS: A 35-year-old woman with blurry vision and proptosis was found to have multiple cavernous hemangiomas of the orbit. The tumors were excised completely without complication, and the patient subsequently regained her vision. Fifteen years later, the patient had recurrent, multiple cavernous hemangiomas in the same orbit. Further investigations for vague back pain revealed a presumed cavernous hemangioma in the liver and a presumed schwannoma arising from the neural foramen of the spine at the level of T2 through T4. CONCLUSION: Multiple cavernous hemangiomas may recur after complete excision and may exist with concurrent systemic tumors.

Novel technique to control hypersecretion from a transplanted autologous submandibular salivary gland for keratoconjunctivitis sicca.

PURPOSE: To present a novel technique to control hypersecretion from a transplanted autologous submandibular gland (SMG) in a patient with keratoconjunctivitis sicca. METHODS: A 65-year-old man presented with corneal epithelial edema and suspicious ocular surface dysplasia secondary to hypersecretion from a transplanted autologous SMG. The location and function of the gland were evaluated perioperatively using technetium-99m-pertechnetate scintigraphy. The course of the duct was marked with a radiation probe, surgically exposed, and partially ligated with titanium clips. RESULTS: Marked reduction in salivary flow and resolution of corneal edema and ocular surface changes were noted. Conjunctival biopsy showed no evidence of malignancy. Symptoms were stable during a 1-year follow-up period. CONCLUSION: Partial ligation of the transplanted SMG duct may be a simple and reversible technique to control hypersecreting glands with secondary corneal edema and ocular surface changes.

Polymyalgia rheumatica and giant cell arteritis--An ophthalmic emergency.

BACKGROUND: The occurrence of giant cell arteritis (GCA) in the setting of polymyalgia rheumatica (PMR) is not uncommon. It is imperative to recognise the symptoms and signs of GCA in this setting as the treatment of PMR with low dose corticosteroids will not protect the patient against the blinding consequences of GCA. OBJECTIVE: This article reports the case of a woman with PMR who developed sudden and irreversible vision loss due to GCA. DISCUSSION: It is important to recognise GCA in patients with PMR before the onset of permanent visual disability. A rising erythrocyte sedimentation rate in such patients may herald the onset of GCA. Other risk factors for GCA include age over 50 years, female gender, symptoms of ischaemia, and temporal artery abnormalities on examination. The latter two features warrant urgent ophthalmic or rheumatological review.